Cor Pulmonale
Cor Pulmonale is a topic covered in the Select 5-Minute Pediatrics Topics.
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Basics
Description
- Cor pulmonale is right ventricular (RV) failure secondary to an altered pulmonary process that results in a loss of functional capillary vascular bed and in excessive pulmonary artery pressure and pulmonary vascular resistance (PVR).
- Cor pulmonale is not the result of a primary congenital heart defect.
ALERT
- In newborns, the RV muscle mass is comparable to that of the left ventricle.
- RV failure from pulmonary hypertension (PH) occurs but is rare in newborns.
- 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.
Epidemiology
- Cor pulmonale may be found at any age but is typically a result of a long-standing pulmonary process. However, severe bronchopulmonary dysplasia is an increasingly common cause of neonatal PH.
- Primary pulmonary hypertension (PPHN) is most often diagnosed in the 2nd or 3rd decade of life with a female predominance, and it is often diagnosed during pregnancy.
Incidence
- PPHN has an annual incidence of 2 per million.
Prevalence
- Upward of 2 per 1,000 neonatal intensive care unit patients will develop significant cor pulmonale.
- 2% of infants undergoing cardiac surgery will have PH, with an associated mortality of 10–20%.
Risk Factors
Genetics
- Pediatric patients with trisomy syndromes are at high risk for PH.
- Familial PH has been mapped to chromosome 2q32, but this is less frequently found in patients with secondary etiologies of PH.
- Region 2q32 point mutations encode for a defective bone morphogenic receptor 2, a pulmonary vascular smooth muscle receptor that mediates proliferation.
Pathophysiology
- Chronic hypoxia is the 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 hypoventilation, hypoxemia, hypercarbia, and/or acidemia all result in increased RV afterload and decreased RV systolic function.
Etiology
- Parenchymal lung disease (most common)
- Chronic obstructive pulmonary disease
- Cystic fibrosis
- Asthma
- Restrictive lung disease
- Infectious
- Pulmonary toxins
- Pulmonary fibrosis
- Bronchopulmonary dysplasia (combined)
- Upper airway diseases: tonsillar/adenoidal hypertrophy
- Syndromes (Down, Treacher Collins)
- Neuromuscular disorders: Duchenne muscular dystrophy
- Chest wall deformities
Commonly Associated Conditions
- Pulmonary vascular abnormalities
- Collagen vascular diseases
- Pulmonary veno-occlusive disease
- Pulmonary thromboembolism
- PPHN
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Basics
Description
- Cor pulmonale is right ventricular (RV) failure secondary to an altered pulmonary process that results in a loss of functional capillary vascular bed and in excessive pulmonary artery pressure and pulmonary vascular resistance (PVR).
- Cor pulmonale is not the result of a primary congenital heart defect.
ALERT
- In newborns, the RV muscle mass is comparable to that of the left ventricle.
- RV failure from pulmonary hypertension (PH) occurs but is rare in newborns.
- 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.
Epidemiology
- Cor pulmonale may be found at any age but is typically a result of a long-standing pulmonary process. However, severe bronchopulmonary dysplasia is an increasingly common cause of neonatal PH.
- Primary pulmonary hypertension (PPHN) is most often diagnosed in the 2nd or 3rd decade of life with a female predominance, and it is often diagnosed during pregnancy.
Incidence
- PPHN has an annual incidence of 2 per million.
Prevalence
- Upward of 2 per 1,000 neonatal intensive care unit patients will develop significant cor pulmonale.
- 2% of infants undergoing cardiac surgery will have PH, with an associated mortality of 10–20%.
Risk Factors
Genetics
- Pediatric patients with trisomy syndromes are at high risk for PH.
- Familial PH has been mapped to chromosome 2q32, but this is less frequently found in patients with secondary etiologies of PH.
- Region 2q32 point mutations encode for a defective bone morphogenic receptor 2, a pulmonary vascular smooth muscle receptor that mediates proliferation.
Pathophysiology
- Chronic hypoxia is the 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 hypoventilation, hypoxemia, hypercarbia, and/or acidemia all result in increased RV afterload and decreased RV systolic function.
Etiology
- Parenchymal lung disease (most common)
- Chronic obstructive pulmonary disease
- Cystic fibrosis
- Asthma
- Restrictive lung disease
- Infectious
- Pulmonary toxins
- Pulmonary fibrosis
- Bronchopulmonary dysplasia (combined)
- Upper airway diseases: tonsillar/adenoidal hypertrophy
- Syndromes (Down, Treacher Collins)
- Neuromuscular disorders: Duchenne muscular dystrophy
- Chest wall deformities
Commonly Associated Conditions
- Pulmonary vascular abnormalities
- Collagen vascular diseases
- Pulmonary veno-occlusive disease
- Pulmonary thromboembolism
- PPHN
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