Background Primary and secondary aortopathy are frequently encountered in patients with congenital heart disease. The aim of this study is to present our experience and the incidence of primary and secondary adult CHD-associated aortopathy. Methods The cohort is comprised of adult patients with congenital heart disease from the registry of the Eastern Slovakia Institute of Cardiovascular Diseases. Data from the last follow-up examinations are included in this study. In the primary and secondary aortopathy groups were 35 and 12 patients respectively. As a control group were selected 64 patients with non aortopathy associated congenital heart disease (atrial and ventricular septal defect). Results Patients with primary and secondary aortopathy had larger ascending aorta/aortic root diameters than the control group (36.28 (26–49) mm vs 30.25 (21–41) mm p = 0.000113, 33.82 27–49) mm vs 29.03 (19–38)mm p = 0.000366 and 42.1 (30–50) mm vs 30.25 (21–41) mm, p = 0.000106, 35.67 (27–48) mm vs 29.03 (19–38) mm, p = 0.000119 respectively). Moreover, patients with secondary aortopathy had statistically significant larger ascending aorta diameter compared to the patients with primary aortopathy (42.1 (30–50) mm vs 36.28 (26–49) mm p = 0.030). During the follow-up period, were performed only in 2 patients (one from each group) operations on the aortic root and the ascending aorta due to aortic root or ascending aorta dilatation. Conclusion More patients with secondary aortopathy had dilated ascending aorta/ aortic root, as well as larger aortic diameters compare to the patients with primary aortopathy. Routine follow-up of these patients with attention to aortic diameter is necessary.

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Embólia koronárnych artérií je menej častou príčinou akútneho infarktu myokardu. Predstavujeme kazuistiku 22-ročnej pacientky s dvojvtokovou ľavou komorou, defektom komorového septa a pulmonálnou stenózou po Fontanovej operácii s akútnym spodným STEMI na embolickom podklade. Okrem známych rizík vedúcich k trombotickým komplikáciám spojeným s Fontanovskou cirkuláciou, medzi potenciálne predisponujúce faktory trombembolizmu u tejto pacientky patrili vytvorenie trombu v dolnej dutej žile a nedostatočná antikoagulačná liečba. Táto kazuistika predstavuje jednu z prvých dokumentovaných embolických príčin STEMI u pacienta s Fontanovskou cirkuláciou. Doteraz nie je vytvorený konsenzus, že antikoagulačná terapia warfarínom je u pacientov s Fontanovskou cirkuláciou superiórna voči aspirínu v primárnej prevencii trombembolických príhod.

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Background: Primary and secondary aortopathy are frequently encountered in patients with congenital heart disease. The aim of this study is to present our experience and the incidence of primary and secondary adult CHD-associated aortopathy.Methods. The cohort is comprised of adult patients with congenital heart disease from the registry of the Eastern Slovakia Institute of Cardiovascular Diseases. Data from the last follow-up examinations are included in this study. In the primary and secondary aortopathy group were 35 and 12 patients respectively. As a control group were selected 64 patients with non aortopathy associated congenital heart disease (atrial and ventricular septal defect).Results: Patients with primary and secondary aortopathy had larger ascending aorta/aortic root diameters than the control group (36.28 (26-49) mm vs 30.25 (21-41) mm p=0.000113, 33.82 27-49) mm vs 29.03 (19-38)mm p=0.000366 and 42.1 (30-50) mm vs 30.25 (21-41) mm, p=0.000106, 35.67 (27-48) mm vs 29.03 (19-38) mm, p=0.000119 respectively). Moreover, patients with secondary aortopathy had statistically significant larger ascending aorta diameter compared to the patients with primary aortopathy ( 42.1 (30-50) mm vs 36.28 ( 26-49) mm p=0.030). During the follow-up period, were performed only in 2 patients (one from each group) operations on the aortic root and the ascending aorta due to aortic root or ascending aorta dilatation.Conclusion: More patients with secondary aortopathy had dilated ascending aorta/ aortic root, as well as larger aortic diameters compare to the patients with primary aortopathy. Routine follow-up of these patients with attention to aortic diameter is necessary.