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Subject: Cor Pulmonale
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Enlargement and subsequent dysfunction and failure of the right ventricle (RV) in the presence of pulmonary hypertension secondary to abnormalities of the pulmonary system, including disorders of the lung parenchyma, pulmonary circulation, chest wall, and ventilatory mechanisms.
For the purposes of this review, pulmonary arterial hypertension (WHO Group I) will not be considered as a cause of cor pulmonale.
May occur in acute or chronic setting
∽6-7% of all types of adult heart disease in United States
Estimated 10-30% of heart failure admissions in the United States are the result of cor pulmonale, most commonly related to chronic obstructive pulmonary disease (COPD).
Acute: A sudden event, such as large pulmonary embolism (PE), increases resistance to blood flow in the pulmonary vasculature, causing a quick and significant increase of pressure proximally. The RV is unable to overcome this pressure, leading to low RV cardiac output, which ultimately leads to low left ventricle (LV) cardiac output. Increased RV pressures in conjunction with a low cardiac output may cause coronary ischemia, further impairing cardiac output and potentially causing complete cardiovascular collapse.
Chronic: A disorder of the pulmonary system causing chronic hypoxia leads to vasoconstriction of the pulmonary vasculature. Over time, the pulmonary vasculature hypertrophies and the intrinsic vasodilatory mechanisms (mediated by nitric oxide) become dysregulated, leading to increases in pulmonary vasculature resistance. The resulting pulmonary hypertension (WHO Group III) transmits increased pressures and volumes to the thin-walled, low-pressure RV, causing maladaptive remodeling (concentric hypertrophy, followed by eccentric dilation frequently with associated tricuspid regurgitation) and subsequent impairment in RV systolic and diastolic function.
LV failure is not considered a cause of cor pulmonale.
Acute cor pulmonale (most commonly caused by PE)
Chronic cor pulmonale (most commonly caused by underlying pulmonary disorder)
Dyspnea is the most common symptom, although nonspecific; may be present at rest, with exertion, or occur as paroxysmal nocturnal dyspnea
Other pulmonary symptoms: pleuritic chest pain, cough, hemoptysis
General heart failure symptoms: fatigue, lethargy, syncope; exertional angina less likely
Right-sided heart failure symptoms: anorexia, early satiety, right upper quadrant discomfort from hepatic congestion, lower extremity edema
Hoarseness secondary to compression of the left recurrent laryngeal nerve by enlarged pulmonary vessels
Cardiovascular collapse, shock, and/or cardiac arrest may occur in acute or advanced chronic setting.
Peripheral edema is the most common sign of right-sided heart failure, although it is nonspecific.
General: pallor, diaphoresis, cyanosis, tachypnea
Neck: jugular venous distension, with prominent a-wave
Lungs: tachypnea, wheezing
Extremities: clubbing, bilateral lower extremity edema, also signs of deep vein thrombosis (DVT) such as tenderness or unilateral swelling
Left-sided heart failure (WHO Group II)
WHO Groups I and V pulmonary hypertension
Right-sided intrinsic cardiomyopathy
2D echocardiogram 1[C]
Right heart catheterization 1[C]
CBC may show signs of polycythemia due to chronic hypoxia.
Basic metabolic panel (BMP) may demonstrate elevated creatinine secondary to poor cardiac output.
Liver function tests (LFTs) may be abnormal due to hepatic congestion or poor cardiac output secondary to RV failure.
Brain natriuretic peptide (BNP) and cardiac troponin can be elevated secondary to right ventricular strain.
D-dimer may be positive as evidence of underlying thromboembolic pulmonary disease.
Arterial blood gas may show hypercapnia due to COPD.
ECG often shows signs of right-sided enlargement.
Spiral CT scan of chest
Ventilation/perfusion scan (V/Q)
Pulmonary function tests (PFTs)
Treat underlying disease 2[A].
Supportive therapy as necessary
Preservation of cardiac function 4[B]
Ameliorate pulmonary hypertension 1,4[C]
Endarterectomy for chronic thromboembolic disease (WHO Group IV)
Moderate to severe disease refractory to medication may require lung and/or heart transplantation.
Smoking cessation and avoidance of exposure to secondary smoke is strongly recommended.
Level of physical activity should be discussed with physician.
Pregnancy should be avoided.
Patients with cor pulmonale resulting from COPD have a greater likelihood of dying than do similar patients with COPD alone.
Pulmonary arterial pressure is a reliable indicator of prognosis; higher pressure is associated with a worse prognosis.
In patients with COPD and mild disease (PAP 20 to 35 mm Hg), 5-year survival is 50%.
I27.81 Cor pulmonale (chronic)
I26.09 Other pulmonary embolism with acute cor pulmonale
416.9 Chronic pulmonary heart disease, unspecified
415.0 Acute cor pulmonale
83291003 Cor pulmonale (disorder)
49584005 Acute cor pulmonale (disorder)
79955004 Chronic cor pulmonale (disorder)
Treatment of cor pulmonale requires treatment of the underlying disease.
Continuous, long-term oxygen therapy improves life expectancy and quality of life in cor pulmonale.
Referral of patients with cor pulmonale to a specialized center is strongly recommended.