Cor Pulmonale

Define cor pulmonale

*Altered structure (eg, hypertrophy or dilatation) and/or impaired function of the right ventricle that results from pulmonary hypertension* *Right-sided heart disease due to left-sided heart disease or congenital heart disease is NOT considered cor pulmonale*

How do you diagnose cor pulmonale

*Chest radiograph*- - this is not definitive, this just helps rule out other ddx - Frontal view-enlargement of the central pulmonary arteries due to pulmonary hypertension - Lateral view- loss of retrosternal air space due to right ventricular enlargement *Pulmonary function tests* - *impaired diffusion capacity* *Right heart catheterization* - *the gold standard for the diagnosis of for pulmonale* - an elevated central venous pressure and right ventricular end-diastolic pressure - *a mean pulmonary artery pressure ≥25 mmHg at rest* - *no left heart disease* *EKG* *Doppler echocardiography* *MRI*

EKG findings

*EKG* - RVBB, right axis deviation, or signs of both right ventricular hypertrophy and right atrial enlargement - right ventricular hypertrophy includes a dominant R wave in V1 and V2 with prominent S waves in V5 and V6, or small R waves and deep S waves across the precordium

Besides COPD, other common etiologies

*Interstitial lung diseases (ILD)* - Up to 40 percent of patients with IPF have echocardiographic evidence of pulmonary hypertension and cor pulmonale *Any pulmonary vascular disorder associated with pulmonary hypertension* *Sleep-related breathing disorders* (eg, obstructive sleep apnea) are associated with pulmonary hypertension and cor pulmonale in about 20 percent of patients

Treatment strategies in cor pulmonale

*Reduce Rt. Ventricular Afterload* - supplemental oxygen to patients with hypoxemia will mitigate any hypoxic vasoconstriction - surgical thromboendarterectomy (Chronic Thromboembolic Disease) - continuous positive airway pressure therapy (Sleep Apnea) *Reduce Rt. Ventricular Pressure* - Diuretics - be sure not to over diurese *Reduce Rt. Ventricular Contractility* - Digoxin (in case of Lt. sided failure) - Dobutamin and milrinone - increase Rt Ventricular Contractility and reduce afterlaod

Doppler echocardiography

*should show dilated RV* useful for detecting *structural changes* and estimating function As the disease progresses, *right ventricular hypertrophy with paradoxical bulging of the septum* into the left ventricle during systole *Right ventricular dilatation eventually occurs* with abnormal flattening of the interventricular septum 1. Right ventricular dilatation 2. Tricuspid regurgitation 3. Right atrial enlargement and prevent the inferior vena cava from collapsing with inspiration

Magnetic Resonance Imaging

*superior to echocardiography for assessment of right ventricular size and function* (ie, contractility, ejection fraction, wall motion abnormalities), as well as assessment of myocardial mass and viability - can visualize things like sarcoid, amyloid - merely an extra diagnostic step since right heart catheterization is still necessary

Clinical Manifestations

- *Dyspnea on exertion* - Fatigue - Lethargy - Exertional syncope - Exertional angina This is caught most early on in active people that notice increase DOE when exercising

Pulmonary HTN groups associated with cor pulmonale

Associated with any of the etiologies that cause Group 1 PAH (other than left-to-right shunt), Group 3, 4 or 5 PH Chronic obstructive pulmonary disease *(COPD)- most common cause* of cor pulmonale in North America - OSA is a very common etiology

The natural course of cor pulmonale

Cor Pulmonale - *naturally chronic progression* Could be *acute if Rt Ventricle does not adapt* quickly to increased pul. Arterial pressure - this could kill the person COPD complicated by cor pulmonale have worse 4 yr. survival (<50%) than without cor pulmonale (75%).

What is the initial diagnostic test

Doppler echocardiography

Which of the following are part of the evaluation of suspected PH? Echocardiogram HIV, TSH, LFT's and SCL-70 V/Q scan Sleep evaluation (eg. polysomnogram) Lung MRI

Echocardiogram HIV, TSH, LFT's and SCL-70 V/Q scan Sleep evaluation (eg. polysomnogram) *Lung MRI will never be good for the lungs, there is too much air

Which statement(s) regarding Class 3 PH is incorrect? PH can develop in a large number of patients with COPD and OSA Good oximetry during the day can rule out PH due to obstructive sleep apnea Even mild COPD can cause PH Many clinicians consider Class 3 (and 1) as part of cor pulmonale Most patients do not respond to vasodilator therapy

Good oximetry during the day can rule out PH due to obstructive sleep apnea Even mild COPD can cause PH

Which of the following statements is false? Treating the underlying disease is the best therapy for most PH Supplemental oxygen helps when SaO2 < 89% Most patients will benefit from specific vasodilator therapy Class I PH is less common than Class 2 or 3 Echocardiograms have limited diagnostic value in PH

Most patients will benefit from specific vasodilator therapy

Overview

Right heart failure

PE findings

Very similar to pulmonary HTN *increased intensity of the pulmonic component of S2* (which may be palpable) - due to increased pressure closing pulmonary valve a narrowly split S2 (which may be absent if there is a right bundle branch block) a holosystolic murmur at the left lower sternal border characteristic of *tricuspid insufficiency* in more severe disease- a diastolic pulmonary regurgitation murmur

Causes of cor pulmonale

diseases of the lung *(chronic obstructive pulmonary disease)* vasculature (eg, idiopathic pulmonary arterial hypertension) Conflicting Opinion upper airway (eg, obstructive sleep apnea) chest wall (eg, kyphoscoliosis)

If the echocardiogram is technically inadequate or is normal despite a high clinical suspicion...

perform a diagnostic right heart catheterization

What test confirms the diagnosis of cor pulmonale

right heart catheterization