Diagnostyka od tego się wszystko zaczyna. Czy nowy algorytm w Nicei 2018?

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1 Diagnostyka od tego się wszystko zaczyna Czy nowy algorytm w Nicei 2018? Adam Torbicki Department of Pulmonary Circulation Thromboembolic Diseases and Cardiology Center of Postgraduate Medical Education ECZ-Otwock, Poland

2 Już PAP > 19 mmhg oznacza gorsze rokowanie mmhg Maron et al. Circulation 2016.

3 Klasyfikacja nadciśnienia płucnego?????? Eur Heart J 2015 doi: /eurheartj/ehv317, Eur Respir J, /

4 Leczenie celowane na tt. płucne? TAK! niewskazane, może być szkodliwe TAK! ostrożnie niewskazane, może być szkodliwe niewskazane brak danych Eur Heart J 2015 doi: /eurheartj/ehv317, Eur Respir J, /

5 Najważniejsze klinicznie postacie NP Nadcisnienie Płucne (NP) Choroby płuc i oskrzeli, hipoksja częste rzadkie Choroby lewego serca Tętnicze NP (PAH) Inne Zakrzepowo-zatorowe NP (CTEPH)

6 Najważniejsze klinicznie postacie NP Nadcisnienie Płucne (NP) Choroby płuc i oskrzeli, hipoksja częste rzadkie 65 palacz z POCHP, nadciśnienie, migotanie przedsionków przewlekła białaczka szpikowa leczona dasatynibem u osoby z wywiadem zakrzepicy żylnej Choroby lewego serca Tętnicze NP (PAH) Inne Zakrzepowo-zatorowe NP (CTEPH)

8 Najważniejsze klinicznie postacie NP Nadcisnienie Płucne (NP) Choroby płuc i oskrzeli, hipoksja częste Jak postępować? rzadkie Choroby lewego serca Tętnicze NP (PAH) Inne Zakrzepowo-zatorowe NP (CTEPH)

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11 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Low Consider common causes of PH: left heart disease and lung disease Consider other causes of symptoms and signs and/or suggest follow-up Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Refer to PH Expert Center Signs of severe PH/RV dysfunction Consider other causes of PH Check for PAH associated conditions and group 5 PH diseases Comprehensive search for CTEPH risk factors and PH-associated diseases/genes Consider V/Q scan Unmatched perfusion defects? Consider pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed Other PA occlusions Group 4 PH CTEPH Group 1/1 PH PAH PVOD/PCH Group 2, 3 or 5 PH

12 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Low 1 Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? 2 Refer to PH Expert Center Consider other causes of symptoms and signs and/or suggest follow-up Signs of severe PH/RV dysfunction Consider V/Q scan Unmatched perfusion defects? Consider other causes of PH 4 Check for PAH associated conditions and group 5 PH diseases 3 Comprehensive search for CTEPH risk factors and PH-associated diseases/genes 5 Consider 6 pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed 7 Other PA occlusions Group 4 PH CTEPH Group 1/1 PH PAH PVOD/PCH Group 2, 3 or 5 PH

13 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Low

14 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Low Consider common causes of PH: left heart disease and lung disease Consider other causes of symptoms and signs and/or suggest follow-up

15 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? V/Q scan Unmatched perfusion defects? Signs of severe PH/RV dysfunction

16 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? V/Q scan Unmatched perfusion defects? Signs of severe PH/RV dysfunction Consider V/Q scan Unmatched perfusion defects?

17 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Signs of severe PH/RV dysfunction Consider V/Q scan Unmatched perfusion defects?

18 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Signs of severe PH/RV dysfunction Consider V/Q scan Unmatched perfusion defects?

19 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Signs of severe PH/RV dysfunction Consider V/Q scan Unmatched perfusion defects? Other PA occlusions

20 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Signs of severe PH/RV dysfunction Consider other causes of PH Check for PAH associated conditions and group 5 PH diseases Consider V/Q scan Unmatched perfusion defects? Other PA occlusions Group 2, 3 or 5 PH

23 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Refer to PH Expert Center Signs of severe PH/RV dysfunction Consider other causes of PH Check for PAH associated conditions and group 5 PH diseases Comprehensive search for CTEPH risk factors and PH-associated diseases/genes Consider V/Q scan Unmatched perfusion defects? Consider pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed Other PA occlusions Group 2, 3 or 5 PH

24 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Refer to PH Expert Center Signs of severe PH/RV dysfunction Consider other causes of PH Check for PAH associated conditions and group 5 PH diseases Comprehensive search for CTEPH risk factors and PH-associated diseases/genes Consider V/Q scan Unmatched perfusion defects? Consider pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed Other PA occlusions Group 4 PH CTEPH Group 1/1 PH PAH PVOD/PCH Group 2, 3 or 5 PH

25 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Low 1 Consider common causes of PH: left heart disease and lung disease Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? 2 Refer to PH Expert Center Consider other causes of symptoms and signs and/or suggest follow-up Signs of severe PH/RV dysfunction Consider V/Q scan Unmatched perfusion defects? Consider other causes of PH 4 Check for PAH associated conditions and group 5 PH diseases 3 Comprehensive search for CTEPH risk factors and PH-associated diseases/genes 5 Consider 6 pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed 7 Other PA occlusions Group 4 PH CTEPH Group 1/1 PH PAH PVOD/PCH Group 2, 3 or 5 PH

26 Symptoms, signs and/or laboratory tests suggestive of PH Echocardiographic probability of PH High or intermediate Low 1 Consider common causes of PH: left heart disease and lung disease 2 Consider other causes of symptoms and signs and/or suggest follow-up Diagnosis of left heart disease or lung disease confirmed? Chronic occlusions on angio CT? V/Q scan Unmatched perfusion defects? Refer to PH Expert Center Signs of severe PH/RV dysfunction Consider V/Q scan Unmatched perfusion defects? Consider other causes of PH 4 Check for PAH associated conditions and group 5 PH diseases 3 Comprehensive search for CTEPH risk factors and PH-associated diseases/genes 5 Consider 6 pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed 7 Other PA occlusions Group 4 PH CTEPH Group 1/1 PH PAH PVOD/PCH Group 2, 3 or 5 PH

27 Cechy sugerujące lewokomorową etiologię PH Prezentacja kliniczna Echokardiografia Inne Wiek >65 lat Objawy niewydolności LK Cechy zespołu metabolicznego Wywiad w kierunku choroby serca utrwalone AF Nieprawidłowa struktura LK wada zastawkowa powiększenie LP (>4,2 cm) uwypuklenie przegrody międzyprzedsionkowej w prawo koncentryczny przerost mięśnia LK i/lub masy LK, dysfunkcja LK E/E ciśnienia napełniania (dopler) > typ 2-3 mitralnej nieprawidłowości przepływu nieobecność: dominującej dysfunkcji PK śródskurczowe zazębienie spektrum przepływu w tętnicy płucnej EKG: lewogram i/lub LAH AF/Afib LBBB obecność załamka Q Inne metody obrazowe: Linie Kerley a wysięk w opłucnej obrzęk płuc powiększenie LP wg wytycznych ESC

28 Diagnostic algorithm for PAH: Focus on Pre-test Probability of PAH Symptoms, signs, history suggestive of PH Probability of PVH/Lung Disease Metabolic Syndrome? yes First consider Echocardiography enlarged Left Atrium? yes Group 2 (PVH) Chest X-Ray upper lobe diversion? yes Abnormal lung function and DLCO <50% yes Group 3 (Lungs) V/Q Scan Mismatched perfusion defects? yes Group 4 (CTEPH) Group 1 (PAH) High Probability Proceed with dedicated RHC Probability of PAH Uprzejmość Prof. Sean Gaine

29 Diagnostic algorithm for PAH: Focus on Pre-test Probability of PAH Symptoms, signs, history suggestive of PH Metabolic Syndrome? yes Echocardiography enlarged Left Atrium? 2 First consider? Probability of PVH/Lung Disease yes Group 2 (PVH) Chest X-Ray upper lobe diversion? yes Abnormal lung function and DLCO <50% yes Group 3 (Lungs) V/Q Scan Mismatched perfusion defects? yes Group 4 (CTEPH) Group 1 (PAH) High Probability Proceed with dedicated RHC Probability of PAH Uprzejmość Prof. Sean Gaine

30 Echocardiographic signs suggesting pulmonary hypertension (in addition to tricuspid regurgitation velocity measurements) 3 RV>LV domination Eur Heart J 2015 doi: /eurheartj/ehv317, Eur Respir J, 2015

31 Echocardiographic signs suggesting precapillary pulmonary hypertension 3 RV>LV domination Eur Heart J 2015 doi: /eurheartj/ehv317, Eur Respir J, 2015

32 Assessment at the PH Expert Center Comprehensive search for CTEPH risk factors and PH-associated diseases/genes 5 Consider 6 pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed 7 Group 2, 3 or 5 PH Possibly Group 1 or 4 with comorbiditiess Specific treatment with early reassessment (< 3 months)? Multifactorial PH Consider specific treatment with very early reassessment (< 1 months)?

33 Assessment at the PH Expert Center Comprehensive search for CTEPH risk factors and PH-associated diseases/genes 5 Consider 6 pulmonary angiograpy RHC with vasoreactivity, exercise, fluid challenge as needed 7 Other PA occlusions Group 4 PH CTEPH Vasoreactive Group 1/1 PH PAH PVOD/PCH Group 2, 3 or 5 PH with venous component Possibly Group 1 or 4 with comorbiditiess Specific treatment with early reassessment (< 3 months)? Multifactorial PH Consider specific treatment with very early reassessment (< 1 months)?

34 Diastolic Pulmonary Gradient (DPG) warunkuje rokowanie w gr. 2 NP z wysokim TPG Pacjenci z RHC N= 3,107 Groupa 2 NP N= 1,094 TPG < 12 mmhg N= 604 TPG > 12 mmhg N= 490 DPG > 7 mmhg N= % pacjentów miałą NP wtórne do LK, 55% bierne 36% z tych z TPG > 12 miało DPG > 7 mmhg (16% całej populacji) DPG < 7 mmhg N= 311 Gerges C et al. CHEST 2012 Cut-off for DPG determined by ROC-curve analysis

35 Co może zrobić ośrodek referencyjny podczas RHC Hemodynamiczne testy obciążeniowe dla pacjentów granicznych Test PAP Pw CO SvO2 Obciążenie płynami Bierne uniesienie kkd? Test z /ILO? Test wysiłkowy Grupa 2

36 Co może zrobić ośrodek referencyjny podczas RHC Hemodynamiczne testy obciążeniowe dla pacjentów granicznych Test PAP Pw CO SvO2 Obciążenie płynami Bierne uniesienie kkd Test z /ILO? Test wysiłkowy Grupa 3

38 Wnioski Diagnostyka różnicowa NP u osób: starszych z chorobami współistniejącymi po zatorowości płucnej jest trudna i wymaga standaryzacji: testów obciążeniowych sposobu oceny próbnie włączanej terapii rejestrowania uzyskanych danych dla późniejszej zbiorczej analizy.

39 bo przecież wszystko zaczyna się od diagnostyki Adam Torbicki Department of Pulmonary Circulation Thromboembolic Diseases and Cardiology Center of Postgraduate Medical Education ECZ-Otwock, Poland