Abstract
Tetralogy of Fallot (TOF) with unilateral absence of pulmonary artery and the anomalous coronary artery is a rare combination. Detailed preoperative evaluation of coronary artery anatomy is must to prevent injury to the major vessels crossing right ventricular outflow tract. We report a rare association of single coronary artery with left circumflex artery crossing right ventricular outflow tract close to the pulmonary annulus in tetralogy of Fallot with absent left pulmonary artery in 11-year-old girl. Though there is a great diversity of coronary anomalies in tetralogy of Fallot, the prepulmonic course of left circumflex artery crossing the right ventricular outflow tract (RVOT) close to the pulmonary annulus has rarely been described in the literature. The patient underwent successful primary single lung intracardiac repair. Right ventricular outflow tract obstruction was treated by handmade valved pericardial autologous conduit and release of the tethering of hypoplastic native unicuspid pulmonary valve leaflet maintaining its integrity.
Highlights
The incidence of anomalous coronary artery (ACA) in Tetralogy of Fallot (TOF) is 2–9%.1 A single coronary artery arising from either the right or left coronary sinus is seen in 1.8-4.2% of these patients.[1]
No association with single coronary artery and left circumflex artery crossing the right ventricular outflow tract (RVOT) has been described in these patients
The cardiac catheterization study showed single coronary artery arising from right aortic sinus with anomalous course of left circumflex artery crossing the RVOT (Figure 1A/Supplementary Video S1)
Summary
The incidence of anomalous coronary artery (ACA) in TOF is 2–9%.1 A single coronary artery arising from either the right or left coronary sinus is seen in 1.8-4.2% of these patients.[1]. A single coronary artery arising from either the right or left coronary sinus is seen in 1.8-4.2% of these patients.[1] About 2-10% of patients with TOF have an anomalous coronary artery crossing the RVOT thereby challenging the surgical skills of surgeon during RVOT reconstruction.[1,2] TOF with unilateral absence of pulmonary artery is a rare variant with incidence of 0.953.23%.3. No association with single coronary artery and left circumflex artery crossing the RVOT has been described in these patients. The cardiac catheterization study showed single coronary artery arising from right aortic sinus with anomalous course of left circumflex artery crossing the RVOT (Figure 1A/Supplementary Video S1). The left circumflex artery was crossing RVOT, very close to the pulmonary annulus (Figure 2A).
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