Untreated congenital heart disease leading to Eisenmenger syndrome: Cultural Implications and barriers to care

Pulmonary arterial hypertension in children can occur from many causes, but when secondary to excessive pulmonary blood flow due to congenital heart disease, timely management is essential. Excessive pulmonary blood flow can occur in ventricular septal defects (VSDs) and in patent ductus arteriosus (PDA), among other defects, and if longstanding, can lead to irreversible changes to the pulmonary vasculature and development of Eisenmenger syndrome (ES). We present the case of a pediatric patient who developed ES due to a hemodynamically significant PDA and VSD, as well as a sizable atrial septal defect, that were not evaluated for many years in the setting of both language and cultural barriers to accessing care in the Amish community. While demonstrating slight improvements in oxygen saturations with medical therapies tailored toward the family's cultural needs, her prognosis remains guarded. We review the essential need for the medical community to proactively provide care to patient populations with identifiable barriers and to appropriately intervene to prevent morbidity and mortality.