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

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 c...

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Main Authors: Matteo Saccocci, Francesco Ferraro, Stefania Blasi, Niccolò Del Zanna, Emmanuel Villa, Antonio Messina, Marco Cirillo, Zean Mhagna, Margherita Dalla Tomba, Giovanni Troise
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2021-01-01
Series:Heart Views
Subjects:
Online Access:http://www.heartviews.org/article.asp?issn=1995-705X;year=2021;volume=22;issue=3;spage=214;epage=219;aulast=Saccocci
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author Matteo Saccocci
Francesco Ferraro
Stefania Blasi
Niccolò Del Zanna
Emmanuel Villa
Antonio Messina
Marco Cirillo
Zean Mhagna
Margherita Dalla Tomba
Giovanni Troise
author_facet Matteo Saccocci
Francesco Ferraro
Stefania Blasi
Niccolò Del Zanna
Emmanuel Villa
Antonio Messina
Marco Cirillo
Zean Mhagna
Margherita Dalla Tomba
Giovanni Troise
author_sort Matteo Saccocci
collection DOAJ
description 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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spelling doaj.art-8b484537dfd640e9a7beca895db282542022-12-21T21:24:43ZengWolters Kluwer Medknow PublicationsHeart Views1995-705X2021-01-0122321421910.4103/HEARTVIEWS.HEARTVIEWS_17_21First case of tricuspid valve surgery in a neurofibromatosis type 1 patientMatteo SaccocciFrancesco FerraroStefania BlasiNiccolò Del ZannaEmmanuel VillaAntonio MessinaMarco CirilloZean MhagnaMargherita Dalla TombaGiovanni TroiseThe 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.http://www.heartviews.org/article.asp?issn=1995-705X;year=2021;volume=22;issue=3;spage=214;epage=219;aulast=Saccoccicardiac anomaliescardiac surgeryneurofibromatosistricuspid valvevon recklinghausen
spellingShingle Matteo Saccocci
Francesco Ferraro
Stefania Blasi
Niccolò Del Zanna
Emmanuel Villa
Antonio Messina
Marco Cirillo
Zean Mhagna
Margherita Dalla Tomba
Giovanni Troise
First case of tricuspid valve surgery in a neurofibromatosis type 1 patient
Heart Views
cardiac anomalies
cardiac surgery
neurofibromatosis
tricuspid valve
von recklinghausen
title First case of tricuspid valve surgery in a neurofibromatosis type 1 patient
title_full First case of tricuspid valve surgery in a neurofibromatosis type 1 patient
title_fullStr First case of tricuspid valve surgery in a neurofibromatosis type 1 patient
title_full_unstemmed First case of tricuspid valve surgery in a neurofibromatosis type 1 patient
title_short First case of tricuspid valve surgery in a neurofibromatosis type 1 patient
title_sort first case of tricuspid valve surgery in a neurofibromatosis type 1 patient
topic cardiac anomalies
cardiac surgery
neurofibromatosis
tricuspid valve
von recklinghausen
url http://www.heartviews.org/article.asp?issn=1995-705X;year=2021;volume=22;issue=3;spage=214;epage=219;aulast=Saccocci
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