>educational:

>other:

>administrative:

>contact us:

>resp notes:

>downloads:

>go back to:

Cor Pulmonale

Introduction

Definition: Right heart failure secondary to chronic (arterial ) pulmonary hypertension

- Arterial pulmonary hypertension
- Normal resting pulmonary artery pressure = around 25 / 8 mmHg
- Pulmonary hypertension = > 30 / 15 mmHg

[Return to top]

---

Differential Diagnosis

Primary Pulmonary Hypertension (PPH)
- sporadic
- familial (25 %)

Chronic hypoxia
- OSA (obstructive sleep apnoea)
- COPD
- severe asthma
- neuromuscular disease (myasthenia / kyphoscoliosis)
- high-altitude residence

Decreased vascular bed
- collagen vascular diseases (scleroderma / CREST / MCTD)
- acute PE
- chronic PEs
- HIV
- toxins / drugs
- ARDS

Volume overload
- L - R cardiac shunt (ASD / VSD / AVSD)
- high-output congestive cardiac failure (CCF): (anaemia / hyperthyroid)

Pressure overload
- ­ LAP (MS / MR / AS / coarctation / LVF)
- venous obstruction (lymphadenopathy / mediastinal fibrosis)

[Return to top]

---

Clinical features

History
- chest pain
- exertional dyspnoea
- syncope
- fatigue

Examination
- cyanosis
- ­JVP (prominent a-waves)
- parasternal heave
- loud P2
- early diastolic murmur (Graham-Steell)
- functional tricuspid regurgitation
- oedema
- hepatomegaly

[Return to top]

---

Investigations

ECG
- RAH (right atrial hypertrophy): P-pulmonale
- RVH (right ventricular hypertrophy): R axis deviation, R in V1, T-inversion in RV leads

CXR
- ­RA / RV
- prominent pulmonary arteries

Bloods
- Hb (secondary polycythaemia)

ABGs
- decreased PaO2
- +/- ­ PaCO2

Lung function tests
- ? fibrosis

Echo
- RVF

Cardiac catheterisation
- >30 mmHg

[Return to top]

---

Management of Cor Pulmonale

- 50% mortality at 5 yrs
- Treat underlying cause

Respiratory failure
- O2
- ± long-term oxygen therapy (if COPD)

CCF
- diuretics
- digoxin

Primary pulmonary hypertension*
- Warfarin: cannot rule out emboli as cause
- Vasodilators: Ca ²⁺ antagonists, PGI analogue: ilaprost (IV), endothelin-antagonists: bosentan (PO)**

Heart-lung transplant
*also for pulmonary hypertension secondary to connective tissue disease
**bosentan is effective in pulmonary HTN (either PPH or in scleroderma) – BREATHE-1, NEJM (2002)

[Return to top]

---