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Left Ventricular Fibroma Mimicking an Acute Coronary Syndrome

2006; Elsevier BV; Volume: 82; Issue: 5 Linguagem: Inglês

10.1016/j.athoracsur.2006.03.034

1552-6259

Hrvoje Gašparović, Vedran Ćorić, Davor Miličić, Gordana Rajsman, Ivan Burčar, Ranka Štern-Padovan, Ivan Jelić,

Vascular Malformations and Hemangiomas

Cardiac fibromas are exceedingly rare neoplasms. We report the case of a 21-year-old woman who presented with symptoms that were initially misinterpreted as an acute coronary syndrome. Radical surgical resection was undertaken and was considered curative, as the mass histology was consistent with a benign fibroma. Cardiac fibromas are exceedingly rare neoplasms. We report the case of a 21-year-old woman who presented with symptoms that were initially misinterpreted as an acute coronary syndrome. Radical surgical resection was undertaken and was considered curative, as the mass histology was consistent with a benign fibroma. Cardiac fibromas are predominantly diagnosed in children in whom they follow rhabdomyomas in incidence as the second most common tumor in that age group. Even though they appear well delineated, they are not encapsulated. They typically occur as solitary lesions and do not have a propensity for spontaneous regression, as is the case with rhabdomyomas. We report the case of a 21-year-old woman with a recent onset of chest pain and no previous cardiac history. Her electrocardiogram revealed nonspecific ST-segment elevation abnormalities in inferior leads. Multiple episodes of sustained monomorphic ventricular tachycardia were noted. A sestamibi cardiac perfusion scan showed an irreversible defect in the inferior portion of the left ventricle. An echocardiogram showed preserved left ventricular function, absence of valvular abnormalities, and a 4 × 3 cm mass, the origin of which could not be clearly identified (Fig 1). Because it was impossible to distinguish by echocardiography whether the mass was a primary cardiac neoplasm or originated from the pericardium and compressed the left ventricle, the diagnostic algorithm was expanded to include multi-slice computed tomographic imaging. The multi-slice computed tomographic imaging located the tumor within the left ventricular wall (Fig 2). No evidence of neoplastic dissemination was evident. After a left heart catheterization that revealed no pathology of the coronary arteries and no sign of pathologic vascularization, the patient was referred for surgical management. The operation was performed with the aid of cardiopulmonary bypass. The intraoperative findings were consistent with a well-delineated tumor encased within the inferior surface of the left ventricular wall (Fig 3). Even though no clear encapsulation was seen, a dissection plane was developed that made a radical excision of the tumor possible. During the course of the dissection, the left ventricular chamber was opened. The proximity of the mitral valve apparatus warranted close inspection of the valve. This was achieved through a separate left atriotomy, which facilitated testing the mitral valve for competence with a bulb syringe. With the integrity of the mitral valve preserved, the patient was weaned off cardiopulmonary bypass without difficulty. Her postoperative recovery had been compromised by a streptococcal line infection that responded favorably to penicillin. Pathohistologic scrutiny revealed elongated fibroblasts interspersed within collagen and elastin fibers, which was consistent with a fibroma.Fig 2Sagittal computed tomographic image of the tumor. The arrows highlight the borders of the neoplasm. (Ao = aorta; LV = left ventricle; SVC = superior vena cava; TM = tumor.)View Large Image Figure ViewerDownload (PPT)Fig 3Intraoperative image of the left ventricular tumor (arrows).View Large Image Figure ViewerDownload (PPT) Cardiac fibromas are primarily diagnosed in children and adolescents. The wide spectrum of clinical manifestations include chest pain, dyspnea, conduction abnormalities, intracavitary obstructions, congestive heart failure, and sudden death secondary to malignant ventricular arrhythmias [1Padalino M.A. Basso C. Milanesi O. et al.Surgically treated primary cardiac tumors in early infancy and childhood.J Thorac Cardiovasc Surg. 2005; 129: 1358-1363Abstract Full Text Full Text PDF PubMed Scopus (78) Google Scholar, 2Freedom R.M. Lee K.J. Macdonald C. Taylor G. Selected aspects of cardiac tumors in infancy and childhood.Pediatr Cardiol. 2000; 21: 299-316Crossref PubMed Scopus (172) Google Scholar]. However, some tumors remain clinically silent. Cardiac fibromas are solitary lesions, and although they are circumscribed, they lack true encapsulation. They tend to infiltrate the surrounding myocardium and may include calcific deposits [2Freedom R.M. Lee K.J. Macdonald C. Taylor G. Selected aspects of cardiac tumors in infancy and childhood.Pediatr Cardiol. 2000; 21: 299-316Crossref PubMed Scopus (172) Google Scholar]. Their benign histology correlates well with their clearly defined borders and absence of metastatic potential. These tumors originate from fibroblasts and myofibroblasts, sharing the gross and microscopic appearance of their soft tissue counterparts [2Freedom R.M. Lee K.J. Macdonald C. Taylor G. Selected aspects of cardiac tumors in infancy and childhood.Pediatr Cardiol. 2000; 21: 299-316Crossref PubMed Scopus (172) Google Scholar, 3Isaacs H. Fetal and neonatal cardiac tumors.Pediatr Cardiol. 2004; 25: 252-273Crossref PubMed Scopus (236) Google Scholar]. No propensity for spontaneous regression has been demonstrated [2Freedom R.M. Lee K.J. Macdonald C. Taylor G. Selected aspects of cardiac tumors in infancy and childhood.Pediatr Cardiol. 2000; 21: 299-316Crossref PubMed Scopus (172) Google Scholar]. The diagnostic algorithm begins with echocardiography, but it may be expanded to include magnetic resonance imaging or computed tomography. Both of the latter imaging methods allow for explicit insights into the regional topography and degree of tumor invasiveness. Cardiac fibromas have a predilection for the left ventricle, which may be reflective of its fibroblast rich interstitium [4Cho J.M. Danielson G.K. Puga F.J. et al.Surgical resection of ventricular cardiac fibromas: early and late results.Ann Thorac Surg. 2003; 76: 1929-1934Abstract Full Text Full Text PDF PubMed Scopus (114) Google Scholar]. The presentation in the neonate differs in that the tumor more commonly involves the interventricular septum [3Isaacs H. Fetal and neonatal cardiac tumors.Pediatr Cardiol. 2004; 25: 252-273Crossref PubMed Scopus (236) Google Scholar]. The microscopic appearance of cardiac fibromas is one of uniform fibroblastic cells interspersed within a collagenous matrix [3Isaacs H. Fetal and neonatal cardiac tumors.Pediatr Cardiol. 2004; 25: 252-273Crossref PubMed Scopus (236) Google Scholar]. The preferred therapeutic option is radical surgical excision. However, optimal long-term results have been attained with subtotal resections in certain patients [4Cho J.M. Danielson G.K. Puga F.J. et al.Surgical resection of ventricular cardiac fibromas: early and late results.Ann Thorac Surg. 2003; 76: 1929-1934Abstract Full Text Full Text PDF PubMed Scopus (114) Google Scholar]. A more radical approach is favored by some authors, and heart transplantation has been advocated for patients with unresectable cardiac fibromas [5Valente M. Cocco P. Thiene G. Cardiac fibroma and heart transplantation.J Thorac Cardiovasc Surg. 1993; 106: 1208PubMed Google Scholar]. The patient reported herein presented with symptoms mimicking an acute coronary syndrome. Further diagnostic workup revealed that her symptoms as well as the perfusion defect seen on a sestamibi scan were related to a left ventricular neoplasm rather than coronary malperfusion. Radical excision of the tumor was feasible and was followed-up by restoration of the structural integrity of the left ventricle. The proximity of the tumor to the mitral valve apparatus presented a surgical challenge, but its preservation proved to be feasible. The operation was supplemented with intraoperative transesophageal echocardiography, which demonstrated a competent mitral valve without evidence of a residual tumor. She continues to do well at her 6-week follow-up examination.