Catastrophic antiphospholipid syndrome with myocarditis in p
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The present case has been published in the The Journal of Obstetrics and Gynecology of India. Catastrophic antiphospholipid syndrome (CAPS) is a disorder characterized by multi-organ failure caused due to multiple small vessel thrombosis associated with thrombotic microangiopathy.

A 25-year-old third gravida woman was referred at 27-week and 3-day period of gestation with complaints of shortness of breath and uncontrolled hypertension. She had a history of previous two missed abortions at 6–8-week gestation, and during the workup for recurrent pregnancy loss she was diagnosed to have APS (antiphospholipid syndrome) with aCL IgG and IgM positive. She was started on aspirin in inter-conceptional period and on low molecular weight (LMW) heparin as soon as she became pregnant.

She developed pre-eclampsia at 25-week gestation, and blood pressure (BP) was inadequately controlled on alpha-dopa, labetalol and nifedipine. She was also given dexamethasone for foetal pulmonary maturation. On admission, she was breathless at rest, i.e. New York Heart Association (NYHA) Class 4 with tachycardia, tachypnoea and blood pressure of 160/100 mmHg.

Her fundus examination showed grade 1 hypertensive retinopathy, and 2D echocardiography showed moderate to severe mitral regurgitation with the presence of an eccentric posterolateral jet and normal biventricular function. Initial chest X ray was suggestive of pulmonary oedema. Obstetric sonography showed a single live foetus in breech presentation with an estimated foetal weight of 865 g. A provisional diagnosis of pre-eclampsia with severe features with partial HELLP syndrome was made.

She was admitted in the obstetric high dependency unit for stabilization. On the third day of her hospitalization, she developed signs and symptoms of imminent eclampsia and pregnancy was terminated by emergency caesarean section with the delivery of a live born female with weight of 1020 g and APGAR scores of 7 and 9 at one and 5 min of life. She received magnesium sulphate for eclampsia prevention till 24 h after delivery.

Anticoagulation with LMW heparin was restarted 24 h after delivery. On the post-partum day 2, she developed shortness of breath with NYHA class IV symptoms, and chest auscultation revealed diffuse bilateral basal chest crepitation. Chest X ray was suggestive of pulmonary oedema, and diuretics were administered. Her course progressively worsened, and she developed hypotension and cardiogenic shock and was shifted to the cardiac care unit.

Echocardiography was repeated which revealed severe mitral regurgitation with global left ventricular hypokinesia and ventricular ejection fraction of 20–25%. A provisional diagnosis of CAPS with myocarditis was made, and she was given intravenous immunoglobulin (IVIg) in a dosage of 1 g/kg body weight. Subsequently, repeat echocardiography showed a worsening ejection fraction of 10–15%. On the post-partum day 5, she developed cardiac arrest and expired.

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Dr. A●●●●●●●a S●●●i and 3 others like this2 shares
Dr. S●●●●●v S●●●●●1
Dr. S●●●●●v S●●●●●1 Obstetrics and Gynaecology
This patient left us at a controversial point to analyse and ponder whether the surgical intervention we did undertake in order to save the life of the mother primarily and the premature baby was definitely of less concern. I think with little more care the baby could have been delivered vaginally even though a breech, with proper counselling regarding the outcome and the mother could have stood the whole process better. Is there a justifiable place of vaginal delivery in such cases as grave as this !... Read more
Feb 8, 2019Like