ABSTRACT
The Rhythmia™ system (Boston Scientific, Natick, MA, USA) facilitates the rapid acquisition of high-resolution electroanatomical and activation maps. However, there are limited data on its efficacy and safety in pediatric and adult congenital heart disease (CHD) patients. In a retrospective, observational cohort study, adult CHD and pediatric patients followed by pediatric cardiology underwent electrophysiologic study using the Rhythmia™ electroanatomic mapping system. Variables examined included the number of electroanatomical maps required, acquisition time, procedure time, fluoroscopy time, radiation dosage, and rate of recurrent arrhythmia. Twelve consecutive patients, including six male patients (50%), were included with an average age of 27.7 years (range: 11-64 years). Seven (58%) of these patients had a diagnosis of CHD [moderate complexity in two (17%) and great complexity in five patients (42%)] and 10 (83%) patients underwent ablation. A total of 37 high-density maps were created in 12 procedures, with a median of 8,140 mapping points, taking a median of 631 seconds. The median procedure time was 189.5 minutes. The median fluoroscopy time was 0.9 minutes, with eight (67%) patients receiving no fluoroscopy at all. Recurrence occurred in one patient (8%) over a median follow-up duration of 16 months (interquartile range: 12.8-17.3 months). No adverse periprocedural events were recorded. This study suggests the use of high-density electroanatomic mapping in adult CHD patients showed potential for rapid acquisition of highly detailed maps with minimal fluoroscopy time or risk of periprocedural events in the studied population.
ABSTRACT
Patients with congenital heart disease (CHD) are one of the fastest growing populations in cardiology, and valvular pathology is at the center of many congenital lesions. Derangements in valvular embryology lead to several anomalies prone to dysfunction, each with hemodynamic effects that require appropriate surveillance and management. Surgical innovation has provided new treatments that have improved survival in this population, though has also contributed to esotericism in patients who already have unique anatomic and physiologic considerations. Conduit and prosthesis durability are often monitored collaboratively with general and specialized congenital-focused cardiologists. As such, general cardiologists must become familiar with valvular disease with CHD for appropriate care and referral practices. In this review, we summarize the embryology of the semilunar and atrioventricular (AV) valves as a foundation for understanding the origins of valvular CHD and describe the mechanisms that account for heterogeneity in disease. We then highlight the categories of pathology from the simple (e.g., bicuspid aortic valve, isolated pulmonic stenosis) to the more complex (e.g., Ebstein's anomaly, AV valvular disease in single ventricle circulations) with details on natural history, diagnosis, and contemporary therapeutic approaches. Care for CHD patients requires collaborative effort between providers, both CHD-specialized and not, to achieve optimal patient outcomes.
