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Successful application of the Norwood procedure for infants without hypoplastic left heart syndrome
Abstract
Although the first-stage Norwood procedure mostly has been used for hypoplastic left heart syndrome, there are other anomalies in which the Norwood procedure can be applied. Since 1991, 18 newborns without hypoplastic left heart syndrome underwent a first-stage Norwood procedure. All had a hypoplastic aortic annulus, ascending aorta, and transverse aorta. Ten had normally related great arteries: aortic atresia or aortic stenosis with inadequate left ventricle in 4, mitral atresia or stenosis in 4, and interrupted aortic arch in 2. The 8 others had double-outlet right ventricle with mitral atresia or complete transposition with a hypoplastic right ventricle. Age ranged from 2 to 77 days (median, 6 days) and weight from 2.4 to 4.4 kg (mean, 3.0 kg). The patients with interrupted aortic arch simultaneously underwent primary repair of the interruption. There were 17 hospital survivors (94%). There have been no late deaths in follow-up from 4 to 30 months (mean, 15.5 months). Thirteen children have had subsequent creation of a bidirectional Glenn shunt with takedown of the original systemic to pulmonary shunt. The 2 with interrupted aortic arch underwent a Rastelli-type biventricular repair. These results show that the Norwood procedure can be applied to infants without hypoplastic left heart syndrome who have hypoplastic aortas and excessive pulmonary blood flow with very low mortality and excellent palliation.
... Some other malformations may present with the same physiology as HLHS, such as double-inlet left ventricle (DILV), tricuspid atresia with transposition, unbalanced atrioventricular septal defect (AVSD), and some complex forms of double-outlet right ventricle (DORV) with left ventricular outflow tract obstruction (LVOTO) [1,5,6,[9][10][11]13]. An association often exists between hypoplasia of the aortic arch and coarctation or interrupted aortic arch. ...
... In agreement with our results, few other studies failed to find a significant difference in operative survival between patients with other forms of single-ventricle physiology and patients with HLHS undergoing the Norwood procedure [5,9,11]. Kanter et al. [9] also applied the Norwood concept successfully for patients with variants of HLHS and showed that a heterogeneous group of neonates with aortic obstruction and unrestrictive pulmonary blood flow without classic HLHS can undergo successful Norwood palliation with low morbidity and mortality. ...
... In agreement with our results, few other studies failed to find a significant difference in operative survival between patients with other forms of single-ventricle physiology and patients with HLHS undergoing the Norwood procedure [5,9,11]. Kanter et al. [9] also applied the Norwood concept successfully for patients with variants of HLHS and showed that a heterogeneous group of neonates with aortic obstruction and unrestrictive pulmonary blood flow without classic HLHS can undergo successful Norwood palliation with low morbidity and mortality. Mosca et al. [11] reported an 89 % operative survival rate with the Norwood procedure performed for tricuspid atresia or double-inlet right ventricle and TGA with arch anomalies. ...
Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p
... 2 Surgical solutions to this unique patient population included-single stage palliation with the Norwood operation or biventricular repair with a Ross/ Konno/Arch repair. 3 The former commits a patient to a shunted-single ventricle physiology when he or she has 2 adequate ventricles, whereas the later has a high early mortality rate (33%). 4 The Yasui operation was developed to address this patient population by creating a Damus-Kaye-Stansel connection between the atretic ascending aorta and the pulmonary trunk then baffling the left ventricular outflow through the VSD to the systemic circulation. ...
Objective
The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation.
Methods
We retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR).
Results
Twenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention.
Conclusions
The Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.
... 3 It can be done as one-stage versus two-stage repair that includes initial modified Norwood procedure followed by Rastelli-type repair. [4][5][6][7][8][9] Unlike the single ventricle pathway, Yasui procedure has the advantage of biventricular repair that results in normal serial pulmonary systemic circulation (Qp:Qs = 1:1) with normal saturated blood being ejected in the systemic circulation. 2 The overall results of Yasui procedure have been variable with reported operative mortality of 6.7%, 2 and actuarial 10-year survival ranging between 78% 8 and 87.8%. 3 The aim of our study is to describe our experience with Yasui procedure in the current era comparing the overall outcome between patients who had one-stage versus two-stage repair, the need for reintervention and associated major morbidity and mortality. ...
Background
Yasui procedure is surgical repair intended to preserve biventricular function for patients with left ventricle outflow tract obstruction associated with aortic arch lesions and ventricular septal defect.
Methods
Retrospective chart review analysis of all patients who had Yasui procedure (2008–2020) comparing midterm outcome of one versus two stage repair.
Results
Twenty patients (70% female) underwent Yasui procedure in our center. Eight patients (40%) had left ventricle outflow tract obstruction /interrupted aortic arch, 7 patients (35%) had left ventricle outflow tract obstruction /coarctation of aorta, 3 patients (15%) had double outlet ventricle and ventricular septal defect that were unattainable for tunneling to one of the semilunar valves without creating obstruction, and 2 patients (10%) had aortic atresia with hypoplastic aortic arch. All patients had associated ventricular septal defect. Fifteen patients (75%) had one-stage repair and 5 patients (25%) had two-stage repair. Means age and weight for one and two-stage repair were 1.3 ± 2 months, 13.4 ± 11.5 months and 3.3 ± 0.6 kg, 7.8 ± 3.4 kg, respectively. During follow up, 8 patients (40%) required re- intervention, mainly for right ventricle–pulmonary artery conduit either dilation or replacement. The average duration of follow up was 5 years with nil mortality.
Conclusion
Yasui procedure is effective approach for children who have left ventricle outflow tract obstruction associated with aortic arch anomalies and ventricular septal defect. Survival rate with single or staged repair is comparably good. During the first 5 year of follow up, nearly 40% of operated patients required re-intervention.
... Transcatheter interventions (balloon septostomy, balloon dilation) are recommended for a recurrent r-FO, but they not universally effective. [408,[453][454][455][456] (class IIa, level C). However, the diameter of a PTFE shunt is the primary determinant of its resistance. ...
The IPCCC is a standardized international system of paediatric and congenital cardiac nomenclature that should be used in all registries, databases and research studies in the domain of paediatric and congenital cardiac care, including those related to HLHS. This recommendation is level 1 class C [15, 16, 700]. Multi-institutional databases and registries allow benchmarking of data concerning death, patterns of practice, morbidity and postoperative length of stay [540]. As a level 1 class C recommendation, all paediatric and congenital cardiac teams should routinely assess their own data against national and international benchmarks using multi-institutional databases and registries [540, 697–699, 701]. © 2020 European Association for Cardio-Thoracic Surgery. All rights reserved.
... Although it is not definitely proven in humans, myocyte mitotic activity and the potential for hyperplasia allegedly persist for 3 to 6 months after birth (8). According to the clinical experience in a previous study, in a patient with a normal mitral valve who underwent a Norwood-type operation as a first-stage palliation because of subaortic obstruction, biventricular repair using a Rastelli-type repair was successfully performed as a definitive repair (9). We think that early improvement of forward flow through the mitral valve might be effective for growth of a hypoplastic left ventricle. ...
The strategy for an infant with congenital mitral stenosis should be determined by three important factors: left ventricular volume, the degree of the systemic outflow tract obstruction, and the type of mitral valve dysfunction. A successful staged biventricular repair in early infancy for a patient who had congenital mitral stenosis with short chordae, hypoplastic left ventricle and coarctation of the aorta, and the long-term results are described. There were the following important hemodynamic factors that led to the successful biventricular repair in the patient. Total systemic output was barely supplied through the hypoplastic left ventricle after closure of the ductus arteriosus on admission. The neonate underwent repair of coarctation of the aorta alone as the initial stage at 9 days after birth. Also, spontaneous closure of the foramen ovale following repair of coarctation of the aorta accelerated the progressive left ventricular growth. Open mitral commissurotomy with an interatrial fenestration using the modified Brawley's approach was performed for a 40-day-old infant. Good left ventricular growth and good mitral valve function have been observed for 18 years after open mitral commissurotomy. Appropriate early augmentation of left ventricular inflow through the mitral valve might be effective for growth of a hypoplastic left ventricle. J. Med. Invest. 64: 187-191, February, 2017
... Kanter et al. [7] reported that the Norwood operation allows for adequate relief of aortic obstruction, protection of the pulmonary vasculature, and preservation of myocardial function, which hopefully will facilitate later corrective operations. Similarly, Jacobs et al. [6] reported that the Norwood operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circulation in anticipation of either the Fontan operation or a biventricular repair [6]. ...
At our institution, the strategy for patients with bicuspid aortic valve, aortic valve stenosis (CoA), or interrupted aortic arch (IAA)] with ventricular septal defects (VSDs) as well as normal left ventricular (LV) volume and mitral valve size consists of two parts. The Norwood operation is applied as the first palliation for this group of patients. Second, the decision whether the patients are to undergo the Rastelli operation or a univentricular repair is made depending on the size of the right ventricle after the Norwood operation. This study aimed to examine whether the aforementioned surgical strategy for this group of patients is adequate or not. Seven patients undergoing the Norwood operation as the first palliation for bicuspid aortic valve, aortic valve stenosis (CoA with VSD, and three patients had IAA with VSD. Six patients underwent biventricular repair, and one patient had univentricular repair due to the small RVEDVI (74 % of normal). The patients with 80–90 % of normal RVEDVI had higher BNP and higher CVP than those with more than 90 % of normal RVEDVI after the Rastelli operation, whereas the patient undergoing the Fontan operation had a low BNP level. In conclusion, the described strategy for patients with severe aortic hypoplasia and aortic stenosis with VSD as well as normal LV and mitral valve size is reasonable.
... The trial's rejection of neonates as subjects, because they lack legal capacity to consent, contrasts starkly with the accepted practice of performing phase I studies on infants and children for conditions that do not present in adults. Examples of such interventions include surfactant for respiratory distress syndrome, 60 novel cardiac surgery techniques for congenital heart disease, 61 drug treatment and dietary restriction for metabolic disorders, 62,63 techniques for treating and preventing neonatal brain injury, [64][65][66] and treatment of extremely low birth weight. 67 The OTC disease phenotype, and indeed, many other disease phenotypes, presents differently at different ages, meaning gene therapy is not necessarily the most appropriate treatment option for older patients with milder phenotypes that can be managed acceptably by less invasive means. ...
Risk is an inescapable aspect of clinical research and is increasingly pertinent to the gene therapy field as the imperative for clinical trial activity grows. In recent years, the widely reported occurrence of serious adverse events (SAEs) in gene therapy studies, including trials for ornithine transcarbamylase (OTC) deficiency, X-linked severe combined immunodeficiency (SCID-X1), and rheumatoid arthritis, has heightened fear in public perceptions of gene therapy. Although it is essential to be cognizant of the risks involved in gene therapy research, there is a danger that gene therapy may become too risk-averse. If the field is to make progress, it is necessary to understand how risk is defined in gene therapy research, how understandings of risk differ, how risk is assessed, how decisions about risk are made, and how gene therapy risks are communicated to subjects and research participants during the informed consent process. In addition to minimizing the risks of clinical research through extensive preclinical safety studies, attention should be given to how decisions about risk and risk acceptability are made by researchers and subjects, and to the methods used to communicate risks to patients. Critical attention to risk will help ensure that the safety of subjects is protected, while also enabling research to develop better treatments for patients.
... Jacobs et al. have reported the experience with the Norwood operation for lesions other than hypoplastic left heart syndrome and revealed an overall mortality of 28%, with 13 of 18 survivors all deemed to be good Fontans [20]. Others have reported a similar success with the use of the Norwood procedure as a ®rst stage palliation with an equivalent rate of progression into the subsequent Fontan pathway [21]. Ilbawi et al. have also been advocates of a primary pulmonary-to-systemic anastomosis in patients with SV with AAO because of the consistent appearance of severe SAS [22]. ...
The outcomes of initial pulmonary artery banding (PAB)+/-coarctation repair are compared with the Norwood operation in newborns with single ventricle (SV) and systemic obstruction (SO).
Between January 1987 and July 2000, 22 patients (median age, 12 days) with SV and aortic arch obstruction (AAO), subaortic stenosis (SAS), or both underwent surgery. Two initial surgical approaches were used: PAB+/-coarctation repair (group I, seven patients); Norwood type operation (group II, 15 patients).
The overall mortality was 32% (seven of 22 patients). There was no late mortality. The mortality in group I was 43% versus 27% in group II. Recently, there has been no mortality following the Norwood operation in the last eight patients operated since 1995. Of the survivors, nine patients have undergone the Fontan operation and four patients have had the bidirectional Glenn (BDG) with no deaths. There was one repair of supravalvar aortic stenosis at the time of BDG in group II as opposed to eight reinterventions for SAS and/or AAO in four patients in group I (P=0.01).
PAB+/-coarctation repair for SV and SO is associated with a high mortality and a high reoperation rate for SAS or recurrent AAO. Although the Norwood operation was also associated with a high mortality early on, it can now be performed with excellent outcome. This improvement, combined with a low reintervention rate for SAS or AAO, suggests that the Norwood operation is likely to emerge as the procedure of choice for SV and SO.
... Several previous reports [9 -14] have advocated the use of inspired CO 2 after a Norwood procedure. When details of the delivery strategy were provided, the policy usually involved inspired CO 2 with increased ventilation [9, 10, 12, 14], although in one report [13], inspired CO 2 was used to achieve a PaCO 2 of 45 to 55 mm Hg. These reports suggested that inspired CO 2 improves outcome. ...
Mortality in the early postoperative period after the Norwood procedure remains substantial. Inspired carbon dioxide (CO2) has been suggested to improve hemodynamic status in this setting. Inspired CO2 can be delivered by one of two strategies, ie, with or without an accompanying increase in minute ventilation. The hemodynamic effects of these two strategies have not previously been studied in a controlled fashion.
Seventeen infants (median age, 9 days; range, 4 to 49 days) undergoing Norwood procedures were prospectively enrolled in this crossover study. Patients were studied while sedated, paralyzed, and mechanically ventilated 1 day to 6 days after operation. The inspired oxygen fraction was kept constant (mean value, 0.24 +/- 0.01). Measurements were made at five time points: 1 = baseline; 2 = inspired CO2 with increased ventilation; 3 = baseline; 4 = inspired CO2 alone; and 5 = baseline. Mixed venous oxygen saturation was monitored using indwelling lines in the superior vena cava.
Inspired CO2 with increased ventilation produced a rise in mean airway pressure with no change in arterial CO2 tension or pH. This strategy had no effect on hemodynamic status or oxygen delivery. Inspired CO2 alone produced a rise in arterial CO2 tension and a fall in arterial pH (respiratory acidosis). This strategy resulted in significant improvement in both variables of systemic oxygen delivery: mixed venous oxygen saturation increased from 48% +/- 2% to 56% +/- 2% (p < 0.05), and arteriovenous oxygen saturation difference decreased from 3% +/- 2% to 26% +/- 2% (p < 0.05).
Inspired CO2 after the Norwood procedure can improve oxygen delivery. This improvement occurs only if minute ventilation is kept constant. There is no improvement if minute ventilation is increased. Clinical use of inspired CO2 may be limited by the accompanying fall in pH. Differentiation of cerebral from total-body effects of inspired CO2 will require further study.
... By avoiding subaortic stenosis, rather than treating it once it has occurred, this approach has the potential of avoiding ventricular hypertrophy, thereby improving suitability for a Fontan procedure [9,10] 1980s and early 1990s [1, 14, 19, 20]. More recently, operative mortalities below 20% have been reported for Norwood procedures carried out for defects other than hypoplastic left heart syndrome [22][23][24][25]. Mosca and associates reported the use of a modified Norwood procedure in 38 patients with a single left ventricle and ventriculoarterial discordance [12]. ...
Background:
The infant with a single ventricle and excessive pulmonary blood flow requires early protection of the pulmonary vascular bed to insure suitability for a subsequent Fontan procedure. The traditional approach, pulmonary artery banding, has had disappointing results. We have pursued an alternate strategy: division of the pulmonary artery, and placement of a systemic-to-pulmonary artery shunt. Potential sites of systemic outflow tract obstruction are simultaneously bypassed, by either a Damus-Kaye-Stansel, or modified Norwood procedure.
Methods:
From January 1996 to June 2001, 22 infants were treated by this strategy. Patients with hypoplastic left heart syndrome were excluded. Median age was 18 days (range 2 days to 6 months). In addition to pulmonary artery division and shunt, 3 of 22 patients underwent a Damus-Kaye-Stansel procedure, and 13 of 22 patients underwent a modified Norwood procedure.
Results:
There were no operative deaths, and one late death. Actuarial survival beyond 30 months was 90%. At follow-up catheterization in 22 patients, median transpulmonary gradient was 7 mmHg (range 4 to 18), and median pulmonary vascular resistance 1.9 Wood units (range 0.9 to 3.3). Twenty-one patients have undergone a subsequent bidirectional superior cavopulmonary connection, and 6 a Fontan procedure, with no deaths. No patient developed subaortic stenosis, or aortic arch obstruction. Neoaortic insufficiency was none or trivial in 12 patients, mild in 3, and moderate in 1.
Conclusions:
In patients with a functional single ventricle and excessive pulmonary flow, a strategy of pulmonary artery division and shunt, along with prophylactic bypass of systemic outflow obstruction, carries low operative and midterm mortality. It provides consistent protection of the pulmonary vascular bed, avoids subaortic stenosis and aortic arch obstruction, minimizes neoaortic insufficiency, and ensures suitability for progression along a Fontan pathway. These results provide a comparison for alternate strategies, including pulmonary artery banding.
... As discussed above, a main argument against complete neonatal repair relates to the extensive myocardial ischemic time and bypass time that the neonatal immature myocardium would undergo [10,11,15]. The addition of a ventriculotomy may further comprise ventricular function in this group of patients. ...
Background:
In general, neonates with severe left ventricular outflow tract obstruction, aortic valvar stenosis or atresia, and arch hypoplasia with either interruption or coarctation, and a small left ventricle undergo Norwood palliation followed classically by a bidirectional cavopulmonary shunt and eventual modified Fontan. However, a subset of patients, usually neonates with a ventricular septal defect, may have adequate left ventricle and mitral valve sizes making them candidates for future biventricular repair (BVR). In view of the long-term advantage of BVR, the feasibility and outcome of this approach was studied. Additionally, echocardiographic data were reviewed in an attempt to develop objective prognostic criteria for selection of patients suitable for BVR.
Methods:
During a 4-year period, 8 of 58 infants undergoing Norwood palliation were identified as potential two-ventricle candidates. Their mean age was 6 days. Diagnoses included aortic atresia (n = 1), or aortic valve stenosis and subaortic stenosis (n = 7), with an interrupted aortic arch in 3 and coarctation in 4. All patients had a ventricular septal defect and a left ventricle that was considered to be apex forming. Mean mitral valve size was 11 mm (z-score = -1.7). Mean aortic valve size was 4.1 mm (mean z-score = -8.4).
Results:
All 8 patients survived Norwood palliation. Six subsequently underwent BVR with ventricular septal defect closure and a right ventricle to pulmonary artery conduit at a mean age of 7 months. One patient is awaiting repair, and 1 underwent a cavopulmonary shunt. At the time of BVR, mean mitral valve z-score was essentially unchanged at -1.4 (14 mm). No early deaths or late deaths occurred during a mean follow-up of 32 months.
Conclusions:
A small subset of patients requiring Norwood palliation as newborns may be candidates for eventual BVR with low risk. In general, patients suitable for BVR have a mitral valve z-score of more than -3 and a normal-sized left ventricle. Recognition of neonatal BVR candidates enables consideration of complete neonatal repair. However, single-stage repair needs to be compared with the excellent results obtainable with the staged approach.
... The more recent application of the Norwood operation for lesions such as tricuspid atresia with discordant ventriculo-arterial connections, or hearts with two well-developed ventricles in which biventricular repair is either difficult or impossible, such as some forms of double outlet right ventricle with non-committed ventricular septal defect, hearts with straddling atrioventricular valves, and severely unbalanced atrioventricular septal defects, has shown that use of the term "Norwood operation" no longer implies the diagnosis of hypoplastic left heart syndrome. [19][20][21] The diagnosis of the syndrome, therefore, should not be defined by the use of the Norwood operation to repair it. ...
The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia. Although the majority of the patients are suitable only for functionally univentricular repair, a small minority may be candidates for biventricular repair. The nature of the syndrome was a topic for discussion at the second meeting of the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, the Nomenclature Working Group, held in Montreal, Canada, over the period January 17 through 19, 2003. Subsequent to these discussions, the Nomenclature Working Group was able to create a bidirectional crossmap between the nomenclature initially produced jointly on behalf of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons, and the alternative nomenclature developed on behalf of the Association for European Paediatric Cardiology. This process is a part of the overall efforts of the Nomenclature Working Group to create a comprehensive and all-inclusive international system of nomenclature for paediatric and congenital cardiac disease, the International Paediatric and Congenital Cardiac Code. In this review, we discuss the evolution of nomenclature and surgical treatment for the spectrum of lesions making up the hypoplastic left heart syndrome and its related malformations. We also present the crossmap of the associated terms for diagnoses and procedures, as recently completed by the Nomenclature Working Group.
... Although UVR largely neu-tralizes the inadequacy of the left ventricle, others have demonstrated improved survival after Norwood palliation in situations in which the left heart is not hypoplastic. 8,9 Larger indexed length of the dominant ventricle was associated with improved survival in UVR. Because the dominant ventricle was almost exclusively the right ventricle, this variable may reflect the functional adequacy of the right ventricle in assuming the systemic role. ...
In critical left ventricular outflow tract obstruction, the common perception that biventricular physiology is superior to univentricular physiology has led to a bias favoring biventricular repair. We hypothesized that pursuit of biventricular repair in borderline candidates increases mortality.
Between 1994 and 2001, 362 neonates with critical left ventricular outflow tract obstruction were prospectively enrolled by 26 institutions. Initial procedure indicated intended univentricular repair (n = 223; 84 deaths) or biventricular repair (n = 139; 39 deaths). Parametric risk-hazard analysis identified predictors of death for univentricular and for biventricular repair, which allowed prediction of the 5-year univentricular survival advantage for every infant. Survival was scrutinized for children managed discordantly to univentricular survival advantage predictions.
Incremental factors for death after univentricular repair were as follows: tricuspid regurgitation, smaller mitral annulus z-score, smaller indexed dominant ventricular length, and presence of a large ventricular septal defect; risk factors after biventricular repair were as follows: minimum left ventricular outflow tract diameter, endocardial fibroelastosis, left ventricular dysfunction, and smaller mid-aortic arch. These variables formed the univentricular survival advantage tool (all P < .0001, R(2) = 0.92). Discordant management was more common with biventricular than with univentricular repair (56% vs 21%; P < .01). Discordant pursuit of biventricular repair was associated with significantly more observed versus expected deaths (biventricular repair 30 vs 14; P < .001; univentricular repair 20 vs 13; P = .02). Survival after biventricular repair is sensitive to changes in univentricular survival advantage values, especially in borderline candidates. In contrast, univentricular repair survival is insensitive to changes in univentricular survival advantage values.
Inappropriate pursuit of biventricular repair in borderline candidates is more frequent and more consequential in survival terms than is inappropriate pursuit of univentricular repair. Use of the univentricular survival advantage tool will help identify infants for whom univentricular repair may be a better choice than attempting biventricular repair.
In the 40 years since inception, use of the Norwood procedure has expanded to other complex single ventricle pathologies, with successive technical modifications resulting in steadily improving results. This chapter discusses the stage one of the Norwood procedure with modifications to the aortic arch repair and shunt.
Objectives
The aim of this study is to present our experience with three patients who underwent a neonatal Yasui procedure, and to show the new decision-making algorithm.
Methods
This is a series of three neonates operated on at our hospital between 2017 and 2022.
Results
All the patients underwent a primary Yasui. The duration of cardiopulmonary bypass was 275, 249 and 391 min, in patient 1, 2 and 3, respectively. After surgery, the Intensive Care Unit stay of patients 1, 2 and 3 was 29, 22 and 24 days, respectively. The patients were discharged in good condition. Subsequent complications during follow-up included the need for percutaneous intervention in patient 1 for the implantation of a stent in the right pulmonary branch (at 6 months postoperatively) and a stent in the right ventricle-pulmonary artery conduit (at 42 months). Patient 2 required right ventricle-pulmonary artery conduit replacement and repair of moderate-severe left ventricular outflow tract obstruction at 16 months postoperatively. Patient 3 needed a reoperation at 3 months postoperatively due to aortic arch stenosis at different levels and a residual ventricular septal defect. Currently, all patients are alive with adequate echocardiographic biventricular function.
Conclusions
In experienced centers, primary Yasui repair can be performed in the neonatal period with satisfactory results.
Background:
The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution.
Methods:
Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29). Outcomes were parametrically modeled, and risk factors associated with early and late death were analyzed.
Results:
Competing-risks analysis showed that, at 2 years after the operation, 24% of patients had died or received transplantation and 75% had undergone a Glenn shunt. At 5 years after the Glenn shunt, 10% of patients had died or received transplantation, 62% had undergone Fontan, and 28% were alive awaiting Fontan. Overall 8-year survival was 70%. Outcomes after Norwood included extracorporeal membrane oxygenation use in 9 (14%), unplanned reoperation in 13 (20%), hospital death in 10 (15%), and interstage death in 8 (12%), with 8-year survival of 66%. Outcomes after PAB+COA included extracorporeal membrane oxygenation use in 1 (3%), unplanned reoperation in 9 (30%), hospital death in 1 (3%), and interstage death in 3 (10%), with 8-year survival of 76%. There was an association trend between underlying anatomy and survival (hazard ratio [HR], 2.1; 95% confidence interval [CI], 0.9 to 4.7; p = 0.087). On multivariable analysis, factors associated with death were extracorporeal membrane oxygenation use (HR, 5.5; 95% CI, 1.9 to 15.9; p = 0.002), genetic syndromes/extracardiac anomalies (HR, 3.5; 95% CI, 1.5 to 8.2; p = 0.003), and weight of 2.5 kg or less (HR, 3.0; 95% CI, 1.3 to 7.2; p = 0.012).
Conclusions:
Anatomic and patient characteristics influence palliation outcomes in neonates born with single-ventricle anomalies associated with aortic arch obstruction. Although the Norwood operation is applicable in most of these patients, the PAB+COA strategy is a valid alternative in well-selected patients.
Tricuspid atresia (TA) is a heterogeneous single-ventricle anomaly in which initial presentation and, consequently, timing and mode of palliation vary based on morphology and degree of pulmonary or systemic outflow obstruction. We report current era palliation outcomes and examine whether morphologic and, subsequently, surgical factors influence survival.
From 2002 to 2012, 105 infants with TA underwent surgical palliation. Competing risks analyses modeled events after first-stage surgery (Glenn versus death) and after Glenn (Fontan versus death) and examined risk factors affecting outcomes.
Seventy-eight patients (74%) required neonatal first-stage palliation, including modified Blalock-Taussig shunt (n = 46, 44%), Norwood (n = 18, 17%), and pulmonary artery band (n = 14, 13%), whereas 27 (26%) received primary Glenn as their initial surgery. Hospital mortality was 5 patients (4.8%). Competing risks models showed that by 1 year after first-stage surgery, 15% of patients had died and 83% had undergone Glenn. By 5 years after Glenn, 2% of patients had died and 80% had undergone Fontan. Overall 8-year survival was 84%. On multivariable analysis, risk factors for mortality were genetic/extracardiac anomalies (hazard ratio 7.0, 95% confidence interval: 2.4 to 20.6, p < 0.001) and pulmonary atresia (hazard ratio 4.4, 95% confidence interval: 1.6 to 12.2, p = 0.004). Survival was not affected by initial palliation type (p = 0.36), ventriculoarterial discordance (p = 0.25), systemic outflow obstruction (p = 0.84), or arch obstruction (p = 0.62).
Despite morphologic and physiologic variations necessitating different palliative sequences, multistage palliation outcomes of various TA subtypes are comparable and generally good, with the exception of patients with associated genetic/extracardiac anomalies. The bulk of mortality is interstage, indicating continued opportunity for improvement in monitoring and managing patients during this critical period.
Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
Subsequent to increased experience with the Norwood operation in children with hypoplastic left heart syndrome (HLHS), its application has expanded to allow palliation of single-ventricle (SV) malformations other than HLHS. We describe current palliation outcomes in this group of SV patients.
Between 2002 and 2012, 65 of the 303 Norwood operations were performed in non-HLHS SV patients. Competing risk analysis modeled events after Norwood and after subsequent Glenn and examined risk factors affecting outcomes.
Competing risk analysis showed that one year following Norwood, 24% of patients had died or received transplantation, 72% had undergone Glenn, and 4% were alive awaiting Glenn/Kawashima. Five years following Glenn, 9% of patients had died, 68% had undergone Fontan, and 23% were alive awaiting Fontan. Overall seven-year survival following Norwood was 68%. On multivariable analysis, mortality risk factors were unplanned cardiac reoperation (hazard ratio [HR]: 4.0 [1.5-10.6], P = .006), right dominant ventricle morphology (HR: 3.3 [1.3-8.3], P = .012), and postoperative extracorporeal membrane oxygenation (HR: 3.1 [1.1-9.0], P = .035).
Operative death and interstage mortality continue to be problematic following Norwood palliation for non-HLHS SV variants. Outcomes seem comparable to those reported for HLHS, however they are influenced by underlying pathology; children with dominant left ventricle morphology (tricuspid atresia and double inlet left ventricle) have superior survival compared to those with dominant right ventricle morphology (mitral atresia, unbalanced atrioventricular septal defect, and most patients with atrial isomerism). Unplanned reoperations for technical imperfections diminish survival. Large multicenter studies might be warranted to better identify high-risk patients and provide guidance toward improving their survival.
© The Author(s) 2014.
Linksobstruktionen können entsprechend der sequentiellen Analyse auf der Ebene der Lungenvenen, des Vorhofseptums, der Mitralklappe,
der Ausflussbahn des linken Ventrikels, der Aortenklappe, der aszendierenden Aorta und des Aortenbogens nachweisbar sein.
Als mögliches Substrat für das Zurückbleiben der Entwicklung der linken Herzhälfte und damit der Ausbildung dieser Obstruktionen
bis hin zum „ypoplastischen Linksherzsyndrom“ (HLHS; ▸ Kap. 14.5) werden intrauterine Flussbehinderungen auf Vorhofebene,
z. B. ein restriktives oder verschlossenes Foramen ovale, angesehen mit der Folge eines reduzierten Flusses zum linken Herzen.
Da dabei jede proximale Stenose auch zu einer distalen Hypoplasie oder Stenose führen kann, bestehen häufig gleichzeitig mehrere
Obstruktionen (Hess 2002).
Patients with double inlet left ventricle (DILV)/transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. The aim of this study is to evaluate the outcomes of initial pulmonary artery banding (PAB) compared with the Norwood-type reconstruction in neonates.
A retrospective study of children with DILV and similar morphologies presenting between 1982 and 2012. The outcomes of initial PAB (n = 17) are compared with a Norwood-type reconstruction (n = 20). The two groups were similar with respect to age, gender, weight, noncardiac syndromes, and Fontan completion. Mean follow-up was longer in PAB patients (13.3 ± 9.8 years) versus Norwood (4.5 ± 3.0 years, p = 0.001).
Survival was 75% at eight years in the Norwood group versus 71% in the PAB group (p = 0.76). Mortality in the Norwood group was higher before 2002 (p = 0.06). The age of patients who underwent a bidirectional Glenn shunt was significantly higher for PAB group (PAB, 9.1 ± 1.4 months vs. Norwood, 6.1 ± 1.6 months; p < 0.001). Freedom from any type of reintervention (systemic outflow obstruction or coarctation) was similar (Norwood, 83% vs. PAB, 71%; p = 0.62). Freedom from heart block with a pacemaker insertion was significantly better for Norwood patients (Norwood, 89% vs. PAB, 41%; p = 0.001).
The Norwood-type reconstruction provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining an unobstructed systemic outflow tract and avoiding the risk of heart block. Survival does not differ depending on the type of procedure. Patients with PAB show comparable satisfactory early and long-term results, with an increasing reoperation risk and heart block remaining a major concern.
Background: Norwood procedure is used as the first stage in the palliative treatment of the hypoplastic heart syndrome and can be used, with some technical modifications, in other forms of univentricular heart with aortic stenosis or hypoplasia. These patients have a high mortality (50%), derived from the procedure itself and from their abnormal physiological status. Aim: To report our experience with the Norwood procedure. Patients and methods: Retrospective analysis of all patients subjected to the Norwood procedure between February 2000 and June 2003. Results: Thirteen patients (9 females, age range 5-60 days and median weight of 3.3 kg) were operated. Eight had hypoplastic heart syndrome and five had a single ventricle with aortic arch hypoplasia. The diagnosis was done in utero in eight patients. All technical variations, according to the disposition and anatomy of the great vessels, are described. Cardiac arrest with profound hypothermia was used in all and regional cerebral perfusion was used in nine. Three patients died in the perioperative period and three died in the follow up (two, four and 10 months after the procedure). Gleen and Fontan procedures were completed in five and one patients, respectively. Conclusions: Our results with the Norwood procedure are similar to other series. There is an important mortality in the immediate operative period and prior to the Glenn procedure (Rev Méd Chile 2004; 132: 556-63).
The Yasui procedure is employed in neonates with interrupted aortic arch and left ventricular outflow tract obstruction (IAA/LVOTO) or aortic atresia-severe stenosis with ventricular septal defect (AA/VSD) and 2 adequate-sized ventricles. This combines a Norwood arch reconstruction with a Rastelli operation establishing a biventricular repair.
From 2002 to 2011, 21 neonates aged 3 to 55 days (mean 12.2 days, median 7 days) had IAA/LVOTO (n=13), AA/VSD (n=7), or AA/IAA with aortopulmonary window (n=1); ten (48%) had genetic abnormalities (8 with DiGeorge syndrome). Based on clinical characteristics and surgeon preference, 6 had a primary Yasui repair (4 AA/VSD, 2 IAA/LVOTO); 15 were staged with an initial Norwood repair (3 AA/VSD, 12 IAA) followed by Yasui completion in 13 (2 await completion) 4.3 to 26.6 months later (median 6.9 months).
Early mortality was zero with no interstage deaths in the staged patients. One patient died 2 months after staged repair. Since biventricular repair, 8 survivors (44%) had reoperation for conduit replacement (n=6), recurrent LVOTO (n=1), or a residual VSD (n=1). No patient requires a pacemaker. There were 3 late deaths after biventricular repair, all in patients with genetic syndromes and IAA/LVOTO. Actuarial survival after initial operation was 100% at 1 year and 75% at 5 years. Actuarial freedom from reoperation or death after biventricular repair was 14% at 5 years.
The Yasui operation is effective for patients with IAA/LVOTO and AA/VSD. Primary and staged repair have comparable results. Reoperation after biventricular repair seems inevitable, mostly for conduit replacement. Genetic factors may affect long-term survival.
Norwood's operation provides satisfactory palliation for neonates with hypoplastic left heart syndrome. The dominant physiologic features of hypoplastic left heart syndrome, ductal dependency of the systemic circulation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. Theoretically, these should be equally amenable to palliation by Norwood's operation. Between January 1990 and June 1994, 60 neonates with malformations other than hypoplastic left heart syndrome underwent initial surgical palliation by Norwood's procedure. Diagnoses included single left ventricle with levo-transposition of the great arteries (12); critical aortic stenosis (8); complex double-outlet right ventricle (8); interrupted aortic arch with ventricular septal defect and subaortic stenosis (7); ventricular septal defect, subaortic stenosis, and coarctation of the aorta (7); aortic atresia with large ventricular septal defect (6); tricuspid atresia with transposition of the great arteries (6); heterotaxy syndrome with subaortic obstruction (3); and other (3). There were 10 hospital deaths and 50 survivors (83% survival). After the introduction of inspired carbon dioxide therapy into the postoperative management protocol (1991), 42 of 47 patients survived (89% survival). Mortality was independent of diagnosis and essentially the same as that for hypoplastic left heart syndrome. With minor technical modifications, Norwood's operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circulation in anticipation of either a Fontan procedure or a biventricular repair.
A young child with [S, L, L] segmental anatomy, double-inlet leftventricle, transposition of the great arteries, rudimentary
rightventricle, and mildly restrictive bulboventricular foramen is reported, inwhom intraoperative temporary snaring of the
modified Blalock- Taussigshunt resulted in instantaneous and dramatic volume contraction of the leftventricle, decrease in
bulboventricular foramen size, and increase of thegradient across the latter from 10 mm Hg preoperatively to 50 mm Hg. Amodified
Damus-Stansel-Kaye procedure using autogenous aortic tissueresulted in unobstructed aortic outflow; in addition, a bidirectionalcavopulmonary
shunt was performed. The importance of early relief of actualor potential aortic outflow obstruction in hearts with restrictivebulboventricular
foramen is emphasized.
The spectrum of hypoplastic left heart disease is diverse but the surgical repair is strictly dichotomous, culminating in either a univentricular or a biventricular surgical repair. Although aortic atresia with a ventricular septal defect historically has been managed by conversion to a univentricular physiology, a biventricular repair occasionally has been attempted in stages or in conjunction with the implantation of multiple allografts or prosthetic conduits. Our repair strategy recently has evolved to the use of a modified single-stage biventricular repair using only autologous tissues without conduits.
Retrospective analysis (1989 to 1997) of neonates with aortic atresia with a ventricular septal defect.
Five neonates underwent repair of aortic atresia with a ventricular septal defect. One died in the hospital. The mean age and weight of the neonates who underwent repair were 7.8 days (range, 2 to 17 days) and 3.2 kg (range, 3 to 3.6 kg), respectively. Three neonates had a univentricular repair and 2 had a modified biventricular repair. The latter two procedures were successful and the patients were discharged from the hospital.
Long-term results are lacking to attest to this surgical modification's superiority over either the standard multistage univentricular operation or the single-stage biventricular repair using multiple conduits. However, we are optimistic that routine use of this modification will enable a greater percentage of neonates to undergo a biventricular repair without the need for serial conduit revisions or future aortoplasty.
We compared the Norwood stage I operation for hypoplastic left heart syndrome and other complex malformations with ductus-dependent systemic circulation.
A retrospective study of 194 patients who underwent a Norwood stage I palliation between 1990 and 1998 was conducted. Malformations in 131 patients were classified as hypoplastic left heart syndrome, defined as aortic and mitral atresia or severe stenosis, normal segmental anatomy, intact ventricular septum, and hypoplasia of the left ventricle. Sixty three patients had other lesions: hypoplastic left ventricle with ventricular septal defect (n = 18), unbalanced complete atrioventricular canal (n = 9), complex double-outlet right ventricle (n = 14), double-inlet left ventricle (n = 11), tricuspid atresia with transposition of the great arteries (n = 6), and others (n = 5), including heterotaxia.
Operative (>30 days) and 1-year survivals were lower for patients with hypoplastic left heart syndrome than for those with other lesions (63.4% vs 81%, P =.008, and 51.2% vs 71.4%, P =.02, respectively). The presence of a nonhypoplastic left ventricle (n = 27) was associated with higher operative and 1-year survivals (96.3% vs 64.7%, P =.002; 88.9% vs 52. 7%, P <.001). A restrictive atrial septal defect and prematurity tended to increase mortality across both groups. Cox proportional hazards regression indicated that a single right ventricle was the most important independent predictor of death (P <.001). Operative mortality for all patients undergoing the stage I procedure decreased from 38.5% (1990-1994) to 21.4% after 1994 (P =.02).
The survival of patients with malformations other than hypoplastic left heart syndrome after the Norwood procedure is greater than for those with hypoplastic left heart syndrome. Staged palliation is valid surgical therapy in these patients, with good results in intermediate follow-up.
Hypoplastic left heart syndrome (HLHS) encompasses a spectrum of structural cardiac malformations that are characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity and mass. The severe end of the spectrum consists of aortic atresia and mitral atresia with a nonexistent left ventricle, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, and milder degrees of left ventricular hypoplasia, recently described as hypoplastic left heart complex (HLHC). Although the overwhelming majority of the patients can only have a univentricular repair, a small minority of patients with HLHS, particularly those that are described as having HLHC, may be candidates for biventricular repair. In this paper, the extant nomenclature for HLHS is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Nomenclature and Database Committee and representatives from the European Association for Cardiothoracic Surgery. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented, which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing, and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
The Norwood procedure can be applicable as a first stage palliation in children who can eventually undergo a biventricular repair. Although usual management of these patients is a primary neonatal repair, in selected patients staged approach with a Norwood procedure in the neonatal period followed by a Rastelli procedure in the infancy for conversion to two-ventricle physiology has been used alternatively.
We report our experiences on two infants who underwent a previous palliation with the Norwood procedure for lesions other than hypoplastic left heart syndrome and converted to two-ventricle physiology by the use of a Rastelli-type procedure. This report examines considerations in biventricular repair after the Norwood procedure especially need for ventricular septal defect enlargement and approach to placement of the right ventricle to pulmonary artery conduit.
Both of the infants who underwent staged approach with an initial Norwood procedure for lesions other than hypoplastic left heart syndrome survived the operations and were clinically well at mid-term follow-up.
In selected patients, the staged approach is an alternative in management of malformations other than hypoplastic left heart syndrome which share the important physiologic features of aortic outlet obstruction and ductal dependency of systemic circulation. We recommend routine enlargement of ventricular septal defect and proper positioning of the conduit at the time of subsequent biventricular repair.
Thyroid hormone has important effects on cardiovascular performance. This study was performed to evaluate the changes in levels of triiodothyronine following the first stage of reconstruction for hypoplastic left heart syndrome.
We enrolled 14 newborns with hypoplastic left heart syndrome scheduled for first stage reconstruction. Blood samples were obtained pre-, intra-, and post-operatively. Levels of free and total triiodothyronine were determined by radioimmunoassay. Statistical comparison was performed using Wilcoxon's signed rank test.
The levels of free triiodothyronine decreased from a baseline of 355+/-31 pg/dl to 205+/-21 pg/dl upon the institution of bypass, and declined to a level of 135+/-9 pg/dl at 24 hours postoperatively. Similarly, levels of total triiodothyronine decreased from 101+/-15 ng/dl to 65+/-4 ng/dl upon the institution of bypass, and continued to decline during the first 24 hours postoperatively. Levels of free and total triiodothyronine had returned to baseline by the fifth postoperative day.
The data demonstrate significant decreases in levels of free and total triiodothyronine during the early postoperative period. These changes in levels of thyroid hormone may have adverse effects on cardiac function during this phase of recovery.
Management of newborns with interrupted aortic arch (IAA) remains challenging. Associated severe left ventricular outflow tract obstruction (LVOTO) have often led to increased mortality with neonatal biventricular repair. We review our experience with an alternative approach for this complex surgical problem.
From May 1991 to June 1999, 28 neonates were treated for IAA. Thirteen of 28 neonates (46%) had type B IAA, ventricular septal defect (VSD) and severe LVOTO (Z value -2 to -7; mean -5 +/- 1.7). Mean age was 8 days (3 to 23 days old) with average weight of 3.3 kg (2.4 to 4.2 kg). Eight of 13 (62%) had anomalous right subclavian artery. Ten of 13 (77%) had thymic aplasia and chromosome 22 region qll deletion. All 13 patients were treated initially with a modified Norwood procedure.
There were no perioperative deaths. Complications included 2 patients with recurrent arch stenosis treated with balloon dilatation. Two patients had systemic arterial shunt revision. Follow-up ranged from 2 to 99 months old (mean 39 months). There were 2 late deaths unrelated to any operation. Nine of 12 patients had a second stage palliation consisting of a bidirectional Glenn shunt. Six patients went on to have biventricular repairs (3 Ross-Konno, 2 Rastelli, 1 VSD closure with LVOT resection). One patient had a modified Fontan operation and 5 patients are awaiting potential biventricular repair.
Children with IAA and severe LVOTO may be managed by initial Norwood palliation with an excellent outcome likely. This initial "univentricular" approach has enabled eventual successful biventricular repair despite severe LVOTO.
Patients with hypoplastic left heart syndrome (HLHS) and associated malformations undergo Norwood palliation or potentially a two-ventricle repair.
Since 8/99, 8 patients with typical HLHS and two with DILV underwent Norwood/Fontan palliation (group I). Three other patients (group II) had two-ventricle repair. Anatomy was: aortic atresia, coarctation, unrestrictive VSD (patient 1), hypoplastic mitral and aortic valve, arch and LV, coarctation (patients 2 and 3). Surgical procedures were Norwood arch reconstruction with either Rastelli operation (patient 1) or ASD-closure (patients 2 and 3).
Operative mortality in group I was 1/8 (day 22; RV-failure). Two patients died before Glenn (sepsis, RV-failure). Six patients underwent Glenn procedure successfully. No patient died in group II. Echocardiography after 13 +/- 7.4 months showed mild homograft dysfunction (patient 1) and an LVOT-gradient of 20 mmHg (patient 3). Clinical condition of all survivors in both groups is good.
Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two-ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.
Background:
Members of the Southern Thoracic Surgical Association (STSA) have presented important pediatric cardiothoracic surgery papers at the annual meetings over the last 50 years. In order to determine the influence of these presentations on the practice of surgery, a review was undertaken. Early papers were characterized by emerging advances in open-heart surgery, anatomic congenital heart studies, and electrophysiologic discoveries that extended life with pacemakers. Later years were characterized by innovative myocardial preservation methods, improved cardiopulmonary bypass techniques, expanded homograft availability, emphasis on accurate repairs, intraoperative transesophageal echocardiography, and cardiopulmonary transplantation.
Methods:
All but one of the scientific programs of the annual meetings (that of 1964) were located. The programs were reviewed and 180 presentations were identified on topics in congenital heart disease, pediatric thoracic disease, and pediatric thoracic wall abnormalities. Of those 180 oral presentations, 155 manuscripts (86%) were eventually published or in press and available for critical review and analysis. Manuscripts were grouped by diagnosis or therapeutic intervention. We determined a "cumulative citation frequency" (CCF), which measures the number of times an article is cited in the bibliography of related papers in the universe of participating journals. The selected manuscripts were compared with the historic landmark contributions and the existing trends at the time, and the number of articles both by individual authors and from institutions were tallied.
Results:
Grouping by authors and institutions showed that 100 of 155 pediatric cardiothoracic manuscripts (65%) originated from 13 institutions. The CCF for the 20 leading articles ranged from 26 to 93.
Conclusions:
This historical STSA 50-year record of pediatric cardiothoracic advances was accomplished in a milieu of collegial respect and camaraderie. Our annual meetings over the years have provided a venue for thoracic surgeons to share their ideas, innovations, and scientific inquiry. These contributions have significantly affected the practice of pediatric cardiothoracic surgery. The STSA has worked for 50 years and we trust that it will work for another 50 years and beyond.
Norwood procedure is used as the first stage in the palliative treatment of the hypoplastic heart syndrome and can be used, with some technical modifications, in other forms of univentricular heart with aortic stenosis or hypoplasia. These patients have a high mortality (50%), derived from the procedure itself and from their abnormal physiological status.
To report our experience with the Norwood procedure.
Retrospective analysis of all patients subjected to the Norwood procedure between February, 2000 and June 2003.
Thirteen patients (9 females, age range 5-60 days and median weight of 3.3 kg) were operated. Eight had hypoplastic heart syndrome and five had a single ventricle with aortic arch hypoplasia. The diagnosis was done in utero in eight patients. All technical variations, according to the disposition and anatomy of the great vessels, are described. Cardiac arrest with profound hypothermia was used in all and regional cerebral perfusion was used in nine. Three patients died in the perioperative period and three died in the follow up (two, four and 10 months after the procedure). Gleen and Fontan procedures were completed in five and one patients, respectively.
Our results with the Norwood procedure are similar to other series. There is an important mortality in the immediate operative period and prior to the Glenn procedure.
The surgical treatment of hypoplastic left heart syndrome is still a challenge. Few selected large volume centers that adopted protocols focused on understanding of post-Norwood pathophysiology have reduced their mortality rates to around 15%. The inherent inefficacy of the parallel circulation in Norwood operation lends itself to problems related to postoperative management of these patients crucially revolving around keeping a balance between systemic blood flow (Qs) and pulmonary blood flow (Qp). This paper describes the physiology of the Norwood principle, the importance of an adequate hemodynamic assessment, to guide the different postoperative management options.
A 2.6 kg female with the diagnosis of type "B" interruption of the "right" aortic arch and mitral atresia underwent stage I palliation with translocation and anastomosis of the distal right carotid artery to the descending aorta. The distal main pulmonary artery was anastomosed to the under surface of the neoaortic arch. A modified Blalock-Taussig shunt was constructed between the left side innominate artery and the left pulmonary artery. The child awaits stage II palliation.
The evolution of the present approach to the newborn with hypoplastic left heart is outlined. Preoperatively, maintenance of ductal patency with prostaglandin E1 and balancing of systemic and pulmonary blood flow are essential. Operative details of the first-stage palliation and the definitive second-stage procedure are described. The more recent adoption of an intermediate-stage hemi-Fontan procedure is also described. Since January 1989, 151 patients have been treated using this three-stage approach, with 109 early survivors. Seventy-eight have undergone the hemi-Fontan operation with nine deaths (5 of whom came to this stage early nonelectively because of shunt failure or ventricular dysfunction). Twenty-seven of the 78 patients undergoing hemi-Fontan operation have subsequently undergone definitive Fontan procedures with no deaths.
During the past 14 years, 28 infants (23 males) underwent valvotomy for severe aortic stenosis at the Children's Hospital Medical Center in Boston. The median age was two months and the oldest patient was six months old. Congestive heart failure was present in all but two babies. Preoperatively, the electrocardiogram was abnormal in all, with left ventricular hypertrophy and a strain pattern being present in 19. At cardiac catheterization, the peak systolic ejection gradient (PSEG) ranged from 35-130 mm Hg (median 90 mm Hg). Associated cardiac lesions were present in 39%. Twenty-four infants underwent valvotomy with inflow occlusion. Four patients were operated upon using cardiopulmonary bypass. There were eight early and two late deaths. The 18 survivors have been followed from six months to 11 years (median five years). Only four are symptomatic. Mild aortic regurgitation developed postoperatively in six patients, moderate in one and severe enough to require valve replacement in another one. The electrocardiogram improved postoperatively in 15 patients, but became entirely normal only in one. Five children required a repeat valvotomy for residual stenosis 1-10 years after the original procedure (median four years). At this second operation, the majority of the valves were flexible and noncalcified, and valvotomy was accomplished without difficulty. One child who underwent valve replacement for aortic regurgitation at age two years is well seven years later.
The optimal Fontan-type operation greatly depends on appropriate initial palliation. Several surgical techniques have been used in infancy to palliate patients with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis. The two most common are pulmonary artery banding and Damus-Norwood procedures. Palliative arterial switch operation is another surgical option that was used in this early series of seven infants. The principle of this operation is to "switch" the subaortic obstruction into a subpulmonary obstruction; the coronary artery relocation on the large pulmonary trunk creates a harmonious aortic root and the connection of the rudimentary ventricular chamber to the pulmonary artery trunk creates a natural protection of the pulmonary vascular bed through the restrictive bulboventricular foramen. Seven infants with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis underwent a palliative arterial switch operation. All infants had an associated aortic arch obstruction of various degrees, including one with interrupted aortic arch, five with coarctation with severe arch hypoplasia, and one with isolated arch hypoplasia. There were three with double-inlet left ventricle, three with tricuspid atresia, and one with transposition of the great arteries with ventricular septal defect and severe right ventricular hypoplasia. The subaortic obstruction was patent at birth in five patients who underwent a palliative switch operation in the first 2 months of life, and rapidly occurred following a previous neonatal pulmonary artery banding associated with arch repair in two patients who underwent a switch operation at 5 and 8 months of age, respectively. The operation includes aortic arch repair without prosthetic material, an atrial septectomy, and the arterial switch. An associated pulmonary shunt was required in five patients and a pulmonary artery banding in one. There was one early death in a patient with [S,L,L] anatomy and congenital atrioventricular block, leading to an early mortality of 14% (95% confidence limits: 1% to 28%). There was one late death. Four survivors are waiting for a Fontan-type procedure, and one survivor had satisfactory right ventricular growth. Early palliative arterial switch operation offers several advantages: reconstruction of a harmonious aortic root, natural protection of the pulmonary bed through the restrictive bulboventricular foramen, prevention of deleterious myocardial hypertrophy, and arch reconstruction without the introduction of a foreign material. This aggressive technique may provide a satisfactory palliation in infants with univentricular hearts and ventriculoarterial discordance, when the bulboventricular foramen/aortic anulus ratio is less than 0.8 or when the subaortic stenosis is severe enough to be associated with an arch obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)
Since 1985, 354 neonates have undergone palliative reconstruction for hypoplastic left heart syndrome with 109 early deaths and 12 late deaths. Of the survivors, before 1989, 77 patients underwent a subsequent modified Fontan operation, consisting of baffling the atrial septal defect to the tricuspid valve (initial 25 patients) or intraatrial baffling of the inferior vena cava to the pulmonary arteries and superior vena cava (52 patients). There were 17 early deaths and three late deaths. Major serous effusions developed in 42 patients (54%) after Fontan operation. Since 1989, a staged approach to Fontan's operation was undertaken in an effort to reduce the volume load of the right ventricle as early as possible, to minimize the impact of rapid changes in ventricular geometry and diastolic function that can accompany a primary Fontan operation, and to reduce effusive complications. Thus, at a mean age of 6 months, 121 patients have undergone closure of aortopulmonary shunt, augmentation of central pulmonary arteries, and association of the superior vena cava with the branch pulmonary arteries (hemi-Fontan procedure). Of these, 61 patients have already undergone completion of the Fontan procedure with six early deaths and three late deaths. Major serous effusions developed in 28 patients (46%) with the staged Fontan. For perspective, the contemporary experience since January 1991 consists of 58 neonates who have undergone initial palliation with 11 deaths (19%), 17 patients who have undergone the hemi-Fontan procedure with one death (6%), and 21 patients who have undergone completion of the Fontan operation with one death (5%).(ABSTRACT TRUNCATED AT 250 WORDS)
Circulatory and metabolic homeostasis in patients with hypoplastic left heart syndrome is dependent on a delicate balance between systemic and pulmonary blood flow. Hypocarbia can result in a marked decrease in pulmonary vascular resistance accompanied by pulmonary overcirculation, systemic hypotension, metabolic acidosis, and death. This report illustrates that early and precise control of the arterial carbon dioxide tension using inspired carbon dioxide can be effective in preventing or treating instability arising during management of a patient with hypoplastic left heart syndrome.
Banding the pulmonary trunk may exacerbate or promote the development of subaortic stenosis in patients with double inlet ventricle or tricuspid atresia with a dominant left ventricle and discordant ventriculoarterial connection and, therefore, may be an inappropriate palliative procedure for such patients. To examine this possibility, 102 consecutive infants were studied who presented with this anatomy between 1972 and 1987. Obstruction of the aortic arch was present in 52 patients. In 28 patients (17 with aortic arch obstruction), subaortic stenosis was already apparent at presentation. Of the remaining 74 patients, 19 received no palliative surgery and 55 underwent banding of the pulmonary trunk either with (n = 22) or without (n = 33) aortic arch repair. Outcome was significantly worse in patients with associated aortic arch obstruction. All such patients either died or developed subaortic stenosis by 3 years of age (survival free of subaortic stenosis 0 of 22 versus 22 of 33 for patients with isolated banding of the pulmonary trunk, p less than 0.001). After isolated banding, there was a lower ratio of the ventricular septal defect to ascending aorta diameters at presentation in the patients who developed subaortic stenosis than in the patients who did not (0.60 +/- 0.08 versus 1.03 +/- 0.15, p less than 0.001). Of the latter, 18 (95%) of 19 patients fulfilled criteria for a Fontan procedure at recatheterization. Thus, the presence of aortic arch obstruction is associated with rapid development of subaortic stenosis after banding of the pulmonary trunk. Alternative initial surgery, even though high risk, may be indicated. In the absence of such obstruction, banding the pulmonary trunk can be performed at reasonable risk and, provided that the ventricular septal defect is of adequate size, satisfactorily prepares most patients for a later Fontan procedure.
In the setting of a single ventricle, subaortic stenosis may be enhanced by pulmonary artery banding and may later contraindicate a Fontan operation. The Norwood operation may prove a preferable alternative in some infants as a preparatory procedure. We have successfully used this procedure as the initial operation to palliate a newborn with tricuspid atresia, transposition of the great arteries, coarctation, and severe arch hypoplasia secondary to a restrictive bulboventricular foramen.
Successful palliative repair of aortic atresia and hypoplastic aortic arch associated with tricuspid atresia in a neonate is described. The repair consisted of reconstruction of the hypoplastic aortic arch with an equine pericardial patch, division of the patient ductus arteriosus, connection of the pulmonary artery to the aorta, implantation of the proximal part of the ascending aorta into the main pulmonary artery, and anastomosis of a polytetrafluoroethylene graft 5 mm in diameter between the right ventricular outflow tract and the central pulmonary artery, which was transferred anteriorly to the main pulmonary artery.
Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.(ABSTRACT TRUNCATED AT 250 WORDS)
Surgical treatment of hypoplasia of the ascending aorta is a difficult problem. An approach using proximal pulmonary artery to distal aorta bypass with distal pulmonary artery banding was employed in 19 patients with an ascending aorta less than 6 mm in diameter but a patent aortic outflow. There were 11 male and 8 female neonates and infants, and weight ranged from 2 to 4.5 kg (median weight, 3.5 kg). Four patients had transposition of the great arteries, 12 had subaortic stenosis, and 5 had interrupted aortic arch. There was one operative death. Cerebral cyanosis developed in 3 of the 4 patients with transposition of the great arteries and necessitated additional operations within the first year postoperatively. Therefore, palliation with a pulmonary artery to descending aorta conduit and banding was abandoned in such patients. All 14 surviving patients with normally related great arteries had successful palliation and growth of left heart structures. Subsequent procedures included a Fontan/Damus procedure in 1 patient, patch aortoplasty and pulmonary arterioplasty in 5 patients, arch reconstruction with aortic valvulotomy and pulmonary arterioplasty in 2, double arch reconstruction in 2, and simple patch aortoplasty with plans for subsequent pulmonary arterioplasty and ventricular septal defect closure in 1. Results demonstrate that in patients with a hypoplastic ascending aorta, this radical method of palliation can result in growth of right and left heart structures and thus provides the possibility of biventricular repair.
Optimal prevention and treatment of subaortic stenosis (SAS) in the univentricular heart with subaortic outlet chamber and high pulmonary blood flow remains controversial, especially when complicated by aortic arch obstruction. Herein we analyze our surgical results. Group 1 consisted of 11 infants (mean age, 10 days) with univentricular heart and SAS. Ten required repair of interrupted aortic arch (n = 7) or coarctation with hypoplastic arch (n = 7). Four patients had relief of SAS by either Damus-Kaye-Stansel connection (n = 2) or aortopulmonary window (n = 2), with three operative deaths and one late death. Six had one-stage arterial switch and atrial septectomy with arch repair (5/6) with one operative death and one late death. Two survivors have progressed to bidirectional cavopulmonary shunt, a third has had a Fontan operation, and a fourth awaits Fontan. In group 2, 11 children required operation for acquired SAS after pulmonary artery banding. Nine have progressed to Fontan operation with either staged (n = 3) or concurrent (n = 6) relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Fontan mortality was 11% (70% confidence limits, 2% to 32%). Group 3 consisted of 3 patients without pulmonary artery banding who had SAS diagnosed at Fontan evaluation. All 3 survived Fontan operation and relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Group 4 consisted of 1 patient with previous pulmonary artery banding (no SAS) who underwent Fontan operation but required Damus-Kaye-Stansel connection 30 months later for SAS.(ABSTRACT TRUNCATED AT 250 WORDS)
Failure of infants with critical aortic stenosis to survive after adequate valvotomy despite a left ventricular size that appears to be adequate indicates that additional preoperative anatomic features may contribute to mortality.
Discriminant analysis was used to determine which of several echocardiographically measured left heart structures were independent predictors of survival after valvotomy for neonatal critical aortic stenosis. It was possible to predict outcome after classic valvotomy (two-ventricle-type repair) with 95% accuracy based on mitral valve area, long-axis dimension of the left ventricle relative to the long-axis dimension of the heart, diameter of the aortic root, and body surface area. Left ventricular volume was not a major determinant in this study, in part because patients who had initial valvotomy had been preselected in favor of an adequately sized left ventricle. Patients with multiple small left ventricular structures were found to have significantly improved survival after initial Norwood operation. In contrast, balloon valvotomy with subsequent Norwood procedure was usually unsuccessful.
The adverse effects of small inflow, outflow, and/or cavity size of the left ventricle are cumulative. The accuracy of prediction of outcome based only on preoperative anatomy indicates that adequacy of valvotomy is not generally a limiting factor for survival in this group of patients. It is possible to identify subjects whose chance of survival is better after a Norwood procedure rather than valvotomy, even if left ventricular volume is not critically small.
The fate of 191 infants with double-inlet ventricle was studied to determine the influence of morphologic characteristics at presentation and subsequent management on the potential for, and timing of, definitive repair by the Fontan operation or ventricular septation. At presentation, 136 patients (71%) were potential candidates for a Fontan procedure. Actuarial survival was better than for those deemed unsuitable for either definitive option (n = 55; 68% versus 28% at 1 year; p less than 0.001), but still, only 78 patients (57%) were known to be alive and suitable candidates at 2 years of age. This was largely due to death after presentation with low cardiac output (n = 19) and at palliative operation (20 of 98 surgically treated patients). The adverse events of late sudden death (n = 14) and the development of new features precluding a Fontan operation (n = 18) mostly occurred before 4 years of age (n = 22). Patients requiring no operation and those who underwent a systemic-pulmonary arterial shunt fared better than those who underwent isolated banding of the pulmonary trunk (9/13 and 25/42 alive and suitable versus 14/33; p less than 0.05), and than those who required aortic arch repair together with banding (1/12; p less than 0.01), because of the development of subaortic stenosis in the latter group. In contrast, only 43 patients (23% of all 191 patients) had morphologic features that were additionally compatible with future ventricular septation. Actuarial survival free of adverse events for these 43 patients was similar to that of the 136 patients considered suitable for a Fontan operation. Thus management in infancy must be aimed at maintaining potential for a future Fontan operation, which itself should not be delayed, for most patients, beyond 3 years of age, because of the prevalence of adverse events with increasing age.
Subaortic stenosis is well known to complicate the clinical course of patients with single ventricle or univentricular hearts, and we have previously suggested that the development of subaortic stenosis in such patients may be causal to and/or accelerated by previous banding of the main pulmonary trunk. To further define the relationship between banding of the pulmonary artery in patients with univentricular hearts and the development of subaortic stenosis, we examined the morphologic substrate and timing of the development of subaortic stenosis in 43 patients seen at our institution from January 1, 1970, through June 30, 1985. These 43 patients include all patients in this period with an unequivocal univentricular heart whose longitudinal data was available for follow-up. We excluded patients who died within 1 week of surgery, patients lost to follow-up, and patients with evidence of subaortic stenosis before banding. Thirty-one of 43 patients (72.1%) developed subaortic stenosis subsequent to banding of the main pulmonary artery. The mean age at banding of those patients who developed subaortic stenosis was 0.21 years, and subaortic stenosis was recognized at a mean age of 2.52 years. For the specific cohort of patients whose ventricular morphology was a main chamber of left ventricular type supporting the pulmonary artery and a rudimentary right ventricle supporting the transposed aorta (32 patients), 27 developed subaortic stenosis (84.4%). Subaortic stenosis in the classic form of single ventricle usually results from progressive restriction of a wholly muscular interventricular communication. Banding of the pulmonary artery by producing myocardial hypertrophy undoubtedly accelerates the potential for subaortic stenosis in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 +/- 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 +/- 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 +/- 0.03 and 0.56 +/- 0.06, respectively, compared with 0.81 +/- 0.12 and 0.95 +/- 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction (p less than 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.
Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.
The factors associated with survival in 40 neonates (age less than 28 days) with critical aortic stenosis undergoing either open (22 patients) or closed (18 patients) transventricular aortic valvotomy were reviewed. Significant adverse correlates with survival included evidence of poor perfusion preoperatively (low pH, greater than Grade 2/6 soft ejection systolic murmur) and marked congestive heart failure (hepatomegaly, cardiomegaly, elevated left atrial pressure). Congenital mitral stenosis (anulus less than 11 mm), a small aortic anulus (less than 6.5 mm), and failure to achieve an adequate aortic orifice (greater than 6 mm), at operation were identified as factors associated with increased mortality. Initial perioperative survival was better with closed aortic valvotomy. However, there was no significant difference in overall operative survival between closed (9/18, 50%) and open (8/22, 36%) aortic valvotomy (p = 0.26). The incidence of early reoperation (less than 1 year of age) was greater in perioperative survivors undergoing closed valvotomy (7/13, 54%) rather than open valvotomy (1/10, 10%) (p less than 0.05). In conclusion, long-term survival among patients with critical neonatal aortic valve stenosis remains disturbingly low (13/40, 32%) and has not significantly improved over the past 20 years.
Neonates with ventricular septal defect and aortic arch obstruction frequently have subaortic stenosis resulting from posterior deviation of the infundibular septum. Because the aortic anulus is often hypoplastic, making direct resection of the infundibular septum through the standard transaortic approach difficult, the optimal method of repair is uncertain. From September 1989 through November 1991, seven patients with ventricular septal defect, coarctation (n = 4), or interrupted aortic arch (n = 3) and severe subaortic stenosis underwent repair with use of a technique that included transatrial resection of the infundibular septum. Their ages ranged from 5 to 63 days (median 15 days) and weights from 1.3 to 5.4 kg (mean 3.1 kg). Only one patient was older than 1 month. The systolic and diastolic ratios of the diameter of the left ventricular outflow tract to that of the descending aorta were 0.53 +/- 0.09 mm (standard deviation) and 0.73 +/- 0.11, respectively. At operation, the posteriorly displaced infundibular septum was partially removed through a right atrial approach by resecting the superior margin of the ventricular septal defect up to the aortic anulus. The resulting enlarged ventricular septal defect was then closed with a patch to widen the subaortic area. In each patient the aortic arch was repaired by direct anastomosis. All patients survived operation; there was one late death from noncardiac causes 3 months after repair. The survivors remain well from 3 to 14 months after repair (mean 8 months). All are in sinus rhythm and none has a residual ventricular septal defect. One patient underwent successful balloon dilation of a residual aortic arch gradient late after repair. No patient has significant residual subaortic stenosis, although one has valvular aortic stenosis. This series suggests that in neonates with ventricular septal defect and severe subaortic stenosis resulting from posterior deviation of the infundibular septum, direct relief can be satisfactorily accomplished from a right atrial approach. This method provides effective widening of the left ventricular outflow tract and is superior to palliative techniques or conduit procedures.