Limits...
Papillary fibroelastoma of the aortic valve--a case report and literature review.

Jha NK, Khouri M, Murphy DM, Salustri A, Khan JA, Saleh MA, Von Canal F, Augustin N - J Cardiothorac Surg (2010)

Bottom Line: The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve.The patient recovered well.Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Adult Cardiac Surgery, Institute of Cardiac Sciences, Sheikh Khalifa Medical City Managed by Cleveland Clinic, PO Box-51900, Abu Dhabi-UAE. nk_jha@hotmail.com

ABSTRACT
The prevalence of primary cardiac tumour ranges from 0.0017-0.28% and papillary fibroelastoma is rare but not uncommon benign cardiac neoplasm. Currently, with the advent of higher-resolution imaging technology especially transoesophageal echocardiography such cases being recognized frequently. The clinical presentation of these tumours varies from asymptomatic to severe ischaemic or embolic complications. We herein, present a 50-year-old female patient with a papillary fibroelastoma of the aortic valve arising from the endocardium of the right coronary cusp very close to the commissure between the right and non-coronary cusps. The patient presented with angina-like chest pain and was investigated using echocardiography and CT angiographic modalities in addition to the usual investigations. The differential diagnosis considered was a thrombus, myxoma, Lambl's excrescence and infective vegetation. The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve. The patient recovered well. A review of the literature suggests that the cardiac papillary fibroelastoma is a rare but potentially treatable cause of embolic stroke and other fatal complications, therefore, a strong suspicion; appropriate use of imaging modality, preoperative anticoagulation and urgent surgical resection is warranted. Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours.

Show MeSH

Related in: MedlinePlus

Trans-oesophageal echocardiography showing a mobile, spherical pedunculated tumour mass of 1.2 × 1 cm in size at the right coronary aortic cusp (ME AV short-axis view).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC2965711&req=5

Figure 1: Trans-oesophageal echocardiography showing a mobile, spherical pedunculated tumour mass of 1.2 × 1 cm in size at the right coronary aortic cusp (ME AV short-axis view).

Mentions: A 50-year-old female presented to our hospital for evaluation of chest pain. She had history of recurrent, vague, central chest pain with radiation to inter-scapular area. The pain was compressive and mild in nature and was not associated with effort. The clinical examination and routine blood laboratory investigations were unremarkable. The electrocardiography including stress test was also inconclusive. The chest x ray was normal. A 2-D and transoesophageal echocardiography (TEE) revealed presence of an echodense supra valvular, pedunculated, spherical mass of 1.2 × 1 cm in size about 1.2 cm above the aortic annulus (Figure 1). This supra valvular echogenic mass was found to be moving and displaced during each phase of the cardiac cycle and it was very close to the orifice of the right coronary artery (RCA) (Figure 2 and 3). However, the aortic valve and other cardiac structures were normal. There was no regurgitation of the aortic valve. A contrast-enhanced computerized tomography scan of the chest confirmed the presence of a mildly ill-defined, non-enhancing, hypodense nodular lesion of approximate size 1.0 × 0.8 × 0.7 cm in the aortic root, just adjacent to the origin of right coronary artery (Figure 4). Based upon the findings as above, a differential diagnosis was made which included, thrombus, myxoma, fibroelastoma and inflammatory mass.


Papillary fibroelastoma of the aortic valve--a case report and literature review.

Jha NK, Khouri M, Murphy DM, Salustri A, Khan JA, Saleh MA, Von Canal F, Augustin N - J Cardiothorac Surg (2010)

Trans-oesophageal echocardiography showing a mobile, spherical pedunculated tumour mass of 1.2 × 1 cm in size at the right coronary aortic cusp (ME AV short-axis view).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2965711&req=5

Figure 1: Trans-oesophageal echocardiography showing a mobile, spherical pedunculated tumour mass of 1.2 × 1 cm in size at the right coronary aortic cusp (ME AV short-axis view).
Mentions: A 50-year-old female presented to our hospital for evaluation of chest pain. She had history of recurrent, vague, central chest pain with radiation to inter-scapular area. The pain was compressive and mild in nature and was not associated with effort. The clinical examination and routine blood laboratory investigations were unremarkable. The electrocardiography including stress test was also inconclusive. The chest x ray was normal. A 2-D and transoesophageal echocardiography (TEE) revealed presence of an echodense supra valvular, pedunculated, spherical mass of 1.2 × 1 cm in size about 1.2 cm above the aortic annulus (Figure 1). This supra valvular echogenic mass was found to be moving and displaced during each phase of the cardiac cycle and it was very close to the orifice of the right coronary artery (RCA) (Figure 2 and 3). However, the aortic valve and other cardiac structures were normal. There was no regurgitation of the aortic valve. A contrast-enhanced computerized tomography scan of the chest confirmed the presence of a mildly ill-defined, non-enhancing, hypodense nodular lesion of approximate size 1.0 × 0.8 × 0.7 cm in the aortic root, just adjacent to the origin of right coronary artery (Figure 4). Based upon the findings as above, a differential diagnosis was made which included, thrombus, myxoma, fibroelastoma and inflammatory mass.

Bottom Line: The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve.The patient recovered well.Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Adult Cardiac Surgery, Institute of Cardiac Sciences, Sheikh Khalifa Medical City Managed by Cleveland Clinic, PO Box-51900, Abu Dhabi-UAE. nk_jha@hotmail.com

ABSTRACT
The prevalence of primary cardiac tumour ranges from 0.0017-0.28% and papillary fibroelastoma is rare but not uncommon benign cardiac neoplasm. Currently, with the advent of higher-resolution imaging technology especially transoesophageal echocardiography such cases being recognized frequently. The clinical presentation of these tumours varies from asymptomatic to severe ischaemic or embolic complications. We herein, present a 50-year-old female patient with a papillary fibroelastoma of the aortic valve arising from the endocardium of the right coronary cusp very close to the commissure between the right and non-coronary cusps. The patient presented with angina-like chest pain and was investigated using echocardiography and CT angiographic modalities in addition to the usual investigations. The differential diagnosis considered was a thrombus, myxoma, Lambl's excrescence and infective vegetation. The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve. The patient recovered well. A review of the literature suggests that the cardiac papillary fibroelastoma is a rare but potentially treatable cause of embolic stroke and other fatal complications, therefore, a strong suspicion; appropriate use of imaging modality, preoperative anticoagulation and urgent surgical resection is warranted. Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours.

Show MeSH
Related in: MedlinePlus