152 - MANAGEMENT AND TREATMENT OF SUSPECTED ACUTE CORONARY SYNDROME IN CHILDREN WITH KAWASAKI DISEASE: A GLOBAL PERSPECTIVE

Background: Acute coronary syndrome (ACS) is a rare but potentially fatal complication of Kawasaki disease especially in those with giant coronary artery aneurysms (GiAn). Guidelines differ between institutions and countries with a limited evidence-based recommendations on their management and treatment. We sought to identify variations in the management of suspected ACS in KD patients with GiAn.

Methods: An anonymous online survey was validated and distributed globally to physicians attending to patients who have or have had KD. Responses with regard to surveillance and imaging practices, pharmacological management and surgical or percutaneous interventions were collected and analyzed. Additionally, participant characteristics (region, type and length of practice) were used for correlation and comparison.

Results: Responses were collected from 134 physicians distributed over 5 continents. The majority (90%) were pediatric cardiologists (1/3^rd interventionalists), 10% non-cardiologists responsible for KD patients. There were more interventionalists from South America (48%), followed by Asia and the Middle-East/North-Africa (30% each), then Europe and North America (18% and 11%).  As ACS is often suspected based on symptoms, elevated cardiac enzymes and/or ECG changes, 85% of respondents considered a constellation of these elements to confirm the diagnosis (Figure-1).  The indication for coronary surgical or percutaneous intervention was the most controversial topic, regardless of geographic region (Figure-2). Once ACS is confirmed, the preferred approached was different in CA stenosis versus CA thrombosis. For stenosis, 69% opted for percutaneous intervention and 31% for coronary bypass surgery. Whereas for thrombosis, medical thrombolysis (50%) primed over percutaneous intervention (39%) and bypass surgery (11%). The setting for intervention (surgically or percutaneously) overwhelmingly called to treat young children in a pediatric facility (92%), versus a split between pediatric and adult facility (59% vs 41%) in older children (Figure-3).  Interestingly, there was agreement for combined management including adult and pediatric specialists for both age-scenarios (70% and 82%) (Figure-3).

Conclusion: The survey identified areas of practice variations and preferences for the acute management of GiAn presenting with ACS. Firstly, there is an outcrying evidence of uncertainty in terms of myocardial and coronary assessment for KD patients with GiAn suspected for ACS. Secondly, there is ambivalence towards treatment strategies including the choice of treatment setting in adolescent patients. A formal transfer of knowledge and interdisciplinary exchange between pediatric and adult cardiology competencies are likely to provide robust evidence-based approach in the management of GiAn patients. Improved international effort towards solving these issues are likewise required.