Unusual presentation of aortic disease in Marfan syndrome: Case report

Rowiński, Olgierd; Wojtaszek, Mikołaj; Milczarek, Krzysztof; Cieszanowski, Andrzej; Szmidt, J

doi:10.1016/j.ijcard.2007.04.034

Cited by 4 publications

(3 citation statements)

References 6 publications

(5 reference statements)

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“…2 Among the reported inherited conditions, such as type IV Ehler-Danlos, Marfan, and Loyes-Deitz syndromes, less than 3 visceral arteries were usually involved. 3 -5 Our patient presented with uncontrolled hypertension that was not previously diagnosed and had a history of smoking. Both are considered as risk factors that could have leaded to her presentation.…”

Section: Discussionmentioning

confidence: 92%

“…Various diseases are associated with visceral arterial dissections, including connective tissue diseases (Ehler-Danlos syndrome, Marfan syndrome), Loeys-Dietz syndrome, fibromuscular dysplasia (FMD), and segmental arterial mediolysis. [3][4][5][6][7] Therefore, targeted clinical diagnostic testing and molecular workup is suggested for patients with SMAD.…”

Section: Introductionmentioning

confidence: 99%

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A Rare Case of 7 Simultaneous Arterial Dissections and Review of The Literature

Komem

Halevy

Griton

et al. 2019

Vasc Endovascular Surg

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Objective: Spontaneous multiple artery dissection is a relatively rare phenomenon. Early clinical signs are often nonspecific, making it difficult to diagnose. Case Report: This is a case of a 51-year-old female who presented with spontaneous dissection of 4 visceral arteries, both iliac arteries, and of the right internal carotid artery. The patient underwent urgent successful endovascular repair. Later complications included acute respiratory distress syndrome and pneumonia after massive blood transfusion. She recovered gradually and was discharged after 21 days. Due to this rare presentation, genetic investigation was performed in search of a connective tissue disorder. Results revealed a new COL3A1 subtype mutation. The pathogenicity of this variant remains unclear. Conclusion: We recommend a high index of suspicion for visceral artery dissection in the differential diagnosis for abdominal pain with concurrent uncontrolled hypertension. Early diagnosis and intervention are crucial to reducing the mortality rate.

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Section: Discussionmentioning

confidence: 92%

Section: Introductionmentioning

confidence: 99%

A Rare Case of 7 Simultaneous Arterial Dissections and Review of The Literature

Komem

Halevy

Griton

et al. 2019

Vasc Endovascular Surg

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“…The use of complex endovascular aortic repair in postdissection aneurysms with fenestrated and branched endografts is increasingly in development but currently implemented by a few centers only 9, 10. Therefore, establishing novel, less-invasive therapies for these patients seems mandatory 11 . The technique for sutureless revascularization of the visceral and renal arteries as an off-label use of otherwise approved hybrid vascular grafts seems a promising treatment option for these high-risk patients.…”

Section: Discussionmentioning

confidence: 99%

Open repair of an aortic aneurysm in a patient with Loeys-Dietz syndrome using Gore hybrid vascular branch grafts

Wipper

Tsilimparis

Kölbel

et al. 2015

Journal of Vascular Surgery Cases

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A 44-year-old woman with Loeys-Dietz syndrome (transforming growth factor-b [TGFBR2] gene mutation) presented with a retrograde type B dissection. She developed rapid expansion of the thoracoabdominal aortic false lumen aneurysm. The patient was successfully treated with open thoracoabdominal repair using Gore Hybrid Vascular Grafts (W. L. Gore and Assoc, Flagstaff, Ariz) for revascularization of the celiac trunk, the superior mesenteric artery, and both renal arteries. Follow-up imaging documented patency for all visceral branches. The described off-label use for sutureless revascularization might be a fast, simple, and reliable solution for revascularization during open thoracoabdominal repair. Furthermore, anastomotic aneurysm in patients with connective tissue disease might be prevented by sutureless anastomosis. (J Vasc Surg Cases 2015;1:69-72.) Surgical treatment of aneurysms in patients with genetic aortic syndromes (GAS) is challenging. We present a patient with GAS who was successfully treated with open thoracoabdominal repair using Gore Hybrid Vascular Grafts (GHVGs; W. L. Gore and Assoc, Flagstaff, Ariz) for revascularization of the celiac trunk, the superior mesenteric artery, and both renal arteries. CASE REPORTA 44-year-old woman with suspected GAS and retrograde type B aortic dissection after a ruptured infrarenal aortic aneurysm developed enlarged false lumen aneurysm of the thoracoabdominal aorta. Furthermore, a penetrating aortic ulcer (PAU) had developed at the junction of aortic arch and descending aorta on the minor curvature as well as additional aneurysms of two intercostal arteries and both internal mammary arteries, with a maximal diameter of 19 mm (Fig 1, A).In the referring hospital, the ruptured infrarenal aortic aneurysm had been replaced by a 16-mm Dacron (DuPont, Wilmington, Del) tube graft in an emergency operation under intermittent manual and medicamentous cardiopulmonary resuscitation due to hemodynamic instability. A periprocedural retrograde type B dissection occurred postoperatively.The patient was then transferred to our institution and was initially administered conservative antihypertensive medication. Because of the complicated vascular phenotype, we ordered testing for GAS.During the following 6 weeks, the patient experienced recurrent pain and progressing false lumen expansion (from 47 mm to 53 mm) and finally required immediate intervention (Fig 1, B). Owing to the high rupture risk, treatment could not await test results for GAS.Our interdisciplinary vascular board, consisting of specialists for open and endovascular surgery, cardiac surgery, angiology, cardiology, and connective tissue disease, including GAS, decided to proceed immediately by open thoracoabdominal aortic repair with reimplantation of the visceral branches. The time required to produce and deliver an individualized four-fenestrated endograft would have taken about 2 months, which was considered too long to wait for the repair of this rapidly progressing aneurysm. Moreover, because the patient was y...

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