Background: Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease. This study describes current surgical treatment strategies and experiences in a cohort of patients from 2 congenital cardiac centers in Shanghai and Guangdong in China. Methods: This retrospective study included 768 patients operated on between 2005 and 2014. Although most patients (n=690) underwent conventional repair, a sutureless technique was used in 10% (n=78) of cases. A multilevel mixed-effects parametric survival model and a competing-risk analysis were used to analyze associated risk factors for death and recurrent pulmonary venous obstruction (PVO), respectively. Kaplan-Meier analysis was used to analyze the overall survival. The Nelson-Aalen cumulative risk curve was used to compare distributions of time with recurrent PVO. Results: The mean surgical age and weight were 214.9±39.2 days and 5.4±3.6 kg, respectively. Obstructed TAPVC (PVO) was documented in 192 (25%) of the 768 patients. There were 38 intraoperative deaths and 13 late deaths. A younger age at the time of repair ( P =0.001), mixed ( P =0.004) and infracardiac ( P =0.035) TAPVC, preoperative PVO ( P =0.027), prolonged cardiopulmonary bypass time ( P <0.001), and longer duration of ventilation ( P =0.028) were associated with mortality. The median follow-up was 23.2 months (range; 1–112 months). Among the 717 survivors, recurrent PVO was observed in 111 patients (15%). Associated risk factors for recurrent PVO included preoperative PVO ( P <0.001), infracardiac TAPVC ( P <0.001), mixed TAPVC ( P =0.013), and prolonged cardiopulmonary bypass time ( P <0.001). Sutureless technique was associated with a lower restenosis rate compared with conventional repair in patients with preoperative PVO ( P =0.038), except in newborn patients ( P =0.443). Reintervention for restenosis was performed in 24 patients. The function of most survivors (91%) was classified according to the New York Heart Association as functional class I or II. Conclusions: Surgical correction in patients with TAPVC with a biventricular anatomy can achieve an acceptable outcome. Risk factors such as a younger age at the time of repair, infracardiac and mixed TAPVC, and preoperative PVO were associated with a poorer prognosis.

Aims: Computer-assisted auscultation has become available to assist clinicians with physical examinations to detect congenital heart disease (CHD). However, its accuracy and effectiveness remain to be evaluated. This study seeks to evaluate the accuracy of auscultations of abnormal heart sounds of an artificial intelligence-assisted auscultation (AI-AA) platform we create. Methods and results: Initially, 1397 patients with CHD were enrolled in the study. The samples of their heart sounds were recorded and uploaded to the platform using a digital stethoscope. By the platform, both remote auscultation by a team of experienced cardiologists from Shanghai Children’s Medical Center and automatic auscultation of the heart sound samples were conducted. Samples of 35 patients were deemed unsuitable for the analysis; therefore, the remaining samples from 1362 patients (mean age—2.4 ± 3.1 years and 46% female) were analysed. Sensitivity, specificity, and accuracy were calculated for remote auscultation compared to experts’ face-to-face auscultation and for artificial intelligence automatic auscultation compared to experts’ face-to-face auscultation. Kappa coefficients were measured. Compared to face-to-face auscultation, remote auscultation detected abnormal heart sound with 98% sensitivity, 91% specificity, 97% accuracy, and kappa coefficient 0.87. AI-AA demonstrated 97% sensitivity, 89% specificity, 96% accuracy, and kappa coefficient 0.84. Conclusions: The remote auscultations and automatic auscultations, using the AI-AA platform, reported high auscultation accuracy in detecting abnormal heart sound and showed excellent concordance to experts’ face-to-face auscultation. Hence, the platform may provide a feasible way to screen and detect CHD.

Background: Studies focused on pregnant women with congenital heart disease (CHD)–associated pulmonary hypertension (PH) are scarce and limited by small sample sizes and single-center design. This study sought to describe the pregnancy outcomes in women with CHD with and without PH. Methods: Outcomes for pregnant women with CHD were evaluated retrospectively from 1993 to 2016 and prospectively from 2017 to 2019 from 7 tertiary hospitals. PH was diagnosed on the basis of echocardiogram or catheterization. The incidence of maternal death, cardiac complications, and obstetric and offspring complications was compared for women with CHD and no PH, mild, and moderate-to-severe PH. Results: A total of 2220 pregnant women with CHD had completed pregnancies. PH associated with CHD was identified in 729 women, including 398 with mild PH (right ventricle to right atrium gradient 30–50 mm Hg) and 331 with moderate-to-severe PH (right ventricle to right atrium gradient >50 mm Hg). Maternal mortality occurred in 1 (0.1%), 0, and 19 (5.7%) women with CHD and no, mild, or moderate-to-severe PH, respectively. Of the 729 patients with PH, 619 (85%) had CHD-associated pulmonary arterial hypertension, and 110 (15%) had other forms of PH. Overall, patients with mild PH had better maternal outcomes than those with moderate-to-severe PH, including the incidence of maternal mortality or heart failure (7.8% versus 39.6%; P <0.001), other cardiac complications (9.0% versus 32.3%; P <0.001), and obstetric complications (5.3% versus 15.7%; P 100 ng/L (odds ratio, 1.9 [95% CI, 1.0–3.4], P =0.04) and New York Heart Association class III to IV (odds ratio, 2.9 [95% CI, 1.6–5.3], P <0.001) were independently associated with adverse maternal cardiac events in pregnancy with PH, whereas follow-up with a multidisciplinary team (odds ratio, 0.4 [95% CI, 0.2–0.6], P <0.001) and strict antenatal supervision (odds ratio, 0.5 [95% CI, 0.3–0.7], P =0.001) were protective. Conclusions: Women with CHD-associated mild PH appear to have better outcomes compared with women with CHD-associated moderate-to-severe PH, and with event rates similar for most outcomes with women with CHD and no PH. Multimodality risk assessment, including PH severity, brain natriuretic peptide level, and New York Heart Association class, may be useful in risk stratification in pregnancy with PH. Follow-up with a multidisciplinary team and strict antenatal supervision during pregnancy may also help to mitigate the risk of adverse maternal cardiac events.

A meta-analysis was performed to investigate the risk factors for postoperative pulmonary venous obstruction (PVO) after surgical repair of total anomalous pulmonary venous connection (TAPVC). Data bases including PubMed, Embase, Web of Science and Cochrane Library were searched systematically. The goal was to discuss the risk factors for postoperative PVO after TAPVC. Publications were screened by 2 authors independently for criteria inclusion, methodological quality assessment and data extraction. The Newcastle-Ottawa Scale and the Agency for Healthcare Research and Quality checklist were obtained to assess the quality of the studies. Data were pooled by the random effect model or the fixed effect model according to the heterogeneity test. A total of 16 studies (2,385 participants) were included in the meta-analysis. All included studies were retrospective studies. Six potential risk factors were pooled, 5 of which were significantly associated with postoperative PVO. Patients with preoperative PVO were more likely to suffer from postoperative PVO [odds ratio (OR)=5.27, 95% confidence interval (CI) = (2.75, 10.11), P < 0.01]. Compared with a sutureless procedure, the conventional operative procedure was associated with postoperative PVO [OR = 1.80, 95% CI=(1.20, 2.71), P < 0.01]. A mixed type TAPVC plays a critical role in postoperative PVO [OR = 3.78, 95% CI=(1.08, 13.18), P = 0.04]. Inverse variance analysis showed that longer cardiopulmonary bypass time [hazard ratio (HR)=1.01, 95% CI=(1.01, 1.02), P < 0.00001] and aortic cross-clamp time [HR = 1.01, 95% CI=(1.01, 1.02), P < 0.01] were significantly associated with postoperative PVO. Heterotaxy [OR = 1.18, 95% CI = 0.13, 10.45, P = 0.88] was not statistically significant as a risk factor for postoperative PVO. This meta-analysis may provide a perspective on the risk factors for postoperative PVO after TAPVC, thus leading to more studies predicting postoperative PVO after TAPVC with our findings.