Incidence and risk factors for mitral valve prolapse in severe adolescent idiopathic scoliosis.

Mitral valve prolapse (MVP) is known to be associated with thoracic skeletal anomalies. To determine the incidence and risk factors for mitral valve prolapse in the adolescent population with severe idiopathic scoliosis (IS), a prospective follow-up study on 139 adolescent patients with IS from the Pediatric Orthopedic Service was undertaken. Data collected included age, sex, medical and family history, physical exam, electrocardiogram and echocardiogram, spinal x-rays, and pulmonary function tests. MVP was detected by echocardiogram in 13.6% (19/139) of patients with IS as compared with 3.2% in 154 age- and weight-matched controls (p < 0.006). All patients with MVP were asymptomatic and a systolic click or murmur was detected on the single preoperative exam only in 37% (7/19) of them. Patients with MVP and IS weighed less (45.1 +/- 2.0 vs 51.8 +/- 0.1 kg, p < 0.002) as compared with those IS patients without MVP. The electrocardiogram was abnormal in 21% (4/19) of patients with MVP as compared with only 1.6% (2/120) of patients with IS but no MVP. The two groups did not differ with respect to age at diagnosis, severity of scoliosis, positive family history of scoliosis, or the presence of restrictive lung disease. Though IS was more prevalent in females (79%), the presence of MVP was not related to gender. MVP was persistent in 10 of the 19 patients reevaluated by echocardiogram 2-4 years after spinal surgery. We conclude that MVP is four times more common in patients with severe IS than in the normal adolescent population, and is associated with a lower body weight in IS patients with MVP than in IS patients without MVP. The persistent nature of MVP, even after corrective spinal surgery, may be related to factors other than geometric changes of the heart caused by abnormal thoracic curvature.

Late follow-up after venous switch operation (Mustard procedure) for simple and complex transposition of the great arteries.

Most adolescents and young adults born with complete transposition of the great arteries (TGA) and alive today are survivors of the Mustard operation. This study reports on the serial, long-term (from 10 to > 20 years) follow-up of 85 patients who underwent this operation between 1971 and 1981. Of these, 63 had simple and 22 complex TGA. The age at surgery ranged from 2 days to 17 years. The early mortality rate was 10.5% and the late mortality 9.2%. The actuarial survival rate after 15 years was 86% for simple and 64% for complex TGA. Event-free survival after 15 years was 77% for simple and 46% for complex TGA. Yearly review of electrocardiograms and, less frequently, of Holter tracings disclosed a lower mean resting heart rate and decrease over time in sinus rhythm and an increase in active arrhythmias. Fifty-two percent had resting sinus rhythm and 17% had active arrhythmias at 16 to 20 years of follow-up. Exercise stress testing in 21 patients revealed resumption of sinus rhythm during exercise but significant diminution of endurance time and peak heart rate response. Seven of the survivors (9.2%) required reoperation. Of these, 4 had severe tricuspid regurgitation following patch closure of ventricular septal defect. This study shows gratifying long-term and event-free survival for the majority of patients who underwent surgery by this venous switch procedure.