[Outcome of 30 congenital atrio-ventricular blocks]. / Devenir à long terme de 30 blocs auriculo-ventriculaires complets congénitaux isolés.

Congenital isolated atrio-ventricular block (CAVB) is a rare pathology, and its management is still rather poorly described through international literature. Within the service of pediatric cardiology leaded by Pr Choussat and Dr Jimenez (Cardiologic Hospital Haut-Lévêque of Bordeaux), we collected from 1980 to 2003, 30 isolated congenital CAVB, constituting the purpose of this retrospective study. Average follow-up is 14 +/- 8.8 years. None death occurred. CAVB are discovered at an average age of 4.8 years old; 6 cases were diagnosed in utero, half of them were associated with maternal lupus. Twenty patients on 30 were fitted with stimulator at an average age of 8.7 +/- 6.9 years old, due to symptoms or bradycardy. Epicardic fitting in VVI mode represents 65% of first approaches, it is followed by endocavitary way for 81% of cases. Cardiac stimulation does not prevent from dilated cardiomyopathy. Among 30 patients 10 were not fitted with stimulator, half of them presents chronotrop insufficiency during effort. As a conclusion, our patients show a good long-term vital prognosis; although CAVB discovered in utero lead to worse prognosis for children.

Transcatheter closure of patent foramen ovale in patients with platypnea-orthodeoxia: results of a multicentric French registry.

BACKGROUND: Dyspnea and the decrease in arterial saturation in the upright position in elderly subjects is described as platypnea-orthodeoxia syndrome (POS). POS is secondary to the occurrence of an atrial right-to-left shunt through a patent foramen ovale (PFO). METHODS: This French multicentric study reports on 78 patients (mean age 67 +/- 11.3 years) with POS who had transcatheter closure of the PFO; frequently associated diseases were pneumonectomy (n = 36) and an ascending aortic aneurysm (n = 11). In all patients, the diagnosis was confirmed by transthoracic or/and transesophageal echocardiography. Five different closure devices were used: Amplatz (n = 45), Cardioseal (n = 13), Sideris (n = 11), Das Angel Wings (n = 8) and Starflex (n = 1). Closure was successful in 76 patients (97%). RESULTS: Oxygen saturation increased immediately after occlusion from 84.6 +/- 10.7% to 95.1 +/- 6.4% (p < 0.001) and dyspnea improved from grade 2.7 +/- 0.7 to grade 1 +/- 1 (p < 0.001). A small residual shunt was immediately observed in 5 patients (3 with the Cardioseal device, 1 with the Sideris and 1 with the Amplatz) leading to the implantation of a second device in one case (Cardioseal). Two early deaths occurred unrelated to the procedure (one due to sepsis probably related to pneumonectomy, another due to respiratory insufficiency). Other complications were: a small shunt between the aorta and the left atrium, two atrial fibrillations and a left-sided thrombus which disappeared with anticoagulant therapy. At a mean follow-up of 15 +/- 12 months, there were 7 late deaths related to the underlying disease. CONCLUSION: Percutaneous occlusion of the foramen ovale is safe and gives excellent results thanks to continuing improvement in available devices. This technique enables some patients in an unstable condition to avoid a surgical closure.

[Prosthetic valve thrombosis in pregnancy. A single-center study of 12 cases]. / Thromboses de prothèses valvulaires cardiaques chez la femme enceinte.

Pregnancy in women with mechanical prosthetic heart valves carried an increased risk of thromboembolic complications due to changes in haemostasis. Prosthetic valve thrombosis is a serious complication resulting in high mortality. Ten patients from 20 to 38 years of age had 12 thromboses of mechanical heart prostheses during pregnancy. The prosthesis was mitral in 8 cases and aortic in 4 cases. The prosthesis was a ball valve in 1 case, a tilting disc in 3 cases and bi-leaflet in 8 cases. Initial emergency treatment was surgical in 3 cases and medical in 9 cases (thrombolysis in 7 cases and simple heparin therapy in 2 cases). Secondary surgery was carried out in one patient after failure of heparin therapy. There was one death in the surgical group (4 cases, 25%) and 30% foetal mortality in the surviving women. In the thrombolysis group (7 cases), two women died (28%) after failure of treatment. Both patients had mitral valve prostheses and were in cardiogenic shock. Three women, of the other 5 thrombolysed cases, were able to complete their pregnancies and had healthy babies with no foetal mortality. No per-thrombolytic embolic complications were observed. However, there was one severe bleeding complication which was successfully managed by surgical drainage. Finally, a global success rate of 75% (9 out of 12 patients) and a mortality of 30% (3 maternal deaths in the 10 patients--all with mitral valve protheses) were observed irrespective of the therapeutic protocol used. Thrombosis is the most life-threatening complication for women with prosthetic heart valve during pregnancy. Emergency surgery for valve replacement or thrombectomy is the commonest treatment. Trombolysis is classically limited by the risk of haemorrhagic and thromboembolic complications reported in the literature. In this study, thrombolysis was effective in the 71% of cases with a low risk of haemorrhagic complications.

[Diagnostic and therapeutic value of lymphography in persistent postoperative chylothorax]. / Intérêt diagnostique et thérapeutique de la lymphographie dans un chylothorax postopératoire persistant.

Chylothorax is a rare but generally severe complication of surgery of congenital heart disease. The authors report the clinical history of a young boy with complex congenital heart disease operated on several occasions and who developed severe and recurrent unilateral chylothorac after a bicavo-bipulmonary derivation. Conservative treatment followed by continuous somatostatin infusion was ineffective. Diagnostic Lipiodol lymphography was required before the chylothorax was cured. The authors describe management of this difficult case and discuss the therapeutic possibilities with reference to a brief review of the literature.

[Factors predictive of mortality in Ebstein's anomaly]. / Facteurs prédictifs de mortalité dans la maladie d'Ebstein.

Nearly 150 years after the description of Ebstein's anomaly the prognostic factors are not well known. Besides the foetal and neonatal forms which carry a poor prognosis the adult forms are often well tolerated and pose the difficult problem of management. This was a retrospective study of 73 patients with this malformation followed up between 1967 and 2002 at the University Hospital of Bordeaux. There were 14 deaths (19%) half of which (7) occurred during the first 3 months of life. The factors predictive of mortality (p<0.05) were: age, associated malformations, Classes III-IV of the New York Heart Association, cardiac failure, malaises, cyanosis, a raised cardiothoracic index, and a high mitro-tricuspid displacement indexed to body surface area. Supraventricular arrhythmia and the Wolff-Parkinson-White syndome do not seem to be associated with extramortality. Surgery, with reference to published studies, seems to improve survival, functional status and decrease the prevalence of arrhythmias. It would appear to be justified to propose surgery when functional status declines or when signs of poor prognosis are observed.

[Ectopic junctional tachycardia of the neonate]. / Tachycardie jonctionnelle ectopique du nouveau-né. A propos de 2 cas.

Congenital ectopic junctional tachycardia (EJT) is a rare arrhythmia presenting in the first 6 months of life. It is often resistant to antiarrhythmic drugs and its poor prognosis (35% mortality) explains its often complex management. The authors report two cases which illustrate its unpredictability with a potential to degenerate to serious ventricular arrhythmias. The possibility of progression to atrioventricular block, increased by antiarrhythmic therapy, may lead to implantation of a cardiac pacemaker. The poor outcome of the two babies underlines the severity of these arrhythmias.

[The best of pediatric cardiology in 2002]. / L'essentiel de 2002 en cardiologie pédiatrique.

This past year has been remarkable for considerable advance in the field of interventional cardiology for congenital heart disease. Ostium secundum atrial defect is by now cured by percutaneous closure with excellent results. Closure of patent foramen ovale (PFO) in patients with paradoxical embolism is still controversial. Nevertheless, some studies demonstrate the efficiency of this procedure, reducing the incidence of ischemic events. In cases of platypnoea-orthodeoxia syndrome occurring in patients having undergone pneumonectomy or presenting dilatation of ascending aorta, closure of PFO with an occluder gives spectacular results with disappearance of dyspnea and cyanosis. Other more complex procedures such as closure of membranous ventricular septal defect, replacement of an pulmonary or aortic valve are still to be assessed. As the last point let us underline the progress of foetal cardiology, the studies of sudden death in children an adolescents, and the care of adults with congenital heart disease.

[Ebstein's anomaly]. / Anomalie d'Ebstein.

Since Ebstein's first description in 1866 and the first surgical attempts at treatment in the 1950's, the natural history of this condition remains obscure. The anatomical description contrasts with the clinical and prognostic variability of this malformation. The form observed in the foetal or prenatal periods has a very different prognosis to that observed in adults. The risk factors are not the same even though the anatomical entity is comparable. The difficulty in managing these patients is due to the problems of diagnosis and determination of these prognostic factors. Advances in echocardiography, the key investigation which facilitates diagnosis, and in surgical and ablation techniques, have improved our understanding of this condition and its prognosis.

[Outcome of operated Fallot's tetralogy]. / Devenir des tétralogies de Fallot opérées.

Fifty years after the beginning of cardiac surgery, a large percentage of operated congenital heart disease patients attain adulthood. The tetralogy of Fallot is one of the malformations in which the natural outcome was unfortunately nearly fatal in the long-term. Open heart surgery has radically transformed the prognosis of this, the most common of all cyanotic congenital cardiac malformations. Nowadays, most operated patients lead normal professional and family lives. Lonf-term survival after correction is between 90-95% after 30-35 years. Although surgical repair is satisfactory, the operated heart is not anatomically normal. The patients have a variable degree of devalvulation of the pulmonary outflow tract, a scar on the right ventricle, a patch repairing the ventricular septal defect and scars on the atrium (cannulation for cardiopulmonary bypass). These sequellae expose the patients to a number of complications, notably arrhythmic and sometimes haemodynamic, affecting the right ventricle. The late mortality rate varies from 5 to 13% in the literature. The main causes of death are sudden death and reoperation. Sudden death is the most severe long-term complication but it is uncommon, affecting less than 5% of the population. It is mainly due to ventricular arrhythmias. Certain predisposing factors should be identified during long-term follow-up, among them the presence of haemodynamic abnormalities: systolic overload (residual pulmonary stenosis) or diastolic overload of the right ventricle (pulmonary regurgitation), infundibular aneurysm, right ventricular dysfunction. It would appear to be important to prevent pulmonary regurgitation by preserving the pulmonary valve even if it means persistence of a mild transvalvular pressure gradient. When pulmonary regurgitation is inevitable, follow-up is essential to evaluate the timing of valvulation of the pulmonary orifice. Despite these different complications which must be understood for proper follow-up of these patients, the long-term outcome of operated tetralogy of Fallot remains very good.

[Value of fetal echocardiography in screening for congenital heart diseases. Report of 1,049 consecutive examinations]. / Intérêt de l'échographie cardiaque foetale dans le dépistage des cardiopathies congénitales. A propos de 1,049 examens consécutifs.

Specialised antenatal echocardiographic screening has allowed 1049 consecutive examinations in 992 patients resident in the Aquitaine region over a period of nine years. The most frequently encountered indications are expertise (suspicion of cardiopathy during initial screening) and family history. Expertise allows a higher detection rate (46.5%) compared to the other indications. We have screened 150 cardiopathies in utero and among them 143 were able to followed up after delivery. We have noticed a much greater severity of the anomalies compared to those encountered in paediatric cardiology. An associated chromosomal anomaly was noticed in 9% of cases and more frequently for atrio-ventricular communication and conotruncal cardiopathies. The rate of abortion was 35%, and the mortality for the surviving pregnancies was elevated (35%). Pharmacological treatment was instituted in 95% of cases of sustained supraventricular tachycardia with 81% successful. Diagnosis errors concerned benign cardiopathies above all and did not change the management of the pregnancy. The sensitivity of fetal echocardiography was 93.1% and its specificity 99.1%. This study shows the viability of fetal cardiac echography for the detection of the majority of congenital cardiopathies in a population at risk and in the management of fetal cardiac rhythm disorders. Nevertheless it remains a specialised examination for which the performance depends much on the initial screening performed by gynaecologists. Although the mortality rate of the screened children is elevated, indicating the frequent parental choice of abortion and the complexities of the cardiopathies, antenatal diagnosis has allowed optimisation of the neonatal management for certain curable cardiopathies.

[Placement of an implantable defibrillator via the endocavitary route in a patient presenting with a univentricular heart and a Glenn anastomosis]. / Pose d'un défibrillateur implantable par voie endocavitaire chez une patiente présentant un coeur univentriculaire et une anastomose de Glenn.

We report the observation of a female 45 year old patient presenting with a post-surgery complex congenital cardiopathy, associated with serious ventricular rhythm disorders necessitating the placement of an implantable defibrillator. The palliative surgery performed (cavo-pulmonary Glenn anastomosis) does not allow the usual access to the right ventricle via the superior vena cava. The different possibilities for defibrillator implantation are discussed. These include associating a surgical approach to introduce the bipolar probe with subcutaneous tunnelling to connect the probe to the box.

[The best of 2001. Pediatric cardiology]. / L'essentiel de 2001 en cardiologie pédiatrique.

During this last year, paediatric cardiology has gone through significant changes, although no capital evolution is to be pointed out. Foetal cardiology, initiated some years ago has been remarkably developed. The rate of cardiac anomalies detected is increasing, which leads to very difficult discussions regarding therapeutic abortion and even increasing legal responsibility as evidenced by "arrêt Perruche". Interventional cardiology is more and more resorted to and a reliable, safe and efficient procedure which improved atrial septal defect (ASD) occlusion devices. Data on occlusion of ASD and patent foramen ovale confirm the reliability of these methods. In the field of imaging. Nuclear Magnetic Resonance angiography which gadolinium can, in some cases, replace fluoroscopy with clearer pictures. Reinforced interest is given to Primary Pulmonary Hypertension (PPH) due to genetic studies and new therapies. The rate of familial PPH is about 6%. It is an autosomic dominant disorder located on chromosome 2q31-32. New treatment use various prostacyclines administrated by different ways: subcutaneously, orally or by inhalation. It is too early to assess their impact on the prognosis. Finally, we may note the paradoxical importance of congenital heart disease in adult as an outcome of interventional cardiology and follow-up of operated children becoming adults. As the beginning of the 21st century, the definition of paediatric cardiology has considerably evolved.

[Myocardial infarction in a child with an anomalous left coronary artery arising from the right coronary sinus. Value of echocardiography]. / Infarctus du myocarde chez un enfant et anomalie de naissance du tronc coronaire gauche du sinus coronaire droit. Apport de l'échocardiographie.

An anomalous left coronary artery arising from the right sinus of Valsalva with a trajectory between the aorta and the main pulmonary artery is a rare isolated congenital anomaly carrying a high risk of sudden death and of acute myocardial ischaemia, especially in children and young adults in a context of exercise. It is usually a post-mortem finding. The authors report the rare case of a 12 year old child who suffered acute myocardial infarction on exercise due to this condition. The diagnosis was made in the acute phase by echocardiography. Surgical correction was performed as this is the only means of prevention of sudden death of an ischaemic recurrence. Effort syndromes in children or young adults should lead to a request for echocardiographic examination to exclude the diagnosis in a non-invasive manner as well as those of hypertrophic cardiomyopathy or congenital aortic stenosis.

The short- and mid-term results of bidirectional cavopulmonary shunt with additional source of pulmonary blood flow as definitive palliation for the functional single ventricular heart.

OBJECTIVE: The purpose of this study was to demonstrate the early and late outcomes of bidirectional cavopulmonary shunt (BCPS) as a definitive procedure for the functional single ventricular heart. METHOD: From September 1991 to December 1997, 34 patients underwent a BCPS procedure without a routine conversion to Fontan circulation. The additional source of pulmonary blood flow was left in all patients. Conversion was performed only when it was required for excessive cyanosis. RESULTS: The hospital mortality rate was 8.8% (3/34, 95% confidence limit; 1.9-23%) and the 5-year survival rate was 75% for a mean follow-up period of 33+/-22 months. Seven patients underwent a conversion procedure for remnant or recurrent cyanosis and deterioration of exercise tolerance. Four of these patients died after conversion to Fontan circulation. Twenty-five long-term survivors with BCPSs maintained an arterial oxygen saturation of 84+/-6.1%, and 52% of them had a normal exercise tolerance or mild limitation. No patients developed severe late complications other than recurrent cyanosis. CONCLUSION: Due to the high mortality after conversion to Fontan circulation in patients whose conditions had deteriorated, we could not demonstrate the clear superiority of long-term BCPS over the construction of Fontan circulation for management of the functional single ventricular heart. If deteriorated conditions were successfully managed in the late period, the outcome of long-term BCPS would have been better.

Immunohistological and ultrastructural analysis of the intimal thickening in coarctation of human aorta.

OBJECTIVES: The histological nature and characteristics of aortic coarctation are not clearly defined, the aim of this study is to analyse intimal thickening in aortic coarctation. METHODS: In order to characterize the components of intimal thickening in coarctation, narrowed segments of aorta obtained after surgery from ten children were examined immunocytochemically and by electron microscopy. RESULTS: Histological analysis of aortic coarctation demonstrated a widened subendothelial region with separation of endothelial cells from the internal elastic lamina. Masson's trichrome staining showed a marked increase in extracellular matrix and cell numbers in the intimal thickening compared with normal aorta. Cellular component analysis demonstrated invagination of the intima by smooth muscle actin-positive cells, with a fragmentation of the internal elastic lamina. No proliferating smooth muscle and inflammatory cells were identified in the intima. In order to characterize the smooth muscle cell phenotypes, various smooth muscle cell markers were sought using specific monoclonal antibodies: alpha-smooth muscle actin, smooth muscle-myosin heavy chain, heavy caldesmon, desmin. In moderate coarcted aorta, at least two distinct smooth muscle phenotypes were identified. In the juxtamedial part of the intima smooth muscle, cells were differentiated and expressed all smooth muscle markers; in the subendothelial part of the intimal thickening, the majority of smooth muscle cells expressed only alpha-smooth muscle actin and appeared dedifferentiated. In regions of marked stenosis, a strong expression of smooth muscle-myosin heavy chain, and heavy caldesmon in the intimal thickening pointed to the presence of redifferentiated smooth muscle cells, not still expressing desmin. Electron microscopic examination also revealed a variety of smooth muscle cell phenotypes in the intimal thickening. In the superficial layer, smooth muscle cells appeared to be in the synthetic state, while in the deeper part, both synthetic and contractile components were identified. CONCLUSIONS: These observations indicated that human coarctation was characterized by intimal recruitment of non-proliferating smooth muscle cells with dedifferentiated phenotype. However, the presence of smooth muscle cells with an intermediate phenotype in the narrowest part of the coarctation suggest that the redifferentiation process could participate in the pathogenesis of aortic coarctation.

[Surgical evaluation of transthoracic tridimensional echocardiography in the anatomic study of atrial septal defect]. / Validation chirurgicale de l'échocardiographie tridimensionnelle transthoracique pour l'évaluation anatomique des communications interauriculaires.

The closure of atrial septal defects by interventional catheterisation requires an accurate assessment of their morphology and anatomical relationships. This study evaluated transthoracic three-dimensional echocardiography for the selection of atrial septal defects accessible to an occlusive prosthesis. The transthoracic three-dimensional echocardiographic measurements of 17 patients (4 to 55 years) with ostium secundum atrial septal defects were compared with those of the surgeon in a prospective study. The maximal diameters of the defect, the height of the interatrial septum, the distances to the superior vena cava (postero-superior border) and inferior vena cava (postero-inferior border), to the coronary sinus and the tricuspid valve were measured as a reconstruction of the interatrial septum seen from the right atrium. The aortic border was measured from a three-dimensional view from the left atrium. Thirteen of the 17 investigations (76%) were exploitable. The diameters of the defect varied during the cardiac cycle (p = 0.0002). Ther correlations between the surgical and echocardiographic measurements varied from 0.82 for the maximal diameter to 0.6 for the postero-inferior limits. Three-dimensional echocardiography is capable of detecting all the contra-indications of an occlusive prosthesis: 2 inadequate postero-inferior and 1 inadequate aortic borders, 9 maximal diameters which were too large, 3 insufficiently high atrial septa, 1 double atrial septal defect. The coronary sinus was only visualised in 1 case. Transthoracic three-dimensional echocardiography is a non-invasive technique capable of improving the selection of atrial septal defects for interventional closure. The transoesophageal approach should be reserved for candidates selected by the transthoracic investigation for the detection of small structures (coronary sinus) and when the transthoracic window is poor.

[Cardiac myxoma in patients over 75 years of age. Report of 19 cases]. / Myxomes cardiaques chez le sujet âgé de plus de 75 ans. A propos de 19 observations.

Left atrial myxoma is considered to be exceptionally rare in the elderly. The authors observed and reported on 19 cases occurring in patients over 75 years of age out of a series of 100 myxomas diagnosed between 1962 and 1997, in 12 women and 7 men (mean age: 80 years, range 75 to 89 years). In 3 cases, the myxomas were chance findings at echocardiography but the 16 symptomatic patients (85%) had left ventricular failure (47%), positional symptoms (25%), pyrexia and poor general health (17%) or systemic embolism (17%). The location of the myxoma was the left atrium in all cases, with mitral valve obstruction in 13 of the 19 cases. Eighteen tumours were attached to the interatrial septum and one to the atrial surface of the anterior mitral leaflet. Calcifications were observed in 5 patients. Surgical ablation of the tumour was performed in 15 of the 19 patients. The post-operative course was usually uncomplicated: one patient died of a cerebral haemorrhage. Four patients did not undergo surgery because of patient refusal in 3 cases and major associated morbidity in the other case. These cases were included in the study because the tumours had all the characteristics of myxomas. Though the discovery of a myxoma remains a classical surgical emergency, the presence of quiescent, non-obstructive, well circumscribed and calcified myxomas with a low risk of obstruction and of embolism in elderly high risk patients may be exceptions to this traditional dogma.

[Congenital left ventricular aneurysms and diverticula. Two case reports]. / Anévrysmes et diverticules congénitaux du ventricule gauche. A propos de deux observations.

The authors report the cases of two young adults (25 and 27 years) presenting with congenital left ventricular aneurysm or diverticulum with healthy coronary arteries. This saccular evagination of the ventricular wall is rare. The authors describe a classification distinguishing muscular (contractile) diverticula, composed of the three cardiac tunics, fibrous diverticula and finally aneurysms with a dyskinetic wall. Although the limits of this classification are sometimes poorly defined, it presents a prognostic value, because it appears logical to propose nonsurgical management in the context of muscular diverticula and it seems legitimate to operate on fibrous diverticula and aneurysms due to the risks of rupture, extension, thrombosis or arrhythmia.