Long QT Syndrome (LQTS) is a rare cardiac channelopathy characterized by a high risk of lethal arrhythmias. Pregnancy and post-partum period may induce an increased vulnerability, so an appropriate prevention is needed.
A 31-year-old primigravid woman at 36 weeks of pregnancy was referred to our Cardiomyopathy Unit located in a tertiary centre for LQTS screening. The patient had a family history of LQTS (mother and brother) and Brugada syndrome (cousin). The 12-lead ECG (Figure 1) showed a long QT interval (QT corrected: 508 msec), while the transthoracic echocardiogram was unremarkable. The genetic test performed is still ongoing. The patient reported sporadic episodes of palpitations at rest and the following 24-hour ambulatory ECG revealed a very high burden of polymorphic ventricular arrhythmias, with about sixteen thousand premature ventricular complexes and a non-sustained ventricular tachycardia. Therefore, due to the patient’s high-risk for life-threatening arrhythmic events, metoprolol 25 mg o.d. was initiated and a wearable cardioverter defibrillator (WCD) with remote monitoring was provided during pregnancy and labour. After 3 weeks a caesarean delivery in epidural analgesia was performed and managed by a pregnancy heart team including a cardiologist, gynaecologist, obstetrician, and anaesthetist in close cardiac monitoring. No maternal or infant complications occurred, without arrhythmic episodes detected by the device. The WCD will be maintained also in the post-partum period with a narrow follow-up of the patient.
In pregnant high-risk LQTS patients, beta-blockers are the first line therapeutic option, but they do not able to interrupt lethal arrythmias in out-of-hospital setting. The implantable cardiac defibrillator is not the best choice, due to the concerns about radiation exposure and anaesthesia during pregnancy. Moreover, lead-associated thrombosis and infections during pregnancy were reported in literature. The WCD is the ideal option in these cases. It is an external device suited for transient higher arrhythmic risk periods. Moreover, it allows a remote monitoring of arrhythmic burden (figure 2) which may help clinicians in guiding the follow-up and the therapeutic management of the patient. One drawback is the need of continuous patient’s compliance. Finally, a pregnancy heart team is mandatory in presence of these rare cardiac syndromes.