Special Article – Clinical Case Reports
Austin J Cardiovasc Dis Atherosclerosis. 2016; 3(2): 1024.
Giant Right Atrium Syndrome
Armaro A, Stringi V, Raffa GM* and Follis F
Cardiac Surgery and Heart Transplantation Unit, Department for the Treatment and Study of Cardiothoracic Diseases and Cardiothoracic Transplantation, IRCCS – ISMETT (Istituto Mediterraneo per i Trapianti e Terapie ad alta specializzazione), Palermo, Italy
*Corresponding author: Raffa GM, IRCCS – ISMETT (Istituto Mediterraneo per i Trapianti e Terapie ad alta specializzazione), Via Tricomi 5, 90127 Palermo, Italy
Received: June 21, 2016; Accepted: June 24, 2016; Published: June 28, 2016
Abstract
A case of rare giant right atrium in a woman with previous mitral replacement is described. The etiology is discussed in the light of literature reports.
Keywords: Atriomegaly; Tricuspid regurgitation; Rheumatic heart disease
Case Presentation
A 59-year-old woman presented to the clinic with a history of progressive dyspnea on exertion. Physical examination revealed jugular venous distension, an irregular pulse, and ankle edema, abdominal distension with liver enlargement 8-10 cm below the costal margin, free ascitic fluid and cachexia. The patient had undergone mitral valve replacement with a biological prosthesis in 1981 and subsequently with a mechanical prosthesis in 1996 for rheumatic mitral insufficiency, both times through a sternotomy approach. A chest radiograph showed near-complete opacification of the right upper-mid lung zones, due to marked bulging of the right heart border (Figure 1). A transthoracic echocardiography revealed severe tricuspid insufficiency, massive dilatation of the right atrium with a calculated volume of 700mL, mild dilatation of right ventricle with fractional area change of 44% and normal mitral prosthesis function. Left ventricular ejection fraction was normal. There was no evidence of organic lesions of the tricuspid valve. Cardiac catheterization showed normal coronary arteries and pulmonary pressures. CT scan demonstrated a huge right atrium, (Figure 2) chronic liver disease, moderate as cites and significantly increased diameter of the inferior vena cava. Intraoperative assessment confirmed the diagnostic findings (Figure 3). The patient underwent tricuspid valve replacement with a 31mm biological prosthesis (Figure 3). The postoperative course was uneventful and the patient was transferred to rehabilitation 10 days later.
Figure 1: Chest radiograph showing biological valve prosthesis and markedly
enlarged cardiac silhouette: antero-posterior view.
Figure 2: Chest computer tomography images showing giant right atrium
and biological valve prosthesis. Axial (a), coronal (b) views and 3D CT
reconstruction (c) RA, right atrium; LV, left ventricle.
Figure 3: Surgical view: the huge right atrium and tricuspid valve. TV:
Tricuspid Valve; RAW: Right Atrium Wall.
Discussion
Giant left atrium was first described by Hewett in 1849 and represents a significant risk factor in patients undergoing mitral valve surgery [1]. On the other hand, we found only tree reports of a nonidiopathic giant right atrium in the adult [2-4]. The most common causes of an enlarged right atrium in adults are chronic pulmonary disease, severe mitral abnormalities with pulmonary hypertension, pulmonary emboli and tricuspid stenosis [2]. Malformations with massive enlargement of the right atrium and coronary sinus are rare and usually associated with congenital heart disease in infants and children although some idiopathic cases have been reported [5]. This case represents one of the largest giant right atrium described in adults. We believe that this patient has the extremely rare condition of severe functional enlargement of the right atrium leading to dilatation of tricuspid annulus, severe tricuspid regurgitation and atrial fibrillation with subsequent right heart failure.
References
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- Kurz DJ, Oechslin EN, Kobza R, Jenni R. Idiopathic enlargement of the right atrium: 23-year follow up of a familial cluster and their unaffected relatives. Heart. 2004; 90: 1310-1314.
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