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Valvular heart diseases

Last updated: February 18, 2021

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
		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
Right heart	Pulmonary valve	Congenital Carcinoid syndrome	Pulmonary hypertension (e.g., tetralogy of Fallot, ventricular septal defects) Dilated cardiomyopathy

References:^[2]^[3]^[4]^[5]^[6]^[7]

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's 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 Steel 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 (extremely rare)	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 Aortic Stenosis (AS) - Heart Auscultation - Episode 1 Aortic Regurgitation (AR) - Heart Auscultation - Episode 2 Mitral Regurgitation (MR) - Heart Auscultation - Episode 4 Mitral Stenosis (MS) - Heart Auscultation - Episode 3 Tricuspid Regurgitation (TR) - Heart Auscultation - Episode 12

References:^[4]^[5]^[6]^[8]

Treatment

Symptomatic

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

Causal

Surgery: The choice of procedure is based on the patient's individual risk profile and an evaluation of benefits.
- Valve reconstruction (annuloplasty)
  - Procedure: ring-shaped device attached to the outside of the valve opening to re-establish shape and function of valve
  - Reduced thromboembolic risk compared to mechanical prosthetic valve; but high risk of recurring stenosis
  - Lower mortality rate than valve replacements, though replacements are more durable
- Prosthetic valve replacement
  - Immunosuppression not necessary

Prosthetic heart valve
	Mechanical prosthetic valve	Biological prosthetic valve
Pros	Valve has a long lifespan	Anticoagulation only necessary for 3 months post operation
Cons	Life-long anticoagulation necessary (warfarin, aspirin)	Short lifespan due to sclerotic degeneration May need to be replaced every ten years
Indications	Younger patients Previously anticoagulated patients (e.g., with pre-existing atrial fibrillation)	Older patients Patients with a high risk of bleeding Women with a desire to have children

Interventional valve replacement via catheter
- Transcatheter aortic valve replacement (TAVR)
- Transcatheter mitral valve replacement (TMVR)
Percutaneous balloon valvuloplasty for stenoses

Biological prosthetic heart valve Mechanical prosthetic valve

References:^[8]^[9]

References

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