Search

Summary: Morgagni hernia is a rare malformation (3% of diaphragmatic hernias). This hernia is usually asymptomatic in children. We report on a case revealed by an unusual complication. Severe cyanosis was due to right-to-left atrial shunt through the foramen ovale assessed by 2D echocardiography. Diagnosis of the Morgagni hernia was made with CT scan. The intrathoracic liver compressed the right chambers of the heart causing tamponade. Cardiac compression was reversed after surgery and replacement of the liver in the abdomen. Six months after the surgery, the infant was symptom-free with normal size right chambers of the heart. [ABSTRACT FROM AUTHOR]

Summary: Endocarditis is uncommon in infants and is exceptionally related to Candida albicans on pulmonary banding. We report on a case in a 7-month-old infant who had pulmonary artery banding for a ventricular septal defect and who presented with candidal endocarditis. Banding was chosen because of the patient''s poor trophic and unstable status, which could be risky for surgery involving extracorporeal circulation. A few weeks after the banding, the patient developed systemic Candida infection, which was treated successfully. At 7 months, cardiac failure appeared without fever or inflammatory signs. Cardiac echography showed that the banding was not protective as well as a hyperechogenic image on the pulmonary bifurcation. The angioscan showed a hypodense thrombus. Emergency surgery was performed consisting of pulmonary artery exploration, thrombectomy, and ventricular septal defect closure. The exploration showed a pulmonary artery perforation caused by the infected pseudoaneurysm and the migration of the banding into the pulmonary artery. The anatomopathologic analysis of the vegetation identified multisensitive Candida albicans. After surgery and prolonged antifungal treatment, progression was satisfactory. [Copyright &y& Elsevier]

Purpose: Pediatric Kawasaki ocular involvement is dominated by bulbar conjunctival injection and mild, self-limited anterior uveitis. Posterior segment involvement is rare., Methods/results: Case Report. Despite early efficient treatment including aspirin and intravenous immunoglobulins, a 12-year-old girl developed a severe bilateral global inflammatory ocular involvement including punctuated keratitis, retrodescemetic precipitates, anterior uveitis, vitritis, and bilateral optic disc swelling with papillitis., Discussion: This is the first description of severe bilateral global inflammatory involvement of the eyes in Kawasaki disease (KD). Usually subclinical and self-limited, eye involvement in KD can lead to severe visual impairment., Conclusions: Inflammation of both anterior and posterior segments does not seem to respond to KD-specific treatment and could justify a specific ophthalmologic therapeutic approach.

We report a severe neonatal presentation of Ebstein anomaly with homodynamic aggravation at birth attributed to patent ductus arteriosus and circular shunt. Prenatal diagnosis of Ebstein anomaly of the tricuspid valve with functional pulmonary atresia was made at 37 weeks gestation by fetal two- and three-dimensional echocardiography. A cesarean section was performed in view of poor fetal tolerance. The newborn was born with hydrops and multivisceral failure. A post-natal echocardiography demonstrated a left-to-right shunt across the patent ductus arteriosus and functional pulmonary atresia with severe pulmonary insufficiency with absent forward flow. This created a circular shunt, where blood flowed through the ductus to the pulmonary arteries, retrograde through the pulmonary artery and Ebstein valve, across the patent foramen ovale and out the aorta. Prostaglandin E1 infusion was stopped, resulting in clinical and echocardiographic improvement. The management of the phenomenon of a circular shunt across a patent ductus arteriosus with Ebstein malformation involves promoting early ductal closure by stopping prostaglandin therapy., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

We report a case of fetal myocarditis without conductive abnormality in a pregnant woman with anti-Ro/La antibodies. Fetal echocardiography showed myocarditis with ventricular and valvular hyperechogenicity, which was confirmed by postnatal transthoracic echography. Treatment with dexamethasone (4 mg/day) was started in the 22nd week of gestation. The outcome was good, with the child remaining asymptomatic 2 years later. This observation describes one of the rare forms of fetal myocarditis with favorable outcome., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Morgagni hernia is a rare malformation (3% of diaphragmatic hernias). This hernia is usually asymptomatic in children. We report on a case revealed by an unusual complication. Severe cyanosis was due to right-to-left atrial shunt through the foramen ovale assessed by 2D echocardiography. Diagnosis of the Morgagni hernia was made with CT scan. The intrathoracic liver compressed the right chambers of the heart causing tamponade. Cardiac compression was reversed after surgery and replacement of the liver in the abdomen. Six months after the surgery, the infant was symptom-free with normal size right chambers of the heart., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Endocarditis is uncommon in infants and is exceptionally related to Candida albicans on pulmonary banding. We report on a case in a 7-month-old infant who had pulmonary artery banding for a ventricular septal defect and who presented with candidal endocarditis. Banding was chosen because of the patient's poor trophic and unstable status, which could be risky for surgery involving extracorporeal circulation. A few weeks after the banding, the patient developed systemic Candida infection, which was treated successfully. At 7 months, cardiac failure appeared without fever or inflammatory signs. Cardiac echography showed that the banding was not protective as well as a hyperechogenic image on the pulmonary bifurcation. The angioscan showed a hypodense thrombus. Emergency surgery was performed consisting of pulmonary artery exploration, thrombectomy, and ventricular septal defect closure. The exploration showed a pulmonary artery perforation caused by the infected pseudoaneurysm and the migration of the banding into the pulmonary artery. The anatomopathologic analysis of the vegetation identified multisensitive Candida albicans. After surgery and prolonged antifungal treatment, progression was satisfactory.

Background: The proximal isovelocity surface area (PISA) method is validated to quantify mitral regurgitation (MR) and ventricular shunt (VS). However, the two-dimensional echocardiography (2DE) PISA method assumes a hemispherical distribution of velocity factors proximal to the MR or VS orifice., Aim: To assess the PISA shape by three-dimensional echocardiography (3DE) in a paediatric population with MR or VS. According to the true PISA shape, we suggest different models to calculate the MR or VS volume by the 3DE PISA method., Methods: Thirty-one paediatric patients (aged 1month to 20years, median 69months) were included: 17 had MR and 14 had VS. The orifice area and volume of MR and VS were evaluated by 2DE. 3DE acquired the entire PISA volume at orifice level. The PISA shape was estimated according to three diameters as being hemispherical, prolate hemispheroid, oblate hemispheroid and hemiellipsoid., Results: Data from 28patients were analysed. The PISA shape was variable: hemispherical, 11%; prolate hemispheroid, 43%; oblate hemispheroid, 32%; hemiellipsoid, 14%. Oblate hemispheroids occurred more frequently in the MR group (47%), whereas prolate hemispheroids occurred more frequently in the VS group (62%); hemispheres were scarce in both groups (10%). The mean MR or VS orifices and volumes measured by 2DE and 3DE were significantly different (0.123cm(2) versus 0.094cm(2) and 13.2mL versus 10.1mL, respectively; p=0.019)., Conclusions: 3DE describes the true surface of the PISA shape. In a paediatric population with MR or VS, the PISA is rarely hemispherical but is more often prolate or oblate hemispheroid.

Background: Evaluation of mitral-regurgitation (MR) severity in infants is challenging. Real-time three-dimensional echocardiography (RT3DE) allows accurate left-ventricular volumetric measurements in adults., Aims: To validate RT3DE by measuring stroke volume in a normal paediatric population, then to use this new method to calculate regurgitant volume in paediatric patients with MR., Methods: Fifty-four patients, aged one week to 19 years, (29 without and 25 with MR) had two-dimensional echocardiography coupled with RT3DE left-ventricular volumetric acquisition. Stroke volume was calculated by the Doppler method at the aortic annulus (SV(D)). End-systolic and end-diastolic left-ventricular volumes were measured using the QLab semi-automated method; three-dimensional stroke volume (SV(3D)) was calculated as their difference. In the MR group, regurgitant volume was calculated by the PISA method (RV(PISA)) and as the difference between SV(3D) and SV(D) (RV(3D)). Regurgitant fraction was also evaluated by these methods (RF(PISA) and RF(3D))., Results: Measurement feasibility was 88%. In the normal group, SV(3D) (27.9+/-18.1 ml) was highly correlated with SV(D) (30.7+/-19.6 ml; r=0.98; p<0.000). In the MR group, RV(PISA) (15.7+/-14.4 ml) and RV(3D) (11.0+/-10.2 ml) were well correlated (r=0.83; p<0.001). Regurgitant fractions were also well correlated (RF(PISA)=30.4+/-17.0%; RF(3D)=24.3+/-15.9%; r=0.79, p=0.006)., Conclusion: RT3DE is a simple, rapid and reliable method for evaluating stroke volume in children and may, therefore, be useful for evaluating regurgitant volume and fraction in paediatric patients with MR.

Three-dimensional echocardiography (3DE) enables new views of heart valves and the septa to be imaged. While the previous 3DE system was cumbersome, the recent introduction of live 3DE allowed for routine use of the technique in adult patients. Here, we report our initial experiences in adapting live 3DE and the adult matrix probe to the pediatric population. Thirty-four 3DE examinations were performed on children, aged 1 day to 12 years (n = 23; median 4 years) and fetuses 20-33 weeks in gestation (n = 11; median 25 weeks), many of whom had various congenital heart diseases. The pediatric matrix probe (2-7 MHz) was used for 2D, Doppler, and 3DE. New modalities of the Vision 2007 (Philips) were applied: live, full volume, thick slice, 3D color Doppler, the QLAB system for navigation, and cropping. The pediatric matrix probe allows for complete 2D and 3D echocardiography, and new acoustic windows are now available to perform live 3DE. The higher frequency of the probe increases the 3D image resolution obtained in neonates and fetuses. This advancement allows new views of the aorta, pulmonary valve, septa and intra cardiac anatomy to be captured. Real time 3DE is a feasible method in addition to conventional 2D echocardiography for evaluating congenital heart disease.

Left ventricular diastolic function may change at an early stage in cardiac disease. It is often difficult to assess in daily practice. The use of Doppler tissue imaging at the annulus has been validated in adults. This method is little used in paediatrics and the physiological norms have not been established in children. Forty three children aged 7 days to 241 months were referred for a cardiological opinion with normal echocardiogrammes were included. Myocardial velocities were measured by Doppler tissue imaging of the left and right ventricular walls at different moments of the cardiac cycle in the apical 4-chamber view. A complete study was possible in 39 cases (91%). Doppler tissue imaging was not performed in one case and was incomplete on the right ventricle in 3 children. The median of the lateral mitral tissue E wave (Ea) was 16.3 cm/s and that of the right ventricle was 15.8 cm/s with a tissue Ea/Aa ratio of 2.6 and 1.6 respectively. The median of the tissue S waves was 8.8 cm/s for the left ventricular lateral wall and 13.3 cm/s for the right ventricular lateral wall. The E/Ea ratio of the left ventricular lateral wall was 5.9. Although the velocities of the left ventricular lateral wall were not related to the children's' age or size, the correlations between the E/Ea ratio and age and size were statistically significant. The myocardial velocities of the neonate were characteristic and different to those of the older paediatric population (slower Ea and S waves, faster Aa with a higher E/Ea ratio). The authors conclude that Doppler tissue imaging is feasible in clinical paediatric cardiology. Comparative studies with populations with cardiac disease are necessary to determine pathological values.

The authors report the case of a neonate with transposition of the great arteries (TGA) with pulmonary stenosis (PS) and intact Interventricular septum. The child was born at full term without a prenatal diagnosis with cyanosis developing at the 12th hour. After emergency atrioseptostomy, detransposition was performed on the 6th day. Commissurotomy and shaving of the much thickened bicuspid pulmonary valve was also performed. The postoperative course was complicated by a non-compliant left ventricle. After two months, myocardial ischaemia and persistent obstruction of the outflow tract led to severe cardiac failure and the death of the child. TGA with PS and intact interventricular septum is a very rare cardiac malformation. The prognosis is closely related to the anatomy of the pulmonary valve and the impact on the left ventricle. The therapeutic options are complex and are discussed in relation to this case.

Much progress has been made over the last few years in understanding and classifying neuromuscular diseases. The heart is frequently affected but often in a dissociated manner with respect to the neuromuscular signs although it has a significant impact on the prognosis. In children and adolescents, the dystrophinopathies, especially Duchenne's muscular dystrophy, are the principal problems but the mild arrhythmic events observed seem to be related to left ventricular dysfunction. On the other hand, in myotonic dystrophies (Steinert's disease), ventricular arrhythmias or conduction defects may appear at an early stage of the disease with serious consequences justifying appropriate follow-up and invasive preventive measures. Emery Dreifuss X-linked dystrophy and other laminopathies are rare conditions but are associated with sudden death and cardiomyopathies of the young adult. Specialised cardiological follow-up is justified in childhood from the time of diagnosis. Medication or implantable electric devices may be justified before the end of the second decade of life. Progressive infra-hisian conduction defects have also been reported in Kearns-Sayre oculo-pharyngeal myopathy. Prospective studies are required at this age to determine the natural history of these pathologies that are probably under diagnosed. The present recommendations, which are based mainly on data from adult series, could then be adapted for younger patients.

Cardiac tumors are rare in childhood and can be revealed by arrhythmias. We report the observation of an 18-month-old infant who had an episode of ventricular tachycardia (VT) which resulted in a large intramyocardic tumour diagnosis evocating a left ventricular fibroma. A treatment by amiodarone allowed a stable reduction of the VT. The presence of an intracardiac obstruction or uncontrollable arrhythmias would lead to a surgical resection.

The authors report a case of Staphylococcus Aureus infectious endocarditis in a premature baby. Echocardiography on the 8th day of life showed a large vegetation on the anterior mitral leaflet without significant regurgitation. Intravenous antibiotics and platelet antiaggregant therapy were given. At three weeks of age the vegetation split into two, one part attached to the foramen ovale and the other to the anterior mitral leaflet. At one month, the development of severe mitral regurgitation led to surgical valvuloplasty in a 2 kg neonate, the vegetation on the foramen ovale having spontaneously fragmented. The ablation of the mitral vegetation associated with commissuroplasty reduced the mitral regurgitation. At 3 months after surgery, the child was asymptomatic with a minimal mitral regurgitation.