Ventricular dysfunction and number of non compacted segments in non compaction: non-independent predictors.

BACKGROUND: Isolated ventricular noncompaction (IVNC) is characterized by multiple prominent trabeculations and deep intertrabecular recesses. Some reports prove that the chronic heart failure may occur in approximately half of the patients. In this report we investigate the correlation between the number of non compacted segments and entity of systolic dysfunction from the registry and subregistries of the SIEC. METHOD: To identify the correlation between ventricular dysfunction and number of segments involved in non compaction we evaluated a consecutive series of 238 patients affected by non compaction, from the SIEC (Società Italiana di Ecografia Cardiovascolare) registry. The average age of patients was 41.5 years (range: 1-92 years), 137 were males and 101 females. In 122 cases we found ventricular systolic dysfunctions with an EF average of 34.6%. The number of affected segments by non-compactation and diastolic dysfunction were found to be non-independent predictors of LV systolic dysfunction. CONCLUSION: From the analyses we carried out, it seems that ventricular dysfunction seems to be completely independent from the segment numbers of non compacted segments.

Cardiac Abnormalities of Sudanese Patients with Down's Syndrome and their Short-Term Outcome : Cardiovascular Topics

Background : Congential heart disease (CHD) is an important cause of morbidity and mortality in patients with Down's syndrome (DS). Methods : All patients with DS seen at the Sudan Heart Centre from July 2004 to November 2007 were included in the study. All patients were examined clinically and echocardiographically; and cardiac catheterisation was carried out in selected patients. All patients were prospectively followed up. Results : In the study period; 1 566 patients were evaluated forheart disease. Of these; 80 patients with DS were identified (5). Their ages ranged from 15 days to 18 years. Cardiac abnormalities included atrioventricular septal defect (AVSD) in 38 patients (48); with the complete form in 25; a partialform in seven; AVSD with intact atrial septum in one; and complex AVSD in four patients. In one patient there was AVSD with right atrioventricular valve malformation with severe valve regurgitation and functional pulmonary atresia. The other main lesions were ventricular septal defect (VSD) in 19 patients (23) and tetralogy of Fallot (TOF) in five (6). Cardiac catheterisation was done in four patients with AVSD to measure pulmonary pressures and resistance; and in one patient with patent ductus arteriosus for device closure. Ten percent of the patients had Eisenmenger's syndrome at the time of presentation. Only 15of patients who were in need of surgery were operated on ; all had an uneventful postoperative course and a good outcome at a mean follow-up period of one year. Conclusion : The pattern of CHD in Sudanese patients with DS was comparable with that in the literature; including the rare occurrence of AVSD with intact atrial septum. In addition; we described an unreported association with right atrioventricular valve malformation. Although there was a significant delay in diagnosis and surgery; surgical results and short-term follow up were good

Anticoagulant drugs in noncompaction: a mandatory therapy?

BACKGROUND: Noncompaction of left ventricular myocardium is a rare congenital cardiomyopathy resulting from an incomplete myocardial morphogenesis that leads to the persistence of the embryonic myocardium. This condition is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. It is not clear, in noncompaction of myocardium, whether intertrabecular recesses could be responsible for thrombi formation and thromboembolic complications. METHODS: The prevalence of stroke and echocardiographic finding of thrombus was evaluated in a continuous series of 229 patients (men and women) affected by noncompaction of the left ventricular myocardium, who were included in the SIEC registry. We excluded patients affected by atrial fibrillation. RESULTS: The mean age of the patients was 49.5 years. Fifty percent of the patients were affected by a ventricular systolic dysfunction. The mean period of follow-up was 7.3 years. Only four patients had a history of ischemic stroke. A large thrombus into the left ventricular chamber was observed in a 1-year-old child affected by Behcet's disease (high risk of thrombi formation). CONCLUSION: Noncompaction of the left ventricular myocardium, by itself, does not seem to be a risk factor for stroke or embolic results, so there is no indication for oral anticoagulant therapy.

Supraventricular arrhythmias in noncompaction of left ventricle: is this a frequent complication?

BACKGROUND: Isolated left ventricular noncompaction is the result of incomplete myocardial morphogenesis, leading to persistence of the embryonic myocardium. The condition is recognised by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Whether these intertrabecular recesses are a favorable substrate for supraventricular arrhythmias is unclear, even if the incidence of chronic heart failure seems to be high. RESULTS: We evaluated a continuous series of 238 patients affected by noncompaction. In 4 cases the patients reported palpitations and in 4 an episode of syncope. Periodic holter monitoring was performed every 6 months for 4 years. Only 9 patients had documented atrial fibrillation. In no cases we observed supraventricular tachycardia. CONCLUSIONS: Noncompaction alone does not seem to be a risk factor for supraventricular arrhythmias.

Ventricular tachycardia in non-compaction of left ventricle: is this a frequent complication?

BACKGROUND: Isolated left ventricular non-compaction is the result of incomplete myocardial morphogenesis, leading to persistence of the embryonic myocardium. The condition is recognized by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Whether these intertrabecular recesses are a favorable substrate for ventricular arrhythmias is unclear. Some reports have found that the fatal ventricular arrhythmias may occur in approximately half of the patients. In this report we investigated about this association. METHODS AND RESULTS: In total we evaluated a continuous series of 238 patients affected by non-compaction. Periodic Holter monitoring was performed every 6 months for 4 years. Only 11 patients had documented ventricular tachycardia, which was sustained in two cases and non-sustained in nine. In no cases we observed ventricular fibrillation. CONCLUSIONS: Non-compaction alone does not seem to be a risk factor for malignant ventricular arrhythmias.

Echocardiographic differentiation of atrioventricular septal defects from inlet ventricular septal defects and mitral valve clefts.

Left ventricular inlet/outlet ratio and percent left atrioventricular valve (AVV) guarded by the posterior leaflet are valuable for characterizing atrioventricular septal defect (AVSD). We performed these measurements echocardiographically in normal patients (n = 101), patients who had AVSD (n = 37), those who had isolated perimembranous inlet ventricular septal defect (VSD; n = 12), and those who had "isolated" mitral valve clefts (n = 5). Surgical findings of 38 patients were reviewed. The normal inlet/outlet ratio was 1.03 +/- 0.07. For patients who had AVSD, the ratio was 0.82 +/- 0.06 (95% confidence interval [CI] 0.180 to 0.237, p = 0.001). For patients who had an isolated inlet VSD, this ratio was lower than that in normal patients (0.93 +/- 0.08, 95% CI 034 to 0.116, p = 0.001) but higher than that in patients who had AVSD (95% CI -0.175 to 0.091, p = 0.001). The percent left AVV guarded by the posterior leaflet was 56 +/- 1.4 in normal patients. For patients who had AVSD, it was significantly lower (43 +/- 1.1). For patients who had an isolated VSD and those who had an isolated mitral cleft, it was normal (57 +/- 1.9 and 55 +/- 2.2, respectively). The 2 measurements are valuable in differentiating AVSD from inlet VSD and isolated mitral cleft, but percent left AVV guarded by the posterior leaflet is more specific.