confirmation of congenital heart lesions in the foetus, the care of children with acquired heart disease and the investigation of innocent murmurs of childhood. There is a local perception that the demand for these services far exceeds the available skills in South Africa. In addition to the cardiological needs of our population, the need for surgery for children with heart lesions appears
Subvalvular aneurysms of the left ventricle are very rare and often the cause is uncertain. Very little data in the literature describes subvalvular aneurysms in children and most of the data are derived from case reports. We describe a unique case of a human immunodeficiency virus negative child with a tuberculous subaortic aneurysm observed at different stages of development by serial transthoracic echocardiography. The patient underwent successful cardiac surgery after the initial conservative treatment for tuberculosis (TB).A 3-year-old black boy who was negative for human immunodeficiency virus presented to our institution with a 1-week history of coughing, fever, night sweats, and generalized body swelling. On examination he appeared chronically ill and was underweight for his age. He had generalized lymphadenopathy in the axillary, supraclavicular, cervical, and submandibular regions. He had ascites, hepatomegaly (liver span, 12 cm), and splenomegaly (3 cm below costal margin). The boy was tachypneic with a respiratory rate of 50 breaths/min. He had intercostal and subcostal recession and audible bilateral rhonchi. His heart rate was150 beats/min, with a gallop rhythm present and with muffled heart sounds. There were no murmurs, but he was in congestive cardiac failure. His blood pressure was 80/40 mm Hg.A chest roentgenogram demonstrated cardiomegaly, a widened mediastinum, and paratracheal lymphadenopathy. The C-reactive protein, lactate dehydrogenase levels, and erythrocyte sedimentation rate were elevated. Echocardiography (Fig 1) revealed normal intracardiac anatomy with a large organized and loculated pericardial effusion. A biopsy of a supraclavicular lymph node demonstrated caseating necrosis with sites of granulomatous inflammation. Epitheloid cell granulomas and Langhans giant cells were also seen. The Ziehl Neelsen stain highlighted occasional acid and alcohol fast bacilli, which confirmed the TB. There were no features of lymphoma or malignancy present. Mycobacterium TB was also isolated on cultures of gastric aspirates. A diagnosis of disseminated TB with organized TB pericarditis was made.
Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries L PEPETA, FF TAKAWIRA, PE ADAMS, NH NTSINJANA, BJ MITCHELL, AM CILLIERS AbstractWe report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery.Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.Keywords: pulmonary atresia with ventricular septal defect, anomalous origin of pulmonary artery, collateral arteries, pulmonary hypertension, branchial arches, CATCH22 syndrome An anomalous origin of the pulmonary artery from the aorta (AOPA) is very rare, with the first case of this nature reported by Fraentzel in 1868. 1 The incidence of anomalous left pulmonary artery from the aorta (AOLPA) was reported in one study to be around 17%, 1 with anomalous origin of the right pulmonary artery (AORPA) from the ascending aorta as the more common variant of AOPA, seen in about 83% of cases. Without surgery, the mortality in patients with AOPA is extremely high, reaching as high as 80%. [2][3][4][5] This is due to severe, rapidly progressive pulmonary vascular disease and congestive cardiac failure. Both isolated AOLPA and that associated with other defects have been well described. 1,6,7 The commonest associated congenital cardiac lesion is tetralogy of Fallot, which is seen in about 75% of cases. 1 AOLPA is also associated with a right arch of the aorta in 63% of cases, and in about 38% of cases, an anomalous origin of the right subclavian artery from the descending aorta has been reported as well. 1 Of note, both our cases had a left aortic arch and had no main and right pulmonary arteries, with the only source of blood supply to the right lung being partially stenosed major aorto-pulmonary collateral arteries (MAPCAs).To the best of our knowledge, this series repr...
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.