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.
ObjectiveTo determine the ability of cardiopulmonary exercise testing (CPET) to distinguish chronic thromboembolic pulmonary hypertension (CTEPH) from chronic thromboembolic disease (CTED). MethodsFifty patients diagnosed with CTED and fifty-eight patients with CTEPH in the the First Affiliated Hospital of Guangzhou Medical University from April 2019 to February 2022 were retrospectively included. The basic characteristics including age, gender, body mass index, symptom duration, and N-terminal prohormone of brain natriuretic peptide (NTpro-BNP), parameters of arterial blood gas analysis, right heart catheterization, echocardiography, pulmonary function, and CPET were all compared between patients with CTED and those with CTEPH. ResultsPatients with CTEPH displayed longer symptom duration, increased NTpro-BNP, decreased arterial partial pressure of oxygen, larger right atrial and ventricular diameters, and impaired worse resting pulmonary diffusing function compared with those with CTED (P<0.05). However, there was no statistically significant difference in the resting pulmonary ventilation function between the two groups (P>0.05). Among the CPET parameters of the CTEPH group, peak exercise oxygen uptake per kilogram, oxygen uptake at anaerobic threshold, oxygen pulse, oxygen uptake efficiency slope and oxygen saturation were all decreased, while the minute ventilation-carbon dioxide production at anaerobic threshold (VE/VCO2@AT) and VE/VCO2 slope were increased (P<0.05). However, there was no statistically significant difference in heart rate reserve and breathing reserve (P>0.05). Furthermore, VE/VCO2@AT showed high accuracy for distinguishing CTEPH from CTED (sensitivity, 0.825; specificity, 0.860; and the area under the receiver operating characteristic curve 0.897). ConclusionsPatients with CTEPH showed more significant decreased exercise endurance, diffusion dysfunction, and hypoxemia during exercise and insufficient increase in cardiac output compared with CTED patients. Therefore, it is feasible to apply CPET as a new objective examination to distinguish CTED from CTEPH.
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 observe the efficacy and safety of sildenafil in one case of PoPH patients. Methods A case of PoPH patient who was successfully treated with sildenafil was retrospectively analyzed，and literatures related sildenafil and PoPH were reviewed. Results A case of PoPH patient admitted with dyspnea on extetion was diagnosed with echocardiography，gastroscrope and other examinations. She was treated with sildenafil and responded well by decreased pulmonary arterial pressure，improved exercise tolerance and life quality，without obvious side effects. Literatures review suggested that PoPH is an under-recognized but life-threatening complication of cirrhosis or noncirrhotic portal hypertension with poor prognosis which exists in decompensated chronic liver diseases patients. The most classic symptoms of PoPH is progressive dyspnea on extertion. PoPH patients may benefit from sildenafil therapy with decreased pulmonary arterial pressure and improved life quality. Conclusions Theawareness of PoPH should be increased and Sildenafil may be effective and safe for PoPH patients. However，more evidences from clinical trials are needed.
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.
Objective To investigate the effect of low-flow inhaling NO for short time on postoperative cardiac and pulmonary functions in infants with congenital ventricular septal defect complicated with severe pulmonary hypertension. Methods Forty-five patients with congenital ventricular septal defect complicated with severe pulmonary hypertension from May 2014 to May 2016 in our hospital were enrolled. There were 19 males and 26 females, whose age ranged from 1 to 22 months (average age: 7.2±14.4 months) and weight ranged from 2.7 to 10.5 kg (average weight: 6.8±3.6 kg). The patients were randomly divided into three groups (n=15 in each): the blank group, the prior inhalation group and the posterior inhalation group. The blank group did not inhale NO, and the prior inhalation group inhalated NO for 10 min after tracheal and intubation. After the opening of the aorta, the posterior inhalation group inhaled NO for 10 min. The concentration of NO was 20 × 10–6. The pressure ratio of pulmonary circulation/systematic circulation, heart index and oxygenation index were calculated and the troponin value of the three groups was monitored 10 min after returning to intensive care unit (ICU) and postoperatively 1 h, 3 h and 24 h. Differences among above indicators between three groups were compared. Results The troponin value of the posterior inhalation group within 3 h increased most, followed by the blank group and the prior inhalation group. Postoperatively 1 h and 3 h, the troponin value of the prior inhalation group was significantly less than that of the blank group and posterior inhalation group (P<0.01) and the value on postoperative 24 h in each group was lower than that on postoperative 3 h. The cardiac index of prior inhalation group was higher than that of the blank group and the posterior inhalation group at each time point. Postoperatively 3 h and 24 has well as 10 min after returning to ICU, the cardiac index in prior inhalation group was significantly higher than that of the posterior inhalation group (P<0.05). The pressure ratio of pulmonary circulation/systematic circulation of posterior inhalation group increased more than that of blank group; the differences in two groups were significant between postoperative 3 h and 10 min after returning to ICU (P<0.01). There was no statistical significance in the pressure ratio on postoperative 24 h and 10 min after returning to ICU (P>0.05) in three groups. The index of oxygenation of the prior inhalation group was higher than that of the blank group and the posterior inhalation group and statistically different from that of posterior inhalation group (P<0.05). Conclusion Inhaling NO 10 min preoperatively can reduce the injury to the heart and lung function effectively, but the result is the opposite when inhalating NO 10 min after aorta opening.
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.
ObjectiveTo describe the effect of sequential pulmonary balloon angioplasty for patients with chronic thromboembolic pulmonary hypertension, who was accompanied with progressed pulmonary hypertension after pulmonary endarterectomy surgeries.MethodsFrom 2014 to December 2017, 7 patients were treated with a combination therapy of pulmonary endarterectomy and sequential pulmonary balloon angioplasty. There were 1 male and 6 females at age of 58 (43–59) years. A follow-up period of more than 1 year was accomplished. The result of right sided heart catheterization and ultrasonic cardiogram between and after the pulmonary endarterectomy or balloon angioplasty was collected.ResultsSeven patients were treated with a combination of pulmonary endarterectomy and sequential pulmonary balloon angioplasty, which included 1 patient of single pulmonary balloon angioplasty and 6 patients of multiple pulmonary balloon angioplasties. The balloon dilation times was 2 (2–6), and the number of segments during each single balloon dilatation was 3–5, compared with the first clinical results before the first balloon dilation, systolic pulmonary artery pressure [53 (47–75) mm Hg vs. 45 (40–54) mm Hg, P=0.042), mean pulmonaryartery pressure [38 (29–47) mm Hg vs. 29 (25–39) mm Hg, P=0.043], N terminal-B type natriuretic peptide [1 872 (1 598–2 898) pg/ml vs. 164 (72–334) pg/ml, P=0.018] improved significantly after the last balloon angioplasty. Heart function classification (NYHA) of all the 7 patients were recovered to Ⅰ-Ⅱclasses (P<0.05).ConclusionSequential pulmonary balloon angioplasty after pulmonary endarterectomy can further reduce the patient's right heart after load, improve the heart function for patients with progressed pulmonary hypertension after pulmonary endarterectomy surgeries.
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.
Objective To investigate the effects of simvastatin on the collagen synthesis of rat pulmonary arterial smooth muscle cells ( PASMCs ) induced by hypoxia. Methods Under hypoxic condition, rat PASMCs were cultured with different concentrations of simvastatin. Collagen synthesis of PASMCs with or without simvastatin were measured by 3H-proline incorporation assay. The mRNA expression of TGF-β1 and the contents of super oxide dismrtase ( SOD) ,malondialdehyde ( MDA) in mediumwere also measured. Results The incorporation data of 3H-TdR in the hypoxia group was significantly increased as compared with that in the control group ( P lt;0. 01) , and simvastatin significantly reduced the incorporation data of 3H-TdR induced by hypoxia. The expression of TGF-β1 mRNA in the hypoxia group was significantly increased as compared with that in the control group ( P lt; 0. 01 ) , and simvastatin could significantly inhibited hypoxia-induced expression of TGF-β1 mRNA in a dose-dependent manner. Compared with the hypoxia group, the expression of TGF-β1 mRNA decreased by 55% in simvastatin( 10 - 6mol /L) group ( P lt; 0. 01) , and by 70% ( P lt; 0. 01) in simvastatin ( 10 - 5mol /L) group. Compared with the control group, the activity of SOD was reduced and the contents of MDA were increased significantly in the hypoxia group. Simvastatin can increase the activity of SOD and reduced the content of MDA in a dose-dependent manner. Conclusions Simvastatin can decreases collagen synthesis of PASMCs. This effect might be explained that simvastatin can reduce lipid peroxide and expression of TGF-β1 mRNA.