My Clinical Notes

• Lung disease
  • Asthma
  • COPD
  • Bronchietasis
  • Pulmonary fibrosis
• Pulmonary vascular disease
  • PE
  • Pulmonary vasculitis
  • Primary pulmonary hypertension
  • ARDS
• Thoracic cage abnormality
  • Kyphosis
  • Scoliosis
  • Thoracoplasty
• Neuromuscular disease
  • MG
  • Poliomyelitis
  • Motor neurone disease
• Hypoventilation
  • Sleep apnoea
  • Enlarged adenoids in children
  • Cerebrovascular disease

Clinical features

• Dyspnoea
• Fatigue/syncope
• Cyanosis
• Tachycardia
• Raised JVP with prominant a and v waves
• RV heave
• Loud P2
• Pansystolic murmur (tricuspid regurgitation), Graham Steel murmur (pulmonary regurgitation secondary to pulmonary hypertension due to mitral stenosis)
• Hepatomegaly
• Oedema

Investigations

• FBC – Hb and haematocrit raised due to secondary polycythaemia
• ABG – hypoxia with or without hypercapnia
• CXR – enlarged RA and RV, prominent pulmonary arteries
• ECG – P pulmonale, right axis deviation, right ventricular hypertrophy/strain

Management

• Treat underlying causes e.g. COPD and pulmonary infections
• Treat respiratory failure
  • In the acute situation give 24% O2 if PaO2 is <8kPa, monitor blood gases and only increase in the PaCO2 is stable
  • Consider LTOT
• Treat cardiac failure
  • With diuretics such as furosemide
• Consider venesection if haematocrit is >55%
• Consider heart and lung transplant in the young

Prognosis

• 50% die within 5 years

This entry was posted on Sunday, January 18th, 2009 at 7:06 am and is filed under Respiratory. You can follow any responses to this entry through the RSS 2.0 feed. You can leave a response, or trackback from your own site.