Characteristics and management of cleft mitral valve

OBJECTIVES: We sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV). BACKGROUND: Few studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear. METHODS: We reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded. RESULTS: Twenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients. CONCLUSIONS: A cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.     

Heart failure and diabetes mellitus: epidemiology and management of an alarming association

Diabetes mellitus is a growing epidemic with a prevalence among patients with heart failure (HF) approaching 30%. Diabetes worsens the prognosis of HF, and the pathophysiology is complex and multifactorial. Early detection of subtle alterations in cardiac function by modern tools, such as Doppler echocardiography or brain natriuretic peptide dosage, is thus important in these patients. All drugs known to be effective in HF with systolic dysfunction are also effective in patients with diabetes. Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists also seem particularly useful. Overall, however, little is known about the treatment of diabetic patients with HF, especially in case of preserved systolic function. Ongoing and future trials should help to determine the best treatment for these patients with or without associated diabetes. This review assesses the relationships between diabetes mellitus and HF and discusses the various medical strategies.     

Prenatal diagnosis of anomalous connection of the inferior caval vein to the left atrium associated with common arterial trunk

Anomalous connection of the inferior caval vein to the left atrium is exceedingly rare, and has even been considered by some authors an anatomic and embryologic impossibility. This study demonstrates for the first time the existence of this rare malformation, diagnosed on prenatal echo, and confirmed on post-mortem examination in a 24 WG fetus, in association with a common arterial trunk.