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References
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2. Moser KM, Bloor CM.Pulmonary vascular lesions occur-
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3. Dorfmüller P, Günther S, Ghigna M-R, Thomas de
Montpréville V, et al. Microvascular disease in chronic
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4. Ende-Verhaar YM, Cannegieter SC, Vonk Noordegraaf A,
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5. Klok FA, van der Hulle T, den Exter PL, Lankeit M,
Huisman MV, Konstantinides S.The post-PE syndrome: a
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6. Gopalan D, Blanchard D, Auger WR.Diagnostic evalua-
tion of chronic thromboembolic pulmonary hypertension.
Ann Am Thorac Soc. 2016;13(Suppl 3):S222–39.
Philadelphia, PA WilliamR.Auger
London, UK DeepaGopalan
Preface
xi
1 Case 1: The Evaluation of Chronic Thromboembolic
Pulmonary Hypertension ........................ 1
Manreet Kanwar, Deepa Gopalan,
and William R. Auger
2 Case 2: There’s No Perfusion to that Lung!
An Unusual Presentation for CTEPH .............. 11
Mona Alotaibi, Victor Pretorius,
Deepa Gopalan, and William R. Auger
3 Case 3: Distal Chronic Thromboembolic Disease—
Is Surgery an Option? ........................... 21
Anjali Vaidya, Deepa Gopalan,
and William R. Auger
4 Case 4: The Case for Balloon Pulmonary
Angioplasty .................................... 33
David S. Poch, Deepa Gopalan,
and William R. Auger
5 Case 5: Operable CTEPH …but Is PTE
the Best Option? ................................ 41
Sonja Bartolome, Deepa Gopalan,
and William R. Auger
6 Case 6: Distal Vessel Thromboendarterectomy—
How Far Can We Go? ........................... 51
Angela Bautista, Michael M. Madani,
Deepa Gopalan, and William R. Auger
Contents
xii
7 Case 7: It’s Not CTEPH, but It Is Chronic
Thromboembolic Disease (CTED) ................. 61
Paul R. Fora, Deepa Gopalan,
and William R. Auger
8 Case 8: Another Case of CTED:
Is Surgery the Way? ............................. 71
Colleen McEvoy, Deepa Gopalan,
and William R. Auger
9 Case 9: Pulmonary Artery Sarcoma Mimicking
Massive Thromboembolic Disease ................. 81
Demosthenes G. Papamatheakis, Deepa Gopalan,
and William R. Auger
10 Case 10: Chronic Thrombus and Metastatic
Cancer—An Unexpected CTEPH Mimic ........... 91
Alison S. Witkin and William R. Auger
11 Case 11: Don’t Forget About the Pulmonary Veins! ... 99
Terence K. Trow, Deepa Gopalan,
and William R. Auger
12 Case 12: Pulmonary Veno-Occlusive Disease …
but It Started Out as CTEPH .....................109
Rana Awdish, Sara Hegab, Deepa Gopalan,
and William R. Auger
13 Case 13: Pulmonary Arteritis… The Great
CTEPH Mimic .................................121
Kim M. Kerr, Albert Hsiao, and William R. Auger
14 Case 14: Sarcoidosis and Large Vessel Pulmonary
Vascular Disease—Another CTEPH Mimic .........133
Robert Schilz, Deepa Gopalan,
and William R. Auger
15 Case 15: CTEPH? But the Lung Scan Is Normal! ....141
Victor J. Test, Deepa Gopalan,
and William R. Auger
Contents
xiii
16 Case 16: Acute Versus Chronic Thromboembolic
Disease—Beware!. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .147
Dianne L. Zwicke, Sara Paulus, Deepa Gopalan,
and William R. Auger
17 Case 17: Can Severe Pulmonary Arterial
Pulmonary Hypertension Mimic CTEPH? ..........157
Jean M. Elwing, Deepa Gopalan,
and William R. Auger
18 Case 18: Atrial Septal Defect with Chronic
Thrombus Mimicking CTEPH ....................167
Timothy M. Fernandes, Deepa Gopalan,
and William R. Auger
19 Case 19: Parenchymal Lung Disease
and Chronic Thromboemboli .....................177
Lynette Brown, Deepa Gopalan,
and William R. Auger
Index .............................................185
Contents
xv
Contributors
Mona Alotaibi Pulmonary Fellow, Division of Pulmonary
and Critical Care Medicine, University of California, San
Diego, San Diego, CA, USA
William R. Auger Lewis Katz School of Medicine, Temple
University, Philadelphia, PA, USA
Rana Awdish 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
Sonja Bartolome CTEPH Program, UT Southwestern
Medical Center, Dallas, TX, USA
Pulmonary and Critical Care Medicine, UT Southwestern
Medical Center, Dallas, TX, USA
AngelaBautista CTEPH Program, University of California,
San Diego, San Diego, CA, USA
LynetteBrown Asssociate Professor (Clinical), University of
Utah, Salt Lake, UT, USA
Pulmonary Hypertension Program, Intermountain Medical
Center, Murray, UT, USA
xvi
JeanM.Elwing Pulmonary Hypertension Program, Division
of Pulmonary Critical Care Medicine, University of Cincinnati
College of Medicine, Cincinnati, OH, USA
TimothyM. Fernandes Division of Pulmonary and Critical
Care Medicine, University of California, San Diego, San
Diego, CA, USA
PaulR.Forfia Pulmonary Hypertension, Right Heart Failure
and CTEPH Program, Temple University Hospital,
Philadelphia, PA, USA
DeepaGopalan Department of Radiology, Imperial College
Hospitals, London, UK
SaraHegab 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
AlbertHsiao Asssociate Professor in Residence, Department
of Radiology, University of California, San Diego, San Diego,
CA, USA
Manreet Kanwar Cardiovascular Institute at Allegheny
Health Network, Pittsburgh, PA, USA
Kim M. Kerr Division of Pulmonary and Critical Care
Medicine, University of California, San Diego, San Diego,
CA, USA
MichaelM.Madani University of California, San Diego, San
Diego, CA, USA
Colleen McEvoy Division of Pulmonary and Critical Care
Medicine, Washington University in St. Louis, St. Louis, MO,
USA
Demosthenes G. Papamatheakis Pulmonary, Critical Care
and Sleep Medicine, University of California, San Diego, La
Jolla, CA, USA
Contributors
xvii
Sara Paulus Pulmonary Hypertension Clinic, Aurora St.
Luke’s Medical Center, Milwaukee, WI, USA
DavidS.Poch University of California, San Diego, La Jolla,
CA, USA
Victor Pretorius Division of Cardiovascular and Thoracic
Surgery, University of California, San Diego, San Diego, CA,
USA
Heart Transplant and MCS Program, University of California,
San Diego, San Diego, CA, USA
Robert Schilz CWRU School of Medicine, Cleveland, OH,
USA
Lung Transplantation and Pulmonary Vascular Disease,
University Hospitals of Cleveland, Cleveland, OH, USA
VictorJ.Test Division of Pulmonary Medicine and Critical
Care, Texas Tech University School of Medicine, Lubbock,
TX, USA
Terence K. Trow Pulmonary, Critical Care, and Sleep
Medicine, DynaMed Plus, EBSCO Health Information
Services, Ipswich, MA, USA
Yale Pulmonary Vascular Disease Program, Yale School of
Medicine, New Haven, CT, USA
Anjali 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
Alison S. Witkin Division of Pulmonary and Critical Care
Medicine, Massachusetts General Hospital, Harvard Medical
School, Boston, MA, USA
Dianne L. Zwicke Pulmonary Hypertension Clinic, Aurora
St. Luke’s Medical Center, Milwaukee, WI, USA
Contributors
1© 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_1
Case Presentation
Patient is a 68-year-old African American male who was
referred to us for evaluation of worsening shortness of breath
in the setting of previous history of pulmonary emboli (PE)
and recently diagnosed pulmonary hypertension (PH).
Chapter 1
Case 1: The Evaluation of
Chronic Thromboembolic
Pulmonary Hypertension
ManreetKanwar, DeepaGopalan, andWilliamR.Auger
M. Kanwar (*)
Cardiovascular Institute at Allegheny Health Network,
Pittsburgh, PA, USA
e-mail: manreet.kanwar@ahn.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
2
He was initially diagnosed with unprovoked bilateral deep
venous thrombi (DVT) and PE in 2013. Hypercoagulability
work-up was negative. He was started on warfarin for antico-
agulation which was subsequently discontinued after a year.
He again presented in June 2016 with worsening shortness of
breath from a recurrent PE.Anticoagulation was resumed
with apixaban with some initial improvement in his symp-
toms. An IVC filter was placed (although there was no docu-
mented recurrent DVT). The patient reported increasing
shortness of breath over the following months, with associ-
ated lower extremity swelling and abdominal distention. He
was evaluated as an outpatient and started on diuretics and
treated for bronchitis for several weeks. His symptoms even-
tually started affecting his activities of daily living when he
presented to an outside hospital for evaluation in September
2016, after an event of near syncope following moderate
exertion.
His initial chest radiograph (Fig. 1.1) and lab work were
unremarkable for any “acute findings”.
An echocardiogram showed severe right ventricular dila-
tation (RVEDD 57mm) with moderately reduced right ven-
tricular global systolic function (TAPSE 11 mm) and mild
hypertrophy (Fig. 1.2). His left ventricular ejection fraction
was preserved at 65% and his calculated pulmonary artery
F . Chest radiograph upon presentation
M. Kanwar et al.
3
systolic pressure (PASP) was about 105mmHg, with a small,
circumferential pericardial effusion.
Given his previous history of PEs, he underwent a repeat
CT angiogram of his chest which showed chronic pulmonary
emboli bilaterally (Fig.1.3). The largest thrombus burden was
at the distal portion of the right pulmonary artery with exten-
sion and occlusion of the right lower lobe interlobar pulmo-
nary artery as well as the proximal right middle lobe
pulmonary artery. At that point he was started on diuretics,
F . TTE pre-op (parasternal long-axis view, four-chamber
view; TR jet). The right ventricle is severely dilated with moderately
to severely reduced systolic function. There is moderate hypertrophy
of the right ventricle. The right ventricular systolic pressure is
101mmHg
F . CT angiography of the chest with findings diagnostic of
CTEPH.Enlarged bronchial collateral artery: solid white arrow
Chapter 1. Case 1: The Evaluation of Chronic…