Description

A young boy with an 11-month-old history of Rastelli procedure and unifocalisation for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral artery presented to our emergency department because of ill appearance and diaphoresis. The patient had a fever and exhibited the signs of poor feeding 2 days before presentation. On presentation, respiratory failure, shock and comatose were noted. Echocardiography revealed slightly decreased apical and posterior wall motion compared with the data obtained 7 months ago. ECG showed no remarkable changes from the previously obtained data (figure 1A). The results of the laboratory examination were as follows: troponin T level, 0.52 ng/mL; creatine kinase level, 254 U/L; and brain natriuretic peptide level, 419.0 pg/mL. Contrast-enhanced CT showed no anomaly or contrast defects of the coronary arteries. We suspected septic shock because of the patient’s fever and shock. Mechanical ventilation and the administration of fluid bolus, norepinephrine, dobutamine and antibiotics were initiated. Subsequently, the patient’s circulatory status improved.

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Figure 1

ECG on admission (A) and the day following admission (B). The latter shows slow ventricular tachycardia.

Slow ventricular tachycardia occurred on the day following admission (figure 1B), and apical wall motion was further reduced (video 1). The patient was subsequently diagnosed with Takotsubo cardiomyopathy (TC) based on transient regional left ventricular hypokinesia that extended beyond a single epicardial vessel distribution, presence of sepsis as a physical trigger, abnormality noted on ECG, and elevated levels of troponin T and brain natriuretic peptide, according to the diagnostic criteria for paediatric TC.1 Although mexiletine infusion partially succeeded in controlling ventricular arrhythmia, his blood pressure decreased because of recurrent arrhythmia. The blood pressure increased with the administration of dobutamine. We suspected acute myocarditis as a differential diagnosis; thus, we planned to transfer the patient to another centre for extracorporeal membrane oxygenation. After several hours, his arrhythmia disappeared. Wall motion improved after a week (video 2). His blood, urine and cerebrospinal fluid cultures were negative. The patient was extubated after 7 days; he was discharged 19 days after admission without any morbidity.

TC is typically triggered by stress and is characterised by transient left or right ventricle dysfunction with various wall motion abnormalities (typically apical left ventricular wall motion).1 It is rare in children, particularly young children.2 Very few cases of TC development in children with congenital heart diseases (CHDs) have been reported.3 4 To the best of our knowledge, the present case is the first report on TC development in a child with pulmonary atresia and ventricular septal defect. Although changes on ECG in children with TC are reported,1 arrhythmia has not been indicated, as per our knowledge. Myocardial hypoxia and surgery for CHD may affect stress response. Although TC is often triggered by sepsis in severely ill adults,5 insufficient information is available for children.2 TC triggered by sepsis and septic cardiomyopathy partially overlap.6 Therefore, future studies are warranted to determine the association of TC with CHD and sepsis in children. Thus, when children with CHD who are suspected of having any infection present with decreased cardiac function, TC may be a possible cause in addition to myocarditis and septic cardiomyopathy.