Aortic Root Translocation with Arterial Switch for Transposition of the Great Arteries or Double Outlet Right Ventricle with Ventricular Septal Defect and Pulmonary Stenosis.

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.

Biventricular Repair after Bilateral Pulmonary Artery Banding as a Rescue Procedure for a Neonate with Hypoplastic Left Heart Complex.

Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation. A postoperative echocardiogram showed newly appearing CoA and progressively narrowing PDA, which resulted in the need for biventricular repair 21 days following the palliation surgery. The patient was discharged on postoperative day 13 without complications and is doing clinically well seven months after surgery.

Heart transplantation in pediatric patients: twelve-year experience of the Asan Medical Center.

Heart transplantation is a standard treatment for end-stage heart disease. Pediatric heart transplantation, however, is not frequently performed due to the shortage of pediatric heart donors. This is the first report of pediatric heart transplantation in Korea. Our retrospective study included 37 patients younger than 18 yr of age who underwent heart transplantation at Asan Medical Center between August 1997 and April 2009. Preoperative diagnosis was either cardiomyopathy (n = 29, 78.3%) or congenital heart disease (n = 8, 22.7%). Mean follow up period was 56.9 ± 44.6 months. There were no early death, but 7 late deaths (7/37, 18.9%) due to rejection after 11, 15, 41 months (n = 3), infection after 5, 8, 10 months (n = 3), suspicious ventricular arrhythmia after 50 months (n = 1). There was no significant risk factor for survival. There were 25 rejections (25/37, 67.6%); less than grade II occurred in 17 patients (17/25, 68%) and more than grade II occurred in 8 patients (8/25, 32%). Actuarial 1, 5, and 10 yr survival was 88.6%, 76.8%, and 76.8%. Our midterm survival of pediatric heart transplantation showed excellent results. We hope this result could be an encouraging message to do more pediatric heart transplantation in Korean society.

Coronary artery anomalies and clinically important anatomy in patients with congenital heart disease: multislice CT findings.

In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease.

CT findings of plastic bronchitis in children after a Fontan operation.

BACKGROUND: Plastic bronchitis is a rare cause of acute obstructive respiratory failure in children. Life-threatening events are much more frequent in patients with repaired cyanotic congenital heart disease, and most frequent following a Fontan operation. Commonly, the diagnosis is not made until bronchial casts are expectorated. Detailed CT findings in plastic bronchitis have not been described. OBJECTIVE: To describe the CT findings in plastic bronchitis in children after a Fontan operation. MATERIALS AND METHODS: Three children with plastic bronchitis after a Fontan operation were evaluated by chest CT. Bronchial casts were spontaneously expectorated and/or extracted by bronchoscopy. Airway and lung abnormalities seen on CT were analyzed in the three children. RESULTS: CT demonstrated bronchial casts in the central airways with associated atelectasis and consolidation in all children. The affected airways were completely or partially obstructed by the bronchial casts without associated bronchiectasis. The airway and lung abnormalities rapidly improved after removal of the bronchial casts. CONCLUSION: CT can identify airway and lung abnormalities in children with plastic bronchitis after a Fontan operation. In addition, CT can be used to guide bronchoscopy and to monitor treatment responses, and thereby may improve clinical outcomes.

Multislice CT angiography of interrupted aortic arch.

Interrupted aortic arch (IAA) is defined as complete luminal and anatomic discontinuity between the ascending and descending aorta. Because almost all patients with IAA become critically ill during the neonatal period, they should undergo urgent corrective surgery. This clinical urgency necessitates a fast and accurate noninvasive diagnostic method. Although echocardiography remains the primary imaging tool for this purpose, it is not always sufficient for planning surgical correction of IAA, principally due to a limited acoustic window and the inexperience of imagers. In this context, multislice CT angiography is regarded as an appropriate imaging technique complementary to echocardiography because it is fast, accurate, and objective for the diagnosis of IAA. In this article we describe what cardiac radiologists should know about IAA in their clinical practice, including clinicopathological features, CT features with contemporary surgical methods and postoperative complications, and differentiation from coarctation of the aorta and aortic arch atresia.

Time-resolved three-dimensional contrast-enhanced magnetic resonance angiography in patients who have undergone a Fontan operation or bidirectional cavopulmonary connection: initial experience.

PURPOSE: To evaluate the usefulness of time-resolved three-dimensional (3D) magnetic resonance angiography (MRA) using diluted contrast agent (CA) in patients who had undergone a Fontan operation or bidirectional cavopulmonary connection (BCPC). MATERIALS AND METHODS: Time-resolved 3D MRA (10 dynamic data sets, two seconds per dynamic data set) using parallel imaging and keyhole data sampling was performed on 15 patients (median age=10 years, range=1-20 years) who had undergone a Fontan operation (N=11) or BCPC (N=4). Diluted gadolinium (Gd) contrast agent (CA) was intravenously injected into the arm and/or leg veins. The flow dynamics and morphology of pulmonary circulation, and lung perfusion were assessed. RESULTS: Preferential or balanced pulmonary blood flow from each systemic vein was visualized on time-resolved 3D MRA in all patients. In addition, occlusion/stenosis of the central thoracic vein (N=4) and pulmonary artery (N=6), systemic venous (N=5) and arterial (N=6) collaterals, and lung perfusion defect (N=4) were identified. Persistent hepatic venous plexus, pulmonary arteriovenous malformation, and axillary arteriovenous fistula were delineated in three patients, respectively. CONCLUSION: Time-resolved 3D MRA with diluted CA is useful for evaluating patients who have undergone a Fontan operation or BCPC because it can reveal the flow dynamics and morphology of pulmonary circulation, and lung perfusion status.

Coronary CT angiography and MR angiography of Kawasaki disease.

Although the incidence of coronary artery aneurysms has diminished in patients with Kawasaki disease, coronary artery involvement is still regarded as a major complication of the disease, significantly affecting morbidity and mortality. Recent technical advances in coronary CT angiography (CTA) and MR angiography (MRA) have led to the possibility of using these two imaging methods as minimally invasive alternatives to the more invasive diagnostic catheter angiography in evaluating coronary artery abnormalities, such as aneurysm, stenosis, and occlusion. In this article, we describe imaging techniques and findings of coronary CTA and MRA in Kawasaki disease.

Computed tomography for the diagnosis of congenital heart disease in pediatric and adult patients.

The development of multi-slice spiral computed tomography (CT) has increased the clinical use of cardiac CT imaging in patients with congenital heart disease. Multi-slice CT has the advantages of fast scan speed; high spatial resolution, enabling the acquisition of isotropic volume data; and simultaneous evaluation of airways and lung parenchyma, thus increasing the ability to answer most clinical questions about structural abnormalities in patients with congenital heart disease. When coupled with electrocardiography-gating, multi-slice spiral CT can be used in functional evaluations, including ventricular wall motion, ventricular ejection fraction, and motion of cardiac valves, as well as enabling the performance of high-quality coronary CT angiography. In this article, we review imaging techniques of multi-slice spiral CT and imaging findings in pediatric and adult patients with various congenital heart diseases.

Visibility of the origin and proximal course of coronary arteries on non-ECG-gated heart CT in patients with congenital heart disease.

BACKGROUND: There is little information on the ability of non-ECG-gated cardiac CT to demonstrate the coronary arteries of children. OBJECTIVE: To evaluate the visibility of the origin and proximal course of coronary arteries on non-ECG-gated cardiac CT, in which the coronary artery was not of primary diagnostic concern, in children with congenital heart disease. MATERIALS AND METHODS: From December 2002 to March 2004, 126 cardiac CT examinations from 104 children (median age 11 months; age range 1 day to 15 years) were evaluated. All patients had ventriculo-arterial concordance and no malformations of the great arteries; those with coronary artery anomalies were excluded. Contrast-enhanced 16-slice spiral CT was performed without ECG-gating and multiplanar images for coronary arteries were obtained. The visibility of coronary artery origins was graded on a three-point scale, while nine segments of the arteries were graded on a four-point scale. CT images in which it was possible to trace the coronary arteries were considered diagnostic. The visibility of each whole coronary artery and the origins and proximal four segments of coronary arteries were calculated. The visibility of coronary arteries was also correlated with patient age. RESULTS: The percentage of CT images of diagnostic quality was 49.3% for the whole coronary artery and 81.7% for the origins and proximal four segments. There was a significant positive correlation between the visibility of coronary arteries and age. CONCLUSIONS: Non-ECG-gated cardiac CT, in which the coronary artery is not of primary diagnostic concern, is frequently able to visualize the origin and proximal course of coronary arteries and may be helpful in detecting coronary artery anomalies in children with congenital heart disease.

Horseshoe lung: useful angiographic and bronchographic images using multidetector-row spiral CT in two infants.

Horseshoe lung is a rare congenital pulmonary anomaly of childhood that can be accompanied with other anomalies. The diagnosis has historically been accomplished with invasive catheter angiography and bronchography rather than CT. Two infants with horseshoe lung were recently diagnosed with CT. We report the imaging findings in these two patients with emphasis on angiographic and bronchographic demonstration of key abnormalities of horseshoe lung using multidetector-row spiral CT.