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109© 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_12
Case Presentation
A 23 year old male with no significant past medical history
was transferred from a small community based intensive care
unit (ICU) to a tertiary care referral center for evaluation of
Chapter 12
Case 12: Pulmonary Veno-
Occlusive Disease …but It
Started Out asCTEPH
RanaAwdish, SaraHegab, DeepaGopalan,
andWilliamR.Auger
R. Awdish (*) · S. Hegab
Pulmonary Hypertension Program, Division of Pulmonary and
Critical Care Medicine, Henry Ford Hospital, Detroit, MI, USA
Pulmonary Hypertension Program, Heart and Vascular Institute,
St. John Providence Ascension Health, Southeld, MI, USA
School of Medicine, Wayne State University, Detroit, MI, USA
e-mail: rawdish1@hfhs.org
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
110
pulmonary hypertension. The patient had a 3 month history of
gradually progressive exertional dyspnea where activities such
as rising from seated position or taking a few steps resulted in
severe shortness of breath. He endorsed dizziness and light-
headedness with exertion as well as pre-syncope, though he
denied any syncopal episodes or lower extremity edema. The
patient was a lifelong nonsmoker with no history of alcohol
use. He reported traveling on several long car and airplane
trips in the few months prior to presentation. Evaluation in
the ICU showed blood pressure of 139/79, pulse of 79 beats
per minute, respiratory rate of 20 breaths per minute, and
oxygen saturation of 93% on room air. Physical examination
showed a well-developed, age appropriate male in mild respi-
ratory distress. Cardiac auscultation was significant for S1 and
S2 with accentuated pulmonary component of S2 audible at
the apex, grade 1/6 holosystolic murmur audible at left lower
sternal border. There was no JVD or lower extremity edema
noted. 2-dimensional (2D) echocardiography showed a mildly
reduced left ventricular ejection fraction of 54% without sig-
nificant abnormalities in diastolic parameters, an estimated
right ventricular systolic pressure (RVSP) of 63mmHg with
severe enlargement of the right ventricle, tricuspid annular
plane systolic excursion (TAPSE) 2.04 cm, right ventricular
fractional area change of 29.4% and flattening of the interven-
tricular septum in diastole and systole. Saline contrast bubble
study was negative for evidence of intra-cardiac shunting.
Due to concern for acute pulmonary embolism given
recent travel history, the patient had a computerized
tomography angiogram (CTA) of the chest. (Fig.12.1) This
was negative for acute pulmonary embolism but did show
right and left lower lobe ground glass opacities with diffuse
lymphadenopathy most pronounced in the mediastinal and
subcarinal areas. The patient underwent bronchoscopy with
transbronchial needle aspiration (TBNA) of lymph node sta-
tions 11R, 7 and 4R with no evidence of malignancy.
An evaluation for pulmonary arterial hypertension was
initiated given history and echocardiographic findings.
Serological screening for connective tissue disease in the form
of antinuclear antibody (ANA) and rheumatoid factor (RF)
R. Awdish et al.
111
were negative. Human immunodeficiency virus (HIV) testing
was negative. Pulmonary function testing was consistent with a
restrictive pattern as evidenced by FVC 60% predicted, FEV1
57% of predicted, FEV1/FVC 77, TLC 63% of predicted and
DLCO was significantly reduced at 38% of predicted.
A ventilation perfusion (VQ) scan (Fig.12.2) to screen for
chronic thromboembolic disease revealed numerous segmen-
tal and subsegmental mismatched perfusion defects bilaterally
with modestly decreased perfusion noted to the entire right
lung relative to the left.
Right cardiac catheterization showed, in mmHg, right
atrial pressure (RAP) 8/5/2, right ventricular pressure (RVP)
72/0/2, pulmonary artery pressure (PAP) 73/37/46, pulmonary
capillary wedge pressure (PCWP) 5. Pulmonary arterial oxy-
gen saturation was measured at 62% with a cardiac output
and cardiac index obtained by Fick method of 6.42L/min and
2.69 L/min/m2 respectively. Pulmonary angiogram was also
performed showing multiple cut-off’s in the right upper,
middle, and lower lobes that was interpreted to be due to a
large burden of chronic thrombus in the entire right lung.
Small cut-off also noted in the left upper anterior artery con-
sistent with chronic thrombus (Fig.12.3).
F . CT angiography shows prominent perivascular and
subcarinal tissue
Chapter 12. Case 12: Pulmonary Veno-Occlusive Disease …
112
F . Lung scintigraphy shows R>L bilateral segmental and
subsegmental unmatched defects
A multi-disciplinary meeting was held including the
Pulmonary Hypertension Team, Interventional Cardiology
who had performed angiography, the Chest Radiologist and
CT Surgery. In light of the V/Q scan findings and the interpre-
tation of the PA gram, despite the absence of evident throm-
boembolic disease by CTA, CTEPH was entertained as the
probable diagnosis. There was further concern for the possibil-
ity of large pulmonary venous thrombosis on CTA (Fig.12.4).
The patient was started on unfractionated heparin while
inpatient and discharged home on long term anticoagulation
for chronic thromboembolic disease. His diagnostic testing
was sent to the University of California in San Diego for an
assessment of operability.
R. Awdish et al.
113
F . Pulmonary arteriogram shows a reduction in periph-
eral perfusion or “pruning” in the left lower lobe (arrows)
F . CT angiogram of the chest show a flow disturbance in
the right inferior pulmonary vein (block arrow)
Chapter 12. Case 12: Pulmonary Veno-Occlusive Disease …
114
The patient presented to the hospital one month later with
worsening dyspnea, now occurring at rest. Physical examina-
tion was significant for normal S1 and S2 with prominent P2,
grade 2/6 holosystolic murmur heard best a left upper sternal
border, JVD of 8cmH
2
O, and 2+ lower extremity edema. There
was significant progression of CT imaging findings (as below),
with increase in interlobular septal thickening and areas of
ground glass opacities with stable lymphadenopathy (Fig.12.5).
Due to the very rapid progression of disease, and the con-
stellation of CT findings in conjunction with the hemody-
namic data, pulmonary veno-occlusive disease was favored.
Pulmonary transplant team was consulted and patient under-
went expedited workup and was listed for lung transplant. As
a bridge to transplant, the patient was started on low dose
parenteral epoprostenol at 2ng/kg/min. His right ventricular
failure progressed despite this, as did his hypoxemic respira-
tory failure. A second multi-disciplinary meeting was held,
including CT Surgery, Pulmonary Hypertension and Pulmonary
Transplant and it was agreed to proceed with veno-arterial
(VA) extracorporeal membrane oxygenation (ECMO) for
hemodynamic support as a bridge to lung transplantation.
The patient required support of VA ECMO for two days
prior to undergoing bilateral lung transplantation. He was
discharged to rehab, and subsequently home.
F . CT angiogram shows bilateral diffuse ground glass
opacities (thin arrow), smooth interlobular septal thickening
(notched arrow), pronounced mediastinal and hilar lymphadenopa-
thy (chevron) with a small left pleural effusion(black star)
R. Awdish et al.
115
F . Gross specimen explanted lung. Scale represented by
ruler. Thickened interlobular septa (thick arrow). Evidence of dif-
fuse alveolar damage in intervening tissue (circle). Accentuated
branch points (thin arrow)
F . Verhoeff stain demonstrating thickened interlobar
septa; Magnification ×4. Thickened vein (thin arrow) within fibrotic
interlobar septae (thick arrow)
Histopathology of the explanted lungs was consistent with
pulmonary veno-occlusive disease, arterial intimal fibrosis,
diffuse alveolar damage/acute interstitial pneumonitis. The
patient is alive ten months post-transplant and continues to
do well (Figs.12.6, 12.7, 12.8, and 12.9).
Chapter 12. Case 12: Pulmonary Veno-Occlusive Disease …
116
F . Verhoeff stain magnification ×4 demonstrating arterial
intimal hyperplasia (arrow)
F . H and E stain. Magnification ×4 demonstrating septal
thickening (thick arrow) and thickened venules (thin arrow)
Radiographic Interpretation
Review of the imaging studies in this case warrants several
comments.
R. Awdish et al.
117
Ventilation-Perfusion scintigraphy demonstrates numer-
ous segmental and sub segmental perfusion defects without
corresponding ventilation defect. The perfusion throughout
the entire right lung is also decreased relative to left.
Ventilation is homogenous in both lungs.
Computerized tomography pulmonary angiogram (CTPA)
shows generic features of pulmonary hypertension such as
dilatation of the right atrium and right ventricle with flatten-
ing of the interventricular septum. There are bilateral diffuse
ground glass opacities (thin arrow) smooth interlobular sep-
tal thickening (notched arrow), pronounced mediastinal and
hilar lymphadenopathy (chevron) with a small left pleural
effusion (black star). There is no evidence of acute or proxi-
mal chronic thromboembolic disease. Flow disturbance is
noted within the inferior pulmonary veins with no evidence
of any extrinsic compressive lesion (block arrow); this was
initially interpreted as a large pulmonary venous thrombus.
On catheter pulmonary angiography, there is smooth
change in the calibre of the pulmonary arteries from proxi-
mal to distal bed with no intimal thrombus, pouch defect,
intravascular webs or stenosis. During the capillary phase,
there is heterogeneous reduction of perfusion in the left
upper zone with reduced peripheral perfusion in the mid and
lower zones. These findings are consistent with the presence
of extensive small vessel disease, and are not a feature associ-
ated with chronic thromboemboli. Similar findings were felt
to be present throughout the right, though the quality of the
study limits interpretation.
Clinical Comments
The principal lesson in this case centers on the radiographic
miscues that occasionally occur when dealing with pulmo-
nary veno-occlusive disease (PVOD). Given the rarity of
PVOD, it is difficult to describe what typical patterns mani-
fest in perfusion scans, but the largest series available
described normal perfusion scans as the predominate pattern.
Chapter 12. Case 12: Pulmonary Veno-Occlusive Disease …
118
(see Seferian and colleagues) That lung perfusion scintigra-
phy can be abnormal in PVOD is not often recognized. In the
presented case, despite an initial CT of the chest and pulmo-
nary angiogram without evidence for either acute or chronic
thromboembolic disease, the abnormal perfusion scan briefly
deviated diagnostic focus toward the possibility of
CTEPH. Although the VQ scintigraphy might have been
“high-probability” for thromboembolic disease, the patient’s
clinical course, degree of hypoxemia, very low DLCO, and
CT findings—such as his diffuse adenopathy—ultimately
were highly suspicious for, and led to the diagnosis of, pulmo-
nary veno-occlusive disease.
One of the best reviews of this subject is provided by
Seferian and colleagues. In both PVOD and idiopathic or
inheritable PAH, the majority of patients will have normal or
essentially normal ventilation-perfusion scintigraphy.
However, unmatched perfusion defects were described in 7%
of cases with PVOD, likely a result of focal “downstream”
increase in resistance caused by the narrowing and oblitera-
tion of pulmonary veins and venules. Also of interest in this
same study was that 10% of patients with idiopathic PAH
demonstrated abnormal perfusion scans.
Points ofEmphasis
Mismatched perfusion defects on scintigraphy in a patient
with pulmonary hypertension does not always indicate a pul-
monary arterial occlusive process. Pulmonary veno-occlusive
disease should be considered when a positive scintigraphy is
coupled with an angiogram showing no arterial obstruction.
The conglomeration of CT findings can be very helpful in
making the correct diagnosis.
Diagnosis
1. Pulmonary veno-occlusive disease.
R. Awdish et al.