BACKGROUNDPulmonary artery sling (PAS) is a rare congenital heart anomaly and may cause unexplained respiratory symptoms in infants. Since the non-specific respiratory symptoms of PAS may lead to misdiagnosis, the aim of this study was to clarify the clinical and imaging features of this disease for timely diagnosis and treatment.

METHODSClinical histories, physical examinations and imaging studies were retrospectively evaluated in nine infants with PAS. Chest X-ray, echocardiography and contrast-enhanced computed tomography (CT) with 3-dimensional reconstructions were performed in all patients and three of them received surgical treatment.

RESULTSNine cases included six males and three females with a mean age of (4.3 ± 2.8) months ranging from 2 to 11 months old. All patients had respiratory symptoms including recurrent cough, stridor and wheezing. The onset of symptoms was within 3 months in all cases and three children had symptoms only a few days after birth. The chest X-ray showed pneumonia in all cases. Contrast-enhanced CT showed the tracheal compression at different lengths in every case. The echocardiograph findings of PAS were anomalous origins of the left pulmonary artery from the posterior aspect of the right pulmonary artery. Of the 9 cases, 8 cases were diagnosed correctly by echocardiography. Of the complicated abnormalities, there were one with secundum atrial septal defect, one with patent foramen ovale and three with persistent left superior vena cava. None of them were complicated with significant blood dynamic changes.

CONCLUSIONSInfants with recurrent respiratory symptoms such as chronic cough, stridor and wheezing, should be examined for the possible presence of congenital pulmonary artery sling. As a noninvasive technique, echocardiography is very helpful and should be the first-choice modality for the diagnosis of pulmonary artery sling. Contrast-enhanced CT, clearly demonstrating the anatomy of pulmonary artery sling and the position and extent of trachea compression, is necessary for the final diagnosis and pre-operation evaluation.

Echocardiography ; Female ; Heart Defects, Congenital ; diagnosis ; diagnostic imaging ; physiopathology ; Hemodynamics ; physiology ; Humans ; Infant ; Male ; Pulmonary Artery ; abnormalities ; diagnostic imaging ; physiopathology ; Radiography

Cardiac magnetic resonance (CMR) imaging is now widely used in several fields of cardiovascular disease assessment due to recent technical developments. CMR can give physicians information that cannot be found with other imaging modalities. However, there is no guideline which is suitable for Korean people for the use of CMR. Therefore, we have prepared a Korean guideline for the appropriate utilization of CMR to guide Korean physicians, imaging specialists, medical associates and patients to improve the overall medical system performances. By addressing CMR usage and creating these guidelines we hope to contribute towards the promotion of public health. This guideline is a joint report of the Korean Society of Cardiology and the Korean Society of Radiology.
Cardiomyopathies/diagnosis/radiography ; Cardiotonic Agents/therapeutic use ; Chest Pain/complications/diagnosis/radiography ; Coronary Artery Bypass ; Coronary Artery Disease/*diagnosis/drug therapy/radiography ; Dobutamine/therapeutic use ; Echocardiography ; Heart Defects, Congenital/diagnosis/radiography ; Heart Failure/diagnosis/ultrasonography ; Humans ; *Magnetic Resonance Imaging ; Mucocutaneous Lymph Node Syndrome/complications/diagnosis ; Percutaneous Coronary Intervention ; Prognosis ; Risk Assessment ; Ventricular Function, Left/physiology

Coronary artery problems in children usually have a significant impact on both short-term and long-term outcomes. Early and accurate diagnosis, therefore, is crucial but technically challenging due to the small size of the coronary artery, high heart rates, and limited cooperation of children. Coronary artery visibility on CT and MRI in children is considerably improved with recent technical advancements. Consequently, CT and MRI are increasingly used for evaluating various congenital and acquired coronary artery abnormalities in children, such as coronary artery anomalies, aberrant coronary artery anatomy specific to congenital heart disease, Kawasaki disease, Williams syndrome, and cardiac allograft vasculopathy.
Child ; Child, Preschool ; Coronary Angiography/*methods ; Coronary Vessel Anomalies/*radiography ; Coronary Vessels/*radiography ; Diagnostic Imaging ; Echocardiography/methods ; Female ; Heart Defects, Congenital/radiography ; Heart Diseases/diagnosis/*radiography ; Heart Rate ; Humans ; Magnetic Resonance Angiography/*methods ; Male ; Mucocutaneous Lymph Node Syndrome/radiography ; Tomography, X-Ray Computed/methods

PURPOSE: The clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases. MATERIALS AND METHODS: From July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n=28), hemitruncus arteriosus (HTA) (n=3), type I truncus arteriosus (TA) (n=4), and the aortopulmonary window (APW) (n=3), were comparatively reviewed. RESULTS: All 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnositic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology. CONCLUSION: In case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.
Angiography ; Aorta, Thoracic/*abnormalities/radiography/ultrasonography ; Child ; Child, Preschool ; Diagnosis, Differential ; Echocardiography, Doppler ; Female ; Heart Defects, Congenital/classification/diagnosis ; Humans ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Retrospective Studies