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Objective Patients with repaired tetralogy of Fallot (ToF) have an increased long-term risk of cardiovascular morbidity and mortality. Risk stratification in this population is difficult. Initial evidence suggests that cardiopulmonary exercise testing (CPET) may be helpful to risk-stratify patients with repaired ToF. Methods and results We studied 875 patients after surgical repair for ToF (358 females, age 25.5 ± 11.7 year, range 7–75 years) who underwent CPET between 1999 and 2009. During a mean follow-up of 4.1 ± 2.6 years after CPET, 30 patients (3.4%) died or had sustained ventricular tachycardia (VT). 225 patients (25.7%) had other cardiac related events (emergency admission, surgery, or catheter interventions). On multivariable Cox regression-analysis, %predicted peak oxygen uptake ( V ˙ O 2 %) (p = 0.001), resting QRS duration (p = 0.030) and age (p < 0.001) emerged as independent predictors of mortality or sustained VT. Patients with a peak V ˙ O 2 ≤ 65% of predicted and a resting QRS duration ≥ 170 ms had a 11.4-fold risk of death or sustained VT. Ventilatory efficiency expressed as V ˙ E / V ˙ C O 2 slope (p < 0.001), peak V ˙ O 2 % (p = .001), QRS duration (p = .001) and age (p = 0.046) independently predicted event free survival. V ˙ E / V ˙ C O 2 slope ≥ 31.0, peak V ˙ O 2 % ≤ 65% and QRS duration ≥ 170 ms were the cut-off points with best sensitivity and specificity to detect an unfavorable outcome. Conclusions CPET is an important predictive tool that may assist in the risk stratification of patients with ToF. Subjects with a poor exercise capacity in addition to a prolonged QRS duration have a substantially increased risk for death or sustained ventricular tachycardia, as well as for cardiac-related hospitalizations. [ABSTRACT FROM AUTHOR]

An increase in N-terminus pro-B-type natriuretic peptide (NT-proBNP) during exercise is related to myocardial ischemia, myocardial dysfunction, and inflammatory stress. Its value for patients with a univentricular heart after total cavopulmonary connection (TCPC) is unknown. For 66 patients with TCPC, a cardiopulmonary exercise test was performed. Venous NT-proBNP samples were drawn with the patient at rest and then 2-3 min after peak exercise. The median NT-proBNP during rest was 82 ng/l (range, 11-2,554 ng/l), with 22 patients above the upper reference limit. A higher NT-proBNP during rest was related to a worse peak oxygen uptake (peak $${\dot{\text V}}{\text O}_{2}$$), a higher ventilatory equivalent (VE/VCO slope), and a need for diuretics administration. A small but significant increase in NT-proBNP during exercise at 6 ng/l (range, 0-314 ng/l) was related mainly to its resting value. The relative increase was solely related to a higher body mass and not to any of the investigated functional parameters. Usually, NT-proBNP during rest is not elevated in TCPC patients. If so, it is a valuable predictor of cardiac function. During exercise, only a minor increase in NT-proBNP occurs. Its extent is not related to any of the investigated functional parameters. Maybe the filling restriction from the lungs prevents atrial and ventricular overload as well as BNP secretion in TCPC patients. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Even after repair of aortic coarctation without restenosis there is a high incidence of arterial hypertension. This study was performed to assess the contribution of several inherited gene polymorphisms, which are known to be related to essential hypertension. Patients and methods: 122 patients aged 17–72years, 46 women, and 2–27years after repair of isolated aortic coarctation without restenosis were investigated. Genomic polymorphism of angiotensin converting enzyme (ACE I/D), angiotensinogen (AGT, c.704C>T), angiotensin II receptor type 1 (AGTR1, c.1166A>C), aldosterone synthase (CYP11B2, c.−344C>T), endothelin 1 (EDN1, EDN1/ex5-c.5665G>T), G protein (GNB3, c.825C>T), G protein-coupled receptor kinase 4 (GRK4, c.679C>T), fibrillin 1 (FBN1, VNTR(TAAA)) and two polymorphisms each of the ß1 adrenoreceptor (ADRB1, c.145G>A and c.1165C>G), ß2 adrenoreceptor (ADRB2, c.46A>G and c.79C>G), and endothelial NO synthase (NOS3, intron 4 I/D and NOS3, c.894G>T) were determined by PCR amplification and fragment length analysis. Patients were classified “normotensive”, if they were not on antihypertensive drugs and showed normal blood pressure both on ambulatory measurement and exercise test. Results: None of the investigated genomic polymorphism could be related to hypertension. Only patients with the ACE I/I genotype had a less pronounced nocturnal dipping and patients with a ADRB1 c.1165 C/C genotype had a higher systolic and mean blood pressure at night. Conclusions: Development of late hypertension after aortic coarctation repair could not be related to the investigated genomic polymorphism. The correlation of the ACE I/D and the ADRB1 c.1165C>G polymorphism to nocturnal dipping and blood pressure at nighttime needs further confirmation. [Copyright &y& Elsevier]

Objectives: The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (CPET) in patients who received a Mustard and Senning (M/S) operation. Background: Patients who received an M/S operation have increased long-term risk of cardiovascular morbidity and mortality. Limited information is available on how to stratify risk in this population. Methods: Between 1996 and 2007, 274 adults (age 26.3 ± 8.9 years, range 16 to 50 years) who had received a Mustard (n = 144) or Senning (n = 130) operation in infancy were studied with CPET. During a follow-up of 3.9 ± 2.3 years (range 0.2 to 10.8 years), 12 patients died at an age of 36 ± 14 years, and 46 patients required a cardiac-related emergency (<24 h from the onset of symptom/condition) hospital admission at an age of 30 ± 11 years. Results: At multivariate Cox analysis, the slope of ventilation per unit of carbon dioxide output (VE/VCO2 slope) (hazard ratio: 1.088, p < 0.0001) and percentage of predicted peak oxygen uptake (Vo 2%) (hazard ratio: 0.979, p = 0.0136) were the strongest predictors of death/cardiac-related emergency hospital admission among demographic, clinical, and exercise variables. A VE/VCO2 slope ≥35.4 (hazard ratio: 10.7, 95% confidence interval [CI]: 7.8 to 24.6), and a peak Vo 2% ≤52.3% (hazard ratio: 3.4, 95% CI: 2.5 to 8.2) were associated with an increased 4-year risk of death/cardiac-related emergency hospital admission. Patients who had both a VE/VCO2 slope ≥35.4 and a peak Vo 2% ≤52.3% of predicted value were at highest risk (4-year event rate: 78.8%). Conclusions: CPET provides important prognostic information in adults with M/S operation. Subjects with enhanced ventilatory response to exercise or those with poor exercise capacity have a substantially higher 4-year risk of death/cardiac-related emergency hospital admission. [Copyright &y& Elsevier]

Study Design: This is a retrospective cross-sectional study to analyze mortality and the rate of restenosis in the follow-up of patients after surgical repair of isolated aortic coarctation in infancy. Patients and Methods: From 1974 to 2003, 191 patients underwent surgical repair of aortic coarctation in infancy. Follow-up data of 2,432 patient-years were extracted from the clinical files of our outpatient department or from family practitioners. Results: Five patients died (total mortality 2.6%): 1 patient intraoperatively and 2 within 30 days after surgery (early mortality 1.6%). The other 2 patients died within 3 months. All deaths were contributed to patients that underwent surgery in the years up to 1981 and within the first 43 days of life. Of the surviving 186 patients, 31 had reintervention because of restenosis and another 11 patients had a noninvasive brachial-ankle systolic blood pressure gradient >20 mm Hg, suggesting current restenosis. Risk factors for death or restenosis were a hypoplastic aortic arch and a low body length at surgery. Conclusions: Nowadays, surgical repair of coarctation can be performed in infancy with minimal risk. The restenosis rate is considerably high (23%). It is mainly caused by the size of the whole aortic arch, but can also develop during later follow-up. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

There are contradictory reports whether exercise capacity is reduced in patients on long-term follow-up after coarctation repair. Data from unselected patient groups are missing. In a cross-sectional, long-term follow-up study of a tertiary congenital cardiology referral center, 260 patients (30.2 ± 11.4 years old, 84 women), after surgical repair for isolated aortic coarctation (age at surgery 11.5 ± 11.2 years), underwent a symptom-limited exercise test. Peak workload was 180 ± 52 W, significantly less than the age- and height-related reference values (p <0.0005). A peak workload under 80% of expected was found in 200 patients (77%). Exercise performance of the patients was independent from age at surgery, type of surgery, or the systolic brachial-ankle blood pressure difference. The only exercise-limiting factor found was the chronic administration of diuretics to treat hypertension (p = 0.005). Exercise hypertension, defined as a systolic blood pressure >2 SD above the load-dependent reference value, was found in 73 patients (28%). It was independently related to the systolic brachial-ankle blood pressure difference (p <0.0005) and diuretics administration (p = 0.037). In conclusion, most patients after coarctation repair have a reduced exercise performance. This reduction is not related to the surgical results. Particularly, as these patients are at risk of early atherosclerosis, exercise should be promoted as primary prevention after restenosis, aortic or cerebral aneurysms, and severe exercise hypertension are ruled out. [Copyright &y& Elsevier]

Objectives: To compare images of the aorta obtained with helical CT (HCT) scanning and MRI for the follow-up of adults with coarctation of the aorta (CoA). Design: Longitudinal study. Setting: Department of adult congenital heart disease in a tertiary university hospital. Patients: A total of 37 adults (age range, 16 to 68 years; women, 13) with CoA (after surgery, 34 patients; native, 2 patients; after balloon-angioplasty, 1 patient) Measurements and results: All patients underwent both HCT and MRI of the thoracic aorta within a mean (± SD) time interval of 1.86 ± 1.11 years. Aortic diameters measured at six intrathoracic levels showed a high correlation (r = 0.79 to 0.94). On average, slightly lower diameters were measured with MRI (1.2 mm). But there was a substantial variation between the two measurements with differences of up to 9 mm. All other pathomorphologic abnormalities were detected and classified similarly with both methods. Conclusions: HCT and MRI are similarly useful for the noninvasive evaluation of the thoracic aorta in patients with CoA. But there can be a substantial variation in two subsequent measurements without an overall substantial bias toward larger diameter in one of the two methods. In repetitive studies, changes of the diameters should be interpreted with care, especially when assessing the progression of aortic diameters. [ABSTRACT FROM AUTHOR]

The article presents a letter to the editor discussing the article "Arterial blood gases and oxygen content in climbers on Mount Everest," by MPW Grocott, DS Martin, DZH Levett, R. McMorrow, J. Windsor, and HE Montgomery published in the journal on January 8, 2009.

Open in new tab Download slide OBJECTIVES To evaluate longitudinal systemic ventricular function and atrioventricular valve regurgitation in patients after the neonatal Norwood procedure. METHODS Serial postoperative echocardiographic images before Fontan completion were assessed in neonates who underwent the Norwood procedure between 2001 and 2020. Ventricular function and atrioventricular valve regurgitation were compared between patients with modified Blalock–Taussig shunt and right ventricle to pulmonary artery conduit. RESULTS A total of 335 patients were identified including 273 hypoplastic left heart syndrome and 62 of its variants. Median age at Norwood was 8 (7–12) days. Modified Blalock–Taussig shunt was performed in 171 patients and the right ventricle to pulmonary artery conduit in 164 patients. Longitudinal ventricular function and atrioventricular valve regurgitation were evaluated using a total of 4352 echocardiograms. After the Norwood procedure, ventricular function was initially worse (1–30 days) but thereafter better (30 days to stage II) in the right ventricle to pulmonary artery conduit group (P  < 0.001). After stage II, the ventricular function was inferior in the right ventricle to the pulmonary artery conduit group (P  < 0.001). Atrioventricular valve regurgitation between the Norwood procedure and stage II was more frequent in the modified Blalock–Taussig shunt group (P  < 0.001). After stage II, there was no significant difference in atrioventricular valve regurgitation between the groups (P  = 0.171). CONCLUSIONS The effect of shunt type on haemodynamics after the Norwood procedure seems to vary according to the stage of palliation. After the Norwood, the modified Blalock–Taussig shunt is associated with poorer ventricular function and worse atrioventricular valve regurgitation compared to right ventricle to pulmonary artery conduit. Whereas, after stage II, modified Blalock–Taussig shunt is associated with better ventricular function and comparable atrioventricular valve regurgitation, compared to the right ventricle to pulmonary artery conduit. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES In this study, we aimed to compare infants with univentricular hearts who underwent an initial ductus stenting to those receiving a surgical systemic-to-pulmonary shunt (SPS). METHODS All infants with univentricular heart and ductal-dependent pulmonary blood flow who underwent initial palliation with either a ductus stenting or a surgical SPS between 2009 and 2022 were reviewed. Outcomes were compared after ductus stenting or SPS including survival, probability of re-interventions and the probability to reach stage II palliations. RESULTS A total of 130 patients were evaluated, including 49 ductus stenting and 81 SPSs. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19 patients. There was comparable hospital mortality (2.0% stent vs 3.7% surgery, P  = 0.91) between the groups, but shorter intensive care unit stay (median 1 vs 7 days, P  < 0.01) and shorter hospital stay (median 7 vs 17 days, P  < 0.01) were observed in patients with initial ductus stenting, compared to those with SPS. However, acute procedure-related complications were more frequently observed in patients with ductus stenting, compared with those with SPS (20.4 vs 6.2%, P  = 0.01), and 10 patients needed a shunt procedure after the initial ductus stent. The cumulative incidence of reaching stage II was similar between ductus stenting and SPS (88.0 vs 90.6% at 12 months, P  = 0.735). Pulmonary artery (PA) index (median 194 vs 219 mm2/m2, P  = 0.93) at stage II was similar between patients with ductus stenting and SPS. However, the ratio of the left to the right PA index [0.69 (0.45–0.95) vs 0.86 (0.51–0.84), P  = 0.015] was higher in patients who reached stage II with surgical shunt physiology, compared with patients with ductus stent physiology. CONCLUSIONS After initial ductus stenting in infants with univentricular heart, survival is comparable and post-procedural recovery shorter, but more acute stent dysfunctions and lower development of left PA are observed, compared to acute shunt dysfunctions. The less invasive procedure and shorter hospital stay are at the expense of more stent reinterventions. [ABSTRACT FROM AUTHOR]

Introduction: Well-trained staff is needed to interpret cardiopulmonary exercise tests (CPET). We aimed to examine the accuracy of machine learning–based algorithms to classify exercise limitations and their severity in clinical practice compared with expert consensus using patients presenting at a pulmonary clinic. Methods: This study included 200 historical CPET data sets (48.5% female) of patients older than 40 yr referred for CPET because of unexplained dyspnea, preoperative examination, and evaluation of therapy progress. Data sets were independently rated by experts according to the severity of pulmonary–vascular, mechanical–ventilatory, cardiocirculatory, and muscular limitations using a visual analog scale. Decision trees and random forests analyses were calculated. Results: Mean deviations between experts in the respective limitation categories ranged from 1.0 to 1.1 points (SD, 1.2) before consensus. Random forests identified parameters of particular importance for detecting specific constraints. Central parameters were nadir ventilatory efficiency for CO2, ventilatory efficiency slope for CO2 (pulmonary–vascular limitations); breathing reserve, forced expiratory volume in 1 s, and forced vital capacity (mechanical–ventilatory limitations); and peak oxygen uptake, O2 uptake/work rate slope, and % change of the latter (cardiocirculatory limitations). Thresholds differentiating between different limitation severities were reported. The accuracy of the most accurate decision tree of each category was comparable to expert ratings. Finally, a combined decision tree was created quantifying combined system limitations within one patient. Conclusions: Machine learning–based algorithms may be a viable option to facilitate the interpretation of CPET and identify exercise limitations. Our findings may further support clinical decision making and aid the development of standardized rating instruments. [ABSTRACT FROM AUTHOR]

In our study, we sought to analyse the mid-term results after interventional aortic coarctation (CoA) stenting with sequential dilation of the stent. The data of all 218 patients, who are above the age of 6 years and underwent CoA-stent implantation in our hospital, were retrospectively analysed on the rate of re-interventions, complications and arterial hypertension at a follow-up time of 31 months. To avoid any aortic complications, stents were deployed primarily not in full size and a second cardiac catheterisation for further dilatation was scheduled within 6–12 months after the stent implantation. The median peak invasive systolic pressure gradient declined significantly from 26.2 mmHg to 2.7 mmHg after stenting. There was one procedure related death due to an aortic rupture after stent implantation. There were in total 33 (15.1%) procedure-related complications including femoral artery complications, stent fracture and stent dislocation (in 9, 9 and 7 patients, respectively). In 85 patients a re-dilatation and in 25 patients a second stent-implantation was necessary at the first re-intervention. The systolic blood pressure declined significantly from 144 mmHg to 131 mmHg after stenting. The number of patients being normotensive changed from 18% before stenting to 78.5% after stenting with adjusted antihypertensive medication. Aortic stenting is an effective means for CoA treatment. With sequential dilation of the stent, a very low rate of life-threatening procedural complications and mortality can be achieved. CoA stenting with proper antihypertensive medications results in better control of blood pressure. • Endovascular stent implantation is effective means for aortic coarctation treatment. • With the sequential dilation of the CoA-stent, a low rate of mortality can be achieved. • It also has a very low rate of life-threatening aortic complications. • It should be considered for patients with high risk for aortic complications. [ABSTRACT FROM AUTHOR]

Aims: High-sensitive troponin T (hs-TnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and C-reactive protein (CRP) are established prognostic biomarkers for cardiovascular (CV) morbidity and mortality and frequently used in symptomatic and/or hospitalized adults with congenital heart disease (ACHD). Their prognostic value in clinically stable ACHD has not yet been well established. This study investigates the predictive value of hs-TnT, NT-proBNP, and CRP for survival and CV events in stable ACHD. Methods and results: In this prospective cohort study, 495 outpatient ACHD (43.9 ± 10.0 years, 49.1% female) underwent venous blood sampling including hs-TnT, NT-proBNP, and CRP. Patients were followed up for survival status and the occurrence of CV events. Survival analyses were performed with Cox proportional hazards regression analysis and Kaplan–Meier curves. During a mean follow-up of 2.8 ± 1.0 years, 53 patients (10.7%) died or reached a cardiac-related endpoint including sustained ventricular tachycardia, hospitalization with cardiac decompensation, ablation, interventional catheterization, pacer implantation, or cardiac surgery. Multivariable Cox regression revealed hs-TnT (P = 0.005) and NT-proBNP (P = 0.018) as independent predictors of death or cardiac-related events in stable ACHD, whilst the prognostic value of CRP vanished after multivariable adjustment (P = 0.057). Receiver-operator characteristic curve analysis identified cut-off values for event-free survival of hs-TnT ≤9 ng/L and NT-proBNP ≤200 ng/L. Patients with both increased biomarkers had a 7.7-fold (confidence interval 3.57–16.40, P < 0.001) higher risk for death and cardiac-related events compared with patients without elevated blood values. Conclusion: Subclinical values of hs-TnT and NT-proBNP are a useful, simple, and independent prognostic tool for adverse cardiac events and survival in stable outpatient ACHD. Registration: German Clinical Trial Registry DRKS00015248. Graphical Abstract [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Effects of aortopulmonary collaterals (APCs) on outcomes after the total cavopulmonary connection (TCPC) are unclear. This study evaluated the incidence of APCs before and after TCPC and analysed the impacts of APCs on adverse outcomes. METHODS A total of 585 patients, who underwent TCPC from 1994 to 2020 and whose preoperative angiographies were available, were included. Pre-TCPC angiograms in all patients were used for the detection of APCs, and post-TCPC angiograms were evaluated in selected patients. Late adverse events included late death, protein-losing enteropathy (PLE) and plastic bronchitis (PB). RESULTS The median age at TCPC was 2.3 (1.8–3.4) years with a body weight of 12 (11–14) kg. APCs were found in 210 patients (36%) before TCPC and in 81 (14%) after TCPC. The closure of APCs was performed in 59 patients (10%) before TCPC, in 25 (4.2%) at TCPC and in 59 (10%) after TCPC. The occurrences of APCs before and after TCPC were not associated with short-term or mid-term mortality. The APCs before TCPC were associated with chylothorax (P  = 0.025), prolonged chest tube duration (P  = 0.021) and PB (P  = 0.008). The APCs after TCPC were associated with PLE (P  < 0.001) and PB (P  < 0.001). With APCs following TCPC, freedom from PLE and PB was lower than without (P  < 0.001, P  < 0.001). CONCLUSIONS APCs before TCPC were associated with chylothorax, prolonged chest tube duration and PB. APCs after TCPC were associated with both PLE and PB. The presence of APCs might affect the lymph drainage system and increase the incidence of chylothorax, PLE and PB. [ABSTRACT FROM AUTHOR]

A letter to the editor and its reply is presented in response to the article "Segmental aortic stiffness in children and young adults with connective tissue disorders: relationships with age, aortic size, rate of dilation, and surgical root replacement" by Ashwin Prakash and colleagues.

Abstract Aims Since the number of adults with congenital heart disease (ACHD) is increasing, age-related cardiovascular diseases become a relevant risk for ACHD. While previous studies investigated isolated risk factors only, this study examines the cardiovascular risk of ACHD based on the PROCAM scores. Methods and results From January 2017 to April 2018, 551 ACHD aged 30 years or older (43.9 ± 9.9 years, 48.3% female) were analyzed for their risk factors of major cardiovascular events within the next ten years using the PROCAM quick check and PROCAM health check. Compared to their individual reference, ACHD had a significantly lower absolute cardiovascular event risk in PROCAM quick check (ACHD: 2.5 ± 4.9%, reference: 3.8 ± 5.2%, p <.001) and PROCAM health check (ACHD: 1.8 ± 3.5%, reference: 3.9 ± 5.3%, p <.001). The relative risk of ACHD was 37% lower than in the general population calculated with the PROCAM quick test, and 57% lower with the PROCAM health check. Only 3.4% of the ACHD had a LDL cholesterol higher than 190 mg/dl, 8.3% had a HDL cholesterol lower than 40 mg/dl, and 26.0% had triglyceride higher than 150 mg/dl. Diabetes mellitus was prevalent in 4.0% of the ACHD and 10.9% were current smokers. Conclusion According to the PROCAM risk score, ACHD have a lower 10-year risk for major cardiovascular events compared to a healthy reference population. Whether this lower rate of the established risk factors leads to a lower rate of acquired cardiovascular disease has to be clarified in this particular population. Highlights • Adult patients with CHD have a lower 10-year risk for major cardiovascular events compared to a healthy reference • 8.3% of the ACHD had a HDL cholesterol lower than 40 mg/dl, and 26.0% had triglyceride higher than 150 mg/dl • Diabetes mellitus was prevalent in 4.0% of the ACHD and 10.9% were current smokers [ABSTRACT FROM AUTHOR]

Background Patients with congenital cardiovascular disease involving the right ventricular outflow tract and with prosthetic valves in the heart are at high risk for developing infective endocarditis (IE). Recently, there has been concern about relatively high rates of IE after percutaneous pulmonary valve implantation (PPVI). Although there are factors specific to percutaneous valves that could plausibly contribute to the risk of IE, including procedural steps prior to implantation such as crimping the valved stent or mechanical forces during dilation, little is known about actual risk factors for this complication. Methods and results The purpose of this study was to assess the incidence rate of IE after PPVI in a single centre long-term experience. The cumulative follow-up time comprised 883.4 patient years for 226 transcatheter valves implanted in the pulmonic position. The annualized incidence rate of IE for all patients receiving valved stents in the RVOT was 1.9%. Freedom from IE 8 years after PPVI was estimated at 87%. The probability for valve removal because of IE was estimated after 8 years at 7%. Conclusion In our experience, the incidence rate of IE after PPVI is acceptable and comparable to surgically implanted biological valves. Despite some IE events, freedom from reoperation was high and there was good performance of the valve in long-term follow-up. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). METHODS In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. RESULTS The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8–3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P  = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P  = 0.007, 20 years: P  = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P  < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P  = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P  < 0.001). CONCLUSIONS There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This study aimed to prove if pulmonary artery (PA) size influences survival and if an additional aortopulmonary shunt (APS) promotes left PA growth after bidirectional cavopulmonary shunt (BCPS) in patients with hypoplastic left heart syndrome. METHODS The medical records of patients with hypoplastic left heart syndrome who underwent Norwood procedure and BCPS between 2007 and 2020 were reviewed. Right, left and total (right + left) PA indices were calculated according to Nakata and colleagues. RESULTS A total of 158 patients were included in this study. The median age at Norwood and BCPS was 8 (7–11) days and 3.6 (3.1–4.6) months, respectively. There were 7 hospital deaths and 12 late deaths. Survival after BCPS was 90.3% at 1 year and 86.2% at 2 years. Total, right and left PA indices were 238 (195–316), 136 (101–185) and 102 (75–130) mm2/m2 at the time of BCPS, and they were 237 (198–284), 151 (123–186) and 86 (69–108) mm2/m2 at the time of Fontan. Left PA index decreased significantly between the time of BCPS and Fontan (P  < 0.01). Nine patients needed partial takedown and additional APS due to failing BCPS, but the additional APS did not promote the PA growth significantly. CONCLUSIONS Preoperative PA index did not affect the mortality after BCPS. The partial takedown and additional APS for failing BCPS were unable to improve left PA size. [ABSTRACT FROM AUTHOR]

The atrial baffle repair (ABR) significantly improved the fate of patients with transposition of the great arteries (TGA). However, these patients show impaired exercise tolerance and some present severe decline of systemic ventricular function. Intrinsic myocardial weakness, low heart rate response to exercise and diastolic filling impairment are discussed to be causative. Forty-nine long-term survivors with TGA (median age 23.7 year) after ABR were catheterized with measured oxygen consumption in four conditions (baseline, volume, atrial pacing, dobutamine) and the results were compared to 10 normal controls. Median cardiac output was significantly lower in the ABR group (2.2 vs. 2.6 l/min/m; p = 0.015), and systemic resistance was significantly elevated (28.9 vs. 22.2 U m; p = 0.04) in comparison with normals. While stroke volume rose by 27% in the control group, it dropped by 7% in patients after ABR at atrial pacing (80/min). Stroke volume increase after dobutamine was significantly lower after ABR in comparison with normal controls (34 vs. 106%; p = 0.001). Higher NYHA class ( p = 0.043), degree of tricuspid regurgitation ( p = 0.009) and ventricular function ( p = 0.028) were associated with lower stroke volume increase. Limited exercise capability of patients after ABR for TGA is primarily due to limited diastolic filling of the ventricles due to stiff non-compliant atrial pathways. Elevated systemic resistance may lead to severe myocardial hypertrophy with possible ischemia and contribute to the multifactorial decline of ventricular function in some patients. [ABSTRACT FROM AUTHOR]

OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis. CONCLUSIONS: Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Studies focused on infants with univentricular heart undergoing stage I palliation other than the Norwood procedure remain a topic of great interest. This study evaluated the outcome of infants who underwent systemic to pulmonary shunt (SPS) or pulmonary artery banding (PAB). METHODS Infants who underwent SPS or PAB as stage I palliation between 1994 and 2019 were included. Survival and late systemic ventricular function were evaluated. RESULTS Out of 242 patients, 188 underwent SPS (77.7%) and 54 PAB (22.3%). Main diagnosis included tricuspid atresia, unbalanced atrioventricular septal defects, double inlet left ventricles and single ventricles with other morphology. Thirty-eight patients died before stage II palliation (15.7%). Stage II palliation was performed in 182 patients (75.2%), and mortality between stages II and III was 11 (5.6%). Stage III palliation was performed in 160 (66.1%) patients. Survival at 1, 5 and 15 years after stage I procedure was 81.9, 77.1 and 76.2%, respectively, and similar between both procedures (P  = 0.97). Premature birth [ P  = 0.03, hazard ratio (HR) = 2.1], heterotaxy (P  = 0.006, HR = 2.4) and dominant right ventricle (P  = 0.015, HR = 2.2) were factors associated to mortality. Unbalanced atrioventricular septal defect (P  = 0.005, HR = 4.6) was a factor associated to systemic ventricular dysfunction. CONCLUSIONS In patients with univentricular heart who underwent SPS and PAB as stage I palliation, survival at 15 years was 76%, regardless of th chosen approach. Premature birth, heterotaxy and dominant right ventricle were associated to mortality. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor in response to an article comparing helical computed tomography scanning and magnetic resonance imaging in the follow-up of adults with coarctation of the aorta, which appeared in an issue of the "CHEST."

Open in new tab Download slide OBJECTIVES We sought to identify the prevalence of surgical reintervention on the neo-aorta after Norwood procedure and its impact on long-term outcomes. METHODS We reviewed the medical records of all patients who underwent Norwood procedure. The impacts of surgical neoaortic reintervention on outcomes were analysed in each stage of palliation. RESULTS A total of 335 patients were included in this study. Thirty patients underwent surgical reintervention on the neo-aorta after Norwood procedure. The timing of initial reintervention was before stage II in 13 patients, at stage II in 7, between stage II and stage III in 5, at stage III in 3 and after stage III in 2. A reintervention before stage II was significantly associated with mortality (HR 14.4, 95% confidence interval 6.00–34.6, P < 0.001). In patients who underwent stage II (n = 251), reintervention had no significant impact on mortality. In patients who underwent stage III (n = 188), the previous reintervention was significantly associated with higher mean pulmonary pressure (P = 0.05) and a higher rate of reduced ventricular function (P = 0.002). Greater than mild atrioventricular valve regurgitation was significantly associated with the development of a neoaortic arch stenosis after stage II (P = 0.03). CONCLUSIONS Surgical reinterventions on the neo-aorta were required in each inter-stage phase. A surgical neoaortic reintervention was not related to increased mortality after stage II but significantly associated with a higher rate of reduced ventricular function and elevated mean pulmonary artery pressure. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES We sought to investigate the impact of early postoperative low arterial oxygen saturation on mortality and morbidity after bidirectional cavopulmonary shunt (BCPS). METHODS The medical records of all patients who underwent BCPS between 2013 and 2018 were reviewed. RESULTS A total of 164 patients were included in this study. Forty-seven patients underwent reintervention during hospital stay at median 7 days after BCPS. Before reintervention, 30 patients were intubated or had SpO2 of <75%. All re-interventions for Glenn pathway obstruction and 4 out of 5 venovenous coil embolization resulted in hospital discharge, while high mortality was observed after other re-interventions (atrioventricular valve surgery, thrombolysis, systemic ventricular outflow obstruction relief, extracorporeal membrane oxygenation implantation and diaphragmatic plication). Additional aortopulmonary shunt with pulmonary artery discontinuation was performed in 8 patients who showed severe cyanosis with median SpO2 of 59% under maximal ventilation support. In the univariable Cox regression analysis, the associated factors for mortality before total cavopulmonary connection were reduced ventricular function [hazard ratio (HR) 6.89, 95% confidence interval (CI) 1.76–26.9, P -value 0.006], greater than moderate atrioventricular valve regurgitation (HR 5.89, 95% CI 1.70–20.4, P -value 0.005), SpO2 1 h after extubation (HR 0.87, 95% CI 0.80–0.96, P -value 0.004) and mean pulmonary artery pressure 1 h after extubation (HR 1.14, 95% CI 1.02–1.26, P -value 0.016). CONCLUSIONS After BCPS, unacceptable cyanosis persisted with various aetiologies. Low arterial oxygen saturation within 1 h after extubation is significantly associated with high mortality after BCPS. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Despite improvements in the surgical management of the hypoplastic left heart syndrome and its variant, the Norwood procedure is still associated with substantial mortality and morbidity and extracorporeal membrane oxygenation support is required in some patients. METHODS We reviewed patients with the Norwood procedure between 2007 and 2019. The primary end point of the study was mortality during extracorporeal membrane oxygenation. Secondary end points included morbidity, bidirectional cavopulmonary shunt and Fontan completion. RESULTS Of the 257 patients in whom the Norwood procedure was performed, mechanical support was required in 41 patients (16%). Indications for extracorporeal membrane oxygenation were low cardiac output (n = 16, 39%), hypoxaemia (n = 12, 29%) and inability to wean from cardiopulmonary bypass (n = 9, 22%). The median age at extracorporeal membrane oxygenation was 10.9 days (interquartile range, 7.9–21.2) and veno-arterial support was required in 37 patients (90.2%). Weaning from extracorporeal membrane oxygenation was achieved in 61% (n = 25). Survival to hospital discharge and 1-year survival was 34.6% (standard deviation: 17.1) and 25.7% (standard deviation: 7), respectively. Bidirectional cavopulmonary shunt was performed in 24% (n = 10) and Fontan completion in 7% (n = 3). Preoperative moderate or greater atrioventricular valve regurgitation was independently associated with mechanical support. Implantation of extracorporeal membrane oxygenation in the paediatric catheter laboratory was identified as an independent risk factor for mortality. CONCLUSIONS Moderate or greater atrioventricular valve regurgitation is an independent risk factor for mechanical support after the Norwood procedure. Mechanical support is associated with substantial in-hospital mortality; however, successful Fontan completion was accomplished in some patients. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES This study was performed to determine the clinical and haemodynamic variables associated with early adverse outcomes after the neonatal Norwood procedure. METHODS Patients who underwent the neonatal Norwood procedure between 2001 and 2019 were included. The patient diagnosis, morphological characteristics and haemodynamic parameters were analysed to identify factors associated with length of stay (LOS) in the intensive care unit (ICU) and mortality during the stay. RESULTS A total of 322 patients were depicted. The median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Certain morphological and preoperative parameters, such as birth weight below 2.5 kg, restrictive atrial septal defect, extracardiac anomalies and the diameter of the ascending aorta, were found to be associated with the LOS in the ICU. Analysis using early postoperative haemodynamic variables revealed that systolic arterial pressure, diastolic arterial pressure, serum lactate levels and reduced ventricular function at 2 days postoperatively were associated with the LOS in the ICU. Birth weight <2.5 kg (P  = 0.010), a restrictive atrial septal defect (P  = 0.001) and smaller ascending aorta (P  = 0.039) were associated with death in the ICU. Reduced ventricular function, lower systolic aortic pressure and higher lactate levels at various time points (P  < 0.05) were also associated with ICU deaths. The LOS in the ICU was significantly associated with late mortality (P  < 0.001, Hazard Ratio (HR) = 1.015). CONCLUSIONS The LOS in the ICU after the Norwood procedure was predicted by early postoperative haemodynamic variables, suggesting that good early postoperative haemodynamics determine early recovery. A prolonged stay in the ICU after the Norwood procedure was associated with late mortality. [ABSTRACT FROM AUTHOR]

Objective Multiple studies have demonstrated the predictive value of arterial stiffness parameters like augmentation index (AIx) for cardiovascular events, the onset of hypertension, and the progression of heart failure. There is evidence that arterial stiffness is increased in some diagnostic subgroups of patients with congenital heart defects (CHD). This study aims to investigate AIx in a large cross-sectional cohort of patients with CHD. Patients and methods We prospectively examined 1125 consecutive patients with various congenital heart defects (27.3 ± 12.1 years, 464 female) referred for routine cardiopulmonary exercise testing (CPET) in our institution, and 322 healthy volunteers (29.4 ± 18.4 years, 165 female). AIx was estimated in supine position using the oscillometric Vicorder device (SMT medical, Würzburg, Germany). Afterward patients performed a CPET. Results In multivariable regression, presence of a CHD emerged as independent risk factor for higher AIx (p < .001). AIx was also higher in older (p < .001), smaller (p < .001) and heavier (p < .001) patients and in females (p = .008). Patients with aortic stenosis (p < .001), Tetralogy of Fallot (p < .001), transposition of the great arteries after atrial switch (p < .001) or Rastelli procedure (p = .013) and after Fontan procedure (p = .002) had higher AIx. Higher peak oxygen uptake (p < .001) and an ACE-inhibitor (p = .088) were associated with a lower AIx. Conclusions AIx is increased in patients with CHD. Several diagnostic subgroups are at risk. A better understanding of pathophysiologic mechanisms, genetic predisposition, the role of surgical aortic scars or implanted conduits/patches and medication is needed to define the value of AIx for further cardiovascular risk assessment in this cohort. [ABSTRACT FROM AUTHOR]

To evaluate the prevalence of veno-venous collaterals (VVCs) after total cavopulmonary connection (TCPC) and analyze their impact on outcomes. Patients undergoing TCPC between 1994 and 2022 were evaluated. VVCs were identified using angiograms of cardiac catheterizations and their impact on outcomes was analyzed. A total of 635 patients were included. Median age at TCPC was 2.3 (interquartile ranges (IQR): 1.8–3.3) years. The most frequent diagnosis was hypoplastic left heart syndrome in 173 (27.2%) patients. Prior bidirectional cavopulmonary shunt was performed in 586 (92.3%) patients at a median age of 5.3 (3.6–9.9) months. VVCs were found in 94 (14.8%) patients at a median of 2.8 (0.1–11.8) years postoperatively. The prevalence of VVCs was similar between the dominant right and left ventricle (14.7 vs. 14.9%, p = 0.967). Mean pulmonary artery pressure (16.2 vs. 16.0 mmHg, p = 0.902), left atrial pressure (5.5 vs. 5.7 mmHg, p = 0.480), transpulmonary gradient (4.0 vs. 3.8 mmHg, p = 0.554) and oxygen saturation (81.4 vs. 82.6%, p = 0.103) before TCPC were similar between patients with and without VVCs. The development of VVCs did not affect survival after TCPC (p = 0.161). Nevertheless, VVCs were a risk for the development of plastic bronchitis (PB, p < 0.001). Interventional closure of VVCs was performed in 60 (9.4%) patients at a median of 8.9 (0.6–15.1) years after TCPC, and improvement of oxygen saturation was observed in 66% of the patients. The prevalence of VVCs after TCPC was 15%. VVCs had no impact on survival following TCPC but were associated with a high prevalence of PB. [Display omitted] [ABSTRACT FROM AUTHOR]

To evaluate the relationship of aortopulmonary collaterals and the development of central pulmonary arteries during staged palliation. A total of 287 patients, who underwent staged palliation with bidirectional cavopulmonary shunt and total cavopulmonary connection between 2008 and 2019, had available angiography. Pulmonary artery index was calculated using pulmonary angiography as described by Nakata and colleagues. Aortopulmonary collaterals were observed in 47 (16%) patients at stage II palliation, in 131 (46%) at total cavopulmonary connection, and afterwards in 49 (7%). The interventional closure of aortopulmonary collaterals was performed before stage II in 12 (4%) patients, before Fontan completion in 38 (13%), and afterwards in 39 (14%). Presence of aortopulmonary collaterals before stage II was not associated with the pulmonary artery index (129 vs. 150 mm2/m2, p = 0.176) at stage II. In contrast, aortopulmonary collaterals before the Fontan completion were associated with lower pulmonary artery index (154 vs. 172 mm2/m2, p = 0.005), and right pulmonary artery index (99 vs. 106 mm2/m2, p = 0.006). Patients who underwent interventional closure of aortopulmonary collaterals before total cavopulmonary connection had lower pulmonary artery index (141 vs. 169 mm2/m2, p < 0.001), lower right pulmonary artery index (93 vs. 106 mm2/m2, p = 0.007), and left pulmonary artery index (54 vs. 60 mm2/m2, p = 0.013) at Fontan completion. The presence of aortopulmonary collaterals did not influence pulmonary artery size by the time of stage II. However, presence of aortopulmonary collaterals was associated with under-developed pulmonary arteries at Fontan completion, especially in patients who needed interventional closure of aortopulmonary collaterals. [ABSTRACT FROM AUTHOR]

Objective: This study compared objectively measured and self-reported physical activity (PA) in adults with congenital heart disease (ACHD) to a healthy reference cohort (RC). Patients and Methods: From May 2017 to August 2020, 211 ACHD (39.9 ± 9.7 years, 101 female) and 141 healthy adults (35.9 ± 14.7 years, 76 female) participated in a wearable-based and self-reported PA assessment. Moderate-to-vigorous PA (MVPA) and the step count were recorded with the Garmin vivofit® 3 device for 7 consecutive days. Additionally, subjects were asked to report the number of days they are active for ≥30 min throughout the week. Results: Only 33 (17%) ACHD and 36 (26%) healthy controls (p = 0.030) accumulated the World Health Organization's (WHO) recommendation of 150 min MVPA per week. ACHD were less active per week (ACHD: 40.0 [0.0; 101.0] min. MVPA vs. RC: 75.0 [22.5; 152.5] min. MVPA, p = 0.002) and walked fewer daily steps (ACHD: 8,246 [6,505; 10,434] vs. RC: 9,413 [7,621; 11,654], p = 0.001) than healthy controls. Especially, patients with moderate (p = 0.030), complex (p < 0.001), or surgically corrected (p = 0.008) congenital heart disease accumulated significantly less MVPA than healthy peers throughout the week. A large majority of 72% of ACHD and 58% of the RC overestimated their weekly active days by more than one day. Conclusions: ACHD walked quite a few steps daily but lacked intensity. ACHD was less active than healthy controls and failed to reach international recommendations. They therefore need encouragement toward more intense movement to improve the exercise capacity and lower cardiovascular risk. Self-reported PA showed no agreement to the objectively measured PA. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES A common atrioventricular valve (CAVV) is considered to be a risk factor for early and late deaths in patients with functional single ventricle (FSV). CAVV surgery in patients with FSV is challenging and there is limited knowledge of the outcomes of CAVV repair with univentricular physiology. METHODS We reviewed all CAVV surgical procedures in patients with FSV who underwent univentricular palliation. End points of the study were survival after CAVV surgery and cumulative incidence of reoperation. RESULTS Between 1984 and 2019, 66 children with CAVV and FSV underwent single-ventricle palliation, of whom 45.5% (30/66) required CAVV surgery. Indication for surgery was moderate CAVV regurgitation in 40% (12/30) and severe CAVV regurgitation in 60% (18/30). CAVV repair was performed in 93.3% (28/30) and CAVV replacement in 6.7% (2/30). The median age and weight at surgery were 0.9 years (interquartile range 0.3–1.8) and 6.5 kg (interquartile range 3.9–8.7), respectively. Operative and late mortality were 23.3% and 8.7%, respectively. Survival and cumulative incidence of reoperation at 4 years after CAVV surgery were 68.9% [standard deviation (SD): 8.7] and 35.8% (SD: 9.1), respectively. Fontan completion was achieved in 60% (18/30). Survival at 4 years after birth was 69.7% (SD: 8.5) in 30 patients with CAVV surgery, whereas it was 83% (SD: 6.3) in 36 patients without CAVV surgery (P  = 0.32). CONCLUSIONS CAVV surgery in patients with FSV is associated with substantial mortality and a high incidence of reoperation. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The ideal shunt for pulmonary blood flow, modified Blalock–Taussig shunt (MBTS) or right ventricular–pulmonary artery conduit (RVPAC) is yet to be determined. This study aimed to evaluate outcomes after the Norwood procedure according to the type of shunt. METHODS A total of 322 neonates with hypoplastic left heart syndrome and related anomalies who underwent the Norwood procedure at our institution between 2001 and 2019 were divided into MBTS and RVPAC groups and the outcomes after the Norwood procedure were compared between the groups with respect to mortality after each staged procedure. RESULTS We identified 322 consequent patients who underwent neonatal Norwood procedure for hypoplastic left heart syndrome (271 patients, 84.2%) and its variant (51 patients, 15.8%). RVPAC was performed in 163 (50.6%) patients and MBTS was performed in 159 (49.4%). There were no differences in the rate of early death (11.0% vs 12.6%, P  = 0.69) or late death (7.4% vs 6.9%, P  = 0.87) between the 2 groups after the Norwood procedure, and no significant difference in the number of patients who reached bidirectional cavopulmonary shunt (77.9% vs 76.1%, P  = 0.69), and there was no difference in mortality after bidirectional cavopulmonary shunt (12.3% vs 7.5%, P  = 0.15) or Fontan completion rate (54.0% vs 52.2%, P  = 0.42) between the 2 groups. Survival at 0.5, 1, 3 and 6 years after the Norwood procedure was 81.0%, 73.8%, 67.9% and 67.0% in patients with RVPAC and 77.1%, 73.3%, 69.1% and 67.9% in patients with MBTS. There was no significant difference in the survival between the 2 groups during the median follow-up of 2.6 (interquartile ranges: 0.3–8.4, maximal 18.8) years (P  = 0.97). CONCLUSIONS In neonates undergoing the Norwood procedure, our available data of maximal 18.8 years follow-up showed no significant difference in early mortality, inter-stage attritions, or overall survival, between MBTS and RVPAC. [ABSTRACT FROM AUTHOR]

Abstract: Objective: This study aimed to compare situational and trait anxiety levels in patients with congenital heart disease (CHD) with that in healthy controls and to investigate their correlation to the perceived health status. Patients and methods: From November 2007 to December 2009 in total 879 patients (405 female, 15–71years) with various CHD and 40 healthy controls completed the State-Trait Anxiety Inventory (STAI) to assess state and trait anxiety, the depression scaling instrument CES-D, and the health-related quality of life questionnaire SF-36. Results: In patients with CHD no increased anxiety as a trait could be found (Mann–Whitney Utest, p=.186). Only situational anxiety in the setting of an outpatient department in a tertiary center was increased compared to healthy controls (Mann–Whitney U test, p=.004). Anxiety was closely related to depression and to all of the nine SF-36 dimensions (r=−.149 to r=−.745, p<.001). Conclusions: In general, patients with CHD show a rather normal anxiety pattern compared to healthy controls. These symptoms of anxiety were strongly correlated with the perceived health status. Only situational anxiety levels at the hospital are increased and still remain an important challenge for doctors and psychologists. [Copyright &y& Elsevier]

Abstract: Objective: In patients with congenital heart disease quality of life is only marginally associated with exercise capacity. The aim of this study was to determine the prevalence of depression and its impact on quality of life and exercise capacity. Patients and methods: From November 2007 to October 2009 a total of 767 patients (352 female, 14–67years) with various congenital heart diseases (37 palliated/native cyanotic, 57 Fontan, 74 Transposition of the Great Arteries (TGA) after atrial switch, 50 other TGA, 136 Fallot, 38 Ebstein, 47 Pulmonic stenosis/regurgitation, 68 aortic coarctation, 103 aortic stenosis, 90 isolated shunts, 67 other) completed the health-related quality of life questionnaire SF-36 and the German translation of the “Center for Epidemiologic Studies Depression Scale” (CES-D) to assess depressive symptoms. Afterwards a cardiopulmonary exercise test was performed. Results: Only 66 patients (8.6%) showed depressive symptoms fulfilling the CES-D definition for depression. The total prevalence of depression was lower than in the general population (Wilcoxon test, p<0.001) and did not differ substantially in between the diagnostic subgroups (Kruskal–Wallis test, p=0.195). CES-D score was correlated to all of the nine dimensions of quality of life (r=−0.170 to r=−0.740, p<0.001) and less pronounced to exercise capacity (r=−0.164, p<0.001). Correlation of peak oxygen uptake to quality of life was weaker than the CES-D scores in all subscales of life quality. Conclusions: Patients with congenital heart disease are rarely depressive. However, even minor depressive symptoms have a stronger impact on quality of life than limited exercise capacity as seen in many patients. [Copyright &y& Elsevier]

Abstract: Objective: Patients with cyanotic congenital heart disease without corrective surgery or palliation survive into adulthood, if they have a balanced pulmonary blood flow facilitated by pulmonary stenosis (PS) or Eisenmenger syndrome (ES). Both groups show cyanosis, diminished exercise performance and impaired quality of life. This study aimed to compare the functional outcome of those two cohorts directly. Patients and methods: In total fifty-eight cyanotic patients with cardiac shunts (28 male, 30 female, aged 14–55years) were investigated, twenty-three of them with PS and thirty-five of them with ES. They completed the health related quality of life questionnaire SF-36 and performed a symptom limited cardiopulmonary exercise test. Results: At exercise, oxygen saturation decreased severely and similarly in both groups (PS: 90% to 65% vs. ES: 87 % to 64%). Moreover, hemoglobin levels were comparable in both subgroups. Exercise capacity was markedly reduced, but more diminished in ES (PS: 20.3 (11.9;24.6) ml/min/kg vs. ES: 11.3 (9.7;14.5) ml/min/kg; p <0.001) and ventilatory inefficiency expressed as V̇ E/V̇ CO2 slope was more enhanced in ES (PS: 45.7 (37.6;52.9) vs. ES: 54.6 (43.4;68.7); p =0.005). Oxygen saturation at rest was correlated to peak V̇ O2 (r =0.436; p =0.001) and V̇ E/V̇ CO2 slope (r =−0.388; p =0.003). Self estimated quality of life was poor, with worse results in physical and psychosocial domains in ES group. Conclusions: Despite similar cyanosis, patients with ES show less exercise performance, more ventilation–perfusion-mismatch and a worse quality of life compared to complex cyanotic congenital heart disease patients with PS. Moreover, oxygen saturation at rest predicts exercise capacity and ventilatory efficiency in this cohort. [Copyright &y& Elsevier]

Purpose: We conducted a study to measure the associations of socio-demographic factors with quality of life outcomes among adults with congenital heart disease (CHD).Methods: Results are drawn from a questionnaire-based survey in 676 adults with CHD and compared to age and sex-matched controls of a representative national survey. Subjective outcomes were measured as health-related quality of life (hrQoL), health and life satisfaction. The associations of the subjective well-being with the degree of severity of the underlying heart defect and socio-demographic factors such as educational and employment status were quantified in multiple linear regression models.Results: A significant correlation of the degree of severity of the heart defect was limited to the physical scale of the hrQoL, whereas for the mental scale of the hrQoL and the satisfaction scales, socio-demographic factors showed a stronger association. Furthermore, the associations of socio-demographic factors and subjective well-being were stronger in the patient group than in the control group.Conclusions: Socio-demographic factors can be significantly associated with the subjective well-being of adults with CHD. In order to assist the surgical successes of the past decades, which have ensured the survival of most of these patients into adulthood, increased attention should be paid to these domains in the care of adults with CHD. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Heterotaxy is a known risk factor for morbidity and mortality in single ventricle palliation. In this study, we examined our experience with this challenging group of patients. METHODS Records of patients born between 2001 and 2019 with heterotaxy, who needed staged single ventricle palliation were retrospectively analysed. RESULTS A total of 53 patients were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation. Forty-one (77.3%) patients received a bidirectional cavopulmonary connection. Thirty-one (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. Twelve (22.6%) patients died before second palliation stage. Four (10%) patients died before TCPC. No patient died after TCPC. Independent risk factors for mortality in the multivariate COX regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02–10.2; P  = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27–10.0; P  = 0.02). CONCLUSIONS Mortality and morbidity in patients with single ventricle and heterotaxy are high. Restrictive pulmonary blood flow needing early modulation and greater than mild atrioventricular valve regurgitation at presentation are independent risk factors for mortality. Total anomalous pulmonary venous connection was not identified as a risk factor in the current era. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The present study was aiming to determine whether high mean pulmonary artery pressure before bidirectional cavopulmonary shunt is a risk factor for late adverse events in patients with low pulmonary artery pressure before total cavopulmonary connection (TCPC). METHODS We retrospectively reviewed the medical records of all patients undergoing both bidirectional cavopulmonary shunt and TCPC with available cardiac catheterization data. RESULTS A total of 316 patients were included in this study. The patients were divided into 4 groups according to mean pulmonary pressure: those with pre-Glenn <16 mmHg and pre-Fontan <10 mmHg (Group LL, n  = 124), those with pre-Glenn ≥16 mmHg and pre-Fontan <10 mmHg (Group HL, n  = 61), those with pre-Glenn <16 mmHg and pre-Fontan ≥10 mmHg (Group LH, n  = 66) and those with pre-Glenn ≥16 mmHg and pre-Fontan ≥10 mmHg (Group HH, n  = 65). Group HL showed significantly higher rate of adverse events after TCPC than Group LL (P  = 0.02). In univariate linear analysis, a history of atrial septectomy at stage 1 palliation was associated with low pre-Glenn mean pulmonary artery pressure (Coefficient B −1.38, 95% confidence interval −2.53 to −0.24; P  = 0.02), while pulmonary artery banding was a significant risk factor for elevated pre-Fontan mean pulmonary artery pressure (Coefficient B 1.68, 95% confidence interval 0.81 to 2.56, P  < 0.001). CONCLUSIONS High mean pulmonary artery pressure before bidirectional cavopulmoary shunt (≥16mmHg) remains a significant risk factor for adverse events after TCPC even though mean pulmonary artery pressure decreased below 10 mmHg before TCPC. [ABSTRACT FROM AUTHOR]

Background: The shift toward a preventative approach in medical aftercare of congenital heart disease (CHD) patients has led to encouragement of regular physical activity (PA) in this patient population. Objective measures are crucial in accurately displaying PA levels and have increasingly found their way into clinical research. This review aims to give an overview about quality, methodology, and outcomes of current scientific work on accelerometers objectively assessing PA in patients with CHD. Methods: Systematically researched literature in all relevant databases (PubMed, Cochrane, and Scopus) over the past decade (2009–2019) with history of CHD and accelerometer-based PA assessment was evaluated by 2 independent reviewers according to the Study Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies of the National Heart, Lung, and Blood Institute. Results: Eight articles with 664 pediatric patients with CHD aged 3–18 years (range 10–162 patients), 5 studies with 574 adults with CHD aged 18–63 years (range 28–330 patients), and 3 studies with 177 pediatric patients and adults with CHD aged 8–52 years were included. Two studies were rated "good"; 9, "fair"; and 5, "poor." Methodologies and devices differed substantially across all studies. Conclusions: Overall study quality was fair at best, and due to difficult methodological comparability of the studies, no clear answer on how active patients with CHD really are can currently be given. Larger studies carefully considering collection and processing criteria, and correct reporting standards exploring PA in patients with CHD from different angles are needed. [ABSTRACT FROM AUTHOR]

OBJECTIVES Our aim was to evaluate the results of tricuspid valve repair (TVr) in patients with hypoplastic left heart syndrome during staged reconstruction, focussing on the timing of the repair and the mechanisms of tricuspid regurgitation (TR). METHODS Records of 44 children with hypoplastic left heart syndrome who underwent a total of 62 tricuspid valve (TV) procedures during staged reconstruction were retrospectively analysed. RESULTS TVr was performed before stage II in 4 (9%) patients, at stage II in 23 (52%) patients, between stages II and III in 3 (7%) patients and at stage III in 14 (32%) patients. The median age at the first TV procedure was 5 months. At surgery, TR emanated commonly from the anteroseptal commissure in 21 (48%) patients. Anterior leaflet prolapse was observed most frequently (n  = 23; 52%), followed by septal leaflet restriction (n  = 22; 50%), dilated annulus (n  = 21; 48%) and cleft anterior leaflet (n  = 9; 21%). Surgical techniques included commissuroplasty in 27 (61.4%) patients, leaflet adaptation in 20 (44%) patients, partial annuloplasty in 11 (25%) patients, chordal reconstruction in 10 (23%) patients and cleft closure in 10 (23%) patients. Among all 44 patients, 27 (61%) patients had preoperative grade III TR and 17 (39%) patients had grade IV; postoperatively, there were no patients with grade IV, 25 patients with grade III (57%), 10 patients with grade II (23%) and 6 patients with grade I (14%). Fifteen patients required redo TV surgeries. Reoperation-free survival was 52% at 5 years. Lower weight at initial TVr predicted mortality [hazard ratio (HR) 0.7, P  = 0.044] and reoperation (HR 0.8, P  = 0.015). TVr before stage II was a risk for both reoperation (HR 5.5, P  = 0.042) and TV replacement (HR 36.9, P  = 0.013). Among morphological factors, septal leaflet restriction was a risk for reoperation (HR 4.7, P  = 0.017) and anterior (HR 4.7, P  = 0.037) and posterior (HR 7.3, P  = 0.015) leaflet chordal anomaly for TV replacement. CONCLUSIONS Anterior leaflet prolapse and septal leaflet restriction are the main mechanisms of TR in hypoplastic left heart syndrome. Early-onset TR before stage II predicts worse outcome. Refinements to repair techniques in early infancy, especially for septal leaflet restrictions and chordal anomalies, are mandatory to improve outcomes. [ABSTRACT FROM AUTHOR]

Patients with Fontan circulation have no subpulmonary ventricle and a passive pulmonary perfusion. Considerable percentage of the pulmonary blood flow is driven by pressure shift due to respiration. Impairments in respiratory musculature strength are associated with a reduced exercise capacity. This study investigated the effect of a daily six months inspiratory muscle training (IMT) on exercise and lung capacity in adult Fontan patients. After a lung function and cardiopulmonary exercise test (CPET), 42 Fontan patients (50% female; 30.5 ± 8.1 years) were randomized into either an intervention group (IG), or a control group (CG). The IG performed a telephone-supervised, daily IMT of three sets with 10–30 repetitions for six months. After six months of IMT, the IG did not improve in any exercise and lung capacity parameter compared to CG. VO 2 peak (ΔVO 2 peak: IG: 0.05 [−1.53; 1.33] ml/kg/min vs. CG: −0.50 [−1.20; 0.78] ml/kg/min; p =.784) and FVC (ΔFVC: IG: 0.07 [−0.16; 0.22] l vs. CG:−0.05 [−0.24; 0.18] l; p =.377) remained unchanged, while FEV1 trended to improve (ΔFEV 1 : IG: 0.05 [−0.07; 0.13] l vs. CG: −0.10 [−0.19; 0.03] l; p =.082). Only oxygen saturation at rest improved significantly (ΔSpO 2 : IG: 1.50 [−0.25; 3.00] % vs. CG: −0.50 [−1.75; 0.75] %; p =.017). A daily six months IMT did not improve exercise and lung capacity and lung volumes in Fontan patients. • Fontan patients show reduced forced vital capacity. • Fontan patients show reduced forced expiratory volume in the first second. • Inspiratory muscle training did not improve exercise capacity in Fontan patients. • Inspiratory muscle training did not improve lung capacity in Fontan patients. • Inspiratory muscle training improved oxygen saturation in Fontan patients. [ABSTRACT FROM AUTHOR]

Bilateral superior vena cava (SVC), which occurs following bilateral bidirectional cavopulmonary shunt (BCPS), is an anomaly marked by unique hemodynamics. This study aimed to determine its effects on outcomes after Fontan completion. Among 405 patients who underwent BCPS and total cavopulmonary connection (TCPC) between 1997 and 2017, 40 required a bilateral-BCPS. The dominant SVC prior to TCPC was identified according to the direction of blood flow in the central pulmonary artery, and its relationship to the inferior vena cava (IVC) was classified as a concordant or discordant relationship. Preoperative factors were analyzed to identify the risk factors for specific adverse outcomes. The length of intensive care unit (ICU) stay after TCPC was longer in the 40 patients who underwent bilateral-BCPS than in those who underwent unilateral BCPS (p = 0.024), and the survival rate was lower in the former group than in the latter group (p = 0.004). In the patients who underwent bilateral-BCPS, the dominant SVC was concordant with the IVC in 30 patients and discordant in 10 patients. With regard to whether certain morphological, hemodynamic, and flow dynamics-related variables were risk factors for adverse outcomes following TCPC, a discordant relationship between dominant SVC and IVC was identified as an independent risk factor for both a longer ICU stay (p = 0.037, HR 2.370) and worse survival (p = 0.019, HR 13.880). Therefore, in patients with a bilateral SVC who have previously undergone bilateral-BCPS, a discordant relationship between dominant SVC and IVC might contribute to worse outcomes following TCPC. [ABSTRACT FROM AUTHOR]