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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 causes 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

    • 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

    • Alprostadil early 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

    • Pulmonary valve replaced by diaphragm of tissue, causing obstruction

    • PA normal size

    • Atrial septal defect (ASD) or patent ductus arteriosus (PDA) necessary for survival after first few hours of birth

    • RV and tricuspid annulus typically small

    • RV: Coronary artery fistulas and coronary artery stenoses are common

    • Some coronary flow dependent on increased pressures in RV

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Symptoms and Signs

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  • • Cyanosis present at birth

    • Duct closure: Profound hypoxia, acidosis

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Laboratory Findings

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  • • ECG: Lack of normal RV dominance

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Imaging Findings

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  • Chest film: Diminished flow into lungs

    Echocardiography: Diagnostic

    Catheterization: Define coronary anatomy, RV-dependent coronary circulation

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  • • Echocardiography, catheterization needed

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  • • Chest x-ray

    • ECG

    • Echocardiography

    • Catheterization

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  • • ASD creation or ductus maintenance (alprostadil) necessary for early survival

    • Surgical treatment depends on RV development and right heart dependent coronary circulation

    • Systemic-PA shunt + patch reconstruction of right heart outlow tract (not performed if poor RV development or right-dependent coronary circulation)

    • With further ventricle development, may close shunt and ASD

    • If ventricles do not develop, consider Fontan

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Surgery

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Indications

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  • • Indicated if diagnosed

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Prognosis

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  • • 1-year mortality, 10-20%

    • Prognosis improving

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References

Earing MG et al: Long-term follow-up of patients after surgical treatment for isolated pulmonary stenosis. Mayo Clin Proc 2005;80:871.  [PubMed: 16007892]
Peterson C et al: Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children. Ann Thorac Surg 2003;76:1078.  [PubMed: 14529989]
Poon LK, Menahem S: Pulmonary regurgitation after percutaneous balloon valvoplasty for isolated pulmonary valvar stenosis in childhood. Cardiol Young 2003;13:444.  [PubMed: 14694939]

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