Cor Pulmonale

Descriptive text is not available for this imageBASICS

DESCRIPTION

  • Cor pulmonale refers to right ventricular (RV) compensatory changes leading to RV failure due to a primary disorder of the lungs that leads to pulmonary hypertension (PH), termed “Group 3 PH” in recent World Health Organization (WHO) classifications.
  • Cor pulmonale does not include PH associated with pulmonary arterial hypertension or congenital heart disease (group 1 PH; see “Pulmonary Hypertension” chapter), persistent PH of the newborn (PPHN; known as group 1 PH), or PH associated with left-sided heart disease (group 2 PH).
  • Cor pulmonale is derived from Latin, meaning “pulmonary heart,” is somewhat nonspecific, and is more commonly used in older adults. “RV failure due to lung disease” may be more accurate.

EPIDEMIOLOGY

  • Cor pulmonale may be found at any age as the result of an acute or long-standing pulmonary processes but is more common in adults than pediatrics.
  • Severe bronchopulmonary dysplasia (BPD) is an increasingly common cause of neonatal PH and cor pulmonale.
  • The incidence of pediatric cor pulmonale is unknown and is disease-specific.
  • Incidence of BPD is center-specific but overall estimated to be between 35% and 40% in extremely premature and extremely low-birth-weight infants (aged <28 weeks, weighing <1,000 g).
    • Prevalence of PH in patients with severe BPD is estimated to be 39%, and those patients are at higher risk for developing cor pulmonale.

ETIOLOGY

  • Parenchymal lung disease
  • BPD
  • Obstructive sleep apnea (OSA)
  • Severe silent aspiration
  • Cystic fibrosis
  • Pulmonary fibrosis and interstitial lung disease
  • Chronic lung infections, possibly related to immune deficiencies or immune dysfunction
  • Sickle cell chronic lung disease
  • Restrictive lung disease
  • Chronic obstructive pulmonary disease
  • Neuromuscular disorders: Duchenne muscular dystrophy
  • Chest wall and thoracic insufficiency syndromes
  • Pulmonary toxins
  • Acute causes include pulmonary embolism and acute respiratory distress syndrome (ARDS).

RISK FACTORS

Genetics

  • Pediatric patients with trisomy syndromes are at high risk for PH and cor pulmonale, often related to underdevelopment of the lung or airway obstruction.
  • Genetic causes have been identified for various developmental lung diseases: alveolar capillary dysplasia (ACD) caused by FOXF1 mutations and genetic abnormalities with surfactant production and catabolism. An increasing list of genes have been found to be associated with interstitial lung disease and lung disease caused by immunodeficiencies and immune dysfunction
  • Cystic fibrosis: several CFTR gene mutations identified
  • OSA: trisomy 21, Prader-Willi syndrome, Pierre-Robin sequence, mucopolysaccharidoses

PATHOPHYSIOLOGY

  • Chronic hypoxia is a principal factor, as well as alveolar hypercarbia and/or acidemia, resulting in cascade of endothelial dysfunction and chronic vasoconstriction.
  • In addition, fibrosis and inflammation associated with lung disease can cause alterations in vasoactive mediators such as nitric oxide, endothelin-1, and platelet-derived growth factors to increase smooth muscle tone, intimal proliferation, and a loss of functional pulmonary arteriolar vascular bed.
  • These factors lead to increased pulmonary vascular resistance (PVR) and increased pulmonary artery pressure. The increased afterload on the RV leads to hypertrophy, dilation, dysfunction, and, eventually failure.

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