Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect.

Abstract

Clinical and morphological findings are described in 2 adults with combined valvular pulmonary and aortic stenosis with atrial septal defect, an extremely rare combination of lesions. The direction of the shunt via the atrial septal defect in this combination appears to be dependent on the relative degree of outflow obstruction on the right side compared with the left side of the heart. In 1 patient the degree of obstruction at the pulmonary valve was nearly three times that at the aortic valve and, therefore, the shunt was right to left and the patient was severely cyanotic. In the other patient, the degree of obstruction at the pulmonary valve was slightly less than that at the aortic valve and, therefore, the shunt was entirely left to right and the patient was acyanotic. Diagnosis of an associated obstructive lesion on the left side of the heart in a patient with an obstructive lesion on the right side of the heart is essential because operative relief of the right-sided obstruction without relief of the left-sided obstruction may lead to rapid, fatal pulmonary congestion.

A neonate with isolated combined aortic and pulmonary valvar stenosis

“…Residual pulmonary valvular systolic gradient remained at 8 mm Hg with a mild pulmonary regurgitation and the aortic gradient decreased to 16 mm Hg at the closure of the ductus arteriosus. The extraordinary combination of isolated valvar aortic and pulmonary stenosis is extremely rare and has been seldom reported without any other structural cardiac abnormality [1][2][3][4][5]. In our case, the right sided obstruction was markedly pronounced when compared to the left obstruction and poorly tolerated justifying the pulmonary valvuloplasty.…”

Combined Semilunar Valve Stenoses in Neonates: Management Approaches and Literature Review

“…The combination of semilunar valve stenosis is extremely rare. Older reports described this entity in ages beyond infancy [1,15,17]. At that time, the only available treatment option was surgery and the outcome was poor.…”

“…In absence of an adequate inter-atrial communication, this causes pulmonary venous congestions and elevation of the pulmonary vascular resistance. In such combination, the symptoms depend on the relation between the degree of the right-and left-sided obstruction as well as the presence or absence of an inter-atrial communication, and the shunt direction [17].…”

“…However, the combination of right and left ventricular outflow tracts obstruction is extremely rare. This combination had been reported beyond infancy sporadically in form of single case reports [1,10,15,17]. Patients presented in neonatal age are usually in critical condition.…”

Biventricular Outflow Obstruction Associated With Atrioventricular Septal Defects and Patent Ductus Arteriosus: An Extremely Rare Combination

“…It was speculated that an early presentation indicated severe disease. Almost all the other published cases in the literature were associated with congenital cardiac lesions: ASD was present in nine cases [ 5 - 7 , 10 , 13 - 14 ], VSD was identified in eight cases [ 7 , 9 - 11 , 15 - 16 ], and an atrioventricular canal defect was described in one case [ 12 ]. To the best of our knowledge, our case makes a unique combination to the medical literature on the topic in which the presence of combination of two left-to-right intracardiac shunts (ASD and VSD) and one left-to-right shunt at the great arteries level (i.e., PDA) were first described.…”