Aberrant left coronary artery from the pulmonary artery with patent ductus arteriosus - a case report and review of the literature.

BACKGROUND: Aberrant left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect. Its coexistence with patent ductus arteriosus (PDA) is extremely rare. The high pressures created by the left-to-right shunt in the pulmonary arteries can delay symptoms and create a real challenge in diagnosing ALCAPA. Missing this diagnosis can have severe results, including extensive ischemia and sudden death. CASE PRESENTATION: We present a case of an infant born with a large PDA. Initially treated conservatively, however, due to congestive heart failure and lack of weight gain, she underwent surgical ligation of the PDA at the age of four and a half months. Following surgery, she developed pulmonary edema. Echocardiography revealed decreased ventricular function. ECG revealed ST elevations on lateral leads, and serum troponin was significantly increased. The patient underwent cardiac magnetic resonance imaging (MRI), which revealed signs of wall ischemia and decreased function of the left ventricle (LV) with unclear coronary anatomy. Diagnostic catheterization revealed an ALCAPA. She underwent surgical intervention, and the left coronary artery was re-implanted in the aortic sinus. Follow-up revealed slow improvement of cardiac function. DISCUSSION AND LITERATURE REVIEW: The coexistence of PDA and ALCAPA is a very rare occurrence. We found at least 10 reported cases in the literature. Delayed diagnosis might be detrimental. The prognosis of these patients is variable. CONCLUSION: An unusual post-surgical course following PDA repair requires a high index of suspicion and appropriate evaluation for ALCAPA, preferably with angiography.

Extracorporeal membrane oxygenation in pediatric patients: our experience in the last ten years.

BACKGROUND: The use of extracorporeal membrane oxygenation (ECMO) in children after cardiac surgery is well established. ECMO support is becoming an integral tool for cardiopulmonary resuscitation in specified centers. OBJECTIVES: To review our use of ECMO over a 10 year period. METHODS: All children supported with ECMO from 2000 to 2010 were reviewed. Most of these children suffered from cardiac anomalies. The patients were analyzed by age, weight, procedure, RACHS-1 when appropriate, length of support, and outcome. RESULTS: Sixty-two children were supported with ECMO; their median age was 3 months (range 0-216 months) and median weight 4.3 kg (range 1.9-51 kg). Thirty-four patients (52.3%) needed additional hemofiltration or dialysis due to renal failure. The children requiring ECMO support represented a wide spectrum of cardiac lesions; the most common procedure was arterial switch operation (27.4%, n=17). ECMO was required mainly for failure to separate from the heart-lung machine (n=55). The median duration of ECMO support was 4 days (range 1-14 days); 29 (46.7%) patients were weaned successfully from ECMO during this time period, and 5 of them died during hospitalization, yielding an overall hospital survival rate of 38.7%. CONCLUSIONS: ECMO support has significant survival benefit for patients with post-cardiotomy heart failure. Its early deployment should be considered in cardiopulmonary resuscitation.