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119
Further Reading
Bailey CL, Channick RM, Auger WR, Fedullo PF, Kerr KM, Yung
GL, Rubin LJ. “High probability” perfusion lung scans in pul-
monary veno-occlusive disease. Am J Resp Crit Care Med.
2000;162:1974–8.
Balke L, Both M, Winkler C, Schreiber T, Röcken C, Koch K,
Bewig B. Scintigraphy leading to the misdiagnosis of chronic
thromboembolic disease in a patient with pulmonary veno-occlu-
sive disease. Circulation. 2016;133(16):1627–8.
Montani D, Girerd B, Jais X, Levy M, Amar D, Savale L, Dorfmuller
P, Seferian A, Lau EM, Eyries M, Le Pavec J, Parent F, Bonnet D,
Soubrier F, Fadel E, Sitbon O, Simonneau G, Humbert M.Clinical
phenotypes and outcomes of heritable and sporadic pulmonary
veno-occlusive disease: a population-based study. Lancet Respir
Med. 2017;5(2):125–34.
Seferian A, Helal B, Jaïs X, Girerd B, Price LC, Günther S, Savale
L, Dorfmüller P, Parent F, Sitbon O, Humbert M, Simonneau G,
Montani D.Ventilation/perfusion lung scan in pulmonary veno-
occlusive disease. Eur Respir J. 2012;40:75–83.
Chapter 12. Case 12: Pulmonary Veno-Occlusive Disease …
121© 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_13
Case Presentation
A 30 year old male presented with a 2year history of pro-
gressive dyspnea, chest pain and fatigue. His past medical
history was notable for a heart murmur since childhood
and a subjective reduced exercise tolerance compared to
his contemporaries. An echocardiogram was performed as
Chapter 13
Case 13: Pulmonary
Arteritis… TheGreat
CTEPH Mimic
KimM.Kerr, AlbertHsiao, andWilliamR.Auger
K. M. Kerr (*)
Division of Pulmonary and Critical Care Medicine,
University of California, San Diego, San Diego, CA, USA
e-mail: kmkerr@ucsd.edu
A. Hsiao
Asssociate Professor in Residence, Department of Radiology,
University of California, San Diego, San Diego, CA, USA
W. R. Auger
Lewis Katz School of Medicine, Temple University,
Philadelphia, PA, USA
e-mail: Bill.Auger@tuhs.temple.edu
122
part of his chest pain and dyspnea evaluation and revealed
a mild to moderately dilated right ventricle and pulmonary
hypertension. Right heart catheterization showed RA 8,
PA 90/13 (mean 35), PAOP 8, thermodilution cardiac out-
put 3.1 L/min (cardiac index 1.6 L/min/m
2
), PVR 697
dynscm
−5
. However, a repeat echocardiogram performed
2 months later demonstrated only a mildly enlarged right
atrium with a normal size right ventricle that appeared
severely hypokinetic. This prompted a cardiac MR which
showed normal cardiac chamber sizes with the exception
of a right atrium at the upper limits of normal and mild
right ventricular hypertrophy with some flattening of the
interventricular septum without evidence of intra-cardiac
shunting.
Soon after this evaluation the patient developed acute
respiratory failure while travelling to altitude requiring
mechanical ventilation and hospitalization for two weeks.
He was diagnosed with pneumonia and pulmonary embo-
lism by chest CT and placed on warfarin anticoagulation.
Upon returning home to lower altitude he remained symp-
tomatic and tadalafil was added to his regimen to treat his
pulmonary hypertension. A VQ scan demonstrated
unmatched perfusion defects (Fig.13.1) and his CT angio-
gram (Fig.13.2) was felt to be compatible for chronic throm-
boembolic disease. He was referred for possible pulmonary
thromboendarterectomy.
On initial evaluation at a CTEPH center he reported some
improvement in exercise tolerance, but remained WHO func-
tional class III with one block DOE and exertional lighthead-
edness. Additional PMH included left spontaneous
pneumothorax treated with VATS (report of evaluation for
Marfan’s negative at that time), chronic hoarseness, mild
thrombocytopenia, questionable Protein S deficiency in the
setting of warfarin therapy, and abnormal LFTs with a liver
ultrasound demonstrating early cirrhotic changes. His family
history was remarkable for a sister who required surgery as a
child for a “hole in her heart”. He previously drank 6–12 beers
daily, but had decreased his alcohol intake substantially and
continued to smoke marijuana.
K. M. Kerr et al.
123
Physical exam revealed a very tall and thin Hispanic male
comfortable at rest. HR 65, BP 136/67, RR 16, SpO2 96% on
room air. There was no JVD, but a carotid bruit vs. a transmit-
ted murmur over his left carotid artery was auscultated. Lung
fields were clear with loud pulmonary flow murmurs noted
anteriorly and posteriorly in both lungs. There was no RV lift,
S1 was normal with a loud second heart sound and a soft
systolic murmur without gallop. Abdominal exam was normal
without evidence of bruits. Extremities revealed no edema,
no bruits over proximal arteries, and pulses were all strong
and symmetric except for a diminished right dorsalis pedis
and posterior tibialis pulses.
Laboratories were notable for elevated alkaline phospha-
tase (251U/L) and ALT/AST (72/46U/L) that were improved
from prior labs, BNPP was normal. CBC was normal. A chest
X-ray (Fig.13.3) showed no cardiomegaly or central pulmo-
nary artery enlargement, but blunting of the costophrenic
angle with some diaphragmatic tenting was noted on the left.
WASH IN
ANT Q
LLAT QRLAT QLAO QRAO Q
POST QLPO Q
RPO Q
WASHOUT1 WASHOUT2EQUIL
F . Lung scintigraphy demonstrating a number of
unmatched perfusion defects, predominantly involving the upper
lobes, lingula and right middle lobe
Chapter 13. Case 13: Pulmonary Arteritis…
124
An echocardiogram showed normal right and left sided
chamber size and function, normal valvular function and esti-
mated RV systolic pressure of 121mmHg. Repeat right heart
catheterization was performed: RA 5, RV 110/6, PA 110/16
(mean 43), PAOP 7, cardiac output 4.25L/min, cardiac index
2.19L/min/m
2
, PVR 678 dynesscm
−5
, PA saturation 70%.
a
b
F . (a) Right main pulmonary artery narrowing (white
arrow) throughout its course with marked vessel narrowing of the
left descending PA (chevron). Bronchial arterial collateral vessels
also noted. (open arrow). (b) Proximal to distal (left to right) axial
images of the CT angiogram shown in (a). Small right descending
PA to attenuated proximal RLL segmental vessels to dilatation of
the more distal segmental vessels of the right lower lobe. No CT
evidence for chronic thrombus
K. M. Kerr et al.
125
To better define pulmonary vessel anatomy, digital sub-
traction pulmonary angiography (Fig. 13.4a, b) was per-
formed. On the right, a small main pulmonary artery was
noted with absent perfusion to the posterior RUL, post-
stenotic dilation of the apical RUL, proximal segmental
obstruction of the RML, irregularity and narrowing of the
RLL arteries with absent flow to the superior segment. The
left main pulmonary artery was also small with marked nar-
rowing of the descending PA, absent perfusion to the majority
of the LUL and lingula and flow to the LLL confined to the
posterior basal artery.
These arteriographic findings were felt to be atypical for
CTEPH and more compatible with large vessel arteritis. An
MR angiogram (MRA) was performed of the neck, chest,
and abdomen revealing marked diffuse narrowing of the
distal descending thoracic and upper abdominal aorta with
occlusion of the celiac origin and moderate stenosis of the
superior mesenteric artery, compatible with the diagnosis of
F . PA chest radiograph: no central pulmonary artery
enlargement despite the severe pulmonary hypertension docu-
mented by right heart catheterization
Chapter 13. Case 13: Pulmonary Arteritis…
126
PA Lateral
a
PA Lateral
b
F . (a) Right PA and lateral digital subtraction pulmonary
angiogram. (b) Left PA and lateral digital subtraction pulmonary
angiogram
K. M. Kerr et al.
127
large vessel arteritis. There was also moderate narrowing of
the renal artery origins, along with extensive collateraliza-
tion of the hepatic artery territory via the splenic artery,
likely through gastric and gastroepiploic collaterals. The
pulmonary arteries were also noted to be diminished in cali-
ber (Fig.13.5), particularly centrally with occlusion of some
vessels as seen on the digital subtraction angiogram. The
finding of arteritis in the systemic vessels (Fig.13.6) on CTA
and MRA further substantiated the diagnosis of large vessel
arteritis of the pulmonary arteries resulting in pulmonary
hypertension.
F . Chest MRA depicting small caliber left central PA with
segments of irregular narrowing (open arrow)
Chapter 13. Case 13: Pulmonary Arteritis…
128
Clinical Comments
This case is in many ways atypical for the presentation of
major vessel pulmonary arteritis—one etiology of which is
Takayasu arteritis—though compelling from the perspective
of when to suspect an alternative diagnosis to CTEPH.The
length of time this patient was experiencing cardiopulmonary
symptoms (dating back to childhood), and the severity of
pulmonary hypertension documented by right heart catheter-
ization should have immediately raised concerns that the
initial acute deterioration at altitude may have been an exac-
erbation of right heart failure with the presence of pulmonary
embolism perhaps a “secondary” event. The subsequent
assessment led to a more appropriate consideration of
CTEPH given the sub-acute symptoms, the severity of pul-
monary hypertension, the abnormal VQ, and CT findings
which could easily be misinterpreted as chronic
F . 3D reconstructions from a CTA of the abdominal ves-
sels, with the notable occlusion of the celiac axis and irregular nar-
rowing of the superior mesenteric artery (white arrow)
K. M. Kerr et al.
129
thromboembolic disease such as segments of vessel narrow-
ing and attenuation and even the post-stenotic dilatation
occasionally observed in CTEPH.There was even the pres-
ence of bronchial arterial collateral vessels. The detection of
pulmonary artery bruits or flow murmurs could be indicative
of CTEPH as well, an auscultatory finding in approximately
30% of patients with proximal vessel, chronic thrombotic
lesions. However, the findings that might suggest an alternate
diagnosis are the longstanding presence of cardiopulmonary
difficulties, the extraordinary pulmonary arterial pulse pres-
sure noted on his initial right heart catheterization—a sign of
noncompliant proximal pulmonary vessels—and the appear-
ance of the proximal arteries by CT and on his routine chest
radiograph. With this degree of pulmonary hypertension,
there’s typically significant enlargement of the proximal pul-
monary arteries.
But based on historical features alone, pulmonary arteritis
was not an obvious diagnosis. His gender was an issue;
Takayasu arteritis usually affects young females, reports indi-
cating a rate ten times greater than males. Furthermore, the
problems during childhood raised concerns that congenital
pulmonary vascular disease should be considered, such as
pulmonary artery stenosis which may have similar abnormali-
ties on CT and VQ. However, pulmonary artery stenosis is
typically associated with congenital heart defects such as
Tetralogy of Fallot, pulmonary arterial stenosis of Williams
syndrome, patent ductus arteriosus, or pulmonary valve ste-
nosis. Such lesions are usually detected during childhood
(and none present on this patient’s cardiac MR). The absence
of systemic arterial events was also puzzling, though in retro-
spect, his chronically elevated liver-related enzymes may
have been a manifestation of compromised hepatic vascular
supply. Though pulmonary artery involvement can be the
initial presentation of Takayasu arteritis, this is not the typical
course.
Ultimately, the digital subtraction pulmonary arteriogram
was the study to raise the greatest doubts about chronic
thromboembolic disease as the cause of this patient’s
Chapter 13. Case 13: Pulmonary Arteritis…