Further evaluation and treatment of comorbidities that impact the severity of lung disease should be undertaken with the diagnosis of BPD-PH infants before the initiation of pulmonary arterial hypertension (PAH)-targeted therapy. Studies should include:
· evaluation for intermittent or sustained hypoxemia (oxymetry),
· aspiration (evaluation by OT, consider videofluoroscopy, consider jejunal feeds trial)
· gastroesophageal reflux disease evaluation and treatment,
· structural airways disease with scope by ENT
· Have ECG and CXR baseline at diagnosis of pulmonary hypertension at 36 weeks.
· Consider NT-proBNP level for follow-up in patients with right ventricular dysfunction at diagnosis of pulmonary hypertension
· pulmonary artery and vein stenosis evaluation, left ventricular diastolic dysfunction, and aortopulmonary collaterals.
Management of BPD-PH:
· Consider trial of Naso/Oro-jejunal feeds to avoid silent micro-aspiration
· Optimize respiratory care (suctioning, recruitment with maximal functional residual capacity to avoid V-Q mismatch)
· Consider diuretics (Hydro/Spiro) in the context of pulmonary hypertension in BPD
· Aim saturations ³ 92% for > than 95% of time. Supplemental oxygen therapy should be used to avoid episodic or sustained hypoxemia and with the goal of maintaining oxygen (O2) saturations between 92%- 95% in patients with established BPD and PH. Avoids vasculature vaso-spasm.
· Vaccination for avoidance of pulmonary infections
· Optimize nutrition (less volume more concentrate, aim normal weight/length ratio)
· Severe flare-up with viral / respiratory infections: require rigorous vaccination, avoidance of crowded areas (shopping center, kindergarten).
· Prevnar 13, Synagis, Pneumovax (23 serotypes polysaccharide vaccine) at 2 years
· Influenza vaccine for the whole family and patient > 6 months
· Ensure with oximetry that baby has saturation ³ 92 % for > 95% of the time
· Pulmonary vasodilators should be started with consultation of a pediatric cardiologist in the context of RV dysfunction and/or iso to supra-systemic pulmonary hypertension after optimization of everything above.
If BPD with severe PH goes into PH crisis – Algorithm:
• Patient in acute PH crises - Heterogeneous pulmonary vascular disease with V/Q mismatch
• Consider Re-intubation with sedation, aggressive chest physio and airway toilette + recruitment to minimize V/Q mismatch - Optimize ventilation with “BPD parameters” (long I-Time for CO2 release, lower rate, higher volumes). These lungs are fibrosed with airway disease that tend to collapse. They need usually a higher PEEP (8-10) in order to stent the airway.
• Optimize hemoglobin > 100
• iNO 20 ppm – will reach vessels of lung areas that are ventilated
• Milrinone – Lusitropic medication to promote RV function and filling
• If PH crisis, consider stress dose hydrocortisone 30 mg/m2/day
• Rule out concomitant infection +/- Abx (urine, viral, bacterial, pneumonia, etc.)
• Adjust nutrition, possibly induce diuresis with furosemide if oedema.
• BPD patients that are intubated and sedated do not need as much calories (the ventilator does much of the work and the sedation keeps them from activating metabolic rate). Need to avoid an increase in fat content and consider decreasing significantly calories and fluids.
For home or follow-up (intra-hospital if still hospitalized):
• Home Oxygen needs to be organized for pulmonary hypertension patients
• Parents need to be informed that the whole family needs influenza vaccination
• The patient should not be in kindergarten while still on home oxygen. Avoid spaces with close contacts. Avoidance of viral infection (social distancing).
• Parents need to be trained to recognize signs of resp distress or RV failure: diaphoresis, retraction, work of breathing, cyanosis, abnormal neurological status
• Parents need basic CPR training
• Travelling by plane can be complicated by PH crises – needs assessment for fit to travelling with hypoxic challenge once PH infra-systemic and stable in room air.
• Sildenafil can accentuate GERD, ensure to manage reflux and follow growth: avoid abnormal Weight-Length Ratio. Energy needs can be up to 140 kcal/kg/d (Small volumes but high caloric enrichment).
• Follow Hgb (ensure no anemia), regular blood gaz and electrolytes (especially if on diuretics), +/- Brain Natriuretic peptide if RV failure (baseline and at follow-ups).
• Consider: Sweat test, occasional chest X-ray to rule out aspiration (especially if oral challenges) and CT scan of chest if progressive worsening of clinical status.