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182
Accompanying VQ scintigraphy appears abnormal in both
the ventilation and perfusion components. There are multiple
segmental and subsegmental mismatched perfusion defects,
but in a pattern atypical for that seen in pulmonary embolic
disease. Ventilation is diffusely abnormal on both sides.
The follow up CTPA (Figs.19.4 and 19.5) shows eccentric
clot lining the right main pulmonary artery and “straddling”
across the main and left pulmonary artery. No clot was pres-
ent in the segmental and subsegmental vasculature. There is
mild dilatation of the main pulmonary artery compared to
the adjacent ascending aorta. Lung windows show severe
centrilobular emphysema involving both lungs with large
areas of lung destruction, this worse in the right lung.
These proximal vessel findings are most likely secondary
to in-situ thrombus that has developed as a result of altered
blood flow in areas of destroyed lung as a consequence of
gross emphysema. The presence of central of central clot with
normal or even large segmental vessels should raise the sus-
picion of in-situ thrombus.
Clinical Comments
The possibility that this patient’s pulmonary hypertension
was secondary to chronic thromboembolic disease was rooted
in his initial presentation of acute dyspnea. Though his symp-
toms to a large extent were attributable to his infiltrates and
pneumothorax—there was also a documented DVT and
radiographic evidence for acute pulmonary emboli. And
despite appropriate treatment of these entities, including a
12month course of anticoagulants, he failed to return to his
prior clinical status. The discovery of pulmonary hyperten-
sion by echo, and confirmed by right heart catheterization to
be beyond what might be expected from COPD alone, raised
concerns that another problem might be contributing to his
clinical decline. And with unmatched perfusion defects by
lung scintigraphy, and the finding of eccentric lining throm-
bus on CT, considering chronic thromboembolic disease as a
factor in his ongoing difficulties was reasonable.
L. Brown et al.
183
But despite what might be interpreted as a significant
thrombus burden in the proximal pulmonary vessels, the
assessment as to whether or not thromboendarterectomy
surgery is appropriate should follow consideration of two
observations—that the lining thrombus may not be the prin-
cipal problem, and there may be no clinical value in attempt-
ing to re-perfuse severely damaged lung parenchyma. As has
been discussed with other cases in this book (see Chaps. 19
and 23), the presence of central lining thrombus is not the
reason for pulmonary hypertension in most cases. As Dr.
Gopalan has suggested, the mechanism behind the develop-
ment of lining thrombus is not entirely understood, but is
likely a result of an aberrant flow pattern along the vessel
wall. In particular, this is the probable mechanism in extremely
dilated proximal pulmonary arteries. And not only can it
occur in the context of chronic lung destruction as in this case,
but can also be seen in dilated central vessels associated with
congenital heart disease such as atrial or ventricular defects.
Most notable is the concept that attempted re-perfusion of
damage lung parenchyma—be it emphysema or fibrosis—is
never a clinically sound decision. Though some of the CT and
VQ abnormalities in this case might have been on the basis
of segmental level chronic thromboembolic disease, an end-
arterectomy of the vessels supplying lung regions with exten-
sive emphysema would not have provided clinical benefit and
would have only added risk to an already risky operation.
Having said this, however, on occasion there are patients with
primarily upper lobe emphysema who present with chronic
thromboembolic disease involving vessels supplying relatively
“preserved” lung parenchyma. An endarterectomy of these
vessels can restore perfusion-ventilation “matching” and
prove to be clinically beneficial.
Points ofEmphasis
Thrombendarterectomy in a patient with extensive parenchy-
mal lung disease requires careful assessment of the limited
pulmonary hemodynamic and clinical benefits that can be
Chapter 19. Case 19: Parenchymal Lung Disease…
184
achieved with this approach. Lining thrombus in the central
pulmonary arteries can be observed in such patients.
Diagnoses
1. Emphysema.
2. Lining thrombus involving the proximal pulmonary
arteries.
Further Reading
Moser KM, Fedullo PF, Finkbeiner WE, Golden J.Do patients with
primary pulmonary hypertension develop extensive central
thrombi? Circulation. 1995;91:741–5.
Perloff JK, Hart EM, Greaves SM, etal. Proximal pulmonary arte-
rial and intrapulmonary radiologic features of Eisenmenger
syndrome and primary pulmonary hypertension. Am J Cardiol.
2003;92:182–7.
L. Brown et al.
185
© 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
A
Acute respiratory failure, 122
Atrial septal defect
cardiac magnetic resonance imaging, 171, 172
chest radiograph, 168, 170
CTA, 168, 169, 172
diagnosis, 175
historical and radiographic aspects, 174
lung scintigraphy, 168, 171
multiplanar CT reconstructions, 174
perfusion scintigram, 175
pulmonary hypertension development, 174
SV-ASD, 173
VQ scintigraphy, 172
B
Balloon pulmonary angioplasty
diagnosis, 39
guidelines and clinical practice, 39
lung scintigraphy, 38
post-angioplasty, 37
pulmonary angiogram, 37
pulmonary digital subtraction pulmonary arteriogram, 36
Raynaud’s phenomenon, 34
Index
186
Balloon pulmonary angioplasty (cont.)
ventilation perfusion imaging, 35
vessel density, 38
Bilateral unmatched perfusion defects, 136
C
Catheter pulmonary angiography, 48, 138
acute versus chronic thromboembolic disease, 154
CTED, 68
CTEPH, 5, 7, 15
Central pulmonary arteries, 42
Chest CT angiogram, 103, 104
Chest radiograph, 12
atrial septal defect, 168, 170
CTED, 63, 67
CTEPH, 2, 47
distal vessel thromboendarterectomy, 52, 53, 56
parenchymal lung disease and chronic thromboemboli, 179, 181
pulmonary arteritis, 125
pulmonary veins, 100, 104
sarcoidosis and large vessel pulmonary vascular disease, 134, 138
Chest x-ray
pulmonary arterial pulmonary hypertension mimic CTEPH, 158,
159
pulmonary artery sarcoma, 84, 87
Chronic obstructive pulmonary disease (COPD), 43, 101, 182
Chronic pulmonary embolism, 142
Chronic thromboembolic disease (CTED)
acute vs.
catheter pulmonary angiography, 154
clinical history, 148
CT angiogram, 149, 150
CTPA, 153
diagnoses, 156
duplex venous ultrasound, 149
echocardiogram, 148
family history, 148
intraluminal lling defects, 151, 152
lung ventilation-perfusion scan, 150, 151
perfusion pattern, return of, 152, 153
physical examination, 148
pulmonary hemodynamic prole, 155
transthoracic echocardiogram, 148, 149
cardiovascular exam, 179
catheter pulmonary angiography, 68
chest radiograph, 63, 67, 179, 181
clinic evaluation, vital signs, 72
Index
187
computed tomography pulmonary angiography, 72
CT angiogram, 73, 75, 178, 180, 181
CT pulmonary angiography, 78
diagnosis, 69, 80, 184
digital subtraction pulmonary arteriogram, 64, 65
distal vessel thromboendarterectomy, 57
family history, 62
laboratory assessment, 179
laboratory data, 63
lung scintigraphy, 64, 180
medical history, 61, 62, 71
minute ventilation, 65
organized thrombus endarterectomized at surgery, 77
pulmonary thromboendarterectomy, 77
residual perfusion defects, 68
surgery, 69
surgical outcomes, 79
ventilation perfusion scintigraphy, 73, 74, 78
VQ scintigraphy, 67, 78
Chronic thromboembolic pulmonary hypertension (CTEPH)
calcied thrombus lining, central pulmonary vessels, 45
catheter pulmonary angiography, 5, 7, 48
calcied thrombus lining, central pulmonary vessels, 45
chest radiograph, 2, 42, 47
chronic thromboembolic disease, radiographic evidence for, 145
clot specimen removing, 5, 6
COPD, 43
CT angiography, 3, 7, 142
CT scan, 49
CTPA, 7, 47
diagnosis, 10, 49, 146
diagnostic algorithm, 7, 8
diagnostic guidelines, 9
disease distribution, 48
dyspnea, 41
echocardiogram, 2, 142
exertional dyspnea and functional disability, 141
extensive parenchymal lung disease, 44, 46
features of, 143
lining clot, 144
lining thrombus within enlarged central pulmonary vessels, 146
lung perfusion scan, 143, 144
lung scintigraphy, 145–146
medical history, 43
negative study, 9
oral prostacyclin therapy, 47
PAH with IST, 144
Index
188
Chronic thromboembolic pulmonary hypertension (CTEPH) (cont.)
physical exam, 42
preoperative lung perfusion scintigraphy, 4
pulmonary arteriogram, 46
pulmonary embolism, 43
transplant evaluation, 47
TTE, 2, 3
unusual presentation for
catheter based pulmonary angiogram, 15
computerized tomography pulmonary angiogram, 13
CT angiography, 14, 17
diagnosis, 19
distal arteriopathy, 18
frontal and lateral chest radiographs, 12
lung scintigraphy, 14, 16
organized thrombus removing, 16
pulmonary angiography, 15
ventilation perfusion scan, 44
VQ scintigraphy, 6
Chronic thrombus and metastatic cancer
CT scan, 92
diagnosis, 96
endarterectomy specimen, 93, 94
islands of malignancy cells, 94, 95
medical history, 92
PET-CT, 94
pulmonary function testing, 93
pulmonary tumor emboli, 96
ventilation-perfusion scan, 93
Computerized tomography (CT), 92
Computerized tomography angiogram
acute versus chronic thromboembolic disease, 149, 150
atrial septal defect, 168, 169, 172
CTED, 73, 75
CTEPH, 14, 17, 142
parenchymal lung disease and chronic thromboemboli, 178, 180, 181
pulmonary artery sarcoma, 82, 86, 87
pulmonary veins, 105
pulmonary veno-occlusive disease, 110, 111, 113, 114
Computerized tomography pulmonary angiogram (CTPA), 47
CTED, 72
CTEPH, 6
pulmonary arterial pulmonary hypertension mimic CTEPH, 164
pulmonary veno-occlusive disease, 117
Congenital heart disease, 145
Index
189
D
Deep venous thrombi (DVT), 2
Distal arteriopathy, 18
Digital subtraction pulmonary angiogram
CTED, 64, 65
distal chronic thromboembolic disease, 27, 28
pulmonary arteritis, 129
Distal chronic thromboembolic disease
chronic thrombotic material removing, 26
diagnosis, 31
ECG, 22
echocardiogram, 23, 27
PA and lateral digital subtraction pulmonary angiogram, 27, 28
perfusion scan, 29
prognostic factor, 30
pulmonary angiogram, 24, 25
risk factor, 30
transthoracic echocardiogram, 23
VQ scan, 23, 24, 29
Distal vessel thromboendarterectomy
chest radiograph, 52, 53, 56
chronic thromboembolic disease, 57
diagnosis, 59
endarterectomy, organized thrombus removed at, 56
pulmonary angiogram, 54
pulmonary artery, 55, 56
right heart catheterization, 54
subclavian venogram, 52
surgery evaluation, 58
ventilation perfusion scan, 52, 53
VQ scintigraphy, 56
E
Echocardiogram
acute versus chronic thromboembolic disease, 148
distal chronic thromboembolic disease, 23, 27
Eisenmenger’s syndrome, 145
Emphysema, 179, 182
Endarterectomy, organized thrombus removed at, 56
Extensive parenchymal lung disease, 44, 46
H
Hypoxemia, 99
Index
190
I
Intraluminal lling defects, 151, 152
L
Large vessel pulmonary vascular disease
bilateral unmatched perfusion defects, 136
cardiopulmonary exercise testing, 136
catheter angiography, 138
chest radiograph, 134, 138
chronic thromboembolic disease
catheter-based pulmonary angiography, 136
mimics of, 139
compressive adenopathy and perfusion defects, 137, 138
CT angiogram, 135
CTPA, 138
diagnosis, 140
echocardiogram, 134
extensive soft tissue, 135, 138
nultifactorial causes, 138
perfusion abnormalities, 139
Lung perfusion scan, 143, 144
Lung scintigraphy
atrial septal defect, 168, 171
balloon pulmonary angioplasty, 38
CTED, 64
CTEPH, 14, 16, 146
parenchymal lung disease and chronic thromboemboli, 179, 180
pulmonary arteritis, 122, 123
pulmonary veins, 102
Lung ventilation-perfusion scan, 150, 151
M
MR angiogram (MRA), 125, 127
N
Nonsteroidal therapy, 101
O
Oral prostacyclin therapy, 47
Index
191
P
Paget-Schroetter syndrome, 52
Parenchymal lung disease
cardiovascular exam, 179
chest radiograph, 179, 181
CT angiogram, 178, 180, 181
diagnoses, 184
laboratory assessment, 179
lung scintigraphy, 180
re-perfusion of, 183
PET CT
chronic thrombus and metastatic cancer, 94
pulmonary artery sarcoma, 85–87
Post PE syndrome, 68
Pulmonary angiogram
distal chronic thromboembolic disease, 24, 25
distal vessel thromboendarterectomy, 54
pulmonary veins, 104
Pulmonary arterial pulmonary hypertension mimic CTEPH
chest x-ray, 158, 159, 163
CT chest with pulmonary angiography, 161
CTPA, 160, 164
diagnosis, 165
echocardiogram, 158, 164
laboratory and routine testing, 158
medical history, 158
pulmonary angiography, 163
pulmonary function test, 158, 159
PVOD, 164
right heart catheterization hemodynamics, 162
ventilation perfusion scanning, 161, 165
xenon ventilation scanning, 160
Pulmonary arteriogram
pulmonary veins, 102, 103
pulmonary veno-occlusive disease, 113
Pulmonary arteritis
acute respiratory failure, 122
chest radiograph, 125
diagnoses, 130
digital subtraction pulmonary arteriogram, 129
echocardiogram, 121
initial evaluation, 122
lung scintigraphy, 122, 123
Index