New Heart, Old Problems: Aortopulmonary Collaterals Cause Diastolic Heart Failure in a Pediatric Heart Transplant Recipient

Abstract

IntroductionAortopulmonary collaterals (APCs) frequently develop in children with single ventricle (SV) physiology. The prevalence and clinical impact of APCs after pediatric heart transplant (HT) are unknown. We highlight a case of an infant with failed SV palliation with post-HT diastolic heart failure due to a significant burden of APCs.Case ReportA 1-year old infant with hypoplastic left heart syndrome underwent first and second-stage surgical palliation. He developed end-stage heart failure due to systemic right ventricular systolic dysfunction and subsequently underwent HT (recipient/donor weight: 0.89). The extent of APCs was unknown at the time of HT. Post-HT, he had ongoing evidence of diastolic dysfunction with a significant diuretic requirement, persistently elevated BNPs (≈600-800pg/ml), and chronic kidney disease throughout the first year after HT. He had no evidence of graft rejection or coronary allograft vasculopathy. There was suspicion for APCs given holodiastolic flow reversal in the abdominal aorta seen on echocardiography. Cardiac catheterization revealed diastolic dysfunction with left ventricle end-diastolic pressures of 17-23mmHg. There was a step up of saturation from superior vena cava to pulmonary artery (∆15%). Angiography revealed 6 APCs from the right and left internal mammary, right and left thoracic, and right axillary arteries (Figure 1A). Embolization was performed utilizing 33 coils (Figure 1B). Cerebral near infrared spectroscopy immediately improved from 50s to 80s after coiling. Seven months after this procedure, the BNP decreased to 200-300 pg/ml, the LVEDP decreased to 12mmHg, and his diuretic requirement significantly improved.SummaryAPCs may be a cause of persistent diastolic dysfunction and heart failure after HT in patients with previous SV physiology. Interrogation for APCs in these patients should be considered post-HT to aid in early diagnosis, and treatment may yield significant clinical improvement. Aortopulmonary collaterals (APCs) frequently develop in children with single ventricle (SV) physiology. The prevalence and clinical impact of APCs after pediatric heart transplant (HT) are unknown. We highlight a case of an infant with failed SV palliation with post-HT diastolic heart failure due to a significant burden of APCs. A 1-year old infant with hypoplastic left heart syndrome underwent first and second-stage surgical palliation. He developed end-stage heart failure due to systemic right ventricular systolic dysfunction and subsequently underwent HT (recipient/donor weight: 0.89). The extent of APCs was unknown at the time of HT. Post-HT, he had ongoing evidence of diastolic dysfunction with a significant diuretic requirement, persistently elevated BNPs (≈600-800pg/ml), and chronic kidney disease throughout the first year after HT. He had no evidence of graft rejection or coronary allograft vasculopathy. There was suspicion for APCs given holodiastolic flow reversal in the abdominal aorta seen on echocardiography. Cardiac catheterization revealed diastolic dysfunction with left ventricle end-diastolic pressures of 17-23mmHg. There was a step up of saturation from superior vena cava to pulmonary artery (∆15%). Angiography revealed 6 APCs from the right and left internal mammary, right and left thoracic, and right axillary arteries (Figure 1A). Embolization was performed utilizing 33 coils (Figure 1B). Cerebral near infrared spectroscopy immediately improved from 50s to 80s after coiling. Seven months after this procedure, the BNP decreased to 200-300 pg/ml, the LVEDP decreased to 12mmHg, and his diuretic requirement significantly improved. APCs may be a cause of persistent diastolic dysfunction and heart failure after HT in patients with previous SV physiology. Interrogation for APCs in these patients should be considered post-HT to aid in early diagnosis, and treatment may yield significant clinical improvement.