Assessment of the incidence of fragmented QRS in electrocardiography in patients with systemic sclerosis and systemic lupus erythematosus.

OBJECTIVE: In systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) cardiac involvement is very common, and it might be asymptomatic. We aimed to assess the presence of fragmented QRS (fQRS) - possible indicator of a scar, diffuse damage or conduction system disturbances in those patients. METHODS: We examined 74 patients with SSc, 77 with SLE and 40 healthy controls. The incidence of fQRS in ECG was examined according to criteria by Das et al. Disease severity was estimated by Rodnan and SLICCC/ACRDI scores in SSc and SLE respectively. RESULTS: Patients with SSc were slightly older than those with SLE (53 ± 14 vs 46 ± 14 yrs), women constituted 91% and 88% of studied groups (p = 0.80). The duration of disease in both groups was similar (p = 0.59). Median of Rodnan and SLICCC/ACR-DI scores were 5 and 4 points, respectively. Left ventricle ejection fraction in both groups was similar (65.5 vs 65.1%, p = 0.51), hypertension incidence was lower in SSc (24 vs 48%, p = 0.004), patients with SLE presented prolonged corrected QT interval (432 vs 424 ms, p = 0.03). Of note, fQRS were observed in 34 (46%) SSc and 29 (38%) SLE patients, p = 0.33. Notched S waves were significantly more frequent in SSc (in 26% vs 10%, p = 0.02). fQRS occurred in 3 (7.5%) of healthy subjects only. CONCLUSION: The incidence of fQRS is similar in SSc and SLE, but is much more common than in healthy subjects. It is possible that fQRS may be considered an additional marker of heart involvement in these rare diseases.

Left ventricular diastolic dysfunction and diseases severity contribute to impaired exercise capacity in systemic lupus erythematosus.

OBJECTIVES: Patients with systemic lupus erythematosus (SLE) have a higher risk of myocardial involvement, which can result in ventricular dysfunction. The aim of our study was to estimate potential relationship between exercise capacity assessed by six minute walk test (6MWT) and echocardiographic parameters of left and right ventricular function in SLE patients. METHODS: We prospectively studied 66 SLE patients (57 F, age 44 (20-75) years) and 27 age matched healthy subjects. In addition to routine evaluation, 6MWT and transthoracic echocardiography including LV diastolic dysfunction parameters (E/A, E/É) were performed. RESULTS: While E/A was similar in both groups, E/E' was higher in patients with SLE than in controls, 7.5 (4-22) vs 6.8 (1.6-9.4), p = 0.018. The mean 6MWT distance was significantly shorter in SLE (561.6 ± 150.7 vs 682.6 ± 98.1 m, p < 0.002). Among SLE patients only 53 (80.3%) were capable to walk at least 450 m, while in controls 27 (100%) (p = 0.013). We observed significant correlations between 6MWT distance and SLICC/ACR-DI (rho=-0.44, p < 0.001), E/A (rho = 0.30, p = 0.004), E/E' (rho=-0.36, p < 0.001) in SLE patients. Univariable logistic regression models revealed that SLICC/ACR-DI, E/E', tricuspid regurgitant peak gradient (TRPG), and right ventricular systolic pressure (RVSP) were associated with 6MWT distance lower than < 450 m. ROC curves shown high predictive value of E/E' ratio, TRPG, RVSP in the prediction for 6MWT distance < 450 m. CONCLUSION: Impaired exercise tolerance seems to result mainly from the severity of SLE and LV diastolic dysfunction.

Comprehensive non-invasive assessment of electrocardiographic abnormalities and cardiac arrhythmias in patients with genetically confirmed mitochondrial diseases.

BACKGROUND: There is limited data on cardiac arrhythmias and ventricular repolarization and dispersion abnormalities in patients with mitochondrial diseases (MitD). METHODS: Consecutive 40 patients with genetically proven MitD and 35 healthy controls were studied. Among other examinations all subjects underwent 24-h Holter recording and 12­lead electrocardiography (ECG) with corrected QT (QTc), QT dispersion (QTd), Tp-e and Tp-e/QT ratio assessment. RESULTS: Patients with MitD were 55.4 ± 15.7 years old, the disease duration was 18.5 ± 10.3 years, presented 6 clinical syndromes while mitochondrial and nuclear DNA type of mutation was present in 40 and 60% of cases, respectively. In MitD more frequently 1st degree atrioventricular block and intraventricular conduction defects were observed and also QRS complex duration was increased. Mean values of QTc (p = 0.001), QTd (p = 0.02), Tp-e (p < 0.00001) and Tp-e/QT (p < 0.00001) were significantly higher in MitD than in controls. Correlations between disease duration and PR interval duration (p = 0.003) and Creatine Kinase MB isoenzyme activity (p = 0.02) were found. No differences in depolarization and dispersion parameters were observed according to type of mutation or dominant clinical syndromes. In addition to supraventricular extrasystoles, nonsustained supraventricular tachycardias occurred more frequently in MitD (in 45.0 vs 14.3%, p = 0.0004). Ventricular arrhythmias were rare and observed almost exclusively in subjects with mitochondrial DNA mutation. CONCLUSIONS: In contrast to healthy controls, in MitD patients intraventricular, repolarization and dispersion disturbances were more frequently observed. In addition to bradyarrhythmias observed in some defined MitD syndromes, supraventricular rather than ventricular arrhythmias are more common.

, , ACUTE PULMONARY EMBOLISM BY AMNIOTIC FLUID - A RARE COMPLICATION OF PERINATAL PERIOD THAT SHOULD NOT BE FORGOTTEN.

OBJECTIVE: Amniotic fluid embolism (AFE) is a diagnostically challenging type of pulmonary embolism that occurs when amniotic fluid enters maternal circulation during delivery or postpartum. This obstetric complication is very rare but characterized by high mortality rate. The main symptoms are dyspnea, cardiovascular collapse, disseminated intravascular coagulation (DIC) and even sudden cardiac death. The aim of the article is to draw attention to AFE as a rare but possible and catastrophic complication of perinatal period. The authors present a 28-year-old woman who was admitted to obstetric ward during the first stage of labour. The patient developed sudden deterioration of her medical state with acute respiratory distress symptoms. An emergency cesarean section was performed, complicated by excessive bleeding. After a detailed assessment of the patient's condition and evaluation of the results of additional tests, we diagnosed AFE as the cause of the patient's deterioration. CONCLUSION: Conclusions: The case study shows how unpredictable, unpreventable and dangerous is AFE. It is still one of the main causes of maternal deaths in developed countries. Four diagnostic criteria proposed by the Society for Maternal-Fetal Medicine (SMFM) may accelerate diagnosis. AFE as a medical emergency, requires immediate multidisciplinary response and aggressive treatment. The initial medical care may be facilitated by the application of the general guidelines recommended by SMFM. The case report also emphasizes the need for further research on this disease, in particular on early detection and prevention.

Serum galectin-3 and galectin-3 binding protein levels in systemic lupus erythematosus and cutaneous lupus erythematosus.

INTRODUCTION: The roles of galectin-3 (Gal-3) and galectin-3 binding protein (G3BP) in systemic lupus erythematosus (SLE) are of ongoing interest, but the data are insufficient due to highly limited available studies. There are no data on cutaneous lupus erythematosus (CLE). AIM: To assess serum Gal-3 and G3BP concentrations in SLE patients with and without LE-specific skin lesions, CLE patients and to correlate levels of proteins with clinical and laboratory parameters. MATERIAL AND METHODS: The study included 71 SLE patients with and without LE-specific skin lesions, 23 CLE patients and 40 controls. Gal-3 and G3BP were measured by specific enzyme-linked immunosorbent assays (ELISA). RESULTS: Serum Gal-3 and G3BP concentrations were significantly higher in SLE with and without LE-specific lesions compared to controls, but without differences between SLE groups. Gal-3 and G3BP levels were also elevated in CLE compared to controls (p = 0.001, p = 0.005; respectively). There was a positive correlation between G3BP level and CLASI activity score both in CLE (r = 0.55, p = 0.006) and in SLE patients with LE-specific lesions (r = 0.36, p = 0.02). G3BP and Gal-3 levels did not differ in SLE with LE-specific lesions and CLE. There was a positive correlation between serum G3BP level and the SLEDAI score in SLE patients (r = 0.26, p = 0.03). CONCLUSIONS: Our findings indicate that serum G3BP and Gal-3 are elevated in CLE. Additionally, G3BP might be associated with the extent of skin lesions. There are no differences between G3BP and Gal-3 concentrations in SLE with and without LE-specific skin lesions.

Influence of disease severity and cardiac autonomic tone on ventricular repolarization and dispersion in electrocardiographic assessment of patients with systemic lupus erythematosus.

BACKGROUND: There are no data on the influence of disease severity and cardiac autonomic tone on ventricular repolarization and dispersion in 24-hour Holter monitoring in systemic lupus erythematosus (SLE). METHODS: Consecutive 92 SLE and 51 healthy subjects were studied. The standard 12-lead electrocardiography (ECG), Holter monitoring with heart rate turbulence (HRT) and QT, Tp-e and Tp-e/QT ratio assessment (including corrected values) were performed. Subjects with conditions causing repolarization abnormalities or insufficient number of beats suitable for QT evaluation were excluded (17 SLE and 8 controls). RESULTS: Finally, 75 SLE and 43 sex- and age-matched controls were included to the study. In SLE patients, the median disease severity score (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI)) was 3.0. The mean values of QTc, cTp-e and cTp-e/QTc were significantly higher in SLE patients than in controls. QTc ≥ 460 ms was observed in 18.7% of patients using standard ECG and in 58.7% using Holter monitoring. With Holter monitoring, patients with SLICC/ACR-DI >3.0 presented longer QTc than those with SLICC/ACR-DI ≤3.0 (418±15 vs. 409 ± 16, p = 0.04), while cTp-e and cTp-e/QTc values were similar. Patients with abnormal HRT presented longer cTp-e and higher cTp-e/QTc than those with normal HRT (92 ± 52 vs. 71 ± 16 ms, p = 0.04; 0.244 ± 0.126 vs. 0.187 ± 0.035, p = 0.03), while QTc values were similar. No differences in QT and Tp-e parameters were observed according to disease duration. CONCLUSION: In SLE patients, Holter monitoring revealed QTc prolongation more frequently than standard ECG. Longer QTc values were observed in patients with more advanced disease, while increased cTp-e and cTp-e/QTc were related to cardiac autonomic dysfunction expressed by abnormal HRT.

Comparison of non-invasive assessment of arrhythmias, conduction disturbances and cardiac autonomic tone in systemic sclerosis and systemic lupus erythematosus.

Systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) are connective tissue diseases presenting cardiac complications including different arrhythmias, then direct electrocardiographic comparison may be useful in everyday clinical decision making. We examined 86 adult SSc patients, 76 with SLE and 45 healthy controls. Among other examinations all subjects underwent 24-h Holter monitoring with time-domain heart rate variability and heart rate turbulence evaluation. Patients with various co-existing conditions which might markedly influence arrhythmias and autonomic modulation were excluded from further analysis (SSc n = 12, SLE n = 6). Finally, 76 SSc and 70 SLE subjects were eligible for this study, mean age 51.9 ± 13.1 and 46.5 ± 12.7 years (p = 0.11), with median disease duration 6.0 and 8.5 years (p = 0.15), respectively. As compared to SLE, patients with SSc were characterised by more frequent incidence of various supraventricular and ventricular arrhythmias. As compared to SSc, patients with SLE presented prolonged corrected QT intervals and also significant correlations between corrected QT length and heart rate variability indices. Both SSc and SLE subjects presented impaired sympathetic cardiac autonomic modulation, while indices associated with parasympathetic activity in SLE were not diminished. Disease duration was not associated with arrhythmias' occurrence (except for ventricular tachycardia in SSc, p = 0.02) and also with autonomic function in both groups of patients. Patients with SSc and SLE differ in terms of arrhythmias, conduction disturbances and cardiac autonomic tone. Regular Holter monitoring should be considered as a part of routine evaluation in connective tissue diseases patients, especially in systemic sclerosis.

Tricuspid Regurgitation Peak Gradient (TRPG)/Tricuspid Annulus Plane Systolic Excursion (TAPSE)　- A Novel Parameter for Stepwise Echocardiographic Risk Stratification in Normotensive Patients With Acute Pulmonary Embolism.

BACKGROUND: Patients with intermediate-risk acute pulmonary embolism (APE) are a heterogeneous group with an early mortality rate of 2-15%. The tricuspid annulus plane systolic excursion (TAPSE) and tricuspid regurgitation peak gradient (TRPG) can be used for risk stratification, so we analyzed the prognostic value of a new echo parameter (TRPG/TAPSE) for prediction of APE-related 30-day death or need for rescue thrombolysis in initially normotensive APE patients.Methods and Results:The study group consists of 400 non-high-risk APE patients (191 men, age: 63.1±18.9 years) who had undergone echocardiography within the first 24 h of admission. The TRPG/TAPSE parameter was calculated. The clinical endpoint (CE) was a combination of 30-day APE-related death and/or rescue thrombolysis. The CE occurred in 8 (2%) patients. All patients with TAPSE ≥20 mm (n=193, 48.2%) had a good prognosis. Among 206 patients with TAPSE <20 mm, 8 cases of the CE occurred (3.9%). NPV and PPV for TRPG/TAPSE >4.5 were 0.2 and 0.98, respectively. The CE was significantly more frequent in 19 (9.2%) patients with TRPG/TAPSE >4.5 than in 188 (90.8%) with TRPG/TAPSE ≤4.5 (4 (21.1%) vs. 4 (2.1%), P=0.0005). Among normotensive APE patients with TAPSE <20 mm, TRPG/TAPSE >4.5 was associated with 21.1% risk of APE-related death or rescue thrombolysis. CONCLUSIONS: TRPG/TAPSE, a novel echocardiographic parameter, may be useful for stepwise echocardiographic risk stratification in normotensive patients with APE, and it identifies patients with a poor prognosis.

Management of high risk pulmonary embolism - a single center experience.

BACKGROUND AND AIM: Patients with acute pulmonary embolism (APE) associated with hemodynamic instability, i.e. high-risk APE (HR-APE), are at risk for early mortality and require urgent reperfusion therapy with thrombolysis or embolectomy. However, a considerable proportion of HR-APE subjects is not reperfused but only anticoagulated due to high bleeding risk. The aim of the present study was to assess the management of HR-APE in a single large-volume referral center. METHODS: A single-center retrospective study of 32 HR-APE subjects identified among 823 consecutive patients hospitalized for symptomatic APE. RESULTS: Out of 32 subjects with HR-APE (19 women, age 69 ± 19 years), 20 patients were unstable at admission and 12 subsequently deteriorated despite on-going anticoagulation. Thrombolysis was applied in 20 (62.5%) of HR-APE subjects, limited mainly by classical contraindications in the remainder. Percutaneous pulmonary embolectomy was performed in 4 patients. In-hospital PE-related mortality tended to be higher, albeit insignificantly, in the patients who developed hemodynamic collapse during the hospital course compared to those unstable at admission (67% vs. 40%, p = 0.14). Also, survival was slightly better in 22 patients treated with thrombolysis or percutaneous embolectomy in comparison to 10 subjects who received only anticoagulation (54% vs. 40%, p = 0.2). Major non-fatal bleedings occurred in 7 of 20 patients receiving thrombolysis (35%) and in 2 (17%) of the remaining non-thrombolysed 12 HR-APE subjects. CONCLUSIONS: Hemodynamically instability, corresponding to the definition of HR-APE, affects about 4% of patients with APE, developing during the hospital course in approximately one-third of HR-APE subjects. As almost 40% of patients with HR-APE do not receive thrombolytic therapy for fear of bleeding, urgent percutaneous catheter-assisted embolectomy may increase the percentage of patients with HR-APE undergoing reperfusion therapy. Further studies are warranted for a proper identification of initially stable intermediate-risk APE subjects at risk of hemodynamic collapse despite appropriate anticoagulation.

Cardiac autonomic function in type 1 and type 2 myotonic dystrophy.

OBJECTIVE: The aim of this study was to evaluate cardiac autonomic nervous system function using Holter-derived and standard electrocardiographic parameters in patients with myotonic dystrophy (dystrophia myotonica, DM) and no clinically overt heart involvement. METHODS: Eighty-four DM patients without conditions potentially influencing cardiac autonomic function were enrolled in the study: 44 with DM type 1 and 40 with DM type 2 (mean age 34.9 ± 11.5 and 47.8 ± 13.5 years, respectively). Two corresponding control groups of aged-matched healthy subjects were selected for DM1 (n = 35) and for DM2 (n = 30). Standard electrocardiography for QT interval dispersion and 24-h Holter monitoring with time-domain heart rate variability and heart rate turbulence were performed. RESULTS: No significant differences in time-domain heart rate variability parameters between DM1 or DM2 subjects and controls were observed. However, heart rate turbulence parameters were significantly impaired in DM1 patients as compared to their controls: turbulence onset (p = 0.025), and turbulence slope (p = 0.018). Moreover, turbulence slope was also impaired in DM2 patients (p = 0.042). As compared to controls, we observed an increased QT dispersion, both in DM1 (p = 0.003) and also in DM2 patients (p < 0.0001). No relationship between disease duration or neurological status and time-domain heart rate variability, heart rate turbulence, and QT dispersion was observed. INTERPRETATION: Despite normal time-domain heart rate parameters, impaired heart rate turbulence and increased QT dispersion may suggest cardiac autonomic nervous system dysfunction in DM patients. The present study is the first one in which heart rate turbulence and QT dispersion assessment were examined both in DM1 and DM2 patients.

Supraventricular and Ventricular Arrhythmias Are Related to the Type of Myotonic Dystrophy but Not to Disease Duration or Neurological Status.

BACKGROUND: Patients with myotonic muscular dystrophy (dystrophia myotonica, DM) are at risk of sudden cardiac death due to diverse arrhythmias, especially progressive atrioventricular (AV) conduction abnormalities. However, there are limited data on supraventricular and potentially life-threatening ventricular arrhythmias, especially according to type 1 and type 2 DM. METHODS: A group of 94 unselected consecutive patients with genetically confirmed DM and 45 healthy controls underwent electrocardiography, echocardiography, and 24-hour Holter monitoring. DM1 was diagnosed in 51, while DM2 in 43 patients (with mean age of 37.3 ± 12.5 and 48.3 ± 13.3 years, respectively). RESULTS: DM1 subjects presented more frequently intraventricular conduction defects (29.4% vs 6.6%, P = 0.0003) and first-degree AV block (25.0% vs 4.6%, P = 0.008) than DM2 patients. Nonsustained supraventricular tachycardia (37.2% vs 3.8%, P = 0.001) and nonsustained ventricular tachycardia and/or R-on-T ventricular beats (23.2% vs 7.8%, P = 0.04) were more frequently observed in DM2 than in DM1. No relationship between disease duration and neurological status and occurrence of arrhythmias was observed. Multivariate analysis showed that independent predictor for bradyarrhythmias occurrence was DM1 only (odds ratio [OR] 6.4, 95% confidence interval [CI] 2.0-20.8, P = 0.002), while for supraventricular or ventricular arrhythmias occurrence it was DM2 (OR 4.1, 95% CI 1.5-11.4, P = 0.007) and increased age (OR 1.09, 95% CI 1.05-1.15, P < 0.0001). CONCLUSIONS: In the relatively large groups of DM1 and DM2 patients, we observed frequent various arrhythmias, which warrant their close cardiac monitoring. DM1 subjects when compared to DM2 presented more frequently intraventricular and AV conduction defects. However, all types of tachyarrhythmias (except atrial fibrillation) were more frequently observed in DM2 patients.

Andersen-Tawil syndrome: report of 3 novel mutations and high risk of symptomatic cardiac involvement.

INTRODUCTION: Andersen-Tawil syndrome (ATS) is a potassium channelopathy affecting cardiac and skeletal muscle. Periodic paralysis is a presenting symptom in some patients, whereas, in others, symptomatic arrhythmias or prolongation of QT in echocardiographic recordings will lead to diagnosis of ATS. Striking intrafamilial variability of expression of KCNJ2 mutations and rarity of the syndrome may lead to misdiagnosis. METHODS: We report 15 patients from 8 Polish families with ATS, including 3 with novel KCNJ2 mutations. RESULTS: All patients had dysmorphic features; periodic paralysis affected males more frequently than females (80% vs. 20%), and most attacks were normokalemic. Two patients (with T75M and T309I mutations) had aborted sudden cardiac death. An implantable cardioverter-defibrillator was utilized in 40% of cases. CONCLUSIONS: KCNJ2 mutations cause a variable phenotype, with dysmorphic features seen in all patients studied, a high penetrance of periodic paralysis in males and ventricular arrhythmia with a risk of sudden cardiac death.

Severity of arterial and chronic thromboembolic pulmonary hypertension is associated with impairment of heart rate turbulence.

BACKGROUND: Heart rate turbulence (HRT) impairment is a validated and an independent indicator of cardiovascular death. There are limited data on HRT in pulmonary hypertension (PH), so we assessed potential HRT alterations in PH, especially in relation to its severity. METHODS: Thirty-three out of 41 patients were enrolled in the study aged 49.7 ± 15.9 years (22 with arterial, 11 with chronic thromboembolic PH). Routine evaluations, right heart catheterization, and 24-hour Holter monitoring with heart rate variability and HRT assessment were performed. RESULTS: HRT was significantly impaired in PH patients, as compared to 25 healthy controls: mean turbulence onset (TO) was -0.27% versus -2.60% (P < 0.0001), and median turbulence slope (TS) was 3.13 versus 13.5 msRR (P < 0.0001). Abnormal HRT (TO ≥ 0.0% and/or TS ≤ 2.5 ms/RR) was found in 63.3% of PH patients. Patients with PH and abnormal HRT presented more compromised functional, biochemical, and hemodynamic status than PH patients with normal TO and TS values. Multivariate stepwise regression analysis showed that TO value was related to oxygen desaturation <90% in 6-minute walking test (6-MWT; OR 0.41, P < 0.001) and was related to N-Terminal pro-B type Natriuretic Peptide concentration (OR 0.40, P < 0.001); TS was related to 6-MWT distance (OR 0.53, P < 0.0001). CONCLUSIONS: Patients with arterial or chronic thromboembolic PH are characterized by significant impairment of HRT which is related to the disease severity. We hypothesize that patients with abnormal HRT could be considered as subjects with an increased risk of cardiovascular death, however, it needs further investigation.

Functional class and type of pulmonary hypertension determinate severity of cardiac autonomic dysfunction assessed by heart rate variability and turbulence.

OBJECTIVE: Heart rate variability (HRV) and turbulence (HRT) are validated methods of evaluation of cardiac autonomic nervous system (cANS) dysfunction which is associated with sudden cardiac death. There is limited data on cANS imbalance and its significance,in patients with pulmonary hypertension (PH), so we assessed HRV and especially HRT alterations in PH. METHODS AND RESULTS: Thirty-three out of 41 PH patients were enrolled in the study: 22 (66.7%) with pulmonary arterial hypertension (PAH) and 11 (33.3%) with chronic thromboembolic PH (CTEPH), 11 patients were in World Health Organization Functional Class (WHO-FC) I-II, and 22 others in WHO-FC III-IV. The control group consisted of 25 healthy persons. Routine evaluations, echocardiography, right heart catheterization and 24-hour Holter monitoring with time-domain HRV and HRT evaluation were performed. Most of HRV indices and both of HRT parameters were significantly impaired in PH patients, as compared to control subjects. Abnormal HRT (turbulence onset 0.0% and/or turbulence slope 2.5 ms/RR) was found in 36.4% WHO-FC I-II and in 77.3% WHO-FC III-IV patients (P=0.05). Patients in WHO-FC III-IV or with CTEPH presented compromised HRV and HRT values when compared to patients in WHO-FC I-II or with PAH, respectively. Multivariate analysis showed that abnormal HRT occurrence in PH was influenced by higher WHO-FC only (odds ratio 5.3, 95% CI 1.4-19.9, P= 0.01). CONCLUSION: Higher WHO-FC and probably type of pulmonary hypertension (CTEPH more than PAH) determinate the severity of cANS dysfunction assessed by heart rate variability and turbulence. Potential clinical consequences of our findings need further investigations.

Cardiac arrest and electrical storm due to recurrent torsades de pointes caused by concomitant clarithromycin, cotrimoxazole and amiodarone treatment.

Torsades de pointes (TdP) is a rapid, polymorphic and usually self-terminating ventricular tachycardia associated with the long QT syndrome. Many drugs may cause prolongation of QT interval and be the trigger for TdP occurrence. We present the case of 52-year-old male who was treated with clarithromycin due to bilateral atypical pneumonia. However, on the fourth day of hospitalization he deteriorated, developed pulmonary edema and short cardiac arrest. After successful resuscitation, unfortunately amiodarone and co-trimoxazole were given causing the arrhythmic storm which required many defibrillations. The case highlights the importance of careful QT measurement, appropriate TdP treatment and difficulties resulting from the patient's disagreement for invasive treatment. We think, that knowledge of drug-induced long QT syndrome and its consequences should be widely spread not only in cardiologists, but also in others doctors.

Six-minute walk test reflects neurohormonal activation and right ventricular function in systemic sclerosis patients.

OBJECTIVES: Heart and pulmonary involvement is a leading cause of systemic sclerosis (SSc)-related deaths. The six-minute walk test (6MWT) is a simple and reproducible test commonly used to evaluate exercise capacity. We tried to assess a potential relationship between exercise capacity assessed by 6MWT, echocardiographic parameters of right ventricular function and serum levels of endothelin-1 and NTproBNP. METHODS: We prospectively studied 111 consecutive patients (101F, 10M, age 54.2±13.8 years) with diagnosed SSc (mean disease duration 9±12.4 years) and a group of 21 age-matched subjects (18F, 3M, age 49.3±10.5 years). In addition to routine evaluation, 6MWT and transthoracic echocardiography (Phillips iE 33) were performed. We also measured serum endothelin-1 (Human Endothelin-1 immunoassay R & D Systems) and NT-proBNP levels (Elecsys pro-BNP immunoassay; Roche Diagnostics). RESULTS: The mean 6MWT distance was significantly shorter in the SSc group than in the controls (562.8±60.3 vs. 514.7±102.5 m, p=0.03). In the SSc group 6MWT distance correlated with ET-1 (r=-0.5, p<0.0001), NTproBNP (r=-0.4, p=0.0008) levels, and echocardiographic indices AcT (r=0.4, p=0.0002) and TRPG (r=-0.4, p=0.0011). Moreover, in patients with 6MWT distance <450 m NTproBNP and endotothelin-1 levels were significantly higher than in patients with distance >450 m (311.2, 31.1-17237 vs. 105.3, 5-17670 pg/ml, p=0.0138 and 2.9±2.2 vs. 1.4±0.7 pg/ml, p=0.0032). CONCLUSIONS: Decreased exercise capacity significantly correlates with biochemical and echocardiographic parameters of right ventricular dysfunction and neurohormonal activation providing a potential link for neuroendocrine derangement in patients with SSc.

Heart Rate Variability Impairment Is Associated with Right Ventricular Overload and Early Mortality Risk in Patients with Acute Pulmonary Embolism.

The association between heart rate variability (HRV) and mortality risk of acute pulmonary embolism (APE), as well as its association with right ventricular (RV) overload is not well established. We performed an observational study on consecutive patients with confirmed APE. In the first 48 h after admission, 24 h Holter monitoring with assessment of time-domain HRV, echocardiography and NT-proBNP (N-terminal pro-B-type natriuretic peptide) measurement were performed in all participants. We pre-examined 166 patients: 32 (20%) with low risk of early mortality, 65 (40%) with intermediate-low, 65 (40%) with intermediate-high, and 4 (0.02%) in the high risk category. The last group was excluded from further analysis due to sample size, and finally, 162 patients aged 56.3 ± 18.5 years were examined. We observed significant correlations between HRV parameters and echocardiographic signs of RV overload. SDNN (standard deviation of intervals of all normal beats) correlated with echocardiography-derived RVSP (right ventricular systolic pressure; r = -0.31, p = 0.001), TAPSE (tricuspid annulus plane systolic excursion; r = 0.21, p = 0.033), IVC (inferior vena cava diameter; r = -0.27, p = 0.002) and also with NT-proBNP concentration (r = -0.30, p = 0.004). HRV indices were also associated with APE risk stratification, especially in the low-risk category (r = 0.30, p = 0.004 for SDNN). Univariate and multivariate analyses confirmed that SDNN values were associated with signs of RV overload. In conclusion, we observed a significant association between time-domain HRV parameters and echocardiographic and biochemical signs of RV overload. Impaired HRV parameters were also associated with worse a clinical risk status of APE.

Plasma Troponins Identify Patients with Very Low-Risk Acute Pulmonary Embolism.

INTRODUCTION: Although in the non-vitamin K oral anticoagulants (NOAC) era majority of low-risk acute pulmonary embolism (APE) patients can be treated at home, identifying those at very low risk of clinical deterioration may be challenging. We aimed to propose the risk stratification algorithm in sPESI 0 point APE patients, allowing them to select candidates for safe outpatient treatment. MATERIALS AND METHODS: Post hoc analysis of a prospective study of 1151 normotensive patients with at least segmental APE. In the final analysis, we included 409 sPESI 0 point patients. Cardiac troponin assessment and echocardiographic examination were performed immediately after admission. Right ventricular dysfunction was defined as the right ventricle/left ventricle ratio (RV/LV) > 1.0. The clinical endpoint (CE) included APE-related mortality and/or rescue thrombolysis and/or immediate surgical embolectomy in patients with clinical deterioration. RESULTS: CE occurred in four patients who had higher serum troponin levels than subjects with a favorable clinical course (troponin/ULN: 7.8 (6.4-9.4) vs. 0.2 (0-1.36) p = 0.000). Receiver operating characteristic (ROC) analysis showed that the area under the curve for troponin in the prediction of CE was 0.908 (95% CI 0.831-0.984; p < 0.001). We defined the cut-off value of troponin at >1.7 ULN with 100% PPV for CE. In univariate and multivariate analysis, elevated serum troponin level was associated with an increased risk of CE, whereas RV/LV > 1.0 was not. CONCLUSIONS: Solely clinical risk assessment in APE is insufficient, and patients with sPESI 0 points require further assessment based on myocardial damage biomarkers. Patients with troponin levels not exceeding 1.7 ULN constitute the group of "very low risk" with a good prognosis.

Electrocardiographic criteria of left ventricular hypertrophy in patients with morbid obesity.

BACKGROUND: Obesity is frequently accompanied by systemic hypertension complicated by left ventricular hypertrophy (LVH). Standard electrocardiography (ECG) is generally accepted screening tool for LVH in systemic hypertension. The aim was to assess currently used ECG criteria in the diagnosis of LVH in morbidly obese patients. METHODS: Ninety-five patients (80 women, 15 men) with body mass index ≥ 40 kg/m(2) , prior to scheduled bariatric surgery were included into the study. All patients underwent standard ECG and transthoracic ECG for LVH assessment. RESULTS: Echocardiographically LVH (>110 g/m(2) in women, and >132 g/m(2) in men) was diagnosed in 54 patients (56.8%). None of the ECG criteria showed satisfactory performance in the diagnosing echocardiographically confirmed LVH. Although, Receiving operating curves (ROC) analysis showed that only Romilht-Estes score and Cornell index × QRS complex duration were characterized by area under curve >0.6 (0.662; 0.612, respectively),currently recommended values of both tests (Romilht-Estes score and Cornell index × QRS duration 2436 mm · ms) showed very low sensitivity in morbidly obese patients (0% and 2%, respectively). CONCLUSIONS: Our study showed that none of voltage-based ECG criteria are of value for LVH diagnosis in severely obese patients. Only Romhilt-Estes scale and Cornell indices could be helpful for the identification of LVH in the group of patients with morbid obesity, but their value is far from being satisfactory.

Holter-Derived Autonomic Function, Arrhythmias and Carbohydrate Metabolism in Patients with Class III Obesity Treated with Laparoscopic Sleeve Gastrectomy.

The effects of weight loss following bariatric surgery on autonomic balance, arrhythmias and insulin resistance are still of interest. We prospectively investigated 50 patients with BMI > 40 kg/m2, aged 36.5 (18-56) years who underwent laparoscopic sleeve gastrectomy. Among other examinations, all subjects had 24-h Holter monitoring with heart rate variability (HRV) and heart rate turbulence (HRT) evaluation. After a median of 15 months, BMI decreased from 43.9 to 29.7 kg/m2, the incidence of hypertension decreased from 54 to 32% (p = 0.04) and any carbohydrate disorders decreased from 24 to 6% (p = 0.02). Fasting insulin concentration and insulin resistance index improved significantly (p < 0.001). Improvements in HRV parameters related to the sympathetic autonomic division were also observed (p < 0.001), while HRT evaluation was not conclusive. The enhancement of autonomic tone indices was correlated with reduction of BMI (SDNN-I r = 0.281 p = 0.04; SDNN r = 0.267 p = 0.05), but not with reduction of waist circumference, and it was also associated with decrease of mean heart rate (OR 0.02, 95%CI 0.0-0.1, p < 0.001). The incidence of arrhythmias was low and similar before and after follow-up. In conclusion, improvement of homeostasis of carbohydrate metabolism and autonomic function is observed in relatively young patients after weight loss due to laparoscopic sleeve gastrectomy.