Origin of Both Great Vessels From the Right Ventricle Without Pulmonary Stenosis

Abstract

In a recent communication Neufeld and associates (1961) described the pathological, functional, electrocardiographic, and clinical findings in a group of eight cases of an uncommon condition in which both great vessels arose from the right ventricle in the absence of pulmonary stenosis. In seven of the eight cases of this condition (for which such synonyms as "partial transposition," "Taussig-Bing complex," "double outlet right ventricle" have been offered) the picture both hmmodynamically and clinically resembled that in large ventricular septal defects.One of the patients (Case 4) of the earlier report, which emanated from the Mayo Clinic, presented somewhat different features from the remaining seven and resembled not only the case reported by Taussig and Bing (1949) but also others reported later as examples of the so-called Taussig-Bing complex. Distinguishing features of the latter type of case included cyanosis and higher levels of oxygen saturation of blood in the pulmonary artery than in a systemic artery or the aorta.This difference in groups of patients having apparently similar anatomical findings (that is, origin of both great vessels from the right ventricle without pulmonary stenosis) prompted a review of another series represented by ten cases from the University of Minnesota Hospitals. From this material a report of correlative studies of the anatomical findings with clinical, hcmodynamic, and angiocardiographic findings represents the main purpose of this communication.These studies suggest the value of classifying into two subdivisions the cases manifesting origin of both great vessels from the right ventricle without pulmonary stenosis. Furthermore, we believe, on the basis of the evidence we shall present, that the so-called Taussig-Bing complex represents one of these two subdivisions. Nine of the ten patients with the anomaly came to necropsy, while the tenth (Case 9), later to be classified as an example of Type IIA, was studied only clinically. The anatomical similarities in all cases of origin of both great vessels from the right ventricle regardless of subdivision are as follows.1. The pulmonary trunk and aorta are normally related externally. 2. The aorta originates entirely from the right ventricle lying to the right of the pulmonary trunk.3. The aortic valve lies higher than normal and is located at about the same cross-sectional and coronal body planes as the pulmonary valve.4. Discontinuity of mitral and aortic valvular tissue is the usual finding (in seven of nine cases in the current study). In the nine examples of the malformation for which necropsy specimens are

Double Outlet Right Ventricle Associated with Persistent Common Atrioventricular Canal and Pulmonary Stenosis

“…In our case and in that described by Neufeld et al [4] it was deviated superiorly and to the right (-170°). We suppose that the vector of the AQRS in the association of DORV with pulmonary stenosis and CAVC is the resultant vector of the vectors of AQRS of the single cardiac malformations.…”

“…DORV with pulmonary stenosis is indis tinguishable from the tetralogy of Fallot on the basis of clinical, electrocardiographic, ra diologic and hemodynamic finding [4]; also the clinical symptoms in the association of CAVC and DORV with pulmonary stenosis resemble those in the tetralogy of Fallot [5],…”

Double Outlet Right Ventricle with Discordant Atrioventricular Connections in a Newborn: Case Report and Brief Overview of the Literature

“…In our patient, interventricular communication was in doubly committed position, aorta was parallel and to the left of the pulmonary trunk with usual atrial arrangement [2]. In the literature, double outlet right ventricle has been described with ventricular inversion form with situs solitus, and with atrioventricular concordance in situs inversus [4][5][6]. Double outlet right ventricle with discordant atrioventricular connections have been described in association with both usual and mirror-image atrial arrangements.…”

Taussig-Bing anomaly and coarctation of the aorta in infancy: Surgical options

“…The anatomic classification of DORY is based upon the location of a VSD relative to the great vessels : subaortic, subpulmonary, doubly committed, and noncommitted [15) . The subpulmonic location of the large VSD in the T-B anomaly causes cyanosis due to preferential streaming of blood from the left ventricle into the pulmonary artery and from the right ventricle into the aorta [20,43] . The absence of pulmonary stenosis results in increased pulmonary blood flow, pulmonary hypertension, and congestive heart failure .…”

“…In 1949, Taussig and Bing [39] described the clinical and pathological characteristics of a complex congenital malformation that has come to be classified as a subset of DORY [15,16,[20][21][22]41] . The anatomic classification of DORY is based upon the location of a VSD relative to the great vessels : subaortic, subpulmonary, doubly committed, and noncommitted [15) .…”