Institutional experience with a protocol of early primary repair of double-outlet right ventricle

Surgical management of transposition in the setting of obstruction within the aortic arch

“…6 Obstruction of the aortic arch is reported in at least half of patients with double outlet right ventricle with subpulmonary interventricular communication, the so-called Taussig-Bing malformation. [7][8][9][10][11][12][13][14] On the other hand, interruption of the aortic arch is uncommon. In a recent study by the Congenital Heart Surgeons Society, 15 involving almost 500 patients with interrupted aortic arch, only 26 patients (6 percent) had transposition with ventricular septal defect, and only 8 patients (2 percent) had double outlet right ventricle.…”

“…The arterial switch operation has become well established in the treatment of transposition, 7,8,[20][21][22][23][24][25][26] as well as for the Taussig-Bing malformation. 7,8 The presence of an obstructed aortic arch, nonetheless, presents a formidable surgical challenge. The surgical treatment in this setting has two parts, one dealing with the intracardiac repair, and the other with repair of the coarctation and the aortic arch.…”

Unusual aberrancy of right coronary artery (RCA) in patient with double-outlet right ventricle (DORV) with pulmonary stenosis (PS)

“…Surgical correction of congenital heart disease in the past three decades has witnessed enormous strides as early primary repair for the vast majority of cardiac malformations is now carried out rather than initial palliation. This started with tetralogy of Fallot (TOF), and is now also being performed for other complex lesions such as DORV, with excellent results [6]. It establishes normal anatomic and physiologic conditions as early in life as possible.…”

Rational Approach to Surgical Management of Complex Forms of Double Outlet Right Ventricle with Modified Fontan Operation

“…Double outlet right ventricle (DORV) is characterized by a malposition of the great arteries. It is a primitive mode of ventriculoarterial connection with the aorta arising predominantly from the right ventricle 1–4 . DORV can occur with two functional ventricles representing a distinct clinical entity or it can be part of complex anatomy including that of a single ventricle.…”

“…It is a primitive mode of ventriculoarterial connection with the aorta arising predominantly from the right ventricle. [1][2][3][4] DORV can occur with two functional ventricles representing a distinct clinical entity or it can be part of complex anatomy including that of a single ventricle. The latter presentation exhibits a wide spectrum of anatomic and physiologic variation.…”