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141© Springer Nature Switzerland AG 2020
W. R. Auger, D. Gopalan (eds.), Clinical Cases in Chronic
Thromboembolic Pulmonary Hypertension, Clinical Cases in
Cardiology, https://doi.org/10.1007/978-3-030-17366-1_15
Case Presentation
Fifty-five-year-old male with a medical history significant for
tobacco abuse (>35 pack-years), prior heavy alcohol use, mild
cirrhosis (Child-Pugh class A), hypertension and
hyperlipidemia. There was also the history of right ventricular
dysfunction diagnosed in 2000.
In July 2014, the patient’s exertional dyspnea and func-
tional disability had progressed to WHO functional class
III.His exam notable for a holosystolic murmur, a soft early
Chapter 15
Case 15: CTEPH? But
theLung Scan Is Normal!
VictorJ.Test, DeepaGopalan, andWilliamR.Auger
V. J. Test (*)
Division of Pulmonary Medicine and Critical Care, Texas Tech
University School of Medicine, Lubbock, TX, USA
e-mail: victor.test@ttuhsc.edu
D. Gopalan
Department of Radiology, Imperial College Hospitals, London, UK
e-mail: d.gopalan@nhs.net
W. R. Auger
Lewis Katz School of Medicine, Temple University,
Philadelphia, PA, USA
e-mail: Bill.Auger@tuhs.temple.edu
142
diastolic murmur, P2 accentuation, jugular venous distension
and ankle edema. Lung exam was unremarkable.
An echocardiogram revealed normal left ventricular func-
tion, severe right ventricular dilatation, RVH and severe RV
dysfunction, moderate tricuspid regurgitation, an RV systolic
pressure estimated at 85mmHg, mild to moderate pulmonic
regurgitation, and severe pulmonary artery dilatation (4.7cm).
Left and right heart catheterization were subsequently
performed. There was no atherosclerotic coronary vascular
disease, though his LVEDP was elevated at 22 mmHg. His
pulmonary artery pressure was measured at 100/40 with a
PVR of 9.7 Wood units. There was no significant PVR
response to inhaled nitric oxide.
CT angiography of the chest showed “chronic pulmonary
embolism” with an extensive clot burden involving several
lobar and segmental vessels. The central pulmonary arteries
were severely dilated and the RV was enlarged with thicken-
ing of the RV wall. No lung parenchymal abnormalities were
observed (Fig.15.1a–d).
a
b
F . CT angiogram imaging the proximal pulmonary vessels
(a), progressing down to the lower lobe arteries (b–d). Lining throm-
bus is noted throughout the proximal vessels, which does not extend
into the enlarged segmental lower lobe arteries. There was no evi-
dence for vessel obstruction or narrowing of the pulmonary vessels
with organized clot
V. J. Test et al.
143
A lung perfusion scan was recommended given the CT
findings, and was assessed as “fairly unremarkable” and “low
probability for acute PE” (Fig.15.2).
Lower extremity dopplers were negative and a hyperco-
agulable workup was positive for a heterozygous Factor V
Leiden abnormality.
The patient was ultimately place on diuretics and a soluble
guanylate cyclase stimulator in treatment of his pulmonary
hypertension. This resulted in an improvement in his overall
functional status.
Radiographic Interpretation
CTPA demonstrates features of significant pulmonary hyper-
tension as evidenced by aneurysmal dilation of main
pulmonary artery, markedly enlarged right sided cardiac
chambers and severe right ventricular hypertrophy. There is
reversal of septal curvature with small under-filled left ven-
tricle. There is eccentric non-occlusive clot lining the distal
right and left main pulmonary arteries. The segmental pulmo-
nary artery branches are also dilated with thin rim of clot in
c
d
F . (continued)
Chapter 15. Case 15: CTEPH? But theLung ...
144
multiple vessels. No intravascular webs, stenosis or pouch
defects were present. Lung windows did not reveal mosaic
attenuation. There was no evidence of intracardiac shunt and
the pulmonary venous anatomy was normal. Scintigraphy
shows mottled perfusion but there are no segmental mis-
matched perfusion defects that is typical of CTEPH.
The overall appearances are that of PAH with in-situ
thrombosis (IST).
Lining clot is a well known complication of long standing
PAH (Moser et al.). Proposed mechanism for IST includes
wall shear stress from turbulent flow, vascular dilatation lead-
ing to stasis and endothelial injury creating a procoagulant
F . Perfusion scan of the same patient at the same time
that the CT angiogram in Fig.15.1 was obtained. Other than an
“imprint” of the enlarged central PAs, the perfusion pattern in both
lungs is essentially normal
V. J. Test et al.
145
environment (Chaouat et al., Archer etal.). Like CTEPH,
these clots can also exhibit calcification. Differentiation
between the two conditions is crucial as they have entirely
different therapeutic options. In CTEPH, the segmental ves-
sels tend to be attenuated whilst PAH is associated with
enlarged proximal vessels and peripheral pruning. Other
angiographic features of CTEPH such as truncation of seg-
mental arteries, variation in size of segmental vessels, webs,
stenosis and pouch defects are absent in PAH. Congenital
heart disease can also be associated with IST and is seen in
about one-fifth of the patients with Eisenmenger's syndrome.
Hence, it is important to make a thorough search for the pres-
ence of intracardiac shunts.
Clinical Comments
Though CTEPH has been defined as pulmonary hyperten-
sion in the presence of radiographic evidence for chronic
thromboembolic disease, the case presented here is an exam-
ple of how that definition can sometimes be misconstrued.
What is present in this patient is organized thrombus lining
enlarged central pulmonary vessels—in-situ and non-embolic.
It is a consequence of longstanding pulmonary hypertension
and not the cause of it. Most often this is seen in congenital
heart disease with Eisenmenger’s physiology that has resulted
in massively enlarged proximal pulmonary arteries (see Case
19, Chap. 19). How this develops is not completely under-
stood. As pointed out by Dr. Gopalan, it is possible that the
blood flow juxtaposed to the enlarged vessel is “stagnant”
enough to allow thrombosis to occur; or possibly there’s an
endothelial injury of the proximal vasculature which pro-
motes clot formation. Either consideration is speculative.
Though the extent of thrombus visualized by CT can be
impressive, as the vessels are examined distally, most often
the lining thrombus disappears leaving the vessels intact and
unobstructed. As blood flow through these vessels is unim-
peded, the relatively normal perfusion pattern seen on lung
Chapter 15. Case 15: CTEPH? But theLung ...
146
scintigraphy is not surprising. Consequently, logic dictates
and previous experience has demonstrated that surgical end-
arterectomy of this lining thrombus provides no pulmonary
hemodynamic benefit and should be considered
contraindicated.
Point ofEmphasis
Lining thrombus within enlarged central pulmonary vessels is
typically unobstructive and is not the basis for a patient’s
pulmonary hypertension, but a consequence of it.
Diagnosis
1. Lining thrombus within enlarged central pulmonary
arteries.
2. Pulmonary arterial hypertension.
Further Reading
Archer S, Rich S.Primary pulmonary hypertension: a vascular biol-
ogy and translational research “work in progress”. Circulation.
2000;02:2781–91.
Chaouat A, Weitzenblum E, Higenbottam T.The role of thrombosis
in severe pulmonary hypertension. Eur Respir J. 1996;9:356–63.
Gopalan D, Blanchard D, Auger WR. Evaluation of chronic
thromboembolic pulmonary hypertension. Ann Thorac Soc.
2016;13(Suppl 3):S222–39.
Moser KM, Fedullo PF, Finkbeiner WE, Golden I.Do patients with
pulmonary hypertension develop extensive central thrombus?
Circulation. 1995;91(3):741–5.
Perloff JK, Hart EM, Greaves SM, Miner PD, Child JS.Proximal
pulmonary arterial and intrapulmonary radiologic features of
Eisenmenger syndrome and primary pulmonary hypertension.
Am J Cardiol. 2003;92:182–7.
V. J. Test et al.
147© Springer Nature Switzerland AG 2020
W. R. Auger, D. Gopalan (eds.), Clinical Cases in Chronic
Thromboembolic Pulmonary Hypertension, Clinical Cases in
Cardiology, https://doi.org/10.1007/978-3-030-17366-1_16
Case Presentation
This patient is an 18-year-old, female, college freshman, and
ROTC student. She presented to the student health service
with a chief complaint of SOB, left-sided chest pain, and
exertion-related episodes of presyncope, with one episode of
frank syncope, promptly regaining consciousness after becom-
ing supine on the ground. She was referred to an electro-
physiologist at a nearby hospital for a same day evaluation of
Chapter 16
Case 16: Acute Versus
Chronic Thromboembolic
Disease—Beware!
DianneL.Zwicke, SaraPaulus, DeepaGopalan,
andWilliamR.Auger
D. L. Zwicke (*) · S. Paulus
Pulmonary Hypertension Clinic, Aurora St. Luke’s Medical Center,
Milwaukee, WI, USA
D. Gopalan
Department of Radiology, Imperial College Hospitals, London, UK
e-mail: d.gopalan@nhs.net
W. R. Auger
Lewis Katz School of Medicine, Temple University,
Philadelphia, PA, USA
e-mail: Bill.Auger@tuhs.temple.edu
148
the syncopal episode and persistent tachycardia at 100 bpm
(usual heart rate 60bpm).
Her detailed clinical history included the onset of symp-
toms 6 weeks prior to presentation for medical evaluation.
Although she was in a good state of physical fitness, she was
unable to walk more than one block and developed light-
headedness and presyncope with stair climbing and with any
of her ROTC activities. The syncopal episode occurred dur-
ing ROTC exercises, two weeks before her clinic presenta-
tion, and she was suspended from any further military
training until cleared medically. She denied any history of
similar symptoms, surgical procedures, pregnancy, history of
DVT/PE, chronic lower extremity edema, or injury. She did
take a plane flight and went on a cruise 6 weeks before the
onset of her symptoms. She did report starting estrogen-
containing oral contraceptives 17 months before the onset of
her first clinical symptoms, for the purpose of birth control
and premenstrual dysphoric syndrome.
She had an unremarkable past medical history.
Her family history was notable for her father having expe-
rienced a deep venous thrombosis (DVT) without a known
clotting disorder.
The physical examination was unremarkable, except for a
resting heart rate of 120 bpm and respiratory rate of 20 per
minute. The blood pressure was 100/70 mmHg. The resting
pulse oximetry showed an O
2
saturation of 99% on room air.
A 12-lead electrocardiogram demonstrated normal findings
with a sinus tachycardia. Laboratory studies were normal
except for a mild elevation of the ALT at 50U (normal range
5–35U). A urine pregnancy test was negative.
Initial assessment included an echocardiogram which
demonstrated a low normal LV ejection fraction at 50%, a
severely increased RV size with severely decreased function,
significant shift of the intraventricular septum into the LV
cavity, a severely enlarged right atrium, mild tricuspid regur-
gitation with a RVSP estimated at 68 mmHg, and a small
pericardial effusion. Injected agitated saline did show evi-
dence of an intra-atrial right-to-left shunt at rest (Fig.16.1).
D. L. Zwicke et al.
149
This was followed by the acquisition of a CT angiogram of
the chest. This revealed bilateral pulmonary emboli affecting
the proximal lobar vessels of the right upper and bilateral lower
lobes, extending into the segmental branches. Partially occlud-
ing thrombi are seen within the segmental left upper lobe
branches and a large thrombus is visible in the left lower lobe
pulmonary artery extending into the segmental branches. There
was a small infiltrate versus atelectasis in the anterior left lung
base, and a small amount of anterior pericardial fluid (Fig.16.2).
A duplex venous ultrasound of both lower extremities
disclosed the deep veins to be widely patent with normal flow
and no evidence of thrombus.
She was admitted to the Intensive Care Unit, after initiat-
ing intravenous heparin, with the intention of proceeding
with lytic therapy utilizing tissue plasminogen activator
(TPA). The clinical history related was consistent with a pos-
sible pulmonary embolic event as long as 6 weeks prior; how-
ever her presentation was prompted by worsening symptoms
that were hemodynamically significant.
She was transferred to a tertiary hospital as the patient and
parents requested additional consults prior to moving for-
ward with thrombolysis given lack of consensus in the treat-
ment plan. Hematology and Pulmonary Hypertension/
Cardiology consults were obtained and achieved consensus
was to withhold lytic therapy for concern that she had acute
on chronic pulmonary emboli.
F . Transthoracic echocardiogram showing signs of right
ventricular strain
Chapter 16. Case 16: Acute Versus Chronic …
150
A lung ventilation-perfusion scan was recommended.
(Hospital Day # 3) This showed extensive modest to large
sized peripherally located wedge shaped perfusion defects
bilaterally throughout the lungs, consistent with findings of
pulmonary emboli by CT (Fig.16.3).
On hospital day 6, a right heart catheterization was per-
formed: BP 99/66mmHg, mean arterial pressure 79mmHg,
HR 77 bpm, pulse oximetry on room air 99%. RA mean
6mmHg, RV 40/6mmHg, PA pressure 40/17 (25 mean)mmHg,
b
c
a
F . Axial (a) and coronal views (b, c) of the CT angiogram
demonstrating extensive proximal vessel intraluminal defects in
both lungs consistent with acute pulmonary emboli. There is modest
central PA enlargement and RV enlargement, though no significant
RV hypertrophy
D. L. Zwicke et al.