Pulmonary valve fusion with intact ventricular septum

Celermajer, John M.; Bowdler, J D; Gengos, D. C.; Cohen, Douglas; Stuckey, Douglas

doi:10.1016/0002-8703(68)90132-4

Cited by 26 publications

(3 citation statements)

References 8 publications

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“…However, there is no agreement concerning the nature and frequency of this hypoplasia. Greenwold et al (1956) believed that right ventricular cavity size could be classified as small or large and this view was endorsed by Davignon et al (1961) and Celermajer et al (1968). In contrast, others have contended that cavity size constitutes a spectrum (Gersony et al, 1967; Subjects and methods Thirty-seven infant hearts with pulmonary atresia and intact ventricular septum together with 3 hearts with critical pulmonary stenosis were found in the cardiopathological collections ofthe Brompton Hospital, Cardiothoracic Institute (London); Killingbeck Hospital (Leeds); Institute of Child Health, Alder Hey Hospital (Liverpool); and Grimsby General Hospital (South Humberside).…”

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confidence: 99%

Morphological variations in pulmonary atresia with intact ventricular septum.

Zuberbuhler¹,

Anderson²

1979

Heart

133

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confidence: 99%

Morphological variations in pulmonary atresia with intact ventricular septum.

Zuberbuhler¹,

Anderson²

1979

Heart

133

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“…Although roentgenographic cardiomegaly is a characteristic of pulmonary atresia with IVS type 23' 19 it is uncommonly of the magnitude seen in the patients in this report. Two of the patients described by Schrire, Sutin and Barnard2 are very similar both clinically and radiologically to the neonates in this report.…”

Section: Discussionmentioning

confidence: 51%

“…The infants in this report had large right ventricular cavities and they did not have the prominent trabecular pattern usually visible in patients with pulmonary atresia and IVS type 2 and produced by the encroachment of large muscle bands on the cavity of the right ventricle. 19 The appearance of the right ventricle, together with the widely dilated tricuspid valve annulus and the massively enlarged right atrium, reflects the severe tricuspid regurgitation present in these patients.…”

Section: Discussionmentioning

confidence: 99%

Severe Congenital Tricuspid Incompetence in the Neonate

et al. 1974

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SUMMARYFive neonates with congenital tricuspid incompetence due to severe tethering of the tricuspid valve to the right ventricle by abnormal chordal and papillary muscle attachments are described. The abnormality was called tricuspid valvular dysplasia (TVD) if the basal insertion of the valve was normal and the Ebstein malformation if it was displaced into the sinus portion of the ventricle.In the two infants with isolated TVD and severe tricuspid regurgitation the functional obstruction to right ventricular outflow (ORVO) produced by the high perinatal pulmonary vascular resistance (PVR) made the exclusion of pulmonary atresia difficult, despite selective right ventricular angiocardiography.In three infants the tricuspid valvular abnormality was associated with organic ORVO; pulmonary atresia in two and critical pulmonary valve stenosis in one. In the two infants with pulmonary atresia and intact ventricular septum (IVS) the severe tricuspid incompetence produced a clinical, radiological and hemodynamic profile which was clearly different from that usually seen in infants with pulmonary atresia and IVS and a normal right ventricular cavity (type 2 of Greenwold).

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Pulmonary Atresia with Intact Ventricular Septum

Hoffman

2009

The Natural and Unnatural History of Congenital Heart Disease

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Although congenital pulmonary atresia with an intact ventricular septum is an uncommon malformation, its recognition is important because the results of surgical treatment may be favourable in some cases. Functionally, this entity belongs to a group of congenital malformations each having obstruction to pulmonary flow. This group includes severe pulmonary stenosis, tricuspid atresia, pulmonary atresia with a ventricular septal defect, and the origin of the aorta and pulmonary trunk from the right ventricle with severe pulmonary-stenosis.In these congenital cardiac malformations, the fundamental functional problem and the clinical picture are determined by obstruction to pulmonary blood flow in the right side of the heart. As a result of this obstruction, right-to-left shunts of varying degrees occur at different sites depending on the type of intracardiac communication present. This hlmodynamic phenomenon is usually manifested clinically by early intense cyanosis. Congestive heart failure occurs in most cases, and early death is common. Since the results of surgical treatment vary greatly in cases of these types of cardiac malformation, immediate and proper diagnosis is essential (Benton et al., 1962). Greenwold et al. (1956) made a study of 12 cases of pulmonary atresia with an intact ventricular septum and divided them into two groups on the basis of the size of the right ventricle. The first group included 6 cases in which the right ventricle was minute or small, and the second group included 6 in which it was of normal size or large. Keith, Rowe, and Vlad (1958) described the pathological findings in 46 cases: the right ventricle was of normal size or large in only 15 per cent of the 46 cases. Davignon et al. (1961) correlated the clinical and pathological findings in 20 cases: several of these were previously included in the report by Greenwold et al. (1956). MATERIAL This report is based on the anatomical, clinical, and electrocardiographic aspects of 12 cases of pulmonary atresia with an anatomical or functionally intact ventricular septum, which were observed at the University of Minnesota Hospitals. In an article to be published, Kieffer and Carey (1963) will consider the radiographic aspects of these cases. Other examples of obstructive lesions of the right side of the heart, such as tricuspid atresia, were not included in our study. ANATOMICAL OBSERVATIONSThe right ventricle was minute in 3 cases, small in 6, of normal size in 2, and large in 1 case.Pulmonary Valve and Arteries. The pulmonary valve had no opening but was represented by a fibrous membrane or diaphragm (Fig. 1). Radiating from the centre on the arterial side of the diaphragm were three equidistant ridges or raphes which extended to the wall of the pulmonary trunk.

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Pulmonary valve fusion with intact ventricular septum

Cited by 26 publications

References 8 publications

Morphological variations in pulmonary atresia with intact ventricular septum.

Morphological variations in pulmonary atresia with intact ventricular septum.

Severe Congenital Tricuspid Incompetence in the Neonate

Pulmonary Atresia with Intact Ventricular Septum

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