1Otto-von-Guericke Universität, Medizinische Fakultät, Universitätsklinikum Klinik für Herz- und Thoraxchirurgie Magdeburg, Deutschland; 2Medizinische Hochschule Hannover Klinik für Herz-, Thorax-, Transplantations- und Gefäßchirurgie, OE 6217 Hannover, Deutschland; 3Universitätsklinikum Magdeburg A.ö.R. Klinik für Kardiologie, Angiologie und Pneumologie Magdeburg, Deutschland; 4Universitätsklinikum Magdeburg A.ö.R. Klinik für Herz- und Thoraxchirurgie Magdeburg, Deutschland
Our aim is to demonstrate the fatality of fulminant giant cell myocarditis (GCM) by presenting a rare case of GCM in an 18-year-old female with newly diagnosed Systemic Lupus Erythematosus (SLE) and emphasize challenges in its diagnosis and treatment.
Case presentation
An 18-year-old female patient was transferred to our clinic when she developed biventricular heart failure secondary to presenting with fatigue and fever to a local hospital after experiencing malaise for a total of 6 months prior, while previously only being prescribed iron supplements for anemia. Echocardiography revealed left-ventricular heart failure (left ventricular ejection fraction [LVEF] 5%), and a preliminary diagnosis of SLE was established relying on markedly elevated anti-dsDNA antibody levels. Due to the severity of her condition, the patient was seen by the heart team in our hospital, a collaboration of cardiology and cardiac surgery, where the decision for emergency ECMO (extracorporeal membrane oxygenation) installation and diagnostic myocardial biopsy was made, which confirmed a secondary diagnosis of GCM. Regardless of the initiated immunosuppressive therapy and cardiovascular support, the LVEF did not improve and remained 5-10%. The patient was transferred to Hannover medical school to be listed for a donor heart and received a biventricular assist device (BiVAD) as bridge-to-transplant (BTT). Despite the initial improvement in cardiac function and the overall condition during BiVAD therapy, there were, unfortunately, significant complications in the form of multi organ failure, embolic complications and subsequent hemodynamic deterioration during the course of the treatment, which complicated the recovery process until the patient ultimately passed, mere weeks after her initial presentation.
Discussion
GCM is often highly fatal. The only causative therapy option is aggressive immunosuppressive therapy, which tries to reduce myocardial damage and the resulting need for mechanical circulatory support (MCS) and cardiac transplantation. GCM tends to progress quickly and patients frequently require MCS, therefore, confirmatory diagnostic measures need to be instigated as soon as GCM is suspected. The 2021 European Society of Cardiology (ESC) Guidelines for the diagnosis and treatment of acute and chronic heart failurerecommend myocardial biopsies to be performed in order to differentiate the particular subtypes of myocarditis, after which specific treatment can be implemented. Bridge-to-transplant (BTT), which is often necessary in GCM patients, is also proposed in the 2021 ESC guidelines, but sparse high-quality evidence on whether to implant MCS in the case of cardiogenic shock means the decision still needs to be made by a multidisciplinary team.
Eventually, even though immunosuppressive therapy can prolong survival, heart transplantation is still the only definite treatment choice and BTT needs to be performed regularly and becomes even more relevant nowadays in the face of organ scarcity. However, despite technological advancements, they are constrained by cerebral as well as other complications. Therefore, early diagnosis and treatment of such aggressive myocarditis remain essential to improve patient survival.