Aortic root aneurysm. Diagnosis and treatment

Abstract Loeys–Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.

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Chronic Type A Aortic Dissection and Giant Aortic Root Aneurysm After Aortic Valve Replacement

Aorta ◽

10.12945/j.aorta.2016.15.028 ◽

2016 ◽

Vol 04 (03) ◽

pp. 108-110 ◽

Cited By ~ 2

Author(s):

Andrés Enríquez Puga ◽

Sara Castaño Rodríguez ◽

Blanca Mateos Pañero ◽

Beatriz Castaño Moreira ◽

Luis Fernando López Almodóvar

Keyword(s):

Valve Replacement ◽

Aortic Root ◽

Aortic Surgery ◽

Mechanical Valve ◽

Type A ◽

Chronic Type ◽

Type A Aortic Dissection ◽

Aortic Root Aneurysm ◽

Valve Conduit ◽

Type A Dissection

AbstractWe describe the case of a 61-year-old male with a giant aortic root aneurysm associated with chronic aortic Type A dissection. The patient had been operated on 16 years before due to aortic annuloectasia with mechanical valve replacement. The patient underwent revision aortic surgery with a Bentall-De Bono operation with Svensson modification, using a #21 On-X Valsalva mechanical valve conduit. The postoperative course was uneventful.

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The deadly “bulging balloon” within the mediastinum: a case of a giant aortic root aneurysm in a young patient with Marfan syndrome

10.21203/rs.3.rs-44721/v1 ◽

2020 ◽

Author(s):

JIAYU SHEN ◽

Changping Gan ◽

R.D.T. Rajaguru ◽

Dou Yuan ◽

ZHENGHUA XIAO

Keyword(s):

Decision Making ◽

Aortic Valve ◽

Ct Scan ◽

Young Patient ◽

Marfan Syndrome ◽

Aortic Root ◽

Treatment Decision ◽

Decision Making Process ◽

Aortic Root Aneurysm ◽

Treatment Decision Making

Abstract Introduction: Marfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age. Case: The patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed six-foot-three inches of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. The transthoracic echocardiography (TTE) and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we successfully performed the Bentall procedure based on the intraoperative exploration results. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later. Conclusion It is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. The composite replacement of the aortic valve and ascending aorta should be performed if the patient is not suitable for valve-sparing operation.

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Surgical treatment of aortic root aneurysm: comparison of Bentall procedure and David reimplantation of aortic valve

Vnitřní lékařství ◽

10.36290/vnl.2017.126 ◽

2017 ◽

Vol 63 (10) ◽

pp. 640-645

Author(s):

Jiří Ničovský ◽

Jiří Ondrášek ◽

Jan Černý ◽

Daniela Žáková ◽

Petr Němec

Keyword(s):

Surgical Treatment ◽

Aortic Valve ◽

Aortic Root ◽

Aortic Root Aneurysm ◽

Bentall Procedure

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Abstract 17228: Not Just Another Knick In The Wall: An Unusual Case Of Multiple Arterial Aneurysms

Circulation ◽

10.1161/circ.144.suppl_2.17228 ◽

2021 ◽

Vol 144 (Suppl_2) ◽

Author(s):

Vibha Amblihalli ◽

Ayita Ray ◽

Hafiz Khalid ◽

luigi pacifico

Keyword(s):

Subclavian Artery ◽

Aortic Root ◽

Left Atrial ◽

Unusual Case ◽

Surgical Intervention ◽

Patch Repair ◽

Atrial Wall ◽

Aortic Root Aneurysm ◽

Left Atrial Wall ◽

Arterial Aneurysms

Case Presentation: 47-year-old Liberian woman with a history of latent TB and no prior cardiac history presented with dyspnea, palpitations, and weight loss. She had fevers, tachycardia, and cervical lymphadenopathy. Cardiac exam showed widened pulse pressure, systolic and diastolic murmur, and features of heart failure. TTE showed dilated left ventricle with preserved ejection fraction, aortic root aneurysm compressing left atrium, severe aortic and mitral regurgitation, and moderate pericardial effusion with no tamponade. CT angiogram of neck, chest and abdomen showed right subclavian artery mycotic aneurysm, large left supraclavicular lymphadenopathy, multiple aortic arch, and descending thoracic aorta mycotic aneurysms. She underwent emergent surgical intervention. Intraoperative TEE revealed rupture of aortic root aneurysm into left ventricular outflow tract causing a fistula, perforated anterior mitral leaflet, and distortion of the left atrial wall. She underwent mitral and aortic tissue valve replacement, aortic root replacement, and a pericardial patch repair of the left atrial wall. Subsequently, she underwent right subclavian artery aneurysm resection, right carotid axillary bypass, and vertebral artery reimplantation. Aortic valve pathology was suggestive of endocarditis with negative cultures. Lymph node biopsy revealed non-necrotizing granulomatous inflammation with no evidence of acid-fast bacilli, fungi, and malignancy. Autoimmune workup was negative. A PET CT showed post-surgical inflammatory changes with no evidence of malignancy. Discussion: We describe an unusual case of multiple large arterial aneurysms causing severe valvular insufficiency requiring emergent surgical intervention. The patient underwent extensive workup which was unrevealing. She was treated for subacute bacterial endocarditis and suspected Bechet’s disease. Thus, the quest for a definitive diagnosis continues to elude us.

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Abstract 14908: Anatomic Validation of Induced Pluripotent Stem Cell-derived Aortic Smooth Muscle Cell Model of Loeys Dietz Syndrome

Circulation ◽

10.1161/circ.142.suppl_3.14908 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Albert J Pedroza ◽

Samantha Churovich ◽

Nobu Yokoyama ◽

Ken Nakamura ◽

Cristiana Iosef Husted ◽

...

Keyword(s):

Smooth Muscle ◽

Stem Cell ◽

Aortic Root ◽

Pluripotent Stem Cell ◽

Cell Model ◽

Expression Profiles ◽

Induced Pluripotent Stem Cell ◽

Aortic Root Aneurysm ◽

Ligand Expression ◽

Induced Pluripotent

Introduction: Mutations in TGF-beta (TGF-ß) signaling genes lead to aortic root aneurysm in Loeys Dietz syndrome (LDS). Smooth muscle cells (SMCs) in the proximal aorta develop from two embryologic origins: second heart field (SHF) and neural crest (NC). Induced pluripotent stem cell (iPSC) models simulate these lineages, but direct correlation to clinical disease is lacking. Hypothesis: iPSC-derived SMCs accurately model lineage-specific aortopathy in LDS. Methods: We generated SMC lines from root and ascending aortic surgical tissue and iPSC-derived SMCs through SHF and NC-specific pathways from an LDS patient ( TGFBR1 mutation). Lineage-specific TGF-ß responses were determined by western blot/ELISA. RNA sequencing and RT-PCR identified SMC transcriptomes. Results: Aortic root SMCs showed greater canonical TGF-ß activation (p-SMAD2/3) versus ascending at baseline and with TGF-ß stimulation ( Figure ). Synonymous results were seen in SHF versus NC SMCs from the iPSC pathway. RNAseq identified 1,600 differentially expressed genes between iPSC lineages, including altered TGF-ß receptor and ligand expression profiles. Primary aortic lines validated iPSC data: root SMCs showed enriched TGF-ß receptor 1/2/3 expression (1.7-, 3.9- and 5.9-fold) while ascending SMCs overexpressed TGFB1 and TGFB2 ligands (1.8- and 3.5-fold). Despite discordant TGF-ß activation, SMC contractile gene expression was similar between lineages in aortic and iPSC-SMCs, suggesting alternative downstream effects in LDS aneurysm. Conclusion: iPSC-derived SMCs effectively model lineage-specific aortic root aneurysm pathology, validating this model as a tool for mechanistic testing and therapy discovery.

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