Izhodišča: Anatomska korekcija (ASO) je zdravljenje izbire za D-transpozicijo velikih arterij (D-TGA). Namen raziskave je bil oceniti dolgoročno uspešnost anatomske kirurške korekcije.
Metode: Delno ...retrospektivno smo pregledali dokumentacijo ali ponovno ocenili zdravstveno stanje 38 bolnikov (30 moških, 8 žensk) v času tranzicije mladostnikov v odraslo dobo (starost ob zadnjem kliničnem pregledu 17,1 ± 1,4 let), ki so bili rojeni od leta 2000 do leta 2005 z D-TGA in so imeli ASO. Ocenili smo: funkcijski razred po NYHA, spremembe, ki so ostale, na neoaorti in neoaortni zaklopki, neopulmonalni zaklopki in pljučnih arterijah, funkcijo desnega in levega prekata, telesno zmogljivost in znake ishemije miokarda.
Rezultati: Nihče v skupini ni umrl (interval zaupanja (0,00-0,09). 32 bolnikov (84,2 %) je bilo v NYHA I, 6 bolnikov (15,7 %) je bilo v NYHA II. Pri 83,3 % bolnikov se je bulbus aorte razširil (20,9 ± 2,8 mm/m2, max. 27,7 mm/m2). Pri 27 bolnikih (90 %) je bila prisotna regurgitacija neoaortne zaklopke. Med skupinami brez, z blago ali zmerno neoaortno regurgitacijo ni bilo razlik v širini bulbusa, normaliziranih na telesno površino (p = 0,6). Regurgitacija neopulmonalne zaklopke je bila prisotna pri 58,1 % bolnikov. Zaradi obstrukcije v iztoku iz desnega prekata je bila potrebna ponovna operacija v enem primeru in perkutana dilatacija neopulmonalne zaklopke v enem primeru. Zaradi zapletov na koronarnih arterijah je bil potreben le en kirurški poseg zaradi miokardnega infarkta med naporom.
Zaključek: Pozni rezultati po anatomski korekciji D-TGA so dobri in primerljivi z večjimi centri. Nihče ni umrl, večina bolnikov je bila brez simptomov, z normalno sistolično funkcijo obeh prekatov in z normalno telesno zmogljivostjo. Ponovne operacije in perkutani posegi so bili dokaj redki, toda dolgoročno uspešni.
Abstract Background Adrenal vein sampling (AVS) is essential for diagnostics of primary aldosteronism, distinguishing unilateral from bilateral disease and determining treatment options. We reviewed ...the performance of AVS for primary aldosteronism at our center during first 15 years, comparing the initial period to the period after the introduction of a dedicated radiologist. Additionally, AVS outcomes were checked against CT findings and the proportion of operated patients with proven unilateral disease was estimated. Patients and methods A retrospective cross-sectional study conducted at the national endocrine referral center included all patients with primary aldosteronism who underwent AVS after its introduction in 2004 until the end of 2018. AVS was performed sequentially during Synacthen infusion. When the ratio of cortisol concentrations from adrenal vein and inferior vena cava was at least 5, AVS was considered successful. Results Data from 235 patients were examined (168 men; age 32–73, median 56 years; BMI 18–48, median 30.4 kg/ m 2 ). Average number of annual AVS procedures increased from 7 in the 2004–2011 period to 29 in the 2012–2018 period ( p < 0.001). AVS had to be repeated in 10% of procedures; it was successful in 77% of procedures and 86% of patients. The proportion of patients with successful AVS (92% in 2012–2018 vs . 66% in 2004–2011, p < 0.001) and of successful AVS procedures (82% vs . 61%, p < 0.001) was statistically significantly higher in the recent period. Conclusions Number of AVS procedures and success rate at our center increased over time. Introduction of a dedicated radiologist and technical advance expanded and improved the AVS practice.
Bolezni perikarda so lahko izoliran bolezenski proces, ali pa so del sistemskih bolezenskih stanj. V zahodnem svetu, kjer je prevalenca tuberkuloze nizka, je perikarditis najpogosteje povezan z ...virusnimi okužbami, avtoimunskimi procesi ali pa malignomi. Glede na klinično sliko lahko bolezni perikarda razdelimo v nekaj glavnih sindromov: perikarditis, izoliran perikardni izliv, tamponada srca, konstrikcijski perikarditis in mase/ciste v perikardu. Zadnje smernice Evropskega kardiološkega združenja prinašajo nekaj novosti na področju triažiranja in diagnosticiranja bolnikov z boleznimi perikarda, predvsem pa se jasneje opredelijo do strategij zdravljenja posameznih perikardnih sindromov. Članek povzema evropske smernice in vključuje prilagoditve za naše razmere.
Now 15-year-old girl with glycogen storage disease (GSD) type IIIa (OMIM 232400) developed severe left ventricular obstructive hypertrophy and hepatomegaly while treated with frequent cornstarch ...meals. Subsequently, she was introduced the ketogenic diet; continuous ketosis has been maintained for over the last 4 years. After the introduction of ketogenic diet, a normalization of the cardiomyopathy and improvement of hepatopathy was achieved, with enhanced overall quality of life.
Datasets highlighting effects of ketogenic diet (KD) in a glycogen storage disease type IIIa patient is presented with the longest patient follow up report to date. Now a 15-year old girl with GSD ...type IIIa, diagnosed at 1 year of age, had initially introduced treatment with diet high carbohydrates, according to the recommendations. Progressively she developed left ventricular obstructive hypertrophy, hepatomegaly and skeletal myopathy. At the age of 11 years, she was introduced KD and continuous ketosis has been maintained for over 4 years providing longest reported follow up to date. KD introduction lead to a normalization of left ventricular parameters and ventricular mass and to an improvement in hepatic injury markers and decrease in liver size.
We provided a table with biochemical parameters, a table providing detailed diet composition, tables with cardiac and hepatic measures and figures depicting cardiac NMR images; all the tables/figures are provided referring to the KD introduction (values prior/after). Interpretation of this data can be found in a case report article titled “Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa”.
The exact interaction of factors leading to myocardial dysfunction and fibrosis of the systemic right ventricle (SRV) is not completely understood. Myocardial ischemia and injury associated with a ...supply-demand mismatch of the pressure overloaded SRV are thought to play an important role, however studies confirming this are lacking.
Adult SRV patients were included in this single centre cohort study. All patients underwent a comprehensive diagnostic and imaging workup. A two-day stress-rest SPECT was performed to assess myocardial perfusion. SRV ischemia was defined as decreased segmental tracer uptake during exercise with significant improvement at rest. Contrast enhanced cardiac magnetic resonance imaging (CMR) was also performed in a subgroup of patients without contraindication, to assess focal myocardial fibrosis. Differences between patients with and without SRV ischemia were assessed.
Twenty-three SRV patients (15 with transposition of the great arteries after atrial switch procedure and 8 with congenitally corrected transposition of the great arteries; 5 (22%) females; mean age 38 ± 11 years) were included. Seven (30%) patients had SRV ischemia on SPECT. Late gadolinium enhancement on CMR was more common in patients with SRV ischemia (p = 0.002). However, there was no association between SRV ischemia and different echocardiographic or CMR parameters of SRV systolic function, laboratory markers (high-sensitivity troponin I and NT-proBNP) and exercise capacity.
Our multimodality study showed that SRV ischemia in adult SRV patients was associated with more focal myocardial fibrosis, but not with functional or imaging markers of SRV function.
•The role of myocardial ischemia in dysfunction of the systemic right ventricle is unknown.•Myocardial ischemia of the systemic right ventricle was evident in 30% of adult patients with systemic right ventricle.•Patients with ischemia more commonly had focal myocardial fibrosis.•Myocardial ischemia had no impact on functional or imaging markers of systemic right ventricular function.
We report a case of aortic pseudoaneurysm associated with a fractured bare Cheatham-Platinum stent following stenting for aortic coarctation. These complications were recognised 6 years after the ...implantation procedure and were successfully managed by percutaneous stent graft implantation. Staged approach for stent dilatation might prevent development of aortic pseudoaneurysms. In addition, careful follow-up is warranted after stenting for aortic coarctation, particularly in patients with recognised aortic wall injury.
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