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Pulmonary Capillary Hemangiomatosis (PCH) in a Premature Infant With Congenital Heart Disease FREE TO VIEW

Scott Bickel, MD; Nemr Eid, MD; Edward Kim, MD; Russell Chowning, MD; Robert Debski, MD; Adrian O'Hagan, MD; Ronald Morton, MD
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University of Louisville, Louisville, KY

Chest. 2014;146(4_MeetingAbstracts):694A. doi:10.1378/chest.1991906
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SESSION TITLE: Pediatric Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare entity characterized by the diffuse proliferation of capillaries throughout the pulmonary interstitium, bronchioles, and vascular structures. We report on a premature neonate with complex heart disease with persistent radiographic features at several days of life which was ultimately determined to be PCH.

CASE PRESENTATION: A 2 month old male born at 33 weeks gestation with complex heart disease (large ASD, VSD, severe right ventricular outflow tract obstruction) presented with a persistent right lower lobe opacity on chest radiographs. The patient required increasing respiratory support after having been stable in room air for several weeks. Chest CT showed multiple parenchymal lung nodules. The differential diagnosis includes pulmonary vascular malformations, pulmonary veno-occlusive disease, pulmonary lymphangiectasia, alveolary capillary dysplasia and PCH. Our patient's lung biopsy showed strong diffuse reactivity for Glut1, confirming the diagnosis of PCH (Fig 1). Heart catheterization demonstrated suprasystemic right ventricular pressures. After the procedure, the patient experienced hypoxemic respiratory failure and has been unable to be weaned off of his ventilator. A trial of propranolol and systemic steroids is being attempted to decrease the vascular hyper-proliferation.

DISCUSSION: PCH frequently presents with symptoms of progressively worsening pulmonary hypertension and is often fatal. Rare cases of neonatal or congenital disease have been described [1]. Recently, PCH-like lesions were described in pre-school age children with congenital heart disease [2]. There is a paucity of literature on treatments for the condition, especially in children: prior case reports have reported using doxycycline, steroids and endothelial cell proliferation inhibitors (such as interferon alpha-2a) [3]. In children, however, there are significant concerns over the potential toxicity of immunomodulators. The definitive treatment is lung transplantation.

CONCLUSIONS: Recognition of PCH in infants can be difficult given its rarity, but is important to identify given its potentially significant implications on managing other co-morbid conditions (i.e pulmonary hypertension). Further research into anti-angiogenic therapy in pediatric patients may lead to more effective therapies, especially in those whom transplantation is not an option.

Reference #1: Oviedo A, Abramson LP, Worthington R, Dainauskas JR, Crawford SE. Congenital PCH: Report of two cases and review of the literature. Pediatr Pulmonol. 2003 Sep;36(3):253-6.

Reference #2: Aiello VD, Thomaz AM, Pozzan G, Lopes AA. Capillary hemangiomatosis like-lesions in lung biopsies from children with congenital heart defects. Pediatr Pulmonol. 2014 Mar;49(3):E82-5. Epub 2013 Sep 9.

Reference #3: Bartyil, K, Bded, O., et al. PCH in children and adolescents: report of a new case and a review of the literature. European Journal of Pediatrics. December 2004, Volume 163, Issue 12, pp 731-737

DISCLOSURE: The following authors have nothing to disclose: Scott Bickel, Nemr Eid, Edward Kim, Russell Chowning, Robert Debski, Adrian O'Hagan, Ronald Morton

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