scholarly journals 3D Printed Personalized External Aortic Root Model in Marfan Syndrome with Isolated Sinus of Valsalva Aneurysm Caused by a Novel Pathogenic FBN1 p.Gly1127Cys Variant

Diagnostics ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 1057
Author(s):  
Jung Sun Cho ◽  
Joonhong Park ◽  
Jong Bum Kwon ◽  
Dae-Won Kim ◽  
Mahn-Won Park
Keyword(s):  
Coronary Artery ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Amino Acid Position ◽  
Cardiovascular Complications ◽  
Patient Specific ◽  
Sinus Of Valsalva ◽  
Sinus Of Valsalva Aneurysm ◽  
Root Model ◽  
3D Printed

The major cause of death in Marfan syndrome (MFS) is cardiovascular complications, particularly progressive dilatation of the proximal aorta, rendering these patients at risk of aortic dissection or fatal rupture. We report a 3D printed personalized external aortic root model for MFS with an isolated sinus of Valsalva aneurysm caused by a novel pathogenic FBN1 variant. A 67-year-old female with a history of lens dislocation and retinal detachment in the left eye was admitted for the evaluation of resting dyspnea several months prior. Transesophageal and transthoracic echocardiography revealed severe aortic valve regurgitation and a large left coronary sinus of Valsalva aneurysm in the proband. Sanger sequencing identified a heterozygous p.Gly1127Cys variant in the FBN1 gene; previously, a mutation at this amino acid position was described as pathogenic (p.Gly1127Ser; rs137854468). A 3D printed personalized external aortic root model based on a multidetector computed tomography scan was constructed to illustrate the location of the ostium of the left main coronary artery on the aneurysm of the left coronary artery cusp. Aortic root replacement with the Bentall procedure matched the exact shape of the 3D printed model. Creation of a 3D printed patient-specific model could be useful in facilitating the development of next-generation medical devices and resolving the risks of postoperative complications and aortic root disease.

scholarly journals A Case of Partial Aortic Root Remodeling for the Right Sinus of Valsalva Aneurysm with an Anomalous Origin of the Coronary Artery

2012 ◽  
Vol 41 (2) ◽  
pp. 70-75
Author(s):  
Takayuki Ueno ◽  
Kazuhisa Matsumoto ◽  
Kosuke Mukaihara ◽  
Kenji Toyokawa ◽  
Tomoyuki Matsuba ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Aortic Root ◽  
Anomalous Origin ◽  
Sinus Of Valsalva ◽  
Sinus Of Valsalva Aneurysm ◽  
Aortic Root Remodeling ◽  
The Right

scholarly journals “Sleeve” Sinus Valsalva Repair in Patients with Acute Type A Aortic Dissection

2020 ◽  
Author(s):  
Laichun Song ◽  
Yang Gao ◽  
Ming Xu ◽  
Bo Wang ◽  
Xiaoyong Li ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Coronary Artery ◽  
Aortic Dissection ◽  
Coronary Sinus ◽  
Computed Tomography Angiography ◽  
Aortic Root ◽  
Acute Type ◽  
Sinus Of Valsalva ◽  
Type A Aortic Dissection ◽  
Left And Right

Purpose. The optimal surgical strategy of aortic root in acute type A aortic dissection (ATAAD) is controversial. The aim of this study was to evaluate the feasibility and safety of “Sleeve” sinus Valsalva repair for AAD limited to the non-coronary sinus or partial left and right coronary sinus without involvement coronary artery ostia. Methods: From Sep 2016 to Mar 2019, 20 patients with AAD involving non-coronary sinus or partial left and right coronary sinus Valsalva underwent “Sleeve” sinus Valsalva repair . Multi slice spiral computed tomography angiography (MSCT) and three dimensional reconstruction were routinely performed in all patients to assess the maximal diameters of each segment of the aorta. Results. There was no early death in hospital and one death during the 30-day postoperative period. Re-thoracotomy due to bleeding was necessary in only 1 patient and no bleeding was related to the proximal anastomosis. The post-operative drainage was 390.5±229.3mL. During the following-up, the echocardiography showed the normal sinus of Valsalva and aortic valvular function. The computed tomography angiography showed normal aortic root without endovascular leak or dissection around the sinus of Valsalva. All patients were free from reoperation. Conclusions. “Sleeve” sinus Valsalva repair with Dacron patch for aortic dissection limited to the non-coronary sinus or partial left and right coronary sinus without involvement coronary artery ostia was technically feasible and safe.

scholarly journals Left main coronary artery compression by a left sinus of Valsalva aneurysm

2013 ◽  
Vol 16 (5) ◽  
pp. 713-714 ◽  
Author(s):  
José Martínez-Comendador ◽  
Javier Gualis ◽  
Carlos Esteban Martín ◽  
Alberto Santamaria
Keyword(s):  
Coronary Artery ◽  
Left Main Coronary Artery ◽  
Sinus Of Valsalva ◽  
Left Main ◽  
Sinus Of Valsalva Aneurysm ◽  
Left Sinus Of Valsalva

Coronary Artery Complications Related to Aortic Root Replacement in Marfan Syndrome

2021 ◽  
Author(s):  
Santo K. Ricceri ◽  
Tami J. Bang ◽  
Amanda Goodwin ◽  
Amber Khanna ◽  
Daniel W. Groves ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Aortic Root Replacement

Rare conditions presenting in adulthood

2017 ◽  
Author(s):  
Sara Thorne ◽  
Sarah Bowater
Keyword(s):  
Coronary Artery ◽  
Pulmonary Artery ◽  
Left Coronary Artery ◽  
Sinus Of Valsalva ◽  
Coronary Anomalies ◽  
Arteriovenous Fistulae ◽  
Sinus Of Valsalva Aneurysm

This chapter discusses rare conditions presenting in adulthood, including coronary anomalies (left coronary artery from pulmonary artery [LCAPA], congenital coronary arteriovenous fistulae) and sinus of Valsalva aneurysm.

scholarly journals Severe Dilatation of Coronary Artery Ostium Complicating Sinus of Valsalva Aneurysm: Differential Diagnosis and Review of the Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Paul Bamford ◽  
Nicholas Collins
Keyword(s):  
Differential Diagnosis ◽  
Coronary Artery ◽  
Right Coronary Artery ◽  
Surgical Intervention ◽  
Sinus Of Valsalva ◽  
Sinus Of Valsalva Aneurysm ◽  
Unusual Manifestation ◽  
The Right ◽  
Underlying Pathophysiology

Coronary artery dilatation may be due to various aetiologies including congenital anomalies, atherosclerotic coronary disease, and Kawasakis disease. We describe a case characterised by apparent severe dilatation of the right coronary artery ostium in an asymptomatic male. Subsequent imaging and surgical intervention documented the presence of a sinus of Valsalva aneurysm extending into the ostium of the right coronary artery. This represents an unusual manifestation of a sinus of Valsalva aneurysm. The underlying pathophysiology, differential diagnosis, role of surgical management, and outcomes are discussed.

scholarly journals Short segment coronary artery bypass grafting using radial artery to repair bilateral coronary ostial aneurysms after aortic root replacement in a patient with Marfan syndrome

2021 ◽  
Author(s):  
Kosuke Saku ◽  
Kazuyoshi Takagi ◽  
Tomofumi Fukuda ◽  
Eiki Tayama ◽  
Hiroyuki Tanaka
Keyword(s):  
Coronary Artery ◽  
Coronary Artery Bypass Grafting ◽  
Coronary Artery Bypass ◽  
Marfan Syndrome ◽  
Radial Artery ◽  
Aortic Root ◽  
Artery Bypass ◽  
Aortic Root Replacement ◽  
Bypass Grafting ◽  
Artery Bypass Grafting

Coronary ostial aneurysm is one of the complications after aortic root replacement especially in Marfan syndrome. How to reconstruct the coronary arteries is important problems during reoperation. Herein, we report a case of coronary artery bypass grafting using radial artery to repair bilateral coronary ostial aneurysms after aortic root replacement in a patient with Marfan syndrome.

scholarly journals Rare Case of Ruptured Sinus of Valsalva: Ruptured Into Left Ventricular Cavity

1970 ◽  
Vol 4 (1) ◽  
pp. 96-98
Author(s):  
SDM Taimur ◽  
SR Khan ◽  
MR Karim ◽  
MH Rahman
Keyword(s):  
Left Ventricle ◽  
Aortic Root ◽  
Rare Case ◽  
Interventricular Septum ◽  
Rare Condition ◽  
Left Ventricular ◽  
Left Ventricular Cavity ◽  
Sinus Of Valsalva ◽  
Sinus Of Valsalva Aneurysm ◽  
Left Sinus Of Valsalva

Rupture sinus of Valsalva is a relatively rare condition. We report a rare case of ruptured left sinus of valsalva with aneurysm, presenting with acute left ventricular failure. Transthoracic echocardiography showed an aneurysmal dilatation of the base of interventricular septum and part of the aortic root and a marked turbulent flow from the aortic root to the left ventricle with a continuous systolodiastolic shunting. The patient underwent successful repair of ruptured sinus of valsulva with closure of fistula. During Ventriculotomy the defect was repaired using pledgeted 5/ 0 prolene interrupted sutures and pericardial patch. Key Words: Rupture sinus of valsalva; Aneurysm; Left ventricle DOI: http://dx.doi.org/10.3329/cardio.v4i1.9398 Cardiovasc. J. 2011; 4(1): 96-98

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