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- Title
First Case of Tricuspid Valve Surgery in a Neurofibromatosis Type 1 Patient.
- Authors
Saccocci, Matteo; Ferraro, Francesco; Blasi, Stefania; Del Zanna, Niccolò; Villa, Emmanuel; Messina, Antonio; Cirillo, Marco; Mhagna, Zean; Tomba, Margherita Dalla; Troise, Giovanni
- Abstract
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 transesophageal 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.
- Subjects
ATRIAL septal defects; NEUROFIBROMATOSIS 1; TRANSESOPHAGEAL echocardiography; TRICUSPID valve surgery; CONGENITAL heart disease; TRICUSPID valve; GENETIC disorders; HUMAN abnormalities
- Publication
Heart Views, 2021, Vol 22, Issue 3, p214
- ISSN
1995-705X
- Publication type
Article
- DOI
10.4103/HEARTVIEWS.HEARTVIEWS_17_21