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Ipertensione polmonare

Ipertensione polmonare. Piergiuseppe Agostoni, MD, PhD Centro Cardiologico Monzino, IRCCS Dipartimento di Scienze Cardiovascolari, Università di Milano, Milano Division of Respiratory Disease, University of Washington, Seattle, WA.

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Ipertensione polmonare

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  1. Ipertensione polmonare Piergiuseppe Agostoni, MD, PhD Centro Cardiologico Monzino, IRCCS Dipartimento di Scienze Cardiovascolari, Università di Milano, Milano Division of Respiratory Disease, University of Washington, Seattle, WA

  2. Distribution of values for forced vital capacity (FVC) (upper) and gas transfer index or diffusing capacity for carbon monoxide (DL ) (lower) in 79 primary pulmonary hypertension (PPH) patients (lines from upper left to lower right) and 20 normal controls (lines from lower left to upper right). Values are divided by deciles of percent predicted (%pred). For these measurements, all individuals below 80% of predicted are below the normal 95% confidence limits. Approximately 50% of the PPH patients have a reduced FVC and 75% have a reduced DL. Sun et al., JACC. 2003;41(6):1028-35

  3. Correlation of resting lung function measurements in 79 primary pulmonary hypertension (PPH) patients. Each symbol indicates an individual PPH patient. Upper left: Values are percent predicted (%pred) for FVC and FEV1; upper right: absolute values for FEV1 and MVV; lower left: absolute values for alveolar volume determined from single breath dilution of inert gas (V ′) and TLC determined by body plethysmography or N2 washout method; and lower right: %pred values for DLand FVC. Sun et al., JACC. 2003;41(6):1028-35

  4. Correlations and regression equations for gas transfer index (DL ) versus three cardiopulmonary exercise test parameters of aerobic function (upper = peak O2 uptake; middle = anaerobic threshold; LOWER = peak O2 pulse) in primary pulmonary hypertension (PPH) patients. Each symbol indicates an individual PPH patient. All values and equations are in units of % predicted (%pred). Dotted lines approximate the 95% confidence limits of controls. Sun et al., JACC. 2003;41(6):1028-35

  5. Sun et al., JACC. 2003;41(6):1028-35

  6. EXERCISE EVALUATION • SIX-MINUTE WALKING TEST • COSTANT WORK-LOAD CARDIOPULMONARY TEST • RAMP-PROTOCOL CARDIOPULMONARY TEST

  7. SIX-MINUTE WALKING TEST • EASY TO PERFORM • 6 – 12 MINUTES DURATION • NEED FOR PRELIMINARY FAMILIARIZATION TEST • NEED TO FOLLOW ATS CRITERIA

  8. SIX-MINUTE WALKING TEST • NORMAL SUBJECTS: - VO2p (R = 0.90) • SUBJECTS WITH SECONDARY PULMONARY HYPERTENSION: - VO2p R = 0.52 - Vemax R = 0.53

  9. From: Clinical Correlates and Prognostic Significance of Six-minute Walk Test in Patients with Primary Pulmonary Hypertension. S. Miyamoto et al., Am J Respir. Crit Care Med 2000; 161: 487-492.

  10. From: ClinicalCorrelates and PrognosticSignificance of Six-minuteWalk Test in PatientswithPrimary Pulmonary Hypertension. S. Miyamotoet al., Am J Respir. Crit Care Med 2000; 161: 487-492.

  11. (A)Long-term (7-year) survival in patientswithidiopathicpulmonaryarterialhypertension (IPAH) based on functionalclass (III vs. IV) at the timeofepoprostenolinitiation. P = 0.0001 bylog-rank test (30). (B)Survival in patientswith IPAH treatedwithintravenousepoprostenolaccordingto New York HeartAssociation (NYHA) functionalclass. Estimatedpercentagesofsurvivalforpatients in NYHA functionalclass IV at baseline(dashedline)were 76%, 60%, and 47% at one, two, and threeyears, respectively, ascomparedwith 90%, 76%, and 71% forpatients in NYHA functionalclass III at baseline(solidline) (p < 0.001 by the Cox-Mantellog-rank test) (32). Barst et al., J Am Coll Cardiol. 2004 43(12 Suppl S):40S-47S.

  12. Ulrichet al.,SWISS MED WKLY 20 07;137:573–580

  13. Mean ({+/-}SE) change in 6-minute walking distance from baseline to week 15 in the primary training, control, and secondary training groups (10 members of the control group reentered the study and received 15 weeks of exercise training) Mereles, D. et al. Circulation 2006;114:1482-1489

  14. Cardiopulmonaryexercisetesting and six-minutewalkcorrelations in pulmonaryarterialhypertension. Oudiz RJ, Barst RJ, Hansen JE, Sun XG, Garofano R, Wu X, Wasserman K.

  15. TEST A CARICO COSTANTE Sotto soglia cinetica VO2 (fase iniziale esercizio) Sopra soglia cinetica VO2 (fase iniziale e fase finale esercizio)

  16. Constant workload VO2   3’ 6’ time

  17. Data from a patientwith PPH withillustrationof the componentsof the single-exponentialcurvesusedtodescribe the exercise and recoveryresponses. In each case, the exponentialfunctionstartsafter a timedelay. Exercise: O2 = c + A(1 − e-(t − td)/τ), where c ispreexercisebaselineO2; A isamplitudefrombaselinetoasymptoticvalue; tdistimedelay; and τ istimeconstant. Recovery: O2 = c + Ae-(t − td)/τ, where c isend-recoveryasymptoticO2; A isamplitudeofresponse; tdistimedelay; and τ istimeconstant. Riley et al., J Am Coll Cardiol 2000;36:547–56

  18. Illustrationof the calculationof O2 deficit and EPOC in a patientwith PPH. Excesspostexerciseoxygenconsumption (EPOC) wascalculatedfor a recoveryperiodof 10 min. Rileyet al., J Am CollCardiol 2000;36:547–56

  19. Rileyet al., J Am CollCardiol 2000;36:547–56

  20. O2 kinetics in response to 3 minutes of unloaded cycling exercise in PPH patients and normal control subjects Sun, X.-G. et al. Circulation 2001; 104:429-435

  21. TEST INCREMENTALE Valutazione set point della CO2

  22. VCO2 x 863VE = ---------------------------- [PaCO2 x (1- VD/VT)]

  23. AT VE RESPIRATORY COMPENSATION POINT ISOCAPNIC BUFFERING VCO2 VO2 WORK LOAD REST EXERCISE

  24. AT RESPIRATORY COMPENSATION POINT ISOCAPNIC BUFFERING VE/VO2 VE/VCO2 WORK LOAD EXERCISE REST

  25. AT RESPIR. COMPEN. POINT ISOCAPNIC BUFFERING ETpO2 ETpCO2 WORK LOAD EXERCISE REST

  26. VALUTAZIONE SET POINT CO2 • TEST A CARICO COSTANTE CONDOTTO CON CARICO DI LAVORO EQUIVALENTE AL TAMPONAMENTO ISOCAPNICO. • TEST INCREMENTALE DURANTE TAMPONAMENTO ISOCAPNICO. • IN ENTRAMBI I CASI A PARITA’ DI PRODUZIONE DI CO2.

  27. Measurements of four key exercise variables in 114 paired tests in 42 patients with PAH. Hansen J E et al. Chest 2004;126:816-824

  28. Measurements of four key exercise variables in 114 paired tests in 42 patients with PAH. Hansen J E et al. Chest 2004;126:816-824

  29. Measurements near the AT in 114 paired tests in 42 patients with PAH. Hansen J E et al. Chest 2004;126:816-824

  30. Measurements near the AT in 114 paired tests in 42 patients with PAH. Hansen J E et al. Chest 2004;126:816-824

  31. Paolillo et al., CardiovascPrev and Rehab, 2011

  32. PPH Diagnosis

  33. PULMONARY VASCULAR LIMITATION ClassicalCriteria • (A - a) pO2 INCREASING DURING EXERCISE TO 45 mm Hg OR MORE (corrected for VO2 ?) • DROP IN OXIMETER SATURATION > 3% WITHOUT PaCO2 RISE • VD/VT (measured) INCREASING TO > 30% DURING EXERCISE Agostoni and Butler. Murray and NadelTextbook of Respiratory Medicine

  34. Petco2 at AT as related to the V̇e/V̇co2 at AT, showing likelihood of pulmonary vasculopathy accounting for exertional dyspnea of unknown cause. Yasunobu Y et al. Chest 2005;127:1637-1646

  35. Ferrazza et al., Respiration. 2009;77(1):3-17. Epub 2009 Jan 14.

  36. Correlations of peak {image}O2 (%pred) and ventilatory equivalent for CO2 at AT ({image}E/{image}CO2@AT) vs NYHA symptom class (top panels) and peak {image}O2 and peak O2 pulse vs AT (bottom panels) in PPH patients during CPET Sun, X.-G. et al. Circulation 2001; 104:429-435

  37. O2sat by pulse oximetry at rest, AT, peak V̇o2, and 2-min recovery in control (X) and PPH as related to physiologic severity: mild, moderate, severe, and very severe. Yasunobu Y et al. Chest 2005;127:1637-1646

  38. Oudiz and Sun. Abnormalities in Exercise Gas Exchange in Primary Pulmonary Hypertension. K. Wasserman: CardiopulmonaryExercise Testing and CardiovascularHealth.

  39. Oudiz and Sun. Abnormalities in Exercise Gas Exchange in Primary Pulmonary Hypertension. K. Wasserman: CardiopulmonaryExercise Testing and CardiovascularHealth.

  40. Petco2 during exercise in six representative subjects with PPH according to severity and evidence of an EIS, compared to a normal subject (dashed curve). Normal PPH senza shunt PPH con shunt The dotted line at 3 min shows the start of unloaded pedaling, and the dotted line at 6 min shows the start of increasing work rate exercise. Yasunobu Y et al. Chest 2005;127:1637-1646

  41. Petco2 at rest, AT, peak V̇o2, and recovery in relation to physiologic severity in 52 patients with PPH (4 mild, 17 moderate, 21 severe, and 10 very severe) and 9 normal control subjects. Yasunobu Y et al. Chest 2005;127:1637-1646

  42. Petco2 as a function of percentage of predicted peak V̇o2 at rest (left panel), AT (middle panel), and peak V̇o2 (right panel) for all 52 PPH patients. Yasunobu Y et al. Chest 2005;127:1637-1646

  43. Mixed-expired and end-tidal CO2 distinguishbetweenventilation and perfusiondefectsduringexercisetesting in patientswithlung and heartdiseases. Hansen JE, Ulubay G, Chow BF, Sun XG, Wasserman K.

  44. Values for normal subjects (NOR or N), COPD (or C), LVF (or L), and PAH (or P) during incremental cycle ergometry tests at rest (open symbols), unloaded cycling, AT, and peak exercise (closed symbols). Hansen J E et al. Chest 2007;132:977-983

  45. Mean = SEM Pco2 values in normal, COPD, LVF, and PAH groups during incremental cycle ergometry at rest (open symbols), progressing to unloaded cycling, AT, and peak exercise. Hansen J E et al. Chest 2007;132:977-983

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