Pulmonary artery banding for cardiomyopathy in young children: First trial in China.

AIMS: Heritable dilated cardiomyopathy (DCM) or DCM associated with congenital or acquired left ventricular diseases carries a significant mortality risk. Pulmonary artery banding (PAB) has been proposed as an alternative to heart transplantation. This study aimed to delineate the clinical development, ventricular reverse remodelling, and functional regeneration of the dilated left ventricle, presenting as a pioneering approach in China. METHODS AND RESULTS: This prospective study was initiated in November 2021, involving paediatric patients with a significant dilated left ventricle and preserved right ventricle who underwent surgical PAB. The baseline characteristics and clinical information during follow-up were collected. Seven patients (five boys) with a median age of 240 (148, 1028) days have been included thus far. No procedural or follow-up mortality was observed. The modified Ross functional class improved from treatment to follow-up of 348 (200, 629) days, and the median left ventricular ejection fraction increased from 27.0 (15.0, 34.0) % before surgery to 61.0 (52.0, 68.0) % (P < 0.05); the median left ventricular end-diastolic diameter and corresponding Z-scores decreased from 43.0 (40.0, 55.0) mm [+9.4 (+7.7, +11.7)] to 33.0 (29.0, 39.0) mm [+1.8 (+1.3, +3.8)] (P < 0.05). Functional regeneration of the left ventricle was observed in five patients. Three of them underwent balloon dilation of the PAB to relieve excessively elevated right ventricular pressures. CONCLUSIONS: The application of PAB should adhere to strict criteria. Initial results are promising for infants and even toddlers with a dilated left ventricle and limited probability of spontaneous recovery. PAB can be an alternative when there is a shortage of donor transplants and assist devices, especially for low- and middle-income countries.

Biopsy-detected myocardial fibrosis predicts adverse cardiac events after pulmonary valve replacement in patients with repaired tetralogy of Fallot.

OBJECTIVES: Risk factors associated with adverse cardiac events (cardiac AEs) after pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot are incompletely understood. In this study, we aimed to determine the relationship between histological myocardial fibrosis and cardiac AEs after PVR in patients with rTOF. METHODS: We consecutively collected clinical, cardiac magnetic resonance, echocardiography and electrocardiogram data of 51 patients with rTOF who underwent surgical PVR. The right ventricular outflow tract tissue was collected during the PVR and the degree of histological myocardial fibrosis was determined by a tailor-made automated image analysis method of picrosirius red staining. RESULTS: The median follow-up time was 4.9 years, and 14 patients had cardiac AEs (a composite of heart failure admission and arrhythmia) during follow-up. The total analysis area of myocardial samples was 5782.18 mm2, and the median percentage of myocardial fibrosis was 20.6% (interquartile range 16.7-27.0%), which were significantly elevated in patients with cardiac AEs compared with patients without cardiac AEs (24.1% vs 19.7%, P = 0.007). Right ventricular ejection fraction and left ventricular end-systolic volume index were significantly associated with myocardial fibrosis in multivariable stepwise linear regression analysis (R2 = 0.238). Cox proportional hazards regression identified degree of myocardial fibrosis [hazard ratio 1.127; 95% confidence interval (CI) 1.047-1.213; P = 0.001] and age at PVR (hazard ratio 1.062; 95% CI 1.010-1.116; P = 0.019) were associated with increased risk of cardiac AEs. The incidence of adverse cardiac events was significantly increased when myocardial fibrosis >20.1% and age at PVR >18.2 years. CONCLUSIONS: Histological myocardial fibrosis was associated with biventricular systolic functions in rTOF. Higher myocardial fibrosis and older age at PVR are independent risk factors for the adverse cardiac events after PVR in patients with rTOF.

Handmade tri-leaflet ePTFE conduits versus homografts for right ventricular outflow tract reconstruction.

BACKGROUND: This study aimed to investigate the performance of handmade tri-leaflet expanded polytetrafluoroethylene (ePTFE) conduits in the absence of a suitable homograft. METHODS: Patients who underwent right ventricular outflow tract reconstruction with tri-leaflet ePTFE conduits or homografts between December 2016 and August 2020 were included. The primary endpoint was the incidence of moderate or severe conduit stenosis (≥ 36 mmHg) and/or moderate or severe insufficiency. The secondary endpoint was the incidence of severe conduit stenosis (≥ 64 mmHg) and/or severe insufficiency. RESULTS: There were 102 patients in the ePTFE group and 52 patients in the homograft group. The median age was younger [34.5 (interquartile range: 20.8-62.8) vs. 60.0 (interquartile range: 39.3-81.0) months, P = 0.001] and the median weight was lower [13.5 (10.0-19.0) vs. 17.8 (13.6-25.8) kg, P = 0.003] in the ePTFE group. The conduit size was smaller (17.9 ± 2.2 vs. 20.5 ± 3.0 mm, P < 0.001) and the conduit Z score was lower (1.48 ± 1.04 vs. 1.83 ± 1.05, P = 0.048) in the ePTFE group. There was no significant difference in the primary endpoints (log rank, P = 0.33) and secondary endpoints (log rank, P = 0.35). Multivariate analysis identified lower weight at surgery [P = 0.01; hazard ratio: 0.75; 95% confidence interval (CI) 0.59-0.94] and homograft conduit use (P = 0.04; hazard ratio: 8.43; 95% CI 1.14-62.29) to be risk factors for moderate or severe conduit insufficiency. No risk factors were found for moderate or severe conduit stenosis or conduit dysfunction on multivariate analysis. CONCLUSION: Handmade tri-leaflet ePTFE conduits showed acceptable early and midterm outcomes in the absence of a suitable homograft, but a longer follow-up is needed.

Clinical Outcomes, Predictors, and Surgical Management of Primary Pulmonary Vein Stenosis.

BACKGROUND: Surgical outcomes for primary pulmonary vein stenosis (PPVS) remain unfavorable, and risk factors are still poorly understood. This study evaluated outcomes and risk factors after PPVS repair. METHODS: This retrospective study included 40 patients with PPVS who underwent surgical repair in Fuwai Hospital from 2010 to 2020. Adverse outcomes included overall death, pulmonary vein (PV) restenosis, and reintervention. A univariate and multivariate risk analysis was performed to determine risk factors. RESULTS: The mean follow-up duration was 37.5 ± 31.5 months. Sutureless technique was performed in 7 patients (17.5%), endovenectomy in 9 (22.5%), and patch venoplasty in 24 (60%). Bilateral PV involvement was documented in 12 patients (30%). Overall death, PV reintervention, and restenosis occurred in 15%, 12.5%, and 25% of patients, respectively. Freedom from overall death, PV reintervention, and restenosis at 5 years was 85% ± 6.3%, 88.9% ± 5.2%, and 65.1% ± 13.2%, respectively. Multivariate analysis revealed that bilateral PV involvement was an independent risk factor for death or PV reintervention (hazard ratio, 10.4; 95% confidence interval, 1.9-56; P = .006) and that involvement of the left inferior PV was an independent risk factor for postoperative restenosis of the left inferior PV (hazard ratio, 13.1; 95% confidence interval, 2.2-76.8; P = .004). CONCLUSIONS: Surgical treatment for PPVS remains a challenging issue with imperfect prognosis. Therefore, it is right and appropriate to take close surveillance on mild or moderate stenosis on a single PV. Bilateral and left inferior PV involvement are independent risk factors for adverse outcomes.

Two approaches for newborns with critical congenital heart disease: a comparative study.

BACKGROUND: Prenatal diagnosis and planned peripartum care is an unexplored concept in China. This study aimed to evaluate the effects of the "prenatal diagnosis and postnatal treatment integrated model" for newborns with critical congenital heart disease. METHODS: The medical records of neonates (≤ 28 days) admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020. The patients were divided into "prenatal diagnosis and postnatal treatment integrated group" (n = 47) and "non-integrated group" (n = 69). RESULTS: The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group (5.2 ± 7.2 days vs. 11.8 ± 8.0 days, P < 0.001; 11.9 ± 7.0 days vs. 16.5 ± 7.7 days, P = 0.001, respectively). The weight at surgery also was lower in the integrated group than in the non-integrated group (3.3 ± 0.4 kg vs. 3.6 ± 0.6 kg, P = 0.010). Longer postoperative recovery time was needed in the integrated group, with a median mechanical ventilation time of 97 h (interquartile range 51-259 h) vs. 69 h (29-168 h) (P = 0.030) and with intensive care unit time of 13.0 days (8.0-21.0 days) vs. 9.0 days (4.5-16.0 days) (P = 0.048). No significant difference was observed in the all-cause mortality (2.1 vs. 8.7%, P = 0.238), but it was significantly lower in the integrated group for transposition of the great arteries (0 vs. 18.8%, log rank P = 0.032). CONCLUSIONS: The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns, and surgical intervention could be performed with a lower risk of death, especially for transposition of the great arteries.

Undifferentiated Chordae Tendineae of the Mitral Valve: Large Cohort Study of a Rare Mitral Malformation.

Aims: This study aimed to investigate the pathology, classification, diagnosis, and surgical prognosis of UCMV. Methods and Results: Consecutive paediatric patients with ≥ moderate-severe mitral regurgitation (MR) and mitral stenosis (MS) were recruited between October 2016 and July 2020. UCMV was diagnosed and classified into three grades according to the involvement of chorda groups and MS presence or absence; other mitral lesions were included as controls. Of 207 eligible patients, 75 with UCMV (10.0 m [interquartile range (IQR): 6.0-21.5]) and 110 with other mitral lesions (16.0 m [IQR: 5.0-43.5]) were diagnosed using echocardiography and surgical exploration. The associated chorda groups of UCMV were confirmed to show high agreement between echocardiography and surgery (kappa = 0.857, p < 0.001). At baseline surgery assessment, the UCMV group exhibited worse New York Heart Association functional class, more severe MR and MS grades, and fewer associated complex anomalies (all, p < 0.05) than the control group. After a mean follow-up of 8.3 (IQR:2.7-14.4) months and adjustment for covariates, the UCMV group required longer cardiopulmonary bypass and aortic clamp times, but there were no differences in the incidence of adverse events (p = 0.584). Class III was associated with higher risk of adverse events than classes I and II (p = 0.002). Conclusions: The UCMV spectrum constitutes a primary pathogenesis of paediatric MV dysfunction, which can be optimally diagnosed using echocardiography. Classification based on mitral anatomy and dysfunction can predict the risk of postoperative adverse events.

Anatomic Repair of Left Main Coronary Artery Atresia: Coronary Ostioplasty With Autologous Pulmonary Artery.

BACKGROUND: Left main coronary arterial (LMCA) atresia is a rare coronary arterial anomaly with extremely limited data on the optimal management. We aimed to report our single-surgeon experience of the ostioplasty in patients with LMCA atresia. METHODS: From July 2018 to December 2019, pediatric patients who presented with LMCA atresia and subsequently underwent surgical coronary ostioplasty were recruited into this retrospective study. Concomitant mitral repair was applied when the regurgitation was moderate or more severe. RESULTS: A total of 9 patients diagnosed with LMCA atresia were included. Mitral regurgitation was found in all of them, including 6 (66.7%) severe, 1 (11.1%) moderate, and 2 (22.2%) mild. In addition to ischemic lesions, which were found in 7 (77.8%) patients, structural mitral problems were also common (presented in 7 [77.8%] patients). All the patients underwent coronary ostioplasty with autologous pulmonary arterial patch augmenting the anterior wall of the neo-ostium. Mean aortic cross clamp time and cardiopulmonary bypass time was 88.1 ± 18.9 and 124.6 ± 23.6 minutes, respectively. During a median of 10.9 (range: 3.3 to 17.2) months' follow-up, there was only 1 death at 5 months after surgery. All survivors were recovered uneventfully with normal left-ventricular function; however, with 4 (50.0%) having significant recurrence of mitral regurgitation. CONCLUSIONS: With favourable surgical outcomes, coronary ostioplasty for LMCA atresia may be an option of revascularization. Structural mitral problems presented in majority patients, resulting in the requirement of concomitant mitral repair. However, the optimal technique of mitral repair remains unclear.

Mid-term results of modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection.

OBJECTIVES: Surgical outcomes of supracardiac total anomalous pulmonary venous connection (TAPVC) repair by the posterior technique (PT) remain unsatisfactory. This study aimed to compare the outcomes of the modified L-shaped incision technique with the PT for supracardiac TAPVC repair. METHODS: From January 2009 to December 2019, 121 consecutive patients with supracardiac TAPVC undergoing surgical repair in our institution were included (L-group, n = 53; PT group, n = 68). A propensity score-matched analysis was performed. Patients with single-ventricle physiology or atrial isomerism were excluded. All clinical data were retrospectively analysed. RESULTS: In the unmatched cohort, the median follow-up duration was 33 months (interquartile range 26-65 months). There were 5 operative mortalities (4.1%) and 12 late mortalities (9.9%). Postoperative pulmonary venous obstruction (PVO) was documented in 21 patients. After matching (52 pairs), the overall survival rate in the L-group was 88.2% at both 3 and 5 years. For the propensity score-matched patients with preoperative PVO (n = 20), statistically significant differences (P = 0.002) were found by Kaplan-Meier curves with freedom from death and postoperative PVO at 1 and 3 years of 100% and 85.7% [standard deviation (SD): 13.2%] in the L-group and 90% (SD: 9.5%) and 22.9% (SD: 14.1%) in the PT group, respectively. Multivariable analysis revealed that the use of the PT was an independent risk factor for death and postoperative PVO (hazard ratio 4.12, 95% confidence interval 1.12-15.16; P = 0.03). CONCLUSIONS: The modified L-shaped incision technique provided an acceptable outcome for supracardiac TAPVC repair. Compared with PT, the modified L-shaped incision technique was significantly associated with decreased death and postoperative PVO in patients with obstructed supracardiac TAPVC.

Tricuspid valvuloplasty for isolated tricuspid regurgitation in children.

BACKGROUND: Isolated congenital tricuspid regurgitation other than Ebstein's anomaly was rare especially for children. The objective of this study was to investigate the clinical characteristics and to assess the results of tricuspid valvuloplasty for children with isolated tricuspid regurgitation. METHODS: From January 2010 to June 2019, 10 consecutive patients with isolated tricuspid regurgitation who were unresponsive to drug therapy underwent tricuspid valvuloplasty in our hospital. Patients' clinical data were analysed retrospectively. RESULTS: Mean age at operation was 48.5 ± 31.0 (range: 9-106) months and mean weight at operation was 16.1 ± 6.9 (range: 8.6-33.0) kg. All patients presented severe isolated tricuspid regurgitation. According to pathological lesions, the main causes accounted for chordae tendinea rupture (3/10), leaflet cleft (2/10), mal-connected chordal tendinea to leaflets (2/10), elongated chordae (1/10) and chordae absent (1/10), and severe anterior leaflet dysplasia (1/10). Individualised tricuspid valvuloplasty was adapted to all of them successfully. Post-operative echocardiography showed no tricuspid regurgitation in two patients and mild regurgitation in eight patients. The cardiothoracic ratios on their chest roentgenograms decreased from 0.59 ± 0.05 to 0.54 ± 0.05. At the latest follow-up (50.4 ± 47.2 months), echocardiography showed that mild to moderate tricuspid regurgitation in seven patients, moderate tricuspid regurgitation in three patients, and no patient with severe tricuspid regurgitation. All patients were in NYHA functional class I. CONCLUSIONS: For patients with isolated tricuspid regurgitation who were not well responsive to drug therapy, individualised tricuspid valve repair can achieve an excellent result.

Modified Single Repair Technique for Complete Atrioventricular Septal Defect: A Propensity Score Matching Analysis.

There were controversies about the priority of modified single patch (MSP) technique compared with two-patch (TP) technique for patients with complete atrioventricular septal defect (CAVSD). From 2002 to 2013, patients who diagnosed as balanced CAVSD in our institution were retrospectively included. Patients with unbalanced ventricles or associated anomalies were excluded. The primary endpoint was all-cause mortality and the secondary endpoint consist of left atrioventricular dysfunction or left atrioventricular outflow tract obstruction (LVOTO). A total of 74 patients underwent MSP repair and 102 patients underwent TP repair. After 1:1 propensity matching, there were 46 Rastelli type A CAVSD patients in each group. Patients in MSP group had shorter cardiopulmonary bypass time [median (interquartile range) 73.5 (65.5-95.0) versus 105.0 (88.8-130.0) min, P < 0.001] and aortic cross-clamp time [105.5 (90.0-128.0) versus 143.0 (122.0-184.0) min, P < 0.001]. In total, the primary endpoint occurred in 12 patients, including 2/46 (4.3%) in MSP group and 10/46 (21.7%) in TP group. That 1-year, 3-year, 5-year survival rate can be calculated as 95.6%, 95.6%, 95.6% in MSP group and 92.6%, 80.4%, 78.2% in TP group, P = 0.015. No significant statistical difference found for the secondary endpoints that 7/46 (15.2%) patients occurred moderate or severe LAVVR in MSP group versus 11/46 (23.9%) patients in TP group, P = 0.293. No LVOTO occurred in both groups. Besides the simplicity of technique, MSP technique may be safer.