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76
c
d
F . (continued)
C. McEvoy et al.
77
pulmonary hypertension at rest and significant rise in pulmo-
nary artery pressures with a modest increase in cardiac output.
As a result of her dissatisfaction with her functional status,
and with her exercised induced pulmonary hypertension, she
elected to proceed with surgery.
She underwent pulmonary thromboendarterectomy 1 year
after diagnosis of her acute pulmonary embolism. She was
found to have level 1 disease on the right and level 2 disease
on the left (Fig.8.5). Her post-operative hemodynamics off
vasopressors showed a mean PA pressure 19 mmHg with
cardiac index 3.1L/min/m
2
and PVR<2Wood units. She was
discharged on post-operative day 7 with the recommendation
for life-long anticoagulation.
At follow-up, she is markedly improved and very pleased
with her progress. She is able to do all activities including
exercise without limitation. Six-minute walk distance has
improved to 1505 ft without desaturations and concluding
Borg score of 2.
V/Q scan (Fig.8.6) showed perfusion images with physio-
logic distribution of pulmonary perfusion and resolution of
defects. Echocardiogram demonstrated normal LV and RV
size and function with normal pulmonary pressures.
F . Organized thrombus endarterectomized at surgery
Chapter 8. Case 8: Another Case ofCTED: ...
78
Radiographic Interpretation
Selected images from the presentation CT pulmonary angi-
ography (Fig. 8.1) demonstrates multiple central filling
defects in the right and main pulmonary arteries, segmental
right and left lower lobe vessels. The pulmonary artery to
aorta (PA:AA) ratio is 1.0. The RV/LV ratio was >1.0 (not
shown). There is a small amount of fluid in the superior peri-
cardial recess. These findings are in keeping with acute pul-
monary embolism with CT features of right heart strain. On
the 2month follow-up VQ scintigraphy, there are mismatched
segmental perfusion defect in the right upper lobe and large
lobar defect in the lingula and left lower lobe (Fig.8.2). There
is no interval change in the size and distribution of the defects
on the 6 month VQ (Fig. 8.3). Selected images from the
repeat CT pulmonary angiography shows complete occlusion
of the lingula and left lower lobe with recanalized clot. There
is a proximal web in the right proximal descending PA (best
visualized with changing the window to “lighten” the contrast,
Fig. 8.4b) and tight trifurcation stenosis in the right lower
F . Ventilation perfusion scintigraphy 7 months post-
pulmonary thromboendarterectomy
C. McEvoy et al.
79
lobe. Following successful pulmonary endarterectomy, there
is complete resolution of the perfusion defects with normal
tracer distribution in both lungs (Fig.8.6).
Clinical Commentary
This case is an extension of observations made in Case 10
(Chap. 10). Even after an appropriate period of anticoagula-
tion, there was failure to resolve her thromboembolic burden,
with progression to occlusive organized chronic thromboem-
bolic disease. Despite relatively normal echocardiographic
findings and pulmonary hemodynamics at rest, a more
detailed assessment with exercise right heart catheterization
revealed considerable exertion related pulmonary hyperten-
sion. Her symptoms were therefore felt to be related to pul-
monary hypertension with exercise and/or maladaptation of
the right ventricle with a decrease in RV compliance. In addi-
tion, studies in patients with symptomatic thromboembolic
disease have demonstrated abnormal dead space ventilation
as contributing to their exertional dyspnea. Whether or not
this was an issue with this patient would have required a care-
fully performed cardiopulmonary exercise test.
The decision to proceed with surgery necessitated an
understanding on the patient’s part that the desired surgical
outcomes included the prevention of pulmonary hyperten-
sion with exertion, a reduction in dead space ventilation and
a return of pulmonary vascular reserve. These goals are
clearly different than that seen in CTEPH where the reversal
of pulmonary hypertension and RV dysfunction are the
desired primary outcomes. Debate continues as to whether
PTE should be performed in patients with chronic thrombo-
embolic disease in the absence of pulmonary hemodynamic
compromise. The natural history of this problem is poorly
characterized and as Taboada and colleagues have demon-
strated (see reference below), the surgery carries significant
risk. Though in their series there was no perioperative mor-
tality, complications occurred in 40% of patients. Consequently,
the assessment of risks and anticipated benefits of surgery in
Chapter 8. Case 8: Another Case ofCTED: ...
80
this patient subgroup needs to be individualized. In this case,
at least from a functional standpoint, and with normalization
of her echocardiogram and perfusion scan, a favorable surgi-
cal outcome was obtained.
Points ofEmphasis
Pulmonary thromboendarterectomy surgery can benefit
symptomatic patients with chronic thromboembolic disease,
though an understanding of surgical goals and attendant risks
with this approach are essential.
Diagnosis
1. Chronic thromboembolic disease (CTED).
Further Reading
Held M, Kolb P, Grün M, Jany B, Hübner G, Grgic A, etal. Functional
characterization of patients with chronic thromboembolic dis-
ease. Respiration. 2016;91:503–9.
McCabe C, Preston SD, Gopalan D, Dunning J, Pepke-Zaba
J. Cardiopulmonary exercise testing suggests a beneficial
response to pulmonary endarterectomy in a patient with chronic
thromboembolic obstruction and normal preoperative pulmo-
nary hemodynamics. Pulm Circ. 2014;4:137–41.
McCabe C, White PA, Hoole SP, Axell RG, Priest AN, Gopalan
D, et al. Right ventricular dysfunction in chronic thromboem-
bolic obstruction of the pulmonary artery: a pressure-volume
study using the conductance catheter. J Appl Physiol (1985).
2014;116:355–63.
Taboada D, Pepke-Zaba J, Jenkins DP, Berman M, etal. Outcome of
pulmonary endarterectomy in symptomatic chronic thromboem-
bolic disease. Eur Respir J. 2014;44(6):1635–45.
van Kan C, van der Plas MN, Reesink HJ, van Steenwijk RP, Kloek JJ,
Tepaske R, etal. Hemodynamic and ventilatory responses during
exercise in chronic thromboembolic disease. J Thorac Cardiovasc
Surg. 2016;152:763–71.
C. McEvoy et al.
81© 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_9
Case Presentation
A 68 year old woman started experiencing progressively
worsening shortness of breath during her walks over the
course of a year, more pronounced during inclines. She could
initially walk for 2–3 miles without any symptoms, but she
had to gradually decrease this distance due to dyspnea and
fatigue. Her symptoms were initially attributed to her aging
Chapter 9
Case 9: Pulmonary Artery
Sarcoma Mimicking
Massive Thromboembolic
Disease
DemosthenesG.Papamatheakis, DeepaGopalan,
andWilliamR.Auger
D. G. Papamatheakis (*)
Pulmonary, Critical Care and Sleep Medicine, University
of California, San Diego, La Jolla, CA, USA
e-mail: dpapamatheakis@ucsd.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
82
and she did not seek further attention, however they contin-
ued to worsen. She eventually started feeling lightheaded and
having chest pressure with exertion, at which point she went
to the emergency department. A computed tomogram (CT)
pulmonary angiography was performed showing large central
pulmonary artery filling defects (Fig.9.1). Based on this she
was diagnosed with an acute pulmonary embolus for which
she was treated with catheter directed thrombolysis using
tPA.Based on hospital reports, pulmonary artery pressures
and angiographic imaging did not improve significantly after
this procedure. Although lower extremity ultrasound did not
exhibit any evidence of deep vein thrombosis, due to the size
of the filling defects within the pulmonary vasculature on CT
it was decided to proceed with an inferior vena cava (IVC)
filter placement. During placement, venography revealed a
duplicated IVC, therefore dual IVC filters were deployed.
She was started on a heparin drip after conclusion of the
above procedures, switched to rivaroxaban, and eventually
discharged home.
Past medical history included osteopenia, systemic hyper-
tension, anxiety, gastro-esophageal reflux disease, and cata-
racts for which she had right eye surgery a few years prior.
Her only other surgery was a total abdominal hysterectomy
and oophorectomy more than 15 years before her current
presentation. She had no known allergies, and no history of
prior deep vein thrombosis, or pulmonary emboli. She also
denied any prior estrogen use.
F . Selected images from computed tomography pulmonary
angiography (see “Radiographic Interpretation” section for arrow
explanations)
D. G. Papamatheakis et al.
83
Social history disclosed no alcohol, tobacco, diet pill or
illicit drug use. She worked as a flight attendant prior to retir-
ing and was widowed. Her family history did not include any
clotting disorder or any relatives with prior blood clots. She
noted a rash with penicillin and some pruritus with enoxapa-
rin and with clindamycin.
A few weeks after her discharge, she followed up as an
outpatient with a pulmonologist due to continued shortness of
breath and decreased exercise tolerance. Not only had she not
returned to her baseline, but she occasionally experienced
exertional chest tightness and palpitations that were allevi-
ated with rest. On one occasion, after pushing herself to walk
approximately 200 ft at a brisk pace, she felt dizzy and pre-
syncopal. Her pulmonologist ordered an echocardiogram that
showed a normal left ventricular size and systolic function,
with mild diastolic dysfunction. Abnormal ventricular septal
motion and position were noted, suggesting the presence or
right ventricular pressure overload. The right ventricle was
moderately enlarged and hypertrophied with reduced systolic
function and the right ventricular systolic pressure was
reported at 67mmHg with a TAPSE measured at 1.32cm. The
left atrium was normal, the right atrium was moderately
dilated and a trivial pericardial effusion was noted in addition
to some moderate tricuspid regurgitation. The main pulmo-
nary artery was enlarged and a large mass was seen within it.
Based on these findings and in the context of her more
chronic and progressive symptoms with poor response to anti-
coagulation, chronic thromboembolic pulmonary hypertension
was suspected and she was referred to a CTEPH center for
pulmonary thromboendarterectomy (PTE) surgery evaluation.
During her evaluation for PTE surgery her vital signs were
notable for elevated systemic blood pressure at 150/73, a tem-
perature of 97°F, a respiratory rate of 16, and a SpO2 of 98%
on room air. Her height was 5′ 4″ and her weight was 60.5kg.
On exam, she had mild JVD and an accentuated P2 heart
sound. Although her lungs were clear to auscultation bilater-
ally, a loud flow murmur was heard over her right lung zones
during breath hold. Her only other finding were some lower
extremity varicose veins.
Chapter 9. Case 9: Pulmonary Artery Sarcoma …
84
At that time a chest x-ray showed two IVC filters in place,
some blunting of the left costophrenic angle, enlarged pulmo-
nary arteries, and a prominent right ventricle (Fig.9.2).
Moreover a ventilation perfusion lung scintigraphy scan
showed lack of perfusion to the entire left lung and a wedge
defect of the superior segment of the right middle lobe
(selected views in Fig.9.3).
After the above preliminary testing she underwent a left
heart catheterization to rule out any coronary artery disease
prior to possible PTE surgery that was unremarkable. Based
on her imaging findings and her clinical history it was sus-
pected that CTEPH was a less likely diagnosis. Therefore,
right heart catheterization and digital subtraction pulmonary
angiography were deferred and felt to be of excessive risk
given the degree of proximal vessel occlusion by CT.As part
of her evaluation, she did however undergo a positron
F . Chest radiograph
D. G. Papamatheakis et al.
85
emission tomography scan with fusion computed tomography
images (Fig.9.4).
Following these studies she was admitted to the hospital,
and placed on continuous intravenous heparin drip in antici-
pation of endarterectomy surgery. This was performed a few
days later including pulmonary valve repair and PFO closure.
The surgery was complicated by left lower lobe airway bleed-
ing requiring a bronchial blocker that resolved within a few
days (specimen shown in Fig.9.5).
Although her post-operative course was complicated by
airway bleeding, an episode of aspiration and retained secre-
tions, a small posterior cerebral artery stroke, and persisting
F . Ventilation perfusion scintigraphy (RAO: right anterior
oblique, Lat: lateral)
Chapter 9. Case 9: Pulmonary Artery Sarcoma …