Introduction: Ebstein's anomaly is traditionally described as an isolated anomaly of the right valvular apparatus.In the last decade, associations with left side anomalies have been described, specifically ventricular non-compaction.More recently, the link between this association and the presence of mutations contractile apparatus proteins has been described.Case description: A 34-year-old woman with Ebstein's anomaly diagnosed since childhood had regular follow-up until 2010.The last TTE (2009) describes apical insertion (þ/-25mm;15.4mm/m2 of the crux plane) of the septal leaflet of the tricuspid valve, compatible with Ebstein's anomaly, and minimal tricuspid regurgitation and norma biventricular function.She returned in August 2014 with complains of de novo palpitations.The ECG showed Sinus Rhythm with incomplete RBBB and 24h HOLTER was unremarkable.At this time the TTE showed, in addition to Ebstein's anomaly, a dilated left ventricle with echocardiographic criteria of non-compaction and moderate systolic dysfunction and standard HF therapy was initiated.In October 2014 she reported a pregnancy (unplanned) and ACE inhibitor was suspended.Pregnancy was complicated by pre-eclampsia requiring induction of normal delivery at 39 weeks.In the immediate postpartum, she developed Supra-Ventricular Taquicardia at 190bpm with reversion to sinus after iv labetalol and a few hours later she developed acute pulmonary oedema with severly compromissed LVEF.She responded to vasodilator and diuretic therapy and was discharged from ICU in day 5. Cardiac MRI showed a dilated LV (LVDV 199ml; 122ml/m2) with apical hypertrabeculation with criteria for non-compaction.The LVEF was severely compromised with a LVEF of 30% without edema or late enhancement.and a normal RV global size and function.She was at home at 15th day postpartum on NYHA II-III under standard HF therapy and anticoagulation.After 6 months therapy she was in NYHA II, although and with no improvement of LVEF, so a decision to implant an ICD was made.She maintained palpitations and although HOLTER was normal, episodes of tachycardia (SVT vs VT) were detected in ICD interrogation and she is currnelty being evaluated for and invasive EPS with intent of ablation.At 3 months, her male child was diagnosed with DCM with moderate to severe LVEF compromise and hypertrabeculation and LV lateral wall and apex.The fetal echocardiogram had been normal.Prognostic modifying therapy was initiated, with improvement of LVEF (48% at 14 months).The genetic study of the child identified heterozygosity in a variant of the MYH7 gene (recently related to the association between Ebstein's anomaly þ non-compacted myocardium), later also confirmed in the mother.Conclusions This case describes the importance to look for left size lesions in Ebstein patients, some of the can only become apparent in the adult life.The association with LVNC has particular importance, because of the dismal prognosis and autosomal dominant inheritance.
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