This document summarizes updates on pulmonary hypertension (PH) in three conditions: end stage renal disease (ESRD), sickle cell disease (SCD), and myeloproliferative neoplasms (MPD). For ESRD, PH is very heterogeneous, with some cases resembling group 1 PH and others resembling postcapillary PH due to left heart dysfunction. Ligation of arteriovenous fistulas in kidney transplant recipients resulted in reduced left ventricular mass. For SCD, PH can develop from multiple factors including anemia, nitric oxide depletion, and hypoxemia; prevalence is around 6-10%. PH management in SCD is controversial. For MPD, one study found different characteristics of PH between SCD sub

1. Updates in Group
5 PH:
ESRD, SCD, MPD
Barbara LeVarge MD
University of North Carolina

2. Outline
• Chronic kidney disease (focus on ESRD)
• Sickle cell disease
• Myeloproliferative neoplasms
Not covered: other hemolytic anemias, PLCH, Gaucher disease, glycogen
storage disease, neurofibromatosis, fibrosing mediastinitis, segmental
pulmonary hypertension, complex congenital heart disease

3. PH in end stage renal disease
• Very heterogeneous
• “Group 1-like” insults  PH with high
PVR (precapillary PH)
• Left heart dysfunction  PH with high
PAWP (postcapillary PH)
• High output contributions
• (True group 1 PAH with related/unrelated
ESRD)
https://www.coreimpodcast.com/2022/04/06/pulmonary-hypertension/

4. PH in end stage renal disease
mPAP = PAWP + CO x PVR
wedge pressure
 LV systolic
dysfunction
 LV diastolic
dysfunction
 Valvular disease
 High output failure
 Volume regulation
cardiac output
 AV fistula/graft
 Anemia
pulmonary vascular
resistance
 Uremic endothelial
dysfunction
 Thromboemboli
 Microbubbles
 Abnormal Ca/phos
metabolism
 Inflammation
Other comorbidities
(DM, HTN, OSA, CTD, HIV)

5. PH in end stage renal disease
• Very heterogeneous
• “Group 1-like” insults  PH with high PVR (precapillary PH)
• Left heart dysfunction  PH with high PAWP (postcapillary PH)
• High output contributions
• (True group 1 PAH with related/unrelated ESRD)
• Much of relevant literature treats CKD-PH as a single disease
• Much of relevant literature uses echocardiography only

6. PH in end stage renal disease
• How common?
• Meta-analysis median prevalence in ESRD: 38% (Schoenberg et al, Lung 2020)
• Twice as common in hemodialysis versus peritoneal dialysis
• Greater than 800,000 in US with ESRD, greater than 30 million with CKD
• Few RHC based studies supporting majority (75+%) postcapillary
• Mortality risk with ESRD-PH vs ESRD-no PH: RR 2.02
• ESRD-PH undergoing renal transplant with higher risk of poor outcomes
(mortality, delayed graft function, graft dysfunction/failure)

7. PH in end stage renal disease
• Updates in 2023

8. Composite
outcome
Mortality

9. • N=54, >1 year post transplant
with stable renal function
• Randomized to fistula ligation or
no intervention
• At 6 months, AVF ligation group
with significant decrease in LV
mass, LV volumes, atrial
volumes, proBNP
Editorial, see p 2819
BACKGROUND: Cardiovascular morbidity and mortality remain high in
recipients of a kidney transplant. The persistence of a patent arteriovenous
fistula (AVF) after transplantation may contribute to ongoing maladaptive
cardiovascular remodeling. The ability to reverse this maladaptive remodeling
by ligation of this AVFis unknown. We conducted the first randomized
controlled trial to evaluate the ef fect of AVFligation on cardiac structure and
function in stable kidney transplant r ecipients.
METHODS: In this randomized controlled trial, kidney transplant recipients
(>12 monthsafter transplantation with stable graft function) wer e
randomized to AVFligation or no intervention. All participants underwent
cardiac magnetic resonance imaging at baseline and at 6 months. The primary
outcome wasthe change in left ventricular (L
V) mass. Secondary outcomes
included changes in L
V volumes, left and right atrial areas, L
V ejection fraction,
NT
-proBNP(N-terminal pro-B-type natriuretic peptide) levels, cardiac output/
index, brachial flows(ipsilateral to AVF), and pulmonary artery velocity.
RESULTS: A total of 93 patients were screened, of whom 64 met the
inclusion criteria and were randomized to the AVFligation (n=33) or
control (n=31) group. Fifty-four participants completed the study: 27 in
the AVFligation group and 27 in the control group. On the second cardiac
magnetic resonance scan, a mean decrease of 22.1 g (95% CI, 15.0–29.1)
was observed in LV mass in the AVFligation group compared with a small
increase of 1.2 g (95% CI, −4.8 to 7.2) in the control group (P<0.001).
Significant decreases in LV end-diastolic volumes, LV end-systolic volumes,
cardiac output, cardiac index, atrial volumes, and NT-proBNPwere also seen
in the AVFclosure group (P<0.01). No significant changes wer e observed
in LV ejection fraction (P=0.93) and pulmonary artery velocity (P=0.07).
No significant complications were noted after AVFligation. No changes in
estimated glomerular filtration rate or systolic and diastolic blood pr essures
were observed between cardiac magnetic resonance scans.
CONCLUSIONS: Elective ligation of patent AVFin adults with stable kidney
Nitesh N. Rao, MBBS,
MPhil
Mic
hael B
. S
tokes
, MB
B
S
A
dil R
ajwani, MB
C
hB
, P
hD
S
hahidU
llah, P
hD
K
erryW
illiam
s
D
avidK
ing, MB
B
S
E
wanMac
aulay, MB
C
hB
C
hris
tineH
.R
us
s
ell,MB
C
hB
S
antos
hO
lakkengil, MB
B
S
R
obert P
. C
arroll, MBC
hB
,
P
hD
R
andall J
. F
aull, MB
B
S
,
P
hD
K
arenS
.L
. T
eo, MB
B
S
, P
hD
S
tephenP
. Mc
D
onald,
MB
B
S
, P
hD
*
MatthewI. Worthley
,
MB
B
S
, P
hD
*
P
. T
obyC
oates
, MB
B
S
,
P
hD
*
O
RIG
INAL RESEARCHARTICLE
Effects of Arteriovenous Fistula Ligation
on Cardiac Structure and Function in
Kidney Transplant Recipients
Circulation
* Drs McDonald, Worthley, and Coates
contributed equally.
Key Words: arteriovenous fistula
heart ventricles kidney
Downloaded
from
http://ahajournals.org
by
on
November
12,
2023
C
irculation. 2019;139:2809–2818. DO
I: 10.1161/C
IR
C
U
LATIO
NAHA.118.038505 June18/25, 2019 2809
Editorial, see p 2819
BACKGROUND: Cardiovascular morbidity and mortality remain high in
recipients of a kidney transplant. The persistence of a patent arteriovenous
fistula (AVF) after transplantation may contribute to ongoing maladaptive
cardiovascular remodeling. The ability to reverse this maladaptive remodeling
by ligation of this AVFis unknown. We conducted the first randomized
controlled trial to evaluate the ef fect of AVFligation on cardiac structure and
function in stable kidney transplant r ecipients.
METHODS: In this randomized controlled trial, kidney transplant recipients
(>12 months after transplantation with stable graft function) wer e
randomized to AVFligation or no intervention. All participants underwent
cardiac magnetic resonance imaging at baseline and at 6 months. The primary
outcome was the change in left ventricular (L
V) mass. Secondary outcomes
included changes in L
V volumes, left and right atrial areas, L
V ejection fraction,
NT
-proBNP(N-terminal pro-B-type natriuretic peptide) levels, cardiac output/
index, brachial flows (ipsilateral to AVF), and pulmonary artery velocity.
RESULTS: A total of 93 patients were screened, of whom 64 met the
inclusion criteria and were randomized to the AVFligation (n=33) or
control (n=31) group. Fifty-four participants completed the study: 27 in
the AVFligation group and 27 in the control group. On the second cardiac
magnetic resonance scan, a mean decrease of 22.1 g (95% CI, 15.0–29.1)
was observed in LV mass in the AVFligation group compared with a small
increase of 1.2 g (95% CI, −4.8 to 7.2) in the control group (P<0.001).
Significant decreases in LV end-diastolic volumes, LV end-systolic volumes,
cardiac output, cardiac index, atrial volumes, and NT-proBNPwere also seen
in the AVFclosure group (P<0.01). No significant changes wer e observed
in LV ejection fraction (P=0.93) and pulmonary artery velocity (P=0.07).
No significant complications were noted after AVFligation. No changes in
estimated glomerular filtration rate or systolic and diastolic blood pr essures
were observed between cardiac magnetic resonance scans.
CONCLUSIONS: Elective ligation of patent AVFin adults with stable kidney
transplant function resulted in clinically significant reduction of LV myocardial
mass.
CLINICAL TRIAL REGISTRATION: Australian and New Zealand Clinical
T
rials Registry URL: https://www.anzctr.org.au. Unique Identifier:
ACTRN12613001302741.
© 2019 American Heart Association, Inc.
Nitesh N. Rao, MBBS,
MPhil
Mic
hael B
. S
tokes
, MB
B
S
A
dil R
ajwani, MB
C
hB
, P
hD
S
hahidU
llah, P
hD
K
erryW
illiam
s
D
avidK
ing, MB
B
S
E
wanMac
aulay, MB
C
hB
C
hris
tineH
. R
us
s
ell, MB
C
hB
S
antos
hO
lakkengil, MB
B
S
R
obert P
. C
arroll, MBC
hB
,
P
hD
R
andall J
. F
aull, MB
B
S
,
P
hD
K
arenS
.L
. T
eo, MB
B
S
, P
hD
S
tephenP
. Mc
D
onald,
MB
B
S
, P
hD
*
Matthew I. Worthley
,
MB
B
S
, P
hD
*
P
. T
obyC
oates
, MB
B
S
,
P
hD
*
https://www.ahajournals.org/journal/circ
* Drs McDonald, Worthley, and Coates
contributed equally.
Key Words: arteriovenous fistula
heart ventricles kidney
transplantation magnetic resonance
imaging
Sources of Funding, see page 2817
Downloaded
from
http://ahajournals.org
by
on
November
12,
2023
C
irculation. 2019;139:2809–2818. DO
I: 10.1161/C
IR
C
U
LA
TIO
NAH
A.118.038505 June18/25, 2019 2809
CONCLUSIONS: Elective ligation of patent AVFin adultswith stable kidney
transplant function resulted in clinically significant reduction of LV myocardial
mass.
CLINICALTRIALREGISTRATION: Australian and New Zealand Clinical
T
rialsRegistry URL: https://www.anzctr.org.au. Unique Identifier:
ACTRN12613001302741.
© 2019 American Heart Association, Inc.
https://www.ahajournals.org/journal/circ
Key Words: arteriovenousfistula
heart ventricles kidney
transplantation magnetic resonance
imaging
Sourcesof Funding, see page 2817

10. • N=71 with PH (PASP >35 by echo)
started on sac/val, compared to
N=51 on ARB, 3 month followup
• Single center, retrospective
• 51% sac/val group with AVF
• Greater reduction in PASP with
sac/val than ARB (-15 vs -8)
• Hyperkalemia, hypotension similar,
no discontinuations
• N=23, HFrEF, all started on sac/val,
median followup 132 days
• Single center, retrospective
• Improvement in LVEF (3041%),
hsTnT, sST2
• 17% symptomatic hypotension, no
discontinuations

11. • N=16, ESRD on HD (88% AVF)
denied transplant due to PH
• Had RHC, then admitted for daily
HD/UF to push dry weight as low
as possible, then repeat RHC
• Improved mPAP, PAWP, dry
weight, but not PASP by echo
• 14/16 deemed eligible for
transplant, 11 transplanted with
100% survival at 1 year, mean
serum creatinine 1.3.

12. PH in sickle cell disease
• SCD affects
100,000 people
in US.
• Single point
mutation in beta
globin gene
• A disease of
hemolysis and
vaso-occlusion
Gladwin MT, Vichinsky E. N Engl J Med 2008;359:2254-2265.

13. PH in sickle cell disease
mPAP = PAWP + CO x PVR
wedge pressure
 Myocardial fibrosis
 LV diastolic dysfunction
 Iron overload
 High output heart failure
 Sleep disordered breathing
 CKD from SCD
cardiac output
 Anemia
 Liver disease
pulmonary vascular
resistance
 ↓ Nitric oxide
 ↑ Endothelin-1
 Hypoxemia
 Hypercoagulability
 CTEPH
 Portal hypertension

14. PH in sickle cell disease
• Less than half of PH by echo (TRV 2.5 m/s) end up with mPAP ≥25
• Prevalence (of mPAP ≥25) of 6-10% when RHC performed
• Invasive studies support roughly half is postcapillary PH and half
precapillary PH (and for precapillary PH, PVR often < 3 WU).
• Anemia  lower blood viscosity  PVR is lower in SCD.
• Recommendation for PVR ≥ 2 WU to be considered abnormal in SCD
• Clear signal of higher mortality in SCD-PH.

15. PH in sickle cell disease
• Management
• Investigation for other causes of PH (CTEPH, liver disease, iron overload,
OSA/CSA, etc)
• Hydroxyurea, or exchange transfusions
• Anticoagulation, PTE/BPA for CTEPH
• PAH-specific therapy: controversial
• Consider for precapillary hemodynamics (mPAP ≥25, PCWP ≤15, PVR ≥2 WU)
• walk-PHaSSt (sildenafil RCT): increased risk of hospitalization for VOC

16. PH in sickle cell disease
Am J Respir Crit Care Med. 2014 Mar 15; 189(6): 727–740

17. PH in sickle cell disease
• Updates in 2023

18. • N=58 patients with SCD (41 SS, 2 SB0, 14 SC) with
precapillary PH
• SS/SB0 with higher hemolysis markers/lower Hgb,
lower FVC, lower 6MWD vs SC
• SS/SB0 with similar mPAP, higher cardiac index, lower
PVR vs SC
• SC with 85% prevalence of segmental defects on VQ
scan (vs 9% SS/SB0)

19. • N=25, SS/SB0, median age 19, mean Hgb
9.7, half adults/teens with TRV >2.5 m/s.
• Prospective longitudinal study using CMR
• Myocardial extracellular volume (ECV), a
measure of collagen deposition, significantly
higher in SCD vs controls and other fibrotic
cardiac conditions.
• Increase in ECV associated with diastolic
dysfunction
RED CELLS, IRON, AND ERYTHROPOIESIS
Association between diffusemyocardial brosisand diastolic dysfunction
in sickle cell anemia
Omar Niss,1
Robert Fleck,2
Fowe Makue,3
Tarek Alsaied,3
Payal Desai,4
Jeffrey A. Towbin,5
Punam Malik,1,6,
*
Michael D. Taylor,3,
* and Charles T. Quinn1,
*
1
Division of Hematology, 2
Department of Radiology, and 3
Division of Cardiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; 4
Department
of Internal Medicine, The Ohio State University, Columbus, OH; 5
The Heart Institute, Le Bonheur Children’s Hospital, Memphis, TN; and 6
Division of
Experimental Hematology and Cancer Biology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Key Points
• Diffuse myocardial fibrosis
is a common and novel
mechanism of heart disease
in SCA that can be detected
noninvasively.
• Diffuse myocardial fibrosis
is strongly associated with
diastolic dysfunction in
individuals with SCA.
Sickle cell anemia (SCA)–related cardiomyopathy is characterized by diastolic dysfunc-
tion and hyperdynamic features. Diastolic dysfunction portends early mortality in SCA.
Diastolic dysfunction is associated with microscopic myocardial fibrosis in SCA mice,
but the cause of diastolic dysfunction in humans with SCA is unknown. We used cardiac
magnetic resonance measurements of extracellular volume fraction (ECV) to discover and
quantify diffuse myocardial fibrosis in 25 individuals with SCA (mean age, 23 6 13 years) and
determinetheassociation between diffuse myocardial fibrosis and diastolic dysfunction.ECV
was calculated from pre– and post–gadolinium T1 measurements of blood and myocardium,
and diastolic function was assessed by echocardiography. ECV was markedly increased in all
participants compared with controls (0.44 6 0.08 vs 0.26 6 0.02, P < .0001), indicating the
presenceof diffusemyocardial fibrosis. Seventeen patients (71%)had diastolic abnormalities,
and 7 patients (29%) met the definition of diastolic dysfunction. Participants with diastolic
dysfunction had higher ECV (0.49 6 0.07 vs 0.37 6 0.04, P 5 .01) and N-terminal pro–brain
natriuretic peptide(NT-proBNP; 1916 261 vs 33 6 33pg/mL, P5 .04) but lower hemoglobin (8.4 6 0.3 vs 10.9 6 1.4g/dL, P5 .004) compared
with participants with normal diastolic function. Participants with the highest ECV values (‡0.40) were more likely to have diastolic
dysfunction (P5 .003)and increased left atrial volume(576 11vs 466 12mL/m2
,P5 .04)compared with thosewith ECV<0.4.ECVcorrelated
with hemoglobin (r 5 2 0.46, P 5 .03) and NT-proBNP (r 5 0.62, P 5 .001). In conclusion, diffuse myocardial fibrosis, determined by ECV, is a
common and previously underappreciated featureof SCA that is associated with diastolic dysfunction,anemia,and high NT-proBNP.Diffuse
myocardial fibrosis is a novel mechanism that appears to underlie diastolic dysfunction in SCA. (Blood. 2017;130(2):205-213)
Downloaded
from
http://ashpublications.org/blood/article-pdf/130/2/205/14042
with participants with normal diastolic function. Participants with the highest ECV values (‡0.40) were more likely to have diastolic
dysfunction(P5 .003)andincreased left atrial volume(576 11vs466 12mL/m2
,P5 .04)comparedwiththosewith ECV<0.4.ECVcorrelated
with hemoglobin (r 5 2 0.46,P5 .03)and NT-proBNP(r 5 0.62,P5 .001). In conclusion,diffusemyocardial fibrosis,determined by ECV,is a
commonandpreviouslyunderappreciatedfeatureofSCAthat isassociatedwithdiastolicdysfunction,anemia,andhighNT-proBNP.Diffuse
myocardial fibrosis is a novel mechanism that appears to underlie diastolic dysfunction in SCA. (Blood. 2017;130(2):205-213)
Introduction
Cardiacdiseaseisaleadingcauseof adult mortality andmorbidity in
sickle cell anemia (SCA).1
Diastolic dysfunction, pulmonary hyper-
tension(PH), andincreasedlevelsof N-terminal pro–brainnatriuretic
peptide(NT-proBNP), amarker of myocardial wall stressandcardiac
dysfunction, are all associated with early mortality in SCA.2-4
We
recentlyreportedthatcardiacdysfunctioninSCA canbeexplainedbya
cardiomyopathy with features of both restrictive and hyperdynamic
physiology,5,6
characterized by diastolic dysfunction, left atrial (LA)
enlargement,andnormal systolicfunction(restrictivephysiology)sup-
erimposed on left ventricular (LV) dilation and hypertrophy (hyper-
dynamic physiology). Like other restrictive cardiomyopathies, the
cardiomyopathyofSCAmayleadtoPHandelevatedtricuspidregurgitant
jet velocity (TRV) and predisposestodysrhythmiasand sudden cardiac
death,all of whicharerecognizedbutunexplainedcomplicationsof SCA.
The etiology of diastolic dysfunction underlying the restrictive
physiology of SCA-related cardiomyopathy isunknown. Myocardial
brosis is known to cause diastolic dysfunction and restrictive
physiology in non-SCA populations.7
We found that diastolic
dysfunctioninSCA micewasassociatedwithmicroscopicmyocardial
brosis, abnormal electrophysiology, and transcriptome changes.6
WhetherthissamepathologyoccursinhumanswithSCA isunknown.
Postmortemstudieshaveshownavaryingextentof myocardial brosis
in SCA.8,9
In addition, cardiac magnetic resonance (CMR) studies
using late gadolinium enhancement (LGE) have identi ed focal
myocardial brosisinafewSCA patients.8,10-12
LGEimagingdetects
dense, focal brosis based on differences in gadolinium volume of
distribution between diseased and normal myocardium.13
Therefore,
LGE is insensitive to diffuse myocardial brosis that is seen in
nonischemic cardiomyopathies and some SCA autopsy studies.8,13
Recently,CMRmeasurementof myocardial T1relaxationtimesbefore
and after gadolinium administration has been used to quantify the
myocardial extracellular volumefraction (ECV).13
In theabsenceof

20. • Review of pathology from 30 autopsies involving SCD patients, 1994-2012
• 76% with hypertensive arterial changes
• 80% with recent thrombosis
• 43% with chronic thrombosis
• 87% with PCH-like capillary changes
• 23% venous thickening
• 37% bronchial hypervascularization
German R. Carstens,1,2
Bianca B. A.
Paulino,1
Edgard H. Katayama,1
Luis F. Amato-Lourenc
ßo,1
Guilherme H. Fonseca,3
Rogerio Souza,4
Vera D. Aiello5
and
Thais Mauad1
1
Department of Pathology, Faculdade de Medic-
ina, Universidade de S~
ao Paulo, S
~
ao Paulo, SP,
Brazil, 2
Hospital Eduardo Sch€
utz Schroeder,
Puerto Montt,Chile, 3
Department of Haematol-
ogy, Hospital das Clınicas HCFMUSP, Facul-
dade de Medicina, Universidade de S
~
ao Paulo,
4
Pulmonary Department, Heart Institute
(InCor), Hospital das Clınicas HCFMUSP,
Faculdade de Medicina, Universidade de S
~
ao
Paulo, and 5
Laboratory of Pathology, Heart
Institute (InCor), Hospital das Clınicas
HCFMUSP, Faculdade de Medicina, Universi-
dade de S~
ao Paulo, S~
ao Paulo, SP, Brazil
Received 10 September 2018; accepted for
publication 14 December 2018
Correspondence: Dr. Thais Mauad, Associate
Professor, Department of Pathology, Faculdade
de Medicina, Universidade de S
~
ao Paulo,
Avenida Dr. Arnaldo, 455, room 1155. CEP:
01246-903 S
~
ao Paulo, SP, Brazil.
E-mail: tmauad@
usp.br
Summary
Pulmonary complications are frequen
(SCD), but few studies have described
the lung tissue of 30 deceased SCD
genotype, clinical characteristics, caus
are presented. We quantified the pre
thrombosis and venous thickening. A
demonstrated using CD34 expression
and echocardiography reports were rev
Tissue expression of markers of endo
sion molecule 1, intercellular adhesion
growth factor) was quantified in p
33 years; genotype was SS in 19, SC
males. Hypertensive arterial changes w
recent thrombosis in 80% and old th
was present in 23% and pulmonary
87%. Ten percent of the patients pre
There was no increased expression of
compared to controls. SCD affects the
reflects the multiple burden on lung
upon time.
Keywords: sickle cell, lung, patholo
giomatosis, thrombosis.
Sickle cell disease (SCD) is a well-characterised monogenic
hereditary disorder that results in the synthesis of the abnor-
is associated with a
mortality in older p
botic disease, pulmonary hypertension (PH), sleep disordered
breathing and asthma/recurrent wheezing (Mehari & Klings,
2016). PH in SCD has gained a lot of research attention as it
functionally to pre-capillary PH (Sakao & Tatsumi, 2013).
Restrictive cardiomyopathy with diastolic dysfunction has
been described in patients with SCD, which, in addition to
ª 2019 British Society for Haematology and John Wiley & Sons Ltd
British Journal of Haematology, 2019, 185, 317–326
First published online 10 February 2019
doi: 10.1111/bjh.15795

21. PH in myeloproliferative neoplasms
• Clonal expansion of hematopoietic progenitor cell, differentiation
preserved  mature myeloid cells
• Polycythemia vera, essential thrombocytosis, primary myelofibrosis,
chronic myeloid leukemia
• More emphasis on precapillary disease
• Chronic thromboembolism (CTEPH)
• PAH-like arteriopathy
• TKI-related (dasatinib and others)  group 1 PAH
• Portal hypertension (extramedullary hematopoiesis)
• PVOD or arterial dominant disease
https://imagebank.hematology.org/reference-case/16/essential-thrombocythemia

22. PH in myeloproliferative neoplasms
• Prevalence of MPN in US approximately 300,000
• Reports of prevalence of PH in MPN range 4-58% (recent meta-analysis
with 35% by echo, 7% by RHC)
• Management
• Treatment of underlying MPD (phlebotomy, aspirin, cytoreduction/JAK inhibitor)
• Anticoagulation, PTE/BPA for CTEPH
• PAH specific therapy?

23. PH in myeloproliferative neoplasms
• Updates in 2023

24. • N=90, largest MPN-PH cohort to date.
• 50% CTEPH (dominant PV/ET), 50% group 5
precapillary PH (PMF, PV/ET)
• 5 year survival: 42% CTEPH, 34% group 5 PH

25. Summary
• ESRD/SCD/MPN: presence of PH is a poor prognostic factor.
• ESRD-PH: postcapillary component in majority, assess AVFs (banding,
?ligation), BP/volume removal, treat other comorbidities, optimize as
possible prior to transplant
• SCD-PH: pre- or postcapillary, assess for CTEPH in precapillary
(especially Hgb SC), hydroxyurea or exchanges, need more research
• MPN-PH: assess for CTEPH (especially PV/ET early in disease)

26. Questions?