Cor Pulmonale

Cor Pulmonale



David W. Hannon, MD



Basic Information



Definition


Cor pulmonale is right-sided heart failure or significant right ventricular hypertrophy (RVH) resulting from pulmonary hypertension. It usually implies that the pulmonary hypertension is caused by pulmonary parenchymal disease, airway obstruction, or hypoventilation syndromes rather than by left-sided heart failure, congenital heart disease, or primary pulmonary hypertension syndromes (see the Pulmonary Hypertension chapter in Diseases and Disorders [Section I] for descriptions of pediatric pulmonary hypertension).



ICD-9-CM Code



416.9 Cor pulmonale


Epidemiology & Demographics


Cor pulmonale is much less common in children than in adults, in whom chronic obstructive pulmonary disease and emphysema are common causes.



Clinical Presentation



History



• Underlying disease history (see “Etiology”)

• Dyspnea

• Fatigue and exercise intolerance

• Syncope


Physical Examination



• Prominent right ventricular impulse on precordial palpation

• Loud, narrowly split or single second heart sound

• Pulmonary artery ejection click

• Jugular venous distention

• Edema or ascites (rare)

• Cyanosis and clubbing in severely hypoxemic patients (e.g., cystic fibrosis)


Etiology



• Bronchopulmonary dysplasia or chronic lung disease after prematurity

• Chronic interstitial pneumonitis, including human immunodeficiency virus (HIV) infection

• Cystic fibrosis

• Muscular dystrophies

• Obstructive apnea

• Primary hypoventilation

• Sickle cell anemia with recurrent pulmonary infarction

• Thoracic dystrophies

• Obstructive apnea and hypoventilation are common in children with syndromic diagnoses in which midfacial hypoplasia and other abnormalities in the growth and development of the facial, oral, pharyngeal, and hypopharyngeal structures may be present.

• Examples of associated abnormalities include the following:
image Trisomy 21 (midfacial hypoplasia)

image Marfan syndrome (palatal abnormality)
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Jun 19, 2016 | Posted by in PEDIATRICS | Comments Off on Cor Pulmonale

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