Pulmonary hypertension

diagnosis and management of pulmonary hypertension

1. APPROCH TO A CASE OF PULMONARY HYPERTENSION D R.MD SHAHID IQUBAL Deptt of Medicine NMCH,PATNA

2. INTRODUCTION Definition- Pulmonary hypertension (PH) is a spectrum of diseases involving the pulmonary vasculature, and is defined as an elevation in pulmonary arterial pressures (mean pulmonary artery pressure >22 mmHg).

3. Classification - PH is subcategorized into five major groups : Group I— Pulmonary arterial hypertension (PAH) Group II— PH due to left heart disease Group III— PH due to lung diseases and/or hypoxemia( COPD , Interstitial lung disease, OSA) Group IV— Chronic thromboembolic pulmonary hypertension (CTEPH) Group V— PH with unclear mechanisms

4. Pulmonary Arterial Hypertension PAH is defined as a sustained elevation in resting mPAP ≥ 25 mmHg , and PCWP or left ventricle end-diastolic pressure of ≤15 mmHg based on a right heart catheterization. Idiopathic pulmonary arterial hypertension (IPAH ) is a progressive disease that leads to right heart failure and death PAH associated with HIV, connective tissue disease(systemic sclerosis),and portal hypertension.

5. DIAGNOSIS Clinical presentation- Symptoms of PH- The symptoms of PH are often nonspecific and variable include dyspnea (most common), exercise intolerance, fatigue , palpitations , syncope , chest pain, lower extremity swelling,

6. DIAGNOSIS contd …. On examination- evidence of right heart failure- elevated jugular venous pressure , lower extremity edema, and ascites. cardiovascular examination- prominent second heart sound (loud S2) with loud P2 component ound , a right-sided S3 or S4, and a holosystolic tricuspid regurgitant murmur clubbing in chronic lung diseases , sclerodactyly and telangiectasia in scleroderma, and crackles and systemic hypertension in left-sided systolic or diastolic heart failure

7. Diagnostic Testing- echocardiogram –(TTE) Is screening test Shows hypertrophied and dilated right ventricle with elevated estimated pulmonary artery systolic pressure Identify causes of PH - left ventricular systolic and diastolic dysfunction, valvular disease right heart catheterization - for definitive diagnosis Measure cardiac output and mean right atrial pressure (RAP ) and asses severity condition

8. Diagnostic Testing contd … Chest x-ray- Large central pulmonary arteries with “vascular pruning,” Cardiomegaly due to enlarged RA ,RV Regional oligemia of pulmonary vasculature (chronic thromboembolic disease) Interstitial infiltrates (ILD) Hyperinflated lungs(chronic obstructive lung disease). HRCT- Enlarged pulmonary arteries , peripheral pruning of the small vessels,and enlarged right ventricle and atrium Centrilobular ground-glass infiltrate Ventilation–Perfusion (V/Q) lung scan- Used for excluding chronic thromboembolic disease

9. Diagnostic Testing contd … Pulmonary Function Testing- Spirometry and lung volumes to assess for COPD or restrictive- interstitial lung disease [ILD ]) Diffusing capacity for carbon monoxide (DLCO) reduced in parenchymal lung diseases Arterial blood gas (ABG): Elevated PaCO2 is found in hypoventilation syndrome . Six-minute walk (6MW) or simple exercise test- to evaluate the degree of exertional hypoxemia and to monitor progression and response to therapy Nocturnal oximetry : . Nocturnal desaturations is common finding in PH Nocturnal desaturations can be find in OSAS

10. Diagnostic Testing contd … ECG- RVH, right atrial enlargement , right bundle branch block, and right ventricular strain pattern Acute vasodilator testing .- Performed with a short-acting vasodilator , such as intravenous adenosine, intravenous epoprostenol , or inhaled nitric oxide Definition of a responder is decrease in mPAP by ≥10 mm Hg to an absolute level ≤ 40 mm Hg without a decrese in CO . responders are considered for long-term treatment with CCBs.

11. Diagnostic Testing contd … Laboratories Complete blood counts (CBCs) Blood urea nitrogen, serum creatinine Hepatic function tests B-type natriuretic peptide (BNP) Human immunodeficiency virus (HIV) serology Thyroid-stimulating hormone (TSH) Antinuclear antibody (ANA) Antitopoisomerase antibodies and anticentromere antibodies hepatitis B and C serologies

12. TREATMENT General measures- Avoid pregnancy Immunization for respiratory illness influenza and pneumovax vaccination Minimize valsalva maneuvers-increase risk of syncope cough, constipation,heavy lifting Should stop cigarette smoking

13. Classes of therapy MEDICAL- Diuretics Anticoagulants Inotropic agents Oxygen PAH specific therapy SURGICAL THERAPY- Lung transplantation or heart–lung transplantation Atrial septostomy Septal defect closure

14. Treatment contd … DIURETICS- Principally to treat edema from right heart failure May need to combine classes(loop diuretic +aldosterone antagonist+ thiazides) Careful to avoid too much preload reduction ANTICOAGULANTS- Warfarin is dosed to target international normalized ratio (INR) of 1.5 to 2.5 INOTROPIC AGENT improves right heart function, cardiac output, and symptoms. Dobutamine and milrinone are best for short-term use in extremely decompensated states .

15. PAH SPECIFIC THERAPY CCBs Endothelin receptor antagonists - Bosentan,ambrisentan,masitentan Phosphodiesterase-5 inhibitors - Sildenafil,tadalafil Prostanoids - epoprostenol,treprostinil guanylyl cyclase stimulator- riociguat

16. Treatment contd …. CCBs- Used only in showing positive vasodilator response Diltiazem or nifedipine preferred Endothelin receptor antagonists- Endothelin receptor antagonists target endothelin-1 (ET-1), a potent endogenous vasoconstrictor improve symptoms, 6MWD,and WHO functional class Phosphodiesterase-5 inhibitors- block the enzyme that shuts down nitric oxide–mediated vasodilation and platelet inhibition .

17. Treatment contd …. Prostanoids - epoprostenol,treprostinil Epoprostenol - continuous intravenous infusion improves functional capacity and survival in PAH. Inhaled prostacycline has no risk of infection and infusion site reaction guanylyl cyclase stimulator- Stimulator of nitric oxide receptor Riociguat is an oral soluble guanylyl cyclase stimulator-approved for the treatment of both PAH and CTEPH

18. FOLLOWING RESPONSE TO THERAPY Six minute walk test Echocardiogram Right heart catheterisation BNP Funtional class

19. Take home message- Idiopathic pulmonary arterial hypertension is a progressive disease Transthoracic Echocardiography is screening test Definitive diagnosis by right heart catheterization there is no cure for PAH, current pharmacologic therapies improve morbidity and, in some cases mortality Improved awareness among clinicians and patients could lead to more timely diagnosis that will affect the response to therapy and survival.

20. THANK YOU