Early Bidirectional Glenn Procedure as Initial Surgical Palliation for Functionally Univentricular Heart with Common Arterial Trunk.

We report an unusual case of a 2-month-old baby with a diagnosis of common arterial trunk and double outlet right ventricle with a remote type ventricular septal defect. Taking into consideration the physiologic moment and anatomic findings of the patient, we planned and successfully performed a bidirectional Glenn procedure as its first palliative procedure.

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle"

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle".

Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Double Root Switch Repair for Taussig-Bing Anomaly.

The double root switch was used to repair a Taussig-Bing anomaly in a two-month-old child. The surgery was performed with translocation of the aortic root to the left ventricular outflow tract and the pulmonary root to the right ventricular outflow tract, after closure of the ventricular septal defect with a patch that ultimately directed flow from the left ventricle to the aorta. This surgical approach has the theoretical advantage of reducing the likelihood of subneopulmonary obstruction, left ventricular outflow obstruction, neoaortic dilatation, and regurgitation and therefore has the potential to minimize the mortality and morbidity related to reintervention and reoperation.

Pulmonary root translocation in malposition of great arteries repair allows right ventricular outflow tract growth.

OBJECTIVE: Optimal surgical treatment of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) remains a matter of debate. This study evaluated the clinical outcome and right ventricle outflow tract performance in the long-term follow-up of patients subjected to pulmonary root translocation (PRT) as part of their surgical repair. METHODS: From April 1994 to December 2010, we operated on 44 consecutive patients (median age, 11 months). All had malposition of the great arteries as follows: TGA with VSD and PS (n = 33); double-outlet right ventricle with subpulmonary VSD (n = 7); double-outlet right ventricle with atrioventricular septal defect (n = 1); and congenitally corrected TGA with VSD and PS (n = 3). The surgical technique consisted of PRT from the left ventricle to the right ventricle after construction of an intraventricular tunnel that diverted blood flow from the left ventricle to the aorta. RESULTS: The mean follow-up time was 72 ± 52.1 months. There were 3 (6.8%) early deaths and 1 (2.3%) late death. Kaplan-Meier survival was 92.8% and reintervention-free survival was 82.9% at 12 years. Repeat echocardiographic data showed nonlinear growth of the pulmonary root and good performance of the valve at 10 years. Only 4 patients required reinterventions owing to right ventricular outflow tract problems. CONCLUSIONS: PRT is a good surgical alternative for treatment of patients with TGA complexes, VSD, and PS, with acceptable operative risk, high long-term survivals, and few reinterventions. Most patients had adequate pulmonary root growth and performance.

Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect.

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We report the case of an 18-day-old infant with DORV and extremely rare anatomical features, such as anterior and left-sided aorta and subpulmonary ventricular septal defect (VSD). In addition to the anatomic features, the role of the echocardiogram for guiding the diagnosis and the surgical therapy of this congenital heart disease are discussed.

[Surgical alternatives in the treatment of double-outlet right ventricle]. / Alternativas quirúrgicas en el tratamiento de la doble cámara de salida del ventrículo derecho.

Double-outlet right ventricle (DORV) is a type of ventriculo-arterial connection, in which both great arteries are connected to the morphologically right ventricle in at least 50% of their diameter. The relation of the great arteries with the ventricular septal defect (VSD) and the pulmonary stenosis, are important anatomical aspects in the election of the surgical technique. In this study we describe four patients with DORV, in whom different surgical techniques were used according to the specific anatomy of each case. Two patients had subaortic restrictive VSD, with subpulmonary stenosis in one of them. In the other two patients the VSD was subpulmonary with the aorta anterior and to the left, in one of these existed moreover a severe mixed pulmonary stenosis. It is concluded, that with different surgical techniques according to the specific anatomy of each patient, good results can be obtained in the surgical treatment of these complex congenital malformations.