eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Endocarditis, Fungal

Author: Robert W Tolan Jr, MD, Chief, Division of Allergy, Immunology and Infectious Diseases, The Children's Hospital at Saint Peter's University Hospital; Clinical Associate Professor of Pediatrics, Drexel University College of Medicine
Contributor Information and Disclosures

Updated: Feb 2, 2009

Introduction

Background

Fungal endocarditis (FE) remains a rare infection, although its incidence is increasing because more neonates are in intensive care and more neonates are undergoing cardiac surgical procedures and central hyperalimentation (CHA). It rarely affects native valves and occurs most frequently in neonates as part of a disseminated fungal infection, in patients following cardiac surgery, or in those who develop an intracardiac thrombus or valvular injury due to 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.

Pathophysiology

Approximately one fourth of neonates and children with systemic candidal disease have a demonstrable cardiac lesion. Fungal infection usually occurs in a right-sided intracardiac thrombus or at the site of a valvular injury secondary to a CVC. Fungal endocarditis may also complicate intracardiac surgery or intrathoracic or systemic fungal infection, particularly in those at highest risk.

Frequency

International

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 present.

Mortality/Morbidity

The mortality rate remains 75-90% because of difficulty in making the 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.

Race

No racial predisposition is present.

Sex

A slight male predominance is observed.

Age

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.

Clinical

History

  • Patients with fungal endocarditis (FE) may have a history of cardiac surgery complicated by symptoms of infection, such as fever, deteriorating cardiac status, embolic phenomena, and dehiscence.
  • History of intrathoracic or systemic fungal infection with spread to the heart is rare.

Physical

  • On rare occasions, fungal endocarditis presents as typical bacterial endocarditis, with fever, weight loss, splenomegaly, splinter hemorrhages, Roth spots (pale retinal lesions with surrounding hemorrhage), Osler nodes (painful nodular lesions on the finger and/or toe pads), petechiae, Janeway lesions (painless hemorrhagic plaques on the palms and/or soles), arthritis, and a new or changing heart murmur.
  • Often, an indwelling central venous catheter (CVC) is present. The use of CVC for central hyperalimentation (CHA) is an additional risk factor.
  • Occasionally, positive blood culture results or positive culture results of other tissues and fluids (despite negative blood culture results) are the only evidence.
  • Cardiac involvement, without other symptoms or signs of infection, may be the only clinically apparent feature.
  • An inflow obstruction (superior vena cava syndrome with cough, hoarseness, dysphagia, and/or a full sensation in the ears) due to an infected thrombus may be the sole manifestation of disease.
  • In neonates, symptoms are often nonspecific and include apnea and bradycardia, hypothermia, poor perfusion, feeding intolerance, increased ventilatory support, and evidence of septic emboli. Rarely, a new or changing heart murmur is present.
  • In neonates, Janeway lesions, petechiae, splinter hemorrhages, and evidence of multiple septic emboli have been reported, although Osler nodes and Roth spots have not been reported.
  • In the postoperative period, patients may have symptoms such as fever, cardiac decompensation, a new or changing heart murmur, evidence of embolic phenomena, and dehiscence.
  • Superior vena cava syndrome may manifest as hoarseness, swelling of the face, wheezing or stridor, and/or venous engorgement.

Causes

  • No particular inheritance patterns are associated with fungal endocarditis.
  • Causal organisms include the following:
    • Candida species (two thirds of all reported cases)1
    • Aspergillus species (particularly in postoperative patients and with spread from systemic and pulmonary infections)2
    • Histoplasma capsulatum (causes pericarditis more frequently)
    • Blastomyces dermatitidis, Cryptococcus neoformans, Coccidioides immitis (mostly pericarditis; rarely endocarditis)
    • Mucor species, Torulopsis glabrata, Trichosporon beigelii, Fusarium species (rare)
    • Pseudallescheria boydii (prosthetic valve endocarditis)
  • Risk factors include the following:
    • Neonatal period
    • History of cardiac surgery
    • CVC in place
    • CHA
    • Broad-spectrum antibacterial therapy
    • Intravenous drug use
    • Preexisting valvular lesion or injury, such as congenital heart disease, bacterial endocarditis, rheumatic heart disease, prosthetic valve
    • Transient fungemia after bowel surgery
    • Any condition associated with immune compromise
  • Fungal endocarditis rarely affects native valves.
  • Fungal endocarditis may spread from intrathoracic (particularly pleural-based) infections.

More on Endocarditis, Fungal

Overview: Endocarditis, Fungal
Differential Diagnoses & Workup: Endocarditis, Fungal
Treatment & Medication: Endocarditis, Fungal
Follow-up: Endocarditis, Fungal
References
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Further Reading

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Keywords

fungal endocarditis, FE, arthritis, Aspergillus, bacterial endocarditis, Blastomyces dermatitidis, Candida, candidal endocarditis, candidal infection, cardiac infection, central hyperalimentation, CHA, Coccidioides immitis, Cryptococcus neoformans, disseminated candidal infection, fever, fungal infection, Fusarium, heart murmur, Histoplasma capsulatum, infectious endocarditis, Janeway lesions, Mucor, neonatal sepsis, Osler nodes, overwhelming infection, petechiae, Pseudallescheria boydii, Roth spots, splenomegaly, splinter hemorrhages, superior vena cava syndrome, Torulopsis galbrata, Trichosporon beigelii, weight loss

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Contributor Information and Disclosures

Author

Robert W Tolan Jr, MD, Chief, Division of Allergy, Immunology and Infectious Diseases, The Children's Hospital at Saint Peter's University Hospital; Clinical Associate Professor of Pediatrics, Drexel University College of Medicine
Robert W Tolan Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society for Microbiology, American Society of Tropical Medicine and Hygiene, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, Phi Beta Kappa, and Physicians for Social Responsibility
Disclosure: GlaxoSmithKline Honoraria Speaking and teaching; MedImmune Honoraria Consulting; MedImmune Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Novartis Honoraria Speaking and teaching; sanofi pasteur Grant/research funds Unrestricted research grant; sanofi pasteur  Consulting; sanofi pasteur Honoraria Speaking and teaching; Tap Honoraria Speaking and teaching; Baxter Healthcare Honoraria Speaking and teaching

Medical Editor

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, Heart Rhythm Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Julian M Stewart, MD, PhD, Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College
Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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