1 - Pediatric Infectious Diseases Unit, Dona Estefânia Hospital, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal
2 - Department of Pediatric Cardiology and Reference Center for Congenital Heart Diseases, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal
3 - Member of the European Reference Network for Rare, Low Prevalence Complex Diseases of the Heart (ERN GUARD-Heart)
4 - Pediatric Rheumatology Unit, Dona Estefânia Hospital, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal
- 14th Excellence in Pediatrics Conference (Oral Communication)
Background Kawasaki disease (KD) is the leading cause of acquired heart disease in children of developed countries. Refractory KD occurs in 10-38% of the cases. It is associated with increasing risk for coronary artery aneurysms and treatment remains controversial. Few cases of successful treatment of KD with giant coronary artery aneurysms have been reported.
Case Presentation Summary A previously healthy 8-month-old male infant, with unremarkable medical history, presented with a four-days high-grade continuous fever and maculopapular erythematous rash of the face, trunk, palms and soles, associated with diarrhea, clefted lips and strawberry tongue. Laboratory findings showed elevated inflammatory markers (PCR 250mg/L and ESR 75mm/h) and four criteria of incomplete KD (normochromic normocytic anemia, hypoalbuminemia, ALT elevation and sterile pyuria). Echocardiogram was normal. KD was assumed and IVIg (2g/kg), methylprednisolone (2mg/kg/day), and lysine acetylsalicylate (30mg/kg/day) were initiated but fever resurged and persisted >48h after 1st IVIg. A 2nd dose of IVIg (2g/kg) was administered, after cardiac involvement exclusion, ceasing the fever. But there was a new recurrence of fever and left main coronary (LMCA) / left anterior descending (LAD) and right coronary artery (RCA) dilation were diagnosed. Anticoagulation was initiated and anakinra was prescribed. Sustained apyrexia and rash involution were achieved at this point, but inflammatory markers remained elevated and coronary changes continued to progress, latter developing giant aneurysms. A 3rd dose of IVIg (2g/kg), methylprednisolone pulses (30mg/kg/day) for 5 days and IV infliximab were administered. Apyrexia and normalization of inflammatory markers were reached, but coronary aneurysms continued to increase, reaching their maximum on the 36th day of illness – LMCA with aneurysmal dilatation of 6.7mm (z-score+13.4), LDA with proximal aneurysm of 5.5mm (z-score+18) and distal aneurysm of 7.5mm (z-score+27), and RCA with proximal aneurysm of 6.8mm (z-score+17). Treatment with propranolol was started and titrated under continuous cardiac monitoring until beta blockade was achieved with the aim of reducing the heart rate and the sheer stress of the coronary arteries. This child was discharged 2 months after hospitalization and at the 8-month-follow-up the aneurysms were getting smaller.
Discussion This is a rare case of refractory KD with major late cardiac involvement albeit early recognition and adequate treatment, showing that serial cardiac evaluations to recognize cardiac involvement are important. What makes this case interesting is that coronary aneurysms continued to increase after controlling clinical and laboratory inflammation, and heart rate reduction was essential to minimize the rupture risk of the coronary arteries.
Palavras Chave: Coronary Aneurysms; Kawasaki Disease