Approach to the patient with bicuspid aortic valve and ascending aorta aneurysm

Opinion statement Bicuspid aortic valve (BAV) disease is a common congenital heart valve abnormality accounting for a large number of valve replacements in the United States. Although still incompletely understood, the natural history of BAV disease is severe aortic stenosis and associated ascending aortic dilatation. In addition to the increased risk of endocarditis, aortic dissection and severe aortic valve dysfunction are responsible for most fatal complications. Thus, early and precise recognition of this condition is mandatory. The new American College of Cardiology/American Heart Association recommendations highlight the role of MRI and CT as complimentary tools to echocardiography for the diagnosis and surveillance of the morphology of the aortic valve and ascending aorta. Moreover, better understanding of the cellular mechanisms, including inflammation, bone formation, atherosclerotic-like processes, and aortic wall abnormalities, as well as the heritability and genetic predisposition for the disease, will define the potential for targeted medical therapies in the future. Currently, the treatment of this condition is primarily surgical. Although combined valve and ascending aorta replacement has been the most common surgical approach in the past, the increased cumulative risk of thrombotic and embolic events among these young patients has led to more conservative approaches. Several valve-sparing approaches with comparable mid-term results compared with the classic procedures have recently been reported. However, longer follow-up studies will be helpful to better define the advantages of these new surgical options. After a quick overview of the natural history of the BAV, this article provides an updated approximation of the current knowledge of the pathophysiology as well as the recommendations for the management and treatment of this disease.     

Acute dissection of the ascending aorta complicating aortic coarctation successfully treated with surgery

Vascular complications and arterial hypertension are major risk factors in the evolution of disease connected with coarctation of the aorta. Supra- and sub-stenotic aortic aneurysms are classical findings, whereas a dissecting aneurysm is a rare occurrence. In this study, we report on a case of acute dissection of the ascending aorta complicating a poorly followed-up coarctation of the aorta in a ten-year-old girl. The diagnosis, determined by X-ray of the thorax, was confirmed by echocardiography and magnetic resonance imaging. The first operative stage consisted of treating the aneurysm. After resection of the latter, an impregnated dacron prosthesis was inserted in termino-terminal. The immediate result is satisfactory, and a second operation is planned for the aortic coarctation.     

Valve sparing operation in a patient with aneurysm of ascending aorta with aortic regurgitation and concomitant ischemic heart disease

Bentall procedure is considered to be operation of choice for ascending thoracic aneurysms with aortic regurgitation. The however procedure has some inherent drawbacks (e.g. need of lifetime anticoagulation because of risk of late thromboembolic complications). These drawbacks can be eliminated by the use of valve saving surgery. We present a case of nontraditional valve saving operation combined with myocardial revascularization in a patient with ascending thoracic aneurysm, aortic regurgitation and ischemic heart disease. Technique of sinus of Valsalva plication for narrowing of sino-tubular ridge during prosthetic replacement of the ascending aorta turned out to be effective in this situation. The procedure described requires correct assessment of the state of aortic valve and selection of prosthesis of suitable diameter.     

Long-term follow-up after separate replacement of the aortic valve and ascending aorta.

Between May 1974 and November 1991, 28 patients underwent a separate replacement of the aortic valve and the ascending aorta (20 male, eight female; 32 to 71 years old, x = 52 years). 23 patients were operated for ascending aortic aneurysm, three for chronic and two for acute aortic dissection type A. 17/18 patients living at the beginning of this study were re-investigated after a mean follow-up interval of 8.5 years postoperatively with DSA, thoraco-abdominal CT and echocardiography. 1/8 biological aortic valves and 1/20 mechanical valves had to be replaced (four years and two months postoperatively) for valve degeneration and paravalvular leakage respectively. Three patients developed a sinus of Valsalva aneurysm and were reoperated five, 9.2 and 9.3 years after primary repair. In all three patients histological signs of idiopathic degenerative media disease of the aorta were found. Two other patients presented with a perfused perigraft channel and therefore had to be reoperated. Patients with chronic aortic dissection type A and/or ascending aortic aneurysms presenting clinical or intraoperative signs of degenerative media disease of the aorta should undergo composite graft replacement to preclude formation of sinus Valsalva aneurysms.     

Asymptomatic aortic dissection with large aneurysm of the ascending and the transverse aorta. Report of a case]

The authors present a cae of asymptomatic type I of the DeBakey aortic dissection with a voluminous aneurysm of the false channel. In the absence of suggestive symptoms, the diagnosis was established by chance through two dimensional echocardiography and later confirmed by magnetic resonance imaging. They ignore the period of time between the beginning of the dissection and the diagnosis, but they consider that it could have exceeded five years. During that time, besides as well tolerated aortic incompetence and aneurysmatic enlargement of the false channel, no other problems were detected. Based on this case, the authors are going through the literature and are discussing the importance of some noninvasive imaging techniques in the diagnosis and follow-up of this patients.     

A rare cause of dissection of ascending aorta after aortic valve replacement

We present a 78-year-old man who developed Stanford type A aortic dissection seven years after aortic valve replacement. At the previous operation, the diameter of the ascending aorta was 40 mm. Three years later, computed tomography revealed the diameter to have expanded to 50 mm. Four years later, the patient developed aortic dissection with entry at the ascending aorta, and a graft replacement was performed.     

Rupture of a thoracic aortic aneurysm after prosthetic bypass from the ascending aorta to the descending aorta in a case of congenital aortic arch hypoplasia

An unusual case of a successfully operated ruptured thoracic aneurysm of the aorta with bleeding into the atelectatic pulmonary left lower lobe and acute occlusion of the prosthesis in a 28-year-old woman is reported. The underlying disease was congenital aortic arch hypoplasia still present following implantation of a prosthetic bypass from the ascending aorta to the descending aorta performed in 1963.     

Aortic surgery, coarctation of the aorta, and aortic dissection

During the past year, significant advances were made in the insertion of composite valve grafts for the ascending aorta, replacement of the aortic arch, treatment of thoracoabdominal aortic aneurysms, and in the screening of patients with abdominal aortic aneurysms for cardiac disease. Controversy remains as how to best manage patients with aortic coarctation, and a recent symposium reviewed the techniques extensively. Methods of detecting aortic dissection have evolved, with increasing use of advanced technology such as transesophageal echocardiography and magnetic resonance imaging. The management of the aortic arch in patients with acute aortic dissection has been debated.     

Rupture of a thoracic aortic aneurysm in a patient with hypertension, myocardial infarction, hyperlipidemia and operation for abdominal aortic aneurysm rupture after follow-up for 28 years

A Japanese man who died at age 86 had been followed since the age of 58, when he presented with hypertension of 150/95 mmHg. The patient remained socially active until he died suddenly of a ruptured thoracic aortic aneurysm, although he experienced angina pectoris in August 1974, and myocardial infarction was identified on electrocardiography in October 1974. He underwent operation for rectal cancer in 1987, and an abdominal aortic aneurysm 38mm in diameter was identified at that time. The patient underwent an operation for rupture of the abdominal aortic aneurysm in 1991. A thoracic aneurysm of 40 mm diameter was identified in 1995, and this expanded to 53 mm by 1997. Autopsy revealed a thoracic aortic aneurysm in the arch (8 x 5 x 5 cm) and descending aorta (7 x 7 x 8 cm). A large volume (2,080 ml) of bloody pleural fluid was present