Catheteriza)on for the infant with BPD and concern for PAH Jack - PowerPoint PPT Presentation

10th Interna)onal Conference on Neonatal and Childhood Pulmonary Vascular Disease BPD: Challenges in Lung and Pulmonary Vascular Growth and Development Catheteriza)on for the infant with BPD and concern for PAH Jack Rome, MD March 9, 2017

Disclosures Conflicts of Interest - none However: 1. I am an interven)onal cardiologist 2. I am not a pulmonary hypertension specialist (when we review the list of pa)ents who need to be added on for cath each day, I don’t always jump at the opportunity to do the BPD pts for r/o PAH) 2

What determines when we bring the pa)ent to catheteriza)on? Circulation. 2015;132:2037-2099 Hansmann G et al. Heart 2016;102:ii86 – ii100. 3

Cardiac catheterisation is indicated in all paediatric patients with pulmonary hypertension (PH) to confirm diagnosis, to evaluate the severity and when PH-specific drug therapy is considered. 4

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Context Total of 161 procedures, 48 (30%)<1y Denominator: ~1300 procedures/yr 6

Indica)ons for Catheteriza)on in BPD (should really leave to others) • Evidence on echocardiography of PAH with concern for other � treatable � lesion • PDA, ASD, PVV stenosis • Evidence of PAH with considera)on for high risk medical therapy of pulmonary hypertension • Plan for chronic pulmonary vasodilator rx. Contraindica)on for Cath • Pa)ent in who predicted mortality is high regardless of what one does or does not do. 7

That would go well 8 8

BPD/PAH Anesthe-c Considera-ons* • Residual pulmonary parenchymal disease • Echo evidence of RV hypertension/RV dysfunc)on • Electrolyte indica)ons of compensated chronic respiratory acidosis • Acquired large airway disease (stenosis, malacia, or combina)on) * James Steven, MD Director of Cardiac Anesthesia

CHOP Anesthesia approach • Seda)ve premedica)on: light if significant residual pulmonary disease or severe PAH • Intravenous induc)on with ketamine safest • Controlled ven)la)on: target top-normal pH (oden requires permissive hypercapnia in chronic respiratory acidosis) • Minimal supplemental oxygen at baseline, but may require some • Avoid respiratory depressant intravenous medica)ons • ICU surveillance post-cath if PVR significantly elevated and/ or RV dysfunc)on

Interpreta-on trade-offs • Light seda)on/spontaneous respira)on: • Most likely some respiratory depression that could adversely affect data • More difficult to rescue from � spells � or � crises � • Controlled ven)la)on and vola)le anesthe)c: • Uncertainty about target ven)la)on goals might affect data • Residual anesthe)c effect more easily eliminated • Less likely to have crises and easier to rescue if they do

Conduct of catheteriza-on: Access • Venous access – the right femoral vein – (think about for long-term access pa)ents) • Arterial • In small infants femoral artery access has 20-50% incidence of femoral artery thrombosis • individualize: may not be needed (if exis)ng atrial hole, peripheral arterial line) 12

Hemodynamic Assessment: Baseline • Ini)al blood gas to achieve appropriate baseline • � normalize � ven)la)on and oxygena)on • Oximetry and blood gas determina)ons • rou)ne sampling through right heart for shunt detec)on • Most commonly asd/pfo and/or PDA • evalua)on of baseline oxygena)on • If ASD/pfo led atrial, if desaturated, pvv sampling • In intact atrial septum, arterial • Pressure measurement • RA, RV, each PA and PCWp and pullback through the right heart. • If elevated PCWp, and no atrial hole, retrograde catheteriza)on • Flow measurement • Shunt present- Fick (VO2 comment) • Shunt Absent - TD (note, 5F TD catheter inadequate for pressure measurement) 13

Hemodynamic Assessment: Pulmonary Vasoreac-vity Tes-ng • Our standard: 100% FiO2 + 40PPM NO, 5 min re- equilibra)on • Oximetry/Flow determina)on: • If shunt lesion repeat appropriate oximetry (ABG for dissolved O2 at any site where satura)on > ~94%). • If no shunt, TD output • Arterial satura)on and ABG, if low and there is an atrial hole, assess led atrial sat and ABG • Pressures: repeat systemic and pulmonary arterial and venous pressures 14

Baseline 100% 40PPM Pap 30 22 Lap 5 5 C.I. 4.4 4 PVRi 6.1 4.25 15

7 M male ex 27 wk with chronic lung disease ASD, s/p PDA ligation Echo – diagnosed PAH, treated with sildenafil 16

4-month-old former 23 weeks gesta)on, severe BPD, persistent PDA, and PAH. Hospitalized at an another ins)tu)on and transferred to ours at 3M. Intubated on 20 ppm iNO. PDA patent despite several courses indomethicin 17

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Percutaneous Closure of Patent Ductus Arteriosus in Small Infants With Significant Lung Disease May Offer Faster Recovery of Respiratory Function When Compared to Surgical Ligation Abu Hazeem A, et al. Catheterization and Cardiovascular Interventions 82:526– 533 (2013) 20

2-month-old male with alveolar capillary dysplasia, pulmonary hypertension, readmitted with increased work of breathing and increased oxygen requirement. Shortly after admission, had a code event in the PICU consistent with a pulmonary hypertensive crisis 21

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6 month old former 24wks premature infant, chronic lung disease, surgical NEC, chronic respiratory failure, and PAH. Previously on iNO. Currently mechanically ventilated on 35-40% FiO2. His two most recent echocardiograms have demonstrated evidence of RV hypertension. 23

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Subsequently underwent a sutureless repair for pvv stenosis 27

Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort Linda Mahgoub et al. Pediatr Pulmonol 2017; 9999: 1 – 8 . 28

• 406 caths in 144 pts • 11 systemic embolic events (10 strokes) – Clinical stroke in 8% of pts – 25% of procedures associated with some complica)on So when you approach your interventional cardiologist requesting catheterization for a patient with BPD, PAH, and PVV stenosis, you might understand why s/he doesn’t jump for joy… 29

Predictors of Catastrophic Adverse Outcomes in Children with Pulmonary Hypertension Undergoing Cardiac Catheterization: A Multi-Institutional Analysis From The Pediatric Health Information Systems Database O’Byrne et al. JACC 66:1261-1269, 2015 • 6,339 procedures from 38/43 centers contributing data to the PHIS database • The observed risk of composite outcome was 3.5% (death or ECMO within 1 day of procedure) 30

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Conclusions • Rather than list conclusions, would rather just note that there are many unknowns in this very high risk popula)on regarding – Who should undergo catheteriza)on – When they should – What the conduct of the procedure should be – What the role of inteven)ons should be 32