LVNC is a genetic cardiac disease of emerging importance with a distinct clinical and pathophysiological presentation. The diagnosis of LVNC, however, is often missed, most often as a consequence of ignorance of the condition. Echocardiography is considered the reference standard for the diagnosis of LVNC. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. Because of the familial association of LVNC, first degree relatives should be screened by Echocardiography.

Background: Congenital ventricular diverticula, defined as a protrusion of the free wall of the ventricle including the endocardium, the myocardium, and the pericardium, behave similarly to an accessory ventricular chamber which contracts synchronously with the normal ventricles. Case Presentation: A 42-year-old man presented with functional class II exertional dyspnea, fatigue, and flushing. Transthoracic echocardiography showed deep recesses with the outpouching of the basal-to-mid free wall and septal hyperkinesia. All the echocardiographic data were highly suggestive of isolated right ventricular (RV) noncompaction. Magnetic resonance imaging revealed multiple large outpouchings in the RV free wall, the RV outflow tract, and the basal anterior left ventricular wall, which had a wide neck and a normal contractility. The left ventricular myocardium was hypertrabeculated but did not fulfill the noncompaction criteria of cardiac magnetic resonance imaging. Conclusions: A muscular type of diverticula with prominent trabeculation and normal contractility, but without abnormalities, on both perfusion and gadolinium enhancement images was reported here. Such cases should not be mistaken for noncompaction or pseudoaneurysms.

BACKGROUND: Although isolated left ventricular noncompaction (ILVNC) has been described almost two decades ago, our knowledge about its diagnosis, presentation, echocardiographic features and clinical outcome is sparse. We aimed to assess the echocardiographic and clinical characteristics of ILVNC in a group of patients referred to our center.METHODS: Patients who were referred to a tertiary referral center, affiliated with Mashhad University of Medical Sciences, with primary diagnosis of dilated cardiomyopathy underwent comprehensive echocardiographic evaluation. The diagnosis of ILVNC was made based on the presence of two-structural layer in myocardium; ratio of noncompacted to compacted layers more than 2, and excessive trabeculation in the left ventricle.RESULTS: Final diagnoses of ILVNC were made in 42 patients. Mean age of patients was 32.9±15.6 years (ranging from 9 to 70 years). Females comprised a higher proportion of patients (61.9%) and shortness of breath was the most reported symptom among patients (47.6%). Non-compacted layers were detected in inferior and lateral segments of apex in 97.6% of patients. A total of 26 (61.9%) patients had left ventricle (LV) dysfunction (defined as ejection fraction less than 50%). The only factor that showed significant association with LV dysfunction was the number of affected segments with noncompaction (P=0.008). Reduced ejection fraction was not associated with either age or sex (P=0.437 and P=0.206, respectively).CONCLUSION: Based on the result of the current study, it can be suggested that apex of the heart is the most common site of noncompaction and increasing numbers of affected segments might be associated with LV dysfunction.

Background-NON-COMPACTION of ventricular myocardium (LVNC), also known as LVHT (left ventricular hypertrabeculation), is a rare embryonic cardiomyopathy that is thought to be a consequence of intrauterine arrest of compaction. It is characterized by an excessively prominent trabecular meshwork, which is accompanied by depressed ventricular function, systemic embolism and ventricular arrhythmia. This study was conducted to clarify the clinical features of patients with left ventricular noncompaction (LVNC) who were diagnosed in Shahid Rajaei Cardiovascular Medical Center.Methods and Results- We retrospectively reviewed patients with LVNC between December 2004 and December 2005. A total of twenty-four patients were identified. In 4 patients there were associated cardiac lesions. They consisted of 6 females and 18 males with a mean age of 38.2 years (age range: 13-62 years). The average ejection fraction was 23.3%.The extension of noncompacted myocardium that was observed on 2-D echocardiography, was predominantly at the apex. There were two patients with systemic emboli, one with nonsustained ventricular tachycardia (VT), and one with Wolf-Parkinson-White sydnrome. The most common abnormality in the the electrocardiogram was left bundle branch block.Conclusions- LVNC is most frequently diagnosed primarily by echocardiography and its prevalence seems to be increased with the improvement of cardiac imaging; so echocardiographers should be aware and trained to recognize this abnormality.

Dear Editor, With interest we read the article by Mohammadpour Ahranjani et al. about a nine-year-old girl with heart failure due to systolic dysfunction and left ventricular hypertrabeculation/ noncompaction (LVHT) in whom the association with skin abnormalities led to the diagnosis of Caravajal syndrome (CS) (1). We have the following questions and concerns: mutations in the desmoplakin genes have been identified insomepatients with CS. However, there are also CS-cases in whom no mutations of the desmoplakin gene have been found thus indicating genetic heterogeneity (2).It would be interesting to know if genetic studies were carried out in the presented patient