Surgical Treatment of Pediatric Hypoplastic Left Heart Syndrome Workup

Updated: Dec 20, 2020
  • Author: Ming-Sing Si, MD; Chief Editor: Stuart Berger, MD  more...
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Laboratory Studies

Routine laboratory studies, such as CBC count, platelets, electrolytes, BUN levels, creatinine levels, liver function tests, and coagulation studies, are indicated. In addition to ensuring that laboratory test values return to reference ranges, maintain the hematocrit level at 45% or more if the patient is cyanotic.

Routine serial monitoring of ABGs is important in balancing the relative pulmonary and systemic blood flows in patients with single ventricle physiology (see Medical therapy, Surgical therapy).


Imaging Studies

Chest radiographs reveal cardiomegaly and increased pulmonary vascular markings. In 2% of patients, a reticular pattern of obstructed pulmonary venous return is seen because of a restrictive atrial septal defect.

Diagnosis is made using 2-dimensional and color Doppler echocardiography for determination of cardiac morphology and evaluation of the arch hypoplasia. Color-flow Doppler images reveal that the blood flow in the ascending aorta is typically retrograde.


Other Tests

Electrocardiography reveals right atrial enlargement and right ventricular hypertrophy.


Diagnostic Procedures

Cardiac catheterization is rarely necessary. An exception would be to gain additional information in patients with borderline left ventricle size to assist in the decision-making process regarding the optimal treatment method.