10-year survival of pulmonary arterial hypertension associated with connective tissue disease: insights from a multicentre PAH registry.

OBJECTIVES: To report the 10-year survival rate and prognostic factors of pulmonary arterial hypertension associated with CTD (CTD-PAH) patients, to compare treatment and survival between patients enrolled before and after 2015, and to validate the discrimination of the recommended four-strata model in predicting 10-year survival at follow-up in Chinese CTD-PAH patients. METHODS: This study was derived from a Chinese national multicentre prospective registry study from 2009 to 2019. Medical records were collected at baseline and follow-up, including PAH-targeted therapy and binary therapy (both CTD and PAH-targeted therapy). RESULTS: A total of 266 CTD-PAH patients were enrolled and the 10-year survival rate was 59.9% (median follow-up time: 4.85 years). Underlying CTD (SSc), baseline 6-min walking distance and SaO2 were independent risk factors for 10-year survival. The proportion of patients receiving PAH-targeted combination therapy increased from 10.1% (2009-2014) to 26.5% (2015-2019) and that of binary therapy increased from 14.8% to 35%. The 1-year survival rate increased from 89.8% (2009-2014) to 93.9%, and the 3-year survival rate increased from 80.1% (2009-2014) to 86.5% (both P > 0.05). The four-strata strategy performed well in predicting 10-year survival at follow-up (C-index = 0.742). CONCLUSION: The 10-year survival rate of CTD-PAH patients was reported for the first time. The 10-year prognosis was poor, but there was a tendency for more standardized treatment and better survival in patients enrolled after 2015. The recommended four-strata model at follow-up can effectively predict 10-year survival in CTD-PAH patients.

Characteristics, goal-oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry.

BACKGROUND AND OBJECTIVE: Nationally representative reports on the characteristics and long-term survival of pulmonary arterial hypertension (PAH) from developing countries are scarce. The applicability of the current main risk stratifications and the longitudinal changes in goal-oriented treatments have yet to be elucidated in real-world settings. Therefore, we aimed to provide insights into the characteristics, goal-oriented treatments and survival of PAH in China and to explore the applicability of the main risk stratifications in our independent cohort. METHODS: PAH patients were consecutively enrolled from a national prospective multicentre registry. Data on baseline, follow-up re-evaluation and therapeutic changes were collected. RESULTS: A total of 2031 patients were enrolled, with congenital heart disease (CHD)-PAH (45.2%) being the most common aetiology. The mean age was 35 ± 12 years, and 76.2% were females. At baseline, approximately 20% of the patients with intermediate or high risk received combination treatment. At follow-up, approximately half of the re-evaluated patients did not achieve low-risk profiles, and even among patients who received combination therapy at baseline, 4% of them still worsened. The rate of combination therapy increased significantly from 6.7% before 2015 to 35.5% thereafter. The main risk assessment tools demonstrated good performance for predicting survival both at baseline and at follow-up. CONCLUSION: Chinese PAH patients show both similar and distinct features compared to other countries. Current main risk stratifications can significantly discriminate patients at different risk levels. There were still many patients not achieving low-risk profiles at follow-up, indicating more aggressive treatment should be implemented to optimize the goal-oriented treatment strategy.

Machine learning in a real-world PFO study: analysis of data from multi-centers in China.

PURPOSE: The association of patent foreman ovale (PFO) and cryptogenic stroke has been studied for years. Although device closure overall decreases the risk for recurrent stroke, treatment effects varied across different studies. In this study, we aimed to detect sub-clusters in post-closure PFO patients and identify potential predictors for adverse outcomes. METHODS: We analyzed patients with embolic stroke of undetermined sources and PFO from 7 centers in China. Machine learning and Cox regression analysis were used. RESULTS: Using unsupervised hierarchical clustering on principal components, two main clusters were identified and a total of 196 patients were included. The average age was 42.7 (12.37) years and 64.80% (127/196) were female. During a median follow-up of 739 days, 12 (6.9%) adverse events happened, including 6 (3.45%) recurrent stroke, 5 (2.87%) transient ischemic attack (TIA) and one death (0.6%). Compared to cluster 1 (n = 77, 39.20%), patients in cluster 2 (n = 119, 60.71%) were more likely to be male, had higher systolic and diastolic blood pressure, higher body mass index, lower high-density lipoprotein cholesterol and increased proportion of presence of atrial septal aneurysm. Using random forest survival (RFS) analysis, eight top ranking features were selected and used for prediction model construction. As a result, the RFS model outperformed the traditional Cox regression model (C-index: 0.87 vs. 0.54). CONCLUSIONS: There were 2 main clusters in post-closure PFO patients. Traditional cardiovascular profiles remain top ranking predictors for future recurrence of stroke or TIA. However, whether maximizing the management of these factors would provide extra benefits warrants further investigations.

Clinical features and survival in Takayasu's arteritis-associated pulmonary hypertension: a nationwide study.

AIMS: This study aimed to assess the clinical characteristics and long-term survival outcome in patients with Takayasu's arteritis-associated pulmonary hypertension (TA-PH). METHODS AND RESULTS: We conducted a nationally representative cohort study of TA-PH using data from the National Rare Diseases Registry System of China. Patients with pulmonary artery involvement who fulfilled the diagnostic criteria of Takayasu's arteritis and pulmonary hypertension were included. The primary outcome was the time from diagnosis of TA-PH to the occurrence of all-cause death. Between January 2007 and January 2019, a total of 140 patients were included, with a mean age of 41.4 years at diagnosis, and a female predominance (81%). Patients with TA-PH had severely haemodynamic and functional impairments at diagnosis. Significant improvements have been found in N-terminal pro-B-type natriuretic peptide (NT-proBNP) and haemodynamic profiles in patients with TA-PH receiving drugs approved for pulmonary arterial hypertension. The overall 1-, 3-, and 5-year survival rates in TA-PH were 94.0%, 83.2%, and 77.2%, respectively. Predictors associated with an increased risk of all-cause death were syncope [adjusted hazard ratio (HR) 5.38 (95% confidence interval 1.77-16.34), P = 0.003], NT-proBNP level [adjusted HR 1.04 (1.03-1.06), P < 0.001], and mean right atrial pressure [adjusted HR 1.07 (1.01-1.13), P = 0.015]. CONCLUSION: Patients with TA-PH were predominantly female and had severely compromised haemodynamics. More than 80% of patients in our cohort survived for at least 3 years. Medical treatment was based on investigators' personal opinions, and no clear risk-to-benefit ratio can be derived from the presented data.

Cardiovascular magnetic resonance-assessed fast global longitudinal strain parameters add diagnostic and prognostic insights in right ventricular volume and pressure loading disease conditions.

BACKGROUND: Parameters of myocardial deformation may provide improved insights into right ventricular (RV) dysfunction. We quantified RV longitudinal myocardial function using a fast, semi-automated method and investigated its diagnostic and prognostic values in patients with repaired tetralogy of Fallot (rTOF) and pulmonary arterial hypertension (PAH), who respectively exemplify patients with RV volume and pressure overload conditions. METHODS: The study enrolled 150 patients (rTOF, n = 75; PAH, n = 75) and 75 healthy controls. RV parameters of interest were fast global longitudinal strain (GLS) and strain rates during systole (GLSRs), early diastole (GLSRe) and late diastole (GLSRa), obtained by tracking the distance from the medial and lateral tricuspid valve insertions to the RV epicardial apex on cine cardiovascular magnetic resonance (CMR). RESULTS: The RV fast GLS exhibited good agreement with strain values obtained by conventional feature tracking approach (bias - 4.9%, error limits (± 2·standard deviation) ± 4.3%) with fast GLS achieving greater reproducibility and requiring reduced analysis time. Mean RV fast GLS was reduced in PAH and rTOF groups compared to healthy controls (PAH < rTOF < healthy controls: 15.1 ± 4.9 < 19.3 ± 2.4 < 24.4 ± 3.0%, all P < 0.001 in pairwise comparisons). In rTOF patients, RV fast GLS was significantly associated with metabolic equivalents, peak oxygen consumption (PVO2) and percentage of predicted PVO2 achieved during cardiopulmonary exercise testing. Lower RV fast GLS was associated with subnormal exercise capacity in rTOF (area under the curve (AUC) = 0.822, sensitivity = 72%, specificity = 91%, cut-off = 19.3%). In PAH patients, reduced RV fast GLS was associated with RV decompensated hemodynamics (AUC = 0.717, sensitivity = 75%, specificity = 58%, cut-off = 14.6%) and higher risk of clinical worsening (AUC = 0.808, sensitivity = 79%, specificity = 70 %, cut-off = 16.0%). CONCLUSIONS: Quantitative RV fast strain and strain rate parameters assessed from CMR identify abnormalities of RV function in rTOF and PAH and are predictive of exercise capacity, RV decompensation and clinical risks in these patients. Trial registry Clinicaltrials.gov: NCT03217240.

Characteristics and long-term survival of patients with chronic thromboembolic pulmonary hypertension in China.

BACKGROUND AND OBJECTIVE: The purpose of this study was to report the characteristics and long-term survival of patients with CTEPH treated in three distinct ways: PEA, BPA and medical therapy. METHODS: Patients diagnosed with CTEPH were included in the registry that was set up in 18 centres from August 2009 to July 2018. The characteristics and survival of patients with CTEPH receiving the different treatments were reported. Prognostic factors were evaluated by Cox regression model. RESULTS: A total of 593 patients with CTEPH were included. Eighty-one patients were treated with PEA, 61 with BPA and 451 with drugs. The estimated survival rates at 1, 3, 5 and 8 years were, respectively, 95.2%, 84.6%, 73.4% and 66.6% in all patients; 92.6%, 89.6%, 87.5% and 80.2% in surgical patients; and 95.4%, 88.3%, 71.0% and 64.1% in medically treated patients. The estimated survival rates at 1, 3, 5 and 7 years in patients treated with BPA were 96.7%, 88.1%, 70.0% and 70.0%, respectively. For all patients, PEA was an independent predictor of survival. Other independent risk factors were CHD, cardiac index, PVR, big endothelin-1, APE and 6MWD. CONCLUSION: This is the first multicentre prospective registry reporting baseline characteristics and estimated survival of patients with CTEPH in China. The long-term survival rates are similar to those of patients in the international and Spanish registries. PEA is an independent predictor of survival.

Risk prediction in medically treated chronic thromboembolic pulmonary hypertension.

BACKGROUND: At present, there is no generally accepted comprehensive prognostic risk prediction model for medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients. METHODS: Consecutive medically treated CTEPH patients were enrolled in a national multicenter prospective registry study from August 2009 to July 2018. A multivariable Cox proportional hazards model was utilized to derive the prognostic model, and a simplified risk score was created thereafter. Model performance was evaluated in terms of discrimination and calibration, and compared to the Swedish/COMPERA risk stratification method. Internal and external validation were conducted to validate the model performance. RESULTS: A total of 432 patients were enrolled. During a median follow-up time of 38.73 months (IQR: 20.79, 66.10), 94 patients (21.8%) died. The 1-, 3-, and 5-year survival estimates were 95.5%, 83.7%, and 70.9%, respectively. The final model included the following variables: the Swedish/COMPERA risk stratum (low-, intermediate- or high-risk stratum), pulmonary vascular resistance (PVR, ≤ or > 1600 dyn·s/cm5), total bilirubin (TBIL, ≤ or > 38 µmol/L) and chronic kidney disease (CKD, no or yes). Compared with the Swedish/COMPERA risk stratification method alone, both the derived model [C-index: 0.715; net reclassification improvement (NRI): 0.300; integrated discriminatory index (IDI): 0.095] and the risk score (C-index: 0.713; NRI: 0.300; IDI: 0.093) showed improved discriminatory power. The performance was validated in a validation cohort of 84 patients (C-index = 0.707 for the model and 0.721 for the risk score). CONCLUSIONS: A novel risk stratification strategy can serve as a useful tool for determining prognosis and guide management for medically treated CTEPH patients. TRIAL REGISTRATION: ClinicalTrials.gov (Identifier: NCT01417338).

[Effect of calorie-enriched formula on postoperative catch-up growth in infants with cyanotic congenital heart disease: a propective randomized controlled study].

OBJECTIVE: To study the effect of calorie-enriched formula on postoperative catch-up growth in infants with cyanotic congenital heart disease (CHD). METHODS: A total of 100 infants with cyanotic CHD who underwent surgical operation from January to December, 2017, were randomly divided into a high-calorie group (receiving calorie-enriched formula after surgery) and a conventional group (receiving standard formula after surgery), with 50 infants in each group. All infants were followed up for 6 months. The observation indices included body height, body weight, prealbumin, and N-terminal pro-brain natriuretic peptide before surgery, at the time of ventilator weaning and extubation after surgery, and at 1, 3, and 6 months after surgery. Height-for-age Z-score (HAZ), weight-for-age Z-score (WAZ), and weight-for-height Z-score (WHZ) were also assessed. Adverse reactions were recorded for both groups. RESULTS: There were 25 cases (50%) and 21 cases (42%) of malnutrition in the high-calorie group and the conventional group respectively before surgery (P > 0.05). The nutritional status of the two groups improved 6 months after surgery (P < 0.05). At 6 months after surgery, compared with the conventional group, the high-calorie group had a lower proportion of infants with malnutrition (18% vs 36%, P < 0.05) and also a lower proportation of infants with a WAZ score of < -2 (P < 0.05). The infants with malnutrion in the high-calorie group had higher HAZ, WAZ, and WHZ than those in the conventional group (P < 0.05). No gastrointestinal intolerance was observed in both groups during hospitalization. CONCLUSIONS: Compared with the standard formula, calorie-enriched formula can better help with postoperative catch-up growth in infants with cyanotic CHD.

Efficacy and safety of ambrisentan in Chinese patients with connective tissue disease-pulmonary arterial hypertension: a post-hoc analysis.

BACKGROUND: The efficacy and safety of ambrisentan has been previously evaluated in Chinese patients with pulmonary arterial hypertension (PAH). This post-hoc analysis assessed the efficacy and safety of ambrisentan in a subgroup of connective tissue disease (CTD) patients with PAH. METHODS: In this open-label, single-arm study, patients received ambrisentan 5 mg once daily for 12 weeks, followed by 12-week dose titration period (dose up to 10 mg). Efficacy endpoints included change from baseline in exercise capacity (measured by 6-min walk test [6MWT]), N-terminal pro B type natriuretic peptide (NT-proBNP) plasma levels, WHO Functional Class (FC) and Borg Dyspnoea Index (BDI) scores from baseline to weeks 12 and 24. Safety endpoints included time to clinical worsening and incidence of adverse events (AEs). RESULTS: In total, 71 Chinese patients with CTD-PAH were included in this analysis. Ambrisentan treatment significantly improved exercise capacity (6MWT) from baseline (mean: 366.4 m) to week 12 (63.8 m, p < 0.001) and week 24 (73.2 m, p < 0.001). A significant reduction in NT-proBNP levels was observed from baseline (mean: 1837.5 ng/L) to week 12 (- 1156.8 ng/L, p < 0.001) and week 24 (- 1095.5 ng/L, p < 0.001). BDI scores decreased significantly at week 12 (- 0.6, p < 0.001) and week 24 (- 0.4, p = 0.002) from baseline (mean: 2.7). The WHO FC improved in 29 (40.8%) and 34 (47.9%) patients at weeks 12 and 24, respectively. Adverse events were reported in 52 (73.2%) patients. One patient (1.4%) experienced clinical worsening at week 24. CONCLUSION: Ambrisentan showed significant improvement in exercise capacity and no clinical worsening in the majority of Chinese patients with CTD-PAH in the 24-week treatment period. The AEs observed in the CTD-PAH subgroup were consistent with the known safety profile of ambrisentan in the overall Chinese PAH population. TRIAL REGISTRATION: ClinicalTrial.gov Identifier, https://clinicaltrials.gov/, NCT01808313 Registration date (first time): February 28, 2013.

Where the congenital heart disease meets the pulmonary arterial hypertension, FLNA matters: a case report and literature review.

BACKGROUND: Pediatric patients with genetic disorders have a higher incidence of pulmonary arterial hypertension (PAH) regardless of their heart defects. Filamin A (FLNA) mutation is recently recognized to be associated with pediatric pulmonary disorders, however, the clinical courses of PAH related to the mutation were reported in limited cases. Here, we presented a case and pooled data for better understanding of the correlation between FLNA mutation and pediatric PAH. CASE PRESENTATION: The patient was a 8-month-old female with repeated episodes of pneumonia. Physical examination revealed cleft lip, cleft palate and developmental retardation. Imaging examination showed a small atrial septal defect (ASD), central pulmonary artery enlargement, left upper lobe of lung atelectasis, and pulmonary infiltration. Genetic test showed she carried a de novo pathogenic variant of FLNA gene (c.5417-1G > A, p.-). Oral medications didn't slow the progression of PAH in the patient, and she died two years later. CONCLUSIONS: FLNA mutation causes rare but progressive PAH in addition to a wide spectrum of congenital heart disease and other comorbidities in pediatric patients. We highly recommend genetic testing for pediatric patients when suspected with PAH. Given the high mortality in this group, lung transplantation may offer a better outcome.

Long-Term Follow-Up of Pediatric Patients After Congenital Coronary Artery Fistula Closure.

The surgical closure of congenital coronary artery fistulas (CAF) is associated with excellent immediate outcomes. Few studies have investigated the long-term prognosis in patients who have undergone surgery for the closure of CAF or differentiated among types of CAF or types of surgical procedures. In this study, we performed clinical examinations and computed tomography angiography (CTA) to characterize outcomes after CAF closure in pediatric patients. The medical records of 79 pediatric patients who underwent surgical closure of CAF were retrospectively reviewed. The median age of the patients included in the study at the time of surgery was 3.4 years (range 0.2 to 15.3 years). The patients had been followed up for 11 years (range 1 to 17 years) with electrocardiography, echocardiography, and coronary CTA. There were 67 medium-to-large CAF and 12 small CAF. Twenty-six (32.9%) CAF arose from the branch coronary artery (proximal type); the others arose from the parent coronary artery (distal type). The surgical procedure included endocardial closure in 16 cases, epicardial distal ligation in 51 cases, epicardial proximal and distal ligation in 12 cases. There was no instance of perioperative death among the cases included in the study. Twenty-eight patients were treated with antiplatelet medication postoperatively. No patient required re-operation during the follow-up period. Coronary thrombi were detected in 27 patients (34.2%). There was no instance of myocardial ischemia related to thrombosis. Among the patients with thrombosis, 26 had medium-to-large CAF (96.3%), and 23 had distal-type CAF (85.2%). Average age at surgery was higher among the patients with thrombosis than among the patients without thrombosis (7.4 years vs. 3.3 years, t = 5.509, P = 0.000). Among the patients with distal-type CAF, thrombosis was more common among the patients treated with ligation than treated with endocardial closure (41.5% vs. 16.7%, χ2 = 3.742, P = 0.043). There was no difference in risk for thrombosis between the patients who did vs. did not receive antiplatelet therapy (P = 0.436). The most common complication after CAF closure was thrombosis. Increased risk for thrombosis was associated with large fistulae, distal-type CAF, and older age at presentation. Antiplatelet treatment did not appear to decrease the risk of thrombosis. Among patients with distal-type CAF, risk for thrombosis was lower among patients treated with endocardial closure, compared with patients treated with epicardial ligation.

Soluble ST2 for Prediction of Clinical Outcomes in Patients with ST-Segment Elevation Myocardial Infarction Receiving Primary PCI.

Soluble suppression of tumorigenicity 2 (sST2), a biomarker representing myocardial fibrosis and inflammation, has been applied in risk stratification of patients with myocardial infarction (MI). However, whether primary PCI (PPCI) will eliminate the predictive value of sST2 in STEMI patients has not been well studied. Here, we conducted a prospective clinical trial to evaluate the correlation between sST2 and prognosis in STEMI patients undergoing PPCI. sST2 levels were measured in 295 STEMI patients (60.2 ± 10.8 years) at admission using a high sensitivity assay. Baseline sST2 levels were significantly associated with heart function, biomarkers of inflammation, and myocardial injury. During a 12-month follow-up, 19 patients had major adverse cardiovascular events (MACEs). Greater sST2 was continuously associated with a higher risk of incident MACEs. Such association remained even after adjusting for other risk factors in a multivariate Cox analysis. A baseline sST2 level in the highest quartile (≥ 58.7 ng/mL) was independently associated with mortality (HR: 5.01, 95%CI: 1.02-16.30, P = 0.048). More incident heart failure was seen in the group with greater sST2, however, the association was not significant after adjustment. Therefore, baseline sST2 may be useful to predict MACEs, especially mortality, in STEMI patients receiving PPCI.

An Infectious Pseudoaneurysm Caused by Ventricular Septal Defect Occluder in Patent Ductus Arteriosus Closure in a Two-Year-Old Child.

We present a case of an infectious pseudoaneurysm after patent ductus arteriosus (PDA) closure with a ventricular septal defect (VSD) occluder in a two-year-old child. The aneurysm grew rapidly but was successfully removed in time and the patient survived. To our knowledge, this is the first report of an infectious pseudoaneurysm caused by VSD occluder in PDA closure.

Late-Onset Postoperative Junctional Ectopic Tachycardia in Nonsurgical Procedures.

Postoperative junctional ectopic tachycardia (JET) is a narrow complex tachycardia and most frequently occurs during and after surgical repair of certain types of congenital heart defects. Postoperative junctional ectopic tachycardia may produce unfavorable hemodynamics that prolongs stays in the cardiac intensive care unit and hospital, prolongs time on a ventilator, and occasionally requires the use of extracorporeal membrane oxygenation (ECMO) as rescue therapy. The present report describes a rare case of late-onset postoperative junctional ectopic tachycardia, which occurred 13 days after the deployment of a perimembranous ventricular septal defect (PmVSD) occluder in a 17-year-old female teenager. To the best of our knowledge, late-onset postoperative junctional ectopic tachycardia has not previously been reported as a complication in nonsurgical procedures. In this case, the junctional ectopic tachycardia remained resistant to medicines and the haemodynamic imbalance caused a serious life-threatening situation in the patient. The occluder was removed by an emergent thoracotomy; then, the patient was successfully cured by being supported with extracorporeal membrane oxygenation. The findings suggest that during follow-up management, the physician should pay attention postoperatively to junctional ectopic tachycardia even after discharge from the hospital.

[Clinical characteristics of 195 Chinese patients with WHO Class I pulmonary hypertension].

OBJECTIVE: To analyze the clinical characteristics of WHO Class I pulmonary hypertension (PAH) in central China. METHODS: Data was collected as a part of prospective registry of PAH through Jan. 2009 to Oct. 2013 in Wuhan Asia Heart Hospital. A total of 195 patients were recruited including 144 cases with congenital heart disease with pulmonary hypertension (CHD-PAH) and 51 cases with idiopathic pulmonary hypertension (IPAH). RESULTS: The age of all patients ranged from 1 to 68 years (mean (27.5 ± 13.2) years), 129 cases were female (66.2%). WHO Class I PAH accounted for 91.1%, CHD-PAH 67.3%, IPAH 23.8%, and other 8.9%.WHO function class III/IV in newly diagnosed PAH accounted for 32.3%, the mean 6MWD was (397 ± 74) m. For patients with IPAH, the median time period between onset of symptoms and diagnosis by right heart catheterization was 38 months. The mean pulmonary pressure, pulmonary vascular resistance index, cardiac index of patients with IPAH and CHD-PAH were measured by the right heart catheterization and there was no difference between the two groups. Acute pulmonary vasodilator testing was negative in all patients in this cohort. Cardiac function was improved in the 121 cases who received the targeted drug treatment and 1 patient died out of these 121 patients while 5 cases died out of patients receiving conventional therapy. CONCLUSION: In Central China, Class I pulmonary hypertension is the most predominant type of PAH, the cardiac function and hemodynamic indexes of these patients were significantly impaired at the time of first PAH diagnosis. Most of the patients accepted targeted drug treatment of pulmonary hypertension, but the drug dose used for the targeted drug treatment is not effective enough in these patients.

[Efficacy of endothelin receptor antagonist bosentan on the long-term prognosis in patients after Fontan operation].

OBJECTIVE: To investigate the long-term effect of bosentan on outcome in patients after Fontan operation. METHODS: Patients after Fontan surgery were randomly divided into bosentan group (B, n = 16) and control group (C, n = 23). Bosentan was applied within 7 days after Fontan surgery as follows: at the first month, 7.8125 mg Bid for patients with body weight ≤ 10 kg; 15.625 mg Bid for patients with body weight between 10-20 kg; 31.25 mg Bid for patients with body weight 20-30 kg and 62.5 mg Bid for patients with body weight > 30 kg. At the second month, the bosentan dose was doubled and Bosentan therapy was continued for more than 1 year. Group C didn't take drugs affecting pulmonary artery pressure. All patients were followed up for 2 years and incidence of mortality, protein losing enteropathy, pulmonary arteriovenous fistulae, 6-minute walk test, heart function were compared between the two groups. RESULTS: After 2 years, mortality tended to be lower in group B compared to group C [6.25% (1/16) vs. 21.8% (5/23), P > 0.05]. Incidence of pulmonary arteriovenous fistulae and protein losing enteropathy were significantly lower in group B than in group C (6.25% vs. 34.78%, P = 0.01;6.25% vs. 39.13%, P = 0.02, respectively) . The results of 6-minute walk test[ (485 ± 44) m vs. (302 ± 183) m] and heart function in group B (3 NYHA III/IV patients in group B vs. 14 NYHA III/IV patients in group C, all P < 0.05) were all better than group C. The concentrations of vasoactive factors such as brain natriuretic peptide (BNP, 279.07 ± 128.17 vs. 457.67 ± 221.30), endothelin (ET, 3.30 ± 0.61 vs. 4.98 ± 1.24) and thromboxane (TXA2, 97.2 ± 24.0 vs. 163.22 ± 24.4) were also significantly lower in group B than in group C (all P < 0.05). Prostacyclin (PGI2) level and incidence of arrhythmias were similar between the two groups. There was no thrombotic event in both groups during follow up. CONCLUSION: Bosentan trerapy in patients post Fontan operation could reduce the incidence of pulmonary arteriovenous fistulae and protein losing enteropathy and improve heart function.

Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension.

The etiology of pulmonary arterial hypertension (PAH) is complex, especially the investigation of rare pathogeny is difficult. Congenital portosystemic venous shunt (CPSS) is a rare congenital anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt, and drains directly into the inferior vena cava (IVC) (Howard and Davenport in J Pediatr Surg 32:494-497, 1997).CPSS is an uncommon cause of PAH (Christiane et al. in J Pediatr Gastroenterol Nutr 56:675-681, 2013), and often covered by other pathogenic factors. The clinical manifestations of CPSS-associated PAH are not specific, thus making it difficult to distinguish from PAH caused by other pathogenetic factors based on clinical presentations alone. This is a retrospective analysis of data from six patients with CPSS at a single center. Of these, five were diagnosed as PAH: four were also associated with other predisposing factors of pulmonary hypertension (PH). All patients had high serum bile concentration and high cardiac output. The aim of this retrospective study was to investigate the clinical recognition of PAH secondary to CPSS. The concentration of serum bile acid and cardiac output can be used as two important non-invasive indicators in clinical practice. Thus far, few studies have reported the clinical outcomes of CPSS-associated PAH specifically (Anna et al. in Hepatology 71:658-669, 2020;Franchi-Abella et al. in J Pediatr Gastroenterol Nutr 51:322-330, 2010;Uike et al. in Pediatr Pulmonol 53:505-511, 2018;). In the current study, such patients carried a poor prognosis if left untreated, or treated with pulmonary vasodilators alone.

Effectiveness and feasibility of transcatheter aortic valve replacement in treating combined aortic and mitral regurgitation: a retrospective observational study.

Background: Transcatheter aortic valve replacement (TAVR) is emerging as a less invasive procedure for aortic valve replacement. However, its effectiveness and feasibility in treating combined valvular disease remain controversial. In this study, we explored the clinical effectiveness and safety of TAVR in the treatment of combined aortic and mitral regurgitation. Methods: The basic clinical characteristics and 1-month follow-up of 11 patients with combined aortic and mitral regurgitation who were treated with TAVR at center of structural heart disease, Zhongnan Hospital of Wuhan University from December 2021 to November 2022 were retrospectively analyzed. Echocardiographic parameters of aortic and mitral valve, complications, and all-cause mortality were compared pre- and post-TAVR. Results: Retrievable self-expanding valve protheses were used in all patients, and the protheses were implanted via the transfemoral route in 8 patients and via the transapical route in 3 patients. There were 9 male and 2 female patients with an average age of 74.7±2.7 years. The mean Society of Thoracic Surgeons score was 8.5±1.2. Among the patients, 1 required retroperitoneal sarcoma semi-elective surgery, and 3 of the 5 patients with atrial fibrillation had their rhythm converted to sinus rhythm after operation. No perioperative deaths were recorded. Two patients underwent permanent pacemaker implantations due to high-grade atrioventricular blocks after TAVR. Moderate/severe mitral regurgitation (MR) were mostly secondary to aortic regurgitation (AR) as no rupture of the subvalvular tendon cords or rheumatic changes being recognized during echocardiography before operation. The mean left ventricular end-diastolic diameter (65.5±10.7 vs. 58.6±8.8 mm, P<0.001) and mitral annular diameter (36.7±5.4 vs. 31.5±2.8 mm, P<0.001) was significantly reduced after operation. MR was improved as the ratio of the regurgitant jet area to the left atrial area decreased significantly after operation (24.7%±11.5% vs. 42.4%±6.8% before operation, P<0.001). During the 1-month follow-up, the mean left ventricular ejection fraction was significantly improved (50.0%±9.4% vs. 44.6%±9.3% at admission, P=0.022). Conclusions: TAVR is effective and feasible for high-risk patients with combined aortic and mitral regurgitation.