Prenatal diagnosis of isolated total anomalous systemic venous connection to the left atrium.

We report the prenatal diagnosis and the neonatal follow-up of a patient with isolated total abnormal systemic venous connection to the left atrium. Right-sided and left-sided superior caval veins and the inferior caval vein were all connected to the left atrium. Pulmonary venous return was normal. This was associated with some right ventricular underdevelopment. To our knowledge, this is the first fetal description of this very rare congenital cardiac malformation.

Impact of exercise pulmonary hypertension on postoperative outcome in primary mitral regurgitation.

AIMS: The management of asymptomatic patients with mitral regurgitation (MR) remains controversial. Exercise-induced pulmonary hypertension (ExPHT) was recently reported as a strong predictor of rapid onset of symptoms. We hypothesised that ExPHT is a predictor of postoperative cardiovascular events in patients with primary MR. METHODS AND RESULTS: One hundred and two patients with primary MR, no or mild symptoms (New York heart association (NYHA) ≤2), and no LV dysfunction/dilatation, were prospectively recruited in 3 centres and underwent exercise-stress echocardiography. The presence of ExPHT was defined as an exercise systolic pulmonary arterial pressure >60 mm Hg. All patients were closely followed up and operated on when indication for surgery was reached. Postoperative events were defined as the occurrence of atrial fibrillation (AF), stroke, cardiac-related hospitalisation or death. Among the 102 patients included, 59 developed ExPHT (58%). These patients were significantly older than those without ExPHT (p=0.01). During a mean postoperative follow-up of 50±23 months, 28 patients (26%) experienced a predefined cardiovascular event. Patients with ExPHT had significantly higher rate of postoperative events (39% vs 12%, p=0.005); the rate of events was still higher in these patients (32% vs 9%, p=0.013), even when excluding early postoperative AF (ie, within 48 h). Event-free survival was significantly lower in the ExPHT group (all events: 5-year: 60±8% vs 88±5%, p=0.007, events without early AF: 5-year: 67±7% vs 90±4%, p=0.02). Using Cox multivariable analysis, ExPHT remained independently associated with higher risk of postoperative events in all models (all p≤0.04). CONCLUSIONS: ExPHT is associated with increased risk of adverse cardiac events following mitral valve surgery in patients with primary MR.