Successful pregnancy in left ventricular hypertrabeculation/noncompaction with implanted cardioverter/defibrillator and a variant in the TPM1 Gen (c.425A > T) in mother and child

Pregnancy in left ventricular hypertrabeculation/noncompaction (LVHT) is a matter of concern due to the risk for arrhythmia or heart failure (HF). This risk seems higher if arrhythmias have been already diagnosed before pregnancy. Pregnancies in LVHT cases with implanted cardioverter-defibrillators (ICD) are rarely reported.We report pregnancy of and delivery to a 28-year old patient with hypertrophic phenotype of LVHT with ICD, implanted 11 years previously for secondary prevention of sudden cardiac death, in whom genetic analysis disclosed a variant in the TPM1 Gen (c.425A > T). Until the 28th gestational week (GW), the pregnancy was without problems, then increasing HF due to diastolic dysfunction developed. In GW34, she was treated with atosiban for tocolysis because of premature labor. Due to aggravation of HF, preterm delivery by cesarean section in spinal anesthesia in GW35 was carried out. Delivery and maternal postpartal course were without problems, HF regressed. ICD interrogation did not show any arrhythmia.Pregnancy and delivery can be managed safely in hypertrophic phenotype of LVHT with ICD under interdisciplinary surveillance. Diastolic dysfunction may occur in the third trimester and lead to HF. Tocolytic drugs should be used with caution, even though the drugs are assumed to be safe regarding cardiovascular complications.

Learning objective: Pregnancy and delivery can be managed safely in hypertrophic phenotype of left ventricular hypertrabeculation/noncompaction with implanted cardioverter-defibrillators under interdisciplinary surveillance. Diastolic dysfunction may occur in the third trimester and lead to heart failure. Tocolytic drugs should be used with caution, even though the drugs are assumed to be safe regarding cardiovascular complications.