Analiza ishoda liječenja djece sa sindromom hipoplastičnoga lijevoga srca u Hrvatskoj

doctoral thesis

• Title (english): Analysis of treatment outcome in children with hypoplastic left heart syndrome in Croatia
• Author: Andrea Dasović Buljević
• Mentor: Ivan Malčić (mentor)
• Committee member: nije dostupno (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; Zagreb
• Defense date and country: 2019-07-10, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences
• Universal decimal classification (UDC): 61 - Medical sciences

Abstract

Sindrom hipoplastičnoga lijevoga srca (HLHS, prema engl. hypoplastic left heart syndrome) označava spektar poremećaja razvoja svih sastavnica lijevoga srca. Incidencija HLHS-a u Hrvatskoj prema nacionalnoj populacijskoj studiji iznosi 2,3% od svih srčanih grešaka. Dijagnoza se postavlja ehokardiografijom. Nakon inicijalnog zbrinjavanja, provode se tri palijacijske operacije koje omogućuju funkciju jedine, desne klijetke kao sistemske. To su operacije po Norwood-u, Glenn-u i Fontan-u. Cilj je ovoga rada bio procijeniti ishod liječenja bolesnika s HLHS-om. Ispitanici su svi bolesnici operirani zbog HLHS-a rođeni u Hrvatskoj u periodu od dvanaest godina (1999.-2011.), njih 57 operiranih u dva inozemna centra koji su zbrinjavani u Klinici za pedijatriju KBC-a Zagreb. Glavne metode bile su statističke, a studija kreirana prema kliničkim i demografskim obilježjima. Ishod liječenja u svih ispitanika procijenjen je prema preživljenju nakon i između operacija od Norwood-ove u novorođenačkoj dobi do Glenn-ove (PCPC) te od Glenn-ove tijekom dojenačke dobi do Fontan-ove operacije (TCPC) i nakon završenog palijacijskog kirurškog liječenja.
Najviše ispitanika umrlo je između prve i druge te druge i treće operacije, a potom nastupa mirno razdoblje. Ukupno dugoročno preživljenje određeno Kaplan-Meier-ovom metodom iznosi 54% (43-69%). Gotovo 80% bolesnika ima odličnu kvalitetu života, dok je u malog broja bolesnika ona loša ili vrlo loša (po 3,5%). Sve veći broj bolesnika doživljava odraslu dob.

Abstract (english)

Hypoplastic left heart syndrome (HLHS) indicates the spectrum of developmental disorders of all left heart components. The incidence of HLHS in Croatia is 2.3% among congenital heart malformations. Diagnosis is primarily attributed to echocardiography. After initial stabilisation, the main treatment method are three palliative operations that allow lifelong function of the right ventricle as systemic (operations by Norwood, Glenn and Fontan). The aim of this study was to evaluate the outcomes of treating patients with HLHS. We included 57 patients in 12-year period operated in foreign centers, who have been treated at University hospital center Zagreb, before and in the meantime. The main methods were statistical, and the study was created according to clinical and demographic characteristics. The outcome of treatment was estimated to survive after and between surgery - from Norwood to Glenn (PCPC), Glenn to Fontan (TCPC) and after completion of surgical palliative treatment.
The majority of subjects passed in interstage, followed by a quiet period. Total long-term survival by Kaplan-Meier’s is 54% (43-69%). Almost 80% of patients have excellent quality of life, while negligible number of patients have poor or very poor quality of life (3,5%). An increasing number of patients are experiencing an adult age.

• Keywords: medicina
• Keywords (english): medicine
• Language: croatian
• URN:NBN: urn:nbn:hr:105:963017
• Study programme: Title: Biomedicine and Health Sciences; Study programme type: university; Study level: postgraduate; Academic / professional title: doktor/doktorica znanosti, područje biomedicine i zdravstva (doktor/doktorica znanosti, područje biomedicine i zdravstva)
• Type of resource: Text
• Extent: 54 str.
• File origin: Born digital
• Access conditions: Open access
• Created on: 2023-05-05 08:38:37