chronic thromboembolic pulmonary hypertension
TRANSCRIPT
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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
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Disclosures
Research Support:Actelion, Gilead, United Therapeutics
Consultancy:Bayer
CTEPH PAH
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Natural History of Chronic Thromboembolic
Pulmonary Hypertension
ACUTE
PE CTEPH
Resolution without
Hemodynamic Compromise
96-99%
Genetic / Intrinsic Variables
Prothrombotic Tendencies
Recurrent TE Events
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Natural History of Chronic Thromboembolic
Pulmonary Hypertension
ACUTE
PE CTEPH
Genetic / Intrinsic Variables
Prothrombotic Tendencies
Recurrent TE Events
Small Vessel Changes
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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.
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VQ Scan: Screening Test of Choice
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• 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%
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Pitfalls with CTPA:(Under-Dx CTEPH, Over-Dx PAH?)
PAH-QuERI: PAH dx’d without VQ = 43%
McLaughlin VV et al. CHEST. 2012.
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CTPA: CTE disease?
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VQ Scan (same patient)
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Reichelt et al. Eur J Radiol 2008
CTEPH: Multi-Slice CTA
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Kreitner KFJ, et al. Radiology 2004
CTEPH: MR Angiogram
PRE POST
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CTEPH: Angioscopy
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What’s Impressive?
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CTEPH: Experience Matters
Thorough Endarterectomy Jamieson Type III Disease
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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
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Not Always Just A PTE
Slides courtesy of: W. Auger
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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
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AATS Toronto, May 2010 22
CTEPH Registry - Operability
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AATS Toronto, May 2010 23
Mortality – Center Expertise
%
PEA per yearWilcoxon 2-sample test
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The meeting has been organized by the Association for Research in CTEPH, in close collaboration withPapworth Hospital, a University of Cambridge Teaching Hospital
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CTEPH (blue) vs PAH (green)Pubmed results
YEAR
# o
f A
rtic
les
Total: 3,640
Total: 651
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CTEPH: Hot Topics
• Operability Criteria
• Access/number of PEA Centers
• Role of Medical Therapy
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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?
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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
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Dartevelle P, et al. Eur Respir J 2004
CTEPH: Preoperative PVR
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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
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Residual PH after PEA
Galie N and Kim NH. PATS 2006
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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
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“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
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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)