Hazim J. Safi

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The major function of vascular smooth muscle cells (SMCs) is contraction to regulate blood pressure and flow. SMC contractile force requires cyclic interactions between SMC alpha-actin (encoded by ACTA2) and the beta-myosin heavy chain (encoded by MYH11). Here we show that missense mutations in ACTA2 are responsible for 14% of inherited ascending thoracic(More)
BACKGROUND A genetic predisposition for progressive enlargement of thoracic aortic aneurysms leading to type A dissection (TAAD) is inherited in an autosomal-dominant manner in up to 19% of patients, and a number of chromosomal loci have been identified for the condition. Having mapped a TAAD locus to 3p24-25, we sequenced the gene for transforming growth(More)
Between 43,000 and 47,000 people die annually in the United States from diseases of the aorta and its branches and continues to increase. For the thoracic aorta, these diseases are increasingly treated by stent-grafting. No prospective randomized study exists comparing stent-grafting and open surgical treatment, including for disease subgroups. Currently,(More)
PURPOSE The purpose of this study was to retrospectively identify variables associated with early death and postoperative complications in patients undergoing thoracoabdominal aortic operations. METHODS The data on 1509 patients who underwent 1679 thoracoabdominal aortic repairs between 1960 and 1991 were retrospectively reviewed. The median age was 66(More)
The vascular smooth muscle cell (SMC)-specific isoform of alpha-actin (ACTA2) is a major component of the contractile apparatus in SMCs located throughout the arterial system. Heterozygous ACTA2 mutations cause familial thoracic aortic aneurysms and dissections (TAAD), but only half of mutation carriers have aortic disease. Linkage analysis and association(More)
OBJECTIVE To report the long-term results of our experience using cerebrospinal fluid drainage and distal aortic perfusion in descending thoracic and thoracoabdominal aortic repair. SUMMARY BACKGROUND DATA Repair of thoracoabdominal and thoracic aortic aneurysm by the traditional clamp-and-go technique results in a massive ischemic insult to several major(More)
Non-syndromic thoracic aortic aneurysms and dissections (TAADs) are inherited in an autosomal dominant manner in approximately 20% of cases. Familial TAAD is genetically heterogeneous and four loci have been mapped for this disease to date, including a locus at 16p for TAAD associated with patent ductus arteriosus (PDA). The defective gene at the 16p locus(More)
Graft inclusion and vessel reattachment to openings made in the graft were employed in the treatment of 605 patients with thoracoabdominal aortic aneurysms. These patients were divided into four groups on the basis of the extent of aneurysm. Group I consisted of those patients with involvement of most of the descending thoracic and upper abdominal aorta;(More)
We report the improving surgical results in a consecutive series of 690 patients referred to one of us (E.S.C.) for aortic dissection between December 1956 and September 1989, a substantial portion of whom had dissection as a complication of either previous aortic (n = 113, 16) or previous cardiac (n = 54, 8%) operation. Our initial operation of choice in(More)