Extracorporeal life support in pediatric patients with congenital heart diseases: outcome of a single centre.

AIM: In pediatric patients with congenital heart disease low cardiac output (LCO) is the principal complication after corrective heart surgery. In LCO refractory to all therapeutic options, mechanical circulatory support is the final method to keep these patients alive. In this present study the authors reviewed the outcome of pediatric patients who required mechanical circulatory support after corrective surgery with extracorporeal membrane oxygenation or ventricle assisted devices (VAD). METHODS: A retrospective single centre consecutive cohort study was carried out in children who required different mechanical circulatory support indicated by postcardiotomy low output syndrome between 1991 and 2004. A total of 20 patients received extracorporeal life support. The indications for surgery were: 12 transposition of great arteries, 1 Bland-White-Garland syndrome, 3 tetralogy of Fallot, 1 hypoplasia of aortic arch, 1 total anomalous pulmonary vein connection, and 2 ventricle septum defect. RESULTS: Mean age was 1.29 years. Mean duration of assist was 8.87 days. Seven patients out of 20 survived, six could be discharged after myocardial recovery from LCO and one could be discharged after successful heart transplantation. The overall mortality in patients with extracorporeal life support was 65%. The causes of death were multiorgan failure and bleeding in one case was a VAD related complication. CONCLUSION: The use of extracorporeal life support (ECLS) shows a high mortality rate. However, ECLS can still help to keep some of those patients alive. Mechanical support devices are the ultimate chance to save time, to increase survival and to bridge the time until heart transplantation.

European Contegra multicentre study: 7-year results after 165 valved bovine jugular vein graft implantations.

OBJECTIVE: The valved bovine conduit "Contegra" for RVOT reconstruction became available for clinical use within a 100 % source data monitored and echo core lab controlled prospective European Multicentre Study, carried out from 1999 to 2006. We present the results of this study. METHODS: A total of 165 Contegras were implanted in 8 centres. The mean patient age was 3.9 years (2 days - 18 years, median 2.0). Total follow-up was 687 patient years. Diagnoses included: tetralogy of Fallot (64 patients, 39 %), truncus arteriosus (50, 30 %), double outlet right ventricle (16, 10 %), aortic valve disease/Ross procedure (11, 7 %), pulmonary valve atresia (10, 6 %), transposition of the great arteries (10, 6 %), 4 other malformations (2 %). Previous procedures were: 82 patients (50 %) - none; 37 (22 %) - valved conduit implantation; 14 (8 %) aortopulmonary shunt; 6 (4 %) catheter intervention. Follow-up appointments which included standardised echocardiography investigations were scheduled at 1, 3, 6, and 12 months, then annually. We evaluated freedom from death, explantation, intervention, stenosis, insufficiency, and degeneration. Results were stratified by age, diagnosis group and conduit size. RESULTS: The 5-year freedom-from rates were: explantation - 90 % (for patients aged 1 to 10 years) and 68 % (for younger patients); endocarditis - over 92 %; catheter intervention - 74 % (patients with congenital malformations); stenosis - 75 % and more (any group); insufficiency - 50 % (12 and 14 mm diameter conduits); any event - 13 % (patients under 1 year), 58 % (1 to 10 years), 82 % (> 10 years). Trace or mild insufficiency was a frequent, but not progressive finding. Mild calcification was detected in only 8 examinations. CONCLUSIONS: The performance of the Contegra conduit compares well with that of homografts when used to reconstruct paediatric right ventricular outflow tracts.

Pediatric patients with Kawasaki disease and a case report of Kitamura operation.

Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is an acute, self-limiting, small-vessel vasculitis with an unknown cause that affects children between the ages of 6 months and 5 years. It is the most common cause of acquired coronary artery disease in childhood. Acute myocardial infarction and coronary artery aneurysm are major complications. We present a cohort of patients with KD who were followed up and treated in the Heart Center, North Rhine-Westphalia. Included is a review of important relevant items common to cases of KD, such as clinical data and management, including medical management of the acute condition and the diagnosis and management of coronary vasculitis and aneurysms as well as the application of coronary artery bypass grafting (CABG) in those conditions. Between January 2002 and January 2006, we evaluated the findings and characteristics of 18 pediatric patients with a history of KD and their long-term outcome. The acute illness occurred between the ages of 4 months and 14 years of age. Anomalies of the coronary arteries were found in 6 patients ranging in age from 5 months to 10 years. One patient had acute myocardial infarction; another underwent CABG after 5 years from disease onset at the age of 15 years. Kitamura operation was performed successfully. The other patients are still under observation.Coronary artery aneurysms and stenosis requiring surgery are rare in KD; nevertheless, CABG is the standard therapy when myocardial ischemia is detected. Kitamura operation provides good growth potential and long-term graft patency.

Mechanical valve replacement of the systemic atrioventricular valve in children.

In children with systemic atrioventricular valve disease (SAVVD), reconstructive surgery is the primary goal. However, in cases with severely dysplastic valves or failed repair, valve replacement is the only option. The purpose of this study was to assess the early and late outcome following mechanical valve replacement in SAVVD. Between 1989 and 2003, 31 children underwent mechanical valve replacement (19 St. Jude Medical, 12 Carbomedics) in SAVVD (27 mitral, 3 tricuspid in corrected transposition of the great arteries, 1 common in an univentricular heart) at our institution. The ages ranged from 3 months to 15 years (mean 4 years) and body weight varied between 4.2 and 57 kg (mean 13.3 kg). The size of prostheses ranged between 16 and 31 mm (mean 23.9 mm). The main indication for valve replacement was severe insufficiency of left atrioventricular valve (84%); 84% of the patients had had a previous cardiac operation. The overall hospital mortality was 6.5% The mean follow up was 7.7 years (range 2-13 years). Ninety percent of children represent sinus rhythm, 87% are in NYHA class I. All patients were placed on a regimen of Phenprocoumon aiming to maintain the international normalized ratio (INR) between 3.0 and 4.0. Since 1994, INR self-management of oral anticoagulation was performed either by the patient or his or her parents. There was no anticoagulation-related complication in this patient group. Mechanical valve replacement in left atrioventricular valve disease carries a low operative risk across the spectrum of pediatric age despite previous operations in most cases. Long-term survival and quality of life are good in nearly all cases. The rate of anticoagulation-related complications is very low, especially when INR self management is performed.

Heart transplantation in children after mechanical circulatory support: comparison of heart transplantation with ventricular assist devices and elective heart transplantation.

Heart transplantation (HTx) is an ultimate treatment for children with end-stage heart failure or inoperable congenital heart disease. The supply of hearts is inadequate; therefore, different mechanical support systems must be used as bridge to HTx in pediatric patients with postoperative low output. The use of ventricular assist devices (VADs) as bridge to HTx in children is limited because of size differences. The purpose of this study was to evaluate the overall long-term outcome of pediatric circulatory support before pediatric HTx. From 1989 through 2004, 91 pediatric patients underwent isolated HTx. Seven of them required mechanical support before transplantation. We reviewed retrospectively the course of 91 children (mean age 14.7 years) who underwent HTx. Group A consisted of elective HTx patients who were treated as outpatients before HTx, whereas group B was the VAD-HTx bridging group (n=7; mean age 12.31 +/- 2.8 years). Mean duration of VAD support was 108 +/- 98 days (minimum 1 day, maximum 258 days). Overall survival rate after HTx was 80% at 1 year without significant differences between groups. Five of seven patients survived and could be discharged after successful HTx, for a survival rate of 77%. The mean follow-up period was 16.76 +/- 10.6 months. No differences in posttransplantation long-term survival and rejection episodes occurred between patients transplanted with or without VAD. VAD therapy can keep pediatric patients with end-stage heart failure alive until successful HTx, and bridge to HTx is a safe procedure in pediatric patients. After HTx, survival rates of these children are similar to those of patients awaiting elective HTx.

Mechanical valve replacement in congenital heart defects in the era of international normalized ratio self-management.

As the number of patients with congenital heart defects requiring heart valve replacement increases, the need for durable valve substitutes with good hemodynamic performance and a low incidence of complications becomes more apparent. The use of porcine xenografts is burdened with early fibrocalcific degeneration, whereas the use of mechanical heart valves led to an increased number of thromboembolic events, especially when implanted in the right side of the heart. We report on our experiences implanting bileaflet heart valves in congenital heart defects since the introduction of international normalized ratio (INR) self-management. The data of 68 long-term survivors (33 males, 35 females) who underwent mechanical heart valve replacement in congenital heart defect were reviewed. Patient age at the time of valve replacement ranged from 5 months to 61 years (mean 21 years). Underlying diagnoses were tetralogy of Fallot (n=33), morbus Ebstein (n=4), atrioventricular canal (n=13), truncus arteriosus communis (n=5), transposition of the great arteries (n=10), and congenitally corrected transposition of the great arteries (n=3). In all patients, bileaflet valves were implanted (St. Jude Medical n=40, Carbomedics n=28). Anticoagulation was performed using dicumarol (Marcumar) and INR self-management in all cases. The mean follow-up was 72 months (range 6-132 months; 409 patient-years). Valve thrombosis developed in 3 of 68 patients (4.4%, all three had tetralogy of Fallot, mean age 9.8 years) after a mean follow-up of 3.5 years. In two of these three patients, re-pulmonary valve replacement was necessary, whereas the third patient was treated by thrombolysis. From our experience, we conclude that mechanical heart valve replacement is a good therapy option with a low complication rate for patients with congenital heart defects requiring valve replacement, especially when INR self-management is performed.

Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations.

OBJECTIVE: From October 1989 to June 1998, 60 patients have undergone the Norwood procedure (stage I) at our institution. The results of the staged surgical reconstruction and risk factors were analysed. Typical hypoplastic left heart syndrome (HLHS) and complex lesions with aortic hypoplasia were compared with each other. PATIENTS: Typical HLHS: N = 48, median age 15 days (5-190 days), median weight 3.6 kg (2.6-5.3 kg). Complex lesions (dominant left ventricle): N = 12, median age 59 days (10-884 days), median weight 3.4 kg (2.4-12 kg). RESULTS: Typical HLHS: The stage-I hospital survival was 73% (35/48). It improved from 60% (95% confidence interval: 49-71%) during 1989-1994 to 91% (95% CI: 81-100%) during 1997-1998. Seven patients were lost late. The right ventricular end diastolic diameter (P = 0.015), shortening fraction (P = 0.027), and the presence of an obstructed pulmonary venous return (P = 0.0032) were significant risk factors. 23 children underwent stage-II operation with four (17%) deaths. All survivors experienced an improvement of their statomotoric development. Stage-III operation was performed in 13 patients with no hospital death. Follow up after stage-III procedure was 7 months to 7 years. 4 year actuarial survival, including hospital mortality and deaths at subsequent stages, improved from 28% (95% CI: 18-38%) during 1989-1994 to 58% (95% CI: 48-68%) during 1994-1997. No patient had signs of myocardial insufficiency. Complex lesions: Stage-I hospital survival was 83% (10/12) with no late death. Stage-II was performed in 8 and stage-III in 6 patients with no death respectively. CONCLUSION: In typical HLHS the results have improved over time. Both size and function of the right ventricle determined significantly stage-I survival. An early operation prevents the natural progression of pulmonary blood flow and may weaken all three risk factors. Patients with complex lesions seemed to have better chances of surviving the early postoperative period. The multistage reconstructions have become a realistic option for patients with HLHS and similar lesions, regardless of the morphologic subtype or diminutiveness of the aorta, and lead to an acceptable quality of life.