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Presentation: A 63-year-old previously with preserved systolic function (Figures ratio was within the therapeutic range.
healthy woman presented to the emer- 2A and 2B). A diagnosis of pulmonary Repeat TTE showed interval devel-
gency department (ED) with a 7-day embolism (PE) was made and she was opment of dilated right-sided heart
history of acute dyspnea on exertion admitted for initiation of anticoagula- chambers, increased RV filling pressure,
and substernal chest pressure. She had tion. She was discharged home 2 days abnormal RV relaxation, and moderate
recently returned from a vacation that later in stable condition on enoxaparin tricuspid valve regurgitation (Figures
included air travel. On evaluation, and warfarin therapy. 2C and 2D). CTPA showed enlarge-
she was found to have mild hypoxia, One month later, she returned to ment of the filling defect within the
requiring 1 liter per minute of oxygen the ED with progressive dyspnea, new main pulmonary artery and right-sided
to maintain SpO2 saturation of 92%. right-sided pleuritic pain, and non- segmental branches, as well as new
Her vital signs and physical examination productive cough. She had a heart rate filling defects within the left superior
were otherwise unremarkable. Comput- of 126 beats per minute, and required lobar artery. There were also peripheral
ed tomography pulmonary angiogram supplemental oxygen at 3 liters per airspace consolidations within the right
(CTPA) demonstrated a large filling minute. A prominent S2 was noted lower lobe (Figures 3A-3D). Right
defect within the main pulmonary artery without associated murmurs or gallops. heart catheterization showed mildly
with extension into the right-sided There was no jugular venous distention elevated pulmonary artery pressures
segmental and subsegmental branches or lower-extremity edema. Lung fields (Table 1). A gadolinium-enhanced
(Figures 1A-1D). Cardiac biomarkers were clear to auscultation bilaterally. chest magnetic resonance imaging
were within normal limits. A transtho- Laboratory studies were notable for a (MRI) study showed no enhancement
racic echocardiogram (TTE) showed pro-brain natriuretic peptide level of within the intraluminal lesion, suggest-
mild right ventricular (RV) dilation 1138 pg/mL; international normalized ing a large bland thrombus (Figures 4A
and 4B).
Key Words— computed tomography pulmonary angiogram, dyspnea, hypoxia, pulmonary artery sarco-
mas, thromboembolic disease Management: The patient underwent
Correspondence: levinedj@uthscsa.edu percutaneous catheter-directed throm-
Figure 1A: CTPA representative selection demonstrating a large filling defect within the right pulmonary artery and middle and inferior lobar
arteries (red arrow).
Figure 1B: CTPA representative selection demonstrating filling defects within the right segmental pulmonary arteries (red arrows).
Figure 1C: CTPA representative selection of the lung parenchyma.
Figure 1D: CTPA representative selection demonstrating mild flattening of the interventricular septum without significant RV enlargement. No
significant parenchymal disease is noted.
the walls of the right pulmonary artery stamm in 1923.1 Fewer than 300 cases cific findings. Similarly, ventilation/
into the adjacent lung parenchyma, ne- have been reported in the literature perfusion scans are generally unable
cessitating right pneumonectomy (Figures since that time. The World Health to differentiate between thrombus and
5A and 5B). A complete debulking pro- Organization categorizes PAS into 3 tumor, as both conditions appear as
cedure was not possible because of gross main subtypes: angiosarcoma, leiomyo- discrete perfusion defects.3 CTPA may
evidence of involvement of left pulmonary sarcoma, and intimal sarcoma.2 Clinical be a more useful imaging modality,
artery and its distal branches. A diagnosis presentation is similar regardless of demonstrating large obstructive filling
of metastatic intravascular chondroblastic the tumor subtype, and symptoms may defects in the proximal pulmonary
osteosarcoma was confirmed, complicated include dyspnea, chest pain, coughing, arteries. Nonmalignant thromboembol-
by foci of undifferentiated pleomorphic and hemoptysis. Consequently, many ic disease rarely presents with lesions
sarcoma (Figures 6A and 6B). The post- of these cases are initially misdiagnosed that occupy the entire vascular lumen.3
operative course was complicated by sepsis as acute or chronic thromboembolic This phenomenon has been referred to
and multisystem organ failure. Based on disease. as the “wall eclipsing sign,” and is felt
poor prognosis and rapid deterioration, There is no consensus diagnostic to be pathognomonic for pulmonary
she elected for comfort measures only and algorithm for the evaluation of suspect- artery tumors.4 Gadolinium-enhanced
died two days later. ed PAS. In general, laboratory testing is chest MRI has also been proposed as
of little utility. Echocardiography may a diagnostic imaging study that can
Discussion: Pulmonary artery sarcomas show evidence of pulmonary hyperten- distinguish PAS from PE.5,6 Contrast
(PAS) were first described by Mandel- sion (PH) and RV dysfunction, nonspe- enhancement of the intraluminal lesion
suggests the presence of an intravas- The mainstay of therapy for these fail to respond to appropriate anti-
cular tumor. However, as our case tumors is surgical resection, which may coagulation therapy. PAS should also
illustrates, the absence of contrast en- prolong tumor-free survival.7,8 The role be considered in patients undergoing
hancement is not sufficient to exclude of chemotherapy is unclear, and there evaluation for chronic thromboembolic
intravascular tumors. Another radio- are no consensus recommendations for pulmonary hypertension (CTEPH) who
graphic modality, fluorodeoxyglucose specific therapeutic regimens.9 Overall, have large proximal filling defects. Given
(FDG) positron emission tomography prognosis is poor with mean survival that no single imaging study can rule
(PET), may be helpful in distinguish- rates between 1 and 2 years in most out an intravascular sarcoma, a multi-
ing between thrombi and malignant case series.3,7-9 This is likely due in part modality approach, including CTPA,
lesions. The degree of FDG uptake to delay in diagnosis. A strong index of chest MRI, and PET imaging, may be
tends to be significantly higher in the suspicion is necessary in patients with necessary to improve diagnostic accura-
latter group.6,7 presumed thromboembolic disease who cy. In addition, early referral to a surgical
ERRATUM
The Volume 15, No 1 issue of Advances in Pulmonary Hypertension contained an error in the PH Grand Rounds col-
umn, “Pulmonary Arterial Hypertension: ‘A Journey to Lung Transplant.” The PVR values in Tables 2 and 3 reflect
an incorrect calculation from the original outside medical records. The editors thank Michael Slack, MD, Professor,
Department of Pediatrics, University of Maryland School of Medicine; Director, Pediatric and Adult Congenital Inter-
ventional Cardiac Catheterization Laboratory, University of Maryland Medical Center, Baltimore, for pointing out this
oversight . Dr Slack reinforces that PVR is the basis for many critical clinical decisions and provides the following 3
different ways to express PVR:
Reference Range
. 5 . 3
Measurement dyn s/cm MPa s/m mmHg.min/l or HRU/Wood units
Pulmonary vascular resistance (PVR) 20-130 2-13 0.25-1.6
In cardiac ICUs, the units of dyn.s/cm5 are often used and the conversion factor between this value and Wood units is,
conveniently, 80. During cardiac catheterization, the convention is to use the Cardiac Index (not cardiac output) and
express the PVR in Wood units, which are indexed to body surface area.