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.
ObjectiveTo analyze the outcomes of surgical repair for mixed total anomalous pulmonary venous connection (TAPVC).MethodsBetween 2006 and 2018, a total of 51 patients with mixed TAPVC underwent surgery in our hospital. Patients with such associated anomalies as single ventricle and tetralogy of Fallot were excluded. There were 35 males and 16 females with a median age of 102.0 (59.0, 181.0) days and a median weight of 5.0 (4.1, 6.4) kg. Patients were divided into three categories based on the anatomy: "3+1" pattern (n=38, three pulmonary veins drained at one site, and the other drained at the opposite site); "2+2" pattern (n=9, the pulmonary veins from each lung joined to form a confluence and drained at separate sites); bizarre pattern (n=4, the anatomy could not be classified into the above two patterns).ResultsThere was no in-hospital death. The median follow-up was 41.0 (18.0, 86.5) months. Postoperative pulmonary venous obstruction occurred in 10 patients. Kaplan-Meier survival curves showed no statistically significant difference in postoperative pulmonary venous obstruction among the three groups (P=0.239). Cox risk regression showed that preoperative pulmonary venous obstruction was significantly associated with postoperative pulmonary venous obstruction (P=0.024).ConclusionMixed TAPVC has various anatomic morphologies and requires individualized surgery.
Double outlet right ventricle (DORV) is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair. DORV is mainly defined as the congenital heart disease with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily (>50%) from the right ventricle, associated with continuity or discontinuity between the aorta and mitral valve. DORV can be subclassified by various ways. Now subclassification is usually performed according to the relationship between the ventricular septal defect (VSD) and the great arteries. Various approaches for surgical repair of DORV ranging from single ventricle palliation to biventricular repair are reported from many centers. However, the high-grade guideline of surgical management of DORV is still absent. Hence, we developed the Chinese expert consensus on DORV as the evidence for surgical strategies.
ObjectiveTo investigate the clinical efficacy and application value of percutaneous interventional treatment for structural heart diseases under guidance of ultrasound.MethodsThe clinical data of 1 010 patients with structural heart diseases treated by transcutaneous ultrasound-guided occlusion in our hospital from December 2, 2015 to December 31, 2019 were retrospectively reviewed, including 360 males and 650 females, aged 1-50 years. There were 692 patients of atrial septal defect (603 with central type, 9 combined with arterial catheter, 80 with ethmoid type), 116 patent foramen ovale, 25 ventricular septal defects (3 combined with atrial septal defect), 132 patent ductus arteriosus, 32 pulmonary valve stenosis (3 combined with atrial defect), 1 main pulmonary artery window, and 3 aneurysm rupture of aortic sinus. All patients were diagnosed by transthoracic echocardiography (TTE) before operation. Treatment was accomplished intraoperatively through TTE or transesophageal echocardiography (TEE) via the femoral artery or femoral vein. After operation, echocardiography, electrocardiogram and chest radiograph were reexamined.ResultsSatisfactory results were obtained in 1 005 patients, and 1 patient failed to seal the ventricular defect and was repaired under direct vision, occluder detachment occurred in 5 patients after operation (3 patients of atrial septal defects underwent thoracotomy for Amplatzer device and were repaired, 1 patient of atrial septal defects was closed after removing Amplatzer device, 1 patient of patent ductus arteriosus underwent thoracotomy for Amplatzer device and was sutured), mild pulmonary valve regurgitation occurred after balloon dilation in 2 patients with pulmonary stenosis, a small amount of residual shunt was found in 2 patients with ventricular defect, which disappeared after 3 months of follow-up, and 1 patient of right bundle branch block occurred and disappeared after 1 week. After follow-up of 1-24 months, 3 patients of ethmoidal atrial septal defect were reexamined with mild shunt. The occluder was in good position and the pressure difference of pulmonary valve was significantly reduced. There was no complication such as hemolysis, arrhythmia, embolism or rupture of chordae tendinae.ConclusionPercutaneous transfemoral artery and vein guided by TTE or TEE is safe and effective, with little trauma, no radiation or contrast agent damage, and has significant clinical efficacy and application values.
Fontan operation is still a main procedure for treatment of complex congenital heart disease, such as univentricular heart. Fontan procedure has undergone many revisions since its introduction in 1968. The earlyapplied atriumpulmonary connection has been replaced by total cavopulmonary connection. The midterm and late results of both the intraatrial lateral tunnel and extracardiac total cavopulmonary connections were compared and analyzed in this article. Extracardiac conduit is better. The Fontan circulation failure would appear at last because of nopump function of the right ventricle. Once Fontan circulation failure occurred and could not recover by medicine, heart transplantation is mandatory, but the source of donor heart is lacking. The study of mechanical cavopulmonary assist device, to “biventricularize” the univentricular Fontan circulation, has been developed, which is quite promising. Following the development of diagnostic and treatment techniques for fetal heart disease, the treatment procedure of complex congenital heart disease has been broadened in recent years, such as to prevent the severe aortic stenosis from developing into hypoplastic left heart syndrome with fetal cardiac intervention so as to increase the chance of biventricular repair, and to terminate gestation to decrease its birth rate of complex heart abnormalities, which could not be completely repaired to date.
Objective To explore the feasibility of transcatheter closure of congenital heart disease (CHD) under the guidance of transthoracic echocardiography (TTE). Methods A total of 37 patients with CHD who received transcatheter closure under the guidance of transthoracic echocardiography from November 2013 through November 2015 in our hospital were recruited. There were 15 males and 22 females, aged 1 to 16 years. Among them 32 patients suffered atrial septal defect and 5 patients had patent ductus arteriosus. The transcatheter closure of CHD was performed under the guidance of TTE. The patients underwent echocardiography follow-up at one, three and six months after surgery. Results Closure devices were successfully implanted in 37 patients under TTE guidance. The procedure was simple and safe. During the follow-up, no severe complication such as valvular injury, pericardial effusion, residual shunt and peripheral vascular injury occurred. Conclusion Transcatheter closure of CHD under TTE guidance is a feasible method and worth further clinical application.
ObjectiveTo investigate the effect of pulmonary ultrasound on pulmonary complications in ultra-fast-track anesthesia for congenital heart disease surgery.MethodsIn 2019, 60 patients with congenital heart diseases underwent ultra-fast-track anesthesia in Shenzhen Children's Hospital, including 34 males and 26 females with the age ranging from 1 month to 6 years. They were randomly divided into a normal group (group N, n=30) and a lung ultrasound optimization group (group L, n=30). Both groups were used the same anesthesia method and anesthetic compatibility. The group N was anesthetized by ultra-fast-track, the tracheal tube was removed after operation and then the patients were sent to the cardiac intensive care unit (CCU). After operation in the group L, according to the contrast of pre- and post-operational lung ultrasonic examination results, for the patients with fusion of B line, atelectasis and pulmonary bronchus inflating sign which caused the increase of lung ultrasound score (LUS), targeted optimization treatment was performed, including sputum suction in the tracheal tube, bronchoscopy alveolar lavage, manual lung inflation suction, ultrasound-guided lung recruitment and other optimization treatments, and then the patients were extubated after lung ultrasound assessment and sent to CCU. The occurrence of pulmonary complications, LUS, oxygenation index (OI), extubation time, etc were compared between the two groups.ResultsCompared with the induction of anesthesia and 1 hour after extubation of the two groups, the incidence of pulmonary complications in the group L (18 patients, 60.0%) was lower than that in the group N (26 patients, 86.7%, χ2= 4.17, P=0.040) and the rate of patients with LUS score reduction was higher in the group L (15 patients, 50.0%) than that in the group N (7 patients, 23.3%, χ2=4.59, P=0.032). The correlation analysis between the LUS and OI value of all patients at each time point showed a good negative correlation (P<0.05). Extubation time in the group L was longer than that in the group N (18.70±5.42 min vs. 13.47±4.73 min, P=0.001).ConclusionUltra-fast-track anesthesia for congenital heart disease can be optimized by pulmonary ultrasound examination before extubation, which can significantly reduce postoperative pulmonary complications, improve postoperative lung imaging performance, and help patients recover after surgery, and has clinical application value.
Objective To confirm the changes of pulmonary artery pressure, neo pulmonary artery stenosis and reoperation in children with unilateral absence of pulmonary artery (UAPA) undergoing pulmonary artery reconstruction. Methods The clinical data of the infants with UAPA undergoing pulmonary artery reconstruction in our hospital from February 19, 2019 to April 15, 2021 were analyzed. Changes in pulmonary artery pressure, neo pulmonary artery stenosis and reoperation were followed up. Results Finally 5 patients were collected, including 4 males and 1 female. The operation age ranged from 13 days to 2.7 years. Cardiac contrast-enhanced CT scans were performed in all children, and 2 patients underwent pulmonary vein wedge angiography to confirm the diagnosis and preoperative evaluation. Preoperative transthoracic echocardiography and intraoperative direct pulmonary arterial pressure measurement indicated that all 5 children had pulmonary hypertension, with a mean pulmonary arterial pressure of 31.3±16.0 mm Hg. Pulmonary arterial pressure decreased immediately after pulmonary artery reconstruction to 16.8±4.2 mm Hg. The mean follow-up time was 18.9±4.7 months. All 5 patients survived during the follow-up period, and 1 patient had neo pulmonary artery stenosis or even occlusion and was re-operated. Conclusion Pulmonary artery reconstruction can effectively alleviate the pulmonary hypertension in children with UAPA. The patency of the neo pulmonary artery should be closely followed up after surgery, and re-pulmonary angioplasty should be performed if necessary.
Objective To explore the treatment method of congenital heart disease (CHD) with pulmonary artery hypertension (PAH) in infants with Down syndrome (DS). Methods The clinical data of 60 CHD patients with PAH from March 2015 to August 2016 in our hospital were retrospectively analyzed. There were 30 infants with DS classified as a DS group (trial group, 17 males and 13 females with a mean age of 1.15±0.25 years) and the other 30 patients without DS were classified as a control group (20 males and 10 females with a mean age of 1.24±0.30 years). All the patients underwent surgical treatment and fasudil combined with sildenafil were used to prevent pulmonary hypertension crisis postoperatively. Results There was no significant difference in cardiopulmonary bypass time, aortic cross-clamping time, modified ultrafiltration time and the incidence of postoperative respiratory complications between the two groups. The pulmonary systolic blood pressure significantly decreased at 24 h after operation in the two groups (both P<0.05). The arterial oxygen pressure and oxygenation index of the trial group were lower than those of the control group at 6 h after operation (both P<0.05). The mechanical ventilation time and intensive care time of the trial group were significantly longer than those of the control group (P=0.007 and P=0.000, respectively). There were no reoperations or early death. Conclusion The effects of surgical repair of CHD with PAH in infants with DS are satisfactory by grasping the indication, protecting lung function and controlling PAH in the early postoperative period, although there is a high incidence of pulmonary complications.
Objective To evaluate the impact of an integrated management mode of prenatal diagnosis-postnatal treatment for congenital heart disease (CHD) on perioperative and long-term outcomes of the arterial switch operation (ASO), and to analyze the efficacy of ASO over six years in a single center. Methods This retrospective study analyzed the clinical data of 183 children who underwent ASO at Guangdong Provincial People's Hospital from January 2018 to December 2024. The cohort included 106 patients (57.9%) of transposition of the great arteries with intact ventricular septum (TGA/IVS), 61 patients (33.3%) of transposition of the great arteries with ventricular septal defect (TGA/VSD), and 16 patients (8.7%) of taussig-bing anomaly (TBA). Perioperative indicators were compared between 91 patients in the prenatal-postnatal integrated management group (an integrated group) and 92 patients in the traditional management group (a non-integrated group). Long-term survival and reoperation rates were analyzed using Kaplan-Meier curves. Results The overall perioperative mortality rate was 4.9% (9/183), showing a downward trend year by year. The primary cause of perioperative mortality was low cardiac output syndrome (LCOS), which occurred in 12 patients (6.6% incidence) with a mortality rate of 75%. The integrated group had a higher proportion of males (89% vs. 72.8%, P<0.05) and lower body weight [3.13 (2.75, 3.35) vs. 3.30 (3.00, 3.67), P<0.05] compared to the non-integrated group. The age at surgery was significantly earlier in the integrated group [7 (3, 10) vs. 14 (9, 48), P<0.05], and all children in the Integrated Group underwent ASO within the optimal surgical window (100% vs. 82.6%, P<0.05). Intraoperatively, cardiopulmonary bypass (CPB) time [173 (150, 207) vs. 186 (159, 237), P<0.05] and aortic cross-clamp (ACC) time [100 (90, 117) vs. 116 (97, 142), P<0.05] were significantly shorter in the integrated group. although the integrated group had longer postoperative mechanical ventilation time [145 (98, 214) vs. 116 (77, 147), P<0.05] and higher 48-hour maximum vasoactive inotropic score (VISmax) [15 (10, 21) vs. 12 (8, 16), P<0.05], there was no statistically significant difference in the incidence of severe complications (LCOS, NEC, ECMO) or mortality rate (3.3% vs. 6.5%, P=0.51) between the two groups, despite earlier surgical intervention and a higher proportion of critically ill cases in the integrated group. The length of hospital stay in the emergency surgery group was significantly shorter than that in the elective surgery group [20 (15, 28) vs. 25 (21, 30), P<0.05], suggesting that early surgery may be of potential benefit. A total of 163 patients were successfully followed up for a median of 4.7 years, with a 5-year survival rate of 95.1% and a freedom from reintervention survival rate of 95.1%. There were no late deaths, and the most common postoperative complication was pulmonary artery stenosis. Conclusion The integrated management model allowed critically ill children with lower body weights to safely undergo surgery, significantly optimizing the timing of surgery and shortening intraoperative times. The long-term risk of reoperation after ASO is primarily concentrated on pulmonary artery stenosis, necessitating long-term follow-up and monitoring.