Tetralogy of Fallot

master's thesis

• Title (croatian): Tetralogija Fallot
• Author: Eleas Iecho
• Mentor: Daniel Dilber (mentor)
• Committee member: Daniel Dilber (predsjednik povjerenstva)
• Committee member: Ruža Grizelj (član povjerenstva)
• Committee member: Marija Jelušić (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; Zagreb
• Defense date and country: 2021-12-07, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Pediatrics

Abstract

Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital cardiac
malformations and is characterized by four cardinal features: a large VSD; often a
dynamic right ventricular outflow tract obstruction (RVOTO); an overriding aorta; and
right ventricular hypertrophy (RVH). The severity of the RVOTO, the pressure
gradient between the ventricles, and the proportion of the aorta overriding the VSD
determine the clinical presentation and the severity of this condition. There are
several variants of this condition, that range from mild to very severe, which is
distinguished by the degree of the cyanosis. Exaggeration of the condition might
occur, which is known as hypercyanotic spells, or tet spells. These spells result in
worsening of the right-to-left shunt that leads to a worsening of cyanosis, and if left
untreated it will result in death. In the current era, more and more cases are
diagnosed antenatally due to better screenings during the pregnancy but also due to
improved diagnostic procedures.
The majority of cases are managed by a single-step surgical correction. Due to an
improved surgical technique, most cases of TOF could be completely repaired within
the first 6 months of life. Although, if the case is severe, a two-step procedure must
be done, first-step would be an insertion of a palliative shunt, and after few months
the second-step which is a complete correction.

Abstract (croatian)

Tetralogija Fallot (TOF) jedna je od najčešćih cijanotičnih kongenitalnih srčanih
malformacija i karakteriziraju je četiri kardinalne značajke: veliki VSD; često
dinamička opstrukcija izlaznog trakta desne klijetke (RVOTO); nadmoćna aorta; i hipertrofija desne klijetke (RVH). Ozbiljnost RVOTO-a, gradijent tlaka između
ventrikula i udio aorte koja nadmašuje VSD određuju kliničku sliku i težinu ovog
stanja. Postoji nekoliko varijanti ovog stanja, od blage do vrlo teške, koje se razlikuje
po stupnju cijanoze. Može doći do preuveličavanja stanja, što je poznato kao
hipercijanotične čarolije ili tet čarolije. Ove čarolije rezultiraju pogoršanjem šanta od
desne na lijevu stranu što dovodi do pogoršanja cijanoze, a ako se ne liječe,
rezultirat će smrću. U današnje vrijeme sve se više slučajeva dijagnosticira
antenatalno zbog boljih probira tijekom trudnoće, ali i zbog poboljšanih dijagnostičkih
postupaka.
U većini slučajeva upravlja se kirurškom korekcijom u jednom koraku. Zbog
poboljšane kirurške tehnike, većina slučajeva TOF-a može se u potpunosti popraviti
unutar prvih 6 mjeseci života. Iako, ako je slučaj težak, mora se napraviti zahvat u
dva koraka, prvi bi korak bio umetanje palijativnog šanta, a nakon nekoliko mjeseci
drugi korak koji je potpuna korekcija.

• Keywords: Tetralogy of Fallot; congenital heart defect; hypercyanotic spells; tet spells; right ventricular outflow tract obstruction; nonrestrictive ventricular septal defect
• Keywords (croatian): Fallotova tetralogija; kongenitalna srčana mana; hipercijanotični napadi; tet napadi; opstrukcija izlaznog trakta desne klijetke; nerestriktivni defekt ventrikularnog septuma
• Language: english
• URN:NBN: urn:nbn:hr:105:518861
• Study programme: Title: Medicine (in English language); Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2022-12-19 10:17:12