1. Approximately how many Portopulmonary Hypertension patients have been managed at your center over the last five years?
a. 0-10 b. 10-25 c. 25-50 d. 50-75 e. 75-100
f. more than 100
2. How many patients with Portopulmonary Hypertension do you estimate you manage annually?
a. Less than 5 b. 5-10 c. 10-15 d. 15-20
e. more than 20
3. Of those patients, how many Portopulmonary hypertension patients are currently under your care and being evaluated for liver transplantation?
4. What percentage do you feel like will successfully undergo liver transplantation? (Text Box) 5. Branching Logic: Where do you see patients?
a. If United States – Continue with Survey
b. If Country other than United States – Save Response, Exit Survey 6. Branching Logic: What is your Professional Degree
a. If MD/DO – Continue with Survey
b. If Other degree – Save Response, Exit Survey 7. What is your Age?
a. 20-30 b. 31-40 c. 41-50 d. 51-60 e. 61-70 f. 71+
g. I don’t wish to disclose 8. What is your Specialty?
a. Cardiology b. Pulmonology c. Hepatology d. Other
9. Branching Logic: Display this question if “Other” is selected for Specialty Please write your Specialty (Text Box)
10. How many years of practice have you been caring for patients with Pulmonary Arterial Hypertension? (Text Box)
a. Less than 5 years b. 5-10 years c. 10-20 years
d. More than 20 years
11. Branching Logic: Do you manage Portopulmonary Hypertension after Liver Transplantation?
a. If Yes – Continue with Survey b. If No – Save Response, Exit Survey
12. In your practice, how many patients with Portopulmonary Hypertension undergo liver transplantation annually
a. Less than 5 b. 5-10 c. 10-15 d. 15-20
e. more than 20
13. What UNOS region is your center located in?
Region 1 (Connecticut, Maine, Massachusetts, New Hampshire, Rhode Island, Eastern Vermont)
Region 2 (Delaware, District of Columbia, Maryland, New Jersey, Pennsylvania, West Virginia, Northern Virginia)
Region 3 (Alabama, Arkansas, Florida, Georgia, Louisiana, Mississippi, Puerto Rico)
Region 4 (Oklahoma, Texas)
Region 5 (Arizona, California, Nevada, New Mexico, Utah)
Region 6 (Alaska, Hawaii, Idaho, Montana, Oregon, Washington)
Region 7 (Illinois, Minnesota, North Dakota, South Dakota, Wisconsin)
Region 8 (Colorado, Iowa, Kansas, Missouri, Nebraska, Wyoming)
Region 9 (New York, Western Vermont)
Region 10 (Indiana, Michigan, Ohio)
Region 11 (Kentucky, North Carolina, South Carolina, Tennessee, Virginia)
14. What types of Pulmonary Hypertension Targeted Therapy are used at your center? (Check all that Apply)
a. Oral Phosphodiesterase-5 Inhibitors (sildenafil, tadalafil) b. Oral soluble Guanylate Cyclase Activators (riociguat)
c. Oral Endothelin Receptor Antagonists (Macitentan, Bosentan, Ambrisentan) d. Oral prostacyclin agents (treprostinil, selexipag)
e. Inhaled prostacyclin agents (“Tyvaso”, “Ventavis”)
f. Parenteral Prostacyclin agents (IV epoprostenol, IV Treprostinil, Sub-Cutaneous Treprostinil)
- Branching Logic: Display this question if “Parenteral Prostacyclin” is selected:
1. Preferred Route of Administration IV
2. Preferred Route of administration Sub-Cutaneous
15. How frequently are the following Pulmonary Hypertension Targeted Therapies used?
a. Oral Phosphodiesterase-5 Inhibitors (sildenafil, tadalafil) - 0-25%
- 25-50%
- 50-75%
- 75-100%
b. Oral soluble Guanylate Cyclase Activators (riociguat) - 0-25%
- 25-50%
- 50-75%
- 75-100%
c. Oral Endothelin Receptor Antagonists (Macitentan, Bosentan, Ambrisentan) - 0-25%
- 25-50%
- 50-75%
- 75-100%
d. Oral prostacyclin agents (treprostinil, selexipag) - 0-25%
- 25-50%
- 50-75%
- 75-100%
e. Inhaled prostacyclin agents (“Tyvaso”, “Ventavis”) - 0-25%
- 25-50%
- 50-75%
- 75-100%
f. Parenteral Prostacyclin agents (IV epoprostenol, IV Treprostinil, Sub-Cutaneous Treprostinil)
- 0-25%
- 25-50%
- 50-75%
- 75-100%
16. Do you use Echocardiogram after Liver Transplantation to Guide Adjustment of Targeted Pulmonary Hypertension Therapy in PoPH patients? (Yes/No)
17. Branching Logic: If Yes, display this question:
If Yes, how frequently do you perform echocardiography following liver transplant in PoPH?
every 1-2 months
every 3 months
every 4-5 months
every 6 months
every 7-12 months
Other frequency (please explain) (Text Box)
18. Do you use right heart catheterization after liver transplantation to guide adjustment of targeted pulmonary hypertension therapy in PoPH patients (Yes/No)
19. Branching Logic: If Yes, display this question:
If Yes, how frequently do you perform right heart catheterization following liver transplant in PoPH?
every 1-2 months
every 3 months
every 4-5 months
every 6 months
every 7-12 months
Other Frequency (please explain) (Text Box)
20. Do you use 6-minute walk testing after liver transplantation to guide adjustment of targeted pulmonary hypertension therapy in PoPH patients (Yes/No)
21. Branching Logic: If Yes, display this question:
If Yes, how frequently do you perform 6-minute walk testing following liver transplant in PoPH?
every 1-2 months
every 3 months
every 4-5 months
every 6 months
every 7-12 months
Other Frequency (please explain) (Text Box)
22. Do you use Natiuretic Peptide Levels (BNP, NTproBNP) after liver transplantation to guide adjustment of targeted pulmonary hypertension therapy in PoPH patients (Yes/No) 23. Branching Logic: If Yes, display this question:
If Yes, how frequently do you perform test for BNP/NTproBNP following liver transplant in PoPH?
every 1-2 months
every 3 months
every 4-5 months
every 6 months
every 7-12 months
Other Frequency (please explain) (Text Box)
24. Do you use any other testing modalities after liver transplantation to guide adjustment of targeted pulmonary hypertension therapy in PoPH patients (Yes/No)
25. Branching Logic: If Yes, display this question:
If Yes, please describe the modality and frequency you use (Text Box) 26. Do you routinely collect research samples from your PoPH patients (Yes/No) 27. Branching Logic: If Yes, display this question:
If Yes, please describe the type of sample and frequency with which it is collected (Text Box) 28. How frequently do you adjust PAH therapy in PoPH patients following Liver Transplantation?
I never adjust PAH therapy after Liver Transplantation in PoPH
<10% of the time
10-25% of the time
25-50% of the time
50-75% of the time
I always adjust PAH therapy after Liver Transplantation in PoPH
29. How soon after Liver Transplantation to you wait before adjusting PAH therapy?
Immediately after transplantation
within 1-2 months after transplantation
after 3 months after transplantation
after 4-5 months after transplantation
after 6 months after transplantation
after 7-12 months after transplantation
Other Frequency (please explain) (Text Box)
30. Which PAH therapy modality do you adjust first (Check all that apply)
a. Oral Phosphodiesterase-5 Inhibitors (sildenafil, tadalafil) b. Oral soluble Guanylate Cyclase Activators (riociguat)
c. Oral Endothelin Receptor Antagonists (Macitentan, Bosentan, Ambrisentan) d. Oral prostacyclin agents (treprostinil, selexipag)
e. Inhaled prostacyclin agents (“Tyvaso”, “Ventavis”)
f. Parenteral Prostacyclin agents (IV epoprostenol, IV Treprostinil, Sub-Cutaneous Treprostinil)
- Branching Logic: Display this question if “Parenteral Prostacyclin” is selected:
1. I titrate the dose down by ~25% and re-assess 2. I titrate the dosedown by ~50% and re-assess 3. I titrate the dose down by ~75% and re-assess
4. I titrate medicine off completely (100%) and re-assess 5. Other titration (please explain) (Text Box)
- Branching Logic: Display this question if “Parenteral Prostacyclin” is selected:
1. I titrate the dose down over 1 month and re-assess 2. I titrate the dose down over 1-2 months and re-assess 3. I titrate the dose down over 3 months and re-assess 4. I titrate the dose down over 3-6 months and re-assess 5. Other timeframe for titration (please explain) (Text Box) 31. Which is the last PAH therapy modality you adjust (Check all that apply)
a. Oral Phosphodiesterase-5 Inhibitors (sildenafil, tadalafil) b. Oral soluble Guanylate Cyclase Activators (riociguat)
c. Oral Endothelin Receptor Antagonists (Macitentan, Bosentan, Ambrisentan) d. Oral prostacyclin agents (treprostinil, selexipag)
e. Inhaled prostacyclin agents (“Tyvaso”, “Ventavis”)
f. Parenteral Prostacyclin agents (IV epoprostenol, IV Treprostinil, Sub-Cutaneous Treprostinil)
- Branching Logic: Display this question if “Parenteral Prostacyclin” is selected:
1. I titrate the dose down by ~25% and re-assess 2. I titrate the dosedown by ~50% and re-assess 3. I titrate the dose down by ~75% and re-assess
4. I titrate medicine off completely (100%) and re-assess 5. Other titration (please explain) (Text Box)
- Branching Logic: Display this question if “Parenteral Prostacyclin” is selected:
1. I titrate the dose down over 1 month and re-assess 2. I titrate the dose down over 1-2 months and re-assess 3. I titrate the dose down over 3 months and re-assess 4. I titrate the dose down over 4-6 months and re-assess 5. Other timeframe for titration (please explain) (Text Box)
32. On Average, what length of time does it take to fully discontinue oral/inhaled PoPH therapies after liver transplantation in your patients?
a. 1 month b. 2 months c. 3 months
d. 4-6 months e. 7 months – 1 year f. Greater than 1 year
g. Other (please explain) (Text Box)
33. On Average, what length of time does it take to fully discontinue parenteral prostacyclin therapies after liver transplantation in your patients?
a. 1 month b. 2 months c. 3 months d. 4-6 months e. 7 months – 1 year f. Greater than 1 year
g. Other (please explain) (Text Box)
34. Are there any other comments you would like to share regarding the management of PoPH patients following liver transplantation? (Text Box)
