Noncompaction ventricular myocardium is an unusual cause of cardiomyopathy. It is association with congenital heart defects, most often with outflow obstructive lesions or coronary anomalies. However, no factor could explain the arrest of development of myocardial structure (isolated form). The pathogenesis of isolated noncompaction is thought to be an arrest in endomyocardial morphogenesis. It has been reported that myocardial noncompaction could present as acquired disease. The most common site of involvement is the left ventricle, with right ventricular involvement being reported in a few cases. In this report, we present a case with noncompaction of the right ventricle (RV). Cardiac computed tomography angiography and magnetic resonance imaging demonstrated morphological abnormalities of the RV.
Angiography ; Cardiomyopathies ; Heart Defects, Congenital ; Heart Ventricles* ; Isolated Noncompaction of the Ventricular Myocardium ; Magnetic Resonance Imaging ; Morphogenesis ; Multidetector Computed Tomography ; Myocardium*

Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy that is thought to be caused by arrest of normal endomyocardial embryogenesis. It is usually diagnosed via echocardiography. The three major clinical manifestations of noncompaction are heart failure, arrhythmia and embolic events. We report here on a case of isolated noncompaction of the ventricular myocardium, and the patient was admitted due to palpitation and dyspnea. Sadly, he died of intractable heart failure despite that we administered intensive medical treatment.
Aged* ; Arrhythmias, Cardiac ; Cardiomyopathies ; Dyspnea ; Echocardiography ; Embryonic Development ; Female ; Heart Failure ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Myocardium ; Pregnancy

Isolated left ventricular noncompaction (LVNC) is a rare cardiomyopathy with morphologic characteristics of two distinct myocardial layers i.e., thin compacted epicardial and thick noncompacted endocardial layers. The noncompacted myocardium consists of prominent ventricular trabeculae and deep intertrabecular recesses. It can lead to arrhythmias, heart failure or systemic embolisms. Electrocardiographic patterns of patients with LVNC are various and non-specific; however, the most common findings are intraventricular conduction delay, left ventricular hypertrophy, and repolarization abnormalities. We reported the first case, to the best of our knowledge, of a 29-year-old man who had recent cerebral infarction and incidental LVNC with spontaneous left atrial standstill.
Adult ; Arrhythmias, Cardiac ; Cardiomyopathies ; Cerebral Infarction ; Electrocardiography ; Embolism ; Heart Atria* ; Heart Failure ; Humans ; Hypertrophy, Left Ventricular ; Isolated Noncompaction of the Ventricular Myocardium ; Myocardium ; Stroke*

Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.
Adult* ; Biopsy ; Cardiomyopathies ; Diagnosis ; Echocardiography ; Electrocardiography ; Embryonic Development ; Female ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; Magnetic Resonance Imaging ; Male ; Myocardium* ; Pregnancy ; Syncope ; Tachycardia, Ventricular* ; Young Adult

Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.
Adult* ; Biopsy ; Cardiomyopathies ; Diagnosis ; Echocardiography ; Electrocardiography ; Embryonic Development ; Female ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; Magnetic Resonance Imaging ; Male ; Myocardium* ; Pregnancy ; Syncope ; Tachycardia, Ventricular* ; Young Adult

OBJECTIVE: To retrospectively summarize the cardiac magnetic resonance imaging (CMRI) findings of isolated noncompaction of ventricular myocardium (INVM). MATERIALS AND METHODS: Eleven patients (M:F = 9:2; mean age, 35 years) were evaluated. Steady-state free precession (SSFP), fast spin echo (SE) sequence, SSFP cine imaging, and delayed enhanced inversion recovery spoiled gradient echo (IR-SPGR) sequence were used for showing abnormal myocardium, measuring ratio of noncompacted/compacted myocardium layers (NC/C ratio), and detecting myocardial viability. The left ventricle was divided into nine segments and a NC/C ratio > 2.3 in diastole was used as cutoff value in diagnosing left INVM. The right ventricle was assessed qualitatively. RESULTS: Cardiac MRI indicated left INVM in seven patients, right INVM in one patient and biventricle INVM in three patients. Characteristic CMRI changes included prominent trabeculations, deep intertrabecular recesses and an increase in the NC/C ratio. The most frequently involved segments was left ventricular apex. Three patients had abnormal high signals within the trabecular structures on SE T2 weighted image. One ventricular aneurysm and one apical thrombus were also observed. Delayed enhancement was seen in six of nine patients with subendocardial and transmural patterns. CONCLUSION: There are CMRI features that might be characteristic for INVM.
Adolescent ; Adult ; Female ; Heart Aneurysm/complications/diagnosis ; Heart Ventricles/pathology ; Humans ; Isolated Noncompaction of the Ventricular Myocardium/complications/*diagnosis ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Myocardium/pathology ; Young Adult

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cyanosis ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Heart Murmurs ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Mass Screening ; Pulmonary Atresia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Trabecular Meshwork ; Ventricular Septum ; Wolff-Parkinson-White Syndrome

OBJECTIVETo investigate the clinicopathologic manifestations of non-compaction of ventricular myocardium (NVM).

METHODSClinical data, electrocardiograms, echocardiography images and pathologic changes were studied in five cases of non-compaction of ventricular myocardium.

RESULTSThe patient's ages ranged from 29 to 57 years old, all were males. Abnormal electrocardiograms were obtained in all of the 5 cases. Among them, 3 were diagnosed using echocardiography. Histopathologic examination showed that there were abnormally coarse muscle trabeculation and deep recesses, interlacing in arrangement, over the inner wall of the heart chambers. The compacted myocardium became thinning down gradually from the base to the apex of the heart. The non-compacted myocardium bundles locating close to the endocardium were coarse and orderless in arrangement, nuclei were irregular and abnormal, nevertheless, the arrangement and appearance of the muscle bundles near by the pericardium part were essentially normal and the cell nuclei were evenly distributed.

CONCLUSIONThere are no specific clinical manifestations obtained in patients with non-compaction of ventricular myocardium, however, the pathologic changes are characteristic and a clinical diagnosis can be made by using echocardiography.

Adult ; Cardiomyopathy, Dilated ; diagnostic imaging ; pathology ; Cardiomyopathy, Hypertrophic ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Electrocardiography ; Heart Ventricles ; pathology ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Myocardium ; pathology ; Ultrasonography

Left ventricular hypertrabeculation/noncompaction (LVHT) is an uncommon type of genetic cardiomyopathy characterized by trabeculations and recesses within the ventricular myocardium. LVHT is associated with diastolic or systolic dysfunction, thromboembolic complications, and arrhythmias, including atrial fibrillation, ventricular arrhythmias, atrioventricular block and Wolff-Parkinson-White syndrome. Herein, we describe a patient who presented with heart failure and wide-complex tachycardia. Echocardiography showed LVHT accompanied with severe mitral regurgitation. The electrophysiologic study revealed a fasciculo-ventricular accessory pathway and atrial flutter (AFL). The AFL was successfully treated with catheter ablation.
Arrhythmias, Cardiac ; Atrial Fibrillation ; Atrial Flutter ; Atrioventricular Block ; Cardiomyopathies ; Catheter Ablation ; Echocardiography ; Heart Failure ; Humans ; Isolated Noncompaction of the Ventricular Myocardium ; Mitral Valve Insufficiency ; Myocardium ; Pre-Excitation, Mahaim-Type ; Tachycardia ; Wolff-Parkinson-White Syndrome

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by a hypertrabeculation of the left ventricle. Patients may present with heart failure, arrhythmia, and thromboembolism. LVNC may be isolated or associated with congenital heart defects. The first discovered genetic cause of isolated LVNC was Barth syndrome (BTHS), an X-linked disorder caused by taffazin (TAZ) gene mutation. BTHS is characterized by cardiomyopathy, neutropenia, skeletal myopathy, and growth delay. A newborn male baby was referred to Soonchunhyang University Cheonan Hospital due to cyanosis and dyspnea. Based on findings of cardiomegaly, ST depression, and elevated cardiac enzyme, echocardiography was done, which revealed a hypocontractile, enlarged left ventricle with distinctive trabeculation in the apex. Heparinization for the treatment of myocardial infarction and continuous infusion of milrinone was started. During hospitalization, the TAZ gene mutation was detected in the patient, his mother, and elder sister. After 3 months, the patient was discharged with heart failure medication and aspirin.
Arrhythmias, Cardiac ; Aspirin ; Barth Syndrome* ; Cardiomegaly ; Cardiomyopathies ; Chungcheongnam-do ; Cyanosis ; Depression ; Dyspnea ; Echocardiography ; Heart Defects, Congenital ; Heart Failure ; Heart Ventricles ; Heparin ; Hospitalization ; Humans ; Infant, Newborn* ; Isolated Noncompaction of the Ventricular Myocardium ; Male ; Milrinone ; Mothers ; Muscular Diseases ; Myocardial Infarction* ; Neutropenia ; Siblings ; Thromboembolism