Patrizia , Massimo , Elisabetta , Giovanni , Marco , Egle , Salvatore , Mauro , and Gianluca: Rupture of Aneurysm of Valsalva Sinus and Aortopathy in Bicuspid Aortic Valve: A Case Report and Review of Literature.

Introduction

The bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly, affecting 1–2 % of the population [1]. It is not a localized lesion but appears to be part of a diffuse vascular degenerative disease. Indeed, the most common comorbidity is dilation of thoracic aorta [2]. Clinical manifestations are usually related to valvular and vascular complications, the latter carrying significant morbidity and mortality. Progressive aortic dilation and aneurysm formation do not appear to be dependent from the hemodynamic burden imposed by a malfunctioning BAV, since it occurs in more than 50 % of patients with a normally functioning BAV [3]. The ascending aorta is the most common site involved in this process (about 44 % of patients with BAV vs 20 % at the aortic root) [1]. Other aortic anomalies may, however, be detected. Sinus of Valsalva aneurysm Sinsu of Valsalva aneurysms (SVA) are very rare and can be either congenital or acquired. Congenital aneurysms may result from focal weakness of the elastic lamina at the junction of the aortic media and the annulus fibrosus. Usually, an un-ruptured SVA is clinically silent. However, it may lead to complications, such as compression (heart chambers or coronary arteries) and rupture [4,5]. Therefore, elective surgical repair is recommended even for asymptomatic individuals. The association of SVA with BAV is very uncommon [6]. Also, the aneurysmal dilation of left subclavian artery is exceedingly rare. It may be defined as an aortic diverticulum type III, according to the classification proposed by Salomonowitz. He classified aortic diverticula into following three types:

  1. in left aortic arch aortic diverticulum that gives rise to aberrant right subclavian artery (i.e. the original Kommerell’s diverticulum or lusoria root),

  2. in right aortic arch aortic diverticulum that gives rise to aberrant left subclavian artery (i.e. the Kommerell’s diverticulum in the broad sense), and

  3. aortic diverticulum at the level of aorto-ductal junction, ductus diverticulum (this category is not considered as Kommerel’s diverticulum). There are reports of the “Kommerell’s diverticulum” without aberrant subclavian artery, but it seems reasonable to categorize this kind of dilation into the third type [7,8]. The presence of a right subclavian artery aneurysm in an adolescent with a BAV has been recently reported [9].

Case Report

We present the case of 58 years-old patient who came to our attention for acute onset of chest pain, dyspnea and palpitations at rest. He referred hypertension without previous relevant clinical history. At baseline, he was in sinus rhythm with dilated jugular veins. Auscultation revealed a continuous machinery-type murmur best heard over the left lower sternal border. The blood sample was unremarkable. Transoesophageal echocardiography (TEE) revealed the presence of a BAV type 0 (without raphe) with normal opening leaflets and a mild regurgitation. From the 2D mid oesophageal short axis view it was possible to suppose the presence of a fistulous tract between the right sinus of Valsalva, which appeared quite dilated, and the right atrium just below the tricuspid valve. The colour Doppler and the continuous wave Doppler confirmed the systo-diastolic (with systolic augmentation), left-to-right shunt at this level. The patient underwent pericardial patch closure of the aortic-RA fistulous tract. The following hospitalization was uncomplicated and patient was discharged some days later. The computed tomography has confirmed the BAV with a coronary sinus (with separated origin of a quite calcified left main artery and a hypoplastic right coronary artery) and a non coronary sinus that was dilated (maximum diameter of bulbar region 52 x 38 mm, synotubular junction 43 x 38 mm). The patch previously implanted was identifiable. In the contest of a left-sided type III aortic arch, the descending aorta had a tortuous course with a kinking (minimum diameter 37 x 35 mm) and gave rise to a non aberrant left subclavian artery with anerurismatic dilation of its origin. The patient is asymptomatic and is followed-up in our institution until now.

Figure 1.

Transesophageal echocardiography. Panel A: misesophageal short axis view showing the bicuspid (type 0) aortic valve, with two siuns of Valsalva. The arrowhead indicates the origin of left main coronary artery. Panel B: midesophageal long axis that depicts the asymmetrical opening of the aortic leaflets. Panel C: Mild aortic regurgitation. Panel D: The arrow shows a leak between the non coronary sinus and the right atrium that gives rise to a leftto- right shunt, which is well showed with the Colour Doppler (Panel E). Panel F: continuous, with systolic reinforcement, left-to-right shunt as seen with the Continuous Wave Doppler. [ra: right atrium, rv: right ventricle, la: left atrium, Ao: aorta]

icfj.2016.8.119-g001.jpg

Discussion

The bicuspid aortic valve (BAV) is one of the most common form of congenital heart defects. The clinical manifestations usually relate to function of the aortic valve and acquired complications such as endocarditis. Although much of the original interest has focused on abnormal bileaflet valve, BAV is a more complex entity, whose involvement of the thoracic aorta forms part of the disease spectrum. The most common comorbility is dilation of aorta. BAV is also associated with other congenital vascular (in particular coarctation of aorta) and cardiac defects (i.e. hypoplastic left ventricle, reversal of dominance of coronary artery, atrial septal defect, ventricular septal defect). This suggests a more global development disorder. A genetic predisposition has been reported, and in familial cases, an autosomal dominant inheritance with variable penetrance appears to be advocated. We report the extremely rare case of a patient with true BAV associated with a diffuse aortopathy, involving the aneurysm of the non coronary sinus, that suddenly rupted into the right atrium and required urgent cardiac surgery, and the aneurysmal dilation of a (non aberrant) left subclavian artery, resembling a Kommerell’s diverticulum. In the emergency care unit, TEE easily identified the aortic-to-right atrium fistulous tract in the life-threatening setting of an acute right failure and allowed the prompt choose of the appropriate repair strategy. CT (although carrying disadvantages of irradiation and use of iodine contrast media) due to its availability, short scanning time and high spatial resolution, is of great value in evaluation and follow-up of patients with suspected extensive aortic pathology due to the diagnosis of bicuspid aortic valve.

Figure 2.

Panel A: Frontal chest X-Ray with para aortic and para arterial lines enlargement (grey arrows). Midline sternotomy wires are also detectable (black arrows). Panels B, C, D Axial and, Panel E, coronal MPR contrast medium-enhanced computed tomography images. Panel B shows the bicuspid aortic valve, with an ectasic (non coronary), sinus of Valsalva. Panel C and D: left main and right coronary arteries originating, separately, from the same coronary sinus of Valsalva. In the Panel E the white arrow shows the patch of closure for the aorto-atrial fistula. Panels F and G: contrast mediumenhanced 3D VRT computed tomography images show aortic arch morphology, origin and course of epiaortic vessels and the very tortuous course (kinking) of the descending tract. The arrowhead indicates the aneurismatic dilation (resembling a Kommerell’s diverticulum) of the proximal part of the left subclavian artery. [rcca: right common carotid artery, rsa: right subclavian artery, lcca: left common carotid artery, lsa: left subclavian artery, lm: left main, rca: right coronary artery]

icfj.2016.8.119-g002.jpg

Declarations of Interest

The authors declare no conflicts of interest.

Acknowledgements

The authors state that they abide by the “Requirements for Ethical Publishing in Biomedical Journals” [10].

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Copyright (c) 2016 Patrizia Carità, Massimo Verdecchia, Emanuela Mancini, Giovanni Ferro, Marco Guglielmo, Egle Corrado, Salvatore Novo, Mauro Pepi, Gianluca Pontone

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