Interdisciplinary management of left ventricular noncompaction in pregnancy using a wearable defibrillator

Introduction: Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology, characterized by extensive trabeculations of the left ventricular cavity and a two-layered structure of the left ventricular myocardium. LVHT is diagnosed in children and adults, mainly by echocardiography, and may be associated with heart failure, arrhythmias and embolic events. LVHT is no contraindication for pregnancy, however worsening of cardiac function and development of malignant arrhythmias has been described. Protection against malignant arrhythmias can be provided by implanted defibrillators (ICDs) or wearable defibrillators (Lifevest Firma Zoll). We present the first LVHT case with a lifevest during pregnancy.

Case report: A 27-years old woman with a diagnosis of LVHT since 2 years presented within the 16th week of pregnancy. She was free of cardiac symptoms. Echocardiography showed a left ventricular ejection fraction of 43% (normal > 60%). The family history disclosed that her sister, also suffering from LVHT, had died at age 19 despite an implanted cardioverter-defibrillator (ICD) and the patient's father had died suddenly at age 34 years. Genetic testing was refused. She was on a medication with bisoprolol 5 mg/d. At completed 25 weeks of gestation betamethason was given for respiratory distress syndrom-prophylaxis. Since multiple ventricular ectopic beats and short ventricular ectopic runs were detected by 24-hour monitoring, and considering the family history, it was decided to provide her with a lifevest which she wore with high adherence. In the following weeks, the cardiac situation and brain-natriuretic peptide levels remained stable and no arrhythmias occurred. Within the 34th week of gestation she was hospitalized. Daily cardiotocograms and weekly Doppler-recordings of the umbilical cord detected no abnormalities. The primary Caesarean-section was scheduled at 37 weeks of gestation on a day when the medical staff of all involved departments was present in the operating room. The Caesarean-section was carried out without any problems in epidural anesthesia. The newborn girl had a weight of 2556 g and a length of 47 cm. Apgar score was 9/10/10. Postpartal development was excellent for mother and child. The child did not show any cardiac abnormalities. Clinical and cardiologic follow-up of mother and child 4 months postpartal showed a normal development of the child. The mother continues to take bisoprolol 10 mg/d, but has, so far, not consented to implantation of an ICD.

Conclusion: In view of the limited data and experience with the course of pregnancy in LVHT-patients, a close interdisciplinary monitoring between cardiologist, gynecologists, anaesthesiologists and neonatologists should be carried out. A wearable defibrillator is an alternative to protect against sudden death if a patient is at risk for malignant arrhythmias and an ICD is either unsuitable or undecided.