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CASE REPORT
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
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/HEARTVIEWS.HEARTVIEWS_17_21

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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.


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