Adult Congenital Heart Disease Research Articles

Anxiety and Depression in Adults With Congenital Heart Disease

BackgroundA comprehensive understanding of adult congenital heart disease outcomes must include psychological functioning. Our multisite study offered the opportunity to explore depression and anxiety symptoms within a global sample. ObjectivesIn this substudy of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults With Congenital Heart Disease–International Study), the authors we investigated the prevalence of elevated depression and anxiety symptoms, explored associated sociodemographic and medical factors, and examined how quality of life (QOL) and health status (HS) differ according to the degree of psychological symptoms. MethodsParticipants completed the Hospital Anxiety and Depression Scale, which includes subscales for symptoms of anxiety (HADS-A) and depression (HADS-D). Subscale scores of 8 or higher indicate clinically elevated symptoms and can be further categorized as mild, moderate, or severe. Participants also completed analogue scales on a scale of 0 to 100 for QOL and HS. Analysis of variance was performed to investigate whether QOL and HS differed by symptom category. ResultsOf 3,815 participants from 15 countries (age 34.8 ± 12.9 years; 52.7% female), 1,148 (30.1%) had elevated symptoms in one or both subscales: elevated HADS-A only (18.3%), elevated HADS-D only (2.9%), or elevations on both subscales (8.9%). Percentages varied among countries. Both QOL and HS decreased in accordance with increasing HADS-A and HADS-D symptom categories (P < 0.001). ConclusionsIn this global sample of adults with congenital heart disease, almost one-third reported elevated symptoms of depression and/or anxiety, which in turn were associated with lower QOL and HS. We strongly advocate for the implementation of strategies to recognize and manage psychological distress in clinical settings. (Patient-Reported Outcomes in Adults With Congenital Heart Disease [APPROACH-IS]; NCT02150603)

Anesthetic Challenges in Severe Pulmonary Stenosis Posted for Caesarean Delivery

Isolated Pulmonary stenosis (PS) accounts for 10–12% of adult congenital heart diseases. The effect of PS on pregnancy depends on the severity of the stenosis. There are limited data available in the literature to guide the anesthetic management of pregnant patients with severe PS. We report the successful anesthetic management of a pregnant woman presenting with severe pulmonary re-stenosis 10 years after open pulmonary valvotomy, posted for caesarean section.

Prognostic value of right ventricular dyssynchrony in adults with repaired tetralogy of Fallot

ObjectiveResidual sequelae after surgical repair of tetralogy of Fallot (rTOF) affect clinical outcome. We investigated the prognostic impact of right ventricular (RV) dyssynchrony in adults with rTOF years after the...

Right ventricular fibrosis in adults with uncorrected secundum atrial septal defect and pulmonary hypertension: a cardiovascular magnetic resonance study with late gadolinium enhancement, native T1 and extracellular volume.

Right ventricular (RV) fibrosis represents both adaptive and maladaptive responses to the overloaded RV condition. Its role in pulmonary hypertension (PH) associated with secundum atrial septal defect (ASD), which is the most common adult congenital heart disease (CHD), remains poorly understood. We enrolled 65 participants aged ≥18 years old with uncorrected secundum ASD who had undergone clinically indicated right heart catheterization (RHC), divided into the non-PH group (n = 7), PH group (n = 42), and Eisenmenger syndrome (ES) group (n = 16). We conducted cardiovascular magnetic resonance (CMR) studies with late gadolinium enhancement (LGE) imaging, native T1 mapping, and extracellular volume (ECV) measurement to evaluate the extent and clinical correlates of RV fibrosis. LGE was present in 94% of the population and 86% of the non-PH group, mostly located at the right ventricular insertion point (RVIP) regions. LGE in the septal and inferior RV region was predominantly observed in the ES group compared to the other groups (p = 0.031 and p < 0.001, respectively). The mean LGE scores in the ES and PH groups were significantly higher than those in the non-PH group (3.38 ± 0.96 vs. 2.74 ± 1.04 vs. 1.57 ± 0.79; p = 0.001). The ES and PH groups had significantly higher degrees of interstitial RV fibrosis compared to those in the non-PH group, indicated by native T1 (1,199.9 ± 68.9 ms vs. 1,131.4 ± 47.8 ms vs. 1,105.4 ± 44.0 ms; p < 0.001) and ECV (43.6 ± 6.6% vs. 39.5 ± 4.9% vs. 39.4 ± 5.8%; p = 0.037). Additionally, native T1 significantly correlated with pulmonary vascular resistance (r = 0.708, p < 0.001), RV ejection fraction (r = -0.468, p < 0.001) and peripheral oxygen saturation (r = -0.410, p = 0.001). In patients with uncorrected secundum ASD, RV fibrosis may occur before the development of PH and progressively intensify alongside the progression of PH severity. A higher degree of RV fibrosis, derived from CMR imaging, correlates with worse hemodynamics, RV dysfunction, and poorer clinical conditions.

Evaluation of Post-Operative Atrial Fibrillation after Cardiac Surgery for Adult Congenital Heart Disease

Evaluation of Post-Operative Atrial Fibrillation after Cardiac Surgery for Adult Congenital Heart Disease

VDD-ICD Implantation in a Patient with Dextrocardia and Corrected Transposition of the Great Arteries

We present the case of a 52-year-old female patient on optimal medical therapy (OMT) for heart failure with severely reduced ejection fraction. She was diagnosed with dextrocardia and congenital corrected transposition of the great arteries (ccTGA) by echocardiography. Despite 3 months of OMT and a persisting NYHA II functional state, she was referred for primary prevention ICD implantation. At admission echocardiography revealed a hypertrophic systemic ventricle with an ejection fraction of 27%. Cardiac CT confirmed ccTGA, dextrocardia, a normal extracardiac and epicardial venous system. Coronary artery disease was excluded. Our team opted for left-sided VDD-ICD implantation in case of a need for AV-synchronous pacing and to enhance arrhythmia discrimination. An active fixation shock lead was placed in the mid-septal region of the functional right ventricle. The defibrillation threshold test (DFT) was successful at 20 J. No periprocedural complications were observed. At the 12-month ambulatory visit, device interrogation showed good function, no detected arrhythmias, and no antitachycardia therapy was delivered. Preoperative imaging is essential prior to any invasive cardiac procedure in patients with congenital heart disease. Performing a DFT test at adult congenital heart disease patients may be reasonable.

A motion-corrected deep-learning reconstruction framework for accelerating whole-heart magnetic resonance imaging in patients with congenital heart disease

BackgroundMRI is an important imaging modality for the assessment and management of adult patients with congenital heart disease (CHD). However, conventional techniques for 3D whole-heart acquisition involve long and unpredictable scan times and methods that accelerate scans via k-space undersampling often rely on long iterative reconstructions. Deep-learning-based reconstruction methods have recently attracted much interest due to their capacity to provide fast reconstructions whilst often outperforming existing state-of-the-art methods. In this study, we sought to adapt and validate a non-rigid motion-corrected model-based deep-learning (MoCo-MoDL) reconstruction framework for 3D whole-heart MRI in a CHD patient cohort. MethodsThe previously proposed deep-learning reconstruction framework MoCo-MoDL, which incorporates a non-rigid motion-estimation network and a denoising regularisation network within an unrolled iterative reconstruction, was trained in an end-to-end manner using 39 CHD patient datasets. Once trained, the framework was evaluated in 8 CHD patient datasets acquired with seven-fold prospective undersampling. Reconstruction quality was compared with the state-of-the-art non-rigid motion-corrected patch-based low-rank reconstruction method (NR-PROST) and against reference images (acquired with three-or-four-fold undersampling and reconstructed with NR-PROST). ResultsSeven-fold undersampled scan times were 2.1 ± 0.3 minutes and reconstruction times were ~ 30 seconds, approximately 240 times faster than an NR-PROST reconstruction. Image quality comparable to the reference images was achieved using the proposed MoCo-MoDL framework, with no statistically significant differences found in any of the assessed quantitative or qualitative image quality measures. Additionally, expert image quality scores indicated the MoCo-MoDL reconstructions were consistently of a higher quality than the NR-PROST reconstructions of the same data, with the differences in 12 of the 22 scores measured for individual vascular structures found to be statistically significant. ConclusionThe MoCo-MoDL framework was applied to an adult CHD patient cohort, achieving good quality 3D whole-heart images from ~ 2-minute scans with reconstruction times of ~ 30 seconds.

Lactobacillus plantarum 24-7 improves postoperative bloating and hard stools by modulating intestinal microbiota in patients with congenital heart disease: a randomized controlled trial.

Gastrointestinal symptoms are a common postoperative complication in patients with congenital heart disease (CHD), affecting their postoperative recovery. Probiotic intervention may be a promising therapeutic approach to alleviate postoperative gastrointestinal symptoms. This study aimed to evaluate the potential of Lactobacillus plantarum 24-7 (L. plantarum 24-7) in mitigating postoperative gastrointestinal symptoms and promoting patient recovery. Adult CHD patients scheduled for surgical intervention were recruited. One hundred and twenty patients were randomized and received L. plantarum or placebo intervention twice daily for ten days. Gastrointestinal symptoms were assessed utilizing the Gastrointestinal Symptom Rating Scale (GSRS). Various postoperative variables were analyzed across both groups. Alterations in gut microbiota were evaluated through 16S rRNA sequencing. 112 patients completed the study, with 55 in the probiotic group and 57 in the placebo group. While the disparity in overall postoperative GSRS scores between the two groups did not reach statistical significance (P = 0.067), marked differences were observed in bloating (P = 0.004) and hard stool (P = 0.030) scores. Furthermore, individuals within the probiotic group exhibited lower postoperative neutrophil counts (P = 0.007) and concurrently higher lymphocyte counts (P = 0.001). Variations in the diversity and composition of postoperative gut microbiota were discerned between the probiotic and placebo groups. Remarkably, no probiotic-related adverse events were documented. Supplementation with L. plantarum was well-tolerated and demonstrated partial efficacy in ameliorating gastrointestinal symptoms in postoperative CHD patients. Modulating the gut microbiota may be a potential mechanism by which L. plantarum exerts clinical benefits.

The impact of maternal congenital heart disease on pregnancy outcomes in Malta: a retrospective study

Background: Most female patients with congenital heart disease (CHD) are becoming pregnant. Maternal CHD can have a negative impact on mother and fetus. This is the first study investigating pregnancy outcomes in Maltese grown-up congenital heart disease (GUCH) patients and one of few to compare these with outcomes in women without heart disease. Methods: Known GUCH pregnancies for the period of 2007-2014 were extracted from our database (GUCH cohort) and cardiovascular outcomes retrieved from hospital notes. A control cohort of 540 pregnancies in women without cardiovascular disease was generated through twenty-fold random matching based on subject age from among all pregnancies in Maltese nationals for the same 8-year period. Obstetric and offspring outcomes were compared between the two cohorts. Results: The GUCH cohort consisted of 27 pregnancies in 24 women. Cardiovascular complications occurred in only 1/27 (3.7%) pregnancies. Elective Caesarean sections were commoner (29.6% vs. 15.4%) and unassisted vaginal deliveries less frequent (51.9% vs. 64.6%) in the GUCH cohort (p=0.02). Obstetric complication rates were similar. GUCH women had smaller babies (median 3030 g vs. 3230 g; p=0.045) and showed a trend towards more small-for-gestational age babies (18.5% vs. 8.4%; p=0.08) and congenital malformations (7.4% vs. 2.4%; p=0.06). Conclusions: Despite the potential adverse effects of maternal CHD on mother and fetus, most pregnancies are uncomplicated and outcomes comparable to those in women without heart disease, particularly if baseline clinical status is good. Based on our findings, it is being proposed that prospective mothers be counselled about the possibility of having smaller infants.

The impact of maternal congenital heart disease on pregnancy outcomes in Malta: a retrospective study

Background: Most female patients with congenital heart disease (CHD) are becoming pregnant. Maternal CHD can have a negative impact on mother and fetus. This is the first study investigating pregnancy outcomes in Maltese grown-up congenital heart disease (GUCH) patients and one of few to compare these with outcomes in women without heart disease. Methods: Known GUCH pregnancies for the period of 2007-2014 were extracted from our database (GUCH cohort) and cardiovascular outcomes retrieved from hospital notes. A control cohort of 540 pregnancies in women without cardiovascular disease was generated through twenty-fold random matching based on subject age from among all pregnancies in Maltese nationals for the same 8-year period. Obstetric and offspring outcomes were compared between the two cohorts. Results: The GUCH cohort consisted of 27 pregnancies in 24 women. Cardiovascular complications occurred in only 1/27 (3.7%) pregnancies. Elective Caesarean sections were commoner (29.6% vs. 15.4%) and unassisted vaginal deliveries less frequent (51.9% vs. 64.6%) in the GUCH cohort (p=0.02). Obstetric complication rates were similar. GUCH women had smaller babies (median 3030 g vs. 3230 g; p=0.045) and showed a trend towards more small-for-gestational age babies (18.5% vs. 8.4%; p=0.08) and congenital malformations (7.4% vs. 2.4%; p=0.06). Conclusions: Despite the potential adverse effects of maternal CHD on mother and fetus, most pregnancies are uncomplicated and outcomes comparable to those in women without heart disease, particularly if baseline clinical status is good. Based on our findings, it is being proposed that prospective mothers be counselled about the possibility of having smaller infants.

The impact of maternal congenital heart disease on pregnancy outcomes in Malta: a retrospective study

Background: Most female patients with congenital heart disease (CHD) are becoming pregnant. Maternal CHD can have a negative impact on mother and fetus. This is the first study investigating pregnancy outcomes in Maltese grown-up congenital heart disease (GUCH) patients and one of few to compare these with outcomes in women without heart disease. Methods: Known GUCH pregnancies for the period of 2007-2014 were extracted from our database (GUCH cohort) and cardiovascular outcomes retrieved from hospital notes. A control cohort of 540 pregnancies in women without cardiovascular disease was generated through twenty-fold random matching based on subject age from among all pregnancies in Maltese nationals for the same 8-year period. Obstetric and offspring outcomes were compared between the two cohorts. Results: The GUCH cohort consisted of 27 pregnancies in 24 women. Cardiovascular complications occurred in only 1/27 (3.7%) pregnancies. Elective Caesarean sections were commoner (29.6% vs. 15.4%) and unassisted vaginal deliveries less frequent (51.9% vs. 64.6%) in the GUCH cohort (p=0.02). Obstetric complication rates were similar. GUCH women had smaller babies (median 3030 g vs. 3230 g; p=0.045) and showed a trend towards more small-for-gestational age babies (18.5% vs. 8.4%; p=0.08) and congenital malformations (7.4% vs. 2.4%; p=0.06). Conclusions: Despite the potential adverse effects of maternal CHD on mother and fetus, most pregnancies are uncomplicated and outcomes comparable to those in women without heart disease, particularly if baseline clinical status is good. Based on our findings, it is being proposed that prospective mothers be counselled about the possibility of having smaller infants.

Hospital Care for Adult Patients with Congenital Heart Diseases

Objective: The ideal type of hospital to care for adult congenital heart disease (ACHD) patients is not well known. Hospital competitiveness, clinical volume and market structure can influence clinical outcomes. We sought to understand how hospital competitiveness affects clinical outcomes in ACHD patients in the era prior to the Adult Congenital Heart Association accreditation program. Methods: Patient discharges with ACHD diagnosis codes were filtered between 2006–2011 from an all-payer inpatient healthcare database. Hospital-level data was linked to market structure patient flow. A common measure of market concentration used to determine market competitiveness—the Herfindahl-Hirschman Index (HHI)—was stratified into: more competitive (HHI ≤25th percentile), moderately competitive (HHI 25th to &lt;75th percentile), and less competitive (HHI ≥75th percentile) hospital. Any complication, home discharge and mortality were analyzed with clustered mixed effects logistic regression. The combined impact of HHI and any complication on mortality by interaction was assessed. Results: A total of 67,434 patient discharges were isolated. More competitive hospitals discharged the least number of patients (N = 15,270, 22.6%) versus moderately competitive (N = 36,244, 53.7%) and less competitive (N = 15,920, 23.6%) hospitals. The adjusted odds of any complication or home discharge were not associated with hospital competitiveness strata. Compared to more competitive hospitals, mortality at moderately competitive hospitals (Adjusted Odds Ratio (AOR) 0.79, 95% CI: 0.66–0.94) and less competitive hospitals (AOR 0.79, 95% CI: 0.63–0.98) were lower (p = 0.025). Age, race, elective admission, transfer status, and payer mix were all significantly associated with adjusted odds of any complication, home discharge and mortality (p ≤ 0.05). Having any complication independently increased the adjusted odds of mortality more than 6-fold (p &lt; 0.001), and this trend was independent of HHI strata. Failure to rescue an ACHD patient from mortality after having any complication is highest at less competitive hospitals. Sensitivity analysis which excluded the transfer status variable, showed that any complication (p = 0.047) and mortality (p = 0.01) were independently associated with HHI strata. Conclusions: Whether lower competition allow hospitals to focus more on quality of care is unknown. Hospital competitiveness and outcome seem to have an inverse trend relationship among ACHD patients. Since medical care is frequently provided away from the home area, hospital selection is an important issue for ACHD patients. Further research is needed to determine why competitiveness is linked to surgical outcomes in this population.

Patient factors and geographic barriers influencing excess time between paediatric and adult CHD care.

Over 90% of children with CHD survive into adulthood and require lifelong cardiology care. Delays in care predispose patients to cardiac complications. We sought to determine the time interval to accessing adult CHD care beyond what was recommended by the referring paediatric cardiologist (excess time) and determine risk factors for prolonged excess time. Retrospective cohort study including all patients in the province of Alberta, Canada, age 16-18 years at their last paediatric cardiology visit, with moderate or complex lesions. Excess time between paediatric and adult care was defined as the interval (months) between the final paediatric visit and the first adult visit, minus the recommended interval between these appointments. Patients whose first adult CHD appointment occurred earlier than the recommended interval were assigned an excess time of zero. We included 286 patients (66% male, mean age 17.6 years). Mean excess time was 7.9 ± 15.9 months. Twenty-nine (10%) had an excess time > 24 months. Not having a pacemaker (p = 0.03) and not needing cardiac medications at transfer (p = 0.02) were risk factors for excess time >3 months. Excess time was not influenced by CHD complexity. The mean delay to first adult CHD appointment was almost 8 months longer than recommended by referring paediatric cardiologists. Not having a pacemaker and not needing cardiac medication(s) were risk factors for excess time > 3 months. Greater outpatient resources are required to accommodate the growing number of adult CHD survivors.

Trends in Unplanned Admissions of Patients With Adult Congenital Heart Disease Based on the Japanese Registry of All Cardiac and Vascular Diseases-Diagnosis Procedure Combination Study.

The prevalence of adult congenital heart disease (ACHD) is increasing rapidly and in particular, patients who underwent complicated surgeries are reaching their youth and middle age. Therefore, the need for ACHD treatment will increase, but the current medical situation is unknown. In this study we assessed trends in unplanned admissions in patients with ACHD in Japan.Methods and Results: From the Japanese Registry of All Cardiac and Vascular Diseases-Diagnosis Procedure Combination, a nationwide claim-based database, we selected patients aged >15 years with CHD defined by the International Classification of Diseases, 10th Revision codes. We identified 39,676 admissions between April 2012 and March 2018; 10,444 (26.3%) were unplanned. Main diagnoses were categorized into 3 degrees of complexity (severe, moderate, and mild) and other. Among unplanned admissions, the proportion of the severe group increased with time. Patients in the mild group were significantly older than those in the moderate and severe groups (median age: 70.0, 39.0, and 32.0 years, respectively). There were 765 deaths during hospitalization (overall mortality rate, 7.3%). The odds ratio of death during admission was significantly higher in patients aged >50 years, especially in the moderate group. Patients with moderate or severe ACHD tended to experience unplanned admissions at a younger age. In anticipation of greater numbers of new, severe patients, we need to prepare for their increasing medical demands.

Collaboration in the Interventional Care of the Adult Congenital Heart Disease Patient: Teamwork Is Key

Collaboration in the Interventional Care of the Adult Congenital Heart Disease Patient: Teamwork Is Key

Effects of trimetazidine on cardiac function in adult cyanotic congenital heart disease patients: Protocol for a 3-month multicenter, randomized, double-blind controlled trial

Nearly 20% Patients with cyanotic congenital heart disease (CCHD) are not able to receive surgery. These patients experience a decline in cardiac function as they age, which has been demonstrated to be associated with changes in energy metabolism in cardiomyocytes. Trimetazidine (TMZ), a metabolic regulator, is supposed to alleviate such maladaptation and reserve cardiac function in CCHD patients. This is a randomized, double-blind, placebo-controlled clinical trial. Eighty adult CCHD patients will be recruited and randomized to the TMZ (20 mg TMZ 3 times a day for 3 months) or placebo group (placebo 3 times a day for 3 months). The primary outcome is the difference in cardiac ejection fractions (EF) measured by cardiac magnetic resonance (MRI) between baseline and after 3 months of TMZ treatment. The secondary outcomes include TMZ serum concentration, rate of cardiac events, NYHA grading, fingertip SpO2, NT-proBNP levels, 6-minute walking test (6MWT), KCCQ-CSS questionnaire score, echocardiography, ECG, routine blood examination, liver and kidney function test, blood pressure and heart rate. This trial is designed to explore whether the application of TMZ in adult CCHD patients can improve cardiac function, reduce cardiac events, and improve exercise performance and quality of life. The results will provide targeted drug therapy for CCHD patients with hypoxia and support the application of TMZ in children with CCHD.

Outcomes of permanent pacemakers and implantable cardioverter-defibrillators in an adult congenital heart disease population

BackgroundBrady- and tachyarrhythmias commonly complicate adult congenital heart disease (ACHD). Permanent pacemakers (PPMs) or implantable cardioverter–defibrillators (ICDs) are often utilised to prevent morbidity or mortality related to arrhythmia, but can also be associated with significant morbidity themselves. MethodsWe analysed outcomes from patients in our comprehensive ACHD database who were seen at least twice since 2000 and once since 2018. Of 1953 ACHD patients, 134 had a PPM and 78 had an ICD (47 for primary and 31 for secondary prevention). ResultsFor PPM patients, 41% had a pacing percentage below 33%, 13% had 33–66%, and 46% had above 66%. One fifth required PPM upgrade, most to cardiac resynchronisation therapy, the rest to ICD. There were 33 appropriate ICD shocks in 15 patients (19%) and 34 inappropriate shocks in 13 patients (17%) over a median follow up of 4.6 years (IQR 0.9–8.3 years). Anti-tachycardia pacing was delivered appropriately for 28% of patients and inappropriately for 9%.Apart from inappropriate therapy, one third of PPM and ICD patients had other device-related complications. Acute PPM complications included lead dysfunction requiring revision (2%), pneumothorax (2%), pleural effusion (2%) and pocket infection (2%). ICDs were also acutely complicated by lead dysfunction (4%) as well as pocket hematoma (3%). The most common long-term complication overall was lead dysfunction, affecting one sixth of both PPM and ICD patients. Finally, the rate of device insertion increased significantly with disease severity. ConclusionsAnti-arrhythmic devices can be lifesaving in ACHD patients, but inappropriate therapy and device-related complications are very common.

Single institution retrospective case cohort of adult cyanotic congenital heart disease and associated pheochromocytoma and paraganglioma

Single institution retrospective case cohort of adult cyanotic congenital heart disease and associated pheochromocytoma and paraganglioma

Comorbidities in congenital heart disease: different patterns in childhood and adulthood

BackgroundExisting studies were no exploration of the association between congenital heart disease (CHD) in children and comorbidities. This study was to assess the prevalence and number of comorbidities in CHD among children and adults, and to compare the comorbidity patterns by children and adults using association rule analysis.MethodsPatients identified by the International Classification of Diseases, Ninth Revision (ICD‐9) code in the Medical Information Mart for Intensive Care III (MIMIC-III) 2001–2012 and MIMIC-IV 2008–2018 were included in this cross-sectional study. Association rule analysis was used to explore associations between CHD and comorbidities in children and adults using values of support (%), confidence (%), and lift.ResultsAmong 60,400 eligible patients, 1.54% of adults had CHD and 0.83% of adults had CHD with at least one comorbidity, 13.79% had CHD and 12.37% had CHD with at least one comorbidity in children. The most common comorbidities were circulatory system diseases (53.78%), endocrine diseases (35.76%), and respiratory system diseases (23.46%) in adults with CHD, and the most common comorbidities were perinatal diseases (87.50%) in children with CHD. The comorbidity rate was 90.19% and 56.68% in children and adults, respectively. In children, perinatal diseases, circulatory system diseases, and endocrine diseases had the highest prevalence. The incidence of circulatory system diseases, perinatal diseases and endocrine diseases in CHD adults was confidence = 31.56%, 36.11%, and 23.23%, respectively. Perinatal diseases were common comorbidities among all CHD severity groups in children and adults.ConclusionThe prevalence of comorbidities in children with CHD was higher than that in adults with CHD. The most common comorbidities were perinatal diseases and endocrine diseases among children and adults with CHD, respectively. Our study provided insights into comorbidity patterns in children and adults with CHD.

Long-term outcomes after heart transplantation in adult patients with univentricular versus biventricular congenital heart disease.

Heart transplantation (HT) is the only life-extending therapy in adults with congenital heart disease (CHD) and end-stage heart failure. HT is considered at high risk in complex CHD given the anatomical complexity and past medical history. Little is known about long-term outcomes after HT in these patients. We aimed to evaluate early and long-term outcomes after HT in adult patients with univentricular versus biventricular CHD. This multicentre retrospective cohort study included all adult CHD patients who underwent HT between 1988 and 2021 in 3 tertiary centres. Factors associated with early (<30 days) and conditional long-term survival were assessed in the entire cohort. Over a mean follow-up of 10.1 ± 7.8 years, 149 patients were included, of whom 55 (36.9%) had univentricular CHD. Sixty-four patients died during follow-up including 47 deaths before discharge from hospital. In multivariable analysis, univentricular physiology and female recipient gender were independently associated with a higher risk of early mortality (odds ratio 2.99; 95% confidence interval [1.33-6.74] and odds ratio 2.76; 95% confidence interval [1.23-6.20], respectively). For patients who survived the early period, conditional long-term survival was excellent for both groups and was not different between 2 groups (P = 0.764). Adult CHD patients have a high incidence of overall mortality due to a high rate of early mortality. Univentricular physiology was associated with a significant increased risk of early death compared to biventricular physiology. However, late mortality was excellent and no longer different between the 2 physiologies.