Background: Mechanical heart valves carry signifi cant risk during pregnancy. Aim: To assess the risk of pregnancy and delivery for mother and child in women with mechanical heart valve prosthesis. Methods: Multicenter retrospective observational study of women with mechanical heart valve prosthesis. The regimen with low molecular weight heparin throughout pregnancy (LMWH) was compared to warfarin since the second trimester (W). Results: Out of 419 pregnant women with cardiovascular diseases we assessed 14 women with mechanical heart valve prosthesis (3.3%) who experienced 23 pregnancies. There were 13 pregnancies with aortic valve prosthesis, 8 with prosthesis of atrio-ventricular valves and 2 pregnancies with both aortic and mitral valve prosthesis. LMWH regimen was used in 18 pregnancies, W in 5 pregnancies. Major maternal complications occurred in 65% of pregnancies, including 3 strokes and 3 urgent cardio-surgical procedures, all in women with LMWH regimen. Prosthetic valve thrombosis occurred in 26%, all in the LMWH group; no thrombosis was found in W group, (p = 0.166). Major bleeding occurred in 30% with no difference between LMWH and W groups (p = 0.596). Fetal loss represented 26% of all pregnancies with no difference between LMWH and W group (p = 1). The birth weight was not signifi cantly different between LMWH and W groups (2496 ± 327 g vs. 3132 ± 592 g, p = 0.12).
Conclusion:The rate of maternal and fetal complications in pregnant women with mechanical valve prosthesis is still high. The anticoagulation regimen using warfarin since the second trimester appears to be the safest one. The best prevention of complications is to avoid the implantation of mechanical valve prosthesis in girls and women in fertile age.
Aims
Fabry disease (FD) is a rare X‐linked genetic disorder caused by α‐galactosidase A (AGALA) deficiency. Whereas ‘classic’ variant has multisystemic manifestation, the more recently described ‘later‐onset’ variant is characterized by predominant cardiac involvement that often mimics hypertrophic cardiomyopathy (HCM).
Methods and results
Consecutive unrelated patients with HCM were screened for FD in 16 (out of 17) cardiac centres in the Czech Republic covering specialized cardiology care from June 2017 to December 2018. AGALA activity and globotriaosylsphingosine (lyso‐Gb3) levels were measured in all subjects using the dry blood spot method. FD was suspected in male patients with AGALA activity <1.2 μmol/h/L and in females with either low AGALA activity or lyso‐Gb3 > 3.5 ng/mL. Positive screening results were confirmed by genetic testing. We evaluated 589 patients (390 males, 66%) with HCM (mean maximal myocardial thickness 19.1 ± 4.3 mm). The average age was 58.4 ± 14.7 years. In total, 17 patients (11 males, 6 females) had a positive screening result, and subsequently, six of them (four males and two females) had a genetically confirmed pathogenic GLA mutation (total prevalence of 1.02%). Five of these patients were carrying the p.N215S mutation known to cause a typical later‐onset cardiac FD.
Conclusions
We confirmed the prevalence of FD repeatedly reported in previous screening programmes (approximately 1% irrespective of gender) in a non‐selected HCM population in Central Europe. Our findings advocate a routine screening for FD in all adult patients with HCM phenotype including both genders. The dry blood spot method used led to identification of clearly pathogenic variants.
Bartonella quintana byla v širším povědomí lékařské veřejnosti především počátkem minulého století v souvislosti s výskytem tzv. zákopové horečky. V dnešní době se jedná o ne příliš častého původce infekční endokarditidy (IE) především u pacientů se sníženou funkcí imunitního systému. Záludností této bakterie je, že ve většině případů není detekovatelná v hemokultuře, nebo až po velmi dlouhé kultivaci. Definitivní diagnózu se většinou podaří určit až na základě polymerázové řetězové reakce (PCR) z odebraného chlopenního materiálu. Kazuistika prezentuje případ 69leté ženy přijaté do intenzivní péče původně pro bronchopneumonii, u které byla díky rutinně provedenému vstupnímu echokardiografickému vyšetření diagnostikována těžká chlopenní vada a následně bartonellová endokarditida. Předkládaná kazuistika se snaží vyzdvihnout význam rutinního echokardiografického vyšetření prováděného intenzivistou -nekardiologem a taktéž upozornit na ne příliš častý případ kultivačně negativní endokarditidy.
Předkládáme kasuistiku třicetileté ženy s infekční endokarditidou, u níž byla správná diagnóza stanovena téměř půl roku po začátku obtíží. K vyšetření na kardiologii naší kliniky se nemocná dostala až ve stadiu závažných srdečních komplikací a v celkově vyčerpaném stavu. Přitom již před pěti měsíci byla podrobně vyšetřována pro ischemickou cévní mozkovou příhodu, jejíž příčina nebyla uspokojivě objasněna.
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