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Pulmonary arterial hypertension associated with bronchopulmonary dysplasia and congenital heart disease in preterm infants. A case report of a preterm infant with recurrent pulmonary hypertension after corrective cardiac surgery and review of the literature.

Abstract
In preterm infants with congenital heart disease, concomitant bronchopulmonary dysplasia (BPD) is associated with relatively poor clinical outcomes because of the increased pulmonary vascular resistance and adverse effects of inflammation on the damaged lungs, even after surgery. We present herein a 1-year-old female who developed recurrent pulmonary arterial hypertension 6 months after closure of a ventricular septal defect. She was born at 26 weeks of gestation (birth weight, 470 g), and developed BPD requiring oxygen supplementation. Her systemic-to-pulmonary blood flow ratio was 2.1 preoperatively and 1.0 postoperatively, pulmonary arterial pressure was 61/15 (mean 39) mmHg preoperatively and 41/17 (mean 24) mmHg postoperatively, and pulmonary vascular resistance was 4.2 mmHg/L·minute·m(2) preoperatively and 3.6 mmHg/L·minute·m(2) postoperatively. At 1 year of age, echocardiography showed an increase in her estimated right ventricular pressure, indicating worsening pulmonary hypertension. After 3 years of treatment with oxygen supplementation, prostacyclin, and bosentan, her pulmonary arterial pressure improved to the normal range. The pathophysiology of pulmonary arterial hypertension is heterogeneous in preterm infants with congenital heart disease and concomitant BPD. Careful management of these patients is warranted even after corrective cardiac surgery.
AuthorsJun Muneuchi, Ayako Kuraoka, Mamie Watanabe, Yoshie Ochiai, Kunitaka Joo
JournalInternational heart journal (Int Heart J) Vol. 56 Suppl Pg. S22-5 ( 2015) ISSN: 1349-3299 [Electronic] Japan
PMID25787794 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)
Topics
  • Bronchopulmonary Dysplasia (complications, diagnostic imaging)
  • Echocardiography
  • Female
  • Humans
  • Hypertension, Pulmonary (complications)
  • Infant
  • Infant, Premature, Diseases (surgery)
  • Postoperative Complications

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