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(Circulation. 2001;104:e132.)
© 2001 American Heart Association, Inc.

Images in Cardiovascular Medicine

"Petrified" Right Ventricle in Long-Standing Naxos Arrhythmogenic Right Ventricular Cardiomyopathy

Cristina Basso, MD, PhD; Adalena Tsatsopoulou, MD; Gaetano Thiene, MD; Aris Anastasakis, MD; Marialuisa Valente, MD; Nikos Protonotarios, MD

From the Institute of Pathology, University of Padua, Italy (C.B., G.T., M.V.); Yannis Protonotarios Medical Center Hora Naxos, Greece (A.T., N.P.); and the Department of Cardiology, University of Athens, Greece (A.A.).

Correspondence to Gaetano Thiene, MD, FESC, Istituto di Anatomia Patologica, Via A. Gabelli 61, 35121 Padova, Italy. E-mail cardpath{at}unipd.it

A 19-year-old asymptomatic man was evaluated because of his sister’s sudden death due to Naxos disease, a recessive form of arrhythmogenic right ventricular (RV) cardiomyopathy associated with palmoplantar keratoderma and woolly hair. It is caused by a plakoglobin mutation. Twelve-lead ECG showed inverted T waves in V1 through V6, incomplete right bundle branch block, and epsilon waves (Figure 1). Frequent ventricular extrasystoles were recorded on a 24-hour ECG. On echocardiography and angiography, the right ventricle was dilated and diffusely hypokinetic, but the left ventricle appeared normal. During 14 years of follow-up, he had 2 episodes of well-tolerated sustained ventricular tachycardia followed by biventricular progression leading to heart failure (New York Heart Association class III to IV) without arrhythmias. The patient died at 33 years of age while waiting for heart transplantation.

View larger version (72K): [in this window] [in a new window]   Figure 1. The 12-lead ECG shows inverted T waves (V1 to V6), incomplete right bundle branch block (QRS duration, 140 ms in V1), and epsilon waves.

At gross examination and nuclear magnetic resonance (Figures 2 and 3), huge cardiomegaly (heart weight, 630 g) and paper-thin RV walls with transmural myocardial loss were evident, together with subepicardial left ventricular fatty infiltration. A calcified fibrous plaque, 7x9 cm in size, was discovered at the level of the RV outflow tract and confirmed by postmortem x-ray (Figure 4). Histological study showed transmural right and left ventricular myocardial atrophy, with fibrofatty replacement embedding a few surviving myocytes. The peculiar massive calcification of the RV outflow tract, possibly functioning as a ventricular disconnection, might explain the decreased arrhythmogenicity at the end-stage.

View larger version (113K): [in this window] [in a new window]   Figure 2. Parasternal long-axis view of the heart shows paper-thin RV walls, transmural myocardial loss, subepicardial left ventricular fatty infiltration, and calcified fibrous plaque at the level of RV outflow tract.

View larger version (84K): [in this window] [in a new window]   Figure 3. Corresponding long-axis nuclear magnetic resonance scan with diffuse bright signal on anterior RV wall and subepicardial bright signal on the left ventricular wall.

View larger version (95K): [in this window] [in a new window]   Figure 4. Postmortem x-ray reveals diffuse calcification of the anterior RV wall.

Acknowledgments

Supported by the 5th Framework Program Research and Technological Development of the European Commission, Contract N. QLG1-CT-2000-01091 "ARVC/D."

Footnotes

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke‘s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke‘s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

This article has been cited by other articles:

				C. Basso, T. Wichter, G. A Danieli, D. Corrado, E. Czarnowska, G. Fontaine, W. J McKenna, A. Nava, N. Protonotarios, L. Antoniades, et al. Arrhythmogenic right ventricular cardiomyopathy: clinical registry and database, evaluation of therapies, pathology registry, DNA banking Eur. Heart J., March 2, 2004; 25(6): 531 - 534. [Full Text] [PDF]

This Article Extract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Basso, C. Articles by Protonotarios, N. Search for Related Content PubMed PubMed Citation Articles by Basso, C. Articles by Protonotarios, N. Related Collections Congestive Myocardial cardiomyopathy disease Electrocardiology CT and MRI Arrhythmias, clinical electrophysiology, drugs