A case of atrial septal defect associated with anomalous sinoatrial node artery in pulmonary atresia with intact ventricular septum

An 11-year-old boy affected by pulmonary atresia with intact ventricular septum (AP-IVS) was listed for percutaneous pulmonary valvuloplasty and closure of multi-fenestrated atrial septal defect (ASD). Intraprocedural transesophageal echocardiography arose the suspect of abnormal coronary artery pattern while selective angiography documented a single sinoatrial node artery (SANa) with an unusual retro-aortic course. As consequence, we proceeded to effectively close the defects with a not self-centering device placed in the most central side hole. This case supports the hypothesis that sometimes arrhythmic complication during ASD closure procedures might be due to unrecognized injury of the SANa.

Commissural Malalignment as a Predictor of Coronary Artery Abnormalities in Patients with Transposition of Great Arteries

Background In patients with transposition of the great arteries (TGA), commissural malalignment (CM) between semilunar valves may be associated with abnormal coronary (CA) pattern. We intend to assess the degree of CM with incidence of unusual CA anatomy. Methods We proposed a ratio to measure the distance of both ends of the anterior facing sinuses of the pulmonary valve from the facing commissure of the aortic valve. We labeled it as D1 and D2 distance. A ratio (C ratio) of the smaller distance (either D1 or D2 whichever is shorter) over the sum of both D1 and D2 was taken (D1 or D2 whichever is shorter/D1+D2). We related this ratio with the incidence of the unusual CA anatomy in D-TGA patients Results We had a total of 158 patients. We defined the point beyond which the C-Ratio becomes significantly associated with abnormal coronary artery pattern, this represents the median effective level (EL50). The EL50 of the C-Ratio was found to be equal to 31% (0.31). The prediction revealed that the CA pattern would most probably be usual when there is a minor commissural malalignment (C-Ratio less than the EL50) and most probably be unusual when there is a major malalignment (C-Ratio is greater than the EL50). The sensitivity was 71% and the specificity 88% (p-value <0.0001). Conclusions The C-Ratio helps to categorize the degree of CM as minor (less than 0.31) or major (more than 0.31). A higher C-Ratio predicts a higher incidence of unusual CA pattern

Commissural Malalignment as a predictor of coronary artery abnormalities in patients with transposition of great arteries

Background In patients with transposition of the great arteries (TGA), commissural malalignment (CM) between semilunar valves may be associated with abnormal coronary (CA) pattern. We intend to assess the degree of CM with incidence of unusual CA anatomy. Methods We proposed a ratio to measure the distance of both ends of the anterior facing sinuses of the pulmonary valve from the facing commissure of the aortic valve. We labeled it as D1 and D2 distance. A ratio (C ratio) of the smaller distance (either D1 or D2 whichever is shorter) over the sum of both D1 and D2 was taken (D1 or D2 whichever is shorter / D1 + D2). We related this ratio with the incidence of the unusual CA anatomy in D-TGA patients. Results We had a total of 158 patients. We defined the point beyond which the C-Ratio becomes significantly associated with abnormal coronary artery pattern, this represents the median effective level (EL50). The EL50 of the C-Ratio was found to be equal to 31% (0.31). The prediction revealed that the CA pattern would most probably be usual when there is a minor commissural malalignment (C-Ratio less than the EL50) and most probably be unusual when there is a major malalignment (C-Ratio is greater than the EL50). The sensitivity was 71% and the specificity 88% (p-value < 0.0001). Conclusions The C-Ratio helps to categorize the degree of CM as minor (less than 0.31) or major (more than 0.31). A higher C-Ratio predicts a higher incidence of unusual CA pattern.

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

OBJECTIVESRepair 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.METHODSWe 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).RESULTSThe 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.CONCLUSIONSRepair 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.

Percutaneous Coronary Intervention in Different Anomalous Coronary Arteries - An Observational Study

Anomalous coronary arteries are uncommon but clinically significant, depending upon its ostial origin, course & distribution such patient may be asymptomatic or may present with angina, acute myocardial infarction, arrythmias, syncope & sudden cardiac death. Management of such cases may be medical, interventional & surgical as well. Some times it is difficult to cannulate such anomalous origin with conventional guiding catheters and requires different catheters unusual for such arteries. Between January 2002 to June 2012 a total of 3,110 elective PCI procedure were performed in NICVD & other cardiac centers in Dhaka, out of which twentyone cases were in different varities of anomalous coronary artery. We report here successful PCI in twenty-one cases having eight verities of abnormal coronary artery with failure in one case. Percutaneous coronary intervention of anomalous coronary artery origins may be difficult, which require appropriate catheter selection and different tricks. Success depends on patience and careful decision of the operator. DOI: http://dx.doi.org/10.3329/uhj.v8i1.11663 University Heart Journal Vol. 8, No. 1, January 2012