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Pediatric PAH and Hyperthyroidism: A Potentially Fatal Combination

Christine Trapp

Robert Elder

Adrienne Gerken

Aviva Sopher

Shulamit Lerner

Gaya Aranoff

Erika Berman Rosenzweig


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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 06.22.2012

Presentation Type: Abstracts

Christine M. Trapp MD1, Robert W. Elder MD2, Adrienne T. Gerken1, Aviva B. Sopher MD1, Shulamit Lerner MD1, Gaya Aranoff MD1, Erika B. Rosenzweig MD2

Division of Pediatric Endocrinology1 and Division of Pediatric Cardiology2, New York-Presbyterian Hospital, Columbia University College of Physicians and Surgeons, NY, NY; US.

BACKGROUND: Patients with pulmonary arterial hypertension (PAH) who develop hyperthyroidism are at risk for acute cardiopulmonary decompensation and death. The association between PAH and autoimmune thyroid dysfunction has been described, but the exact link has not been defined. Little is known about outcomes in PAH patients who develop hyperthyroidism and even less in the pediatric population.

METHODS: We performed a retrospective review of the pulmonary hypertension database at Columbia University Medical Center to identify pediatric patients with pre-existing PAH who developed hyperthyroidism between 1999 and 2011.

RESULTS: Eight pediatric PAH patients with hyperthyrodism were identified: 5 with idiopathic PAH (IPAH) and 3 with heritable PAH (HPAH) (Table). The mean age of PAH diagnosis was 10.7 years; the mean age at hyperthyroidism diagnosis was 14.1 years. All were female and all were receiving continuous IV epoprostenol (Epo) prior to developing hyperthyroidism. Five of 8 patients presented with acute cardiopulmonary decompensation; in 3 of the 8 patients hyperthyroidism was detected during routine screening of thyroid function test (TFT) abnormalities. The mean WHO functional class (FC) at the outpatient visit just prior to hyperthyroidism was class II and worsened to a mean FC IV at the time of hyperthyroid presentation. All had goiter documented at presentation and at least 6 of the 8 had thyroid antibodies. Of the 5 patients who presented with acute cardiopulmonary decompensation, only 1 survived; this was the only patient who underwent emergency thyroidectomy.

CONCLUSIONS: Based on the potential for serious clinical deterioration, surveillance for hyperthyroidism should be performed regularly in pediatric patients with PAH, particularly those on IV Epo. TFTs should be checked urgently in pediatric PAH patients with acute decompensation without an identifiable trigger or with symptoms of hyperthyroidism. Aggressive treatment of the hyperthyroid state may be warranted including escalation of targeted PAH therapy, beta blockade and emergency total thyroidectomy if indicated. Elective thyroidectomy or radioactive iodine ablation should be considered for clinically stable PAH patients with early or mild hyperthyroidism to avoid potentially lifethreatening cardiopulmonary decompensation.

Pediatric PAH and Hyperthyroidism: A Potentially Fatal Combination