Login | Users Online: 152  
Home Print this page Email this page Small font sizeDefault font sizeIncrease font size   
Home | About us | Editorial board | Search | Ahead of print | Current Issue | Archives | Submit article | Instructions | Subscribe | Advertise | Contact us
Year : 2021  |  Volume : 22  |  Issue : 3  |  Page : 214-219

First case of tricuspid valve surgery in a neurofibromatosis type 1 patient

1 Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy
2 Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia; Department of Cardiovascular Surgery, University Hospital Policlinico A. Gemelli, Rome, Italy
3 Cardiac Surgery Unit; Heart Failure Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy

Correspondence Address:
Dr. Matteo Saccocci
Poliambulanza Foundation Hospital, Via L. Bissolati 57, 25122, Brescia
Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and Permissions

The neurofibromatosis is a large class of different genetic disorders: Neurofibromatosis type 1, type 2, type 3 (or Schwannomatosys), which have different clinical characterization. Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen disease, represents 95% of the total cases. It is a complex autosomal dominant disorder with multisystem involvement, frequently associated to cardiac malformation. We present the case of a 52-years-old male affected by NF-1 with severe tricuspid regurgitation and atrial septal defect (ASD). No previous report about tricuspid valve surgery in NF-1 are available in the literature. A complete perioperative assessment was performed, including dermatologist evaluation, angio-CT scan and trans-esophageal echocardiography. The patient underwent uneventfully tricuspid valve replacement and ASD closure, with no wound complication even at 6-months follow-up. Treating congenital malformation in patient with complex genetic disorders like NF-1 is safe and can be resolutive, permitting to reduce long-term risk of complications for the patients. Preoperative assessments are fundamental, as well as in-hospital care and expertise on congenital heart defects.

Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)

 Article Access Statistics
    PDF Downloaded34    
    Comments [Add]    

Recommend this journal