This document discusses pulmonary hypertension (PH), defined as a mean pulmonary arterial pressure over 25 mmHg. PH is classified into 5 groups based on etiology. Common causes include left heart disease (group 2) and idiopathic pulmonary arterial hypertension (group 1). Symptoms are nonspecific but relate to right ventricular dysfunction. Diagnosis requires right heart catheterization to confirm PH and assess severity. Evaluation includes echocardiogram, CT, V/Q scan, PFTs, and labs. Prognosis correlates with functional class and exercise capacity.

1. PULMONARY HYPERTENSION
(PART 1)
DISCUSSED BY :
DR KUNWAR SIDHARTH SAURABH
REFERENCE –
ERS GUIDELINES 2018

2. DEFINITION
• PULMONARY HYPERTENSION (PH) IS DEFINED AS “MEAN”
PULMONARY ARTERIAL PRESSURE > 25 mmHg, AT REST “ASSESED BY
RIGHT HEART CATHETERISATION.”
• NORMAL PAPm – 14 + 3, UPPER LIMIT OF 20mmHg.
• PAPm of 21- 24  falls in grey zone.
• Nothing as PH on exercise.

4. ANY THING RELATED TO LEFT
HEART IS POST CAPILLARY

5. CLINICAL CLASSIFIACTION
• DIVIDED INTO 5 GROUPS AS PER THE ETIOLOGY .
• GROUP 1,3,4,5 FALLS IN PRECAPILLARY GROUP
• GROUP 2 & 5 FALLS IN POSTCAPILLARY GROUP.
• GROUP 2 IS THE MOST COMMON CAUSE

8. HYPOXIA

14. WHO FUNCTIONAL CLASS

16. GENETICS
• M/C MUTATION IS IN BMPR2- 75% FAMILIAL CASES
• BMPR1B, SMAD9,CAV1, KCNK 3 , EIF2AK4 ARE SOME OTHERS
• CONSANGUINOUS MARRIAGE INCRESES CHANCE OF HERITABLE PAH,
SHOWING ITS RECESSIVE PATTERN OF TRANSMISSION.

17. CLINICAL PRESENTATION
• Symptoms are non specific
• Usually related to Right Ventricular dysfunction.
• Shortness of breath, fatigue, weakness , angina, syncope.
• Dry cough, exercise induced vomiting.
• At rest symptoms occurs only in advanced cases showing signs of RHF.

18. Symptoms due to Mechanical complications
• Hemoptysis – rupture of hypertrophied bronchial artery.
• Hoarseness – pulmonary artery dilation compressing left recurrent
laryngeal nerve.
• Wheeze – caused by large airway compression.
• Angina – due to compression of Left Main coronary artery.

19. PHYSICAL SIGNS
• LEFT PARASTERNAL SHIFT
• ACCENTUATED PULMONARY COMPONENT OF S2
• RV THIRD HEART SOUND
• PANSYSTOLIC MURMUR OF TR
• DIASTOLIC MURMUR OF PR.
• SIGNS OF RHF
• WHEEZE AND CRACKLES ARE USUALLY ABSENT ( EXCEPT SOME
CASES , USUALLY GROUP 2)

20. ECG

21. CXR
PRUNNING

22. PFT & ABG
• CAN IDENTIFY UNDERLYING PARENCHYMAL LUNG DISEASE.
• DECREASED “DLCO” IN MOST PATIENTS DEFINED AS <45% OF
PREDICTED ASSOCIATED WITH POOR OUTCOME.
• COPD IS A CAUSE OF HYPOXIC PH.
• THE PREVELANCE OF NOCTURNAL HYPOXAEMIA AND CENTRAL SLEEP
APNOEA ARE HIGH IN PAH.

23. ECHO
• Transthoracic echocardiography is used to image the effects of PH on
the heart and estimate PAP from continuous wave Doppler
measurements.
• Echocardiography should always be performed when PH is suspected
and may be used to infer a diagnosis of PH in patients in whom
multiple different echocardiographic measurements are consistent
with this diagnosis.

24. • The estimation of systolic PAP is based on the peak tricuspid
regurgitation velocity (TRV) taking into account right atrial pressure
(RAP) as described by the simplified Bernoulli equation.
• RAP can be estimated by echocardiography based on the diameter
and respiratory variation in diameter of the inferior vena cava (IVC):
an IVC diameter <2.1 cm that collapses >50% with a sniff suggests a
normal RA pressure of 3mmHg (range 0–5 mmHg), whereas an IVC
diameter >2.1 cm that collapses <50% with a sniff or <20% on quiet
inspiration suggests a high RA pressure of 15 mmHg (10-20)

25. • When peak TRV is technically difficult to measure (trivial or mild
tricuspid regurgitation) some laboratories use contrast
echocardiography [e.g. agitated saline administered by intravenous
(i.v.) injection], which may improve the Doppler signal, allowing
measurement of peak TRV velocity.
• In patients with severe tricuspid regurgitation, TRV may be
significantly underestimated and cannot be used to exclude PH.
• Overestimation may also occur. PH cannot be reliably defined by a
cut-off value of TRV.

29. V/Q SCAN
METHOD OF CHOICE IN CTEPH

30. NOTE
• ALTHOUG CT IS DONE MORE COMMONLY, IT IS NOT EVIDENCE
BASED AS V/Q SCAN REMAINS THE GOLD STANDARD IN
DIAGNOSING CAUSES OF PH.

31. HRCT
• CT may raise a suspicion of PH in symptomatic patients or those
examined for unrelated indications by showing an increased PA
diameter (⩾29 mm) and pulmonary:ascending aorta diameter ratio
(⩾1.0). A segmental artery:bronchus ratio >1 : 1 in three or four lobes
has been reported to have high specificity for PH
• Contrast CT angiography is helpful in evaluating the surgical
accessibility.

32. Cardiac MR imaging
• CMR imaging is accurate and reproducible in the assessment of RV
size, morphology and function and allows non-invasive assessment of
blood flow, including stroke volume, CO, pulmonary arterial
distensibility and RV mass.
• Also suitable in pregnancy and patients with contrast allergy.

33. Blood Tests
• Not useful in diagnosis but routines are required.
• General routine blood check up
• Thyroid profile is MUST
• Liver enzymes may be raised in patients with endothelin receptor
antagonist therapy.
• NT-proBNP.
• Tests for connective tissue diseases,

34. USG abdomen
• To rule out PORTAL HYPERTENSION.

35. RIGHT HEART CATHETERISATION
• RHC is required to confirm the diagnosis of PAH and CTEPH, to assess
the severity of haemodynamic impairment and to undertake
vasoreactivity testing of the pulmonary circulation in selected
patients.
• The threshold to perform left heart catheterization in addition to RHC
should be low in patients with clinical risk factors for coronary artery
disease
• All pressure measurements should be determined at the end of
normal expiration (breath holding is not required).

36. • Inhaled nitric oxide (NO) at 10–20 parts per million (ppm) is the
standard of care for vasoreactivity testing, but i.v. epoprostenol, i.v.
adenosine or inhaled iloprost can be used as alternatives .
• A positive acute response is defined as a reduction of the mean PAP
⩾10 mmHg to reach an absolute value of mean PAP ⩽40 mmHg with
an increased or unchanged CO.
• The use of CCBs, O2, phosphodiesterase type 5 inhibitors or other
vasodilators for acute vasoreactivity testing is discouraged.

43. EVALUATION OF SEVERITY
• WHO FUNCTIONAL CLASS IS ONE OF THE MOST POWERFUL
PREDICTOR OF SURVIVAL.
• EXERCISE CAPACITY – 6MWT, CPET.
• NO SPECIFIC BIOCHEMICAL MARKER – NT-proBNP LEVELS CO-RELATE
WITH MYOCARDIAL DYSFUNCTION .