Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children.

BACKGROUND: We compared the long-term results of surgical valvotomy (S) versus balloon valvuloplasty (BV) for pulmonary valve stenosis in infants and children. METHODS: Results after surgical pulmonary valvotomy (with concomitant ASD/VSD closure) (n = 62, age 2.9 +/- 3.5 years) and balloon valvuloplasty (n = 108, age 3.6 +/- 3.9 years) were analyzed. Transvalvular mean pressure gradient decrease, freedom from reintervention for restenosis, pulmonary valve insufficiency, and tricuspid valve insufficiency were considered. RESULTS: Mean pressure gradient decreased significantly more in the surgical group (from 64.8 +/- 30.8 mm Hg to 12.8 +/- 9.8 mm Hg at a mean follow-up of 9.8 years) than after BV (decreasing from 66.2 +/- 21.4 mm Hg to 21.5 +/- 15.9 mm Hg after a mean of 5.4 years; p < 0.001). Moderate pulmonary valve insufficiency occurred in 44% after surgery, and in 11% after BV (p < 0.001). Tricuspid valve insufficiency occurred in 2% after surgery, and in 5% after BV. Restenosis occurred in 3 surgical patients (5.6%), 2 patients required reoperation, and 1 patient required a balloon valvotomy. Restenosis developed in 13 BV patients (14.1%): 6 patients were redilated and 7 patients required surgery. Surgical valvotomy led to significantly less reinterventions than balloon valvuloplasty (p < 0.04). CONCLUSIONS: Surgical relief of pulmonary valve stenosis produces lower long-term gradients and results in longer freedom from reintervention. Balloon valvuloplasty may remain, despite these results, the preferred therapy for isolated pulmonary valve stenosis, because it is less invasive, less expensive, and requires a shorter hospital stay. Surgery should remain the exclusive form of therapy in the presence of concomitant intracardiac defects, which need to be addressed.

Prenatal diagnosis of the fetus with hypoplastic left heart syndrome management and outcome.

OBJECTIVES: To review our 13-year experience with prenatally detected hypoplastic left heart syndrome (HLHS) of which management remains controversial. MATERIAL AND METHODS: Retrospective study of the management and outcome in all cases of HLHS diagnosed prenatally in a tertiary referral center for pediatric cardiology and cardiac surgery between January 1988 and July 2001. RESULTS: The diagnosis of HLHS was made in 32 fetuses. One mother had two pregnancies associated with HLHS. In 16 cases parents opted for termination of pregnancy and in five for compassionate care. Four fetuses died in utero, and seven patients received a palliative reconstructive Norwood procedure. In seven fetuses, associated anomalies were detected: three chromosomal and structural and four only structural. In six fetuses, other associated intracardiac anomalies were detected. Of seven infants operated, six had no associated anomalies and only one is alive at an age of 17 months. CONCLUSION: The low percentage of intention to treat among patients in our center (34%) is in accordance with the percentage found in another study from the UK (36.2%), but differs significantly from reported series across the Atlantic (67%). Prenatal diagnosis of the HLHS provides opportunities not only for getting patients in optimal preoperative condition when surgery is offered, but also for in-depth counseling of the parents on this severe malformation. A minority of parents faced with the difficult decision of possible termination of pregnancy, compassionate care or the Norwood strategy, choose surgical treatment which might be based on socioreligious differences and the interpretation of the long-term quality of life.

Comparable systemic ventricular function in healthy adults and patients with unoperated congenitally corrected transposition using MRI dobutamine stress testing.

BACKGROUND: Failure of the systemic right ventricle (RV) often complicates adult survival in unoperated or physiologically repaired congenitally corrected transposition of the great arteries (CCTGA). Healthy controls schematically represent an optimal outcome of anatomic repair, which is increasingly performed to treat CCTGA. Magnetic resonance imaging dobutamine stress testing measures cardiac reserve, and sets to compare the left ventricle of controls with the systemic RV of unoperated and physiologically repaired patients with CCTGA. METHODS: Baseline and stress magnetic resonance imaging (maximum dobutamine dose, 15 microg/kg/min) assessed systemic RV function in 13 minimally or asymptomatic adult patients with CCTGA (unoperated, n = 7; physiologically repaired, n = 6). The left ventricles of 11 healthy age-matched adults served as controls. RESULTS: Baseline and stress end-diastolic volumes similar between the systemic RV of unoperated patients and the left ventricle of controls, as well as base end-systolic volumes. Stress ejection fraction was lower in unoperated and physiologically repaired patients (70 +/- 6% and 60 +/- 5%, respectively, vs healthy controls (84 +/- 8%). However, comparable with healthy controls, both subsets of CCTGA patients responded appropriately to dobutamine stress, as illustrated by similar RV stroke volume, heart rate, mean blood pressure, and cardiac index. CONCLUSIONS: Compared with the left ventricles of healthy controls, both patient groups had larger systemic RV volumes, diminished ejection fraction, but an appropriate response to dobutamine stress. Values of unoperated patients are closer to normal than physiologically repaired patients. Magnetic resonance imaging dobutamine may help to define the subgroups of CCTGA patients with favorable anatomy, whereby asymptomatic adult survival could be anticipated without the need for an operation.