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Disclosures. Pulmonary Hypertensive Crisis. Case Scenario. Objectives. Question. Definition: Pulmonary Hypertension. No financial disclosures

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Disclosures CRASH 2015 Pulmonary Hypertensive Crisis Mark Twite MA MB BChir FRCP No financial disclosures Some drugs discussed are an off-label application Children s Hospital Colorado & University of
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Disclosures CRASH 2015 Pulmonary Hypertensive Crisis Mark Twite MA MB BChir FRCP No financial disclosures Some drugs discussed are an off-label application Children s Hospital Colorado & University of Colorado Anschutz Medical Campus 2 Objectives After 30 minutes, understand: 1. Pathophysiology of pulmonary hypertension (PH) 2. Anesthetic management of PH 3. Strategies to prevent and treat a PH crisis 3 Requires Broviac catheter placement and lung biopsy Last Echocardiogram report Tricuspid regurgitant (TR) jet 5m/sec (BP 110/70) No PFO Patient s medications Epoprostenol sodium (Flolan ) Sildenafil (Revatio ) Bosentan (Tracleer ) Aspirin Nifedipine 4 Question What is the degree of this patient s PH? 1. Supra-systemic 2. Systemic 3. Sub-systemic Definition: Pulmonary Hypertension mpap 25 mmhg at rest Normal mean pulmonary artery pressure (mpap) 15mmHg independent of age, gender, ethnicity During exercise mpap increases slightly dependent on age and level of exertion PVRI 3 Wood units m 2 In association with variable degrees of: Pulmonary vascular remodeling Vasoconstriction In-situ thrombosis 5 Nef Heart 2010;96: ECHO: Systolic TR Velocity Requires Broviac catheter placement and lung biopsy Last Echocardiogram report TR jet 5m/sec (BP 110/45) No PFO Patient s medications Epoprostenol sodium (Flolan ) Sildenafil (Revatio ) Bosentan (Tracleer ) Aspirin Nifedipine Bernoulli Equation spap = 4v 2 + RAP = 4(5 2 ) + 10 = 110 mmhg Limitations: Need TR Assumes perfect alignment between Doppler and TR jet 7 Rich Chest 2011;139: Relationship between spap and mpap Definition: Pulmonary Hypertension mpap = (0.61 x spap) + 2 mmhg spap 40 mmhg highly suggestive PH mpap 25 mmhg at rest Normal mean pulmonary artery pressure (mpap) 15mmHg PVRI 3 Wood units m 2 Chemla Chest 2009;135: Nef Heart 2010;96: Pulmonary Vascular Resistance Cardiac Cath: Gold standard PVR ΔP PVR = ΔP/flow (TPG) = mpap LAP = mpap LAP/CO Hemodynamics PVR = mpap LAP / CO Normal PVR Wood units m 2 ( dynes/s/cm 5 ) PH PVR 3 Wood units m 2 (240 dynes/s/cm 5 ) Wood units x 80 = dynes/s/cm 5 2. Vasoreactivity testing Short acting pulmonary vasodilator ino, IV adenosine Drop in mpap 10 mmhg Responders ( 10%) may benefit from calcium channel blockers 3. Rule out associated disease states Pulmonary vein disease 12 Non-invasively monitor patients with PH? Right ventricle (RV) function Ability of the RV to cope with progressive increase in PA pressure Determines patient s functional capacity and survival Other ECHO parameters (observer angle dependent): TAPSE (Tricuspid Annular Plane Systolic Excursion) Tei index Non-invasively monitor patients with PH? Increase of 0.1 in S:D ratio associated with 13% increase in yearly risk for lung transplant or death. S:D ratio 1.4 associated with worse: RV function hemodynamics exercise capability clinical status 13 Alkon Am J Cardiol 2010;106: Non-invasively monitor patients with PH? Menon Clin Med Insights: Cardiology 2014:8 4D-Cardiac MRI 3D + time RV function indices correlate with PA pressures from right heart cath 15 PH Classification: What increases mpap? PVR mpap = mpap LAP/CO = LAP + (CO x PVR) 1. LAP LV systolic / diastolic dysfunction Mitral valve stenosis / regurgitation 2. CO Congenital heart disease with L to R shunt 3. PVR Pulmonary parenchymal disease Thromboembolic disease Strumpher JCVA 2011;25:687 WHO I V Classification 16 WHO Classification Conferences 1973 Geneva 1988 Evian 2003 Venice 2008 Dana Point 2011 Panama Pulmonary Vascular Research Institute Classification specific to children 2013 Nice, France Evolving research & improved understanding WHO Classification Group I Group II Left sided heart disease Group III Lung disease and/or hypoxia Group IV Chronic embolic/thrombotic Group V Miscellaneous Ivy Curr Opin Cardiol 2012;27: Group I WHO Classification Idiopathic Heritable: TGF-β Family (BMPR2, ALK-1, Endoglin) Drugs: Fenfluramine, methamphetamine Herbal supplements: St. John s Wort Associated with: Congenital heart disease Connective tissue disease (SLE) HIV Chronic hemolytic anemias (Sickle cell disease) Schistosomiasis Pulmonary veno-occlusive disease (PVOD) 19 Group II Left heart disease Mitral valve disease Group III Lung disease and/or hypoxia COPD BPD Interstitial lung disease Sleep-disordered breathing High altitude Group IV Chronic embolic/thrombotic Group V Miscellaneous Myeloproliferative disorders Metabolic disorders 20 Pediatric Pulmonary Hypertension PH: Congenital Heart Disease Chromosomal or Genetic syndrome Biventricular Circulation mpap 25mmHg & PVRI 3 Wood units m 2 Positive vasodilator response is a fall in mpap and PVRI by 20% with no change in CO Pathological insults on a growing lung Lung development abnormalities Univentricular Circulation following cavopulmonary anastomosis PVRI 3 Wood units m 2 or TPG 6 mmhg EVEN IF mpap 25mmHg 21 Del Cerro Pulm Circ 2011;1: Adult: Epidemiology & Survival Pediatric: Epidemiology & Survival Rare disease 5-15 cases / million REVEAL Age of presentation increased from 35yrs in 1980s to 53yrs Male:Female 1:4 1yr survival 80-90% 3yr survival 60% TOPP 1 Global registry At diagnosis: Median age 7yrs Dyspnea & fatigue 43% other disorders 85% CHD, 12% BPD Chromosome anomalies 13% (Trisomy 21) REVEAL 2 USA registry At diagnosis: Median age 7yrs Dyspnea mpap 56mmHg PVRI 17 Wood units m 2 5yr survival 75% Benza Chest 2012; Berger Lancet 2012; 379: Barst Circulation 2012;125: Requires Broviac catheter placement and lung biopsy Question With no PFO on ECHO, what may happen during an acute increase in PVR? Last Echocardiogram report TR jet 5m/sec (BP 110/45) No PFO Patient s medications Epoprostenol sodium (Flolan ) Sildenafil (Revatio ) Bosentan (Tracleer ) Aspirin Nifedipne Option BP SpO Physiology of Pulmonary Circulation Pulmonary vascular bed is a high-flow low-pressure circulation system Large cross sectional area High compliance arterioles with thin walls (less smooth muscle cells) Pressure and resistance are 10% of systemic circulation Sympathetic nervous system innervation Pathophysiology of Pulmonary Circulation Poiseuille s Law R = π r 4 p / 8nl 8mm 1 x R 4mm 16 x R 2mm 256 x R Pulmonary arteries Constrict with hypoxia (Euler-Liljestrand reflex) and relax with hyperoxia Respond to changes in cardiac output and airway pressure FIXED REACTIVE Structural changes decrease X-sectional area Vascular smooth muscle contraction PH: Pathology Plexiform lesion PH: Pathology A. Vasculopathy of the pulmonary arteriole cells 1. Endothelial Intimal hyperplasia 2. Smooth muscle cell (SMC) Medial hypertrophy 3. Adventitial Proliferation Less distensible vessel Fixed vs. Reactive B. Vasoconstriction C. Thrombosis 29 Cool Chest 2005;128: Normal 30 PH: Ventilation PVR changes with ph and PaO 2 Ventilation strategy for PH: Oxygen Tidal volume 6ml/kg Rate mild hypocarbia Optimum PEEP Recruitment maneuvers Drain pleural effusions/pneumothorax Adapted from West s Essential Physiology 10 th Ed. 31 Rudolph J Clin Invest 1966;45: Discussion What will be your airway and ventilation strategy to facilitate the lung biopsy? Concerns? Does anyone want a type & screen or cross match? Requires Broviac catheter placement and lung biopsy Last Echocardiogram report TR jet 5m/sec (BP 100/45) No PFO Patient s medications Epoprostenol sodium (Flolan ) Sildenafil (Revatio ) Bosentan (Tracleer ) Aspirin Nifedipine Endothelin Pathway Bosentan (Tracleer ) ET A & ET B antagonist Ambrisentan (Letairis ) ET A selective antagonist Prostacyclin Pathway Epoprostenol (Flolan ) Treprostinil (Remodulin ) Iloprost (Ventavis ) Requires Broviac catheter placement and lung biopsy Flolan running via PIV NPO status good Nitric Oxide Pathway ino Sildenafil (Revatio ) Tadalafil (Adcirca ) 35 36 Question What is this patient s risk of cardiac arrest under general anesthesia? times LESS 2. SAME times GREATER times GREATER 37 PH: Peri-operative risk Population Procedures (n) Cardiac arrest (%) Death (%) Reference All children All (1,089,200) Morray et al. Anesthesiology 2000;93:6-14 All children heart disease All except cardiac surgery (88,639) Flick et al. Anesthesiology 2007;106: Cardiac cath (4,454) Bennett et al. Pediatr Anesth 2005;15: All except cardiac surgery (256) Carmosino et al Anesth Analg 2007;104: Cardiac cath (141) Carmosino et al Anesth Analg 2007;104: Cardiac cath (70) Taylor et al Br J Anaesth 2007;98: Cardiac cath (128) Williams et al Pediatr Anesth 2010;20: Adapted from Friesen Pediatr Anesthesia 2008;18: PH: Peri-operative risk Population Procedures (n) Cardiac arrest (%) Death (%) Reference All children All (1,089,200) Morray et al. Anesthesiology 2000;93:6-14 All children heart disease All except cardiac surgery (88,639) Flick et al. Anesthesiology 2007;106: Cardiac cath (4,454) x Bennett et al. Pediatr Anesth 2005;15: All except cardiac surgery (256) Carmosino et al Anesth Analg 2007;104: Cardiac cath (141) Carmosino et al Anesth Analg 2007;104: Cardiac cath (70) Taylor et al Br J Anaesth 2007;98: Cardiac cath (128) Williams et al Pediatr Anesth 2010;20: Adapted from Friesen Pediatr Anesthesia 2008;18: Discussion What is your plan for induction of anesthesia? Use the existing PIV? Drugs? 40 PH: Goals of anesthetic management 1. Avoid increases in pulmonary vascular resistance (PVR) Hypoxia, Hypercarbia, Metabolic acidosis Sympathetic stimulation secondary to noxious stimuli (endotracheal intubation, surgery, tracheal suctioning) 2. Avoid systemic hypotension Decreases coronary artery blood flow leading to myocardial ischemia and ventricular dysfunction A rapid increase in PVR to a point where PAP SBP leads to RV failure especially if there is no PFO (or atrial septostomy) 41 Hemodynamic effects of anesthetic drugs 42 Preparation ino in the room Set-up and working, weaning plan to avoid rebound PH Anesthesia drugs 4% topical lidocaine spray for vocal cords Balanced technique Resuscitation drugs ready Communicate with surgeon High risk patient Local anesthetic Post-op plan Communicate with PH team 43 Pulmonary Hypertensive Crisis Pulse-oximetry desaturation Sats 70% Hypotensive BP 60/30 Tachycardia Bradycardia HR 30 ECG ST segment changes 44 PH: Pathophysiology of RV Failure Pulmonary Hypertensive Crisis Treatment Rationale / Therapy Administer 100% O 2 P A O 2 and P a O 2 will PVR Hyperventilate PVR is directly related to PaCO 2 Exclude pneumothorax Optimize ventilation Mean Airway Pressure Avoid P alv P art Correct metabolic acidosis PVR is directly related to H + level Administer pulmonary vasodilators ino Analgesia Decrease sympathetic mediated PVR Support cardiac output Adequate preload, epinephrine, vasopressin Price Crit Care 2010;14:R ECMO Support cardiac output and oxygenation 46 Disposition and Follow-up Extubated and returned to the ICU Returned emergently to the OR 3 hours later Bleeding via Chest Tube Hypoxic and hypotensive Summary Knowledge & therapy of PH is an evolving field Patients with systemic PH are HIGH risk Goals for anesthesia in PH Preparation Good airway management Balanced anesthetic drug technique 47 48 Thankyou! 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