Primary Pulmonary Hypertension & Cor Pulmonale

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1 Primary Pulmonary Hypertension & Cor Pulmonale Arohumam Kan, Kingsley Shih, Lerly Luo, Chris Cochrane PHYL 422, Sally Osborne

2 What is pulmonary hypertension? It may be defined as having a mean Pulmonary Arterial Pressure (Ppa) : >25 mmhg at rest or >30 mmhg during exercise Normal resting Ppa = 14.3 ± 3.3 mmhg. This low pressure is due to the large cross-sectional area of the pulmonary circulation low resistance. in pulmonary vascular resistance or pulmonary blood flow results in pulmonary hypertension. Primary Pulmonary Hypertension (PPH) Secondary Pulmonary Hypertension Cor pulmonale The etiology of this condition is unknown. This condition can be attributed to complications of many pulmonary, cardiac and extrathoracic conditions. Cor pulmonale is enlargement of the right ventricle as a consequence of disorders of the respiratory system. Unrelieved pulmonary hypertension, regardless of the underlying cause, leads to right ventricular failure. Hoeper, M.M. (2009). The new definition of pulmonary hypertension. European Respiratory Journal 34,

3 Respiratory Mechanics Expiratory airflow limitation and premature airway closure Airway narrowing and bronchial wall thickening VC Dynamic Hyperinflation in Exercise Possible vasculature-airway coupling Airway Radius PPH associated with nitric oxide (NO) production & Endothelin-1

4 Ventilation & Perfusion Ventilation (V) = amount of air that reaches the alveoli (g/min) Perfusion (Q) = amount of blood that reaches the alveoli (L/min) V/Q ratio measures the efficiency of gas exchange: - 1L air holds 210mL oxygen - 1L blood holds 200mL oxygen ideal V/Q ratio = 1.05 >1 excess ventilation <1 excess perfusion Figure retrieved from West J (2004). Respiratory Physiology, 7th edition.

5 Gas Exchange Gas exchange depends on: Fick's Law of Diffusion: Vgas = (A D[ P])/T D and P= constant A changed by Q or volume in capillaries... V/Q mismatch... Amount of RBC in capillaries = Vol. dependent Good or bad? Depends on where in the lung! But how do we know?... Hoeper, M. M., M. W. Pletz, H. Golpon, and T. Welte. "Prognostic Value of Blood Gas Analyses in Patients with Idiopathic Pulmonary Arterial Hypertension." European Respiratory Journal 29.5 (2007): Print.

6 Arterial Blood Gases Arterial gas pressures: - Hypocapnic (PaCO2 < 4.25kPa) = Physiologic dead space? (vasoconstriction, embolism) = V/Q - Hypoxemic + P(A-a)O2 = Physiologic shunt? (edema) = V/Q - Depends on cause... In particular areas of the lung: Pul. edema, emphysema = Diffusion Thrombo-emboli = Perfusion = G.E. Hypoxic v/c = better V/Q = G.E. Polycythemia = Q = G.E. Hoeper, M. M., M. W. Pletz, H. Golpon, and T. Welte. "Prognostic Value of Blood Gas Analyses in Patients with Idiopathic Pulmonary Arterial Hypertension." European Respiratory Journal 29.5 (2007): Print.

7 Pulmonary Function Pulmonary Hypertension as related to pulmonary function: Class 1: PH without limitations or overt symptoms Class 2: Comfortable at rest Minimal limitation to ordinary exercise including: dyspnea, fatigue, chest pain or near syncope Class 3: Comfortable at rest Marked limitation to activity Class 4: Dyspnea or fatigue may be present at rest Asymptomatic activity not possible Show signs of right heart failure -> Cor Pulmonale Nausser and Stites. Am Fam Physician May 1;63(9):

8 "The heart and lungs are intricately related. Whenever the heart is affected by disease, the lungs will follow, and vice versa. Pulmonary heart disease is by definition a condition when the lungs causes the heart to fail." -Wikipedia

9 Cor Pulmonale Increased resistance and pressure in the lungs causes the right heart to work harder Hypertrophy of the right side of the heart Chronic PH leads to straining of the right heart and ultimately failure, known as Cor Pulmonale

10 Causes of PH & Cor Pulmonale

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