Peripartum Cardiomyopathy in Neuroblastoma Patient. Pregnancy alone vs Chemotherapy




Patel, Harsh
Jipescu, Daniel
Patel, Aman
Burkholz, Michael


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Background/Abstract: Although known for decades, etiology for peripartum cardiomyopathy (PPCM) is still eluding. It’s not only a challenging disease for physician but also for the patients due to it’s complexity in management and very high recurrence rate with subsequent pregnancies. Several risk factors for PPCM include: age [greater than] 30, multiparity, eclampsia, pre-eclampsia, and prolonged tocolytic therapy with beta agonists. Here we present a case of a young caucasian woman with history of neuroblastoma that developed severe peripartum cardiomyopathy leading to cardiac cirrhosis. Case Report: This is a 22-year-old Caucasian female with past medical history of fetal alcohol syndrome, neuroblastoma at birth status post chemotherapy, portal hypertension status post banding, and recently diagnosed peripartum cardiomyopathy. The patient’s pregnancy was complicated with eclampsia, which resulted in a C-Section emergent birth. 2 weeks after birth, patient started complaining of dyspnea on exertion, productive cough with clear sputum, orthopnea and lower extremity swelling. Initial workup with CXR revealed pulmonary vascular congestion, a TTE with EF 5-10%, diffuse hypokinesis, grade 3 diastolic dysfunction, and pulmonary artery peak pressure of 35-40 mmHg, physical examination revealed: pulmonary rales, 2+ pitting LE edema, dyspnea on exertion. Patient was diagnosed with CHF at the time and discharged on Carvedilol and Furosemide; however, 2 weeks later her LE edema continued, this time presenting with worsening dyspnea and tachycardia. Bilateral LE duplex was negative for DVT, but a CTA Chest with contrast revealed a PE in subsegmental Left Lower lobe for which she was started on Apixaban. Her EF remained the same. CHF medications were optimized with Lisinopril, Metoprolol Succinate, Lasix, and Digoxin. Initial hypercoagulable workup was negative, however, the CTA Chest revealed liver lesions. MRI of abdomen revealed a cirrhotic liver secondary to combination of hepatic venous congestion from RHF, chronic portal vein thrombosis, portal hypertension, gastrohepatic varices. The patient’s current therapy for her CHF is: Digoxin, Furosemide, Betablockers, and Milrinone drip. She will be sent for evaluation for liver and heart transplant. Discussion/Conclusion: PPCM is a rare but serious condition with high maternal and fetal morbidity and mortality. It is idiopathic but many theories implicate potential role of maternal or fetal hormones, immunological, genetic or environmental factors. Early diagnosis is paramount with strong clinical suspicion, lab markers and echocardiography. Management includes treating heart failure symptomatically as well as potential use of newer therapies targeting immunological system as well as anti prolactin therapy with Bromocriptine and in severe cases cardiac transplant. However, no randomized clinical studies are available till date to support newer therapies. Some questions that we proposed during literature reviews are: is there an early lab or imaging marker (either maternal or fetal) that can predict PPCM and thus prevent or slow the severity. More studies are needed to answer these puzzling questions.