Arun Chandran

An institutional review of the value of computed tomographic angiography in the diagnosis of congenital cardiac malformations.

The ultra-fast, thin-cut computerised tomographic angiogram is an efficient method to diagnose extracardiac lesions associated with congenital cardiac disease. For the purposes of this review, we evaluated various facets of the technique as used in 30 patients who were referred for diagnosis of congenital cardiac disease. The technique had high diagnostic accuracy, with a sensitivity of 93 percent in 15 of these patients referred for either interventional catheterisation or surgery. There were no immediate side-effects associated with the scanning procedure. The scan was also found to be more cost-effective as compared to an alternative noninvasive modality for imaging modality, namely magnetic resonance imaging. The angiographic technique, however, does expose the child to between 2 and 2.5 rems of radiation, despite the short period of scanning, of 10 plus or minus 2 seconds.

Evaluating the extracardiac Fontan conduit by multislice computed tomography: an emerging modality.

Subsequent failure of the Fontan circulation had resulted in signs of failure of the right heart, so revision was achieved by placing an extracardiac conduit between the inferior caval vein and the undersurface of the bifurcation of the pulmonary arteries. During the postoperative period, we used cardiac computed tomography to evaluate the patency of the extracardiac conduit. Figure 1 (arrow) demonstrates a cross-sectional view of the patent extracardiac conduit, while the conduit is shown in coronal view in Figure 2 (arrow). In Figure 3, we provide a three-dimensional reconstruction that clearly visualizes the external appearance of the entire Fontan circuit (arrow). Our experience highlights again the ability of cardiac computed tomography to delineate, in a non-invasive Figure 1. Figure 2.

Case of persistent left superior vena cava draining via the left atrium with a normal coronary sinus utilizing cardiac CT

With an estimated incidence of ≈ 0.5%-2% of the population and 10% of patients with congenital heart disorders, persistent LSVC is the most common venous anomaly of the chest. Most drain via the right atrium. Rarely (7.5% of cases), the persistent LSVC may drain directly into the left atrium. These are typically seen with a coronary sinus type atrial septal defect with many investigators believing that, embryologically, a normal coronary sinus cannot exist in the setting of a persistent LSVC draining into the left atrium. There have been few case reports of this in the setting of a normal coronary sinus. With the assistance of cardiac CT, we were able to show the existence of this rare entity. The CT images provided guidance for a successful endovascular repair.

Left Atrial Appendage Aneurysm Characterized by Multimodal Imaging

We present a four-year-old female with an incidental finding of a congenital left atrial appendage aneurysm who underwent surgical resection with excellent results. This case highlights the importance of multimodal imaging in the diagnosis and characterization of this rare condition.

Severe Direct Hyperbilirubinemia as a Consequence of Right Heart Failure in Congenital Heart Disease.

Adult literature documents increased cholestasis in right heart failure yet is poorly documented in the pediatric population. We describe three infants with congenital heart disease who developed significantly elevated direct bilirubin levels of 43, 23, and 12 mg/dL, respectively, in the absence of hepatic dysfunction. The common hemodynamic pathophysiology in these infants is right heart dysfunction with moderate to severe tricuspid regurgitation in the setting of low perfusion state. Right heart dysfunction in infants can result in severe conjugated bilirubin, likely as a consequence of venous congestion and can be used as an indirect marker of right heart dynamics

Hybrid Stage 1 Palliation in a Patient With Hypoplastic Left Heart Syndrome and Unusual Decompressing Levoatrial Cardinal Vein in Turner Syndrome: Utility of Multimodality Imaging.

A newborn with prenatal diagnosis of Turner syndrome with hypoplastic left heart syndrome (HLHS) was born at term at our institution without perinatal complications. Prostaglandin (PGE) infusion was started, and the initial transthoracic echocardiogram (TTE) confirmed HLHS with severe mitral and aortic valve stenosis. There was a restrictive atrial communication with a large tortuous, decompressing levoatrial cardinal vein (LACV) that drained in a very unusual fashion to the superior vena cava (Figure 1A and B and Video 1). Family wished to pursue every available option, despite known poor outcomes with traditional stage 1 Norwood palliation in the 1 Pediatric Cardiology, University of Florida, Gainesville, FL, USA 2 UF Health Congenital Heart Center, University of Florida College of Medicine, Gainesville, FL, USA 3 Department of Pediatrics, Congenital Heart Center, University of Florida, Gainesville, FL, USA

Coronary Cardiac Fistula Evaluation by Cardiac CTA for Management

We present the case of a 5-year-old otherwise healthy female referred to our clinic for evaluation of a continuous cardiac murmur heard at the left upper sternal border. A differential diagnosis of patent ductus arteriosus versus coronary cardiac fistula versus arteriovenous fistula was considered based on the physical examination. Initial transthoracic echocardiogram demonstrated a large right coronary artery (RCA) to right ventricle (RV) fistula with a very dilated (7 mm) proximal RCA (Supplementary Video S1). There was associated moderate right heart dilation. On further discussion with our pediatric cardiothoracic surgeon and interventional cardiologist, initial consensus was to first obtain a prospectively gated pediatric cardiac computed tomographic angiography (CTA) to better delineate morphology of the coronary cardiac fistula especially with respect to elucidating the proximity of the distal communication of the coronary cardiac fistula to the posterior descending artery prior to establishing surgical versus interventional candidacy. The CTA confirmed the anatomy of the RCA to RV fistula and demonstrated diffuse ectasia of the RCA (7.2 mm; z score of 14.5). Maximum intensity projection with vessel tracking software clearly delineated the fistulous connection at the base of RV (Figure 1A). Three-dimensional reformation from the CTA demonstrated the dilated RCA and its fistulous connection to the RV (Figure 1B, Supplementary Video S2). The RCA was massively dilated secondary to the increased flow from the fistula (Figure 1C, Supplementary Video S3). The CTA findings were determined to be conducive for device closure of the coronary cardiac fistula and the patient successfully underwent percutaneous catheter-directed coil occlusion of the fistula (Figure 1D, Supplementary Videos S4-S6). She remains asymptomatic on anticoagulation for ongoing dilation of her RCA. Our case highlights the utility of cardiac CTA in delineating aberrant pediatric coronary anatomy, allowing for assessment of operative candidacy and treatment planning (surgical vs interventional) especially as it pertains to delineating the baseline morphology but also minimizing the risk of potential device-related complications such as compression of adjacent structures or embolization. Cardiac CTA clearly delineated the anatomy of the coronary cardiac fistula while complementing the functional assessment provided by echocardiogram.

Case of double-outlet right ventricle after repair with pulmonary arteriovenous malformations using cardiac CT

We present a case where cardiac CT clearly defines the complex anatomy of a 22 year old man with double outlet right ventricle who is status bilateral bidirectional Glenn and Fontan conduit. This case also shows the association of pulmonary arteriovenous malformations with Fontan and Glenn procedures.

Dual connection of single pulmonary vein in partial anomalous pulmonary venous return

We present the case of a 6-month-old infant born premature at 29 weeks with perinatal stroke and postnatal hypoxia. Echocardiogram was suspicious for partial anomalous pulmonary venous return (PAPVR). Cardiac CT showed an unusual variant of PAPVR, with a vertical vein having a dual connection superiorly to the left innominate vein and inferiorly to the morphologic left atrium. This unusual variant has the potential for right-to-left flow with a possibility of systemic hypoxia and paradoxical embolism.

Aorto-Pulmonary Window in an Adult: Utility of Multimodality Imaging

From the *Department of Pediatrics, University of Florida, Gainesville, Florida; †Department of Radiology, University of Florida, Gainesville, Florida; ‡Department of Cardiology, University of Florida, Gainesville, Florida; and the Congenital Heart Center, University of Florida, Gainesville, Florida. Manuscript received November 8, 2010; accepted November 19, 2010. A46-year-old man with a history of cardiothoracic surgery at 20 years of age of uncertain type sought treatment for worsening dyspnea on exertion and episodes of dizziness. He had central cyanosis, clubbing, and baseline saturations of 96% that dropped to 86% with exercise. Initial transthoracic echocardiogram (A, Online Video 1) showed moderate right ventricular hypertrophy with dysfunction and right ventricular hypertension. It was also indicative of a residual bidirectional aortopulmonary shunt at the level of the prior surgically placed patch (B, Online Video 2). Cardiac magnetic resonance imaging confirmed the presence of the right ventricular hypertrophy and associated dysfunction (C, Online Video 3) with late gadolinium enhancement (D, Online Video 4) and the small bidirectional aortopulmonary shunt (E, Online Video 5). This multimodality imaging contributed to the subsequent Published by Elsevier Inc. doi:10.1016/j.jacc.2010.11.076