Scimitar syndrome and anomalous origin of the circumflex artery from the main pulmonary artery in infancy: a case report.

We present a neonate with an antenatal diagnosis of Scimitar syndrome and aortic arch hypoplasia. After delivery, computerised tomography scan additionally revealed an anomalous origin of the circumflex coronary artery from the main pulmonary artery. The management of this rare combination is discussed.

Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome: A Multicenter International Study.

OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND: Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.

Abnormal left ventricular diastolic function at late follow-up after repair of total anomalous pulmonary venous drainage: the impact of altered ventricular loading in utero.

BACKGROUND: Assessment of diastolic function has not been described after repair of total anomalous pulmonary venous drainage (TAPVD), but studies of exercise capacity demonstrate impaired performance in this population despite normal systolic function. We postulated that diastolic impairment might contribute to this finding. METHODS: We analyzed echocardiographic variables from 28 patients with repaired TAPVD and compared these with data from 32 healthy controls (normals) and 21 subjects with repaired transposition of the great arteries (TGA). RESULTS: Left ventricular (LV) end-diastolic volumes were smaller in the TAPVD group (median, 50 mL/m(2) compared with a median of 64 mL/m(2) in TGA and 67 mL/m(2) in normals; P < .001 in each case). LV diastolic function in the TAPVD group was impaired. Mitral early to late ratio was increased (median, 2.7 in TAPVD compared with a median of 1.9 in TGA [P = .047] and 2.1 in normals [P = .021]). LV isovolumic relaxation time was reduced (median, 50 milliseconds in TAPVD compared with a median of 70 milliseconds in both TGA and normals; P < .001 in each case). Late diastolic and systolic tissue Doppler velocities were lower and the E/e' ratio was higher in the TAPVD group. CONCLUSIONS: Patients with repaired TAPVD are usually regarded as having excellent outcomes, but the finding of LV diastolic dysfunction in this population warrants more careful follow-up. We postulate that the diastolic impairment in these patients is the result of relative unloading of the LV during early cardiac development. These findings may also have implications in considering therapeutic approaches for hypoplastic ventricles in attempting to achieve biventricular repair.