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Unilateral absence of left pulmonary artery with absent pulmonary valve syndrome
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  • Sachin Mahajan,
  • Sudhansoo Khanna,
  • Sanjeev Naganur,
  • JAVID RAJA
Sachin Mahajan
Post Graduate Institute of Medical Education and Research
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Sudhansoo Khanna
Post Graduate Institute of Medical Education and Research
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Sanjeev Naganur
Post Graduate Institute of Medical Education and Research
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JAVID RAJA
Post Graduate Institute of Medical Education and Research
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Abstract

Tetralogy Of Fallot (TOF) with absent pulmonary valve (APV) and unilateral absence of pulmonary artery (UAPA) is an extremely rare congenital cardiac anomaly. “Complete” unilateral absence implies absence of both intrapericardial and hilar segments. Co- existence of both free pulmonary regurgitation (PR) and decrease in the cross sectional area for right ventricular outflow tract (RVOT) in this substrate, are detrimental for right ventricular (RV) function. Early intervention to preserve RV function is imperative. Durable and competent valve in RVOT can be extremely helpful in improving the overall prognosis. We present a case of a two year old child, TOF with APV, absent left pulmonary artery (ALPA) who underwent RVOT reconstruction, with a pulmonary valve (PV) made from polytetrafluorethylene (PTFE) membrane. To our knowledge construction of PV using Graham Nunn technique, has not been reported with this morphology.