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CHAPTER 61 ■ Anesthesia for Non-cardiac Surgery in Children With Congenital Heart Disease 983<br />

systolic murmur with maximal intensity at the apex in the left<br />

lateral position has to be considered organic. 12 Innocent functional<br />

cardiac murmurs are frequently heard in almost 50% of healthy<br />

children. 13 In all cases, the distinction between organic and func -<br />

tional murmur should be considered in combination with the<br />

clinical assessment. This should include the following four steps:<br />

(1) count heart rate; (2) assess for the color of the nails, lips, and<br />

skin; (3) appreciate the precordial impulse; and (4) evaluate the<br />

amplitude of the pulses at all extremities. 11 Any anomaly that raises<br />

suspicion should have a focused cardiac evaluation by a cardi -<br />

ologist. A diagnosis of an innocent murmur is supported by<br />

a negative clinical history, normal growth and development, the<br />

lack of symptoms related to cardiac failure, normal pulse and<br />

blood pressure in all four extremities, and normal saturation with<br />

normal chest x-ray and electrocardiogram (ECG).<br />

Preoperative Tests<br />

12-Lead ECG<br />

A 12-lead ECG should always be considered, especially in the<br />

presence of an arrhythmia and/or PHTN. Signs of PHTN on ECG<br />

include the presence of a P-wave amplitude greater than 2.5 mm<br />

in II, III, and aVF, and/or greater than 1.5 mm in V1 (reflecting<br />

right atrial enlargement: P pulmonale), right axis deviation greater<br />

than 110, and a dominant R-wave in V1 (>7 mm). As for the<br />

physical examination, it is important to have a systematic approach<br />

to the ECG (rhythm and conduction) to avoid missing subtle<br />

abnormalities that may have clinical importance.<br />

Chest X-Ray<br />

The chest x-ray may be useful in evaluating the importance of the<br />

cardiomegaly and the pulmonary vascularization. In the presence<br />

of pulmonary vascular disease, children will have cardiomegaly<br />

and peripheral pruning of the vascular tree with a hypertranslucent<br />

appearance in association with dilatation of the hilar and proximal<br />

vessels. Whereas in case of hypoperfusion of the lungs, a homo -<br />

geneous translucent appearance is seen such as in TOF with the<br />

abnormal bootlike shape to the heart.<br />

Cardiac Echocardiogram<br />

Cardiac echocardiogram should be reconsidered if there is no<br />

information available or if the last cardiology visit date was more<br />

than 6 to 9 months earlier. Results can be compared with previous<br />

ones and should be discussed with the cardiologist to better<br />

determine ventricular function and cardiac anatomy.<br />

Laboratory Testing<br />

Laboratory testing is more dependent on the presence or absence<br />

of cyanosis and/or the type of surgery that is scheduled.<br />

In the presence of a cyanosis, a hematocrit over 60% is more<br />

likely to be associated with coagulation abnormalities. These<br />

anomalies are related to the degree of chronic hypoxemia as<br />

well as to the degree of hyperviscosity. 14 The most frequently<br />

observed anomalies include thrombocythemia, platelet dysfunc -<br />

tion, hypofibrino genemia, and a deficit in several clot factors.<br />

Aspirin, heparin, oral anti-vitamin K, and other anticoagulation<br />

therapies, frequently prescribed in these children, may worsen<br />

the clotting.<br />

It is crucial to check the electrolytes especially in children<br />

receiving digoxin and/or diuretics and/or angiotensin-converting<br />

enzyme (ACE) inhibitors.<br />

PREOPERATIVE PREPARATION<br />

Preoperative Medication<br />

In general, no medication should be stopped before surgery,<br />

particularly if the child is using diuretics, ACE inhibitors, or<br />

antiarrhythmic agents. Very often, aspirin is prescribed in children<br />

with systemic to pulmonary arterial shunt and its interruption may<br />

lead to a life-threatening thrombosis, particularly if oxygenation<br />

relies on the shunt.<br />

Children with TOFs are often on propanolol to prevent<br />

infundibular spasm of the right ventricle and the occurrence<br />

of a spell. In case of cardiac failure, optimization of the medical<br />

treatment could be required and digoxin concentration levels<br />

should be adapted to reach concentration between 1 and 1.5 ng/<br />

mL (without exceeding 2 ng/mL). 15 The use of diuretics may lead<br />

to hypochloremia, metabolic alkalosis, and hypokalemia that<br />

can potentiate digoxin toxicity. Thus, preoperative administration<br />

of K + to correct hypokalemia may be necessary and should<br />

not exceed 0.5 mmol/kg/h. Although vasodilators such as ACE<br />

inhibitors are of great value in the treatment of cardiac failure, they<br />

might induce arterial hypotension on induction of anesthesia and<br />

may be stopped just the night before surgery. Finally and in very<br />

rare cases, some children with severe cardiac failure may benefit<br />

from a dobutamine infusion or other inotrope or vasodilator<br />

before anesthesia.<br />

Endocarditis Prophylaxis<br />

The incidence of infectious endocarditis with cardiac disorder<br />

is on the order of 35 times higher than that reported in the<br />

normal children. 16 More than half affect those with CHD, with<br />

33% occurring in children with cyanotic complex cardiopathy. 17<br />

As a consequence, the highest incidence of endocarditis is found<br />

in children who have had definitive or palliative surgery for<br />

cyanotic heart disease. 18<br />

The American Heart Association (AHA) has recently revised<br />

the previous complex guidelines. Prophylaxis is now recommended<br />

only for patients with these four conditions 19 : (1) prosthetic cardiac<br />

valve, (2) previous history of infective endocarditis, (3) certain<br />

types of congenital heart disease (unrepaired cyanotic CHD,<br />

including palliative shunts and conduits; completely repaired<br />

CHD with prosthetic material or device, whether placed by surgery<br />

or by catheter intervention, during the first 6 mo after the<br />

procedure; and repaired CHD with residual defects at the site or<br />

adjacent to the site of a prosthetic patch or prosthetic device [which<br />

inhibit endothelialization]),and (4) cardiac transplantation with<br />

valvulopathy.<br />

In addition, the new guidelines restricted the use of prophylaxis<br />

for dental procedures that involve manipulation of gingival tissue<br />

or the periapical region of teeth or perforation of oral mucosa;<br />

incision or biopsy of respiratory tract mucosa; and procedures on<br />

infected skin or musculoskeletal structures. Gastrointestinal or<br />

genitourinary procedures do not require any prophylaxis, but<br />

if the urine is colonized by enterococcus, eradication before<br />

invasive urinary procedures should be considered. Prophylaxis is<br />

based on the administration of a single dose of amoxicillin, 30 to

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