This document provides an overview of pulmonary hypertension (PH), including its history, clinical findings, investigations, classification, pathophysiology, and management. Some key points: - PH can be caused by various underlying conditions and diseases. A thorough workup is needed to determine the specific type and cause. - Common presenting symptoms are dyspnea and fatigue. Physical exam may reveal signs of right heart failure as disease progresses. - Initial screening tests include echocardiogram, ECG, CXR. Right heart catheterization is the gold standard for diagnosis and assessing severity. - PH is classified into 5 types based on underlying pathophysiology. The main types are PAH, PH related to lung/

1. Dr Arya Mahapatra
NRS Medical College
APPROACH TO PULMONARY
HYPERTENSION

2. • Outline
a) Introduction
b) History
c) Clinical findings
d) Investigations
e) Diagnostic algorithm
f) Discussion of PH
g) Management

3. Introduction
• Pulmonary Hypertension diagnosis is important.
• PH is underdiagnosed.
• Different diseases cause PH.
• Numerous factors influence the management of PH patients.
• Thinking Physiologically is useful in approach to PH.

4. HISTORY
• Most patients will present with dyspnea and/or fatigue.
• Whereas edema, chest pain, presyncope, and syncope are less
common and associated with more advanced disease.
• In early phases of PAH, the physical examination is often unrevealing.
• As the disease progresses, there may be evidence of right ventricular
failure with elevated jugular venous pressure, lower extremity
edema, and ascites.

5. Clinical Findings
• Cardiovascular examination may reveal
A. Elevated JVP
B. Hypertension
C. Accentuated P2 component of the second heart sound,
D. Right-sided S3 or S4
E. Mid diastolic murmur of MS
F. Ejection systolic murmur at pulmonary area.
G. Holosystolic tricuspid regurgitant murmur.

8. • It is also important to seek signs of the diseases that are commonly
concurrent with PH.
• Clubbing may be seen in some chronic lung diseases (DPLD)
• Crepitations in lung field.
• Air entry
• Sclerodactyly, telangiectasia may signify scleroderma (or the limited
cutaneous form, CREST [calcinosis, Raynaud’s phenomenon,
esophageal dysmotility, sclerodactyly, and telangiectasia]),

9. Investigation
• ECG may show right ventricular hypertrophy or strain.
• CXR may reveal enlargement of pulmonary arteries and obliteration
of the retrosternal space.
• Echocardiography with agitated saline (bubble) study is the most
important initial screening test.
• Elevated estimated pulmonary artery systolic pressure (>35 mmHg)
or a hypertrophied or dilated right ventricle support the diagnosis of
PH.

10. • Important additional information can be gleaned about specific
etiologies of PH, such as
1. valvular disease,
2. left ventricular systolic and diastolic function,
3. left atrial enlargement, and
4. intracardiac shunt.

11. ECG
• Diagnostic criteria
• Right axis deviation of +110° or more.
• Dominant R wave in V1 (> 7mm tall or R/S ratio > 1).
• Dominant S wave in V5 or V6 (> 7mm deep or R/S ratio < 1).
• QRS duration < 120ms (i.e. changes not due to RBBB).

12. • Supporting criteria
• Right atrial enlargement (P pulmonale).
• Right ventricular strain pattern = ST depression / T wave inversion in
the right precordial (V1-4) and inferior (II, III, aVF) leads.
• S1 S2 S3 pattern = far right axis deviation with dominant S waves in
leads I, II and III.
• Deep S waves in the lateral leads (I, aVL, V5-V6).

17. Additional tests focusing on functional
capacity
• 6-minute walk distance (6-MWD) assessment, which also aids in
assessing prognosis.
• Cardiopulmonary exercise testing (CPET) differentiates between
cardiac and pulmonary causes of dyspnea and includes measuring
peak volume of oxygen consumption, which is an integrated
parameter of cardiopulmonary fitness and also useful in
prognosticating PH.
• Invasive hemodynamic monitoring with right heart catheterization
(RHC) is the gold standard for PH diagnosis and severity assessment.

19. • PULMONARY FUNCTION AND LUNG IMAGING
a. In PAH, an isolated reduction in diffusing capacity of the lungs for
carbon monoxide (DLCO) is a classic finding.
b. HRCT- Enlargement of the main pulmonary artery, right ventricle,
and atria, as well as peripheral pruning of small vessels.
c. CT is also critical for distinguishing co-morbid interstitial lung
disease, emphysema, or overlap syndromes that include fibrosis
and obstructive pulmonary disease.

20. • SLEEP STUDIES
1. Nocturnal desaturation is a common finding in PH even in the
absence of sleep-disordered breathing.
2. Thus, all patients should undergo nocturnal oximetry screening,
regardless of whether classic symptoms of obstructive sleep apnea
or obesity-hypoventilation syndrome are present.
• ASSESSMENT OF PULMONARY ARTERIAL THROMBOSIS
• Serology for HIV screening.

22. Discussion
• Pulmonary hypertension (PH) is a heterogenous disease involving
pathogenic remodeling of the pulmonary vasculature, which
increases pulmonary artery pressure and vascular resistance.
• Pulmonary arterial hypertension (PAH) is an uncommon, but distinct,
PH subtype characterized by the interplay between molecular and
genetic events that cause an obliterative arteriopathy and symptoms
of dyspnea, chest pain, and syncope.
• If left untreated, PH carries a high mortality rate, largely owing to
decompensated right heart failure.

23. Pathophysiology
• Pulmonary circulation is a low resistance, high compliance circuit.
During exercise it gets engorged and can accommodate 3times its
blood volume( high compliance)
• Ohm’s Law V=IR
• Pulmonary circulation: mPAP= (COXPVR) + LAP
• Systemic circulation: mean Aortic Pressure= (COXSVR) + RAP
• Normally LVEDP= LAP= PCWP
• As because SVR>>PVR, Mean Aortic Pressure is much more than
mPAP.

24. • In PAH the pathologic lesions involve mainly the distal pulmonary
arteries (< 500 μm in diameter).
• Characterized by
1. Medial hypertrophy, intimal proliferative and fibrotic changes
(concentric, eccentric)
2. Adventitial thickening
3. Moderate perivascular inflammatory infiltrates
4. Complex lesions (plexiform, dilated lesions)
5. Thrombotic lesions

25. • Upregulation of Endothelin pathway : vasoconstriction and
antiproliferation.
• Downregulation of NO pathway : inhibition of cGMP
• Downregulation of Prostacycline pathway : PGI2 / cAMP/
Prostacycline inhibited.

27. Classification
• Type 1 PAH
• Type 2 Secondary to left heart disease. ( Increased LAP/ PCWP)
aka PVH.
• Type 3 Secondary to Lung disease and hypoxia.
• Type 4 Chronic Thrombo Embolic PH
• Type 5 Miscellaneous.

28. Hemodynamic Definition
• Normal values
I. mPAP <20 mmHg
II. PVR <3 Wood’s unit
III. LAP/ PCWP <15 mmHg
PH mPAP PVR LAP/PCWP
Type 1 >20 >3 <15
Type 3 ,, ,, ,,
Type 4 ,, ,, ,,
Type 2 >20 <3 <15

29. Type 1/ WHO group 1
• PAH
• Idiopathic PAH is the MC variety. Young Female.
• Heritable- BMPR2 gene mutation ( 70% with germline mutation), ALK
1
• Drug and Toxin induced
a) Dasatinib (TKI)
b) Anorexigens ( Fen-Phen, Aminorex)
c) Methamphetamine

30. • Disease associated-
a) CTD – MC Scleroderma ( CREST/ Limited Cutaneous), SLE
b) Congenital Heart Disease- ASD, VSD, PDA with Eissenmenger
Syndrome.
c) Portal Hypertension ( Splanchnic blood bypasses liver)
d) HIV
e) Schistosomiasis

31. Pulmonary Hypertension Associated with Left Heart
Disease
• Patients with PH due to left ventricular systolic dysfunction, aortic
and mitral valve disease, and heart failure with preserved ejection
fraction (HFpEF) are classified in WHO group 2.
• The hallmark is elevated LAP with resulting pulmonary venous
hypertension.

32. Pulmonary Hypertension Associated with
Lung Disease
• COPD, DPLD
• PH is also diagnosed in diseases of mixed obstructive/restrictive
pathophysiology: bronchiectasis, cystic fibrosis, mixed obstructive-
restrictive disease marked by fibrosis in the lower lung zones, and
emphysema predominantly in the upper lung zones.

33. Management
• General measures
1) Salt restriction <2.4 gm/d
2) Immunization for respiratory illness – Influenza and pneumonia
vaccine.
3) Minimize valsalva maneuver (cough, constipation, heavy lifting). It
can increase the risk of syncope.
4) Avoid pregnancy, as there is 30-50% maternal mortality and 15%
fetal mortality.

34. • Medical Management
i. PAH specific therapy.
ii. Oxygen: to prevent hypoxemia. As because Pulmonary circulation
reacts to hypoxia by vasoconstriction.
iii. Digoxine- shown to increase RV contraction.
iv. Anti coagulants in CTEPH
v. Diuretics ( judicious use)
• Surgical Management Atrial septostomy , Lung transplant.

35. • PAH specific therapy
• Endothelin receptor antagonists – Bosentan, Ambrisentan
• Guanyle Cyclase Stimulant Riociguat
• PDE 5 inhibitor sildenafil, Tadalafil
• Prostanoid analogue Epoprostenol, Iloprost,
• CCBs