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High Morbidity and Mortality in Premature Infants with Pulmonary Arterial Hypertension Secondary to Bronchopulmonary Dysplasia

Vincent Aluquin

G. Sabharwal

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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 06.22.2012

Presentation Type: Abstracts

Aluquin VPR, Sabharwal G.

Penn State Children’s Hospital, Hershey, Pennsylvania, USA

BACKGROUND: Pulmonary arterial hypertension (PAH) is the most significant cardiovascular complication of bronchopulmonary dysplasia (BPD), a chronic lung disease affecting 15,000 premature infants in the US yearly. Despite reported high mortality in these patients, there is paucity of data on its onset and clinical outcomes.

METHODS: We reviewed the characteristics, onset of PAH, medical therapy, morbidity and mortality in 24 BPD patients with PAH. PAH was established by echocardiography and/or cardiac catheterization.

RESULTS: Their mean gestational age was 25.7 + 1.7 weeks and mean birth weight was 679 + 259 grams. Among the 24 babies, 54% were females and 46% were born small for gestational age. PAH was initially diagnosed at 106 + 36 days of life, mainly by echocardiography. Since they had a previous normal echocardiogram at 47 + 44 days, we predict that PAH can be manifested in BPD patients as early as 2 months of life. 83% of patients had Nissen fundoplication and G tube placement, 50% had patent ductus arteriosus ligation and 75% had tracheostomy tubes. Five out of 24 infants died (21%) with 2 occurring in-hospital. Twenty two survivors were discharged on either a home ventilator or nasal cannula at 6.9 + 2.7 months. All except two were on pulmonary vasodilators, most frequently sildenafil (91%). During the first year after discharge, there was an average of 3 hospital readmissions per patient, with 30/62 (48%) readmissions due to pneumonia and BPD exacerbation. On follow-up (28.2 +14 months), 50% of children continued to have weights below the 10th percentile.

CONCLUSIONS: Clinically significant PAH may begin at 2 months of age in preterm babies with BPD. These infants have poor growth, significant co-morbidities, frequent readmissions after discharge and a high mortality rate. There is a need to improve surveillance and management of these high-risk patients.