Here we report a case of successful heart transplantation (HT) in a patient with high risk on HT due to her complex congenital heart disease and its consequent complications; physiologic single lung and severe pulmonary arterial hypertension. HT was successfully performed in our patient using meticulous perioperative management techniques, such as control of collateral vessels, appropriate donor selection, pulmonary vasodilator therapy, post-transplant extracorporeal membranous oxygenation support, and atrial septostomy for right ventricular unloading.
Keywords: Heart defects, congenital; heart transplantation; pediatrics; physiologic single lung; pulmonary arterial hypertension.
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