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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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placed on 5L oxygen via nasal cannula and transferred to our
institution for further management.
His past medical history was also significant for ongoing
smoking with over 20 pack year history of tobacco abuse. He
denied any history suggestive of connective tissue disease,
liver or kidney disease, malignancy, thyroid or hematological
disorders, or other risk factors for pulmonary hypertension.
His EKG showed right axis deviation with borderline right
ventricular hypertrophy in a sinus rhythm. V/Q perfusion
images revealed large mismatched defects bilaterally
(Fig.1.4).
Right heart catheterization showed significantly elevated
right-sided filling pressures with a right atrial (RA) pressure
of 20mmHg, a PAP of 71/33mmHg (mean 45) and a pulmo-
nary capillary wedge pressure of 11mmHg. His cardiac index
was marginal at 2.2L/min/m
2
and his calculated pulmonary
vascular resistance (PVR) was 9.1 Wood Units. Pulmonary
angiogram showed lack of perfusion in his right lower and
middle lobes with multiple other findings consistent with
CTEPH (Fig.1.5a, b). Coronary angiogram revealed no sig-
nificant obstructive disease.
His pulmonary function tests showed moderate obstruc-
tive lung disease with an FEV1 of 2.1L.
RT ANTERIOR LT
RT LATERAL LT LATERAL
LT POSTERIOR RT RT RAO LT
LT RPO RT RT LAO LT
LT LPO RT
F . Preoperative lung perfusion scintigraphy
M. Kanwar et al.
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He was admitted for aggressive diuresis, and given his
hemodynamic presentation we used inotropic support with
IV milrinone for 72h prior to surgery for volume and right
ventricular optimization. After obtaining informed consent,
he underwent pulmonary thromboendarterectomy (PTE)
surgery with the right side circulatory arrest time of 32 and
16min on the left side (Fig.1.6).
He was extubated the following morning and weaned off
of his inotropic support over the next 3days. Off support, his
postoperative PA pressures were RA 6mmHg, PA 33/9mmHg
(mean 19), PCWP 7mmHg, CI 2.7L/min/m
2
, PVR 2.7Wood
Units, BP 107/82. He was discharged home 7days later and
had a follow-up right heart catheterization 6 months later,
F . (a, b) Catheter-based pulmonary angiography illustrat-
ing features of chronic thromboembolic disease. Angiographic web
depicted by solid white arrow
Chapter 1. Case 1: The Evaluation of Chronic…
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which demonstrated a mean PAP of 23 and a PVR of
2.8 Wood Units. He continues to do well at his 1 year
follow-up.
Radiological Interpretation
A more detailed interpretation of the radiographic studies
reveals that the chest radiograph in Fig.1.1 is remarkable for
cardiomegaly and asymmetric dilatation of the proximal
pulmonary arteries (right is larger than the left) with reduced
pulmonary vascular markings in the periphery.
VQ scintigraphy (Fig.1.4) reveals lobar perfusion defects
present in the right lower lobe and lingula, with wedge
shaped segmental perfusion defect in the left lower lobe.
Ventilation was homogenous in both lungs.
Computerized Tomography Pulmonary Angiography
(CTPA) (Fig.1.3) shows intimal thrombus in the distal right
F . Clot specimen removed at the time of pulmonary throm-
boendarterectomy
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main pulmonary artery with occlusion of the right lower lobe.
On the left, there is eccentric intimal thrombus around the
distal left main pulmonary artery with proximal occlusion of
the lingula. There is attenuation of the segmental branches of
the left lower lobe with loss of peripheral arcades. Right
atrium and right ventricle are markedly dilated with flatten-
ing of the interventricular septum. There is enlargement of
the bronchial arteries (solid white arrow) and small pericar-
dial and pleural effusions.
Catheter pulmonary angiography (Fig. 1.5a, b) demon-
strates intimal irregularity of the distal right main pulmonary
artery and proximal right lower lobe vessel with a proximal
web (solid white arrow) followed by a pouch defect with a
thin trickle of contrast medium. The right middle lobe vessel
is occluded. There is a proximal stenosis in the origin of the
posterior segment of the right upper lobe with post-stenotic
dilatation. On the left, intimal thrombus is present in the dis-
tal left main pulmonary artery with segmental occlusion of
the lingula, and abrupt truncation of the anterior basal
branch of the left lower lobe with mid segmental attenuations
of the posterior and lateral basal segments. Note that in the
capillary phase there is good perfusion in the left upper lobe
and lack of perfusion in the lingula and left lower lobe.
Clinical Comments
This case is an illustration of a “typical” approach to the
evaluation of CTEPH, though it did not follow the diagnostic
guidelines that have been promoted over the past years
(Fig.1.7). With the background history of pulmonary embolic
events, and his presentation showing volume overload,
screening for the presence of pulmonary hypertension and
right heart disease with echocardiography was in line with the
recommendations as depicted in Fig.1.7. However, it can be
argued that obtaining CT angiography of the chest to investi-
gate the possibility of recurrent pulmonary emboli was com-
pletely appropriate. The fact that the diagnosis of chronic
Chapter 1. Case 1: The Evaluation of Chronic…
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thromboembolic disease was established with this study can
be attributed to an appropriate radiographic interpretation,
and when all the data were considered, the clinical picture
was that of operable CTEPH.The additional benefit of CT
was its effectiveness in ruling out coexisting mediastinal or
parenchymal lung disease, along with competing diagnoses
involving the pulmonary vessels such as a sarcoma (Chap. 12).
In order to completely delineate or “map-out” this patient’s
chronic thromboembolic disease, lung scintigraphy and
catheter- based pulmonary angiography were performed. To
better establish surgical risk, pulmonary function studies (to
rule out co-existing airways disease) and right heart catheter-
Symptoms, signs, history suggestive of CTEPH
Echocardiographic probability of PH (Table 8)
High or intermediate probability of PH
CTEPH possible
CTEPH ruled out
Work-up for PH/PAH
(Figure 1)
Refer to PH/CTEPH
expert centre
CT pulmonary angiography
Right heart catheterization
+/- Pulmonary angiography
V/Q scan
a
Mismatched perfusion defects?
Yes
CT = computed thomography;CTEPH = chronic thromboembolic pulmonary hypertension;
PAH = pulmonary arterial hypertension;PH = pulmonary hypertension;
V/Q = ventilation/perfusion.
a
CT pulmonary angiography alone may miss diagnosis of chronic thromboembolic
pulmonary hypertension.
No
F . CTEPH diagnostic algorithm. Galiè et al. 2015 ESC/
ERS guidelines for the diagnosis and treatment of pulmonary
hypertension. Eur Respir J 2015; 46:903
M. Kanwar et al.
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ization were obtained to complete the evaluation. In many
centers performing PTE surgery, coronary angiography is
additionally obtained in certain age groups or in patients with
CAD risk factors as it is prudent to surgically address signifi-
cant coronary vascular disease when present.
The recommended diagnostic guidelines have been pro-
moted to emphasize a number of key points. It has been
asserted that lung scintigraphy is the screening study of choice
for CTEPH. To a large extent this is based on a study by
Tunariu and colleagues (2007) which demonstrated a greater
sensitivity of lung scintigraphy in the detection of CTEPH
compared to CT angiography. Though subsequent investiga-
tions performed at CTEPH experienced centers have chal-
lenged these findings (see He et al. 2012) it has been
consistently observed that distal vessel chronic thromboem-
bolic disease can be difficult to detect with CT by inexperi-
enced diagnosticians. Consequently, the finding of an abnormal
lung perfusion study even with a “negative for PE” CTA war-
rants further evaluation for chronic thromboembolic disease
and CTEPH.And although lung scintigraphy lacks specificity
for CTEPH, a “negative study” showing a normal perfusion
pattern effectively rules out operable CTEPH.The algorithm
in Fig. 1. 7 further suggests that in the setting of suspected
CTEPH, seeking the advice of a group with experience in the
diagnosis and management of this disease can be helpful.
Confirming the diagnosis, establishing surgical candidacy, and
sorting through optimal treatment options can be
challenging.
Points ofEmphasis
For an at-risk patient and for those with unexplained exer-
tional dyspnea and exercise intolerance…no matter what
age….pulmonary vascular disease and/or CTEPH should be
explored. The establishment of CTEPH as a diagnosis can
lead to a potential cure with a surgical endarterectomy.
Chapter 1. Case 1: The Evaluation of Chronic…
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Diagnosis
1. Operable chronic thromboembolic pulmonary hypertension.
Further Reading
Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, etal. 2015 ESC/
ERS guidelines for the diagnosis and treatment of pulmonary
hypertension. The joint task force for the diagnosis and treat-
ment of pulmonary hypertension of the European Society of
Cardiology (ESC) and the European Respiratory Society (ERS).
Eur Respir J. 2015;46:903–75.
Gopalan D, Blanchard D, Auger WR. Diagnostic evaluation of
chronic thromboembolic pulmonary hypertension. Ann Am
Thorac Soc. 2016;13(Suppl 3):S222–39.
He J, Fang W, Lv B, He JG, Xiong CM, Liu ZH, He ZX. Diagnosis
of chronic thromboembolic pulmonary hypertension: comparison
of ventilation/perfusion scanning and multidetector computed
tomography pulmonary angiography with pulmonary angiogra-
phy. Nucl Med Commun. 2012;33(5):459–63.
Renapurkar RD, Shrikanthan S, Heresi GA, Lau CT, Gopalan
D.Imaging in chronic thromboembolic pulmonary hypertension.
J Thorac Imaging. 2017;32(2):71–88.
Tunariu N, Gibbs SJR, Win Z, Gin-Sing W, Graham A, Gishen P,
AL-Nahhas A.Ventilation-perfusion scintigraphy is more sen-
sitive than multidetector CTPA in detecting chronic thrombo-
embolic pulmonary disease as a treatable cause of pulmonary
hypertension. J Nucl Med. 2007;48:680–4.
M. Kanwar et al.
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11© 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_2
Chapter 2
Case 2: There’s No
Perfusion tothat Lung!
AnUnusual Presentation
forCTEPH
MonaAlotaibi, VictorPretorius, DeepaGopalan,
andWilliamR.Auger
M. Alotaibi
Pulmonary Fellow, Division of Pulmonary and Critical Care
Medicine, University of California, San Diego, San Diego, CA, USA
V. Pretorius (*)
Division of Cardiovascular and Thoracic Surgery, University
of California, San Diego, San Diego, CA, USA
Heart Transplant and MCS Program, University of California,
San Diego, San Diego, CA, USA
e-mail: gpretorius@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
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12
Case Presentation
A 56-year-old white female presented with bilateral humeral
fractures after a mechanical fall and worsening exertional dys-
pnea. She was hypoxemic in the emergency department with
oxygen saturations of 84% on room air normalizing with 2L
nasal cannula O
2
supplementation. Vital signs showed a pulse
of 82, BP of 147/95, respiratory rate of 18, and a temperature of
98.6 °F. Physical exam was notable for normal first heart sound,
loud P component of second heart sound and grade I/VI holo-
systolic murmur at the left lower sternal boarder. No dullness
to percussion of the lungs was appreciated. No pulmonary
bruit was auscultated. Lower extremities showed bilateral pit-
ting edema, without clubbing or cyanosis. An initial troponin
was 0.01 μg/L. N-Terminal proBrain natriuretic peptide
(NT-proBNP) was 467pg/mL. An electrocardiogram (ECG)
revealed a normal sinus rhythm. A chest x-ray (Fig.2.1) was
F . Frontal and lateral chest radiographs show asymmetri-
cally enlarged left pulmonary artery, small right pulmonary artery; a
hypovascular right lung with a relatively smaller right hemithorax
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read as showing an enlarged left pulmonary artery with diffuse
bronchovascular markings; in addition, there was right lung
volume loss with a reduction in vascular markings.
Past medical history included left leg DVT and pulmonary
embolism in 1987 with a placement of IVC filter and has been
on anticoagulation with warfarin ever since. Her breathing
never returned to baseline since. She has history of heparin
induced thrombocytopenia (HIT), anti-thrombin 3 deficiency,
and hypertension.
Social history was notable for no alcohol, illicit drug use or
tobacco use.
An initial echocardiogram revealed mild right atrial dila-
tion, mild right ventricular dilation and dysfunction with mild
tricuspid regurgitation and a pulmonary artery systolic pres-
sure (PASP) estimate of 32mmHg. The left atrium was mildly
dilated. The left ventricle was normal in size and systolic
function. No pericardial effusion was present. Bubble con-
trast administration revealed no evidence of shunting.
Serologies for connective tissue disease and human immuno-
deficiency virus were negative. Spirometry tests revealed a
forced expiratory volume of 1.96L (84% of predicted), forced
vital capacity of 2.37 L (76% of predicted), with a ratio of
80%. A ventilation-perfusion scan was performed and
revealed non- perfusion of the entire right lung (Fig.2.2).
A computerized tomography pulmonary angiogram
revealed abrupt termination of the distal right main pulmonary
artery with unopacification of more distal right pulmonary
arteries suggestive of chronic thromboembolism (Fig.
2.3).
Right heart catheterization revealed a right atrial mean
pressure (RAP) of 12 mmHg, pulmonary artery pressure
(PAP) of 70/31 with a mean of 47mmHg, a pulmonary wedge
pressure (PAWP) of 21mmHg, left ventricular end diastolic
pressure (LVEDP) of 11 mmHg, cardiac output (CO) of
7.20 L/min, cardiac index (CI) of 3.32 L/min, resulting in a
pulmonary vascular resistance (PVR) of 3.6 Wood units.
Consultation with the pulmonary hypertension specialist
resulted in a diagnosis of WHO group 4 chronic thromboem-
bolic pulmonary hypertension (CTEPH).
Chapter 2. Case 2: There’s No Perfusion tothat Lung…
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