Ischemic stroke as the first manifestation of peripartum cardiomyopathy: a therapeutic and diagnostic challenge

A 17-year-old woman with a history of bronchial asthma was transferred from the general pediatric ward to our cardiology department due to severe impairment of left ventricular (LV) systolic function and heart failure (HF) symptoms for several days. Six months earlier, she had a vaginal delivery (first pregnancy, without delivery complications, 40th week of gestation, neonate with Apgar 10). She was hospitalized in the pediatric ward for 46 days due to ischemic stroke (4 months after the delivery) with left hemiplegia. Computed tomography angiography revealed an occlusion of the right internal carotid artery and its intracranial branches. The patient did not present any typical HF symptoms before admission. Echocardiography (ECHO) showed a thrombus in the LV, enlarged left ventricular end-diastolic dimension (LVEDD) of 70 mm, and generalized hypokinesis with left ventricular ejection fraction (LVEF) of 20%. Laboratory tests showed elevated levels of N-terminal pro–brain-type natriuretic peptide (NT-proBNP) at 5855 pg/ml (reference range [RR], <⁠112 pg/ml), troponin T at 0.216 ng/ml (RR, <⁠0.022 ng/ml), C-reactive protein at 31.02 mg/l (RR, <⁠3.1 mg/l), white blood cells at 13.44 × 10⁹/l (RR, 4–10 × 10⁹/l), and D-dimer at 2.37 μg/ml (RR, <⁠0.5 μg/ml). Due to a long time between the stroke symptoms and diagnosis, the patient was not referred for thrombectomy. Treatment with enoxaparin was initiated, followed by warfarin. The size of the LV thrombus gradually reduced until the thrombus resolved completely, but the LVEF was unstable, as it increased maximally to 44% and then decreased to 15%. On admission to the cardiology department the patient presented with persistent left hemiparesis, central facial nerve palsy and crackles at the base of the right lung. Her blood pressure was 98/85 mm Hg, heart rate 101 bpm, and NT-proBNP level 4904 pg/ml. ECHO revealed LVEDD of 72 mm, LVEF of 15%, LV global longitudinal strain (GLS) of –8.7%, and pericardial effusion (Figure 1A and 1B). ECHO showed sinus tachycardia and QTc prolongation (551 ms). Holter ECHO did not reveal any arrhythmia. Cardiac magnetic resonance showed LV dilatation, late gadolinium contrast enhancement of the nonischemic type (basal and middle segments of the anterolateral and posterolateral wall) without thrombus, with increased LV trabecular walls without signs of myocardial edema (Figure 1C and 1D). During the hospitalization, a VVI cardioverter-defibrillator was implanted for primary prevention. An effective and adequate high-energy intervention due to ventricular fibrillation was observed the next day. Predischarge ECHO showed an improvement in LVEF to 20%–25% and LV GLS to –10%. An increase in NT-proBNP to 7358 pg/ml was observed. Pharmacotherapy consisted of warfarin dosed according to international normalized ratio, aspirin, bisoprolol, spironolactone, dapagliflozin, ivabradine, and furosemide. Despite early intolerance of sacubitril / valsartan or angiotensin-converting enzyme inhibitor (symptomatic hypotension), after a 1.5-month follow-up sacubitril / valsartan at 24/26 mg twice daily was initiated and was well tolerated. At this time, the following values of clinical markers were observed: NT-proBNP 3837 pg/ml, LVEDD 72 mm, LVEF 20%–25%, LV GLS –8.4%, as well as pericardial effusion, and stable clinical state. Taking into consideration the medical history, laboratory tests, and additional examination, peripartum cardiomyopathy (PPCM) was diagnosed. Coagulation disorders were excluded based on additional tests (protein C, S, antithrombin, factors V, VII, VIII, von Willebrand, mutations of the MTHFR gene [A1298C] and prothrombin gene [G20210A]).

PPCM presents as HF secondary to LV systolic dysfunction, usually with LVEF below 45%, in the third trimester of pregnancy or within 6 months following the delivery, without any other identifiable cause.¹ Major geographic variations in incidence exist (1–100 per 10 000 live births).² PPCM is associated with a mortality rate of around 6% at 6 months, but has a high chance of myocardial recovery of around 50% reported up to 3 years after the diagnosis.² HF and pregnancy are both procoagulant conditions, and the rate of thromboembolic events is high.^3,4 However, the etiology of PPCM is still not fully understood.⁵ PPCM is relatively rare and may have an atypical course, with the dominant picture resulting from thrombotic complications.