1/187. Aortic stenosis and coronary artery disease caused by alkaptonuria, a rare genetic metabolic syndrome.alkaptonuria is a rare metabolic disease in which homogentisic acid deposits occur in various body tissues. We present a case of alkaptonuria which resulted in aortic stenosis and coronary artery disease due to homogentisic acid deposition.- - - - - - - - - - ranking = 1keywords = coronary (Clic here for more details about this article) |
2/187. Iatrogenic left main coronary artery stenosis.Iatrogenic left main coronary artery stenosis is a potentially life-threatening complication of cardiac valve replacement surgery due to injury by perfusion cannulas. This requires prompt clinical recognition and diagnosis by repeat coronary angiography, and treatment by early coronary artery bypass grafting. We present 3 patients who had normal coronary arteries prior to valve replacement surgery, and who developed severe left main coronary artery stenosis after surgery. Accelerating angina and refractory ventricular arrhythmia were presenting clinical manifestations. coronary artery bypass grafting was successfully performed in all 3 patients.- - - - - - - - - - ranking = 1.8keywords = coronary (Clic here for more details about this article) |
3/187. Surgical angioplasty of left main coronary stenosis complicating supravalvular aortic stenosis.We successfully treated obstruction of the main coronary artery, not aortic valve leaflet adhesion to the intimal shelf, complicating supravalvular aortic stenosis by modifying the Brom aortoplasty. An autologous pericardial patch was used to enlarge the left main coronary artery as well as the stenotic aorta. This modification allows simple and effective restoration of coronary blood flow, while maintaining the Brom procedure's merit of achieving anatomic geometry of the aortic root in such patients.- - - - - - - - - - ranking = 1.4keywords = coronary (Clic here for more details about this article) |
4/187. Application of successive principles of repair to correct supravalvular aortic stenosis.The diffuse form of supravalvular aortic stenosis may extend in the takeoff of the coronary and arch arteries. Relief of the supravalvular stenosis requires a harmonious enlargement of the sinotubular junction to maintain aortic valve competence. The technique we used in a 9-year-old boy involved patch enlargement of all affected structures. Deep hypothermic circulatory arrest and retrograde cerebral perfusion was used during repair of the aortic arch and arch arteries.- - - - - - - - - - ranking = 0.2keywords = coronary (Clic here for more details about this article) |
5/187. Characterization of a novel cellular defect in patients with phenotypic homozygous familial hypercholesterolemia.Familial hypercholesterolemia (FH) is characterized by a raised concentration of LDL in plasma that results in a significantly increased risk of premature atherosclerosis. In FH, impaired removal of LDL from the circulation results from inherited mutations in the LDL receptor gene or, more rarely, in the gene for apo B, the ligand for the LDL receptor. We have identified two unrelated clinically homozygous FH patients whose cells exhibit no measurable degradation of LDL in culture. Extensive analysis of dna and mRNA revealed no defect in the LDL receptor, and alleles of the LDL receptor or apo B genes do not cosegregate with hypercholesterolemia in these families. FACS((R)) analysis of binding and uptake of fluorescent LDL or anti-LDL receptor antibodies showed that LDL receptors are on the cell surface and bind LDL normally, but fail to be internalized, suggesting that some component of endocytosis through clathrin-coated pits is defective. Internalization of the transferrin receptor occurs normally, suggesting that the defective gene product may interact specifically with the LDL receptor internalization signal. Identification of the defective gene will aid genetic diagnosis of other hypercholesterolemic patients and elucidate the mechanism by which LDL receptors are internalized.- - - - - - - - - - ranking = 0.0037360010999456keywords = circulation (Clic here for more details about this article) |
6/187. Alkaptonuric aortic stenosis: a case report.alkaptonuria is a rare disease of phenylalanine, aromatic amino acids, and tyrosine metabolism. Because of a genetic deficiency of the enzyme homogentisic acid oxidase, an accumulation of homogentisic acid causes ochronotic pigment deposition. The most common clinical manifestations are arthropathy, urinary calculi and discoloration, cutaneous and cartilaginous pigmentation, and cardiac valvular disease. Arthropathy and aortic stenosis are the most debilitating manifestations of the disease. A case of alkaptonuric aortic stenosis is described. A 75-year-old woman with a history of alkaptonuria presented in the emergency department with complaints of progressive dyspnea. Upon examination, the patient was hypertensive, tachypneic, and tachycardic with premature ventricular contractions. She had pitting edema of the lower extremities and complaints of generalized weakness. Chest x-rays revealed congestive heart failure and pulmonary edema. diuretics were administered, and a continuous nitroglycerin infusion was initiated in the emergency department. The patient was admitted for further evaluation. The patient's respiratory status continued to decline. She was intubated endotracheally 1 day after admission. Subsequent cardiac evaluation revealed an ejection fraction of 35%, severe aortic stenosis, mild coronary artery disease, ischemic cardiomyopathy, and anteroapical akinesis. A dobutamine infusion was instituted for persistent hypotension, and renal dose dopamine was initiated for oliguric renal failure. The patient underwent an emergency operation for an aortic valve replacement with a Dacron patch 10 days after admission. cardiopulmonary bypass and mild hypothermia were used during the procedure. The patient's hemodynamic status remained tenuous throughout the procedure. Although the first attempt to wean off cardiopulmonary bypass failed, the second attempt was successful with the aid of an intra-aortic balloon pump, inotropic support, and atrioventricular pacing. These measures were maintained during transport to the surgical intensive care unit. In the intensive care unit, the patient did not have an audible blood pressure or a palpable pulse without the support of the intra-aortic balloon pump and atrioventricular pacing. Coarse atrial fibrillation was the underlying electrocardiogram rhythm in the absence of atrioventricular pacing. sodium bicarbonate was given without improvement. After discussion with the family, all life support measures were discontinued. The patient died 10 minutes after her arrival in the intensive care unit. alkaptonuria's pathogenesis is manifested as both local and systemic in nature. collagen vascular diseases share a similar pattern of multisystem involvement. Despite the negative outcome for the patient described, valuable insight can be obtained by studying this case and noting the anesthetic considerations specific to collagen vascular diseases in general.- - - - - - - - - - ranking = 0.2keywords = coronary (Clic here for more details about this article) |
7/187. Pulmonary stenosis caused by extrinsic compression of an aortic pseudoaneurysm of a composite aortic graft.Pulmonic stenosis and stenosis of the right ventricular outflow tract related to extrinsic compression have been described in patients with tumors, in a patient with a pericardial cyst, and in patients with vascular abnormalities as an unruptured sinus of valsalva aneurysm, a giant coronary artery pseudoaneurysm and an aortic arch aneurysm. Composite graft replacement of the ascending aorta and aortic valve with reimplantation of the coronary arteries has some inherent complications. Our case report describes a patient with a pericomposite graft aneurysm presenting as a stenosis of the pulmonary artery, detected by Doppler echocardiography.- - - - - - - - - - ranking = 0.4keywords = coronary (Clic here for more details about this article) |
8/187. Surgical angioplasty of the left main coronary artery in non-atherosclerotic lesions.Surgical angioplasty of the left main coronary artery confers several advantages over conventional bypass surgery: unrestricted forward flow is provided to the entire coronary bed and graft material is spared. The literature contains many reports of surgical angioplasty of atherosclerotic stenoses. The technique is described in five patients with non-atherosclerotic disease of the left main coronary artery: three children (a 7 year old girl who had undergone an arterial switch operation shortly after birth; a 9 year old boy with congenital supravalvar aortic stenosis; and a 10 year old girl with Kawasaki's disease) and two adults (a 51 year old woman with post-radiation stenosis; a 53 year old man with acute dissection). All patients had an uneventful recovery and are free from symptoms with a widely open left main trunk. Although technical difficulties are increased in these patients, excellent results can be achieved with this approach.- - - - - - - - - - ranking = 1.4keywords = coronary (Clic here for more details about this article) |
9/187. Supravalvular aortic stenosis, williams syndrome and sudden death. A case report.Supravalvular aortic stenosis (SVAS) is an uncommon but well characterized congenital narrowing of the ascending aorta above the level of the coronary arteries. It can be a familial disorder, can occur sporadically, or associated with williams syndrome (WS) which is a neurodevelopmental disorder affecting connective tissue and the central nervous system. Sudden death is a well-known complication of non-syndromic SVAS but few cases have been reported associated with WS. We present a case of sudden death in a woman with the diagnosis of SVAS and WS since the age of 3 years who refused surgical correction and died at the age of 27 years. At autopsy, the aorta and pulmonary trunk were narrowed and the walls showed peculiar microscopical characteristics. In the cardiac conduction system the His bundle was small and intramyocardial. The incidence, pathology, pathogenesis and prognosis of both conditions (SVAS and WS) are reviewed.- - - - - - - - - - ranking = 0.2keywords = coronary (Clic here for more details about this article) |
10/187. Living related donor liver transplantation in a patient with severe aortic stenosis.We report the successful anaesthetic management of a young girl with Alagille's syndrome and severe aortic stenosis (resting pressure gradient 88 mm Hg) undergoing living related donor liver transplantation (LRDLT). The patient had end-stage liver disease and LRDLT was performed before replacement of the aortic valve. Anaesthesia was conducted uneventfully with the aid of a pulmonary artery catheter. intra-aortic balloon pumping was used in the perioperative period for protection against myocardial ischaemia. Total clamping of the inferior vena cava was avoided during surgery and volume administration was guided by the pulmonary artery pressure. A stable circulation was maintained in the reperfusion period. The patient was discharged from hospital on day 54 after operation with normal liver function. Two years later her aortic valve was replaced successfully.- - - - - - - - - - ranking = 0.0037360010999456keywords = circulation (Clic here for more details about this article) |
| | Next -> |