Anasarca Revealing Severe Cardiac Involvement Due to Behçet's Disease (BD): A Case Report.

Behçet's disease (BD) is a systemic vasculitis which is most often manifested by recurrent oral aphthosis, genital aphthosis, and ocular involvement with sometimes visceral damage, in particular neurological, digestive, vascular, or renal. We report the case of a 21-year-old man admitted for anasarca who revealed severe cardiac involvement associating endomyocardial fibrosis, intracardiac thrombi and involvement of the tricuspid valve in the context of BD diagnosed a posteriori. Cardiac involvement is exceptional during BD, especially as a mode of entry into the disease. It can be particularly severe, hence the need for early diagnosis, rapid and sometimes aggressive management. Close monitoring is also necessary in order to watch for the occurrence of visceral manifestations, particularly in young patients.

Extracardiac conduit as alternative repair for scimitar syndrome.

Scimitar syndrome has been repaired by different surgical procedures including intracardiac baffle technique, reimplantation of scimitar vein (SV) to the right atrium or the left atrium (LA). However, several anatomical variations such as short venous collector coursing deeply within the lung hilum with infradiaphragmatic drainage make the repair more challenging with conventional repair techniques. We present an alternative method for repair using a tube graft to connect the SV to the LA.

Adults forms of scimitar syndrome.

Scimitar syndrome is a rare malformation defined as the partial or total anomalous pulmonary venous return of the right lung veins to the inferior vena cava just above or below the diaphragm. Severe forms of the disease are diagnosed in infancy and childhood. However, because of the mild form of the syndrome in adult patients, they remain asymptomatic and few cases are reported in the literature. We report two adults cases of this syndrome with one presenting with chest discomfort.

[Persistence of the 5th aortic arch associated with interruption of the aortic arch]. / Persistance du 5ème arc aortique associé à une interruption de l'arche aortique.

We report a case of persistence of the 5th aortic arch associated with total interruption of the aortic arch. This clinical case shows the diagnostic pitfall of the persistence of the 5th aortic arch and its beneficial hemodynamic effect. Preoperative clinical picture was misleading, due to the persistence of femoral pulses and clinical signs of left-to-right shunt via a wide ductus arteriosus. The diagnosis was intraoperatively adjusted on the basis of blood pressure monitoring using catheter placed into the femoral artery.

Congenital left atrial appendage aneurysm: Atypical presentation.

Congenital left atrial appendage aneurysm is a rare condition caused by dysplasia of the atrial muscles. We report a case of a 14-year-old boy, with a 5-month history of cough and in sinus rhythm. Transthoracic echocardiography and computerized tomographic angiography confirmed the aneurysm of the left atrial appendage which was resected through median sternotomy on cardiopulmonary bypass. This case is presented not only for its rarity but also for its atypical clinical presentation.

[Cardiac pseudotumor revealing Behçet's disease]. / Pseudotumeur cardiaque révélant une maladie de Behçet.

Intracardiac thrombosis is a rare complication of Behçet's disease (BD), which may manifest as intracardiac tumor. In half of cases, its detection precedes the diagnosis of MB. High mortality rates may be related to post-surgical complications and/or pulmonary arteries involvement. We report the case of a 29-year old young patient, with a previous history of bipolar aphthosis, who underwent surgery after the detection of right atrium and ventricle tumor. Anatomo-pathological examination showed thrombus and MB was diagnosed in the postoperative period. Patient's evolution was favorable under medical treatment based on corticosteroids, colchicine and vitamin K antagonists (AVK). The detection of intracardiac mass in a young subject should suggest the diagnosis of cardiac thrombus and Behçet's disease, even in the absence of ethnic or geographical risk factors.

[Myxoma involving posterolateral leaflet: about a case]. / Myxome de la petite valve mitrale: à propos d'un cas.

Cardiac myxomas are the most common type of primary cardiac tumors. They mainly affect the interatrial septum and exceptionally the heart valves. Surgical excision remains the only therapeutic alternative. We here report the case of a 69-year old patient with no significant pathological history suffering from NYHA class II-III dyspnea associated with lipothymia. Transthoracic echocardiography showed a tight calcified aortic narrowing with aortic valve gradient of 58 mmHg. A sessile mass of 15mm diameter inserted into the posterolateral leaflet, without stenosis or mitral regurgitation evoking an atypical localization of myxomaor or fibroelastoma was detected at the level of the mitral valve. The examination was supplemented by ETO which confirmed the diagnosis of a mass involving the posterolateral leaflet. The patient underwent surgery via median sternotomy, under conventional extracorporeal circulation. Left atriotomy allowed to objectify a sessile mass of 15mm of diameter involving the auricular wall of the friable and easily cleavable posterolateral leaflet. Cauterization of the implant base via electric scalpel was then performed without any additional gesture on the posterolateral leaflet. Anatomopathologic analysis of the surgical specimen confirmed the diagnosis of myxoma. The patient also underwent aortic valve replacement with mechanical prosthesis. The postoperative course was uneventful. The patient was discharged on postoperative day 8. Cardiac myxoma involving the mitral leaflet is very rare. Surgical procedure attempting to resect the widest possible surgical margins remains the only therapeutic option to avoid the risk of recurrence.

Early results for active infective endocarditis.

INTRODUCTION: Cardiac surgery is frequently needed during active phase of infective endocarditis (IE). The purpose of this study was to analyze the immediate and late results and determine the risk factors for death. METHODS: We retrospectively reviewed 101 patients with IE operated in the active phase. The mean age was 40.5 ± 12.5 years. 16 patients (15.8%) were diagnosed with prosthetic valve endocarditis (PVE). 81 (80.9%) were in NYHA functional class III-IV. Blood cultures were positive in only 24 cases (23.9%). RESULTS: in-hospital mortality rate was 17.9% (18 cases). Multivariate analysis indentified five determinant predictor factors: congestive heart failure (CHF), renal insufficiency, high Euroscore, prolonged cardiopulmonary bypass time (> 120 min) and long ICU stay. The median follow-up period was 4.2 (2-6.5) years. Overall survival rate for all patients who survived surgery was 97% at 5 years and 91% at 10 years. CONCLUSION: Despite high in-hospital mortality rate, when patients receive operation early in the active phase of their illness, late outcome may be good.

[Giant congenital intrapericardial left atrial appendage aneurysm: about a case and review of the literature]. / Anévrysme congénital géant intra péricardique de l'auricule gauche: à propos d'un cas avec revue de la littérature.

Left atrial appendage aneurysm is a very rare heart anomaly. It may be congenital or acquired, secondary to inflammatory or degenerative processes. Most cases are asymptomatic. The prevalence of these lesions in pediatric age has been very rarely reported. As it can cause potentially fatal arrhythmias or thrombus, surgery is required immediately after diagnosis. This study reports the case of a 14-year-old boy with rapidly progressive dyspnea, palpitations, sensation of repetitive dizziness and fainting, in whom congenital left atrial appendage aneurysm was detected. Diagnosis was based on coronary CTA data. The patient was successfully treated with surgical resection of the aneurysm.

[Subaortic diaphragm surgery]. / La chirurgie du diaphragme sous aortique.

Subaortic diaphragm is characterized by a certain clinical latency and low morbi-mortality. Surgery remains the treatment of choice despite the real risk of long-term recurrence. Our study involved 18 patients with subaortic diaphragm operated between April 1994 and March 2011. The average age was 18.1 ± 9.7 years, 11 patients were male. The diaphragm was fibrous in 13 patients and fibromuscular in 5 patients. All patients underwent diaphragm resection associated with myectomy, aortic plasty, closure of ventricular septal defect and permeable ductus arteriosus ligation in 3, 3, 2 and 2 patients respectively. Operative mortality was zero and there were no cases of postoperative conduction disorder. With a median follow-up of 44.3±36.8 months, there was no late death. Two patients had a diaphragm recurrence which required reoperation with good evolution. The current trend in diaphragm surgery is towards early interventions and more extensive resections. However, the risk of recurrence requires a systematic and close ultrasound monitoring.

Evaluation of penetrating cardiac stab wounds.

BACKGROUND: The purpose of this study was to identify factors associated with unfavourable outcome following stab wounds to the heart in order to improve selection of patients who may benefit from resuscitative effort. METHODS: From February to March, variables were collected from medical records of patients sustaining cardiac trauma. The inclusion criterion was the presence of a penetrating cardiac injury confirmed intraoperatively. RESULTS: Ninety-eight patients were admitted with penetrating cardiac injury. The mortality rate was 60 %. Fifty-seven patients had unrecordable blood pressure at admission and emergency department thoracotomy was done in twelve patients. The AAST-OIS score was higher in non survivors group (4,21 vs 4,49). Multivariate analysis identified tamponade, associated injuries, right ventricular laceration as the most predictive variables for mortality. DISCUSSION: Stab wounds should be separated from gunshots wounds as the former mechanism has different pathophysiological issue. Patients arriving without signs of life may benefit from aggressive resuscitative efforts depending on transport time. CONCLUSION: Penetrating cardiac injuries are highly lethal condition. Cardiac tamponade, right ventricle lacerations and associated extra-cardiac injuries are independent risk factors of death.

Resection of secreting cardiac pheochromocytoma with and without cardiopulmonary bypass.

We report two cases of cardiac pheochromocytoma, the first with superior vena cava obstruction and the second involving the left atrium and extending to the posterior wall of the aorta. Both tumors were resected with disease-free margins, with and without the use of cardiopulmonary bypass, respectively. The patients remain asymptomatic at 8-year and 1-year follow-ups, respectively.