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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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35
no edema, clubbing or cyanosis of her extremities and periph-
eral pulses were intact without bruits.
Laboratory findings were notable for an NT-pro BNP of
2793pg/mL, Hgb of 15.3g/dL and serum creatinine of 1.0mg/
dL.Hypercoagulable work up was negative.
Ventilation perfusion imaging (Fig.4.1) demonstrated nor-
mal ventilation with multiple, large perfusion defects in the
bilateral lung fields.
Echocardiogram revealed an RVSP of 82 mmHg, a severely
enlarged right ventricle, a trivial pericardial effusion and a
TAPSE of 1.3cm. Hemodynamics obtained during right heart
catheterization were: mean RA pressure 12 mmHg, PAP
83/34 mmHg, mean PA pressure 52 mmHg, PCWP 10mmHg.
Fick CO 2.63 L/min (CI 1.54 L/min/m
2
), thermodilution CO
2.77L/min (CI 1.62L/min/m
2
) and PVR 16.2Wood Units. Testing
with inhaled nitric oxide was negative for vasoreactivity.
A non-selective digital subtraction angiogram was per-
formed (Fig.4.2) to evaluate her lung scan findings and dem-
onstrated features atypical for CTEPH.
WASH IN
ANT Q
LT LAT Q RT LAT QLAO QRAO Q
POST Q LPO QRPO Q
WASHOUT1WASHOUT2EQUIL
F . Lung scintigraphy demonstrating multiple unmatched
perfusion defects in both lungs
Chapter 4. Case 4: TheCase forBalloon Pulmonary....
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36
While there were several features suggestive of chronic
thromboembolic disease on her pulmonary angiogram, the small
vessel caliber size throughout, along with a discordance between
the degree of pulmonary hypertension and her “thrombus bur-
den” suggested an atypical presentation for a pulmonary vasculi-
tis. (see Case) However, an MR angiogram of the systemic great
vessels was obtained and showed no evidence for vasculitis in the
aorta, mesenteric, renal, subclavian or carotid arteries.
Even with vasculitis ruled out, the atypical and discordant
angiographic features were to such a degree that it was
assessed endarterectomy surgery might offer little benefit
and, with her degree of right heart compromise, carry a high
procedural mortality. The decision was made to start combi-
nation oral pulmonary hypertension targeted therapy with
riociguat and macitentan followed by balloon pulmonary
angioplasty (BPA).
Approximately 3 weeks after initiating macitentan and
riociguat the patient returned for the first session of balloon
pulmonary angioplasty. Her dyspnea on exertion was mod-
estly improved but she remained NYHA functional class
III.NT-pro BNP had improved to 1022pg/mL and she walked
360m on 6min walk testing. Repeat right heart catheteriza-
tion immediately prior to the first BPA session demonstrated
the benefits of her PAH targeted medical therapy. Her mean
PA pressure had decreased to 45mmHg and her Fick CO had
increased to 4.26L/min (CI 2.52L/min/m
2
) and thermodilu-
F . Pre-intervention pulmonary digital subtraction pulmo-
nary arteriogram
D. S. Poch et al.
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37
tion CO 3.77L/min (CI 2.22L/min/m
2
), resulting in a reduc-
tion of PVR from 16 to 9–10 Wood Units. Selective angiography
revealed very small caliber vessels in every lung segment.
Most of her lower lobe segmental vessels were no more than
2 mm in diameter prior to BPA (Fig.4.3). The approach to
initial BPA sessions was necessarily cautious given her severe
pulmonary hypertension and small caliber of her vessels.
After eight sessions of balloon pulmonary angioplasty, all
PH targeted therapies had been discontinued and the patient
was NYHA Functional Class II.Right heart catheterization
prior to her ninth and final session of BPA revealed PA
49/16mmHg, mean PA pressure 30 mmHg Fick CO 5.81L/
min (CI 3.28L/min/m
2
), thermodilution CO 4.73L/min (CI
2.67L/min/m
2
) and a PVR of 3.4–4.2Wood Units. Follow-up
lung scintigraphy (Fig.4.4) revealed areas of improved perfu-
sion, particularly in her lower lobes.
Radiographic Interpretation
Frontal and lateral views of the selective pulmonary angio-
gram demonstrated that the segmental branches bilaterally
were of generally small caliber with attenuation as the vessels
reach the mid third of the lungs. There was a generalized pau-
city of vasculature in the peripheral third of the lungs with
lack of sub-pleural perfusion in the parenchymal phase, this
slightly more prominent on the right. A “pouch lesion” was
present in the right middle lobe with a thin trickle of contrast
medium extending from the convexity. There were segmental
stenoses in the medial, anterior and lateral basal right lower
lobe and anterior branch of the left lower lobe (Fig.4.2).
The right lower lobe stenoses were much better delineated
on the dedicated super-selective angiogram performed prior
to balloon pulmonary angioplasty. Post-angioplasty, there was
angiographic improvement, with restoration of flow beyond
the diseased segments (Fig.4.3). This improvement was mir-
rored on the perfusion scintigraphy with resolution of some
of the perfusion defects when compared to the pre-treatment
scintigram (Fig.4.4).
Chapter 4. Case 4: TheCase forBalloon Pulmonary....
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38
F . Immediate increase in vessel density to right lower lobe
after BPA session
ANT Q
a
b
POST Q LPO Q RPO Q
LT LAT Q RT LAT Q LAO Q RAO Q
F . Pre- (panel a) and Post (panel b) BPA lung scintigraphy
showing an improvement in perfusion primarily in the lower lobes
(arrows)
D. S. Poch et al.
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39
Clinical Comments
Published guidelines, and clinical practice at most expert
CTEPH centers have advocated that balloon pulmonary
angioplasty should be reserved for those CTEPH patients
with lesions felt to be inoperable, or in those patients with
co-morbidities that preclude surgical consideration. It was
only after discussion with a multidisciplinary team of experts
skilled in the surgical approach to CTEPH that this patient
was excluded from PTE surgery. Even after MRI made the
diagnosis of vasculitis less likely, the unusual pulmonary
angiographic features raised significant concern that PTE
would be technically challenging given the small caliber of
vessels and offer limited hemodynamic benefit. There is
clearly some subjectivity that led to this decision. Challenging
cases such as this emphasize that PTE operability decisions
are best handled by multidisciplinary teams, experienced in
the evaluation and management of the CTEPH patient.
This case further stresses the step-wise approach to angio-
plasty in establishing meaningful endpoints. Though there
remained numerous…and large …perfusion defects on lung
scintigraphy, the patient achieved significant hemodynamic
improvement off all PH targeted medical therapy, improve-
ment in Nt Pro BNP, improvement in 6min walk testing and
dramatic improvement in functional status.
Point ofEmphasis
For those patients with inoperable chronic thromboembolic
pulmonary hypertension, balloon pulmonary angioplasty can
be a viable option to provide a clinically meaningful improve-
ment in pulmonary hemodynamics and functional status.
Diagnosis
1. Inoperable chronic thromboembolic pulmonary
hypertension.
Chapter 4. Case 4: TheCase forBalloon Pulmonary....
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40
Further Reading
Mahmud E, Madani MM, Kim NH, et al. Chronic thromboem-
bolic pulmonary hypertension: evolving therapeutic approaches
for operable and inoperable disease. J Am Coll Cardiol.
2018;71(21):2468–86.
Ogawa A, Satoh T, Fukuda T, Sugimura K, Fukumoto Y, etal. Balloon
pulmonary angioplasty for chronic thromboembolic pulmonary
hypertension: results of a multicenter registry. Circ Cardiovasc
Qual Outcomes. 2017;10(11):pii:e004029.
Ogo T. Balloon pulmonary angioplasty for inoperable chronic
thromboembolic pulmonary hypertension. Curr Opin Pulm Med.
2015;21(5):425–31.
D. S. Poch et al.
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41© 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_5
Case Presentation
A 66-year old male presents to the hospital with increased dys-
pnea and was admitted to the hospital with acute on chronic
hypoxemic respiratory failure. He had been on his usual 3–4L/
min of oxygen by nasal cannula but just could not catch his
breath. He denied fever, wheezing or a productive cough.
Chapter 5
Case 5: Operable CTEPH …
but Is PTE theBest Option?
SonjaBartolome, DeepaGopalan, andWilliamR.Auger
S. Bartolome (*)
CTEPH Program, UT Southwestern Medical Center,
Dallas, TX, USA
Pulmonary and Critical Care Medicine, UT Southwestern Medical
Center, Dallas, TX, USA
e-mail: Sonja.Bartolome@UTSouthwestern.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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In the emergency department a 100% oxygen nonrebreather
mask was required to maintain oxygen saturations >90%. On
physical exam he had a pulse of 112, was tachypneic at 22
breaths per minute, blood pressure was 118/73 and he was
afebrile. His lung exam revealed no wheezing but a few rales
in the right base and a pulmonary flow murmur on the right.
His cardiac exam revealed a markedly increased pulmonic
component of the second heart sound, a grade 2/6 systolic
murmur at the right upper sternal border, a right ventricular
S3, and a right ventricular heave. He had JVD to the jaw and
2+ lower extremity pitting edema to the knees. He had a nor-
mal white blood cell count, his hemoglobin was elevated at
17.6mg/dL, platelet count was normal, electrolytes and renal
function were normal and an N-terminal proBrain natriuretic
peptide was 5354 pg/mL. A portable chest X-ray was per-
formed (Fig.5.1) which revealed marked cardiomegaly and
large pulmonary arteries. Lasix was administered and he was
admitted to the intensive care unit.
Prior to presentation, he was unable to walk through large
grocery stores without stopping. However, he drives, cares for
himself, and does his other activities of daily living. He is
F . Admission chest radiograph demonstrating cardiomeg-
aly and massively enlarged central pulmonary arteries
S. Bartolome et al.
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43
using 3L oxygen by nasal cannula at all times but his oxygen
saturations drop to the 70s when walking short distances.
Recently in clinic his 6-min walk test showed a distance of
134 m with oxygen saturations 80–86% on 25 L per nasal
cannula.
His pulmonary function testing reveals an FEV1 of 1.89L
(54% of predicted), FVC of 3.66L (78% predicted), and an
FEV1/FVC ratio of 51%. The patient was unable to perform
the lung volume or DLCO maneuver due to dyspnea and
hypoxemia.
By provided history, at age 19 he was diagnosed with a pul-
monary embolism after presenting with hemoptysis and was
anticoagulated for several months but then enlisted in the
United States Army and stopped his warfarin. He stayed off
of the warfarin until 1990 when he presented with dyspnea
and was found to have an “acute PE in the main pulmonary
artery.” He again was treated with warfarin but only took it for
1–2years and then was lost to follow-up. He presented to a
health care provider for routine care and was restarted on
anticoagulation in 2000 and has been on it ever since. He has
been a heavy smoker for the past 30years and was diagnosed
with chronic obstructive pulmonary disease (COPD) a decade
ago by pulmonary function testing. He started using oxygen
about 8years ago. The patient has had frequent admissions for
COPD exacerbation characterized by productive cough, dys-
pnea, wheezing and an increased oxygen requirement. At the
time of each episode he is treated with antibiotics and steroids
and improves. His pulmonologist noted he had pulmonary
hypertension, and recognized that it might be related to
chronic thromboembolic disease after a ventilation/perfusion
scan and computed tomography of the chest were obtained.
He was started him on an oral prostacyclin approximately
8months prior to presentation. The patient felt much worse
on this and his oxygen requirement had been increasing.
Other past medical history included a gastric ulcer, chronic
atrial fibrillation, gout, a chainsaw accident with a hand injury
and squamous cell skin cancer. He has been evaluated for a
thrombophilia but none was found.
Chapter 5. Case 5: Operable CTEPH …but Is PTE....
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44
Social History is notable for smoking 3/4 pack per day for
30years. Upon presentation he was smoking e-cigarettes. He
has a significant alcohol history and consumed at least 20
cans of beer per week for 30years. He now drinks 1–2 beers
per week. He worked in oil field, drilling. In the Army, he was
a transportation security gunner.
Family history was significant for coronary artery disease
but no clotting disorders, pulmonary emboli or deep vein
thrombosis.
A ventilation perfusion scan was performed. There were
segmental V/Q mismatches in the anterior left upper lobe,
the lingula, and to a lesser extent, in the LLL.Overall perfu-
sion to the right lung was reduced sparing a small region of
the anterior right lower lobe (Fig.5.2).
A CT angiogram was performed revealing large pulmo-
nary arteries with lining, calcified clot in the main pulmonary
arteries and bilateral segmental branches, and an enlarged
right atrium and ventricle (Fig.5.3).
High resolution computed tomography of the chest was
then obtained due to his history of parenchymal lung disease.
This revealed centriacinar and paraseptal emphysema,
dilation of the main pulmonary artery with calcification along
F . Abnormal perfusion scintigraphy with distinct segmen-
tal defects involving primarily the left lung
S. Bartolome et al.
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