Aneurysm of mitral-aortic intervalvular fibrosa—a review

Objective: Aneurysm of mitral-aortic intervalvular fibrosa is a rare condition. The purpose of this report is to describe in detail the etiology, clinical presentation, morphologic aspects, diagnostic modalities, treatment options, and outcome to consolidate the understanding of this infrequently encountered distinct anomaly. Methods: We studied English language literatures published from 1960 to December 2015, and the articles were searched from Pub med. A total of 81 articles containing 98 cases were studied, and we also included two cases from our institution, making the total number for analysis 100. Their age ranged from 3 months to 91 years, and male-female ratio was 2.7:1. Endocarditis and aortic valve surgery were the most frequent causes for the development of aneurysm with aortic regurgitation and bicuspid aortic valve as contributing factors. Various diagnostic modalities are available, but conventional echocardiography is useful in diagnosis of this entity especially transesophageal echocardiography. Surgery was the recommended treatment modality in 85%, but in 7% surgery was refused because of the high risk of surgery, and 3% were sent for palliative care especially with associated comorbidity making surgery more risky or asymptomatic patients with very small-sized aneurysm. Results: The surgical outcome is reported excellent, especially with aortic valve replacement and repair of aneurysm at 3 years of follow-up. Outcome in patients with extensive repair for active infective endocarditis had almost 34% mortality. Patients who did not undergo surgery may encounter a thrombus in the aneurysm, risk of endocarditis, and rupture. The mortality rate was 14% due to varied reasons. Conclusion: Aneurysm of mitral-aortic intervalvular fibrosa is rare. Symptoms and signs of infection, chest pain, heart failure or shortness of breath, and cerebrovascular accidents are the presenting features. Transesophageal echocardiography is the diagnostic modality aided by computed tomography and magnetic resonance imaging. Surgical correction is the treatment of choice. Watchful observation may be considered after careful evaluation of associated high-risk features in high-risk cases for surgery and in asymptomatic patients who refuse surgery. © 2017, Indian Association of Cardiovascular-Thoracic Surgeons.