ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.
Objective To construct a risk prediction score model for serious adverse event (SAE) after cardiac catheterization in patients with adult congenital heart disease (ACHD) and pulmonary hypertension (PH) and verify its predictive effect. Methods The patients with PH who underwent cardiac catheterization in Wuhan Asian Heart Hospital Affiliated to Wuhan University of Science and Technology from January 2018 to January 2022 were retrospectively collected. The patients were randomly divided into a model group and a validation group according to the order of admission. The model group was divided into a SAE group and a non-SAE group according to whether SAE occurred after the catheterization. The data of the two groups were compared, and the risk prediction score model was established according to the results of multivariate logistic regression analysis. The discrimination and calibration of the model were evaluated using the area under the receiver operating characteristic (ROC) curve and the Hosmer-Lemeshow test, respectively. Results A total of 758 patients were enrolled, including 240 (31.7%) males and 518 (68.3%) females, with a mean age of 43.1 (18.0-81.0) years. There were 530 patients in the model group (47 patients in the SAE group and 483 patients in the non-SAE group) and 228 patients in the validation group. Univariate analysis showed statistical differences in age, smoking history, valvular disease history, heart failure history, N-terminal pro-B-type natriuretic peptide, and other factors between the SAE and non-SAE groups (P<0.05). Multivariate analysis showed that age≥50 years, history of heart failure, moderate to severe congenital heart disease, moderate to severe PH, cardiac catheterization and treatment, surgical general anesthesia, and N-terminal pro-B-type natriuretic peptide≥126.65 pg/mL were risk factors for SAE after cardiac catheterization for ACHD-PH patients (P<0.05). The risk prediction score model had a total score of 0-139 points and patients who had a score>50 points were high-risk patients. Model validation results showed an area under the ROC curve of 0.937 (95%CI 0.897-0.976). Hosmer-Lemeshow goodness-of-fit test: χ2=3.847, P=0.797. Conclusion Age≥50 years, history of heart failure, moderate to severe congenital heart disease, moderate to severe PH, cardiac catheterization and treatment, general anesthesia for surgery, and N-terminal pro-B-type natriuretic peptide≥126.65 pg/mL were risk factors for SAE after cardiac catheterization for ACHD-PH patients. The risk prediction model based on these factors has a high predictive value and can be applied to the risk assessment of SAE after interventional therapy in ACHD-PH patients to help clinicians perform early intervention.
Objective To analyze the curative effect of nitric oxide (NO) and bosentan on treatment of the interruption of aortic arch (IAA) with ventricular septal defect (VSD) and serious pulmonary hypertension (SPH). Methods Thirty-two children with IAA and VSD combined SPH from January 2015 to May 2017 confirmed by cardiac CT and ultrasound in Children’s Hospital of Hebei Province were enrolled including 17 males and 15 females, aged 1.10-4.30 months (mean, 2.71±0.98 months) and weighing 3.33-6.10 kg (mean, 4.57±0.88 kg). The 32 children were randomly divided into two groups (n=16 in each), a NO group and a bosentan group. All the patients underwent interruption of aortic arch and ventricular septal defect repair. When patients returned to cardiosurgery intensive care unit (CSICU) half an hour later, patients in the NO group inhaled NO 20 ppm for 36 h and those in the bosentan group were given bosentan by nasogastric feeding 15 mg, twice a day. The cardic index, pulmonary/systemic pressure ratio, oxygenation index at 3 h, 6 h, 12 h, 24 h, 36 h after surgery were evaluated, and the differences between the two groups were compared. Results The pulmonary/systemic pressure ratio in the two groups increased at first and then decreased, while oxygenation index in the two groups decreased at first and then increased, and the differences in the same groups at the adjacent time points were statistically significant (P<0.05). The cardiac index in the two groups decreased at first and then increased, the differences in the same groups at the adjacent time points were statistically significant, except for 6 h and 12 h after surgery in the bosentan group (P>0.05). At postoperative 6 h, 12 h, the oxygenation index in the NO group was significantly higher than that in the bosentan group, and the pulmonary/systemic pressure ratio in the NO group was less than that in the bosentan group (P<0.01). The cardiac index in the NO group was higher than that of the bosentan group after 6 h, 12 h, 24 h of operation, which were statistically significant (P<0.05), and the cardic index of children in the NO group was greatly higher than that in the bosentan group after 12 h of surgery (P<0.01); at the same time point, the corresponding indexes were not statistically significant between the two groups (P>0.05). Conclusion NO inhalation in the treatment of IAA with VSD and SPH in children with early postoperative SPH is better than the bosentan, but in the late postoperative period, the effect is similar.
Objective To investigate the relation of spinal ventricular septal angle (SVSA) measured by computer tomographic pulmonary angiography (CTPA) and pulmonary vascular resistance (PVR) measured by right heart catheterization in patients with chronic thromboembolic pulmonary hypertension (CTEPH) .Methods Eighty-nine patients with CTEPH (male 57, female 32; 53.08 ±12.43 years) were recruited as a CTEPH group, and 89 patients without pulmonary artery hypertension and pulmonary embolismwere recruited as a control group. The CTEPH patients received CTPA before right-heart catheterization and pulmonary angiography. SVSA and pulmonary artery obstruction indexes including Qanadli Index and Mastora index were evaluated by two radiologists.Results SVSA was 65.13°±12.26°and 39.69°±5.84°in the CTEPH group and the control group respectively, with significant difference between two groups ( t =14.479, P = 0.000) . Qanadli index of the CTEPH patients was( 42.50 ±17.67) % , which had no correlation with SVSA ( r= 0.094, P = 0.552) . Mastora index was ( 30.02 ±15.53) % , which also had no correlation with SVSA ( r=0.025, P =0.873) . SVSA had a moderate positive correlation with PVR ( r =0.529, P =0.000) and a weak positive correlation with right atriumpressure ( r =0.270, P =0.010) . Area under ROC was 0.764 and sensitivity, specificity for PVR≥1000 dyne· s· cm- 5 was 0.714 and 0.778 respectively when SVSA≥67.55°. Conclusion SVSA measured by CTPA can be used as a better predictor for evaluating PVR in CTEPH patients.
Objective To systematically evaluate the impact of pulmonary hypertension (PH) on the prognosis of patients with severe aortic stenosis (AS) undergoing transcatheter aortic valve replacement (TAVR). Methods A computerized search was conducted in CNKI, Wanfang Data, VIP, CBM, PubMed, The Cochrane Library, EMbase, and Web of Science databases from inception to June 2023 for cohort studies on the prognostic impact of PH in severe AS patients undergoing TAVR. Two researchers independently screened the literature, extracted data, and assessed the quality of included studies. Stata 17.0 software was used for meta-analysis. Results A total of 16 cohort studies were included, all with Newcastle-Ottawa Scale scores≥7. Meta-analysis results showed that, compared with AS patients without PH, those with PH had significantly higher 1-year all-cause mortality after TAVR [OR=2.10, 95%CI (1.60, 2.75), P<0.01], 30-day all-cause mortality [OR=2.09, 95%CI (1.54, 2.83), P<0.01], and cardiovascular mortality [OR=1.49, 95%CI (1.18, 1.90), P<0.01]. The differences between the two groups in major bleeding events, stroke, myocardial infarction, pacemaker implantation, and postoperative renal failure were not statistically significant. For outcome indicators with significant heterogeneity, subgroup analyses were performed based on PH measurement methods, diagnostic criteria, and different types of PH. The results showed that most subgroup combined results were consistent with the overall findings and that heterogeneity was significantly reduced. Conclusion PH significantly increases the 30-day all-cause mortality, 1-year all-cause mortality, and cardiovascular mortality in patients with severe AS undergoing TAVR.
ObjectiveTo explore the hemodynamic effects of inhaled nitric oxide (iNO) on postoperative hemodynamic in patients with cyanotic congenital heart disease (CHD) combined with decreased pulmonary blood flow.MethodsFrom 2014 to 2018, there were 1 764 patients who received corrective repair of cyanotic CHD with decreased pulmonary blood flow in the Department of Pediatric Cardiac Surgery of Fuwai Hospital. We included 61 patients with the ratio of right ventricular systolic pressure to systolic blood pressure (SBP) ≥75% after weaning from cardiopulmonary bypass. There were 41 males and 20 females, with the age of 20.5 (9.0, 39.0) months and weight of 12.5±7.8 kg. The patients were divided into two groups: a conventional group (33 patients, conventional therapy only) and a combined therapy group (28 patients, iNO combined with conventional therapy). The hemodynamics during the first 24 hours after iNO therapy and the in-hospital outcomes of the two groups were investigated and compared.ResultsThere was no statistical difference between the two groups in demographic characteristics and surgical parameters (P>0.05). The hemodynamic effects of iNO within 24 hours included the decrease in the vasoactive inotropic score (VIS, 21.6±6.6 vs. 17.3±7.2, P=0.020) along with the increase in blood pressure (SBP: 73.7±9.7 mm Hg vs. 90.8±9.1 mm Hg, P<0.001) , the decrease in central venous pressure (10.0±3.1 mm Hg vs. 7.9±2.1 mm Hg, P=0.020), the decrease in lactate (2.2±1.7 mmol/L vs. 1.2±0.5 mmol/L, P<0.001) and increase in urine output [2.8±1.7 mL/(kg·h) vs. 4.9±2.2 mL/(kg·h), P<0.001]. The decrease of VIS at 24 h after the surgery in the conventional therapy group was not statistically significant (22.1±7.9 vs. 20.0±8.5, P=0.232). Besides, we discovered that the need for renal replacement therapy (RRT) was less in the combined therapy group than that in the conventional therapy group, especially in the moderate complicated surgery [risk adjustment in congenital heart surgery (RACHS-1) ≤3] subgroup (9.5% vs. 40.7%, P=0.016).ConclusionIn pediatric patients after corrective repair of cyanotic and pulmonary blood follow decreased CHD with increased pulmonary vascular resistance, iNO combined with conventional therapy can improve the hemodynamics effectively. Compared with the conventional therapy, the combined therapy with iNO can decrease the VIS and the need for RRT, which is beneficial to the postoperative recovery of patients.
Congenital heart disease refers to the structural or functional abnormality of the macrovascular in the heart or thoracic cavity caused by the failure of the formation of the heart and large blood vessels during the embryonic development or the abnormal closure of the heart or the closure of the channel after birth. In the past few years, a new and broader definition of structural heart disease has been gradually proposed. Structural heart disease narrowly refers to the pathological and physiological changes of the heart caused by abnormal anatomical structures in the heart, including congenital heart disease. A few decades ago, congenital heart disease was considered as a pediatric disease, because most patients with severe lesions rarely survive to adulthood. Due to recent advances in echocardiography, anesthesia, intensive care, percutaneous intervention, especially cardiac surgery in recent decades, the treatment and intervention strategies for congenital heart disease in children have been greatly improved, a fatal defect in childhood can now be successfully repaired or alleviated. Because of these successes, more than 90% of congenital heart disease patients are expected to survive to adulthood, which has led to emerge a new population: adult patients with congenital heart disease. Adult congenital heart disease patients are different from children. Pulmonary hypertension leads to right heart failure and eventually progresses to whole heart failure. The appearance of Eisenmenger syndrome leads to severe cyanosis and worsening of the disease. At present, the continuous development of mechanical assisted circulation support devices and heart or cardiopulmonary transplantation technology has increased the survival rate of end-stage adult congenital heart disease patients with heart failure. The high incidence of cardiovascular events in pregnant patients requires comprehensive multidisciplinary team care and early coordination planning for delivery, including early counseling for pregnancy-related risks, close monitoring of cardiac function and regular scan of fetal assessment. The prenatal and postpartum integrated diagnosis and treatment model and the development of intrauterine treatment technology reduce the incidence of congenital heart disease in adults from the source through fetal intervention. Other complications such as arrhythmia, infective endocarditis, cerebrovascular accidents, and other medical underlying metabolic diseases also challenge future diagnosis and treatment. The incidence and epidemiology of adult congenital heart disease, pulmonary hypertension and end-stage heart failure complications, as well as prenatal and postpartum integrated diagnosis and treatment and intrauterine treatment are summarized in this review.
ObjectiveTo explore the method and feasibility of establishing patent ductus arteriosus (PDA) model in Bama miniature pig by using autologous jugular vein, and to provide a large animal model for the development of PDA occluder and the study of pulmonary hypertension associated with congenital heart disease. MethodsFive male Bama miniature pigs weighing about 45 kg were selected to gain the PDA model of the autogenous jugular vein, which was fixed by glutaraldehyde and anastomosed between the ascending aorta and the main pulmonary artery. The patency of PDA was confirmed by echocardiography and angiocardiography immediately and one week after the operation. Two animals were selected to undergo transcatheter closure of PDA via femoral vein 1 week after the operation, and the rest were euthanized to obtain PDA and lung tissue for pathological examination. ResultsThe PDA model was successfully established in all five animals with a success rate of 100.0%. Immediately and 1 week after the operation, echocardiography and angiography showed that PDA blood flow was unobstructed, and hematoxylin-eosin staining showed that PDA endothelialization was good. One week after the operation, two animals were successfully treated with transcatheter femoral vein occlusion. The pathological examination of lung tissue showed thickening of the intima and muscular layer of pulmonary arterioles, thickening of pulmonary interstitium and infiltration of neutrophils. ConclusionIt is safe and feasible to establish a large animal model of PDA by using autogenous jugular vein anastomosis between the ascending aorta and the main pulmonary artery. The model can be used for the development of PDA interventional occlusive devices and the pathophysiological study of congenital heart disease-related pulmonary hypertension.
Abstract: Objective To analyze the results of preoperative evaluation of the operability of chronic thromboembolic pulmonary hypertension (CTEPH), and to find parameters to define the inconsistency between the operable lesions of CTEPH and pulmonary vascular resistance (PVR). Methods A total of 133 patients with CTEPH admitted into Anzhen Hospital were enrolled for preoperative assessment of operability from March 2002 to May 2010. There were 86 males and 47 females with an age of 49.10±22.70 years. The patients were divided into operable group (group A, n=82，age of 47.80±21.60 years) and inoperable group (group B, n=51, age of 50.30±23.40 years) according to the assessment suggestion. We evaluated the occluded pulmonary segment（OPS） of all the patients through pulmonary ventilation/perfusion scintigraphy， and measured the plasma aminoterminal Btype natriuretic peptide (NT-pro BNP) and PVR. Then the ratio of NT-pro BNP to OPS and PVR to OPS were calculated. [CM(159mm]Results Out of the 82 patients with CTEPH in group A, 81 were positive in the anesthesia test and were subject to pulmonary thromboendarterectomy(PTE). In the whole cohorts of operated patients, there was one early death due to persistent pulmonary hypertension and right heart failure. The specificity of the anesthesia test was 98.78%. Eighty patients (98.77%) were followed up in this group for a period of 1 to 95 months (42.70±28.40 months). During the followup, there was one late death due to pulmonary artery hypertension crisis. Among the 51 patients with CTEPH in group B, there were 32 patients (62.75%) with surgically inaccessible lesions, 13 patients (25.49%) with surgical accessible CTEPH concomitant with severe diseases, and 6 patients (11.76%) with inconsistency between the surgical accessible lesion and high PVR. The ratio of NT-pro BNP to OPS and PVR to OPS for the 81 positive patients in group A was in the range of 80-150 pg·ml-1/OPS and 50-100 dyn·s·cm-5/OPS, respectively. The ratio of NT-pro BNP to OPS (315.00±83.00 pg·ml-1/OPS vs. 115.60±40.50 pg·ml-1/OPS, P=0.000) and PVR to OPS (190.00±57.00 dyn·s·cm－5/OPS vs. 76.40±26.30 dyn·s·cm-5/OPS, P=0.000) for the 6 patients with incosistency between the surgical accessible lesion and high PVR in group B were significantly higher than that for the 81 positive patients in group A. Conclusion Surgically inaccessible CTEPH lesions, CTEPH concomitant with severe diseases, and inconsistency between surgical accessible lesion and high PVR are the three most frequent reasons for denying PTE procedure. The ratio of NTpro BNP to OPS and PVR to OPS may serve as the parameters to define the inconsistency between the surgical accessible lesion and high PVR. Anesthesia test before the PTE procedure may serve as the last evaluation method for the assessment of the operability of CTEPH.
Objective To explore current results after staged operations in patients with functional single ventricle anomalies and pulmonary hypertension. Methods We retrospectively analyzed the clinical data of 129 patients with functional single ventricle anomalies and pulmonary hypertension undergoing pulmonary artery banding in our hospital between April 2008 and December 2015. There were 81 males and 48 females. There were 71 patients with double outlet of right ventricle, 17 patients with tricuspid atresia, 7 patients with transposition of great arteries, 33 patients with uni-ventricular heart, and one patient with complete atrio-ventricular septal defect. The surgical results, transition to Glenn procedure and subsequent transition to Fontan procedure were analyzed. Results The 129 patients underwent 159 operations of pulmonary artery banding totally. Hospital mortality was 4.7% (6/129). Nine patients were lost to follow-up. Eighty-seven (67.4%) patients underwent the second-stage Glenn procedure, and 43 patients(33.3%) underwent third-stage Fontan procedure. Two patients died after Glenn and 3 patients died after Fontan separately. There were 32 patients who accompanied with coarctation, interruption of aortic arch, heterotaxy, total anomalous pulmonary venous connection or atrio-ventricular valve regurgitation. Fifteen (46.9%) patients succeeded in transition to Glenn, and 6 (18.8%) patients succeeded in transition to Fontan. Fourteen patients developed obstruction of left ventricular outlet tract or bulbo-ventricular foramen. Conclusion Early pulmonary artery banding is an acceptable strategy for patients with single ventricle anomalies and pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good. Accompanied complex anomalies are risk factors for lower ratio of transition to Glenn and Fontan procedure.