Computed Tomographic 3-Dimensional Virtual Dissection Aiding in Diagnosis and Surgical Planning of a Rare Form of Obstructed Supracardiac Total Anomalous Pulmonary Venous Connection With an Unusual Scimitar-Like Arrangement.

We present a case of a newborn with a rare presentation of obstructed supracardiac total anomalous pulmonary venous connection who required emergent cannulation to extracorporeal membrane oxygenation (ECMO). Computed tomographic angiography of the heart was performed and using novel virtual dissection techniques aided in surgical planning and guidance. Computed tomographic angiography can be successfully performed in neonates with complex congenital heart disease on ECMO without adjustment of flows to aid in surgical management and novel virtual dissection techniques aid in complex anatomical delineation and spatial orientation with noncardiac structures. The preoperative imaging in this case allowed for appropriate and detailed presurgical planning and contributed to the excellent outcome of this patient.

Normal pediatric data for isovolumic acceleration at the lateral tricuspid valve annulus-a heart rate - dependent measure of right ventricular contractility.

BACKGROUND: Quantitative assessment of right ventricular (RV) function is important for the management of patients with congenital heart disease. Tissue Doppler (TDI)-derived myocardial acceleration during isovolumic contraction (IVA) is an echocardiographic measure of contractility that is independent of loading conditions. The aim of this study was to establish normative data for IVA at the lateral tricuspid valve annulus (RV IVA). METHODS: This is a retrospective study of 340 children who had normal echocardiograms. We analyzed RV IVA in relation to age, body surface area (BSA), gender, heart rate (HR), tricuspid annular plane systolic excursion (TAPSE), and TDI-derived S' (RV S'). RESULTS: RV IVA showed a positive correlation with HR (r = 0.58, P<0.001) and a negative correlation with age and BSA (r = -0.44, P<0.001, for both). RV IVA ranged from 2.3 m/sec(2) (±2 SD: 1-3.7 m/sec(2) ) at a HR of <60 beats per minute (bpm) to 4.9 m/sec(2) (±2 SD: 3.5-6.4 m/sec(2) ) at a HR of >160 bpm. When RV IVA was corrected for HR (RV IVAc), it became independent of HR, age, BSA, and gender. The mean RV IVAc was 2.3 m/sec(1.5) (±2 SD: 1.1-3.6 m/sec(1.5) ). There was a correlation of normalized RV IVAc with normalized TAPSE and RV S'. CONCLUSION: RV IVA, a marker of RV contractility, shows a strong dependence on HR. HR corrected RV IVA (RV IVAc) is independent of age, BSA, gender, and HR. We suggest measuring RV IVAc routinely in patients at risk for RV dysfunction.