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  • • A congenital heart lesion that decreases pulmonary arterial blood flow resulting in a right-to-left shunt

    • Cyanosis and decreased oxygen delivery cause compensatory polycythemia (Hct > 70%) and spontaneous thrombosis

    • Exercise, acidosis, pain worsens cyanosis, can cause hypoxic spells

    • Squatting increases systemic resistance, causing increased pulmonary flow and oxygen saturation

    • β-Blockers (decreases spasm), fluid intake, HCO3 administration, norepinephrine (increases systemic resistance) may help decrease hypoxia

    • Clubbing due to proliferation of capillaries and AV fistulas in extremities

    • Bronchial and mediastinal arteries enlarge

    • Ductus arteriosus maintains flow to lungs during fetal development

    • Early administration of alprostadil can allow time for optimization before definitive treatment

    • Operative options to increase pulmonary flow:

    • -Blalock-Taussig shunt: Subclavian artery to ipsilateral pulmonary artery (PA) end to side fashion

      -Modified Blalock-Taussig shunt: Subclavian to PA using PTFE

      -Glenn: Superior vena cava (SVC) to PA shunt

      -Fontan: SVC and inferior vena cava (IVC) rerouted to PA

      -Excision of obstructive muscle, patch enlargement of infundibulum, and valve replacement

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Tricuspid Atresia

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  • • Lack of communication between RA and RV

    • Usually RV small

    • Ventricular septal defect (VSD) common, usually restrictive

    • Connections of great arteries to ventricles abnormal in 30%

    • Degree of obstruction correlates to systemic and pulmonary blood flow

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Ebstein Anomaly

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  • • Septal and posterior leaflets of tricuspid valve small displaced toward RV apex

    • Portion of RV thin and atrialized

    • Associated atrial septal defect (ASD) and patent foramen ovale (PFO)

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Symptoms and Signs

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Tricuspid Atresia

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  • • Symptoms relate to degree of ASD/VSD restriction, great vessels anatomy

    • Clinical presentation can vary

    • Majority have some degree of cyanosis no obstruction to systemic output

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Ebstein Anomaly

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  • • Cyanosis, arrhythmias common

    • 50% develop right heart failure, hypoxia, hepatomegaly, dysrhythmias

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Imaging Findings

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  • Echocardiography: Diagnostic

    Catheterization: Determine suitability for repair and potential correction of ASD

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  • • Echocardiography and catheterization

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  • • Echocardiography and catheterization

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Tricuspid Atresia

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  • • ASD balloon septoplasty: If restrictive

    • Blalock-Taussig often done initially, provide pulmonary flow

    • Glenn followed by Fontan to complete 3 stage repairs

    • Goal: To provide adequate, not excessive pulmonary flow, minimize RV overload

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Ebstein Anomaly

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  • • Oversew atrialized portion of RV, correct tricuspid valve incompetence (successful in 50% of cases)

    • Newer techniques involve correction of RV and tricuspid valve

    • Some cases, irreparable

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References

Dearani JA et al. Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease. Ann Thorac Surg. 2000;69:S106.
Knott-Craig CJ et al. Neonatal repair of Ebstein's anomaly: indications, surgical technique and medium-term follow-up. Ann Thorac Surg. 2000;69:1505.  [PubMed: 10881831]

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