CHRONISCHE TROMBOEMBOLISCHE PULMONALE HYPERTENSIE (CTEPH) EPIDEMIOLOGIE, FYSIOPATHOLOGIE EN DIAGNOSTIEK MARION DELCROIX
PH CLASSIFICATION (NICE 2013) Group
Description
Group 1 Pulmonary arterial hypertension (PAH) Group 2 Pulmonary hypertension (PH) due to left heart disease Group 3 PH due to lung diseases and/or hypoxia Group 4 Chronic thromboembolic PH (CTEPH) and … Group 5 Unclear or multifactorial mechanisms Galie et al, EHJ and ERJ 2015
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Galie et al, EHJ and ERJ 2015
CTEPH DEFINITION mean PAP 25 mmHg abnormal ventilation-perfusion (V/Q) scan
1. 2. – –
3. 4.
one or more segmental-sized or larger mismatched perfusion defects
abnormal CT scan and/or abnormal pulmonary angiography showing typical findings of CTEPH at least 3 months of anticoagulation
Registry including * only PAH and CTEPH cases, † only incident PAH and CTEPH cases, or ‡ only incident CTEPH patients; prevalence and incidence are expressed in cases per million inhabitants and per million inhabitants per year, respectively; § evolution over the years; # country with highest incidence (unpublished data)
Delcroix et al, Ann Am Thorac Soc 2015 (in press)
HISTORY OF PE IN CTEPH PATIENTS CTEPH risk factors(2) CTEPH associated
Pepke-Zaba et al, Circulation 2011; 124: 1973 ISHLT 2011
ANGIOGENESIS
Quarck et al, Eur Respir J 2015
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ANGIOGENESIS 120
120
100
100
Percent survival
Percent survival
Long-term survival PEA 2004-2009
80 60 40 20
Late Ang>0.8
80
p=0.01
60
Late Ang1 (CT) • sPAP >60 mmHg (TTE)
CTEPH prediction score5: CTEPH risk factors4 1. unprovoked PE Splenectomy, VA shunt for 2. hypothyroidism hydrocephaly, chronic inflammatory 3. symptom onset >2 weeks before PE diagnosis disorders, non-O blood group 4. RV dysfunction on CT or TTE (at 6 months) 5. no diabetes mellitus 6. no thrombolytic therapy or embolectomy
1. Pepke-Zaba J et al. Circulation 2011;124:1973–81. 2. Guérin L et al. Thromb Haemost 2014;112:598–605. 3. Klok FA et al. Thromb Res 2011;128:21–6. 4. Bonderman D et al. Eur Respir J 2009;33:325–31. 5. Klok et al. J Thromb Haemost 2015
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FACTORS SUGGESTIVE FOR CTEPH AFTER ACUTE PE At the time of index PE
In the follow up of acute PE
Massive and recurrent PE1
New or worsened dyspnea
PH already present2:
RV hypertrophy (ECG) and increased NTproBNP3
• RV/LV diameter >1 (CT) • sPAP >60 mmHg (TTE)
CTEPH prediction score5: CTEPH risk factors4 1. unprovoked PE Splenectomy, VA shunt for 2. hypothyroidism hydrocephaly, chronic inflammatory 3. symptom onset >2 weeks before PE diagnosis disorders, non-O blood group 4. RV dysfunction on CT or TTE (at 6 months) 5. no diabetes mellitus 6. no thrombolytic therapy or embolectomy
1. Pepke-Zaba J et al. Circulation 2011;124:1973–81. 2. Guérin L et al. Thromb Haemost 2014;112:598–605. 3. Klok FA et al. Thromb Res 2011;128:21–6. 4. Bonderman D et al. Eur Respir J 2009;33:325–31. 5. Klok et al. J Thromb Haemost 2015