[Anomalous origin of the left coronary artery from the pulmonary artery in infants: clinical features and the perioperative treatment strategies].

OBJECTIVE: To investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).

METHOD: Data of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed. The patients' cardiac function was evaluated with echocardiography during follow-up.

RESULT: Most patients presented with symptoms of heart failure, such as tachypnea, diaphoresis, poor feeding, failure to thrive etc. Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients, endocardial hyperplasia in 5 patients, dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients. The left ventricular ejection fraction (LVEF) was (45.5±13.9)% (25%-77%). The left ventricular fractional shortening (LVFS) was (22.0±7.3)% (12%-38%). Twenty one patients underwent cardiovascular CT scan. Left coronary artery originated from left posterior sinus in 9 patients, from right posterior sinus in 1 patient, from lower main pulmonary artery in 5 patients, from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital, only 1 case who underwent angiography was diagnosed correctly. Two patients were misdiagnosed in 8 patients first-presented in our hospital. Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan. The preoperative therapies included using inotropic agents, diuretics and vasodilators according to cardiac function. Two patients underwent left coronary artery orifice ligation. Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system. Patients of NYHA IV with moderate mitral regurgitation (MR) and NYHA III with severe MR underwent mitral annuloplasty. If LVEF was less than 30% after weaning from cardiopulmonary bypass, blood pressure could not be maintained with medication, or lactates increased progressively, extracorporeal membrane oxygenation (ECMO) was demanded. Two patients showed low cardiac output syndrome immediately after surgical procedures, died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries, duration of ventilation was 7-500 hours, 11 of them were supported with ventilator for less than 60 hours. The mean length of hospital stay was (23.4±13.9) d (8-65 d). The follow-up duration ranged from 1-91 months (median 28.5 months). One case was lost to follow up. The patient died from infection 3 months after discharge. The cardiac functions of the remaining 22 patients were improved. The size of left ventricle of 14 patients recovered to normal. LVEF increased to the normal level in 20 cases. No patient underwent redo procedure.

CONCLUSION: The accurate diagnosis can be made based on history, electrocardiogram, echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants. Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.