A 37 year old woman with left ventricular non compaction syndrome (an autosomal dominant condition where the left ventricular myocardium remains trabeculated) booked in her first pregnancy at 9+/40. Preconceptually, she was advised against pregnancy in view of the risks of thrombosis, cardiac failure and arrhythmia. At booking, echocardiography demonstrated an EF (ejection fraction) of 28%, which improved to 36% with Furosemide. Low molecular weight heparin was started as thromboprophylaxis but β blockade was declined by the patient until after 12/40 (Bisoprolol 1.25mg OD).
From booking a multi-disciplinary approach was taken. At 28+2/40 she developed reduced exercise tolerance and orthopnoea associated with a reduction in her EF to 31%. Her symptoms were managed by titrating up her diuretic doses.
At 34+4/40 cardiac function deteriorated further (EF = 25%). Delivery was recommended, with steroid cover, but the patient declined this. After discussion with her cardiologist she agreed and a live baby boy was delivered by LSCS under epidural anaesthesia. Post-partum she was cared for on HDU with IV Furosemide and ionotrope support. She was discharged on day five with Furosemide and Amiloride and having converted to Warfarin anticoagulation.
Conclusion The management of cardiomyopathy in pregnancy, particularly when there is poor adherence to medical advice, will be discussed. A proactive and multi-disciplinary approach to her care resulted in a live birth without significant compromise to maternal health.
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