Valvular Heart Disease, Emergency Medicine

Basics

Description

Mitral stenosis:
- Obstruction of diastolic blood flow into the left ventricle (LV)
Mitral regurgitation:
- Inadequate closure of the leaflets allows retrograde blood flow into the left atrium (LA).
- Acute: Pressure overload in LA and pulmonary veins causing acute pulmonary edema
- Chronic: LV volume overload with dilatation and hypertrophy with LA enlargement
Aortic stenosis:
- Obstruction of LV outflow with increased systolic gradient
- Progressive increase in LV systolic pressure and concentric hypertrophy
Aortic regurgitation:
- Acute LV pressure and volume overload leading to left-heart failure and pulmonary edema
- Chronic volume overload with LV dilation and hypertrophy

Pregnancy is associated with significant hemodynamic changes that can aggravate valvular heart disease and increase the risk of thromboembolic events.

Degenerative valvular disease is most common (aortic stenosis and mitral regurgitation)
Aortic valve replacement is the most common surgical procedure

Etiology

Mitral stenosis:
- Rheumatic fever
- Cardiac tumors
- Rheumatologic disorders (lupus, rheumatoid arthritis)
- Myxoma
- Congenital defects: Parachute valve
Mitral regurgitation (acute):
- Ruptured papillary muscle (infarction, trauma)
- Papillary muscle dysfunction (ischemia)
- Ruptured chordae tendineae (trauma, endocarditis, myxomatous)
- Valve perforation (endocarditis)
- Weight-loss medications (fenfluramine, dexfenfluramine)
Aortic stenosis:
- Congenital aortic stenosis: Male > female (4:1)
- Congenital bicuspid valve (1 " 2%)
- Rheumatic aortic stenosis
- Calcific aortic stenosis
Aortic regurgitation:
- Infective endocarditis
- Rupture of sinus of Valsalva
- Acute aortic dissection
- Chest trauma
- Following valve surgery
- Bicuspid aortic valve
- Rheumatic fever
- Weight-loss medications (fenfluramine, dexfenfluramine)
- Collagen vascular or connective-tissue diseases
- Systematic lupus erythematosus
- Marfan syndrome
- Pseudoxanthoma elasticum
- Ankylosing spondylitis
- Ehlers " Danlos syndrome
- Polymyalgia rheumatica

Diagnosis

Signs and Symptoms

Mitral stenosis:
- Malar flush ( "mitral facies " )
- Prominent jugular A-waves
- Right ventricular lift
- Loud S1
- Opening snap
- Low-pitched diastolic rumble
- Exertional dyspnea
- Fatigue
- Palpitations
- Paroxysmal nocturnal dyspnea
- Orthopnea
- Hemoptysis
- Systemic emboli
- Pulmonary edema
Mitral regurgitation:
- Acute pulmonary edema
- Jugular venous pressure (JVP) exhibits cannon A-waves and giant V-waves.
- Harsh blowing apical crescendo " decrescendo murmur radiating to the axilla
- Palpable thrill at apex
- S3 and S4
- Palpitations
- Atrial fibrillation
- Dyspnea
- Orthopnea
- Nocturnal paroxysmal dyspnea
- Peripheral edema
- Systemic emboli
- Normal JVP
- Left ventricular hypertrophy (LVH)
- Apical high-pitched pansystolic murmur
- Decreased or obscured S1
- Widely split S2
- S3
Aortic stenosis:
- Exertional angina
- Syncope (during exercise)
- CHF (initially diastolic failure, then systolic)
- Sudden death secondary to ventricular fibrillation
- Harsh crescendo " decrescendo (diamond-shaped) systolic murmur at aortic focus radiating to carotids
- Absent aortic component of S2
- Delayed upstroke in peripheral pulse (pulsus parvus et tardus)
- S4 gallop
- Ejection click
Aortic regurgitation:
- Fatigue
- Dyspnea on exertion
- Paroxysmal nocturnal dyspnea
- Orthopnea
- Syncope
- Acute pulmonary edema
- High-pitched blowing decrescendo diastolic murmur at aortic area
- Accentuated A2 heart sound
- Wide pulse pressure
- Corrigan pulse (collapsing pulse)
- Duroziez sign (to-and-fro murmur)
- De Musset sign (head bobbing with systole)
- Quincke pulse (nail bed pulsations)
- Austin Flint murmur (soft diastolic rumble)

Essential Workup

History and symptoms
Thorough cardiopulmonary exam
ECG

Diagnosis Tests & Interpretation

Lab

Blood cultures
Presumed endocarditis
CBC:
- Anemia

Imaging

CXR:
- Mitral stenosis:
  - Enlarged LA
  - Pulmonary vascular congestion (Kerley B lines)
  - Prominent pulmonary arteries
- Mitral regurgitation:
  - LV and LA enlargement in chronic cases
  - Pulmonary edema and normal LV and LA dimensions in acute cases
- Aortic stenosis:
  - LVH
  - Aortic calcification
  - Dilation of ascending aorta
  - Pulmonary congestion and cardiomegaly
- Aortic regurgitation:
  - Acute = normal cardiac silhouette and pulmonary edema
  - Chronic = enlarged LV and dilated aorta
ECG:
- Quality assessment of valvular structures
- Measurements of flow through valves
- Identification of regurgitation
- Ventricular dilatation or hypertrophy
Spiral CT scan:
- To exclude aortic dissection with acute aortic regurgitation

Diagnostic Procedures/Surgery
EKG:

Mitral stenosis:
- LA enlargement (broad notched P-waves)
- RV hypertrophy
- Right axis deviation
- Atrial fibrillation
- Acute mitral regurgitation:
- Left atrial enlargement
- LVH
- Left axis deviation
Aortic stenosis:
- LVH most common
- Atrial fibrillation
- Interventricular conduction delay
- Complete AV block
Aortic regurgitation:
- Acute = LV strain
- Chronic = LVH and strain

Differential Diagnosis

See Etiology.

Treatment

Pre-Hospital

Avoid vasodilators in aortic stenosis.

Initial Stabilization/Therapy

ABCs
Administer oxygen.
Monitor and measure pulse oximetry.
IV access

Ed Treatment/Procedures

Mitral stenosis:
- Treat symptoms of CHF.
- Rate control if in atrial fibrillation
- Digoxin
- ²-blockers
- Heparin (if new-onset atrial fibrillation)
- Diuretics
- Endocarditis prophylaxis/education
Mitral regurgitation:
- Differentiate between acute and chronic MR:
- Acute:
  - Afterload reduction (nitroglycerin, morphine, or sodium nitroprusside)
  - Diuresis
  - Intra-aortic balloon pump (temporizing for urgent surgery)
- Chronic:
  - Diuresis
  - Nitrates
  - Hydralazine
  - ACE inhibitor
  - Digoxin
  - ²-adrenergic blocker (ventricular rate control)
  - Calcium antagonist (ventricular rate control)
  - Heparin (if atrial fibrillation)
  - Endocarditis prophylaxis/education
Aortic stenosis:
- Gentle diuresis if CHF
- Mild hydration if hypotensive and not in CHF
- Avoid nitrates and afterload reduction.
- Digoxin
- Intra-aortic balloon pump (temporize for surgery)
- Endocarditis prophylaxis/education
Aortic regurgitation:
- Chronic:
  - Preload and afterload reduction
  - Digoxin
  - Diuretics
  - Endocarditis prophylaxis/education
- Acute:
  - Preload and afterload reduction
  - Intra-aortic balloon pump
  - Urgent surgery

Medication

Atenolol: 0.3 " 2 mg/kg/d PO, max. 2 mg/kg/d (peds: 1 " 2 mg/kg/dose PO daily suggested)
Digoxin: 0.5 mg bolus IV, then 0.25 mg IV q2h up to 1 mg; 0.125 " 0.375 mg/d PO
Diltiazem: 0.25 mg/kg IV over 2 min (repeat in 15 min PRN with 0.35 mg/kg) then 5 " 15 mg/h
Enalapril: 1.25 mg IV q6h; PO 2.5 " 10 mg BID (peds: 0.1 " 0.5 mg/kg/d PO div. q12 " 24h; max.: 0.58 mg/kg/d or 40 mg/d
Esmolol: IV: 500 ¼g bolus, then 50 " 400 ¼g/kg/min
Furosemide: 20 " 80 mg/d PO/IV/IM; titrate up to 600 mg/d for severe edematous states (peds: 1 mg/kg IV/IM slowly under close supervision; not to exceed 6 mg/kg)
Heparin: 80 U/kg IV bolus, then 18 U/kg/h drip, adjust to maintain partial thromboplastin time 1.5 " 2 control (INR 2 " 3)
Hydralazine: 10 " 25 mg IV q2 " 4h (peds: 0.1 " 0.5 mg/kg IM/IV q4 " 6h; max. 20 mg/dose)
Metoprolol: 5 mg IV q2min 3 doses; then 50 mg PO q6h 48 hr
Nitroglycerin: Start at 20 ¼g/min IV and titrate to effect (up to 300 ¼g/min); SL 0.3 " 0.6 mg PRN; USE NON-PVC tubing. Start at 5 ¼g/min, titrate up by 5 ¼g/min every 3 " 5 min until desired effect. Topical 1/2 " 2 in of 2% q6h (peds: 0.25 " 0.5 ¼g/kg/min IV, increase by 0.5 " 1 mg/kg/min; max. 20 ¼g/kg/min)
Phentolamine: 5 mg bolus IV, then 1 " 2 mg/min IV infusion
Propranolol IV: 1 " 3 mg at 1 mg/min
Sodium nitroprusside IV: 0.5 ¼g/kg/min; increase in increments of 0.5 to 1 ¼g/kg/min q5 " 10min up to 10 ¼g/kg/min
Amoxicillin: 2 g PO 1 h before the procedure; alternatively, 3 g PO 1 h before the procedure, followed by 1.5 g PO 6 h after the initial dose:
- Pediatric dose: 50 mg/kg PO 1 h before procedure
Ampicillin: 2 g IV/IM 30 min before the procedure (peds: 50 mg/kg IV/IM 30 min before the procedure)
Clindamycin: 600 mg PO 1 h before procedure (peds: 20 mg/kg PO 1 h before procedure; not to exceed 600 mg)

Follow-Up

Disposition

Admission Criteria

New-onset atrial fibrillation
CHF/pulmonary edema
Hemodynamically unstable
Acute mitral or aortic regurgitation
Cardiac ischemia
Angina
Syncope
Arrhythmias

Discharge Criteria

Hemodynamic stability
Unchanged ECG
Resolution of CHF symptoms with diuresis
Chronic mitral regurgitation

Issues for Referral

For patients who are candidates for outpatient management, close follow-up with a cardiologist to assess severity of valvular disease and need for cardiac surgery
Educate patient about risks of valvular heart disease and need for antibiotic prophylaxis with dental and medical procedures.

Pearls and Pitfalls

In patients with chest pain and aortic stenosis, nitrates are contraindicated.

Additional Reading

Bonow RO, Cheitlin MD, Crawford MH, et al. Task Force 3: Valvular heart disease. J Am Coll Cardiol. 2005;45(8):1334 " 1340.
Carabello BA, Crawford FA. Valvular heart disease. N Engl J Med. 1997;337(1):32 " 41. [Erratum: N Engl J Med. 1997;337:507].
Chen RS, Bivens MJ, Grossman SA. Diagnosis and management of valvular heart disease in emergency medicine. Emerg Med Clin North Am. 2011;29(4):801 " 810.
Elkayam U, Bitar F. Valvular heart disease and pregnancy part I: Native valves. J Am Coll Cardiol. 2005;46:223 " 230.
Rahimtoola SH. The year in valvular heart disease. J Am Coll Cardiol. 2013;61(12):1290 " 1301.
Roldan CA, Shively BK, Crawford MH. Value of the cardiovascular examination for detecting valvular heart disease in asymptomatic subjects. Am J Cardiol. 1996;77:1327 " 1331.

Codes

ICD9

394.0 Mitral stenosis
424.0 Mitral valve disorders
424.90 Endocarditis, valve unspecified, unspecified cause
395.0 Rheumatic aortic stenosis
429.5 Rupture of chordae tendineae
429.6 Rupture of papillary muscle
746.4 Congenital insufficiency of aortic valve
746.89 Other specified congenital anomalies of heart

ICD10

I05.0 Rheumatic mitral stenosis
I34.0 Nonrheumatic mitral (valve) insufficiency
I38 Endocarditis, valve unspecified
I06.0 Rheumatic aortic stenosis
I51.1 Rupture of chordae tendineae, not elsewhere classified
I51.2 Rupture of papillary muscle, not elsewhere classified
Q23.1 Congenital insufficiency of aortic valve
Q24.8 Other specified congenital malformations of heart

SNOMED

368009 Heart valve disorder (disorder)
79619009 Mitral valve stenosis (disorder)
48724000 Mitral valve regurgitation (disorder)
72011007 Rheumatic aortic stenosis (disorder)
274098004 Rupture of chordae tendineae (disorder)
5919001 Rupture of papillary muscle
70320004 congenital anomaly of heart valve (disorder)
72352009 bicuspid aortic valve (disorder)

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