Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. It is characterized by an interventricular communication with an overriding aorta, subpulmonary obstruction, and consequent right ventricular hypertrophy. The potential for late complications is an important concern for growing number of survivors after surgical repair, although long-term survival rates are excellent. Progressive pulmonary valve regurgitation leading to right heart failure and arrhythmias are common late complications and major reasons of mortality. In this review, we focus on research progress of pathogenesis and treatment of late complications after TOF repair, and the importance of long-term follow-up is emphasized.
Abstract: Objective To evaluate the longterm results of surgical treatment of tetralogy of Fallot (TOF) in adults and discuss the perioperative treatment skills. Methods From January 2000 to March 2008, 149 patients older than 14 years with tetralogy of Fallot received surgical treatment in Changhai Hospital. Among the patients, there were 78 males and 71 females with ages ranged from 14 years to 53 years and the average age was 26.3 years. Twenty patients had previous pulmonary arterial shunts before radical treatment. A total of 129 patients underwent primary radical treatment. Thirtyeight patients received a right ventricular outflow tract patch, 107 patients had transannular patch, and 4 patients had homograft aorta with valves. Results Hospital mortality was 4.0%(6/149). Four patients died of low cardiac output syndrome (LCOS), and multiple organ failure, and 2 patients died of acute renal failure. The postoperative complications included pleural effusion in 11 patients, pulmonary edema in 10 patients, severe LCOS in 9 patients, severe cardiac arrhythmia in 7 patients, reoperation for excessive bleeding in 7 patients, reintubation in 6 patients, and residual ventricular septal defect (VSD) in 5 patients (two of them had reoperation for residual VSD repair and 2 received transcatheter closure of VSD). One hundred and thirtyfour patients were followed up for 3 to 102 months (47.2±28.6 months) with a followup rate of 93.7%(134/143). Late death occurred in 2 patients, one of whom died of secondary infective endocarditis and the other had a sudden death 29 months after operation. During the followup, one patient had residual VSD (2 mm), but had a normal life. The peak systolic right ventricletopulmonary artery pressure gradient exceeded 40 mmHg in 4 patients. Two patients had severe pulmonary regurgitation. A total of 132 patients survived and had an improved life. One hundred and twentyone patients had class Ⅰ heart function (NYHA), and 11 patients in class Ⅱ. Conclusion The pathophysiologic conditions of the patients with tetralogy of Fallot in adults are very complicated due to longterm right ventricle outlet stricture and chronic hypoxia. Preoperative evaluations and postoperative treatment of complications are necessary. The systemicpulmonary arterial shunts should be performed when hypotrophy of the pulmonary arteries or left ventricles exists. Repair of tetralogy of Fallot in adults has acceptable morbidity and mortality rates with goodlongterm outcomes.
Objective To summarize the experiences of surgical intervention for tetralogy of Fallot(TOF) in early infancy and to discuss the relevant issues about primary treatment procedures in the period. Methods We retrospectively analyzed the clinical operative information of 21 patients in their early infancy (less than 6 months) with TOF treated in Children’s Hospital of Shanghai from June 2008 to August 2010. There were 14 males and 7 females with a mean age of 4.86±1.15 months and a mean body weight of 6.84±1.33 kg. All patients were diagnosed by heart color Doppler ultrasound. Four patients underwent CT or magnetic resonance imaging（MRI） or right heart catheter arteriography examination. The McGoon ratio was 1.86±0.41 and the pulmonary artery index（PAI） was 142.54±59.46 mm2/m2. The ventricular septal defect (VSD) was closed with autologous pericardium using continuous sutures through right atrium (19 cases) or right ventricle (2 cases). Transannular repair was performed when pulmonary valve annulus was one standard deviation less than the normal Z value (18 cases). If the annulus diameter approached or reached the normal Z value, the valve annulus was preserved and pericardium was used to enlarge the right ventricular outflow tract(RVOT) and the main pulmonary artery (3 cases). Results There was one death due to heart failure on the 15th day after operation, one patient had acute laryngeal edema after removal of endotracheal intubation on the second day after operation, and received reintubation and assisted ventilation for three days. All the other patients recovered well. Eighteen patients were followed up for 9.89±6.47 months. Their heart functions were in modified Ross class I or II. Echocardiography during the followup showed that RVOT pressure was 21.20±12.27 mm Hg (8.10-45.14 mm Hg); pulmonary incompetence (PI) was mild in 10 cases, moderate in 5 cases, and no severe PI occurred. Two cases of residual VSD were spontaneously closed. Compared with the early postoperative period, RVOT pressure and PI levels were not significantly different (Pgt;0.05). Right heart function was good.onclusion Early complete repair of TOF yields good surgical results. Transatrial repair of intracardiac pathology and retaining pulmonary valve annulus can be safely applied to yield good postoperative right ventricular function.
Abstract: Objective To analyze the surgical treatment of tetralogy of Fallot (TOF) with anomalous coronary artery (ACA) crossing the right ventricular outflow tract (RVOT), in order to improve the outcome of the disease. Methods The clinical data of 26 patients of TOF with ACA crossing the RVOT of Fu Wai Hospital from Oct.1996 to Feb.2006 were analyzed retrospectively. A double ventriculotomy superior and inferior to ACA were used in 11 patients, one ventriculotomy inferior to ACA were used in 6 patients and superior to ACA for 4 patients, 2 patients needed extra cardiac conduits, and 3 patients received other approaches. Results There were 2 operative death （7.7%）and no late deaths. Follow-up was extended 1 to 100 months, all of them had no residual ventricular septal defect(VSD) and their right ventriclepulmonary artery gradient were 27.3±15.6 mmHg. Conclusion Preoperative identification of ACA in patients with TOF is necessary. The surgeon should be careful in inspection of distribution of coronary artery during operation, and undergo the individualized surgical procedures based on the extent of RVOT obstruction and distribution of the ACA.
Objective To compare perioperative results between transventricular and transatrialtransventricular approaches in repairing tetralogy of Fallot (TOF), and to improve the surgical results. Methods The data of 1 423 consecutive patients who underwent complete repair of TOF between January 1998 and December 2007 were reviewed. 736 patients were repaired by the transventricular approach,and 687 patients by the transatrialtransventricular approach. Results Patients repaired by transventricular approach decreased from 100% in 1998 to 65% in 2002, and by transatrialtransventricular approach increased from 35% in 2002 to 79% in 2007. Aortic clamping time, cardiopulmonary bypass (CPB) time,mechanical ventilation time,and intensive care unit (ICU) stay in patients repaired by transatrialtransventricular approach had less than those in patients repaired by transventricular approach. No difference in transvalve patch ratio.There was lower morbidity in patients repaired by transatrialtransventricular approach in one to two organ systems dysfunction than that in patients repaired by transventricular approach. No difference in three or more organ systems dysfunction between them. Rate of residual ventricular septal defect(VSD), right ventricule to mean pulmonary artery (MPA) pressure gradient, tricuspid regurgitation, pulmonary artery regurgitation and arrhythmia in patients repaired by transatrialtransventricular approach were less than those in patients repaired by transventricular approach. Reoperative rate and mortality in patients repaired by transatrialtransventricular approach were less than those in patients repaired by transventricular approach. Conclusion TOF repair by the transatrialtransventricular approach fits to the actual conditions in China.
Abstract: Objective To investigate the method of improving effect, by investigating and analyzing the possible risk factors affecting shortterm outcome after total correction of tetralogy of Fallot (TOF). Methods Data of 219 patients who received total correction of TOF were divided into two groups according to the length of postoperative stay in hospital and recovery of heart function in the near future. Group A(n=110): patients had good recovery of heart function classified as gradeⅠorⅡ（NYHA classification）, and could smoothly be discharged from the hospital within two weeks without serious complications. The left ventricular ejection fraction (LVEF) had to exceed to 0.50 during 6 months followup visit. Group B(n=109): patients had worse recovery of heart function classified as grade Ⅱ or Ⅲ, and could not be discharged within two weeks with severe complications. LVEF was less than 0.50 during 6 months followup visit. The clinical data of two groups were compared, and risk factors affecting shortterm outcome after total correction of TOF operation were analyzed by logistic regression and model selection. Results There were good recovery of heart function classified as gradeⅠorⅡ(NYHA classification）in discharge, no death, and LVEF all exceeded to 0.50 in group A; there were 8 deaths in group B (7.34 %), and recovery of heart function was worse classified as grade Ⅱ or Ⅲ, with LVEF being less than 0.50（Plt;0.01）. Amount of postoperative daily thoracic drainage, assisted respiration time, time of inotropic agent stabilizing circulation, and the average length of postoperative stay in group A were all less or short than those in group B（Plt;0.01）. But the bypass and clamping time of group B were exceeded group A. The ratio of patching astride annulus in group B was greater than that in group A, and Nakata index was less than that in group A(Plt;0.01). The results of logistic regression and model selection indicate: age at repair (OR=0.69), oxygen saturation(OR=0.98), haematocrit before operation (OR=0.94), and patching astride annulus (OR=46.86), Nakata index (OR=16.90), amount of postoperative daily thoracic drainage (OR=0.84), presence of arrhythmia(OR=0.87), and wound infection(OR=63.57) have significant effect with shortterm outcome after total correction of TOF operation. Conclusions The probable methods to improving effect of shortterm outcome after total correction of TOF are an earlier age at repair, decreasing haematocrit, rising oxygen saturation before surgery, performing a palliative operation facilitating development of arteriae pulmonalis in earlier time, improving the surgical technique, and strengthening the perioperative care.
The aim of this study was to clarify characteristics of cardiovascular malformation in patients associated with tetralogy of Fallot (TOF) by using dual-source computed tomography (DSCT) angiography. We retrospectively analyzed DSCT angiography of 99 consecutive patients with TOF. In addition to typical CT features of TOF in all patients, the DSCT angiography showed 27 cases (27.27%) of atrial septal defect, 14 cases (14.14%) of patents ductus arteriosus, 11 cases (11.11%) of bicuspid pulmonary valve, 18 cases (18.18%) of congenital coronary artery malformation, 22 cases (22.22%) of right aortic arch, 12 cases (12.12%) of persistent left superior vena cava, 8 cases (8.08%) of retro-aortic innominate vein and 9 cases (9.09%) of pulmonary venous anomalous. DSCT is capable of displaying anatomical characteristics of cardiovascular malformation in patients with TOF.
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.