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Anomalous origin of the left coronary artery from pulmonary artery constitutes 0.5% of all CHD (Boutsikou M, Shore D, Li W, et al, Int J Cardiol 261: 49–53, 2018). Fifteen percent survive into adulthood undiagnosed and 90% present with sudden death (Yau JM, Singh R, Halpern EJ, Fischman D, Clin Cardiol 34: 204–210, 2011; Alexi-Meskishvili V, Berger F, Weng Y, Lange PE, Hetzer R, J Card Surg 10: 309–315, 1995). We describe an enigmatic case of a 29-year-old female who presented after an aborted cardiac arrest and was diagnosed with anomalous origin of the left coronary artery from pulmonary artery.
A term female with prenatally diagnosed D-Transposition of the great arteries, large membranous ventricular septal defect with inlet extension, moderate secundum atrial septal defect, and large patent ductus arteriosus (Fig 1) was born by scheduled caesarean section. Transthoracic echocardiogram confirmed the anatomy with both coronary arteries arising from a single sinus with separate ostia. The right coronary artery arose from right posterior facing sinus (Fig 2). The left coronary artery arose anomalously from the same sinus adjacent to the right coronary artery ostium, coursing posterior to the aorta, with brief intramural and interarterial course before bifurcating into the left anterior descending and left circumflex coronary arteries (Figs 3 and 4). As a result of this unique coronary pattern, she underwent unroofing of the intramural left coronary artery noted on opening the aortic root to the coronary ostium. Both coronary buttons were harvested and this large button was then divided into two buttons. The left coronary artery button was implanted with a trapdoor technique, right coronary artery button was implanted, and the remainder of the arterial switch procedure along with LeCompte maneuver was completed uneventfully, with closure of the atrial and ventricular septal defects. The post-operative course was uneventful and the patient was discharged on the seventh post-operative day. At discharge, the patient had normal biventricular systolic function, no residual intracardiac shunt, and robust antegrade flow in the reimplanted coronary arteries. The patient was growing well at the fourth month post-operative visit with normal biventricular function, patent coronaries, and outflow tracts.
Aortic dissection causes significant morbidity and mortality in adults and treatment guidelines are based on well-documented risk factors. Conversely, dissection after orthotopic heart transplantation is very rare, especially in the absence of infection, hypertension, or donor–recipient aortic size mismatch. Several forms of CHD are associated with aortic dilatation, but the incidence of aortic dissection and aneurysm in children is also low, which makes use of adult guidelines in children challenging. We present a 17-year-old Amish female with a homozygous gene mutation in the MYBPC3 gene known to cause lethal, infantile hypertrophic cardiomyopathy. She underwent orthotopic heart transplantation and then developed an asymptomatic aortic dissection despite no known risk factors.
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