Transatrial Repair of Double Outlet Right Ventricle with L-Malposition of the Great Arteries

An eight-month-old, 7.8 kg infant with a base line oxygen saturation measuring in the high 80% range was diagnosed with a double outlet right ventricle (DORV) prenatally. There was also suspicion of right ventricular outflow tract (RVOT) obstruction. Associated anomalies included bilateral superior venae cavae with no bridging vein, presence of patent foramen ovale, and the right coronary artery crossing the pulmonary artery prior to entering the right atrioventricular grove. The repair was performed using a median sternotomy, aortic cannulation, and tri-caval cannulation at mild hypothermia. Gross anatomy showed features of DORV with the aorta located anterior and to the left of the main pulmonary artery. After achieving antegrade cardioplegic arrest, a right atriotomy was performed and the intracardiac anatomy was evaluated. Upon evaluation, the pathway from the left ventricle to the aorta was found to be long. A 14 mm Hemashield graft was cut in half longitudinally and used to create an intraventricular tunnel from the left ventricle to the aorta. Of note, the patient had a quadricuspid aortic valve.

The baffle was secured with running 6-0 polypropylene suture and further supported with multiple interrupted 6-0 sutures reinforced with bovine pericardial pledgets placed in a horizontal mattress fashion. The aortic and tricuspid valves were tested and deemed competent. The RVOT was evaluated with an appropriately sized dilator and found to be adequate.

Next, a limited aortotomy was performed to evaluate the left ventricular outflow tract (LVOT) and ensure no obstruction to the intracardiac baffle. The patent foramen ovale was then closed and the heart was de-aired after closure of all incisions. Lastly, the cross-clamp was removed. The patient regained his normal sinus rhythm and was subsequently weaned off cardiopulmonary bypass without difficulty on low-dose inotropic support.

The intracardiac pressures were measured directly and confirmed no significant gradient across with the RVOT or LVOT. Transesophageal echocardiogram confirmed widely patent RVOT and LVOT with competent aortic and tricuspid valves in addition to good biventricular functions.

The chest was closed in the standard fashion and the patient was extubated in the operating room. The patient continued to do well during his follow-up period.

References

1. T G DiSessa, A D Hagan, C Pope, L Samtoy, W F Friedman. Two dimensional echocardiographic characteristics of double outlet right ventricle. Am J Cardiol 1979 Nov;44(6):1146-54

2. J K Kirklin, A D Pacifico, J W Kirklin. Intraventricular tunnel repair of double outlet right ventricle. J Card Surg. 1987 Jun;2(2):231-45

3. Meng H, Pang KJ, Li SJ, Hsi D, Yan J, Hu SS, Hua ZD, Wang H. Biventricular Repair of Double Outlet Right Ventricle: Preoperative Echocardiography and Surgical Outcomes. World J Pediatr Congenit Heart Surg. 2017 May;8(3):354-360
4. Ishiyama M, Kurosawa H, Shinoka T, Imai Y. Double-outlet right ventricle with left malposition of the great arteries and total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg. 2007 Feb;55(2):57-60

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