pulmonary hypertension

1. PULMONARY HYPERTENSION
MENTOR: DR. MANOJ SHRESTHA ,MD(IOM), MD CARDIOLOGY(PAK)
PRESENTED BY : BHUPENDRA BAJGAIN (INTERN CARDIOMEDICINE)

2. PULMONARY
HYPERTENSION
spectrum of disease involving pulmonary vasculature and defined as an
elevation in pulmonary arterial pressure( MPAP >22mmhg or pulm.
systolic pressure >36 mmHg)

3. ANATOMY AND PHYSIOLOGY:
1. mean pulmonary arterial pressure
(MPAP)
2. pulmonary capillary wedge pressure
(PCWP)
3.transpulmonary gradient
(MPAP-PCWP)

4. CLASSIFICATION
1. GROUP 1-PULMONARY ARTERIAL HYPERTENSION
2. GROUP 2- PULMONARY HTN A/W LEFT HEART DISEASE
3. GROUP 3- PUL. HTN A/W LUNG DISEASE
4. GROUP 4- PH A/W CTED
5. GROUP 5- OTHER CONDITIONS: SARCOIDOSIS, SICKLE CELL
DISEASE,SCHISTOSOMIASIS

5. PATHOBIOLOGY
VASOCONSTRICTION, VASCULAR PROLIFERATION,THROMBOSIS,INFLAMMATION
VASCULAR REMODELLING
DECREASED COMPLIANCE OF PULMONARY VASCULATURE
INCREASED RIGHT VENTRICULAR AFTERLOAD/ TOTAL PULMONARY VASCULAR RESISTANCE
INCREASED MEAN PULMONARY ARTERIAL PRESSURE & DECREASED CO
COMPENSATORY TACHYCARDIA
DECREASED FILLING TIME AND DECREASED PRELOAD
In advanced stage, PAP decreases

6. MOLECULAR DETERMINANTS OF
PATHOGENESIS OF PAH:
 ALTERATIONS IN REGULATORS OF PROLIFERATION
 ALTERATIONS IN INFLAMMATORY MEDIATORS
 ALTERATIONS IN VASCULAR TONE
 HYPOXIA INDUCED REMODELLING
 ALTERATIONS IN TGF-B SIGNALING PATHWAYS

7. Clinical features: NONSPECIFIC,INSIDIOUS AND OVERLAP
WITH ASTHMA ,OTHER LUNG DISEASES&CARDIAC DISEASES
 symptoms:
 dyspnea, excercise intolerance
 fatigue
 chest pain
 pre syncope, syncope
 lower extremety swelling
 signs
 evidence of rt ventricular failure:
i.e. eleveated JVP,lower extremity
edema,ascites,hepatomegaly,pulsa
tile live
 auscultatory; prominent S2 with
loud P2, right sided S3 or S4,
holosystolic tricuspid regurgitant
murmur
 clubbing, sclerodactyly,
telangiectasia, CLD stigmata

8. DIAGNOSIS
1. ECHOCARDIOGRAM WITH BUBBLE STUDY:IMPORTANT INITIAL
SCREENING TEST, Estimate PASP, assess RV charecteristics
2. LABORATORIES- CBC, BUN, S. Cr, LFT, BNP, HIV SEROLOGY
TSH, ANA
3.ELECTROCARDIOGRAPHY: Signs of rt heart enlargement

9. 4. RIGHT HEART CATHETERISATION(RHC):
RHC WITH PULM VASODILATATION TEST
5. CARDIOPULMONARY EXCERCISE TEST(CPET):
6. PULMONARY FUNCTION TEST:
 spirometry and lung volumes
 DLCO
 ABG
 6MW
 Nocturnal oxymetry
6.CHEST RADIOGRAPH: CENTRAL PULMONARY ARTERIES
ENLARGEMENT,VASCULAR PRUNING AND CARDIOMEGALY

10. 7. HRCT CHEST
 ENLARGED PULMONARY ARTERIES, PERIPHERAL PRUNNING OF SMALL
VESSELS, ENLARGED RIGHT VENTRICLE AND RT ATRIUM
 VENOUS CONGESTION INCLUDING CENTRILOBULAR GROUNDGLASS
INFILTRATE AND THICKENED SEPTAL LINES
8. V/Q LUNG SCAN:
9. CT PULMONARY ANGIOGRAPHY:

12. PHARMACOLOGIC TREATMENT OF PAH
1. PROSTANOIDS
2. ENDOTHELIN RECEPTOR ANTAGONISTS
3. NITRIC OXIDE PATHWAY
4. COMBINATION THERAPY
5. PDE5 INHIBITORS
6. SOLUBLE GUANYLATE CYCLASE STIMULATOR

15. SURGICAL MANAGEMENT
 LUNG TRANSPLANTATION OR HEART/LUNG
TRANSPLANTATION
 ATRIAL SEPTOSTOMY
 SEPTAL DEFECT CLOSURE

16. REFERENCES
 Harrisons principle of internal medicine 20th edition
 The Washington manual of medical therapeutics 35th edition

17. THANK YOU