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Traditional failing Fontan hemodynamics (high CVP with low output) and possible therapeutic options. For systemic ventricular systolic dysfunction, conventional anti-HF strategies might be successful. In addition, pulmonary artery dilators could also be effective. For AVV impairment, surgical options should be considered. ACEI = angiotensin converting enzyme inhibitor; AOP = aortic pressure; ARB = angiotensin II receptor blocker; AVV = atrioventricular valve; AVVP/R = atrioventricular valvuloplasty or replacement; CRT = cardiac resynchronization therapy; CVP = central venous pressure; EM = coil embolization to collateral vessels; ETB = endothelin receptor blocker; HF = heart failure; HOT = home oxygen therapy; IVN = catheter intervention; LAP: functional left atrial pressure; MP = muscle pump; NO = nitric oxide; PDE5I = phosphodiesterase 5 inhibitor; PGI2 = prostaglandin I2; PTA = percutaneous transluminal angioplasty; RP = respiratory pump; Rp = pulmonary artery resistance; Rs = systemic artery resistance; S/IVC = superior vena cava/inferior vena cava.
Source publication
Fontan circulation is generally characterized by high central venous pressure, low cardiac output, and slightly low arterial oxygen saturation, and it is quite different from normal biventricular physiology. Therefore, when a patient with congenital heart disease is selected as a candidate for this type of circulation, the ultimate goals of therapy...
Context in source publication
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Objective
Fontan circulation maintains preload and cardiac output by reducing venous capacitance (Cv) and increasing central venous pressure (CVP). The resultant congestive end-organ damage affects patient prognosis. Therefore, a better circulatory management strategy to ameliorate organ congestion is required in patients with Fontan circulation. T...
Citations
... As shown in Table 3, the area fraction of the great vessel area, including the Fontan tract, was also significantly lower when the point of chest compression was moved to the right by the width of the sternum. The Fontan tract is less compressed, so the preload can be secured during CPR [13]. Therefore, the results of our study can support the background theory of the 2020 PALS guidelines. ...
Background
We aimed to identify the ideal chest compression site for cardiopulmonary resuscitation (CPR) in patients with a single ventricle with dextrocardia corrected by Fontan surgery.
Methods
The most recent stored chest computed tomography images of all patients with a single ventricle who underwent Fontan surgery were retrospectively analysed. We reported that the ideal chest compression site is the largest part of the compressed single ventricle. To identify the ideal chest compression site, we measured the distance from the midline of the sternum to the point of the maximum sagittal area of the single ventricle as a deviation and calculated the area fraction of the compressed structures.
Results
58 patients (67.2% male) were analysed. The mean right deviation from the midline of the sternum to the ideal compression site was similar to the mean sternum width (32.85 ± 15.61 vs. 31.05 ± 6.75 mm). When chest compression was performed at the ideal site, the area fraction of the single ventricle significantly increased by 7%, which was greater than that of conventional compression (0.15 ± 0.10 vs. 0.22 ± 0.11, P < 0.05).
Conclusions
When performing CPR on a patient with Fontan circulation with dextrocardia, right-sided chest compression may be better than the conventional location.
... The case PVR-Cr represents a high-PVR Fontan failure state, in which the CVP is maintained at 18 mmHg for a long-time, chronically, which is far over the optimal limit (14 mmHg) of an ideal Fontan patient [12,36]. It is wellestablished that even a quantum decrease in CVP (2 to 6 mmHg) is effective and will delay the Fontan failure [16,40,43,46]. ...
End-stage Fontan patients with single-ventricle (SV) circulation are often bridged-to-heart transplantation via mechanical circulatory support (MCS). Donor shortage and complexity of the SV physiology demand innovative MCS. In this paper, an out-of-the-box circulation concept, in which the left and right ventricles are switched with each other is introduced as a novel bi-ventricle MCS configuration for the “failing” Fontan patients. In the proposed configuration, the systemic circulation is maintained through a conventional mechanical ventricle assist device (VAD) while the venous circulation is delegated to the native SV. This approach spares the SV and puts it to a new use at the right-side providing the most-needed venous flow pulsatility to the failed Fontan circulation. To analyze its feasibility and performance, eight SV failure modes have been studied via an established multi-compartmental lumped parameter cardiovascular model (LPM). Here the LPM model is experimentally validated against the corresponding pulsatile mock-up flow loop measurements of a representative 15-year-old Fontan patient employing a clinically-approved VAD (Medtronic-HeartWare). The proposed surgical configuration maintained the healthy cardiac index (3–3.5 l/min/m2) and the normal mean systemic arterial pressure levels. For a failed SV with low ejection fraction (EF = 26%), representing a typical systemic Fontan failure, the proposed configuration enabled a ~ 28 mmHg amplitude in the venous/pulmonary waveforms and a 2 mmHg decrease in the central venous pressure (CVP) together with acceptable mean pulmonary artery pressures (17.5 mmHg). The pulmonary vascular resistance (PVR)—SV failure case provided a ~ 5 mmHg drop in the CVP, with venous/pulmonary pulsatility reaching to ~ 22 mmHg. For the high PVR failure case with a healthy SV (EF = 44%) pulmonary hypertension is likely to occur as expected. While this condition is routinely encountered during the heart transplantation and managed through pulmonary vasodilators a need for precise functional assessment of the spared failed-ventricle is recommended if utilized in the PVR failure mode. Comprehensive in vitro and in silico results encourage this novel concept as a low-cost, more physiological alternative to the conventional bi-ventricle MCS pending animal experiments.
... Below 82% there was a high likelihood of increased length of hospitalization. It is still unclear how lower oxygen saturation can cause increased venous congestion; a suggested mechanism is through a direct increase in pulmonary vascular resistance via induction of pulmonary vasoconstriction 13 . Low oxygen saturation prior to Fontan might also result from Fontan dysfunction, leading to increased flow across the fenestration with more desaturation 14 . ...
Background:
The Fontan procedure is the final stage of a three-stage palliation process in patients born with a univentricular heart as part of hypoplastic left heart syndrome (HLHS) or other pathologies with a univentricular heart. As essential as this procedure has proven to be for such cases, the Fontan physiology diminishes cardiac output and expands systemic venous pressure, which then leads to venous congestion that can be complicated by protein-losing enteropathy (PLE). This retrospective study aimed to identify the predictors of such complications in all patients who underwent completion of the Fontan procedure at our center (Sheikh Khalifa Medical City/SKMC) in the past eight years.
Methods:
This study examined the medical records of patients who underwent completion of Fontan repair at our center since the inauguration of the cardiac surgery program of SKMC in the United Arab Emirates (UAE) - 01 Jan 2012 to 31 Dec 2020. Exclusion criteria included the absence of any of the undermentioned data in patient files. Patients were divided into two groups: those who developed PLE and those who did not. For each group, the following data were collected: demographics data (current age and age at completion of Fontan), clinical and laboratory data (oxygen saturation, serum albumin), echocardiographic data (classification of original cardiac diagnosis, degree of atrio-ventricular valve regurgitation, ventricular functions), hemodynamic data (mean pressures of superior vena cava and pulmonary arteries before Fontan completion), operative data (type of initial palliation, type of Fontan, presence of fenestrations and its size) and the need for any cardiac intervention prior to Fontan completion, such as atrio-ventricular valve repair, peripheral pulmonary stenting and arch balloon dilatation.
Results:
Of the 48 included patients,13 (25%) developed PLE. Multivariate regression analysis proved that the best predictors of PLE were superior vena cava mean pressure (P = 0.012) and the degree of atrio-ventricular valve regurgitation (P = 0.013). An oxygen saturation <83% prior to Fontan completion was 92% sensitive in predicting PLE after Fontan completion.
Conclusion:
This is a single-center study of the predictors of PLE after Fontan procedure and, as expected from similar studies, SVC pressure higher than 11 mmHg and moderate-to-severe atrio-ventricular valve regurgitation were predictors of Fontan failure. The higher prevalence of PLE in our cohort, as well as lower cut-offs of SVC pressure that can predict complications, may be related to the predominance of hypoplastic left heart in the operated patients, which has been the main referral center for cardiac surgeries in UAE in the last decade.
... However, due to lack of a functioning subpulmonary ventricle, reactive high CVP presents to create additional driving pressure for the pulmonary circulation. 1 Complications develop because of venous congestion or decreased cardiac output (CO). Common comorbidities included ventricular failure, arrhythmias, thromboembolic events, liver disease, and protein-losing enteropathy. ...
... Therefore, the systemic perfusion pressure is consequently low, leading to end-organ failure. 1,4 In this condition, inotropic agents and pulmonary vasodilators could regulate stroke volume and reduce CVP level, respectively. However, there are some Fontan patients who have high CVP but high or normal CO instead. ...
... Conventional heart failure medications, such as ACEI, ARB, and aldosterone antagonists, have been reported to have anti-inflammatory and antifibrogenic effects in Fontan-associated liver disease, while carvedilol and metoprolol may be considered for systolic dysfunction. 1,5,15 However, caution must be exercised when using vasodilatory medications in Fontan patients with renal dysfunction as these medications may worsen hemodynamic balance in patients with elevated CVP and high CO. 1 In such cases, vasoconstrictors like midodrine, which increase SVR, have been suggested to be more effective and may be used to treat protein-losing enteropathy. 6 In our case, the use of vasopressors has shown to be helpful in this scenario of Fontan circulation. ...
The modified Fontan operation, is a standard palliative surgical procedure for patients with single ventricular complex congenital heart disease. This procedure improves the patient’s oxygenation saturation and may achieve long-term survival. Owing to the unique hemodynamic status, substantial morbidity and mortality occur while hemodynamic conditions do not provide sufficient oxygenation and perfusion. Fontan failure thus developed. We report a case of a 16-year-old girl, having a history of Fontan failure and cirrhosis, whose hemodynamic condition and renal insufficiency were successfully treated with intravenous and oral vasopressors.
... Moreover, the overlapped radioactive tracers on the nutmeg-like findings elucidated the lymphatic connection of the TD and abnormal lymphatic dilatation in the cervical and left hilar lung areas ( Fig. 3 B). SPECT/MRL findings suggested PLPS, and the results of cardiac catheterization indicated the hemodynamic profile of the patient were inconsistent with a failing Fontan circulation [13] . ...
... "Failing Fontan circulation" is a term commonly used in clinical settings, but its definition is still uncertain. The hemodynamic characteristics of Failing Fontan anecdotally include elevated CVP, decreased cardiac output, and decreased SaO 2 , although there is no numerical threshold for each parameter [13] . In our case, the results of cardiac catheterization (ie, CVP of 12 mmHg, CI of 3.5 L/min * m 2 , and SaO 2 of 96%) were inconsistent with the characteristics of Fontan failure. ...
A preschool male patient with an extensive cardiac surgical history developed refractory chylothorax after a total cavopulmonary connection. Neither lymphoscintigraphy nor single-photon emission computed tomography (SPECT)/computed tomography could identify the lymphatic system leakage sites. Non-contrast heavy T2-weighted magnetic resonance lymphangiography (MRL) was performed to visualize the lymphatic system. Nevertheless, distinguishing lymphatic ducts from other watery structures of the patient remained difficult. Therefore, non-contrast MRL and SPECT images were fused. This hybrid diagnostic tool elucidated the pathophysiology of the prolonged chylothorax; pulmonary lymphatic perfusion syndrome and illustrated the anatomical connection of the thoracic duct and an abnormally dilated lymphatic network in the neck and left hilar regions. Subsequent intranodal lymphangiography with ethiodized oil confirmed these findings. SPECT/MRL may become an alternative modality for revealing the mechanism of prolonged chylothorax by visualizing the lymphatic system when dynamic contrast-enhanced magnetic resonance lymphangiography is unavailable.
... Thus, regular liver imaging and liver function tests are recommended after the Fontan procedure [56]. After the development of cirrhosis and portal hypertension due to Fontan-associated liver disease, intrinsic substances such as nitric oxide increase and lead to systemic vasodilation [57]. This abnormal systemic vasodilation cause inappropriately reduced systemic vascular resistance and systemic blood pressure and causes end-stage heart failure with inappropriately high cardiac output. ...
Cardiovascular diseases and liver diseases are closely related. Non-alcoholic fatty liver disease has the same risk factors as those for atherosclerotic cardiovascular disease and may also be a risk factor for atherosclerotic cardiovascular disease on its own. Heart failure causes liver fibrosis, and liver fibrosis results in worsened cardiac preload and congestion. Although some previous reports regard the association between cardiovascular diseases and liver disease, the management strategy for liver disease in patients with cardiovascular diseases is not still established. This review summarized the association between cardiovascular diseases and liver disease. In patients with non-alcoholic fatty liver disease, the degree of liver fibrosis progresses with worsening cardiovascular prognosis. In patients with heart failure, liver fibrosis could be a prognostic marker. Liver stiffness assessed with shear wave elastography, the fibrosis-4 index, and non-alcoholic fatty liver disease fibrosis score is associated with both liver fibrosis in patients with liver diseases and worse prognosis in patients with heart failure. With the current population ageing, the importance of management for cardiovascular diseases and liver disease has been increasing. However, whether management and interventions for liver disease improve the prognosis of cardiovascular diseases has not been fully understood. Future investigations are needed.
... The Fontan tract is less compressed, so the preload can be secured during CPR. 13 Therefore, the results of our study can support the background theory of the 2020 PALS guideline. In addition, it may be more helpful for Fontan circulation patients with dextrocardia. ...
We aimed to find the ideal chest compression point undergoing cardiopulmonary resuscitation (CPR) in patients with a single ventricle with dextrocardia corrected by Fontan surgery. The most recent saved chest computed tomography images of all patients 8 years or older with a single ventricle who underwent Fontan surgery were retrospectively analysed. We defined the ideal chest compression point as the largest part of the compressed single ventricle. To find the ideal chest compression point, we measured the distance from the midline of the sternum to the point of the maximum sagittal area of the single ventricle as a deviation and calculated the area fraction of the compressed structures. Fifty-eight patients (male 67.2%) were analysed. The mean right deviation from the midline of the sternum as the ideal compression point was almost like the mean sternum width (32.85 ± 15.61 vs. 31.05 ± 6.75 mm). When chest compression was performed at the ideal point, the area fraction of the single ventricle statistically significant increased by 7%, wider than conventional compression (0.15 ± 0.10 vs. 0.22 ± 0.11, p < 0.05). When performing CPR on a patient with Fontan circulation with dextrocardia, right-sided chest compression is better than the conventional location.
... Actually, in our case series, 3 patients had tolerable central venous pressure, and 3 patients had high cardiac index. Therefore, pathophysiologic interactions between the cardiovascular system and advanced noncardiovascular end-organ dysfunction are supposed to play major roles in Fontan circulatory failure [7,8]. Fontan circulatory failure cannot be explained by the contemporary concept of heart failure according to ventricular ejection fraction, that is, heart failure as a result of a reduced or preserved ejection fraction [9]. ...
Sodium glucose cotransporter-2 (SGLT-2) inhibitors have been widespread in patients with heart failure; however, there is little information regarding its feasibility and safety among patients after the Fontan procedure. We presented five adults after the Fontan procedure who were treated with SGLT-2 inhibitors. All patients reduced oedema and/or pleural effusion despite other conjunct medications were ineffective. Although we did not measure the urine volume in all patients, all patients themselves reported an increase in urinary output after the administration of a SGLT-2 inhibitor. In addition, administration of a SGLT-2 inhibitor resulted in weight loss (4/5), an increase in systemic oxygen saturation (4/5), an increase in serum albumin level (4/5), an increase in estimated glomerular filtration ratio (4/5), and a decrease in plasma brain natriuretic peptide level (4/5). Our case series supported the feasibility and safety of SGLT-2 inhibitors in patients with Fontan circulatory failure, although the exact changes in urinary output were unknown in all patients. Further investigation will be required to explore a diuretic effect by SGLT-2 in patients after the Fontan procedure.
... The causes of seizure may have been multifactorial, including history of autism, the state of cardiac ischemia that might happen which decreases the seizure threshold, and hypoxia. Of note, patients with cardiac condition and Fontan procedure tend to have a lower-than-normal oxygen saturation (2). This case also illustrates the significant impact of COVID-19 pandemic on access to healthcare, particularly for cancer patients. ...
Background:
Cardiac liver cirrhosis secondary to Fontan procedure has been associated with hepatocellular carcinoma at a younger age. However, Fontan associated liver disease and combined hepatocellular-cholangiocarcinoma has not been previously reported. Combined hepatocellular-cholangiocarcinoma is a rare cancer that accounts for 2-5% of primary liver tumors and poses significant diagnostic and treatment challenges. This case highlights these needs and potential screening and treatment considerations. Herein we describe a case of combined hepatocellular-cholangiocarcinoma in a patient with autism, congenital heart disease, and Fontan procedure.
Case description:
The patient is a 27-year-old male who presented with a liver mass detected on MRI performed in the context of a rising alpha-fetoprotein during a screening visit. Biopsy of the mass revealed a combined hepatocellular-cholangiocarcinoma which was staged as localized. Due to the COVID-19 pandemic and subsequent halt of all elective surgeries, the patient received local therapy with chemoembolization followed by pembrolizumab. The disease progressed though, and therapy was changed to gemcitabine plus cisplatin. Patient received 2 cycles of therapy, after which he and his family decided to transfer medical care to Memorial Sloan Kettering. Next generation sequencing of the tumor revealed TP53 and FGFR2 mutations. By then patient was also found to have lung metastasis. To help address the hepatocellular carcinoma, lenvatinib was added. Patient had sustainable disease control for about a year, yet eventually developed thrombocytopenia complicated by an episode of gastrointestinal bleeding. With a worsening performance status, adverse events of the treatment, and recurrent hospitalizations, a goals of care discussion with his family led to the discontinuation of active cancer therapy and patient was started on best supportive care. Patient remained in active follow-up until the time of this report and passed away less than a year from initiating best supportive care alone.
Conclusions:
This challenging case raises awareness towards screening and monitoring all patients with Fontan procedure for Fontan associated liver disease and liver cancers, including combined hepatocellular-cholangiocarcinoma. To the best of our knowledge, this is the first description of combined hepatocellular-cholangiocarcinoma occurring in the context of cardiac cirrhosis. The management difficulties that led to altering the goals of care, is another reminder of the dynamic nature of the care oncologists would provide.
... When focusing on patterns of failing conditions, 2 headings are useful: (1) early failure in which the patients do not adapt to the circulation soon after the Fontan-type procedure, and (2) late failure in which the once-adapted Fontan circulation becomes less efficient long afterwards. 171, 973 Usually, in the former there is reduced cardiac output with higher CVP, while in the latter there is increased cardiac output with higher CVP. 171 Late failure may be a result of some complications of the bodily organs. ...
