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Clinical case

Intrapericardial rupture of a mitral subannular aneurysm: A case report and review of the literature Anévrisme sous-annulaire mitral rompu dans le péricarde : cas clinique et revue de la littérature I.B. Diop a , M. Leye a,b , A.D. Diallo a , E.H.M. Sarr a , S.J. Manga a , L.L. Diene a , M. Jobe c,∗ a

b

Service de cardiologie, centre hospitalier universitaire de Fann, Dakar, Senegal Unité de formation et de recherches des sciences de la santé, université de Thiés, Thiés, Senegal c Service de cardiologie, CHU Aristide Le Dantec, Dakar, Senegal Received 5 March 2014; accepted 20 January 2015

Abstract Mitral subannular aneurysm is a rare heart disease that can have many different forms of clinical presentations. It was first described in young men of African descent and was later reported in other geographical areas of the world. The etiopathogenesis as per data from the literature can be congenital, acquired or idiopathic. We report the case of a 19-year-old male in whom we made the diagnosis of mitral subannular aneurysm. The evolution was fatal following a rupture of the aneurysm into the pericardium. Through this case report, we stress the importance of echocardiography in the diagnosis of this condition. In resource-limited countries, the prognosis is unfortunately often unfavorable especially the ruptured forms. Therefore, a high degree of suspicion is needed to make a prompt diagnosis and timely surgical intervention. © 2015 Published by Elsevier Masson SAS. Keywords: Mitral subannular aneurysm; Intrapericardial rupture

Résumé L’anévrisme sous-annulaire mitral est une cardiopathie rare qui peut avoir des présentations cliniques multiples. Il a été initialement décrit surtout chez de jeunes patients d’origine africaine, puis secondairement signalé dans différentes zones géographiques du monde. Des données de la littérature, il ressort au plan étiopathogénique, qu’il peut être congénital, acquis ou idiopathique. Nous rapportons le cas d’un patient de 19 ans chez qui nous avons fait le diagnostic d’anévrisme sous-annulaire mitral, l’évolution a été fatale suite à une rupture intra-péricardique de l’anévrisme. À travers cette observation, nous soulignons l’importance de l’échographie cardiaque dans le diagnostic de cette affection. Dans les pays à faibles ressources, le pronostic est malheureusement souvent défavorable dans ses formes rompues. © 2015 Publié par Elsevier Masson SAS. Mots clés : L’anévrisme sous-annulaire mitral ; Rupture intra-pédicardiale

1. Introduction

∗ Corresponding author. Service de cardiologie, CHU Aristide Le Dantec, BP 3001 Dakar, Senegal. E-mail address: [email protected] (M. Jobe).

Mitral subannular aneurysm was first described in Nigeria [1]. This disease was later reported in other countries in subSaharan Africa and also in other parts of the world [2–4]. Its clinical presentation varies and its diagnosis has been facilitated by transthoracic and transesophageal echocardiography, multidetector scanner and more recently by magnetic resonance imaging [2,4,5]. Surgery is the cornerstone of treatment.

http://dx.doi.org/10.1016/j.ancard.2015.01.013 0003-3928/© 2015 Published by Elsevier Masson SAS.

Please cite this article in press as: Diop IB, et al. Intrapericardial rupture of a mitral subannular aneurysm: A case report and review of the literature. Ann Cardiol Angeiol (Paris) (2015), http://dx.doi.org/10.1016/j.ancard.2015.01.013

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Fig. 1. a: 2D transthoracic echocardiography longitudinal long axis view showing mitral subannular aneurysm in diastole; b: 2D transthoracic echocardiography apical 4-chamber view showing aneurysm.

However, the prognosis is often not favorable even with surgery [2,4,6]. 2. Case presentation We report the case of a 19-year-old male who presented with a two-month history of chest pain with stage III dyspnea of the New York Heart Association classification. There was no significant past medical history. He was hemodynamically stable with an oxygen saturation of 99% on room air, heart rate of 118 beats per minute and a blood pressure of 90/50 mmHg. The apical impulse was deviated from the mid-clavicular line. Cardiac auscultation found regular heart sounds with an apical systolic murmur with an intensity of 3/6 radiating to the left axilla. The peripheral pulses were well palpated, and were of good volume and symmetrical. The rest of the clinical examination was normal apart from cryptic hypertrophied tonsils. Echocardiography (Figs. 1 and 2) found a significant dilatation of the left heart chambers with a left atrial antero-posterior diameter of 49 mm, left ventricular diameter of 68 mm and 45 mm in telediastole and telesystole respectively. There was a preserved systolic left ventricular function. We noted a severe mitral insufficiency (regurgitant orificial area of 0.47 cm2 and a regurgitant volume of 42 mL/cycle) by dilatation of the mitral annulus (53 mm) and faulty coaptation of the two mitral leaflets.

The mitral leaflets were moderately thickened with the subvalvular apparatus slightly modifed. We noted the presence of a voluminous subannular aneurysm whose antero-posterior diameter was 45 mm, and with an area of 32 cm2 containing a spontaneous contrast of grade 2+, but without intra-aneurysmal thrombus. The overall kinetics of the walls of the left ventricle was homogeneous and correct. Moderate tricuspid regurgitation was noted with a severe pulmonary hypertension estimated at 62 mmHg, for a right atrial pressure of 10 mmHg. Laboratory tests found a hypochromic microcytic anemia with a hemoglobin level of 9.3 g/dL and an anti-streptolysin O level of 1600 IU/L, and raised inflammatory markers with a C-reactive protein of 48 mg/L and a fibrinogenemia of 5.72 g/L. HBsAg test was negative. The tuberculin skin test was negative as well as the search for acid-fast Bacilli in sputum. Serology for HIV-1 and HIV-2 were negative. Blood cultures however could not be obtained. The medical treatment given was in the form of furosemide 80 mg daily, spironolactone 50 mg daily, captopril 50 mg daily associated with treatment with a low molecular weight heparin. The evolution was marked by the appearance of mainly persistent nocturnal chest pains associated with fever and tachycardia with examination findings of muffled heart sounds and signs of bilateral pleural effusion. Blood count found a further decrease in hemoglobin level to 8.3 g/dL associated with a thrombocytosis of 490,000/mm3 . Follow-up echocardiography (Fig. 3a and b) demonstrated the occurrence of a pericardial effusion predominant facing the lateral wall of the left ventricle measuring 15 mm without signs of compression or haemodynamic compromise. The indication for surgical treatment of the aneurysm was made but the patient died before surgery. 3. Discussion

Fig. 2. Colour Doppler transthoracic echocardiography 4-chamber view in systole showing mitral subannular aneurysm and a severe mitral insufficiency.

Mitral subannular aneurysm was described for the first time in Nigeria [1] in 1962. This condition was later described in many African series and in the world [2–5,7]. It is a disease whose cause remains unclear, with rare and varied etiologies. Because of its predilection in black people, genetic origin has often been suggested [2–4]. Combination with tuberculosis, rheumatic fever, infection with Chlamydia pneumoniae or Takayasu’s arteritis have also been reported [2,6–8]. This suggests the role of infection and inflammation in the pathogenesis

Please cite this article in press as: Diop IB, et al. Intrapericardial rupture of a mitral subannular aneurysm: A case report and review of the literature. Ann Cardiol Angeiol (Paris) (2015), http://dx.doi.org/10.1016/j.ancard.2015.01.013

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Fig. 3. a: 2D transthoracic echocardiography 4-chamber view showing the presence of a pericardial effusion facing the lateral and apical walls of the left ventricle and a left pleural effusion following aneurysmal rupture; b: Doppler transthoracic echocardiography 4-chamber view showing a colour flow in the pericardium proving aneurysmal rupture into the pericardial sac.

of this disease. On the other hand, some publications refer to congenital forms without any underlying infection or trauma [4,6]. In our case, this inflammatory context was found in association with an evolving rheumatic picture suspected due to the presence of cryptic enlarged tonsils and ASO level of 1600 IU/L. But the evolving rheumatic picture was not confirmed by a second sample as the patient died before this was possible. The clinical presentation varies depending on the circumstances that may occur in the form of stenocardia, heart failure, palpitations and edema of the lower limbs [5] or it may be discovered during the evaluation of a systolic murmur or cardiomegaly [2]. Ventricular arrhythmias may also be the presenting clinical picture [9]. In our patient, the mode of presentation was the occurrence of chest pain associated with heart failure. The clinical examination reveals almost always the presence of a systolic murmur of mitral regurgitation of varying intensity [2,5], which was present in our patient. The electrocardiographic features depends on the underlying condition usually showing a sinus rhythm with non-specific signs [2], which sometimes maybe consistent with signs of infarction [9]. Echocardiography enables in all cases to make the diagnosis of aneurysm showing a localized dilatation of the wall forming a pocket of variable size in free communication with the left ventricular cavity just below the posterior leaflet of the mitral valve around the posterolateral aspect of the left ventricle, the mitral subannular region [2,9]. Most often, it is associated with mitral valve insufficiency of variable degrees. Echocardiography is also used to assess the systolic function of the left ventricle, to look for wall motion abnormalities and to study the status of other valves as well as the pulmonary artery pressure. Transesophageal Doppler echocardiography is used to confirm the diagnosis and to better define the lesions [2,5]. CT and magnetic resonance imaging are not indispensable in the diagnosis, they are used to further explore findings from transthoracic echocardiography, and provide information on

the coronary arteries and also help avoid performing coronary angiography in some cases [4]. Open-heart surgery is the gold standard in the treatment of mitral subannular aneurysm. Two techniques are commonly used: an extracardiac technique to deal with the aneurysm by epicardial means that is to say, under the wall of the aneurysm. The intrapericardial route or antunes approach is to go through the atrioventricular sulcus through the left atrium [6]. The evolution in the absence of surgery is usually marked by a rupture into a heart chamber [10,11] or into the pericardium [12], as was the case in our patient. Improved surgical techniques and intensive care services have significantly reduced the rate of mortality [2,4–6,9]. In sub-Saharan Africa, the difficulty lies in the surgical management, which is not always possible due to the scarcity and inexperience of existing cardiac surgery centres in the management of acute cardiovascular conditions requiring lifesaving surgery [2]. 4. Conclusion Mitral subannular aneurysm is a rare but serious heart disease, needing a high degree of suspicion for a prompt timely diagnosis. Most series have been reported in black people, although clinical observations have been described around the world. The causes are diverse. Echocardiography allows in most cases to make a reliable and accurate diagnosis. Improved surgical techniques and intensive care has altered the prognosis of this condition. Hence, the need for the surgery as soon as the diagnosis is made to prevent progression to rupture. Authors’ contributions IBD, ML and MJ conducted the literature search, drafted the first manuscript, performed language correction, and participated in article design and coordination. EMS and SJM conducted the echocardiography and participated in manuscript

Please cite this article in press as: Diop IB, et al. Intrapericardial rupture of a mitral subannular aneurysm: A case report and review of the literature. Ann Cardiol Angeiol (Paris) (2015), http://dx.doi.org/10.1016/j.ancard.2015.01.013

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draft. ADD and LLD critically revised the manuscript for important intellectual content and participated in investigation studies and critically evaluated the article. All authors read and approved the final manuscript.

[3]

Consent

[5]

All authors declare that written informed consent was obtained from the patient’s father for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor of this journal.

[6]

Disclosure of interest

[8]

The authors declare that they have no conflicts of interest concerning this article.

[9]

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Please cite this article in press as: Diop IB, et al. Intrapericardial rupture of a mitral subannular aneurysm: A case report and review of the literature. Ann Cardiol Angeiol (Paris) (2015), http://dx.doi.org/10.1016/j.ancard.2015.01.013