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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Respiratory or pulmonary vascular disease

  • Elevated jugular venous pressure, hepatomegaly, peripheral edema

  • Normal or near-normal left ventricular (LV) function and filling pressures

GENERAL CONSIDERATIONS

  • Alteration in the structure and function of the right ventricle (RV) caused by pulmonary hypertension (PH)

  • Pathophysiology: pulmonary vasoconstriction due to alveolar hypoxia or blood acidemia; reduced cross-sectional area of the pulmonary vascular bed

  • Causes:

    • – Lung disorders (eg, emphysema, pulmonary fibrosis)

    • – Acute or chronic pulmonary embolism

    • – Increased blood viscosity (eg, polycythemia vera, sickle cell disease, macroglobulinemia)

    • – Pulmonary arterial hypertension

  • Acute right heart failure may accompany acute respiratory failure or pulmonary embolism

  • RV hypertrophy predominates in chronic pulmonary hypertensive states

  • RV dilatation and dysfunction predominantly occur with acute cor pulmonale

  • Acute cor pulmonale is usually due to acute pulmonary thromboembolism

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Symptoms may be vague and subtle, especially in the early stages

  • Fatigue, exercise intolerance

  • Dyspnea

  • Syncope

  • Chest discomfort (usually exertional)

  • Palpitations

  • Dizziness

  • Cough

  • Hemoptysis

PHYSICAL EXAM FINDINGS

  • Tachypnea

  • Tachycardia

  • Systemic hypotension

  • Elevated jugular venous pressure

  • Labored respiratory efforts with retractions of the chest wall

  • Hyperresonance to lung percussion

  • Diminished breath sounds

  • Pulmonary wheezes

  • Left parasternal lift

  • Distant heart sounds

  • Accentuated pulmonic component of the S2

  • Split second heart sound that increases with inspiration

  • Holosystolic murmur at the lower sternal border (tricuspid regurgitation)

  • Diastolic murmur along the left sternal border due to pulmonary regurgitation may be present

  • Right-sided S3 and S4

  • Hepatomegaly; pulsatile liver (with severe tricuspid regurgitation)

  • Abdominal fluid wave from ascites

  • Peripheral edema

  • Cyanosis

DIFFERENTIAL DIAGNOSIS

  • Right heart failure due to left heart failure

  • RV myocardial infarction

  • Constrictive pericarditis

  • Other cardiac causes of right heart failure, such as pulmonic stenosis or atrial septal defect

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • CBC (polycythemia may be present)

  • Serum electrolytes (including calcium, magnesium),

  • Blood urea nitrogen and creatinine

  • Serum uric acid level (may be elevated from diuretics)

  • Liver function tests (elevated with hepatic congestion)

  • Coagulation studies

  • Antinuclear antibody and rheumatoid factor, hypercoagulability laboratories, HIV test, thyroid function test, serum alpha1-antitrypsin (if deficiency is suspected) to exclude causes of PH

ELECTROCARDIOGRAPHY

  • Sinus tachycardia

  • Atrial fibrillation or flutter

  • Premature atrial contractions

  • Multifocal atrial tachycardia

  • Junctional tachycardia

  • Low-voltage QRS in patients with chronic obstructive pulmonary disease (COPD)

  • Right atrial enlargement (P pulmonale)

  • Right bundle branch block

  • RV hypertrophy: R/S amplitude ratio > 1 mV in lead V1 and right-axis deviation (QRS axis of +110°)

IMAGING STUDIES

  • Chest x-ray findings vary with underlying disorder

  • Transthoracic echocardiography: RV hypertrophy may be present

    • – RV dilatation with reduced systolic function

    • – Septal deviation toward the LV during systole

    • – Color-flow Doppler ...

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