Nejm 1997;336:32

Including aortic stenosis (AS), aortic insufficiency (AI), mitral stenosis (MS), mitral regurgitation (MR) (Nejm 2001;345:740), tricuspid stenosis (TS), and tricuspid insufficiency (TI)

Cause:Many are rheumatic

Pathophys:Rheumatic pancarditis causes chordae fibrosis and shortening, and valvular vegetations at points of trauma. MS always present in RHD to some degree; hemoptysis in MS is due to shunts from pulmonary veins to bronchial veins. 70% of all pts with chronic MR in the 1960s was due to RHD, while 30% due to chordae rupture from myocardial infarction, endocarditis, and trauma. Severe AI alone rarely is due to RHD; usually from trauma, syphilis, dissecting aneurysm, endocarditis, SLE, RA, ankylosing spondylitis. AS now rarely (10%) is due to RHD, most is calcific aortic stenosis on bicuspid or previously normal valves (Nejm 2008;359:1395)

AS most common in elderly now; MS/MI in females age 20-40; TS/TI always accompanies mitral or aortic disease but occurs in only 2-4% of patients with rheumatic heart disease (RHD)

Sx:

MS: exertional dyspnea, orthopnea, hemoptysis

MR: exertional dyspnea, orthopnea

AS: angina, CHF, syncope

AI: gradual CHF sx

Si:

MS: atrial fibrillation; mid-diastolic murmur with presystolic accentuation even in atrial fibrillation (Nejm 1971;285:1284); opening snap; loud S₁

MR: loud systolic murmur, radiates to axilla

AS: CHF, loud systolic murmur radiates into carotids and left shoulder (uniquely); worsens with amyl nitrite inhalation in contrast to MR and VSD

AI: early diastolic murmur; this typical murmur is present in 73% (sens), and is heard only 8% of the time in patients who lack AI (92% specif—Ann IM 1986;104:599). Austin Flint murmur of functional MS narrowed by AI regurgitant jet. Very soft S₁ as mitral valve floats closed from the AI and indicating urgent need for surgery

MS: slow progression

MR: even if asx, if severe, has a 40% 5-yr mortality (Nejm 2005;352:875)

AS: if heavily calcified, <0.07 cm² but asx, 80% 4-yr mortality (Nejm 2000;343:611, 652); after angina 50% survive 5 yr; after syncope 50% survive 3 yr; after CHF 50% survive 2 yr

MS: CHF, SBE, emboli

AS: angina, sudden death, CHF, gi angiodysplasia with gi bleed disappears after valve replacement (Ann IM 1986;105:54). After onset of any of the 3 sx, mortality is 75% at 3 yr unless valve replaced (Mayo Cl Proc 1987;62:986)

Lab:

Cardiac cath:MS significant valve diameter <1.2 cm², no sx if >2.5 (L. Cobb 1971). AS critical if <0.07 cm²/M² (Nejm 1997;336:32)

Noninv:2D echo and Doppler for valves, chamber sizes, and estimates of valve diameters or regurgitation amounts; in AI, 90⁺% sens/specif (Ann IM 1986;104:599)

Xray:Chest, in MS shows large left atrium, pulmonary artery, and right ventricle, and may show valvular calcifications; in AS, may show 4-chamber enlargement, valvular calcifications usually only with significant disease

Rx:

MR: Repair before EF <60% or end diastolic ventricular dimension <45 mm; if due to full flail leaflet, repair sooner rather than later since prognosis w/o repair poor (Nejm 1996;335:1417)

MS: may do closed commissurotomy or percutaneous catheter commissurotomy via interatrial septum (Nejm 1994;331:961), which may diminish embolization if done early (Ann IM 1998;128:885); valve replacement if calcifications/clot, porcine (Nejm 1981;304:258) or bovine pericardium w higher structural failure rates vs mechanical w higher bleeding cmplc (Nejm 1993;328:1289). Control Afib w digoxin or -blocker; anticoagulate w warfarin especially if Afib, concomitant AI, or clot by echo (Ann IM 1998;128:885)

AI: vasodilator rx w nifedipine or ACEI does not delay LV dysfunction development or consequent valve replacement (Nejm 2005;353:1342). Repair before EF <55% or end diastolic ventricular dimension <55 mm

AS: valve replacement if angina, CHF, or syncope; even in elderly (>80 yr) if healthy, eg, 60% 5-yr survival (Jags 2008;56:255), and perhaps before sx develop. For high-risk pts, perhaps percutaneous new valve placement (Nejm 2010;363:1597, 1667)

of CHF: anticoagulate if platelet survival <3 d (Nejm 1974;290:537) or evidence of emboli; rheumatic fever penicillin prophylaxis; SBE prophylaxis.

Surgical: valve choices (Nejm 1996;335:407); w mechanical valve replacement, ideal protime INR is 3-4 or 3-5 for maximal benefit/risk ratio (Nejm 1995;333:11); w bioprosthetic valves, warfarin to INR = 2-3 × 3 mo, then just ASA unless other embolic risk factors like Afib, dyskinetic segment, etc; in pregnancy after valve replacement, warfarin is teratogenic in 1st trimester (25%) so heparin is used but is less than adequate anticoagulation (Nejm 1986;315:1390) and usually combined w ASA (for normal and abnormal heart sounds w various valves see table in Nejm 1996;335:410)