BACTERIAL MYOCARDITIS IN SICKLE CELL PATIENT WITH ACUTE CHEST SYNDROME

Abstract

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: Sickle cell anemia is an autosomal recessive disorder leading to chronic hemolytic anemia secondary to abnormal hemoglobin. Sickle cell anemia has numerous clinical consequences such as acute pain crisis, acute chest syndrome, osteomyelitis, and renal failure. The most common cause of death for sickle cell patients is infection. A rare cause of death for sickle cell patients is infection secondary to viral myocarditis; and even rarer is bacterial myocarditis. In this case, our sickle cell patient passed away from a staphylococcal bacterial myocarditis. CASE PRESENTATION: A 44-year-old female with a significant past medical history of Sickle Cell Disease (Hemoglobin SS) presented to the emergency department with complaints of shortness of breath, 5 days of fever with abdominal pain and dysuria. Initial pertinent investigations showed a hemoglobin of 5.3, WBC 23.5, and mild hypokalemia with an acute kidney injury with elevated BUN/Creatinine (71/2.31). The urine analysis was positive, although the culture was negative. Blood cultures were drawn. Ceftriaxone and IV fluids were started. A CT abdomen and chest was obtained and showed mixed alveolar and interstitial opacities in bilateral lower lobes consistent with acute chest syndrome, hepatomegaly, and an enlarged heart. She was transferred to a tertiary center due to acute sickle cell pain crisis complicated by AKI, and acute chest syndrome. Patient was given a PCA pump with hydromorphone. Hematology recommended starting hydroxyurea and transfusing 2 units packed red blood cells. Troponin levels became elevated. Echocardiogram showed no wall motion abnormalities and a normal ejection fraction (50-55%). Patient continued to clinically deteriorate, so an exchange transfusion was started. During the exchange transfusion the patient coded and passed away. An autopsy was performed and showed interstitial neutrophilic infiltrates within the myocardium with micro abscesses with gram positive cocci identified. Blood cultures were positive for Staphylococcus aureus. The cause of death was identified as staphylococcal bacterial myocarditis. DISCUSSION: Sickle cell patients are susceptible to serious adverse outcomes from infections; notably infections are the leading cause of death in sickle cell patients. Death is often sudden and unexpected. Our patient was an example of an extremely rare cause of staphylococcal bacterial myocarditis with a concomitant acute chest syndrome that may have contributed to masking the underlying myocarditis and thus ultimately leading to death. CONCLUSIONS: Bacterial myocarditis is a very rare but potential cause of death in patients with sickle cell disease and the symptoms could be concealed from the clinician due to and overlapping acute chest syndrome. REFERENCE #1: Manci EA, Culberson DE, Yang YM, et al; Investigators of the Cooperative Study of Sickle Cell Disease. Causes of death in sickle cell disease: an autopsy study. Br J Haematol. 2003 Oct;123(2):359-65. REFERENCE #2: Cooper LT Jr. Myocarditis. N Engl J Med. 2009 Apr 9;360(15):1526-38. REFERENCE #3: McGee M, Shiel E, Brienesse S, et al; Staphylococcus aureus Myocarditis with Associated Left Ventricular Apical Thrombus. Case Rep Cardiol. 2018 May 23;2018:7017286. DISCLOSURES: No relevant relationships by Chad DeMott, source=Web Response No relevant relationships by Badri Giri, source=Web Response No relevant relationships by Elspeth Springsted, source=Web Response