Cardiac performance assessment during cardiopulmonary exercise test can improve the management of children with repaired congenital heart disease.

BACKGROUND: Children with repaired congenital heart disease (CHD) have impaired maximal aerobic capacity (VO2max). Determining the causes of their VO2max alteration remains challenging. Cardiac output measure using thoracic impedancemetry during cardiopulmonary exercise tests (CPET) can help to understand the determinants of VO2max in children with open-heart repaired CHD. METHOD: We analyzed CPET in 77 children with repaired CHD. Among them, 55 patients had residual lesions. Patients with repaired CHD were compared with 44 age-matched healthy individuals. Maximal oxygen content brought to capillaries (QO2max) and oxygen muscle diffusion capacity (DO2) were assessed using cardiac output measure, Fick principle and simplified Fick law. RESULTS: In the 55 patients with residual lesion, VO2max, QO2max and DO2 were lower than those of controls (76.1 vs 86% of theoretical value, p < 0.01; 2.15 vs 2.81 L/mn, p < 0.001; 24.7 vs 28.8 ml/min/mmHg, p < 0.05). Decrease in QO2max was due to both impaired stroke volume and chronotropic insufficiency (48 vs 53 ml/m2 and p < 0.05; 171 vs 185/min p < 0.001). Patients without residual lesion (22/77) had normal VO2max with lower maximal heart rate compensated by higher SV (p < 0.05). CONCLUSION: Aerobic capacity was normal in children without residual lesions after CHD repair. Patients with residual lesion have impaired VO2max due to both lower central and peripheral determinants. Measuring cardiac performance during CPET allowed a better selection of patients with altered cardiac reserve that can benefit from residual lesion treatment and find the good timing for intervention. Detection of peripheral deconditioning can lead to a rehabilitation program.

[Transfer of cardiac catheterisation from a paediatric to an adult cardiological centre: results at 3 years]. / Transfert de l'activité de cathétérisme cardiaque d'un centre pédiatrique à un centre de cardiologie de l'adulte: trois ans d'activité

The advances of surgical and interventional treatment of congenital heart diseases have allowed a large number of patients with congenital heart disease to reach adult age. This population involves almost 0.3/1000 of total population in West Europe and North America and can be estimated around 200000 patients in France. Patients with operated Tetralogy of Fallot, benign forms of pulmonary atresia with ventricular septal defect, simple or complex transposition of the great arteries usually survive beyond childhood. These patients can need repeated interventions to treat lesions of native or reconstructed pulmonary arteries and/or aortic arch, to occlude residual shunts, to treat pulmonary incompetence. More complex heart diseases such as single ventricle, rarely allow survival until the adult age. The majority of these patients undergo heart transplant, often made difficult by multiple cardiac surgeries, anomalies of pulmonary arteries, chronic cyanosis, aorto-pulmonary shunts. Patients with relatively simple or complex congenital heart diseases need to be followed-up in specialized units, like those created more than twenty years ago in the United States, Canada, and United Kingdom. Interventional cardiac catheterisation play a major role in the management of this population. The results of 3 years of activity in a new centre treating GUCH patients are illustrated.

Stroke after surgical treatment of sinus venosus type atrial septal defect: percutaneous treatment.

BACKGROUND AND PURPOSE: Sinus venosus-type atrial septal defect can be associated with anomalous drainage of the upper right pulmonary vein into the superior vena cava. Surgical correction provides reconstruction of superior vena cava and rerouting of the pulmonary vein into the left atrium. SUMMARY OF CASE: We report the case of a young woman who experienced 2 strokes 11 years after surgical repair of sinus venosus-type atrial septal defect. CONCLUSIONS: Interventional treatment resolved the superior vena cava stenosis, rerouted the pulmonary vein into the left atrium and abolished the right to left shunt.

Bosentan for the treatment of pulmonary arterial hypertension associated with congenital heart defects.

BACKGROUND: Bosentan is an effective first-line therapy in New York Heart Association (NYHA) III patients with idiopathic pulmonary arterial hypertension (PAH). Pre-clinical data support the rationale for the potential benefit of bosentan in PAH associated with congenital heart disease (CHD). MATERIALS AND METHODS: We performed a retrospective analysis of patients with PAH-associated CHD who were treated with bosentan on top of conventional therapy. Bosentan was started at 62.5 mg bid for 4 weeks, then titrated to 125 mg bid. New York Heart Association (NYHA) functional class, 6-min walking distance (6MWD), Borg dyspnoea index, arterial oxygen saturation and cardiopulmonary haemodynamic data (cardiac output, pulmonary blood flow and systemic and pulmonary vascular resistances) were collected at baseline and at follow up. RESULTS: Twenty-seven patients (23 females, mean 35 +/- 15 years) with NYHA class III-IV PAH-associated CHD (not repaired in 23 cases) were treated with bosentan for a mean 18.3 +/- 9.9 months. Bosentan improved 6MWD from 298 +/- 92 m at baseline to 355 +/- 82 m at 3 months (P = 0.0002) and to 364 +/- 92 m (P = 0.0001) at the last follow up (mean 15.2 +/- 9.7 months). At the last follow up, 13 patients had improved (= 1 NYHA class) and 14 remained stable. A favourable effect was observed in pulmonary blood flow and pulmonary vascular resistance for the 11 available patients. No change in pulse oximetry or liver enzyme elevation was reported. CONCLUSIONS: Bosentan improves exercise capacity, functional class and haemodynamics in most patients with PAH-associated CHD, without serious side-effects, suggesting bosentan may be an important treatment option for these patients.

[Arteriovenous fistula of the renal vessels due to septic embolism in a patient with bacterial endocarditis]. / Fistule artério-veineuse des vaisseaux rénaux par embolie septique au cours d'une endocardite bactérienne.

A 27 year-old woman developed acute pain of the right flank during the course of an infectious endocarditis. A septic arteriovenous fistula of the renal vessels of a solitary functioning kidney was demonstrated. The cardiac valvular lesions required a prosthetic aortic and mitral replacement valves. An attempt to occlude the fistula by embolization with several coils was unsuccessful and led to surgery: extracorporeal repair enabled complete closure of the fistula. During the long-term follow-up, the aortic prosthetic valve had to be changed. Renal function was satisfactory and remained stable over time. Renal arteriovenous fistula is an exceptional complication of bacterial endocarditis despite the frequency of septic emboli during the course of the disease.

[Coronary fistula in tetralogy of Fallot: a rare complication of embolisation]. / Embolisation d'une fistule coronaire chez une patiente opérée d'une tétralogie de Fallot: survenue d'une complication rare.

The authors report the spontaneous detachment of a coil during embolisation of a coronary fistula in a 34 old year woman with operated tetralogy of Fallot. The fistula originated from an isolated circumflex artery and drained into the infundibulum. By retrograde approach, a microcatheter was successfully advanced into the distal part of the fistula, one coil loop was fixed through the fistula mouth and two in its distal portion. During positioning of the proximal part of the coil, it suddenly detached into the microcatheter. Coaxial snaring of the coil was attempted, however this manoeuvre produced progressive elongation and repeated ruptures of the coil. Finally, the procedure was abandoned; surgical extraction of the coil and suture of the fistula were successfully performed. Percutaneous transcatheter closure of coronary fistulae, although generally feasible is burdened by complications due to tortuous anatomy, poor stabilisation of catheters and lack of specific materials.

[Percutaneous pulmonary valve replacement: towards a modification of the prosthesis]. / Remplacement valvulaire pulmonaire par voie percutanée: vers une modification de la prothèse.

Percutaneous pulmonary valve replacement has recently been introduced as an alternative to surgery, pushing back the frontiers of interventional catheterisation. The authors report their experience in this field and focus on the complications of the method. From September 2000 to November 2002, 14 patients with a failing artificial pulmonary outflow tract were included in this protocol of clinical research. The authors attempted pulmonary implantation of a bovine jugular vein valve fitted in a vascular stent under general anaesthesia. The implantation was possible in all patients. Three had dynamic obstruction due to the implanted valve. This was due to infiltration of blood between the valve and the stent. The problem was resolved by: implanting a second valve in one patient: redialating the valve stent in two other patients. However, the last patient had to be reoperated 7 weeks after the implantation because of recurrence of the obstruction. One valve had to be explanted one year after implantation because of severe infectious endocarditis after unprotected dental treatment. Two patients experienced fracture of the stent but without valve dysfunction. Despite these complications, the clinical state of all patients improved during follow-up. The authors conclude that non-surgical pulmonary valve replacement is feasible, thereby increasing the therapeutic arsenal of interventional catheterisation. Simple modifications should result in fewer problems of dynamic obstruction and stent fracture, and lead to proposing this technique for native pulmonary outflow tracts.

[Cyanotic heart disease in the adult]. / Les cardiopathies cyanogènes de l'adulte.

Patients with cyanotic heart disease may have an acceptable quality of life but are exposed to several complications: polycythaemia, often beneficial but with its risks: hyperviscosity, hyperuricaemia, thrombocytopaenia, blood clotting abnormalities; and the other complications of right-to-left shunt: cerebral abscess, cerebral embolism, endocarditis. The hypoxia may be improved by interventional catheterisation or palliative surgery. The Eisenmenger syndrome is life-threatening in pregnancy or during general anaesthesia. These patients are at risk and sometimes have iatrogenic complications, so usual cardiological treatment may be dangerous: diuretics, ACE inhibitors, oral anticoagulants, antiarrhythmics.

[Followup of adults with a Fontan-type cavopulmonary derivation]. / Surveillance d'un adulte ayant eu une dérivation de type Fontan ou cavopulmonaire.

The Fontan procedure is a technique used in single ventricle syndromes and initiates a circulation which bypasses the right ventricle. It was described by Fontan in 1971. Many variations of this operation have been described and classified under the term of total cavopulmonary derivation. These operations result on long-term functional benefit. However, the 15 year survival rate is only 55 to 80%. This procedure (often carried out in childhood) is considered to be palliative rather than curative. The mortality is due to long-term complications, the frequency of which increases with the length of follow-up of the patient. The causes are: the Fontan circulation itself (arrhythmias, thrombosis, inferior caval stasis (hepatic, exsudative enteropathy), recurrence of cyanosis): the underlying cardiac disease (ventricular failure, atrioventricular valvular regurgitation); other complications (endocarditis and extracardiac, in particular orthopedic problems). Any of these complications may be an indication for cardiac transplantation, a high risk procedure because of previous multiple surgical interventions.

[Baloon angioplasty with stent implantation in recoarctation of the aorta: an attractive alternative]. / Dilatation percutanée suivie d'implantation de stent dans les recoarctations de l'aorte: une alternative séduisante.

The results of transcatheter balloon angioplasty in teenagers and adults with aortic recoarctation are uncertain. Therefore, there is a current trend to prefer a more complex procedure including the implantation of a stent. This study deals with 8 patients aged 7 to 25.3 years (median: 15 years), weighing 20 to 68 kg. (median: 57) and having undergone resection of an aortic coarctation during infancy (24 days to 4 years). All had their lower limb pulses diminished or abolished, elevated blood pressure at rest (and at exercise in the 5 tested patients), and left ventricular hypertrophy. MRI documented the lesion and helped to select seven patients whose stenosis was short and remote enough from the origin of the main aortic collateral. In one case, the decision to stent was taken as an emergent measure to treat an aortic dissection which appeared shortly after balloon dilatation. The effectiveness of the procedure was immediate in all patients with a 50% increase in diameter of the dilated area, total relief of the gradient, drop to normal values of the blood pressure. These good results persisted at follow-up (3-24 months) in 6 patients, with moderate hypertensive rebounds in the last 2. There were 2 technical problems (premature burst of the balloon, asymmetrical inflation of the stent like an "Eiffel Tower") that could finally be overcome and should no longer occur with the new specially designed so-called "BIB" balloons. Would long term follow-up confirm these early results, one should conclude that this method offers an attractive, safe and effective option to surgery for adolescents and adults with late recoarctation of the aorta.

[Quality of life of adults with univentricular hearts]. / Qualité de vie des adultes atteints de coeur univentriculaire.

This study sought to evaluate the quality of life of surviving adults with univentricular heart and to determine the impact of sociodemographic and clinical characteristics on quality of life. The health records of 89 survivors with univentricular heart living in France at the time of the study were reviewed. They were 17 to 49 years old (median: 21). Sixty-seven patients answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The scores of patients who answered the questionnaire were compared with national norms. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. The Duke scores of adults with univentricular heart were similar to those of the normal population. Cyanosis predicted a worse score for physical (p = .05) and perceived health measures (p = .02). The higher educational level predicted a better score for physical (p .004), mental (p = .01), and general health measures (p = .02). Orthopaedic problems worsened social score (p = .05). Psychosocial problems worsened pain score (p = .04). Mitral atresia, in comparison with the other anatomical types, worsened perceived health score (p = .02). Finally, patients younger than 23 years scored better for almost all of health and dysfunction measures. In conclusion, despite repeated interventions and other disease-related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life. These patients probably developed copying mechanisms, easing the psychological stress, and oriented themselves towards a different set of values in everyday life so that to accept disabilities and to recalibrate personal expectations.

Quality of life and perceived health status in surviving adults with univentricular heart.

OBJECTIVE: To evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics. DESIGN AND SETTING: Retrospective, cross sectional study conducted in a regional paediatric cardiology centre. PATIENTS: The health records of 89 survivors with univentricular heart (median age 21 years; range 17-49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. RESULTS: The Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures. CONCLUSIONS: Despite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life.

Impaired cardiac adrenergic innervation assessed by MIBG imaging as a predictor of treatment response in childhood dilated cardiomyopathy.

OBJECTIVE: To evaluate the prognostic value of metaiodobenzylguanidine (MIBG) imaging in childhood cardiomyopathy. DESIGN: Prospective cohort study. SETTING: Tertiary referral centre. PATIENTS: 40 children (21 boys, 19 girls; mean (SD) age, 7.0 (5.6) years) with heart failure resulting from idiopathic dilated cardiomyopathy (n = 23) or various other disorders (n = 17). METHODS: At the initial examination, cardiac (123)I-MIBG uptake and release, circulating noradrenaline (norepinephrine) concentration, x ray cardiothoracic ratio, and echocardiographic variables were recorded. Cardiac MIBG uptake was obtained by measuring the heart to mediastinum activity ratio on the planar image obtained four hours after MIBG injection. MIBG washout rate was evaluated using relative decrease in cardiac activity measured at 20 minutes and four hours. Patients were treated with angiotensin converting enzyme inhibitors, diuretics, and digitalis, and were followed up for 12 (10) months. Fifteen patients did not respond to medical treatment (12 heart transplants; three deaths), and 25 did respond (improved or stable). RESULTS: Cardiac MIBG uptake was positively correlated with x ray cardiothoracic index (r = 0.55, p = 0.0008) and echocardiographic left ventricular fractional shortening (r = 0.68, p < 0.0001). Among all the clinical and laboratory variables tested, multivariate discriminant analysis showed that the only independent predictor of an unfavourable outcome was a low MIBG uptake (p < 0.001). Survival curves had a mean threshold value of 1.54 for MIBG uptake. CONCLUSIONS: Impaired cardiac adrenergic innervation is strongly related to adverse outcome in children with dilated cardiomyopathy, independently of the aetiology. MIBG imaging may help to stratify risk in such patients.

[Stenosis of the superior caval canal after Mustard and Senning procedures: treatment by dilatation and stent implantation. Two case reports]. / Sténoses du chenal cave supérieur après interventions de Mustard et Senning: traitement par dilatation et implantation d'endoprothèses vasculaires. A propos de deux observations.

The authors report two cases of stenosis of the superior caval canal after Mustard and Senning procedures for transposition of the great arteries in patients paced for atrial arrhythmia. During cardiac catheterisation, it was possible to treat the stenosis by perforation in one case and by balloon dilatation in the second, followed by the implantation of two stents to reestablish vascular patency. In one patient, two pacing catheters were implanted by an endovascular approach without complications three months after stenting; in the other case, epicardial pacing was required because the patient's condition could not wait for endothelialisation of the stent before implanting the pacing catheters. This technique of revascularisation may also be used in children with venous stenosis after implantation of endocavitary pacing catheters, in which the pacing system has to be changed.

[Therapy and prognosis of infectious complete atrioventricular block in children]. / Traitement et pronostic du bloc auriculo-ventriculaire complet infectieux de l'enfant.

From 1983 to 1997, we have studied ten children with complete atrioventricular block likely due to myocarditis in order to assess its prognosis and to define a therapeutic strategy. Their age ranged from 6 days to 16 years (median: 4.1 years). All were admitted for sudden complete block, with symptoms in seven: syncope or fainting, seizures, collapse. Three had an asymptomatic bradycardia which was detected on routine auscultation in children with fever or already hospitalized; fever was present in 5. The disease was related to infection on biological data in 4 cases (1 listeriosis and 3 seroconversions for Epstein Barr or cytomegalic or Coxsackie B viruses), on a myocardial biposy in 1 case and on scintigraphic data in 1 case. In the remaining 4, indirect arguments were considered such as infectious context, normal recent ECG, favourable outcome. Five children were given intravenous isoprenalin with ventricular tachycardia in 3. Five were treated with steroids and 3 with specific antiviral agents. Seven patients were paced temporarily. One child died, 6 recovered totally and 3 have a permanent block with a definitive pacemaker implanted in 2. In conclusion, sudden acquired complete atrioventricular blocks are often ill-tolerated in children and have to be treated with transient pacing. Recovery occurs as a rule but some of these blocks may be definitive. Infective myocarditis is likely to be the cause of the disease even if the pathogen agent cannot always be identified.

Characteristics and results of epicardial pacing in neonates and infants.

The results of epicardial pacing in infants with isolated congenital complete atrioventricular block (CAVB) are reported. Thirty-four patients, aged 1 day to 20 months (22 patients < 1 month), were paced between 1988 and 1998. Thirty had bradycardia < 50 beats/min with symptoms in 12 patients, and 4 patients were paced because of associated ventricular ectopy or prolonged QT interval. In thirty cases, the electrodes were implanted through a left thoracotomy and connected to an abdominal generator; in four, the subxyphoid approach was preferred. Twenty-two children had dual chamber units. There was no operative death, but three patients died later of cardiomyopathy. Seven infants were reoperated for electrode displacement, infection, exit block, and pacemaker sensitivity. Chronic ventricular thresholds ranged from 0.3 to 2 V except in one case (4 V) and proper atrial sensing was lost in two cases. All children are doing well and the generator has lasted at least 5 years in 16 cases. In conclusion, epicardial pacing in infants with CAVB can be done with satisfactory results. There was no mortality in relation with pacing and thresholds have improved with the use of steroid-eluting electrodes. The deep location of the generator prevents cutaneous erosion and allows implantation of large units with a longer life duration.