Ghassan Chehab

Fatal cardiac atherosclerosis in a child 10 years after liver transplantation: A case report and a review

We hereby review liver transplantation for homozygous familial hypercholesterolemia and report the case of a 14-year-old girl presenting with severe bilateral coronary ostial stenosis and tight supra-valvular aortic narrowing 10 years after liver transplantation. Despite normalization of the lipids after liver transplantation, the patient showed evidence of severe cardiac atherosclerosis 10 years later and died of apparent sepsis.

PULMONARY ARTERY ACCELERATED FLOW REVEALING HODGKIN'S LYMPHOMA.

We present a case in which transthoracic echocardiography was the first diagnostic tool to suspect mediastinal Hodgkins lymphoma by revealing a change in the hemodynamic of left pulmonary artery flow, and it was used as a follow-up method for monitoring treatment efficacy by demonstrating a normalization of pulmonary artery hemodynamics.

False and True Acute Rheumatic Fever : A Lebanese Experience

OBJECTIVES The main aim of this study is to evaluate the significance of several clinical and laboratory findings presented by a large number of pediatric patients who were suspected to have acute rheumatic fever (ARF) by physicians who ordered echocardiogram for confirmation and for avoiding overuse of unnecessary antibiotics. These suspecting reasons include elevated ASO titers and recurrent tonsillitis. Another aim is to stress the role of Doppler echocardiogram in confirming the diagnosis of ARF by detecting rheumatic heart disease (RHD) in suspected cases. MATERIALS AND METHODS 401 patients were registered in the National Registry of the Pediatric and Congenital Heart Disease (RNCPC) from April 1, 2002 until the end of March 2013 as suspected cases of ARF. The clinical reasons that brought suspicion are discussed and evaluated in accordance to modified Jones criteria. The patients are distributed into two groups: 1) confirmed ARF and 2) different diagnosis. All patients underwent echocardiogram. Patients came from different geographic areas and were referred by a variety of physicians. RESULTS 48 patients out of 401 suspected cases turned to have confirmed ARF (12%). In this group, the predominant suspecting reasons are reported as heart murmur in 29/48 (60.4%), arthritis in 28/48 (58.3%), arthralgia with reduced physical activity in 15/48 (31%) and chorea in 5/48 (10%). In the different diagnosis group with a high ASO titer 174/353 (49.3%), isolated arthralgia 91/353 (25.8%) and combined elevated ASO with arthralgia in 44/353 (12.5%) were the predominant suspecting reasons. Doppler echocardiography could detect 38/48 cases of active carditis and subclinical carditis (SCC). CONCLUSION Heart murmur, arthritis, arthralgia with reduced physical activity and chorea are the main clinical reasons that lead to suspicion in the ARF group. High ASO titers, arthralgia, and combination of high ASO titers with arthralgia are the main presenting symptoms in the group with a different diagnosis. We concluded that elevated ASO titer and normal ESR with arthralgia not reducing physical activity can exclude ARF. Doppler echocardiography is found to be a gold standard key to confirm acute rheumatic heart disease and ARF.

Insertion Linéaire des Valves Auriculo-Ventriculaires Chez les Enfants Avec et Sans Syndrome de Down

RÉSUMÉ • OBJECTIF : Étudier différents paramètres rencontrés dans l’insertion linéaire des valves auriculoventriculaires (ILVAV) chez les patients indemnes de trisomie 21 (T21 ou syndrome de Down) et ceux porteurs de cette anomalie. MATÉRIEL : Un groupe de patients (n = 6240) âgés de 1 à 16 ans, avec et sans cardiopathie congénitale (CC), inscrits au cours d’une période de 6 ans (1 juillet 200530 juin 2011) au Registre national de cardiologie pédiatrique et congénitale, Société libanaise de cardiologie. MÉTHODE : Les enfants ont été divisés en 2 groupes. Groupe I : enfants porteurs de T21 ; groupe II : enfants indemnes de T21. Dans les deux groupes, la fréquence de l’ILVAV isolée ou associée à d’autres CC (en dehors du canal atrioventriculaire), la répartition garçon-fille ainsi que les différentes anomalies cardiaques associées ont été étudiées. Les groupes ont été comparés par les tests non paramétriques. Une valeur de p < 0,05 est considérée significative. RÉSULTATS : Le groupe I comprend 155 patients dont 31 (20%) avec une ILVAV, isolée dans 22 cas (14,2%) et associée à une CC dans 9 cas (5,8%). Le groupe II comprend 5995 patients dont 14 (0,2%) sont porteurs d’une ILVAV avec p < 0,00001. Une nette prédominance masculine existe : 78% dans le groupe I et 56% dans le groupe II. Chez les patients avec ILVAV isolée, 86% étaient des garçons. Des antécédents de T21 sont retrouvés chez 3/7 des enfants sans T21 avec ILVAV isolée. Un patient T21 et chez qui une communication interauriculaire (CIA), type ostium primum avec ILVAV fut diagnostiquée durant le 1 mois de vie, a vu son défaut septal disparaître cinq mois plus tard. CONCLUSION : L’ILVAV isolée est beaucoup moins fréquente dans la population sans T21 mais ayant fréquemment des antécédents familiaux de T21 avec une nette prédominance masculine. Mots-clés : Liban, cardiopathie congénitale, sex ratio, ostium primum ABSTRACT • OBJECTIVE : Study various parameters encountered in the linear insertion of the atrioventricular valves (LIAVV) in patients with and without Down’s syndrome (DS). MATERIAL : A group of patients (n = 6240) aged from 0 to 16 years with and without congenital heart disease (CHD) registered over 6 years (1.07.2005-30.06.2011) in the National Registry of the Pediatric and Congenital Heart Disease of the Society of Cardiology in Lebanon. METHOD : Children were divided in two groups; Group I : children with DS and group II : children without DS. In the two groups, the frequency of LIAVV whether isolated or associated with other CHD (excluding the atrioventricular canal), the distribution boy-girl and the various associated cardiac anomalies were studied. We used nonparametric tests for comparing the two groups. The p value < 0.05 was considered significant. RESULTS : Group I consists of 155 patients including 31 (20%) with LIAVV, isolated in 22 cases (14.2%) and associated with CHD in 9 cases (5.8%). Group II was composed of 5995 patients ; the incidence of LIAVV was 0.2% (14 patients) with a p value < 0.00001. Regarding sex, there was a marked male predominance : respectively 78% and 56% in groups I and II. In patients with isolated LIAVV, 86% of patients were males. Family history of DS is found in 3/7 children without T21 with isolated LIAVV. A patient, in whom an atrial septal defect ostium primum type with LIAVV was diagnosed during the first month of life, has seen his septal defect closed spontaneously five months later. CONCLUSION : Encountered predominantly among a male population, the isolated LIAVV is less frequent in children without DS but often with family history of Down’s syndrom.