Noncompaction Of The Ventricular Myocardium Research Articles

Noncompaction of the ventricular myocardium mimicking ischemic cardiomyopathy

A 68-year-old woman was admitted to our hospital because of left ventricular failure. Myocardial perfusion single-photon emission computed tomography demonstrated a non-reversible perfusion defect in the anterolateral left ventricular segments and reduced ejection fraction, findings consisted with ischemic cardiomyopathy accompanied by lateral wall infarction. Coronary angiogram was normal, but the left ventriculogram showed prominent trabeculations in the apical and anterolateral segments. The left ventricular ejection fraction was 28%. Cine magnetic resonance imaging demonstrated prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Myocardial hypoperfusion or necrosis in the noncompacted myocardium may be the cause of myocardial damage and possibly the basis of left ventricular failure.

Elderly manifestation of non-compaction of the ventricular myocardium

Non-compaction of the ventricular myocardium (NCVM) is a rare disorder of myocardial morphogenesis usually diagnosed in paediatric age associated with high mortality rates. Among reported cases, NCVM has been described in only six patients > or = 70 years. We describe the case of a 74-year-old male with NCVM involving the left ventricle, representing one of the oldest patients ever reported in the literature. Of note, this case was characterized by late appearance of NCVM and rapid deterioration of clinical conditions. The wide age range of symptom onset from prenatal to geriatric age supports the hypothesis that NCVM is a multifactorial rather than a simple congenital disorder.

Noncompaction of the ventricular myocardium combined with polycystic kidney disease

Noncompaction of the ventricular myocardium (NVM) is a rare cardiac abnormality of unknown etiology. The condition is characterized by prominent and excessive trabeculations in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Polycystic kidney diseases are characterized by the formation of multiple cysts in the kidneys and liver and, less frequently, in the pancreas. Cardiovascular abnormalities including hypertension, mitral valve prolapse, and intracranial aneurysms are also frequently recognized. However, polycystic kidney disease and isolated ventricular noncompaction have not previously been correlated. Here, we describe one case of isolated noncompaction of ventricular myocardium with polycystic kidney disease, coupled with a progressive worsening of heart failure. We confirmed these abnormalities using contrast echocardiography, abdominopelvic computed tomography, and cardiac magnetic resonance imaging.

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.

Intrafamilial variability of noncompaction of the ventricular myocardium

Noncompaction of the ventricular myocardium (NVM) is a relatively uncommon form of cardiomyopathy characterized by a highly trabeculated myocardium. This report describes the clinical and genetic evaluation of a 3-generation kindred. Family members were initially evaluated by 2-dimensional echocardiography. Most family members with signs of NVM were further evaluated by magnetic resonance imaging. Genetic analyses included mutational screening of the taffazin (TAZ) and alpha-dystrobrevin (DTNA) genes. Eight family members had signs of NVM. Considerable interindividual variation was noted in terms of spatial distribution and severity of affected regions and ventricular dysfunction. Depending on which of 2 previously proposed quantitative diagnostic criteria were used and where ventricular myocardial measurements were taken, between 4 and 7 of these individuals had findings that were considered diagnostic. Magnetic resonance imaging served as a useful adjunct for confirming or establishing diagnoses in all 8 individuals. No mutation was found in TAZ or DTNA. This kindred demonstrates the remarkably wide phenotypic spectrum that can be seen in familial cases of NVM, ranging from prenatal/neonatal lethality to a complete lack of symptoms. The fact that all 8 affected individuals either have shown improvement in ventricular function or symptoms during childhood or have been asymptomatic indicates that NVM can have a relatively benign course. The degree and nature of cardiac involvement are also quite varied, and there is a weak correlation with ventricular function and symptoms. Evaluation of families with NVM requires careful assessment that uses a combination of imaging techniques and diagnostic criteria.

Noncompaction of the ventricular myocardium

Noncompaction of the ventricular myocardium (NCVM) is an as-yet unclassified cardiomyopathy. It is supposedly exceedingly rare,1 but appears to be underdiagnosed.2,3 NCVM is associated with a high incidence of congestive heart failure, arrhythmias, and thromboembolic events.1,3,4 The reported mortality is of concern.1,3,4 The diagnosis is easily missed. Familiarity with the characteristic imaging features is mandatory.

Long-term Survival of a Patient with Isolated Noncompaction of the Ventricular Myocardium

Isolated noncompaction of the myocardium (INCM) is a rare, congenital, unclassified cardiomyopathy. It is caused by a disorder in endomyocardial morphogenesis in the absence of other structural disease. INCM is characterized by numerous prominent trabeculations and deep intertrabecular recesses in the myocardium. Frequently, INCM is associated with an increased incidence of heart failure, arrhythmias, and cardioembolic events with high morbidity and mortality. We describe a 28-year-old woman experiencing symptoms of heart failure since she was 4 years old. She had been intensively investigated and misdiagnosed as having dilated, restrictive, and apical hypertrophic cardiomyopathies. Cardiac magnetic resonance and echocardiography recently revealed the actual diagnosis of INCM. The patient is alive and well, taking vasodilators and warfarin. Herein, we describe the long-term follow-up of this patient and demonstrate that some patients have a favorable prognosis. In addition, the improvement in noninvasive cardiac imaging has revealed a higher prevalence of INCM, previously undetected.

Non-Compaction of the Ventricular Myocardium

Non-Compaction of the Ventricular Myocardium

Demographics and Clinical Characteristics of the Isolated Noncompaction of the Ventricular Myocardium

Background. Echocardiography is a reliable procedure for the diagnosis of the isolated noncompaction of the ventricular myocardium (INVM). We studied the demographics and clinical characteristics of echocardiographically diagnosed INVM.Methods. We analyzed two-dimensional and color Doppler echocardiograms in 16,623 consecutive patients from June 2000 to February 2005.Results. There were 99 INVM patients (68 males) with a prevalence was 0.6% (0.7% in males, 0.5% in females). Ten male patients had left ventricular dysfunction. Twenty-nine male patients and 10 female patients had mitral valve prolapse. Of 95 INVM patients, 38 male patients and 20 female patients had thoracic deformity, and 18 male patients and 7 female patients had thoracic deformity and mitral valve prolapse.Conclusions. Echocardiographically diagnosed INVM is not an uncommon disease. Left ventricular function is almost normal in many patients (86.9%). Thoracic deformity and/or mitral valve prolapse are often associated with INVM.

Cardioverter defibrillator implantation in an adult with isolated noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium is an unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. Fatal 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 isolated noncompaction of the ventricular myocardium in whom implantation of automatic cardioverter defibrillator was thought to be effective to prevent the risk of sudden cardiac death.

Non-Compaction-Kardiomyopathie

Isolated non-compaction of the ventricular myocardium (INVM), also known as left ventricular hypertrabeculation or spongy myocardium, belongs to the "unclassified" cardiomyopathies according to the World Health Organization. The main characteristic of this entity is a prominent trabeculation of the left ventricle with deep intertrabecular recesses communicating with the ventricular cavity. The pathomechanism of INVM is thought to be an arrest in cardiac myogenesis with persistence of embryonic myocardial morphology. The most frequent clinical manifestations include congestive heart failure, ventricular arrhythmias and systemic thromboembolic events. The therapy of INVM comprises standard medical therapy for heart failure.

Isolated Ventricular Non-Compaction in Adults With Idiopathic Cardiomyopathy: Cardiac Magnetic Resonance and Pathologic Characterization of the Anomaly

Non-compaction of the ventricular myocardium is an anomaly of myocardial morphogenesis that leads to persistence of the embryonic myocardium with an excessively prominent trabecular meshwork and deep intertrabecular recesses. This report describes 3 cases of isolated left ventricular non-compaction in adults leading to terminal heart failure. We describe their distinctive myocardial histopathology and prospective diagnosis by cardiac magnetic resonance imaging (CMR). Heart transplantation was the only treatment option for all 3 of these severely ill patients. Isolated ventricular non-compaction should be considered in patients with severe idiopathic cardiomyopathy whose symptoms appear in early adulthood, and consideration given for early transplantation. Non-invasive imaging with CMR can confirm the diagnosis.

Isolated Left Ventricular Noncompaction: A Diagnosis to Consider With Left Ventricular Wall Akinesis

Abstract: A 12-year-old boy presented with isolated noncompaction of the ventricular myocardium (INVM), a rare cardiomyopathy of childhood. Echocardiography revealed spongy myocardium with deep intratrabecular recesses at the left ventricular apex. Gated blood-pool scintigraphy revealed an akinetic lateral wall and apex with a normal ejection fraction.

Clinical characteristics and morphologic features of isolated noncompaction of the ventricular myocardium: electrocardiogram-gated magnetic resonance imaging can elucidate more accurate biventricular structures

Isolated noncompaction of the ventricular myo-cardium (INVM) is a rare congenital disorder characterized by an arrest of myocardial morphogenesis. We experienced a case of a 23-year-old man, referred to our emergency room, whose symptoms included shortness of breath. ECG-gated cardiac magnetic resonance imaging depicted distinctive features of INVM in both the left and right ventricles. We present the clinical characteristics and morphological features of this case in comparison with some recent reports.

Asymptomatic noncompaction myocardium and familial probable partial penetrant disease

Isolated noncompaction of the ventricular myocardium is thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. Clinical manifestations are highly variable, from no symptoms to congestive heart failure, arrhythmias, and systemic thromboemboli. Both familial and sporadic forms have been described, and mutations of certain genes have been related with this entity. Partial penetration of the disease is reported. We report an asymptomatic infant accomplishing the echocardiographic criteria, and his mother, showing prominent trabeculations and recesses, but not fulfilling all the diagnosis criteria.

Noncompaction of Ventricular Myocardium in a Patient with Congenitally Corrected Transposition of the Great Arteries Treated Surgically: Case Report

Noncompaction of the ventricular myocardium is a rare disorder that represents numerous prominent trabeculations and intratrabecular recesses in the ventricles. It is believed to represent not only an arrest in endomyocardial morphogenesis but also an unclassified cardiomyopathy. The pathology has been almost invariably associated with other congenital cardiac malformations. A female patient with noncompaction of the myocardium of both ventricles and congenitally corrected transposition of the great arteries (cTGA), situs inversus totalis, and atrial and ventricular septal defects is described. When she was 7 days old a permanent pacemaker was implanted because of complete heart block. Prazosin (Minipress), an alpha-receptor blocker, was administered, and the cardiac ejection fraction showed a striking increase from 20% to 42%. Despite careful and regular follow-up evaluations, the general condition of the patient slowly worsened. Five months after surgery she died of hepatorenal failure and low cardiac output. This case report is thought to be the first description of congenital complete heart block, cTGA, and situs inversus totalis with noncompaction of the myocardium of both ventricles.

Ultrasonic diagnosis of isolated noncompaction of the ventricular myocardium in adults

To apply echocardiography in diagnosis of isolated noncompaction of ventricular myocardium (INVM) in adults. Six patients with INVM underwent echocardiographic examination using HP5500 ultrasound system with the frequency of 2 approximately equals 4 MHz, and the observation was focused on ventricularmyocardium and endocardium at one-third of the apex. All 6 patients showed typical echocardiographic images characterized by an altered structure of the ventricular myocardium with extremely thickened, hypokinetic segments consisting of two layers: a thin, compacted epicardium and an extremely thickened endocardium, resulting in an extremely thickened ventricular wall. Echocardiography should be the first choice to be applied in diagnosis of INVM, which is a distinct entity of specific cardiomyopathy in adults.

Noncompaction of the myocardium complicated by coronary artery embolism

Isolated noncompaction of the ventricular myocardium is an unusual form of cardiomyopathy resulting from an arrest of compaction of the loosely interwoven meshwork of myofibers that embryologically exist within the myocardium causing multiple trabeculations and deep intertrabecular recesses within the ventricular myocardium. The clinical features associated with ventricular compaction consist of ventricular dysfunction, arrythmias, and systemic and pulmonary thromboemboli. We hereby describe a 30-year-old man with isolated noncompaction of the ventricular myocardium who first presented with an acute coronary artery embolism.

An unusual cause of heart failure identified by echocardiography in an octogenarian

Non-compaction of the ventricular myocardium is a rare congenital cardiomyopathy that carries an unfavorable prognosis. It is associated with a high incidence of progressive heart failure, thromboembolism and malignant arrhythmias. Echocardiography currently remains the imaging modality most commonly used for diagnosis. We describe an unusual case of isolated non-compaction of the left ventricle in an octogenarian male to highlight the need for echocardiography in all patients with suspected heart failure, in order to determine aetiology, prognosis and assess treatment.

Reply: Value of Cardiac Magnetic Resonance Imaging in the Diagnosis of Noncompaction of the Ventricular Myocardium

Reply: Value of Cardiac Magnetic Resonance Imaging in the Diagnosis of Noncompaction of the Ventricular Myocardium