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Endocarditis in Adults with Congenital Heart Disease Cover

Endocarditis in Adults with Congenital Heart Disease

Open Access
|Jun 2026

Figures & Tables

Table 1

Distribution of causative microorganisms in infective endocarditis among patients with congenital heart disease across population studies.

STUDY (ref)STREPTOCOCCI (%)STAPHYLOCOCCUS AUREUS (%)OTHER STAPHYLOCOCCI (CONS) (%)ENTEROCOCCI (%)OTHER PATHOGENS (%)
Niwa et al.1543.329.85.510.2
Snygg-Martin et al.819.322.47.89.8
Verheugt et al.1446.028.016.9
Havers-Borgersen et al.716.4 (viridans)17.54.45.8†
Van Melle et al.4029.622.78.77.010.1

[i] †Includes Coxiella burnetii, Streptococcus bovis, and other rare pathogens

Table 2

Rare microbiological agents causing infective endocarditis. spp: species

Brucella sppC. burnetiidBartonella sppT. whipplei
Mycoplasma sppLegionella sppFungiMycobacteria
Table 3

2023 European Society of Cardiology revised criteria for diagnosis of infective endocarditis (IE).

MAJOR CRITERIA
Blood culture positive for IE
Typical IE-related organisms isolated from two independent blood cultures: Oral streptococci, Streptococcus gallolyticus (formerly S. bovis), HACEK group, S. aureus, E. faecalisPersistent positivity of blood cultures for microorganism compatible with IE, defined as:
  • – at least two positive blood cultures of blood samples drawn > 12 hour apart

  • – Positivity of all three, or the majority of four or more separate blood cultures, with at least one hour between the first and last sample

A single positive blood culture for C. burnetii or a phase I IgG antibody titer greater than 1:800
Imaging positive for IE
Structural or metabolic abnormalities suggestive of IE identified on cardiac valves, perivalvular or periprosthetic regions, or intracardiac foreign material. These findings may be detected using: echocardiography (either transthoracic or transesophageal), cardiac computed tomography, 18F-fluorodeoxyglucose positron emission tomography, white blood cell single photon emission tomography/computed tomography
MINOR CRITERIA
Risk factors (ie, predisposing heart condition at high or intermediate risk of IE or people who inject drugs)
Fever (body temperature above 38°C)
Embolic vascular dissemination (including clinically apparent or incidentally detected lesions)
Systemic or pulmonary emboli or infarcts, abscessesHematogenous osteoarticular septic complicationsMycotic aneurysms
Intracranial ischemic or hemorrhagic lesionsConjunctival hemorrhagesJaneway’s lesions
Immunological phenomena
GlomerulonephritisOsler nodes, Roth spotsRheumatoid factor
Microbiological evidence
Positive blood culture that do not meet the definition of a major criterionSerological evidence of active infection caused by a microorganism compatible with IE
Infective endocarditis classification
Definite infective endocarditis
2 major criteria1 major criterion and at least 3 minor criteria5 minor criteria
Possible
1 major criterion and 1 or 2 minor criteria3-4 minor criteria
Rejected
Does not meet criteria for definite or possible at admission with or without a firm alternative diagnosis
Figure 1

Diagnostic pathway for suspected infective endocarditis (IE) in adults with congenital heart disease (ACHD). ECG: electrocardiogram; CT: computed tomography; 18-FDG PET: 18F-fluorodeoxyglucose positron emission tomography; WBC: white blood cell; SPECT: single-photon emission computed tomography

Table 4

Surgical indication for infective endocarditis based on the 2023 European Society of Cardiology guidelines. NVE: native valve endocarditis; PVE: prosthetic valve endocarditis; HF: heart failure; AVB: atrioventricular block; HACEK: Haemophilus, Aggregatibacter, Cardiobacterium, Eikenella, and Kingella

Emergency surgery is indicated in patients with aortic or mitral NVE or PVE when acute severe regurgitation, valve obstruction, or fistula formation leads to refractory pulmonary edema or cardiogenic shock (I – B)Urgent surgery is indicated in aortic or mitral NVE or PVE when severe acute regurgitation or obstruction cause HF symptoms or echocardiographic evidence of poor hemodynamic tolerance (I – B)
Urgent surgery is indicated when there is evidence of uncontrolled local infection (abscess, false aneurysm, fistula, enlarging vegetation, prosthetic dehiscence, new AVB) (I – B)In IE caused by fungal or multidrug-resistant pathogens, surgery is recommended urgently or electively depending on the hemodynamic status of the patient (I – C)
Urgent surgery should be considered in patients with IE and persistent positive blood cultures for more than one week or ongoing sepsis despite appropriate antimicrobial therapy and adequate management of metastatic infectious foci (IIa – B)Urgent surgery should be considered in PVE caused by S. aureus or non-HACEK gram-negative bacteria (IIa – C)
Urgent surgery is indicated in aortic or mitral NVE or PVE when vegetations remain ≥ 10 mm after one or more embolic events despite appropriate antimicrobial treatment (I – B)Urgent surgery is recommended in IE with vegetation ≥ 10 mm when another indication for surgery is present (I – C)
In low-risk surgical candidates with aortic or mitral IE, urgent surgery may be considered for vegetations ≥ 10 mm even in the absence of severe valve dysfunction or clinical embolic events (IIb – B)
Figure 2

Proposed management pathway for infective endocarditis (IE) in adults with congenital heart disease (ACHD).

DOI: https://doi.org/10.14797/mdcvj.1799 | Journal eISSN: 1947-6108
Language: English
Page range: 136 - 149
Submitted on: Feb 9, 2026
Accepted on: Apr 28, 2026
Published on: Jun 30, 2026
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services

© 2026 Andrea Busti, Giuseppe A. Annoni, Carlo Pace Napoleone, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.