Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum

Abstract

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one such case which was diagnosed prenatally to have tetralogy of Fallot with absent pulmonary valve and identified postnatally to have nonconfluent pulmonary artery branches in addition. We discuss the conundrum of respiratory management in this patient pre- and postoperatively due to a unique ventilation perfusion mismatch problem, which varies between the two lungs.

Keywords

tetralogy of Fallot absent pulmonary valve syndrome patent ductus arteriosus nonconfluent pulmonary arteries

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References

Rao

Anderson

Edwards

. Anatomic variations in the tetralogy of Fallot. Am Heart J. 1971;81(3):361–371.

Arsan

Akalin

Gursu

Isbir

. Autologous right pulmonary artery tissue for repair of left pulmonary artery originating from left patent ductus arteriosus in a patient with tetralogy of Fallot and absent pulmonary valve. Pediatr Cardiol. 2011;32(7):986–989.

Cho

Park

Lee

. A variant of tetralogy of Fallot with absent pulmonary valve: left pulmonary artery originate from left patent ductus arteriosus. Pediatr Cardiol. 2010;31(1):153–154.

Zucker

Rozin

Levitas

Zalzstein

. Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome. Cardiol Young. 2004;14(4):402–408.

Fischer

Neches

Beerman

, et al. Tetralogy of Fallot with absent pulmonic valve: analysis of 17 patients. Am J Cardiol. 1984;53(10):1433–1437.

Norgaard

Alphonso

Newcomb

Brizard

Cochrane

. Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up. Eur J Cardiothorac Surg. 2006;29(5):682–687.

Podzimkova

Hickey

Slavik

Leanage

Chan

. Absent pulmonary valve syndrome with intact ventricular septum: role of ductus arteriosus revisited. Int J Cardiol. 1997;61(2):109–112.

Qureshi

Burkhart

Julsrud

Cetta

. Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome. Tex Heart Inst J. 2014;41(6):664–667.

Solana-Gracia

Crossland

Mitchell

Wren

Griselli

. Tetralogy of Fallot with absent pulmonary valve syndrome, right aortic arch, and disconnected left pulmonary artery. World J Pediatr Congenit Heart Surg. 2013;4(2):206–209.

10.

Saygi

Kilinc

Irdem

Ozturk

Guzeltas

. Combination of tetralogy of Fallot with absent pulmonary valve and left pulmonary artery originating from patent ductus arteriosus: a rare association in an infant. Anatol J Cardiol. 2015;15(5):E15–16.

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