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Chronic Thromboembolic Pulmonary Hypertension Nick H. Kim, M.D. Associate Clinical Professor of Medicine Pulmonary and Critical Care Medicine Director, Fellowship Program Director, Pulmonary Vascular Program University of California, San Diego

Chronic Thromboembolic Pulmonary Hypertension

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Page 1: Chronic Thromboembolic Pulmonary Hypertension

Chronic Thromboembolic

Pulmonary Hypertension

Nick H. Kim, M.D.

Associate Clinical Professor of Medicine

Pulmonary and Critical Care Medicine

Director, Fellowship Program

Director, Pulmonary Vascular Program

University of California, San Diego

Page 2: Chronic Thromboembolic Pulmonary Hypertension

Disclosures

Research Support:Actelion, Gilead, United Therapeutics

Consultancy:Bayer

CTEPH PAH

Page 3: Chronic Thromboembolic Pulmonary Hypertension

Natural History of Chronic Thromboembolic

Pulmonary Hypertension

ACUTE

PE CTEPH

Resolution without

Hemodynamic Compromise

96-99%

Genetic / Intrinsic Variables

Prothrombotic Tendencies

Recurrent TE Events

Page 4: Chronic Thromboembolic Pulmonary Hypertension

Natural History of Chronic Thromboembolic

Pulmonary Hypertension

ACUTE

PE CTEPH

Genetic / Intrinsic Variables

Prothrombotic Tendencies

Recurrent TE Events

Small Vessel Changes

Page 5: Chronic Thromboembolic Pulmonary Hypertension
Page 6: Chronic Thromboembolic Pulmonary Hypertension

Clinical Classification of Pulmonary

Hypertension (Dana Point 2008)

1. PAH

• Idiopathic PAH

• Heritable

• Drug- and toxin-induced

• Persistent PH of newborn

• Associated with:

−CTD

−HIV infection

−portal hypertension

−CHD

−schistosomiasis

−chronic hemolytic anemia

1’. PVOD and PCH

2. PH Due to Left Heart Disease

• Systolic dysfunction

• Diastolic dysfunction

• Valvular disease

3. PH Due to Lung Diseases and / or Hypoxia

• COPD

• ILD

• Other pulmonary diseases with mixed

restrictive and obstructive pattern

• Sleep-disordered breathing

• Alveolar hypoventilation disorders

• Chronic exposure to high altitude

• Developmental abnormalities

4. CTEPH

5. PH With Unclear Multifactorial Mechanisms

• Hematologic disorders

• Systemic disorders

• Metabolic disorders

• Others

Simonneau G et al. J Am Coll Cardiol. 2009;54:S43-S54.

Page 7: Chronic Thromboembolic Pulmonary Hypertension
Page 8: Chronic Thromboembolic Pulmonary Hypertension

VQ Scan: Screening Test of Choice

Page 9: Chronic Thromboembolic Pulmonary Hypertension

• Retrospective: compared with DSA

• Of 78 CTEPH pts confirmed by DSA:VQ: 75 high, 1 intermediate, 2 lowCTPA: 40 positive, 38 negative

• VQ: sens 96-97%, spec 90-95%

• CTPA: sens 51%, spec 99%

Page 10: Chronic Thromboembolic Pulmonary Hypertension

Pitfalls with CTPA:(Under-Dx CTEPH, Over-Dx PAH?)

PAH-QuERI: PAH dx’d without VQ = 43%

McLaughlin VV et al. CHEST. 2012.

Page 11: Chronic Thromboembolic Pulmonary Hypertension

CTPA: CTE disease?

Page 12: Chronic Thromboembolic Pulmonary Hypertension

VQ Scan (same patient)

Page 13: Chronic Thromboembolic Pulmonary Hypertension

Reichelt et al. Eur J Radiol 2008

CTEPH: Multi-Slice CTA

Page 14: Chronic Thromboembolic Pulmonary Hypertension

Kreitner KFJ, et al. Radiology 2004

CTEPH: MR Angiogram

PRE POST

Page 15: Chronic Thromboembolic Pulmonary Hypertension

CTEPH: Angioscopy

Page 16: Chronic Thromboembolic Pulmonary Hypertension
Page 17: Chronic Thromboembolic Pulmonary Hypertension

What’s Impressive?

Page 18: Chronic Thromboembolic Pulmonary Hypertension

CTEPH: Experience Matters

Thorough Endarterectomy Jamieson Type III Disease

Page 19: Chronic Thromboembolic Pulmonary Hypertension

UCSD Surgical Experience1984 - 2012

0

20

40

60

80

100

120

140

160

1984 1985 1986 1987 1988 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012

PTE SurgeriesProjected

Slides courtesy of: W. Auger

Page 20: Chronic Thromboembolic Pulmonary Hypertension

Not Always Just A PTE

Slides courtesy of: W. Auger

Page 21: Chronic Thromboembolic Pulmonary Hypertension

Perioperative Mortality Rates: UCSD

0

2

4

6

8

10

12

14

1615.8

9.4

7.6

3.8

5.3

6.7

8.3

5.2

12.3

7.4

4.5 4.55.3

8.5

2.6

5.74.8

3.42.6 2.5

3.2

00.8

%

Slides courtesy of: W. Auger

Page 22: Chronic Thromboembolic Pulmonary Hypertension

AATS Toronto, May 2010 22

CTEPH Registry - Operability

Page 23: Chronic Thromboembolic Pulmonary Hypertension

AATS Toronto, May 2010 23

Mortality – Center Expertise

%

PEA per yearWilcoxon 2-sample test

Page 24: Chronic Thromboembolic Pulmonary Hypertension

The meeting has been organized by the Association for Research in CTEPH, in close collaboration withPapworth Hospital, a University of Cambridge Teaching Hospital

Page 25: Chronic Thromboembolic Pulmonary Hypertension

CTEPH (blue) vs PAH (green)Pubmed results

YEAR

# o

f A

rtic

les

Total: 3,640

Total: 651

Page 26: Chronic Thromboembolic Pulmonary Hypertension

CTEPH: Hot Topics

• Operability Criteria

• Access/number of PEA Centers

• Role of Medical Therapy

Page 27: Chronic Thromboembolic Pulmonary Hypertension

CTEPH and Operability Assessment

1) Is there chronic thromboembolic disease?

2) What is the PVR?

3) How experienced is your surgeon?

4) How experienced is your pre/post-operative team?

Page 28: Chronic Thromboembolic Pulmonary Hypertension

Preoperative Evaluation

• Is there CTE (proximal) disease? VQ scan / PA angio / angioscopy

CT angio / MRA / PA ultrasound

• Is there microvascular (inoperable) disease? Hemodynamic to radiographic discrepancy / Expert

opinion

Page 29: Chronic Thromboembolic Pulmonary Hypertension

Dartevelle P, et al. Eur Respir J 2004

CTEPH: Preoperative PVR

Page 30: Chronic Thromboembolic Pulmonary Hypertension

Of 22 deaths (4.4%):

• 17 (77%) had residual pulmonary hypertension

• Post-op PVR > 500 dsc-5 had 30.6% mortality

• Post-op PVR < 500 dsc-5 had 0.9% mortality

Jamieson SW, et al. Ann Thorac Surg 2003

500 cases 1998 – 2002

Page 31: Chronic Thromboembolic Pulmonary Hypertension

Residual PH after PEA

Galie N and Kim NH. PATS 2006

Page 32: Chronic Thromboembolic Pulmonary Hypertension

Rationale for Medical Therapy

• Pathologic evidence of concomitant small vessel disease (Moser & Bloor ’93, Yi et al ‘00)

• Hemodynamic progression in the absence of new perfusion defects (Moser & Bloor ’93)

• Discordance between hemodynamics and radiographically apparent burden of disease (Azarian

R, et al. ’97)

• Numerous uncontrolled reports of PAH-specific therapies having beneficial results in select CTEPH patients

• Availability of PAH therapy versus PEA center

Page 33: Chronic Thromboembolic Pulmonary Hypertension

“Inoperable” CTEPH: RCTs

• Iloprost (AIR), n=57*6MW+FCOlschewski H, et al. N Engl J Med 2002; 347:322-9

• Sildenafil (UK Pilot Study), n=196MWSuntharalingam J, et al. Chest 2008; 134:229-36

• Bosentan (BENEFIT), n=1576MW or PVRJais X, et al. JACC 2008; 52:2127-34

Page 34: Chronic Thromboembolic Pulmonary Hypertension

CTEPH Summary

• Defining role of medical treatment(to be continued…)

• Screen PH with VQ scan

• Refer to PEA centers(not just for surgery but also for adjudication)

• Always consider surgery(operability is dependent on numerous factors)