Valvular Heart Diseases
Valvular heart diseases occur when one or more of the heart's four valves malfunction. These valves regulate blood flow through the heart. When they become diseased, they may not open fully (stenosis) or close tightly (regurgitation), leading to various heart problems.
Types of Valvular Heart Diseases
1. Aortic Stenosis
➭ Epidemiology: Affects 25% of all valvular heart diseases.
➭ Pathophysiology: Calcification of the aortic valve leaflets narrows the opening, restricting blood flow.
➭ Clinical Presentation: Dyspnea, angina, syncope, heart failure.
➭ Diagnosis: Echocardiogram, EKG, cardiac catheterization.
➭ Treatment: Aortic valve replacement (AVR) or transcatheter aortic valve replacement (TAVR).
2. Aortic Regurgitation
➭ Epidemiology: 25% of valvular heart diseases.
➭ Pathophysiology: Incomplete closure of the aortic valve allows blood to leak back into the left ventricle.
➭ Clinical Presentation: Asymptomatic initially, later dyspnea, orthopnea, fatigue, heart failure.
➭ Diagnosis: Echocardiogram, EKG, chest X-ray.
➭ Treatment: Medical management with diuretics and vasodilators, surgical aortic valve replacement.
3. Mitral Stenosis
➭ Epidemiology: Primarily affects young women.
➭ Pathophysiology: Rheumatic heart disease leads to thickening and calcification of the mitral valve leaflets, narrowing the opening.
➭ Clinical Presentation: Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, hemoptysis, right heart failure.
➭ Diagnosis: Echocardiogram.
➭ Treatment: Percutaneous balloon valvuloplasty or mitral valve replacement.
4. Mitral Regurgitation
➭ Epidemiology: Can occur at any age.
➭ Pathophysiology: Incomplete closure of the mitral valve allows blood to leak back into the left atrium.
➭ Clinical Presentation: Asymptomatic initially, later fatigue, dyspnea, orthopnea, atrial fibrillation, heart failure.
➭ Diagnosis: Echocardiogram.
➭ Treatment: Medical management with diuretics and vasodilators, surgical mitral valve repair or replacement.
5. Pulmonary Stenosis
➭ Epidemiology: Diagnosed in young individuals.
➭ Pathophysiology: Congenital heart diseases like tetralogy of Fallot and Noonan syndrome cause narrowing of the pulmonary valve.
➭ Clinical Presentation: Asymptomatic initially, later exertional dyspnea, angina, syncope, right heart failure.
➭ Diagnosis: Echocardiogram, EKG.
➭ Treatment: Balloon valvuloplasty.
6. Pulmonary Regurgitation
➭ Epidemiology: Occurs after surgical management of congenital heart diseases.
➭ Pathophysiology: Incomplete closure of the pulmonary valve allows blood to leak back into the right ventricle.
➭ Clinical Presentation: Asymptomatic initially, later exertional dyspnea, fatigue, edema, congestion.
➭ Diagnosis: Echocardiogram, EKG, cardiac MRI.
➭ Treatment: Medical management with diuretics and ACE inhibitors, surgical valve replacement.
7. Tricuspid Stenosis
➭ Epidemiology: More common in men, often associated with mitral stenosis.
➭ Pathophysiology: Rheumatic heart disease leads to thickening and calcification of the tricuspid valve leaflets.
➭ Clinical Presentation: Mid-diastolic murmur at the left lower sternal border, right atrial enlargement, systemic venous congestion.
➭ Diagnosis: Echocardiogram, EKG.
➭ Treatment: Loop diuretics, surgical valve repair or replacement.
8. Tricuspid Regurgitation
➭ Epidemiology: Associated with mitral regurgitation.
➭ Pathophysiology: Incomplete closure of the tricuspid valve allows blood to leak back into the right atrium.
➭ Clinical Presentation: Fatigue, dyspnea, right heart failure.
➭ Diagnosis: Echocardiogram, EKG, cardiac MRI.
➭ Treatment: Heart failure medications, surgical valve repair or replacement.
Infective Endocarditis
Infective endocarditis is a serious infection of the heart's inner lining, valves, and blood vessels. It occurs when bacteria or other microorganisms enter the bloodstream and attach to damaged heart tissue.
Risk Factors: Age > 60 years, illicit IV drug use, poor dentition, comorbidities.
Clinical Presentation: Fever, anorexia, weight loss, fatigue, Janeway lesions, Roth spots, petechiae, Osler's nodes, splinter hemorrhages, septic emboli.
Diagnosis: Blood cultures, echocardiogram.
Treatment: Antibiotics, surgical valve replacement.
Aortic Valve Diseases
Aortic Stenosis
Aortic stenosis is a condition where the aortic valve narrows, restricting blood flow from the heart to the body. This narrowing can lead to various complications, including heart failure.
Epidemiology
➭ Affects 25% of all valvular heart diseases.
➭ Most common cause in individuals over 70 is degenerative heart disease.
➭ Most common cause in individuals under 70 is bicuspid aortic valve.
Pathophysiology
➭ Calcification of the aortic valve leaflets leads to obstruction of blood flow.
➭ The heart compensates by increasing afterload, causing left ventricular hypertrophy (LVH) to maintain ejection fraction (EF) and systolic function.
➭ This increased pressure affects the left atrium, pulmonary artery, and right ventricle.
Clinical Manifestations
➭ Asymptomatic until the valve area narrows to less than 1 cm².
➭ Progression leads to:
➭ Dyspnea (most common)
➭ Angina (5 years after dyspnea)
➭ Syncope (3 years after angina)
➭ Heart failure (2 years after syncope)
On Exam
➭ Harsh systolic ejection crescendo-decrescendo murmur at the right upper sternal border (RUSB) radiating to the carotids.
➭ Pulsus parvus et tardus (small, delayed carotid pulse)
➭ Widened pulse pressure
Diagnosis
➭ Transthoracic echocardiogram (TTE) is usually sufficient.
➭ EKG shows left ventricular hypertrophy (LVH)
➭ Cardiac catheterization is done before surgery to assess the severity of the stenosis.
Treatment
➭ Medical Management: None, as it does not alter the course of the disease.
➭ Surgical Management:
➭ Aortic Valve Replacement (AVR) is recommended if the patient is symptomatic, LVEF is ≤50%, and valve area is ≤1 cm².
➭ Mechanical valves require lifelong anticoagulation.
➭ Bioprosthetic valves are less durable but minimally thrombotic.
➭ Transcatheter aortic valve replacement (TAVR) is an option for high-risk patients.
Prognosis
➭ 10-year survival rate after AVR is 60%.
➭ If left untreated, the prognosis is poor, with severe aortic stenosis leading to a high risk of perioperative mortality (15-20%).
Aortic Regurgitation
Aortic regurgitation (AR) is a condition where the aortic valve does not close properly, allowing blood to leak back into the left ventricle during diastole.
Epidemiology
➭ 25% of valvular heart diseases.
➭ Chronic AR is often associated with valvular disease or enlargement of the aortic root.
Pathophysiology
➭ Incomplete closure of the aortic valve during diastole leads to regurgitation of blood into the left ventricle.
➭ This increases preload, causing left ventricular hypertrophy and dilation to maintain cardiac output.
➭ Over time, this can lead to left ventricular failure.
Clinical Manifestations
➭ Chronic AR:
➭ Asymptomatic for a long time.
➭ Signs of heart failure may develop later: dyspnea, orthopnea, diaphoresis, fatigue
➭ Acute AR:
➭ Sudden onset of pulmonary edema, cardiogenic shock.
On Exam
➭ Chronic AR:
➭ Diastolic decrescendo blowing murmur at the left upper sternal border (LUSB)
➭ Mid-late diastolic rumble at the apex (Austin Flint murmur)
➭ Widened pulse pressure
➭ Water-hammer pulse (Corrigan's pulse)
➭ Rapid diastolic fluttering of the anterior mitral leaflet (characteristic sign on echocardiogram)
➭ Acute AR:
➭ Low-pitched early diastolic murmur after S2
Diagnosis
➭ Echocardiogram is the diagnostic test of choice.
➭ EKG may show left axis deviation and LVH.
➭ Chest X-ray may show cardiomegaly.
Treatment
➭ Chronic AR:
➭ Medical management with diuretics and vasodilators.
➭ Surgical aortic valve replacement is recommended if EF is <65%.
➭ Acute AR:
➭ Immediate medical management with IV diuretics, vasodilators, and inotropes.
➭ Urgent surgical aortic valve replacement is necessary.
Prognosis
➭ The prognosis depends on the severity of AR and the presence of associated conditions.
➭ Early surgical intervention improves long-term survival.
Mitral Valve Diseases
The mitral valve, located between the left atrium and left ventricle of the heart, plays a crucial role in directing blood flow. When this valve malfunctions, it can lead to significant heart problems. Let's explore the two main types of mitral valve diseases: mitral stenosis and mitral regurgitation.
Mitral Stenosis
Mitral stenosis is a condition where the mitral valve narrows, restricting blood flow from the left atrium to the left ventricle. This narrowing can lead to various complications, including heart failure.
Epidemiology:
➭ Primarily affects young women (onset between 30-40 years)
➭ Incidence has decreased due to improved rheumatic fever management
Etiology:
➭ Most commonly caused by rheumatic heart disease, leading to thickening and calcification of the valve leaflets.
Pathophysiology:
➭ The narrowed mitral valve obstructs blood flow, causing increased pressure in the left atrium.
➭ This leads to left atrial enlargement, pulmonary hypertension, and right ventricular hypertrophy.
➭ Over time, this can lead to right-sided heart failure.
Clinical Presentation:
➭ Pulmonary Symptoms: Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and hemoptysis.
➭ Cardiac Symptoms: Right heart failure (jugular venous distension, edema, hepatomegaly) and atrial fibrillation.
➭ Physical Exam: Prominent S1, opening snap, and a diastolic rumble at the apex (best heard in the left lateral decubitus position).
Diagnosis:
➭ Echocardiogram: Reveals narrowed mitral valve with thickened leaflets and reduced motion.
Treatment:
➭ Medical Management: Diuretics, beta-blockers, and calcium channel blockers can help manage symptoms and control heart rate.
➭ Surgical Management: Percutaneous balloon valvuloplasty is recommended for symptomatic patients or those with a mitral valve area ≤1 cm².
Mitral Regurgitation
Mitral regurgitation occurs when the mitral valve does not close properly, allowing blood to leak back into the left atrium during systole.
Epidemiology:
➭ Can occur at any age but is most common in adults aged 50-70 years.
➭ Can be primary (leaflets/chordae) or secondary (mitral valve prolapse, rheumatic heart disease).
Pathophysiology:
➭ Retrograde flow from the left ventricle to the left atrium increases left atrial and pulmonary pressures.
➭ Left ventricular volume and pressure also increase, leading to left ventricular dilation and hypertrophy.
➭ Over time, this can lead to left ventricular failure.
Clinical Presentation:
➭ Asymptomatic until late stages.
➭ Chronic MR: Fatigue, dyspnea, orthopnea, and atrial fibrillation.
➭ Acute MR: Sudden onset of pulmonary edema, cardiogenic shock.
Physical Exam:
➭ Chronic MR: Blowing holosystolic murmur at the apex, radiating to the axilla. Wide splitting of S2, S3 gallop.
➭ Acute MR: Low-pitched early diastolic murmur, narrow pulse pressure, jugular venous distension, pulmonary congestion.
Diagnosis:
➭ Echocardiogram: Reveals regurgitant jet and left atrial size.
➭ Severe MR: Ventricular dilation and reduced ejection fraction.
Treatment:
➭ Medical Management: Diuretics and vasodilators can help manage symptoms but do not alter the course of the disease.
➭ Surgical Management: Mitral valve repair or replacement is recommended if the patient is symptomatic, has left ventricular dysfunction, or has atrial fibrillation.
Mitral Valve Prolapse
Mitral valve prolapse (MVP) is a condition where one or both of the mitral valve leaflets bulge upward into the left atrium during systole.
Epidemiology:
➭ Affects young women (15-35 years)
➭ Associated with connective tissue disorders (Marfan syndrome, Ehlers-Danlos syndrome)
Pathophysiology:
➭ Myxomatous degeneration of the mitral valve leaflets leads to abnormal tissue structure and function.
Clinical Presentation:
➭ Most patients are asymptomatic.
➭ Autonomic dysfunction: Anxiety, palpitations, syncope.
➭ Severe MR: Fatigue, dyspnea, heart failure.
➭ Physical Exam: Mid-late systolic click at the apex, narrow pulse pressure, low body weight.
Diagnosis:
➭ Echocardiogram: Reveals posterior leaflet bulge and regurgitation, if present.
Management:
➭ Reassurance and beta-blockers if autonomic dysfunction is present.
Pulmonary Valve Diseases
The pulmonary valve, located between the right ventricle and the pulmonary artery, plays a crucial role in directing blood flow to the lungs. When this valve malfunctions, it can lead to significant heart problems. Let's explore the two main types of pulmonary valve diseases: pulmonary stenosis and pulmonary regurgitation.
Pulmonary Stenosis
Pulmonary stenosis is a condition where the pulmonary valve narrows, restricting blood flow from the right ventricle to the pulmonary artery. This narrowing can lead to various complications, including right ventricular hypertrophy and right heart failure.
Epidemiology:
➭ Diagnosed in young individuals.
➭ Primarily a congenital condition.
Etiology:
➭ Congenital heart diseases like tetralogy of Fallot and Noonan syndrome.
Pathophysiology:
➭ The stenotic pulmonary valve creates a pressure gradient between the right ventricle and the pulmonary artery.
➭ This increases afterload on the right ventricle, leading to right ventricular hypertrophy and dysfunction.
➭ Over time, this can lead to right heart failure.
Clinical Presentation:
➭ Asymptomatic in mild cases.
➭ Severe stenosis: Exertional dyspnea, angina, syncope.
➭ Physical Exam: Harsh systolic ejection crescendo-decrescendo murmur at the left upper sternal border (LUSB), radiating to the neck. Increased intensity with inspiration.
➭ Signs of right heart failure: Edema, jugular venous distension, hepatomegaly.
Diagnosis:
➭ Echocardiogram: Reveals right ventricular hypertrophy, thickened leaflets with restricted systolic excursion.
➭ EKG: Right axis deviation, right ventricular hypertrophy, right atrial enlargement.
Treatment:
➭ Medical Management: Diuretics for right heart failure symptoms.
➭ Surgical Management: Balloon valvuloplasty is the treatment of choice for asymptomatic patients with evidence of severe stenosis or symptomatic patients.
Pulmonary Regurgitation
Pulmonary regurgitation occurs when the pulmonary valve does not close properly, allowing blood to leak back into the right ventricle during diastole.
Epidemiology:
➭ Occurs after surgical management of congenital heart diseases like tetralogy of Fallot or after percutaneous pulmonary balloon valvotomy.
Pathophysiology:
➭ Retrograde blood flow from the pulmonary artery to the right ventricle increases right ventricular pressure and afterload.
➭ This leads to right ventricular dilation and hypertrophy.
➭ Over time, this can lead to right heart failure.
Clinical Presentation:
➭ Asymptomatic or signs of right heart failure: Exertional dyspnea, fatigue, edema, congestion.
➭ Physical Exam: Graham Steell murmur, a brief decrescendo early diastolic murmur at the LUSB, heard best with full inspiration.
Diagnosis:
➭ Echocardiogram: Reveals regurgitant jet and right ventricular dilation.
➭ EKG: Right ventricular hypertrophy, right atrial enlargement.
➭ Cardiac MRI: To assess right ventricular function.
Treatment:
➭ Medical Management: Diuretics, ACE inhibitors.
➭ Surgical Management: Valve replacement is indicated for symptomatic patients, severe regurgitation, or severe right ventricular dilation.
Tricuspid Valve Diseases
The tricuspid valve, located between the right atrium and right ventricle, plays a crucial role in directing blood flow within the heart. When this valve malfunctions, it can lead to significant heart problems. Let's explore the two main types of tricuspid valve diseases: tricuspid stenosis and tricuspid regurgitation.
Tricuspid Stenosis
Tricuspid stenosis is a condition where the tricuspid valve narrows, restricting blood flow from the right atrium to the right ventricle. This narrowing can lead to various complications, including right atrial enlargement and right heart failure.
Epidemiology:
➭ More common in men.
➭ Does not occur as an isolated lesion - most commonly associated with mitral stenosis.
Etiology:
➭ Primarily rheumatic heart disease.
Pathophysiology:
➭ The stenotic tricuspid valve obstructs blood flow from the right atrium to the right ventricle.
➭ This increases right atrial pressure, leading to right atrial enlargement and systemic venous congestion.
Clinical Presentation:
➭ Initial symptoms: May be due to progression of associated mitral stenosis.
➭ On exam: Mid-diastolic murmur at the left lower sternal border (LLSB) 4th ICS.
➭ Opening snap: Usually later than that of mitral stenosis.
Diagnosis:
➭ EKG: Right atrial enlargement (tall, peaked P waves in lead II).
➭ Echo: Thickened TV leaflets, turbulent flow, right atrial dilation.
Treatment:
➭ Medical Management: Loop diuretics to manage fluid overload.
➭ Surgical Management: Valve repair or replacement for symptomatic and severe tricuspid stenosis.
Tricuspid Regurgitation
Tricuspid regurgitation occurs when the tricuspid valve does not close properly, allowing blood to leak back into the right atrium during systole.
Etiology:
➭ Primary: Annular dilation in the setting of right ventricular remodeling.
➭ Secondary (functional): Associated with mitral regurgitation.
Pathophysiology:
➭ Incompetent tricuspid valve leads to retrograde blood flow from the right ventricle to the right atrium.
➭ This increases right atrial pressure, leading to right atrial enlargement and right heart failure.
Clinical Presentation:
➭ Early: Fatigue and dyspnea (exertional).
➭ Late: Cachexia, cyanotic. Symptoms of right heart failure.
➭ On exam: Holosystolic, blowing, high-pitched murmur at the left lower sternal border.
➭ Murmur increases with inspiration (Carvallo's sign), distinguishing it from mitral regurgitation.
Diagnosis:
➭ EKG: Right atrial enlargement, atrial fibrillation.
➭ Echo: Right ventricular dilation, tricuspid regurgitation.
➭ Cardiac MRI: To assess right ventricular function.
Treatment:
➭ Medical Management: Heart failure medications (beta blockers, ACE inhibitors, diuretics).
➭ Surgical Management: Valve repair is preferred over replacement.
➭ Severe regurgitation: Undergoing left-sided valve surgery.
Infective Endocarditis
Infective endocarditis (IE) is a serious infection of the heart's inner lining, valves, and blood vessels. It occurs when bacteria or other microorganisms enter the bloodstream and attach to damaged heart tissue, forming a vegetation. This vegetation can then break off and travel to other parts of the body, causing serious complications.
Risk Factors
➭ Age > 60 years
➭ Illicit IV drug use
➭ Poor dentition
➭ Female
➭ Comorbidities - increased risk of infection
Pathophysiology
➭ Bacterial infection of the endothelium and valves.
➭ Mitral valve most commonly involved.
➭ Tricuspid valve if history of IV drug use.
Types of IE
➭ Acute: Virulent organisms (Staph aureus, Streptococci, Enterococci)
➭ Subacute: Less virulent organisms (Strep viridans, from oral flora, indolent)
➭ IV Drug Use: Staph epidermidis
➭ Prosthetic Valve: Staph epidermidis (if within 60 days)
Clinical Presentation
➭ Fever, anorexia, weight loss, fatigue
➭ Janeway lesions (rashes on palms), Roth spots (retinal hemorrhages), petechiae, Osler's nodes (tender nodules on fingertips), splinter hemorrhages, septic emboli
Diagnosis
➭ TTE > TEE
➭ CBC - leukocytosis, anemia, ESR elevated, rheumatoid factor
➭ Blood cultures - 3 sets 1 hr apart from different sites
➭ Modified Duke Criteria: 2 major OR 1 major + 3 minor OR 5 minor
Treatment
➭ Empiric after 2 blood cultures - vancomycin
➭ Duration: 6 weeks post-first negative culture
➭ MSSA -> Nafcillin or oxacillin (penicillin)
➭ MRSA -> IV vancomycin x 6 weeks
➭ HACEK -> Ceftriaxone + ampicillin OR ciprofloxacin x 6 weeks
➭ Prosthetic valve: If hemodynamically unstable, vancomycin, gentamicin AND cefepime OR antipseudomonal agent. After culture, add rifampin. Stop gentamicin after two weeks
➭ Surgical - valve replacement (HF, persistent, invasive, prosthetic, fungal, emboli)
