Valvular heart diseases

Last updated: March 25, 2022

Summary

Valvular heart diseases can take the form of stenosis, insufficiency (regurgitation), or a combination of the two. These defects are typically acquired as the result of infections, underlying heart disease, or degenerative processes. However, certain congenital conditions can also cause valvular heart diseases. Acquired defects are found primarily in the left heart as a result of higher pressure and mechanical strain on the left ventricle. The type of valvular disease determines the type of cardiac stress and subsequent symptoms. Valvular stenosis leads to a greater pressure load and concentric hypertrophy, while insufficiencies are characterized by volume overload and eccentric hypertrophy of the preceding heart cavities. Diagnostic procedures typically include ECGs, chest x-ray, and echocardiograms. Management consists of interventional or surgical procedures to reconstruct or replace valves, as well as medical treatment of possible heart failure.

Epidemiology

Aortic stenosis
- Most common valve defect in industrialized countries
- Mostly degenerative
- Degenerative stenosis usually becomes symptomatic after the age of 75 and is most common in men.
- Aortic stenosis in young people is usually secondary to congenital defects (e.g., bicuspid aortic valve).
Aortic regurgitation
- Age of onset: 40–60 years
- Severity increases with age
Mitral stenosis: symptom onset between 20 and 39 years ^[1]
Mitral regurgitation
- Overall prevalence of 0.6 to 2.4 %
- Second most common valve defect
- More common in women
Tricuspid valve defects: occur in < 1% of the population
Pulmonary valve defects: rare outside of congenital conditions

Epidemiological data refers to the US, unless otherwise specified.

Etiology

Valvular heart defects may either be acquired or congenital. Acquired defects are more common and typically occur secondary to infections (postinflammatory), degenerative processes, or heart disease.

Etiology of valvular heart conditions ^[2]^[3]
		Valve stenosis	Valve regurgitation
Left heart	Mitral valve	Rheumatic fever Rheumatic diseases (e.g., SLE, RA)	Mitral valve prolapse Dilated cardiomyopathy Ischemic heart disease (e.g., following myocardial infarction) Degenerative calcification Rheumatic fever Infective endocarditis
Left heart	Aortic valve	Degenerative calcification (most common) Rheumatic endocarditis Congenital (e.g., unicuspid, bicuspid, or hypoplastic valve)	Acute: infective endocarditis, aortic dissection type A, chest trauma Chronic Bicuspid aortic valve Connective tissue diseases (e.g., Marfan syndrome, Ehlers-Danlos syndrome) Rheumatic fever Rheumatic diseases (e.g., Behcet disease, RA, SLE)
Right heart	Tricuspid valve	Rheumatic fever Infective endocarditis (mostly IV drug abuse)	Right ventricular dilation (e.g., in right-sided heart failure) Infective endocarditis (IV drug use) Rheumatic fever Connective tissue diseases (e.g., Marfan syndrome) Carcinoid syndrome Pulmonary hypertension (cor pulmonale; secondary to mitral stenosis) Left-sided heart failure
Right heart	Pulmonary valve	Congenital Carcinoid syndrome	Pulmonary hypertension (e.g., tetralogy of Fallot, ventricular septal defects) Dilated cardiomyopathy

Clinical features

All valvular defects can eventually lead to symptoms of heart failure as a result of excessive strain on the ventricles.

Physical examination

Complete heart examination: see cardiovascular examination and auscultation of the heart for details.

Auscultation in valvular defects
	Maximum point	Murmur	Characteristics
Aortic stenosis	Aortic valve (parasternal 2^nd right intercostal space) Erb point	Harsh crescendo-decrescendo systolic ejection murmur	Radiation to the carotids Soft S₂ Possibly ejection click
Aortic regurgitation	Aortic valve (parasternal 2^nd right ICS) Erb point	Diastolic murmur with a decrescendo Possible additional quiet systolic murmur	Immediately following the 2^ndheart sound (“immediate diastolic murmur”) Austin Flint Murmur
Mitral stenosis	Heart apex (midclavicular 5^th left ICS)	Delayed diastolic murmur with a decrescendo	“Tympanic” 1^stheart sound Mitral opening murmur/opening snap (OS)
Mitral valve prolapse	Heart apex (midclavicular 5^th left ICS)	Late-systolic crescendo	Midsystolic high-frequency click (due to the tensing of the chordae tendinae) Loudest before S₂
Mitral regurgitation	Heart apex (midclavicular 5^th left ICS) Left axilla	Holosystolic murmur 3^rdheart sound audible Quiet 1^stheart sound	Blowing Radiation into the axilla
Pulmonary stenosis	Pulmonary valve (parasternal 2^nd left ICS)	Crescendo-decrescendo ejection systolic murmur	Possible radiation into the back Possible early systolic pulmonary ejection click and/or widely split 2^ndheart sound
Pulmonary regurgitation	Pulmonary valve (parasternal 2^nd left ICS)	Diastolic murmur with a decrescendo	Graham Steell murmur: high-frequency decrescendo diastolic murmur
Tricuspid stenosis (extremely rare)	Tricuspid valve (parasternal 4^th left ICS)	Delayed diastolic murmur with a decrescendo Possible pre-systolic crescendo
Tricuspid regurgitation	Tricuspid valve (parasternal 4^th left ICS)	Holosystolic murmur	Augmentation of the murmur's intensity with inspiration (Carvallo sign)

Anatomical locations and auscultatory sites for heart valves Cardiovascular Examination - Clinical Examination of the Heart Phonocardiogram of systolic heart murmurs Phonocardiogram of diastolic murmurs Aortic Stenosis (AS) - Heart Auscultation - Episode 1 Aortic stenosis - Heart Murmur - Clinical examination | Δ AMBOSS Aortic Regurgitation (AR) - Heart Auscultation - Episode 2 Aortic regurgitation - Heart murmur - Clinical examination | Δ AMBOSS Osmosis: Aortic valve disease (bicuspid, tricuspid) - stenosis, regurgitation, symptoms Mitral Stenosis (MS) - Heart Auscultation - Episode 3 Mitral Regurgitation (MR) - Heart Auscultation - Episode 4 Mitral regurgitation - Heart murmur - Clinical examination | Δ AMBOSS Osmosis: Mitral valve disease (regurgitation, stenosis) - causes, symptoms, pathology Tricuspid Regurgitation (TR) - Heart Auscultation - Episode 12 Osmosis: Tricuspid Valve Disease- causes, symptoms, diagnosis, treatment, pathology Osmosis: Pulmonary valve disease - causes, symptoms, diagnosis, treatment, pathology

References:^[4]^[5]

Treatment

Symptomatic treatment

Treatment of heart failure
Endocarditis prophylaxis
Prevention of thromboembolism (if necessary)

Causal treatment

Surgical options

The choice of procedure is based on the patient's individual risk profile and an evaluation of the risks and benefits. Options include:

Valve reconstruction (annuloplasty)
- Procedure: a ring-shaped device is attached to the outside of the valve opening to reestablish the shape and function of the valve
- Reduced thromboembolic risk compared to a mechanical prosthetic valve but high risk of recurring stenosis
- Lower mortality rate than valve replacements; however, replacements are more durable
Prosthetic heart valve replacement

Overview of prosthetic heart valve replacement options
	Mechanical prosthetic valve	Biological prosthetic valve
Indications	Younger patients (< 65 years of age) Patients who already take anticoagulants (e.g., those with preexisting atrial fibrillation)	Older patients (≥ 65 years of age) Patients with a high risk of bleeding Women with a desire to have children
Advantages	Immunosuppression is not necessary.
Advantages	Valve has a long lifespan	Anticoagulation is only necessary for 3 months after surgery.
Disadvantages	Life-long anticoagulation is necessary (e.g., with warfarin, aspirin).	Short lifespan due to sclerotic degeneration (biological prosthetic valves may need to be replaced every 10 years)
Complications ^[6]	Thromboembolism Patient-prosthesis mismatch A phenomenon caused by the implantation of a prosthetic valve with a functional area that is too small for the cardiac demand of the patient Leads to left ventricle hypertrophy and impaired exercise capacity, and increases the risk of other cardiac events Associated with increased mortality Can be prevented by choosing a valve that is appropriate for the patient's body size Prosthetic valve endocarditis Prosthetic valve stenosis Valve dysfunction Hemolytic anemia

Mechanical prosthetic valve Biological prosthetic heart valve

Interventional procedures

Transcatheter aortic valve replacement (TAVR)
- A minimally invasive, percutaneous procedure in which the aortic valve is replaced through an endovascular technique
- A collapsible replacement valve is inserted via a catheter and placed over the native valve
- Once the replacement valve is expanded, it pushes the old valve aside and assumes its function.
Transcatheter mitral valve replacement (TMVR)
Percutaneous balloon valvuloplasty
- Used to treat cardiac valvular stenosis
- A balloon is advanced into the target valve (either percutaneously or transapically) and inflated, opening the stenotic valve

References:^[5]^[7]

References

Iung B, Vahanian A. Epidemiology of Acquired Valvular Heart Disease. Can J Cardiol. 2014; 30 (9): p.962-970. doi: 10.1016/j.cjca.2014.03.022 . | Open in Read by QxMD
Lincoln J, Garg V. Etiology of Valvular Heart Disease. Circulation Journal. 2014; 78 (8): p.1801-1807. doi: 10.1253/circj.cj-14-0510 . | Open in Read by QxMD
Waller BF. Etiology of pure tricuspid regurgitation.. Cardiovasc Clin. 1987; 17 (2): p.53-95.
Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease. J Am Coll Cardiol. 2017; 70 (2): p.252-289. doi: 10.1016/j.jacc.2017.03.011 . | Open in Read by QxMD
Vesey JM, Otto CM. Complications of prosthetic heart valves. Curr Cardiol Rep. 2004; 6 (2): p.106-111. doi: 10.1007/s11886-004-0007-x . | Open in Read by QxMD
Acker MA, Parides MK, Perrault LP, et al. Mitral-valve repair versus replacement for severe ischemic mitral regurgitation. N Engl J Med. 2014; 370 (1): p.23-32. doi: 10.1056/NEJMoa1312808 . | Open in Read by QxMD

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