Pulmonary Regurgitation With Ventricular Septal Defect and Pulmonic Stenosis—tetralogy of Fallot Variant

Durnin, Robert E.; Willner, Richard; Virmani, Shubi; Lawrence, Tsun-Yee K.; Fyler, Donald C.

doi:10.2214/ajr.106.1.42

Cited by 19 publications

(1 citation statement)

References 17 publications

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“…Resulting anectasia, atelectasis or obstructive emphysema of a lung lobe or a whole lung further complicates the course of the disease in most cases (6,8,10,12,13,15,16,28,29,33,34). Due to the intimate anatomical relation between the tracheobronchial tree and the pulmonary arteries compression of trachea and/or bronchi frequently occurs.…”

Section: Compression Of the Tracheobronchial Treementioning

confidence: 99%

Das Syndrom der Pulmonalklappenaplasie mit Ventrikelseptumdefekt-anatomie und Embryologie

et al. 1979

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Four cases of absent pulmonary valve in combination with ventricular septal defect are reported. In this syndrome hypo- and dysplasia of the pulmonary valve is constantly associated with a big ventricular septal defect, formation of a huge pulmonary artery aneurysm and absence of the ductus arteriosus. Presence or absence of a right ventricular outflow tract obstruction is the criteria for classification into two forms. Absence of the pulmonary valve, right ventricular outflow tract obstruction and a malalignment-type ventricular septal defect produced by a conotruncal malseptation process represent the primary complex of malformations. Consecutive intrauterine cardiac failure is most probably prevented by prenatal closure of the ductus arteriosus. Pulmonary artery aneurysm and also dilatation of the right ventricular outflow tract as well as a whole lot of other coexisting deformities can be explained by a cascade of hemodynamical sequelae started by this ductus closure in utero. An embryological scheme explaining the genesis of this syndrome is derived from a morphological analysis of the constituting incoherent-appearing anatomical features.

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Section: Compression Of the Tracheobronchial Treementioning

confidence: 99%

Das Syndrom der Pulmonalklappenaplasie mit Ventrikelseptumdefekt-anatomie und Embryologie

et al. 1979

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Tetralogy of Fallot with “Absent” Pulmonary Valve

Freedom¹,

Rabinovitch²

1992

Neonatal Heart Disease

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Absent aortic and pulmonary valves: Investigation of three fetal cases with cystic hygroma and review of the literature

et al. 1994

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Absent semilunar valve was found in three fetal cases with cystic hygroma. Two cases which simultaneously showed absent aortic and pulmonary valves (AAV and APV, respectively) had double-outlet right ventricle. The third case, which lacked only the aortic valve, had atrioventricular septal defect and anomalous origin of the right subclavian artery. Two of the three cases had a markedly hypoplastic thymus. Fifteen AAV and 179 APV cases, including the above-mentioned cases and others reported elsewhere, were discussed with special reference to the pathogenesis of absent semilunar valve. Of the 15 AAV cases, hypoplasia of the left heart was observed in 11 cases (73.3%), double-outlet right ventricle in 5 (33.3%), and aortic arch malformations in 6 (40.0%). In the 179 APV cases, there were 111 tetralogy of Fallot (62.0%) and 44 right-sided aortic arch (24.6%). DiGeorge anomaly was found in one AAV and eight APV cases. These results indicate a pathogenesis that is possibly related to hemodynamic abnormalities or abnormal neural crest cells. Further investigation will be needed to elucidate a more definite pathogenesis of absent semilunar valve.

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Pulmonary Regurgitation With Ventricular Septal Defect and Pulmonic Stenosis—tetralogy of Fallot Variant

Cited by 19 publications

References 17 publications

Das Syndrom der Pulmonalklappenaplasie mit Ventrikelseptumdefekt-anatomie und Embryologie

Das Syndrom der Pulmonalklappenaplasie mit Ventrikelseptumdefekt-anatomie und Embryologie

Tetralogy of Fallot with “Absent” Pulmonary Valve

Absent aortic and pulmonary valves: Investigation of three fetal cases with cystic hygroma and review of the literature

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