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Background— The timing of pulmonary valve replacement (PVR) for free pulmonary incompetence in patients with congenital heart disease remains a dilemma for clinicians. We wanted to assess the determinants of improvement after PVR for pulmonary regurgitation over a wide range of patient ages and to use any identified predictors to compare clinical outcomes between patient groups. Methods and Results— Seventy-one patients (mean age 22±11 years; range, 8.5 to 64.9; 72% tetralogy of Fallot) underwent PVR for severe pulmonary regurgitation. New York Heart Association class improved after PVR (median of 2 to 1, P <0.0001). MRI and cardiopulmonary exercise testing were performed before and 1 year after intervention. After PVR, there was a significant reduction in right ventricular volumes (end diastolic volume 142±43 to 91±18, end systolic volume 73±33 to 43±14 mL/m 2 , P <0.0001), whereas left ventricular end diastolic volume increased (66±12 to 73±13 mL/m 2 , P <0.0001). Effective cardiac output significantly increased (right ventricular: 3.0±0.8 to 3.3±0.8 L/min, P =0.013 and left ventricular: 3.0±0.6 to 3.4±0.7 L/min, P <0.0001). On cardiopulmonary exercise testing, ventilatory response to carbon dioxide production at anaerobic threshold improved from 35.9±5.8 to 34.1±6.2 ( P =0.008). Normalization of ventilatory response to carbon dioxide production was most likely to occur when PVR was performed at an age younger than 17.5 years ( P =0.013). Conclusions— A relatively aggressive PVR policy (end diastolic volume <150 mL/m 2 ) leads to normalization of right ventricular volumes, improvement in biventricular function, and submaximal exercise capacity. Normalization of ventilatory response to carbon dioxide production is most likely to occur when surgery is performed at an age ≤17.5 years. This is also associated with a better left ventricular filling and systolic function after surgery.

Improved medical care of congenital heart disease patients increased survival into adulthood from 15% in the 1960s to over 85% in the current era. As a consequence, the prevalence of adult congenital heart disease (ACHD) increased rapidly, 1 which is estimated to be .1 million ACHD patients in North America and 1.2 million in Europe. The growing number and aging of ACHD patients led to an overall increase in hospitalizations over the last decade and a substantial increase in patients presenting with heart failure (HF) ( 20%). 2 The incidence of first HF-admission was 1.2 per 1000 patientyears in the Dutch national 'CONCOR' registry. Patients admitted with HF had a five-fold higher risk of death than those not admitted. From the same registry, the mortality was 2.8% during a follow-up period of 24 865 patient-years. Chronic HF (26%) and sudden death (19%) were recorded most frequently. The median age at death from HF was 51.0 years (range: 20.3 -91.2 years). 3 In another ACHD cohort, sudden death (26%) was the most common cause of death, followed by progressive HF (21%) and perioperative death (18%). 4 Although patients with ACHD may not readily report symptoms, clinical HF is documented in 22.2% of patients with a Mustard

Background-Percutaneous pulmonary valve implantation was introduced in the year 2000 as a nonsurgical treatment for patients with right ventricular outflow tract dysfunction. Methods and Results-Between