Management of Pulmonary Hypertension in Critical care settings

1. Pulmonary Hypertension
Dr Zainab

2. Objectives
• General Definition
• Grades
• Clinical Presentation
• Workup
• Diagnotic Algorithm
• Treatment & Management

3. Definition
• Pulmonary hypertension, defined as a mean pulmonary arterial
pressure greater than 20 mm Hg at rest or greater than 30 mm Hg
during exercise, is often characterized by a progressive and sustained
increase in pulmonary vascular resistance that eventually may lead to
right ventricular failure.

4. GRADES of Pulmonary Hypertension
• The World Health Organization (WHO) has divided pulmonary
hypertension into five groups on the basis of similarities in
pathophysiology, clinical presentation, and therapeutic options

6. Clinical Presentation

8. Differential Diagnosis
• Aortic stenosis
• Atrial myxoma
• Atrial Septal Defect
• Chronic Obstructive Pulmonary Disease (COPD)
• Chronic Pulmonary Embolism
• Dilated Cardiomyopathy
• Emphysema
• Hepatopulmonary Syndrome
• Hypertrophic Cardiomyopathy
• Interstitial Lung Disease
• Mitral Regurgitation
• Mitral Stenosis
• Obesity-Hypoventilation Syndrome
• Obstructive Sleep Apnea (OSA)
• Restrictive Cardiomyopathy
• Restrictive Lung Disease
• Ventricular Septal Defects

9. Workup
1. LAB TESTS:
• CBC, S/E, RFTs, Uric acid, LFTs, PT/APTT, Iron status and NT-proBNP.
• Collagen-vascular disease screening can be performed by measuring
antinuclear antibody (ANA) levels, as well as rheumatoid factor (RF)
and antineutrophil cytoplasmic antibody (ANCA). When there is
clinical suspicion for scleroderma, anti-Scl-70, anticentromere, and
anti-U1-RNP antibodies can also be checked. Biological markers of
Antiphospholipid syndrome.
• HIV, Thyroid function tests

10. Cardiac Workup
2. ECG:

11. 3. Echocardiography

12. Cardiac Workup
4. Cardiac Magnetic Resonance Imaging
5. Cardiopulmonary exercise testing: to assess the underlying
pathophysiological causing exercise intolerance.

13. Respiratory Workup:
6. Chest X-ray

14. Workup(Cont..)
7. Pulmonary function tests and ABGs:
• These tests may show an obstructive pattern suggestive of COPD or a
restrictive pattern suggestive of an interstitial lung disease.
• DLCO may be normal in patients with PAH.
• A severely reduced DLCO(less than 45% predicted) with normal PFTs
can be found in PAH with SSc, PVOD, and in PH with group 3.
• PaO2 may be normal or slighlty reduced and PaCO2 typically lower
than normal because of alveolar hyperventilation.

15. 8. Ventilation-Perfusion Lung Scan:
• It is recommended to rule out or detect signs of CTEPH in suspected
or newly diagnosed PH.
9. Non-Contrast and Contrast CT chest:
• CT-chest: Enlarged PA diameter(>30mm), RVOT wall thickness (>6mm)
and septal deviation greater than 140°(or RV:LV ratio >1).
• CT-PA to detect direct and indirect signs of CTEPH.
• Dual energy CT (DECT)
• Digital Subtraction Angiography (DSA)

16. 9. Abdominal Ultrasound
10. Right heart Catheterization: gold standard for diagnosing and
classifying pulmonary hypertension.
11. Vasoreactivity testing
12. Fluid Challenge
13. Genetic Counselling and testing

18. Diagnosis

19. Severity and Risk Assessment
1. Clinical Parameters
2. Imaging of heart (Echo, Cardiac MRI)
3. Hemodynamics
4. Exercise capacity
5. Biochemical markers

21. Pathophysiology and Therapeutic targets

22. Treatment

23. Management of PH in ICU settings
1. Decision Regarding PH medications in ICU:
• Home PAH medicines should be continued beacuse they reduce PVR.
• Abrupt cessation or dose reduction can lead to rapid dexompensation
& death.
• Three available classes of medications usually used for PAH:
a. Pulmonary Vasodilators-prostacyclin analogues(Esoprostenol-drug
of choice in PH with RV failure)
b. Phosphodiestrase type 5 antagonists(sildenafil,taldenafil)
c. Endothelin antagonists(Bosentan-for opd basis treatment, not used
in icu settings)
d. Inhaled nitric oxide

24. 2. Fluid Management:
• Mericulous volume management to optimize cardiac filling pressures.
• Elevated RA/RV pressures lead to systemic venous congestion and
cause malperfusion and injury to other organs like liver, kidney and
intestines.
• Intravenous diuretics and hemofiltration markedly improve
hemodynamics. Digoxin for atrial tachyarythmias and anticoagulants
for thrombotic arteriopathy in idiopathic PH.

25. 3. Hemodynamic support with Vasopressors and Inotropes:
• Goal is to restore cardiac output and maintaining systemic blood pressures
while reducing PVR.
• Our goal is to maintain: SVR>PVR, MAP>mPAP and systolic BP>systolic PAP,
with cardiac index higher than 2 Litres per min per metre square.
• Dobutamine and milrinone can be used to reduce SVR and PVR, while
increasing cardiac output in RV failure.
• Milrinone may reduce SVR more than PVR and may cause systemic
hypotension, so a vasopressor like norepinephrine can be used to tackle it
as norepinephrine doesn’t reduce PVR but improves RV-PA coupling.
• Phenylephrine should be avoided as it increases PVR.

27. 4. Mechanical Circulatory Support:
• ECMO can be used as bridge to recovery and bridge to
transplantation.
• Should only be used in patients with realistic plan of lung transplant
or recovery.

28. 5. Respiratory Support:
• Effect of positive pressure ventillation include a reduction in venous
return, reduction in RV preload and increased RV afterload from
compression of pulmonary vasculature.
• High PEEP can worsen hypoxia and hypercapnia
• Intubation and mechanical ventillation in patients in patients with PH
and RV failure can cause acute changes in RV preload and afterload
along with systemic vasodilation and hypotension that lead to tapid
decompensation and death.

29. Relationship b/w alveolar volume/pressure
and PVR.

30. 6. Balloon Atrial setptostomy
7. Lung Transplantation