Right ventricular involvement in a patient with isolated noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium (INVM) is an unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. Although right ventricular involvement is not uncommon, the correct diagnosis is often difficult by echocardiography. In this report, we describe a patient with INVM in whom magnetic resonance imaging was useful to detect right ventricular morphological and functional abnormalities.     

Familial isolated noncompaction of the left ventricular myocardium.

Noncompaction of the ventricular myocardium (sometimes referred to as 'spongy myocardium') is believed to represent an arrest in endomyocardial morphogenesis. The gross anatomical appearance is characterized by numerous excessively prominent trabeculations and deep intertrabecular recesses. Distinct morphological features can be diagnosed on two-dimensional echocardiography. We present here a family of isolated noncompaction of the left ventricular myocardium, in which 5 affected individuals suggested the presence of some genetic abnormalities in this disorder.     

Left ventricular aneurysm associated with isolated noncompaction of the ventricular myocardium

A 66-year-old woman was admitted to our hospital because of left ventricular failure and nonsustained ventricular tachycardia. Two-dimensional echocardiography demonstrated prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Myocardial perfusion scintigraphy demonstrated a defect in the anterobasal left ventricular segment. Coronary angiogram was normal, but the left ventriculogram showed an aneurysm in the anterior myocardial segments. This is the first reported case with isolated noncompaction of the ventricular myocardium associated with left ventricular aneurysm.     

Cardiac resynchronization and cardioverter defibrillation therapy in a patient with isolated noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium (INVM) is an unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. Left ventricular failure and ventricular arrhythmias may occur in approximately half of the patients and account for half of the death in this disorder. In this report, we describe a patient with INVM in whom cardiac resynchronization and cardioverter defibrillation therapy was effective for the improvement of left ventricular function and for the prevention of ventricular arrhythmias.     

Adult case of isolated noncompaction of left ventricular myocardium revealed at hospitalization for tuberculous pleuritis

A 48-year-old man was diagnosed with isolated noncompaction of the left ventricular myocardium. He had been suffering from dyspnea during light exercise since early February 1997, which worsened with time. Eventually, he visited our hospital on February 14. He was admitted urgently because orthopnea was observed and chest radiogram showed massive left pleural effusion. The diagnoses were pulmonary tuberculosis and tuberculous pleuritis. Echocardiography at admission showed generalized hypokinesis of the left ventricle, so we suspected that his condition was complicated by myocarditis. However, virus antibody levels were not elevated, and no obvious findings compatible with myocarditis or cardiomyopathy were obtained by right ventricular myocardial biopsy. Left ventricular contractility remained low and a trabecular mesh structure was seen at the left ventricular apex. Thus, the diagnosis was isolated noncompaction of the left ventricular myocardium. This disorder has been highlighted in pediatric patients, but few adult cases have been reported.     

Isolated noncompaction of the left ventricular myocardium in an elderly patient.

Noncompaction of the ventricular myocardium (NVM) is a rare disorder of endomyocardial morphogenesis characterized by numerous, prominent trabeculations and deep intertrabecular recesses. It is commonly associated with congenital heart disease, but the isolated form (INVM) is not associated with other structural heart diseases. Clinical reports of INVM have been limited to a few case reports and small series of pediatric patients. INVM is considered to be a form of congenital abnormal endomyocardial morphogenesis caused by abnormal cessation of the embryonic development of the ventricular myocardium; most reported cases have been pediatric patients, and autopsy cases of elderly patients have been quite rare. In the present case, an elderly female had INVM associated with severely disturbed left ventricular (LV) function and an enlarged left ventricle similar to dilated cardiomyopathy. The echocardiogram showed prominent trabeculations and deep intertrabecular recesses of the LV walls, especially in the posterior and apical areas. LV contrast echocardiography revealed markedly protruberant trabeculations, which were also observed with computed tomography. Five years later, the patient died of refractory heart failure and ventricular fibrillation. The autopsy revealed numerous excessively prominent trabeculations in the LV myocardium, with deep intertrabecular recesses containing thrombi.     

Stroke and ventricular dysfunction in a patient with isolated left ventricular noncompaction

A 44-year-old woman developed a cardioembolic stroke. Transthoracic echocardiography demonstrated isolated noncompaction of the ventricular myocardium. Left ventricular systolic function was mildly depressed, which severely decreased during 3 months after discharge. The embolic stroke might occur when the ventricular systolic function had begun to deteriorate. The proper time to start anticoagulation in isolated noncompaction of ventricular myocardium patients may be when left ventricular systolic function decreases below normal.     

Preserved systolic function with isolated left ventricular noncompaction in an elderly patient.

Isolated left ventricular noncompaction (IVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. Clinical presentations of IVNC include systolic and diastolic dysfunction, systemic embolism and ventricular arrhythmias. In recent years there has been an increasing awareness of this anomaly; however, especially in elderly, clinical characterization and natural course of IVNC are still in question. In this case, we report a case of a 78-year-old patient with the diagnosis of IVNC with preserved left ventricular (LV) systolic function. To the best of our knowledge, this is the oldest IVNC case with preserved LV systolic function in the literature.     

Perforation of the right ventricular free wall by an ICD lead in a patient with isolated noncompaction of the ventricular myocardium

A 74-year-old patient with isolated noncompaction of the ventricular myocardium (INVM) underwent implantable cardioverter defibrillator (ICD) implantation because of sustained ventricular tachycardia. The post operative course was complicated by perforation of the right ventricular free wall by the ICD lead. The type of the active fixation transvenous ICD lead and the type of background cardiac disease are thought to be major risk factors for perforation.     

Isolated noncompaction of the ventricular myocardium in a patient presenting with subarachnoid hemorrhage

ObjectiveHyperhomocysteinemia and protein C deficiency have synergistic effect on the onset of thrombotic disease.MethodsWe report a 42-year old woman with myocardial infarction and venous thrombosis in whom recognition of heterozygous MTHRF gene mutation, hyperhomocysteinemia, and protein C deficiency.ResultsThe patient was treated successfully with coronary artery bypass graft surgery and systemic anticoagulation.ConclusionsOur report emphasize that a combined hyperhomocysteinemia, and protein C deficiency may be a high risk factor for arterial and venous thromboembolic events in young adults. These patients might be candidates for indefinite anticoagulation.     

Isolated noncompaction of ventricular myocardium

Isolated noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The diagnosis can be made echocardiographically, and the entity may be associated with problems of cardiac rhythm. We describe two illustrative cases, with Wolf-Parkinson-White syndrome in one, and left bundle branch block in the other. We emphasize that children with problems of rhythm and findings suggestive for left ventricular cardiomyopathy, either hypertrophic or dilated; should be investigated to exclude isolated noncompaction of the ventricular myocardium.     

Adult case of isolated ventricular noncompaction discovered by complete atrioventricular block.

A 25-year-old male was admitted to hospital with a 3-day history of worsening faintness. The electrocardiogram showed complete atrioventricular block. Echocardiography showed generalized hypokinesis and prominent trabeculations ranged from the apex to the mid-ventricular lateral wall of the left ventricle as well. Furthermore, trabeculations in the left ventricle were seen in his sister and brother. Thus, isolated ventricular noncompaction (IVNC) was diagnosed and a permanent pacemaker was implanted. Common clinical symptoms of IVNC are heart failure, ventricular arrhythmias, and embolic events. This is the first reported adult case of IVNC disclosed by the presence of complete atrioventricular block.