Valvular heart disease

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Valvular heart disease Microchapters
Patient Information
Classification Aortic stenosis Aortic regurgitation Mitral stenosis Mitral valve prolapse Mitral regurgitation Tricuspid stenosis Tricuspid regurgitation Pulmonary stenosis Pulmonary regurgitation
Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Tarek Nafee, M.D. [2]
Synonyms and keywords: VHD, Valve disease, Heart valve disease, Valvular dysfunction

Overview

Valvular heart disease (VHD) is the pathological defect affecting one of the four valves of the heart: aortic valve, mitral valve, pulmonic valve, or tricuspid valve. VHD may be congenital or acquired. Congenital causes of VHD include tetralogy of Fallot, Ebstein's anomaly, Noonan syndrome, congenital rubella syndrome, and bicuspid valve among others. Acquired causes of VHD include rheumatic heart disease, infective endocarditis, senile calcification of valves, or valve deformities secondary to structural changes of the myocardium (e.g. dilated cardiomyopathy). Regardless of the underlying cause, VHD may result in valve stenosis, valve regurgitation or , in some cases, valve prolapse. Valvular heart disease can often be asymptomatic and may go undiagnosed. In patients who develop symptoms suggestive of valvular heart disease, cardiac auscultation for heart murmurs is often the first step of a focused physical examination to rule out valvular heart disease. Echocardiography is the gold standard diagnostic modality for valvular heart disease. In addition to a thorough and focused physical examination, a well-performed echocardiogram aids the clinician in determining the severity of the disease, the prognosis, and the need for surgical intervention.

Classification

Valvular heart diseases are typically classified according to the valve that is affected as well as the nature of the pathological defect as follows:

• Aortic Valve
  • Aortic stenosis
  • Aortic regurgitation
• Mitral Valve
  • Mitral stenosis
  • Mitral regurgitation
  • Mitral valve prolapse
• Tricuspid valve
  • Tricuspid stenosis
  • Tricuspid regurgitation
• Pulmonary valve
  • Pulmonic stenosis
  • Pulmonic regurgitation

Differential Diagnosis

Clinicians may differentiate among different valvular heart diseases on the basis of the characteristics of the murmur and collecting a thorough patient history, as shown in the following table:

Valvular Disease	Common causes	Murmur Description						Interventions that Change Murmur Intensity
		Pitch	Timing	Best Heard Location	Shape	Other Features	Radiation	Valsalva Maneuver	Abrupt Standing	Hand Grip	Abrupt Squatting	Inhalation
Aortic stenosis	Senile calcification Calcification of bicuspid valve Other causes	High	Systolic	Right second intercostal spaces	Crescendo-Decrescendo	Systolic Ejection Click with bicuspid valves	Radiates towards the neck (Gallavardin dissociation)	↓	↓	-	↑	-
Subaortic stenosis	Hypertrophic obstructive cardiomyopathy	High	Systolic	Right second intercostal spaces	Crescendo-Decrescendo	-	No radiation	↑	↑	-	↓	-
Aortic regurgitation	Calcification of bicuspid valve Senile calcification Rheumatic heart disease Iatrogenic (after valvuloplasty) Other causes	High	Diastolic	Right third intercostal spaces (Erb's point)	Decrescendo	May present an early diastolic rumble at the apex (Austin-Flint murmur)	-	-	-	↑	-	-
Mitral stenosis	Infective endocarditis Rheumatic heart disease Mitral annular calcification Other causes	Low (rumbling)	Diastolic	Left ventricular apex	Decrescendo-Crescendo	Opening snap	No radiation	-	-	-	-	-
Mitral regurgitation	Infective endocarditis Ischemic heart disease Papillary muscle rupture Dilated cardiomyopathy Other causes	High	Systolic	Left ventricular apex	Holosystolic	Blowing sound	Usually radiates to the axilla	↓	-	↑	-	-
Mitral valve prolapse	Marfan's syndrome Myxomatous degeneration of the valve Papillary muscle injury Other causes	High	Systolic	Complete precordial area	Late systolic	Mid-systolic click	No radiation or may radiate to the axilla	↑	↑	-	↓	↓
Tricuspid stenosis	Rheumatic heart disease Carcinoid heart disease Cardiac tumors Other causes	Low	Diastolic	Left fourth or fifth intercostal spaces	-	Opening snap	No radiation	-	-	-	↑	↑
Tricuspid regurgitation	Pulmonary hypertension Right ventricular failure Iatrogenic Carcinoid heart disease Other causes	Low	Systolic	Left lower sternal border	Holosystolic	-	No radiation	-	-	-	-	↑ (Carvallo's sign)
Pulmonary stenosis	Congenital Rubella Syndrome Tetralogy of Fallot Carcinoid heart disease Other causes	High	Systolic	Left second intercostal spaces	Crescendo-Decrescendo	Wide split S2	Slight radiation to the neck	-	-	-	↑	↑
Pulmonary regurgitation	Carcinoid heart disease Pulmonary hypertension Rheumatic heart disease Iatrogenic (Tetralogy of Fallot repair) Other causes	High	Diastolic	Left second and third intercostal spaces	Decrescendo	Blowing sound	No radiation	-	-	-	-	↑ (Carvallo's sign)

2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR Guideline for the Evaluation and Diagnosis of Chest Pain: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines

Recommendations for Acute Chest Pain With Valvular Heart Disease

Class I

1.In patients presenting with acute chest pain with suspected or known history of VHD, TTE is useful in determining the presence, severity, and cause of VHD. (Level of Evidence: C-EO)

2.In patients presenting with acute chest pain with suspected or known VHD in whom TTE diagnostic quality is inadequate, TEE (with 3D imaging if available) is useful in determining the severity and cause of VHD. (Level of Evidence: C-EO)

Class IIa

1.n patients presenting with acute chest pain with known or suspected VHD, CMR imaging is reasonable as an alternative to TTE and/or TEE is nondiagnostic. (Level of Evidence: C-EO)