The Year in Congenital Heart Disease

Abstract

Long-Term Management of Tetralogy of Fallot (TF)Articles related to long-term management of the status of older children and young adults after repair of TF include issues related to assessment of pulmonary regurgitation and timing pulmonary valve replacement as well as indications for and results of implantation of defibrillators.Gengsakul et al.(1) report a matched cohort study of 82 patients with pulmonary valve replacement (PVR) after TF repair without a history of ventricular arrhythmia who were compared with non-PVR control subjects. Oxygen consumption at peak exercise did not change in either group. For PVR subjects, there was a significant reduction in pulmonary valve regurgitation (PR), tricuspid valve regurgitation (TR), and right ventricular (RV) size and dysfunction on echocardiography. Symptoms and functional class improved after PVR, but a change in arrhythmia was not detected. There were no sudden deaths in the PVR subjects, whereas 4 patients died suddenly in the non-PVR group.Meijboom et al.(2) report on 67 patients who were followed from 15 Ϯ 3 years until 27 Ϯ 3 years after TF surgery. Twenty-two patients had mild-to-moderate PR, and no significant changes occurred during follow-up. Of 45 patients with severe PR and severe RV dilation, 28 (62%) remained free of symptoms and did not undergo PVR. No additional changes in RV size or exercise capacity were found. Seventeen patients had symptoms and underwent PVR; 9 of 17 improved clinically and echocardiographically, and QRS duration shortened. Refraining from PVR in asymptomatic patients led to no measurable deterioration in 25 of 28 patients. Referring symptomatic patients for PVR led to improvement in 9 of 17 patients. This article highlights the dilemma in following asymptomatic patients with significant PR. Although most showed no measurable deterioration, in 24% there were questionable or unsatisfactory results for this type of management.Henkens et al.(3) analyzed the influence of pre-operative PR severity and RV size and function on outcome in 27 adults who had cardiac magnetic resonance imaging (MRI) before and after PVR. PR was not related to RV dimensions or function before PVR, and severity of PR did not influence changes in RV dimensions after PVR. These data suggest that the timing of PVR should be based on indexed RV end-systolic volume and corrected RV ejection fraction (EF) rather than severity of PR. It is unclear how PR cannot be related to RV dimensions in this article. Nevertheless, the preceding 3 reports emphasize that diligence is needed in follow-up of these patients and MRI, plus exercise studies can help in providing optimal timing for PVR before deterioration of RV function occurs. This can be a difficult enterprise despite all of our efforts. If we had a perfect valve it would be much easier, but such is not the case.Khairy et al. (4) report on implantable cardioverterdefibrillators (ICDs) in TF patients. A total of 121 patients were enrolled and followed for a median of 3.7 years. The ICDs were implanted for primary pr...