Tetralogy of Fallot and Absence of Left Pulmonary Artery

Introduction

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital lesion with a diverse clinical presentation [1]. More frequently, UAPA is associated with severe cardiovascular anomalies such as tetralogy of Fallot (TOF) or septal defects. There are few reports in the literature describing such a dilemma; however, there is still a lack of consensus on the best diagnostic and therapeutic approach for these patients [2].

Here, we report on a case of TOF accompanied by an absent left pulmonary artery and further complicated by major aortopulmonary collateral arteries (MAPCA).

References

• 1 Welch K, Hanley F, Johnston T, Cailes C, Shah M J. Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up.  Ann Thorac Surg. 2005;  79 1399-1402
• 2 Ten Harkel A D, Blom N A, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature.  Chest. 2002;  122 1471-1477
• 3 Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood.  Chest. 1995;  108 670-676
• 4 Apostolopoulou S, Kelekis N L, Brountzos E N, Rammos S, Kelekis D A. “Absent” pulmonary artery in one adult and five pediatric patients: imaging, embryology, and therapeutic implications.  AJR Am J Roentgenol. 2002;  179 1253-1260
• 5 Ergun P, Erdogan Y, Turkkani M, Gokcek A. Isolated unilateral absence of a aulmonary artery: an unusual cause of recurrent pulmonary infection.  Archives of Pulmonary. 2003;  4 221-224
• 6 Raja S G, Danton M D, MacArthur K J, Pollock J C. Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects.  J Cardiothorac Vasc Anesth. 2007;  21 203-207
• 7 Brancaccio G, Toscano A, Bevilacqua M, Di Chiara L, Parisi F. Bosentan and sildenafil: should the combination therapy be a valid alternative in childhood to prostacyclin infusion?.  Pediatr Transplant. 2007;  11 110-112
• 8 Taugchi T, Ikeda K, Kume K, Nagasaki A, Goto S, Matsuo S, Hasuo K, Tamura S. Isolated unilateral absence of left pulmonary artery with peribronchial arteriovenous malformation showing recurrent hemoptysis.  Pediatr Radiol. 1987;  17 316-318
• 9 Reddy V M, McElhinney D B, Amin Z, Moore P, Parry A J, Teitel D F, Hanley F L. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients.  Circulation. 2000;  101 1826-1832