Acute fulminant carditis presenting with sustained ventricular tachycardia, and recovery after extracorporeal cardiopulmonary resuscitation

Abstract

A 12-year-old boy consulted a local physician with complaints of cough, abdominal pain, shortness of breath and general malaise. Medications for symptomatic relief and bed rest were suggested. The flu-like symptoms were relieved on the 2nd day, and the general malaise with repeated vomiting, chest pain and chest tightness attenuated on the 3rd day. A chest x ray showed multiple pneumonic patches with borderline cardiomegaly. Poor left ventricular function was noted, and the left ventricular ejection fraction was reduced to 21%. Although multiple episodes of sustained ventricular tachycardia were converted by six repeated cardiac defibrillations and a xylocaine (intravenous) bolus infusion, his general condition went downhill to shock and proceeded to several episodes of heart standstill that necessitated cardiopulmonary resuscitation. Extracorporeal cardiopulmonary resuscitation was installed via femoral cannulation. Cardiac function progressively recovered to normal, and extracorporeal membrane oxygenation was removed on the 7th day. The patient completely recovered and was discharged on the 15th day with no neurological sequelae.

BACKGROUND

Fulminant myocarditis (FM) is characterised by rapid onset of cardiac arrest, usually following a flu-like illness. Early referral from a local physician, confirmation of the diagnosis at the emergency room or intensive care unit, prompt installation of intensive cardiovascular support including extracorporeal membrane oxygenation (ECMO), and subsequent renal dialysis against multiorgan failure can be lifesaving; however, reports of experience with providing extracorporeal cardiopulmonary resuscitation (ECPR) to paediatric patients with acute FM is quite limited. In addition, the clinical presentation has rarely been described in detail. Pediatric Advanced Life Support (PALS) protocols are the current standard of care for managing paediatric cardiac arrest. Paediatric cardiac arrest most often has a poor outcome with conventional cardiopulmonary resuscitation (CPR). This report describes a case with FM in which paediatric cardiac arrest and sustained ventricular tachycardia refractory to PALS protocols were successfully treated with ECPR during the acute stage of the illness.

CASE PRESENTATION

A 12-year-old boy consulted a local physician with complaints of a mild cough, shortness of breath, abdominal pain and general malaise. Medications for symptomatic relief and bed rest were suggested. The flu-like symptoms were relieved on the 2nd day, and the general malaise with repeated vomiting, chest pain and chest tightness was attenuated on the 3rd day, after which he was referred to a local hospital. Severe dyspnoea, ventricular tachycardia and pneumonia were noted. Since an ECG demonstrated high-degree atrioventricular block, diffuse ST-T ischaemic change, and bizarrely widened ventricular tachycardia (fig 1), the patient was referred to our hospital the same day. His past history was non-contributory. A physical examination at admission revealed a clear consciousness level, height of 102 cm, weight of 40 kg, body temperature of 38°C, blood pressure of 90/70 mmHg, a coarse breathing sound, severe tachypnoea with suprasternal retraction (with a respiratory rate of 32/min), tachycardia (with a heart rate of 130/min), cardiac murmur (−), galloping rhythm (+) by auscultation, a flat and soft abdominal region, a prolonged capillary refilling time, and pale lips. After direct admission to the paediatric intensive care unit, although multiple episodes of sustained ventricular tachycardia attacks were converted by six repeated cardiac defibrillations (initial dose of 100 J (2.5 J/kg) and the subsequent three-stacked shock sequences with 150 J (4 J/kg) and a xylocaine (intravenous) bolus infusion, his general condition went downhill to shock and proceeded to several episodes of heart standstill converted with CPR. ECPR was installed via right femoral cannulation. Continuous venovenous haemodialysis (CVVD) was installed with ECMO due to initial oliguria and for washing out the cytokines. Massive haemothorax over the right lung was drained on the 4th day. Disseminated intravascular coagulation was suspected on the 5th day (D-D dimmer of 3.98). Fresh frozen plasma was given thereafter. Cardiac function progressively recovered to normal, and the ECMO and CVVD were removed on the 7th day.

Figure 1.

ECG at admission (3rd day).

INVESTIGATIONS

Laboratory data on admission (3rd day) revealed leukocytosis (white blood cells of 22.7×10⁹/l; segment/lymphocytes of 87%/10%), an electrolyte imbalance (potassium of 5.2 mmol/l), hyperglycaemia (blood sugar of 508 mg/dl), and an elevated troponin I level (18.37 ng/ml). Chest plain film showed patchy consolidation in the right lung field and left peripheral middle lung field (fig 2). The cardiac echo showed a dilated left ventricular chamber (LVIDS/LVIDD 43.1 mm/47.7 mm) with a diminished eject fraction (21%). Viral and bacterial screening tests were all negative. Chest CT on the 4th day showed multiple large patchy consolidation over the bilateral lungs, and this favoured a diagnosis of severe pneumonia or acute respiratory distress syndrome (fig 3). Fibrebronchoscopy on the 4th day revealed intrapulmonary inflammation.

Figure 2.

Chest plain x ray at admission (3rd day).

Figure 3.

Chest CT scan (4th day).

DIFFERENTIAL DIAGNOSIS

Pneumonia with acute respiratory distress syndrome, pulmonary embolism and idiopathic ventricular tachycardia.

TREATMENT

Conventional CPR, ECPR (initial ECMO setting: flow of 1.55 l, pump speed of 2560/min, fraction of inspired oxygen (FiO₂) of 80%, O₂ flow of 1.5 l, and heater temperature of 37°C) and continuous venovenous haemodialysis.

OUTCOME AND FOLLOW-UP

The patient completely recovered and was discharged on the 15th day with no definite neurological sequelae (during a follow-up time of 3 months).

DISCUSSION

Acute FM is clinically characterised by preceding non-specific extracardiac symptoms and rapid onset of dyspnoea associated with ECG changes. The clinical course is usually stormy with a clinical presentation of imminently lethal cardiogenic shock, pulseless ventricular arrhythmia, or cardiac arrest.^1,2 Early recognition of the clinical picture and prompt installation of adequate life support after confirming the diagnosis are crucial to providing better chances of recovery for these patients.

PALS protocols are the current standard of care for managing paediatric cardiac arrest.³ However, FM carries a high mortality, ranging from 50% to ∼75% under conventional CPR procedures without immediate mechanical circulatory support. ECPR is the rapid deployment of ECMO to provide immediate cardiovascular support for patients who have cardiac arrest unresponsive to conventional CPR measures. ECPR has been used as an extension of traditional cardiopulmonary resuscitation. Several series have reported that ECPR in children with FM refractory to conservative CPR measures was a significant factor in their improved survival.^4,5 A recent report further suggested that the potential survival benefit persists for adult patients with in-hospital cardiac arrest of heterogeneous cardiac origins, even if conventional CPR continued for more than 10 min.⁶ Although there are risks associated with ECMO support and therefore it should be used judiciously,⁷ we propose FM as a potential subgroup of cardiac arrest patients who could benefit from ECPR. We have no experience of using therapeutic hypothermia in combination with ECPR. Mild therapeutic hypothermia becomes as standard therapy for patients with in-hospital cardiac arrest and asystole who are comatose after return of spontaneous circulation.⁸ Nevertheless, the injured myocardial cells are very sensitive to changes in temperature. Hypothermia may compromise cardiac performance and cause serious arrhythmic adverse effect in patients with FM.⁹ Further studies are required to prove its beneficial effects in these children. This report describes a case with FM in which cardiac arrest and sustained ventricular tachycardia refractory to PALS protocols were successfully treated with ECPR during the acute stage of the illness.

LEARNING POINTS

• For practitioners: it is important not to overlook the early manifestations of acute carditis and ensure quick referral for further diagnostics.

• For residents in hospitals: residents in the emergency room and intensive care unit should be able to recognise the rapid progression of this disease entity and request an immediate cardiac confirmatory diagnosis.

• For attending physicians: understanding the limitations of conventional cardiopulmonary resuscitation and quickly organising extracorporeal cardiopulmonary resuscitation standby and installation are crucial to saving lives.

• For the Pediatric Advanced Life Support (PALS) committee (American Heart Association): we provide this evidence-based case report to support revision of PALS management.