Pierre Viart

Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium

Objective: To assess long term outcome of patients who underwent Mustard or Senning repair for transposition of the great arteries up to 30 years earlier. Design: Retrospective review of medical records. Setting: The six university hospitals in Belgium with paediatric cardiology departments. Patients: 339 patients were reviewed, of whom 124 underwent the Mustard procedure and 215 the Senning procedure. This represents almost the entire population of patients in Belgium with either simple or complex transposition. Main outcome measures: Mortality, morbidity, functional abilities, social integration. Results: Overall mortality was 24.2%. Early mortality (⩽ 30 days after surgery) accounted for 16.5%, late mortality for 7.7%. Actuarial survival of early survivors at 10, 20, and 30 years after surgery was 91.7%, 88.6%, and 79.3%, respectively. Patients in the Senning cohort had a slightly better survival rate than those in the Mustard cohort (NS). Baffle obstruction occurred more often after Mustard repair (15.3%) than after the Senning procedure (1.4%). Arrhythmia-free survival did not differ between the two cohorts, but was determined by the complexity of the transposition. Survivors of the Senning cohort had better functional status, and tended to engage in more sports activities. Conclusions: The long term outcome for patients surviving the Mustard or Senning operation was favourable in terms of late mortality, morbidity, functional, and social status. Overall mortality in the Senning cohort did not differ from the Mustard group, but Senning patients had better functional status, greater participation in sports activities, and fewer baffle related problems.

Early results of valved bovine jugular vein conduit versus bicuspid homograft for right ventricular outflow tract reconstruction

BACKGROUND Homograft conduits are preferable for right ventricular outflow tract reconstruction in children, but their limited availability remains a major concern. Recently, a valve-containing segment of bovine jugular vein (Contegra, Medtronic Inc, Minneapolis, MN) has been introduced as a potential alternative conduit. METHODS Early clinical and echocardiographic results of right ventricular outflow tract reconstruction were retrospectively compared between 41 children (mean age, 1.9 years), receiving a Contegra conduit and 36 patients (mean age, 2.7 years) with a size-reduced pulmonary homograft. RESULTS Clinical outcome was comparable with two early deaths in the homograft group and one in the Contegra group. There were no conduit-related complications in either population. Early echocardiographic assessment showed only trivial to mild regurgitation in 9 homografts versus 17 Contegra conduits. The peak gradient across the right ventricular outflow tract conduit was comparable for both groups, although a larger number of patients, treated with a downsized homograft, had a small gradient at the distal junction with the pulmonary arteries (12 versus 6 patients). None of the patients had a gradient at the valvar level. CONCLUSIONS The valved bovine jugular vein conduit offers a promising substitute for right ventricular outflow tract reconstruction in infants and children, with an early hemodynamic performance that compares favorably with downsized, bicuspid homografts. Clinical advantages are greater shelf availability and the natural continuity between valve and conduit, which allows proximal infundibular shaping without additional material. However, durability must be determined, even though most of these children will require right ventricular outflow tract reoperation after outgrowing the conduit.

Cardiac surgery with extracorporeal circulation in 23 infants weighing 2500 g or less: short and intermediate term outcome

OBJECTIVE AND METHODS From September 1990 to February 1997, 23 consecutive critically ill infants (12 males, 11 females) weighing 2500 g or less underwent cardiac surgery necessitating extracorporeal circulation (ECC). A retrospective study was carried out to evaluate short- and intermediate-term outcome. Mean weight at operation was 2265 g (range 1750-2500 g). Mean age at operation was 24 days. The indications for surgery were transposition of the great arteries (TGA; 7), ventricular septal defect (VSD; 4), aortic stenosis (AS; 3), univentricular heart (UVH; 2), tetralogy of Fallot (TOF; 2), interrupted aortic arch (IAA; 2), atrial septal defect (ASD; 1), atrioventicular septal defect (AVSD; 1) and total abnormal pulmonary venous return (TAPVR; 1). All patients were in NYHA class IV; 17 patients (74%) were intubated pre-operatively. RESULTS The mean aortic cross-clamping time was 40 min. Twelve patients required deep hypothermia (<20 degrees C) with total circulatory arrest (mean duration 19 min). All patients were successfully weaned from extracorporeal circulation (ECC). Five patients left the operating room with an open sternum (mean duration before closure: 3.5 days). Mean duration of artificial ventilation was 10.6 days; of inotropic support 6.7 days and of intensive care stay 17.8 days. Severe complications were observed in 19 patients (83%): cardiac failure requiring high inotropic support (13), sepsis (7), and acute renal insufficiency (5). One patient needed a ventricular assist device. Five patients (22%) died in the intensive care unit (ICU): 2 AS with fibroelastosis, 2 IAA with VSD. and 1 UVH with pulmonary atresia. At discharge from the ICU, 7 patients were receiving no treatment. Mean duration of follow-up was 32 months (range 2-80 months). We had 2 reoperations: 1 for right ventricular outflow tract obstruction 1 year after a switch operation and 1 for mitral valve replacement 1 year after total abnormal pulmonary venous return repair (death 30 days post mitral valve replacement). Survival at I year was 73%. At the last clinical examination 16 patients were in NYHA class I. CONCLUSION Despite the severity of pre-operative cardiac disease, early surgical repair with ECC in infants weighing 2500 g or less is feasible with tolerable mortality yet with significant early morbidity.

Combined repair of upper sternal cleft and tetralogy of fallot in an infant

We report on a 2-month-old infant with the unusual association of an upper sternal defect and tetralogy of Fallot. Surgical correction of the cardiac disease consisted of closing the ventricular septal defect and relief of the right ventricular outflow tract by infundibuloplasty. The already less compliant bony thorax of the infant made direct approximation of the upper sternal defect only possible with adjuvant bilateral chondrotomies. Sternal cleft repair is advised during the very first weeks of life, during which complete correction of the rarely associated tetralogy of Fallot can be successfully performed.

Spectrum and workload of consultation activities in a Belgian tertiary paediatric cardiac centre

Abstract Background. Few data are available on the spectrum and frequency of issues addressed in the paediatric cardiology consultation service of tertiary academic hospitals. Methods. Those activities were collected prospectively during 6 months. Results. A total of 967 consultations were performed. The origin was mostly the medical ward (n=535), the intensive care unit (n=195), the neonatal unit (n=97), the operating room (n=84) and the nursery (n=44). In 553 cases, a heart disease was previously known and the most common reasons of consultation were postoperative (n=279) and preoperative evaluations (n=129). Thirteen newborns had a prenatal suspicion of congenital heart disease, which was confirmed in 11 cases. For the other 401 consultations, the most common clinical concerns included cardiac function in oncological disease (n=60), murmur (n=48), syncope (n=33), diabetes mellitus (n=28), prematurity (n=27), syndrome (n=19), unexplained stridor or respiratory distress (n=19) and unexplained fever (n=15). There were new diagnoses of congenital heart disease, acquired heart disease and arrhythmias in 35, 17 and 5 cases, respectively. Conclusions. The workload of the paediatric cardiology consultation service is increasing alarmingly. These data may be helpful in future planning of consultant manpower and in curriculum development in cardiac training of students and residents.

Association of a Cor Triatriatum Sinister and a right Partial Anomalous Pulmonary Venous Return. A case report

Abstract A 56-day old baby girl referred for recurrent respiratory tract infections and stridor, showed a rare association of a Cor Triatriatum Sinister (CTS) and a right Partial Anomalous Pulmonary Venous Return (PAPVR), in the form of a stenosed Scimitar vein, draining the right pulmonary veins to the subdiaphragmatic inferior vena cava (IVC), on echography and angiography. The baby underwent successful surgical repair of the CTS, by resection of the obstructive false membrane and reconnection of the Scimitar vein to the left atrium. At the age of 5 years, the child is doing well without any evidence of obstruction on the right or the left pulmonary venous drainage.

Cavopulmonary connection after repair of pulmonary vein stenoses

We report a case of heterotaxia with single-ventricle physiology undergoing successively pulmonary artery banding (PAB) at 8 months of age, repair of pulmonary vein (PV) stenoses at 15 months of age, and a fenestrated total cavopulmonary connection (TCPC) combined with repair of a regurgitant common atrioventricular (AV) valve and a Damus-Stansel operation for a restrictive subaortic ventricle at 5 years of age, followed by percutaneous closure of the fenestration. The child is now 10 years old and is, to the best of our knowledge, the only reported long-term survivor of a Fontan type procedure done after repair of PV stenoses.

Nonvalved homografts of thoracic aorta in operation for complex congenital cardiac disease.

BACKGROUND In contrast to the wide and successful use of valved aortic and pulmonary homografts, the non-valved prolongation of the thoracic aorta for the repair of some complex congenital heart diseases has rarely been described. We present here our experience with the use of descending aorta and aortic arch homografts as non-valved conduits for the surgical repair of complex cardiac malformations in 8 patients. METHODS One atriopulmonary conduit replacement four extraatrial cavopulmonary connections, and one intraatrial cavopulmonary repair were achieved by means of a homograft of the descending aorta with a diameter of 15 to 17 mm. Three pulmonary unifocalization procedures were carried out in 2 more patients using a curved homograft of the aortic arch with a diameter of 18 mm. Except for 1 patient, whose incompetent common atrioventricular valve was replaced with a mechanical prosthesis, all other patients were managed without anticoagulation. RESULTS No conduit-related complications were seen during a follow-up of 18 to 42 months. One patient died perioperatively during an emergency central repair of the right ventricular outflow tract after bilateral unifocalization with arch homografts. Another patient died suddenly 4 months after cavopulmonary connection. The remaining 6 patients are currently doing well. CONCLUSIONS Regardless of the complexity of the underlying malformations, we are encouraged to use nonvalved thoracic aortic homografts in the repair of congenital cardiac diseases because of the reduction in thrombotic, hemorrhagic, and infectious complications associated with their use; convenience in handling; and their versatility in size and shape.