Dental management and preventive strategies in pediatric patients with congenital or acquired heart disease: a systematic review

2.1. Protocol and registration

This study was designed as a systematic review of the literature on oral health status and dental management in children and adolescents with congenital heart disease (CHD) or other cardiovascular conditions that predispose to infective endocarditis (IE). This systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement [42].

2.2. Search processing

A structured electronic search was performed in three databases: PubMed, Scopus, and Embase. The search covered the period from January 2000 to October 2025 and was restricted to articles written in English, involving human participants, and with full text available. Combinations of Medical Subject Headings (MeSH) and free-text terms were used, including but not limited to:

• for the cardiac condition: congenital heart disease, “congenital heart defect, “pediatric cardiology”, “heart disease”, “cardiac disease”
• for the oral domain: “oral health”, “dental health”, “dental caries”, “tooth decay”, “plaque”, “gingivitis”, “periodontal disease”
• for care and prevention: “dental care”, “dental treatment”, “oral hygiene”, “preventive dentistry”, “oral health education”, “prophylaxis”, “infective endocarditis”

Database-specific search strings were adapted to each platform’s syntax. In addition, the reference lists of relevant articles and key guidelines were manually screened to identify further eligible studies (snowballing).

2.3. Inclusion criteria

Studies were considered eligible if they met all of the following inclusion criteria:

• Population: children or adolescents aged 0–18 years, with a diagnosis of congenital heart disease and/or other cardiovascular conditions associated with increased IE risk (as defined by the individual studies or by major cardiology guidelines).
• Content: The study had to report data on at least one of the following: oral health status (e.g., caries indices dmft/DMFT, plaque, gingivitis, periodontal parameters); access to and utilization of dental care; oral hygiene behaviors, dietary habits, knowledge/attitudes of patients and/or caregivers; preventive or educational interventions specifically targeting pediatric cardiac patients.
• Study design: The following designs were eligible: observational studies (cross-sectional, case-control, cohort); interventional studies (e.g., preventive programs, educational interventions); mixed-methods studies with a quantitative component.
• Publication type and language: original research articles published in peer-reviewed journals, written in English, published between 2000 and October 2025, available in full text.

2.4. Exclusion criteria

• studies including exclusively adult patients, or where pediatric data could not be separated from adult data;
• articles focusing solely on dental or orthodontic treatment without specific reference to children with CHD or cardiovascular disease;
• case reports and small case series with fewer than five patients (except for rare exceptions considered uniquely informative and explicitly discussed as such);
• narrative reviews, systematic reviews, meta-analyses, editorials, conference abstracts, and letters without original data;

2.5. PICO Question

The review was conducted using the PICO criteria:

Participants: children and adolescents with congenital heart disease;

Interventions: structured oral health prevention programs, dental follow-up protocols, or educational interventions for patients and/or caregivers;

Comparisons: standard care or healthy peers when available;

Outcomes: caries experience, plaque and gingival indices, oral hygiene behaviors, use of dental services, and expected impact on infective endocarditis risk.

2.6. Data processing

Two reviewers (G.L. and L.M.) independently consulted the databases to collect studies and rated their quality based on selection criteria. The selected articles were downloaded in Zotero (Version 6.0.15). Any divergence between the two authors was resolved through discussion with a senior reviewer (F.I.).

3.1. Study Selection

The database search identified 409 records, of which 310 were retrieved from PubMed, 58 from Scopus, and 41 from Embase (Figure 1). After removing 62 duplicate records, 347 unique articles remained for title and abstract screening.

During this first screening phase, 293 records were excluded because they were clearly unrelated to pediatric cardiac populations, did not address oral health or dental management, or did not meet the predefined eligibility criteria. The full texts of the remaining 54 articles were retrieved and assessed for eligibility.

Following full-text evaluation, 42 reports were excluded as off-topic or insufficiently focused on oral health management, preventive protocols, or endocarditis prophylaxis in children with cardiac or cardiovascular diseases. Ultimately, 12 studies satisfied all inclusion criteria and were incorporated into the qualitative synthesis. The overall selection process is illustrated in the PRISMA flow diagram (Figure 1).

3.2. Study Characteristics

The 12 included studies, published between 2001 and 2025, investigated different aspects of oral health care in pediatric patients with congenital or acquired cardiovascular diseases. Most studies adopted an observational, cross-sectional design or survey-based approach, while a smaller number used cohort or interventional designs focusing on preventive or educational programs. Across the studies, sample sizes varied widely, ranging from small single-center cohorts to larger multicentre or population-based samples of children and adolescents with congenital heart disease or other cardiac conditions at risk of infective endocarditis. Several articles included comparison groups of healthy peers, whereas others examined only pediatric cardiac patients. The most frequently reported outcomes were dental caries experience (e.g., dmft/DMFT indices), plaque and gingival indices, and qualitative or quantitative measures of oral hygiene behaviors and dental service utilization. Some studies specifically evaluated preventive protocols or educational interventions, such as structured oral health programs or targeted counseling for families, and their impact on knowledge, behaviors, or clinical oral health indicators. The characteristics of the included studies are summarized in Table 2.

The risk-of-bias assessment is summarized in Table 3.

Study (first author, year)	Population & selection	Outcome measurement	Confounding / comparability	Follow-up / completeness	Overall risk of bias
Bsesa et al., 2023 [26]
Downing et al., 2022 [34]
Raad & Shihab, 2023 [19]
Schulz-Weidner et al., 2021 [35]
Erbas Unverdi et al., 2024 [27]
Sivertsen et al., 2018 [23]
Kolçakoğlu et al., 2024 [37]
Gonnella et al., 2021 [39]
Wilson et al., 2007 [3]
Delgado et al., 2023 [4]
Al-Karaawi et al., 2001 [31]
Dunlop et al., 2013 [32]

3.3 Quality Assessment and Risk of Bias of Included Articles

A descriptive risk-of-bias assessment was performed for all twelve included articles across four main domains: (1) Population and selection (clarity of inclusion criteria, representativeness and potential selection bias); (2) Outcome measurement (use of validated clinical or histological indices versus self-reported data); (3) Confounding/comparability (presence of control groups and/or statistical adjustment for key covariates); and (4) Follow-up/completeness of data (for longitudinal or interventional studies). Each domain was rated as low risk (), with some concerns (), or high risk (), and an overall qualitative judgment was assigned. Guideline and modeling papers were evaluated for methodological transparency and consistency with current evidence, but were interpreted primarily as contextual rather than primary clinical data. The summary “traffic-light” assessment is presented in Table 3. Overall, the methodological quality of the included studies was heterogeneous (Table 3). Only one clinical trial (Sivertsen et al., 2018) was judged to have an overall low risk of bias, with clearly defined inclusion criteria, standardized clinical outcome measures, and a structured preventive intervention [23]. Most of the remaining clinical and observational studies raised concerns or indicated a high risk of bias, particularly regarding confounding and participant selection. Several studies were based on single- center convenience samples or highly selected patient groups (e.g., children referred for treatment under general anesthesia in Kolçakoğlu et al., 2024, or a single case in Gonnella et al., 2021), which limit generalisability and increase the likelihood of selection bias [37,39]. In terms of outcome measurement, caries and gingival status were often assessed using validated clinical indices, and histological analyses were performed in the study by Raad and Shihab (2023), supporting a relatively low risk of measurement bias in this domain [19]. However, many studies also relied on parent- or self-reported questionnaires to capture oral hygiene behaviors, dental attendance, and perceived oral health (Bsesa et al., 2023; Downing et al., 2022; Dunlop et al., 2013), which are inherently vulnerable to recall and social desirability biases [26,32,34]. Confounding factors such as socioeconomic status, general health, dietary habits, and access to care were seldom fully controlled for; only large survey data (Downing et al., 2022) allowed partial adjustment for sociodemographic variables, whereas smaller clinical studies often lacked multivariable analyses [32,34].

Follow-up and data completeness were mainly affected in interventional and longitudinal studies (Schulz-Weidner et al., 2021; Sivertsen et al., 2018; Kolçakoğlu et al., 2024), where relatively short observation periods and limited information on drop-outs introduce additional uncertainty about the durability of the reported effects [23,35,37]. Guideline and modeling papers (Wilson et al., 2007; Delgado et al., 2023; Al-Karaawi et al., 2001) were generally methodologically transparent and consistent with current evidence [3–4,31]. Taken together, these considerations indicate that the strength of the available evidence is moderate at best and that the findings of this review should be interpreted with caution in light of the underlying risk of bias.

4.1 Burden of oral disease in pediatric cardiac patients

Several studies consistently show that children with cardiac disease carry a higher burden of oral pathology than their healthy peers. In the cross-sectional study by Bsesa et al. (2023), children with congenital heart disease (CHD) exhibited greater caries experience and worse oral health-related quality of life compared with age-matched controls, underscoring the clinical and functional impact of oral disease in this population [26]. Similarly, Erbas Unverdi et al. (2024), in a cohort of children with various systemic diseases, including cardiac conditions, reported higher caries indices and more negative behavior at the first dental visit in medically compromised patients than in healthy controls, again suggesting an increased oral disease burden in those with chronic illnesses [27]. At a population level, Downing et al. (2022) analyzed data from the National Survey of Children’s Health. They found that children with heart conditions were more likely to have parent-reported poor oral health and unmet dental needs than children without heart disease, even after adjustment for sociodemographic factors [34]. This large-scale evidence complements the smaller clinical cohorts and confirms that the oral health gap is not limited to single centers or specific settings. The retrospective study by Kolçakoğlu et al. (2024) provides a more extreme picture of disease burden: children with CHD referred for comprehensive dental treatment under general anesthesia frequently presented with extensive carious involvement, multiple extractions, and complex restorative needs, indicating long-standing untreated disease [37]. From a biological perspective, the histological investigation by Raad and Shihab (2023) demonstrated enamel hypomineralisation and dentinal irregularities in primary teeth from children with CHD, compared with controls [19]. These structural alterations may reflect chronic hypoxia, metabolic disturbances, or medication effects during tooth development and likely contribute to the increased susceptibility to caries observed clinically. Taken together, these five studies indicate that pediatric cardiac patients not only experience more oral disease but often present at more advanced stages, with implications for quality of life, treatment complexity, and potential systemic risk.

4.2 Oral-hygiene behaviors and access to dental care

The same studies also shed light on the behavioral and organizational determinants underlying this excess burden. In Bsesa et al. (2023), parents of children with CHD reported difficulties in maintaining regular oral hygiene and preventive dental visits, often due to the competing demands of cardiac management, frequent medical appointments, and general fatigue [26]. Erbas Unverdi et al. (2024) observed that children with systemic diseases, including cardiac conditions, exhibited more negative behavior during dental treatment than their healthy peers, which may make both families and clinicians more reluctant to pursue regular dental care [27]. Using nationally representative data, Downing et al. (2022) showed that children with heart conditions were less likely to have had a preventive dental visit in the previous year and more likely to have unmet dental needs than children without heart disease [34]. These differences persisted even after adjustment for sociodemographic variables, suggesting that barriers extend beyond socioeconomic status and include health-system factors and disease-specific challenges. The series by Kolçakoğlu et al. (2024) illustrates the downstream consequences of these behavioral and access barriers: many children with CHD reached dental services only when extensive rehabilitation under general anesthesia was required. This pattern implies that opportunities for earlier, less invasive, and more preventive management are frequently missed. Overall, Theme 2 highlights a “double vulnerability”: children with cardiac disease have a greater need for preventive oral care and, at the same time, reduced access to timely and regular dental services [37].

4.3 Preventive and interventional programs

Only a few studies investigated structured preventive or interventional approaches specifically targeting children with CHD. In a longitudinal interdisciplinary program, Schulz-Weidner et al. (2021) implemented tailored oral-hygiene instruction, professional cleaning, and preventive counseling in collaboration with pediatric cardiology. They reported improvements in plaque control, gingival conditions, and parental awareness over time, suggesting that targeted interventions can partially offset baseline disadvantages [35]. The randomized controlled trial by Sivertsen et al. (2018) provides the strongest experimental evidence in this area. Children with congenital heart defects enrolled in an interdisciplinary preventive program showed better oral hygiene and gingival health than those receiving standard care. This study, which was judged to have an overall low risk of bias, demonstrates that structured preventive care can effectively improve short-term oral outcomes in pediatric cardiac patients [23]. The role of prevention is also indirectly emphasized by Kolçakoğlu et al. (2024), where the need for comprehensive treatment under general anesthesia reflects a failure of earlier preventive and restorative strategies. Similarly, the case report by Gonnella et al. (2021), describing the anesthetic management of a child with cardiofaciocutaneous syndrome undergoing dental treatment, highlights the clinical and organizational complexity involved in addressing oral disease late in medically fragile patients. Collectively, these four studies indicate that interdisciplinary preventive programs are feasible and beneficial in the short term. However, the evidence remains limited by small sample sizes, short follow-up periods, and a lack of long-term data on sustained caries reduction, avoidance of extractions, or impact on cardiac outcomes [37,39].

4.4 Infective endocarditis prevention, guidelines, and professional decision-making

Prevention of infective endocarditis represents a major concern in the care of children with CHD and other high-risk cardiac conditions. The influential AHA guideline by Wilson et al. (2007) and the more recent ESC document by Delgado et al. (2023) provide the conceptual framework for IE prevention: antibiotic prophylaxis is reserved for a small subset of very high-risk patients and procedures, while the maintenance of good oral health and the timely management of oral infections are emphasised as fundamental strategies to reduce bacteraemia and IE risk [3–4]. Within this guideline context, Al-Karaawi et al. (2001) developed a theoretical model estimating IE risk associated with dental procedures in children with severe CHD [31]. Their analysis suggests that although the absolute risk is low, it is not negligible, and that decisions on prophylaxis should be individualized, considering both the underlying cardiac condition and the expected bacteremia associated with different dental interventions. This modeling work supports the shift from an “antibiotic-centered” approach towards an “oral-health-centered” paradigm, in which controlling oral inflammation and infection is at least as important as, and often more sustainable than, indiscriminately extending prophylaxis. The survey study by Dunlop et al. (2013) among members of the American Academy of Pediatric Dentistry shows that translation of these principles into daily practice remains challenging. The authors found substantial variability in how pediatric dentists interpret IE prophylaxis recommendations and manage clinical scenarios involving children with heart disease, with some respondents over-prescribing antibiotics and others expressing uncertainty about indications. These findings highlight the need for clear, shared protocols and strong communication between cardiology and dentistry, so that guideline recommendations are applied consistently and appropriately. Overall, Theme 4 underscores that while the theoretical and guideline rationale for prioritizing oral health in IE prevention is robust, its implementation in routine care remains incomplete and heterogeneous and requires closer interdisciplinary alignment [32].

4.5 Methodological considerations and directions for future research

Across all themes, the strength of the evidence is tempered by methodological limitations, as highlighted in the risk-of-bias assessment. Most clinical and observational studies (Bsesa et al., 2023; Erbas Unverdi et al., 2024; Downing et al., 2022; Raad & Shihab, 2023; Kolçakoğlu et al., 2024; Gonnella et al., 2021) are cross-sectional or descriptive, often based on small, single- center, or highly selected samples, with limited control of confounding factors [19,26–27,34,37,39]. Several key variables, such as oral hygiene behaviors and dental attendance, are measured using parent- or self-report questionnaires (Bsesa et al., 2023; Downing et al., 2022; Dunlop et al., 2013), which are inherently prone to recall and social desirability biases [26,32,34]. The interventional and longitudinal studies (Schulz-Weidner et al., 2021; Sivertsen et al., 2018; Kolçakoğlu et al., 2024) involve relatively small sample sizes and short follow-up periods, with incomplete reporting on drop-outs, so the long-term durability of the observed improvements remains uncertain [23,35,37]. Heterogeneity in outcome measures (different caries indices, behavioral scales, and definitions of “poor oral health”) further complicates comparisons and precludes meta-analysis. None of the included studies directly assessed the incidence of IE in relation to oral health or preventive programs, so the presumed systemic benefit of improved oral care remains biologically plausible but not empirically demonstrated. Future research should therefore prioritize multicentre, longitudinal and methodologically robust designs, with standardized, validated outcome measures for caries, gingival health and oral-health behaviors, and, where feasible, evaluation of cardiac outcomes in relation to oral-health trajectories and preventive interventions.

4.6 Clinical implications

Despite these limitations, the twelve included articles converge on a coherent message: oral health must be regarded as a fundamental component of comprehensive cardiac care in children and adolescents [43]. Pediatric cardiac patients have greater biological and clinical need for preventive oral care (Theme 1) but face multiple behavioral and system-level barriers to accessing it (Theme 2). Interdisciplinary preventive programs (Theme 3) and adherence to modern IE prevention guidelines that emphasize oral hygiene over routine prophylaxis (Theme 4) offer realistic avenues to reduce the burden of oral disease and potentially mitigate systemic complications. In practical terms, this implies early and systematic referral of children with cardiac disease to pediatric dental services, integration of basic oral-health screening and counseling into cardiology visits, clear written protocols for dental management and IE prophylaxis, and ongoing education for both families and healthcare professionals. Strengthening these interdisciplinary pathways is essential to translate the existing, albeit imperfect, evidence into meaningful improvements in both oral and cardiovascular outcomes in this vulnerable population.