Tetralogy of Fallot and abnormal coronary artery: use of a prosthetic conduit is outdated

Abstract

OBJECTIVES Repair of tetralogy of Fallot (ToF) can be challenging in the presence of an abnormal coronary artery (CA) in 5–12% of cases. The aim of this study was to report our experience with ToF repair without the systematic use of a right ventricle-to-pulmonary artery (RV-PA) conduit. METHODS We conducted a monocentric retrospective study from 2000 to 2016, including 943 patients with ToF who underwent biventricular repair, of whom 8% (n = 76) presented with an abnormal CA. Mean follow-up time was 50 months (1 month–18 years). RESULTS The most frequent CA anomaly was the left descending artery arising from the right CA (n = 47, 61.8%). The median age at repair was 7.7 months (1.8 months–16 years). Thirteen patients (17%) required prior palliation, mostly systemic pulmonary shunts for anoxic spells in the neonatal period. Surgical repair allowed us to preserve the annulus in 40 patients (53%) by combining PA trunk plasty, commissurotomy and infundibulotomy under the abnormal CA. If the annulus had to be opened (n = 35, 46%), a transannular patch was inserted after a vertical incision of the PA trunk and extended obliquely on the RV over the anomalous crossing CA (with an infundibulotomy under the abnormal CA). Three patients (4%) required the insertion of an RV-PA conduit (1 valved tube and 2 RV-PA GORE-TEX tubes with annulus conservation). The early mortality rate was 4% (n = 3); none of the deaths was coronary related. Four patients (5%) required reoperation (2 early and 2 late reoperations) for residual pulmonary stenosis, 3 of whom had annulus preservation during the initial repair. The mean RV/left ventricle (LV) pressure ratio and an RV/LV pressure ratio >2/3 were identified as risk factors for right ventricular outflow tract (RVOT) reinterventions (P = 0.0026, P = 0.0085, respectively), RVOT reoperations (P = 0.0002 for both) and reoperation for RVOT residual stenosis (P = 0.0002, P = 0.0014, respectively). Two patients underwent pulmonary valve replacement. Freedom from late reoperation was 100% at 1 year, 97% at 5 years and 84% at 10 and 15 years. CONCLUSIONS Repair of ToF and abnormal CA can be performed without an RV-PA conduit, with an acceptable low reintervention rate. The high early mortality rate in this series remains a concern. If any doubt remains about the surgical relief of the RVOT obstruction, the RV/LV pressure ratio should always be measured in the operating room.

Coronary anomalies in tetralogy of Fallot – A meta-analysis

“…Transpulmonary, the pulmonary trunk can be enlarged with a patch. Using this approach Pontailler et al [53] succeeded in 53% of cases to spare the pulmonary valve annulus. It is advised to wait with total repair of TOF until the child is at least 8 kg to make surgery technically easier.…”

Coronary Artery Anomalies and Their Impact on the Feasibility of Percutaneous Pulmonary Valve Implantation

“…Our findings correspond with previous data describing coronary artery anomalies in patients with tetralogy of Fallot. Among 943 surgical patients with tetralogy of Fallot Pontailler et al found 76 (8%) with anomalous coronary arteries crossing the right ventricular infundibulum [ 8 ]. The most frequent anomalies were origin of the main or an accessory LAD from the right aortic sinus accounting for 62% of these anomalies.…”

“…Major infundibular branches were present in about 20%. Abnormal origin of the right coronary artery from the left aortic sinus or from the LAD was described in 12% while the remaining 6% represented patients with a single coronary originating either from the right or left aortic sinus [ 8 ]. Quite similar distributions of anatomic variants were reported by Ruzmetov et al among 43 patients with tetralogy of Fallot and anomalous coronary arteries [ 9 ].…”

“…Quite similar distributions of anatomic variants were reported by Ruzmetov et al among 43 patients with tetralogy of Fallot and anomalous coronary arteries [ 9 ]. While these coronary anomalies no longer represent a significant risk factor for corrective surgery which frequently can be performed without the use of a right ventricular to pulmonary artery conduit, they remain important for the planning of later repeat surgery or PPVI [ 8 , 9 ]. Abnormal coronary artery anatomy was demonstrated in 34/226 (15%) patients with tetralogy of Fallot who underwent catheterization and testing for intended PPVI [ 7 ].…”

“…In patients with origin of the left anterior descending coronary artery (LAD) from the right aortic sinus the LAD crosses the right ventricular outflow tract (RVOT) in close proximity to a potential landing zone. This coronary anomaly is not uncommon in patients with tetralogy of Fallot, who represent a major percentage of potential candidates for PPVI [7][8][9]. The aim of this study was to assess the frequency and relevance of CAA impacting the feasibility of PPVI based on retrospective evaluation of all patients who underwent invasive testing in our institution.…”