| bronchopulmonary sequestration (BPS) is usually a rare congenital anomaly, which is most frequently extralobar or intralobar. The case of a patient with positional congenital anomaly--dextrocardia (situs thoracalis inversus) and intrapulmonary sequestration (IPS) is presented. Clinical and radiological characteristics of EPS and IPS are discussed, and new combinations of congenital anomalies with bronchopulmonary sequestration are described, dextrocardia and intrapulmonary sequestration. The importance of the algorithm of diagnostic examinations is emphasized, from detection of bronchopulmonary sequestration on the chest roentgenogram to establishing a definite diagnosis by means of angiography. ( info) |
2/163. Peters' anomaly: dominant inheritance in one pedigree and dextrocardia in another. Two case reports are described to illustrate the unusual occurrence of dominant inheritance of Peters' anomaly and the concomitant occurrence of Peters' anomaly with colobomatous microphthalmos and dextrocardia. Studies of additional families are necessary to determine conclusively the pathogenesis, genetic mode of inheritance, ocular and systemic associated malformations, and proper management of this complex entity. ( info) |
3/163. Accessory mitral valve tissue causing severe subaortic stenosis with dextrocardia in a premature newborn. We report an unusual case of left-ventricular outflow obstruction caused by accessory mitral valve tissue associated with dextrocardia and ventricular septal defect in a seven-day-old, 2200 grams premature infant, who was referred with a heart murmur. The diagnosis was made by two-dimensional and Doppler echocardiography which demonstrated the accessory tissue as well as a 100 mmHg peak systolic gradient between the left ventricle and the aorta. Ten days after the presentation the infant underwent emergency surgery after respiratory arrest and recurrent episodes of syncope. The accessory mitral valve tissue and its fibrous extension were excised and the ventricular septal defect was closed. We believe that surgical treatment of patients with accessory mitral valve tissue should be performed early because of the possibility of acute deterioration. Combined aortotomy and interatrial approach is very helpful in evaluating the anatomy and the mitral valve function as well as delineating the tissue to be excised. ( info) |
4/163. Neonatal arterial switch operation for transposition of the great ateries in a patient with mirror image dextrocardia and situs inversus totalis. The neonatal arterial switch operation has become the standard therapy for D-transposition of the great arteries in the absence of left ventricular outflow tract obstruction. We describe our experience of successful arterial switch operation after balloon atrial septostomy in a 5-day-old infant girl who had atrial and visceral situs inversus totalis, mirror image dextrocardia, and D-transposition of the great arteries. To our knowledge, ours is the first report of this operation in a patient with this anatomy. ( info) |
5/163. Omental flap for mediastinitis after median sternotomy in asplenia syndrome and gut malrotation. We describe a 12-year-old patient with asplenia syndrome and gut malrotation who, after an interim step before a modified Fontan operation, developed life-threatening mediastinitis. A flap of the omentum arising from the transverse colon, longitudinally located in the left of the abdomen, was created and transferred to the mediastinum after a division of the arterial arcade at its most caudal extent. Her postoperative course was uneventful, and 12 months later, the modified Fontan operation was successfully completed. Although visceral heterotaxy results in an omental deformation, it does not preclude the use of an omental flap. ( info) |
| This is a case report of an unusual case of a patient with dextrocardia and "situs inversus totalis" who presented with unstable angina. coronary angiography revealed severe main stem and severe triple vessel coronary artery disease. The patient later underwent successful emergency coronary artery bypass graft surgery. To the authors' knowledge this is the first reported case in malaysia and also, the first ever report in the literature of multiple vessel coronary artery grafting, including the use of the right internal mammary artery. ( info) |
7/163. Fetal diagnosis of atrioventricular septal defect with dextrocardia in trisomy 18. On prenatal ultrasound atrioventricular septal defect is often diagnosed in infants with down syndrome based on an abnormal four-chamber view of the heart. A unique case of atrioventricular septal defect with dextroposition of the heart in an infant with trisomy 18 is presented. ( info) |
| A patient with dextrocardia who suffered his first myocardial infarction after approximately 26 hours of a diagnostic sleep deprivation protocol is described. The infarction started about 3 hours after a significant improvement in mood, which persisted during and after infarction. Total sleep deprivation may be an acute risk factor for myocardial infarction. ( info) |
9/163. Left lower quadrant pain of unusual cause. The differential diagnosis of left lower quadrant abdominal pain in an adult man includes, among others, sigmoid diverticulitis; leaking abdominal aortic aneurysm; renal colic; epididymitis; incarcerated hernia; bowel obstruction; regional enteritis; psoas abscess; and in this rare instance, situs inversus with acute appendicitis. We report a case of situs inversus totalis with left-sided appendicitis and a brief review of the literature. There were several subtle indicators of total situs inversus present that were missed by the physicians and surgeons who initially evaluated the patient prior to surgery. Computed tomography scan with contrast, however, revealed the diagnosis immediately, and treatment was successfully initiated. ( info) |
| Marfan's syndrome is an inherited connective tissue defect that affects many organs, especially of the musculoskeletal, ophthalmic and cardiovascular systems, and may be associated with some rare conditions. Here, we report the first known case of Marfan's syndrome, combined with situs inversus totalis with dextrocardia and discrete subaortic stenosis and aortic insufficiency in a 22-year-old woman. ( info) |