Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome

Alport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, corn...

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Main Authors: Amornpol Anuwatworn, Prince Sethi, Kelly Steffen, Orvar Jonsson, Marian Petrasko
Format: Article
Language:English
Published: Hindawi Limited 2017-01-01
Series:Case Reports in Cardiology
Online Access:http://dx.doi.org/10.1155/2017/1705927
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spelling doaj-8da711ac323341b7b6ad19a4b66c1d4f2020-11-24T20:54:10ZengHindawi LimitedCase Reports in Cardiology2090-64042090-64122017-01-01201710.1155/2017/17059271705927Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport SyndromeAmornpol Anuwatworn0Prince Sethi1Kelly Steffen2Orvar Jonsson3Marian Petrasko4University of South Dakota Sanford School of Medicine, Sanford USD Medical Center, Sanford Cardiovascular Institute, Sioux Falls, SD, USAUniversity of South Dakota Sanford School of Medicine, Sanford USD Medical Center, Sanford Cardiovascular Institute, Sioux Falls, SD, USAUniversity of South Dakota Sanford School of Medicine, Sanford USD Medical Center, Sanford Cardiovascular Institute, Sioux Falls, SD, USAUniversity of South Dakota Sanford School of Medicine, Sanford USD Medical Center, Sanford Cardiovascular Institute, Sioux Falls, SD, USAUniversity of South Dakota Sanford School of Medicine, Sanford USD Medical Center, Sanford Cardiovascular Institute, Sioux Falls, SD, USAAlport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina. The resulting disorder includes hereditary nephritis, corneal opacities, anterior lenticonus, fleck retinopathy, temporal retinal thinning, and sensorineural deafness. Aortic and aortic valve pathologies have been described as extrarenal manifestations of AS in multiple case reports. One case report described intramural hematoma of the coronary artery. We report the first case of true spontaneous coronary artery dissection (SCAD) with an intimal flap as a very rare manifestation of AS. The patient is a 36-year-old female with history of AS with chronic kidney disease, hypertension, and obesity who presented to the emergency room with acute onset of substernal chest pain radiating to her neck and arms. Troponin was elevated, and ECG showed transient 1 mm ST-segment elevation in the inferior leads. Subsequent coronary angiography revealed localized dissection of the left circumflex artery. Percutaneous coronary angioplasty was performed and her symptoms improved. This case illustrates that SCAD may be a manifestation of AS patients with chest pain.http://dx.doi.org/10.1155/2017/1705927
collection DOAJ
language English
format Article
sources DOAJ
author Amornpol Anuwatworn
Prince Sethi
Kelly Steffen
Orvar Jonsson
Marian Petrasko
spellingShingle Amornpol Anuwatworn
Prince Sethi
Kelly Steffen
Orvar Jonsson
Marian Petrasko
Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome
Case Reports in Cardiology
author_facet Amornpol Anuwatworn
Prince Sethi
Kelly Steffen
Orvar Jonsson
Marian Petrasko
author_sort Amornpol Anuwatworn
title Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome
title_short Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome
title_full Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome
title_fullStr Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome
title_full_unstemmed Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome
title_sort spontaneous coronary artery dissection: a rare manifestation of alport syndrome
publisher Hindawi Limited
series Case Reports in Cardiology
issn 2090-6404
2090-6412
publishDate 2017-01-01
description Alport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina. The resulting disorder includes hereditary nephritis, corneal opacities, anterior lenticonus, fleck retinopathy, temporal retinal thinning, and sensorineural deafness. Aortic and aortic valve pathologies have been described as extrarenal manifestations of AS in multiple case reports. One case report described intramural hematoma of the coronary artery. We report the first case of true spontaneous coronary artery dissection (SCAD) with an intimal flap as a very rare manifestation of AS. The patient is a 36-year-old female with history of AS with chronic kidney disease, hypertension, and obesity who presented to the emergency room with acute onset of substernal chest pain radiating to her neck and arms. Troponin was elevated, and ECG showed transient 1 mm ST-segment elevation in the inferior leads. Subsequent coronary angiography revealed localized dissection of the left circumflex artery. Percutaneous coronary angioplasty was performed and her symptoms improved. This case illustrates that SCAD may be a manifestation of AS patients with chest pain.
url http://dx.doi.org/10.1155/2017/1705927
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