ACR Appropriateness CriteriasPulmonary Hypertension
Kathleen Brown, MD,* Antonio J. Gutierrez, MD,* Tan-Lucien H. Mohammed, MD,w
Jacobo Kirsch, MD,z Jonathan H. Chung, MD,y Debra Sue Dyer, MD,y
Mark E. Ginsburg, MD,8 Darel E. Heitkamp, MD,z Jeffrey P. Kanne, MD,#
Ella A. Kazerooni, MD,** Loren H. Ketai, MD,ww J. Anthony Parker, MD, PhD,zz
James G. Ravenel, MD,yy Anthony G. Saleh, MD,88 Rakesh D. Shah, MD,zz
Robert M. Steiner, MD,## Robert D. Suh, MD,* and Expert Panel on Thoracic Imaging
Abstract: Pulmonary hypertension (PH) may be idiopathic or
related to a variety of diseases. The diagnosis, accurate assessment
of etiology and severity, prognosis, treatment response, and follow-
up of PH can be achieved using a diverse set of diagnostic
examinations. In this review, the role of imaging in the evaluation
of PH as suggested by the American College of Radiology
Appropriateness CriteriasExpert Panel on Thoracic Imaging has
been discussed. The American College of Radiology Appropriate-
ness Criteriasare evidence-based guidelines for specific clinical
conditions that are reviewed every 2 years by a multidisciplinary
expert panel. The development and review of the guidelines include
an extensive analysis of current medical literature from peer-
reviewed journals and the application of a well-established con-
sensus methodology (modified Delphi) to rate the appropriateness
of imaging and treatment procedures by the panel. In those
instances in which evidence is lacking or not definitive, expert
opinion may be used to recommend imaging or treatment.
Key Words: pulmonary hypertension, pulmonary arterial hyper-
tension, imaging, Appropriateness Criterias
(J Thorac Imaging 2013;28:W57–W60)
SUMMARY OF LITERATURE REVIEW
Pulmonary hypertension (PH) is a complex disorder
and may be idiopathic or related to a variety of diseases. In
pulmonary arterial hypertension (PAH), progressive nar-
rowing of the small pulmonary arteries and arterioles
results in increased pulmonary vascular resistance, which
may ultimately lead to right ventricular failure and death.
The first hemodynamic definition proposed in 1973 defined
PAH as an increase in main pulmonary arterial pressure
(PAP), with a mean PAP>25mm Hg at rest or > 30mm Hg
with exercise, in the presence of a pulmonary capillary
wedge pressure r15mm Hg.1Subsequently, a clinical
classification of PH was introduced with 5 major categories,
with similarities in pathophysiological mechanisms, clinical
presentations, and therapeutic options.2Most recently, the
Fourth World Symposium recommended a revised hemo-
dynamic definition of PH as a mean PAPZ25mm Hg
without inclusion of exercise criterion, in view of evidence
that the cutoff level of >30mm Hg did not clearly differ-
entiate PH from physiological response to exercise.3–5
The 5 categories of PH are as follows: group 1, PAH;
group10, pulmonary veno-occlusive
pulmonary capillary hemangiomatosis; group 2, PH asso-
ciated with left heart diseases; group 3, PH associated with
lung respiratory disease and/or hypoxia; group 4, PH due
to chronic thromboembolic disease; and group 5, PH with
an unclear and/or a multifactorial mechanism.6
The diagnosis, accurate assessment of etiology and
severity, prognosis, treatment response, and follow-up of
PH can be achieved using a diverse set of diagnostic
examinations in a reasonable manner that is tailored for
each specific patient,7with a goal of early detection and
accurate characterization so that appropriate medical or
surgical therapy can be instituted8(Table 1).
Overview of Imaging Modalities
Historical studies have demonstrated that the chest
radiograph is an appropriate tool for evaluating patients at
risk for PH and for the detection or confirmation of the
diagnosis of PH.9,10The chest radiograph may reveal evi-
dence of diffuse lung disease, such as interstitial lung disease
and emphysema, or evidence of pulmonary venous hyper-
tension. However, the chest radiograph is relatively insen-
sitive for detecting mild PH. In clinical practice, although
chest radiography is insensitive for the detection of mild to
moderate PH, it is nonetheless recommended in the initial
This article is a revised version of the American College of Radiology
Appropriateness CriteriasPulmonary Hypertension. Practitioners
are encouraged to refer to the complete version at www.
Reprinted with permission of the American College of Radiology.
From the *Ronald Reagan UCLA Medical Center, Los Angeles, CA;
wVirginia Mason Medical Center, Seattle, WA; zCleveland Clinic,
Weston, FL; yNational Jewish Health, Denver, CO; 8Department
of Surgery, Society of Thoracic Surgeons, Columbia University,
New York, NY; 88The American College of Chest Physicians, New
York Methodist Hospital, Brooklyn, NY; zzNorth Shore Uni-
versity Hospital, Manhasset, NY; zDepartment of Radiology,
Indiana University, Indianapolis, IN; #Department of Radiology,
University of Wisconsin School of Medicine and Public Health,
Madison, WI; **Department of Radiology, University of Michigan
Medical Center, Ann Arbor, MI; wwUniversity of New Mexico,
Albuquerque, NM; zzSociety of Nuclear Medicine, Beth Israel
Deaconess Medical Center, Boston, MA; yyDepartment of Radi-
ology, Medical University of South Carolina, Charleston, SC; and
##Department of Radiology, Temple University, Philadelphia, PA.
The American College of Radiology (ACR) seeks and encourages col-
laboration with other organizations on the development of the ACR
Appropriateness Criteriasthrough society representation on expert
panels. Participation by representatives from collaborating societies
on the expert panel does not necessarily imply individual or society
endorsement of the final document.
Jeffrey P. Kanne is a consultant for PTC Therapeutics ($13,000) not
relevant to ACR activities or imaging. For the remaining authors
no conflicts of interest were declared.
Reprints: Kathleen Brown, MD, American College of Radiology, 1891
Preston White Drive, Reston, VA 20191 (e-mail: kbrown@
r American College of Radiology. Reprinted with
AMERICAN COLLEGE OF RADIOLOGY
Appropriateness Criteria Review
J Thorac Imaging?Volume 28, Number 4, July 2013www.thoracicimaging.com|W57
evaluation of adult patients with unexplained dyspnea or
other symptoms of possible PH or for the evaluation of
patients at risk for PH.
Echocardiography is noninvasive, widely available,
reproducible, and relatively inexpensive. The lack of radi-
ation exposure makes it a valuable examination for serial
follow-up studies. Patients at high risk for development of
PAH may benefit from screening using Doppler echo-
cardiography.11At-risk patients include: (1) individuals
with known genetic mutation–associated PAH or with a
first-degree relative with idiopathic PAH; (2) those with
scleroderma spectrum of disease; (3) patients with con-
genital heart disease and systemic-to-pulmonary shunts;
and (4) patients with portal hypertension before liver
Doppler echocardiography allows estimation of right
ventricular systolic pressure and pulmonary diastolic pres-
sure. Doppler echocardiography is also useful for evaluat-
ing cardiac anatomy such as chamber enlargement, valvular
function and morphology, left ventricular systolic and
diastolic dysfunction, and the presence of pericardial effu-
sion. An echocardiographic contrast or “bubble” study
using agitated saline may be useful for detecting intra-
Ventilation-Perfusion (V/Q) Scans
The algorithm recommended by the American College
of Cardiology Working Group calls for scanning in all
patients with unexplained PH.13V/Q scans are particularly
useful in determining chronic thromboembolic pulmonary
hypertension (CTEPH) and differentiating CTEPH from
other causes of PH. A normal or low-probability scan
essentially excludes the diagnosis of CTEPH with a sensi-
tivity of 90% to 100% and a specificity of 94% to 100%.6
The V/Q scan may be normal in other causes of PH.
Right Heart Catheterization
Right heart catheterization is the gold standard for the
diagnosis of PAH.11Right heart catheterization directly
measures PAP and cardiac function. The vasoreactivity of
pulmonary circulation should be tested using a short-acting
drug and by taking direct pressure measurements. Such
tests may identify patients likely to respond to long-term
therapy with calcium channel blockers.6,14
Catheter pulmonary angiography remains the stand-
ard of care for assessing operability in patients with
CTEPH, although some authors have demonstrated that
computed tomography pulmonary angiography (CTPA) is
as reliable as digital subtraction angiography in the evalu-
ation of CTEPH.15Angiography and CTPA may be used
for evaluating possible arteriovenous malformations, and
CTPA, magnetic resonance imaging (MRI), and positron
emission tomography (PET) may be used for evaluating
Computed Tomography (CT)
Chest CT and CTPA may be useful in assessing the
clinical classification of PAH.16A number of findings on
CT have been shown to be useful in evaluating possible
PAH, including the ratio of pulmonary artery diameter to
aortic diameter17and the main pulmonary artery dia-
meter.18More recent studies have shown that the sensitivity
and specificity of the main pulmonary artery diameter vary
widely, depending on the etiology of the lung disease.19,20
High-resolution CT is indicated for evaluating diffuse
lung disease and for screening of patients with chronic
unexplained dyspnea (see the ACR Appropriateness Cri-
teriastopics on “Chronic Dyspnea—Suspected Pulmonary
Origin”21and “Dyspnea—Suspected Cardiac Origin”).
Diffuse lung diseases associated with PH include interstitial
lung disease and emphysema, sarcoidosis, connective tissue
disease, and pulmonary Langerhans cell histiocytosis. The
diagnosis of several rare diseases associated with PAH, such
as pulmonary veno-occlusive disease and pulmonary
capillary hemangiomatosis, may be suggested by high-res-
olution CT.16,22,23CTPA is increasingly used to evaluate
thromboembolic disease, and it has become the standard of
care at many institutions. Some authors have suggested that
the greater expertise in interpretation of CTA supports the
use of CTA rather than V/Q scans for initial evaluation of
patients with suspected CTEPH.24
MRI/MRA can provide reproducible quantification of
morphologic and functional changes occurring in PH that
can aid in diagnosis and help evaluate disease severity and
prognosis.8,25–29Small studies have shown that MRI can
also be used as a noninvasive method for obtaining
TABLE 1. Clinical Condition: Pulmonary Hypertension
9 Catheterization and
examinations. Both should
typically performed before
examinations. Both should
typically performed before
CTA chest (noncoronary)
Tc-99m V/Q scan lung
MRI heart function and
MRA pulmonary arteries
with right heart
CT chest without contrast
May be performed with
5 If there is concern for an
occult ILD, HRCT may be
— US echocardiography
Rating scale: 1, 2, 3=usually not appropriate; 4, 5, 6=may be
appropriate; 7, 8, 9=usually appropriate.
HRCT indicates high-resolution CT, ILD, interstitial lung disease; US,
Brown et alJ Thorac Imaging?Volume 28, Number 4, July 2013
W58|www.thoracicimaging.com CopyrightrAmerican College of Radiology. Reprinted with permission.
functional information to monitor treatment response and
can be performed serially to monitor changes in right
ventricular parameters as a response to medical therapy. By
detecting increases in right ventricular stroke volume,
reversion of the septal shift, and decreases in right ven-
tricular mass, MRI can assess the long-term effects of
MRI has proven to be essential for the diagnosis and
characterization of congenital heart abnormalities32and
has become an important tool for detecting and quantifying
cardiovascular shunt lesions while providing information
on right heart function (group 1.4.4).33Of utmost impor-
tance is the ability of MRI to detect and characterize shunts
that are difficult to identify on echocardiography, including
sinus venosus atrial septal defects, atrioventricular septal
defects, and partial anomalous pulmonary venous return.
Given its high diagnostic sensitivity and specificity and
lack of ionizing radiation, MRI may be used as an adjunct
or as a comprehensive alternative to current first-line or
invasive examinations at many tertiary centers. This is
particularly important for young patients for whom the
risks from repeated radiation exposure are greater and for
patients with significant comorbidities that result in greater
risk from repeated right heart catheterizations.
FDG-PET allows in vivo imaging of metabolic proc-
esses and is complementary to the structural/anatomic
information provided by cross-sectional imaging modal-
ities. FDG-PET is well established for the diagnosis and
management of malignancy but is becoming a valuable
imaging modality for the characterization and diagnosis of
various inflammatory conditions.34–36The use of FDG-
PET in the evaluation of PH is extremely limited, but it may
be useful in distinguishing rare cross-sectional mimics of
chronic thromboembolic PH, including pulmonary artery
sarcoma and medium to large vessel vasculitis (eg,
Takayasu arteritis), both of which will demonstrate
increased FDG uptake.35,36
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