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.

Keywords: Arrhythmia; Atrial flutter; Case report; Congenital heart disease; Supraventricular tachycardia; Turner syndrome.

Figure 1

(A) Baseline electrocardiogram in cardiology clinic at 8 days of age showing sinus tachycardia with a heart rate of 182 beats per minute, a northwest axis, and right ventricular hypertrophy with strain pattern. (B) Electrocardiogram one hour later showing orthodromic reentrant supraventricular tachycardia with retrograde conduction at 350 beats per minute. The retrograde p-waves are noted with arrows.

Figure 2

Apical four-chamber view showing a non-apex forming (arrow) mildly hypoplastic left ventricle with increased trabeculations (*). LV, left ventricle; RV, right ventricle.

Figure 3

Suprasternal notch view showing the normal drainage of the right lower (RLPV), left upper (LUPV), and left lower pulmonary veins (LLPV) into the left atrium (LA). The absence of the right upper pulmonary vein (RUPV) is shown with the *, suggestive of partial anomalous pulmonary venous return (PAPVR) of the RUPV.

Figure 4

Right parasternal view showing the anomalous venous drainage of the right upper pulmonary vein (RUPV) into the right superior vena cava (SVC). RA, right atrium.

Figure 5

Supraventricular tachycardia at 266 beats per minute that converted to sinus rhythm after administration of intravenous adenosine 0.2 mg/kg in the pediatric intensive care unit. Note the retrograde p-waves (arrows), consistent with orthodromic retrograde conduction.

Figure 6

Supraventricular tachycardia at 288 beats per minute with spontaneous resolution noted on Holter monitoring. The retrograde p-waves are noted with arrows.