Cor Pulmonale
Cor Pulmonale: Excerpt from The 5-Minute Pediatric Consult
Brian D. Hanna, MD, PhD
Cor Pulmonale - BASICS
Cor Pulmonale - 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 resistance (PVR).
- Cor pulmonale is not the result of a primary congenital heart defect.
- In newborns, the RV muscle mass is comparable to that of the 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.
Cor Pulmonale - epidemiology
- Cor pulmonale may be found at any age, but is typically due to a long-standing pulmonary process. However, severe BPD 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.
Cor Pulmonale - incidence
- PPHN has a yearly incidence of 2/106.
- The incidence of cor pulmonale is dependent on the severity of the underlying lung pathology.
Cor Pulmonale - prevalence
- Upwards of 2/1000 NICU patients will develop significant cor pulmonale.
- 2% of infants undergoing cardiac surgery will have PH with an associated mortality of 10–20%.
Cor Pulmonale - risk factors
Cor Pulmonale - 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 with secondary etiologies of PH.
- Region 2q32 point mutations encode for defective bone morphogenic receptor 2, a pulmonary vascular smooth muscle receptor that mediates proliferation.
Cor Pulmonale - 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.
Cor Pulmonale - etiology
- Parenchymal lung disease (most common)
- Chronic obstructive pulmonary disease:
- 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
Cor Pulmonale - associated conditions
- Pulmonary vascular abnormalities
- Collagen vascular diseases
- Pulmonary veno-occlusive disease
- Pulmonary thromboembolism
- PPHN
Cor Pulmonale - DIAGNOSIS
Cor Pulmonale - signs & symptoms
Cor Pulmonale - history
- Fatigue
- Failure to thrive/weight loss
- Dizziness
- Syncope
- Exercise intolerance
- Chest pain (secondary to RV ischemia)
- Palpitations
- Hemoptysis
Hemoptysis is a life-threatening emergency and heralds a poor prognosis for any patient with PH.
Cor Pulmonale - physical exam
- Tachycardia
- Parasternal RV impulse
- Cyanosis may be evident.
- Hepatomegaly, jugular venous distention, peripheral edema
- A loud, narrowly split or single second heart sound (P2), RV gallop, holosystolic murmur right of the sternum (tricuspid regurgitation), and/or diastolic murmur at the left upper sternal border (pulmonary insufficiency)
In the newborn to puberty, an abnormally increased RV impulse is best felt under the xiphoid sternum.
Cor Pulmonale - tests
Cor Pulmonale - lab
- Brain-type natriuretic peptide is an excellent biomarker of RV diastolic dysfunction and is elevated with worsening cor pulmonale.
- Decreased PaOPolycythemia may be consistent with chronic hypoxemia.
Cor Pulmonale - imaging
- Chest x-ray: Cardiomegaly from RV dilation and main pulmonary artery enlargement
- ECHO. RV dilation, RV hypertrophy, pulmonic insufficiency, and RV pressure estimate from tricuspid regurgitation and/or intraventricular septal position
- V/Q scan is beneficial to rule out thromboembolic disease.
Cor Pulmonale - diag proced-surgery
- EKG: May show right atrial enlargement, RV hypertrophy, and T-wave inversion
- Cardiac catheterization, although invasive, remains the gold standard.
- Lung biopsy is usually contraindicated in the face of PH and significant lung disease.
Cor Pulmonale - differencial diagnosis
- Congenital heart disease with PH and right-to-left shunt (Eisenmenger syndrome)
- Obstruction of pulmonary venous return, both anatomic obstruction and left ventricular failure
- Pulmonary veno-occlusive disease
- Alveolar capillary dysplasia
Cor Pulmonale - TREATMENT
Cor Pulmonale - general measures
- The primary goal is reduction of the abnormally elevated pulmonary artery pressure and the RV workload.
- If at all possible, address the primary etiology (i.e., tonsillectomy/adenoidectomy in a patient with obstructive upper-airway disease).
- Fluid boluses are poorly tolerated and rarely augment systemic BP.
- Oxygen (nocturnal oxygen)
- Diuretics (if pulmonary congestion)
- Bronchodilators (theophylline)
- Digoxin (may improve RV contractility)
- Anticoagulants
- Pulmonary vasodilators:
- Nitric oxide
- Prostacyclin
- Calcium-channel blockers (only if >1 year old and cardiac output is not compromised)
- Sildenafil
- Atrial septostomy (in select cases, may improve cardiac output but at the expense of hypoxemia)
- Lung or heart–lung transplantation
- Usually self limited activity
- No competitive sports
- Arginine, a nitric oxide donor, has been used; however, the increased amino acid concentrations are proliferative and may worsen the long term prognosis.
Cor Pulmonale - medication
Cor Pulmonale - first line
- Oxygen to keep saturations >90%
- Anticongestive medications (digoxin, diuretics)
Cor Pulmonale - second line
Vasodilator therapy with care not to worsen the intrapulmonary shunt
Cor Pulmonale - surgery
Consider tracheostomy, Nissen fundoplication, and G-tube early
Cor Pulmonale - FOLLOW UP
Cor Pulmonale - prognosis
- Patients with reversible lung disease usually have a better prognosis.
- Patients with cor pulmonale are at risk for sudden death because of the inability to augment cardiac output with exercise, growth, or febrile illnesses.
- Numerous medical therapies and lung transplantation may improve long-term survival.
- Long-term survival is variable and depends on the age at onset of pulmonary changes and the underlying conditions (e.g., Down syndrome) that may adversely affect survival.
- Death often occurs in the 2nd or 3rd decade of life.
Cor Pulmonale - complications
Aside from the underlying lung process, the chronic hypoxia results in polycythemia, decreased systemic oxygen delivery, and RV failure secondary to the inability of the RV to handle the excessive afterload.
Cor Pulmonale - patient monitoring
Home oxygen saturation monitoring is indicated when night OCor Pulmonale - bibliography
- Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126(Suppl):35S–62S.
- Bandla HP, Davis SH, Hopkins NE. Lipoid pneumonia: A silent complication of mineral oil aspiration. Pediatrics. 1999;103(2):E19.
- Brouillette RT, Fernback SK, Hunt CE. Obstructive sleep apnea in infants and children. J Pediatr. 1982Jan100(1):31–40.
- Perkin RM, Anas NG. Pulmonary hypertension in pediatric patients. J Pediatr. 1984;105(4):511–522.
- Rashid A, Ivy D. Severe paediatric pulmonary hypertension: New management strategies. Arch Dis Child. 2005;90:92–98.
- Simonneau G, Gallie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(Suppl S):5S–12S.
- Wessel DL, Adatia I, Thompson JE, et al. Delivery and monitoring of inhaled nitric oxide in patients with pulmonary hypertension. Crit Care Med. 1994;22(6):930–938.
- Wheller J, George Bl, Mulder DG, et al. Diagnosis and management of postoperative pulmonary hypertensive crisis. Circulation. 1979;70:1640–1644.
Cor Pulmonale - CODES
Cor Pulmonale - icd9
- 415.0 Acute cor pulmonale
- 416.9 Chronic pulmonary heart disease, unspecified (Cor pulmonale [chronic] not otherwise specified)
Cor Pulmonale - FAQ
- Q: Is cardiac catheterization indicated in all patients with cor pulmonale?
- A: Yes. Although a great deal of information can be learned from echocardiogram, direct pulmonary artery pressure/resistance measurements require an invasive procedure. In addition, assessment of the reactivity of the pulmonary vascular bed to various agents (oxygen, prostacyclin, and calcium-channel blockers) is best performed in the catheterization laboratory.
- Q: Is nocturnal oxygen therapy beneficial?
- A: Nocturnal oxygen has been speculated to delay the progression of cor pulmonale in some select patients with obstructive sleep hypoxemia.
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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» Next page: Pulmonary Hypertension (The 5-Minute Pediatric Consult)
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