Tricuspid Atresia

• • 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

Tricuspid Atresia

• • 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

Ebstein Anomaly

• • 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)

Symptoms and Signs

Tricuspid Atresia

• • 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

Ebstein Anomaly

• • Cyanosis, arrhythmias common

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

Imaging Findings

• • Echocardiography: Diagnostic

  • Catheterization: Determine suitability for repair and potential correction of ASD

• • Echocardiography and catheterization

• • Echocardiography and catheterization

Tricuspid Atresia

• • 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

Ebstein Anomaly

• • 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

References