Pediatric pulmonary arterial hypertension and hyperthyroidism: a potentially fatal combination.
Publication/Presentation Date
7-1-2012
Abstract
CONTEXT: Patients with pulmonary arterial hypertension (PAH) who develop hyperthyroidism are at risk for acute cardiopulmonary decompensation and death.
CASES AND SETTING: We present a series of eight idiopathic PAH/heritable PAH pediatric patients who developed hyperthyroidism between 1999 and 2011. Institutional Review Board approval was obtained; informed consent was waived due to the retrospective nature of the series. All eight patients were receiving iv epoprostenol; five of the eight patients presented with acute cardiopulmonary decompensation in the setting of hyperthyroidism. In the remaining three patients, hyperthyroidism was detected during routine screening of thyroid function tests. The one patient who underwent emergency thyroidectomy was the only survivor of those who presented in cardiopulmonary decline.
EVIDENCE SYNTHESIS: Aggressive treatment of the hyperthyroid state, including emergency total thyroidectomy and escalation of targeted PAH therapy and β-blockade when warranted, may prove lifesaving in these patients. Prompt thyroidectomy or radioactive iodine ablation should be considered for clinically stable PAH patients with early and/or mild hyperthyroidism to avoid potentially life-threatening cardiopulmonary decompensation.
CONCLUSIONS: Although the association between hyperthyroidism and PAH remains poorly understood, the potential impact of hyperthyroidism on the cardiopulmonary status of PAH patients must not be ignored. Hyperthyroidism must be identified early in this patient population to optimize intervention before acute decompensation. Thyroid function tests should be checked routinely in patients with PAH, particularly those on iv epoprostenol, and urgently in patients with acute decompensation or symptoms of hyperthyroidism.
Volume
97
Issue
7
First Page
2217
Last Page
2222
ISSN
1945-7197
Published In/Presented At
Trapp, C. M., Elder, R. W., Gerken, A. T., Sopher, A. B., Lerner, S., Aranoff, G. S., & Rosenzweig, E. B. (2012). Pediatric pulmonary arterial hypertension and hyperthyroidism: a potentially fatal combination. The Journal of clinical endocrinology and metabolism, 97(7), 2217–2222. https://doi.org/10.1210/jc.2012-1109
Disciplines
Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods
PubMedID
22622024
Department(s)
Administration and Leadership
Document Type
Article