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14
F . CT angiography demonstrated complete obstruction of
the distal right main PA.There was no evidence for a mediastinal
mass or adenopathy. Bronchial collaterals are indicated by the arrow
WASH IN
ANT Q POST Q
EQUIL
F . Lung scintigraphy showing absence of perfusion to the
right lung; Xenon ventilation documenting a smaller right lung with
resultant hemidiaphragm elevation, correlating with chest radio-
graph findings
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Pulmonary angiography revealed complete proximal
occlusion of main right pulmonary artery without major ves-
sel chronic thromboembolic disease in the left lung (Fig.2.4).
Referral to CTEPH center experienced with pulmonary
thromboendarterectomy surgery was requested.
Pulmonary thromboendarterectomy was ultimately per-
formed with the removal of chronic thrombotic material as
pictured in Fig.2.5. Considerable “back-bleeding” in the right
pulmonary arteries was observed during cardiopulmonary
bypass. This resulted in a significant improvement in pulmo-
nary hemodynamics with the following postoperative values:
CVP 7, PAp 40/16 (25 mean), a thermodilution CO 4.9L/min,
F . Catheter based pulmonary angiogram shows complete
occlusion of the right main pulmonary artery; on the left side there’s
segmental vessel tortuosity without angiographic features for
chronic thromboembolic disease. Poor opacification of the left sided
vessels was due to a high output state
Chapter 2. Case 2: There’s No Perfusion tothat Lung…
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a cardiac index of 2.3L/min/m
2
. The only postoperative com-
plication was a transient episode of atrial fibrillation. Early
postoperative lung scintigraphy revealed partial reperfusion
of her right lung (Fig.2.6).
F . Organized thrombus removed at time of PTE
WASH IN
ANT Q POST Q
EQUIL
F . Lung scintigraphy after PTE surgery showing partial
reperfusion of the right lung
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F . Coronal view of CT angiography pictured in Fig. 2.3.
Very large bronchial collateral vessel going to right lung (arrow)
Radiographic Interpretation
Selected images from computerized tomography pulmonary
angiography demonstrate a pouch defect in the distal right
main pulmonary artery with complete occlusion of the lobar,
segmental and subsegmental arteries in the right lung
(Fig.2.3). There are multiple enlarged bronchial collaterals,
seen best on the coronal view (Fig.2.7, open arrow).
Clinical Comments
The first observation relevant to this case is that there are a
number of clinical entities that are more commonly associ-
ated with non-perfusion of an entire lung than occlusion of
a main pulmonary with organized, chronic thrombus.
Extrinsic compression of the pulmonary artery from a medi-
astinal or hilar mass (adenopathy or neoplasm) or intrinsic
disease of the artery as seen in vasculitis (e.g., Takayasu’s
Chapter 2. Case 2: There’s No Perfusion tothat Lung…
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vasculitis, see Chap. 17) or a tumor (e.g. angiosarcoma, see
Chap. 12) are in the diagnostic differential. In an adult,
rarely is this finding due to pulmonary artery agenesis, espe-
cially in the absence of ipsilateral airway or parenchymal
lung disease. This serves as the basis for the recommenda-
tion that a patient displaying such significant compromise in
lung perfusion requires computed tomography or magnetic
resonance imaging of the chest to assist the clinician in mak-
ing the correct diagnosis.
For those patients with chronic thromboembolic obstruc-
tion, and who are candidates for thromboendarterectomy
surgery, there are numerous challenges to confront.
Experience has shown that approximately 30% of patients
with complete occlusion of a main pulmonary artery will not
reperfused that lung even with a complete endarterectomy of
the clot. And in another third of patients, incomplete reperfu-
sion can be expected. Which outcome a patient will experi-
ence following a thromboendarterectomy is difficult to
predict, though an extended period of occlusion seems to
decrease the chance of reperfusion. This is especially the case
if there is accompanying lung volume loss and scarring. A
poor outcome is obviously more problematic if a patient
presents with pulmonary hypertension and right heart dys-
function making it imperative that reperfusion and pulmo-
nary hemodynamic benefit are achieved with endarterectomy
surgery.
In addition, the reasons for suboptimal re-perfusion are
unclear. The development of a distal arteriopathy with
extended proximal occlusion of a main pulmonary artery has
been suggested as a potential mechanism for this outcome.
“Competitive blood flow” from systemic collateral vessels
has also been proposed. As seen in many CTEPH patients,
and illustrated in this case presentation, obstruction of major
pulmonary arteries promotes the compensatory development
of an extensive collateral systemic circulation, which is
important to maintain tissue viability. Whether this collateral
circulation is an essential factor in the development of a distal
arteriopathy or is responsible for the inhibition of pulmonary
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arterial blood flow after endarterectomy remain areas of
ongoing research. In the presented case, what occurred was
partial reperfusion of the right lung with recruitment of
enough of the pulmonary vascular bed to result in significant
pulmonary hemodynamic and symptomatic improvement.
Whether or not this incomplete re-perfusion relates to the
extensive collateral systemic blood flow is not known.
However, as has been the case in similar patients, subsequent
improvement in perfusion of the endarterectomized lung
tends to occur over time as long as anticoagulation is
maintained.
Points ofEmphasis
Complete scintigraphic absence of perfusion to an entire lung
requires cross sectional diagnostic imaging to ascertain the
cause. For those patients with chronic thrombotic occlusion,
pulmonary thromboendarterectomy can be an effective inter-
vention to re-establish blood flow, though may be best per-
formed early in the course of the disease.
Diagnoses
1. Complete occlusion of a main pulmonary artery with
chronic thromboembolus.
2. WHO group IV pulmonary hypertension.
Further Reading
Cho S-R, Tisnado J, Cockrell CH, et al. Angiographic evaluation
of patients with unilateral massive perfusion defects in the lung
scan. Radiographics. 1987;7(4):729–45.
Dorfmüller P, Günther S, Ghigna MR, etal. Microvascular disease
in chronic thromboembolic pulmonary hypertension: a role
for pulmonary veins and systemic vasculature. Eur Respir J.
2014;44:1275–88.
Chapter 2. Case 2: There’s No Perfusion tothat Lung…
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Hirsch AJ, Moser KM, Auger WR, Channick RN, Fedullo
PF. Unilateral pulmonary artery thrombotic occlusion: is dis-
tal arteriopathy a consequence? Am J Respir Crit Care Med.
1996;154(2):491–6.
Kitcher A, Pretorius V, Kerr K, Fedullo P, Kim NH, Poch D, Madani
M, Jamieson S, Auger W. Complete pulmonary artery occlu-
sion from chronic thromboembolic disease: outcomes follow-
ing pulmonary thromboendarterectomy surgery. J Heart Lung
Transplant. 2014;32(4S):S54.
Laczika K, Lang IM, Quehenberger P, et al. Unilateral chronic
thromboembolic pulmonary disease associated with combined
inherited thrombophilia. Chest. 2002;121:286–9.
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.
Moser KM, Olson LK, Schlusselberg M, Dembitsky WP. Chronic
thromboembolic occlusion in the adult can mimic pulmonary
artery agenesis. Chest. 1989;95:503–8.
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21© 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_3
Case Presentation
A 63year old white male with a history of hyperlipidemia and
sick sinus syndrome requiring placement of a permanent pace-
maker presented with dyspnea on exertion. A CT angiogram
Chapter 3
Case 3: Distal Chronic
Thromboembolic
Disease—Is Surgery
anOption?
AnjaliVaidya, DeepaGopalan, andWilliamR.Auger
A. Vaidya (*)
Pulmonary Hypertension, Right Heart Failure, and CTEPH
Program, Advanced Heart Failure and Cardiac Transplant, Lewis
Katz School of Medicine, Temple University, Philadelphia, PA, USA
Pulmonary Hypertension, Right Heart Failure, and CTEPH
Program, Lewis Katz School of Medicine, Temple University,
Philadelphia, PA, USA
e-mail: Anjali.vaidya@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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of the chest at the time of presentation was read as showing
no pulmonary embolus. Right heart catheterization revealed
pre-capillary pulmonary hypertension, and he was started on
bosentan for idiopathic pulmonary arterial hypertension
(iPAH). After 9 months, this was discontinued due to ele-
vated liver activity enzymes, and he was transitioned to silde-
nafil. One year later, for progressive dyspnea and right heart
failure related to pulmonary hypertension, he was initiated
on inhaled treprostinil. Subsequently, ambrisentan was added
to his regimen. Two years thereafter, on this combination
regimen, a repeat right heart catheterization was performed:
mean PA pressure (mPAP) of 84mmHg, cardiac output (CO)
5.2 L/min, pulmonary capillary wedge pressure (PCWP) of
10 mmHg, and pulmonary vascular resistance (PVR) of
14.2 Wood Units. Another CT angiogram of the chest was
performed, again showing no evidence for acute pulmonary
emboli. He remained on this regimen for 2 years, and after an
episode of syncope, was transitioned from inhaled treprostinil
to intravenous epoprostenol during a local hospital
admission.
Five years after his initial presentation, he was referred to
a Pulmonary Hypertension Cardiologist for further evalua-
tion. He reported exertional dyspnea and syncope with symp-
toms limiting his activities of daily living and World Health
Organization functional class III.Physical exam was notable
for BP 124/56, pulse 89, respiratory rate 14, and oxygen satu-
ration of 95% on room air. His jugular venous pressure was
8cm of water with ‘v’ waves to 12cm of water, with positive
abdominojugular reflux. An accentuated P2 and a III/VI
holosystolic tricuspid regurgitation murmur that augmented
with inspiration at the left lower sternal border were heard.
No pulmonary bruits were heard. There were sharp contour
arterial pulses without peripheral edema.
An electrocardiogram (ECG) revealed sinus rhythm with
an incomplete right bundle branch block, right ventricular
hypertrophy, and evidence of right heart strain with right
sided precordial and inferior T wave inversions. His 6 min
walk distance (6MW) was 331m with oxygen desaturation to
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91%. N-terminal proBrain natriuretic peptide (NT-ProBNP)
was 1178, Cr 0.96, and Na 138. ANA and HIV were negative.
Review of his transthoracic echocardiogram revealed a
small pericardial effusion, small and underfilled left ventricle
with hyperdynamic function (LVEF 75%), severe tricuspid
regurgitation, transmitral and tissue Doppler pattern consis-
tent with normal left heart filling pressures, severe systolic
interventricular septal flattening, mid-systolic notching of the
right ventricular outflow tract pulse wave Doppler profile
with a severely reduced acceleration time, severe RV enlarge-
ment (RV:LV ratio 1.8), RVSP estimated at 100 mmHg, and
tricuspid annular plane systolic excursion (TAPSE) of 2.3cm
(Fig.3.1).
Social history was notable for no alcohol or drug use, and
no history of anorexigen, methamphetamine, or other illicit
drug use. There was no family history of pulmonary hyperten-
sion, autoimmune disease, or thrombophilia.
His sildenafil dose was escalated, epoprostenol dose up-
titrated, and an initial ventilation perfusion (V/Q) scan was
performed, which revealed multiple unmatched perfusion
defects in the bilateral upper and lower lobes (Fig.3.2).
ab
F . (a, b) Preoperative echocardiogram showing enlarge-
ment of the right atrium (RA) and right ventricle (RV) with flatten-
ing of the interventricular septum (arrow)
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