Pulmonary Hypertension, short presentation.

1. Pulmonary Hypertension
Dr. Saurabh Bhardwaj
MBBS, MD(Gen Med), DrNB (Cardiology)

2. Definition
‘ a syndrome characterized by marked remodeling of the pulmonary
vasculature and a progressive rise in the pulmonary vascular load,
leading to hypertrophy and remodeling of the right ventricle.’
Pulmonary hypertension is currently defined hemodynamically by a
mean pulmonary arterial pressure of higher than 20 mm Hg at rest, as
measured by right heart catheterization

3. Classification- 6th WSPH, France- 2018

6. History and Symptoms
• Symptoms are nonspecific- exertional dyspnea, fatigue, chest pain, and fluid
retention, as well as syncope in advanced cases.
• Presence of underlying disease such as HIV infection or liver or connective-tissue
disease or a history of exposure to drugs or toxins should heighten suspicion
concerning PH.
• A major diagnostic challenge is to rule out other forms of PH for which
management should focus principally on the underlying disease, so risk factors for
or symptoms of left-sided heart disease or chronic lung disease are important to
consider

7. Physical findings include
• an increased second pulmonic sound,
• a murmur of tricuspid regurgitation, and
• evidence of right ventricular fluid overload (e.g., increased jugular venous
pressure and pedal edema).
• Other findings might suggest an underlying cause of PH, including sequelae of
chronic liver or rheumatologic disorders.

8. Evaluation
• ECG
• Chest X ray
• Echocardiogram
• Laboratory studies
• Ventilation –Perfusion Lung Scintigraphy
• Pulmonary Function tests
• Cardiac MRI
• Overnight Oximetry
• Functional Assesment
• Right Heart Catheterization

9. ECG
• P- pulmonale secondary to right atrial enlargement,
• Right axis deviation,
• Right ventricular enlargement, often with a strain pattern
• Common accompanying arrhythmias include atrial fibrillation, atrial flutter, and
multifocal atrial tachycardia in addition to premature atrial and ventricular
contractions

10. Chest X ray
• enlarged main and hilar pulmonary artery shadows.
• “pruning” or attenuation of the peripheral vasculature and
• right ventricular enlargement, which is best appreciated on the lateral view.
• Other findings may point to an associated diagnosis, such as hyperinflation with flat diaphragms
(COPD) or pulmonary venous congestion (left-sided heart disease)

11. 2D ECHO
Common echocardiographic features of PH include
• right atrial enlargement,
• right ventricular enlargement and dysfunction,
• small underfilled left-sided heart chambers,
• interventricular septal flattening,
• tricuspid regurgitation with elevated velocity,
• reduced tricuspid annular plane systolic excursion (TAPSE).
• left ventricular systolic or diastolic dysfunction alongwith aortic and mitral
valvular heart disease are easily assessed on echocardiogram.

13. Right Heart Catheterization
• Oxygen saturation (superior and inferior vena cavas, pulmonary and systemic
arteries)
• Right atrial pressure
• Right ventricular pressure
• Pulmonary artery pressure
• Left-sided filling pressure (PAWP, left atrial pressure, or left ventricular end-
diastolic pressure [LVEDP])
• CO/cardiac index
• PVR
• Systemic blood pressure
• Heart rate
• Response to acute vasodilators (inhaled NO, iv epoprostenol)

14. Management

15. Treatment
1. Calcium channel blockers
2. Prostanoids
3. Endothelin receptor antagonists
4. Phosphodiesterase inhibitor 5
5. Soluble Guanylate Cyclase Stimulator

16. Thank you.
• References
• Braunwald’s Heart Disease, A Textbook of Cardiovscular Medicine, 11e
• ESC 2022 Guidelines, Pulmonary Hypertension
• NEJM 2021; 385:2361-2376, Pulmonary Hypertension