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. 1970 Jul;32(4):483–490. doi: 10.1136/hrt.32.4.483

Congenital absence of the pulmonary valve

Fergus J Macartney 1,1, Graham A H Miller 1,2
PMCID: PMC487358  PMID: 5433308

Abstract

The clinical, radiological, electrocardiographic, and haemodynamic findings in 7 patients with the syndrome of congenital absence of the pulmonary valve with ventricular septal defect are reported. Findings included a pansystolic murmur and a low-pitched delayed diastolic murmur at the left sternal border with no pulmonary valve closure sound, an rsR′ pattern in V1 on the electrocardiogram, and a chest x-ray showing a large heart and gross dilatation of the main, left or right pulmonary arteries but normal or reduced vascularity of the peripheral lung fields. Cardiac catheterization showed a left-to-right shunt at ventricular level in every case, though in one the shunt was bidirectional. Despite right ventricular systolic pressures at least 75 per cent of systemic level in every patient, the pulmonary artery pressure was invariably normal. Pulmonary stenosis was shown to exist at infundibular level, at valve ring level, or at both. Selective right ventricular angiography was diagnostic: the main and right or left pulmonary arteries were aneurysmally dilated and there was an abrupt transition in calibre between these vessels and their immediate branches; enlargement of the right pulmonary artery caused distortion and downward displacement of the left atrium in 4 patients; in 2 there was a flat `diaphragm' in the pulmonary valve position.

The diagnosis was confirmed in 3 patients by necropsy. The pulmonary valve was represented by at most a rudimentary fringe of tissue. Histological examination of the pulmonary arteries in one patient showed a correlation between the dilatation of the artery and the structure of its wall.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BOUSVAROS G. A., DEUCHAR D. C. The mumur of pulmonary regurgitation which is not associated with pulmonary hypertension. Lancet. 1961 Oct 28;2(7209):962–964. doi: 10.1016/s0140-6736(61)90798-x. [DOI] [PubMed] [Google Scholar]
  2. CAMPEAU L. A., RUBLE P. E., COOKSEY W. B. Congenital absence of the pulmonary valve. Circulation. 1957 Mar;15(3):397–404. doi: 10.1161/01.cir.15.3.397. [DOI] [PubMed] [Google Scholar]
  3. D CRUZ I. A., LENDRUM B. L., NOVAK G. CONGENITAL ABSENCE OF THE PULMONARY VALVE. Am Heart J. 1964 Dec;68:728–740. doi: 10.1016/0002-8703(64)90122-x. [DOI] [PubMed] [Google Scholar]
  4. EDWARDS J. E. Functional pathology of the pulmonary vascular tree in congenital cardiac disease. Circulation. 1957 Feb;15(2):164–196. doi: 10.1161/01.cir.15.2.164. [DOI] [PubMed] [Google Scholar]
  5. HEATH D., EDWARDS J. E. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958 Oct;18(4 Pt 1):533–547. doi: 10.1161/01.cir.18.4.533. [DOI] [PubMed] [Google Scholar]
  6. LAVENNE F., TYBERGHEIN J., BRASSEUR L., MEERSSEMAN F. Complexe d'Eisenmenger avec insuffisance pulmonaire par absence de valvules. Acta Cardiol. 1954;9(3):249–262. [PubMed] [Google Scholar]
  7. LEATHAM A., VOGELPOEL L. The early systolic sound in dilatation of the pulmonary artery. Br Heart J. 1954 Jan;16(1):21–33. doi: 10.1136/hrt.16.1.21. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. MILLER R. A., LEV M., PAUL M. H. Congenital absence of the pulmonary valve. The clinical syndrome of tetralogy of Fallot with pulmonary regurgitation. Circulation. 1962 Aug;26:266–278. doi: 10.1161/01.cir.26.2.266. [DOI] [PubMed] [Google Scholar]
  9. ONESTI S. J., Jr, HARNED H. S., Jr Absence of the pulmonary valve associated with ventricular septal defect. Am J Cardiol. 1958 Oct;2(4):496–501. doi: 10.1016/0002-9149(58)90337-0. [DOI] [PubMed] [Google Scholar]
  10. RUTTENBERG H. D., CAREY L. S., ADAMS P., ADAMS P., Jr, EDWARDS J. E. ABSENCE OF THE PULMONARY VALVE IN THE TETRALOGY OF FALLOT. Am J Roentgenol Radium Ther Nucl Med. 1964 Mar;91:500–510. [PubMed] [Google Scholar]
  11. Soulié P., Vernant P., Sterba S., Bouchard F., Albou E., Lanfranchi J., Letac B. Insuffisance pulmonaire congénitale et communication interventriculaire. (Etude clinique, hémodynamique et angiocardiographique) Arch Mal Coeur Vaiss. 1967 Feb;60(2):172–202. [PubMed] [Google Scholar]
  12. VENABLES A. W. Absence of the pulmonary valve with ventricular septal defect. Br Heart J. 1962 May;24:293–296. doi: 10.1136/hrt.24.3.293. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Wagenvoort C. A., Nauta J., van der Schaar P. J., Weeda H. W., Wagenvoort N. Effect of flow and pressure on pulmonary vessels. A semiquantitative study based on lung biopsies. Circulation. 1967 Jun;35(6):1028–1037. doi: 10.1161/01.cir.35.6.1028. [DOI] [PubMed] [Google Scholar]

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