A female infant was born with a large PDA; she developed early congestive heart failure and was initially treated conservatively with diuretics, ACE inhibitors, and high-caloric feeding, and followed up in the pediatric cardiology clinic.
Repeat echocardiography showed a PDA 4–5 mm in diameter with a left-to-right shunt across and a low-pressure gradient. The left atrium, left ventricle, and pulmonary arteries were all dilated, as expected. Cardiac function was normal and hyperdynamic in some tests. The coronaries were reported to be normal in origin and course in multiple tests by experienced sonographists and pediatric cardiologists.
The original management plan was to allow the patient to gain enough weight, making trans-catheter device occlusion of the PDA feasible. However, weekly follow-ups indicated worsening congestive heart failure, reflected by failure to thrive as well as recurrent respiratory infections requiring hospitalization.
Following prolonged hospitalization due to RSV bronchiolitis at the age of 4 months and the persistent lack of weight gain, it was decided to perform surgical ligation of the PDA.
At the age of 4.5 months, the PDA was ligated through a lateral thoracic incision with an unremarkable surgical course.
Following surgery, on post-surgical day (POD) 2, the patient was weaned off mechanical ventilation and catecholamine support. However, dyspnea, tachypnea, and tachycardia persisted. Chest X-ray on POD 3 revealed worsening congestion (picture 1), and ECG revealed pronounced ST elevations on lateral leads. Echocardiography revealed significantly decreased left ventricle function, assessed through "eyeballing" and fractional shortening measurement of 10–15%. Troponin was measured and found to be significantly increased, reaching a maximum of 5330 ng/L.
A differential diagnosis was suggested, including an iatrogenic surgical cause, infectious myocarditis, and coronary anomalies missed in the echocardiography.
On POD 6, the patient underwent a cardiac MRI, which thinning of the myocardium and hypokinesia of the lateral wall and septum, increased T2 weighted signal indicating edema, and late gadolinium enhancement of the endocardium (pictures 2–3). The left ventricle and atrium were dilated, with a left ventricular ejection fraction measured at 20%. The coronary anatomy was not clear.
On POD 7, she underwent diagnostic catheterization, confirming the diagnosis of ALCAPA (picture 4).
She underwent emergent surgical intervention on the same day, during which the left coronary artery was successfully re-implanted in the aortic sinus. The surgery was without complications. She was weaned off cardiopulmonary pump easily and transferred to the pediatric intensive care unit (PICU).
Following surgery, she had a prolonged course due to decreased LV function. Extubation was performed on POD 5, and she was completely weaned off catecholamine support by POD 12. Her PICU length of stay was 25 days, and the total length of stay was 30 days. Prior to discharge, troponin decreased to 358 ng/L, and echocardiography revealed some improvement of cardiac function.
Since her discharge, seven months ago as of the writing of this report, she has been re-hospitalized a total of 7 times. The first re-hospitalization has been just 5 days following her discharge. Most readmissions were due to social factors; however, in two cases, she had respiratory infections with decompensation of her heart failure requiring PICU hospitalization.
Follow-up revealed slow and gradual improvement of cardiac function with wall motion abnormalities and dilated cardiomyopathy. Due to slow improvement, she completed a coronary CT and invasive angiography, both demonstrated patent coronaries arising from the aorta. Her last follow-up was at the age of eleven months, seven months following surgery. She appears well with only mild tachypnea and is gaining weight slowly. Echocardiography revealed dilated LV and left atrium, lateral and septal wall motion abnormalities, and fractional shortening of 23%. The re-implanted left coronary artery appeared patent with normal flow. She continues medical treatment and receives furosemide, captopril, and digoxin, which she tolerates well.