Anomalies of the Aorta and Pulmonary Arteries Complicating Ventricular Septal Defect

Morgan, John J. B.; Pitman, Robert M.; Goodwin, J. F.; Steiner, R. E.; Hollman, Arthur

doi:10.1136/hrt.24.3.279

Cited by 32 publications

(12 citation statements)

References 12 publications

(3 reference statements)

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“…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). In view of the importance of this malformation in the differential diagnosis of Fallot's tetralogy the following case is reported.…”

supporting

confidence: 54%

Double outlet right ventricle with pulmonary stenosis.

Shetty¹,

Martin²

1967

Heart

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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.

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supporting

confidence: 54%

Double outlet right ventricle with pulmonary stenosis.

Shetty¹,

Martin²

1967

Heart

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“…In addition, all the published cases of double outlet right ventricle with pulmonary stenosis in which clinical data were reported are analysed and compared with our series. Two patients with double right ventricle with pulmonary stenosis who have been previously diagnosed at this hospital and reported by Morgan et al (1962) are included.…”

Section: Methodsmentioning

confidence: 99%

“…Witham (1957) was the first to give this condition the name of double outlet right ventricle; he showed that cyanotic patients with this disorder could be divided into two subgroups depending on the site of obstruction to blood flow: those with pulmonary stenosis resembled the tetralogy of Fallot, while those with a high pulmonary vascular resistance and a right-toleft shunt simulated the Eisenmenger complex. Morgan et al (1962) reported double outlet right ventricle with and without pulmonary stenosis, and used a similar classification as follows: Type 1. Acyanotic.…”

mentioning

confidence: 99%

Double outlet right ventricle with pulmonary stenosis.

Dayem¹,

Preger²,

Goodwin³

et al. 1967

Heart

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“…Recently the lesion has been diagnosed during life (Neufeld, DuShane, Wood, Kirklin, and Edwards, 1961;Neufeld, Lucas, Lester, Adams, Anderson, and Edwards, 1962;Morgan, Pitman, Goodwin, Steiner, and Hollman, 1962;Hollman, personal communication), and we know of about 12 patients who have been treated successfully by surgery (McGoon, 1961 ;Redo, Engle, Holswade, and Goldberg, 1963 ;Kirklin, Harp, and McGoon, 1964).…”

mentioning

confidence: 99%