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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3902_Библиотеки_им_академика_М_И_Перельмана
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55
ab
cd
F . (a–d) Frontal (panel a) and lateral (panel b) views of the
left pulmonary artery. Frontal (panel c) and lateral (panel d) views
of the right pulmonary artery
Chapter 6. Case 6: Distal Vessel ......
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Radiographic Interpretation
A more detailed interpretation of the studies presented
reveals that the chest radiograph demonstrates a dilated right
atrium and enlargement of proximal pulmonary arteries with
peripheral pruning. There is parenchymal scarring in the right
mid zone. The blunted costophrenic angle could be due to
chronic right basal pleural reaction secondary to the previous
documented episode of right haemothorax with a small effu-
sive component.
VQ scintigraphy shows normal ventilation and multiple
mismatched bilateral perfusion defects, the location of which
are as described in the text. Of note, the majority of defects
are segmental in size.
The frontal and lateral views of selective pulmonary angio-
grams of the right and left pulmonary arteries are presented
F . Organized thrombus removed at endarterectomy, pri-
marily from the subsegmental vessels (Level IV resection)
A. Bautista et al.
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in Fig.6.3. On the right, in the upper lobe, there is segmental
web in the apical branch and proximal stenosis with post ste-
notic dilatation in the anterior branch, proximal pouch in the
lower lobe with segmental web in the lateral branch and
proximal irregular stenosis in the medial branch. Overall,
there is paucity of vessels in the mid to distal third of the
lungs, particularly in the upper and mid zones. On the left, in
the lower lobe, the posterior and lateral segmental vessels are
attenuated, there was a truncated apical segment, and distal
vessel narrowing of the anterior segmental branch. There are
segmental webs in the apico-posterior branch of the upper
lobe and lobar occlusion of the lingula. There is severe reduc-
tion in perfusion in the lower lobe as evidenced by the lack of
parenchymal blush in the capillary phase.
Overall, this is a case of chronic thromboembolic disease
with mixed disease distribution. Although there is disease in
the lobar and segmental vessels, on balance, the extent of
demonstrable disease is out of proportion to the markedly
elevated pulmonary vascular resistance of 1091dynes/s/cm
-5
.
This implies there is significant small vessel component.
Whilst it is the accepted norm that there should be concor-
dance between the extent of disease seen on imaging and the
pulmonary hemodynamics, there is no exact formula linking
the two parameters. It is more of an art rather than an exact
science and therefore, decision regarding operability should
not be based purely on the basis of uni-modality imaging.
Clinical Comments
There are several points of interest illustrated by this case.
The first comes from the observation that significant chronic
thromboembolic disease was diagnosed in this 15-year-old
girl, emphasizing that CTEPH can occur even in pediatric
patients. As is often the case in this age group, a prothrom-
botic state was demonstrated (see Madani et al. 2011).
However, an additional risk factor for the development of
thromboembolic disease was likely related to repetitive
Chapter 6. Case 6: Distal Vessel ......
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injury of her subclavian vein with her athletic activities, ulti-
mately leading to the diagnosis of Paget-Schroetter syn-
drome. The initial diagnosis of pleurisy and exercise-induced
asthma was also not surprising, given the absence of specific
symptoms to suggest thromboembolic disease and the rela-
tive rarity of CTEPH, especially in pediatric patients.
However, that’s one of the reasons behind presenting this
case, to acknowledge that in the absence of an evident cause
for the new onset of exertional dyspnea…regardless of age
group…pulmonary vascular disease needs to be considered.
And given the degree of pulmonary hypertension discovered
during her initial hospitalization, it was evident that CTEPH
was already present.
The other point to be made is that the evaluation for sur-
gery was somewhat misleading. The size of the perfusion
abnormalities by lung scintigraphy, and the pulmonary angio-
graphic findings which suggested lobar disease on the right
(in the descending PA), and segmental disease in the right
upper lobe and throughout the pulmonary vascular bed on
the left, correctly created the impression that these lesions
were accessible to endarterectomy. However, the actual orga-
nized thrombi removed were primarily from the distal seg-
mental and subsegmental arteries, which would be consistent
with what one would expect given the source of clot was from
the upper extremities veins. This emphasizes the importance
of having the surgical expertise to perform distal vessel end-
arterectomies, especially in those clinical scenarios where
distal disease is likely. This case also illustrates the discrep-
ancy that occasionally occurs between the preoperative
assessment and what is discovered at surgery. But most
importantly, this presentation highlights the significant pul-
monary hemodynamic and clinical benefits that can be
achieved with a properly performed distal vessel thromboen-
darterectomy. As available information has documented that
the best chance for a cure comes with PTE surgery, every
patient with CTEPH…regardless of chronic thrombus loca-
tion within the pulmonary vessels…should have a complete
surgical assessment by experienced CTEPH diagnosticians
and surgeons.
A. Bautista et al.
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Point ofEmphasis
Though technically a more difficult surgery requiring consid-
erable expertise, a properly performed thromboendarterec-
tomy in patients with distal vessel CTEPH can lead to
significant improvements in pulmonary hemodynamics, func-
tional status and quality of life. Careful patient selection is
critical to a successful outcome.
Diagnosis
1. Distal vessel chronic thromboembolic pulmonary
hypertension.
Further Reading
Auger WR, Kim NK, Fernandes T, et al. Distal vessel endarterec-
tomy: results from a single institution. J Thorac Cardiovasc Surg.
D’Armini AM, Morsolini M, Mattiucci G, etal. Pulmonary endarter-
ectomy for distal chronic thromboembolic pulmonary hyperten-
sion. J Thorac Cardiovasc Surg. 2014;148:1005–12.
Madani M, Mayer E, Fadel E, Jenkins DP. Pulmonary endarterec-
tomy: patient selection, technical challenges, and outcomes. Ann
Am Thorac Soc. 2016;13(Suppl 3):S240–7.
Madani MM, Wittine LM, Auger WR, etal. Chronic thromboem-
bolic pulmonary hypertension in pediatric patients. J Thorac
Cardiovasc Surg. 2011;141:624–30.
Chapter 6. Case 6: Distal Vessel ......
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61© 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_7
Case Presentation
Twenty-eight-year-old male without significant past medical
history who was very physically active until 2015 when he
was diagnosed with essential hypertension. He noted heart
rates in the 90s when he normally had been running in the
low 50s. He was a competitive rower in college and cycled
long distance, performing at least 20 century rides and mul-
Chapter 7
Case 7: It’s Not CTEPH,
but It Is Chronic
Thromboembolic Disease
(CTED)
PaulR.Forfia, DeepaGopalan, andWilliamR.Auger
P. R. Fora (*)
Pulmonary Hypertension, Right Heart Failure and CTEPH
Program, Temple University Hospital, Philadelphia, PA, USA
e-mail: Paul.Fora@tuhs.temple.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
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tiple 200 miles rides. By September 2015, he noted more
labored breathing with exertion but he was still able to cycle.
He stopped his beta blocker due to insomnia side effect. He
vacationed in a remote country in October 2015 and noted
much worsening symptoms of shortness of breath. He was
diagnosed with pulmonary embolism and was given tPA
after echocardiogram showed PASP of 109mmHg, (though
CTA and VQ were not performed). He was hospitalized for
1month and was ultimately anticoagulated with Eliquis.
Although his symptoms improved, he was not able to
resume previous activities at the desired level. He still worked
out 3h a week for 25–30min using the elliptical machine. On
infrequent occasions when he did cycle, he could cover 2h
before experiencing fatigue. He did not note dyspnea. He
coached his kid’s rugby and noticed that he can no longer
sprint. He was felt to be WHO functional class I. He denied
chest pain, lightheadedness, syncope, hemoptysis, lower
extremity edema. He did note oxygen desaturation during
exercise, going down to 89%. V/Q scan from July 5, 2016 and
January 9, 2017 were essentially unchanged showing right
upper lobe, lingula, questionable left lower lobe perfusion
defects. Echocardiogram from August 12, 2016. showed nor-
mal RA, RV size and RV systolic function, normal LV size and
systolic function, no evidence of pulmonary hypertension.
His past medical history was notable only for his pulmo-
nary embolism as above and essential hypertension. There
was a questionable history of left popliteal DVT (the patient
reported pain and swelling in 2003) for which he was treated
for a short period with aspirin. Prior surgeries included a sep-
toplasty (2011), left ACL reconstruction (2000), bilateral vari-
cocelectomy (2007) and a dermoid cyst removal as a child. On
review of systems, the patient denied constitutional symptoms
of fevers, chills, malaise, weight loss, weight gain, a known
coagulopathy, connective tissue disease, Raynaud’s phenom-
ena, previous exposure to stimulants, anorexiants, trauma.
Family history was remarkable for venous thromboembo-
lism experienced by his father (PE), uncle, grand aunt (DVT).
With concerns over the persistent perfusion defects on VQ
scan, and his dissatisfaction with his functional limitations, a
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referral was initiated to a CTEPH center specializing in
thromboendarterectomy.
On exam, the patient was a muscular, well developed male,
BP 131/89, HR 72 and room air O2 saturation of 99%.
HEENT exam was unremarkable; there was no thyromegaly
or jugular venous distension. Lung fields were clear with nor-
mal diaphragmatic excursion; no pulmonary flow murmurs.
No palpable RV lift; S2 splitting with inspiration and no mur-
mur or S3 gallop. No hepatomegaly. No lower extremity
edema or venous stasis skin changes.
Laboratory data included a BUN 14, creatinine 1.40, liver
transaminase levels within normal limits. ProBNP 13. Urinalysis
negative for protein. White blood cell count 6.1, hemoglobin
15.2, hematocrit 45.7. Platelets 311,000. INR 1.0. PTT 38.2.
EKG: normal sinus rhythm, 83 beats per minute. Normal QRS
axis and R wave progression across the precordium. His echo-
cardiogram (June 2017) demonstrated normal LV size and
function (estimated EF 69%); right atrium and right ventricu-
lar size were normal with normal RV systolic function, no tri-
cuspid regurgitation, and no pericardial effusion.
On chest radiograph (Fig.7. 1 ) there was no cardiomegaly
or central PA enlargement. Lungs were well expanded with-
out infiltrates.
F . Chest radiograph at time of evaluation for thromboen-
darterectomy
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Lung scintigraphy (Fig.7. 2 ) demonstrated large right upper
lobe and lingular perfusion defects, with a smaller unmatched
defect in the anterior right lower lobe.
Results of the right heart catheterization showed pulmo-
nary hemodynamics at rest of right atrial mean of 6mmHg,
PA pressure 31/13 mmHg (20 mean), PAOP 10 mmHg,
thermodilution cardiac output of 7.2 L/min (cardiac index
3.17L/min/m
2
) and calculated PVR 112dyns/cm
5
. Exertion
of his upper extremities resulted in a PA pressure of
46/12mmHg (28 mean), a pulmonary capillary wedge pres-
sure of 12 mmHg, and a thermodilution cardiac output
13.6 L/min (cardiac index 5.99 L/min/m
2
); calculated PVR
was 94dyns/cm
5
.
Pulmonary angiography revealed evidence for chronic
thromboembolic disease at the lobar level of a right upper
lobe vessel and lingula (Fig.7. 3 ).
Though there was radiographic evidence for operable
chronic thromboembolic disease, in the absence of significant
WASH IN
ANT Q
LLAT Q
RLAT Q
LAO QRAO Q
POST QLPO Q
RPO Q
WASHOUT1 WASHOUT2EQUIL
F . Lung scintigraphy demonstrating unmatched perfusion
defects in both lungs
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exercise limitation and with essentially normal pulmonary
hemodynamics at rest and during modest exertion, it was
assessed that the risk of a surgical thromboendarterectomy
was unwarranted and unlikely to result in a noticeable clini-
cal benefit. However, to provide a more complete physiologic
evaluation at higher levels of exercise, a cardiopulmonary
exercise test (CPET) was requested.
The patient exercised up to 271W for a total exercise time
of 11min 1s and was reported stopping because of dyspnea
(3 out of 10) At rest, minute ventilation (VE) at rest was
13.5 L/min, maximum voluntary ventilation was 194L/min,
and resting oxygen saturation was 99% (pulse oximetry). The
patient’s physiological dead space proportion (VD/VT) at
rest, estimated from the transcutaneous PCO2 (PtcCO2) and
exhaled PCO2 (PexCO2), was 0.31. Anaerobic threshold,
based on the rates of change in VE/VO2, VE/VCO2 and R
was reached at a VO2 of 1.692L/min (16.6mL/kg/min), 59%
of predicted peak VO2. At the point of AT, the VE/VCO2
ratio was 28, the RQ was 0.95 and the heart rate was 139 BPM
(81% of the predicted max). At peak exercise, VO2 (3.505L/
min, 34.4 mL/kg/min) was 123% of the predicted peak
F . Digital subtraction pulmonary arteriogram: “pouch
defect” proximal right upper lobe (open arrow), and occluded lin-
gula (closed arrow)
Chapter 7. Case 7: It’s Not CTEPH, but It Is Chronic…
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