- This disease also known as Pulmonary haert disease.
- This disease solely caused by primary disorders of respiratory system
- If this disease is chronic then right ventricular hypertrophy develops progressively but in case of acute cor pulmonale right ventricular dilatation may occur due to acute increase pressure instead of hypertrophy.
- Right sided ventricular disease caused by a primarily left sided heart disease or congenital heart disease is not considered as cor pulmonale.
- Almost any chronic lung disease or condition causing prolonged low oxygen blood flow can lead to cor pulmonale
Causes of cor pulmonale:
- Chronic obstructive pulmonary disease ( COPD )***
- Primary pulmonary hypertension***
- Cystic fibrosis
- Interstitial lung disease ( Due to scarring of lung tissue )
- Obstructive sleep apnea
- Recurrent pulmonary embolism ( Chronic blood clots in the lungs)
- Sarcoidosis
- Kyphoscoliosis
- Pneumoconiosis
- Altitude sickness
- Sickle cell anemia
- Loss of lung tissue following lung trauma or surgery
Acute causes
- Acute massive pulmonary embolism***
- Acute respiratory distress sysndrome ( ARDS )
- Acute exacerbation of cor pulmonale
Mnemonics for causes of Cor pulmonale: PuLmonary HTN
P = Pulmonary vascular disease
L = Lung disease
H = Hypoventilation
T= Thoracic abnormality
N = Neuromuscular disease
P = Pulmonary vascular disease
- Primary pulmonary hypertension
- Pulmonary embolism ( Acute massive PE or Chronic recurrent PE )
- Pulmonary vasculitis
- Sickle cell anemia
- ARDS
- Parasite infestation
L = Lung disease
- COPD
- Asthma ( Severe, Chronic)
- Bronchiectasis
- Cystic fibrosis
- Interstitial lung disease
- Loss of lung tissue following lung trauma or surgery
- Sarcoidosis
- Pneumoconiosis
H = Hypoventilation
- Obstructive sleep apnea
- Cerebrovascular disease
- Adenoids enlargements in children
T= Thoracic abnormality
- Kyphosis
- Scoliosis
N = Neuromuscular disease
- Polioymyelitis
- Motor neurone disease
- Myasthenia gravis
Pathophysiology
1, Pumonary vasoconstriction
- Due to respiratory disease and poor lung function can leads to alveolar hypoxia and blood acidemia.
- Alveolar hypoxia and blood acidemia can results in vasoconstriction of pulmonary arteries
- This mechanism leads to pulmonary hypertension and if not treated cor pulmonale ensues
2, Anatomic changes in pulmonary vascular bed
- It occurs secondary to parenchymal lung disease or alveolar lung diseases. e.g Emphysema in COPD, interstitial lung disease, massive pulmonary emobolism, cystic fibrosis.
- This causes increased in pulmonary pressure
3, Hyperviscosity of blood
- Increased blood viscosity is secondary to polycythemia vera ( secondary), sickle cell anemia, macroglobulinemia
4, Increased blood flow in pulmonary vasculature
5, Idiopathic or primary pulmonary hypertension
All above mechanism finally leads to increased pulmonary hypertension and later develop cor pulmonale
Clinical presentation
Symtpoms:
- Shortness of breath ( Initially exertional dyspnea but in severe cases can occurs at rest )
- Wheezing
- Fatigue and syncopal attack
- Chest discomfort with anginal pain due to RV ischemia or pulmonary artery stretching
- Chronic wet cough
- Hemoptysis
- Ascites
- Hepatomegaly and hepatic congestion secondary to right heart failure
- Anorexia due to hepatic involvement
- Right upper quadrant abdominal pain and discomfort due to hepatomegaly and congestion
- Abnormal heart sounds
- Hoarseness of voice due to compression of left recurrent laryngeal nerve by dilated pulmonary artery ( very rare )
Inspection:
- Tachypnea
- Laboured respiratory efforts with retractions of respiratory muscles
- Enlargement of prominent neck and facial veins
- Cyanosis: Bluish dicolouration of face
- Jaundice due to hepatomegaly and congestion
Palpation:
- Hepatomegaly
- Pedal edema
Auscultation:
- Wheezing
- Splitting P2 sound in early stages
- Systolic ejection murmur with sharp ejection click heard in pulmonary area
- Diastolic pulmonary regurgitation
- S3 or S4 sound may be heard
- Systolic murmur of tricuspid regurgitation may be heard
Percussion:
- Hyperresonance sound in case of COPD
- Dull percussion note or fluid thrill in case of ascites