Pediatric and young adult patients with congenital heart disease (CHD) represent a population with unique medical issues, who pose a challenge to the primary care physician (PCP). Advances in pediatric cardiology and cardiac surgery have resulted in an increasing number of survivors with CHD.1 As a result, PCPs are now more likely to encounter patients with varying severity of CHD in their daily practice. In this chapter, we aim to discuss some common pediatric issues that may be encountered while taking care of this subset of patients.
Oral health is an integral part of general health, and according to the Centers for Disease Control and Prevention, dental caries are the most prevalent infectious disease among U.S. children.2 More than 40% of children have tooth decay by the time they reach kindergarten, and more than 52 million hours of school are lost each year because of dental problems.2,3 Oral disease poses a significant burden in patients with CHD, not only by increasing the risk of acute and subacute endocarditis, but also by exposing these children to the risk associated with oral procedures. The morbidity and mortality of endocarditis is significant.4,5 One important objective of the PCP caring for patients with CHD is to guide families to prevent oral pathology.4,5 Oral disease increases morbidity and mortality in children with CHD by different mechanisms. The mouth is a portal of entry for microbial infection. Bacteremia may cause injury by directly damaging the epithelium and indirectly by generating an inflammatory response. Through these mechanisms and potentially others, periodontitis may further increase the known risk of endocarditis. More recently, an association between periodontitis and increasing atherogenesis and thromboembolic phenomena has been described in adults.4 In more severe cases, oral disease may also compromise nutrition, which in turn may have a deleterious effect on the course of the CHD.
Children with CHD have variable cardiovascular reserve; those with more tenuous hemodynamics are at higher risk of complications during dental intervention. For example, some local anesthetics with vasoconstrictors like epinephrine may be contraindicated in patients with refractory dysrhythmias. Some patients with CHD, such as patients with prosthetic valves or implanted devices, may require anticoagulation with agents like warfarin or aspirin. Stopping these agents to prevent severe bleeding may be appropriate during the much needed dental procedure but may increase the risk of valve or intracardiac thrombosis. Alternative strategies such as transition to heparin or enoxaparin and careful control of the coagulation profile before surgical intervention may be indicated. Consultation with hematology and coagulation experts may be essential in some of the complex cases. Stress and pain secondary to the dental procedure may generate catecholamine release enough to compromise hemodynamic stability. In the attempt to limit stress and pain in this vulnerable population, the clinician may have a lower threshold to use sedation; this poses other risks, some of which we will discuss latter in this chapter.4,5 Keeping these thoughts in mind, the PCP should target the higher risk populations to emphasize the importance of preventive care and oral health maintenance.
An oral health risk assessment should be administered periodically, and PCPs should apply the guidelines set by the American Academy of Pediatrics (AAP) regarding dental health.2 Among these are dietary counseling for optimal oral health and administration of fluoride based on an individual’s caries risk. The AAP has also introduced the concept of establishing a dental home by the first year of age. The term “dental home” alludes to a specialized primary dental care service expected to provide the following: (1) an accurate risk assessment for dental diseases and conditions; (2) an individualized preventive dental health program based on the risk assessment; (3) anticipatory guidance about growth and development issues (eg, teething, digit or pacifier habits, feeding practices); (4) a plan for emergency dental trauma and information about proper care of the child’s teeth and gingival tissues; (5) information regarding proper nutrition and dietary practices, comprehensive dental care in accordance with accepted guidelines, and periodicity schedules for pediatric dental health; and (6) referrals to other dental specialists, such as endodontists, oral surgeons, orthodontists, and periodontists. Parents sometimes encounter significant difficulties accessing pediatric dental services. The PCP plays a crucial role in helping these families establish a “dental home.”1,3,6
Once periodontal disease is present, interventions must be carefully planned to minimize risk. A significant concern is the risk of endocarditis. The efficacy of antibiotic prophylaxis as a preventive measure is debatable.5 Newer strategies for better oral hygiene for the future include photodynamic therapy in which the inactivation of microorganisms is based on localization of a photosensitizer in bacteria. The photosensitizer, activated by low doses of visible light, reacts by generating free radicals that are toxic to bacteria.7,8
Prevention of infective endocarditis (IE) by appropriate administration of antibiotics prior to procedures known to cause bacteremia has been a subject of interest for decades. The initial recommendations regarding antibiotic prophylaxis for IE were made by the American Heart Association (AHA) in 1955.5 Since then, 9 iterations of AHA recommendations for antibiotic prophylaxis have been made. The last statement was published in 20075 and was a revision of the statement that had been in place since 1997. The fundamental principles that the IE antibiotic prophylaxis guidelines were based on are as follows:
IE is uncommon, but it is associated with significant morbidity and mortality. Prevention is preferable.
Certain underlying cardiac conditions place the patient at increased risk for developing IE.
Bacteremia with certain organisms (such as streptococci, staphylococci, and enterococci) is more commonly associated with IE.
Certain invasive procedures, including dental, respiratory, gastrointestinal, and genitourinary (GU) tract procedures, have been associated with transient bacteremia.
Antimicrobial prophylaxis has proven to be effective for prevention of experimental IE in animals.5,9,10
Keeping these premises in mind and making the assumption that, in humans, antimicrobial prophylaxis may also be effective for prevention of IE associated with dental, respiratory, gastrointestinal, or GU tract procedures, antibiotic prophylaxis guidelines have been set forth.7 Over the years, researchers have questioned the premises on which IE prophylaxis guidelines were based. The AHA appointed a multidisciplinary group, including members of the American Dental Association, the Infectious Disease Society of America, and the AAP, to regularly revise the recommendations as new literature was published.5,9,10 The last IE prophylaxis guidelines published in 2007 reflect expert opinion based on relevant analysis of the literature published between 1950 and 2007 regarding (1) procedure-related bacteremia and IE; (2) in vitro susceptibility data of the most common microorganisms that cause IE; (3) results of prophylactic studies in animal models of experimental endocarditis; and (4) retrospective and prospective studies of prevention of IE. Most of the literature available was related to dental interventions.
The absolute risk for IE from dental procedures is impossible to measure precisely. Still, based on existing literature, it is estimated that if dental treatment causes 1% of all cases of Viridans streptococci IE annually in the United States, then the overall risk, in the general population, may be as low as 1 case of IE per 14 million.5
Estimates of absolute risk of IE from dental procedures in patients with heart disease indicate that IE is a very rare complication. It was estimated that in patients with mitral valve prolapse, the risk of IE was 1 in 1.1 million procedures; in patients with CHD, the risk was 1 in 475,000 procedures; and in patients with rheumatic heart disease, the risk was 1 in 142,000 procedures. The 2 populations with the highest estimated absolute risk were patients with prosthetic cardiac valves (absolute risk of 1 in 114,000 dental procedures) and patients with previous IE (risk of 1 in 95,000 dental procedures).5
The efficiency of antibiotic prophylaxis in reducing bacteremia is questionable. Although it has been shown that dental procedures are associated with bacteremia, transient bacteremia is also common during daily activities like flossing and tooth brushing (20%-68%), use of wooden tooth picks (20%-40%), use of water irrigation devices (7%-50%), and chewing food (7%-51%). Because these activities are far more common causes of bacteremia than dental visits, it makes the premise of antibiotic prophylaxis for dental procedures questionable and highlights the importance of maintenance of good oral health in the prevention of IE.5
The antibiotic prophylaxis guidelines for IE from 2007 express the consensus of expert opinion on available literature and are designed to be interpreted based on class and level of evidence (LOE) (Table 16-1). When selecting the patient population eligible for IE prophylaxis, the 2007 recommendations shifted their focus to include a specific subset of patients with complex heart disease who were more likely to have the risk of increased mortality or long-term morbidity from IE (Table 16-2). This emphasis is in contrast to the 1997 recommendations that gave greater importance to increased risk of overall acquisition of IE in all complex and noncomplex CHD.
| Class of Recommendation | Interpretation |
|---|---|
| I: Benefit >>>> risk | Should be performed/administered |
| IIa: Benefit >> risk | Reasonable to perform/administer |
| IIb: Benefit ≥ risk | May be considered to be performed/administered |
| III: Risk ≥ benefit | Should not be performed/administered |
| Level of Evidence | Recommendation Based on: |
| A: Multiple populations evaluated | Randomized clinical trials and meta-analysis |
| B: Limited populations evaluated | Single randomized trial and non-randomized studies |
| C: Very limited populations evaluated | Expert opinion/consensus, case studies |
|
Antibiotic regimens for IE prophylaxis are mainly targeted to patients undergoing dental procedures (Table 16-3). However, these recommendations may also be applied to patients undergoing other procedures (such as GU interventions) when prophylaxis is indicated. In these cases, the choice of antibiotics may vary, and certain rules apply. The recommendation is that antibiotics should be administered in a single dose before the procedure. If this window is missed, antibiotics may still be given within 2 hours after the procedure. If the patient is febrile prior to the procedure, blood cultures should be considered. IE related to transient bacteremia, secondary to daily activities, such as flossing and tooth brushing, needs to be ruled out.
| Regimen: Single Dose 30-60 Minutes Before Procedure | |||||
|---|---|---|---|---|---|
| Situation | Agent | Adults | Children | ||
| Oral | Amoxicillin | 2 g | 50 mg/kg | ||
| Unable to take oral medication | Ampicillin OR Cefazolin or ceftriaxone | 2 g IM or IV 1 g IM or IV | 50 mg/kg IM or IV 50 mg/kg IM or IV | ||
| Allergic to β-lactams; able to take oral medication | Cephalexina,b OR Clindamycin OR Azithromycin or clarithromycin | 2 g 600 mg 500 mg | 50 mg/kg 20 mg/kg 15 mg/kg | ||
| Allergic to β-lactams or unable to take oral medication | Cefazolin or ceftriaxone† or Clindamycin | 1 g IM or IV 600 mg IM or IV | 50 mg/kg IM or IV 20 mg/kg IM or IV | ||
Transient Viridans streptococcal bacteremia is the main concern during invasive dental procedures. Interventions for which IE prophylaxis is considered reasonable include all dental procedures that involve manipulation of gingival tissue or periapical region of teeth or perforation of the oral mucosa. Antibiotic prophylaxis is not recommended for mucosal bleeding associated with local anesthesia through noninfected tissue, dental radiographs, placement or removal of orthodontic appliances, tooth eruptions, or incidental trauma (class IIa, LOE C).
Strains of Viridans streptococci resistant to penicillin have increased significantly. Antibiotics such as vancomycin and fluoroquinolones are not recommended due to concerns of excessive use, increasing resistance, and a low risk–benefit ratio. The antibiotics of choice for prophylaxis for dental procedures are amoxicillin or cephalosporins and macrolides in special situations when antibiotic allergies are encountered (Table 16-3).5
IE antibiotic prophylaxis may be reasonable for respiratory tract procedures in which incision or biopsy of respiratory mucosa is made, for example tonsillectomy and adenoidectomy (class IIa, LOE C). Procedures like bronchoscopy, where there is no disruption of the respiratory mucosa expected, do not require antibiotic prophylaxis. Regimens detailed in Table 16-3 may be applied if underlying bacterial infection is suspected or confirmed; if organisms resistant to regimens detailed in Table 16-3 are suspected, alternative regimens should be used.5
Antibiotics may be indicated in patients undergoing elective GU procedures, with the aim of eradicating enterococci in colonized patients or in patients with enterococci urinary tract infection (class IIb, LOE B). If the procedure is not elective, it may be reasonable to give antibiotic prophylaxis, including therapeutic antibiotic coverage for enterococci (class IIb, LOE B). Ampicillin and amoxicillin are the agents of choice. If the there is a contraindication to a β-lactam antibiotic, vancomycin can be used. Expert consultation should be obtained when prophylaxis is against multiresistant organisms.5
Antibiotic prophylaxis with a penicillin or a cephalosporin may be considered for procedures on infected skin or musculoskeletal tissue. Under these circumstances, the choice of antibiotics should include coverage against Staphylococcus and β-hemolytic Streptococcus (class IIb, LOE C). Vancomycin and clindamycin can be used as alternatives depending on bacterial resistance patterns and patient allergies.5
Ideally, dental treatment should be completed before cardiac surgery because the absence of infective oral disease is preferred. Patients undergoing placement of prosthetic valves, intracardiac or intravascular prosthetic material, and transvenous pacemakers are candidates for perioperative antibiotic prophylaxis (class I, LOE B). Prophylaxis prior to surgery should be primarily targeted at Staphylococcus aureus species. Antibiotic choice should be based on the hospital’s specific susceptibility patterns. Nonetheless, a first-generation cephalosporin is a class I, LOE A recommendation. If methicillin- resistant S aureus or Staphylococcus epidermidis species are highly prevalent, vancomycin may be considered (class IIb, LOE C).5
Patients with CHD may have other comorbidities that merit special considerations:
Patients receiving long-term antibiotics for indications other than IE: Some patients may already be on daily antibiotics for issues such as rheumatic fever prophylaxis or urinary tract prophylaxis or because they are immunocompromised. If patients are undergoing a procedure for which prophylaxis is recommended, the suggestion of the AHA is to use an antibiotic from a different class instead of increasing the dose.5,9
Patients taking anticoagulation: Intramuscular injections as a mode of administering antibiotic prophylaxis should be avoided because of risk of hematoma (class I, LOE A). Oral administration is the preferred option. In patients who do not tolerate the enteric route, intravenous is the preferred alternative.5
One of the significant changes in the 2007 AHA IE prophylaxis recommendations is that antibiotic prophylaxis is no longer recommended for patients undergoing gastrointestinal procedures (class III, LOE B). This change is based on new literature that indicates that normal gut flora, including enterococci, and polymicrobial intra-abdominal infections are uncommon IE pathogens. The literature associating enterococci bacteremia to gastrointestinal procedures is scant, and reported cases are anecdotal. Alternatively, antibiotics may be recommended if a patient with an active gastrointestinal infection is undergoing a procedure. In this case, targeted antibiotic therapy should be considered, and coverage for enterococci is reasonable (class IIb, LOE B).5,9
Education about pregnancy and contraception should be an integral part of the healthcare plan in the patient with CHD. Increasing survival in this patient population has resulted in more females with CHD reaching, and surviving beyond, childbearing years. Recent studies have shown that there needs to be a better effort in improving pregnancy and contraception counseling. As Vigil et al.11 described in their study, which included 536 females with CHD, up to 43% of women reported they received no counseling regarding contraception. Forty- eight percent of women claimed they had not been informed of pregnancy-related risks before being seen by a specialist.11 In addition, up to 45% of subjects in another study reported using or having used contraception methods that were contraindicated because of their cardiac diagnosis.12 It is the responsibility of the healthcare team to work as an interdisciplinary group in educating this growing population regarding their fertility and the risks of pregnancy. The importance of education is specifically recognized in preventing unplanned pregnancy in females with more severe forms of CHD. In these patients, an unplanned pregnancy could lead to severe health consequences, including death, for both the mother and the fetus.
Counseling adolescents with CHD regarding pregnancy and contraception should begin early. This population is heterogeneous and includes females with simple cardiac lesions who can be counseled as healthy adolescents and women with more severe lesions who need individualized consideration. In counseling, the healthcare provider must take into account the risk of pregnancy to the patient and the unborn child, specific contraindications intrinsic to each contraception method and medications used by the patients, and the patient’s capabilities and preferences.12–14
Pregnancy imposes prohibitively high mortality to both mother and fetus in patients with Eisenmenger syndrome. In these patients, methods of contraception with the lowest failure rate need be considered. Other patients at high risk for complications during pregnancy include women with mechanical valves, Fontan circulation, or significant myocardial dysfunction. In these patients, contraception methods with low failure rates should also be recommended.12
The American College of Cardiology/AHA guidelines for adults with CHD emphasize the importance of these women being counseled regarding the genetics of their heart disease, the risk of offspring being affected, risks of fetal demise, prematurity, and maternal morbidity and mortality. All medications the patient is taking need to be reviewed, and the teratogenic potential for the fetus needs to be considered.13
There are limited data on the safety of contraception methods in patients with CHD, so information is extrapolated from women with no CHD. The general categories of contraceptives and their risks are listed in Table 16-4.
| Contraceptive Agent | Risks and Advantages |
|---|---|
| Combined hormonal contraception (estrogens and progestins) | ↑ risk of thromboembolism ↑ LDL and triglyceride, ↓ HDL cholesterol ↑ glucose intolerance ↑ risk for hypertension |
| Progesterone-only formulations | No thrombosis risk |
Failure rate high (5%-10%) Fluid retention | |
| Intrauterine device (IUD) | +/− ↑ risk of infection (IE) |
| Barrier methods | +/− efficacy is user dependent No specific contraindications |
| Sterilization | Most effective for high-risk patients |
Permanent Involves a surgical intervention, anesthesia |
Estrogen-based formulations have been shown to increase risk of thromboembolism, have a negative impact on lipid profile (decreasing high-density lipoproteins and increasing low-density lipoproteins and triglycerides), increase the risk of glucose intolerance, and increase the risk of hypertension. Failure rate during the first year is 3% to 8%.12 The 2008 American College of Cardiology/AHA guidelines for adults with CHD recommend that estrogen-containing oral contraceptive pills not be prescribed to patients at risk for thromboembolism (eg, patients who have undergone Fontan palliation), patients with significant cyanosis, patients with atrial fibrillation, and patients with pulmonary hypertension.13 Although not included in the 2008 American College of Cardiology guidelines, patients who require anticoagulation are not ideal candidates for estrogen-based pills. This group includes patients just mentioned and patients with mechanical valves, due to the increased risk related to the known interaction of estrogen with warfarin.12
Medroxyprogesterone and progesterone-only pills are an alternative to combined hormonal contraception. They have less effect on blood pressure, are not contraindicated in smokers, and do not increase the risk of thrombosis. Their most significant drawbacks are the high failure rates (5%-10% in the first year) and their association with increased fluid retention, which may compromise hemodynamic stability,12,13 as in patients with Eisenmenger syndrome, other causes of pulmonary hypertension, severe cardiomyopathy, Marfan syndrome with dilated aorta, or severe aortic stenosis. If the decision is still to use progesterone-based contraception in these specific cardiac conditions, forms of slow-release contraception are preferred (intramuscular injections) because they are more reliable.12 Per the AHA guidelines, levonorgestrel (Plan B), may be used in women with cyanotic CHD and with pulmonary hypertension.13
The use of intrauterine devices should be individualized for patients with CHD. The suggested increased risk for endocarditis is controversial, so risk–benefit ratios should be considered based on the individual patient.13 Intrauterine devices are not recommended in patients with Fontan circulation because they are at risk for severe vagal response during device placement.12
Condoms, spermicidal products, and diaphragms are recommended as adjunctive methods. They have no specific contraindications, but efficiency and compliance are highly user dependent.
In summary, it is mandatory that PCPs, cardiologists, and gynecologists caring for patients with CHD become proficient in educating this growing heterogeneous population of young adults regarding their fertility, pregnancy risk, and choice of contraception. We must strive to give these women the choice of making informed decisions regarding their reproductive years.
The Advisory Committee on Immunization Practices annually publishes an immunization schedule for persons age 0 through 18 years that summarizes recommendations for currently licensed vaccines. Healthcare providers are referred to the Centers for Disease Control and Prevention website15 to review the most recent guidelines. Patients with CHD are subject to the same vaccination guidelines, with some extra requirements that are discussed in the following sections.
Respiratory syncytial virus (RSV) is considered a significant cause of morbidity and mortality in some patients with CHD.16,17 Candidates for RSV prophylaxis include children younger than 24 months with hemodynamically significant acyanotic congenital cardiac lesions and cyanotic heart disease. The AAP has come to the consensus that the patients most likely to benefit from RVS prophylaxis are infants younger than 12 months of age who are receiving medication to control congestive heart failure, infants with moderate to severe pulmonary hypertension, and infants with cyanotic heart disease.16,17
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