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Cor Pulmonale Overview of Cor Pulmonale

Management
Updated: Mar 03, 2016
Author: Derek Leong, MD; Chief Editor: Henry H Ooi, MD, MRCPI more...

OVERVIEW OF COR PULMONALE MANAGEMENT

Etiology and Pathophysiology of Cor Pulmonale

The pathophysiology of cor pulmonale is a result of increased right-sided filling pressures from
pulmonary hypertension that is associated with diseases of the lung. The increased afterload
leads to structural alterations in the right ventricle (RV) including RV hypertrophy (RVH) which
can be seen in chronic cor pulmonale.

Acute cor pulmonale: pulmonary embolism (more common) and acute respiratory distress
syndrome (ARDS). The underlying pathophysiology in a massive pulmonary embolism causing
cor pulmonale is the sudden increase in pulmonary resistance. In ARDS, RV overload can occur
due to mechanical ventilation and the pathologic features of the syndrome itself. Mechanical
ventilation, especially higher tidal volumes, requires a higher transpulmonary pressure.

In the case of ARDS, cor pulmonale is associated with an increased possibility of right-to-left
shunting through a patent foramen ovale, which carries a poorer prognosis. [2]

Several different pathophysiologic mechanisms can lead to pulmonary hypertension and,

subsequently, to cor pulmonale. The World Health Organization (WHO) has five classifications
for pulmonary hypertension, and all except one of these groups can result in cor pulmonale
(WHO Classification group 2 is pulmonary artery hypertension due to left ventricular [LV]
dysfunction). [3] Note the following WHO classifications:

Group 1: Pulmonary artery hypertension, including heritable causes; connective-tissue

disorders, including scleroderma; and other idiopathic causes
Group 3: Pulmonary hypertension due to lung disease and/or hypoxia; these disorders
include chronic obstructive pulmonary disease (COPD), which is the most common cause
of for pulmonale. There have been studies correlating the degree of hypoxia with the
severity of cor pulmonale. Other disorders that can result in cor pulmonale in this group
include interstitial lung sisease (ILD) and obstructive sleep apnea (OSA)
Group 4: Chronic thromboembolic pulmonary hypertension; blood clots that form in the
lungs can lead to increased resistance, pulmonary hypertension and, subsequently, cor
pulmonale
Group 5: Pulmonary hypertension caused by other diseases or conditions, including
sarcoidosis, polycythemia vera (which can lead to increased blood viscosity and,
subsequently, pulmonary hypertension), vasculitis, and other disorders.

The end result of the above mechanisms is increased pulmonary arterial pressure and
resistance.

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RV and LV output

The RV is a thin-walled chamber that is a better volume pump than a pressure pump. It is better
suited to adapt to changing preload than afterload. With an increase in afterload, the RV systolic
pressure is increased to maintain the circulatory gradient. At a critical point, a further increase in
pulmonary arterial pressure and resistance produces significant RV dilatation, an increase in RV
end-diastolic pressure, and RV circulatory failure.

A decrease in RV output leads to a decrease in LV filling, which results in decreased cardiac

output. Because the right coronary artery originates from the aorta, decreased LV output causes
decreased right coronary blood flow and ischemia to the RV wall. What ensues is a vicious cycle
between decreases in LV and RV output.

RV and LV morphogenesis

Genetic investigations have confirmed that morphogenesis of the right and left ventricle
originated from different sets of progenitor cells. Their differing embryologic origins could explain
the differing rates of hypertrophy of the right and left ventricles. [4]

RV overload

RV pressure and volume overload is associated with septal displacement toward the left
ventricle. Septal displacement, which can be visualized on echocardiography, is an additional
factor that decreases LV filling and output in the setting of cor pulmonale and RV enlargement.

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