Correspondence Address: Dr. Rajesh Krishnachandra Shah Shri Krishna Hospital, 223 Samarth Nagar, Aurangabad - 431 001, Maharashtra India
Source of Support: None, Conflict of Interest: None
Congenital aneurysm of the mitral aortic intervalvular fibrosa (MAIVF) is a relatively rare condition. It was initially described in the African population and subsequently reported in other racial groups. Many of the subaortic aneurysms arise from the MAIVF; association with submitral aneurysm (SMA) is even rare. They more commonly occur as a pseudoaneurysm of the MAIVF-P, which is usually due to a complication of endocarditis or aortic valve surgery. Diagnosis is usually with echocardiography, and complete confirmation and anatomical assessment are done by computerised tomography and cardiac magnetic resonance imaging, which are useful for surgical evaluation. Surgery is the only treatment when it is large or symptomatic or has related complications, such as rupture, compression of adjacent structures, embolic events, mitral regurgitation, or heart failure. Here, case is presented of a congenital variety of MAIVF aneurysm with congenital SMA, in a 31-year-old female. She presented with breathlessness. She had mitral regurgitation, and had to undergo surgical repair and mitral valve replacement. This may be the first case reported of a congenital MAIVF aneurysm with a congenital SMA.
Keywords: Mitral aortic intervalvular fibrosa aneurysm, pseudoaneurysm of the mitral aortic intervalvular fibrosa, submitral aneurysm
How to cite this article: Shah RK. Congenital Mitral Aortic Intervalvular Fibrosa Aneurysm with Submitral Aneurysm. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:383-6
How to cite this URL: Shah RK. Congenital Mitral Aortic Intervalvular Fibrosa Aneurysm with Submitral Aneurysm. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2021 Apr 13];4:383-6. Available from: https://www.jiaecho.org/text.asp?2020/4/3/383/303929
Congenital aneurysm of mitral aortic intervalvular fibrosa (MAIVF) is a rare condition, and the association with a congenital submitral aneurysm (SMA) is still rare. More commonly seen is the pseudoaneurysm of the MAIVF (MAIVF-P), which is usually a result of complications such as endocarditis or surgery on these valves. These patients become symptomatic only when complications occur. Echocardiography is the primary modality of investigation for the diagnosis, but multimodality approach is required for confirmation and anatomical evaluation before surgery. Surgery is the only treatment modality for the condition.
A 31-year-old female presented with complaints of gradually increasing breathlessness of 3–4 months duration, which was Grade III at presentation. She also complained of palpitations of 1-month duration. Her pulse was 116/min and blood pressure was 110/70 mmHg. She had no signs of cardiac failure. Cardiovascular examination revealed a pansystolic murmur at the apex conducted to the axilla. There was no history suggestive of rheumatic fever, cyanosis, or prolonged fever in the past.
Electrocardiogram showed atrial flutter, left atrial enlargement in the form of “P” mitrale, and nonspecific ST-T changes [Figure 1]. The chest X-ray showed cardiomegaly and mild pulmonary congestion [Figure 2]. Parasternal long-axis view in echocardiography revealed a blind pouch extending between the aortic valve and aorta anteriorly and the anterior mitral leaflet posteriorly. The pouch was anterior and medial to the left atrium and measured 42 mm × 34 mm [Figure 3] and [Video 1]. The parasternal short-axis view confirmed the presence of the pouch between the anterior mitral leaflet and left atrium inferiorly and the aorta superiorly, with multiple septations in it [Figure 4] and [Video 2]. The apical four-chamber view also showed the presence of the pouch and the SMA [Figure 5] and [Video 3]. There was malcoaptation of the mitral leaflets, and color flow mapping showed the presence of severe mitral regurgitation (MR) in the parasternal long view [Video 4]. The vena contracta of the jet was 6.4 mm. A second opinion of a senior cardiologist taken who did the three-dimensional transesophageal echocardiography and confirmed it to be a MAIVF aneurysm [Figure 6] and [Video 5]. computerized tomography (CT) scan was done for further anatomical evaluation, which confirmed the diagnosis. The patient was advised surgery as it was the only available option for the condition. Preoperative cardiac magnetic resonance imaging (cMRI) was done, which revealed the presence of the MAIVF aneurysm with a thrombus in it. It also showed the SMA of size 20 mm × 9 mm, which also had a thrombus of 3 mm size in it [Figure 7]. During the course of preoperative investigations, ultrasonography of the abdomen and pelvis revealed her to be pregnant, with a gestation of 6 weeks and 4 days. The pregnancy was medically terminated, and she ultimately underwent cardiac surgery. The procedure performed was left ventricular (LV) endoaneurysmorrhaphy of a large aneurysm of the intervalvular LV fibrosa and SMA with bovine pericardium. The mitral valve was replaced with a 25 SJM bi-leaflet valve. The approach used was a bicameral one (transaortic and trans-right atrial transseptal). After 14 days, she had breathlessness, which was due to a larger pleural effusion on the right side which was drained with an intercostal approach. The patient was discharged in 2 days.
Congenital aneurysm of the MAIVF is a relatively rare condition. It was initially described in the African population and subsequently reported in other racial groups. Many of the subaortic aneurysms arise from MAIVF.
The more commonly seen is the MAIVF-P which is usually due to complications of endocarditis or aortic valve surgery. Congenital variety is very rare and the association with congenital SMA is even rare. This may be the first case reported in the literature.
Left and noncoronary leaflets of the aortic valve share fibrous continuity with the anterior leaflet of the mitral valve. The thick ends of this fibrous tissue are in continuation with the ventricular musculature on both sides and are called right and left fibrous trigones. The MAIVF is a central triangular area bounded by these right and left trigones. As it is a relatively avascular area, it is very prone for weakening and abscess formation after aortic and, less commonly, mitral valve surgeries.
Since most patients are offered surgery, the natural course of uncomplicated and asymptomatic MAIVF is unknown. A patient with MAIVF aneurysm can remain asymptomatic or present with dyspnea or infective symptoms, suggestive of endocarditis. They can even present as angina due to compression of coronary arteries. Another common presentation can be due to congestive cardiac failure. Various complications of MAIVF-P aneurysms have been reported. The common complications are enlargement of a pseudoaneurysm, leading to compression of adjacent structures including the left atrium, coronary, and pulmonary arteries. As they are very close to the left atrium and aorta, they may rupture into anyone resulting in a fistula. Mitral annulus dysfunction may also lead to functional mitral regurgitation. MAIVF aneurysm is usually diagnosed with echocardiography and confirmed with cardiac computed tomography (CT) and cMRI, which are useful for surgical evaluation.
The recommended treatment for MAIVF aneurysm is surgery in both asymptomatic and symptomatic patients. Surgery is the only treatment when it is large or symptomatic or has related complications such as rupture, compression of adjacent structures, embolic events, MR, or heart failure. The patch repair in MAIVF aneurysm can be carried out with or without aortic root reconstruction.
Congenital aneurysm of MAIVF is a very rare condition. More common is the pseudoaneurysm, which can be due to several causes such as endocarditis of the aortic or mitral valve and postaortic valve surgery. Association with SMA is still rare. It is usually asymptomatic. Symptoms occur when there are complications such as thromboembolic phenomenon, rupture, and endocarditis or heart failure. Surgery is the only treatment for both symptomatic and asymptomatic patients. The connective tissue deficiency could be the cause of simultaneous occurrence of MAIVF aneurysm and SMA.
This may be the first case reported of congenital MAIVF aneurysm and SMA in the same patient.
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Joshi SS, Jagadeesh AM, Furtado A, Bhat S. Transesophageal echocardiography in surgical management of pseudoaneurysm of mitral-aortic intervalvular fibrosa with aneurysms of right sinus of Valsalva and left main coronary artery. Ann Card Anaesth 2013;16:40-3. [PUBMED] [Full text]
Ghersin E, Litmanovich D, Agmon Y, Milo S. Pseudoaneurysm of the mitral-aortic intervalvular fibrosa following aortic valve replacement-Diagnosis and dynamic evaluation with multidetector CT and transesophageal echocardiography. Interact Cardiovasc Thorac Surg 2005;4:502-4.