Anomalous aortic origin of the right coronary artery from the left aortic sinus is a rare congenital anomaly that is generally repaired during adolescence when the condition is associated with symptoms. It is rarely diagnosed in infancy. Similarly, a quadricuspid pulmonary valve is also a rare finding, and there are scant data to evaluate whether this malformation of the pulmonary valve is suitable to be used for a Ross operation. This report describes a case in which both these anomalies coexisted in an infant who underwent a successful Ross-Konno operation.A quadricuspid pulmonary valve (QPV) is a rare congenital cardiac anomaly of uncertain significance, and there are scant data to evaluate whether this malformation of the pulmonary valve is suitable to be used for a Ross operation. 1-3 We describe a case in which this anomaly existed in an infant who underwent a successful Ross-Konno operation. Our patient also had an associated anomalous aortic origin of the right coronary artery (AAORCA), which made the repair somewhat more complex.A 39-week gestational age male infant weighing 3.2 kg was born with interrupted aortic arch type B, hypoplastic left heart complex, and a large posterior malaligned ventricular septal defect. The hypoplastic bicuspid aortic valve measured 4.4 mm (z score, À4.5), the left ventricular major axis was 25 mm, with a minor axis of 9 mm and a long axis of the heart of 32 mm, and the mitral valve measured 10 mm (z score, À0.87). The Congenital Heart Surgeons' Society scores of 1 and 2 favored a biventricular repair. 4 He underwent a primary repair of the interrupted aortic arch with anterior augmentation of the anastomosis using autologous treated pericardium and pericardial patch closure of a large posterior malaligned ventricular septal defect and a large atrial septal defect.His early postoperative course was uncomplicated, and he was thriving at home with good weight gain and no feeding difficulty. However, the left ventricular outflow tract obstruction continued to increase over the next 3 months. By echocardiography, the peak gradient over the subvalvular and valvar areas was recorded as 90 mm Hg, and cardiac catheterization confirmed a multilevel gradient of 60 mm Hg ( Figure 1A). In addition, the right coronary artery arose from the left sinus very close to the left main coronary artery ostium ( Figure 1B).At 4 months of age, he underwent reoperation for subaortic resection. Because the subaortic scarring involved the hypoplastic aortic valve leaflets, a Ross-Konno operation was performed. His intraoperative findings confirmed the high takeoff of the right coronary artery from the left sinus very close to the ostium of the left main coronary artery. There was an interarterial course, making the harvesting of the pulmonary root more complicated, especially in the context of reoperative aortic root surgery in an infant. Furthermore, once the pulmonary root had been harvested, it was found to be a quadricuspid valve, with a small rudimentary fourth posterior leaflet. A generous cuff...

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Ebstein's anomaly is a congenital heart disease that results from failure of delamination of the tricuspid valve with resulting apical displacement of the septal and posterior leaflets of the tricuspid valve. Age at presentation can vary greatly but neonatal presentation is associated with extraordinary high mortality rates. Comprehensive multispecialty care is required starting at the time of fetal diagnosis. Fetal echocardiography is vital in monitoring progression of the disease in utero. Fetal echocardiogram can evaluate for complications such as arrhythmias, pericardial effusion, or fetal hydrops. Post-natal evaluation should include evaluation of functional pulmonary atresia or circular shunt. Despite advances in surgical technique for Ebstein's anomaly, mortality for it remains high with early surgical intervention. Aggressive medical management should be used to support patients with Ebstein's anomaly during the neonatal period. Surgical procedures for neonatal Ebstein's vary widely from systemic to pulmonary shunts with or without tricuspid valve closure to tricuspid valve repair.

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