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Table of Contents
Year : 2019  |  Volume : 9  |  Issue : 3  |  Page : 169-170

Supraventricular tachycardia as presenting feature of Nevi, Atrial myxoma, Myxoid neurofibromatosis, and Ephelides Syndrome

1 Consultant-Intensivist, Intensive Care Unit, Kokilaben Dhirubhai Ambani Hospital and Medical Research Center, Mumbai, India
2 Clinical Cardiologist, Department of Cardiology, Sardar Patel Heart Institute, Ankleshwar, Gujarat, India

Date of Web Publication3-Dec-2019

Correspondence Address:
Dr. Sonali Vadi
Kokilaben Dhirubhai Ambani Hospital and Medical Research Center, Mumbai - 400 053, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JICC.JICC_7_19

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NAME composed of Nevi, Atrial myxoma, Myxoid neurofibromatosis, and Ephelides is a rare autosomal dominant syndrome with unknown prevalence. NAME syndrome has variable clinical manifestations, including skin pigmentation, neurofibromatosis, congestive cardiac failure, or even stroke as a result of tumor emboli. To the best of our knowledge, there are no prior published reports of supraventricular tachycardia as the initial presenting finding in a patient with NAME syndrome, a common arrhythmia signaling a rare syndrome. We describe a woman who presented with supraventricular tachycardia. Her thorough clinical examination with detailed workup revealed the features of NAME syndrome.

Keywords: Atrial myxoma, Nevi, Atrial myxoma, Myxoid neurofibromatosis, and Ephelides syndrome, supraventricular tachycardia

How to cite this article:
Vadi S, Mehta M. Supraventricular tachycardia as presenting feature of Nevi, Atrial myxoma, Myxoid neurofibromatosis, and Ephelides Syndrome. J Indian coll cardiol 2019;9:169-70

How to cite this URL:
Vadi S, Mehta M. Supraventricular tachycardia as presenting feature of Nevi, Atrial myxoma, Myxoid neurofibromatosis, and Ephelides Syndrome. J Indian coll cardiol [serial online] 2019 [cited 2020 Jul 10];9:169-70. Available from:

  Background Top

NAME syndrome, a subset of Carney complex encompasses Nevi, Atrial myxoma, Myxoid neurofibromatosis, and Ephelides. Sporadic or familial, atrial myxomas can manifest with a variety of presentations. Following is the narration of a patient who presented rather unusually, palpitations secondary to supraventricular tachycardia due to an underlying atrial myxoma.

  Case Scenario Top

A 60-year-old female presented to the emergency room with palpitations. Her heart rate was at 150 beats/min with blood pressure of 90/60 mmHg. Electrocardiogram revealed supraventricular tachycardia. Pharmacological cardioversion reverted rhythm to normal. Her serum electrolyte levels returned normal. Thyroid profile was within the normal limits. An unsatisfactory window of transthoracic echocardiogram revealed a left atrial mass. Hence, transesophageal echocardiography was performed for surgical planning that showed a left atrial mass likely myxoma attached to the septum [Figure 1]. Preoperative coronary angiogram was normal. Detailed physical examination revealed scattered nevi [Figure 2], multiple neurofibromas [Figure 2] and [Figure 3], as also ephelides [Figure 3]. A week later, she underwent excision of the left atrial myxoma uneventfully. A neurofibroma was also biopsied then. Postoperative course was stable. Histopathology revealed atrial myxoma with myxoid changes in the neurofibroma. Subsequently, she was discharged home. She has remained asymptomatic at 1½ years of follow-up.
Figure 1: Transesophageal echocardiography: Marker at zero degree; view is modified to optimize left atiral (LA) mass visualization. LA myxoma occupies nearly 80% of LA; myxoma is attached to interatrial septum

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Figure 2: Nevi

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Figure 3: Neurofibromas and ephelides

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  Discussion Top

Cardiac myxomas usually occur sporadically. However, about 7% are associated with Myxoma syndrome,[1] with autosomal dominant inheritance. Myxoma syndromes include Carney complex and its subsets NAME syndrome, and Lentigines, Atrial myxoma, Mucocutaneous myxoma, Blue nevi syndrome. These usually present between the third and sixth decades.

Myxomas commonly present with the “myxoma triad” obstructive symptoms such as intracardiac obstruction of blood flow with syncopal attacks, cardiac failure, or complete occlusion of the valvular opening leading to sudden cardiac death, constitutional symptoms, namely, fever, loss of weight, fatigue, arthralgia, myalgia, and embolic symptoms involving the skin[2] or central nervous system leading to stroke (ischemic or hemorrhagic). Clinical manifestations depend on the size and location of the myxoma. Cardiac auscultation may disclose accentuated first heart sound, apical diastolic murmur, or tumor plop. Myocardial invasion may lead to conduction abnormalities or arrhythmias presenting as palpitations.[3] Dermatological manifestations in NAME syndrome comprise nevi (moles), ephelides (freckles), and neurofibromas. Careful physical examination disclosed the patient to possess all the said dermatological characteristics. However, she had never sought medical opinion for her apparent skin traits.

Cardiac myxomas are benign neoplasms that can be differentiated from intracardiac thrombus or vegetation by echocardiography, the investigation of choice. An electrocardiogram may be normal, show atrial enlargement depending on the site or reveal arrhythmias,[4],[5] supraventricular tachycardia as seen in our patient.

Management includes surgical excision. Recurrence is seen with incomplete resection[1] and in 10%–25% of cases of familial myxomas.[6] Anticoagulation does not help as emboli are fragments of the myxoma.

  Conclusion Top

When inquiring into the history and performing a physical examination, a clinician sets up list of differentials of the common causes of clinical features. Once common causes are eliminated, the focus should be diverted to the rarer etiologies, looking for cues that will support the hypothesis. Supraventricular tachycardia, an arrhythmia commonly encountered in the intensive care unit, can be a harbinger of a rare disease as seen in the case deliberated above. By presenting this case, we wish to highlight the significance of recognizing a common presentation of an uncommon disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

McCarthy PM, Piehler JM, Schaff HV, Pluth JR, Orszulak TA, Vidaillet HJ Jr., et al. The significance of multiple, recurrent, and “complex” cardiac myxomas. J Thorac Cardiovasc Surg 1986;91:389-96.  Back to cited text no. 1
Bolognia JL, Braverman IM. Skin manifestations of internal disease. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison's Principles of Internal Medicine. 18th ed. New York: McGraw Hill Education; 2011. p. 405-23.  Back to cited text no. 2
Loscalzo J. Palpitations. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison's Principles of Internal Medicine. 18th ed. New York: McGraw Hill Education; 2011. p. 295-6.  Back to cited text no. 3
Seol SH, Kim DI, Jang JS, Yang TH, Kim DK, Kim DS. Left atrial myxoma presenting as paroxysmal supraventricular tachycardia. Heart Lung Circ 2014;23:e65-6.  Back to cited text no. 4
Nina VJ, Silva NA, Gaspar SF, Rapôso TL, Ferreira EC, Nina RV, et al. Atypical size and location of a right atrial myxoma: A case report. J Med Case Rep 2012;6:26.  Back to cited text no. 5
Singh SD, Lansing AM. Familial cardiac myxoma – A comprehensive review of reported cases. J Ky Med Assoc 1996;94:96-104.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3]


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