[Diagnosis of congenital heart disease in a nonselected population in Upper Normandy: retrospective study between 2003 and 2007]. / Diagnostic anténatal des cardiopathies congénitales dans une population à bas risque en Haute-Normandie : étude rétrospective de 2003 à 2007.

UNLABELLED: Routine prenatal ultrasound screening for the detection of possible cardiopathy has existed in Upper Normandy since 1987, including the continuous training of obstetric ultrasonographers. We evaluated the profitability and the expected benefit of prenatal detection in a nonselected population in this region. METHODS: A retrospective study was undertaken from October 2003 to September 2007 in the cardiopediatric units of Upper Normandy. All fetuses and infants with a diagnosed major cardiac defect were classified into 3 groups: no possibility of anatomic surgical repair (group 1), risk of early decompensation (group 2), and anatomic surgical repair possible but without early decompensation (group 3). Prenatal and postnatal mortality and morbidity were reported. RESULTS: One hundred and sixty-five major congenital heart defects were detected prenatally and 68 postnatally. The prenatal detection rate was 71% (93, 53, and 77% for groups 1, 2, and 3, respectively; p<0.0001). The rate of pregnancy termination was 92, 17, and 45%, respectively. The mortality rate tended to be higher in the undiagnosed group of urgent neonatal heart cases (10.6% vs 4.4%). The prenatal prevalence of abnormal karyotype was 21% and was 11.5% for congenital malformation syndrome. CONCLUSION: Prenatal detection of major cardiac defects has continued to attain high success in Upper Normandy. However, 50% of urgent neonatal heart cases often remain undiagnosed, and therefore the neonatologist must treat this patient population with particular care. Prenatal diagnosis can reduce preoperative mortality and morbidity of cardiopathy with a risk of early decompensation with specific neonatal intensive care.

Hypoplastic transverse arch and coarctation in neonates. Surgical reconstruction of the aortic arch: a study of sixty-six patients.

From Jan. 1, 1983, to Jan. 1, 1988, 66 consecutive neonates with coarctation and severe hypoplasia of the transverse arch underwent coarctation repair by resection of the coarctation and reconstruction of the aortic arch. Mean age at operation was 14 +/- 8 days, ranging from 2 to 30 days; 63% of the newborn infants were less than 2 weeks of age. The coarctation was isolated in 23%, associated with a ventricular septal defect in 39%, and associated with complex anomalies in 38%, including 16 cases of transposition of the great arteries or doublet-outlet right ventricle plus ventricular septal defect, two cases of simple transposition, two of corrected transposition plus ventricular septal defect, and five cases of "hypoplastic" left ventricle. The surgical technique comprises a wide resection of the coarctation extended to the contiguous ductal tissue followed by the reconstruction of the aortic arch in bringing the descending aorta into the concavity of the aortic arch. This technique is able to relieve the obstruction of the aortic arch provided that (1) the descending aorta is widely dissected to allow mobilization and (2) the incision of the transverse arch is extended proximal to the ostium of the left carotid artery. The operation was performed through a left thoracotomy in 62 patients and through a sternotomy in four additional neonates with transposition and ventricular septal defect who underwent a one-stage repair with aortic reconstruction, closure of the defect, and arterial switch. The overall early mortality rate (less than 30 days) was 14% (9/66; 95% confidence limits = 5% to 22%), including four deaths occurring within the first month, at a concomitant or subsequent repair of the associated anomaly. There were six late deaths, all related to the associated lesions. The overall mortality rate was 23% (15/66; 95% confidence limits = 13% to 33%). The mean follow-up was 21 +/- 10 months, ranging from 6 to 66 months. Actuarial survival rates at 5 years are 72% +/- 10% for the overall group; 87% +/- 17% for simple coarctation; 88% +/- 12% for coarctation and ventricular septal defect; and 52% +/- 18% for complex coarctation. The rate of recurrent coarctation was 12.5% (95% confidence limits = 2% to 23%), leading to five reoperations with no deaths. Freedom from reoperation was 89.5% +/- 9% at 5 years. This technique of coarctation repair offers several advantages: low operative mortality, complete relief of the left ventricular obstruction, wide resection of the ductus tissue, absence of prosthetic material, and preservation of the left subclavian artery.

Prevention of endothelial dysfunction in small and large arteries in a model of chronic heart failure. Effect of angiotensin converting enzyme inhibition.

Chronic heart failure (CHF) impairs endothelium-dependent vasodilatation of large conductance arteries. We investigated whether a similar reduction also occurs in small arteries, and whether such a reduction can be prevented by the angiotensin converting enzyme inhibitor perindopril (P) in a rat model of CHF (left coronary artery ligation). After 1 month treatment with placebo or P (2 mg/kg/day), rats were anesthetized and arterial pressure, left ventricular end-diastolic pressure, and central venous pressure were measured with a micromanometer. Segments of aorta and mesenteric artery (mean diameter, 281 +/- 8 microns) were then isolated, cannulated, and perfused at constant pressure using an arteriograph. Responses to increasing concentrations of acetylcholine (Ach), nitroprusside, and to 10(-4) mol/L NG-nitro-L-arginine methyl ester (L-NAME) were studied after preconstriction by phenylephrine. Heart failure resulted in a decrease in systolic and diastolic pressures, an increase in left ventricular end-diastolic and central venous pressures, and a significant depression of Ach-induced dilatation of the mesenteric artery (maximal dilatation, from 90 +/- 4% to 63 +/- 4%, P < .05) but not of the aorta (from 56 +/- 8% to 45 +/- 5%, NS) without any modification in the endothelium-independent vasodilatation induced by nitroprusside. In the group treated by the angiotensin converting enzyme (ACE) inhibitor perindopril, systolic and diastolic pressures were slightly decreased, whereas left ventricular end diastolic, central venous pressures, and the endothelium-dependent vasodilating response to Ach were normalized. Responses to L-NAME were not affected by CHF or perindopril. Perindopril also decreased hypertrophy, as evidenced by a significantly lower heart weight in treated rats.(ABSTRACT TRUNCATED AT 250 WORDS)

[Disproportion in diameter of the cardiac chambers and great arteries in the fetus. Contribution to the prenatal diagnosis of coarctation of the aorta]. / Déséquilibre dans le diamètre des cavités cardiaques et des vaisseaux chez le foetus.

The aim of this study was to identify simple echocardiographic criteria suggesting the presence of coarctation of the aorta in the antenatal period. This was a retrospective cooperative study of 43 cases of foetal echocardiography referred to a paediatric cardiologist for abnormal dimensions of the left cardiac chambers and vessels compared with 102 control foetus. Eighteen (41.8%) had abnormalities of the aortic arch at birth. Thirteen of the 18 (72%) neonates had aortic arch abnormalities when disequilibrium with a small left heart was observed before 25 weeks amenorrhea. The ratio between the right and left ventricular dimensions was abnormally high in foetus with functional disequilibrium similar to the foetus with coarctation: the difference between the two groups was not significant. The ratio of pulmonary artery to aortic dimension was higher in the foetus with coarctation of the aorta than with functional disequilibrium. The difference was significant: p < 0.0001. The diameter of the aortic arch in foetus with a future coarctation was much smaller than the mean of the controls, except in 4 cases. The majority of the foetus without left-sided obstacles at birth had normal aortic arches. An early disequilibrium, a high pulmonary artery/aortic ratio and the small size of the aortic ischmus were the main elements suggestive of abnormalities of the aortic arch, especially in the early prenatal period.

[Congenital aortic valve stenosis treated by dilatation. Apropos of a case with hemodynamic control 12 months later]. / Rétrécissement valvulaire aortique congénital traité par dilatation.

Intraluminal dilatation of congenital aortic valve stenosis was attempted in a 14-year old boy. Significant improvement was obtained, with a fall in transaortic gradient from 80 to 30 mmHg. A control haemodynamic examination performed 12 months later confirmed that the result was stable; there was no aortic leakage, and myocardial hypertrophy had begun to regress at echocardiography. This case is of interest in that dilatation is less costly than surgical commissurotomy. However, this technique cannot be widely used until satisfactory long-term results have been demonstrated in a large population of children.

[Prognosis and outcome of idiopathic dilatation of the right atrium in children. A cooperative study of 15 cases]. / Pronostic et évolution de la dilatation idiopathique de l'oreillette droite chez l'enfant.

Idiopathic dilatation is a rare abnormality corresponding to isolated aneurysmal dilatation of the right atrium, the outcome of which is not well known. Therefore a multicentric retrospective study was set up by the paediatric working group of the French Society of Cardiology recensing 7 boys and 8 girls who were diagnosed with this condition between 1971 and 1993. Ten of the children were asymptomatic and the diagnosis was suggested by the chest X-ray: one neonate had cardiac failure secondary to atrial tachycardia. The diagnosis has been facilitated by echocardiography since 1980. In this series, since 1993, four diagnoses were made antenatally. The outcome was variable : eight children are alive and well with follow-up periods ranging from 2 to 15 years (average 6 years) : four children have had cardiac arrhythmias : benign atrial extrasystoles (1 case), junctional reentrant tachycardia (1 case). The other two had more severe arrhythmias with flutter in a 7 year-old and one neonatal atrial tachycardia. The outcome was favourable with medical treatment. Three children underwent surgical atrial resection : the outcome has been good in these 3 cases with follow-up periods of 4, 13 and 18 years. This series shows that idiopathic dilatation of the right atrium is usually a well tolerated abnormality but unexpected complications may arise which can be severe such as arrhythmias, or which may be potentially threatening such as interatrial thrombosis. Management consists of either follow-up to diagnose complications which require appropriate treatment of systematic surgical correction as some authors suggest.

[Right atrial thrombosis; an unusual late complication of the Fontan procedure. Apropos of a case]. / Thrombose de l'oreillette droite; complication tardive inhabituelle d'une intervention de Fontan. A propos d'un cas.

The authors report the case of a large right atrial thrombosis causing acute cardiac failure in an 18 year old patient with tricuspid atresia who had undergone 3 operations: Waterston shunt at 2 months of age, Fontan procedure at 3 years of age with reoperation at 13 years of age. The diagnosis was made at echocardiography and angiography. Surgical management comprised ablation of the thrombus and a tunneling of the right atrium between the inferior vena cava and the atriopulmonary conduit. The immediate postoperative course was complicated by a slowly regressive neurological deficit. The medium-term outcome (one year) is satisfactory with antiarrhythmic and anticoagulant therapy. A review of the literature showed that these thromboses are not exceptional in the early postoperative period for a variety of reasons. Secondary thrombosis is often related to arrhythmias, thus requiring clinical, electrocardiographic and echocardiographic follow-up of these patients and the prescription of antiarrhythmic and anticoagulant therapy. The diagnostic methods are discussed. Echocardiography may be sufficiently explicit to make potentially risky angiography unnecessary.

[Aneurysm of the membranous ventricular septum. Apropos of 4 cases surgically treated]. / Anévrysme du septum membraneux. A propos de 4 cas opérés.

Four patients with aneurysms of the membranous ventricular septum were operated either because of complications or for associated malformations. The authors describe the anatomical, radiological and, above all, the echocardiographic and angiographic features of this malformation. Surgical management is necessary in complicated cases or when there are associated congenital malformations. The information provided by echocardiography in the diagnosis and follow-up of ventricular septal defects indicates that this malformation is not as rare as was previously thought.

[Anatomic correction of simple transposition of the great vessels during the neonatal period. 5 years' experience in 235 children]. / Correction anatomique en période néonatale de la transposition simple des gros vaisseaux. Une expérience de cinq and portant sur 235 enfants.

From March 1984 to August 1989, anatomic surgical correction was performed on 235 newborn infants (2 to 32 days old, mean 8.5 +/- 4) with simple transposition of the great arteries. Ninety-four percent of the patients underwent balloon atrial septostomy before the operation. The anatomy of the coronary arteries according to the Yacoub classification was as follows: type A, 160 patients (68%); type B, 5 patients (2%); type C, 11 patients (5%); type D, 41 patients (17%); and type E, 18 patients (8%). Coronary artery relocation was performed according to the Yacoub technique with some modifications, and the pulmonary artery reconstruction was done using the Lecompte manouver in association with a pericardial patch. The perioperative mortality rate was 8.9 percent for the entire series, significantly higher for the type C (46%, p less than 0.01) and significantly lower for the type D (0%, p less than 0.05). Seven patients were lost of follow-up. The follow-up ranged from 1 to 63 months (mean 21 +/- 14). Four late deaths occurred: 2 from myocardial infarction, 1 from pulmonary hypertension and one from a thrombotic occlusion of the superior Vena cava. One patient had an acute posterior myocardial infarction five months after the operation and his left ventricular contractility is normal at echocardiography three years later. Supravalvular pulmonary stenosis occurred in 17 patients but only two were operated on with no death. Mild aortic regurgitation occurred in nine patients. Ninety eight percent of the survivors have a normal life, with no cardiovascular symptoms and receive no medication.

[Endovascular treatment of an extralobar sequestration with cardiac failure]. / Traitement endovasculaire d'une séquestration extralobaire avec insuffisance cardiaque.

Extralobar sequestration usually presents during the first months or years of life by respiratory distress, cyanosis or infection. Cardiac failure is a rare mode of presentation. If surgery is the usual treatment, radiological endovascular treatment has occasionally been described. We report the case of a newborn who underwent endovascular treatment at 19 days. Clinical status is excellent at 5.5 year follow-up.

[Hypoplasia of abdominal aorta, rare cause of hypertension in childhood]. / Hypoplasie de l'aorte abdominale, cause rare d'hypertension artérielle du grand enfant.

BACKGROUND: The majority of children with secondary hypertension have a renal abnormality or renovascular lesions. Coarctation of the aorta is also a classical cause, rarely located to the abdominal aorta. CASE REPORTS: Two girls, 11 and 12 years-old, were suspected of having recent sustained hypertension. Pulsed-wave doppler ultrasonography and angiography showed abdominal aortic hypoplasia associated with renal artery stenosis, unilateral in one patient and bilateral in the other. Both patients became normotensive 10 and 18 months, respectively, after corrective vascular surgery. CONCLUSION: Examination of the abdominal part of the aorta is mandatory in all patients with hypertension.

[Preoperative mortality in transposition of the great vessels]. / Mortalité préopératoire de la transposition isolée des gros vaisseaux.

UNLABELLED: The aim of the study was to report the incidence and causes of preoperative deaths in isolated transposition of the great vessels and to describe the clinical findings in these neonates. PATIENTS AND METHODS: In five French centers of pediatric cardiology, data of all the neonates with isolated transposition of the great vessels who died before arterial switch operations between January 1986 and June 1996 were obtained from reviewing hospital files, echocardiography records and autopsy reports. RESULTS: Among 199 neonates with transposition of the great vessels, 20 (9.9%) died before surgery. The death was related to intracranial haemorrhage in one premature neonate, severe and early hypoxemia in 13 full-term patients (group A) and later sudden collapse in six patients (group B). In group A, the symptoms occurred within 20 minutes after the birth and included cyanosis (n = 12), acute respiratory distress (n = 8), and shock (n = 4). Despite assisted ventilation (n = 13), bicarbonate infusion (n = 12), prostaglandin E1 (n = 7), inotropic drugs (n = 5) and balloon atrioseptostomy (n = 7), death occurred at the median age of five hours. The patent foramen ovale was absent or tiny in ten patients, normal in one patient and not specified in two patients. The ductus arteriosus was patent in ten patients and not specified in three patients. In group B, the neonates were initially in a good hemodynamic condition. Unexplained death occurred between two and five days after the birth: one infant with a large patent foramen ovale did not receive prostaglandin E1, four patients died a few hours after an angiographic study or a balloon atrioseptostomy was performed in a catheterization laboratory, and one child suffered from a cerebral anoxia due to a tightened cord. CONCLUSION: We conclude that the high preoperative mortality rate in isolated transposition of the great vessels is mainly due to absent or small atrial shunt. These findings suggest that only prenatal diagnosis of transposition of the great vessels with immediate balloon atrioseptostomy could avoid a fatal outcome.

[Microdeletion of 22q11 and conotruncal cardiopathies: contribution of prenatal diagnosis]. / Microdélétion 22q11 et cardiopathies conotroncales: apport du diagnostic prénatal.

OBJECTIVE: We report our experience on prenatal diagnosis of 22q11 deletion by fluorescent in situ hybridation (FISH). PATIENTS AND METHODS: From February 1997 to April 1998, prenatal diagnosis of 22q11 deletion was performed in 13 cases of congenital conotruncal heart defects. FISH was carried out using D22S75 DiGeorge's chromosome region probe. RESULTS: Microdeletions of 22q11 were detected in 4 fetuses with tetralogy of Fallot (3 cases) and pulmonary atresia with ventricular septal defect (1 case). Termination of pregnancy was performed in two cases for severe congenital heart defect. A third malformed fetus died immediately after a blood sampling procedure. The last fetus, with a tetralogy of Fallot malformation, was born and underwent corrective cardiac surgery. The dysmorphic features of this fetus was suggestive of DiGeorge's syndrome, and the development status was normal. CONCLUSION: Prenatal detection of 22q11 only played a minor role in the decision to terminate the pregnancy in our study.

[Interstitial emphysema of the right lung. Treatment with bronchial occlusion in a premature infant]. / Emphysème interstitiel du poumon droit. Traitement par occlusion bronchique chez un prématuré.

Interstitial emphysema of the right lung occurring as a complication of hyaline membrane disease in a premature infant was successfully treated by bronchial occlusion with a balloon catheter. This technique was simpler and safer than selective intubation of the left main bronchus.

[Neonatal heart transplantation. First results]. / Transplantation cardiaque néonatale. Premiers résultats.

Two 5- and 17-days old neonates with hypoplastic left heart syndrome respectively underwent orthotopic heart transplantation. The donor-recipient weight ratio was +58 percent and +88 percent; ischemic time was 144 and 167 min. The immunosuppressive protocol included thymoglobulin during the induction period and a classical 3-drug therapy, with a rapid tapering off of prednisone over 3 weeks. No infectious complication was observed; each infant experimented one episode of acute rejection, successfully treated with prednisolone. Forty-three months and 10 months later, the 2 children are doing well, with normal renal function and normal growth. No late rejection episode was observed. Heart transplantation in neonates is feasible, the short-term and mid-term results are good. Despite important ethical problems, heart transplantation represents a great hope for neonates with inoperable congenital heart defects.

Double-lumen aortic arch by persistence of fifth aortic arch: A new case associated with coarctation.

A coarctation of aorta with double-lumen aortic arch due to persistence of an embryonic fifth aortic arch was recognized in a 13-day-old girl and successfully repaired using enlargement of the aorta by side-to-side anastomosis of the fourth and fifth aortic arches.

[Trial of prevention of coronary aneurysm in Kawasaki's disease using plasma exchange or infusion of immunoglobulins]. / Essai de prévention des anévrismes coronaires de la maladie de Kawasaki par échanges plasmatiques ou perfusion d'immunoglobulines.

Coronary aneurysms are present in 15-25% of cases of Kawasaki disease and are responsible for the morbidity and rapid mortality in this disease. We treated 20 children aged 20 +/- 16 months, less than 15 days (8 +/- 3.2) after onset of the disease, either by plasma exchange (7 exchange transfusions and one plasmapheresis) or by high dose intravenous gammaglobulins. No coronary anomalies or cardiac dysfunction prior to or after therapy (mean follow-up 8.3 +/- 4 months) was found. Tolerance to therapy was good and the course of the disease was arrested by exchange transfusions or shortened by gammaglobulin infusion. We conclude as have the Japanese, that this therapy is efficacious and safe, and should be initiated early.

[Scimitar syndrome in infants. Physiopathology and therapeutic implications in 12 cases]. / Le syndrome du cimeterre du nourrisson. Physiopathologie et déductions thérapeutiques dans 12 cas.

Twelve children, aged 6 days to 6 months (mean: 42 +/- 23 days) were diagnosed as presenting with the scimitar syndrome. In this syndrome, anomalous pulmonary venous drainage, hypoplasia of the right pulmonary artery and parenchyma, variety of "sequestrum" of the right pulmonary artery and parenchyma, variety of "sequestrum" of the right pulmonary base usually fed by subdiaphragmatic branches of the aorta are associated. Respiratory disorders were constant, severe in 8 cases. X-rays showed "dextro-cardia" and "hypoplasia of the right lung". Endocardiac investigation assessed the diagnosis and showed pulmonary arterial hypertension in 11 cases, with (5) or without (6) associated heart defect. One case with good tolerance remains under simple supervision; 4 were operated on from the associated lesions only and died; 7 took benefit of a simple surgical removal of the branches feeding the sequestrum, with one death and 6 successes. The poor prognostic significance of the sequestrum is thus indirectly demonstrated and the value of its devascularization is emphasized.

Dilated coronary sinus in prenatal echocardiography; identification, associations and outcome.

The dilated coronary sinus (DCS) has only recently been clearly visualised in the fetus due to progress in prenatal echography. This is a retrospective study of 22 fetuses presenting with DCS revealed by prenatal echography. We report the circumstances leading to the detection of a DCS and the neonatal outcome of these fetuses. The coronary sinus was defined as dilated depending on its visualisation in cross-section from the '4 chamber' view, as well as a pseudo inter-atrial septal defect from a more posterior view. In each case the gestational age, circumstances of detection, associated anomalies and postnatal outcome are reported. The circumstances were: evaluation of a clearly identified DCS in four cases and during detailed fetal echocardiography because of suspected congenital heart disease in 18 cases. Five cases were associated with a cardiac anomaly, three with an extracardiac anomaly, six with both cardiac and extracardiac anomaly and eight were isolated. Postnatal outcome was related to the associated anomaly. In conclusion, it is important that the echography image be correctly interpreted, as a DCS often implies possible associated defects and therefore affects prognosis. When not associated with other anomalies this condition is not considered serious.