OBJECTIVE: Many adult patients with congenital heart disease (ACHD) are still afflicted by premature death. Previous reports suggested natriuretic peptides may identify ACHD patients with adverse outcome. The study investigated prognostic power of B-type natriuretic peptide (BNP) across the spectrum of ACHD in a large contemporary cohort.
METHODS: The cohort included 3392 consecutive ACHD patients under long-term follow-up at a tertiary ACHD centre between 2006 and 2019. The primary study endpoint was all-cause mortality.
RESULTS: A total of 11 974 BNP measurements were analysed. The median BNP at baseline was 47 (24-107) ng/L. During a median follow-up of 8.6 years (29 115 patient-years), 615 (18.1%) patients died. On univariable and multivariable analysis, baseline BNP [hazard ratio (HR) 1.16, 95% confidence interval (CI) 1.15-1.18 and HR 1.13, 95% CI 1.08-1.18, respectively] and temporal changes in BNP levels (HR 1.22, 95% CI 1.19-1.26 and HR 1.19, 95% CI 1.12-1.26, respectively) were predictive of mortality (P < .001 for both) independently of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. Patients within the highest quartile of baseline BNP (>107 ng/L) and those within the highest quartile of temporal BNP change (>35 ng/L) had significantly increased risk of death (HR 5.8, 95% CI 4.91-6.79, P < .001, and HR 3.6, 95% CI 2.93-4.40, P < .001, respectively).
CONCLUSIONS: Baseline BNP and temporal BNP changes are both significantly associated with all-cause mortality in ACHD independent of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. B-type natriuretic peptide levels represent an easy to obtain and inexpensive marker conveying prognostic information and should be used for the routine surveillance of patients with ACHD.

暂无摘要。

Metabolic disorders were a health problem for many adults with congenital heart disease, however, the differences in metabolic syndrome-related metabolite levels in adults with congenital heart disease compared to the healthy population were unknown.
We collected 18 studies reporting metabolic syndrome-associated metabolite levels in patients with congenital heart disease. Data from different studies were combined under a random-effects model using Cohen\'s d values.
The results found that the levels of total cholesterol (Cohen\'s d -0.68, 95% CI: -0.91 to -0.45), high-density lipoprotein cholesterol (Cohen\'s d -0.63, 95% CI: -0.89 to -0.37), and low-density lipoprotein cholesterol (Cohen\'s d -0.32, 95% CI: -0.54 to -0.10) were significantly lower in congenital heart disease patients compared with controls. Congenital heart disease patients also had a lower body mass index (Cohen\'s d -0.27, 95% CI: -0.42 to -0.12) compared with controls. On the contrary, congenital heart disease patients had higher levels of hemoglobin A1c (Cohen\'s d 0.93, 95% CI: 0.17 to 1.70) than controls. Meanwhile, there were no significant differences in triglyceride (Cohen\'s d 0.07, 95% CI: -0.09 to 0.23), blood glucose (Cohen\'s d -0.12, 95% CI: -0.94 to 0.70) levels, systolic (Cohen\'s d 0.07, 95% CI: -0.30 to 0.45) and diastolic blood pressure (Cohen\'s d -0.10, 95% CI: -0.39 to 0.19) between congenital heart disease patients and controls.
The lipid levels in patients with congenital heart disease were significantly lower than those in the control group. These data will help in the health management of patients with congenital heart disease and guide clinicians.
CRD42022228156.

Data on the evolution of congenital heart disease (CHD) in China remain scarce. Based on a Chinese echocardiography database, we analyzed the observed rate (OR) and spectrum changes of CHD over the past 18 years with a focus on the congenital aortic valve malformation (CAVM) and Adult CHD (ACHD).
The transthoracic echocardiographic data of 682,565 records from 2003 to 2020 were retrospectively reviewed at Fujian Medical University Union Hospital, China. A total of 37,200 CHD cases were recruited in this study. Over the three periods (from 2003 to 2008, 2009-2014, to 2015-2020), the OR of Total CHD decreased (106.72, 90.64, and 67.43 per 1000 cases, respectively); the proportion of Simple CHD to Total CHD increased (80.96%, 83.41%, and 87.97%, respectively), with a decrease in the proportion of Complex CHD (18.11%, 15.51%, and 10.42%, respectively) (p < 0.05 for all). The proportion of ACHD increased in most types of CHD [Total CHD: 25.79%, 27.84%, and 31.43%; CAVM: 69.02%, 73.42%, and 78.16%; CAVM with aortic stenosis (AS): 67.42%, 70.73%, and 79.25%; respectively, p < 0.05 for all], with a much higher proportion in both CAVM and CAVM with AS than in the other CHD types. The proportion of CHD patients receiving intervention increased over the designated periods.
This study depicts the longitudinal changes of CHD in the Chinese population with a single-center echocardiographic data, revealing an increased proportion of Simple CHD, ACHD (including CAVM and CAVM with AS), and a decreased OR of Total CHD and proportion of Complex CHD.

BACKGROUND: Right coronary artery (RCA) fistulized to the coronary sinus is rare condition in adult cardiac anomalies, and the management and operative indication are controversial.
METHODS: We describe the case of a 45-year female patient who presented with exertional dyspnea, accompanied by intermitted lower limbs and facial edema. She was diagnosed with severe tricuspid regurgitation second to a severely dilated RCA fistulized to the coronary sinus. After multidisciplinary discussion, she underwent surgery through routine medium sternotomy, the right atrium was opened under cardiopulmonary bypass. The coronary arteriovenous fistula from the distal portion of RC to a severely enlarged coronary sinus was found. Trans-coronary sinus closure of the fistula was performed with continuous stitching and a tricuspid ring annuloplasty was done. The patient recovered uneventful post operation.
CONCLUSIONS: According to current literatures, surgical treatment was adopted for this case, instead of endovascular intervention. The optimal approach for these cases should consider the heart\'s anatomical characteristics. But we need to be aware of the occurrence of myocardial infarction and tricuspid regurgitation in the early and late stage after operation.

The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease.
The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease.
This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360 days, inappropriate shocks, and delivery of appropriate therapy were assessed.
The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30 days and 14.7% at 360 days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring ≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%.
This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations.

UNASSIGNED: Adults with unrepaired tetralogy of Fallot (ToF) are common in developing countries. Long-term overload of the right ventricle places adult patients at risk for postoperative right heart failure after primary repair, which contributes to morbidity and mortality. The effect of pulmonary valve replacement (PVR) in reducing postoperative morbidity and mortality in adults has never been validated.
UNASSIGNED: We conducted a retrospective cohort study in adults (age ≥18 years) with ToF undergoing primary repair from January 2014 to December 2019 at our institution. Patients were divided into three groups according to techniques used to enlarge the right ventricle outflow tract (RVOT). Baseline variables and perioperative outcomes were collected. The primary endpoint was operative mortality. Secondary endpoints were incidences of right heart failure and stage 3 acute kidney injury (AKI).
UNASSIGNED: A total of 56 patients were enrolled (mean age 41.5±11.7 years, 30 females, 53.6%). They were divided into three groups designated as the following: TA-PVR group for trans-annular patch enlargement with PVR; TA group for trans-annulus patch enlargement without PVR; and group AP for annulus preservation. Four patients (7.1%) died postoperatively, all due to right heart failure. All twelve patients in the TA-PVR group survived. There was no significant difference in mortalities among groups. Ten patients (17.9%) developed right heart failure after surgery with no significant difference among groups. Three patients (5.4%) developed stage 3 AKI after surgery, none belonging to the TA-PVR group, however, not statistically significant.
UNASSIGNED: Right heart failure is a common complication after primary repair of adult ToF. Trans-annulus patch enlargement should be cautiously selected in this population. PVR with trans-annulus patch enlargement may be a promising technique to protect against postoperative right heart failure and mortality when annulus preservation is not feasible.

The unique malformation of congenitally corrected transposition of the great arteries (cc-TGA) makes the pulmonary outflow tract (POT) a possible origin of atrial tachycardia (AT).
The purpose of this study was to investigate the mapping characteristics of ATs successfully ablated at the POT in patients with cc-TGA.
Patients with cc-TGA with AT eliminated at the POT were analyzed. Activation mapping of the atria and POT was performed under the guidance of a 3-dimensional electroanatomic mapping system. The activation pattern of these chambers was investigated, with the local activation time (LAT; using coronary sinus ostium as a reference) of the earliest activation site (EAS) being compared.
AT eliminated at the POT was documented in 5 of 6 patients with cc-TGA. The EAS was at the right anteroseptal region with a LAT of 33 (21-120) ms in the right atrium and at the septal wall with a comparable LAT (26, 47, and 26 ms; P = .604) in the left atrium. The EAS of the POT was in the vicinity of the left-facing pulmonary sinus cusp in 3 cases and the nonfacing pulmonary sinus cusp in 2 cases, with a LAT of 106 (28-134) ms preceding both atria. Ablation at this site successfully eliminated AT in all 5 cases.
AT arising adjacent to the POT is not an uncommon tachycardia in patients with situs solitus-type cc-TGA and can be safely eliminated by ablation targeting the EAS in the POT.

Background There are limited data about cardiac resynchronization therapy (CRT) in adult congenital heart disease. We aimed to assess early and late outcomes of CRT among patients with adult congenital heart disease. Methods and Results We retrospectively studied 54 patients with adult congenital heart disease (median age, 46 years; range, 18-73 years; 74% men) who received CRT implantation (biventricular paced >90%) between 2004 and 2017. Clinical and echocardiographic data were analyzed at baseline and early (mean±SD: 1.8±0.8 years) and late (4.7±0.8 years) follow-up after CRT. Compared with baseline, CRT was associated with significant improvement at early follow-up in New York Heart Association functional class, QRS duration, and cardiothoracic ratio (P<0.05 for all); improvement in New York Heart Association class was sustained at late follow-up. Among patients with a systemic left ventricle (LV; n=39), there was significant increase in LV ejection fraction and reduction in LV end-systolic volume at early and late follow-up (P<0.05 for both). For patients with a systemic right ventricle (n=15), there was a significant early but not late reduction in systemic right ventricular basal and longitudinal diameters. Eleven patients died, and 2 had heart transplantation unrelated to systemic ventricular morphological characteristics. Thirty-five patients (65%) responded positively to CRT, but only baseline QRS duration was predictive of a positive response. Conclusions CRT results in sustained improvement in functional class, systemic LV size, and function. Patients with a systemic LV and prolonged QRS duration, independent of QRS morphological characteristics, were most likely to respond to CRT.

BACKGROUND: The traditional approach for adult congenital heart disease combined with valvular disease is surgical treatment under cardiopulmonary bypass (CPB). This approach has a high incidence of postoperative complications, especially in patients with pulmonary hypertension and old age. We present two patients in whom the hybrid procedure was used to treat congenital malformations, followed by valve formation and replacement surgery.
METHODS: A 63-year-old man had a muscular ventricular septal defect complicated by mitral regurgitation and a 57-year-old man had patent ductus arteriosus complicated by aortic stenosis. In both of the patients, the congenital malformation was successfully treated by the hybrid procedure, followed by valve repair or replacement. Both patients had no complications. A post-procedure echocardiogram showed no residual shunt across the duct.
CONCLUSIONS: Our findings suggest that the hybrid procedure is a useful alternative for treating adult congenital heart disease with valvular heart disease. This procedure reduces the surgical incision and difficulty of surgery, shortens the CPB time, avoids residual leakage after surgery, and reduces recovery and hospitalization times.