Management of Myosin Heavy Chain 11-Associated Familial Thoracic Aortic Aneurysm and Dissection During Pregnancy in Two Siblings.
Two pregnant siblings presented with thoracic aortic dissection during the second trimester. A pathogenic MYH11 was identified following the first sibling's diagnosis. The second sibling, previously known to be at risk but lost to follow-up, reengaged during pregnancy, tested positive for the familial variant, and dissected before her initial evaluation. This case highlights the importance of genetic diagnosis, surveillance, and multidisciplinary care in managing heritable thoracic aortic disease during pregnancy.
- Front Matter
7
- 10.1016/j.xjon.2021.01.013
- Mar 5, 2021
- JTCVS Open
The impact of genetic factors and testing on operative indications and extent of surgery for aortopathy
- Research Article
2541
- 10.1161/cir.0b013e3181d4739e
- Apr 6, 2010
- Circulation
2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease
- Front Matter
329
- 10.1016/j.ejvs.2018.09.016
- Oct 12, 2018
- European Journal of Vascular and Endovascular Surgery
Editor's Choice – Current Options and Recommendations for the Treatment of Thoracic Aortic Pathologies Involving the Aortic Arch: An Expert Consensus Document of the European Association for Cardio-Thoracic Surgery (EACTS) & the European Society for Vascular Surgery (ESVS)
- Research Article
125
- 10.1016/j.athoracsur.2008.06.074
- Oct 17, 2008
- The Annals of Thoracic Surgery
Outcome of Endovascular Treatment of Acute Type B Aortic Dissection
- Research Article
360
- 10.1213/ane.0b013e3181dd869b
- Aug 1, 2010
- Anesthesia & Analgesia
TABLE OF CONTENTSPreamble ……………………………….280 Introduction ………………………281 1.1. Methodology and Evidence Review … . .2811.2. Organization of the Writing Committee …2831.3. Document Review and Approval …… .2831.4. Scope of the Guideline ……………283 1.4.1. Critical Issues …………… . .2831.5. Glossary of Terms and
- Research Article
39
- 10.1016/j.athoracsur.2010.04.111
- Oct 22, 2010
- The Annals of Thoracic Surgery
In Search of Blood Tests for Thoracic Aortic Diseases
- Research Article
1052
- 10.1161/cir.0b013e3181d47d48
- Apr 6, 2010
- Circulation
2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease: Executive Summary
- Discussion
11
- 10.1016/j.hlc.2016.10.023
- Dec 24, 2016
- Heart, lung & circulation
Update on the Diagnosis and Management of Inherited Aortopathies, Including Marfan Syndrome.
- Front Matter
3
- 10.1016/j.jtcvs.2022.05.016
- May 16, 2022
- The Journal of Thoracic and Cardiovascular Surgery
Risk prediction for thoracic aortic dissection: Is it time to go with the flow?
- Research Article
26
- 10.1016/j.ejvs.2005.10.016
- Dec 20, 2005
- European Journal of Vascular and Endovascular Surgery
Evolving Experience of Percutaneous Management of Type B Aortic Dissection
- Research Article
34
- 10.1161/circulationaha.105.564682
- Aug 23, 2005
- Circulation
As children, we learned that a balloon blown up to its limit of elasticity would pop. For similar aortic ballooning, we hardly know more. Furthermore, in the 21st century, with a nascent epidemic of aortic-related deaths, there have been no consequential advances in preventing the loss of aortic elasticity or in describing the etiology or injury that causes aortic disease. Nor is there an explanation as to why, in some people the aorta “pops” and in others, the aorta dissects. See p 1098 In the United States between 1999 and 2001, at least 129 533 people died from diseases of the aorta and its branches, excluding carotid and coronary disease—an average of 43 199/year, according to the Centers for Disease Control and Prevention ICD-10 codes (Table). The upper limit could potentially exceed 46 817 per year. This number is greater than the ≈40 000 people who die annually from breast cancer, homicides, pancreatic cancer, colon cancer, prostate cancer, or motor vehicle accidents.1 Despite this, little research and even less funding have been allocated to aortic disease research, possibly because disease of a supposedly utilitarian pipe that conveys blood to a pantheon of organs engenders less interest or sympathy in comparison with, for example, cancer. Furthermore, the dismal prognosis of aortic disease, a marker of systemic problems despite successful surgery, has not roused much concern, with a 5-year average survival rate of only 60% in most patients.2 This figure does not differ much from that of stage IB lung cancer. Even the aortic disease–related deaths of media figures such as Albert Einstein, Lucille Ball, Conway Twitty, and John Ritter have fomented little interest. As Western populations age, this most common cause of deaths including sudden death will become an increasing factor in long-term survival. View this table: Deaths Related to Aortic Disease …
- Front Matter
- 10.1016/j.jtcvs.2020.07.071
- Jul 25, 2020
- The Journal of Thoracic and Cardiovascular Surgery
Commentary: The elusive perfect criterion for aortic intervention
- Research Article
424
- 10.1161/01.cir.0000155243.70456.f4
- Mar 22, 2005
- Circulation
Marfan syndrome (MFS) is a heritable disorder of the connective tissue with a prevalence of ≈1 in 3000 to 5000 individuals. The condition is inherited in an autosomal dominant manner with complete penetrance but demonstrates variable expression with significant intra- and interfamilial variation. Approximately 25% of patients do not have a family history and represent sporadic, new mutations for the condition. The cardinal features of MFS involve the cardiovascular, ocular, and skeletal systems. The most life-threatening complication of MFS is thoracic aortic aneurysms leading to aortic dissection, rupture, or both. This article focuses on medical and surgical treatment of aortic disease in patients with MFS and addresses the treatment of aortic disease in children and pregnant women with the condition. The most common cardiovascular complication in patients with MFS is progressive aortic root enlargement initially occurring at the sinuses of Valsalva. Ascending aortic aneurysm can precipitate acute type A aortic dissection, aortic rupture, aortic regurgitation (AR), or all 3, and these complications were the primary cause of death before the advent of successful preventive therapies. Treatment of the aorta consists of regular imaging to detect and quantify progression of aortic dilation, β-adrenergic receptor antagonist therapy, and prophylactic aortic repair when the dilation reaches a sufficient size to threaten dissection or cause AR. Before the era of open-heart surgery, the majority of patients with MFS died prematurely of rupture of the aorta, with an average life expectancy of 45 years.1 The success of current medical and surgical treatment of aortic disease in MFS has substantially improved the average life expectancy, extending it up to 70 years.2,3 Cardiovascular manifestations in MFS also include valvular disease involving the mitral valve, aortic valve, or both. Mitral valve prolapse is the most prevalent valvular abnormality, affecting 35% to 100% of patients.4 Mitral …
- Research Article
- 10.1016/j.mayocp.2017.10.026
- Jun 22, 2018
- Mayo Clinic Proceedings
53-Year-Old Man With Progressive Dyspnea and Orthopnea
- Research Article
- 10.31083/hsf50270
- Jan 22, 2026
- The Heart Surgery Forum
Endovascular therapy provides a new treatment modality for patients with aortic disease. By avoiding the morbidity of open surgery, endovascular approaches make treatment possible for a larger array of patients. However, the durability and long-term survival benefit of endovascular aortic intervention require further discussion and additional follow-up. We believe that the characterization of the role of endovascular therapy involves close risk-benefit analysis based on patient risk, disease presentation, native and pathological anatomy, and long-term outlook. Through review of the randomized prospective literature and relevant retrospective data, we explore the role of catheter-based solutions in abdominal and thoracic aortic disease, with a focus on aortic aneurysm and aortic dissection (AD). For patients with appropriate anatomy, endovascular aortic repair (EVAR) has largely supplanted open aortic repair (OAR) in the treatment of abdominal aortic aneurysm (AAA), both in the elective setting and during rupture. Similarly, thoracic endovascular aortic repair (TEVAR) has gained popularity in treating disease of the descending thoracic aorta, in both aneurysmal degeneration and AD. Similar adoption has been seen in treating other disease states, namely traumatic aortic injury. However, we recognize the current limitations of endovascular therapy and detail the innovations being pursued to advance endovascular therapy in the future.