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Exercise Induced PAH in a Patient with Compensated Cardiac Disease Background: Hemodynamic and Functional Response to Sildenafil Therapy

L. Nikolaidis

N. Memon

B. O'Murchu

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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 06.22.2012

Presentation Type: Abstracts

Nikolaidis L, Memon N, O'Murchu B,

Temple University, Philadelphia, PA, USA

BACKGROUND: Exercise-induced PAH (EI-PAH) is a relatively rare form in the spectrum of PAH. Although few reports suggest response to either PDE-5 or ETA therapies, neither of these approaches is currently FDA approved or backed by solid clinical trial data. Further, EI-PAH in the setting of concomitant cardiac disease –although well compensated- is even less well defined and unclear as far as therapeutic options.

METHODS: We describe a 54 year old man who complained of worsening exertional dyspnea and fatigue for over 2 years, consistent with NYHA III symptoms. He had history of a bioprosthetic AVR and compensated mild LV dysfunction. His ECHO showed LVEF 45-50 % and normal prosthetic valve gradients and function. Physical exam and extensive biochemical profile were normal. Multiple cardiac and pulmonary investigations, including ECHO, TEE, left and right heart catheterization (RHC), cardiac MRI, nuclear stress testing, as well as high resolution CT of chest, PFT and sleep study were unremarkable. Because the patient described dyspnea only with exertion, he underwent a cardiopulmonary exercise test (CPET) as well as a RHC combined with symptom-limited, bedside bicycle ergometry.

RESULTS: The findings demonstrated EI-PAH, in addition to his compensated left sided heart disease. Based upon these data, the patient was treated with sildenafil 20 mg TID for 2 years. This resulted in improvement of his symptoms, hemodynamics and functional class, as evidenced in the table.

 

BASELINE

1 year on PDE-5

2 years on PDE-5

RHC

REST

EXERCISE

REST

EXERCISE

REST

EXERCISE

PASP

22

70

24

40

15

48

PADP

6

20

5

9

4

12

mPAP

13

46

14

23

9

27

PCWP

6

9

10

11

5

7

SvO2

69

38

78

60

75

80

CO

4.5

3.8

5.8

3.5

4.8

6.5

TPG

7

37

4

12

5

20

PVR

1.3

9

1.7

5.5

1.5

3.4

CPET

VO2 max

13.7

18.5

17.5

Ve/VCO2

41.5

33.5

30.5

RQ

1.2

1.11

1.07

CONCLUSIONS: We conclude that EI-PAH may indeed coexist with other cardiac conditions and should be investigated as a potential source of otherwise unexplained exertional symptoms out of proportion to the cardiac dysfunction. Our patient responded favorably to sildenafil therapy