Nonseptal right atrial aneurysm: case report and review of literature

Cardiovascular Pathology 19 (2010) e79 – e83

Case Report

Nonseptal right atrial aneurysm: case report and review of literature Kathryn Mersbach a , Aditya Mehra b , Joseph Jaworski a , Billie Fyfe a,⁎ a Department of Pathology, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA Division of Cardiology, Department of Medicine, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA

b

Received 31 July 2008; received in revised form 16 January 2009; accepted 25 January 2009

Abstract An aneurysm of the portion of the right atrium classically referred to as the subeustachian sinus is reported in a 75-year-old man with cardiac amyloidosis, AL phenotype, related to underlying multiple myeloma. A review of literature confirms the rarity of nonseptal right atrial aneurysms and their propensity to involve the subeustachian area of the right atrium which may be an intrinsic area of weakness in the atrial wall. The coincident amyloidosis in our current case suggests that hemodynamic factors may have played a role in the development of the aneurysm. © 2010 Elsevier Inc. All rights reserved. Keywords: Non-septal right atrial aneurysm; Cardiac amyloidosis; Subeustachian sinus

1. Case report A 75-year-old man with multiple myeloma (IgG kappa phenotype), coronary artery disease, diabetes mellitus, and head and neck cancer was admitted to the hospital for aspiration pneumonia. On the day of admission, he became bradycardic and unresponsive in the emergency department, but was successfully resuscitated. An electrocardiogram a few days later showed sinus tachycardia with normal voltage, left axis deviation, and frequent premature ventricular complexes. An echocardiogram showed normal systolic and diastolic function with mild left ventricular hypertrophy. No cardiac aneurysm was identified. The patient's 2-month hospital course was complicated by continued ventilator-dependent respiratory failure and various infections. He died after unsuccessful cardiopulmonary resuscitation for asystole. 2. Pathology At autopsy, the 450-g heart had thickened RV at 0.9 cm and LV at 2.0 cm. The atrial endocardium and the ⁎ Corresponding author. Department of Pathology MEB 212, UMDNJRobert Wood Johnson Medical School, One Robert Wood Johnson Place, New Brunswick, NJ 08901, USA. Tel.: +1 732 235 8114; fax: +1 732 235 8124. E-mail address: [email protected] (B. Fyfe). 1054-8807/09/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.carpath.2009.01.003

myocardium had a waxy appearance. The atria were dilated and along the posterolateral aspect of the right atrium there was an outpouching of atrial wall that looped below the AV sulcus and coronary sinus (Fig. 1). From the interior aspect of the right atrium, the opening to this outpouching was wide mouthed and it appeared to involve the area of the trabeculated atrium classically anatomically described as the subeustachian sinus [1] (Fig. 2). The interatrial septum was unremarkable and specifically there was no evidence of patent foramen ovale, atrial septal defect, or atrial septal aneurysm. Additionally, there was no evidence of Ebstein's anomaly; the tricuspid valve was normally situated. There was no thrombosis or endocardial thickening in the aneurysm and the wall was extremely thin, measuring 0.1 cm. Microscopic examination of the wall revealed fibrous replacement of myocardium in this area (Fig. 3). Congo red stain for amyloid was positive, surrounding individual atrial myocytes with associated atrophy (Fig. 4). The remainder of the myocardium demonstrated remarkable amounts of amyloid distributed as nodules in the interstitium, perimyocytic, and perivascular, as well as involving cardiac valves. The nature of the amyloid protein was confirmed with immunohistochemical stains against kappa and lambda light chains (anti-kappa and anti-lambda rabbit polyclonalantibody, Ventana, Tucson, AZ, USA), confirming a kapparestricted light chain staining of the amyloid protein, in

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Fig. 1. Posterior aspect of heart demonstrating right atrial aneurysm from the exterior aspect (arrow).

Fig. 3. Low power view of aneurysm wall with extensive fibrosis (Trichrome 40×).

keeping with the clinically noted IgG kappa multiple myeloma (Figs. 5 and 6) 3. Discussion The identification of an unusual nonseptal right atrial aneurysm (NSRAA) which appeared to be an acquired

Fig. 4. Bright red fluorescence of Congo red positive amyloid material in atrial aneurysm wall (fluorescence microscopy, Congo red stain 100×).

Fig. 2. Opening of the aneurysm from the interior of the right atrium demonstrating wide-mouthed orifice beneath coronary sinus (*) leading into thin-walled aneurysm sac (arrow). Also note the waxy appearance of the endocardium due to amyloid deposition and the intact interatrial septum.

Fig. 5. Immunohistochemical staining of atrial aneurysm wall with kappa light chain antibody was markedly positive (immunoperoxidase 200×).

K. Mersbach et al. / Cardiovascular Pathology 19 (2010) e79–e83

Fig. 6. Immunohistochemical staining of atrial aneurysm wall with lambda light chain antibody was negative (immunoperoxidase 200×).

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aneurysmal dilatation of an area of the trabeculated right atrium termed the subeustachian sinus prompted our review of the English medical literature. The clinicopathologic features of the current and the 35 reported cases of NSRAA are listed in Table 1 [2–34]. NSRAA has been reported to occur from 1 day to 75 years of age. There is a slight male predominance (21:12— two cases without reported gender). Diagnosis is often made by transthoracic echocardiography but other imaging modalities are also used. Multiple aneurysms more commonly occur in younger individuals (3 weeks, 5 months, 9 and 12 years), but one report of multiple aneurysms in a 50-year-old was found. Single aneurysms are more frequent and often involve the subeustachian sinus area. The subeustachian sinus is a shallow area in the trabeculated portion of the right atrium. Posterior to this is an even smaller pouch that has been termed the fossa of His

Table 1 Clinicopathologic features of NSRAA Ref. Age (years) and sex Aneurysm Location

Associated diseases

Presentation

[2] [3] [4] [5] [6] [7]

23 F 43 F 5 months M 12 F 10 days M 9M

None None None Congestive heart failure Ventricular septal defect, atrial septal defect Dilated cardiomyopathy

Chest pain, arrhythmia Arrhythmia Arrhythmia Arrhythmia Asymptomatic Arrhythmia, SOB

[8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [21] [21] [22] [23] [24] [25] [26] [27] [28] [29] [30]

50 M 1 day M 7M 52 M 15 M 34 M 36 M 72 F 39 F 40 M 4 months M 27 F 52 M 19 F 25 M 44 M 30 F 9M 9 months M 7 days F 9F 42 F 39 F 15 months M 56 M

From RA covering entire heart Anterior to coronary sinus 5 saccular aneurysms near RA appendage 3 aneurysms between sinus node and RA appendage Posterior RA 3 aneurysms between anterior wall and RA appendage 3 diverticula over entire RA Lateral RA wall Anterior RA crossing right AV groove Unknown Near RA appendage Trabeculated portion of RA free wall RA free wall Anteromedial RA wall Lateral RA wall Anterior free wall of RA, superior to tricuspid valve Unknown Lateral RA wall Anterior free wall of RA, near crista terminalis Free wall of RA above tricuspid valve Unknown Free wall of RA Trabeculated portion of RA Inferior to RA appendage near right anterior AV groove Unknown Lateral RA wall Free wall of RA Superior to tricuspid valve Lateral RA wall Trabecular portion of RA Lateral RA wall

Chest pain Asymptomatic Asymptomatic Arrhythmia SD Arrhythmia Chest pain SOB Arrhythmia Arrhythmia, chest pain Asymptomatic Arrhythmia Arrhythmia Chest pain Arrhythmia Asymptomatic Asymptomatic Chest pain s/p MVA Cyanosis Asymptomatic Arrhythmia Arrhythmia, chest pain Arrhythmia Cough, edema Chest pain

[31] [32] [33] [34]

39 F 3 weeks 49 4 months M 75 M

Unknown Lateral and anterior RA wall Unknown Posteromedial to RA Lateral RA, superior to inferior vena cava

None None None None Hypertrophic cardiomyopathy None Coronary artery disease None None None Atrial septal defect Patent foramen ovale None None None None Ebstein's anomaly a None Del 8(p23.1) Patent foramen ovale None Atrial septal defect with left to right shunt Atrial septal defect None Atrial fibrillation, pulmonary embolism, Ebstein's anomaly a Patent foramen ovale Patent foramen ovale, atrial septal aneurysm None Patent foramen ovale Systemic AL amyloidosis

RA, right atrium; MVA, motor vehicle accident; SOB, shortness of breath; SD, sudden death. a

Misdiagnosed Ebstein's anomaly.

Arrhythmia Asymptomatic Fatigue Asymptomatic Asymptomatic

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[35]. We feel that this may be an area of inherent weakness in the atrial wall. The involvement of this area in NSRAA is also interesting given the frequency of arrhythmic presentation. Sixteen patients presented with arrhythmia and three of these had atrial flutter. It is known that atrial flutter often arises from the subeustachian isthmus [36]. The most frequent presentation after arrhythmia is as an asymptomatic finding, but it is interesting to note that sudden death and cyanosis have been reported with NSRAA. Associated heart defects have included patent foramen ovale, atrial septal defect, and ventricular septal defect, and two patients were misdiagnosed as Ebstein's anomaly. Complications include thrombus formation with pulmonary or paradoxical embolization. Therapy is individualized to the patient but has often entailed surgical intervention. Our case of amyloidosis and other reports of NSRAA in ASD with left to right shunt and hypertrophic cardiomyopathy support the view that these aneurysms may occur in situations of hemodynamic stress that lead to aneurysmal dilatation of this area of congenital weakness [12,27]. In other situations, with aneurysms occurring in very young infants, there appears to be a congenital cause. Some have postulated a congenital absence of myoblasts, perhaps related to viral or other insult leading to loss of embryonic muscle [4]. In summary, we report the first case of NSRAA arising in a patient with amyloidosis. We identify the subeustachian sinus as an area frequently involved by these aneurysms and we postulate that in our case this lesion formed in part because of the amyloid-associated hemodynamic stresses placed on this area of congenital weakness of the atrial wall. References [1] Wilcox B, Anderson R. Surgical anatomy of the heart. New York: Churchill Livingstone, 1985. p. 2.5–2.13. [2] Morrow AG, Behrendt DM. Congenital aneurysm (diverticulum) of the right atrium. Clinical manifestations and results of operative treatment. Circulation 1968;38(1):124–8. [3] Sheldon WC, Johnson CD, Favaloro RG. Idiopathic enlargement of the right atrium. Report of four cases. Am J Cardiol 1969;23(2): 278–84. [4] Varghese PJ, Simon AL, Rosenquist GC, Berger M, Rowe RD, Bender HW. Multiple saccular congenital aneurysms of the atria causing persistent atrial tachyarrhythmia in an infant. Report of a case successfully treated by surgery. Pediatrics 1969;44(3):429–33. [5] Olsson SB, Blomström P, Sabel K, William-Olsson G. Incessant ectopic atrial tachycardia: successful surgical treatment with regression of dilated cardiomyopathy picture. Am J Cardiol 1984;53:1465–6. [6] Petit A, Eicher JC, Louis P. Congenital diverticulum of the right atrium situated on the floor of the coronary sinus. Br Heart J 1988;59(6): 721–3. [7] Miyamura H, Nakagomi M, Eguchi S, Aizawa Y. Successful surgical treatment of incessant automatic atrial tachycardia with atrial aneurysm. Ann Thorac Surg 1990;50(3):476–8. [8] Morishita Y, Kawashima S, Shimokawa S, Taira A, Kawagoe H, Nakamura K. Multiple diverticula of the right atrium. Am Heart J 1990;120(5):1225–7.

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