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Familial arrhythmogenic cardiomyopathy masquerading as peripartum cardiomyopathy
Abstract Number: T-35
Abstract Type: Case Report/Case Series
New onset heart failure in the parturient can represent de novo disease in the form of peripartum cardiomyopathy (PPCM) or preexisting, subclinical disease unmasked by the physiologic stresses of pregnancy.(1) We present the anesthetic management of a woman who developed ventricular bigeminy and a decreased ejection fraction (EF) in labor, which was ultimately diagnosed as heritable arrhythmogenic cardiomyopathy (ACM).
A 28 year old G1P0 with hypothyroidism was admitted for induction of labor at 40+5 weeks. Several hours after initiation of oxytocin, she developed an irregular heart rate, palpitations, and mild dyspnea. She denied personal or family cardiac history. EKG revealed bigeminy. Hypomagnesemia (1.1 mg/dl) was the only lab abnormality. When bigeminy failed to resolve after repletion of magnesium, cardiology was consulted. Echocardiogram revealed frequent ectopy with a moderately reduced EF (35-45%) concerning for PPCM. An early labor epidural and arterial line were placed and low dose beta blockade initiated.
Hours later, the patient underwent Cesarean delivery for failure to progress with slow, sequential dosing of her labor epidural. She received 18ml epidural 2% buffered lidocaine with epinephrine 1:200,000, 1500ml crystalloid, and oxytocin 1 unit bolus followed by 7.5 unit/hr infusion. EBL was 700ml. She was hemodynamically stable through surgery. Postoperatively, she was monitored for 1 day in the cardiac care unit and ultimately discharged on POD 5 on low dose beta blockade, furosemide, and magnesium supplementation.
In outpatient cardiology follow up, the patient revealed that her father, paternal uncle, and cousin all died suddenly between age 30-40, attributed by the family to a curse rather than medical disease. 24 hour cardiac event monitor showed 20.4% PVCs. A cardiac MRI revealed right ventricular (RV) fatty deposits and persistently decreased EF of 40%, suggestive of ACM. Confirmatory genetic testing is pending. The patient was fitted for an external cardiac defibrillator vest with plans for an internal cardiac defibrillator (ICD).
ACM is an autosomal dominant disorder characterized by fibro-fatty replacement of the RV myocardium. It is recognized as a leading cause of ventricular arrhythmias and sudden cardiac death in young adults, both of which are largely preventable with antiarrhythmics and ICDs.(1) ACM has variable penetrance and more commonly manifests in males; however, the physiologic stresses of pregnancy and labor may unmask symptoms in females.(2) Our case illustrates the importance of carefully elucidating family and cardiac history in patients with presumed PPCM. The identification of this life threatening genetic disease masquerading as PPCM allowed for treatment likely to increase this patient’s life expectancy.
1) Trends Cardiovasc Med. 2014; S1050-1738
2) Eur J Obstet Gynecol Reprod Biol. 2006; 127(2):186-9