First Case of Tricuspid Valve Surgery in a Neurofibromatosis Type 1 Patient.

Autor: Saccocci M; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy., Ferraro F; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy.; Department of Cardiovascular Surgery, University Hospital Policlinico A. Gemelli, Rome, Italy., Blasi S; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy., Del Zanna N; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy.; Department of Cardiovascular Surgery, University Hospital Policlinico A. Gemelli, Rome, Italy., Villa E; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy., Messina A; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy., Cirillo M; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy.; Heart Failure Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy., Mhagna Z; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy., Tomba MD; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy., Troise G; Cardiac Surgery Unit, Cardiovascular Department, H Poliambulanza Foundation, Brescia, Italy.
Jazyk: angličtina
Zdroj: Heart views : the official journal of the Gulf Heart Association [Heart Views] 2021 Jul-Sep; Vol. 22 (3), pp. 214-219. Date of Electronic Publication: 2021 Oct 11.
DOI: 10.4103/HEARTVIEWS.HEARTVIEWS_17_21
Abstrakt: 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.
Competing Interests: There are no conflicts of interest.
(Copyright: © 2021 Heart Views.)
Databáze: MEDLINE
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