The syndrome of pulmonary stenosis with patent foramen ovale

Abstract Two cases of pulmonary stenosis with intact interventricular septum and patency of the foramen ovale are reported, with an analysis of twenty-seven additional autopsied cases from the literature. This series is compared with the reports of autopsied cases of pulmonary stenosis with both septa closed, of the tetralogy of Fallot and of the Eisenmenger syndrome. Pulmonary stenosis with patent foramen ovale is characterized by chronic cyanosis, polycythemia and clubbing. In the degree of cyanosis it occupies an intermediate place between the tetralogy of Fallot and the Eisenmenger syndrome. Pulmonary stenosis with closed septa is essentially a noncyanotic lesion. On the basis of cyanosis, cases of pulmonary stenosis with and without patency of the foramen ovale are placed in different classes of congenital heart disease. Otherwise these two diseases are clinically and pathologically very similar and differ from the tetralogy of Fallot in important respects. The most distinctive feature of pulmonary stenosis with patent foramen ovale is the x-ray appearance of the cardiac shadow which is characterized by a poststenotic dilatation of the pulmonary artery and its branches. This again places the condition in an intermediate position between the small shadows of the pulmonary vessels in the tetralogy of Fallot and the very prominently dilated and congested pulmonary vessels of the Eisenmenger syndrome. Other clinical features and pathologic findings of this lesion are discussed and evidence is presented that it is a well defined clinical entity with enough distinctive features to make possible diagnosis during life. Next to the tetralogy of Fallot it is the most important congenital cardiac lesion in adults with chronic cyanosis, polycythemia and clubbing. Pulmonary stenosis with patent foramen ovale represents a conspicuous exception to the rule that cyanotic congenital heart disease with dilated pulmonary arteries is unsuitable for surgical relief.