Abstract

<p>Progress in diagnosis and treatment has led to a significant improvement in the fate of children born with tetralogy of Fallot, with the perioperative mortality of 2-3% and a 30-year survival rate of 90%. However, the majority of these patients have residual postoperative morphological and hemodynamic disorders, as well as heart rhythm disorders, primarily due to the volume loading of the right ventricle caused by chronic pulmonary regurgitation. Improved surgical techniques have reduced early mortality to less than 3%, but the annual mortality rate has increased 20-30 years after initial surgical remediation, mainly due to unwanted cardiac events. In longitudinal monitoring of patients after repair of tetralogy of Fallot, early detection of morphological and hemodynamic residual disorders both in asymptomatic and symptomatic patients is of great importance. The purpose of this is a timely identification of new therapeutic measures (e.g. pulmonary valve replacement) in order to improve the course and outcome of treatment. Cardiovascular magnetic resonance imaging is a diagnostic method that provides the most precise and accurate estimation of individual parameters of cardiac dysfunction and poor outcomes. Furthermore, it plays a significant role in defining the predictive ability of individual parameters. Previous research has shown that the increase in mortality risk coincides with progressive dilation and right ventricular dysfunction, by means of ventriculo-ventricular interaction and left ventricular dysfunction. Identification of parameters that can predict the high-risk patients for future unwanted cardiac events such as ventricular tachycardia and cardiac insufficiency can assist the customization of a therapeutic approach leading to improvement in quality of life and patient's survival rate.</p>

Highlights

  • Napredak u dijagnostici i liječenju doveli su do značajnog poboljšanja sudbine djece rođene sa tetralogijom Fallot sa perioperativnim mortalitetom od 2-3% i tridesetogodišnjom stopom preživljavanja od 90%

  • Cardiac magnetic resonance is the preferred method for evaluating right ventricular size and function in patients

  • symptomatic patients is of great importance

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Summary

Kratak sadržaj

Napredak u dijagnostici i liječenju doveli su do značajnog poboljšanja sudbine djece rođene sa tetralogijom Fallot sa perioperativnim mortalitetom od 2-3% i tridesetogodišnjom stopom preživljavanja od 90%. Magnetna rezonanca srca je dijagnostička metoda koja pruža najprecizniju i najtačniju procjenu pojedinih parametara srčane disfunkcije i loših ishoda kao i definisanje prediktivne vrijednosti pojedinih parametara. Ključne riječi: Tetralogia Fallot, disfunkcija desne komore, magnetna rezonanca srca, prognoza. Tetralogia Fallot (TOF) je najčešća cijanogena urođena srčana mana (USM) koja čini 6-7% svih urođenih mana srca. Patoanatomski supstrat TOF čine: veliki perimembranozni defekt međukomorske pregrade, dekstroponirana aorta koja jaše nad septumom za 10-50%, opstrukcija izlaznog trakta desne komore i hipertrofija desne komore. Težinu bolesti određuje stepen opstrukcije izlaznog trakta desne komore, koja je obično infundibulo-valvularna, mada se značajne stenoze mogu javiti i na nivou trunkusa plućne arterije i grana plućne arterije. Krajnji stepen opstrukcija izlaznog trakta desne komore je plućna atrezija, kada se plućna cirkulacija napaja preko ductus arteriosus persistens ili patoloških aorto-pulmonalnih kolaterala [1, 2]

Istorijski podaci
Incidenca i prevalence
Kliničke karakteristke
Rezidualne postoperativne sekvele
Findings
Zamjena plućne valvule
Full Text
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