Pulmonary Hypertension Flashcards
PULMONARY HYPERTENSION
• Mean PAP >25mmHg at rest measured by
Right Heart Catheterisation; WHO
classification includes PH due to lung
disease/Cor Pulmonale (WHO III) and
Chronic Thromboembolic PH (WHO IV)
• Patients with progressive Pulmonary
Hypertension develop RCH, Dilatation,
Failure and Death
• Presents as Dyspnoea, Fatigue, Weakness,
Angina, Syncope or Abdominal Distention; Clinical signs include RVH, Loud P2, Soft Pansystolic
Murmur (Tricuspid Regurgitation) or Early Diastolic Murmur (Pulmonary Regurgitation)
o RHF presents as ↑JVP, Ascites, Peripheral Oedema, Hepatomegaly
• Following Acute PE 0.5% to 2.0% of patients will develop CTEPH (WHO IV)
Investigations for Pulmonary Arterial Hypertension
• CXR – Enlargement of Pulmonary Arteries and Major branches; Marked Pruning of Peripheral
Arteries; Lung Fields are Lucent and there may be Right Atrial and Ventricular Enlargement
• ECG – RVH, RA Dilatation (P Pulmonale in II); Useful to detect Left Ventricular involvement
• Echo – Estimation of PAP using Bernoulli equation
• Cardiac MRI for Congenital Heart Disease or assessing RV function
• FBC, U/Es, LFT, TFT, Serological Assays for CTD, HIV and Hepatitis
• Right Heart Catheterisation – Confirm PAP, PWP, CO, PVR and reactivity
o Vasodilator challenge to identify benefit to treatment; >10mmHg decline to reach
mPAP of <40mmHg without decline in CO; Only for Idiopathic PAH
Treatment of Pulmonary Arterial Hypertension
• Lifestyle – Physical activity but non-exertional; Advised against conception (High mortality;
use barrier, POP, coil), Supplementary O2 on plans at 2L/min if ↓NYHA and hypoxia <8 kPa
o Vaccination – Against Influenza and Pneumococcus
o Elective Surgery – Use of Epidural Anaesthesia rather than General Anaesthetic
• Oral Anticoagulation – IPAH, Heritable PAH, PAH; INR target 2-3
• Diuretics – RHF and Fluid Overload
• Digoxin for patients with Tachyarrhythmias
• CCB for IPAH patients who demonstrate response to vasodilator challenge; RHC in 3-4 months
to assess response to therapy
• Prostacyclin (Epoprostenol) – Improves survival in IPAH and APAH; Aerosol, IV or SC
• Endothelin Receptor Antagonists – Bosentan (Dual receptor) and Sitaxsentan (ETA selective)
• PDE5 Inhibitors – Sildenafil and Tadalafil produce vasodilation in Pulmonary Vasculature and
reduce Cell Proliferation; Symptomatic relief and improved exercise capacity in IPAH
• Surgical management includes Atrial Septostomy, Pulmonary Endarterectomy (For CTEPH)
and Lung/ Heart-Lung Transplantation
