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J Am Coll Cardiol, 2004; 44:23-24 © 2004 by the American College of Cardiology Foundation |
* Stanford University Medical Center, Palo Alto, California, USA
*
Reprint requests and correspondence: Dr. Daniel J. Murphy, Jr., Stanford University Medical Center, Pediatric Cardiology, 750 Welch Road, Suite 305, Palo Alto, California 94306, USA.
murphyd{at}stanford.edu
The field of congenital cardiology is dominated by surgical repair and catheter intervention for residual or recurrent structural disease and, increasingly, as a primary therapy for structural disease. There were a number of abstracts concerning catheter closure of atrial septal defects (ASDs) and patent foramen ovale. Those studies suggest that closure of these defects in the catheterization laboratory preserves ventricular function as compared with surgery (1), may actually reduce migrainous events (2), reduces right ventricular (RV) size, and improves symptoms. Several studies have demonstrated that ASD closure is beneficial even in patients older than 50 to 60 years (3,4). Also, two studies (5,6) showed that an adequate septal rim may or may not be necessary for closure success and device stability. These devices are currently under investigation and improvement, and we expect more information on outcomes at future Scientific Sessions.
Pacing is becoming increasingly important, both in the pediatric age range and also in adults who survive childhood CHD. In a study from Stanford University and the University of Chicago (7), six patients with congenital complete atrioventricular block were converted to biventricular pacing. All of the patients had been treated for heart failure and several were listed for heart transplant before biventricular pacing conversion. In this group of children, the ejection fraction increased from 34% to 57%, and five of six had improved physical growth.
In another study from the University of Chicago (8), multisite pacing was employed in 30 patients with univentricular heart. The use of multisite pacing produced a increased cardiac index in 15 of 16 patients in whom it was measured. Multisite pacing also resulted in a decreased QRS duration, which may be a marker for the risk of sudden death, in 28 of 30 patients. Finally, in a novel use of three-dimensional echocardiography, a marked decrease in asynchrony was demonstrated with the use of multisite pacing in this population.
In an acute study from Cincinnati Children's Hospital (9), eight patients with tetralogy of Fallot underwent acute RV resynchronization therapy during their stay in the cardiac intensive care unit. Atrioventricular sequential pacing improved hemodynamic parameters, but there was no demonstrated outcome benefit with resynchronization. A spirited discussion about what defines RV resynchronization and what sites should be used ensued, and we can expect more studies related to novel pacing modes in the pediatric and congenital cardiac population.
Many patients with congenital cardiac defects have right-sided heart defects involving the pulmonary valves. Pulmonary valvotomy, early in childhood, generally results in significant pulmonary regurgitation. Pulmonary valve replacement is frequently necessary at some point in time and may be beneficial at an early age. A large number of studies were presented at this meeting regarding pulmonary valve replacement. These studies demonstrate that pulmonary valve replacement reduces RV volume and shortens the QRS duration (10). Pulmonary valve replacement also improves functional capacity, as measured by peak oxygen consumption (11) and normalizes RV volume, but only if performed before the RV volume reaches 150% of normal size measured by magnetic resonance imaging (12).
Finally, in a provocative retrospective review from the Mayo Clinic (13), investigators studied 10 patients in whom mechanical pulmonary valves were placed. In this series, there was only one valve replacement, and that was for outgrowth because of size. There were no valve failures.
Hypoplastic left heart syndrome has historically been one of the most deadly forms of CHD, and surgical strategies and medical management have improved the survival rate. In a single-center series, investigators from the Children's Hospital of Wisconsin reported 50 of 51 hospital survivals (14). Twenty-seven of those patients had interstage events at home between that surgery and their next surgery, with only one death, for an overall survival to the second stage of 49 of 51 infants. This is really remarkable for a lesion that was 100% fatal 20 years ago.
On a more sobering note, a consortium report from 29 surgical centers (15) demonstrated that better surgical survival was associated with high institutional volume, and that there was no relation to the number of procedures performed by an individual surgeon. Of concern, however, many of the centers had poor outcomes, with survival rates as low as 25% in this population.
Neurodevelopmental outcomes after neonatal repair of total anomalous pulmonary venous return was the subject of a study from the Children's Hospital of Philadelphia (16). The researchers demonstrated that for the group as a whole, there were somewhat lower performance IQ scores and impaired fine motor skills. Clearly, there is a challenge to not only allow these children to survive, but also to improve their neurocognitive outcomes and quality of life.
Congenital cardiology also includes care for fetuses with CHD. Investigators from Virginia Commonwealth University (17) presented a large retrospective review of fetuses with restriction or closure of the foramen ovale and showed that this particular occurrence in the fetus is extremely high risk: the process of foramen restriction was shown to progress, and four of the six babies died after birth.
In a very provocative presentation from the group at Boston Children's Hospital, who last year at this meeting presented their initial experience with aortic valve dilation in the fetus, in utero creation of an ASD was described (18). The study population comprised seven fetuses with a hypoplastic left heart and closed or restrictive ASDs. There was no maternal mortality and only one intrauterine death. Five of the infants were live-born at term and four of them are alive. This experience suggests that fetal atrial septostomy is feasible and appears to be safe for the mother and relatively safe for the fetus.
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