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Mortality Estimates for Severe Bronchopulmonary Dysplasia Complicated by Pulmonary Hypertension

Steven Walker

H. L. Meluskey

Brian Hanna

D. Munson

H. Kirpalani

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Conference: 2011 PH Professional Network Symposium

Release Date: 09.22.2011

Presentation Type: Abstracts

S. Walker, MS, CRNP; H. L. Meluskey, BS, BSN, RN; B. D. Hanna, MD, PhD; D. Munson, MD; H. Kirpalani, BM, MSc. 
Pediatric Cardiology & Neonatology, The Children's Hospital of Philadelphia, Philadelphia, PA.

BACKGROUND: Pulmonary hypertension (PH) may become a predominant clinical feature in severe bronchopulmonary dysplasia (BPD), defined as an increased oxygen requirement at 36 weeks gestational age in infants with chronic lung disease of prematurity.

PURPOSE: We hypothesized that, in a severely affected cohort, survivors of BPD associated PH would have milder PH at presentation, and pre-specified clinical variables (see table) would predict survival.

MATERIALS/METHODS: BPD and PH severity and treatment modalities were determined from a retrospective chart review of infants that presented between 01/04 and 09/09. Excluded infants had congenital lung or major cardiac malformations. Severe PH was defined by an echo finding right ventricular pressure as >systemic. Cox proportional hazards modeling assessed prediction of death using antenatal factors, in-hospital variables, and specific pulmonary vasodilator therapy.

RESULTS: The clinical data (Mean + SD or %), of 33 infants transferred at 21.8 + 11.5 weeks of age was evaluated. The group FiO2 was 0.77 + 0.28, while 66% were ventilated at referral. PH was severe in 73%. Therapeutic interventions included fundoplication (45%), gastrostomy (52%), tracheostomy (39%); specific PH therapies: sildenafil (72 %); prostacyclin (21 %). Overall 8 patients (24%) died, all before 2 yrs, with 2 yr Kaplan Meier survival of 75%. Follow-up for survival was 3.3 + 1.6 yrs. Cox proportional hazards modeling showed mode of enteral feeding at death or discharge predicted mortality (p=0.002). Antenatal characteristics, PH severity and type of PH treatment did not predict mortality. At follow-up 43% of the survivors had a reduction in PH therapy.

CONCLUSIONS: Even with aggressive PH therapy the mortality at 2 yrs, of infants with severe BPD associated PH was 24%, when treatment was started at > 5 months of age. With our results mortality cannot be predicted from severity of PH. To confirm the survival benefit from post-pyloric feeding, or the effectiveness of specific PH therapies requires a prospective, multi-centered evaluation.

PRE-SPECIFIED CLINICAL VARIABLES

 

Antenatal

Gestational Age
Antenatal Steroids
Birth weight (SGA status)

Admission

fIO2
PaCO2
Postnatal Age

PH Severity

Moderate vs. Severe
Treatment Type
Fundoplication

Follow-Up

Days of Hospitalization
Total Years of Follow-Up