Abstracts

Rationale: West Syndrome is an age-specific, often refractory, epileptic encephalopathy characterized by infantile spasms (IS), distinctive EEG findings (hypsarrythmia) and neurodevelopmental regression. Adrenocorticotropic hormone (ACTH) is the only medication approved by the FDA for primary treatment of IS/West Syndrome. Hypertension and hypertrophic cardiomyopathy (HCM) are well-documented complications of ACTH therapy for IS, but other cardiac effects have not been described. Methods: We report a patient with IS who developed HCM and pericardial effusion leading to cardiac tamponade during her ACTH weanResults: Our case was a 6 month old female with IS in her final week of a 3 week ACTH wean after having received 4 weeks of high dose intramuscular ACTH. She presented to the ED with complaints of poor feeding, lethargy, labored breathing and cyanosis. Chest x-ray revealed cardiomegaly. Echocardiogram demonstrated HCM and pericardial effusion. The patient developed tachycardia, hypotension, and pulsus paradoxus, suggestive of tamponade physiology, and required bedside pericardiocentesis in the Pediatric ICU. To our knowledge, pericardial effusion in association with ACTH therapy has not been described. Review of the literature revealed three cases in which pericardial effusion was associated with hypocortisolemia due to primary or secondary adrenal insufficiency. Other causes of pericardial effusion were ruled out in each case, and symptoms were responsive to cortisol replacement therapy. In two cases, effusion recurred on attempted discontinuation of therapy. These findings, along with the timing of our patient s presentation during the ACTH wean, suggest that our patient may have developed iatrogenic adrenal insufficiency leading to cardiac tamponade secondary to hypocortisolemia. Conclusions: This is the first documented case of pericardial effusion and tamponade during ACTH usage/wean. Our case underscores the importance of considering cardiac sequela of ACTH therapy in the treatment of IS and suggests a connection between low systemic ACTH, cortisol levels, and pericardial effusion. The mechanism by which low cortisol may cause pericardial effusion remains unclear and warrants further investigation.