We report a 10-month-old girl withKCNT1(c1420C > T; p. Arg474Cys, R474C) mutation-associated epileptic encephalopathy, systemic-to-pulmonary artery “collateralopathy”, and intermittent QTc prolongation. Spontaneous regression of systemic-to-pulmonary artery collateral-mediated left heart dilation was noted in this patient, a finding which was ominous as it heralded the onset of severe pulmonary hypertension. The structural and electrical phenotypic features ofKCNT1mutation-associated heart disease, including the novel findings noted in our patient, are discussed in detail.
25 March 2019 in Pacing and Clinical Electrophysiology
In contrast to adults, severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) usually leads to a mild illness in children. However, a few children have been reported to have severe manifestations including pneumonia, acute kidney injury, multi-organ failure and cardiac injury. This review focuses on cardiac involvement during SARS-CoV-2 infection and the recently described likely immune mediated post-COVID-19 syndrome. Therapeutic strategies for cardiac dysfunction in both these settings are briefly discussed.