Surgical treatment of preterm infants and small infants with critical and complex congenital heart disease

    We retrospectively analyzed the clinical data of 849 cases of critically complex congenital heart disease aged less than 6 months who were surgically treated in our hospital from January 2003 to October 2008. Mean age: 4.15±2.32 months (1 day-6 months). Mean weight: 4.42±0.56 kg (1.0-7 kg).  The types of disease included: combined respiratory distress with patent ductus arteriosus (PDA) in 48 cases; combined pulmonary hypertension with ventricular septal defect (VSD/PH) in 511 cases, complete large vessel dislocation (D-TGA) in 35 cases; double outlet right ventricle (DORV) in 37 cases; aortic constriction (CoA) or combined intracardiac malformation in 16 cases; interrupted aortic arch (IAA) in 5 cases; complete pulmonary venous ectopic (TAPVC) in 26 cases; tetralogy of Fallot (TOF) in 97 cases; complete atrioventricular septal defect (CAVC) in 19 cases; pulmonary atresia with intact ventricular septum (PA/IVS) in 8 cases; permanent arterial trunk in 3 cases; main pulmonary artery window in 2 cases; right pulmonary artery originating from the ascending aorta in 2 cases; triatrial heart in 4 cases; severe pulmonary stenosis (PS) in 16 cases; atrial septal defect (ASD) in 11 cases; tricuspid atresia (TA) in 3 cases; coronary artery fistula (CAF) in 3 cases; congenital vascular ring malformation: double aortic arch in 1 case; right ventricular tumor in 1 case. Preoperative respiratory failure with ventilator was used in 52 cases. All children were diagnosed by cardiac ultrasound or/and 64-row CT of cardiac great vessels, except for pulmonary artery atresia with preoperative cardiac catheterization.  Surgical procedures: 16 cases of PDA in preterm infants were ligated openly at the bedside of NICU; 3 cases of pulmonary artery circumferential ligation in functional single ventricle with PH; 2 cases of body-pulmonary artery bypass in functional single ventricle with severe cyanosis; 1 case of coronary artery fistula ligation under non-CPB; 4 cases of aortic constriction alone had end-to-end aortic anastomosis via the left thoracic route for aortic constriction resection. All other patients underwent direct intracardiac surgery under extracorporeal circulation, ASD repair under normothermic extracorporeal cardioversion in 9 cases, PS enlargement with autologous pericardium in 16 cases, and bidirectional Glenn’s procedure in 2 cases.  Four cases of combined PA/IVS with PDA/ASD underwent right ventricular outflow tract pulmonary artery pericardial patch enlargement with simultaneous bypass of the innominate artery and right pulmonary artery. In 511 cases of non-restricted VSD and combined malformations were repaired in one stage under medium- and low-temperature extracorporeal circulation or deep low-temperature low-flow and stopped circulation, and VSD was repaired continuously or intermittently via the right atrium or pulmonary artery. 28 cases of D-TGA and 6 cases of Taussig-Bing underwent aortic reversal, of which 2 cases underwent rapid second-stage aortic reversal; the other 7 cases underwent staged aortic reversal within 3-1 years.  In 31 cases, the right ventricular double outlet was selected according to the different anatomical types; 17 cases of aortic constriction and aortic arch disruption combined with VSD/PDA were corrected in one stage through a median sternotomy, with resection of aortic constriction and end-to-end anastomosis of the descending aorta. 12 cases of TAPVC were performed in the supracardiac type through the supracardiac route, between the ascending aorta and superior vena cava, with the right pulmonary artery pulled in a cephalad direction, exposing the top of the left atrium and anastomosing it to the summary vein. In 10 intracardiac cases, the atrial septal tissue between the top of the coronary sinus and the ASD was dissected and the ASD was repaired with an autologous pericardial patch. in 3 subcardiac cases, the vertical vein was ligated at the level of the transverse septum and the pulmonary vein coaptation was anastomosed to the posterior wall of the left atrium. in 97 TOF cases, except for 4 cases with central palliation staged surgery, the rest were repaired via the right atrium with poorly aligned VSD and autologous pericardial enlargement of the right ventricular The CAVSD was repaired by the double-piece method in 10 cases, completely repaired by the single-piece pericardial method in 4 cases, and by the modified single-piece method in 5 cases. All patients underwent ultrafiltration or (and) modified ultrafiltration after stopping extracorporeal circulation. HCT increased from 20.2±2.4% to 32.1±2.8% before and after ultrafiltration, with a mean of 387±115 ml of filtered fluid. time of extracorporeal circulation: 89.24±37.46 (41-208 min), time of aortic block: 49.07±24.15 (18-119 min) , time to circulatory arrest 23.45±14.67 minutes (15-39 minutes).  One case of TAPVC had difficulty in deconditioning after surgery and was revived with low-flow assisted circulation for 3 hours. There were 28 cases of delayed chest closure, which were closed 1–3 days after surgery. Postoperatively, they were admitted to ICU for monitoring. Postoperative ventilator use time: 3 hours-90 days, mean 17.5 ± 9.7 hours, ICU stay: 2-90 days, mean 4.9 ± 1.5 days.  RESULTS: There were 30 postoperative deaths, with a mortality rate of 3.62%. Among them, 4 cases of D-TGA combined with coronary artery malformation, 2 cases of combined LVOTO2, 2 cases of uncontrollable bleeding, 1 case of rapid phase II death from mycobacterial infection 3 months after surgery, 1 case of spontaneous liver rupture 1 week after surgery, 1 case of postoperative ischemic-hypoxic encephalopathy parental abandonment; 1 case of Taussig-Bing with intracardiac tunnel repair; 2 cases of IAA; 2 cases of subcardiac TAPVC; 3 cases of VSD combined with malformation and 5 cases of TOF died from pulmonary hemorrhage renal failure; 1 case of permanent arterial trunk died from mycobacterial infection 3 months after surgery; 1 case of single atrium combined with left ventricular dysplasia; 1 case of VSD/PH combined with tracheal stenosis died 19 days after surgery; 1 case of III degree dystrophy CoA/VSD/PDA/PH died from asphyxia 18 days after surgery; 1 case of DORV/PH postoperative renal failure parents abandoned treatment; 1 case of severe hypoxemia after B-T shunt for TOF/PA/PDA at 2 months. The postoperative complications were: 23 cases of hypocardial discharge, 5 cases of residual shunt secondary surgery, 18 cases of infiltration syndrome, 9 cases of mediastinal infection sternal doctomy; 12 cases of pleural effusion, 33 cases of pneumonia pneumonia, 28 cases of pulmonary hemorrhage, and 9 cases of renal failure. The postoperative follow-up was 1 month-5 years, and the surviving children had good cardiac function and significantly accelerated growth and development.  Conclusion: With the improvement of the level of pediatric congenital heart diagnosis, surgical techniques and postoperative monitoring, surgical operation for critical and complex congenital heart disease in preterm and small infants is feasible, and the survival rate of the operation can be acceptable.

Support Us

Discussion

Share your experience, or seek help from fellow patients.

Other Languages

English Deutsch Français Español Português 日本語 Bahasa Indonesia Русский