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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| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Cardiology |
| Online Access: | http://dx.doi.org/10.1155/2017/1705927 |
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| author | Amornpol Anuwatworn Prince Sethi Kelly Steffen Orvar Jonsson Marian Petrasko |
| author_facet | Amornpol Anuwatworn Prince Sethi Kelly Steffen Orvar Jonsson Marian Petrasko |
| author_sort | Amornpol Anuwatworn |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-8da711ac323341b7b6ad19a4b66c1d4f |
| institution | Kabale University |
| issn | 2090-6404 2090-6412 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
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| series | Case Reports in Cardiology |
| spelling | doaj-art-8da711ac323341b7b6ad19a4b66c1d4f2025-02-03T01:12:59ZengWileyCase 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 |
| 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 |
| title | 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_short | Spontaneous Coronary Artery Dissection: A Rare Manifestation of Alport Syndrome |
| title_sort | spontaneous coronary artery dissection a rare manifestation of alport syndrome |
| url | http://dx.doi.org/10.1155/2017/1705927 |
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