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Background: The long-term evolution of pulmonary vascular resistance (PVR) after ventricular septal defect (VSD) repair is unknown. This study serially evaluated resting and exercise PVR after VSD repair in childhood., Methods: Patients were enrolled from the outpatient Adult Congenital Heart Disease clinic of the University Hospitals Leuven and compared to age- and gender-matched controls. Participants underwent resting and exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. Total PVR was calculated as the ratio of mean pulmonary artery pressure (mPAP) to cardiac output (CO). The slope of the mPAP-CO curve (exercise PVR) was obtained using linear regression analysis., Results: Twenty-seven patients (mean age 31 ± 7 years, 70% male) and 18 controls were included. At baseline, patients had larger right ventricular (RV) end-diastolic areas (10 ± 2 vs 9 ± 1 cm 2 /m 2 , p = 0.001) and lower tricuspid annular plane systolic excursion (TAPSE) (17 (17-19) vs 26 (22-28) mm, p < 0.001). After 1.1 (1.0-1.5) years follow-up, similar differences in RV areas and TAPSE were found. Patients reached lower peak workload and cardiac index compared to controls at each time point. Peak total PVR was higher (Baseline: 2.7 ± 0.8 vs 2.2 ± 0.3 mm Hg/L/min, p = 0.005; Follow-up: 2.9 ± 0.9 vs 2.1 ± 0.3 mm Hg/L/min, p < 0.001) and the mPAP-CO slope was steeper (Baseline: 2.2 ± 0.8 vs 1.7 ± 0.3 mm Hg/L/min, p = 0.008; Follow-up: 2.5 ± 0.9 vs 1.6 ± 0.3 mm Hg/L/min, p < 0.001) in patients. The mPAP-CO slope in patients correlated inversely with peak oxygen uptake (R = -0.41 and - 0.45, p = 0.036 and 0.022, baseline and follow-up, respectively)., Conclusion: Despite repair, VSD patients seem to show altered pulmonary hemodynamics and RV impairment at rest and exercise, supporting life-long follow-up., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: When elevated pulmonary artery pressure (PAP) is assessed by echocardiography, right heart morphology is always considered. The goal of this study was to evaluate how right heart geometry changes with increasing right ventricular pressure load., Subjects and Methods: Data from patients undergoing transthoracic echocardiography with subsequent right heart catheterization within a time period of 6 months were retrospectively analysed. First, Spearman-rho coefficients between mean PAP and right heart parameters were calculated. Second, the population was divided into tertiles according to mean PAP and Kruskal-Wallis variance analysis between variables was performed., Results: Fifty-four patients (23 female, median age 77 years, IQ range 63-83) were selected. Mean PAP (median 27 mmHg, IQ range 24-36), right atrial (RA) dilatation (median 1, IQ range 0-2), tricuspid insufficiency (TI) severity (median 1.5, IQ range 0-2) and right ventricular (RV) dilatation (median 0, IQ range 0) were included. Significant correlations with mean PAP were found for RA dilatation (rho = 0.380; P = 0.005) and TI severity (rho = 0.294; P = 0.032). No correlation with RV dilatation could be shown (rho = 0.241; P = 0.081). Across the tertiles [(1) mean PAP 25 mmHg; (2) mean PAP 26-30 mmHg; (3) mean PAP > or = 31 mmHg)], variance analysis showed a significant increase in RA dilatation (P = 0.009) and TI severity (P = 0.040). No change in RV dilatation across groups was observed (P = 0.216)., Conclusions: RA dilatation and TI severity significantly increase with increasing RV pressure load. No increase in RV dilatation was observed in the studied population. Hence, absence of RV dilatation does not exclude the presence of elevated PAP.

Objective: To evaluate if development of fragmented QRS (fQRS) complexes, a marker of inhomogeneous ventricular activation due to myocardial fibrosis, is associated with adverse outcome in adults after Mustard/Senning repair for d-transposition of the great arteries (d-TGA).Methods: Adults with atrial switch repair for d-TGA were selected from the database of a tertiary care hospital. Exclusion criteria were systemic right ventricular (RV) assist device or heart transplantation (HTx) before the age of 16, or fQRS already present at first visit to the Adult Congenital Heart Disease clinic. A blinded expert reader retrospectively analysed all available ECGs after the age of 16 for the presence of fQRS. The appearance of fQRS was modelled for each patient as a time-dependent variable. Cox regression was performed to assess the relationship between covariates and the composite endpoint of cardiovascular mortality, HTx or systemic RV assist device.Results: Records of 89 patients (34% female, 42% Mustard repair) were analysed. At latest follow-up, fQRS was noted in 26 patients (29%). Over a median follow-up time of 16.9 (IQR 12.6-22.9) years, the composite endpoint occurred in nine patients (10%). In multivariable Cox analysis, appearance of fQRS (HR 14.11; 95% CI 1.42 to 140.12) and development of severe RV dysfunction (HR 11.36; 95% CI 2.08 to 62.17) were significantly associated with the composite endpoint.Conclusions: Appearance of fQRS complexes on a 12-lead ECG is associated with adverse outcome in adults after atrial switch repair for d-TGA. In this population, fQRS detection might be a promising and easily implementable tool to identify patients at risk for adverse events. [ABSTRACT FROM AUTHOR]

Background Discharge from follow-up after closure of isolated congenital shunt lesions in childhood was common practice in the past. The aim of the present study was to recall these patients to evaluate their current status. Methods Patients included in the database of pediatric and congenital heart disease of our tertiary center with repaired secundum atrial septal defect (ASD) or ventricular septal defect (VSD) before the age of 18 years, and discharged from follow-up, were invited for clinical and echocardiographic check-up. Results Forty-six ASD patients (age 30 ± 7 years, 37% male) responded. Median age at ASD repair was 6 (IQR 4–8) years. All but one functioned in NYHA class I. Eight (17%) patients reported palpitations. No patient developed pulmonary hypertension (PH). Right ventricular (RV) dilatation was present in 7 (15%). RV fractional area change (FAC) was < 35% in 7 (15%), TAPSE < 17 mm in 12 (26%). Forty-seven VSD patients (age 34 (IQR 29–40) years, 57% male) participated. Median age at VSD repair was 4 (IQR 1–5) years. Six (13%) patients functioned in NYHA class II. Seventeen (36%) patients reported palpitations. Four (9%) patients presented PH. Left ventricular dilatation was present in 4 (9%), RV dilatation in 6 (13%). RV FAC was < 35% in 7 (15%), TAPSE < 17 mm in 17 (36%). Seven (15%) patients had dilated ascending aorta. Conclusions Patients with closure of isolated secundum-type ASD in childhood do well, but some have persistent RV dilatation and dysfunction. By contrast, more patients after VSD closure were symptomatic and presented with RV dilatation and dysfunction, PH, and a dilated ascending aorta. [ABSTRACT FROM AUTHOR]

Background/objectives Pulmonary arterial hypertension is an important complication in hemodynamically relevant atrial septal defects (ASD) and negatively affects outcome. This retrospective study aimed at (1) estimating the prevalence of pulmonary hypertension (PH) in patients with secundum ASD and (2) identifying predictors of PH development or persistence after ASD closure. Methods Consecutive patients with an isolated secundum ASD from the Belgian Registry on Adult Congenital Heart Disease were studied. Demographic, clinical, echocardiographic and invasive hemodynamic measurements were analyzed. PH was defined upon the echocardiographic PH probability (tricuspid regurgitation velocity ≥ 2.9 m/s). Results PH prevalence in the entire ASD population (295 patients, 68.8% females, mean age 46 ± 21 years) was 15.9% compared to 13.3% in patients after ASD closure. PH after ASD closure was significantly related to mortality (p = 0.001), atrial arrhythmia (p < 0.001) and right heart failure (p = 0.019). Age at repair was the most important predictor for PH (HR 1.11). In the highest tertile of age at repair (> 55 years), PH prevalence was the highest (34%) and mean pulmonary artery pressure (mPAP) at catheterization before was related to PH after closure (HR 1.09). Twenty patients in the PH group had mPAP < 25 mm Hg before closure. Conclusions PH in closed secundum ASD patients is not uncommon. Its prevalence was the highest when the defect was repaired above 55 years of age. Clinical outcome was worse. PH may even develop despite normal mPAP before closure. The present findings raise the question whether the cutoff value for mPAP before closure should be age-adjusted. [ABSTRACT FROM AUTHOR]

Background To evaluate the relationship between right ventricular (RV) systolic dysfunction at rest and reduced exercise capacity in patients with a systemic RV (sRV). Methods All patients with congenitally corrected transposition of the great arteries (ccTGA) or complete TGA after atrial switch (TGA-Mustard/Senning) followed in our institution between July 2011 and September 2017 who underwent cardiac imaging within a six-month time period of cardiopulmonary exercise testing (CPET) were analyzed. We assessed sRV systolic function with TAPSE and fractional area change on echocardiogram and, if possible, with ejection fraction, global longitudinal and circumferential strain on cardiac magnetic resonance (CMR) imaging. Results We studied 105 patients with an sRV (median age 34 [IQR 28–42] years, 29% ccTGA and 71% TGA-Mustard/Senning) of which 39% had either a pacemaker (n = 17), Eisenmenger physiology (n = 6), severe systemic atrioventricular valve regurgitation (n = 14), or peak exercise arterial oxygen saturation < 92% (n = 17). Most patients were asymptomatic or mildly symptomatic (NYHA class I/II/III in 71/23/6%). Sixty-four percent had evidence of moderate or severe sRV dysfunction on cardiac imaging. Mean peak oxygen uptake (pVO2) was 24.1 ± 7.4 mL/kg/min, corresponding to a percentage of predicted pVO2 (%ppVO2) of 69 ± 17%. No parameter of sRV systolic function as evaluated on echocardiography (n = 105) or CMR (n = 46) was correlated with the %ppVO2, even after adjusting for associated cardiac defects or pacemakers. Conclusions In adults with an sRV, there is no relation between echocardiographic or CMR-derived sRV systolic function parameters at rest and peak oxygen uptake. Exercise imaging may be superior to evaluate whether sRV contractility limits exercise capacity. [ABSTRACT FROM AUTHOR]