Relationship of Aortopulmonary Collaterals and Pulmonary Artery Development During Staged Single Ventricle Reconstruction.

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.

Brady-arrhythmias requiring permanent pacemaker implantation during and after staged Fontan palliation.

BACKGROUND: Brady-arrhythmia requiring pacemaker implantation remains one of the Fontan-specific complications before and after total cavopulmonary connection. METHODS: A retrospective analysis of 620 patients who underwent total cavopulmonary connection between 1994 and 2021 was performed to evaluate the incidence of brady-arrhythmia and the outcomes after pacemaker implantation. Factors associated with the onset of brady-arrhythmia were identified. RESULTS: A total of 52 patients presented with brady-arrhythmia and required pacemaker implantation. Diagnosis included 16 sinus node dysfunctions, 29 atrioventricular blocks, and 7 junctional escape rhythms. Pacemaker implantation was performed before total cavopulmonary connection (n = 16), concomitant with total cavopulmonary connection (n = 8), or after total cavopulmonary connection (n = 28, median 1.8 years post-operatively). Freedom from pacemaker implantation following total cavopulmonary connection at 10 years was 92%. Twelve patients needed revision of electrodes due to lead dysfunction (n = 9), infections (n = 2), or dislocation (n = 1). Lead energy thresholds were stable, and freedom from pacemaker lead revision at 10 years after total cavopulmonary connection was 78%. Congenitally corrected transposition of the great arteries (odds ratio: 6.6, confidence interval: 2.0-21.5, p = 0.002) was identified as a factor associated with pacemaker implantation before total cavopulmonary connection. Pacemaker rhythms for Fontan circulation were not a risk factor for survival (p = 0.226), protein-losing enteropathy/plastic bronchitis (p = 0.973), or thromboembolic complications (p = 0.424). CONCLUSIONS: In our cohort of patients following total cavopulmonary connection, freedom from pacemaker implantation at 10 years was 92% and stable atrial and ventricular lead energy thresholds were observed. Congenitally corrected transposition of the great arteries was at increased risk for pacemaker implantation before total cavopulmonary connection. Having a pacemaker in the Fontan circulation had no adverse effect on survival, protein-losing enteropathy/plastic bronchitis, or thromboembolic complications.

Impact of calorie intake and weight gain after Norwood procedure on the outcome of stage II palliation.

BACKGROUND: This study aimed to assess the impact of caloric intake and weight-for-age-Z-score after the Norwood procedure on the outcome of bidirectional cavopulmonary shunt. METHODS: A total of 153 neonates who underwent the Norwood procedure between 2012 and 2020 were surveyed. Postoperative daily caloric intake and weight-for-age-Z-score up to five months were calculated, and their impact on outcome after bidirectional cavopulmonary shunt was analysed. RESULTS: Median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Modified Blalock-Taussig shunt was used in 95 patients and right ventricle to pulmonary artery conduit in 58. Postoperatively, total caloric intake gradually increased, whereas weight-for-age-Z-score constantly decreased. Early and inter-stage mortality before stage II correlated with low caloric intake. Older age (p = 0.023) at Norwood, lower weight (p < 0.001) at Norwood, and longer intubation (p = 0.004) were correlated with low weight-for-age-Z-score (< -3.0) at 2 months of age. Patients with weight-for-age-Z-score < -3.0 at 2 months of age had lower survival after stage II compared to those with weight-for-age-Z-score of -3.0 or more (85.3 versus 92.9% at 3 years after stage II, p = 0.017). There was no difference between inter-stage weight gain and survival after bidirectional cavopulmonary shunt between the shunt types. CONCLUSION: Weight-for-age-Z-score decreased continuously throughout the first 5 months after the Norwood procedure. Age and weight at Norwood and intubation time were associated with weight gain. Inter-stage low weight gain (Z-score < -3) was a risk for survival after stage II.

Tachyarrhythmia after the total cavopulmonary connection: incidence, prognosis, and risk factors.

OBJECTIVE: The purpose of this study is to evaluate the incidence and outcomes regarding tachyarrhythmia in patients after total cavopulmonary connection. METHODS: A retrospective analysis of 620 patients who underwent total cavopulmonary connection between 1994 and 2021 at our institution was performed. Incidence of tachyarrhythmia was depicted, and results after onset of tachyarrhythmia were evaluated. Factors associated with the onset of tachyarrhythmia were identified. RESULTS: A total of 52 (8%) patients presented with tachyarrhythmia that required medical therapy. Onset during hospital stay was observed in 27 patients, and onset after hospital discharge was observed in 32 patients. Freedom from late tachyarrhythmia following total cavopulmonary connection at 5, 10, and 15 years was 97, 95, and 91%, respectively. The most prevalent late tachyarrhythmia was atrial flutter (50%), followed by supraventricular tachycardia (25%) and ventricular tachycardia (25%). Direct current cardioversion was required in 12 patients, and 7 patients underwent electrophysiological study. Freedom from Fontan circulatory failure after onset of tachyarrhythmia at 10 and 15 years was 78% and 49%, respectively. Freedom from occurrence of decreased ventricular systolic function after the onset of tachyarrhythmia at 5 years was 85%. Independent factors associated with late tachyarrhythmia were dominant right ventricle (hazard ratio, 2.52, p = 0.02) and weight at total cavopulmonary connection (hazard ratio, 1.03 per kilogram; p = 0.04). Type of total cavopulmonary connection at total cavopulmonary connection was not identified as risk. CONCLUSIONS: In our large cohort of 620 patients following total cavopulmonary connection, the incidence of late tachyarrhythmia was low. Patients with dominant right ventricle and late total cavopulmonary connection were at increased risk for late tachyarrhythmia following total cavopulmonary connection.

Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.

OBJECTIVES: Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge. METHODS: We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation. RESULTS: Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9-18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively. CONCLUSIONS: In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Impact of early postoperative haemodynamic and laboratory parameters on outcome after the Fontan procedure.

OBJECTIVE: To identify early postoperative haemodynamic and laboratory parameters predicting outcomes following total cavopulmonary connection. METHODS: Patients who underwent total cavopulmonary connection between 2012 and 2021 were evaluated. Serial values of mean pulmonary artery pressure, mean arterial pressure, peripheral oxygen saturation, and lactate levels were collected. The influence of these variables on morbidities was analyzed. Cut-off values were calculated using the receiver operating characteristic analysis. RESULTS: A total of 249 patients were included. All patients had previous bidirectional cavopulmonary shunt. Median age and weight at total cavopulmonary connection were 2.2 (1.8-2.7) years and 11.7 (10.7-13.4) kg, respectively. All patients were extubated in the ICU at a median of 3 (2-5) hours after ICU admission. Postoperative pulmonary artery pressure, around 12 hours after extubation, was significantly associated with chest tube drainage (p = 0.048), chylothorax (p = 0.021), ascites (p = 0.016), and adverse events (p = 0.028). Receiver operating characteristic analysis revealed a cut-off value of 13-15 mmHg for chest tube drainage and chylothorax and 17 mmHg for ascites and adverse events. Mean arterial pressure 1 hour after extubation was associated with prolonged chest tube drainage (p = 0.015) and adverse events (p = 0.008). Peripheral oxygen saturation 6 hours after extubation (p = 0.003) was associated with chest tube duration and peripheral oxygen saturation 1 hour after extubation (p < 0.001) was associated with ascites. Lactate levels on 2nd postoperative day (p = 0.022) were associated with ascites and lactate levels on 1st postoperative day (p = 0.009) were associated with adverse events. CONCLUSIONS: Higher pulmonary artery pressure, lower mean arterial pressure, lower peripheral oxygen saturation, and higher lactate in early postoperative period, around 12 hours after extubation, predicted in-hospital and post-discharge adverse events following total cavopulmonary connection.

[Pulmonary hypertension in adults with congenital heart disease in light of the 2022-ESC-PAH guidelines - Part II: Supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesions, left ventricular disorders, univentricular hearts), interventions, intensive care, follow-up, future perspectives]. / Pulmonale Hypertonie bei Erwachsenen mit angeborenen Herzfehlern im Lichte der 2022-ESC-PAH-Leitlinien.

The number of adults with congenital heart defects (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary hypertension (PH), which may develop early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart disease" is addressed only relatively superficial in these guidelines. Therefore, in the present article, this topic is commented in detail from the perspective of congenital cardiology.

Development of Weight and Height Age z-Score after Total Cavopulmonary Connection.

OBJECTIVE: We aimed to analyze somatic growth of patients after total cavopulmonary connection (TCPC) as well as to identify factors influencing postoperative catch-up growth. METHODS: A total of 309 patients undergoing TCPC at 4 years old or less between 1994 and 2021 were included. Weight for age z-score (WAZ) and height for age-z-score (HAZ) at TCPC and at postoperative time between 1 and 3 years were calculated. Factors influencing somatic growth were analyzed. RESULTS: Most frequent diagnosis and initial palliation were hypoplastic left heart syndrome (HLHS) (34%) and the Norwood procedure (51%), respectively. Median age and weight at TCPC were 2.0 (IQR: 1.7-2.5) years and 11.3 (10.5-12.7) kg, respectively. Median 519 days after TCPC, a significant increase in WAZ (-0.4 to -0.2, p < 0.001) was observed, but not in HAZ (-0.6 to -0.6, p = 0.38). Older age at TCPC (p < 0.001, odds ratio [OR]: 2.6) and HLHS (p = 0.007, OR: 2.2) were risks for low WAZ after TCPC. Older age at TCPC (p = 0.009, OR: 1.9) and previous Norwood procedure (p = 0.021, OR: 2.0) were risks for low HAZ after TCPC. Previous bidirectional cavopulmonary shunt (BCPS) was a protective factor for both WAZ (p = 0.012, OR: 0.06) and HAZ (p = 0.028, OR: 0.30) at TCPC. CONCLUSION: In patients undergoing TCPC at the age of 4 years or less, a significant catch-up growth was observed in WAZ after TCPC, but not in HAZ. Previous BCPS resulted to be a protective factor for a better somatic development at TCPC. HLHSs undergoing Norwood were considered as risks for somatic development after TCPC.

Abnormalities in pulmonary function and volumes in patients with CHD: a systematic review.

BACKGROUND: Lung function and cardiac function are naturally correlated by sharing the thoracic cage and handling the whole cardiac output sequentially. However, lung function studies are rare in patients with CHD, although results worthy of investigation could be expected. This review summarises existing studies with the lung function parameters (spirometry and body plethysmography) in CHD patients during the last decade. METHODS: A systematic review was performed in the relevant database (PubMed, Cochrane, and Scopus) in studies including paediatric and adult patients with CHD where lung parameters (spirometry, body plethysmography) were investigated from January 2010 to December 2020. Two independent reviewers evaluated the studies according to the Study Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies of the National Heart, Lung, and Blood Institute. RESULTS: Eight studies investigated patients with Fontan palliation including 704 patients (306 female). Four studies included patients after repaired tetralogy of Fallot examining 219 patients (103 female), with one study using double. Further six studies included 3208 (1324 female) children and adults with various CHDs. Overall, four studies were categorised as "good", ten as "fair", and four as "poor". While the measurements were consistently standardised, references to calculate %predicted differed substantially across all studies. All evaluated studies showed reduced forced vital capacity in the majority of CHD patients. CONCLUSIONS: Many CHD patients have a reduced forced vital capacity independent of their underlying defect. Spirometry should not only follow a standardised measure according to ATS (update 2019) but also stick to the 2012 GLI reference values.

[New definition and classification of pulmonary hypertension]. / Die neue Definition und Klassifikation der pulmonalen Hypertonie.

In the recent ESC/ERS guidelines on the diagnosis and management of pulmonary hypertension (PH) several important changes have been made in respect of the definition and classification of PH.The mPAP cut-off for defining PH was lowered. PH is now defined by an mPAP > 20 mmHg assessed by right heart catheterization. Moreover, the PVR threshold for defining precapillary PH was lowered. Precapillary PH is now defined by a PVR > 2 WU and a pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg. Furthermore, the increasing evidence for the clinical relevance of pulmonary exercise hemodynamics led to the reintroduction of exercise pulmonary hypertension (EPH) 1. EPH is characterized by a mPAP/CO-slope > 3 mmHg/L/min during exercise testing. In the classification of PH five groups are distinguished: Pulmonary arterial hypertension (group 1), PH associated with left heart disease (group 2), PH associated with lung diseases and/or hypoxia (Group 3), PH associated with pulmonary artery obstructions (group 4) and PH with unclear and/or multi-factorial mechanisms (group 5).In the following guideline-translation we focus on novel aspects regarding the definition and classification of PH and to provide additional background information.

[Diagnostic Algorithm and Screening of Pulmonary Hypertension]. / Diagnose Algorithmus der pulmonalen Hypertonie und Screening-Verfahren.

The new guidelines for the diagnosis and treatment of pulmonary hypertension include a new diagnostic algorithm and provide specific recommendations for the required diagnostic procedures, including screening methods. These recommendations are commented on by national experts under the auspices of the DACH. These comments provide additional decision support and background information, serving as a further guide for the complex diagnosis of pulmonary hypertension.

[Pulmonary arterial hypertension in congenital heart disease - Part I]. / Pulmonale arterielle Hypertonie bei Erwachsenen mit angeborenen Herzfehlern ­ Teil I.

The number of adults with congenital heart disease (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary arterial hypertension (PAH), which may develop early in untreated CHD. Despite timely treatment of CHD, PAH often persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart defects" is addressed only relatively superficially in these guidelines. Therefore, this article addresses the perspective of congenital cardiology in greater depth.

Adults with Congenital Heart Disease Move Well but Lack Intensity: A Cross-Sectional Study Using Wrist-Worn Physical Activity Trackers.

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.

Do children with congenital heart defects meet the vaccination recommendations? Immunisation in children with congenital heart defects.

INTRODUCTION: Congenital heart defects (CHDs) are the most common congenital malformations. Patients with CHD have a higher morbidity and mortality rate and are at greater risk for infectious diseases. The risk might even be higher if complex CHD occurs and if CHD is associated with additional co-morbidities. Therefore, immunisations in these children are essential. MATERIALS AND METHODS: Individuals were recruited at the outpatient centre of the Department of Congenital Heart Defects and Pediatric Cardiology at the German Heart Center Munich in the time between February 2016 and February 2017. Included were children between 23 months and 17 years and a diagnosis of CHD. The vaccination certificate aimed to assess the immunization status. RESULTS: In total, 657 children with CHD were included and analysed. Regarding primary immunisation, only 34 % (n = 221) of the children reached the complete vaccination status within the allowed catch-up time. Among these primary immunisation rates, vaccinations against Hepatitis B, Meningococci, Varicella and Pneumococci were found to have the lowest coverage with all being below 80%. The vaccination rate was partly influenced by the previously performed number of surgeries but not by the diagnosis of specific genetic diseases. At the age of school entry, the immunisation rate in children with CHD was also lower than in the comparable healthy population. CONCLUSION: The vaccination coverage rate in children with CHD is lower than in comparable healthy children, although this is a vulnerable patient group. Further education of parents and treating physicians of children with CHD regarding vaccination is still needed.

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry.

The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with "stable" Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial's eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in "stable" patients.

E-Health Exercise Intervention for Pediatric Patients with Congenital Heart Disease: A Randomized Controlled Trial.

OBJECTIVE: To improve health-related physical fitness (HRPF) (primary outcome) and health-related quality of life (HRQoL) with a web-based motor intervention program in pediatric patients with congenital heart disease (CHD). STUDY DESIGN: Overall, 70 patients (13.0 ± 2.6 years; 34% girls) aged 10-18 years with moderate or complex CHD severity were randomly allocated 1:1 to an intervention or control group. The intervention group trained 3 times per week for 20 minutes in a web-based exercise program over a period of 24 weeks. The control group followed lifestyle per usual. At baseline and follow-up HRPF was assessed via 5 tasks of the FITNESSGRAM and converted to a HRPF z score. HRQoL was assessed with KINDL self-report questionnaire. RESULTS: In total, 61 patients completed the follow-up. There was no change in total HRPF z score (intervention group: 0.14 ± 0.38 vs control group: 0.09 ± 0.38, P = .560) and total HRQoL (intervention group: -1.73 ± 8.33 vs control group: 1.31 ± 7.85, P = .160) after the 24-week web-based exercise intervention. This was true for all subcategories of HRPF and HRQoL. There were no adverse events associated with the web-based exercise intervention. CONCLUSIONS: We found that 24 weeks of web-based exercise intervention with an aimed volume of 60 minutes of exercise per week was safe but did not improve HRPF and HRQoL in children with moderate or complex CHD. TRIAL REGISTRATION: Clinicaltrials.gov: NCT03488797.

Objective Physical Activity Assessment in Clinical Congenital Heart Disease Research: A Systematic Review on Study Quality, Methodology, and Outcomes.

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.

Children with Congenital Heart Disease Are Active but Need to Keep Moving: A Cross-Sectional Study Using Wrist-Worn Physical Activity Trackers.

OBJECTIVE: To compare daily physical activity of children with congenital heart disease (CHD) with healthy peers measured using wearables bracelets in a large cohort. Additionally, subjectively estimated and objectively measured physical activity was compared. STUDY DESIGN: From September 2017 to May 2019, 162 children (11.8 ± 3.2 years; 60 girls) with various CHD participated in a self-estimated and wearable-based physical activity assessment. Step-count and moderate-to-vigorous physical activity were recorded with the Garmin vivofit jr. for 7 consecutive days and compared with a reference cohort (RC) of 96 healthy children (10.9 ± 3.8 years; 49 girls). RESULTS: Children with CHD were active and 123 (75.9%) achieved 60 minutes physical activity on a weekly average according to the World Health Organization criteria as 81 (84.3%) of the healthy peers did (P = .217). After correction for age, sex, and seasonal effects, only slightly lower step count (CHD: 10 206 ± 3178 steps vs RC: 11 142 ± 3136 steps; P = .040) but no lower moderate-to-vigorous physical activity (CHD: 80.5 ± 25.6 minutes/day vs RC: 81.5 ± 25.3 minutes/day; P = .767) occurred comparing CHD with RC. In children with CHD higher age (P = .004), overweight or obesity (P = .016), complex severity (P = .046), and total cavopulmonary connection (P = .027) were associated with not meeting World Health Organization criteria. Subjective estimation of daily moderate-to-vigorous physical activity was fairly correct in half of all children with CHD. CONCLUSIONS: Even though the majority is sufficiently active, physical activity needs to be promoted in overweight or obese patients, patients with complex CHD severity, and in particular in those with total cavopulmonary connection.

Flow Dynamics of Bilateral Superior Cavopulomonary Shunts Influence Outcomes After Fontan Completion.

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.

Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein's Anomaly.

BACKGROUND: Exercise capacity is a well-defined marker of outcome in congenital heart disease. We analyzed seventeen cardiovascular magnetic resonance (CMR) derived parameters and their correlation to exercise capacity in patients with Ebstein's anomaly (EA). METHODS: Fifty-four surgery free patients, age 5 to 69 years (median 30 years) prospectively underwent CMR examination and cardiopulmonary exercise testing (CPET). The following volume/flow parameters were compared with peak oxygen uptake as the percentage of normal (peakVO2%) using univariate and multivariate analysis: right and left ventricular ejection fraction (RVEF and LVEF), the indexed end-diastolic and end-systolic volumes (RVEDVi, RVESVi, LVEDVi, and LVESVi), the indexed stroke volumes (RVSVi and LVSVi), the total normalized right and left heart volumes; the total right to left heart volume ratio (R/L-ratio). The indexed antegrade flow (ante), indexed net flow (net) as well as cardiac index (CI) in the aorta (Ao) and pulmonary artery (PA) were used. RESULTS: RVEF (R2 0.2788), indexed flow PA net (R2 0.2330), and PA ante (R2 0.1912) showed the best correlation with peakVO2% (all p < 0.001) in the univariate model. Further significant correlation could also be demonstrated with CI-PA, LVEF, LVSVi, Aorta net, RVESVi, and Aorta ante. Multivariate analysis for RVEF and indexed net flow PA revealed a R2 of 0.4350. CONCLUSION: Functional CMR parameters as RVEF and LVEF and flow data of cardiac forward flow correlate to peakVO2%. Evaluation of the indexed net flow in the pulmonary artery and the overall function of the right ventricle best predicts the maximal exercise capacity in patients with EA.