The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success of modern medicine. With the development of diagnostic techniques, surgical procedures and interventional techniques, more than 90% of CHD children can survive to adulthood. Consequently, the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle-aged adults but also patients aged above 60 years. Standardized guidelines can provide good theoretical support for the comprehensive management of adult CHD. Ten years after the European Society of Cardiology guidelines for the management of grown-up CHD released in 2010, the new version was officially released in August 2020. The new version of guidelines updated the classification and stratification of diseases, comprehensive intervention methods and intervention timing, and put forward some new concepts, new intervention standards and methods. For adult CHD that has not been repaired or needs to be repaired again, the indication and mode of surgical intervention and perioperative management have a great impact on the prognosis. The new version of the guidelines provides a detailed description of the surgical and intervention indications and methods for different diseases, and clarifies the management methods for high-risk groups. This article attempts to interpret this newly updated guideline from the perspective of a surgeon, sort out several key diseases introduced by the guideline, and strives to provide a concise and actionable guideline for domestic counterparts.
Objective To investigate the value of systemic-normothermic/cardiac-hypothermic cardiopulmonary bypass（CPB）on operation of congenital heart disease. Methods Thirty patients of congenital heart disease were randomly divided into two groups, the normothermia group（n=15）and hypothermia group（n=15）. The changes of CPB time, aortic cross-clamp time,operation time and postoperative drainage and the value of blood cell were observed. Results The duration of CPB （37. 5 ±11. 6rain vs. 51. 6± 12. 0 min, P〈0. 05） and operation time （2.2± 0.6h vs. 2. 7±0. 5h, P〈0. 01） in normothermia group were shorter than those of hypothermia group statistically, the differences of postoperative drainage and the value of blood cells between two groups were not statistically significant. Conclusion The use of systemic-normothermic/cardiac-hypothermic CPB on operation of congenital heart disease shows that the time of operation is shorter remarkly , and it could be clinically used safely.
ObjectiveTo explore the midterm therapeutic effect of modified Blalock-Taussing shunts (MBTs) in the treatment of tetralogy of Fallot. MethodsWe retrospectively analyzed the clinical data of 69 children with tetralogy of Fallot undergoing MBTs in Shanghai Xinhua Hospital between July 2006 and January 2013. There were 44 males and 25 females with mean age of 17.97±24.73 months (ranged from 2 months to 10 years). The patients weighted from 4 to 24 (9.00±4.03) kg. All the MBTs between subclavian artery and pulmonary artery were performed through right or left posterior lateral incision. ResultsThe patients were followed up for 6-36 months including 57 patients with 6 months following-up, 33 patients with 6 months and 12 months following-up, 16 patients with 12 months and 24 months following-up, and 11 patients with 24 months and 36 months following-up. There was significant growth in McGoon ratio during the first 12 months follow-up (preoperative vs. 6 months:1.09 ±0.33 vs. 1.40 ±0.40, P=0.00; 6 months vs. 12 months:1.29±0.31 vs. 1.36±0.33, P=0.00). There was no obvious growth in McGoon ratio after 12 months (12 months vs. 24 month:1.22±0.31 vs. 1.19±0.32, P=0.14; 24 months vs. 36 months:1.22±0.23 vs. 1.23±0.20, P=0.45). The left ventricular end diastolic volume index (LVEDVI) increased significantly in 6 months after MBTs (preoperative vs. 6 months:29.60±10.12 ml/m2 vs. 49.18±11.57 ml/m2, P=0.00), but there was no significant growth after 6 months. There was no significant decline in left ventricular ejection fraction (LVEF) after MBTs. ConclusionThe MBTs can significantly promote the growth of McGoon ratio in 12 months of patients with tetralogy of Fallot, but there is no obvious growth of McGoon ratio after 12 months. MBTs can significantly improve left ventricular development within 6 months, and it won't lead to excessive expansion of the left ventricle when we extend follow-up time. The MBTs affects little on cardiac function of patients with tetralogy of Fallot.
ObjectiveTo analyze clinical outcomes of sutureless technique for patients undergoing surgical correction of total anomalous pulmonary venous connection (TAPVC). MethodsBetween July 2007 and December 2013, 132 consecutive TAPVC patients underwent surgical correction in Guangdong Cardiovascular Institute. Those patients with such associated congenital cardiac anomalies as single ventricle and right atrial isomerism were excluded from this study. All the patients underwent biventricular repair. Preoperatively, all the patients received echocardiography, and most patients received CT scan to know the development of pulmonary veins. Preoperative diagnosis was confirmed by intraoperative exploration. According to different surgical techniques, all the patients were divided to a conventional technique group and a sutureless technique group. In the conventional technique group, there were 69 patients including 54 males (78.3%)and 15 females (21.7%)with their median age of 60 (30, 225)days and median body weight of 4.85 (3.50, 6.35)kg. In the sutureless technique group, there were 63 patients including 48 males (76.20%)and 15 females (23.8%)with their median age of 90 (30, 210)days and median body weight of 4.58 (3.72, 6.20)kg. Follow-up was performed till January 1, 2014. ResultsIn-hospital mortality (4.8% vs. 7.2%, χ2=1.414, P=0.720)and postoperative overall mortality (4.8% vs. 13.0%, χ2=2.733, P=0.098)of the sutureless technique group were both lower than those of the conventional technique group, although there was no statistical difference. Postoperative incidence of pulmonary venous obstruction (PVO)of the sutureless technique group was significantly lower than that of the conventional technique group (1.6% vs. 10.1%, χ2=4.236, P=0.040). Cox proportional-hazards regression showed that conventional technique and preoperative PVO were significant risk factors for postoperative PVO (P=0.023, P=0.016). Conventional technique was not significantly correlated with postoperative mortality (P=0.060). ConclusionSutureless technique can significantly lower postoperative incidence of PVO for patients with supracardiac TAPVC.
ObjectiveTo analyze the effect of modified B-T shunt for the treatment of complex congenital heart disease. MethodsWe retrospectively analyzed the clinical data of 150 B-T case times performed in 143 patients with complex congenital heart disease in Shanghai Xinhua Hospital between July 2006 and January 2013.There were 100 case times for male patients and 50 case times for female patients with age of 2-756 (20.17 ±80.37) months and weight of 4-63 (8.86 ±9.69) kg. ResultsThere were 5 in-hospital deaths (mortality at 3.50%). Three patients occurred abnormal bleeding (2.10%). Five patients (3.50%) performed the second B-T because of shunt occlusion. And the other patients recovered uneventfully. A total of 129 case times were followed up for 6-48 (14.38±10.05) months. Seven B-T case times (5.43%) were performed in 6 patients again because of shunt occlusion during the follow-up. Three patients died during the follow-up (mortality at 2.33%). A total of 88 patients of survival underwent corrective surgery or stage Ⅱ palliative surgery (68.22%). Main pulmonary artery have a significant increase in diameter during the follow-up(t=-15.18, P=0.00). Postoperative diameters of left pulmonary artery (t=-13.27, P=0.00), right pulmonary artery (t=-15.94, P=0.00), and right pulmonary artery (t=2.44, P=0.02) increased with statistical differences compared with preoperative values. Growth in ipsilateral pulmonary of B-T is better than that of the contralateral pulmonary (t=2.44, P=0.02). McGoon ratio increased significantly after B-T (t=10.10, P=0.00). Ejection fraction value was slightly lower than the preoperative value (t=2.77, P=0.00). Left ventricular mass index increased significantly compared with the preoperative value(t=-9.26, P=0.00). ConclusionsThe modified B-T shunt has been proved to be safe and effective in treating for complex congenital heart disease. It can significantly promote the development of pulmonary artery, especially the ipsilateral pulmonary of B-T. Small McGoon ratio and pulmonary atresia are the risk factors for limiting the further development of pulmonary. Appropriate diameter of B-T shunt choice according to preoperative pulmonary diameter and the weight of the patients is the basis to ensure successful operation and a good prognosis.
Objective To explore the efficacy of humidified high flow nasal cannula ( HHFNC) for respiratory failure after ventilator weaning in post-operative newborns and infants with congenital heart disease. Methods FromJanuary 2010 to August 2010, 33 newborns and infants [ ( 7. 8 ±8. 4) months, range 3 days to 36 months; weight ( 6. 6 ±3. 6) kg, range 2. 2 to 19. 6 kg] were treated with HHFNC ( 22 cases) and routine oxygen therapy ( 11 cases) for respiratory failure following ventilator weaning after operation of congenital heart disease. Symptoms, blood oxygen saturation ( SpO2 ) , partial pressure of oxygen( PaO2 ) , partial pressure of carbondioxide ( PaCO2 ) , incidence rate of re-intubation, duration of ICU, and hospital stay were assessed and compared between the HHFNC group and the routine oxygen therapy group.Results There were no statistical significance in the duration of ICU, hospital stay, duration of mechanical ventilation, or infection rate between the HHFNC group and the routine oxygen therapy group ( P gt; 0. 05) . But the incidence rate of re-intubation was lower in the HHFNC group than that in the routine oxygen therapy group. Meanwhile SpO2 and PaO2 increased and PaCO2 decreased significantly in the HHFNC group ( P lt;0. 05) . Conclusion HHFNC shows a clinical improvement rapidly and efficiently in preventing respiratory failure after ventilator weaning in post-operative newborns and infants with congenital heart disease.
ObjectiveTo analyze the early outcomes of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) patients with severe left ventricular dysfunction after surgical repair, and to explore the predictors for extracorporeal membrane oxygenation (ECMO) support for these patients.MethodsThe clinical data of ALCAPA patients with severe left ventricular dysfunction (left ventricular ejection fraction<40%) who underwent coronary artery reimplantation in the pediatric center of our hospital from 2013 to 2020 were retrospectively analyzed. The patients were divided into an ECMO group and a non-ECMO group. Clinical data of the two groups were compared and analyzed.ResultsA total of 64 ALCAPA patients were included. There were 7 patients in the ECMO group, including 4 males and 3 females aged 6.58±1.84 months. There were 57 pateints in the non-ECMO group, including 30 males and 27 females aged 4.34±2.56 months. The mortality of the patients was 6.25% (4/64), including 2 patients in the ECMO group, and 2 in the non-ECMO group. The postoperative complications rate was significantly higher in the ECMO group than that in the non-ECMO group (P=0.041). There were statistical differences in the cardiopulmonary bypass time [254 (153, 417) min vs. 106 (51, 192) min, P=0.013], aortic cross-clamping (ACC) time (89.57±13.66 min vs. 61.58±19.57 min, P=0.039), and preoperative left ventricular end-diastolic diameter/body surface area (132.32±14.71 mm/m2 vs. 108.00±29.64 mm/m2, P=0.040) between the two groups. Multivariate logistic regression analysis showed that ACC time was an independent risk factor for postoperative ECMO support (P=0.005). Receiver operating characteristic (ROC) curve analysis showed that the area under the ROC curve was 0.757, the sensitivity was 85.70%, specificity was 66.70%, with the cut-off value of 66 min.ConclusionACC time is an independent risk factor for postoperative ECMO support. Patients with an ACC time>66 min have a significantly higher risk for ECMO support after the surgery.
ObjectiveTo summarize clinical experience and outcomes of extracardiac conduit total cavopulmonary connection (TCPC)for surgical treatment of complex congenital heart diseases. MethodsClinical data of 52 patients who underwent extracardiac conduit TCPC from September 2006 to December 2012 in Department of Cardiac Surgery, Guangzhou General Hospital of Guangzhou Military Command were retrospectively analyzed. There were 12 patients who received one-stage extracardiac conduit TCPC. There were 40 patients who received two-staged extracardiac conduit TCPC after bidirectional Glenn procedure. Clinical data of all the patients were analyzed. Mortality, morbidity, length of hospital stay and intensive care unit (ICU)stay, mechanical ventilation time, change of arterial oxygen saturation (SaO2)were compared between the 2 groups. ResultsTwo patients (3.8%)died postoperatively including 1 patient with severe low cardiac output syndrome and another patient with multiple organ dysfunction syndrome. Fifty patients were discharged successfully. Mechanical ventilation time, length of ICU stay and hospital stay of the 40 patients who received two-stage extracardiac conduit TCPC were significantly shorter than those of the 12 patients who received one-stage extracardiac conduit TCPC. There was no statistical difference in postoperative morbidity, SaO2 (two-staged vs. one-staged:93%±3% vs. 94%±3%)or mortality (two-staged vs. one-staged:2.5% vs.8.3%)between the 2 groups (P > 0.05). Forty-five patients (90%)were followed up for 6-52 months, and there was no death during follow-up. At 3 months after TCPC, all the patients had heart function of class I or II, and echocardiography showed patent cavopulmonary anastomosis. ConclusionExtracardiac conduit TCPC is a simple procedure, can produce more physiological hemodynamic results, and can be performed for patients who cannot undergo biventricle procedure. Compared with one-stage extracardiac conduit TCPC, two-staged extracardiac conduit TCPC has wider surgical indications, can produce better postoperative recovery, and is easier to perform.
Objective To summarize our clinical experience of pulmonary artery banding （PAB） for the treatment of complex congenital heart diseases as a palliative procedure.?Methods?From January 1997 to November 2010, 138 patients with complex congenital heart diseases underwent PAB in Fu Wai Hospital. There were 87 male patients and 51 female patients with their age of 22.2±26.5 months and average body weight of 7.5±4.6 kg. All the 138 patients were divided into 3 groups according to the purpose of PAB：left ventricular retraining （group 1, n=55）, initial procedure for functional single ventricle with unobstructed pulmonary blood flow （group 2, n=32） and initial palliative procedure followed by later biventricular repair （group 3, n=51）. The intraoperative and postoperative clinical parameters of all participants were observed, and follow-up was made for these 3 groups of patients.?Results?The in-hospital mortality of PAB was 5.1% （7/138）. Three patients underwent re-banding procedure to adjust the size of banding. In group 1, there was 1 postoperative death. Among the 55 patients, 36 patients with dextro-transposition of great arteries received PAB at an average age of 19.6±29.5 months, 29 patients of whom underwent concomitant modified Blalock-Taussig shunt. After an average training interval for 42 days, 83.3% of them（30/36）successfully received arterial switch operation. The other 19 patients in group 1 with isolated corrected transposition of great arteries received PAB at an average age of 45.3±27.2 months. Afteran average training interval for 9 months, 42.1% of them （8/19） successfully received double-switch operation. In group 2, there was 2 postoperative death. Thirty-two patients with functional single ventricle and unobstructed pulmonary blood flow received PAB at an average age of 14.1±14.9 months. Their postoperative mean pulmonary artery pressure decreased significantly from 34.00±10.00 mm Hg to 23.00±7.40 mm Hg, and their oxygen saturation of blood （SpO2） significantly decreased from 92.60%±5.90% to 83.30%±6.30%. After a median interval of 2 years, 18.8% of them （6/32） underwent right heart bypass operation. In group 3, there was 4 postoperative death. Fifty-one patients received PAB at an average age of 20.60±25.60 months. After PAB procedure, the ratio of systolic pulmonary artery pressure and systolic blood pressure significantly decreased from 0.81±0.14 to 0.46±0.15, and their SpO2 significantly decreased from 93.10%±7.60% to 85.00%±10.00%. After a median interval of 6 months, 23.5% of them （12/51） received biventricular repair.?ConclusionAlthough PAB is a palliative procedure with comparatively high risks, it still plays an indispensable role in terms of protecting pulmonary vascular beds, retraining ventricular function and two-stage surgical correction for the treatment of complex congenital heart diseases.
Objective To investigate how to choose the methods of surgical treatment for Scimitar syndrome. Methods From Jan. 1999 to July 2004, the clinical data of 12 patients with Scimitar syndrome were analyzed retrospectively, 10 patients underwent repair by intra-atrial baffles approach, one patient by connecting scimitar vein and left atrium with artificial blood vessel under cardiopulmonary bypass, and one patient by directly reimplanting the scimitar vein to left atrium without cardiopulmonary bypass. Results All the 12 patients had no perioperative or late deaths and none of them required reoperation. Follow-up was extended from 1 to 36 months, echocardiography demonstrated a patent anastomosis in all patients without any evidence of restenosis. Conclusions Surgical approaches to Scimitar syndrome is based on the anatomic and pathologic features presented in each case. Approriate method will have good result.