Chronically ill children with congenital heart defects

Cilj diplomskog rada je ukazati na probleme koji su prisutni kod kronično bolesne djece sa srčanim grješkama. Radi se o preglednom radu u kojemu želim naglasiti važnost ove posebne populacije te obuhvatiti sve aspekte njihove kronične bolesti. Prirođene srčane grješke najteže su anomalije jednog organskog sustava te najčešći uzrok smrti u perinatalnoj i ranoj neonatalnoj dobi. Mogu se javiti samostalno kao izolirane malformacije ili u sklopu nekih sindroma te združene s ekstrakardijalnim anomalijama (1). Također su najčešće anomalije koje prelaze u kroničnu bolest zbog čega se formirao javnozdravstveni problem odraslih s prirođenim srčanim grješkama. Svaka grana medicine neprestano napreduje i dolazi do novih spoznaja pa tako i pedijatrijska kardiologija koja predstavlja prvu stepenicu u zbrinjavanju ove patologije. Kako bi se pružio optimalan terapijski pristup, najbitnije je naglasiti rano postavljanje indikacije za fetalnu ehokardiografiju i prepoznavanje točne dijagnoze iza čega slijedi pravovremena intervencija. Kao i kod svake kronične bolesti, i u ovoj je nužan multidisciplinaran pristup. Također, djeca s PSG-om su izložena brojnim operacijama, interventnim postupcima, čestim kontrolama te boravcima u bolnici. Prirođene srčane grješke najčešće se razvrstavaju prema Clarkovoj klasifikaciji koja se zasniva na pretpostavci da relativno malo razvojnih mehanizama uzrokuje širok spektar fenotipskih oblika grješaka (2). Klinička slika i komplikacije, koje se mogu razviti, nisu jednake kod svih srčanih grješaka. Kontinuitet skrbi, kako u dječjoj dobi tako i u odrasloj dobi, igra najbitniju ulogu u smanjenju komplikacija i smrtnosti bolesnika sa PSG-om. Važno je naglasiti i obitelj koja nosi veliku ulogu u svladavanju prepreka i suočavanju s teškoćama koje dolaze s ovom kroničnom bolešću.The aim of the diploma thesis is to indicate problems present in chronically ill children with heart defects. It is a scientific review in which I would like to point out the importance of this special population and cover all the aspects of their chronic illness. Congenital heart malformations are the most difficult anomalities of ones body system and the most frequent cause of death in perinatal and early neonatal age. They can appear individually as isolated malformations or accompanied with other extracardial anomalies (1). They are also the most common malformations marked by transition into a chronic disease. Therefore, today a public health entity known as adults with congenital heart disease has been established. Pediatric cardiology, like all branches of medicine, is constantly advancing and obtaining new scientific knowledge and represents the first step in the management of this pathology. The optimal assessment is associated with early indication for fetal echocardiography, the most important procedure, and establishment of an accurate diagnosis that leads to on-time intervention. Just like in every chronic illness, a multidisciplinary approach is necessary. Children with CHD are exposed to numerous operations, interventional procedures, frequent check-ups, and hospitalizations. Congenital heart malformations are most commonly classified by Clark’s categorization, which is based on the assumption that merely a few developing mechanisms cause a wide spectrum of phenotypic forms of defects (2). The clinical presentation and complications are not the same for all congenital heart defects. Continuity of care in childhood, as well as in adulthood, plays the most important role in reducing complications and mortality of patients with CHD. It is important to emphasize the essential role of the patient’s family in overcoming all the obstacles and coping with the difficulties that come with this chronic illness