3/12/2019 Chronic PH – a spectrum illness Enhanced Precision in care of Neonate with cPH! • Reliability of clinical assessment • Optimal approach to diagnosis • Therapeutic precision Patrick McNamara Professor of Paediatrics & Internal Medicine • Preventative approach Senior Associate Scientist, University of Iowa Chronic Pulmonary Hypertension – Spectrum illness Case Dysregulation of pulmonary vascular bed and/or right ventricular failure • Ex-24 week twin • Ex-22 week infant • Hx Meconium Inspissation • Hx prolonged ventilation Incidence 28% in patients with CLD at 36 weeks Weismann 2017 J Peri [stoma], PDA –medical HFJV, rec sepsis Mortality rate up to 38% in severe BPD Khemani 2007 J Pediatr therapy, • Tracheostomy/G-tube Challenges • nCPAP FiO 2 0.3 • sIMV 30/5, FiO 2 0.25 1. Impact of confounding illnesses • Day 84 Screening TnECHO • Day 104 Screening TnECHO 2. Variable adaptive response of RV to elevated afterload 3. Echo limited to subjective appraisal of pressure and function TnECHO : RV systolic pressure 85 TnECHO : Normal mmHg, mod dilated RV 4. It doesn’t exist or it doesn’t matter 1
3/12/2019 Evaluation of Chronic Pulmonary Hypertension • How reliable are methods of evaluation? • How precise are diagnostic methods? • Are screening thresholds temporally appropriate? • Is longitudinal monitoring consistent? 36 weeks Perinatal Factors Postnatal Factors Gestation Lung disease Chorioamnionitis Ventilation, Oxygen Resuscitation PDA Sepsis Sepsis/NEC 2
3/12/2019 Survey of management of chronic PH in premature babies in Canadian Perspectives: Echo Screening at 36 weeks Neonatal Network and National Research Network NICUs Michelle Baczynski, Emer Finan, Patrick McNamara, Edward Bell, Amish Jain Altit et al 2018 • Response from 37 tertiary NICUs [29/44 CNN, 9/16 NRN] • 57% centers routine echo screening for cPH [almost always ‐ respiratory support at 36 weeks CGA (71%)] • Only 17% (5 sites) performed RH catheterization • 86% reported use of pulmonary vasodilators Case Course Prior to TnECHO Assessment • Preterm infant with antenatal diagnosis possible coarctation of the aorta and omphalocoele Cardiology: Serial echo – coarctation ruled out TnECHO….. PDA 2.4mm PDA 1.6mm PDA 1.1mm • Gestational Age: 28+3 Progressive respiratory decompensation • Birthweight: 1105g 2 6 16 41 48 Postnatal Day Baseline HR ⇡ gradually to 180s; irritable Baseline HR 120s FiO 2 80% • Initial course unremarkable, CPAP in room air x 5 days then low Intubation; BP 95/60 well FiO 2 40‐50% flow 50mL/min. CPAP Low Flow 30‐ sedated FiO 2 0.21 50mL/min 3
3/12/2019 Dilated Right Heart Severe Pulmonary Hypertension & low CO Paradoxical septal motion RVSp at least 120mmHg + RAp Systolic BP = 95mmHg Dilated RA & RV Dilated main PA with mild PI Very low left ventricular output – 70 ml/min/kg Clinical Course Course Prior to TnECHO Assessment • Respiratory decline until FiO 2 1.0 despite appropriate recruitment, normal CO 2 , sedation and muscle relaxation Cardiology: Serial echo – coarctation ruled out PDA 2.4mm PDA 1.6mm PDA 1.1mm 40% R ⇢ L 30% R ⇢ L 40% R ⇢ L TnECHO….. • Pulmonary vasodilator therapy initiated sequentially: – Inhaled nitric oxide – Milrinone titrated up using echo guidance to a max dose of 0.9mcg/kg/min – IV prostacyclin Progressive respiratory 2 6 16 41 48 Postnatal Day decompensation Baseline HR ⇡ gradually to 180s; irritable • Right heart dysfunction with systemic hypotension treated with dobutamine, Baseline HR 120s vasopressin, hydrocortisone CPAP Low Flow 30‐ Intubation; FiO 2 80% FiO 2 0.21 50mL/min FiO 2 40‐50% BP 95/60 well sedated • Ongoing progressive desaturation [70‐75%] and right heart dysfunction; on postnatal day 56 ICU support withdrawn with parents consent 4
3/12/2019 Diagnostic criteria used Comprehensive PH EVALUATION 1. RV pressure HARM CHD RVSP – TR jet PDA shunt Septal wall motion PAAT:RVET ratio 2. RV Function Subjective NORMAL RV fractional area % TAPSE Tissue Doppler / Strain 3. Impact of PH RV output Pulm vein S/D Vmax BENEFIT PH McNamara and Jain 2015 LV function & output Baczynski et al 2017 E-PAS I: Tricuspid Regurgitation dependant on RV function Estimated sPAP vs Cath derived PAP Bernoulli Equation [RVSP = 4 Vmax 2 + Right Atrial Pressure] (i) Normal patients: TR absent or trivial (< 2 m/sec) Mourani et al 2009 (ii) Underestimates RVSP if RV dysfunction 5
3/12/2019 Afterload and Heart function ‐ continuum II: Septal Wall Motion impacted by LV pressure RV LV Septum Convex Septal Flattening Septal Bowing Normal ½ to 2/3 systemic Systemic or above (i) False negatives if Systemic Hypertension (ii) Impacted by RV vs LV systolic function Reliability of qualitative assessment of RV III Reliability of RV Assessment dilation, septal flattening and RV systolic function All (n=60) Controls (n=30) PH (n=30) RV Dilation All 0.14 (0.02), p<.001 0.12 (0.04), p=.004 0.12 (0.03), p<.0001 Expert 0.15 (0.06), p=.003 0.13 (0.02), p<.001 0.17 (0.07), p=.009 Novice 0.13 (0.05), p=.003 0.22 (0.07), p=.002 0.02 (0.06), p=.38 Septal Curvature All 0.2 (0.02), p<.001 0.08 (0.03), p=.004 0.22 (0.03), p<.0001 Expert 0.23 (0.05), p<.001 0.09 (0.08), p=.13 0.23 (0.08), p=.001 Novice 0.21 (0.05), p<.001 0.06 (0.07), p=.18 0.29 (0.07), p<.0001 RV dysfunction All 0.3 (0.03), p<.001 0.11 (0.05), p=.01 0.33 (0.04), p<.0001 Expert 0.31 (0.06), p<.001 0.13 (0.01), p=.1 0.32 (0.08), p<.0001 Novice 0.35 (0.06), p<.001 0.06 (0.1), p=.3 0.4 (0.08), p<.0001 6
3/12/2019 Dilated RV with Septal Flattening Triaging TnECHO at 36/40 • Ex-preterm 22/40 infant • CGA 36 week infant • 2150 grams • nCPAP • 30% oxygen, SpO2 92% A: 36 +2 weeks PMA B: 36 +1 weeks PMA C: 37 +5 weeks PMA PEEP 9, FiO 2 43% PEEP 7, FiO 2 47% PEEP 8, FiO 2 35% B: Large left to right Atrial Septal Defect C: Pulmonary Pressure = Systemic Level RVO = 428ml/min/kg LVO = 211ml/min/kg RVSp estimated at 75mmHg + Rap High RVET:PAAT ratio with Systemic systolic of 81mmHg notched PA Doppler 7
3/12/2019 Stenotic Pulmonary Veins Chronic Pulmonary Hypertension – A Spectrum • Resistance mediated Vasodilator – Lung disease – Remodeling • Flow mediated Shunt modulation – Chronic hsDA / ASD – AV malformations • cPH mimicking Disease Specific – Pulm. Vein stenosis Peak Gradient 2.96m/s – Hypertension Summary • cPH is a physiologic spectrum disorder characterized by elevated PAP and impaired RV performance • TnECHO provides enhanced diagnostic precision and longitudinal monitoring that is individualized to ambient physiology • RV performance in health and disease requires prospective consideration 8
3/12/2019 Severe RV dysfunction Septal Hypertrophy Impaired LV Filling ASD and BPD risk [Acute PH/MAP] Treatment – reduce MAP as is Treatment – inotropic Treatment – volume, possible, transition dopamine support, prostin if PDA reduce MAP, vasopressin, Kumar 2018 J Pediatr to vasopressin or restrictive remove all positive inotropy norepinephrine 25 (86%) reported use of pulmonary vasodilators 9
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