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Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report

INTRODUCTION: Single ventricle and truncus arteriosus are both rare congenital cardiac syndromes with limited survival. Their occurrence together is extremely uncommon and prolonged survival is exceptionally rare. We present the case of a patient who had both of these defects with survival to age 45...

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Autores principales: Porter, Inna, Vacek, James
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2008
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2424060/
https://www.ncbi.nlm.nih.gov/pubmed/18513397
http://dx.doi.org/10.1186/1752-1947-2-184
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author Porter, Inna
Vacek, James
author_facet Porter, Inna
Vacek, James
author_sort Porter, Inna
collection PubMed
description INTRODUCTION: Single ventricle and truncus arteriosus are both rare congenital cardiac syndromes with limited survival. Their occurrence together is extremely uncommon and prolonged survival is exceptionally rare. We present the case of a patient who had both of these defects with survival to age 45. CASE PRESENTATION: We describe the vase of a 45-year-old man with the unusual occurrence of two very rare congenital cardiac defects. He was found to have both truncus arteriosus and single ventricle with long survival. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today, this case does indicate the efficacy of the management options available at the time and place of the patient's contacts with the medical care system in Belarus. We discuss the findings, frequency, classification, and management of both of these congenital defects. CONCLUSION: This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment. As the management of these patients is constantly evolving, and interventional techniques are improving, patients such as this with prolonged survival will be more common, with each case providing insights to future treatment. Challenges in management may include prior care provided in health care systems with limited resources.
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spelling pubmed-24240602008-06-11 Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report Porter, Inna Vacek, James J Med Case Reports Case Report INTRODUCTION: Single ventricle and truncus arteriosus are both rare congenital cardiac syndromes with limited survival. Their occurrence together is extremely uncommon and prolonged survival is exceptionally rare. We present the case of a patient who had both of these defects with survival to age 45. CASE PRESENTATION: We describe the vase of a 45-year-old man with the unusual occurrence of two very rare congenital cardiac defects. He was found to have both truncus arteriosus and single ventricle with long survival. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today, this case does indicate the efficacy of the management options available at the time and place of the patient's contacts with the medical care system in Belarus. We discuss the findings, frequency, classification, and management of both of these congenital defects. CONCLUSION: This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment. As the management of these patients is constantly evolving, and interventional techniques are improving, patients such as this with prolonged survival will be more common, with each case providing insights to future treatment. Challenges in management may include prior care provided in health care systems with limited resources. BioMed Central 2008-05-30 /pmc/articles/PMC2424060/ /pubmed/18513397 http://dx.doi.org/10.1186/1752-1947-2-184 Text en Copyright © 2008 Porter and Vacek; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Porter, Inna
Vacek, James
Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report
title Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report
title_full Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report
title_fullStr Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report
title_full_unstemmed Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report
title_short Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report
title_sort single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2424060/
https://www.ncbi.nlm.nih.gov/pubmed/18513397
http://dx.doi.org/10.1186/1752-1947-2-184
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