Skip to main content
  • American Heart Association
  • Science Volunteer
  • Warning Signs
  • Advanced Search
  • Donate

  • Home
  • About this Journal
    • Editorial Board
    • General Statistics
    • Circulation Doodle
      • Doodle Gallery
      • Circulation Cover Doodle
        • → Blip the Doodle
    • Information for Advertisers
    • Author Reprints
    • Commercial Reprints
    • Customer Service and Ordering Information
    • Subscribe to AHA Journals
  • All Issues
  • Subjects
    • All Subjects
    • Arrhythmia and Electrophysiology
    • Basic, Translational, and Clinical Research
    • Critical Care and Resuscitation
    • Epidemiology, Lifestyle, and Prevention
    • Genetics
    • Heart Failure and Cardiac Disease
    • Hypertension
    • Imaging and Diagnostic Testing
    • Intervention, Surgery, Transplantation
    • Quality and Outcomes
    • Stroke
    • Vascular Disease
  • Browse Features
    • AHA Guidelines and Statements
      • Recently Published Guidelines
    • Bridging Disciplines
    • Circulation at Major Meetings
    • Special Themed Issues
    • Global Impact of the 2017 ACC/AHA Hypertension Guidelines
    • Circulation Supplements
    • Cardiovascular Case Series
    • ECG Challenge
    • Hospitals of History
      • Brigham and Women's Hospital
      • Hartford Hospital
      • Hospital Santa Maria del Popolo, Naples, Italy
      • Instituto do Coração-INCOR (São Paulo, Brasil)
      • Minneapolis City Hospital
      • Parkland Hospital: Dallas, Texas
      • Pennsylvania Hospital, Philadelphia
      • Pitié-Salpêtrière Hospital
      • Royal Infirmary of Edinburgh, Scotland
      • Tufts Medical Center
      • University of Michigan
      • Uppsala University Hospital
      • Vassar Brothers Medical Center (Poughkeepsie, NY)
      • Wroclaw Medical University
      • Women's College Hospital, Toronto, Canada
      • Henry Ford Hospital, Detroit, Michigan
      • Instituto Nacional de Cardiología Ignacio Chávez – INCICh México City, México
      • Kuang-Tien General Hospital (Taichug, Taiwan)
    • On My Mind
    • Podcast Archive
    • → Subscribe to Circulation on the Run
    • →Circulation FIT Podcast 2018
    • → #FITFAVs
  • Resources
    • Instructions for Authors
      • Accepted Manuscripts
      • Revised Manuscripts
    • → Article Types
    • → General Preparation Instructions
    • → Research Guidelines
    • → How to Submit a Manuscript
    • Journal Policies
    • Permissions and Rights Q&A
    • Submission Sites
    • Circulation CME
    • AHA Journals RSS Feeds
    • International Users
    • AHA Newsroom
  • AHA Journals
    • AHA Journals Home
    • Arteriosclerosis, Thrombosis, and Vascular Biology (ATVB)
    • Circulation
    • → Circ: Arrhythmia and Electrophysiology
    • → Circ: Genomic and Precision Medicine
    • → Circ: Cardiovascular Imaging
    • → Circ: Cardiovascular Interventions
    • → Circ: Cardiovascular Quality & Outcomes
    • → Circ: Heart Failure
    • Circulation Research
    • Hypertension
    • Stroke
    • Journal of the American Heart Association
  • Facebook
  • Twitter

  • My alerts
  • Sign In
  • Join

  • Advanced search

Header Publisher Menu

  • American Heart Association
  • Science Volunteer
  • Warning Signs
  • Advanced Search
  • Donate

Circulation

  • My alerts
  • Sign In
  • Join

  • Facebook
  • Twitter
  • Home
  • About this Journal
    • Editorial Board
    • General Statistics
    • Circulation Doodle
    • Information for Advertisers
    • Author Reprints
    • Commercial Reprints
    • Customer Service and Ordering Information
    • Subscribe to AHA Journals
  • All Issues
  • Subjects
    • All Subjects
    • Arrhythmia and Electrophysiology
    • Basic, Translational, and Clinical Research
    • Critical Care and Resuscitation
    • Epidemiology, Lifestyle, and Prevention
    • Genetics
    • Heart Failure and Cardiac Disease
    • Hypertension
    • Imaging and Diagnostic Testing
    • Intervention, Surgery, Transplantation
    • Quality and Outcomes
    • Stroke
    • Vascular Disease
  • Browse Features
    • AHA Guidelines and Statements
    • Bridging Disciplines
    • Circulation at Major Meetings
    • Special Themed Issues
    • Global Impact of the 2017 ACC/AHA Hypertension Guidelines
    • Circulation Supplements
    • Cardiovascular Case Series
    • ECG Challenge
    • Hospitals of History
    • On My Mind
    • Podcast Archive
    • → Subscribe to Circulation on the Run
    • →Circulation FIT Podcast 2018
    • → #FITFAVs
  • Resources
    • Instructions for Authors
    • → Article Types
    • → General Preparation Instructions
    • → Research Guidelines
    • → How to Submit a Manuscript
    • Journal Policies
    • Permissions and Rights Q&A
    • Submission Sites
    • Circulation CME
    • AHA Journals RSS Feeds
    • International Users
    • AHA Newsroom
  • AHA Journals
    • AHA Journals Home
    • Arteriosclerosis, Thrombosis, and Vascular Biology (ATVB)
    • Circulation
    • → Circ: Arrhythmia and Electrophysiology
    • → Circ: Genomic and Precision Medicine
    • → Circ: Cardiovascular Imaging
    • → Circ: Cardiovascular Interventions
    • → Circ: Cardiovascular Quality & Outcomes
    • → Circ: Heart Failure
    • Circulation Research
    • Hypertension
    • Stroke
    • Journal of the American Heart Association
Images in Cardiovascular Medicine

Cardiac Rhabdomyoma in an Adult With a Previous Presumptive Diagnosis of Septal Hypertrophy

Ricardo Wage, Henryk Kafka, Sanjay Prasad
Download PDF
https://doi.org/10.1161/CIRCULATIONAHA.107.744904
Circulation. 2008;117:e469-e470
Originally published June 2, 2008
Ricardo Wage
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Henryk Kafka
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sanjay Prasad
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Tables
  • Supplemental Materials
  • Info & Metrics
  • eLetters

Jump to

  • Article
    • Sources of Funding
    • Footnotes
    • References
  • Figures & Tables
  • Supplemental Materials
  • Info & Metrics
  • eLetters
Loading

An 18-year-old woman had been complaining of palpitations and had suffered several episodes of syncope. When the patient was 6 years of age, echocardiography had revealed a localized area of septal hypertrophy without left ventricular outflow tract obstruction, and she was diagnosed with hypertrophic cardiomyopathy. Because of her recent symptoms, she was referred for a number of neurological and cardiac investigations, including cardiovascular magnetic resonance imaging (CMR).

On CMR, there was a localized mass in the interventricular septum that extended from the level of the aortic valve toward the midcavity for a distance of approximately 50 mm, measuring 30 mm at its maximum width. On steady-state free precession cine, it appeared to be homogeneous without any definite demarcation from surrounding myocardium (Figure, A and B, and Movies I through V), and there was limited systolic motion of that part of the septum. There was no evidence of left ventricular outflow tract obstruction. No calcification was noted. T1-weighted imaging showed a homogeneous isointense signal compared with surrounding myocardium (Figure, C). T2 short tau inversion recovery images showed a hyperintense signal within the mass (Figure, D) that differentiated it from the surrounding myocardium. Perfusion imaging demonstrated complete opacification of the full thickness of the mass with almost immediate opacification of the central portion (Movie VI). There was early gadolinium uptake in the mass with enhancement that was more marked than in the surrounding myocardium (Figure, E). Abnormal late enhancement was visible across the entire thickness of the mass (Figure, F).

Figure1
  • Download figure
  • Open in new tab
  • Download powerpoint

Figure. A, Short-axis still image from an steady-state free precession cine obtained through the proximal septum. The markedly thickened septum (arrow) appears to be homogeneous with the rest of the left ventricle (LV) and right ventricle (RV) myocardium. B, Still image from an steady-state free precession cine showing the LV outflow tract (arrow). Despite the marked enlargement of the septum, there was no measured gradient in the LV outflow tract. C, Short-axis turbo spin echo image with T1 weighting demonstrates that the signal from the markedly thickened septum is isointense compared with the rest of the LV myocardium. D, Short-axis T2 short tau inversion recovery image demonstrates a hyperintense signal within the thickened septum, suggesting a different structure from the surrounding myocardium. E, Short-axis image obtained early after gadolinium injection with enhancement of the LV and RV cavities, as well as the myocardium, including the thickened septum. F, Short-axis image obtained late after gadolinium injection. Normal myocardium appears black. There is marked abnormal late enhancement throughout the entire segment of thickened septum.

We concluded that the markedly thickened appearance of the septum was due not to localized left ventricular hypertrophy but to the presence of a hamartoma. Subsequently, the patient’s episodes of syncope were classified as seizures, and further neurological investigation led to a diagnosis of tuberous sclerosis. This combination of her CMR imaging features and the presence of tuberous sclerosis resulted in a final diagnosis of cardiac rhabdomyoma.

CMR is well established in the evaluation of patients with hypertrophic cardiomyopathy and is ideally suited to the assessment of possible cardiac tumors. Factors in determining tumor type by CMR scanning include location and imaging features, such as intensity with T1 and T2 weighting, and whether there is contrast uptake.1,2 Location and imaging features in the present case were certainly in keeping with rhabdomyoma except for the fact that there was only a single tumor, as rhabdomyomas are frequently multiple. The pattern documented in the present case suggested replacement of myocardium by abnormal tissue and supported the diagnosis of a tumor.

Cardiac rhabdomyoma is found in more than 50% of children with tuberous sclerosis complex.3,4 There can be partial or complete resolution of the cardiac tumors, even as tumors elsewhere fail to regress; yet, cardiac rhabdomyoma has been documented to increase in size or appear de novo in some patients.3 Because cardiac rhabdomyoma tends to resolve, the patient is usually followed clinically unless the tumor gives rise to important left ventricular outflow tract obstruction, left ventricular failure, or arrhythmias. In this patient who has a tumor and episodes of loss of consciousness ascribed to seizures, it will be important to ensure that a future diagnosis of symptomatic arrhythmia is not missed.

Sources of Funding

Dr Kafka has received financial support as a Detweiler Fellow of the Royal College of Physicians and Surgeons of Canada.

Disclosures

None.

Footnotes

  • The online-only Data Supplement, which contains Movies I through VI, is available with this article at http://circ.ahajournals.org/cgi/content/full/117/22/ e469/DC1.

References

  1. ↵
    Luna A, Ribes R, Caro P, Vida J, Erasmus JJ. Evaluation of cardiac tumors with magnetic resonance imaging. Eur Radiol. 2005; 15: 1446–1455.
    OpenUrlCrossRefPubMed
  2. ↵
    Restrepo CS, Largoza A, Lemos DF, Diethelm L, Koshy P, Castillo P, Gomez R, Moncada R, Pandit M. CT and MR imaging findings of benign cardiac tumors. Curr Probl Diagn Radiol. 2005; 34: 12–21.
    OpenUrlCrossRefPubMed
  3. ↵
    Freedom RM, Lee KJ, MacDonald C, Taylor G. Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol. 2000; 21: 299–316.
    OpenUrlCrossRefPubMed
  4. ↵
    Józwiak S, Kotulska K, Kasprzyk-Obara J, Domanska-Pakiela D, Tomyn-Drabik M, Roberts P, Kwiatkowski D. Clinical and genotype studies of cardiac tumors in 154 patients with tuberous sclerosis complex. Pediatrics. 2006; 118: 1146–1151.
    OpenUrlCrossRef
View Abstract
Back to top
Previous ArticleNext Article

This Issue

Circulation
June 3, 2008, Volume 117, Issue 22
  • Table of Contents
Previous ArticleNext Article

Jump to

  • Article
    • Sources of Funding
    • Footnotes
    • References
  • Figures & Tables
  • Supplemental Materials
  • Info & Metrics
  • eLetters

Article Tools

  • Print
  • Citation Tools
    Cardiac Rhabdomyoma in an Adult With a Previous Presumptive Diagnosis of Septal Hypertrophy
    Ricardo Wage, Henryk Kafka and Sanjay Prasad
    Circulation. 2008;117:e469-e470, originally published June 2, 2008
    https://doi.org/10.1161/CIRCULATIONAHA.107.744904

    Citation Manager Formats

    • BibTeX
    • Bookends
    • EasyBib
    • EndNote (tagged)
    • EndNote 8 (xml)
    • Medlars
    • Mendeley
    • Papers
    • RefWorks Tagged
    • Ref Manager
    • RIS
    • Zotero
  •  Download Powerpoint
  • Article Alerts
    Log in to Email Alerts with your email address.
  • Save to my folders

Share this Article

  • Email

    Thank you for your interest in spreading the word on Circulation.

    NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

    Enter multiple addresses on separate lines or separate them with commas.
    Cardiac Rhabdomyoma in an Adult With a Previous Presumptive Diagnosis of Septal Hypertrophy
    (Your Name) has sent you a message from Circulation
    (Your Name) thought you would like to see the Circulation web site.
  • Share on Social Media
    Cardiac Rhabdomyoma in an Adult With a Previous Presumptive Diagnosis of Septal Hypertrophy
    Ricardo Wage, Henryk Kafka and Sanjay Prasad
    Circulation. 2008;117:e469-e470, originally published June 2, 2008
    https://doi.org/10.1161/CIRCULATIONAHA.107.744904
    del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo

Related Articles

Cited By...

Subjects

  • Intervention, Surgery, Transplantation
    • Cardiovascular Surgery
  • Imaging and Diagnostic Testing
    • Imaging
  • Heart Failure and Cardiac Disease
    • Cardiomyopathy

Circulation

  • About Circulation
  • Instructions for Authors
  • Circulation CME
  • Statements and Guidelines
  • Meeting Abstracts
  • Permissions
  • Journal Policies
  • Email Alerts
  • Open Access Information
  • AHA Journals RSS
  • AHA Newsroom

Editorial Office Address:
200 Fifth Avenue, Suite 1020
Waltham, MA 02451
email: circ@circulationjournal.org
 

Information for:
  • Advertisers
  • Subscribers
  • Subscriber Help
  • Institutions / Librarians
  • Institutional Subscriptions FAQ
  • International Users
American Heart Association Learn and Live
National Center
7272 Greenville Ave.
Dallas, TX 75231

Customer Service

  • 1-800-AHA-USA-1
  • 1-800-242-8721
  • Local Info
  • Contact Us

About Us

Our mission is to build healthier lives, free of cardiovascular diseases and stroke. That single purpose drives all we do. The need for our work is beyond question. Find Out More about the American Heart Association

  • Careers
  • SHOP
  • Latest Heart and Stroke News
  • AHA/ASA Media Newsroom

Our Sites

  • American Heart Association
  • American Stroke Association
  • For Professionals
  • More Sites

Take Action

  • Advocate
  • Donate
  • Planned Giving
  • Volunteer

Online Communities

  • AFib Support
  • Garden Community
  • Patient Support Network
  • Professional Online Network

Follow Us:

  • Follow Circulation on Twitter
  • Visit Circulation on Facebook
  • Follow Circulation on Google Plus
  • Follow Circulation on Instagram
  • Follow Circulation on Pinterest
  • Follow Circulation on YouTube
  • Rss Feeds
  • Privacy Policy
  • Copyright
  • Ethics Policy
  • Conflict of Interest Policy
  • Linking Policy
  • Diversity
  • Careers

©2018 American Heart Association, Inc. All rights reserved. Unauthorized use prohibited. The American Heart Association is a qualified 501(c)(3) tax-exempt organization.
*Red Dress™ DHHS, Go Red™ AHA; National Wear Red Day ® is a registered trademark.

  • PUTTING PATIENTS FIRST National Health Council Standards of Excellence Certification Program
  • BBB Accredited Charity
  • Comodo Secured