The Year in Congenital Heart Disease

Abstract

With the increasing number of excellent papers related to congenital heart disease, it has become more difficult to select those that are perceived to be particularly critical to scientific developments in the field. I have chosen in this review to focus almost entirely on results and to follow surgical-or catheterbased interventions that highlight the complex conditions that surgeons and cardiologists now are able to repair or palliate successfully. Sections chosen are tetralogy of Fallot (TOF), Fontan operation, cardiac surgery in general, transposition of the great arteries (TGA), hypoplastic left heart syndrome (HLHS), aortic valve surgery, aortoplasty, and ventricular septal defect (VSD) management. These particular areas were chosen because of the importance to the general field of congenital heart disease, and many of the areas highlighted continue to plague investigators as they attempt to improve long-term outcomes. TOFPapers about TOF include issues related to initial intervention assessment of pulmonary regurgitation (PR) timing of pulmonary valve replacement (PVR) as well as late follow-up and implication for management.Tamesberger et al.(1) reported 90 consecutive infants who underwent early repair: 25 neonates repaired due to duct-dependent pulmonary circulation or severe hypoxemia and 65 infants Ͻ4 months old with elective repair. Their results indicated no 30-day mortality, and late mortality of 2% with median follow-up of 4.7 years. Seven of 88 (8%) needed reoperation and 12 of 88 required reintervention. The younger patients did not differ significantly in intensive care unit stay, ventilator days, hospital stay, complications, or reoperation. There were more frequent transannular patches and reinterventions in the younger infants. These are excellent results, but will such early repair result in more severe PR and the need for more frequent PVR? Dohlen et al.(2) reported right ventricular outflow tract (VOT) stenting in 9 infants because of cyanosis or ductdependency and adverse risk factors. Stenting improved O 2 saturation from 73% to 94%. Median left pulmonary artery (PA) z-score increased from Ϫ4.9 to Ϫ1.5, right PA increased from Ϫ3.7 to Ϫ0.8, and Nakata index increased from 56 to 150 mm 2 /m 2 . There were no procedural complications. Six patients have undergone repair with no deaths. This is another method to palliate infants who are duct-dependent with adverse risk factors and have a pulmonary valve that is deemed unsalvageable. Stenting allows infants to obtain a greater age and size when repair theoretically is safer.Papadopoulos et al. (3) reported repair of pulmonary valves in 5 children and 2 adults. All had severe PR associated with right ventricular (RV) dilation and dysfunction after primary right VOT reconstruction. There were no operative or late deaths. All valves were repaired successfully with a mean PR grade of 1.3 post-operatively. Mean transvalvular gradient was 20 mm Hg for children and 23 mm Hg for adults. The mean RV dilation index decreased significantly from 0.85 to...

Congenital Heart Disease in the Adult

“…Patients in this group who develop moderate-to-severe PR undergo PV replacements as adults but without consistent improvement of RV systolic function postoperatively. 55 Overall postoperative survival in patients with TOF is approximately 90% at approximately 30 years after surgery. 56 …”