Issam El-Rassi

Surgical management of a circumflex aneurysm with fistula to the coronary sinus

We report the successful management of a circumflex coronary artery aneurysm with fistula to the coronary sinus. Our strategy aimed at closing the fistula and grafting the obtuse marginal artery. The calcified aneurysm was left intact, and showed secondary thrombus formation on the postoperative angiogram.

GATA5 mutation homozygosity linked to a double outlet right ventricle phenotype in a Lebanese patient

GATA transcription factors are evolutionary conserved zinc finger proteins with multiple roles in cell differentiation/proliferation and organogenesis. GATA5 is only transiently expressed in the embryonic heart, and the inactivation of both Gata5 alleles results in a partially penetrant bicuspid aortic valve (BAV) phenotype in mice. We hypothesized that only biallelic mutations in GATA5 could be disease causing.

Treatment of Heyde's Syndrome by Aortic Valve Replacement.

Heyde’s syndrome is the association between calcific aortic stenosis and gastrointestinal bleeding due to angiodysplasia. Alterations in von Willebrand factor due to turbulence across the diseased aortic valve have been incriminated in the pathophysiology of this syndrome. Replacement of the aortic valve has been reported to stop the bleeding, but this is debatable. Along with a review of the relevant medical literature, we hereby report a 68 year old patient with aortic stenosis and severe recurrent gastrointestinal bleeding that completely subsided following aortic valve replacement.

Three-dimensional printed models for surgical planning of complex congenital heart defects: an international multicentre study

OBJECTIVES To evaluate the impact of 3D printed models (3D models) on surgical planning in complex congenital heart disease (CHD). METHODS A prospective case-crossover study involving 10 international centres and 40 patients with complex CHD (median age 3 years, range 1 month-34 years) was conducted. Magnetic resonance imaging and computed tomography were used to acquire and segment the 3D cardiovascular anatomy. Models were fabricated by fused deposition modelling of polyurethane filament, and dimensions were compared with medical images. Decisions after the evaluation of routine clinical images were compared with those after inspection of the 3D model and intraoperative findings. Subjective satisfaction questionnaire was provided. RESULTS 3D models accurately replicate anatomy with a mean bias of -0.27 ± 0.73 mm. Ninety-six percent of the surgeons agree or strongly agree that 3D models provided better understanding of CHD morphology and improved surgical planning. 3D models changed the surgical decision in 19 of the 40 cases. Consideration of a 3D model refined the planned biventricular repair, achieving an improved surgical correction in 8 cases. In 4 cases initially considered for conservative management or univentricular palliation, inspection of the 3D model enabled successful biventricular repair. CONCLUSIONS 3D models are accurate replicas of the cardiovascular anatomy and improve the understanding of complex CHD. 3D models did not change the surgical decision in most of the cases (21 of 40 cases, 52.5% cases). However, in 19 of the 40 selected complex cases, 3D model helped redefining the surgical approach.

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.

Transfer Technique of an Anomalous Coronary Artery From the Anterior Pulmonary Artery

We describe a simple rerouting technique for an anteriorly situated anomalous left coronary artery from the pulmonary artery (ALCAPA), extending the left main coronary trunk with autologous aortic and pulmonary tissue. This technique is reproducible. It provides a tension-free aortocoronary anastomosis and a full potential for future growth.

Acute thrombosis of abdominal aortic aneurysm during cardiac surgery.

Aortic thrombosis has been described in the medical literature as a rare and catastrophic complication of abdominal aortic aneurysms. However, it has only been reported once in cardiac surgical settings. We report a unique case of thrombosis of an abdominal aortic aneurysms during the course of cardiac surgery, in a fully anticoagulated patient on cardiopulmonary bypass. Prompt diagnosis and immediate surgical management were critical for a successful outcome.

Surgical repair of partial atrioventricular defect

Long-term survival rate of patients operated for partial atrioventricular (AV) canal is lower than that of the general population, and late complications are relatively significant: between 10 and 30% of operated patients present with left AV valve regurgitation, and up to 25% have to be reoperated for valve repair or replacement, left ventricular outflow tract obstruction or residual atrial septal defect. Because the left AV valve regurgitation is the most common complication following surgery, technical details in the surgical management of the mitral valve are the most important aspects of this procedure; for example, the decision to close the cleft and to perform an annuloplasty. The presence of mitral valve anomalies in 7-28% of the cases complicates further the surgical management of these valves. This article will describe in detail the operative technique of partial AV canal repair, and review the relevant literature.

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.