8.

Chronic cor pulmonale

 Definition- referred to as pulmonary heart disease, is defined as dilation and
hypertrophy of the right ventricle with/without right sided heart failure in response to
diseases of the pulmonary vasculature (HT) and/or lung parenchyma.
 Etiology-
o Pulmonary parenchymal- sarcoidosis, COPD, sillicosis
o Pulmonary vascular- Pulmonary hypertension,
Pickwick syndrome,
o Thoracic- pleural adhesions, kyphoscoliosis
 Pathogenesis- the common pathophysiologic mechanism in
each case is pulmonary hypertension that is sufficient to lead
to RV dilation, with or without the development of
concomitant RV hypertrophy. The systemic consequences of
cor pulmonale relate to alterations in cardiac output as well
as salt and water homeostasis. Anatomically, the RV is a thin-
walled, compliant chamber that is better suited to handle
volume overload than pressure overload. Thus, the sustained
pressure overload imposed by pulmonary hypertension and
increased pulmonary vascular resistance eventually causes
the RV to fail. Chronic cor pulmonale is associated with a
slowly evolving and progressive pulmonary hypertension that
leads to initial modest RV hypertrophy and subsequent RV dilation.
o Contributing factors to pulmonary hypertension: anatomic loss of pulmonary
vessels leading to decrease of cross sectional area and the increase of resistance
of the pulmonary vessels. Pulmonary hypertension appears only after >50% of
the vascular tree has been lost. Spasm of pulmonary vessels leads to hypoxemia.
Pulmonary blood flow is increased as a compensatory mechanism leading to
elevation of pulmonary pressure. Polyglobulia and high hematocirt are also
compensatory. There is increase of intraalveolar pressure with prolonged
expiration.
o Hemodynamics- normal pulmonary capillary and venous pressure, pulmonary
systolic pressure is elevated to more than 30mmHg followed by right ventricular
hypertrophy, dilation, tricuspid regurgitation and right heart failure.
 Classification
o Latent- no significant RV hypertrophy. Pulmonary artery systolic pressure is
normal but after physical exercise rises to 27mmHg.
o Manifest- PASP >20mmHg at rest. RV hypertrophy is present. No signs of HF.
o Decompensated- abnormally elevated PASP at rest. RV hypertrophy present and
signs of RV heart failure.
 Clinical- compensated and decompensated (respiratory and heart failure stage)
o Symptoms- related to the underlying pulmonary disorder. Dyspnea, the most
common symptom, is usually the result of the increased work of breathing
secondary to changes in elastic recoil of the lung. Tussive or effort-related
syncope may occur because of the inability of the RV to deliver blood adequately
 to the left side of the heart. Abdominal pain and ascites that occur with cor
pulmonale are similar to the right-heart failure that ensues in chronic HF.
o Signs- Many of the signs encountered in cor pulmonale are also present in HF
patients with a depressed EF, including tachypnea, elevated jugular venous
pressures, hepatomegaly, and lower-extremity edema. Patients may have
prominent v waves in the jugular venous pulse as a result of tricuspid
regurgitation. Other cardiovascular signs include an RV heave palpable along the
left sternal border or in the epigastrium. The increase in intensity of the
holosystolic murmur of tricuspid regurgitation with inspiration (“Carvallo᾽s sign”)
may be lost eventually as RV failure worsens. Cyanosis is a late finding in cor
pulmonale and is secondary to a low cardiac output with systemic
vasoconstriction and ventilation-perfusion mismatches in the lung.
 Diagnosis
o ECG- in severe pulmonary hypertension shows P pulmonale, right axis deviation,
and RV hypertrophy. Increased amplitude >2mm in II, III, avR, V1 and V2.
Incomplete/complete RBBB with rSR or RSR on V1. Atrial tachyarrhythmia’s.
o Xray- enlargement of the main pulmonary artery, the hilar vessels, and the
descending right pulmonary artery. Heart has an increased transversal diameter
and when lying horizontally the right contour of the heart is formed by the right
atrium.
o Echocardiography- useful for measuring RV thickness and chamber dimensions as
well as the anatomy of the pulmonary and tricuspid valves. The interventricular
septum may move paradoxically during systole in the presence of pulmonary
hypertension. As noted, Doppler echocardiography can be used to assess
pulmonary artery pressures.
o Right-heart catheterization is useful for confirming the diagnosis of pulmonary
hypertension and for excluding elevated left-heart pressures (as a cause for right-
heart failure.
 Differential Diagnosis- all left sided pathology leading to pulmonary HT, constrictive
pericarditis, acquired heart disease, pulmonary stenosis and TR.
 Treatment-
o Suppression of pulmonary infection with antibiotics
o For respiratory failure- O2, bronchodilators, seretolytics
o Prophylaxis of respiratory insufficiency
o Decreasing risk factors: smoking, exposure to dusts, metals & humidity.
o For heart failure- diuretics, digitalis
o BETA BLOCKERS Contraindicated causes bronchospasm.
o Decreasing PHT by giving- theofiline, beta2mimetics, vasodilators,
prostaglandins, endothelin receptor antagonists.