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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Format: | Article |
Language: | English |
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Wolters Kluwer Medknow Publications
2021-01-01
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Series: | Heart Views |
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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. |
first_indexed | 2024-12-18T02:02:51Z |
format | Article |
id | doaj.art-8b484537dfd640e9a7beca895db28254 |
institution | Directory Open Access Journal |
issn | 1995-705X |
language | English |
last_indexed | 2024-12-18T02:02:51Z |
publishDate | 2021-01-01 |
publisher | Wolters Kluwer Medknow Publications |
record_format | Article |
series | Heart Views |
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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