Neonatal arrhythmias in Turner syndrome: a case report and review of the literature

Abstract

Background While left-sided congenital heart defects have been well described in females with Turner syndrome (45, X), the literature is scarce regarding arrhythmias in this patient population. Case summary A full-term neonate referred to cardiology was found to have a non-apex forming left ventricle and partial anomalous pulmonary venous return. During the echocardiogram, she developed atrial flutter, followed by orthodromic reentrant supraventricular tachycardia (SVT). She was started on propranolol and eventually switched to sotalol due to breakthrough SVT. A genetics evaluation revealed Turner syndrome with complete monosomy X (45, X). The patient is now 18 months old and has not had any further arrhythmias. Discussion We present a rare case of atrial flutter followed by supraventricular tachycardia in a neonate with Turner syndrome and left-sided heart defects. This case highlights the importance of early and precise investigation of cardiac abnormalities in neonatal patients, especially among females with Turner syndrome given their relatively higher risk of cardiovascular disease compared to the general population.

(Arrhythmogenic potential in Turner syndrome patients)

“…Pouze dvě práce referují o supraventrikulárních arytmiích u pacientek s TS, jedna z nich je kazuistické sdělení z roku 2020 dokládající běh fl utteru síní, následované paroxysmem ortodromní atrioventrikulární reentry tachykardie na podkladě skryté spojky u novorozence s TS a druhá práce zachytila u tří pacientek běhy atrioventrikulární nodální reentry tachykardie, u dvou fi brilaci síní a u jedné atrioventrikulární reentry tachykardii na podkladě skryté spojky. 12,13 V naší skupině pacientek s TS byla prevalence krátkého PR stanovena na 13 % ve srovnání s 5 % ve studii autorů Bondy a spol., respektive 4,3 % ve studii Noordmanové a spol. 8,9 Obdobně je ve výše uvedených studiích popsána i signifi kantně vyšší tepová frekvence u dívek i žen s TS, jejíž příčina je shodná s možnými důvody krátkého intervalu PR.…”

Organ Abnormalities Caused by Turner Syndrome

“…Although the exact mechanisms underlying the increased risk of cardiac arrhythmias in patients with TS are not fully understood, structural abnormalities in patients with TS, such as aortic stenosis and BAV, can lead to changes in blood flow and increased stress on the heart muscle. Patients with TS also have longer QT intervals than normal women, which may be associated with tachycardia [87].…”