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Key Features

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Essentials of Diagnosis
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  • Mild cyanosis at birth

  • Minimal auscultatory findings

  • Rapid onset of shock with ductal closure

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General Considerations
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  • Hypoplastic left heart syndrome (HLHS)

    • Includes several conditions in which obstructive lesions of the left heart are associated with hypoplasia of the left ventricle

    • Occurs in 1.4–3.8% of infants with congenital heart disease

    • Stenosis or atresia of the mitral and aortic valves is the rule

  • In the neonate, survival depends on a patent ductus arteriosus (PDA) because antegrade flow into the systemic circulation is inadequate or nonexistent

  • The PDA provides the only flow to the aorta and coronary arteries

  • Prenatal diagnosis aids in counseling for the expectant parents and planning for the delivery of the infant at or near a center with experience in treating HLHS

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Clinical Findings

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  • Neonates appear stable at birth because the ductus is patent

  • Deterioration occurs rapidly as the ductus closes, with shock and acidosis secondary to inadequate systemic perfusion

  • Oxygen saturation may actually increase for a period of time as the ductus closes due to increased blood flowing to the lungs

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Diagnosis

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Imaging
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  • Chest radiography

    • May be relatively unremarkable on first day of life, with the exception of a small cardiac silhouette

    • Later, demonstrates cardiac enlargement with severe pulmonary venous congestion if the PDA has begun closing or if the baby has been placed on supplemental oxygen increasing pulmonary blood flow

  • Echocardiography

    • Diagnostic

    • Color-flow Doppler imaging shows retrograde flow in the ascending aorta

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Diagnostic Procedures
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  • Electrocardiography

    • Shows right axis deviation, right atrial enlargement, and right ventricular hypertrophy with a relative paucity of left ventricular forces

    • Small Q wave in lead V6 may be absent

    • qR pattern is often seen in lead V1

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Treatment

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Medical
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  • Initiation of PGE1 is essential and lifesaving

  • Later management depends on balancing pulmonary and systemic blood flow

  • Supplemental oxygen is avoided because it decreases pulmonary resistance and leads to further increases in pulmonary blood flow

  • Nitrogen is used in some centers to decrease inspired oxygen to as low as 17%

    • Results in increased pulmonary arterial resistance, which encourages systemic blood flow and improves systemic perfusion

    • Systemic afterload reduction also increases systemic perfusion

    • Patients must be monitored carefully

  • Adequate perfusion can usually be obtained by keeping systemic O2 saturation between 65% and 80%, or more accurately a PO2 of 40 mm Hg

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Surgical
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  • Staged surgical palliation is the most common management approach

  • Heart transplantation indications

    • Failed surgical palliation

    • Systemic RV fails (often in adolescence or young adulthood)

  • Some centers offer a "hybrid" approach, in which surgeons and interventional cardiologists collaborate

    • Chest is opened surgically and the branch pulmonary arteries are banded, to limit pulmonary blood ...

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