Hugues Dessy

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.

The effect of bosentan in patients with a failing Fontan circulation.

OBJECTIVES To investigate the effect of bosentan in patients with a failing Fontan circulation. DESIGN A multicentric open label, non-controlled study. SETTING 5 tertiary care centres for congenital cardiology. PATIENTS We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks. MAIN MEASURES OF OUTCOMES: We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded. RESULTS Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued. CONCLUSIONS Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

Echocardiographic and Radionuclide Pulmonary Blood Flow Patterns After Transcatheter Closure of Patent Ductus Arteriosus

BACKGROUND Transcatheter occlusion of patent ductus arteriosus (PDA) has been associated with protrusion of the occluder device into the left pulmonary artery (LPA). This study was conducted to evaluate the significance of occluder protrusion and its implications for potential obstruction of the LPA and associated decrease of left lung perfusion. METHODS AND RESULTS Fifty-two patients underwent successful transcatheter PDA occlusion over a period of 5 years. In this study, 49 were reexamined between March and June 1995. In addition to clinical and echocardiographic examination, lung scintigraphy was performed. Protrusion into the LPA was present in 5 of 49 patients (10%). In these patients, maximal flow velocity in the LPA was significantly (P < .01) increased. Decreased left lung perfusion, defined as < 40% of total pulmonary blood flow, was found in 7 of 49 patients (14%). Although mean left pulmonary perfusion was significantly (P = .02) decreased in patients with protrusion, there was considerable overlap with patients without protrusion, and only a weak correlation was found (r = -.35, P = .01) between flow-velocity and left lung perfusion. CONCLUSIONS Our results demonstrate that protrusion of the device in the LPA is an infrequent finding. If present, it is associated with increased maximal flow velocity in the LPA and diminished left lung perfusion. However, echocardiography and lung scintigraphy are weakly correlated: Increased maximal blood flow velocities in the proximal LPA proved to be a poor indicator or impaired left lung perfusion. Also, decreased perfusion occurs in the absence of echocardiographic evidence of device protrusion.

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.

Epidemiology of heart failure in a tertiary pediatric center

In contrast to the adult age group, epidemiologic studies on heart failure (HF) in the pediatric population are lacking. The aim of this prospective study was to analyze the epidemiology of HF during infancy and childhood.

Noncardiac comorbidities of congenital heart disease in children

Background: Noncardiac conditions may complicate heart disease care, contribute to the progression of the disease and alter the response to treatment. The aim of this prospective study was to evaluate the proportion of cardiac children with such significant conditions.

Virtual autopsy by computed tomographic angiography of the fetal heart: a feasibility study

To determine the feasibility of postmortem computed tomographic (pm‐CT) angiography for fetal heart evaluation.

Delayed recognition of congenital heart disease

Objective: The aim of this prospective study was to evaluate the proportion of children with delayed recognition of congenital heart disease (CHD). Methods: Of the 744 children with CHD primarily diagnosed during a 10 year period in one hospital, the patients were identified where the diagnosis of CHD was established with a significant delay. Results: Sixty six patients (8.9%) had delayed diagnosis of CHD. Among patients with cyanotic CHD, 10.4% (7 of 67 cases) were referred after they had initially been discharged home from the birth clinic. Among patients with acyanotic CHD, 8.7% (59 of 677) of all children and 35.1% (59 of 168) of the children who required surgery or interventional catheterisation were referred at an age where elective repair should have already been performed or needed immediate treatment because of their haemodynamic status. Of the 66 patients with delayed diagnosis, one infant with cor triatriatum died at admission because of delayed referral and 10 children had severe complications: preoperative cardiogenic shock in seven cases of aortic coarctation and one case of endocardial fibroelastosis, pulmonary hypertensive crisis in one child after delayed repair of a ventricular septal defect, and infectious endocarditis after dental care in a teenager with undiagnosed moderate aortic stenosis, who required Ross operation a few months later. Discussion and conclusion: A substantial proportion of CHD was detected with relevant delay. In all cases of late diagnosis, clinical cardiac findings were present that should have alerted the physician on the possible presence of underlying CHD.

Magnetic resonance imaging in the normal fetal heart and in congenital heart disease

To evaluate prospectively the feasibility of magnetic resonance imaging (MRI) for assessment of the fetal heart for congenital heart disease (CHD).

Percutaneous transhepatic access for ablation of atypical atrial flutter in complex congenital heart disease.

A 12-year-old boy with complex congenital heart disease presented with symptomatic incessant atrial flutter resistant to antiarrhythmic medication. He had 5 previous surgical interventions for (1) a single left ventricle, (2) D-transposition of the great vessels, and (3) interruption of the aortic arch type C. As a result, he had a complete aorto-bifemoral reconstruction, an atrioseptectomy, and a complete cavopulmonary derivation with fenestration to the right atrium, which was subsequently closed by an Amplatz umbrella device for poor oxygen saturation (with a residual cavo-atrial communication). Due to previous surgical interventions and a thrombosis of the right internal jugular vein, he had no transcutaneous venous access except the left internal jugular vein and the hepatic vein. Percutaneous transhepatic access was obtained using a needle positioned below the right costal margin and guided with ultrasound and contrast visualization of the hepatic vein (Figure 1A and B). A single 3.5 mm externally irrigated-tip ablation catheter (Navistar, Biosense Webster, Diamond Bar,