Lesław Szydłowski

Blood pressure regulation and resting heart rate abnormalities in adolescent girls with polycystic ovary syndrome

OBJECTIVE To assess the risk of cardiovascular disease on the basis of biochemical, echocardiographic, and 24-hour blood pressure (BP) monitoring parameters in adolescent girls with polycystic ovary syndrome (PCOS). DESIGN Cross-sectional study. SETTING Academic and research institution. PATIENT(S) Thirty-four obese and nonobese girls with PCOS were evaluated and compared with body mass index-matched girls with regular menses. INTERVENTION(S) None. MAIN OUTCOME MEASURE(S) Androgens, gonadotropins, lipids, and fasting and oral glucose tolerance test-stimulated glucose and insulin concentrations were measured. Echocardiographic assessment and 24-hour BP monitoring were done. RESULT(S) Compared with obese controls, obese girls with PCOS had significantly higher 24-hour mean BP, day mean BP, day diastolic BP, and diastolic BP nighttime dip (75.5 ± 4.5 mm Hg vs. 71.7 ± 3.7 mm Hg; 78.2 ± 5.0 mm Hg vs. 73.6 ± 4.0 mm Hg; 67.6 ± 4.9 mm Hg vs. 63.7 ± 3.7 mm Hg; and 20.2% ± 5.2% vs. 15.0% ± 6.6%, respectively). Obese girls with PCOS had significantly higher night heart rate than obese controls (60.4 ± 5.6 beats per minute vs. 61.7 ± 4.8 beats per minute). Left ventricle end-diastolic (4.6 ± 0.3 cm vs. 4.2 ± 0.2 cm) and end-systolic diameter (3.0 ± 0.3 cm vs. 2.7 ± 0.2 cm) were also significantly higher in nonobese girls with PCOS than in nonobese controls; however, all values were still within the accepted range of normal limits. CONCLUSION(S) Higher night heart rate in obese girls with PCOS and higher day BP but preserved diastolic nocturnal dip in nonobese girls with PCOS may be regarded as early cardiovascular disease risk factors.

Age-and sex-dependent mRNA expression of KCNQ1 and HERG in patients with long QT syndrome type 1 and 2.

Introduction The main goal of this study was to examine the patient age and sex dependent expression of KCNQ1 and HERG genes that encode potassium channels responsible for the occurrence of long QT syndrome (LQTS). Material and methods The study enrolled 43 families whose members suffered from LQTS type 1 (LQTS1) or 2 (LQTS2) or were healthy. The study attempted to prove that β-actin is a good endogenous control when determining the expression of the studied genes. Examination of gene expression was achieved with quantitative real-time PCR (QRT-PCR). Expression of the investigated genes was inferred from the analysis of the number of mRNA copies per 1 μg total RNA isolated from whole blood. Results Significantly lower KCNQ1 and KCNH2 mRNA levels in healthy females than healthy males were observed (p = 0.032; p = 0.02). In male patients both transcripts were expressed at a lower level (p = 0.0084; p = 0.035). The comparison of transcriptional activity of KCNQ1 and KCNH2 in healthy adults and children revealed higher KCNQ1 and lower KCNH2 mRNA levels in healthy adults (p = 0.033; p = 0.04), higher KCNQ1 and lower KCNH2 mRNA levels in adult patients below 55 years old than in adults over 55 years old (p=0.036; p = 0.044), and significantly higher KCNQ1 and lower KCNH2 mRNA levels in adult patients (over 55 years) than in paediatric patients (below 15 years) (p=0.047; p = 0.08). Conclusions The results support the hypothesis that KCNQ1 and HERG gene expression is influenced by age and gender in human patients with long QT syndrome and in healthy subjects.

Analysis of risk factors and prospective evaluation of cardiovascular complications of Kawasaki disease in children: a single centre study

BACKGROUND Kawasaki disease (KD) remains a diagnostic challenge due to its nonspecific clinical symptoms. Delayed treatment initiation increases the risk of coronary complications. AIM To evaluate the risk of coronary artery involvement and perform a prospective analysis of its course in children hospitalised due to KD. METHODS KD was diagnosed in 38 children, including 25 boys and 13 girls, aged 1.5-118 months (median 37.5 months). We assessed the risk of cardiac complications in relation to the presence of a complete or incomplete form of the disease, age, gender and laboratory test results, as well as the timing of treatment initiation. Thirty-six children were followed for 1-9 years in a cardiology clinic. RESULTS More than 80% of patients with KD were younger than 5 years. Eleven (29%) of them had an incomplete form of the disease. Coronary artery abnormalities were found in 10 (26%) children, insignificantly more often among those within complete KD. Each day of treatment delay increased the complication rate by almost 1.5 (OR 1.45, p = 0.009). Treatment initiated 10 days after the onset of the disease increased this risk almost nine times (OR 8.99, p = 0.007). No significant differences in respect to age (p = 0.431), gender (p = 0.744) and laboratory test results were found between the groups with and without coronary complications. A complete regression of coronary artery involvement was seen in 7 children, and partial regression was seen in one child. One child died and another needed coronary artery bypass grafting. CONCLUSIONS Coronary artery aneurysms developed at a similar rate in both complete and incomplete forms of KD and the only significant risk factor was the timing of treatment initiation. In young children with fever of unknown cause lasting longer than 5 days, echocardiography is warranted. Despite a tendency for coronary artery aneurysms to regress, late complications may occur and all children require long-term follow up in a cardiology clinic.

N-terminal prohormone brain natriuretic peptide-proBNP levels in ventricular arrhythmias in children.

Ventricular arrhythmias are the most common consequences of structural and functional heart diseases, but cases with no evident pathology are also observed. A parameter indicating asymptomatic circulatory failure could support decisions related to possible treatment of ventricular arrhythmias.

Arrhythmogenic right ventricular dysplasia:clinical study.

Arrhythmogenic right ventricular dysplasia (ARVD) is a complex arrhythmogenic cardiomyopathy, characterized by a partial or total replacement of the right ventricular myocytes by fatty and fibrous tissue. In this study, we present a case of ARVD in 17 year old girl, who was admitted to the hospital after syncope with ventricular arrhythmia. The echocardiography did not demonstrate structural cardiac abnormalities but the magnetic resonance recently showed thinning of the right ventricular wall. The girl was treated with the lidocaine, amiodarone and next, after radiofrequency catheter ablation she was receiving metoprolol. The girl has remained asymptomatic for four years of follow‐up.

Left Ventricular Diastolic Dysfunction Assessed by Conventional Echocardiography and Spectral Tissue Doppler Imaging in Adolescents With Arterial Hypertension

AbstractCompared to conventional echocardiography, spectral tissue Doppler imaging (s-TDI) allows more precise evaluation of diastolic cardiac function. The purpose of this study was to conduct s-TDI to analyze the slow movement of the left ventricular (LV) myocardium in adolescents with systemic arterial hypertension (HT) and to determine whether patients with HT suffer from LV diastolic dysfunction. The study group comprised 69 consecutive patients (48 boys and 21 girls aged 14–17 years [mean, 15.5 ± 1.1 years]) with primary HT, and the control group comprised 48 healthy participants (24 boys and 24 girls aged 14–17 years [mean, 15.8 ± 1.3 years]). Physical examinations, 24-hour arterial blood pressure monitoring, conventional 2-dimensional and Doppler echocardiography, and s-TDIs were performed. Analysis revealed that study group participants were significantly heavier and had greater LV mass indices than controls (P < 0.001). There were no differences between the velocities of E waves (peak early filling of mitral inflow), but the deceleration times of the mitral E waves were significantly shorter whereas the A waves survived longer in the study group than in the control group. The velocities of A waves (peak late filling of mitral inflow) were elevated (P = 0.041), and the E/A wave pattern (E/A = 1.8 ± 0.4) was normal. These results suggest pseudonormalization, a type of LV diastolic dysfunction in adolescents with HT.In the study group, when the sample volume was positioned at the septal or lateral insertion site of the mitral leaflet, the e′ wave velocity was significantly depressed whereas the a′ wave velocity was elevated, compared to those of the control group (P < 0.001).The e′/a′ ratios from the septal and lateral insertion sites were lower, whereas the E/e′ ratio from the septal insertion site was significantly higher in the study group, similar to that seen in atrial reversal velocity (P < 0.001).These findings indicate that using sTDI to find and measure diastolic LV failure is valuable when the probe is placed at the septal and lateral mitral valve annuli during examination.Changes in the myocardium appear similar to those seen in adults.

Tracheal agenesis: perioperative management of one stage correction.

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Validation of Standard and New Criteria for the Differential Diagnosis of Narrow QRS Tachycardia in Children and Adolescents.

AbstractTo establish an appropriate treatment strategy and determine if ablation is indicated for patients with narrow QRS complex supraventricular tachycardia (SVT), analysis of a standard 12-lead electrocardiogram (ECG) is required, which can differentiate between the 2 most common mechanisms underlying SVT: atrioventricular nodal reentry tachycardia (AVNRT) and orthodromic atrioventricular reentry tachycardia (OAVRT). Recently, new, highly accurate electrocardiographic criteria for the differential diagnosis of SVT in adults were proposed; however, those criteria have not yet been validated in a pediatric population.All ECGs were recorded during invasive electrophysiology study of pediatric patients (n = 212; age: 13.2 ± 3.5, range: 1–18; girls: 48%). We assessed the diagnostic value of the 2 new and 7 standard criteria for differentiating AVNRT from OAVRT in a pediatric population.Two of the standard criteria were found significantly more often in ECGs from the OAVRT group than from the AVNRT group (retrograde P waves [63% vs 11%, P < 0.001] and ST-segment depression in the II, III, aVF, V1–V6 leads [42% vs 27%; P < 0.05]), whereas 1 standard criterion was found significantly more often in ECGs from the AVNRT group than from the OAVRT group (pseudo r′ wave in V1 lead [39% vs 10%, P < 0.001]). The remaining 6 criteria did not reach statistical significance for differentiating SVT, and the accuracy of prediction did not exceed 70%. Based on these results, a multivariable decision rule to evaluate differential diagnosis of SVT was performed.These results indicate that both the standard and new electrocardiographic criteria for discriminating between AVNRT and OAVRT have lower diagnostic values in children and adolescents than in adults. A decision model based on 5 simple clinical and ECG parameters may predict a final diagnosis with better accuracy.

Simplified Automated Right Ventricular Overdrive Pacing for Rapid Diagnosis of Supraventricular Tachycardia

Objectives: The purpose of this study was to prospectively evaluate the feasibility and diagnostic value of right ventricular overdrive pacing (RVOP) during supraventricular tachycardia (SVT) using a 2-catheter approach with automatic pacing from the right ventricular inflow (RVIT) and outflow tract (RVOT). Methods: One hundred and thirty-six consecutive patients (with 138 arrhythmias, mean age 36 ± 20 years, range 4-95) were enrolled in this study. Only coronary sinus and ablation catheters were used. RVOP was delivered from RVIT and then from RVOT. Each attempt consisted of 10 synchronized beats delivered at a cycle length of 10-40 ms longer than the tachycardia cycle length. Results: RVOP was sufficient to confirm the transition zone within the first 9 beats in the majority of SVTs. Atrial perturbation (acceleration, delayed) in the transition zone was detected in all patients with orthodromic atrioventricular (AV) reentry. Patients with typical AV nodal reentry, atypical AV nodal reentry and atrial tachycardia did not show atrial timing perturbation during fusion complexes of RVOP. Conclusions: Synchronized RVOP from RVIT or RVOT is an easy and accurate method for the quick and reliable differential diagnosis of SVT in various clinical settings, particularly when only a limited number of catheters are used.

Early results of aortic arch reconstruction and bilateral pulmonary artery banding: modification of the Norwood operation for treatment of hypoplastic left heart syndrome

BACKGROUND In the period from 2003 to the end of 2015, 96 Norwood I procedures were performed in the Paediatric Heart Surgery Department in Katowice, Poland, in children with hypoplastic left heart syndrome (HLHS). AIM This paper presents a retrospective analysis of early surgical results. METHODS The patients consisted of two groups: group I included 59 children operated on in the years 2003-2012, in whom the stage I Norwood procedure with the Sano modification was performed with the aortic arch reconstructed by use of a ho-mogenous pulmonary artery patch or a bovine pericardial patch. Group II included 37 children after our modification of the Norwood I procedure, in which the aortic arch was reconstructed with an extracellular matrix patch and bilateral pulmonary artery banding was done. RESULTS Aortic cross-clamping time was significantly shorter in group II (mean 52; range 38-62 min) than in group I (mean 57; range 39-72 min; p < 0.009). Eighteen (30.5%) children in group I and six (16.2%) in group II died. Although this dif-ference did not reach statistical significance (p = 0.12), it suggested that improved outcomes with the modified procedure are possible. The cause of death in group I was significantly more frequently due to massive postoperative bleeding (n = 6; 33.3%) than in group II (n = 1; 16.7%; p = 0.046). CONCLUSIONS The introduction of this new surgical technique reduced postoperative bleeding rates, shortened the operation time, and might improve the mortality rate in the first-stage surgical treatment of children with HLHS.