PubMed · 9117612
The source did not provide an abstract. Follow the original record for more information.
Explore connections, maps & timelines
T Ikeda, T Ban. 1996. [Persistent fifth aortic arch].. https://pubmed.ncbi.nlm.nih.gov/9117612/
Cite the original work for its findings. Save a collection to share your selection of sources.
KEEP EXPLORING
Interrupted aortic arch (IAA) type B is a congenital heart defect believed to be caused by an anomaly of bronchial arch mesenchymal development. IAA type B has been associated with DiGeorge syndrome (DGS), which includes conotruncal heart defects, T-cell immunodeficiency, hypocalcemia, and facial abnormalities. The great majority of DGS cases are associated with hemizygous deletions at the chromosome 22q11 locus. The present study was designed to establish the involvement of the 22q11 locus in the etiology of IAA type B, independently from the typical DGS phenotype. An evaluation was performed on 73 patients with conotruncal heart defects using fluorescence in situ hybridization (FISH) analysis with probes from the 22q11 DGS locus. From this group, 7 patients were deleted (including 4 of the 11 patients with IAA type B). FISH analysis was extended to a total of 22 patients with IAA type B and 11 of these (50%) were deleted. FISH and Southern blot analyses using additional markers within the DiGeorge chromosomal region were performed on patients found not to be deleted in the initial FISH screening. No small deletions or rearrangements were detected. In our patient population, a single, specific genetic defect is the basis for one half of the IAA type B cases. These data suggest that IAA type B is one of the most etiologically homogeneous congenital heart defects. A 22q11 deletion in IAA type B may or may not be associated with the typical DGS phenotype. Therefore, IAA type B, per se, should be an indication for 22q11 deletion testing.
Vascular compression of the airway is a significant cause of respiratory compromise in children. While the indications for surgical repair are sometimes life threatening, they can also be subtle. This retrospective study examines 45 surgical cases of tracheobronchial compromise secondary to vascular compression at a large children's hospital between July 1983 and February 1996. A total of 34 were diagnosed with innominate artery compression, ten with a double aortic arch and one with an anomalous right subclavian artery. The 45 patients, 25 male and 20 female, ranged in age from 12 days to 11 years at surgery (average 13 months). A total of 21 (47%) presented with proven or suspected episodes of cyanosis or apnea. All 45 patients had evidence of vascular compression during microlaryngoscopy and bronchoscopy. The diagnosis was confirmed by magnetic resonance imaging (MRI) in 23/45 (51%), barium swallow in 22/45 (49%) and aortogram in 3/45 (7%). There was one death. One patient had a tracheotomy before surgery and continues to require it after surgery. Complete resolution of symptoms was achieved in 39/45 (87%) with five requiring more than one operation before their symptoms resolved completely. A total of four patients experienced a recurrence of symptoms within a variable length of time after surgery. Surgical indications and treatment alternatives will be discussed.
PURPOSE: To review recent developments in the diagnosis, clinical epidemiology, pathology, and management of atherosclerosis of the thoracic aorta, especially atherosclerosis of the thoracic aorta as a source of embolization. DATA SOURCES: MEDLINE searches, bibliographies of published papers, and consultation with experts in the field. STUDY SELECTION: English-language publications on atherosclerosis of the thoracic aorta were selected. DATA SYNTHESIS: During the last 6 years, the increasing use of transesophageal echocardiography has shown that atherosclerotic plaque in the thoracic aorta is a source of otherwise unexplained embolic events, including stroke, transient ischemic attack, and peripheral emboli. Retrospective studies have documented a strong independent association between larger lesions (4 mm to 5 mm) and previous embolic disease, and prospective studies have shown that patients with these lesions have a high risk for future events (in one study, the risk for stroke was 12%; in another, the risk for cerebral or peripheral events was 33% in a follow-up period of just 14 months). These lesions also pose a serious risk for embolization caused by manipulation of the aorta during catheterization, intra-aortic balloon-pump placement, and cannulation of the aorta for heart surgery. Pathologic examination has shown atherosclerotic plaque, often with superimposed thrombi that account for the mobile components seen on transesophageal echocardiography. The management of patients who have atherosclerotic lesions in the thoracic aorta has not been determined prospectively. However, anticoagulation may help prevent emboli, as it does for patients who have thrombi in other locations, such as the left atrium and the left ventricle. CONCLUSIONS: Protruding atherosclerotic lesions in the thoracic aorta, often with superimposed mobile thrombi, are an important cause of embolic disease. Transesophageal echocardiography should be considered in the work-up of patients who have unexplained embolic events.