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 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.
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.
Objective
To discuss the safety and validity of pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) patients with severe right heart failure (RHF).
Methods
PEA procedures were performed on 36 patients in Fu Wai Hospital from January 2015 to April 2016. There were 28 males and 8 females, with a mean age of 46.56±11.85 years. According to the New York Heart Association (NYHA) cardiac function classification, 36 patients were divided into preoperative severe RHF group (grade Ⅲ-Ⅳ,n=28) and preoperative without severe RHF group (grade Ⅱ,n=8). Hemodynamic parameters before and after PEA were recorded and 3-18 months' follow-up was done.
Results
All the patients having PEA surgeries had an obvious decrease of mean pulmonary arterial pressure (from 49.53±13.14 mm Hg to 23.58±10.79 mm Hg) and pulmonary vascular resistance (from 788.46±354.60 dyn·s/cm5 to 352.89±363.49 dyn·s/cm5, bothP<0.001). There was no in-hospital mortality among all the patients. Persistent pulmonary hypertension occurred in 2 patients, perfused lung in 2 patients, pericardial effusion in 2 patients. No mortality was found during the follow-up period. All patients improved to NYHA grade Ⅰ-Ⅱ (WHO grade Ⅰ-Ⅱ), and only 2 patients remained in the NYHA grade Ⅲ (P<0.01).
Conclusion
The CTEPH patients having PEA surgeries had an obvious improvement in both their hemodynamics results and postoperative heart function, which in return could improve their quality of life.
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.
Medical nitric oxide (NO) flow control system plays an important role in lowering pulmonary hypertension. The design requirements, overall scheme, delivery system and hardware circuits of a medical NO flow control system were introduced in this paper. Particularly, we proposed the design of NO delivery system and hardware circuits in detail. To deliver nitric oxide of a variable concentration, the designed system needs to work with a ventilator. The system can adjust and monitor the inhaled nitric oxide concentrations and send out sound and light alarms when the inhaled nitric oxide concentrations are out of the set range. To validate reliability and efficacy, we measured specifications such as linearity, stability and response time of the proposed NO flow control system by continuously administering nitric oxide into inspiratory circuit to deliver nitric oxide of variable concentrations to a test lung. The experiments showed that these specifications can meet the desired requirements.
Pulmonary hypertension due to left heart disease (PH-LHD) is the most common in various types of pulmonary hypertension. Although there are many treatments for pulmonary hypertension, it may be harmful when we adopt treatment without detrimental diagnosis and classification of pulmonary hypertension. Therefore, it is very crucial to have accurate diagnosis and classification of pulmonary hypertension before making treatment decisions. However, there are still some difficulties in the classification of pulmonary hypertension in clinical work. It is a great challenge with limited treatment to solve the PH-LHD which often has complicated pathophysiological mechanisms of precapillary and postcapillary pulmonary hypertension. Here, we review the research status of PH-LHD.
ObjectiveTo study the diagnosis and treatment of aortopulmonary window (APW) associated with severe pulmonary hypertension.MethodsThe clinical data of 23 patients with APW undergoing surgical treatment in The First Affiliated Hospital of Air Force Medical University from 2010 to 2018 were retrospectively reviewed. There were 9 male and 14 female patients. The age was 3-132 (4.63±2.14) months. The weight was 3.3-35.0 (17.3±3.6) kg.ResultsWindows were situated in the proximal of semilunar valve (type Ⅰ) in 8 patients, and distal of the aorta (type Ⅱ) in 14 patients, from proximal to distal (type Ⅲ) in only 1 patient. Eleven patients were isolated APW, the others were combined with cardiac defects. The mean pulmonary artery pressure was 68.4±7.5 mm Hg. All patients underwent surgical correction under general anesthesia and hypothermia cardiopulmonary bypass. All patients were discharged uneventfully, with an average follow-up time of 4 years. The patients showed good outcomes and no residual shunt after surgery, and the pulmonary artery pressure decreased to normal.ConclusionAPW is an uncommon congenital cardiac anomaly. The clinical presentation is an excessive left-to-right shunt, and most patients present early in life. Development of pulmonary hypertension and pulmonary vascular resistance is usually rapid. Operative treatment is indicated as soon as the diagnosis is established, regardless of the patient’s age, and most patients after surgery have a good long-term outcome.
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.
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.
