Clinicopathologic findings in congenital aneurysms of the great vessels

American Journal of Medical Genetics
L C AdèsD M Milewicz

Abstract

We describe the clinical, histopathologic, and angiographic findings in four children with congenital abnormalities of the great vessels of unknown cause, comprising either single or multiple arterial aneurysms, aortic/arterial dilatation, vessel tortuosity, or combinations of these abnormalities. Two children had early and severe respiratory distress due to aneurysmal compression of the trachea. All children had diffuse dilatation of several arteries, and two children also had tortuosity of multiple arteries. Progression of these abnormalities was clearly evident in one child, in whom diffuse vessel irregularity and tortuosity affected intra-abdominal, and intra and extra-cranial arteries. One child died at 5 years, while the other three have undergone successful surgical repair in the first 3 months of life and are now well, between age 2.5 and 7 years. The phenotype of each child appears unique but all have in common the rare finding of aneurysms of the aorta and main pulmonary artery. Congenital aortic aneurysms did not occur as an isolated finding in any of these children.

References

Aug 1, 1976·The Journal of Pediatrics·S ChenV L Willman
Sep 1, 1979·Chest·F J FrickerD B Lerberg
Jul 1, 1978·The British Journal of Surgery·D W Short
Jun 13, 1975·Pediatric Radiology·R V Dutton, E B Singleton
Dec 1, 1991·Journal of Pediatric Surgery·S A Saad, A May
Dec 1, 1987·The Annals of Thoracic Surgery·J P ChangM J Sheih
Dec 1, 1969·The American Journal of Medicine·S ImahoriJ C Brennan
May 18, 1967·The New England Journal of Medicine·M B Howorth
Sep 1, 1967·Circulation·A Ertugrul
Feb 1, 1983·Journal of Pediatric Surgery·M SchillerK Abu-Dalu
Apr 1, 1983·Journal of Pediatric Surgery·K TakayanagiS Kuwait
Apr 1, 1982·Archives of Surgery·G C HunterM Chvapil
Jan 1, 1981·The Journal of Pediatrics·P ProcopisD M Danks
Apr 1, 1994·Matrix Biology : Journal of the International Society for Matrix Biology·J F Bateman, S B Golub
Mar 29, 1996·American Journal of Medical Genetics·E A PutnamD M Milewicz
Apr 1, 1958·The American Journal of Medicine·H LEPOWO MUREN

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Citations

Sep 29, 2009·Orphanet Journal of Rare Diseases·Marco RitelliMarina Colombi
Nov 26, 2008·Cardiovascular Research·Chao-Hung ChengYuan-Tsong Chen
Sep 22, 2009·The Annals of Thoracic Surgery·Abdulaziz Al-KhaldiYasser Mohammed
Dec 10, 2013·Journal of Forensic Sciences·Theresa PowerRoger W Byard
Mar 14, 2007·Heart, Lung & Circulation·Sylvio C ProvenzanoPeter W Grant
Jul 22, 2008·The Annals of Thoracic Surgery·Roland HetzerVladimir Alexi-Meskishvili
Feb 24, 2017·Journal of Forensic Sciences·Roger W Byard
Apr 1, 2004·American Journal of Medical Genetics. Part a·Rita GardellaMarina Colombi
Jan 13, 2018·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Aude BeyensBert Callewaert
Jul 15, 2009·Pediatric Cardiology·M PilatiG Puppini
Oct 18, 2019·Antioxidants & Redox Signaling·Annekatrien BoelÉva Margittai
Apr 9, 2020·International Journal of Molecular Sciences·Jorge NuchePilar Escribano Subías
Nov 6, 2004·American Journal of Medical Genetics. Part a·Marja W WesselsPatrick J Willems
Sep 14, 2004·Journal of Vascular and Interventional Radiology : JVIR·Marco A CuraAlan Benvenisty

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