תוצאת חיפוש

Familial Occurrence of Ebstein Anomaly

Ruti Margalit-Stashefski, Avraham Lorber, Eyal Margalit

Family Practice Unit, Kupat Holim Klalit, Haifa, Pediatric Cardiology Unit, Rambam Hospital, Haifa and Ophthalmology Dept., Hadassah--University Hospital, Jerusalem

Ebstein anomaly is a rare congenital disease which affects location, structure and mobility of the tricuspid valve, and right atrium and ventricle. Although most cases are sporadic, familial occurrence has been reported. We report 2 brothers born with Ebstein anomaly. The parents were first degree cousins and there were 8 other children. 2 daughters were born with other congenital heart anomalies, 1 with ventricular septal defect and the other with severe pulmonary artery stenosis. We suggest that in some families, Ebstein anomaly is an autosomal dominant disease with different expression in the sexes.

Q Fever Endocarditis and Bicuspid Aortic Valve

Ora Shovman, Jacob George, Yehuda Shoenfeld

Medial Dept. B and Autoimmune Disease Research Unit, Chaim Sheba Medical Center, Tel Hashomer and Sackler of Medicine, Tel Aviv University

Q fever is caused by the rickettsia Coxiella burnetti, an obligate intracellular bacterium acquired by inhalation of infected dust from subclinically infected animals. Q fever may be acute or chronic; the chronic form mostly presents as endocarditis. Immunocompromised states and underlying heart disease are the most important risk factors. Usually the symptoms of Q fever endocarditis are nonspecific and diagnosis is often established very late. New criteria for diagnosis include a single blood culture positive for Coxiella burnetti, positive Q fever serology and characteristic echocardiographic studies. We describe a 49-year-old man with bicuspid aortic valve admitted with fever, weight loss and a new heart murmur. The diagnosis of Q fever endocarditis was established by positive Q fever serology, and an echocardiogram showing vegetations and valvular dysfunction. This case suggests that Q fever endocarditis should be considered in patients with "sterile" endocarditis.