- Cor pulmonale refers to right ventricular (RV) failure secondary to a pulmonary process that results in a loss of functional capillary vascular bed and in increased pulmonary artery pressure and pulmonary vascular resistance (PVR).
- Cor pulmonale is not used to describe right heart failure associated with congenital heart defects or primary pulmonary vascular disease. It results from pulmonary hypertension (PH) associated with diseases of the lung, termed “Group 3 PH” in recent World Health Organization (WHO) classifications.
- RV failure from PH occurs but is rare in newborns. At birth, RV muscle mass is normally comparable to that of the left ventricle.
- RV failure in newborns is usually a consequence of hypoxemia, ischemia, metabolic acidosis (e.g., persistent fetal circulation), and/or premature restriction/closure of the intrauterine ductus arteriosus.
- Cor pulmonale may be found at any age but is the result of acute or long-standing pulmonary processes.
- Severe bronchopulmonary dysplasia is an increasingly common cause of neonatal PH and cor pulmonale.
The incidence of cor pulmonale is unknown and is disease-specific.
- Upwards of 2 per 1,000 neonatal intensive care unit patients will develop significant cor pulmonale.
- At least 20% of infants with severe bronchopulmonary dysplasia will develop PH and are at risk for cor pulmonale.
- Pediatric patients with trisomy syndromes are at high risk for PH and cor pulmonale, often related to airway obstruction.
- To date, seven genes have been associated with heritable pulmonary arterial hypertension, most notably bone morphogenetic protein type 2 (BMPR2). Genetic etiologies are less frequently found in patients with secondary etiologies of PH, including those related to lung disease.
- Genetic causes have been identified for various developmental lung diseases, including alveolar capillary dysplasia (ACD) and surfactant protein abnormalities.
- Chronic hypoxia is a principal factor, resulting in a cascade of endothelial dysfunction with pulmonary vasoconstriction, followed by the development of PH.
- A variety of vasoactive mediators may be responsible for the effect on vasomotor tone.
- Alveolar hypercarbia and/or acidemia worsen the increased RV afterload and decreased RV systolic function.
- Parenchymal lung disease (most common)
- Chronic obstructive pulmonary disease
- Cystic fibrosis
- Restrictive lung disease
- Pulmonary toxins
- Pulmonary fibrosis
- Bronchopulmonary dysplasia (combined)
- Upper airway diseases causing obstructive sleep apnea (OSA): tonsillar/adenoidal hypertrophy
- Syndromes (Down, Treacher Collins)
- Neuromuscular disorders: Duchenne muscular dystrophy
- Chest wall and thoracic insufficiency syndromes
- Acute causes include pulmonary embolism and acute respiratory distress syndrome (ARDS).
- Genetic syndromes
- Pulmonary vascular abnormalities
- Pulmonary arterial hypertension
- Collagen vascular diseases
- Pulmonary veno-occlusive disease
- Pulmonary thromboembolism
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