HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text 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 Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Francesca Viscardi Alessandro Mazzucco Giovanni Battista Luciani Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Viscardi, F. Articles by Luciani, G. B. Search for Related Content PubMed Articles by Viscardi, F. Articles by Luciani, G. B. Related Collections Congenital - cyanotic Valve disease

J Thorac Cardiovasc Surg 2009;137:751-753
© 2009 The American Association for Thoracic Surgery

Brief Communication

Familial fetal-type rhabdomyoma of the tricuspid valve in the neonate: Malignant course for a benign disease

^a Division of Cardiac Surgery, University of Verona, Verona, Italy
^b Division of Pediatrics, University of Verona, Verona, Italy
^c Division of Pathology, University of Verona, Verona, Italy

Received for publication November 17, 2007; accepted for publication March 23, 2008.

^_* Address for reprints: Giovanni Battista Luciani, MD, Division of Cardiac Surgery, University of Verona, O. C. M. Piazzale Stefani 1, Verona, 37126, Italy. (Email: gbluciani@yahoo.com).

The first 300 words of the full text of this article appear below.

We report 2 brothers with prenatal diagnosis of tricuspid valve masses, both requiring aggressive intensive care resuscitation shortly after birth and surgical excision of the tumor, which proved to be a fetal-type rhabdomyoma. The unique pathologic and clinical features of this rare disease are discussed.

Clinical Summary

Patient 1. The first child had a critical neonatal period due to severe tricuspid valve stenosis, caused by 3 obstructive cardiac masses and severe pulmonary hypertension. Treatment included balloon atrial septostomy to maintain cardiac output and mechanical ventilation, with prostaglandin E1 (PGE1) infusion and inhaled nitric oxide (NO) for 2 months after birth. Discharge therapy with sildenafil and bosentan was continued until the time of elective surgical resection of tricuspid valve masses (Figure 1, A), at 11 months of age. Histology showed only dystrophic calcification. Excellent tricuspid valve function and no recurrence of cardiac masses were documented at follow-up echocardiogram 1 year after operation. The child has no clinical nor laboratory evidence of tuberous sclerosis.

View larger version (73K):   Figure 1. A, Operative view of the 3 tricuspid valve masses via right atriotomy in patient 1. All 3 valve leaflets were encased in firm, amorphous sclerocalcified masses. B, Operative view of the tricuspid valve masses via right atriotomy in patient 2. A single, large, and friable sclerocalcified mass, adherent to the right atrial free wall and obstructing almost completely the tricuspid valve orifice, was present. Residual tricuspid valve tissue was barely visible.