We report the clinical and molecular findings in a patient with a mild form of recessive dystrophic epidermolysis bullosa and aortic insufficiency. To our knowledge, this is the first report of association between dystrophic epidermolysis bullosa and abnormalities of the aortic valve. Analysis of the COL7A1 gene has revealed two new mutations, a 20-bp duplication and a splice site mutation.

background: recent interests have mainly focused on the roles of serum calcium and phosphorus and their product (ca-p product) in the development of valvular heart disease. the present study assessed the relationship between the ca-p product and the severity of valvular heart disease in end-stage renal disease (esrd) patients undergoing chronic hemodialysis. methods: this cross-sectional study ...

introduction aortic surgery has made much progress recently. yet the combination of aortic surgery and coronary artery bypass grafting has remained a major issue for the surgeons. patient and observation herein, we present a case of severe aortic insufficiency with aortic aneurysm concomitant with single coronary artery disease on the right coronary artery. the patient underwent bentall procedu...

from may 1994 to december 1996 thirty patients received viable fresh aortic homografts. mean age was 55 years (13 to 70), male to female ratio was 2/1, 24 operations were elective whereas 6 were semiurgent. predominant lesions were aortic stenosis or regurgitation in lb patients, aortic valve endocarditis in 10, prosthetic valve dysfunction in 3, and aortic root pathology in one patient. from t...

Variations in certain slopes of the carotid pulse which exist among normal subjects, patients with aortic valvular disease, and those with hypertrophic subaortic stenosis were examined. Records from 256 subjects were quantified with computer technics. The externally recorded volume pulses were related to simultaneously recorded brachial artery pressures (cuff); thus the slopes are expressed in ...

Variations in certain slopes of the carotid pulse which exist among normal subjects, patients with aortic valvular disease, and those with hypertrophic subaortic stenosis were examined. Records from 256 subjects were quantified with computer technics. The externally recorded volume pulses were related to simultaneously recorded brachial artery pressures (cuff); thus the slopes are expressed in ...

Although classic and standard procedure for patients with ascending Aorta aneurysm with Sever aortic Regurgitation is Bentall surgery but in some selected cases this complex surgery was not performed with different reason like advanced age and coexisting comorbidity involving renal and hepatic and lung Function. In our cases 75 years old man with chronic renal failure ( Cr= 3.2 ) who was known ...

OBJECTIVES Surgery for aortic root aneurysm without valve stenosis is increasingly being transformed from the Bentall procedure to valve-sparing aortic root remodelling or reimplantation. In this report, a new repair option is explored, with full functional 'restoration' of the aortic root complex using a geometric annuloplasty ring, leaflet repair, and sinus/ascending aortic replacement with a...

OBJECTIVES To prove whether different indications for valve sparing aortic root reconstruction may have an impact on the outcome and longevity of the repair. METHODS From July 1993 to March 2003, the reimplantation technique for valve sparing aortic root reconstruction was applied to 232 patients. In 44 patients, indication for operation was acute aortic dissection type A (AADA). These patien...