Cardiac Catheterization is Unnecessary in the Evaluation of Patients with Pulmonary Hypertension: CON
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1 Cardiac Catheterization is Unnecessary in the Evaluation of Patients with Pulmonary Hypertension: CON Dunbar Ivy, MD The Children s s Hospital Heart Institute 1 Diagnostic Evaluation: Right Heart Cardiac Catheterization Gold Standard Establish diagnosis Ascertain etiology Evaluate vasoreactivity Establish severity & prognosis Guide treatment McGoon M, Gutterman D, Steen V, et al. Chest 2004;126;14S-34S 2
2 Pulmonary Arterial Hypertension Sustained elevation of mean pulmonary arterial pressure to > 25 mm Hg, with a mean pulmonary capillary and left atrial pressure < 15 mm Hg at rest Pulmonary Vascular resistance > 3 Units X m 2 Simonneau G, et al. J Am Coll Cardiol 2009;54:S43-S54 3 Echo Doppler Estimate of PA pressure
3 Limitations of Doppler Estimate of PA Systolic Pressure Requires a good acoustic window Requires a good and properly aligned Doppler signal Tricuspid regurgitation has to be present Can be affected by incorrect assumption of right atrial pressure Pulmonary Vein Stenosis IN BPD 6
4 Pulmonary Vein Stenosis 7 Diagnostic Evaluation: Right Heart Cardiac Catheterization Gold Standard Establish diagnosis Ascertain etiology Evaluate vasoreactivity Establish severity & prognosis Guide treatment McGoon M, Gutterman D, Steen V, et al. Chest 2004;126;14S-34S 8
5 What Is the Cause of Pulmonary Hypertension? Pulmonary Hypertension: Types Post-Capillary PH (PCWP>15 mmhg; ; PVR nl) PAH Respiratory Diseases PE Atrial Myxoma Cor Triatriatum MV Disease VC RA RV PA PC PV PV compression PVOD LA LV LVEDP Ao Systemic HTN AoV Disease Pre-capillary PH (PAH) PCWP<15 15 mmhg PVR > 3 Wu Myocardial Disease Dilated CMP-ischemic/non ischemic/non-isc. Hypertrophic CMP Restrictive/infiltrative CMP Obesity and others 2 9 BPD and PAH Mourani, Ivy, Rosenberg, Fagan, Abman. J Peds 2008;152:
6 Diagnostic Evaluation: Right Heart Cardiac Catheterization Gold Standard Establish diagnosis Ascertain etiology Evaluate vasoreactivity Establish severity & prognosis Guide treatment McGoon M, Gutterman D, Steen V, et al. Chest 2004;126;14S-34S 11 Treatment Overview Barst R, et al. J Am Coll Cardiol 2009;54:S78-S84
7 Conventional treatment Yes Acute vasodilator No Responder Non Responder Calcium Channel Blocker Incomplete Incomplete Bosentan oral Sildenafil oral Tadalafil oral Iloprost inhaled Treprostinil sc Treprostinil inhaled Combination of drugs No Right heart failure Yes Incomplete Incomplete IV prostanoids (Epoprostenol, Treprostinil) Atrial septostomy Incomplete Transplantation Tissot, Ivy, Beghetti Pulmonary Vascular Reactivity Testing Acute - Classic - Decrease in mpap of at least 20% - with the PVR decreasing 20% - with no change or an increase in cardiac output Acute -Revised - Decrease in mpap of at least 10 mmhg - with the mpap decreasing 40 mmhg - with a normal or high cardiac output 14
8 Diagnostic Evaluation: Right Heart Cardiac Catheterization Gold Standard Establish diagnosis Ascertain etiology Evaluate vasoreactivity Establish severity & prognosis Guide treatment McGoon M, Gutterman D, Steen V, et al. Chest 2004;126;14S-34S 15 Hemodynamics NIH Registry Frequency Normal Range Mean Pulmonary Artery Pressure (mm Hg) Frequency Normal Range Pulmonary Vascular Resistance Index (L/Min/M 2 ) Frequency Normal Range Right Atrial Pressure (mm Hg) Frequency Normal Range Cardiac Index (L/Min/M 2 ) Rich S et al. Ann Intern Med. 1987;107:
9 Hemodynamic Predictors of Survival in Patients With PPH: A National Prospective Registry Variable Odds Ratio (95% CIs) Right atrial pressure (mean) 1.99 ( ) Pulmonary arterial pressure (mean) 1.16 ( ) Cardiac index 0.62 ( ) D Alonzo GE, et al. Ann Intern Med. 1991;115: PAH Determinants of Risk Determinants of Risk Clinical evidence of RV failure Lower Risk No Higher Risk Yes Progression Gradual Rapid WHO class II, III IV 6MW distance Longer (>400 m) Shorter (<300 m) BNP Minimally elevated Very elevated Echocardiographic findings Hemodynamics Minimal RV dysfunction Normal/near normal RAP and CI Pericardial effusion, significant RV dysfunction High RAP, low RAP McLaughlin VV and McGoon MD. Circulation, In press.
10 Predictors of Outcome on Epoprostenol Variables Hazard Ratio (95% CI) p value Age >44 yrs 1.17 ( ) Gender (female/male) 0.95 ( ) Anorexigenic (yes/no) 0.95 ( ) Raynaud s phenomenon (presence/absence) 1.38 ( ) History of syncope (yes/no) 0.75 ( ) History of right-sided heart failure (yes/no) 2.19 ( ) NYHA FC IV vs III 2.24 ( ) Six-minute walk distance 250 m 2.20 ( ) Baseline hemodynamics mrap 12 mm Hg mpap <65 mm Hg PAOP 10 mm Hg CI 1.80 L/min/m 2 TPR 35.4 U/m 2 msap 93 mm Hg Heart rate 88 beats/min Systolic index 21.4 ml/m 2 Svo % 2.74 ( ) 1.72 ( ) 0.87 ( ) 0.96 ( ) 0.65 ( ) 1.24 ( ) 0.76 ( ) 1.46 ( ) 1.54 ( ) Variables Hazard Ratio (95% CI) p value NYHA FC III/IV vs I/II 8.75 ( ) < Six-minute walk distance <380 m 2.62 ( ) Increaase in distance walked <112 m from baseline 1.09 ( ) Dose of epoprostenol achieved <14 ng/kg/min 0.60 ( ) mrap >10 mm Hg 3.57 ( ) mpap <59 mm Hg 1.94 ( ) Decrease in mpap 10% relative to baseline 1.25 ( ) CI 2.4 L/min/m ( ) Increase in CI < ( ) L/min/m 2 relative to baseline TPR 25 U/m ( ) Decrease in TPR <30% relative to baseline 1.90 ( ) Svo 2 <62% 2.64 ( ) Sitbon O et al. J Am Coll Cardiol. 2002;40: Diagnostic Evaluation: Right Heart Cardiac Catheterization Gold Standard Establish diagnosis Ascertain etiology Evaluate vasoreactivity Establish severity & prognosis Guide treatment McGoon M, Gutterman D, Steen V, et al. Chest 2004;126;14S-34S 20
11 Guide Treatment: Epoprostenol dosing Timing of listing for lung transplantation Timing for addition or change of therapy Performance of atrial septostomy 21
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