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161
F . Ventilation/perfusion scanning with significant bilateral
patchy perfusion defects
F . CT chest with pulmonary angiography revealed
enlarged pulmonary arteries (arrow) without evidence of acute or
chronic pulmonary emboli
Chapter 17. Case 17: Can Severe Pulmonary ....
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162
V/Q was concerning for chronic thromboembolic pulmonary
hypertension (CTEPH); however, after consultation with an
expert CTEPH Center and detailed review of imaging, it was
determined that V/Q changes were attributable to his pulmo-
nary arterial hypertension and there was no radiographic
evidence of significant chronic thromboembolic disease. He
was diagnosed with WHO Group I pulmonary arterial hyper-
tension. He was initiated on oral pulmonary vasodilator
therapy with significant symptomatic improvement. His exer-
cise tolerance increased and presyncope with exertion
resolved. He remains active with NHYA functional class II
symptoms.
T . Right heart catheterization hemodynamics revealing
signicant precapillary pulmonary hypertension with elevated pul-
monary vascular resistance
Hemodynamic findings
Right atrial (RA) 4mmHg
Right ventricular (RV) 65/4mmHg
Pulmonary artery (PA) 64/25 (38) mmHg
Pulmonary artery wedge (PAWP) 4mmHg
Inferior vena cava (IVC) Saturation 67%, Hgb
12.9g/dL
Right atrium (RA) Saturation 69%, Hgb
12.9g/dL
Pulmonary artery (PA) Saturation 66%, Hgb
12.9g/dL
Systemic saturations Saturation 90%
Thermodilution cardiac output (CO) 4.5L/min
Thermodilution cardiac index (CI) 2.2L/min/m
2
Thermodilution systemic vascular
resistance (SVR)
1529dyn-s/cm
5
Thermodilution pulmonary vascular
resistance (PVR)
7.6 WU, 604dyn-s/cm
5
J. M. Elwing et al.
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163
Radiographic Interpretation
The frontal and lateral chest radiographs demonstrate nor-
mal cardiac silhouette with clear lungs. VQ scintigraphy
shows mottled perfusion bilaterally with small mismatched
perfusion defects. There are generic features of pulmonary
hypertension on the CT pulmonary angiography such as
mildly dilated and hypertrophied right ventricle and flatten-
ing of interventricular septum. The left atrium and left ven-
tricle are not enlarged and there’s no evident intracardiac
shunt. There is no evidence of acute or chronic thromboem-
bolic disease. The lungs are unremarkable. Catheter
pulmonary angiography shows good opacification of pulmo-
nary vasculature with smooth vessels that demonstrate areas
of abnormal tapering to the periphery (“pruning”). There are
no specific features of thromboembolic disease.
Abnormal perfusion on scintigraphy is a well-recognized
feature of non-thrombotic PAH and is most likely a reflection
of underlying pulmonary vasculopathy (Fishman et al.).
Whilst the perfusion defects in PAH typically tends to be
mostly patchy and in a non-anatomical distribution, occasion-
ally segmental defects have also been described. A recent
study using SPECT VQ found perfusion abnormalities with
no demonstrable thromboembolic disease on corroborative
pulmonary angiography in as many as 43% of cases with
F . Pulmonary angiography with bilateral equal perfusion
without evidence of luminal narrowing or vascular obstruction
Chapter 17. Case 17: Can Severe Pulmonary ....
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164
PAH with worse hemodynamics and clinical outcome in
patients with global perfusion defects (Chan etal.). Pulmonary
veno-occlusive disease (PVOD) is another important differ-
ential to consider in the setting of pulmonary hypertension
and false positive VQ (Bailey etal.; see Case 13, Chap. 13).
CTPA is a useful discriminator as the triad of interlobular
septal thickening, diffuse ground glass opacification and
mediastinal lymphadenopathy are hallmarks of PVOD rather
than PAH.These features were not present on the CT in the
current case. As Group 2, 3 and 4 were also excluded by imag-
ing, the diagnosis is WHO Group 1 disease.
Clinical Comments
The presented case provides a “textbook” illustration of a
logical progression in the evaluation of a patient’s dyspnea.
The absence of an obvious basis for this complaint accompa-
nied by an unremarkable physical exam, routine laboratory
studies, electrocardiography, chest radiograph and PFTs, led
to an investigation for pulmonary vascular disease. With the
echocardiogram then suggestive of pulmonary hypertension,
screening for CTEPH with lung scintigraphy is the appropri-
ate next step. This possible diagnosis was further enhanced
given the pro-thrombotic risk factor of a previous splenectomy
and the prior DVT history (the methylenetetrahydrofolate
reductase polymorphism is not a significant risk factor for
thrombosis). Despite the abnormal perfusion scan, the pat-
tern of perfusion abnormality was very suggestive of distal
vessel disease. CT angiography of chest as a diagnostic tool
was entirely appropriate to further define the VQ findings.
Without CT evidence for chronic thromboembolic lesions,
the question might be raised as to the rationale for further
diagnostic testing. As CTA has been shown to underestimate
CTEPH within the segmental and subsegmental vessels
(Sugiura et al.), it was appropriate to proceed with digital
subtraction pulmonary angiography to complete the evalua-
tion. This ultimately ruled out chronic thromboembolic dis-
ease as the basis for this patient’s pulmonary hypertension.
J. M. Elwing et al.
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165
Finally, confirmatory right heart catheterization was essential
to document the degree of pulmonary hypertension and right
heart dysfunction to guide therapy.
Point ofEmphasis
Ventilation perfusion scanning is an essential part of the
evaluation of patients with pulmonary hypertension. The
absence of perfusion defects reliably rules out chronic throm-
boembolic disease. However, patchy perfusion can be seen in
the setting of pulmonary arterial hypertension and can mimic
CTEPH. This often requires assessment with pulmonary
angiography to confirm the presence of chronic thromboem-
bolic disease.
Diagnosis
1. WHO group I pulmonary arterial hypertension (precapil-
lary pulmonary hypertension).
Further Reading
Bailey CL, Channick RN, Auger WR, etal. High probability perfu-
sion lung scans in pulmonary venoocclusive disease. Am J Respir
Crit Care Med. 2000;162(5):1974–8.
Chan K, Ioannidis S, Coghlan JG, etal. Pulmonary arterial hyper-
tension with abnormal V/Q single-photon emission computed
tomography. JACC Cardiovasc Imaging. 2018;11(10):1487–93.
Fishman AJ, Moser KM, Fedullo PF.Perfusion lung scans vs pulmo-
nary angiography in evaluation of suspected primary pulmonary
hypertension. Chest. 1983;84(6):679–83.
Suigura T, Tanabe N, Matsuura Y, etal. Role of 320-slice CT imaging
in the diagnostic workup of patients with chronic thromboem-
bolic pulmonary hypertension. Chest. 2013;143:1070–7.
Worsley DF, Palevsky HI, Alavi A.Ventilation-perfusion lung scan-
ning in the evaluation of pulmonary hypertension. J Nucl Med.
1994;35(5):793–6.
Chapter 17. Case 17: Can Severe Pulmonary ....
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167© 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_18
Case Presentation
A 31year old Filipino male with no significant past medical
history presented with complaints of two years of progres-
sively worsening dyspnea on exertion with associated weight
loss of 20lb. He had an acute deterioration after a trip to visit
family in the Philippines. Upon landing back in the United
States, he was found to be profoundly hypoxemic and cyanotic.
Chapter 18
Case 18: Atrial Septal
Defect withChronic
Thrombus Mimicking
CTEPH
TimothyM.Fernandes, DeepaGopalan,
andWilliamR.Auger
T. M. Fernandes (*)
Division of Pulmonary and Critical Care Medicine,
University of California, San Diego, San Diego, CA, USA
e-mail: tfernandes@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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168
He has transferred to the local Emergency room and found to
have SpO2in the low 80s % with only a modest improvement
in oxygenation with supplemental oxygen. On exam, he was
tachycardic with a prominent P2 component of the second
heart sound. There was no S3. He had jugular venous disten-
tion to the jaw and pretibial edema. There was prominent
clubbing of the digits of the hands and feet. Upon further
questioning, it was unclear as to the length of time the patient
exhibited clubbing and nailbed cyanosis. His feeling was that
this was how “fingers should look”. Given his travel history,
there was concern for acute pulmonary embolism so the
patient was sent for a CT pulmonary angiogram (Fig.18.1a–c).
The CT demonstrated a severely enlarged main pulmonary
artery consistent with pulmonary hypertension and “severe
bilateral pulmonary emboli”. He underwent a transthoracic
echocardiogram which revealed severe tricuspid regurgitation
with and right ventricular systolic pressure estimated at
112mmHg. The patient was then transferred to a pulmonary
thromboendarterectomy center for evaluation of potential
chronic thromboembolic pulmonary hypertension.
Hospital Course: A chest radiograph (Fig.18.2) and venti-
lation- perfusion scan (Fig. 18.3a, b) were performed. The
perfusion scan revealed multiple unmatched perfusion
defects in the anterior left upper lobe, lateral basal left lower
lobe and lateral segment of the right middle lobe. There was
uptake of technetium macro aggregated albumin seen in the
brain and kidneys consistent with a right-to-left shunt.
A repeat transthoracic echocardiogram with agitated
saline was ordered and demonstrated a severely enlarged
right atrium with early positive bubble study, concerning for
an atrial septal defect. The patient underwent right heart
catheterization with shunt run with results as follows:
• Saturation Run: Systemic pulse oximetry 82–84%, high
SVC saturation 45%, low SVC saturation 44%, mid RA
saturation 62%, IVC saturation 46%, right ventricle satu-
ration 61%, PA saturation 59%.
• Baseline hemodynamics:
• Mean right atrial pressure of 2.
T. M. Fernandes et al.
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169
a
b
F . CTA of the chest showing occlusive thrombus in a left
upper lobe (panel b, blue arrow) and right middle lobe pulmonary
artery (panel c, open blue artery). Eccentric lining thrombus in a
markedly enlarged right main PA, and in dilated left upper lobe,
right lower lobe segmental vessels also noted (panels a, c, white
arrows)
Chapter 18. Case 18: Atrial Septal Defect withChronic…
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170
F . Chest radiography (AP view) demonstrates cardio-
megaly with aneurysmal dilatation of the proximal pulmonary arter-
ies. There is no obvious pulmonary artery calcification
c
F . (continued)
T. M. Fernandes et al.
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171
• Right ventricular systolic pressure of 88, right ventricular
end-diastolic pressure of 4.
• Pulmonary artery pressure 92/51, mean pulmonary artery
pressure 64.
• Pulmonary capillary wedge pressure 4.
• Cardiac output calculations: Qs was equal to 2.17 L/min
with cardiac index of 1.4 m
−2
. Qp was calculated using a
few assumed pulmonary venous saturations. Assuming a
pulmonary venous saturation of 95%, Qp was equal to
2.36L/min, 1.5m
−2
.
Given the step-up in saturation seen in the right atrium, a
cardiac MR was ordered to further assess cardiac anatomy.
This revealed total right-sided anomalous pulmonary venous
drainage with both the upper and lobe right-sided pulmonary
veins draining back into the right atrium (Fig.18.4). This was
associated with mixed atrial septal defects including a sinus
venosus defect (Fig.18.4) and an ostium secundum ASD.
For management of his pulmonary hypertension due to
Eisenmenger’s syndrome (an atrial septal defect with right-
to- left shunt, cyanosis, pulmonary hypertension) and total
right-sided anomalous pulmonary venous drainage, the deci-
sion was made for medical management. The patient was
started on intravenous epoprostenol, bosentan (an endothe-
ab
WASH IN
ANT
Q
LLAT
QRLAT QLAO Q RAO Q
POST QLPO Q RPO Q
POST KIDNEY
WASHOUT1 WASHOUT2EQUIL
F . Lung scintigraphy showing unmatched perfusion
defects (panel a) as described in the text, with uptake evident in the
kidneys suggesting a right to left shunt (panel b)
Chapter 18. Case 18: Atrial Septal Defect withChronic…
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