Ross-Konno Operation in an Infant With a Quadricuspid Pulmonary Valve and Anomalous Aortic Origin of the Right Coronary Artery

Abstract

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...

Quadricuspid Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve

“…Literature has reported on three cases with both QPV and QAV [ 14 , 30 , 60 ], six with both QPV and bicuspid aortic valve [ 2 , 25 , 56 , 77 ], and two with both QAV and bicuspid pulmonary valve [ 78 , 79 ]. These three coexistences are extremely rare combinations.…”

“…Only 1 of 57 cases with QPV diagnosed after 1973 presented with a coronary arterial abnormality [ 77 ]. Coronary ostium malformation of QAV was 8.8% [ 80 ]; 10.2% of QAV had a coronary malformation [ 18 ].…”

“…Five cases with QPV had a conotruncal anomaly. Two neonates of aortic atresia with interrupted aortic arch were diagnosed as QPV at autopsy [ 64 ], and an infant with interrupted aortic arch complex was diagnosed as QPV at the time of Ross operation [ 77 ]. 1 (0.63%) of 150 cases with tetralogy of Fallot had QPV, which could not be diagnosed by both CT and TTE [ 75 ].…”

“…[ 88 ] concluded that up-to-date knowledge of aortic valve reconstruction may be applied to increasing challenging anatomy, such as a non-trileaflet pulmonary autograft. In a 4-month infant with QPV (type-b) for which the Ross–Konno procedure was performed, mild AR occurred 2 years later [ 77 ]. QPV, found unexpectedly at the time of operation, can be used as an autograft in the Ross operation, particularly in neonates and infants in whom no good alternatives for aortic valve replacement exist [ 77 ].…”

“…In a 4-month infant with QPV (type-b) for which the Ross–Konno procedure was performed, mild AR occurred 2 years later [ 77 ]. QPV, found unexpectedly at the time of operation, can be used as an autograft in the Ross operation, particularly in neonates and infants in whom no good alternatives for aortic valve replacement exist [ 77 ]. A complete evaluation of the morphology of both semilunar valves in patients with coronary ostial malformations is advised [ 78 ].…”