What should be evaluated in an echocardiogram (echo) for a child with Down syndrome undergoing orchidopexy?

Preoperative Echocardiographic Evaluation for Down Syndrome Children Undergoing Orchidopexy

All children with Down syndrome require a comprehensive echocardiogram before orchidopexy surgery, regardless of normal cardiac physical examination findings, because physical examination alone misses significant cardiac defects in approximately 20% of cases. 1, 2

Structural Cardiac Anomalies to Assess

Atrioventricular Septal Defects (AVSD)

• AVSD is the most common cardiac anomaly in Down syndrome, present in 33-44% of cases with congenital heart disease, and complete AVSD carries higher perioperative mortality (13% vs 5%) requiring specific anesthetic considerations. 1
• Evaluate for both complete and partial AVSD, assessing the degree of valve regurgitation and shunt flow. 3, 1
• Document tricuspid and mitral valve dysplasia commonly associated with AVSD. 1

Ventricular Septal Defects (VSD)

• Assess VSD size, location, and hemodynamic significance, as moderate to large VSDs affect anesthetic management and perioperative risk. 1
• Use Doppler to quantify shunt flow and determine direction of shunting across the defect. 3, 1

Tetralogy of Fallot

• Present in approximately 11-13% of Down syndrome children with cardiac disease. 1
• Evaluate the degree of right ventricular outflow obstruction, VSD size, aortic override, and right ventricular hypertrophy. 1

Patent Ductus Arteriosus (PDA)

• Specifically assess for persistence beyond 7 days of age, as this represents pathologic rather than physiologic shunting. 1
• Document patency, direction and degree of shunting at the ductal level using Doppler. 3, 1

Functional and Hemodynamic Assessment

Pulmonary Hypertension Evaluation

• Children with Down syndrome develop pulmonary vascular disease earlier and more rapidly than other children, and pulmonary hypertension represents the highest perioperative risk, potentially contraindicating elective surgery until adequately treated. 3, 1
• Use Doppler echocardiography to estimate pulmonary artery pressures and assess for elevated pulmonary vascular resistance. 3, 1
• Evaluate for right-to-left shunting patterns that indicate elevated pulmonary pressures. 3

Ventricular Function

• Assess both left and right ventricular systolic and diastolic function, as poor ventricular function significantly impacts perioperative risk. 1
• Document ventricular compensation for volume overload from shunts or valvular disease. 3

Valvular Assessment

• Evaluate all valve configurations and assess severity of any regurgitant lesions. 3, 1
• Document chamber dilation secondary to valvular disease. 1

Additional Critical Elements

Chamber Size and Cardiomegaly

• Document chamber size and dilation, as cardiomegaly may indicate volume overload from shunts or valvular disease. 3, 1

Pericardial and Intracardiac Thrombus

• Rule out pericardial effusion, as pericardial disease could complicate anesthesia. 3, 1
• Assess for thrombus formation, particularly important in children with cyanotic lesions or previous cardiac interventions. 1

Coronary Artery Assessment

• Evaluate origin, size, and flow in coronary arteries, especially if there is history of Kawasaki disease or other inflammatory conditions. 3

Common Pitfalls to Avoid

• Do not rely on physical examination alone: Physical examination has only 80% sensitivity and normal findings do not exclude significant cardiac disease requiring surgical consideration. 1, 2
• Do not delay echocardiography: Even asymptomatic children require evaluation, as 15-20% with normal physical examinations have abnormal echocardiograms, with some requiring eventual surgery. 2
• Do not underestimate pulmonary hypertension risk: Children with Down syndrome are particularly susceptible to pulmonary hypertension crises during anesthesia, making this assessment critical for anesthetic planning. 1