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Objectives: This study aimed to evaluate whether fetal echocardiographic parameters were predictive of the postnatal surgical treatment required for fetuses with Tetralogy of Fallot (TOF). Methods: The fetal echocardiographic and postnatal clinical data of all cases of prenatal TOF at Xinhua Hospital from 2016 to 2020 were reviewed. Patients were categorized based on the operation type, and cardiac parameters were compared between groups. Results: Of the 37 fetuses assessed, the development of the pulmonary valve annulus (PVA) was significantly poorer in the transannular patch group. Patients with a prenatal PVA z‐score (Schneider's method) ≥ –2.645, a PVA z‐score (Lee's method) ≥ –2.805, a PVA/aortic valve annulus diameter ratio ≥.697, and a pulmonary annulus index ≥.823 were more likely to undergo pulmonary valve‐sparing surgery. There was a strong correlation between prenatal and postnatal PVA z‐scores. The PVA growth potential was greater in the pulmonary valve‐sparing surgery group. Conclusions: PVA‐related parameters evaluated by fetal echocardiography can predict the type of surgical intervention required and are valuable in improving prenatal counseling in fetal cases of TOF. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Our goal was to evaluate the risk-adjusted effects of operative and non-operative repair on long-term outcomes in patients with congenitally corrected transposition of the great arteries and left ventricular outflow tract obstruction (CCTGA/LVOTO). METHODS We retrospectively reviewed 391 patients with CCTGA/LVOTO from 2001 to 2020 (operative group, 282; non-operative group, 109) in 3 centres in China. The operative group included 73 patients who underwent anatomical repair and 209 patients who underwent non-anatomical repair. The median follow-up time was 8.5 years. The inverse probability of treatment weighted-adjusted Cox regression and a Kaplan–Meier analysis were used to evaluate long-term outcomes. RESULTS Operative repair did not reduce the hazard ratio (HR) of death, tricuspid regurgitation or New York Heart Association functional class III/IV, but a significantly increased HR was observed for pulmonary valve regurgitation [HR, 2.84; 95% confidence interval (CI), 1.10–7.33; P  = 0.031]. Compared with that in the non-operative group, anatomical repair resulted in significantly increased HRs for death (HR, 2.94; 95% CI, 1.10–7.87; P  = 0.032) and pulmonary valve regurgitation (HR, 9.71; 95% CI, 3.66–25.77; P  < 0.001). Subgroup analysis showed that in patients with CCTGA/LVOTO with moderate or worse tricuspid regurgitation (≥ moderate), anatomical repair significantly reduced the HR of death. An inverse probability of treatment weighting-adjusted Kaplan–Meier analysis showed that the survival rates at 5 and 10 days postoperatively were 88.2 ± 4.0% and 79.0 ± 7.9%, respectively, in the anatomical repair group; these rates were significantly lower than the rates in the non-operative group (95.4 ± 2.0% and 91.8 ± 2.8%; P  = 0.032). CONCLUSIONS For patients with CCTGA/LVOTO, operative repair does not provide superior long-term outcomes, and anatomical repair results in a higher incidence of death. However, in patients with CCTGA/LVOTO with tricuspid regurgitation ≥ moderate, anatomical repair can reduce the risk of death in the long term. [ABSTRACT FROM AUTHOR]

Background: Mucopolysaccharidosis Type II (MPS II) is a rare, progressive and ultimately fatal X-linked lysosomal storage disorder caused by mutations in the iduronate-2-sulfatase (IDS) gene. This report conducted a retrospective analysis to investigate the clinical characteristics, genotypes and management strategies in a large cohort of Chinese patients with MPS II. Methods: In this study, we explored 130 Chinese patients with MPS II between September 2008 and April 2022. Clinical manifestations, auxiliary examination, IDS pathogenic gene variants and IDS enzyme activity, surgical history were analysed in the study. Results: A total of 130 patients were enrolled and the mean age at diagnosis was 5 years old. This study found the most common symptoms in our patients were clawlike hands, followed by coarse facial features, birthmarks (Mongolian spot), delayed development, inguinal or umbilical hernia. The most commonly cardiac manifestations were valve abnormalities, which were mitral/tricuspid valve regurgitation (71.9%) and aortic/pulmonary valve regurgitation (36.8%). We had found 43 different IDS pathogenic gene variants in 55 patients, included 16 novel variants. The variants were concentrated in exon 9 (20% = 11/55), exon 3 (20% = 11/ 55) and exon 8 (15% = 8/55). A total of 50 patients (38.5%) underwent surgical treatment, receiving a total of 63 surgeries. The average age of first surgery was 2.6 years, and the majority of surgery (85.7%, 54/63) was operated before 4 years old. The most common and earliest surgery was hernia repair. Three patients were died of respiratory failure. Conclusion: This study provided additional information on the clinical, cardiac ultrasound and surgical procedure in MPS II patients. Our study expanded the genotype spectrum of MPS II. Based on these data, characterization of MPS II patients group could be used to early diagnosis and treatment of the disease. [ABSTRACT FROM AUTHOR]

Today, strategy of repair remains controversial and rare analyses on annular integrity associated with outcomes in complete repair by RVOT incision were performed in a large TOF cohort in China. This is a retrospective cohort study involving patients with TOF who had undergone complete repair by RVOT incision between January 2012 and December 2017 in Fuwai hospital. The primary outcome was a composite of reintervention, significant pulmonary regurgitation (PR) and significant annular peak gradient (APG). Multivariate Cox proportional-hazards model analyses were used to assess the relationships between annular integrity and outcomes. In total, 1673 survival patients with the median age of 318 days were included, and 1002 were male. During a median follow-up of 49 months, 538 participants developed the primary outcome (27 reinterventions). Multivariate Cox analyses showed that compared with AS, TAP was associated with an increased risk of primary outcome (adjusted HR, 1.94 [95% CI 1.60–2.37]) and the results remained unchanged even in most subgroups defined. In secondary outcomes analyses, TAP is associated with a higher risk of reintervention (adjusted HR, 3.32 [95% CI 1.25–8.79]) and significant PR (adjusted HR, 2.51 [95% CI 2.00–3.16]). However, TAP is not associated with a decreased risk of significant APG (adjusted HR, 1.33 [95% CI 0.94–1.88]). PVA integrity preservation is important in complete repair of TOF with RVOT incision. TAP is associated with a higher risk of reintervention and significant PR, and with a similar risk of significant APG. Significant APG in AS patients at discharge has a downtrend over time. [ABSTRACT FROM AUTHOR]

The current study was to report our initial experiences of fetal pulmonary valvuloplasty (FPV) for fetuses with pulmonary atresia with intact ventricular septum (PA/IVS) and critical pulmonary stenosis (CPS), including case selection, technical feasibility, and the effects of FPV on utero and postnatal outcome. Two fetuses with PA/IVS and three fetuses with CPS were enrolled between September 2016 and April 2018. All fetuses were with concomitant severe right ventricular dysplasia and growth arrest. Parameters of right cardiac development and hemodynamics, including tricuspid/mitral annulus ratio (TV/MV), right ventricle/left ventricle long-axis ratio (RV/LV), tricuspid valve inflow duration/cardiac cycle ratio (TVI/CC), degree of tricuspid regurgitation (TR), and blood flow direction of arterial duct and ductus venosus, were evaluated using echocardiogram. FPV was performed trans-abdominally under ultrasound guidance. Echocardiogram was performed post-FPV and every 2–4 weeks thereafter until delivery. The median gestational age at the time of FPV was 28 weeks. From technical perspective, pulmonary balloon valvuloplasty was successfully performed and the opening of pulmonary valve was improved in all fetuses in 2–4 weeks. However, progressive restenosis was observed in four fetuses with gestation advancing, and re-atresia occurred in two PA/IVS fetuses at 36th and 37th weeks' gestation, respectively. The growth trajectories of TV/MV, RV/LV, and TVI/CC were improved in the 1st week after FPV and then slowed down along with pulmonary valve restenosis. All fetuses were born alive and underwent postnatal interventions, including pulmonary balloon valvuloplasty in three fetuses and surgical procedures in two fetuses. During follow-up, three fetuses turned to be biventricular, one became one and a half ventricular at 1-year old, and one died of neonatal infection. Although pulmonary valve restenosis might occur as gestation advancing, FPV seems to be a safe and feasible procedure to improve the growth trajectories of right heart for fetuses with PA/IVS and CPS. [ABSTRACT FROM AUTHOR]

Background. Acute lung injury (ALI) is a rare but life-threatening pulmonary complication of transcatheter arterial chemoembolization (TACE) for hepatocellular carcinoma (HCC). The aim of this study was to characterize the common risk factors, clinical features, imaging findings, treatments, and outcomes of acute lung injury caused by TACE. Methods. A retrospective study was performed on all TACE-associated ALI cases that were diagnosed at authors' hospital from January 2015 to June 2018. Results. The study included 14 ALI cases where the mean age of patients was 60.9±11.7 years (range 41-82 years), with a mean onset time of 2.4±1.6 d after TACE. Of the 14 patients, 8 patients (57.1%) developed acute respiratory distress syndrome (ARDS). 7 patients (50%) had underlying chronic respiratory disease and hepatic arteriovenous fistula was detected in 6 patients (42.6%), both of which were significantly higher than control group (P<0.05). Dyspnea (92.9%) was the most common symptoms. Pleural effusion (64.3%), diffuse pulmonary infiltration (42.9%), and accumulation of Lipiodol in lung field (42.9%) were frequent radiologic abnormalities. 11 patients (78.6%) achieved remission after treatment, and the 30-day mortality rate was approximately 21.4%. Patient's median survival time after the development of ALI was merely 4.3 months, which was obviously worse than control group (4.3 months vs. 13.5 months, P<0.05). Conclusion. This study illustrates that TACE-associated ALI is a rare pulmonary complication with a high mortality rate. We infer that pulmonary Lipiodol embolization might be one of the main causes of TACE-associated ALI. Thus, HCC patients who are at high risk should be closely evaluated and monitored during TACE to avoid such potentially fatal complication. [ABSTRACT FROM AUTHOR]

Background Although the Ross operation requires double-valve replacement for aortic valve disease, it has been shown to provide excellent hemodynamic results and is associated with low morbidity and mortality rates. We reviewed our long-term experience after completion of the Ross procedure. Methods Between October 1994 and February 2009, 58 consecutive patients underwent a Ross procedure at our institution. The right ventricular outñow tract was repaired with a cryopreserved pulmonary homograft. All patients were scheduled for a yearly study thereafter that ended at the time of death or at closure of the follow-up visit. Mean follow-up was 8.2 ± 3.5 years (range: 1.8 to 16.2 years).Results There were two early deaths (3%) and one late death (2%). Actuarial survival at 16 years was 94.8 ± 3.1%. One patient required reoperation and died during reoperation 1.5 years after his first Ross operation. Of the 55 survival patients, 42 patients (76%) were in NYHA functional class 1 and 13 patients (24%) were in NYHA functional class II. Grade 1 or grade 2 autograft regurgitation was observed in seven patients (12%) at 1 year after the surgery. The sinus of Valsalva diameters were all<40 mmin these seven patients. Freedom from hemodynamically relevant autograft regurgitation was 88 ± 2.8% at 16 years. After surgery, left ventricular function was significantly improved and left ventricular end-diastolic diameter recovered to normal over the long term. None of the patients required reoperation due to pulmonary homograft failure. Conclusions The Ross procedure can be safely performed in patients with aortic valve disease. To date, mortality, morbidity, and reoperation rates are very low. Reasons for these superior results may include the following: (1) the diameter of the aortic valve annulus matching that of the pulmonary valve and (2) patients were monitored with antihypertensive medications to keep systolic blood pressure under 120 mm Hg to delay pressure lesions to the pulmonary autograft. [ABSTRACT FROM AUTHOR]

Aims: The aim of this study was to assess the international procedural and short-term to midterm experience with the new percutaneous Venus P-valve., Methods and Results: Retrospective data of patient characteristics, clinical and imaging follow-up of Venus P-valve implantation outside China were collected. Thirty-eight patients underwent attempted Venus P-valve implantation between October 2013 and April 2017. Thirty-seven valves were successfully implanted during 38 procedures. There was one unsuccessful attempt and there were two valve migrations, one of which required surgical repositioning. The mean follow-up was 25 months with no short-term or midterm valve failure or deterioration in performance. Frame fractures occurred in 27% of patients. The cohort demonstrated a statistically significant reduction in pulmonary regurgitation fraction and indexed right ventricular diastolic volumes at six and 12 months., Conclusions: Implantation of the Venus P-valve has provided satisfactory short-term to midterm results with high success and low complication rates in an inherently challenging patient substrate.

Aims: The aim of the study was to evaluate the midterm safety and efficacy of a self-expanding valve (Venus P-valve) in the treatment of patients with pulmonary regurgitation and a native right ventricular outflow tract (RVOT) in China., Methods and Results: Patients who had moderate or severe pulmonary regurgitation after surgical repair of the RVOT with a transannular or RVOT patch were included in the study. Fifty-five patients (67% female; average age 28.7±12.4 years) from six different hospitals in China were enrolled. The procedure success rate was 98.2%. In the one failure, the patient experienced valve dislodgement two days after the procedure. During the 12-month follow-up, two patients died, one due to infective endocarditis. Three other patients developed infective endocarditis. Two patients developed atrial flutter, and one patient had a pulmonary embolism. Echocardiography examinations at 12 months showed that two patients had mild pulmonary regurgitation, and 19 patients had trace pulmonary regurgitation. No paravalvular regurgitation occurred. The mean peak pulmonary gradient was 16.3±7.4 (range 4-38) mmHg. Compared with the baseline data, the right ventricular end-diastolic volume index (RVEDVI) was reduced from 137.6±15.8 mL/m2 to 83.9±16.0 mL/m2 (p<0.001), and the New York Heart Association (NYHA) class was significantly improved (p<0.01)., Conclusions: The one-year results of the China Venus P-valve study show considerable promise for a hitherto unmet need in patients with pulmonary regurgitation and an enlarged native RVOT.