Mitral Valve Prolapse in Young Patients.

In brief: From 1% to 5% of young individuals are found to have, on clinical or echocardiographic examination, a prolapsing mitral valve. The majority of these patients are asymptomatic, and require from the physician an explanation of this defect and reassurance that the condition usually remains mild. Beta-blocking drugs are prescribed for patients with disabling chest pain, dizziness, or palpitation, or if potentially serious rhythm disorders develop. Physicians caring for these young patients must manage each case individually and must remain mindful that the natural course studies come from a perspective of only 25 years.

Aortic thrombosis in the neonate.

The case histories of two infants with aortic thrombosis are presented. Clinically, an obstructive aortic arch lesion was suspected because of a systolic pressure gradient between the upper and lower extremities in one infant and unequal peripheral pulses with lower limb cyanosis in the other. Noninvasive studies were inconclusive, the only abnormality noted echocardiographically being severe left ventricular dysfunction. Cardiac catheterization including angiography defined the severity of the obstruction and location of the thrombus in both babies. The thrombus originated within a ductus arteriosus aneurysm in one infant and was located in the ascending aorta and transverse arch in the other. Although successful surgical removal was accomplished, both babies died unexpectedly in the early postoperative period.

Development of pulmonary arteriovenous shunt after superior vena cava-right pulmonary artery (Glenn) anastomosis. Report of four cases.

Four patients with cyanotic congenital heart disease who had previously undergone superior vena cava-right pulmonary artery (Glenn) anastomosis developed pulmonary arteriovenous malformations that resulted in significant intrapulmonary right-to-left shunting. This abnormality was documented by selective angiography, oximetry, and contrast echocardiogrphy. It may be a major cause of late clinical deterioration in patients treated with the Glenn anastomosis.

Ebstein's malformation. Surgical experience at the Mayo Clinic.

At the Mayo Clinic, 13 patients with Ebstein's malformation have undergone surgical repair since 1963. Their ages ranged from 18 months to 51 years (median 13 years). Ten patients were in Functional Class III or IV. Marked cardiomegaly, cyanosis, paradoxic emboli, and dysrhythmias secondary to Wolff-Parkinson-White syndrome were indications for operation in the remaining 3 patients. A wide range of anatomic variations was encountered. All 5 patients who underwent tricuspid annuloplasty with plication of the atrialized segment of the right ventricle survived operation. Of 5 patients who underwent prosthetic valve replacement, only one survived. Other procedures included atrial septal defect closure alone in one patient, atrial septal defect closure and relief of pulmonary stenosis in one patient, and tricuspid annuloplasty alone in one patient. One patient had concomitant mapping and division of anomalous conduction pathways. Functional classification improved in 8 of 10 operative survivors. There were two late sudden deaths; both patients had had preoperative dysrhythmias. The data suggest that results are improved when the atrialized segment of the right ventricle is dealt with during repair of the tricuspid valve. A combined ventricular plication and tricuspid annuloplasty yielded better early and late results than did valve replacement.

Truncus arteriosus and previous pulmonary arterial banding: clinical and hemodynamic assessment.

Twenty-seven patients with truncus arteriosus and previous pulmonary arterial banding were evaluated 1 1/2 to 14 years (mean 7 years) after banding. Ages at the time of cardiac catheterization ranged from 3 to 18 years (mean 9 years). Current symptoms were severe in five patients and were related to truncal valve incompetence or decreased pulmonary blood flow (or both) rather than to age, duration of palliation or band location. Twenty-one of 22 patients with two pulmonary arteries were considered to be in a hemodynamically operable state at the time of study. The condition of three of five patients with a single pulmonary artery was subsequently found inoperable because of severe pulmonary vascular disease in the lung supplied by the single pulmonary artery. In patients with two pulmonary arteries, demonstration of low pressure in at least one normal-sized pulmonary artery established operability. Postoperative pressure measurements correlated well with preoperative prediction of operability, with 19 of 20 patients having a pulmonary arterial pressure less than 70 percent of systemic levels after repair. Bilateral pulmonary arterial binding may be more effective than central arterial banding (which frequently produces severe obstruction to the right pulmonary artery) in preventing pulmonary vascular obstructive disease in patients with truncus arteriosus who have two pulmonary arteries. Patients with truncus arteriosus and a single pulmonary artery with pulmonary arterial banding remain at high risk for the development of pulmonary vascular obstructive disease.