Moredock Elisabeth,  Fulmer J.,  Collard Michael,  Pfeifer Cory

Final Pr. ID: Poster #: EDU-086

Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder that can cause respiratory arrest during sleep. It is sometimes referred to as "Ondine's Curse" in reference to a fictional character who had to remember to breathe based on a spell cast by a jilted lover. The number of cases has been reported to be near 1,000. The purpose of this educational exhibit is to describe CCHS and emphasize its implications for pediatric radiology. Read More

Authors:  Moredock Elisabeth , Fulmer J. , Collard Michael , Pfeifer Cory

Keywords:  CCHS, neuroblastoma, diaphragm pacer

Leonhardt Leah

Final Pr. ID: Poster #: CR-001

Cardiac strangulation is an unusual phenomenon occuring in pediatric cardiac surgical patients. During implantation of epicardial leads, a loop is made to allow for somatic growth of the heart. The mechanism proposed describes somatic growth of the pediatric heart after placement of circumferential leads, whereby they become adherent and contribute to a compressive process. This pattern has a unique radiologic appearance, and is important for radiolgists to recognize, as the entitiy has a very high mortality and morbidity, leading to ventricular compromise, valvular dysfyunction and coronary vessel compresseion.

We present two cases of cardiac strangulation by epicardial leads at our instution. The first case is a 7 year-old who presented with ventricular fibrillation and cardiac arrest, while the second was discovered following arterial switch procedure. In both cases, cardiac CTA demonstrates extrensic compression of coronary vessels, with variable ventricular deformity.

Interestingly, a majority of reported cases of strangulation occur after placement of leads at the anterior surface of the pericardium, with diaphragmatic placement appearing less frequeently as a cause. It can be inferred that this complication should be more highly considered in patients with circumferential looping over the anterior pericardium on conventional radiography, prompting more advanced imaging. Early recognition of this phenomenon is sure to prevent the highly morbid complications associated with this entity. Read More

Authors:  Leonhardt Leah

Keywords:  pacemaker, pacer