Long-term outcome after the ross procedure in 173 adults with up to 25 years of follow-up.

OBJECTIVES: The potential risk of autograft dilatation and homograft stenosis after the Ross procedure mandates lifelong follow-up. This retrospective cohort study aimed to determine long-term outcome of the Ross procedure, investigating autograft and homograft failure patterns leading to reintervention. METHODS: All adults who underwent the Ross procedure between 1991-2018 at the University Hospitals Leuven were included, with follow-up data collected retrospectively. Autograft implantation was performed using the full root replacement technique. The primary end-point was long-term survival. Secondary end-points were survival free from any reintervention, autograft or homograft reintervention-free survival, and evolution of autograft diameter, homograft gradient and aortic regurgitation grade over time. RESULTS: A total of 173 adult patients (66% male) with a median age of 32 years (range 18-58 years) were included. External support at both the annulus and sinotubular junction was used in 38.7% (67/173). Median follow-up duration was 11.1 years (IQR, 6.4-15.9; 2065 patient-years) with 95% follow-up completeness. There was one (0.6%) perioperative death. Kaplan-Meier estimate for 15-year survival was 91.1% and Ross-related reinterventionfree survival was 75.7% (autograft: 83.5%, homograft: 85%). Regression analyses demonstrated progressive neoaortic root dilatation (0.56 mm/year) and increase in homograft gradient (0.72 mmHg/year). CONCLUSIONS: The Ross procedure has the potential to offer excellent long-term survival and reintervention-free survival. These long-term data further confirm that the Ross procedure is a suitable option in young adults with aortic valve disease which should be considered on an individual basis.

Aorta pathology and pregnancy-related risks in adult congenital cardiac disease: does the aorta dilate during pregnancy?

Background: Aortic dilatation and pregnancy are major concerns in women with aortopathy (AOP). This single-centre retrospective analysis focuses on the evolution of aortic diameters during and after pregnancy in women with Marfan syndrome (MS), Turner syndrome (TS) and bicuspid aortic valve (BAV) aortopathy. Methods and results: Thirty-eight women who had one or more single pregnancies were included. The ascending aorta was measured during pregnancy and postpartum. During pregnancy, a significant increase of diameters of the sinus aortae (median 1.4 mm; [-1.3; 3.8]) and ascending aorta (median 2.1 mm; [0.0; 4.0]) was noted. Systemic hypertension gives dilation of the aorta, but it did not influence the overall trajectory during pregnancy. Conclusion: Significant aortic dilatation is noted during pregnancy in women with underlying AOP, even persisting in the long term. Pre-existing systemic hypertension is associated with larger aortic diameters prior to pregnancy. More research on a larger study population however is needed.

Biomarkers in transposition of the great arteries after arterial switch operation: A pilot trial with deep phenotyping.

INTRODUCTION: Transposition of the great arteries (TGA) is a cyanotic congenital heart defect for which the arterial switch operation (ASO) is the preferred surgical repair. This study wanted to investigate whether a panel of biomarkers could identify morphologic as well as hemodynamic changes obtained by cardiac magnetic resonance (CMR). METHODS: Forty-four adult patients were included. Blood samples were collected to measure a broad range of biomarkers (galectin-3, ST2, GDF-15, PINP, ICTP, PIIINP, IGF-1, NT-proBNP, and hs-Tn). CMR was performed at rest and during exercise to assess cardiac function and morphology. Explorative statistics were performed between biomarker levels and CMR findings. RESULTS: All patients were asymptomatic. While galectin-3, GDF-15, and NT-proBNP levels were within normal ranges, increased ST2, PINP, PIIINP, and ICTP levels were found in 20.5%, 34.1%, 45.5%, and 27.3% of patients, respectively. Moreover, 3 and 2 patients, respectively, showed elevated IGF-1 and hs-Tn levels. Although the ejection fraction of both ventricles was within normal limits, impaired cardiac reserve was found in 20 and 25% of patients for left and right ventricle, respectively. CMR revealed no evidence of diffuse interstitial fibrosis, while 4 patients showed focal ischemic scarring. However, no significant associations between serum biomarkers and CMR data could be detected. CONCLUSION: The results suggest that in asymptomatic ASO-repaired TGA patients serum level biomarkers are elevated and that this increase is not associated with morphological changes nor with a decreased cardiac reserve. Further study with larger sample sizes is required to draw conclusions with greater confidence.

Left atrial strain in patients after arterial switch operation for transposition of the great arteries.

BACKGROUND: Left atrial (LA) strain, comprising LA reservoir, conduit and contractile function could add mechanistic information of patients after arterial switch operation (ASO) for transposition of the great arteries (TGA). ASO patients might have abnormal ventriculoarterial coupling, which makes them vulnerable to left ventricle (LV) dysfunction and results in reduced exercise capacity. This explorative study aimed to evaluate the relation between LA strain, atrial size, ventricular function, and exercise data obtained by cardiopulmonary exercise testing (CPET). METHODS: In a cohort of 44 patients (71% male, mean age 25 ± 4 years) LA strain was measured using transthoracic speckle-tracking echocardiography. Further assessment involved standard echocardiography, CPET evaluation, and blood sampling. LA strain values were compared to normal values. Correlations were calculated. Regression analysis with all strain variables to the CPET data was performed. RESULTS: LA reservoir, conduit and contractile strain were normal in 30%, 89% and 50% of the patients, respectively. LA reservoir/contractile strain correlated to LV ejection fraction (ρ 0.310/-0.159, respectively) and LA reservoir/conduit strain correlated to the LA volume index (ρ 0.336/-0.357, respectively). None of the individual LA strain parameters were associated with the CPET variables. In multivariate regression analysis, LA contractile strain was significantly associated with the percentage of predicted maximal heart rate (ß - 2.555). CONCLUSIONS: These data suggest that in TGA patients after ASO repair LA strain is impaired and correlates with LA size and LV function. However, impaired LA strain wasn't associated with the standard CPET parameters. As such, clinical significance needs to be further unravelled.

Short-term results of serial cardiopulmonary exercise testing in adults with repaired coarctation of the aorta.

BACKGROUND: Data on the evolution of exercise capacity in adults with repaired coarctation of the aorta (CoA) are scarce. We aimed to investigate the evolution and change of measures of exercise capacity obtained by cardiopulmonary exercise testing (CPET) in adults with repaired CoA. METHODS: Patients 16 years of age and older with CoA, who performed at least two maximal CPETs in our institution, were included in the study. The first and last available tests were used for comparative statistical analysis of common exercise variables. RESULTS: Sixty patients (43 men) performed serial maximal CPET. Mean age at first assessment was 30 ± 10 years (range 17-68). Mean time between first and last assessment was 3.5 years (range 1-7). Mean peak VO2 was 85.6 ± 20.4% of the predicted value at the initial test, and 87.0 ± 20.5% at the final test (p = 0.294). There were no significant differences in the mean values of oxygen pulse, VO2 at anaerobic threshold, systolic and diastolic blood pressures and peak heart rate between the two assessments. There was a slightly higher VE/VCO2 slope at the final test (p = 0.047). Higher age and Borg scale were found to be related with a decline in percent-predicted peak VO2 from initial to final assessment. CONCLUSION: In adults with repaired CoA, we found no significant change in peak VO2 during a mean follow-up of 3.5 years, yet a small increase in VE/VCO2 slope was observed. Higher age was predictive for a decline in percent-predicted peak VO2, starting in the third decade of life.

Short-term Prognostic Value of Heart Failure Diagnosis in a Contemporary Cohort of Patients With Adult Congenital Heart Disease.

BACKGROUND: Heart failure (HF) is the primary cause of premature death in adult congenital heart disease (ACHD). This study aimed to describe the impact of a HF diagnosis on short-term prognosis and to investigate the added prognostic value of an HF diagnosis to the ACHD Anatomic and Physiologic classification (ACHD-AP). METHODS: This study included 3995 patients followed in a tertiary care centre (last follow-up after January 1, 2010). Survival curves were plotted, and predictors of the primary end point (death, heart transplantation, or ventricular assist device [VAD]) were identified with the use of Cox proportional hazard models and compared with the use of Harrell's C-statistic. RESULTS: Mean age at baseline was 35.7 ± 13.3 years. The prevalence of ACHD-HF was 6.4%. During a median follow-up of 3.1 years (IQR 2.1-3.6 years), 27.3% of ACHD-HF patients reached the primary end point, compared with 1.4% of ACHD patients without HF. Event-free survivals were 78.3%, 61.9%, and 57.5% at 1, 3, and 5 years in ACHD-HF patients, compared with 99.3%, 98.3%, and 98.0% in ACHD patients without HF (P < 0.001). An HF diagnosis (HR 6.9, 95% CI 4.3-11.2) and the physiologic classification (HR 2.6, 95% CI 1.9-3.7) were independently associated with the primary end point. The addition of HF to the ACHD-AP classification yielded a Harrell's C-index of 0.8631, providing a significant improvement over the ACHD-AP classification alone (P = 0.0003). CONCLUSIONS: The risk of mortality, transplantation, or VAD is increased in ACHD-HF patients. An HF diagnosis appears to be a valuable prognostic marker in addition to the ACHD-AP classification.

Adverse functional remodelling of the subpulmonary left ventricle in patients with a systemic right ventricle is associated with clinical outcome.

AIMS: Early recognition of adverse remodelling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to short-term clinical evolution in this population. METHODS AND RESULTS: Thirty-three patients (76% male) with sRV (atrial switch repair for D-transposition of the great arteries and congenitally corrected transposition of the great arteries) underwent detailed phenotyping including exercise cardiac magnetic resonance and were followed over mean follow-up time of 3 years. Mean age was 40 ± 8 (range 26-57) years at latest follow-up. Adverse outcome was a composite of heart failure (HF) and tachyarrhythmia. Descriptive statistics and univariate cox regression analyses were performed. When compared with baseline: (i) most patients remained in New York Heart Association functional class I (76%), (ii) the degree of severity of the systemic atrioventricular valve regurgitation rose, and (iii) more electrical instability was documented at latest follow-up. Six (18%) of a total of 9 events were counted as first cardiovascular events (9% HF and 9% arrhythmia). NT-proBNP, oxygen pulse, left ventricle end-diastolic volume index (LVEDVi), and stroke volume index (SVi) of the subpulmonary left ventricle (LV) both in rest and at peak exercise were significantly associated with the first cardiovascular event. CONCLUSION: NT-proBNP was by far the best prognostic marker for clinical outcome. Adverse remodelling with increase of LVEDVi and SVi of the subpulmonary LV at rest and during exercise were associated with worse clinical outcome. We theorize that remodelling of the subpulmonary ventricle might be an early sign of a failing sRV circulation.

Heart failure related to adult congenital heart disease: prevalence, outcome and risk factors.

AIMS: Information on the prevalence, outcome and factors associated with heart failure in patients with adult congenital heart disease (CHD) (ACHD-HF) is lacking. We aimed at assessing the prevalence and outcome of ACHD-HF, the variables associated with ACHD-HF, and the differences between major anatomical/pathophysiological ACHD subgroups. METHODS AND RESULTS: We included 3905 patients (age 35.4 ± 13.2 years) under active follow-up in our institution (last visit >2010). Outcome of ACHD-HF cases was compared with sex- and age-matched cases. Univariable and multivariable binary logistic regression with ACHD-HF diagnosis as a dependent variable was performed. Overall prevalence of ACHD-HF was 6.4% (mean age 49.5 ± 16.7 years), but was higher in patients with cyanotic CHD (41%), Fontan circulation (30%), and a systemic right ventricle (25%). All-cause mortality was higher in ACHD-HF cases when compared with controls (mortality rate ratio 4.67 (2.36-9.27); P = 0.0001). In multivariable logistic regression analysis, age at latest follow-up [per 10 years; odds ratio (OR) 1.52; 95% confidence interval (CI) 1.31-1.77], infective endocarditis (OR 4.11; 95%CI 1.80-9.38), history of atrial arrhythmia (OR 3.52; 95%CI 2.17-5.74), pacemaker implantation (OR 2.66; 95% CI 1.50-4.72), end-organ dysfunction (OR 2.41; 95% CI 1.03-5.63), New York Heart Association class (OR 9.28; 95% CI 6.04-14.25), heart rate (per 10 bpm; OR 1.27; 95% CI 1.08-1.50), ventricular dysfunction (OR 3.62; 95% CI 2.54-5.17), and pulmonary hypertension severity (OR 1.66; 95% CI 1.21-2.30) were independently related to the presence of ACHD-HF. Some variables (age, atrial arrhythmia, pacemaker, New York Heart Association, and ventricular dysfunction) were related to ACHD-HF in all anatomical/physiological subgroups, whereas others were not. CONCLUSIONS: ACHD-HF is prevalent especially in complex CHD and is associated with poor prognosis. Our data provide insight in the factors related to ACHD-HF including differences between specific anatomical and physiological subgroups.

15-Year follow-up of regional right and left ventricular function after the Senning operation: a Colour-Doppler myocardial imaging study.

INTRODUCTION: Despite right ventricular (RV) dysfunction being a major concern in Senning patients, long-term follow-up data is lacking. This study aimed (1) at evaluating regional (base-mid-apex) RV and left ventricular (LV) function using Colour-Doppler myocardial imaging over a 15-year follow-up period and (2) at comparing results with matched controls. METHODS: For the longitudinal analysis (2004-2019), we compared systolic and diastolic function in 10 Senning patients. For the cross-sectional analysis, we compared the subaortic RV (sRV) of Senning patients with the RV and LV of matched controls and the subpulmonary LV (spLV) of Senning patients with the LV of matched controls. RESULTS: The longitudinal analysis of sRV function showed a significant decrease in apical peak systolic strain (-17 ± 7% vs -12 ± 4%; p = 0.025) and apical peak systolic strain rate (-1.1 ± 0.3s-1 vs -0.8 ± 0.4s-1; p = 0.012). spLV function showed a significant decrease in peak systolic velocity (mid; p = 0.013 and apex; p = 0.011) and peak systolic strain rate (mid; p = 0.048). The cross-sectional analysis revealed significant lower values for basal, mid and apical peak systolic velocity, peak systolic strain rate, peak systolic strain of the sRV of Senning patients when compared to both LV and RV of matched controls (all p < 0.05). CONCLUSION: Our study showed that systolic and diastolic sRV function did not change over a 15-year follow-up period, except in the apical region. There was a decline in spLV systolic function, which may be of clinical value. On the other hand, when compared to age- and gender-matched controls, the sRV of Senning patients exhibits significantly decreased measurements of longitudinal systolic function.

Comparison of risk stratification models for pregnancy in congenital heart disease.

BACKGROUND: Pregnancy in women with congenital heart disease (CHD) is associated with increased risk for maternal cardiac complications. Several risk stratification models are used to predict adverse cardiac outcome in women with CHD who become pregnant. This study was set up as an exploratory study to provide a head-to-head comparison of the 4 most commonly used models: CARPREG, CARPREG II and ZAHARA risk scores and mWHO risk classification. METHODS AND RESULTS: We randomly selected 100 women from the database of paediatric and congenital heart disease of the University Hospitals Leuven. Individual pregnancy risk scores were retrospectively calculated and summarized in a weighted average risk for each risk stratification model. To evaluate accuracy of each model, the weighted average risk was plotted against the actual observed number of "cardiac events" as defined in the respective risk models. Maternal adverse cardiac events occurred in 8% of our study population. Weighted average risks were plotted versus the observed number of events for each model: 10.1% versus 4.0% for CARPREG, 8.6% versus 5.0% for CARPREG II, 11.1% versus 8.0% for ZAHARA and 12.4% versus 8.0% for the mWHO classification. CONCLUSION: All risk models overestimated maternal cardiac risk. The ZAHARA risk model appeared to be a closer reflection of maternal risk in our cohort of CHD patients. More research on a larger study population is needed.

Covered stent placement for treatment of coarctation of the aorta: immediate and long-term results.

OBJECTIVES: This study aimed to describe the safety and efficacy of covered stents in patients with coarctation of aorta (CoA) for immediate and long-term follow-up. BACKGROUND: Covered stents are increasingly being used in (re)CoA, mainly to reduce the risk of aortic wall injuries (AWI). However, limited data are available on intermediate and long-term outcome. METHODS: In 89 patients (67.4% male) with a mean age of 23.9 ± 15.8 (min max range 5.1-71.6) years were 102 covered stents implanted (January 2003 - December 2017). Short-term pre/post-implant hemodynamics and angiographic data were reported. Changes in blood pressure, the use of antihypertensive drugs and complications were recorded during follow-up. RESULTS: The procedural success rate was 100%. The mean invasive ascending-to-descending aorta systolic gradient under general anaesthesia decreased from 25 ± 16 mmHg to 4 ± 7 mmHg (p < 0.001). After a mean follow-up time of 6.6 ± 3.7 years, there was a persistent improvement of the mean systolic blood pressure gradient between right arm and leg (-7 ± 18 vs 38 ± 24 mmHg; p < 0.001). A larger proportion of patients required antihypertensive medication (33.7% vs 50.0%, p = 0.017) and needed ≥ 2 drugs (20.2% vs 27.4%, p = 0.066) to control blood pressure. Long-term adverse events were found in 4.5% of patients [covered stent fracture (n = 3), aneurysm formation (n = 2)]. CONCLUSIONS: Covered stent implantation for CoA is highly successful, safe and results in a persistent hemodynamic improvement in the immediate and long-term outcome. Lifelong follow-up with additional antihypertensive drug treatment is mandatory to maintain favourable hemodynamic results after stenting. CONDENSED ABSTRACT: Long-term follow-up data on covered stents in patients with coarctation of the aorta are scarce. A cohort of 89 patients was reviewed. The procedural implantation success rate was 100%. The invasive gradient decreased from 25 ± 16 mmHg to 4 ± 7 mmHg (p < 0.001). After follow-up of 6.6 ± 3.7 years, there was a persistent improvement of the clinical systolic blood pressure gradient (-7 ± 18 vs 38 ± 24 mmHg; p < 0.001). However, a larger proportion of patients required antihypertensive medication (33.7% vs 50.0%, p = 0.017). Covered stent implantation results in favourable hemodynamic effects, but lifelong follow-up with additional antihypertensive drug treatment is mandatory to maintain these results.

Perioperative Risk Factors for Mortality in Patients Undergoing Surgery for Ebstein's Anomaly at a Single Institution: A Retrospective Analysis.

BACKGROUND: Mortality after cardiac surgery for Ebstein's anomaly ranges from 2.5% to 31%. Independent predictors for mortality and morbidity remain poorly defined because of the low incidence of this congenital anomaly. To identify potentially modifiable factors, this retrospective study investigates the prognostic value of perioperative variables for mortality and morbidity. METHODS: We reviewed the charts of 171 patients with Ebstein's anomaly who were at one point in follow-up at our center. Only patients who underwent cardiac surgery for this anomaly were included. The primary endpoint was a composite of mortality or rehospitalization for cardiac reasons within 1 year of surgery. Logistic regression and Cox regression models were used to study the predictive value of various variables. RESULTS: We identified 32 patients (median age 12 years; range 7 days to 70 years) who underwent a total of 49 surgical procedures for Ebstein's anomaly at our institution between November 1987 and March 2015. The following variables were significantly associated with the primary outcome: increased severity of tricuspid valve stenosis (odds ratio 2.089; 95% confidence interval 1.175 to 3.713) and right ventricular dysfunction (1.826; 1.109 to 3.006), partial corrective surgery (versus corrective surgery) (6.709; 1.436 to 31.344), occurrence of major postoperative complications (5.460; 1.419 to 21.008), and increased length of stay in the intensive care unit (1.051; 1.010 to 1.093). A better outcome was observed with the use of intraoperative cardioplegic arrest (0.185; 0.063 to 0.550), atrial septal defect closure during surgery, and longer duration of surgery (0.991; 0.984 to 0.998). CONCLUSION: Several patient-specific characteristics and perioperative characteristics were associated with a poorer outcome after cardiac surgery for Ebstein's anomaly. The outcome seems to be primarily determined by the severity of the valve dysfunction and right ventricular performance, with only a minor role for perioperative surgical or anesthetic technical determinants.

Outcome of arterial switch operation for transposition of the great arteries. A 35-year follow-up study.

BACKGROUND: Arterial switch operation (ASO) is today the first-choice surgical treatment for patients with transposition of the great arteries. Long-term outcome data still remain scarce. Moreover, the course of these patients is not uneventful. Therefore, we wanted to evaluate long-term outcome and determine on which variables to focus during follow-up. METHODS: Clinical records of 318 patients who underwent ASO between October 1981 and July 2018 were reviewed. Perioperative, post-operative, and interventional data were collected to determine mortality and the need for re-intervention. Descriptive statistics and Kaplan-Meier survival analysis were performed. RESULTS: Mean follow-up time was 11.1 SD 8.5 years (range 0-35) with a mean age of 12.5 SD 9.0 years (range 0-37) at latest follow-up. In-hospital mortality was 7.5% and overall survival 90.9% for a maximum follow-up time of 35 years. Causes of early mortality were cardiogenic shock, severe pulmonary hypertension, septic shock and multiple organ failure. Causes of late mortality were cardiogenic shock, severe pulmonary hypertension, pacemaker lead fracture and fire death. Re-intervention free survival at 5-year was 91.6%, at 10-year 90.7%, at 20-year 79.2%. For all survivors, the most frequent sequelae after ASO were pulmonary artery stenosis (80.9%), of which 13.5% needed an intervention. The threshold for intervening on lesions at the level of the pulmonary artery bifurcation was higher and the percutaneous re-intervention rate was higher for non-bifurcation lesions. CONCLUSIONS: Despite a relatively high peri-operative mortality, TGA patients have an excellent overall long-term survival. However, a large proportion of patients requires re-interventions, mainly for pulmonary artery stenosis.

Outcome of the Glenn procedure as definitive palliation in single ventricle patients.

OBJECTIVES: In selected single ventricle patients, a Glenn procedure (SV-Glenn) may be considered as definitive palliation. Either the patient is unsuited to progress to a Fontan circulation or a SV-Glenn circulation is preferred. This study aimed at describing the clinical course, and long-term mortality/morbidity of SV-Glenn patients. METHODS: All SV-Glenn patients followed at the University Hospitals Leuven before May 2018 were included. Patients who underwent, or were awaiting, TCPC completion and those who underwent a Glenn in the setting of a biventricular circulation one-and-a-half repair (OAHR), were excluded. RESULTS: Of 65 Glenn-only patients identified, 21 (32%) had OAHR, whereas 44 (68%) were SV-Glenn patients. Of SV-Glenn patients, 19 died within 6 months after the Glenn procedure. Of 25 SV-Glenn survivors, median age at Glenn was 6.3 (IQR 1.2-29.7) years. Eight were unsuited for TCPC completion; in 17 SV-Glenn was preferred over TCPC completion. Over a median follow-up time of 11 (IQR 3-18) years after the Glenn procedure, 5 (20%) patients died. At latest follow-up 10 (40%) had heart failure, 5 (20%) had atrial and 4 (16%) ventricular arrhythmias, 2 (8%) a thromboembolic event, 7 (28%) required pacemaker implantation, and 2 (8%) had infective endocarditis but none developed cirrhosis or protein-losing enteropathy. Mean saturation at latest follow-up was 87 ± 7%. CONCLUSION: SV-Glenn patients represent a unique and heterogeneous patient population. Outcome was reasonable, although comorbidities, such as heart failure and arrhythmias were not uncommon. In SV-Glenn patients, 'classic' complications related to Fontan physiology, such as cirrhosis and protein-losing enteropathy, were absent.

Percutaneous closure of an uncommon aortic pseudoaneurysm after arterial switch repair: a case report.

BACKGROUND: The development of an aortic pseudoaneurysm is a rather rare but potentially fatal complication after cardiac surgery for aortic valve and aorta disease. If a pseudoaneurysm is left untreated, it carries a substantial risk of rupture, thrombosis with subsequently systemic embolization, and compression of the surrounding structures. CASE SUMMARY: We describe a case of a transcatheter repair of a more complex and uncommon pseudoaneurysm following aortic valve replacement in a patient with a history of arterial switch repair for transposition of the great arteries. The pseudoaneurysm originated from the aortic wall and connected to the left ventricular outflow tract (LVOT). The connection to LVOT was closed with a duct occluder, the neck to the aortic wall with an atrial septal defect occluder. After 1 month, the complete pseudoaneurysm was successfully thrombosed. DISCUSSION: Percutaneous closure of a complex pseudoaneurysm after arterial switch repair is feasible and safe. However, lifelong follow-up is needed to determine the late results after transcatheter interventions.

Advanced care planning in adult congenital heart disease: Transitioning from repair to palliation and end-of-life care.

As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2]. The scientific adult CHD (ACHD) community has been working hard to identify variables related to worse outcomes, modifying those where possible in order to improve survival. Indeed, survival in adults with CHD has increased, but consequently, on top of CHD-related complications, patients are increasingly exposed to the standard cardiovascular risk factors. Therefore, a program for lifelong coaching on health behavior and life style management becomes indispensable. More emerging is that a substantial number of patients, in particular those with complex heart defects, will eventually end up in a stage with hardly any medical or interventional options left. Our healthcare provision has to be prepared to organize care for this specific group of patients who will die prematurely and require the timely development and establishment of advanced care planning. Advanced care planning should preferentially be set-up in expert CHD centers. The long-lasting relationship in ACHD care with healthcare providers offers an excellent basis with regards to prognosis, advanced care planning and end-of-life issues.

Advanced Imaging to Phenotype Patients With a Systemic Right Ventricle.

Background Reduced ventricular function and decreased exercise capacity are widespread in adults with complete transposition of the great arteries after atrial switch ( TGA -Mustard/Senning) and congenitally corrected TGA (cc TGA ). Advanced imaging techniques may help to better phenotype these patients and evaluate exercise cardiac response. Methods and Results Thirty-three adults with a systemic right ventricle (70% TGA -Mustard/Senning, 37±9 years of age, 24% female, 94% New York Heart Association class I- II ) underwent echocardiogram, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging at rest and during a 4-stage free-breathing bicycle test. They were compared with 12 healthy controls (39±10 years of age, 25% female, all New York Heart Association class I). TGA -Mustard/Senning patients had a higher global circumferential strain (-15.8±3.6 versus -11.2±5.2%, P=0.008) when compared with cc TGA , whereas global longitudinal strain and systemic right ventricle contractility during exercise were similar in both groups. Septal extracellular volume ( ECV ) in cc TGA was significantly higher than in TGA -Mustard/Senning (30.2±2.0 versus 27.1±2.7%, P=0.005). During exercise, TGA -Mustard/Senning had a fall in end-diastolic volume and stroke volume (11% and 8%, respectively; both P≤0.002), whereas cc TGA could increase their stroke volume in the same way as healthy controls. Because of a greater heart rate reserve in TGA -Mustard/Senning ( P for interaction=0.010), cardiac index and peak oxygen uptake were similar between both patient groups. Conclusions Caution should be exercised when evaluating pooled analyses of systemic right ventricle patients, given the differences in myocardial contraction pattern, septal extracellular volume, and the exercise response of TGA -Mustard/Senning versus cc TGA patients. Longitudinal follow-up will determine whether abnormal exercise cardiac response is a marker of earlier failure.

Up to 11 years of experience with the Melody valved stent in the right ventricular outflow tract.

AIMS: The aim of the study was to report up to 11 years of follow-up after Melody valve implantation in the pulmonary position. METHODS AND RESULTS: This was a single-institution non-randomised prospective observational study of all Melody valves in the pulmonary position after discharge between 2006 and 2017 (n=188). Mean age was 19.4 years (SD 13.2). The indication was stenosis (45%), regurgitation (33%) and mixed (22%). Pre-stenting was performed in all except the initial four patients. In stenotic lesions the peak gradient was 36 mmHg PIG (SD 12.0) after 11 years and in regurgitant lesions the maximal regurgitation was 2/4. Stent fractures were observed in 8.6%; only one grade III fracture required redo PPVI. Surgical removal was carried out in seven (3.7%), redo PPVI in five (2.7%). Endocarditis was diagnosed in 19 (10.2%) patients at a median of 2.3 years (0.7-8.8) after Melody implantation. Three were surgically removed early because of persistent infection, 16 were sterilised; six required replacement (three surgical, three redo PPVI). There were no valve- or procedure-related deaths. CONCLUSIONS: The Melody valve shows a good preserved leaflet function up to 11 years after implantation. The main reason for graft failure was endocarditis, although in half of those patients no reintervention was needed. After pre-stenting, stent fractures led very exceptionally to reintervention.

Right ventricular systolic dysfunction at rest is not related to decreased exercise capacity in patients with a systemic right ventricle.

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 ±â€¯7.4 mL/kg/min, corresponding to a percentage of predicted pVO2 (%ppVO2) of 69 ±â€¯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.

Management of acute heart failure in adult patients with congenital heart disease.

Heart failure is an increasing reason for hospitalization and the leading cause of death in patients with adult congenital heart disease (ACHD). Recently, the European Society of Cardiology and the American Heart Association published consensus documents on the management of chronic heart failure in ACHD patients. However, little data and/or guidelines are available for the management of (sub)acute heart failure. The ACHD population is heterogeneous by definition and often has complex underlying anatomy, which could pose a challenge to the physician confronted with the ACHD patient in (sub)acute heart failure. Recognizing the underlying anatomy and awareness of the possible complications related would result in better treatment, avoid unnecessary delays, and improve outcomes of the ACHD patient with (sub)acute heart failure. This review focuses on the management of (sub)acute heart failure in ACHD with specific attention to lesion-specific issues.