This document discusses a case study of a patient with complex cardiopulmonary issues. The patient presented with COPD exacerbation and was found to have pulmonary hypertension. Initial right heart catheterization found severely elevated pulmonary pressures and pulmonary vascular resistance. After treatment with diuresis and initiation of CPAP, a second catheterization found improved pressures. The document analyzes whether the patient's condition represents pulmonary arterial hypertension alone or a combination of pre-capillary and post-capillary pulmonary hypertension based on the various comorbidities and hemodynamic data. It also discusses challenges in categorizing patients who do not neatly fit classification criteria.

1. PH in the Real World
Case Studies
Terry Fortin MD
Duke Pulmonary Vascular Disease Center
November 4, 2022

2. • Mean PAP > 20 mmHg
1
• Wedge < 15 mmHg
2
• PVR > 3 WU (or less)
3
Definition of PAH Clinical, Hemodynamic
6th World Symposium 2018
6th World Symposium on PH 2018

3. Groups of PH (Framework)
 1. Pulmonary Arterial Hypertension (PAH)
 2. PH owing to left heart disease
 3. PH owing to lung diseases and/or
hypoxia
 4. Chronic thromboembolic pulmonary
hypertension (CTEPH)
 5. PH with unclear multifactorial mechanisms
 (ESRD, Cancer, Hemoglobinopathy, Sarcoid…

4. Real World : PH or PAH ?
54 yo sickle cell/thallasemia Hgb 7-8
Rheumatoid arthritis SLE overlap/MCTD
CKD on hemodialysis (high output)
History Mitral Valve replacement for remote
endocarditis
Morbid obesity, (BMI>50) OSA on CPAP
LV EF is 45%, HTN, DM, TIA, CAD
Pulmonary embolus x 2 but VQ neg
PFDs with moderate restrictive/ obstructive
lung disease.
Later develops cirrhosis

5. WCM How did we get from A
to B. What if B came first?
 RH Cath 4/22
 RA 22
 RV 110/20
 PA 104/45 mean 68
 PCWP 28
 Output 7 L/min
 Index 3.2
 PVR 5.7 WU
 ABG 7.25/90/107
 Hgb 17.9
 RH Cath 7/22
 RA 8
 RV 50/10
 PA 52/29 mean 34
 Wedge 14
 output 5.2 L/min
 Index 2.5
 PVR 3.9

6. WCM 4/2022
 65 yo presents to OSH with “COPD flare and NSTEMI”
 EMS called nonresponsive, 02 sat 60% Placed on BiPAP
 History COPD since 2017, Flare 1-2 times per year . Not on 02
 At OSH ABG 7.1/88/124 on 02
 Elevated troponins, LFTs, AKI, pBNP 1700, Hgb 18
 CT ruled out PE, No pneumonia
 History of sleep apnea x years but never used machine
 On inhalers for COPD No PFDs
 On transfer hypercarbic and hypoxic respiratory failure.
 Diuresis, on /off bipap, ABX and Steroids

7. WCM

11. WCM Right and Left Heart Cath 4/2022
Off BIPAP on FI02 30%
 RA 22
 RV 110/20
 PA 104/45 mean 68
 PCWP 28
 AO 129/80 mean 96
 CO 7 L/min CI 3.2
 PVR 5.7 WU No Step up to suggest Shunt
 7.25/90/107 Hgb 17.9 Sat 95%
 Placed back on BIPAP post case
 Two vessel CAD TIMI 3 Flow 80% mid LCx,
 Tandom 80% RCA proximal and mid lesions
 Negative volume about 3 liters+ by this time

12. Pulmonary Vascular Resistance (PVR) increases
with
hypoxemia,
acidosis,
hypercarbia,
increased catacholamines or sympathetic tone
Illness , inflammation,
Thrombosis
 Very high or low lung volumes increase PVR
Activation of neurohormones negatively impacts
function of RV as well
Relevant principles

13. Treatment???
 Diuresis
 Give pulmonary vasodilator (inhaled prostacyclin)
 1 and then 2
 Diuresis, initiate CPAP/BIPAP/Trilogy
 IV Prostacyclin
 Dual oral therapy with endothelin antagonist and PDE 5
inhibitor
 Repeat Cath before Discharge?
 More Work up
 CT with No PE, ANA normal, HIV normal, Some ongoing
work up for liver disease
 Had stents to his RCA and LCx

14. My Plan
 Diuresis
 Sent home with Trilogy Machine
 02 for exertion Per PT assessment
 Shock Liver resolved. AKI resolved
 VBG before Discharge 7.43 pC02 48 post using trilogy in
hospital
 Follow up with Pulmonologist / Sleep
 Seen back in my clinic in 10 Days
 Decreased diuretic, pBNP much improved
 Feeling best he has been in years
 Plan for repeat Right heart cath after on his Sleep machine
for 2 to 3 months as long as he is well

15. WCM 1st clinic visit
 PFDs Group 3 Disease
 FVC 3.33 L 76%
 FEV1 1.28 L 38%
 FEV1/FVC 38
 FEF25-7% 14%
 TLC 110%
 RV 178%
 DLCO 13.72 52%
 Walk is 393 meters (76% predicated)room air

16. WCM RH Cath 7/2022
 RA 8, PA 52/29 mean 34, Wedge 14
 Cardiac output is 5.2L/min with index of 2.4
 PVR is 3.9 WU, SVR 13 with mean BP of 78
 TPG 20, DPG 15
 pBNP is 148 ( down from 1700)
 Previous cath pre trilogy and diuresis
 RA 22, Mean PAP 68, wedge 28 (group 2)

17.  Passive or Post Capillary pressures cause congestion of
venous bed and passive PH
 Increased PA pressure with high wedge normal PVR
 Can then have reversible vasoconsttriction
 TPG < 12 and /or DPD or DPG <7
Reactive or precapillary PH physiologic/anatomic remodeling of
arterioles to compensate or protect upstream pulmonary
venous changes
 PVR>3, TPG >12, DPD or DPG >7
 Early may be reversible and more Chronic irreversible
 Combined pre and post capillary PH
Group 2 Pulmonary HTN

18. WCM Sep 2022
 Normalization of p BNP pBNP 143 ( < 225 normal)
 ANA negative, Alpha -1 Antitrypsin normal
 Negative VQ
 Bicarb decreased from 35 to 27
 Work up for Liver Disease /Steatohepatitis
 Completed cardiopulmonary Rehab
 No Hospitalizations or COPD flares since 4/22
 Compliant with CPAP
 Set up for echo next visit

19. WCM
 What Group At least Group 2 and 3 ?1
 Pre and post capillary PH Initially more Pre
 TPG 68-28 =44
 DPG 45-24 = 11
 But high wedge 28
 Does it matter that his pH was 7.25, and pC02 90?
 Group 3
 Sleep issues
 COPD ( Would this be different if ILD)
 VQ was low probability

20. Patients do NOT always follow the
rules
 Cpc PH Combined Pre and post capillary PH
 Group 3 and Group 2 risk factors/ disease
 IS PAH really PAH?
 Relative Severity of each component or factor
 Would we think about this differently if the lung
disease was ILD and not COPD.
 If the 2nd cath was first cath
 If initial wedge was 20 and not 30

21. Does the Data Make Sense
and is Data accurate
 Timing of the data
 During hospitalization with some concomitant illness
 Rapid diuresis and wedge pressure looks OK
 Initial CXR suggests otherwise
 Re equilibrate
 Inconsistencies of data
 Echo looks bad Cardiac output is good
 CT or other testing suggest other disease process