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Vasospastic (Prinzmetal) Angina

Cardiology > Valvular Heart Disease

Valvular Heart Disease

1. Knuuti J, Wijns W, Saraste A, Capodanno D, Barbato E, Funck-Brentano C, et al. 2019 ESC Guidelines for the diagnosis and management of chronic coronary syndromes. Eur Heart J. 2020;41(3):407-477.
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DOI: https://doi.org/10.1093/eurheartj/ehz425


2. Fihn SD, Gardin JM, Abrams J, Berra K, Blankenship JC, Dallas AP, et al. 2012 ACCF/AHA/ACP/AATS/PCNA/SCAI/STS guideline for the diagnosis and management of patients with stable ischemic heart disease. J Am Coll Cardiol. 2012;60(24):e44-e164.
PMID: 23182125
DOI: https://doi.org/10.1016/j.jacc.2012.07.013


3. Khan MA, Hashim MJ, Mustafa H, Baniyas MY, Al Suwaidi SKBM, AlKatheeri R, et al. Global epidemiology of ischemic heart disease: Results from the Global Burden of Disease Study. Cureus. 2020;12(7):e9349.
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4. Ibanez B, James S, Agewall S, Antunes MJ, Bucciarelli-Ducci C, Bueno H, et al. 2017 ESC Guidelines for the management of acute myocardial infarction in patients presenting with ST-segment elevation. Eur Heart J. 2018;39(2):119-177.
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5. Amsterdam EA, Wenger NK, Brindis RG, Casey DE Jr, Ganiats TG, Holmes DR Jr, et al. 2014 AHA/ACC guideline for the management of patients with non–ST-elevation acute coronary syndromes. J Am Coll Cardiol. 2014;64(24):e139-e228.
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  Background 

Valvular Heart Disease (VHD) refers to damage or dysfunction of one or more of the heart’s four valves—mitral, aortic, tricuspid, or pulmonary. It may involve stenosis (narrowing of the valve orifice), regurgitation (backflow of blood due to incomplete valve closure), or both. These abnormalities impair normal blood flow through the heart and can lead to heart failure, arrhythmias, thromboembolism, or sudden cardiac death. 

Classification

By Valve Affected: 

    • Aortic valve disease: Aortic stenosis (AS), Aortic regurgitation (AR) 
    • Mitral valve disease: Mitral stenosis (MS), Mitral regurgitation (MR), Mitral valve prolapse (MVP) 
    • Tricuspid valve disease: Tricuspid regurgitation (TR), Tricuspid stenosis (TS) 
    • Pulmonic valve disease: Pulmonic stenosis (PS), Pulmonic regurgitation (PR) 

By Onset: 

    • Acute valvular disease: Often due to infective endocarditis, acute MI (papillary muscle rupture), or trauma 
    • Chronic valvular disease: Often due to rheumatic fever, degenerative calcification, or congenital defects 

By Severity: 

    • Classified as mild, moderate, or severe based on echocardiographic criteria 

Epidemiology 

    • Sex: Aortic stenosis is more common in men; mitral valve prolapse more common in women 
    • Age: Prevalence increases with age due to degenerative changes 
    • Race/Region: Rheumatic valve disease more common in low- and middle-income countries 
    • Social Status: Low socioeconomic groups may have higher rheumatic disease incidence and lower access to valve interventions 

Pathophysiology

Valvular heart disease involves dysfunction of one or more of the heart’s valves—aortic, mitral, tricuspid, or pulmonary—leading to stenosis (narrowing) or regurgitation (incompetence/leakage).

  • Stenosis occurs when a valve becomes narrowed due to fibrosis, calcification, or congenital defects, leading to increased pressure load on the chamber proximal to the valve. For example, aortic stenosis causes left ventricular hypertrophy due to pressure overload.

  • Regurgitation (or insufficiency) happens when a valve fails to close completely, allowing backward flow of blood, resulting in volume overload. This causes chamber dilation (e.g., mitral regurgitation leads to left atrial enlargement and left ventricular dilation).

Over time, these abnormal hemodynamic stresses lead to:

  • Myocardial remodeling

  • Reduced cardiac output

  • Heart failure

  • Arrhythmias (e.g., atrial fibrillation in mitral valve disease)

 

  Etiology

I) Causes of Valvular Heart Disease:

    • Degenerative (Calcific): Common in elderly; affects aortic and mitral valves 
    • Rheumatic heart disease: Causes stenosis and regurgitation, especially mitral valve 
    • Congenital: Bicuspid aortic valve, Ebstein anomaly 
    • Infective endocarditis: Acute destruction or chronic scarring of valve leaflets 
    • Ischemic heart disease: Papillary muscle dysfunction or rupture causing regurgitation 
    • Myxomatous degeneration: In MVP 
    • Radiation-induced valvular fibrosis 

II) Risk Factors

    • Age > 65 years 
    • History of rheumatic fever 
    • Congenital heart disease 
    • Hypertension 
    • Chronic kidney disease 
    • Endocarditis 
    • Connective tissue disorders (e.g., Marfan syndrome) 

 

  Clinical Presentation

I) History (Symptoms)

    • Aortic stenosis: Exertional dyspnea, angina, syncope 
    • Aortic regurgitation: Wide pulse pressure, palpitations, fatigue 
    • Mitral stenosis: Dyspnea, orthopnea, PND, hemoptysis 
    • Mitral regurgitation: Fatigue, palpitations, dyspnea 
    • Right-sided valve disease: Abdominal bloating, peripheral edema, hepatic congestion 

II) Physical Exam (Signs)

Vital Signs: May show hypotension or wide pulse pressure 

Cardiac Exam 

    • AS: Systolic crescendo-decrescendo murmur at right upper sternal border, radiating to carotids 
    • AR: Diastolic decrescendo murmur at left sternal border 
    • MS: Diastolic murmur with opening snap at apex 
    • MR: Holosystolic murmur at apex radiating to axilla 
    • TR: Holosystolic murmur increasing with inspiration 
    • Elevated JVP (right-sided disease) 
    • Displaced apical impulse (LVH or dilatation)  

Pulmonary Exam: 

    • Pulmonary rales (left-sided heart failure)  

Abdomen:

    • Hepatomegaly, ascites (right-sided failure) 

Peripheral:  

    • Peripheral edema 

  Differential Diagnosis (DDx)

    • Heart failure due to non-valvular causes 
    • Hypertrophic cardiomyopathy 
    • Congenital heart diseases (e.g., ASD, VSD) 
    • Pulmonary hypertension 
    • Constrictive pericarditis 
    • Infective endocarditis (when presenting acutely) 
    • Myocardial ischemia/infarction 

 

  Diagnostic Tests

Initial Tests: 

I) Echocardiography (TTE/TEE):

Mainstay in diagnosing VHD

    • Assess valve morphology and function 
    • Measure gradients and regurgitant volume 
    • Determine ejection fraction 

II) Electrocardiogram (EKG):

May show signs of chamber enlargement or arrhythmias 

III) Chest X-ray: 

    • Cardiomegaly 
    • Pulmonary congestion (in left-sided lesions) 
    • Enlarged pulmonary arteries (in right-sided lesions) 

IV) BNP or NT-proBNP:

Elevated in heart failure

Additional Testing:

I) Cardiac MRI:

Detailed anatomy and function when echo inconclusive 

II) Cardiac catheterization:

For hemodynamics and coronary anatomy pre-surgery 

III) CT angiography:

To evaluate valve calcification and plan interventions 

 

  Treatment

I) Medical Management:

    • For Heart Failure Symptoms
    • Diuretics for volume overload 
    • ACE inhibitors, beta-blockers cautiously (not in severe AS without specialist input) 
    • Rate control: In MS with atrial fibrillation (beta-blockers, calcium channel blockers) 
    • Anticoagulation
    • Atrial fibrillation (especially with MS) 
    • Mechanical valve replacements (lifelong warfarin) 
    • Endocarditis prophylaxis: In select cases (e.g., prosthetic valves) 

II) Surgical/Interventional:

    • Aortic valve replacement (AVR)
    • Indicated for severe symptomatic AS or AR with reduced EF 
    • Mitral valve repair/replacement
    • Severe symptomatic MR or MS 
    • Tricuspid valve repair: In severe TR with other valve surgeries 
    • Transcatheter Aortic Valve Replacement (TAVR): For high-risk surgical patients with severe AS 
    • Percutaneous mitral balloon valvotomy: For rheumatic MS with favorable anatomy 

  Consults

    • Cardiology: All patients with moderate/severe valvular disease 
    • Cardiothoracic surgery: For evaluation of surgical candidates 
    • Interventional cardiology: For TAVR or percutaneous mitral interventions 
    • Infectious disease: In suspected or confirmed endocarditis 
    • Primary care: For comorbidity management (e.g., hypertension, diabetes) 

 

  Patient Education

    • Importance of medication adherence 
    • Endocarditis prevention education 
    • Recognizing signs of decompensation (dyspnea, edema, palpitations) 
    • Avoid high-intensity activity in severe stenosis until evaluated 
    • Low-sodium diet if heart failure present 
    • Annual influenza and pneumococcal vaccines 
    • COVID-19 vaccination per guidelines 

 

  Follow-Up

    • Regular echocardiograms to monitor disease progression (every 6–12 months in severe disease) 
    • Monitor for development of symptoms (even in asymptomatic severe cases) 
    • Evaluate for arrhythmias, especially atrial fibrillation 
    • Assess need for surgical or transcatheter intervention 
    • Reinforce lifestyle changes and follow-up for chronic comorbidities 
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