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A Rare Cause of Peripartum Cardiomyopathy
Abstract Number: F1C-1
Abstract Type: Case Report/Case Series
A 27-year-old G4P3003 Amish female with no history presented at 32.2 weeks gestational age with dyspnea and tachycardia. CXR and CT revealed cardiomegaly. Transthoracic echocardiogram (TTE) showed a significantly dilated LV, LVEF 10-15%, and severely decreased RV function. She was admitted to the ICU for medical management but required emergent cesarean section due to elevated biventricular pressures and severely reduced mixed venous oxygen saturation. Intra-operative course was uncomplicated but required inotropic and vasoactive infusions. Post-operatively, management included aggressive diuresis, afterload reduction, and swan-guided inotropic therapy. On POD 7, she experienced ventricular fibrillation cardiac arrest requiring emergent placement of Impella CP and initiation of V-A ECMO. Sans cardiac recovery, the patient was bridged to transplantation with a Heartmate III LVAD on POD 11. Post-VAD medical management included aggressive RV support. The baby also showed symptoms of heart failure and underwent genetic testing which revealed proprionic acidemia (PA).
PA is a rare autosomal recessive condition characterized by a deficiency of propionyl-CoA-carboxylase, which is important in the metabolism of odd chain fatty acids and branched chain amino acids. Deficiency results in an accumulation of organic acids, specifically proprionic acid, which are toxic to many tissues including the myocardium . The incidence of disease is < 1:100,000, however, there is an increased incidence in the Amish community .
There are few reported cases of pregnancy in women with PA; all were uneventful as the diagnosis was known a priori . Usually, PA is a childhood diagnosis and has many manifestations including dilated cardiomyopathy and long QT syndrome . There is evidence of a cardiac phenotype specific to the Amish which presents at a later age; hence our patient’s delayed diagnosis . Pregnancy depletes carnitine which worsens cardiomyopathy. Carnitine prevents toxic metabolite build up in mitochondria. European guidelines regarding management of PA in pregnancy include close monitoring of protein and carnitine levels, carnitine replacement, protein restriction, and IV glucose therapy during labor and delivery . There is currently no consensus regarding delivery method. Some advocate for cesarean section to reduce the catabolic state induced by the stress of vaginal delivery; however successful vaginal deliveries have been reported. In summary, this shows the complexity of care for a parturient with PA and the adverse outcomes that can occur without timely diagnosis and management.
1. J Schwoerer, et al. Successful pregnancy and delivery in a woman with proprionic academia from the Amish community. Molecular Genetics and Metabolism Reports. (2016); 8:4-7.
2. N Mungan, et al. Proprionic acidemia: a Turkish case report of a successful pregnancy, labor, and lactation. Journal of Pediatric Endocrinology and Metabolism. 29(7) (2016); 863-866.