Slide 1

Pulmonary Hypertension Christina T. Sheridan, MD Pediatric cardiologist October 16, 2014

Slide 2

Disclosures I have no financial disclosures.

Slide 3

Neonatal Pulmonary HTN Incidence ~2/1000 term births Three main types Lung parenchymal diseases Idiopathic: normal parenchyma, but remodeled vasculature Hypoplastic vasculature Symptoms: tachypnea, increase O2 requirement or vent settings, differential O2 sats in upper and lower limbs

Slide 4

Normal transition PVR falls rapidly immediately after birth due to Mechanical stretch of lung as pulmonary blood flow increases 10x Decreasing CO2 tension Increasing O2 tension Increase in local production of vasodilators (NO- cGMP pathway)

Slide 5

Differential pre-post ductal sats To arms To legs

Slide 6

Etiologies Lung parenchymal diseases Infection MEC aspiration Respiratory distress syndrome Idiopathic Premature closure of the PDA due to maternal use of NSAIDS or maternal use of SSRIs Can cause RV failure and hydrops in utero Hypoplastic vasculature Diaphragmatic hernia, chest masses, etc

Slide 7

Work-up CXR Head US : rule out intraventricular bleed Lab work: Infection? Polycythemia? Echocardiogram: Rule out structural defects Assess pulmonary veins Assess direction of PDA and PFO shunts Quantify degree of PAp via septal curvature and tricuspid regurgitation (TR) jet

Slide 8

Quantifying degree of TR PISA refers to the degree of color bleed of a regurgitant jet. The vena contracta is the narrowest width of the TR jet where the max velocity is measured by Doppler Image source: www.echobasics.de

Slide 9

TR jet by numbers Pressure gradient = 4V 2 (ex: V=3m/s; RVsp=36mmHg) Image source: www.adhb.govt.nz

Slide 10

Definitions Normal PVR (pulmonary vascular resistance) is 1-3 Woods units after about 2 months of age Normal SVR (systemic vascular resistance) is 15-30 Woods units Normal RVsp