1/14. 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 = 1keywords = vascular disease (Clic here for more details about this article) |
2/14. ochronosis: an unusual finding at aortic valve replacement.The condition known as ochronosis refers to the accumulation of oxidized homogentisic acid in the connective tissues of alkaptonuric patients. The diagnosis is usually made from the triad of degenerative arthritis, ochronotic connective tissue pigmentation and urine that turns dark brown or black on alkalinization. Cardiovascular disease is a less well appreciated aspect of this disorder. A patient with ochronosis of his stenotic aortic valve is reported. The role of the pigment in the genesis of the valve degeneration is discussed.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
3/14. Trapped renal arteries: functional renal artery stenosis due to occlusion of the aorta in the arch and below the kidneys.Acute renal failure is a well recognized complication from the use of angiotensin-converting enzyme inhibitors in patients with severe bilateral renovascular disease. A 54-year-old woman presented with acute pulmonary edema with intractable hypertension and a history of lower limb claudication. The addition of lisinopril to her antihypertensive regimen resulted, within 48 h, in the development of acute renal failure that remitted with cessation of the drug. She was found to have a heavily calcified occlusion of her aortic arch and another occlusion of the aorta below the renal arteries. Angiography and Doppler ultrasonography showed normal renal arteries. This is the first reported case of angiotensin-converting enzyme inhibitor-induced renal failure occurring in a patient with atherosclerotic occlusion of the aorta. The literature on suprarenal aortic occlusion is reviewed to determine the manner of presentation, prevalent risk factors and physical findings that typify this unique clinical entity.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
4/14. Antegrade selective coronary angiography via the transseptal approach in a patient with severe vascular disease.We describe a case of a woman with severe vascular disease in whom retrograde access to the aortic root was limited by both aortoiliac and axillary disease. Transseptal catheterization was performed in anticipation of percutaneous aortic valvuloplasty. Selective antegrade angiography was successfully performed using catheters introduced through the transseptal sheath.- - - - - - - - - - ranking = 2.5keywords = vascular disease (Clic here for more details about this article) |
5/14. Hutchinson-Gilford progeria syndrome with severe calcific aortic valve stenosis and calcific mitral valve.The case of a 12-year-old girl with clinical features of progeria with severe calcific valvar aortic stenosis is presented. The mitral valve showed the presence of calcium, and peripheral vascular disease was also present, though there was no family history of this. Aortic valve replacement was deferred because of insufficient data relating to this condition. The genetics and phenotypic mechanisms of the disease are reviewed. In view of the association of progeria with valve disease, all patients should undergo electrocardiography and echocardiography as part of their routine work-up.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
6/14. Dual coronary artery fistula in a patient with aortic valve stenosis.Bilateral coronary artery fistulae originating from both right and left coronary arteries are rare congenital abnormalities that, in the adult population, are often associated with other acquired cardiovascular diseases. We briefly describe a 63-year-old woman with a dual coronary artery fistula and severe aortic stenosis. Both anomalies were successfully corrected surgically.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
7/14. Aortic occlusion in patients treated with cisplatin-based chemotherapy.cisplatin-based chemotherapy is one of the most common chemotherapy regimens that is complicated by thromboembolic events. A wide spectrum of vascular events exists, including venous and arterial thromboses of varying severity and location. However, total occlusion of the aorta is very unusual. We describe two patients with atherosclerotic vascular disease who developed occlusion of the abdominal aorta after cisplatin-based chemotherapy.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
8/14. williams syndrome in one dizygotic twin.williams syndrome is a disorder of unknown etiology with a characteristic facial appearance, vascular disease and infantile hypercalcemia. Most cases are sporadic. We report the case of a dizygotic male twin with the williams syndrome. This appears to be the first report of twins with only one affected. This suggests a mutational event as the cause of williams syndrome.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
9/14. Contractures in patients with williams syndrome.williams syndrome is a multisystem disorder, including a characteristic facies and habitus. Intellectual, neurologic, and cardiovascular dysfunction occur; vascular disease may be progressive. Joint contractures affected 10 of a group of 20 children and young adults. In 3 of 10 patients, the contractures were severe enough to interfere with daily activities; in 3 the effect was only moderate. The contractures developed in early childhood and usually did not improve or worsen with time. Large and small joints were affected and usually were symmetrical. Their occurrence did not correlate with vascular disease. The contractures could not be attributed to a neurologic cause.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/14. Aortic valvuloplasty: potential nursing diagnoses.The use of transluminal balloon angioplasty has become widespread in the treatment of cardiovascular diseases. Since critical care nurses are responsible for the assessment and management after the procedure in the critical care unit, the authors provide information on the pathophysiology and potential nursing diagnoses for these patients. In this way, the critical care nurse can support the patient before, during, and after the procedure.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
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