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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....

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