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Transcript
Aortic Stenosis Causes Valvular AS - Congenital bicuspid valve o Suspect if in younger patients (40s-50s) o Presence of ejection click suggests bicuspid valve - Calcific degenerative valve o Suspect if in older patients (60s80s) - Rheumatic heart disease o Note: Isolated Aortic valve RHD is uncommon – usually tgt with mitral valve disease - Others (rare): Type II Hyperlipidemia, Radiation, SLE, Rheumatoid Subvalvular - Hypertrophic obstructive cardiomyopathy o Dynamic stenosis & LVOT obstruction o Due to genetic causes - Discrete subaortic membrane o Congenital malformation Supravalvular (Due to narrowing of ascending aorta or supravalvular fibrous diaphragm causing obstruction) - Williams Syndrome o Elfin facies, Pointed chin, Small and widely spaced teeth, Mental retardation Clinical Features Usually asymptomatic until AS is moderately-severe Symptoms: - Angina [Survival: 5 years] o ↑ Myocardial demand (LVH Ischemia of hypertrophic myocardium) o ↓ Coronary Blood Flow (Prolonged systolic ejection time ↓ diastole & coronary perfusion pressure) - Syncope [Survival: 3 years] o Exertional/exercise-induced syncope o Exercise ↑ left ventricular obstruction ↓ cardiac output ↓ Blood pressure Syncope - Dyspnea [Survival: 2 years] o Indication of LV failure! o Forward: Failure of LVH to compensate/maintain EF ↓ Ejection fraction Dyspnea o Backward: Pressure overload LVH ↓ LV compliance ↑ LV diastolic pressure Dyspnea Associated features: - Skin & mucosal bleeding o Acquired Type 2A von Willebrand syndrome secondary to severe AS o Mechanism: High shear stress as vWF passes through stenotic aortic valve Induce structural changes in shape of vWF Susceptible to proteolysis by ADAMST13 Loss of vWF o a/w Heyde’s syndrome: Gastrointestinal Angiodysplasia o Normal hemostasis is restored following aortic valve replacement - Familial hypercholesterolemia Differentials: - Aortic sclerosis o No radiation to carotids o No signs of severity (e.g. reverse splitting of S2, S4) - Pulmonary Stenosis - Microangiopathic Hemolytic Anemia (MAHA) Signs of Severity Murmur characteristics: - Late-peaking ESM - Reverse Splitting of S2 o Mild: A2 P2 o Moderate: A2 & P2 (single S2) o Severe: P2 A2 (delayed aortic valve closure) - Soft/inaudible A2 o Due to poorly mobile stenotic valve - Presence of S4 o Due to left ventricular failure Pulse/Apex beat: - Pulsus parvus et tardus o Slow-rising, small volume pulse - Narrow pulse pressure - Heaving apex beat Complications: - Cardiac failure Staging of Valvular Heart Disease - Stage A: At Risk o Patients with risk factors for developing valvular heart disease - Stage B: Progressive o Asymptomatic mild-tomoderate valvular heart disease - Stage C: Asymptomatic Severe o C1: Compensated LV/RV function (e.g. LVEF >50%) o C2: Decompensated LV/RV function (e.g. LVEF<50%) - Stage D: Symptomatic Severe Investigations Echocardiography (Imaging modality of choice!) - Assess Morphology of aortic valve o Confirm Diagnosis o Determine Etiology: Bicuspid, calcific degeneration, rheumatic o If aortic valve morphology is normal, likely to be supra/subvalvular AS - Assess Severity o Based on Valve Area & Trans-aortic pressure gradient Valve Area Pressure Gradient Mild >1.5cm2 <20mmHg Mod 1.0-1.5cm2 20-40 Severe <1.0cm2 >40mmHg - Assess LV Systolic Function o LVEF<50% in Severe AS Indication for surgery! Electrocardiogram - LV Hypertrophy with strain pattern (indicating pressure overload) - LA Enlargement, L Axis Deviation, LBBB Chest X-Ray - Aortic valve calcification (best seen on lateral view) - Post-stenotic dilation of ascending aorta Management Patients with severe valvular heart disease should be evaluated by a multidisciplinary Heart Valve Team whenever intervention is considered, comprising of cardiologist, cardiac surgeon, radiologist (cardiac imaging), anesthesiologist and nurses. Non-Pharmacologic Activity restriction - Avoid vigorous physical exertion in moderate & severe AS Regular follow-up for development of symptoms Pharmacologic No medical treatment to delay progression of AS Medical management of complications (e.g. Heart failure) Avoid certain medications: - Negative inotropes (ACE-I, BetaBlockers) - Venodilators (e.g. Nitrates) - Diuretics Surgical Aortic Valve Replacement - Indication for surgery: o Severe aortic stenosis o + 1 of the following: Symptomatic [D] Note: Survival rates drop drastically with onset of symptoms! – hence intervention is needed!! Ejection Fraction <50% [C2] Undergoing other cardiac surgery [C1,C2, D] (e.g. CABG, Aortic root surgery, Mitral valve repair) [Class I Recommendation based on ACC/AHA 2014 guidelines] Note: 3 indications in General: - Symptomatic AS (invariably severe) - Asymptomatic Severe AS with LVEF <50% - Asymptomatic Mod-severe AS undergoing cardiovascular operation Transcatheter Aortic Valve Implantation (TAVI) - Percutaneous prosthetic valve insertion under fluoroscopic & TEE guidance - Indicated for patients who are candidates for AVR but have prohibitive surgical risk