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      Drugs & Diseases > Pediatrics: Cardiac Disease and Critical Care Medicine

      Pediatric Fungal Endocarditis

      Updated: Dec 21, 2020
      • Author: Sandy N Shah, DO, MBA, FACC, FACP, FACOI; Chief Editor: Syamasundar Rao Patnana, MD  more...
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      Overview

      Background

      Fungal endocarditis (FE) is a rare infection in pediatrics. Its incidence is increasing because more neonates are in intensive care units (ICUs), are undergoing cardiac surgical procedures, and are receiving hyperalimentation (CHA). [1]

      Fungal endocarditis rarely affects native valves. Rather, it occurs most frequently in neonates as part of a disseminated fungal infection, in patients following cardiac surgery, and in those who develop an intracardiac thrombus or valvular injury due to the presence of a central venous catheter (CVC).

      Fungal endocarditis is often difficult to diagnose because the presentation may be nonspecific, and the disease typically occurs in otherwise critically ill patients with confusing clinical pictures. [2, 3, 4]

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      Pathophysiology

      In pediatric patients, one fourth of fungal endocarditis cases have systemic candidal infection with cardiac involvement. These usually are associated with a right-sided intracardiac thrombus at the site of valvular injury due to the presence of a central venous catheter (CVC). [5]

      Fungal endocarditis may complicate intracardiac surgery as well as complicate intrathoracic or systemic fungal infection in those at highest risk.

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      Etiology

      No particular inheritance patterns are associated with fungal endocarditis.

      Causal organisms include the following:

      • Candida species (two thirds of all reported cases) [6]

      • Aspergillus species (particularly in postoperative patients, with spread from systemic and pulmonary infections, and in immunocompromised hosts) [7, 8]

      • Histoplasma capsulatum (causes pericarditis more frequently)

      • Blastomyces dermatitidis, Cryptococcus neoformans, [9] Coccidioides immitis (mostly pericarditis; rarely endocarditis)

      • Mucor species, Torulopsis glabrata, Trichosporon beigelii, Fusarium species (rare)

      • Pseudallescheria boydii (prosthetic valve endocarditis)

      • Scedosporium species [10]

      Risk factors include the following:

      • Neonatal period

      • History of cardiac surgery (eg, palliative shunt procedures, complex intracardiac repairs, vascular patches, vascular grafts, prosthetic valves) [11]

      • CVC in place

      • CHA

      • Broad-spectrum antibacterial therapy

      • Intravenous drug use

      • Preexisting valvular lesion or injury, such as congenital heart disease (eg, ventricular septal defect, atrial septal defect, patent ductus arteriosus, tetralogy of Fallot), [11] bacterial endocarditis, rheumatic heart disease

      • Transient fungemia after bowel surgery

      • Any condition associated with immune compromise (eg, transplantation, leukemia)

      Fungal endocarditis rarely affects native valves.

      Fungal endocarditis may spread from intrathoracic (particularly pleural-based) infections.

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      Epidemiology

      Incidence

      Fungi cause 0-12% (average 1.1%) of infectious endocarditis cases in children worldwide. Thus, the incidence rate is approximately 1.5-4 cases per 10 million children. Most published series are from the United States and other developed countries. Two thirds of fungal endocarditis is candidal. Among those in the neonatal intensive care unit (NICU), 1% develop disseminated candidal infection. Despite recent rises in frequency, this remains a rare infection, with reported cases numbering less than a few hundred in patients of any age.

      Data are too limited to document the incidence of fungal endocarditis in the developing world. As many risk factors for the disease are associated with advanced medical care, a direct relationship between the availability of these technologies and the frequency of this infection is likely.

      Race-, sex-, and age-related demographics

      No racial predisposition is present.

      A slight male predominance is observed.

      Increasingly, the age distribution of cases is bimodal. The number of cases reported is rising in neonates and, gradually with age, in adults in their second decade of life. In a recent review of the published literature, 48% of fungal endocarditis cases were in infants (93/192); 57 of these were in premature infants. [12]

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      Prognosis

      Morbidity/mortality

      Prognosis is improving because of advances in intensive and operative care, but the survival rate remains low. Chen et al reviewed the records of 8 patients treated at their center over a 12-year period and reported an in-hospital mortality rate of 25%; 2 additional deaths occurred within 4 months of discharge. [13] Ganesan et al performed a review of 192 published cases and reported a 56% overall mortality rate for pediatric fungal endocarditis. [12]

      The mortality rate remains high because of the difficulty in making a timely diagnosis, lack of effective antifungal antibiotics, need for surgical intervention in most cases, presence of underlying or predisposing conditions, and frequent comorbid conditions in these typically critically ill neonates and children.

      Complications

      The most common complications in survivors are associated with embolic phenomena, postoperative issues, and underlying or predisposing conditions.

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      Patient Education

      Survivors may require subacute bacterial endocarditis prophylaxis, depending on residual cardiac anatomy or abnormalities.

      For patient education resources, see the Infections Center, as well as Candidiasis (Yeast Infection).

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