Transposition of the great arteries with ventricular septal defect and pulmonary stenosis.

Abstract

SUMMARY A postmortem investigation was carried out of 19 heart specimens with transposition of the great arteries, ventricular septal defect, and congenital subvalvular pulmonary stenosis. Certain types of obstruction appeared to be closely related to other features of the hearts.In (Elliott et al., 1963;Shaher et al., 1967aShaher et al., , 1967b Daicoffet al., 1969;Rastelli et al, 1969;Fisher et al., 1970;Imamura et al., 1971;Quero-Jimenez et al., 1973;Shaher, 1973;Quero-Jimenez and Perez Martinez, 1974;Lancelin et al., 1975; QueroJimenez et al., 1975;Shrivastava et al., 1976; Buihlmeyer et al., 1977; Quero-Jimenez etcal., 1977;Van Praagh, 1977).If we exclude cases with other malformations, nonvalvular pulmonary stenosis in transposition of the great arteries with ventricular septal defect in the published reports is described in about 7 to 25 per cent of cases of transposition coming to necropsy (Lev et al., 1961;Elliott et al., 1963;Shaher et al., 1967b;Paul et al., 1968;Imamura et a!., 1971;Quero-Jimenez et al., 1973;Shaher, 1973;Van Praagh, 1977).Received for publication 1 August 1977 In our collection of 150 heart-lung specimens with d-transposition of the great arteries, there were 19 specimens with the combination transposition of the great arteries, ventricular septal defect, and clinical subvalvular pulmonary stenosis. In this paper a detailed description is given of the various structures, causing the subvalvular stenosis. DefinitionsTransposition of the great arteries is said to occur when the aorta arises from the morphologically right ventricle and the pulmonary trunk arises from the morphologically left ventricle. The infundibular septum is the structure interposing between two semilunar valves (Lev, 1972;Anderson et al., 1977), and in normal hearts it is incorporated on top of the ventricular septum; in the case of a malorientation of the infundibular septum a malalignment ventricular septal defect may result (Fig. la, 2a). The anterolateral muscle bundle of the left ventricle separates the anterior leaflet of the mitral valve from the anterior border of the left ventricle. It borders on the left sumilunar cusp and extends into the anterolateral wall of the left ventricle (Fig. lb, 2b) 494

Rastelli procedure for transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. Early and late results in 41 patients (1971 to 1978).

“…"7 Subvalvular stenosis may be caused by a fibrous shelf, a fibromuscular tunnel, herniation of accessory tricuspid tissue through the ventricular septal defect, abnormal attachment of the mitral valve, aneurysm of the membranous ventricular septum, or septal hypertrophy. [18][19][20][21] Direct attempts to resect this stenotic area in combination with a Mustard operation and ventricular septal defect closure may not alleviate the gradient-because of the arrangement of the mitral valve, ventricular septum, and coronary arteries.22 More recently, good results with the relief of left ventricular outflow tract obstruction combined with the Mustard operation have been achieved.2' 24 However, the Rastelli procedure offers several advantages in this group of patients by providing adequate relief of the left ventricular outflow tract obstruction while transferring the left ventricle and mitral valve to the systemic arterial circulation. It should, therefore, avoid any concern about late right ventricular function and the tricuspid regurgitation sometimes seen after the Mustard procedure.…”

Comparison of types of pulmonary stenosis with the state of the ventricular septum in complete transposition of the great arteries

“…Elliott and associates [6] found VSD in 22 (35%) of 60 cases of complete transposition . Van Gils and associates [17] found VSD in 40 (27%) of 150 specimens . Idriss and associates [7] examined 94 hearts and found VSD in 41 (43 .5%) .…”

“…Van Gils' group [17] has suggested that malorientation of the infundibular septum and association of the so-called malalignment type of VSD leads to certain forms of subvalvular PS . Support for this theory is provided by Quero Jimenez and Perez Martinez [12], who described five hearts with LV conus and malalignment of the infundibular septum with associated supracristal VSD .…”

“…Obstruction to flow occurs either solely or in combination from valvular stenosis, subvalvular fibromuscular or fibrous stenosis, or bulging of the ventricular septum into the LV cavity [3,15] . Others have described anomalous attachment of the anterior leaflet of the mitral valve to the ventricular septum resulting in obstruction to pulmonary flow [6,9,10,14,17] . Deal and associates [5] undertook an echocardiographic study of patients with VSD and transposition and noted an increased incidence of abnormalities of the tricuspid valve when compared with patients with an intact septum and d-TGA (64% vs 0%) .…”

The Spectrum of Congenital Heart Disease with Transposition of the Great Arteries from the Cardiac Registry of the University of Padua

“…The types of left outflow tract obstruction in complete TGA were the same found in hearts with concordant VA connections. The stenosis was located at subvalvular or valvular level (unicuspid, bicuspid, or dysplastic valve) ( 27 , 28 ). The subvalvular stenosis was caused by hypertrophy and bulging of the muscular ventricular septum, in isolation or associated to a fibrous shelf.…”