Willem A. Helbing

Objectives Data on long-term outcomes after surgical repair of pulmonary valve stenosis are limited. This study evaluated survival, clinical outcomes and quality of life (QoL) after surgery during childhood. Methods Single centre, longitudinal cohort study evaluating consecutive patients with pulmonary valve stenosis who underwent surgical repair between 1968–1980 and were evaluated every decade since 1990. Results Of the original cohort of 89 operated patients, 11 died (12%), including 2 who died within 30 days postsurgery (2%), and 7 (8%) were lost to follow-up. Survival at 50 years follow-up was 87%, which was not significantly different from the GDP. Of the remaining 71 survivors, 32 refrained earlier from participating in this cohort study, leaving 39 eligible, of whom 34 (87%) participated again (50% male, median age 48 years) with a median follow-up of 45 (range 40–52) years. Event-free survival was 50%, with supraventricular tachycardia (14%) and reintervention (13%) being the most frequent events, although less frequently in the last 10 years. At last follow-up, biventricular function was preserved in most patients. Reduced right and left ventricular ejection fraction (EF) was found in 33% and 13%, respectively. Exercise capacity and maximum rate of oxygen consumption were mildly impaired in 14% and 32% of patients. Patients who underwent an infundibulectomy during initial surgery were significantly more likely to undergo reintervention (HR=8.32, p=0.003). Patient-reported QoL scores remained stable over time and consistently exceeded those of the age-matched GDP. Conclusion Fifty-year survival after surgery for pulmonary valve stenosis was excellent and comparable to the GDP. Most patients maintained preserved ventricular function, functional capacity and excellent QoL. Routine lifelong follow-up may not be necessary for all patients, but should be considered for those who underwent an infundibulectomy or have residual lesions.

Introduction Reduced exercise performance is common in patients with repaired tetralogy of Fallot (rToF) and relates to adverse outcomes. The effects of high-intensity interval training (HIIT) in rToF are unknown. Furthermore, it is unknown if home-based, online-monitored exercise training is effective in rToF. We aimed to examine the feasibility, safety and effects of home-based HIIT in patients with rToF. Methods Patients aged 12–30 years (n=34, median age 16 (IQR 14–23)) with rToF were randomised to a 12-week home-based HIIT programme or standard of care (SOC). HIIT was prescribed in three 30 min sessions/week, with heart rate telemonitoring. SOC included exercise as usual. Co-primary end points were mean differences in peak oxygen consumption (VO2) and maximal workload, analysed using linear mixed-effects (LME) models. Secondary end points were: echocardiography, ECG and MRI-derived measures of cardiac structure and function. Results No serious adverse events occurred. After HIIT, peak VO2 increased from 38.3 (34.0–42.5) mL/kg/min to 40.9 (36.6–45.2) mL/kg/min (∆ 4.0 mL/kg/min, compared with control period (95% CI 1.52 to 6.53)), workload increased from 204 (174–234) W to 218 (188–248) W (∆ 17 W, compared with control period (95% CI 5 to 30)). No changes in cardiac volumes and ejection fraction were observed after HIIT. VO2 improved after HIIT independent of baseline fitness, body mass index and biventricular size. Notably, lower right ventricular ejection fraction (RV EF) was associated with greater improvement. Conclusion HIIT significantly enhances exercise capacity in adolescents and young adults with rToF without adverse effects on cardiac structure or function, although the sample size limits the detection of subtle changes and can be successfully performed at home with online monitoring. This intervention offers a feasible alternative to traditional, on-site supervised exercise programmes, for patients with rToF, including those with lower RV EF. Trial registration number NTR2731.

We used a porcine model of sequential loading with pulmonary artery banding and transannular patch mimicking tetralogy of Fallot to test ventriculo-arterial coupling as a marker of early right ventricular dysfunction. Ventriculo-arterial coupling is preserved despite right ventricular dysfunction and afterload is decreased. Pulmonary regurgitation results in low afterload following pulmonary artery pressure drop during diastole. Early systolic ejection is increased and correlates with ventriculo-arterial coupling.

BACKGROUND: Very preterm-born infants are at risk for developing bronchopulmonary dysplasia (BPD), a chronic lung disease. Nowadays, the majority of these infants reach adulthood. Very preterm-born young adults are at risk for developing pulmonary arterial (PA) hypertension later in life. An early sign of PA hypertension is increased PA stiffness. This study aims to use cardiovascular magnetic resonance to compare PA stiffness using PA relative area change (RAC) and pulse wave velocity (PWV) to identify early signs for PA hypertension in young adults born very premature, with and without BPD. METHODS: Twenty preterm-born young adults with and 20 without BPD underwent cardiovascular magnetic resonance and were compared with 20 at-term-born young adults. RAC was calculated as the percentage change between the maximal and minimal areas of the PA. PWV was calculated using a method that simultaneously compares flow and area increase in the pulmonary artery during early systole. RESULTS: In 57 of 60 patients, PWV and RAC measurements could be performed. Preterm-born young adults with BPD showed increased PWV compared with preterm-born young adults without BPD (median [25th–75th percentile] 2.07 m/s [1.45–3.05] versus 1.61 m/s [1.18–1.85]; P =0.04) and at-term-born young adults (1.35 m/s [1.08–2.23]; P =0.04). RAC was decreased in both preterm-born young adults with (62% [56–82]; P P <0.01), compared with at-term-born young adults (101% [87–122]). CONCLUSIONS: Preterm-born young adults with BPD show increased PA stiffness as measured by PWV compared with preterm-born young adults without BPD and at-term-born young adults; RAC was decreased in both preterm-born groups compared with at-term controls. This noninvasive method of measuring PA stiffness might be a valuable tool to identify individuals at risk for early signs of PA hypertension in this population.

Background: Lymphatic disorders such as protein‐losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers. Methods and Results: A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty‐one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178 ‐ 313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein‐losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1 ‐ 4) procedures per year and 12 (interquartile range, 5 ‐ 15) interventions in total per center. Conclusions: This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence‐based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.

Willem A Helbing; The obesity epidemic meets complex congenital heart disease: challenges for prevention., European Journal of Preventive Cardiology, , zwa