Pulmonary hypertension made easy Dr / Waseem Omar, MD Lecturer of Cardiology, Al- Azhar University
Definition Mean pulmonary arterial pressure >25 mmHg, or a systolic pulmonary artery pressure of >40 mmHg at rest.
Methods of measurement Invasive: pulmonary artery catheters (i.e. Swan- Ganz catheter). Non invasive: assessment of TR velocity by continuous Doppler by echo (4 X TV2+ RAP).
Mechanisms of Pulmonary Hypertension Hypoxic vasoconstriction: chronic hypoxia causes pulmonary vasoconstriction. causes: COPD, chronic alveolar hypoxia. Decreased area of pulmonary vascular bed: causes: collagen vascular disease, HIV infection, drugs and toxins, thrombotic or embolic disease, inflammatory, interstitial fibrosis, • Volume and pressure overload pulmonary artery pressure will not rise in otherwise normal lung until pulmonary blood flow exceeds 2.5x the basal rate causes: congenital systemic-to-pulmonary shunts (e.g. VSD, ASD, PDA), portopulmonary HTN, left-sided heart conditions,
II: Pulmonary HTN due to Left Heart Disease Left-sided atrial or ventricular heart disease (e.g. LV dysfunction) Left-sided valvular heart disease (e.g. aortic stenosis, mitral stenosis)
III: Pulmonary HTN due to Lung Disease and/or Hypoxia. Parenchymal lung disease (COPD, interstitial fibrosis, cystic fibrosis) Chronic alveolar hypoxia (chronic high altitude, alveolar hypoventilation disorders, sleep-disordered breathing)
IV: Chronic Thromboembolic Pulmonary HTN (CTEPH) V: Pulmonary HTN with Unclear Multifactorial Mechanisms Hematologic disorders Systemic disorders (e.g. sarcoidosis ) Metabolic disorders Extrinsic compression of central pulmonary veins
Symptoms Symptoms of systemic congestion Dyspnea Fatigue Retrosternal chest pain Syncope Symptoms of underlying disease
Signs Loud, palpable P2 RV heave Right-sided S4 (due to RVH) Systolic murmur (tricuspid regurgitation) If RV failure: right sided S3, increased JVP, positive HJR, peripheral edema ,
Investigations CXR: enlarged central pulmonary arteries and attenuation of peripheral Vs ( bruning ), cardiac changes due to RV enlargement (filling of retrosternal air space). ECG: RVH/right-sided strain. 2-D echo doppler : assessment of right ventricular systolic pressure. Cardiac catheterization: direct measurement of pulmonary artery pressures (necessary to confirm diagnosis). PFTs: for assessmnet of underlying lung disease: CT angiogram: to assess lung parenchyma and possible PE. Serology: ANA positive in 30% of patients with primary pulmonary HTN;
General rules Mild physical activity Routine influenza and pneumococcal vaccine. Digoxin Anticoagulant Avoid high altitude. Oxygen therapy to maintain SO2 ≥ 90%. Diuretics. Contraception as PHT increase mortality by 30-50%.
Specific treatment I: No effective treatment CCBs. prostanoids , Endothelin receptor antagonists, PDE5 inhibitors Lung transplantation