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Cardiovascular Disease |

A Tale of Right Ventricular Cardiac Myxoma

Sivaranjani Penna, MD; Ankush Lahoti, MD; Rizwan Sardar, MD; Frank Mcgeehen, MD
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Lankenau Medical Center, Wynnewood, PA


Chest. 2013;144(4_MeetingAbstracts):151A. doi:10.1378/chest.1704851
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Abstract

SESSION TITLE: Cardiovascular Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Cardiac myxomas are benign tumors, can be asymptomatic but can also have distressing consequences including sudden death. These can be sporadic but recurrence, multiplicity and uncommon locations should raise the suspicion of genetic syndromes[1]. Carney complex (CNC) is such a genetic syndrome which is an autosomal dominant multiple neoplasia involving skin, cardiac, neural and endocrine systems with cardiac myxomas being the second common manifestation after skin lentigenes.

CASE PRESENTATION: A 46 year old Caucasian male with a known history of paroxysmal atrial fibrillation (AF) and benign thyroid hyperplasia presented with an acute episode of AF. Physical exam was significant for brownish macules on his lips, conjunctivae and the body. Transthoracic echocardiogram (TTE) showed a right ventricular mass attached to interventricular septum. Surgical resection revealed a bilobed structure, beneath which was a small sessile mass also excised. Pathology of both masses confirmed the diagnosis of myxomas. Two year follow up TTE revealed another mass in left atrium which revealed to be myxoma too. Its unusual location in right ventricle, multiplicity and recurrence along with thyroid hyperplasia, skin findings and cardiac myxoma led to the diagnosis of CNC.

DISCUSSION: The Carney complex (CNC) was first described in 1985 by Dr. J. Aidan Carney, as the combination of myxomas, spotty pigmentation and endocrine over activity [2]. Amongst cardiac myxomas, left atrial cardiac myxomas are the most common (75%) [3] followed by right atrial (20%) and ventricular being the least common (5 %)[3]. Up to 7% of all the cardiac myxomas are associated with CNC. They are notorious for recurrence. So far, there have reports of associations of cardiac myxomas with CNC at various locations but none arising from the right ventricle as described above.

CONCLUSIONS: This case illustrates that uncommon location, multiplicity and recurrence of cardiac myxoma should raise the suspicion of a genetic syndrome. As per our knowledge, this is the first reported case of right ventricular cardiac myxoma in association with CNC.

Reference #1: Carney JA, Hruska LS, Beachump GD, Gordon H. Dominant inheritance of the complex myxomas, spotty pigmentation and endocrine overactivity Mayo Clinic proc. 1986 Mar;61(3):165-72

Reference #2: Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore) 1985 Jul;64(4):270-83

Reference #3: MacGowan SW, Sidhu P, Aherne T, et al. Atrial myxoma: National incidence, diagnosis and surgical management. Ir J Med Sci 1993 Jun;162(6):223-6.

DISCLOSURE: The following authors have nothing to disclose: Sivaranjani Penna, Ankush Lahoti, Rizwan Sardar, Frank Mcgeehen

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