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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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45
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F . Calcified thrombus lining the central pulmonary vessels.
(Panels a–c) Proximal vessel narrowing of the lingula (Panel b,
arrow) from chronic thromboembolic disease, along with reduction
in vascularity of the RML (Panel c, open arrow) and vessel attenua-
tion (Panel d, arrow) due to involvement of the central vessels with
calcified thrombus. Panel d also showing right atrial enlargement
and hypertrophy of the RV free wall
its walls and the right and left segmental pulmonary arteries
(Fig.5.4).
Pulmonary angiography was performed suggesting central
chronic thromboembolic disease with web defects and dis-
crete vessel narrowing in the RUL, RLL, and LUL, absent
perfusion to the RML, a “pouch defect” in the RUL, occluded
segmental vessels in the LLL and lingula, with turbulent con-
trast filling in both main pulmonary arteries (Fig.5.5).
Chapter 5. Case 5: Operable CTEPH …but Is PTE....
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abc
F . (a–c) Extensive parenchymal lung disease demonstrated
on high resolution computed tomography
F . Frontal views of the pulmonary arteriogram demonstrat-
ing changes consistent with chronic thromboembolic disease
Right and left heart catheterization revealed a right atrial
pressure of 12mmHg, pulmonary artery pressure of 103/42
(63) mmHg, a left ventricular end diastolic pressure of
5mmHg, and a cardiac output of 6.43L/min with a cardiac
index of 2.95L/min/m
2
while on inotropic support. Coronary
angiography revealed no significant coronary disease.
S. Bartolome et al.
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During this evaluation, the patient was in the ICU on a
high flow nasal cannula. The oral prostacyclin therapy was
titrated to off because of concerns that it may be worsening
ventilation/perfusion mismatching and an oral phosphodies-
terase type-5 inhibitor was titrated up to a high dosage. He
was diuresed aggressively and his decompensated right ven-
tricular failure was treated with a dopamine infusion to
decrease right ventricular pressure/volume overload. The
patient tolerated this well and after approximately 2.5weeks
in the ICU and another 1.5weeks on the hospital floor he was
transferred to home on 6L per nasal cannula. He was evalu-
ated for pulmonary thromboendarterectomy including a
second review by an experienced center and it was deter-
mined he was a poor candidate due to his degree of pulmo-
nary parenchymal disease. A transplant evaluation was
pursued and completed while the patient was an inpatient and
he was listed for transplant shortly thereafter. He underwent
successful lung transplantation approximately 1month later.
He is doing well at home and is no longer needing oxygen.
Radiographic Interpretation
Chest radiograph shows cardiomegaly with marked proximal
pulmonary artery dilatation. There is calcification, seen most
prominently in the main PA.Subsegmental atelectasis is pres-
ent in the right lower lobe. There is a small left pleural
effusion. Overall, the appearances are that of long standing
PH with likely pulmonary congestion.
The findings on perfusion scan with the presence of large
bilateral segmental perfusion defects is highly suspicious for
thromboembolic disease rather than PAH as the underlying
aetiology.
CTPA shows dilated and hypertrophied right ventricle
with reversal of septal curvature and an enlarged right atrium
with compression of the under-filled left atrium. There is
eccentric thrombus with peripheral calcification in the dilated
right and left main pulmonary arteries. Associated with this
Chapter 5. Case 5: Operable CTEPH …but Is PTE....
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finding is proximal vessel narrowing of the lingula, a reduc-
tion in vascularity of the RML, and vessel attenuation of the
medial left lower lobe artery. Eccentric lining thrombus is
also evident in one of the right lower lobe segmental vessels.
The lung windows demonstrate paraseptal and centrilobular
emphysema with upper lobe predominance.
Selected views from a catheter pulmonary angiogram shows
eccentric intimal thrombus in the right main PA, extending in to
the right lower lobe. There is a pouch defect with complete
occlusion of the middle lobe. Proximal webs are present in seg-
mental right upper lobe. The anterior, posterior and lateral
basal segments of the right lower lobe are occluded. There is
stenosis with post stenotic dilatation of medial branch of the
right lower lobe. On the left, there is intimal thrombus in main
PA and left lower lobe vessel where there is a proximal stenosis
followed by post stenotic dilatation. The superior segment of
the lower lobe is occluded and anterior and lateral segments are
truncated. There is segmental web disease in the left upper lobe
and truncated appearance to the lingular branches. A salient
feature seen bilaterally is the extreme tortuosity of the vessels
in general. This is seen in very longstanding PH.The known PA
wall calcification is difficult to appreciate on the angiogram
although there is excellent depiction of the vessel lumen, this is
a well- recognized limitation of catheter angiography.
Clinical Comments
This case has features of severe pulmonary hypertensions
secondary to very long standing chronic thromboembolic
disease. Disease distribution is proximal and radiologically
amenable to surgery but an important co-morbidity in the
form of emphysema has been revealed on the thoracic
CT.Patients with significant parenchymal lung disease derive
little benefit from pulmonary endarterectomy, as revascular-
ization of regions affected with either significant emphysema
or interstitial lung disease can cause profound refractory
hypoxemia and respiratory failure post-surgery (see Madani
et al. 2016). This observation has resulted in one of the
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maxims of PTE surgery… “never attempt to reperfuse bad
lung” (also see Chap. 19). An exception to this rule is when
the emphysema is limited to the upper lobes and chronic
thromboembolic disease involves vessels supplying preserved
lung parenchyma. Surgery in this situation has the potential
to be beneficial as there will be improvement in matching
ventilation and perfusion. One should however be very care-
ful when assessing the emphysema severity. CT has been
extensively validated as a tool for assessment of the presence,
pattern, and severity of emphysema. But it should be remem-
bered that visual quantification of emphysema burden on CT
scan is a weak predictor of lung function and individuals with
similar levels of physiological impairment may have very dif-
ferent CT appearances. Hence, it is important to complement
the CT changes with objectives measures of lung function.
Consequently, though this patient’s history was compelling
for recurrent pulmonary embolic events, and his radiographic
studies showed evidence for operable chronic thromboem-
bolic disease, the extent of his air flow obstruction, his paren-
chymal lung disease, and other comorbidities made it unlikely
thromboendarterectomy alone would result in meaningful
clinical improvement.
Point ofEmphasis
The presence of operable chronic thromboembolic disease
does not by itself establish the surgical candidacy of a patient
for pulmonary thromboendarterectomy. A consideration of
comorbidities and an assessment that there’s a high likeli-
hood of achieving a favorable outcome need to factored in
the decision to proceed with PTE surgery.
Diagnosis
1. Chronic thromboembolic pulmonary hypertension with
decompensated right heart failure.
2. Centriacinar and paraseptal COPD—BODE index of 6.
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Further Reading
Auger W, Kerr K, Kim N, Fedullo P. Evaluation of patients with
chronic thromboembolic pulmonary hypertension for pulmonary
thromboendarterectomy. Pulm Circ. 2012;2(2):155–62.
Madani M, Eckhard M, Fadel E, Jenkins P.Pulmonary endarterec-
tomy: patient selection, technical challenges and outcomes. Ann
Am Thorac Soc. 2016;13(S3):S240–7.
S. Bartolome et al.
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51© 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_6
Chapter 6
Case 6: Distal Vessel
Thromboendarterectomy—
How Far Can WeGo?
AngelaBautista, MichaelM.Madani, DeepaGopalan,
andWilliamR.Auger
A. Bautista (*)
CTEPH Program, University of California, San Diego,
San Diego, CA, USA
e-mail: m2bautista@ucsd.edu
M. M. Madani
University of California, San Diego, San Diego, CA, USA
e-mail: mmadani@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
Case Presentation
Fifteen-year-old female who had been previously healthy,
playing competitive volleyball all of her life. In March 2014,
during softball tryouts, she noted chest pain and dyspnea with
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running. She was seen by her primary care physician and
diagnosed with musculoskeletal pain, pleurisy and exercise-
induced asthma. By October of 2014, she could not walk up
one flight of stairs. Further workup revealed a pulmonary
embolus and a right subclavian DVT.She was initially placed
on warfarin, however, she developed a right hemothorax in
the area of the right lower lobe infarct, which required chest
tube placement and had approximately 1.5L drained.
During the same hospitalization, a right subclavian veno-
gram showed a nonocclusive thrombus directly adjacent to
the first rib consistent with Paget-Schroetter syndrome. On
right heart catheterization, her pulmonary pressures were
70/20 with a mean of 40 with an echocardiogram revealing
right atrial and right ventricular enlargement along with
RVH. She also developed an occlusive thrombus involving
her left basilic vein at a previous IV site despite having thera-
peutic INRs and was deemed a “coumadin failure”. She was
eventually discharged home on Eliquis.
At the time of her evaluation 2months later at a special-
ized center for CTEPH patients, she was experiencing WHO
functional class III symptoms. She could walk for approxi-
mately 25ft and not quite half a flight of stairs before experi-
encing dyspnea. She complained of chest pain with
overexertion and had some lightheadedness when bending
forward. She denied syncope, cough, hemoptysis, lower
extremity edema. She was started on diuretics due to liver
congestion and was also placed on supplemental oxygen at
2L during sleep and with exertion.
No family history of blood clots or bleeding disorders. She
denies tobacco use, alcohol and illicit drug use.
Her chest radiograph revealed blunting of the right costo-
phrenic angle with peripheral scarring of the right upper lobe.
The right atrium was enlarged and the central pulmonary
arteries were prominent (Fig.6.1).
Ventilation perfusion scan showed perfusion defects to the
apical posterior segments of the left upper lobe, superior lin-
gula, and the anterobasal and lateral basal segments of the
left lower lobe. In the right lung there are defects in the right
upper lobe, right middle lobe and basal segments of the right
lower lobe (Fig.6.2).
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F . Preoperative chest radiograph showing a prominent
right atrial shadow, with central PA enlargement
WASH IN
ANT Q
LT LAT Q
RT LAT Q
LAO
QR
AO Q
POST QLPO Q
RPO Q
WASHOUT1 WASHOUT2EQUIL
F . Preoperative lung scintigraphy demonstrating bilateral
mismatched perfusion defects
Chapter 6. Case 6: Distal Vessel ......
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An echocardiogram revealed RA and RV severely enlarged
with depressed RV systolic function. LV size is small, with
normal systolic function, TR velocity 4.5m/s with PA systolic
pressure 82mmHg plus CVP, cardiac output 4.4L/min, mild
tricuspid regurgitation, absent pericardial effusion.
Laboratory data was as follows: Sodium 142, potassium 4.0,
chloride 103, bicarbonate 24, BUN 15, creatinine 1.14, blood
glucose 99. Liver function tests within normal limits except
total bilirubin mildly up at 1.54. Urinalysis was negative for
protein. White blood cell count 6.9, hemoglobin 13.6, hemato-
crit 41.5, platelets 259,000. INR 1.3, PTT 39.6.
Right heart catheterization showed right atrial mean pres-
sure 10mmHg, PA pressure 101/28 with a mean of 58mmHg,
Pulmonary capillary wedge pressure 13mmHg, Cardiac out-
put 3.30L/min, cardiac index 1.95L/min/m
2
, Pulmonary vas-
cular resistance 1091dynes/sec/cm
-5
.
Pulmonary angiogram on the right side demonstrated a
“pouch defect” in the right lower lobe; webs in the right
upper lobe apical and anterior vessels. On the left side, the
anterior medial vessels to the lower lobe were narrowed at
the distal segmental and subsegmental level with a reduction
in vascularity to the posterior LLL.Otherwise, there was a
“pouch defect” in the superior segment, proximal occlusion
of the lingula and a web in posterior left upper lobe (Fig.6.3).
She was ultimately determined to have operable CTEPH
and underwent pulmonary thromboendarterectomy with a
total circulatory arrest time of 67min; 35min for the right side
and 32 min for the left. Postoperative hemodynamics were
CVP 12, PAP 47/18 (28), mean 19mmHg, CO 5.30L/min, CI
3.2L/min/m
2
and estimated PVR 256dynes/s/cm
-5
(Fig.6.4).
In the absence of major postoperative difficulties, this
patient was discharged from hospital on postoperative day 9,
with the recommendation for lifelong anticoagulation.
Subsequent hematologic evaluation showed her to have
“elevated PAI-1 levels”. Over the 3years following her sur-
gery, she has returned to a normal functional status (high
school activities, lifting weights, running), and able to engage
in her first year of university studies.
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