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Non-compaction of the left ventricle is an extremely rare cardiomyopathy resulting from a defective morphogenesis of the endomyocardium. It results in an architecturally aberrant ventricular wall consisting of two layers: a compacted layer and a loose interwoven meshwork with prominent trabeculae and deep intertrabecular recesses that communicate w...
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... man presented to the emergency room because of sudden onset of dizziness, slurred speech, and weakness of the right-sided extremities. Diagnosis of dilated cardiomyopathy had been established five months prior to this incident, when he developed signs and symptoms compatible with congestive heart failure. Conventional treatment for heart failure was instituted. Physical examination revealed mild dysarthria, 0/5 strength in the right upper extremity, and 2/5 strength in the right lower extremity. A grade 2/6 pansystolic murmur at the left lower sternal border was also appreciated. The remainder of the examination was within normal limits. Head computed tomography (CT) scan failed to reveal evidence of acute intracranial pathology and within the next 24 hours, neurologic symptoms had resolved, for which a diagnosis of Transient Ischemic Attack (TIA) was established. A transthoracic echocardiogram with contrast media demonstrated a hypokinetic and markedly dilated left ventricle with a double-layer arrangement consisting of a thin epicardial muscle layer and a thick endocardium ( Figure 1) consisting of multiple trabeculae and deep recesses (Figure 2), suggestive of Left Ventricular Non- Compaction (LVNC). Over the following six months, the patient had multiple hospitalizations due to recurrent thromboembolic events and decompensated heart failure. The patient was referred to an electrophysiologist for placement of an implantable cardioverter-defibrillator (ICD). During the procedure, the patient developed an intractable ventricular arrhythmia and subsequently died in spite of appropriate resuscitation measures. Autopsy confirmed a grossly enlarged heart, weighing 980g. The left ventricular (LV) wall demonstrated segmental thickening with a two-layer arrangement consisting of a thin compacted epicardial layer and a thick, non-compact endocardial layer with prominent trabeculations and deep recesses comprising over 50% of the LV wall (Figure 3), consistent with LVNC. Microscopic examination of the tissue revealed focal fibrosis (Figure 4). First-line relatives of this patient were submitted to echocardiographic studies, but no abnormalities have been reported so far. Noncompaction of the left ventricle (LVNC) is an extremely rare cardiomyopathy resulting from defective morphogenesis of the endomyocardium. The architecturally aberrant ventricular wall consists of two layers: a compacted layer, and a loose interwoven meshwork with prominent trabeculae and deep intertrabecular recesses that communicate with the left ventricular cavity (1-4). As a result, the myocardium is thickened and may be easily confused with other –albeit more common- cardiomyopathies. This probably explains why even though occasional reports corresponding to LVNC had been published during the second half of the twentieth century (5), the condition remained unrecognized until 1984, and it is still included under “unclassified” cardiomyopathies by the World Health Organization [WHO] ...
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Citations
... There have been few proposed functional theories about trabeculae in the literature, but some suggested that trabeculae improve nourishment of myocardium during development [6,9], may help buffer the kinetics of blood flow in the ventricle [9,10], or assist in emptying blood more completely during systole [11]. Furthermore, excessive numbers of free-running trabeculae occur in pathological conditions, most notably left ventricular noncompaction, characterized by a layer of prominent trabeculae and deep intertrabecular recesses at least twice as thick as the compacted myocardium, which results in heart failure (HF) [12][13][14]. Prominent trabeculae occur in dilated cardiomyopathy, acquired LV hypertrophy due to hypertension, and congenital heart disease [6,12,15]. Trabecular hypertrophy also occurs in hypertrophic cardiomyopathy [5,[16][17][18]. ...
The role of trabeculae carneae in modulating left ventricular (LV) diastolic compliance remains unclear. The objective of this study was to determine the contribution of trabeculae carneae to the LV diastolic compliance. LV pressure-volume compliance curves were measured in six human heart explants from patients with LV hypertrophy at baseline and following trabecular cutting. The effect of trabecular cutting was also analyzed with finite element model simulations. Our results demonstrated that LV compliance improved after trabecular cutting (p<0.001). Finite element simulations further demonstrated that stiffer trabeculae reduce LV compliance further, and that the presence of trabeculae reduced the wall stress in the apex. In conclusion, we demonstrate that integrity of the LV and trabeculae is important to maintain LV stiffness and loss in trabeculae leads to more LV compliance.
... The literature search yielded 26 adult cases (14 females, mean age 36 years, range 21-70 years) whose hearts have been described pathomorphologically either after cardiac transplantation (n = 16) or at autopsy (n = 10) [3,6,7,[16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33]. In previously reported cases the hearts were opened along the long axis in 6 cases, along the short axis in 15 cases, and in 5 cases the axis was not reported. ...
Aim of this study was to assess pathomorphologic findings (PATHO) in patients with echocardiographically (ECHO) diagnosed left ventricular hypertrabeculation/noncompaction.
ECHO-criteria for LVHT were: >3 trabeculations, moving synchronously with the compacted myocardium, and forming the noncompacted part of a two-layered myocardium. At autopsy, the hearts were investigated according to the pathologists' preferences.
Twelve patients (2 females, age 27-81years) were included. Seven suffered from neuromuscular disorders, 5 patients were not investigated neurologically. The specimens were acquired after explantation during heart transplantation (n=1), death due to heart failure (n=6), sudden death (n=2), pneumonia (n=2) and stroke (n=1). Eight hearts were investigated without fixation and 4 after formaldehyde fixation. The hearts were opened along the long-axis, in 3 hearts additional short-axis cuts were carried out. At PATHO the trabecular meshwork was better visible in the formaldehyde-fixed hearts than in the fresh hearts. Differentiation from papillary muscles was easier on the long-axis cuts, whereas the two-layered structure was better visible on short-axis cuts. The trabecular pattern was similar in patients with neuromuscular disorders and those who did not undergo neurologic investigation. Subendocardial fibrosis was found in each case. Due to the complex three-dimensional geometry, it was impossible to count the number of trabeculations.
Formaldehyde-fixation should be performed when comparing ECHO with PATHO findings in LVHT. Long-axis as well as short-axis cuts should be carried out in order to assess the course of trabeculations and the extent of the two-layered structure. Subendocardial fibrosis in LVHT deserves further research.
... One patient died during the procedure, the other a few weeks later at home, and the third was treated with a left ventricular assist device followed by cardiac transplantation after three months. In all three patients ICD implantation was carried out for primary prophylaxis of SCD [120][121][122]. ...
Arrhythmias in left ventricular hypertrabeculation/noncompaction (LVHT) comprise sustained or non-sustained ventricular tachycardia (VT) (n=135), atrial fibrillation (AF) (n=96) AV block (n=55) and QT prolongation (n=47). The prevalence differs between children and adults. In children most frequent are WPW-syndrome (n=24), AV block (n=24), VT (n=17) and bradycardia (n=15). In adults most frequent arrhythmias are VT (n=118), AF (n=95), QT prolongation (n=42) and AV block (n=31). Some arrhythmias are more frequently reported in children than in adults like WPW-syndrome (24 vs. 17 patients), second-degree AV block (4 vs. 0 patients), bradycardia (15 vs. 3 patients) and ventricular fibrillation (VF) (9 vs. 5 patients). There are nearly no pediatric cases with AF (1 vs. 95 patients). In 120 patients implantable cardioverters/defibrillators have been implanted for primary or secondary prevention of sudden cardiac death. The pathomechanisms of arrhythmias in LVHT are largely unknown, especially if patients with LVHT and neuromuscular disorders are more prone to arrhythmias than patients without. There is a need to clarify risk factors for VT or VF because 19% of LVHT patients with VT or VF have a normal systolic function and demonstration of systolic dysfunction is no reliable risk marker. Data about long-term follow-up of LVHT patients with implanted cardioverters/defibrillators are necessary since the indication for prophylactic implantation is still unclear. AF in LVHT increases the embolic risk, thus it would be useful to know which LVHT patients who have sinusrhythm at baseline are prone to develop AF in order to start early with anticoagulant therapy.
... Left ventricular noncompaction, previously known as ''spongy left ventricular myocardium,'' is a rare, unclassified cardiomyopathy [1,2]. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses that lie in continuity with the left ventricle cavity. ...
Left ventricular noncompaction, previously known as ‘‘spongy left ventricular myocardium,’’ is a rare, unclassified cardiomyopathy [1, 2]. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses that lie in continuity with the left ventricle cavity. The echocardiographic diagnostic criteria present the ‘‘gold standard’’ for diagnosis [1, 3]. Rarely this abnormality might be associated with other congenital heart defects, and then angiography is a useful tool. Because of this there are only a few angiographic images of noncompaction of the left ventricle (NCLV) in the literature. Prompt recognition of the disease is mandatory because of its high mortality and morbidity rates in symptomatic patients. Thus, we present here the angiographic images of an infant with such an anomaly. The infant was born normally after an uneventful pregnancy to healthy unrelated parents. The female patient’s birth weight was 2.8 kg. She was admitted at the age of 3 months with signs of severe respiratory distress. A loud systolic murmur of 3/6 intensity was audible at the left sternal border. There were crepitations in her chest as well as hepatomegaly. Chest X-ray showed cardiomegaly (cardiothoracic index 0.63) and plethoric lung fields. Echocardiogram showed perimembranous ventricular septal defect; increased pulmonary pressure; and a hypertrophied, spongiform, poorly contractile left ventricle. The ejection fraction was 38%, and there were intramural recesses communicating with the left ventricle cavity, with blood flowing in and out from the ventricle. A ratio of 2.25:1 was calculated between the thicknesses of the noncompacted and compacted myocardial layers in systole (Figs. 1 and 2). Angiography was performed after the patient was clinically stabilized. Left ventricular angiography (Fig. 3) showed the left ventricle with a wide spongy-like layer and the perimembranous ventricle septal defect. The pressure was increased in the right ventricle (70/0/13 mmHg) and in the pulmonary artery (53/10/30 mmHg). The child was referred for surgical intervention. The ventricular septal defect (VSD) was closed with a 9-mm Dacron patch. The postoperative period was uncomplicated. Because the patient had residual congestive heart failure due to NCLV at 12month follow-up, pharmacotheraphy has been continued up to the present (digitalis, diuretics, and angiotensin-converting enzyme inhibitors).
Introduction
Left ventricular hypertrabeculation, also named “noncompaction” (LVHT) is a cardiac abnormality which is detected by pathoanatomic investigation or during cardiac surgery. Imaging techniques visualize LVHT by ventriculography, echocardiography, cardiac magnetic resonance imaging(CMRI) and computed tomography(CT).
Areas covered
We aimed to assess 1) how often the definition of LVHT was validated against a criterion standard, 2) if inter- and intra-observer agreement was assessed, and 3) how often LVHT was associated with genetic diseases. A literature search disclosed 58 cases whose hearts were investigated pathoanatomically and by ≥1 imaging technique. Echocardiography was most frequently(95%) compared with pathoanatomy, followed by cMRI(31%), ventriculography(7%) and CT(5%). Intra- and inter-observer agreement was more frequently assessed for cMRI definitions and yielded more consistent results than for echocardiographic definitions. Since genetic findings were only reported from 4 of the 58 cases, no association with imaging findings could be carried out.
Expert opinion
Correlation between pathoanatomic investigations with imaging techniques will hopefully contribute to reliable and uniformly accepted definitions of LVHT. Most probably, the echocardiographic definition of LVHT will be a synthesis of the currently used definitions, integrating short axis and four-chamber views. A refinement of cMRI definitions, considering pathoanatomic and echocardiographic investigations, seems necessary to avoid overdiagnosis.
Introduction: When >3 trabeculations associated with interventricular recesses are found, this is termed “left ventricular hypertrabeculation/noncompaction”(LVHT). Cardiac-imaging methods detect LVHT in all ages, isolated or associated with extracardiac, especially neuromuscular disorders(NMDs). Many issues about LVHT are unclear. The review gives an update about pathomorphologic findings in patients >14 years and the role of NMDs in LVHT.
Areas covered: A PubMed-search for the terms “noncompaction“ or “non-compaction“ or “hypertrabeculation“ AND “autopsy“ or “biopsy” or “ultrastructure” or “electron microscopy” AND “neuromuscular” or “myopathy” or “neuropathy” was carried out from 1985 to July 2018.
Expert commentary: Macroanatomic (n=65), histopathologic (n= 59) and ultrastructural (n=7) reports were found. A comparison with echocardiography was described in 45 cases. Measurements of non-compacted and compacted layer were only given from hearts investigated in short-axis cuts after formaldehyde-fixation. Endocardial, subendocardial and interstitial fibrosis were frequent findings. When LVHT-patients were systematically investigated, a NMD was found in 80%, most frequently mitochondrial disorders, Barth syndrome, zaspopathy, and myotonic dystrophy type 1. LVHT does not seem to be a special type of cardiac involvement of NMDs. NMDs affect prognosis in LVHT as well as LVHT affects prognosis in patients with Duchenne muscular dystrophy.
Non‐compaction of the left‐ventricular myocardium is an extremely rare cardiomyopathy. The most common clinical manifestations are heart failure, ventricular arrhythmia, thromboembolism, and sudden cardiac death. The condition is diagnosed by two‐dimensional echocardiography or magnetic resonance imaging. We report a rare case of a 55‐year‐old man with coexistence of left ventricular non compaction cardiomyopathy, significant coronary artery disease and massive thrombus formation within recesses in left ventricular cavity.
A 41-year-old female was brought to the ER after a presyncope and absence episode while riding a bike. She recalled no prodromi. Her physical and neurological examinations were unremarkable; past medical, family, substance, and travel history bland. She reported a constant tiredness, sporadic slight cephalgia, responsive to acetaminophen, and a recent syncope while jogging, resulting in a fall, circumstances of which she could not recall. Non-compaction cardiomyopathy is a type of cardiomyopathy that was first described 25 years ago. Its molecular genetic basis is not yet fully clear, and the same is true of its diagnosis, treatment, and prognosis. Further study of these matters is needed.
Left ventricular non-compaction cardiomyopathy (LVNC) is a rare disorder characterized by a thick myocardial wall with two distinct layers consisting of compacted and noncompacted myocardium. The major clinical manifestations of LVNC have been described as heart failure, cardiac arrhythmias, and cardioembolic events. In this report we present a case of a young woman with LVNC who presented with aborted sudden cardiac death. This is the second case of LVNC reported in Puerto Rico, but the first presenting with this complication. Recent advances in the field of cardiology allow the identification and diagnosis of this disease; thus, preventive and treatment strategies could be established for this potentially life-threatening condition. LVNC has to be considered in young patients presenting with sudden cardiac death.
