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J Thorac Cardiovasc Surg 2008;136:538
© 2008 The American Association for Thoracic Surgery

Letter to the Editor

Right atrial papillary fibroelastoma associated with atrial septal defect, persistent superior vena cava, and coronary artery disease

Cipriano Abad, MDa, Pilar De la Rosa, MDb

a Department of Cardiovascular Surgery, Hospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran Canaria, Spain
b Department of Pathology, Hospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran Canaria, Spain

To the Editor:

We read with interest the article by Lauten and coworkers1Go regarding the successful resection of a right atrial papillary fibroelastoma (PF). We have additional surgical experience with this infrequent location of the neoplasm.

A 60-year-old man with a history of arterial hypertension and a transient ischemic attack underwent a transthoracic echocardiogram, which demonstrated a mass inside the right atrium (RA). Further cardiologic evaluation including transesophageal echocardiogram and complete cardiac catheterization and coronary arteriography disclosed a tumor in the RA, fenestrated atrial septal defect (ASD), persistent left superior vena cava (LSVC), and severe coronary artery disease with tight stenosis in the proximal left anterior descending coronary artery (LAD) and middle right coronary artery (RCA).The patient underwent surgery on June 6, 1998. A median sternotomy was performed. Under standard cardiopulmonary bypass, with cannulation of the aorta, both venae cavae, and the LSVC, the aorta was crossclamped. Intermittent cold blood cardioplegia was administered and the three venae cavae were snared. A right atriotomy was performed, a 2 x1.5-cm gelatinous tumor attached to the muscular endocardium of the RA was completely removed, an ostium secundum type of ASD was closed with an autologous pericardial patch, and a double coronary bypass graft to the LAD and RCA was carried out. The patient had an uncomplicated surgery and recovery and was discharged home 10 days after the operation. Gross and histologic examination of the mass was typical of PF. At present, 9 years 6 months after the operation, the patient remains asymptomatic. A recent transthoracic echocardiogram showed no intracavitary masses, closure of the interatrial septum, and normal left ventricular function.

PF is an endocardial tumor that represents the second or third most common cardiac neoplasm, accounting for less than 10% of all primary benign heart tumors. It is most frequent in the left side of the heart and generally develops in the heart valves; however, other endocardial heart locations have also been described. Right PF arising from the nontricuspid valve or interatrial septum is extremely uncommon. We only found a few surgically treated patients.1-7Go To the best of our knowledge, excision of a free-wall PF in association with closure of an ASD and coronary artery bypass grafting has not been previously reported.

References

  1. Lauten A, Strauch JT, Wipperman J, Walers T. A rare type of right tumor in a 66-year-old woman. J Thorac Cardiovasc Surg 2007;133:251-252.[Free Full Text]
  2. Gallas MT, Reardon MJ, Reardon PR, De Felice CA, Raizner AE, Mody DR. Papillary fibroelastoma. A right atrial presentation. Tex Heart Inst J 1993;20:293-295.[Medline]
  3. Singh H, Alam M, Schwartz D, Martin JR. Right atrial papillary elastoma: an uncommon location. Echocardiography 2004;21:273-274.[Medline]
  4. Hindupur S, Schwabe JL. Papillary fibroelastoma of the right atrium: an atypical presentation. A case report and review of the literature. J Cardiovasc Surg 2005;46:589-591.[Medline]
  5. Gabbieri D, Rossi G, Barutti L, Corghi L, Zaca F, Sarandria D, et al. Papillary fibroelastoma of the right atrium as an unusual source of recurrent pulmonary embolism. J Cardiovasc Med (Hagerstown) 2006;7:273-278.
  6. Lotto A, Earl UM, Owens A. Right mass.Thrombus, myxoma, or cardiac papillary fibroelastoma?. J Thorac Cardiovasc Surg 2006;132:159-160.[Free Full Text]
  7. Kim RW, Jeffery ME, Smith MJ, Wilensky Rl, Woo EY, Woo YJ. Minimally invasive resection of papillary fibroelastoma in a high-risk patient. J Cardiovasc Med (Hagerstown) 2007;8:639-641.[Medline]




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