Aortopulmonary window. Experience of eleven cases.

Aortopulmonary (AP) window is a communication between the ascending aorta and the main pulmonary artery, in the presence of two separate arterial valves arising from separate subarterial ventricular outflow tracts. It is a rare anomaly that accounts for approximately 0.1% to 0.2% of all congenital heart disease. The purpose of this study was to assess clinical features, surgical treatment and outcome of patients with aortopulmonary window referred to a tertiary pediatric cardiac center over a 30-year period. Eleven patients were diagnosed with AP window, ten with the proximal type and one with the distal type defect. Age at first evaluation ranged from three days to 13 years (mean 44.5+/-63.3 months; median three months). Echocardiography enabled correct diagnosis in the four most recent cases. Seven patients underwent closure of the AP window through a transaortic approach and three patients underwent ligation. Simultaneously, significant associated cardiac anomalies were corrected in three patients: correction of interrupted aortic arch in two patients and closure of ventricular septal defect and Dacron patch enlargement of the right ventricular outflow tract in one patient. One patient was not operated because of fixed high pulmonary vascular resistance. Operative mortality was 10% (1/10) and there were no late deaths. Mean follow-up was 10+/-4.9 years. All nine surviving operated patients are asymptomatic, without medication, with no residual defects and without pulmonary hypertension. In conclusion, advances in diagnostic and surgical approaches to AP window in recent years have enabled earlier intervention with good outcome.

Sotalol in the treatment of fetal tachyarrhythmia.

BACKGROUND: Fetal tachycardia is an uncommon condition that if sustained may lead to fetal death. There is no consensus regarding the optimum treatment. The aim of this study was to evaluate the safety and efficacy of sotalol in the treatment of fetal tachycardia. METHODS: This was a retrospective study of patients treated for fetal tachycardia with sotalol in a pediatric cardiology department over a ten-year period. RESULTS: There were eight fetuses treated for supraventricular tachycardia and sotalol was used in six of them. Mean gestational age was 30 weeks. None of them had congenital heart disease; two fetuses had hydrops and one had hydrocephalus. All had supraventricular tachycardia and two had atrial flutter. Drug treatment was successful in establishing sinus rhythm in five of the six fetuses treated with sotalol and no adverse effects were recorded in the mothers. There were no deaths. Supraventricular tachycardia was present in three infants at birth. CONCLUSIONS: In this study sotalol was successful and safe in the treatment of fetal tachycardia, but given the small sample size, more studies are needed to validate this conclusion.

Hypoplastic left heart syndrome with in utero closed foramen ovale: case report.

We report a case of a newborn with a prenatal diagnosis of hypoplastic left heart syndrome (HLHS), referred at 37 weeks of gestation. The fetal echocardiogram had shown classic morphologic aspects of HLHS with patent mitral valve and aortic valve atresia. The atrial septum was thickened and the foramen ovale (FO) appeared to be patent. It was a normal full-term delivery, with birth weight of 2540 g. Orotracheal intubation and mechanical ventilation were immediately necessary, as well as intravenous prostaglandin, due to clinical deterioration. Transthoracic echocardiography performed in the first hour of life confirmed the diagnosis, but doubts were raised about the patency of the FO. Cardiac catheterization was performed and Rashkind balloon atrial septostomy was attempted, without success. The baby died and pathologic examination revealed HLHS with intact atrial septum, dilated coronary sinus and pulmonary lymphangiectasia. In this case, the dilated coronary sinus led to a mistaken prenatal diagnosis of FO patency with left-to-right shunt.

Subaortic stenosis following anatomic correction for transposition of the great arteries.

The authors present a case report of a 12-year-old boy with diagnosis of transposition of the great arteries and ventricular septal defect, who underwent an arterial switch operation plus closure of the septal defect. On follow-up, left ventricular outflow tract obstruction was detected, initially mild. Last year, he started complaining of fatigue on exercise. Severe subaortic stenosis was diagnosed and surgical repair was performed with good results. Subaortic stenosis is a rare complication on follow-up of patients who have undergone an arterial switch operation, particularly those with transposition of the great arteries and ventricular septal defect.

Permanent junctional reciprocating tachycardia: an incessant tachycardia in children.

UNLABELLED: Permanent junctional reciprocating tachycardia is an infrequent form of reentrant supraventricular tachycardia but is the commonest incessant tachycardia in childhood. The persistent nature of the tachycardia can lead to heart failure secondary to left ventricular dysfunction and the arrhythmia is often refractory to drug control. STUDY OBJECTIVE: To review the main clinical features and therapeutic options currently available for this arrhythmia. DESIGN: Retrospective study. PATIENTS: A group of 5 children with a diagnosis of permanent junctional reciprocating tachycardia. RESULTS: Age at presentation ranged between 14 days and 12 years. Three were asymptomatic. The first echocardiogram displayed left ventricular dilation and decreased shortening fraction in one child. Pharmacological therapy was initially successful in all. During follow-up (0.2-4.5 years) the arrhythmia became resistant to medication in one case and radiofrequency ablation was carried out. CONCLUSIONS: Permanent junctional reciprocating tachycardia has a wide range of clinical presentations. Pharmacological therapy is recommended, but radiofrequency ablation is the definitive treatment and only depends on patient age.