Pulmonary Hypertension

Relevant clinical signs

• Peripheries
  • Clubbing
  • Cyanosis
  • Pulse – rate and rhythm
  • Central cyanosis
  • Raised jugular venous pulsation, giant v waves
• Precordium
  • Palpation
    • Apex beat – may be displaced if caused by intracardiac shunts
    • Right ventricular heave
    • Palpable thrills
  • Auscultation
    • Loud S1 (mitral stenosis)
    • Loud P2 (pulmonary hypertension)
    • Pan-systolic murmur at lower left sternal edge (tricuspid regurgitation from PH)
    • Early diastolic murmur at pulmonary area (Graham-Steele murmur)
    • Pan-systolic murmur radiating to axilla (mitral regurgitation)
    • Murmurs of VSD, ASD and PDA disappear with development of Eisenmenger’s syndrome
• Lungs
  • Coarse crepitations of bronchiectasis
  • Fine crepitations of pulmonary fibrosis
  • Pulmonary oedema

Causes of pulmonary hypertension

• Primary pulmonary hypertension
• Secondary pulmonary hypertension
  • Intracardiac shunts
    • Atrial septal defect
    • Ventricular septal defect
    • Patent ductus arteriosus
  • Left sided valvular heart disease
    • Mitral stenosis
    • Mitral regurgitation
    • Aortic regurgitation
    • Aortic stenosis
  • Chronic hypoxia
    • Chronic thromboembolic pulmonary hypertension
    • Interstitial lung disease
    • Obstructive airways disease
    • Bronchiectasis

Investigations

• Chest radiograph: dilated pulmonary arteries, cardiomegaly, overt lung disease
• Electrocardiogram: dominant R wave in V1 (right ventricular hypertrophy)
• Trans-thoracic echocardiogram
  • Pulmonary artery systolic pressure > 25mmHg
  • Look for underlying aetiology – e.g. mitral stenosis, mitral regurgitation
• Right heart catheterization – responsiveness to vasopressors
• High-resolution computed tomography scan of the lungs
• V/Q scan / CTPA – chronic thromboembolic pulmonary hypertension

Management

• Multidisciplinary team approach
• Patient education, counselling on contraception, avoid pregnancy
• Anticoagulation for primary pulmonary hypertension (target INR 2 – 3)
• Diuretics for congestive cardiac failure
• Pulmonary vasodilators
  • Calcium channel blockers
    • Nifedipine and diltiazem
    • Contraindicated in patients with right heart failure
    • Response to CCB associated with 95% 5-year survival
  • Endothelin antagonists
    • Bosentan and ambrisentan
  • Phosphodiesterase-5 inhibitors
    • Sildenafil and tadalafil
  • Prostaglandin analogues
• Long-term oxygen therapy
• Surgery
  • Atrial septostomy – palliative procedure, creation of right-to-left shunt
  • Heart and lung transplantation

Summary

Sir, this patient has pulmonary hypertension. On examination of the peripheries, there are no stigmata of infective endocarditis. The patient is not clubbed, and there is no peripheral or central cyanosis. The jugular venous pressure is elevated, and there are giant v waves. On examination of the precordium, the apex beat is not displaced. There is a right ventricular heave, with no other palpable thrills. The heart sounds are dual, with no loud S1 to suggest underlying mitral stenosis. The pulmonary component of the second heart sound is loud. In addition, there is an early diastolic murmur over the pulmonary area, likely representing a Graham-Steele murmur of functional pulmonary regurgitation secondary to pulmonary artery dilation. There is also a pan-systolic murmur heard loudest over the lower left sternal edge and in full inspiration. In the context of pulmonary hypertension and giant v waves, this likely represents tricuspid stenosis. The lung bases are clear to auscultation. There is pitting oedema of the legs.

Causes of pulmonary hypertension include primary pulmonary hypertension, left-sided heart disease and chronic hypoxia secondary to chronic lung disease such as pulmonary fibrosis, bronchiectasis or chronic thromboembolic pulmonary hypertension. There is no evidence of left-sided valve disease on clinical examination.

In summary, this patient has pulmonary hypertension, likely complicated by functional tricuspid and pulmonary regurgitation.