Double outlet right ventricle with pulmonary stenosis.

Abstract

Although transposition of the great vessels is a well-known clinical and pathological entity, the partial transposition complex, resulting in the origin of both the aorta and the pulmonary trunk from the right ventricle, is rare. The association of this malformation with pulmonary stenosis has been described previously (Braun et al., 1952;Witham, 1957;Neufeld, DuShane, and Edwards, 1961;Morgan et al., 1962; Levy et al., 1962 and others angle with a distinct "a " wave. The apex beat was in the sixth left intercostal space and was very forceful and localized. There was a right ventricular heave. There was no thrill. A grade 2/4 pansystolic murmur was heard in the third left intercostal space and under the clavicle a continuous murmur was audible. The second heart sound was single. A loud atrial gallop was heard in the mitral area. The chest radiograph (Fig. 1) showed a cardiothoracic ratio of 57 per cent with right and left ventricular enlargement. The aortic shadow was not prominent and the main pulmonary artery was small. The lungs were underfilled. The electrocardiogram (Fig. 2) showed sinus rhythm. The mean QRS axis was + 135°; there was a tall peaked P wave in lead II and a biphasic P in VI, suggesting both right and left atrial hypertrophy. A tall R wave was present in Vl and very deep S waves in V2, V3, and V4. The T waves over V5 and V6 were deeply inverted. The findings suggested biventricular hypertrophy. A phonocardiogram confirmed the clinical auscultatory findings. The fourth heart sound was clearly shown over the mitral area in the medium frequency tracing.Although the patient had previously been diagnosed as having Fallot's tetralogy, these findings made the diagnosis untenable. The most formidable objection was provided by the electrocardiographic evidence of left ventricular hypertrophy, which was conspicuous, and the occurrence of a left atrial gallop suggesting additional left-sided disease.An angiocardiogram was planned, but on the day of the proposed investigation the patient suddenly collapsed and died, in spite of attempts at resuscitation.At necropsy the heart was very large, weighing 573 g. Both ventricles were greatly hypertrophied. The aorta and pulmonary trunk were normally related externally, but further examination showed that the aorta originated entirely from the right ventricle lying to the right of a normally situated pulmonary trunk and separated from the atrio-ventricular valvular tissue by a horizontal limb of the crista supraventricularis (Fig. 3 A, B). The aortic and pulmonary valves lay approximately in the !79 on 11 May 2018 by guest. Protected by copyright.

Double outlet right ventricle with spontaneously developing pulmonary outflow obstruction.

“…Double outlet right ventricle with spontaneously developing pulmonary outflow obstruction 939 the defect below the pulmonary and the aortic valves. Double outlet right ventricle may be associated with pulmonary stenosis (Dayem et al, I967;Neufeld, DuShane, and Edwards, I96I;Witham, I957;Hallermann et al, 1970;Shetty and Martin, I967). According to Edwards et al (I965) this association is found only in cases with infracristal defects (Type I).…”