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A Case of Peripartum Cardiomyopathy Supported by VA-ECMO


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A Case of PeripartumCardiomyopathy Supported by VA- ECMO

AlexandraBaker1, Francisca Caetano1, Susanna Price1ShahanaUddin1, Richard Trimlett1DeepaArachchillage1

1Royal Brompton and HarefieldNHS Foundation Trust, London, UK.

Introduction Peripartumcardiomyopathy (PPCM) is a rare, severe and idiopathic type of heart failure in previously healthy young women, during late pregnancy or immediately after delivery. In severe cases refractory to full medical therapy, mechanical cardiovascular support may be needed as a bridge to recovery or transplantation.

Case Report A 26-year-old, 38-week pregnant woman (G7P2) presented to obstetric triage after 4 weeks of chest pain (treated as reflux) and worsening oedema. She was found to be hypertensive, oedematous to her waist and on lying flat became acutely hypoxic. She immediately underwent emergency intubation, ventilation and caesarean section resulting in delivery of a live infant.  A transthoracic echocardiogram was performed in theatre, which revealed a moderately dilated left ventricle (LV) with secondary mitral regurgitation and severe left ventricular systolic dysfunction, ejection fraction <30%.

Post-operatively the patient remained in a low cardiac output state, despite escalating inotropic support, culminating in a cardiac arrest with successful resuscitation.  She was retrieved to an ECMO centre on Veno-Arterial Extracorporeal Membrane Oxygenation (VA-ECMO). An Intra-Aortic Balloon Pump (IABP) was inserted for LV off-loading.

Transoesophagealechocardiogram (TOE) showed a large RA thrombus (in association with the drainage cannula) and a possible patent foramen ovale(PFO) increasing simultaneously the risk of pulmonary embolism, but also of stroke due to paradoxical embolism, particularly during ECMO decannulation. She thus required systemic anticoagulation, but due to bleeding complications (from the caesarean wound) only a low heparin level was maintained. 

To treat the PPCM she was loaded with levosimendanand commenced on bromocriptine. Serial echocardiography demonstrated improvement in biventricular function. On Day 7 post-ECMO cannulationa bubble study excluded any intra-cardiac shunt, and the patient was decannulatedfrom ECMO. Subsequently, the IABP was removed and the patient was extubated. By Day 11, her LV was of normal dimensions, and the ejection fraction >40%. She was repatriated to her local hospital on chronic heart failure therapy and under surveillance of the heart failure team.

Discussion This case demonstrates several challenges in the assessment and management of PeripartumCardiomyopathy:

The non-specific nature of its presenting symptoms can make it difficult to differentiate from physiological and symptomatic changes in pregnancy. 
PPCM can be difficult to differentiate from other causes of cardiomyopathy diagnosed during/after pregnancy. 
A pregnant patient presenting with cardiogenic shock may have additional causes for circulatory collapse, including pregnancy-induced hypertension and eclamplsia, or amniotic fluid embolism. 
Although increasingly used and recommended in current guidelines, the effects of bromocriptineremain difficult to predict in individual patients1.

VA-ECMO has been described as a management option in patients refractory to conventional medical treatment, INTERMACS I, as a bridge to cardiac recovery or transplantation. Its use must be weighed against potential complications, including stroke, infection, circuit failure, bleeding and thrombosis. 

Treatment with bromocriptineand the peripartumstate put the patient at high risk of thrombus formation. Therefore, collaboration with haematologists and imaging experts (to diagnose/exclude thrombus and intra-cardiac communication) is essential to mitigate the risk of paradoxical embolism resulting in stroke in this young cohort of patients.2

References 1. Regitz-Zagrosek, V, Roos-Hesselink, J, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. European Heart Journal, Volume 39, Issue 34, 7 September 2018, Pages 3165–3241. 2. Mehta, H, Eisen, H, Cleveland, J. Indications and Complications for VA-ECMO for Cardiac Failure. American College of Cardiology 2015 [https://www.acc.org/latest-in-cardiology/articles/2015/07/14/09/27/indic...

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