STUDY DESIGN: A retrospective study. OBJECTIVES: To analyze natural history and clinical features of scoliosis developed after surgery for congenital heart disease and to investigate the influence of cardiac surgery on the onset and progression of scoliosis. MATERIALS AND METHODS: Three hundred and five patients who had been operated for congenital heart disease from Jan.1988 to Dec.1990 and followed up for more than 5 years were analyzed. The curve which was more than 10 degrees on radiography was defined as significant scoliosis and the patients with congenital spinal anomalies were excluded. We compared age at surgery, cardiac disease and effect of cyanosis between scoliosis and non-scoliosis group. Furthermore, in scoliosis group, prevalence, onset of significant scoliosis, and manifestation of curve according to side of thoracotomy were assessed. RESULTS: Fifty-six patients(18.4%) had scoliosis of more than 10 degrees. As for location, high thoracic in 19, thoracic in 23, double thoracic in 8, thoraco-lumbar in 5 and double major in 1. Of the 42 patients with thoracic or high thoracic curves, 17(40%) showed convexity to the left. The magnitudes of curves were less than 40 degrees except 1. There was no significant difference in prevalence(p=0.513) and Cobb angle(p=0.634) between cyanosis and acyanosis group. Scoliosis developed between 3 and 6 years after cardiac operation in 26 patients(46%). In high thoracic curve, 6 of 7 patients with left thoracotomy demonstrated convexity to the left and 5 of 6 patients with right thoracotomy demonstrated convexity to the right(p=0.026). CONCLUSIONS: The prevalence of scoliosis after surgery for congenital heart disease was 18.4%. High thoracic and left thoracic curves were more frequent compared to idiopathic curves and a half of them developed 3 to 6 years after cardiac surgery. Most patients did not have severe curve to need surgical intervention and there was no correlation between severity of scoliosis and age at cardiac operation and cyanosis. In high thoracic curve, the tendency for the curve to be convex to the side of cardiac approach was demonstrated.
Child* ; Cyanosis ; Heart Defects, Congenital* ; Humans ; Natural History ; Prevalence ; Radiography ; Retrospective Studies ; Scoliosis* ; Thoracic Surgery ; Thoracotomy

Heart Defects, Congenital ; diagnostic imaging ; Humans ; Organ Size ; Radiography, Thoracic ; Syndrome

CT is increasingly being used for evaluating the cardiovascular structures and airways in the patients with congenital heart disease. Multi-slice CT has traditionally been used for the evaluation of the extracardiac vascular and airway abnormalities because of its inherent high spatial resolution and excellent air-tissue contrast. Recent developments in CT technology primarily by reducing the cardiac motion and the radiation dose usage in congenital heart disease evaluation have helped expand the indications for CT usage. Tracheobronchomalacia associated with congenital heart disease can be evaluated with cine CT. Intravenous contrast injection should be tailored to unequivocally demonstrate cardiovascular abnormalities. Knowledge of the state-of-the-art CT imaging techniques that are used for evaluating congenital heart disease is helpful not only for planning and performing CT examinations, but also for interpreting and presenting the CT image findings that consequently guide the proper medical and surgical management.
Contrast Media ; Electrocardiography ; Heart Defects, Congenital/complications/*radiography ; Humans ; Imaging, Three-Dimensional ; Radiation Dosage ; Tomography, Spiral Computed ; *Tomography, X-Ray Computed/methods ; Tracheomalacia/complications/congenital/radiography

A 6-month-old mature intact female Siamese cat presented with exertional dyspnea. Diagnostic studies revealed pleural effusion, grade 4/6 left basal systolic murmur, deep S-wave in electrocardiograph leads I, II, and III, cardiomegaly with pleural effusion on radiography, pulmonic systolic (~5.8 m/sec) and tricuspid (3.6 m/sec) regurgitant jets, atrial septal defect, and a hypoplastic right outflow tract. Based on these results, the case was diagnosed as pulmonic stenosis with atrial septal defect. To the best of our knowledge, this is the first case report describing pulmonic stenosis with atrial septal defect in a cat in Korea.
Animals ; Cardiomegaly ; Cats* ; Dyspnea ; Electrocardiography ; Female ; Heart Defects, Congenital ; Heart Septal Defects, Atrial* ; Humans ; Infant ; Korea ; Pleural Effusion ; Pulmonary Valve Stenosis* ; Radiography ; Systolic Murmurs

We present an unusual case of an intracardiac Eustachian valve cyst observed concurrently with atresia of the coronary sinus ostium, a persistent left superior vena cava (LSVC) and a bicuspid aortic valve. There have been several echocardiographic reports of Eustachian valve cysts; however, there is no report of multidetector computed tomography (MDCT) findings related to a Eustachian valve cyst. Recently, we observed a Eustachian valve cyst diagnosed on MDCT showing a hypodense cyst at the characteristic location of the Eustachian valve (the junction of the right atrium and inferior vena cava). MDCT also demonstrated additional cardiovascular anomalies including atresia of the coronary sinus ostium and a persistent LSVC and bicuspid aortic valve.
Aged ; Aortic Valve/abnormalities/radiography ; Cysts/*radiography ; Echocardiography, Transesophageal ; Heart Atria/abnormalities/radiography ; Heart Defects, Congenital/*radiography/surgery ; Humans ; Male ; *Tomography, X-Ray Computed ; Vena Cava, Inferior/abnormalities/radiography ; Vena Cava, Superior/abnormalities/radiography

Unilateral absence of a pulmonary artery is an uncommon anomaly, which presents as an isolated lesion or in combination with other congenital heart disease such as TOF or PD^. We encountered three cases of isolated unilateral absence of a pulmonary artery;one was left pulmonary artery agenesis with right sided aortic arch and the others were right pulmonary artery agenesis with left sided aortic arch. Plain chest radiograph showed considerable loss of unilateal lung volume and lack of ipsilateral hilar shadow. Pulmonary angiogram which was done in two cases, revealed proximal interruption of a pulmonary artery. Chest CT was done in only one case, on which right pulmonary artery was absent and was replaced by adipose tissue. CT with its clean demonstration pulmonary artery without any evidence of aquired obstruction of a pulmonary artery by pulmonary embolism or tumor invasion, maybe a valuable method for evaluaton of the unilateral absence of a pulmonary artery.
Adipose Tissue ; Aorta, Thoracic ; Heart Defects, Congenital ; Lung ; Pulmonary Artery* ; Pulmonary Embolism ; Radiography, Thoracic ; Tomography, X-Ray Computed

Scimitar syndrome is a rare congenital anomaly that is characterized by hypoplasia of the right lung and the right pulmonary artery with anomalous pulmonary venous drainage to the inferior vena cava. The scimitar vein is usually visible on chest radiographs, but may be obscured by the heart. It is essential for surgical correction to establish the point of drainage of the anomalous vein and associated anomalies. There are recent reports of familial total anomalous pulmonary venous return suggesting heritable forms of this anomaly. Although genetic factors are believed to have important roles in congenital heart disease, few genes involved in heart development have been located. We report a case of familial chromosome 9 inversion with Scimitar syndrome in an offspring who presented with dextrocardia. Evaluation with magnetic resonance cineangiograph imaging demonstrated an anomalous pulmonary vein draining into the inferior vena cava above the diaphragm and hypoplasia of the right lung and the right pulmonary artery. Chromsome analysis showed pericentric inversion of chromosome 9, inv 9 (p13, q21), in the patient and his mother as well. A brief review of the related literature is also included.
Chromosomes, Human, Pair 9 ; Dextrocardia ; Diaphragm ; Drainage ; Heart ; Heart Defects, Congenital ; Humans ; Lung ; Mothers ; Pulmonary Artery ; Pulmonary Veins ; Radiography, Thoracic ; Scimitar Syndrome* ; Veins ; Vena Cava, Inferior

The assessment of the accuracy of Electron Beam Computed Tomography (EBCT) for the follow-up of pulmonary vascular system after the shunt operation in cyanotic congenital heart diseases was purpose of the study. The study group consists of 16 consecutive patients with cyanotic congenital heart disease who had Blalock-Taussig (BT) shunt (n=7), bi- directional cavo-pulmonary shunt (BCPS, n=7) and unifocalization (n=2). EBT images were obtained on systolic phase under EKG gating and after intravenous administration of contrast agent. We evaluated the shunt patency, anatomy of intrapericardial pulmonary artery, parenchymal pulmonary vessels and background lung attenuation for the pulmonary blood flow, and the presence of systemic arterial and venous collaterals. Angiography (n=12) and echocardiography (n=16) were used as the gold standards. EBCT was consistent with angiogram in detecting the shunt patency and in depicting the anatomy of the intrapericardial pulmonary artery. Occlusion of the BT shunts was not detected in 2 patients by echocardiography. Diffuse or focally decreased pulmonary flow on EBCT in 8 patients was consistent with the pulmonary hemodynamics pattern revealed by cardiac catheterization. Uneven attenuation between lobes was related with multifocal supply of pulmonary flow or occlusion of lobar pulmonary arteries. Systemic collateral arteries were observed in 5 at the corresponding site of the decreased pulmonary flow. Systemic venous collaterals seen in all patients following BCPS were eventually draining to the inferior vena cava in 5 and to the left atrium in 2. EBCT provided accurate information of the pulmonary vascular system after shunt and has unique advantage over echocardiography in assessing patency of BT shunt or unifocalization tubes within the pleural space, the estimation of regional difference in pulmonary hemodynamics, and the detection of systemic collateral vessels. Therefore EBCT may provide useful information about the timing of definitive correction and the need for a second shunt or an interventional procedure prior to total repair.
Child ; Child, Preschool ; Collateral Circulation ; Female ; Heart Defects, Congenital/physiopathology/*radiography/*surgery ; Human ; Infant ; Male ; Pulmonary Artery/*surgery ; Pulmonary Circulation ; Subclavian Artery/*surgery ; *Tomography, X-Ray Computed

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

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