Pulmonal hypertensjon Arne K Andreassen Kardiologisk avdeling Oslo
- Slides: 40
Pulmonal hypertensjon Arne K. Andreassen Kardiologisk avdeling Oslo Universitetssykehus Rikshospitalet
Clinical classification of pulmonary hypertension 1. Pulmonary arterial hypertension (PAH) 2. Pulmonary hypertension due to left heart disease 3. Pulmonary hypertension due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions 5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
Clinical classification of PH Galie N, et al. Eur Heart J 2015; published August 29
Hemodynamic definitions of PH Galie N, et al. Eur Heart J 2015; published August 29
Right heart catheterization (Swan -Ganz catheter)
Clinical classification of pulmonary hypertension 1. Pulmonary arterial hypertension (PAH) 2. Pulmonary hypertension due to left heart disease 3. Pulmonary hypertension due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions 5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
Pulmonary arterial hypertension Galie N, et al. Eur Heart J 2015; published August 29
Patogenese ved PAH Gaine S. JAMA 2000; 284: 3160 -4
Survival in IPAH Percentage surviving Formula for survial: 100 A(x, y, z) = e(0. 007352 x + 0. 0526 y – 0. 3275 z) 80 Where x = MAP y = RAP z = CI 60 40 20 1 2 3 4 5 Years of follow-up D`Alonzo GE et al. Ann Intern Med 1991; 115: 343 -9
Ekkokardiografi og histologi
Right-heart catheterization
Akutt vasodilatasjonstest Respondere Ikke – respondere MAP og PAR reduseres til nær normale verdier Ca-blokkere Endotelinblokkere PDE-5 blokkere Prostaglandiner
Targets for current therapies in PAH
Targeted drug therapy in Norway Drug NYHA-class Dosage Calcium channel blockers Endothelin receptor antagonists Ambrisentan Bosentan Macitentan II, III 5 -10 mg X 1 125 mg X 2 10 mg X 1 Phosphodiesterase type-5 inhibitors Sildenafil Tadalafil II, III 20 mg X 3 40 mg X 1 Soluble guanylate cyclase stimulators Riociguat II, III 2. 5 mg X 3 IP-receptor stimulators Selexipag II, III 1600 mg X 2 Prostanoids Epoprostenol (iv) Iloprost (inh) Treprostinil (iv, sc) III-IV III 15 -50 ng/kg/min 10µg X 6 -9 inh 20 -50 ng/kg/min Andreassen AK et al Tidsskr Nor Legeforen 2011; 131: 1285 -8
Observed and estimated survival in IPAH N = 32 Andreassen AK et al Tidsskr Nor Legeforen 2011; 131: 1285 -8
Meta-analysis of randomized trials in PAH Trials: 21 N=3140 Duration 14. 3 weeks All-cause mortality Among controls: 3. 8% Reduction in mortality: 43% (RR 0. 57; 95% CI 0. 35 -0. 92; p=0. 023) Galié N et al. Eur Heart J 2009; 30; 394 -403
Lung transplantation Yusen RD, et al. J Heart Lung Transplant 2013; 32: 965 -78
Clinical classification of pulmonary hypertension 1. Pulmonary arterial hypertension (PAH) 2. Pulmonary hypertension due to left heart disease 3. Pulmonary hypertension due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions 5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
Left ventricular heart failure and PH Rosenkranz S, et al. Eur Heart J 2016; 37: 942 -54
RV failure added to LV failure N = 377 VVEF < 35% Ghio S et al. J Am Coll Cardiol 2001; 37: 183 -8
Treatment algorithm for systolic heart failure Mc. Murray JJ. N Engl J Med 2010; 362: 228 -38
Clinical classification of pulmonary hypertension 1. Pulmonary arterial hypertension (PAH) 2. Pulmonary hypertension due to left heart disease 3. Pulmonary hypertension due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions 5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
Cor pulmonale (WHO 1963): ”hypertrophy of the RV resulting from diseases affecting the function and/ or structure of the lungs, except when these pulmonary alterations are the result of diseases that primarily affect the left side of the heart, as in congenital diseases” Høyrekateterisering (m/1 L O 2) RA (mm. Hg) 6 MAP (mm. Hg) 27 PCV (mm. Hg) 7 CI (L/min/m 2) 2, 2 PAR (WU) 4, 9 HR (min-1) 84 AP metn. (%) 57 Ao metn. (%) 90
COPD and PH N=409 LTx 1991 -2010 Distribution of PH Partial pressures of O 2 and CO 2 Accounted for m. PAP variance Survival Andersen K, et al. J Heart Lung Transplant 2012; 31: 373 -80
Clinical classification of pulmonary hypertension 1. Pulmonary arterial hypertension (PAH) 2. Pulmonary hypertension due to left heart disease 3. Pulmonary hypertension due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions 5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
Pulmonary angiograms
Pulmonary endarterectomy
Long term outcome in CTEPH Delcroix M, et al. Circulation 2016; 133: 859 -71
Surgical specimen Type I disease (10 %) Type II disease (70 %) Type III disease (20 %) Madani M M and Jamieson SW. Advances in Pulmonary Hypertension 2007; 6(2): 83 -91
CTEPH international registry – patient disposition Pepke-Zaba J et al. Circulation 2011; 124: 1973 -81
Heart 2013; 99: 1415– 1420. doi: 10. 1136/heartjnl-2012 -303549
BPA of occluded vessels
Hemodynamics pre- and post-PBA Andreassen AK et al. Heart 2013; 99: 1415 -20
RV following BPA: reverse remodeling
CT angiogram pre- and post BPA Kataoka M et al. Circ Cardiovasc Interv 2012; 5: 756 -762
CHEST-1 (Riociguat: Adempas®) Ghofrani H-A et al. N Engl J Med 2013; 369: 319 -29
Clinical classification of pulmonary hypertension 1. Pulmonary arterial hypertension (PAH) 2. Pulmonary hypertension due to left heart disease 3. Pulmonary hypertension due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions 5. Pulmonary hypertension with unclear and/or multifactorial mechanisms
PH with unclear and/or multifactorial mechanisms 5. 1 Hematological disorders: myeloprolifertaive disorders, splenectomy, hemolytic anemia 5. 2 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis 5. 3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders 5. 4 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis
PH with unclear and/or multifactorial mechanisms: tumoral obstruction Freim Wahl SG et al. Tidsskr Nor Legeforen 2012; 132: 1877 -80
Diagnostic algorithm for PH Mc. Laughlin VV et al. Circulation 2006; 114: 1417 -31
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