Coronary myocardial bridging in Noonan syndrome: definitive diagnosis with high-resolution CT.

Noonan syndrome is an autosomal dominant disorder reported at an incidence of 1 in 1000-2500 live-born patients. Pulmonary stenosis with a dysplastic pulmonary valve and hypertrophic cardiomyopathy are often associated with this syndrome. We report on a 9-year-old patient affected by Noonan syndrome with left ventricle hypertrophy, in whom a sudden clinical presentation of chest pain after effort led to the clinical suspicion of associated coronary myocardial bridging, which was confirmed by high-resolution CT. We also review the literature on this topic.

CT angiography of the artery of Adamkiewicz with 64-section technology: first experience in children.

BACKGROUND AND PURPOSE: Preoperative localization of the artery of Adamkiewicz (AKA) may be useful in selected children in prevention of ischemic spinal complications of spinal or thoracoabdominal aortic surgery. The aim of our study was to investigate the ability of 64-section CT for assessing the AKA in children. MATERIALS AND METHODS: Forty children (mean age, 7.5 +/- 5 years) underwent thoracic imaging with a 64-section CT scanner after intravenous injection of 1.5 mL/kg of contrast agent. Volumetric reconstructions were obtained for every patient. Identification of the AKA was performed on the basis of continuity from the aorta to the anterior spinal artery with a characteristic hairpin turn. Identification of the AKA and determination of its origin and course were analyzed by 2 independent radiologists. RESULTS: The AKA was successfully visualized in 38 patients (95%). Twenty-seven (71.1%) AKAs originated on the left side, whereas 11 (28.9%) originated on the right side. It was seen to originate at the level of the left 5th intercostal artery in 1 patient, the left 8th in 4, the left 9th in 15, the left 10th in 5, the left 11th in 2, the right 8th in 2, the right 9th in 6, the right 10th in 2, and the right 12th in 1. CONCLUSION: Sixty-four-section CT angiography depicted the AKA in a very high percentage (95%) of children. The results of this study suggest that 64-section CT angiography is a viable noninvasive technique that could be an alternate to selective angiography for delineating the AKA.

[Transcatheter exclusion of a giant aneurysm of the left pulmonary artery using covered stents]. / Exclusion d'un anévrisme géant de l'artère pulmonaire gauche par cathétérisme interventionnel: utilisation de stents couverts.

An 11 year-old boy with an Alagille syndrome was referred for an urgent echocardiography because of new onset of febrile hemoptysis. Follow-up in our institution to that point had shown severe hypoplastic pulmonary arteries. The diagnosis of tricuspid endocarditis complicated by pulmonary embolisms and aneurysm of the left pulmonary artery was done. Despite effective antiobiotics, the evolution was marked by rapid progression in size of the left pulmonary aneurysm. Since the surgery was not an option because of high risk of death, this aneurysm was treated by a trans-catheter technique using covered stents (CP stents, Numed Inc). Its exclusion allowed to stop its progression in size and to avoid its rupture.

[Spontaneous idiopathic chylopericardium in childhood]. / Chylopericarde idiopathique spontané de l'enfant.

Here we report a case of a primary idiopathic chylopericardium in a 13 years old child. Pericardial effusion was diagnosed because the child suffered chest pain and fatigue. Pericardial drainage was performed and 800mL of chylous fluid was evacuated. Extensive investigations were performed but no cause could be found. Thoracic CT scan, lymphoscintigraphy and MRI did not evidence any communication between the thoracic duct and pericardium. After 2 recurrences of pericardial effusion while the child was on a medium chain triglycerides regimen, it was decided to ligate the thoracic duct and to do a partial pericardectomy. The result was excellent with complete resolution of the pericardial effusion and no recurrence since 3 years.

Right to left shunt through interatrial septal defects in patients with congenital heart disease: results of interventional closure.

OBJECTIVE: To study the effects of closure of interatrial communications associated with a right to left shunt in patients with congenital heart disease (CHD) who had a biventricular repair. DESIGN: Retrospective study. SETTING: Tertiary referral centre. PATIENTS: 15 patients with CHD with right to left shunt through an interatrial communication: three had repaired tetralogy of Fallot, five had repaired pulmonary atresia with intact ventricular septum, four had Ebstein's disease, and three had other CHDs. Two patients had had a stroke before closure of the interatrial communication. INTERVENTIONS: Percutaneous atrial septal defect (n = 6) or persistent foramen ovale (n = 9) closure. All patients underwent an exercise test before and after interatrial communication closure. RESULTS: Five patients were cyanotic at rest. During exercise, mean (SD) oxygen saturation diminished from 93.9 (3.8)% to 84.3 (4.8)% (p < 0.05). Interatrial communication closure led to an immediate increase of oxygen saturation from 93.9 (3.8)% to 98.6 (1.6)% (p < 0.05). At a median follow up of three years (range 0.5-5) all but one patient with a residual atrial septal defect had normal oxygen saturation at rest and during exercise. Maximum workload increased from 7.2 (1.9) to 9.0 (2.2) metabolic equivalents (p < 0.001). CONCLUSIONS: Percutaneous closure of interatrial communications associated with a right to left shunt allows restoration of normal oxygen saturation at rest, avoidance of desaturation during exercise, and improvement of exercise performance in patients with CHD.

[Relationship between aortic arch shape and blood pressure response after coarctation repair]. / Relation entre l'architecture de la crosse aortique et la pression artérielle après cure de coarctation.

The mechanisms of secondary hypertension after repair of coarctation of the aorta are not well understood. Abnormalities of the architecture of the aortic arch and their consequences on blood pressure have not been studied. In order to study the relationship between abnormalities or aortic arch architecture and resting blood pressure ninety-four patients without re-coarctation were followed up prospectively from 1997 to 2004 (mean age 16.9 +/- 8.1 years; mean weight 57.5 +/- 18.3 Kg; interval since surgery 16.3 +/- 5.4 years). All underwent MRI angiography of the thoracic aorta which enabled the abnormalities to be classified in 3 groups: gothic arch, crenellated arch and roman arch. Twenty-four patients (25.5%) were hypertensive and 70 (74.4%) normotensive. There were 40 gothic arches (42.5%). 14 crenellated arches (15%) and 40 roman arches (42.5%). Gothic arches were more commonly observed in the hypertensive patients (18/40, [45%, 95% CI 31-62]) than the crenellated arches (4/14, [28.5%, 95% CI 7-48]) or the roman arches (2/40, [5%, 95% CI 2-12]). Only the gothic arch was independently correlated with hypertension on multivariate analysis. The authors conclude that gothic deformation of the aortic arch is an independent predictive factor of hypertension in patients operated for coarctation with an excellent result on the isthmic region. Patients with a gothic appearance of their aortic arch should be followed up closely.

[Successful recanulization of superior venous vessels: a new challenge for interventional pediatric cardiology]. / Reperméabilisation des gros troncs veineux supérieurs: un nouveau défi pour la cardiologie interventionnelle pédiatrique.

UNLABELLED: The use of long-term central venous catheters is a routine in chronic pediatric diseases. Thrombotic complications progressively reduce the central venous capital and hamper the long-term management of these patients. OBSERVATION: We report two cases of obstruction of the central upper venous system and discuss of the techniques used to repermeabilize venous axes before the placement of a new central line. CONCLUSION: The control of the permeability of the central veins should be performed before any withdrawal of central catheters, repermeabilization of the venous axes being simpler when the central catheter is kept in place in the occluded vessel.

Late coronary complications after arterial switch operation for transposition of great arteries. Clinical and therapeutic implications.

The arterial switch operation is the procedure of choice for correction of transposition of the great arteries. Although offering excellent long term results, this procedure is burdened by the risk of acute and subacute coronary occlusion. No guide-lines exist for the management of acute or chronic ischemia in this setting. We briefly review the literature and present the results of our institution.

Delayed surgery in pericardial teratoma with neonatal hydrops.

Timing of neonatal surgery in cases of pericardial teratoma with hydrops is not standardised. We report two cases of hydropic premature newborns with pericardial teratoma in which surgery was delayed until respiratory and haemodynamic stabilisation. Mature teratoma was removed on day 3. The newborns were weaned from the ventilator on postoperative day 5 and 10, respectively. Both infants were doing well at 18 months, suggesting delayed surgery may be feasible and effective.

Cardiac abnormalities 15 years and more after adriamycin therapy in 229 childhood survivors of a solid tumour at the Institut Gustave Roussy.

The purpose of this paper was to determine the cardiac status in children 15 years or more after adriamycin therapy for a solid tumour. Of the 447 pts, 229 pts were fully studied and 218 were not. The following cardiac evaluations were proposed to all the 447 consecutive patients (pts): (1) cardiac Doppler US by one of two expert cardiologists; (2) cardiac rhythm and conduction abnormalities including 24-hour holter ECG; (3) (131)l-mlBG myocardial scintigraphy; (4) serum brain natriuretic peptide levels at rest; (5) an exercise test with VO(2) max measurement. The radiation doses delivered to 6 points in the heart were estimated for all patients who had received radiotherapy. Congestive heart failure was diagnosed in 24 of 229 (10%) evaluated pts, with a median interval of 15 years (0.3-24 years) from the first symptom after adriamycin treatment. Among the 205 remaining pts, 13 asymptomatic pts (6%) had severe (n=4) (FS<20%) or marked (n=9) (20< or =FS<25%) systolic dysfunction. In the 192 others, the median meridional end-systolic wall stress was 91 (53-135) and it exceeded 100 g cm(-2) in 52 pts. Using a Cox model, only the cumulative dose of adriamycin and the average radiation dose to the heart, were identified as risk factors for a pathological cardiac status. In conclusion, the risk of cardiac failure or severe abnormalities increases with adriamycin treatment, radiotherapy and time since treatment, even after a follow-up of 15 years or more. In our series, after an average follow-up of 18 years, 39% of the children had a severe cardiac dysfunction or major ventricular overload conditions. The risk increases with the dose of adriamycin and radiation received to the heart, without evidence for threshold.

[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.

[Early complications of stenting in children and young adults with congenital heart disease]. / Complications précoces de l'implantation de stents chez l'enfant et le jeune adulte ayant une cardiopathie congénitale.

We report the early complications of stent implantation. Since 1/1/97 to 1/12/02, we implanted 91 stents in 76 patients. Median age and weight were 15 years (range 0.3-43) and 49 kg (range 3-96). Primary diagnosis were: pulmonary atresia with ventricular septal defect (n = 17), tetralogy of Fallot (n = 13), aortic coarctation (n = 15), transposition of the great arteries (n = 3), truncus arteriosus (n = 6), univentricular heart (n = 7), other anomalies (n = 28). Indications to stent implantation were: stenosis of the pulmonary outflow and/or pulmonary branches (n = 44), aortic recoarctation (n = 14), native aortic coarctation (n = 1), systemic venous channels obstruction (n = 14), other (n = 3). The stents were: Palmaz (n = 57), non premounted CP (n = 8), premounted CP (n = 3), Palmaz Corinthian (n = 19), self-expanding (n = 4). Procedural complications occurred in 24 patients (31%) and included: 11 balloon bursting, 8 stent slipping, 1 stent migration, 4 wall dissections. Five patients (21%) needed urgent surgery. Sixty three percent of complications occurred during pulmonary outflow or pulmonary branches stenting, 25% during stenting of the aortic arch, 12% during other procedures. All but 2 complications were observed with non premounted stents. In conclusion, the use of not premounted stents, although widely diffuse, has a not negligible incidence of complications, leading to longer procedures, to the use of a large variety of materials and needing more than occasionally a surgical solution. A larger use of premounted stents, when possible, is recommended.

[Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiopathy]. / Facteurs prédictifs de bloc auriculo-ventriculaire complet tardif après traitement chirurgical des cardiopathies congénitales.

The late occurrence of complete atrio-ventricular block (CAVB) after cardiac surgery is rare but potentially responsible for cases of late sudden death. We searched for factors allowing prediction of this complication, retrospectively reviewing the case notes of 11 patients in hospital with complete AVB, 2 months to 10 years after correction of a cardiac malformation. All had a normal pre-operative ECG. The diagnosis had been made based on symptoms in 8 patients: syncope or collapse (4 cases) symptoms on effort (3 cases) or fatigue (1 case). In the others the diagnosis had been made on ECG. The block was infra-His in 5 patients who had electrophysiology. ECG analysis showed that all the patients had CAVB immediately post-operatively lasting 3-14 days. After restoration of conduction the ECGs showed the following anomalies compared to the pre-operative ECGs: long PR (1 case), long PR + right bundle branch block (2 cases), long PR + left axis deviation (1 case), RBBB + left deviation or rotation of the QRS axis (3 cases), long PR + RBBB + left axis deviation (4 cases). All of these patients had been fitted with a cardiac stimulator. In conclusion, the children who had CAVB immediately post-operatively lasting more than 48 hours and who then had an ECG showing different QRS compared to the pre-operative QRS and/or long PR had a risk of late complete AVB. These patients should have electrophysiology and a stimulator must be implanted in those who have an infra-His block.

[Cardiac retransplantation in childhood. Questions and answers]. / Retransplantation cardiaque en pédiatrie. Interrogations et réponses.

Cardiac transplantation in childhood remains a long-term palliative procedure and it is probable that an increasing number of patients undergoing this procedure in childhood will require a new transplant one day. Coronary artery disease of the transplanted heart is the main indication. The results (in terms of survival and morbidity) in the literature and in the authors' experience are encouraging and justify the indication for retransplantation in cases of severe coronary disease of the transplanted heart. The indication is not as clear-cut in transplantation performed early or as an emergency for primary graft dysfunction, which carries a high mortality. Renal failure and allo-immunisation are not contra-indications to this procedure. Occasionally, procedures of coronary revascularisation provide time to wait for a new transplant. In the absence of more effective preventive or curative treatment of coronary disease of the transplant, the good results of retransplantation justify the procedure for this indication and reinscription on the waiting list for cardiac transplantation.

[Evaluation of long-term outcome of Senegalese children sent abroad for cardiac surgery]. / Evaluation au Sénégal du devenir des enfants transférés pour chirurgie cardiaque.

The incidence of childhood heart disease in developing countries is high, but access to cardiac surgery is limited. This mismatch has given rise to numerous humanitarian programs aimed at sending children abroad for surgical treatment. However little is available about the long-term outcome of these interventions. In 1999 we conducted a retrospective study of 168 Senegalese children undergoing follow-up at the Principal Hospital in Dakar after being transferred to Europe or the Ivory Coast for surgical treatment thanks to the Terre des Hommes Association. A total of 85 children presented congenital heart disease (CHD) and 83 presented acquired heart disease (AHD). Fifteen patients did not undergo surgery due to either contraindications or preoperative death. At the end of study, 23 children had been lost to follow-up mostly from the CHA group and presumably some were cured. Outcome was verifiable in the remaining 145 patients with a median follow-up of 5.6 years. Ninety-seven patients were cured or undergoing surveillance. Quality of life was better in the CHD group (p = 0.047). Forty-eight patients died including 16 in the CHD group and 32 in the AHD group. Perioperative mortality (n = 19) was lower and late mortality (n = 29) was higher in the AHD group (p = 0.005). In the AHD group compliance with surveillance was better for children with valve prostheses. In children treated for isolated mitral valve insufficiency, late mortality was higher after valve replacement than valve repair (p = 0.04). In absence of comparative study data, high mortality was due in part to the long delay between the decision to send the patient abroad and the actual evacuation. These findings support humanitarian action to promote cardiac surgery in developing countries.

[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.

Assessment of muscular ventricular septal defect closure by transcatheter or surgical approach: a three-dimensional echocardiographic study.

AIMS: Previous classification of muscular ventricular septal defects (VSDs) visualized on two-dimensional echocardiography relied on artificial divisions of the septum. New visualization of the ventricular septum integrating the third dimension would facilitate communication between cardiologists and surgeons. The objectives of this study were (1) to assess in patients with muscular ventricular septal defects the accuracy of left ventricular three-dimensional echocardiographic reconstructions in demonstrating the position, the size and the tissue rims of the defects; (2) to compare findings by three-dimensional echocardiography with those obtained by surgical and transcatheter approaches. METHODS AND RESULTS: Twenty-six patients, aged from one month to 40 years, with muscular ventricular septal defects underwent three-dimensional echocardiographic study. From the left ventricular three-dimensional echocardiographic reconstructions, the localization, the maximal diameter and the tissue rim of the defect were analysed and compared with surgical or transcatheter findings. Optimal three-dimensional echocardiographic reconstructions were obtained in 22 patients. Nineteen had a single muscular ventricular septal defect and three had multiple muscular ventricular septal defects. The muscular ventricular septal defect localizations were the inlet septum in three, the outlet septum in three, the mid-muscular septum in 14 and the apex in eighth. In 10 patients who underwent surgical closure, the correlation between three-dimensional echocardiography and surgery for muscular ventricular septal defect maximal diameter was y=0 x 95 x +0.13 (r=0.98; P<0.001). The agreement between three-dimensional echocardiographic and intraoperative findings on muscular ventricular septal defect localization were complete. In five patients who underwent transcatheter closure, the mean difference between three-dimensional echocardiographic maximal diameter and stretched diameter was 1 x 8+/-0 x 5 mm. CONCLUSION: The three-dimensional echocardiographic left ventricular views provide a new and easily communicated visualization of various muscular ventricular septal defects. Such new imaging should contribute to the surgical and transcatheter treatments of muscular ventricular septal defects.

[Marfan syndrome in the newborn and infants less than 4 months: a series of 9 patients]. / Syndrome de Marfan chez le nouveau-né et le nourrisson de moins de 4 mois: une série de 9 patients.

We report a series of 9 children with neonatal Marfan syndrome. The diagnosis was made on a striking facial dysmorphia associated with arachnodactyly type skeletal anomalies. They all had cardiovascular anomalies: aortic dilatation 9 times, and mitral and/or tricuspid valve prolapse 8 times. Three children underwent ascending aorta replacement with the Yacoub technique. There was one operative death in a young infant and the two survivors required further surgery for gross mitral insufficiency and an aneurysm of the horizontal aorta. Three children died before the age of 1 year from cardiac insufficiency linked to massive leaks of all the valves. The three youngest children in the series did not undergo any procedure and were followed up for a slowly growing dilatation of the initial aorta. The prognosis of neonatal Marfan syndrome is weighted with a heavy mortality. The timing of surgical intervention in case of excessive dilatation of the aortic root is difficult to determine. It depends not only on the aortic diameter but also on the aortic valve function and the existence of other mitral or tricuspid lesions. Surgical treatment for lesions of the initial aorta and the atrioventricular valves improves the short-term prognosis but the risk of re-intervention is higher.