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Chronic Thromboembolic
Pulmonary Hypertension (CTEPH):
Myths Regarding CTEPH and Its Management
To learn more, go to CTEPH.com
CTEPH Definition
C
hronic thromboembolic pulmonary
hypertension, or CTEPH, is defined
as mean pulmonary arterial pressure
(mPAP) of at least 25 mmHg and
pulmonary capillary wedge pressure
(PCWP) of no more than 15 mmHg in the
presence of multiple chronic or organized
occlusive thrombi or emboli in the elastic
pulmonary arteries (the main, lobar,
segmental, subsegmental) after at least
3 months of effective anticoagulation.
Defining CTEPH
After 3 months of therapeutic
anticoagulation
•m
PAP ≥25 mmHg
•P
CWP ≤15 mmHg
•C
hronic/organized occlusive
thrombi/emboli
CTEPH is a complex and misunderstood
disease, and numerous myths about
CTEPH and its treatment persist. Here,
we present evidence that should help
dispel some commonly held myths
about the diagnosis of CTEPH and about
pulmonary thromboendarterectomy (PTE)
surgery, the only potentially curative
treatment for CTEPH.
Diagnosis
• A prospective follow-up study by Pengo et al of 314 consecutive patients
MYTH:
If a patient recovers from an
acute pulmonary embolism,
she won’t go on to develop
CTEPH.
E
vidence: While the likelihood of
developing CTEPH after an acute
pulmonary embolism (PE) is small, it is not
negligible. CTEPH is a rare complication
of a common disease.
found that 3.8% eventually developed CTEPH within 2 years of their first
symptomatic episode of acute PE
•There are several factors that increase
the risk that CTEPH will eventually
develop subsequent to an acute PE.
These include:
–Large perfusion defects
–Recurrent or idiopathic PE
–Young or old age
–Persistent PH 6 months after the
embolism has occurred
–Systolic PAP >50 mmHg at initial
manifestation of PE
•Recognizing this risk, the ACCF/AHA
2009 expert consensus document
on pulmonary hypertension (PH)
recommends that a ventilation/perfusion
(V/Q) scan be performed in patients
who show symptoms of PH 3 months
after having an acute PE
•Studies report that about a quarter to
as many as 63% of CTEPH patients
may present without a history of acute
PE or deep vein thrombosis
Pengo V et al. N Engl J Med. 2004;350:22572264. Piazza G, Goldhaber SZ. N Engl J Med.
2011;364:351-360. McLaughlin VV et al.
Circulation. 2009;119:2250-2294. Lang IM.
N Engl J Med. 2004;350:2236-2238. PepkeZaba J et al. Circulation. 2011;124:1973-1981.
To learn more about CTEPH, please visit CTEPH.com
Chronic Thromboembolic Pulmonary Hypertension (CTEPH):
Myths Regarding CTEPH and Its Management
E
MYTH:
The V/Q is unnecessary in
the evaluation of pulmonary
hypertension.
S
C
vidence: Multiple PH guidelines—
including those from the Fifth
World Symposium on Pulmonary
Hypertension (WSPH) CTEPH Task
Force—recommend that the V/Q scan
be considered an essential CTEPH
screening tool as part of the PH
diagnostic algorithm.
The diagram below communicates a
mnemonic, SCAR, developed by the
WSPH CTEPH Task Force. Along with
echocardiography, the V/Q scan provides
information that leads to a suspicion
of CTEPH, but it is only with right heart
catheterization (RHC) and pulmonary
angiography that a diagnosis of CTEPH
can be confirmed.
Suspect
• Echocardiogram
•V
/Q scan
Confirm
• Right heart catheterization
• Pulmonary angiogram (or CTPA, MRA)
A
R
•Computed tomographic pulmonary
angiography (CTPA) may be used to
provide complementary diagnostic
information, but it does not replace the
V/Q scan as a screening test
–In a retrospective study of 227
patients, Tunariu et al reported
CTPA had a sensitivity for
detecting CTEPH of 51%, while
the V/Q scan had a sensitivity
>96%
–The WSPH noted that use of
CTPA as a screening tool instead
of V/Q scanning, “may lead to
potential misdiagnosis of PAH and
underdiagnosis of CTEPH”
Assess Risk
• Hemodynamics
• Comorbidities
• Surgeon/CTEPH team experience
•The V/Q scan is the preferred and
recommended method of screening for
CTEPH
–Every patient being evaluated
for PH should undergo a V/Q scan
if CTEPH has not already been
excluded by a prior normal
V/Q scan
Galiè N et al. Eur Heart J. 2009;30:24932537. Kim NH et al. J Am Coll Cardiol. 2013;
62:D92-D99. Tunariu N et al. J Nucl Med.
2007;48:680-684.
V/Q scan showing perfusion defects.
Image courtesy of Dr Paul Forfia,
Right Heart Failure, and Pulmonary
Thromboendarterectomy Program
at Temple University Hospital,
Philadelphia.
To learn more about CTEPH, please visit CTEPH.com
Chronic Thromboembolic Pulmonary Hypertension (CTEPH):
Myths Regarding CTEPH and Its Management
Assessing Operability For PTE Surgery
•A recent study from the UK of a cohort
MYTH:
PTE surgery cannot be
safely performed in patients
who have more severe PH
or who are elderly, obese, or
otherwise very sick.
E
vidence: There are no absolute
contraindications to PTE surgery
based on severity of pulmonary
hypertension, age, body mass index,
or presence of comorbidities.
•Madani and colleagues from University
of California, San Diego (UCSD) reported
a series of 500 patients who had PTE
surgery and found that among the
patients who had preoperative pulmonary
vascular resistance (PVR) >1000 dynes,
which indicates comparatively severe
pulmonary hypertension, overall mortality
was 4.1% vs 1.6% in patients who had
preoperative PVR <1000
of 411 total patients (see table below),
including 103 who were aged 70 or
more years, showed that short-term
in-hospital mortality rates did not
significantly differ between the younger
and the older patients: 4.6% vs 7.8%
Berman M et al. Eur J Cardiothorac Surg.
2012;41:e154-160.
•A recent analysis of data collected
by researchers at UCSD evaluated
outcomes after PTE for CTEPH in
a cohort that included 120 patients
who were obese (body mass index
[BMI] >30) and 208 patients who were
not obese (BMI<30) demonstrated
no significant difference in mortality
(2.4% for obese vs 0.8% for nonobese
patients) or post-PTE hemodynamics
between the groups
Kerr KN et al. Am J Respir Crit Care Med.
2013;187:A3316.
•Patients who have underlying
cardiovascular disease may be able
to undergo corrective procedures—
eg, valve replacement or coronary
artery bypass grafting—at the same
time as PTE
•Those with hepatic or renal
insufficiency at baseline may have
complications during the perioperative
period, but in the long term, hepatic
and renal function may improve along
with the general improvements in
CTEPH following PTE surgery
Thistlethwaite PA. Ann Thorac Surg. 2001;72:
13-19. Marshall PS et al. Clin Chest Med.
2013;34:779-797.
Any major surgery may pose a greater
risk for older patients as compared
with younger patients. Similarly, obesity,
serious comorbidities, and severity of
PH certainly need to be considered
by the experienced CTEPH team
assessing a patient’s operability for PTE
surgery. However, none of these factors
automatically disqualifies patients from
potentially curative PTE surgery.
Madani M et al. Ann Thorac Surg. 2012:94:
97-103.
Comparable mortality rates in older and younger patients
(1/2006-3/2011)
Time Frame
<70 years, n=308
≥70 years, n=103
P
In hospital
4.6%
7.8%
.21
1 year
8.6%
14.1%
2 year
10.1%
15.9%
3 year
12.3%
15.9%
.07
To learn more about CTEPH, please visit CTEPH.com
Chronic Thromboembolic Pulmonary Hypertension (CTEPH):
Myths Regarding CTEPH and Its Management
PTE Surgery
MYTH:
PTE surgery is still
an experimental and
dangerous procedure.
E
vidence: PTE surgery is not
experimental. Rather, it’s a wellestablished, reproducible procedure that
represents the only potential cure for
patients who have CTEPH.
• The PTE surgical technique was
developed and first performed in 1970.
– The blue table documents the PTE
history of a single experienced
center. Mortality rates have
consistently decreased, such that
none of their last 260 PTEs, as
reported by Madani et al in 2012,
resulted in death
Jamieson SW et al. Ann Thorac Surg. 2003;
76:1457-1464. Madani MM et al. Ann Thorac
Surg. 2012;94:97-103.
• Since its inception over 4 decades ago,
the safety of PTE surgery has improved
markedly, such that experienced centers
have been able to achieve in-hospital
mortality rates of <5%
Jenkins DP et al. Eur Respir J. 2013;41:735742.
Mortality data post-PTE at a single experienced center
Timeline
N
1970-1990
200
1994-1998
500
1998-2002
500
1999-2006
1000
2006-2010
500
Last consecutive patients
260
up to 12/2010
– The green table shows mortality
there are about 300 PTE
• Today,
– At
the most experienced centers,
•
rates reported by 27 centers
stratified by the number of PTEs
performed each year, a measure
of surgical experience
96.5% survive to discharge
Mayer et al. J Thorac Cardiovasc Surg. 2011;
141:702-710.
Drug therapy can be used
in place of PTE surgery.
Registry data: 26 European, 1 Canadian center
(2/2007-1/2009, n=386 patients with PTE)
Average no. of PTE per year
In-hospital deaths
Deaths 1 year after PTE
1-10
E
11-50
>50
Combined
7.4%4.7%3.5% 4.7%
11.1%
is the standard and recommended
• PTE
treatment for CTEPH, and it is the only
treatment option that can potentially
cure CTEPH.
from the WSPH makes it
• Guidance
vidence: According to the WSPH
CTEPH Task Force, drug therapy is
surgeries performed each year in the
US, and the geographic distribution
of experienced centers spans the
continental United States
PTE
surgery provides lasting
symptomatic and hemodynamic
benefit to most patients who
undergo it
Mayer E. Eur Respir Rev. 2010;19:64-67.
no substitute for PTE in a CTEPH patient
who has operable disease.
MYTH:
Mortality
17%
8.8%
4.4%
5.2%
2.2%
0%
clear that all patients with CTEPH
should be assessed for operability,
and those who have operable disease
7.3%
6.0%
should be referred for PTE without
delay.
the role of “bridging” with
• Furthermore,
drug therapy in CTEPH has not been
sufficiently studied and should be
reserved for controlled investigation.
Kim NH et al. J Am Coll Cardiol.
2013;62:D92-D99.
To learn more about CTEPH, please visit CTEPH.com
This material was reviewed by Gustavo Heresi-Davila, MD, of Cleveland Clinic, Cleveland OH and
Ivan M. Robbins, MD, of Vanderbilt University Medical Center, Nashville TN.
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PP-400-US-1773. May 2015
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