CONGENITAL PULMONARY VALVE REGURGITATION

CONGENITAL PULMONARY VALVE REGURGITATION (see also [For More Information] ). Because the degree of regurgitation is seldom more than moderate, and because the right ventricle readily adapts to low pressure volume overload, most patients tolerate this anomaly into adulthood, through middle age, and occasionally into their sixth, seventh, or even eighth decade.60–62 However, right heart failure may occur in older adults after decades of stability, because acquired cardiopulmonary disorders serve to increase the amount of regurgitation across the congenitally incompetent pulmonary valve.5

LUTEMBACHER’S SYNDROME

LUTEMBACHER’S SYNDROME. The disorder consists of a congenital atrial septal defect upon which acquired mitral stenosis is imposed.5 Mitral stenosis augments the left-to-right interatrial shunt, while the atrial septal defect decompresses the left atrium and reduces the mean left atrial pressure and the gradient across the stenotic mitral valve. Lutembacher’s patient was a 61-year-old woman who had seven pregnancies,63 and Firkett’s patient was a 74-year-old woman who had 11 pregnancies.64 The oldest recorded patient was an 81-year-old woman who experienced no cardiac symptoms until her 75th year.65 These favorable reports should not obscure the fact that survival in patients with ostium secundum atrial septal defects is unfavorably influenced by mitral stenosis that augments the left-to-right shunt and predisposes to atrial fibrillation and right ventricular failure.5

SINUS OF VALSALVA ANEURYSM

SINUS OF VALSALVA ANEURYSM (see also [For More Information] ). The malformation begins as a blind pouch or diverticulum that takes origin from a localized site in one aortic sinus.66 A substantial majority of ruptures occur before age 30 (rarely in infancy or early childhood), generally in men, at an average age of 34 years with a range of 11 to 67 years.66,67 The physiological consequences and clinical course depend upon the rapidity with which the rupture develops, the amount of blood flowing through the abnormal communication, and the chamber (site) that receives the shunt.68 Complete heart block is an occasional cause of syncope or sudden death when a ruptured or unruptured aneurysm penetrates the base of the ventricular septum.69 Small perforations may come to light because of an asymptomatic continuous murmur, because of a right ventricular outflow tract systolic murmur (subpulmonary obstruction by the aneurysm), or because of aortic regurgitation.5 Small chronic perforations are susceptible to infective endocarditis. About 20 per cent of congenital sinus of Valsalva aneurysms are unperforated and are discovered at necropsy, cardiac surgery, or operation for ventricular septal defect, which sometimes coexists with an aortic sinus aneurysm.68 In one of our patients, an 85-year-old man, an unsuspected unperforated aortic sinus aneurysm was diagnosed by echocardiography.

Date: 2014-12-21; view: 1143