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RV enlargement and/or altered function resulting from primary lung disease; leads to RV hypertrophy and eventually to RV failure. Etiologies include:

Symptoms !!navigator!!

Depend on underlying disorder but include dyspnea, cough, fatigue, and sputum production (in parenchymal diseases).

Physical Examination !!navigator!!

Tachypnea, RV impulse along left sternal border, loud P2, right-sided S4; cyanosis, clubbing are late findings. If RV failure develops, elevated jugular venous pressure, hepatomegaly with ascites, pedal edema; murmur of tricuspid regurgitation is common.

Laboratory ECG !!navigator!!

Right axis deviation, RV hypertrophy, and RA enlargement (Chap. 113 Electrocardiography); tachyarrhythmias are common.

Radiologic Studies !!navigator!!

CXR shows RV and pulmonary artery enlargement; if PAH present, tapering of the pulmonary artery branches. Chest CT identifies emphysema, interstitial lung disease, and acute pulmonary embolism; V/Q nuclear scan is more reliable for diagnosis of chronic thromboemboli. Pulmonary function tests and ABGs characterize intrinsic pulmonary disease.

Echocardiogram !!navigator!!

RV hypertrophy; LV function typically normal. RV function can be quantitated by measuring tricuspid annular excursion during systole and Doppler velocity of the RV free wall. RV systolic pressure is estimated from Doppler measurement of tricuspid regurgitant velocity. If imaging is difficult because of air in distended lungs, RV volume and wall thickness can be evaluated by MR imaging.

Right-Heart Catheterization !!navigator!!

Confirms presence of pulmonary hypertension and can exclude left-HF as contributor.

TREATMENT

COR Pulmonale

Aimed at underlying pulmonary disease and may include bronchodilators, antibiotics, oxygen administration, and noninvasive mechanical ventilation. For pts with PAH, pulmonary vasodilator therapy may be beneficial to reduce RV afterload (Chap. 129 Pulmonary Hypertension). See Chap. 135 Pulmonary Thromboembolism and Deep-Vein Thrombosis for treatment of pulmonary embolism.

If RV failure is present, treat as HF, instituting low-sodium diet and diuretics; digoxin is of uncertain benefit and must be administered cautiously (toxicity increased due to hypoxemia, hypercapnia, acidosis). Loop diuretics must also be used with care to prevent significant metabolic alkalosis that blunts respiratory drive.

Outline

Section 8. Cardiology