Pulmonary Hypertension by dhanush krishna

a ppt done by a medical student

1. Pulmonary Hypertension By: Dhanush Krishna V

2. Introduction: Pulmonary artery pressure = Cardiac output(CO) x pulmonary vascular resistance (PVR) + left atrial pressure(LAP) Normal pulmonary artery pressure are as follows: Systolic pressure 15-25 mm Hg Diastolic pressure 5-10 mm Hg Mean pressure 10-15 mm Hg Pulmonary hypertension is present when pulmonary artery systolic pressure is >30 mm Hg, and pulmonary artery mean pressure is >25 mm Hg

3. WHO Classification of Pulmonary hypertension Type 1: Small vessel pulmonary arteriopathy leading to increased PVR – known as Pulmonary artery hypertension (PAH) or Idiopathic Pulmonary Hypertension (IPH) Type 2: Pulmonary Venous Hypertension secondary to left heart disease: Left ventricular systolic dysfunction Left ventricular diastolic dysfunction Valvular heart disease Congenital/ acquired left heart inflow or outflow tract obstruction

4. Type 3 : Secondary to lung disease or hypoxemia: Advanced obstructive or restrictive lung disease (COPD, ILD, Pulmonary fibrosis, bronchiectasis, OSAS, alveolar hypoventilation syndrome, High altitude) Developmental lung disorders Type 4 : Due to Chronic thromboembolic pulmonary hypertension( treatable by surgical intervention) Type 5 : Miscellaneous causes: Chronic hemolytic anemia, sarcoidosis, tumor embolization, myeloproliferative disorder.

5. Pulmonary Artery Hypertension (Type 1) Causes: 1.Most common: Idiopathic 2.Secondary causes: BMPR2 mutation Drugs and toxins- example: Aminorex, Fenfluramine Portal hypertension HIV/schistosomiasis Autoimmune diseases: Limited systemic sclerosis Pathogenesis: Three factors are thought to cause the increased PVR that characterizes the disease.

6. 1 .Vasoconstriction: Under expression of vasodilators like NO and prostacyclin. Overexpression of vasoconstrictors like endothelin 1. 2. Remodelling of pulmonary vessel wall 3. Thrombosis in situ: Blood clots Clinical Presentation : 1.Dyspnea/ breathlessness 2.Angina/ chest pain 3.Features of right ventricular failure 4.Fatigue and weakness 5.Edema/swelling 6.Syncope/passing out

7. Signs in PAH: A wave in JVP Loud P2 with wide splitting of S2 (seen only in RV failure) RVS3 in right heart failure due to increased diastolic filling pressure Ascites/ Pedal edema /TR murmur/ apex shift/ hepatomegaly Gold standard: Pulmonary arteriography Murmurs in PAH: 1. Tricuspid regurgitation: High pitched holosystolic blowing murmur along left sternal border increased on inspiration.

8. 2. Pulmonary stenosis: Ejection systolic murmur 3. Severe pulmonary hypertension: High pitched early diastolic murmur(EDM) Other investigations: Pulmonary function tests : FEVI: Normal FVC: Normal FEVI/FVC: Normal DLCO: Reduced ECHO: To look for RV function and tricuspid regurgitation.

9. Treatment: Based on vasoreactive test: Administration of inhaled NO, a preferential pulmonary arterial vasodilator. Patients with positive vasoreactive test : Calcium channel blockers( 240 mg of nifedipine given). Vasoreactive test negative : 1.Endothelin receptor antagonists(ERAS): Bosentan, Sitaxsentan, Ambrisentan 2.PDE-5 inhibitors: Sildenafil, Tadalafil, Vardenafil 3.Prostanoids: Epoprostenol, Treprostinil, Iloprost

10. Thank You Please keep this slide for attribution