SARS-CoV-2-related pediatric inflammatory multisystem syndrome (PIMS-TS) is a novel syndrome first described in England in May 2020 [1]. It affects children 2–6 weeks after COVID-19 infection whether symptomatic or not. The course of the disease varies from mild to requiring Intensive Care Unit support, with a 2% mortality rate reported [2]. An inherent symptom of PIMS-TS is high fever. Cardiac evaluation is crucial in PIMS-TS patients as the involvement of the cardiovascular system de- termines the course of disease and prognosis.
We present 2 cases of girls aged 5 (Patient 1) and 7 years (Patient 2). They were both admited to hospital due to high unremitting fever, weakness, and mucocutaneous and gastrointestinal symptoms. Patient 2, presented with severe dyspnea and had a history of upper respiratory tract infection with a highly possible COVID-19 etiology 3 weeks earlier.
On admission, the girls presented with fever, polymorphic rash, and conjunctival redness. Patient 2 had hepatomegaly, pleural effusion, and cardiac enlargement on chest X-ray. Laboratory test results in both patients were typical of PIMS-TS with significantly elevated inflammatory parameters, cardiac biomarkers, lymphopenia, hyponatremia, and increased D-dimers. Following PIMS-TS diagnostic and treatment protocol, electro- cardiography (ECG) and echocardiography were performed [3]. ECG showed abnormal repolarization with coved ST-segment eleva- tion ≥2 mm, followed by negative T wave in V1 and V2 precordial leads. Such abnormalities are not characteristic of PIMS-TS.
The patients were treated for PIMS-TS in accordance with the Polish Pediatric Society recommendations [3]. Immunomodulating therapy (immunoglobulins and glucocor- ticosteroids) and acetylsalicylic acid were administered. Patient 2 developed symptoms of cardiogenic shock with severely reduced left ventricular ejection fraction and required inotropic medication. They both recovered without complications. Their ECGs normalized once the fever resolved.
With the aforementioned ECG recordings, both patients met the criteria for the Brugada type 1 ECG pattern [4]. Brugada syndrome phenocopies were excluded [5]. Patients and their first-degree relatives underwent a thor- ough cardiological screening according to the Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society (HRS/EHRA/APHRS) expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes [4].
Patient’s 1 family’s cascade screening led to the patient’s father being subjected to an ajmaline challenge, which revealed a typical Brugada syndrome pattern. Moreover, the index-case paternal grandfather suffered a sudden cardiac death. The girl’s genetic test with Next Generations Sequencing (NGS) test- ing showed a variant of unknown significance in the SCN5A gene. No other family members shared the disease. No significant events were reported in patient’s 2 family. They are currently being investigated to exclude the disease in first-degree relatives of the patient. NGS testing is in progress. Both patients were then given lifestyle change recommendations, according to the HRS/EHRA/APHRS expert consensus statement [4]. However, both families were reassured about the low risk of life-threatening arrhythmias in asymptomatic and incidentally diagnosed patients.
The two cases presented a typical but severe course of PIMS-TS. High and long-lasting fever occurring in PIMS-TS exposed the abnormal electrocardiographic repolarization pattern which led to the diagnosis of Brugada syndrome. In children, ECG in fever is rarely performed, however, as stated, it may lead to a correct diagnosis.
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