Stress cardiomyopathy with posterior reversible encephalopathy syndrome in Eclampsia
Gayathri k, Brindha, Gowsalya
Department of Anaesthesia, Kauvery Hospital, Hosur
Abstract
Eclampsia is a multi-system disorder characterized by new onset of generalized tonic -clonic seizures which typically lasts 60 to 90 secs, occur during antepartum (20 weeks after gestation) intrapartum and postpartum period in a pre eclamptic patient, and has an unpredictable outcome, with high morbidity and mortality for both mother and fetus. Eclampsia as such imposes adverse outcomes; managing it’s complications has its own consequences in the perinatal period. Hereby we are presenting a unique case of a 31 year old ASA 2 hypothyroid primi at 38 weeks of gestation with uneventful antenatal period, who presented to ER with eclamptic fit, developing stress cardiomyopathy & Posterior Reversible Encephalopathy Syndrome ( PRES) in the immediate postoperative period. These two entities are being increasingly reported to occur concomitantly under same circumstances.
Introduction
Incidence of progression to eclampsia occur in approximately 2 to 3% of those with severe preeclampsia, the incidence being 1.6 to 10 per 10000 deliveries in developing countries. The reported prevalence of PRES in those with eclampsia ranges from 21 to 100%. Eclampsia is associated with increased risk of maternal mortality and morbidity, such as abruption placenta, Disseminated Intravascular Coagulation (DIC), pulmonary edema, aspiration pneumonia, acute renal failure, Hemolysis, Elevated Liver Enzymes, Low Platelet count (HELLP) syndrome, PRES and rarely stress cardiomyopathy. Fetal complications include severe prematurity, and IUGR.
Posterior reversible encephalopathy syndrome (PRES), also known as acute hypertensive encephalopathy, is a rare reversible complication of eclampsia, diagnosed with combinations of typical risk factors and clinical features, and supported by radiological findings (2).Because of advances in imaging methods and increasing awareness the association between PRES and eclampsia is becoming increasingly recognised. PRES is regarded as diagnosis of exclusion as there no specific diagnostic criteria. It’s classical presentation is neurological symptoms like headache, visual mpairment, altered mental status, seizures and other focal deficits like numbness along with unique neuro imaging findings of vasogenic edema involving the posterior circulation.
Stress cardiomyopathy is characterized by reversible ventricular dysfunction in the absence of obstructive coronary artery disease. They share a common pathophysiology where catecholamine surge being the triggering factor [5].
Case Presentation
31-Year-old primi at 38 weeks of gestation was brought to ER with c/o 3 episodes of seizures at home each lasted for few seconds. She was brought within half an hour of the event. She had h/o headache the previous night. She is a known case of gestational hypothyroid on T. Thyronorm 50 mcgs since 3rd month of gestational age and she was also known to have a seizure disorder-last episode 10 years back & was not on any medication and had an uneventful antenatal period.
On Examination
On presentation to ER patient was restless, irritable & agitated with vitals of;
HR: 145 bpm, BP: 160/100mmHg, Spo2: 96% RA. After around 5 min of arrival to ER she again had generalized tonic clonic seizures.
Immediately resuscitation measures were started, airway secured (RSI) to prevent aspiration & patient was ventilated with 100% O2 & and placed on left lateral position.
Standard obstetric care was provided with carefully monitoring fetal heart rate. Inj. Magnesium sulphate 4 g bolus given over 20 min to control seizures and it was continued for 24 hrs with the maintenance dose of 2gm per hour, f/b Inj. levitiracetam 1g bolus to prevent further seizure attacks. Adequate control of blood pressure was achieved with inj. Labetalol 10 mg iv in incremental doses to reach the goal of BP < 150/100mmHg.
The patient was classified under ASA grade 4 After getting written and verbal consent, she was taken up for LSCS immediately after initial stabilization, under General Anesthesia.
Intra Op
Intra op was uneventful & she delivered a live girl baby weighing 2.5 kg.
MgSO4 infusion was continued and patient shifted to ICU on mechanical ventilation at the end of the surgery
Post Op
Sedation continued with dexmedetomidine infusion and Magnesium maintenance dose continued for 24 hr, with monitoring of reflexes, creatinine function and urine output.
Basic investigation done was positive for urine albumin (3+) and all other biochemical tests were within normal limits.
CT- Brain
Impression
CT Brain done for seizures showed symmetrical hypodense areas in bilateral occipital & posterior fronto parietal region with f/s/o Posterior reversible encephalopathy syndrome.
Management
- Antiepileptics and antihypertensives were continued as per neurologist advice, and planned for EEG & MRI after stabilization.
- Transthoracic ECHO was performed that revealed f/s/o stress cardiomyopathy with ejection fraction of 30%, severe LV dysfunction, global hypokinesia of LV, good RV function with no PAH.
- Trop I: 345.3, BNP: 892.
- Antiplatelets were administered
- Patients neurological symptoms slowly began to resolve by next day
- Patient was conscious, alert, obeying commands, and haemodynamically stable.
- Repeat ECHO showed improved EF: 50 -55% with adequate LV function. Patient was The patient was observed for 36 hrs.She was discharged on the 3rd postoperative period from ward in stable condition .
Discussion
Despite advancements in medical management eclampsia remains the leading cause of maternal and perinatal morbidity and mortality. Several factors have been associated with increased risk of eclampsia such as black and Hispanic race , advanced maternal age, nulliparity, maternal age less than 20 years, multifetal gestation etc .
Several pathological mechanisms have been implicated in the pathogenesis of eclampsia. Mainly there is alteration in cerebral auto regulation as in hypertensive encephelopathy, endothelial dysfunction due to increased hydrostatic pressure and decreased cerebrovascular resistance with disruption of blood brain barrier (BBB), increased permeability at the BBB, focal cerebral edema, neuro inflammation and neuronal damage . BBB permeability may be increased by the circulating factors such as VEGF (vascular endothelial growth factor) and placental growth factor as seen in preeclamptic patients. Increased oxidative stress in the placental circulation causes oxidative conversion of LDL to ox LDL that generates complex signaling cascades leading to induction of inflammatory pathway and increased production of superoxide which further enhances endothelial dysfunction.
Various case reports of stress cardiomyopathy have been published following status epilepticus in the general population .
Posterior Reversible Encphalopathy Syndrome (PRES)
PRES, also known as reversible posterior leukoenchalopathy syndrome (RPLS), is a clinical imaging syndrome with posterior -predominant vasogenic brain edema, most frequently affecting young or middle aged adults with the mean age of 45years, with female predominance even after excluding obstetric patients.
PRES is a clinico radiological reversible entity that presents with non specific symptoms manifesting over several hours or days [3]
- Encephalopathy develops in 28 to 94% of patients and ranges from mild confusion, altered mental status, to stupor.
- Headache which is dull, diffuse and gradual in onset.
- visual symptoms such as reduced visual acuity, diplopia, field defects, visual hallucinations, and acute cortical blindness in 40% of patients
- Seizures affecting 74 to 87%, and typically occurs within 24 to 48 hrs of presentation. In 3% of patients it may evolve into status epilepticus.
- Patients can also have other neurological deficits, nausea and vomiting .
PRES has various etiologies and may develop as a part of PRES -associated medical conditions hypertension, autoimmune diseases, sepsis, renal disorders like glomerulonephritis, malignancy, solid organ transplantation, toxins, preeclampsia and eclampsia. It may result from medical treatments such as immunosuppressants like tacrolimus, cyclosporine, chemotherapeutic drugs, corticosteroids, intravenous immunoglobulins,, iv contrast, tyrosine kinase inhibitors, linozolid, lithium and hypomagnesimia [1]
Eclampsia on presentation, postpartum eclampsia, Caesarian delivery, use of labetalol during surgery , higher gestational age, recurrent seizures were associated with increased risk of PRES. This condition is also defined as obstetric PRES.The incidence of PRES in Pregnant women was 0.22%. Obstetric PRES is a serious maternal complication endangering life of both mother and foetus in acute stage and hence diagnosing the disease in early stages is mandatory to prevent adverse pregnancy outcomes.
Pathophysiology
PRES is associated with cerebral autoregulatory failure and breakthrough vasodilation due to severe hypertension. As a result there occurs cerebral hyperfusion (hyper perfusion theory) subsequently endothelial dysfunction secondary to circulating endogenous or exogenous toxins leading to blood brain barrier disruption & vasogenic edema[1]. The lesions of vasogenic edema are usually reversible within few days with prompt treatment, and at the same time can exhibit cytotoxic edema, ischemia and infarction (vasospasm theory/“cytotoxic/immunogenic theory) without timely intervention [2].
PRES predominantly affects the posterior circulation as it has less sympathetic innervation to counter reflex parasympathetic vasodilatation.
Posterior reversible enchalopathy syndrome is a misnomer since it can extend beyond posterior cerebrum and though reversible it can progress to permanent cerebral damage and encephalopathy may not present in all cases.
Biomarkers such as placental growth factor, soluble vascular endothelial factor, and serum magnesium, LDH, albumin have been shown to be associated with PRES in eclamptic patient. NFL an important biomarker has been proved to be associated with obstetric PRES .
Serum Neurofilament light chain is a marker of neuroaxonal injury. Neuro filaments are nonspecific cytosketon proteins and composed of neurofilament heavy (NF), medium and light chains (NFL). It is elevated in CSF and blood in acute and chronic neurological conditions and also in neurodegerative disease and in stroke [6].
Diagnosis & Treatment
Neuroimaging is not recommended routinely for patients with eclampsia and done only if the patient has focal neurological signs, recurrent convulsions and prolonged coma.
MRI is the gold standard diagnostic test for PRES & it’s finding being symmetrical hemispheric vasogenic edema affecting subcortical white matter and often extends to the overlying cortex and hyper intensities in the posterior region of brain in 70%. A central-variant (brain stem pattern) affecting the brainstem, basal ganglia, post limb of internal capsule, cerebellum and periventricular regions without cortical and subcortical involvement is seen in few cases. Most patients with PRES will show complete resolution of imaging findings in one or two weeks.
Usually treatment is supportive, gradually controlling blood pressure, and anti seizure medications and treating the underlying pathology. Sudden decrease in blood pressure may increase the risk of cerebral hypoperfusion and ischemia.
As the precipitating factor was eclampsia, in this case she was managed with antihypertensive, antiepileptics.
About treatment of seizures: traditional antiepileptics used for status epilepticus like phenytoin, benzodiazepines , phenobarbital may induce severe adverse effects [1].
Patients with eclampsia have reported long term cognitive difficulties related to memory and concentration in later years
Autonomic and endovascular dysfunction are the central mechanisms contributing to both PRES and stress cardiomyopathy and have been associated with good prognosis with supportive management.
Stress Cardiomyopathy
Eclampsia is associated with a 12 fold increased risk of cardiovascular morbidity such as MI, CVA, acute heart failure, cardiomyopathy or cardiac arrest.
Various case reports of stress cardiomyopathy have been published following status epilepticus in the general population .The actual incidence of stress cardiomyopathy in pregnancy remains unknown.It is a rare transient cardiac dysfunction that has been reported in Pregnant women with multiple triggering conditions.
Stress cardiomyopathy is a condition characterized by non-atherosclerotic form of reversible ventricular systolic dysfunction, in the absence of obstructive coronary artery disease and has typical apical ballooning [5]. It is transitory and triggered by acute emotional stress and hence known as broken heart syndrome or TTCM (Takotsubo cardiomyopathy), most commonly seen among the post menopausal women.
It mimics acute coronary syndrome, presenting with angina type chest pain, syncope or dyspnea, diaphorosis, palpitations, with ischemic ECG changes and modestly elevated cardiac biomarkers-significant rise of troponkin levels, mild elevation of creating kinase and marked elevation of BNP.
Inter TAK Diagnostic Criteria
Pathology
Many are associated with “life time stressors” being the triggering factor. Majority of these patients have preceding emotional or physical stress that leads to catecholamine release causing microvascular dysfunction, myocardial stunning ,coronary & multivessel spasm [7].
Transthoracic ECHO shows akinetic basal and mid anterior walls and normal contractility in the apical segment. Coronary angiogram is the confirmatory test.
Differential diagnosis of stress cardiomyopathy include pulmonary emboli, ischemic obstuctive coronary artery disease, peripartum cardiomyopathy or TCM, spontaneous coronary artery dissection. As opposed to stress cardiomyopathy which mimics ACS, peripartum cardiomyopathy present with features of congestive heart failure. Stress cardiomyopathy also differs in that it can develop shortly after delivery as opposed to peripartum cardiomyopathy, which takes months to manifest clinically. Risk factors for both stress cardiomyopathy and peripartum cardiomyopathy include older age, black race, preeclampsia, multiparty and twin gestations[5]. In addition, psychological stressors and coexisting conditions associated with increased sympathetic response, acute intra cranial disease all can trigger Stress cardiomyopathy.
Differentiating between these two conditions is very important as the management differs entirely. Supportive care and recovery from physical and emotional insult often results in complete recovery of cardiac function.
Stress cardiomyopathy patients usually recover fully and recovers more rapidly as compared to peripartum cardiomyopathy.
Prevention of Eclampsia
For primary prevention of eclampsia low dose aspirin should be considered in high risk groups. Secondary prevention will include weekly monitoring, use of anti hypertensive medications, timely delivery, prophylactic use of magnesium sulphate during labour and immediately after delivery.
Conclusion
Concomitant occurrence of both PRES & stress cardiomyopathy in an eclamptic patient is a rare scenario. But the treating physician has to be aware of these symptoms in preclamptic & eclamptic patients and without timely intervention patient’s condition can deteriorate and result in developing status epileticus, cerebravascular disease, intracranial hypertension, irreversible neurological damage and even death. Stress cardiomyopathy has to be differentiated with peripartum cardiomyopathy .As both of these can arise any time in the antenatal period and may even extend into the post partum period and need for close peri & postpartum follow up ,aggressive blood pressure control and early neuro imaging may have a role in managing these patients. Hence, the risk factors has to be rapidly identified and the underlying etiology should be addressed promptly. Patients should be counseled about the clinical features , early warning signs, treatment and the importance of regular follow up and also about risks in successive pregnancies to be addressed since the rate of rate of recurrent eclampsia is 2%.
References
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- Bahadur A, Mundhra R, Singh R, Mishra J, Suresh G, Jaiswal S, Sinha D, Singh M. Predictors of posterior reversible encephalopathy syndrome (PRES) in women with pre-eclampsia/eclampsia: a retrospective analysis. Cureus. 2022 Nov 13;14(11).
- Yadav D, Garg L, Narwal P, Ladkany R, Franey L. Concomitant takotsubo cardiomyopathy with PRES syndrome: A coincidence or a real heart–brain connection?. Journal of Cardiology Cases. 2015 Aug 1;12(2):48-51.
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- Xiabaco F, Yanlin L et al Serum Neurofilament Light ;a Potential diagnostic and Prognostic biomarker Obstetric Posterior Reversible Encephalopathy Syndrome. Molecular Neurobiology (2021)58;6460-6470
- Takotsubo Cardiomyopathy MimickinMultivessel Coronary Artery Disease FollowingSpinal SurgeryDolores Sanchez Morey , Samer KholokiMedicine, Saint James School of Medicine, The Valley, AIA 2. Internal Medicine, La Grange Memorial HospitalChicago, USA.
Dr. Gayathri k
Senior Consultant – Anaesthesia
Dr. Gowsalya
PG – Resident
