|Year : 2022 | Volume
| Issue : 4 | Page : 144-147
COVID-19-associated multisystem inflammatory syndrome in a child with fulminant myocarditis with successful outcome on extracorporeal membrane oxygenation: A case report
Prameela Joji, Swathy Subhash, N U Nisha Nair, Shiju Kumar, Neetu Gupta, Prijo Philip, Subash Sundarsingh, Shaji Palangadan
Paediatric Intensive Care Unit and Cardiothoracic surgery Unit, KIMSHEALTH, Thiruvananthapuram, Kerala, India
|Date of Submission||06-Apr-2022|
|Date of Decision||23-Jun-2022|
|Date of Acceptance||26-Jun-2022|
|Date of Web Publication||20-Jul-2022|
Dr. Prameela Joji
KIMSHEALTH, Anayara, Thiruvananthapuram - 695 029, Kerala
Source of Support: None, Conflict of Interest: None
Multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome-coronavirus-2 is a new entity which has to be picked up early for better outcomes. This hyperinflammatory syndrome usually presents with multiorgan dysfunction, predominantly affecting cardiovascular, mucocutaneous, and gastrointestinal systems. This case report describes a child with severe MIS-C who presented with fulminant myocarditis and subsequently suffered a cardiac arrest in the emergency department but was successfully revived and brought back to life by timely extracorporeal membrane oxygenation and supportive care.
Keywords: Coronavirus disease-2019, extracorporeal membrane oxygenation, fulminant myocarditis, intravenous immunoglobulin, multisystem inflammatory syndrome in children
|How to cite this article:|
Joji P, Subhash S, Nair N U, Kumar S, Gupta N, Philip P, Sundarsingh S, Palangadan S. COVID-19-associated multisystem inflammatory syndrome in a child with fulminant myocarditis with successful outcome on extracorporeal membrane oxygenation: A case report. J Pediatr Crit Care 2022;9:144-7
|How to cite this URL:|
Joji P, Subhash S, Nair N U, Kumar S, Gupta N, Philip P, Sundarsingh S, Palangadan S. COVID-19-associated multisystem inflammatory syndrome in a child with fulminant myocarditis with successful outcome on extracorporeal membrane oxygenation: A case report. J Pediatr Crit Care [serial online] 2022 [cited 2022 Aug 16];9:144-7. Available from: http://www.jpcc.org.in/text.asp?2022/9/4/144/351515
| Introduction|| |
Multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus-2 has been reported in many countries where coronavirus disease-2019 (COVID-19) pandemic has peaked and/or is fading. Although respiratory manifestations are the hallmark of COVID-19 infection, a vast majority suffer from myocarditis and acute cardiac injury. Here, we are presenting a unique case, where a 5-year-old child presented with hypotensive shock and fulminant myocarditis 4 weeks after exposure to COVID-19 infection, her journey through extracorporeal membrane oxygenation (ECMO) and successful recovery.
| Case Report|| |
A 5-year-old girl presented with a 3-day history of fever, vomiting, and abdominal pain. She was initially treated as an outpatient but as she developed increasing tiredness and lethargy and she was admitted to a nearby hospital. In view of hypotension requiring noradrenaline support, she was referred to our center for further care. The father of the child had tested COVID-19 positive 4 weeks earlier. This child also had a fever for 1 day during that time. With this background history and clinical presentation, MIS-C with myocarditis was strongly suspected.
In the emergency department (ED), she had waxing and waning sensorium, dyspnea, heart rate – 68/min, and hypotension with blood pressure of 80/50 mmHg on norepinephrine 0.1 mcg/kg/min. Auscultation revealed muffled heart sounds. A bedside echocardiogram (ECHO) showed severe left ventricular (LF) dysfunction with ejection fraction >30%. Dobutamine infusion was added to the ongoing norepinephrine. She was electively intubated and invasive ventilation commenced. Despite inotropic and ventilatory support, she continued to be bradycardic; transvenous pacing was planned. Before the procedure, she suffered a cardiac arrest in the ED; she was revived after two cycles of cardiopulmonary resuscitation (CPR).
Following this, she developed an episode of pulseless ventricular tachycardia needing one more cycle of CPR. Considering the clinical scenario, an emergency ECMO was planned. Since our patient presented with repeated cardiac arrest ventricular fibrillation and was on high inotropic support (injection noradrenaline 0.2 mcg/kg/min and injection dobutamine 15 mcg/kg/min), we initiated central cannulation venoarterial ECMO (VA-ECMO) along with epicardial pacing. ECMO circuit consisted of Rotaflow machine and quadrox oxygenator. The return cannula in aorta was 18 Fr. Medtronic cannula and the drainage canula in right atrium was 28 Fr Medtronic cannula.
At initiation, ECMO flow was 2.8 Litres per minute (LPM), Rotations per minute (RPM): 2800, Sweep: 1 LPM, and FiO2: 50%, and mean arterial pressure was maintained at 60 mmHg. Ventilator settings revealed pressure-controlled ventilation with FiO2 35%, inspiratory pressure: 16, RR: 10/min, positive end-expiratory pressure: 7, and generating tidal volume: 70–80 ml.
Anticoagulation revealed heparin infusion with activated clotting time titrated to 180–200 s and activated partial thromboplastin clotting time: 45–50 s.
Blood reports revealed highly elevated inflammatory markers (C-reactive protein 65 mg/L, interleukin-6 100 pg/mL, and ferritin 26975 ng/mL), and deranged renal and hepatic function (aspartate aminotransferase 2789U/L and alanine transferase 1804 U/L) with coagulopathy international normalized ratio of 3.3. She also had evidence of severe myocardial dysfunction. (D-dimer >10,000 ng/mL, NT-proBNP >25,000 pg/mL, Troponin-T 5190 mcg/mL). COVID-19 reverse transcription-polymerase chain reaction (PCR) was negative. Her blood and urine cultures were sterile. Whole-blood PCR for sepsis was also negative. Immunoglobulin M and immunoglobulin G COVID-19 antibody levels were also negative. In view of acute-onset hepatic and cardiac dysfunction, poisoning was also suspected but a toxicology screen was negative.
She was started on pulse methylprednisolone 30 mg/kg for 3 days and intravenous immunoglobulin (IVIg) 2 g/kg within 24 h of admission. In view of severe ischemic hepatitis, liver protective measures were also commenced. Within 12 h of initiation of ECMO, she was weaned off inotropic support. On day 1 of ECMO, electrocardiogram showed wide QRS complexes with P wave and global T-wave abnormalities and nonspecific interventricular conduction abnormality with both right and left bundle branch block likely due to severe global cardiac dysfunction which was confirmed on ECHO as well. On day 3, ECHO showed improvement in cardiac function with moderate LV dysfunction and mild right ventricular dysfunction.
On day 5 of admission, she developed hypotension recurred requiring inotropic support, excessive bleeding, and new-onset thrombocytopenia. Sepsis with disseminated intravascular coagulation was suspected and antibiotics were escalated to meropenem, polymyxin B, and caspofungin. Bleeding was managed conservatively with packed red blood cells, platelet transfusions, and tranexamic acid. Despite new-onset hypotension, her ECHO continued to improve. Following this, she developed oliguric acute kidney injury (AKI) on day 5 requiring hemodialysis. Slow continuous ultrafiltration (SCUFF) was done for 4 days through a separate hemodialysis catheter 11.5 Fr (not connected to ECMO circuit) in the left femoral vein. Blood and urine cultures remained sterile.
By day 9, the bleeding stopped and she was hemodynamically stable off inotropes. She was decannulated from ECMO on day 10 and switched to conventional ventilation. Weaning was planned as per the clinical parameters and epicardial ECHO findings. While doing epicardial ECHO, ECMO flow was reduced and ECMO was stopped briefly for few minutes without creating hemodynamic disturbances. Pacing wires were cut after pulling from the skin 8 days after sternal closure.
Post-ECMO, she developed critical illness myopathy. Magnetic resonance imaging of her brain and spine was normal. She underwent graded physiotherapy and neurorehabilitation. On day 15, she underwent a tracheostomy after a failed continuous positive airway pressure trial. On day 19, the child was weaned off ventilation. Tracheostomy decannulation was done on day 26.
At discharge from the hospital after 6 weeks of stay, she had normal cognition, full upper limb power, and could walk with support. On follow-up, she achieved complete neurorecovery and had a normal ECHO.
| Discussion|| |
The Extracorporeal Life Support Organization Registry shows a steady rise in the number of pediatric ECMOs with a promising increase in survival rates as well. ECMO provides biventricular and respiratory support for the critically ill child. The timing of initiation of ECMO, the patient selection, and underlying indication are important indicators of survival.
Fulminant myocarditis is an acute severe subset of myocarditis which rapidly worsens to multiorgan failure, cardiogenic shock, and death. Literature on the use of ECMO in this setting appears reassuring. Xiong et al. conducted a meta-analysis of six studies encompassing 172 pediatric patients with fulminant myocarditis who required VA-ECMO and reported survival rates ranging between 53.8% and 83.3% with a cumulative rate of 62.9%. In a single-center retrospective cohort study conducted in Vietnam, 37 out of 54 children with acute myocarditis received ECMO; the case fatality rate was 32% among children who required ECMO. In line with our case report, arrhythmias were noted to be an indicator for ECMO.
As in our patient, AKI is a common complication in children requiring ECMO. Children may have AKI before, during, or even after weaning from ECMO. López-Herce published a study wherein it was found that kidney function is significantly impaired in a high percentage of children undergoing ECMO, thus requiring continuous renal replacement therapies. They found that children with normal kidney function at the beginning of ECMO had a lower mortality rate and those surviving to pediatric intensive care unit discharge recover normal renal function.
Apart from renal injury, significant bleeding, neurological injury, and sepsis are other well-known complications of ECMO. Bleeding occurs due to therapeutic or patient-related factors. Intracranial hemorrhage can be missed unless actively sought for. Neurological injury on ECMO may be due to a multitude of causes including hypoxia, hypoperfusion, electrolyte imbalances, or coagulopathy. With the lack of standard guidelines on neuromonitoring in ECMO and the need for deep sedation, especially in children who may not tolerate awake ECMO, the identification of central and peripheral nervous system injury is often delayed. To our knowledge, there have been no studies on the association of critical illness myopathy in patients on ECMO.
In multiple cohorts of children with MISC and severe LV dysfunction, it has been shown that cardiac function returns to normal in about 2–3 days with inotropic support alone. In high acuity cohorts with severe cardiac dysfunction, the utility and success of ECMO have been published in the literature. The earliest cohort of MISC was reported by Riphagen and Whittaker from the UK where 58 children were treated with steroids, IVIg, interleukin antagonists, and supportive measures of which one child who required VA-ECMO succumbed to the illness. They have also noted that sustained arrhythmias led to hemodynamic collapse necessitating ECMO, In the largest cohort of 186 MISC patients reported from the US, eight needed ECMO, of whom three died. Belhadjer et al. reported a multicentric trial from France and Switzerland in 35 children with EF <30%, of whom 10 required ECMO. All patients were successfully weaned off ECMO with no mortality.
There are only a few reports of children with MISC from developing nations like India. We would like to emphasize the severe spectrum of MISC and the utility of ECMO with the successful outcome of our patients even in developing nations with limited resources. Being a novel disease condition, there is no standard protocol devised for the treatment of MISC. Case reports and series can add to the accumulating evidence for successful treatment of children, especially with the pandemic set to strike children. An amalgam of inotropic support, immunomodulation, and ECMO can result in a successful collaboration to treat a critically ill child.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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