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 The earlyapplied atriumpulmonary connection has been replaced by total cavopulmonary connection.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.
ObjectiveTo review our clinical experience of modified Fontan procedure for complex congenital heart disease (CHD) in children. MethodsClinical data of 62 children with complex CHD who underwent modified Fontan procedure in Guangzhou Women and Children's Medical Center from May 2008 to December 2013 were retrospectively analyzed. There were 41 male and 21 female patients with their median age of 4 years(range, 16 months to 14 years) and body weight of 12.5 (8.9-49.5) kg. Diagnosis included functional single ventricle in 45 patients, transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) in 6 patients, corrected TGA with VSD and LVOTO in 6 patients, double outlet right ventricle with severe pulmonary stenosis in 4 patients, and right ventricular dysplasia in 1 patient. Previous procedure included pulmonary artery banding in 10 patients, unilateral bidirectional Glenn shunt in 37 patients, and bilateral bidirectional Glenn shunt in 8 patients. Seventeen patients received single-stage modified Fontan procedure, and 45 patients received two-stage modified Fontan procedure. Extracardiac conduit was used in 56 patients, and lateral tunnel was used in 6 patients. Concomitant procedures included fenestration in 41 patients, atrioventricular valvuloplasty in 6 patients, and pulmonary artery angioplasty in 3 patients. ResultsTwo patients in both single-stage and two-stage groups died postoperatively with mortality of 11.8% and 4.4% respectively (P=0.299). Postoperative mechanical ventilation time, length of ICU stay, chestdrainage duration, postoperative hospital stay and morbidity were not statistically different between single-stage and two-stage group. Mean follow-up was 2.0 ±0.5 years (range, 3 months to 5 years). There were 2 late death in the singlestage group but no late death in the two-stage group. Growth of all survival children was good, and their exercise capacity significantly improved. Percutaneous oxygen saturation was higher than 90%. Echocardiography showed patent superior and inferior vena cava anastomosis without thrombosis, stenosis, atrioventricular valve regurgitation aggravation or pulmonary venous return obstruction. All survival patients were in New York Heart Association class Ⅰ or Ⅱ. None of the patients had arrhythmia, chronic effusion or protein losing enteropathy. ConclusionEarly and mid-term results of modified Fontan procedure were satisfactory for children with complex CHD. For children with high risk factors, staged Fontan procedure can reduce surgical mortality.
ObjectiveTo investigate the effect and safety of the pulmonary vasodilators in pediatric patients after Fontan operation. MethodsThis retrospective study evaluated the clinical utility of pulmonary vasodilators in pediatric patients with Fontan surgery. Between January and December 2013, 42 consecutive patients with single ventricle physiology who underwent a modified Fontan procedure of total cavapulmonary collection (TCPC) were enrolled. After extubated oral intake started, 24 patients (the treated group) received the pulmonary vasodilator treatment, while 18 patients (the untreated group) didn't not receive the treatment. ResultsNo inpatient death occurred after surgery. The primary endpoints were time of stay in hospital and time of chest tube drainage. There was no statistical difference between the two groups. Instead, patients in the treatment group seemed to have longer time of hospital stay (22 to 21 days) and chest tube drainage (14.0 to 8.5 days) than those in the untreated group. Compared with the untreated group, patients in the treatment group were younger (P=0.082) with no statistical difference, and had higher postoperative Lac with statistic difference (P=0.031), longer ventilation time with no statistical difference (P=0.050), and lower postoperative oxygen saturation with statistic difference (P=0.065). No clinically significant adverse events relating to pulmonary vasodilator therapy occurred during this study and, in particular, no significant abnormalities in hepatic, renal function tests were observed in pediatric Fontan patients. ConclusionsPulmonary vasodilator agents were found to be particularly used in the patients with serious conditions in our study. Our study results failed to show significant improvement of pulmonary vasodilator drugs after Fontan surgery in decreasing time of pleural drainage and time of stay in hospital.
ObjectiveTo analyze the outcomes of patients with one-stage Fontan procedure and short-term prognosis at a single institute. Method We retrospectively analyzed clinical records of 116 patients with one-stage Fontan procedure in our hospital from January 2008 through September 2013. There were 77 males and 39 females (36%), with median age 6.27 years (ranged 3.15 to 17.47 years) at the time of surgery and the median weight at 17.5 kg (ranged 10.0 to 80.0 kg). There were 55 patients with standard extracardiac conduit, 13 patients with direct extracardiac connection, 17 patients with intra-atrial or intra/extracardiac conduit, and 31 patients with lateral tunnel. ResultsMedian cardiopulmonary bypass time was 124 (61-256) minutes. Median cross-clamp time was 60 (19-152) minutes. There were six early deaths (5.1%). The overall median time of the cardiac intensive care unit stay was 4 (1-17) days, with a median ventilator support of 7.3 (1.0-181.0) hours. The mean room air saturation was 90.00%±4.68% before discharge. Median length of chest tube drainage was 10 (4-45) days. Multiple logistic analysis confirmed that heterotaxy syndrome was the only independent predictor for postoperative renal insufficiency. Operations with aortic cross-clamping (OR=26.184, 95% CI 1.712-400.451), preoperative sinus mode dysfunction (OR=6.777, 95% CI 1.495-30.721) and cross-clamp time over 60 minutes (OR=1.036, 95% CI 1.002-1.076) were predictors for prolonged chest tube drainage. A total of 110 patients were followed up for 17 (8-47) months with 2 deaths and 1 with thrombosis. No reoperation occurred. ConclusionThe one-stage Fontan procedure can be performed with satisfactory outcomes. Staged strategies for operations may be appropriately loosen for selected elder children.
ObjectiveTo summarize the cardiac-vascular abnormalities and clinical results in patients with Fontan operation for heterotaxy syndrome. MethodWe retrospectively analyzed the medical records of 81 patients who underwent the Fontan operation with heterotaxy syndrome between September 2008 and September 2013. There were 49 male and 32 female patients at age of 3.79 (range 2.07-13.02) years with preoperative room air saturation of 81% (range 63%-97%) and weight of 14.8 (10.0-36.0) kg. ResultsThere were 70 patients in the right atrial isomerism group and left in 11 patients. Dextrocardia was seen in 16 patients, and single atrium in 50 patients. Single ventricle was found in 44 patients, conotruncal defects in 40 patients, pulmonary stenosis in 72 patients, pulmonray atresia in 8 patients, common atrioventricular valve in 61 patients, and bilateral superior vena cava in 54 patients. The staged Fontan procedure was applied in 57 patients and one stage in 24 patients. Operation strategies were included intra/extracardiac conduit (n=17), extracardiac conduit (n=48), lateral tunnel (n=14) and direct cavopulmonray connection (n=2).There were 15 early deaths. Postoperative complications included low cardiac output in 15 patients, hepatic insufficiency in 35 patients, renal insufficiency in 55 patients required peritoneal dialysis and arrhythmia in 28 patients. The room air saturation was 89% (range 78%-98%) before discharge and time of follow-up was from 2 months to 6 years in 64 patients. Thromboembolic events were found in 2 patients who had accomplished conduit replacement operation. ConclusionCompared with reported literatures in western countries, heteraotaxia patients are mostly with right atrial isomerism. Fontan palliation is still the main treatment option and strict indication was needed for satisfactory clinical results.
While the Fontan operation has improved the survival of a generation of children born with a functional single ventricle, it does not recreate a normal circulation. However, significant challenges remain. Early stage mortality risk seems stubbornly high. The risk of late cowplications seriously affect the longterm survival of children. As new techniques and therapies exist, more single ventricle patients survive till adulthood. Therefore, the limits of Fontan procedures is more and more evident. In recent years, the study of mechanical cavopulmonary assisting device, which addresses the limitations of Fontan circulation, has been developed and provided a more stable and effective biventricular of blood flow in the total cavopulmonary connection in existence. This would benefit not only the treatment of late Fontan complications, but also facilitating early surgical repair, which is promising.
The success of staged Fontan palliation for patients with single ventricle is related to low pulmonary vascular resistance (PVR). The complications of high PVR in Fontan physiology are numerous, such as low exercise tolerance, low cardiac output, ventricular function failure and protein-losing enteropathy; eventually it leads to failing Fontan. Therefore, a low PVR is crucial in Fontan patients. Now, targeted therapies decreasing PVR has been an advanced research hotspot in Fontan patients. In this review we present an overview of the safety and efficacy of the therapy with bosentan or sildenafil on elevated pulmonary artery pressure and pulmonary vascular resistance in Fontan patients.
Objective To investigate somatic growth of children undergoing stageⅡ Fontan procedure. Methods Clinical data of 152 children who underwent stageⅡ Fontan procedure in Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine between January 2012 and December 2015 were reviewed. There were 84 males and 68 females at age of 4-16 years. The weight and height of those children in each operative stage were assessed, and WHO Z-score was obtained to describe the pattern of growth of the children. Results Mean weight for age Z-score (WAZ) and height for age Z-score (HAZ) were –0.73 and 0.06 at birth, –1.27±1.37 and –1.27±1.72 before Glenn procedure, –0.47±1.08 and –0.69±1.17 before Fontan procedure, –0.42±1.18 and –0.39±1.48 at follow-up, respectively. A significant decline (P<0.05) in WAZ and HAZ was found before Glenn procedure. After the Glenn procedure, there was significant catch-up growth (P<0.05). The WAZ was stabilized after Fontan procedure, while the HAZ was reversed (P<0.05) continually. Conclusion The somatic growth is impaired in children with stageⅡ Fontan procedure. There is a trough of growth before Glenn procedure. Although catch-up growth occurs after Glenn procedure, low body weight and growth retardation remain at follow-up. Effective interventions such as more intensive nutritional strategies before Glenn procedure should be targeted at those children in the future, so as to improve their growth.
ObjectiveTo investigate the effects of a self-powered conduit in different patients’ models who underwent extracardiac Fontan procedure.MethodsFour children who underwent extracardiac Fontan procedure in Shanghai Children's Medical Center from 2011 to 2017 year were selected. Venae cavae and pulmonary arteries were reconstructed using Mimics 19.0®. In silico, a venturi conduit was introduced to the anastomosis of venae cavae and pulmonary artery. Then computational fluid dynamics simulation was performed using patients’ clinical data.ResultsWhen inferior venae cavae were directly to or to the left of superior venae cavae, the venturi conduit could assist the return of venous blood and reduce the pressures of venae cavae about 0.5 mm Hg. And the pressure differences between venae cavae and pulmonary arteries were about –0.7 mm Hg, which suggested that the conduit could generate right ventricle-like effect.ConclusionThe venturi conduit can reduce the pressure of venae cavae, increase pulmonary circulation flow and improve Fontan hemodynamics.