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Funding Acknowledgements Type of funding sources: None. Introduction Worsening of cardiac function with increased arrhythmic risk is common in cancer patients undergoing chemotherapy. Impaired LV Global Longitudinal Strain (GLS) in these patients despite preserved ejection fraction is a common issue. Recently, myocardial work by speckle-tracking echocardiography has been used to overcome GLS limitations in various conditions, but little is known about its usefulness in the detection of cardiac toxicity. Moreover, left atrial (LA) toxicity may occur early in the course of cancer therapy. The main aim of the study was to assess the cardiotoxic effects of tyrosine kinase inhibitors (TKIs) on patients with Philadelphia chromosome-positive chronic myeloid leukaemia (Ph+ CML) by using novel echocardiographic tools as myocardial work and atrial strain analysis. Methods We retrospectively enrolled Ph+ CML patients treated with TKIs followed at the cardio-oncology outpatient clinic of our hospital from December 2018 to March 2019 who underwent clinical evaluation with ECG and echocardiogram (TTE) before and after one year of treatment with TKIs. Healthy subjects were enrolled in the control group matched for gender, age and cardiovascular risk factors. Myocardial work was derived from the strain-pressure relation, integrating in its calculation the non-invasive arterial pressure. LA longitudinal strain (reservoir, conduit and booster) was obtained from an optimized apical 4-chamber view of the LA. Results The study recruited 32 patients in Ph+ CML group and 32 healthy controls. 39% of patients were treated with Imatinib, 29.3% with Nilotinib, 4.9% with Dasatinib and 4.9% with Ponatinib. Main results are detailed in the Table 1. At one-year follow-up there was a significant reduction compared to baseline in Global Constructive Work (2555.22 ± 564.33 vs 2119.31 ± 700.19; p = 0.0001), Global Work Efficiency (96.13 ± 1.90 vs 94.00 ± 2.96; p = 0.002) and Global Work Index (2340.75 ± 579.57 vs 1938.46 ± 680.23; p = 0.001), and a non-significant reduction in Global Wasted Work (p = 0.393). Regarding left atrial strain analysis at the one-year follow-up there was a statistically significant reduction in LA contractile strain (booster= 14.63 ± 1.408 vs 12.38 ± 1.581; p= 0.018). LA contractile strain reduction was also observed in the comparison with controls (12.38 ± 2.99 vs 14.91 ± 3.09; p = 0.009). Any other significant difference was detected between baseline and FU TTE data in the Ph+ CML group. Conclusions New imaging methods for the study of cardiotoxicity provide an additional tool for early prediction of potential adverse effects of antineoplastic drugs. TKIs therapy leads to an impairment of atrial contractility, which can be detected by atrial strain e myocardial work analysis. Abstract table 1  Abstract figure 1

Na naše oddělení byla přijata 64letá žena k implantaci dvoudutinového kardiostimulátoru. Samotný výkon se obešel bez jakékoli komplikace. Jedna komorová elektroda s aktivní fi xací byla umístěna v hrotu pravé komory. Brzy po implantaci začal pacientku sužovat neproduktivní kašel, jednoznačně související s komorovou stimulací. Transtorakální echokardiografi e prokázala malý perikardiální výpotek podél apikálních segmentů. U pacientky bylo provedeno urgentní CT vyšetření s kontrastní látkou, které potvrdilo perikardiální výpotek a přítomnost elektrody v myokardu hrotu pravé komory. Pro přetrvávání symptomů jsme se rozhodli přemístit pravokomorovou elektrodu do středu mezikomorového septa. Po výkonu kašel okamžitě vymizel.

Takotsubo syndrom (TTS) je formou kardiomyopatie navozené akutním stresem s dysfunkcí levé komory ve většině případů bez obstrukční ischemické choroby srdeční, i když ta může být přítomna v 10-29 % případů. Spouštěčem pro rozvoj takotsubo syndromu může být i málo invazivní výkon spojený s fyzickým nebo emocionálním stresem. Popis případu: Popisujeme případ 87leté ženy s vysokou, již dříve přítomnou zátěží stresem, u níž došlo k rozvoji takotsubo syndromu s neobvyklými symptomy po nekomplikované implantaci trvalého kardiostimulátoru. Závěry: Tento případ ukazuje, že TTS je nutno zvažovat jako potenciální komplikaci po implantaci kardiostimulátoru, zvláště u postmenopauzálních žen. Je třeba vytvořit multicentrické registry a provést další studie, abychom zjistili, jak a u které kategorie pacientů mohou i málo invazivní výkony vést ke vzniku TTS.

Background Anderson-Fabry Disease (AFD, OMIM 301500) is a rare X-linked lysosomal storage disorder caused by GLA gene mutation resulting in a deficit or absent activity of the α-galactosidase A enzyme (α-Gal A). This deficiency involves the impossibility of cleavage of glycophospholipids, resulting in an intralisosomal accumulation of them in different tissues. Due to an incidence of 1 in 80000, AFD is considered the second most common glycosphingolipid storage disorder after Gaucher disease. Cardiac manifestations include left ventricular hypertrophy (LVH) and arrhythmias. The rate of pacemaker implantation (PMI) in AFD has been described to be 25 times higher than in the general population, and the requirement of PMI has been reported to be as high as 8% in some AFD series. In this context, the presence of conduction abnormalities in young patients may suggest an undiagnosed AFD.1 Therefore, early diagnosis is important in AFD because appropriate therapies seem to be more effective when initiated promptly. Purpose Our aim is to detect AFD among relatively patients with unexplained conduction disturbances requiring PMI, not submitted to newborn screening. Methods Among 650 patients afferent to our ambulatory for routinary pacemaker follow-up, we considered a selected population with diagnosis of sinus node dysfunction or atrioventricular block (confirmed by atrial pacing rate ≥ 60% or ventricular pacing percentage ≥ 80%) and an age, at the time of PMI, ranging between ≥40 and ≤70 years old. The exclusion criteria were: patients with previous myocardial infarction; patient whit known cardiac disease (such as hypertrophic cardiomyopathy); patients who underwent cardiac surgery and patients with extracardiac disease with cardiac involvement such as autoimmune disorders. For this cohort of 26 adult patients (13 males; 13 females; mean age 63 ± 7 years) a prospective screening study for AFD was performed. After clinical evaluation, transthoracic echocardiography (analyzing signs of left ventricular hypertrophy) and pacemaker check, a dried blood spot sampled in filter paper was analyzed. This filter paper assay was performed in male patients in order to evaluate the α-Galactosidase A enzyme activity through the detection of Fabry disease biomarkers; only in the case of abnormal values, genetic investigation was performed. In female patients, the analysis was exclusively genetic. Results The analyses revealed 58% (15/26) of patients affected by mild LVH (IVS diameter ranging from 11 to 15 mm). No patient had severe LVH (IVS diameter ≥15 mm) or moderate-severe renal dysfunction (more than stage 3B, GFR below than 30-44 mL/min). In the restrict cohort considered, we found one 69 yo female patient with heterozygosis GLA pathogenic mutation, NM_000169.2:c.638A>C p.(Lys213Thr). She had normal value of liso-Gb3 1,1 ng/ml (n.v. ≤ 1,8 ng/ml). She had mild LVH (IVS diameter 12 mm) and no renal dysfunction. Familiar screening was programmed. Conclusion In a highly selected sample of relatively young patients with conduction disturbances requiring pacemaker implantation, a female patient with genetic mutation causing AFD has been identified. Therefore, it seems that screening efforts should be increased in this patient population. 1 Hemelsoet D, De Keyser J, Van Heuverswyn F et al. Screening for Fabry Disease in Male Patients With Arrhythmia Requiring a Pacemaker or an Implantable Cardioverter-Defibrillator. Circulation. 2021 Feb 23;143(8):872-874. doi: 10.1161/CIRCULATIONAHA.120.051400.