Aortic stenosis

Deletions in the elastin gene (located at 7qll.23) have been found in approximately 90% of subjects with Williams syndrome, a developmental disorder affecting connective tissue and the central nervous system. The mutations, by affecting synthesis of elastin, probably play a causative role in the supravalvular aortic stenosis often found in this condition. A number of skin diseases (eg, scleroderma) are associated with accumulation of elastin. Fragmentation or, alternatively, a decrease of elastin is found in conditions such as pulmonary emphysema, cutis laxa, and aging of the skin. 

Decreased cardiac output Heart failure Sepsis Pulmonary hypertension Aortic stenosis (and other valvular abnormalities) Anesthetics... 

Hyperkaiemia Elevated serum potassium (at least 0.5 mEq/L greater than the upper limit of normal) was observed in 0.4% of hypertensive patients given trandolapril, approximately 1% of hypertensive patients given benazepril, enalapril, ramipril, or moexipril approximately 2% of patients receiving quinapril or lisinopril, approximately 2.6% of hypertensive patients given fosinopril, and approximately 4.8% of CHF patients given lisinopril. Hyperkalemia also occurred with captopril. Vaivuiar stenosis Theoretically, patients with aortic stenosis might be at risk of decreased coronary perfusion when treated with vasodilators, because they do not develop as much afterload reduction as others. 

Patients with the following underlying conditions can be particularly sensitive to the actions of vasodilators, including sildenafil, tadalafil, and vardenafil Those with left ventricular outflow obstruction (eg, aortic stenosis, idiopathic hypertrophic subaortic stenosis) and those with severely impaired autonomic control of blood pressure. 

Valve surgery with severe aortic stenosis or mitral regurgitation... 

Contraindications Advanced aortic stenosis, severe hypotension... 

Serious side effects are rare and result from improper use of these agents, as when intravenous verapamil (or diltiazem) is given to patients with sinus or atrioventricular nodal depression from drugs or disease, or nifedipine to patients with aortic stenosis. 

May include select patients with mild aortic stenosis LVD (NYHA class I)... 

Hywel Davies reported of temporarily treatment of aortic regurgitation with a parachute valve mounted onto a catheter tip in 1965 (34). Twenty-seven years later Andersen and his colleagues described the first experience with a bioprosthetic valve attached to a wire-based stent and mounted on a balloon valvuloplasty catheter (35). In 2002, Alain Cribier performed the first transcatheter valve implantation in an elderly patient with inoperable aortic stenosis using a prototype of a stent-mounted, pericardial, tricuspid aortic valve (36). 

Cribier A, Savin T Saoudi N, et al. Percutaneous transluminal valvuloplasty of acquired aortic stenosis in elderly patients an alternative to valve replacement Lancet 1986 I (8472) 63-67. 

Otto CM, Mickel MC, Kennedy JW, et al. Three-year outcome after balloon aortic valvuloplasty, Insights into prognosis of valvular aortic stenosis, Circulation I 994 89(2) 642-650. 

Cribier A, Eltchaninoff H, Bash A, et al. Percutaneous transcatheter implantation of an aortic valve prosthesis for calcific aortic stenosis first human case description. Circulation 2002 ... 

Cribier A, Eltchaninoff H, Tron C, et al. Early experience with percutaneous transcatheter implantation of heart valve prosthesis for the treatment of end-stage inoperable patients with calcific aortic stenosis. J Am Coll Cardiol 2004 43(4) 698-703. 

Curran ME, Atkinson DL, Ewart AK, Morris CA, Leppert MF, Keating MT. The elastin gene is disrupted by a translocation associated with supravalvular aortic stenosis. Cell 1993 73(1) 159 168. 

Li DY, Toland AE, Boak BB, Atkinson DL, Ensing GJ, Morris CA, Keating MT. Elastin point mutations cause an obstructive vascular disease, supravalvular aortic stenosis. Hum Mol Genet 1997 6(7) 1021-1028. 

Tassabehji M, Metcalfe K, Donnai D, Hurst J, Reardon W, Burch M, Read AP. Elastin genomic structure and point mutations in patients with supravalvular aortic stenosis. Hum Mol Genet 1997 6(7) 1029-1036. 

Load creates hyperhomocysteinemia (HHcy) The chronic pressure or volume overload by hypertension, aortic stenosis, and aortic vena cava fistula increases Hey by volume retention, in addition, by decreasing the activities of MTHFR, MS, CBS, and CGL enzymes (Figure 3.3). During load Hey chelates the Cu2+ in CytC, Cox,... 

Hypertensive emergency is a rare, but life-threatening situation in which the diastolic blood pressure is either over 150 mm Hg (with systolic blood pressure greater than 210 mm Hg) in an otherwise healthy person, or 130 mm Hg in an individual with preexisting complications, such as encephalopathy, cerebral hemorrhage, left ventricular failure, or aortic stenosis. The therapeutic goal is to rapidly reduce blood pressure. 

A 70-year-old woman with a history of mastectomy developed syncope which lasted a few seconds. She had taken tamoxifen 10 mg bd for 10 years and had no history of allergic reactions. Doppler ultrasound showed aortic stenosis and coronary angiography was performed using 150 ml of iopromide (a non-ionic contrast medium, iodine 370 mg/ml). She had visual hallucinations (spiders on the wall, moving curtains) 30 minutes after the injection of iopromide. The symptoms resolved 72 hours later without any specific treatment. Neurological and psychiatric examinations were normal, as were brain MRI and Doppler ultrasound of the carotid and vertebral arteries. 

Thorough cardiac examination should look for possible cardiac source of embolism, including atrial fibrillation, mitral stenosis and prosthetic heart valves. Left ventricular hypertrophy suggests hypertension or aortic stenosis, and a displaced apex from a dilated left ventricle indicates underlying cardiac or valvular pathology. 

Left ventricular thrombus Left ventricular dyskinesis Mitral stenosis Mitral annulus calcification Aortic stenosis... 

A report from the Mayo Clinic has described 27 elderly patients (mean age 71 years) with aortic stenosis in whom dobutamine stress hemodynamic testing was used to assess the severity of the stenosis (12). There were no severe adverse effects, but relatively minor problems occurred in 16 patients, including chest pain and ventricular extra beats (n = 9 each) and atrial dysrhythmias (n — 4). The authors concluded that the procedure appears to be safe in these high-risk patients, although its diagnostic value may be limited. 

Lin SS, Roger VL, Pascoe R, Seward JB, Pelhkka PA. Dobutamine stress Doppler hemodynamics in patients with aortic stenosis feasibility, safety, and surgical correlations. Am Heart J 1998 136(6) 1010-16. 

A 70-year-old man developed heart failure secondary to ischemic heart disease and severe aortic stenosis (1). Furosemide 20 mg/day was replaced by torasemide 5 mg/day. After the second dose he developed oliguria and an erythematous morbilliform rash with palpable violet petechial lesions on the legs. Chest X-ray showed bilateral alveolar infiltrates. Serum creatinine and potassium were raised (212 pmol/1 and 6.7 mmol/1 respectively). Skin biopsy showed leukocytoclastic vasculitis. After withdrawal of torasemide, his renal function improved (serum creatinine 97 pmol/1) and the skin lesions resolved (leaving residual pigmented areas) within 8 days. 

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