Echocardiographic Assessment of Left Ventricular Function in Type 1 Gaucher's Disease

There is predominate opinion among physicians managing type 1 Gauchers' disease (GD) that cardiac involvement is not an issue in these patients. In order to follow this hypothesis, we prospectively investigated 15 adult imiglucerase-treated type 1 GD patients by echocardiography, Doppler, and tissue Doppler echocardiography. This was a case-controlled study with 18 matched healthy volunteers. The obtained data was correlated with the levels of NT-proBNP (brain natriuretic peptide). None of the GD patients had clinical signs of heart disease. In 3 of the 15 patients, we observed echocardiographic signs of aortic and mitral valve calcification. The left ventricular systolic function was within normal limits. Compared to the control group, there was no statistically significant difference observed in the most sensitive indices of left ventricular diastolic function, parameter Em (P = .095), and E/Em ratio (P = .097), as demonstrated by tissue Doppler echocardiography. However, there was a positive correlation between the E/Em ratio and NT-proBNP plasma levels (P = .009). In conclusion, the prospective echocardiographic study of type 1 GD patients did not validate any left ventricular dysfunction. But, the E/Em ratio showed a strong statistical correlation with the most sensitive indicators of heart failure, NT-proBNP. Research on larger groups of patients and the usage of even more sensitive methods as strain-rate imaging will be necessary to confirm eventual myocardial involvement in GD patients

Dolgoročni rezultati po anatomski korekciji D-transpozicije velikih arterij – izkušnja enega centra

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

Longitudinal strain vs. conventional echocardiographic parameters in the first week of life in healthy term newborns

The first week of life is characterized by substantial alterations in hemodynamic conditions. Changes in myocardial contractility will reflect these changes. We aimed to assess right and left ventricular function on the third and seventh days of life in 50 healthy term newborns. To assess myocardial function, we used speckle tracking echocardiography. Pulsed-wave tissue Doppler imaging, M-mode, Doppler and pulsed-wave Doppler were also used to assess ventricular function. We found a significant increase in both right and left longitudinal strain and an increase in systolic and diastolic tissue Doppler velocities, whereas most other parameters remained unchanged. At both time points, the measured parameters were significantly greater for the right ventricle, but the changes with time were similar for both ventricles. We also found an increase in right ventricular outflow tract acceleration time as an indirect sign of decreasing pulmonary vascular resistance and an increase in systolic blood pressure, pointing to increasing systemic vascular resistance. Together with a decreasing proportion of patients with patent ductus arteriosus, the estimated left ventricular cardiac output decreased and right ventricular cardiac output increased but not to a statistically significant degree. In conclusion, the results of our study show how different echocardiographic techniques capture hemodynamic changes and changes in myocardial contractility and compliance. Both longitudinal strain and tissue Doppler imaging parameters seem to offer greater sensitivity in comparison with conventional echocardiographic parameters