X-ray Doses in Relation to Body Mass, Indication, and Substrate During Pediatric Electrophysiological Procedures on the Heart.

The main goal of this study is to determine typical values of dose area product (DAP) and difference in the effective dose (ED) for pediatric electrophysiological procedures on the heart in relation to patient body mass. This paper also shows DAP and ED in relation to the indication, the arrhythmia substrate determined during the procedure, and in relation to the reason for using radiation. Organ doses are described as well. The subjects were children who have had an electrophysiological study done with a 3D mapping system and X-rays in two healthcare institutions. Children with congenital heart defects were excluded. There were 347 children included. Significant difference was noted between mass groups, while heavier children had higher values of DAP and ED. Median DAP in different mass groups was between 4.00 (IQR 1.00-14.00) to 26.33 (IQR 8.77-140.84) cGycm2. ED median was between 23.18 (IQR 5.21-67.70) to 60.96 (IQR 20.64-394.04) µSv. The highest DAP and ED in relation to indication were noted for premature ventricular contractions and ventricular tachycardia-27.65 (IQR 12.91-75.0) cGycm2 and 100.73 (IQR 53.31-258.10) µSv, respectively. In arrhythmia substrate groups, results were similar, and the highest doses were in ventricular substrates with DAP 29.62 (IQR 13.81-76.0) cGycm2 and ED 103.15 (IQR 60.78-266.99) µSv. Pediatric electrophysiology can be done with very low doses of X-rays when using 3D mapping systems compared to X-rays-based electrophysiology, or when compared to pediatric interventional cardiology or adult electrophysiology.

Serum Fibrinogen and Renal Dysfunction as Important Predictors of Left Atrial Thrombosis in Patients with Atrial Fibrillation.

BACKGROUND: As has been shown previously, patients with atrial fibrillation (AF) who have left atrial thrombus (LAT) also have elevated plasma concentrations of fibrinogen. In this study, we tried to determine if this is the consequence of a genetic trait and whether elevated concentrations of fibrinogen could be used to predict LAT in patients with AF. METHODS: We recruited 181 consecutive patients scheduled for pulmonary vein isolation (PVI) or direct current cardioversion. The primary endpoint was the presence of LAT on transesophageal echocardiography (TOE). We recorded routine clinical and biochemical data as well as the polymorphism type of the fibrinogen gene for the ß chain. To control potentially interfering variables, we performed propensity score matching (PSM). Multivariable and univariable logistic regression models (LRM) were computed using the CHA2DS2-Vasc score, the fibrinogen concentration and creatinine clearance as estimated by the Cockcroft-Gault equation. RESULTS: 60 of 181 patients had LAT as detected by TOE. As expected, patients with LAT had significantly higher concentrations of fibrinogen (3.9 vs. 3.6 g/L); p = 0.01 in the unadjusted analysis. After performing PSM, there were no statistically significant differences between the groups, except for creatinine clearance (79.9 vs. 96.8 mL/min); p = 0.01. There were also no differences regarding the -455 G/A ßfibrinogen polymorphism distribution between the two groups. After constructing the LRM, we found no performance enhancement for the CHA2DS2-Vasc score by adding the fibrinogen concentration or creatinine clearance alone, but when all three variables were put together, there was a significant improvement in LAT prediction (AUC 0.64 vs. 0.72), p = 0.026. CONCLUSION: Our study found no evidence of elevated levels of circulating fibrinogen in patients with LAT or a connection between those levels and the A/A and A positive polymorphism. When used together with renal function markers such as creatinine clearance, plasma fibrinogen concentrations can provide additional power to the CHA2DS2-Vasc score for predicting LAT.

Remdesivir use in severe and critical COVID-19 patients might be associated with lower incidence of arterial thrombotic events.

INTRODUCTION: Venous thromboembolism (VTE) and arterial thrombotic (AT) events are a striking feature of severe COVID-19, however, relationship of remdesivir use and the risk of thrombotic events is unknown and has not been investigated before. METHODS: We retrospectively analyzed a cohort of 876 consecutive hospitalized severe and critical COVID-19 patients who were treated with remdesivir and compared them to 876 case-matched control patients. All patients were treated in our tertiary-level institution in period from 10/2020 to 6/2021. VTE and AT were diagnosed by objective imaging and laboratory methods. RESULTS: After exclusion of 71 VTE and 37 AT events present at the time of hospital admission, there were a total of 70 VTE (35 in the remdesivir and 35 in the control group) and 38 AT events occurring during hospitalization (13 in the remdesivir and 25 in the control group). There was a similar cumulative post-admission VTE incidence among both remdesivir and matched control patients (P = 0.287). Significantly lower cumulative post-admission AT incidence was observed among patients treated with remdesivir than among matched control patients (1.7% vs 3.3%, HR = 0.51, P = 0.035). Tendency for lower AT rates was evident in subgroups of patients stratified according to the type of AT, as well as according to the intensity of required oxygen supplementation at the time of remdesivir use. CONCLUSION: Remdesivir use in severe and critical COVID-19 patients might be associated with lower occurrence of AT during hospitalization, whereas similar rates of VTE events were observed among both patients treated with remdesivir and control patients.

Risks associated with prior oral anticoagulation use in hospitalized COVID-19 patients - A retrospective cohort study on 5392 patients from a tertiary centre.

INTRODUCTION: There are conflicting data on prior oral-anticoagulant (OAC) use and outcomes of hospitalized COVID-19 patients. Due to uncertainties regarding associated risks with the prior OAC use, we have investigated this issue in a large cohort of hospitalized COVID-19 patients from our institution. METHODS: We have retrospectively evaluated a total of 5392 consecutive COVID-19 patients hospitalized in our tertiary center institution in period 3/2020 to 6/2021. Majority of patients received low-molecular-weight-heparin thromboprophylaxis and corticosteroids during hospitalization. Patients' characteristics and clinical outcomes were documented as a part of a hospital registry project and were evaluated according to the prior non-OAC, warfarin and direct oral anticoagulants (DOAC) use. RESULTS: Median age was 72 years, median Charlson comorbidity index (CCI) was 4 points. There were 56.2% male patients. Majority of patients had severe (70.5%) or critical (15.8%) COVID-19 on admission. A total of 84.8% patients did not receive prior OAC, 9% were previously anticoagulated with warfarin and 6.2% were previously anticoagulated with DOACs. In the multivariate regression analyses, prior warfarin use was associated increased in-hospital mortality (OR 1.24, P = 0.048) independently of older age (OR 2.12, P < 0.001), male sex (OR 1.27, P < 0.001), higher CCI (OR 1.26, P < 0.001) and severe or critical COVID-19 on admission (OR 22.66, P < 0.001). Prior DOAC use was associated with higher occurrence of major bleeding (OR 1.72, P = 0.045) independently of higher CCI (OR 1.08, P = 0.017). CONCLUSION: Prior OAC use could be associated with worse clinical outcomes during COVID-19 hospitalization. These phenomena might be OAC type specific and persist after multivariate adjustments.

Bone morphogenetic protein 1.3 inhibition decreases scar formation and supports cardiomyocyte survival after myocardial infarction.

Despite the high prevalence of ischemic heart diseases worldwide, no antibody-based treatment currently exists. Starting from the evidence that a specific isoform of the Bone Morphogenetic Protein 1 (BMP1.3) is particularly elevated in both patients and animal models of myocardial infarction, here we assess whether its inhibition by a specific monoclonal antibody reduces cardiac fibrosis. We find that this treatment reduces collagen deposition and cross-linking, paralleled by enhanced cardiomyocyte survival, both in vivo and in primary cultures of cardiac cells. Mechanistically, we show that the anti-BMP1.3 monoclonal antibody inhibits Transforming Growth Factor ß pathway, thus reducing myofibroblast activation and inducing cardioprotection through BMP5. Collectively, these data support the therapeutic use of anti-BMP1.3 antibodies to prevent cardiomyocyte apoptosis, reduce collagen deposition and preserve cardiac function after ischemia.

Acute Occlusion of the Infarct-Related Artery as a Predictor of Very Long-Term Mortality in Patients with Acute Myocardial Infarction.

AIM: The survey's aim was to examine the significance of infarct-related artery (IRA) occlusion (verified angiographically) on very long-term outcomes of patients with acute myocardial infarction, within the STEMI and NSTEMI diagnosis. METHODS: A single-center, nonrandomized, registry-based study on patients treated for acute coronary syndrome with percutaneous coronary intervention between June 2011 and December 2016 was conducted. Patients with angiographically proven IRA occlusion (100% stenosis with TIMI flow 0 distal to occlusion) were categorized as occlusive myocardial infarction (OMI) and patients with patent IRA (50-99% stenosis with TIMI 1-3 flow) were categorized as nonocclusive myocardial infarction (NOMI) and very long-term outcomes were analyzed. Data were collected prospectively from the hospital's PCI registry and the database of the Croatian Institute of Public Health. RESULTS: A total of 2450 patients were included in the study. 796 (32.5%) patients had NOMI and 1654 patients (67.5%) had OMI. According to ECG changes, 1534 patients presented with STEMI (62,6%) and 916 with NSTEMI (37,8%). 88% of STEMI patients presented with OMI and 12% with NOMI, while patients with NSTEMI in 33,8% presented with OMI and in 66,81% with NOMI. A median follow-up was 4.7 years. There was no significant difference in cardiovascular mortality between the groups (14.8% vs 13.1%; OMI vs NOMI, respectively; p=0.374) neither in all-cause mortality (19% vs 21.5%; OMI vs NOMI, respectively; p=0.374). Patients with NSTEMI had a significantly higher very long-term mortality (21.6% vs 18.1%; NSTEMI vs STEMI, respectively; p=0.029). CONCLUSION: The main findings of the study are as follows: (1) total IRA occlusion was not associated with higher long-term mortality; (2) NSTEMI was associated with a higher mortality rate compared with STEMI, independent of angiographic presentation (OMI/NOMI); (3) IRA occlusion was not associated with significantly higher mortality rates in patients with STEMI and NSTEMI, respectively.

Atrial appendages' mechanics assessed by 3D transoesophageal echocardiography as predictors of atrial fibrillation recurrence after pulmonary vein isolation.

BACKGROUND: Although there are numerous studies reflecting predictors of atrial fibrillation (AF) recurrence (AFR) after pulmonary vein isolation (PVI), data on atrial appendages' mechanics is scarce. This study aimed to assess atrial appendages' mechanics by 2-dimensional (2D) and 3-dimenssional (3D) transoesphageal echocardiography (TEE) and to explore its value to predict AFR after PVI. METHODS: Consecutive patients with paroxysmal AF undergoing first PVIwere analysed. 3D and 2D-TEE with tissue Doppler imaging (TDI) and strain analysis was obtained prior to the PVI, including: left atrial appendage (LAA) TDI and strain analysis, LAA ostium surface area, right atrial appendage's TDI velocity and superior vena cava (SVC) ostium surface area. The primary end-point was freedom from any documented recurrence of atrial arrhythmia lasting > 30 s. RESULTS: This single-centre, prospective study included 74 patients with paroxysmal AF (median age 59 years; 36% female; BMI 27.4 ±â€¯4.1 kg/m2, LA volume index 32 ±â€¯11 mL/m2). After a median follow-up of 14 (IQR 10-22) months, 21 (28%) patients had AFR. In a univariate and multivariate Cox-regression analysis LAA TDI velocity (HR 1.48, 95%CI 1.28-1.62, p < 0.001) and LAA ostium surface area(HR 1.58, 95%CI 1.06-1.81, p = 0.033) both independently predicted AFR after single PVI. RAA TDI velocity and SVC ostium surface area were not correlated to AFR. CONCLUSION: Paroxysmal AF patients with lower LAA TDI tissue velocity and LAA ostium surface area have higher risk of developing AFR after PVI. To our knowledge, this is the first study assessing atrial appendages' mechanics in predicting AFR after PVI.Clinical trial registration: www.drks.de(Identifier: DRKS00010495).

The influence of therapeutic hypothermia on the outcomes of cardiac arrest survivors: a retrospective cohort study.

AIM: To determine whether therapeutic hypothermia (TH) improves survival and neurological outcomes in out-of-hospital cardiac arrest (OHCA) survivors. METHODS: This retrospective cohort study enrolled patients treated for OHCA with a return of spontaneous circulation admitted to the Cardiac Intensive Care Unit from October 2000 until March 2019. Data were collected from medical archives. Propensity score matching was used. The primary endpoint was death during hospital stay and secondary endpoint was cerebral performance category (CPC) score at discharge. RESULTS: Out of 152 patients included in the study, 58 (38.7%) underwent TH treatment. After matching (which left 70 patients in the analysis), death during hospital stay occurred less often in TH group (28.6% vs 57.1%, P=0.029), while the difference in CPC score was not significant. Cox proportional hazards model showed the predictors of death during hospital stay to be TH (hazard ratio [HR] 0.29, 95% confidence interval [CI] 0.13-0.68, P=0.004), initial Glasgow Coma Scale score of 3 (HR 7.55, 95% CI 1.44-39.63, P=0.017), and heart failure (HR 2.35, 95% CI 1.02-5.34, P=0.045). TH was not an independent predictor of CPC score. Mann-Whitney U test and linear regression model showed that TH was associated with higher gain in GCS. CONCLUSION: TH was associated with better survival and certain variables suggesting improved neurological outcomes, suggesting that TH is a vital treatment option for comatose OHCA survivors.

Chromogenic anti-FXa assay calibrated with low molecular weight heparin in patients treated with rivaroxaban and apixaban: possibilities and limitations.

INTRODUCTION: Clinical application of rivaroxaban and apixaban does not require therapeutic monitoring. Commercial anti-activated factor X (anti-FXa) inhibition methods for all anti-FXa drugs are based on the same principle, so there are attempts to evaluate potential clinical application of heparin-calibrated anti-FXa assay as an alternative method for direct FXa inhibitors. We aimed to evaluate relationship between anti-FXa methods calibrated with low molecular weight heparin (LMWH) and with drug specific calibrators, and to determine whether commercial LMWH anti-FXa assay can be used to exclude the presence of clinically relevant concentrations of rivaroxaban and apixaban. MATERIALS AND METHODS: Low molecular weight heparin calibrated reagent (Siemens Healthineers, Marburg, Germany) was used for anti-FXa activity measurement. Innovance heparin (Siemens Healthineers, Marburg, Germany) calibrated with rivaroxaban and apixaban calibrators (Hyphen BioMed, Neuville-sur-Oise, France) was used for quantitative determination of FXa inhibitors. RESULTS: Analysis showed good agreement between LMWH calibrated and rivaroxaban calibrated activity (κ = 0.76) and very good agreement with apixaban calibrated anti-Xa activity (κ = 0.82), respectively. Low molecular weight heparin anti-FXa activity cut-off values of 0.05 IU/mL and 0.1 IU/mL are suitable for excluding the presence of clinically relevant concentrations (< 30 ng/mL) of rivaroxaban and apixaban, respectively. Concentrations above 300 ng/mL exceeded upper measurement range for LMWH anti-FXa assay and cannot be determined by this method. CONCLUSION: Low molecular weight heparin anti-FXa assay can be used in emergency clinical conditions for ruling out the presence of clinically relevant concentrations of rivaroxaban and apixaban. However, use of LMWH anti-FXa assay is not appropriate for their quantitative determination as an interchangeable method.

LEFT VENTRICULAR LEAD PLACEMENT FOR PACING AND SENSING IN A PATIENT WITH ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY UNDERGOING ICD IMPLANTATION.

We present a case of a 64-year-old female patient scheduled for implantable cardioverter defibrillator (ICD) implantation due to arrhythmogenic right ventricular cardiomyopathy (ARVC). Dual coil, active fixation ICD lead was introduced through the axillary vein. More than 20 positions were changed in the right ventricle (RV) (outflow tract, high, mid and apical septum, infero-basal, apical and lateral wall). Maximum R wave amplitude was 2 mV with pacing threshold of 0.5 V. Since the sensing was inappropriate, we decided to place the pace/sense lead of the ICD in the coronary sinus. The lead was placed in the basal part of the lateral vein. The pacing threshold was 1.0 V/0.40 ms and R wave was 9 mV. The lead was connected to the ICD sense-pace port and high voltage coils were connected in the usual way. The RV sense-pace lead was capped off. The device sensed an R wave of 7.0 mV 48 hours later. The purpose of this report is to show a possible solution of sensing problems during an ICD implantation in a patient with ARVC.

ROUTINELY AVAILABLE BIOMARKERS AS LONG-TERM PREDICTORS OF DEVELOPING SYSTOLIC DYSFUNCTION IN COMPLETELY REVASCULARIZED PATIENTS WITH ACUTE ST ELEVATION MYOCARDIAL INFARCTION.

The aim of this study was to assess the efficacy of high-sensitivity C-reactive protein (hsCRP), cardiac troponin T (cTnT) and creatine kinase (CK) as long-term predictors of reduced systolic function in patients with acute ST-segment elevation myocardial infarction (STEMI) undergoing primary percutaneous coronary intervention (PCI) with complete revascularization. This prospective study evaluated consecutive patients with acute STEMI who had normal left ventricular ejection fraction (LVEF ≥50%) at admission with single-vessel disease and underwent complete revascularization. Blood samples were collected from admission to day 7. The primary endpoint was reduction of LVEF <50% after 12 months. The study included 47 patients, median age 59±10 years, 74.5% of them men. Patients who developed systolic dysfunction (LVEF <50%) had significantly higher mean values of cTnT after 24 hours (5.11 vs. 2.82 µg/L, p=0.010) and peak values of CK (3375.5 vs. 1865 U/L, p=0.008). There was no significant relation between hsCRP and development of reduced LVEF (p=0.541). In conclusion, cTnT and CK could serve as long-term predictors of reduced left ventricular systolic function (<50%) in acute STEMI patients with normal systolic function at admission, single-vessel coronary disease and complete revascularization during primary PCI.

Nutritional Considerations of Cardiovascular Diseases and Treatments.

Nutritional considerations of many chronic diseases are not fully understood or taken into consideration in everyday clinical practice. Therefore, it is not surprising that high proportion of hospitalized patients with cardiovascular diseases remains underdiagnosed with malnutrition. Malnourished patients have increased risk of poor clinical outcomes, complications rate, prolonged hospital stay, more frequent rehospitalizations, and lower quality of life. The purpose of this review is to recapitulate recent data on nutritional considerations in cardiovascular medicine.

[MYOCARDIAL INFARCTION AS A CONSEQUENCE OF CHILDHOOD POLYARTERITIS NODOSA ­ CASE REPORT].

Polyarteritis nodosa (PAN) is a systemic vasculitis histologically characterized by necrotic lesions of small and medium- sized arteries occurring mostly in their bifurcations. PAN is a multi-organ disorder that affects numerous visceral arteries and leads to inflammation and necrosis, which may result in ischemia of vital organs. The etiology of the disease is unknown. It occurs in four forms - cutaneous (most common), classic, systemic, and microscopic. PAN preferably affects the renal and coronary arteries. The most common findings in the affected blood vessels are aneurysm, thrombosis, and stenosis. Cardiac complications are rare in children, but 35% of patients develop a complication in adulthood. The most common complication in adulthood is cardiac decompensation, which can be explained as a consequence of longstanding hypertension and changes in coronary blood vessels. The incidence of myocardial infarction is low, particularly in young patients. This paper describes a 25-year-old patient with acute myocardial infarction accompanied by changes in the coronary blood vessels and persistent arterial hypertension as a result of PAN diagnosed in childhood.

Prevalence of depressive symptoms among college students and the influence of sport activity.

The present study asses the prevalence of depressive symptoms among college students in Split, Croatia, and positive influence of sport activity on decreasing the depression symptoms. Authors screened all 664 college students of the first year of study. All of them were over the 18 years and the mean age was 19.4 +/- 1.2 years. There were 466 females (70.2%) and 178 (26.8%) males. They answered The Beck Depression Inventory (BDI) and questionnaire about their sport activity (no sport activity, recreational and active in sports). For the purpose of the analysis depressive symptoms were defined as a score of > 11. Chi-square and Mann-Whitney test were used for data analysis. 9.4% of the students had significant depression symptoms (score > 11). No one student had score > 26 (symptoms of major depression). Statistically significant lower score on BDI have students who are active in sports (score median = 3) compared to group of recreational (score median = 4) and in correlation to group who are not active in sports (score median = 5) (Kruskal-Wallis: p < 0.001). In the group of active in sports (N = 254) there are only 5.5% with depressions symptoms, while in the group of non active in sports (N = 60) are 18 depressive (chi2-test: p = 0,005). Females are statistically more depressed than males (chi2-test: p = 0.01). In the female group 49 (10.5%) are depressed, and in the male group are 9 (5%). Compared to gender in separate analysis we did not find correlation of decreasing depression symptoms and sport activity among males (chi2-test: p = 0.47), while in females we find that sport activity has significant effect (chi2-test: p = 0.026). Our results shoved moderate values of depression symptoms among college population in Split, Croatia. More females than males experienced depressive symptoms. While sport activity did not have significant influence on the depression in male population, it has significant influence in reducing the depression symptoms among females.

[Guidelines for diagnosis, therapy and follow up of Anderson-Fabry disease]. / Smjernice za dijagnostiku, lijecenje i pracenje Anderson-Fabryjeve bolesti.

Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the α-galactosidase A (α-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations. Estimates of the incidence range from one per 40,000 to 60,000 in males, and 1:117,000 in the general population. Pain is usually the first symptom and is present in 60%-80% of affected children, as well as gastrointestinal disturbances, ophthalmologic abnormalities and hearing loss. Renal failure, hypertrophic cardiomyopathy, or stroke as the presenting symptom may also be found even as isolated symptoms of the disease. Life expectancy is reduced by approximately 20 years in males and 10-15 years in females, therefore enzyme replacement therapy should be introduced in patients of any age and either sex, who meet treatment criteria for Anderson-Fabry disease.

The association of ventricular tachycardia and endothelial dysfunction in the setting of acute myocardial infarction with ST elevation.

BACKGROUND: Ventricular tachycardia (VT) is frequently seen in ischemic settings like acute myocardial infarction with ST segment elevation (STEMI). Endothelial dysfunction (ED) represents inflammation and the loss of all protective features of the endothelium. We aimed to examine the association between VT and ED in patients with STEMI. MATERIAL/METHODS: The study included 90 subjects (30 with VT and acute STEMI, 30 with STEMI without VT, and 30 controls). Sera of all subjects were tested on ED markers by enzyme immunoassay: sICAM-1 (intracellular adhesive molecule-1), sVCAM-1 (vascular adhesive molecule-1), P- and E-selectins, and VEGF (vascular endothelial growth factor). In addition, CRP (C-reactive protein) was detected. RESULTS: Significantly increased values of low-density lipoprotein, triglycerides, leukocytes, creatinine, and the number of cigarettes smoked were observed among patients with VT+STEMI in comparison to controls. The levels of E-selectin were significantly lower in the VT+STEMI group than in the other groups, while the levels of VCAM-1 were significantly higher in the groups with STEMI and VT+STEMI compared to the controls. Lower levels of VEGF were recorded in STEMI and VT+STEMI groups compared to the control group. A significant correlation between CRP and VCAM-1 in patients with VT +STEMI was demonstrated. CONCLUSIONS: We showed that ED may have a role in the immunopathogenesis of VT in patients with STEMI. The role of sE- selectin and correlation of sVCAM-1 with CRP as possible ED predictive markers in patients with VT+STEMI should be further investigated in a large cohort of patients.

Successful management of the superior mesenteric artery occlusion in 86-year-old female: case report.

Chronic superior mesentery ischemia often presents a clinically asymptomatic diffuse atherosclerotic process. There are no compelling recommendations on the benefits of early revascularization strategy besides antithrombotic prophylaxis and statin treatment. Conversely, long-term prevalence of symptomatic cases in surgical patient cohorts is rarely reported in the literature. Acutization of chronic ischemia has a severe clinical course, so timely recognition may be considered lifesaving. We present a case of an 86-year-old woman hospitalized for acutized atherosclerotic narrowing of superior mesenteric artery. The patient was urgently operated on by aorto-mesenteric ring prosthesis revascularization. Postoperative course was uneventful and the patient regained 10 kilograms in the next few months.

Guidelines for diagnosis, therapy and follow up of Anderson-Fabry disease.

Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the alpha-galactosidase A (alpha-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations. Estimates of the incidence range from one per 40,000 to 60,000 in males, and 1:117,000 in the general population. Pain is usually the first symptom and is present in 60%-80% of affected children, as well as gastrointestinal disturbances, ophthalmologic abnormalities and hearing loss. Renal failure, hypertrophic cardiomyopathy, or stroke as the presenting symptom may also be found even as isolated symptoms of the disease. Life expectancy is reduced by approximately 20 years in males and 10-15 years in females, therefore enzyme replacement therapy should be introduced in patients of any age and either sex, who meet treatment criteria for Anderson-Fabry disease.

Persistent Left Superior Vena Cava in Patients Undergoing Cardiac Device Implantation: Clinical and Long-Term Data.

BACKGROUND: Persistent left superior vena cava (LSVC) is a rare congenital venous anomaly that may be found at the time of cardiac device lead insertion. METHODS: In this case series, we present clinical and long-term data of five patients with LSVC who underwent pacemaker (PM) or cardioverter defibrillator (ICD) implantation during the period of 10 years. RESULTS: Left-sided venous approach was used for device implantation in 3 patients with standard PM indications, whereas a right-sided venous approach and an epicardial approach had to be used in 2 patients who needed an ICD and biventricular PM, respectively. In post implantation period of 44 ± 29 months, one patient died due to stroke, one underwent heart transplantation, and 3 had atrial fibrillation. CONCLUSION: The long-term outcome of patients with persistent LSVC and implanted cardiac devices is mostly influenced by the presence of underlying heart disease.