pm024 the common genotypes of long qt syndrome and the role of electrocardiogram prediction---the...

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Introduction: Up to now there are not studies on comparative response to cardiopul- monary exercise testing (CPET) during transient left bundle branch block (LBBB) and normal intra-ventricular conduction (NIVC) in female athletes. Objectives: The aim of this study is to ll the gap existing to date for comparative studies on the response to CPET during transient LBBB and NIVC in female athletes. Methods: From January 2004 to April 2013 two cases of transient LBBB were detected on 10107 (0.02%) consecutive female athletes referred for cardiac preventive screening to competitive sports activity,. According to protocol both athletes, respectively a 25 years old roller skater and a 27 years old water-polo player, performed a CPET, and were invited to come back every six months for control. Have been made twenty-six CPET, respectively twelve during LBBB and fourteen during NIVC. The rest and peak oxygen uptake (VO2), oxygen pulse (O2 pulse), heart rate (HR), blood pressure, and the peak work load (PWL) sustained, measured during CPET performed in LBBB and NIVC were compared using the primer software. Results: The CPET data collected during LBBB and normal conduction expressed as mean plus/minus standard deviation (SD), and the P value were shown on the table. During the follow up the LBBB spontaneously disappear after fty-four months in both females. Conclusion: This study is the rst to compare the CPET response in female athletes with transient LBBB during the transitory conduction disturbance and NIVC. The above results support the notion that except the minor increase in peak SBP, the peak HR as well as the overall functional capacity expressed as peak VO2 achieved and PWL sustained in NIVC remain unchanged during LBBB. Disclosure of Interest: None Declared PM023 Aerobic Exercise Altered Electrocardiogram And Cardiac Biomarkers In Cachectic Tumor-Bearing Rats Aline Toneto 1 , Luiz Alberto F. Ramos* 1 , Emiliane Salomão 1 , Miguel A. Areas 1 , Maria Cristina C. Gomes-Marcondes 1 1 Biology Structural and Physiology, STATE UNIVERSITY OF CAMPINAS - UNICAMP, Campinas, Brazil Introduction: Cancer-cachexia state promotes intense involuntary weight loss, wasting skeletal muscle protein due to higher proteolysis and/or decreased protein synthesis. Physical exercise can delay the onset of anorexia and cachexia, inhibiting tumor growth and maintain the body sources associated to less fatigue and skeletal muscle mass, being an alternative of co-adjuvant cancer treatment 1 . Objectives: This work evaluated exercise effects on electrocardiogram (ECG) and cardiac biomarkers in the cachectic rats bearing Walker-256 tumor (W) subjected to mild aerobic exercise. Methods: Adult rats were distributed into 4 groups: Ccontrol rats; W- tumor-bearing; ECexercised rats (swimming for 9 weeks) and EWexercised tumor-bearing rats (5 ani- mals per group). The electrocardiogram was evaluated after anesthesia (ketamine 100mg/ Kg. plus xelazine 7mg/Kg intramuscular) during pre-agonic state assayed by LabChart software. Cardiac biomarkers (MPO, TIMP, PAI-total) were evaluated in heart muscle homogenate analysed by multiplex assay (Millipore-Luminex). Difference among groups assayed using one-way ANOVA followed by Bonferronis test (P<0.05). Results: ECG parameters: Heart rate decrease in W and WE groups. QT-c interval tended toincreased only in W rats (P¼0.109); recovering in EW. T wave amplitude increased in EW group.Cardiac biomarkers: MPO and TIMP increasedespecially in W group; PAI-total increased in both tumor-bearing groups, especially in EW rats. Conclusion: Aerobic exercise has benec effects in Walker tumor-bearing rats partly restoring the ventricular function (similar QT-c), but did not prevent the increased cardiac biomarkers related to thrombogenesis processes. Reference 1. SALOMÃO et al, Nutrition and Cancer, 2010, 62(8), 1095. Disclosure of Interest: None Declared PM024 The Common Genotypes of Long Qt Syndrome And The Role Of Electrocardiogram PredictionThe China Experience Cuilan Li 1 , Yuanfeng Gao 2 , Wenling Liu* 2 , Li Zhang 3 , Dayi Hu 2 , and For Chinese Channelopathy Register Investigators 1 Heart Center, 2 Peking University Peoples Hospital, Beijing, China, 3 Lankenau Medical Center, Lankenau Institute for Medical Research, Jefferson Medical College, Philadelphia, United States Introduction: LQTS is an inherited cardiac disorder characterized by QT prolongation and increased risks of sudden death. Mutations in 13 genes have been identied in hereditary LQTS. However, only 60% to 70% of the individuals affected with LQTS could be detected to have mutations in these genes. Approximately 90% of known mutations were on LQTS1-3 causing genes. Objectives: Since the vast majority of mutation carriers of LQT1-3 presented with geno- type-specic ECG patterns, in this study we tested its utility in targeted genotyping in a large cohort of Chinese patients with LQTS. Methods: The ECG-pattern-guided genotyping was conducted in 74.3% (107/144) pro- bands with LQTS enrolled in the Chinese Channelopathy Register Study. Probands with atypical ECG patterns were excluded from this study (n¼37). The initial 12-lead ECG of each patient was evaluated. Individuals were considered affected if they have a prolonged QT interval (QTc > 450ms in male and > 470 ms in females). Patients were predicted as possible LQT1, 2 or 3 based on the presence of ECG patterns typical to each genotype. Based on the ECG predictions, mutational screening were performed using PCR and direct DNA sequence analysis. DNA samples from 50 healthy Han Chinese served as controls. Results: A total of 67 mutations, including 29 novel mutations, were identied. Of which 26 were KCNQ1, 39 KCNH2 and 2 SCN5A mutations, respectively. Among the 107 patients, the ECG-diagnosis results showed that there were 38 LQT1, 65 LQT2 and 4 LQT3, while gene screening results showed that 26, 48 and 2 patients could be diagnosed genetically as LQT1, LQT2 and LQT3, respectively. The mutational positive rate was 71.0% (76/107) for all patients. To be specic, the ECG predicted genotype matched the mutation results in 68.4% (26/38), 73.8% (48/65) and 50.0% (2/4) for LQT1, LQT2 and LQT3, respectively. Conclusion: This study shows that most mutations harbored by LQTS patients are on LQT1- 3 causing genes while LQT2 the most common in Chinese. ECG prediction-guided genotyping is proven time and economically efcient, except for a low predictable rate of ECG- diagnosis in LQT3. Our experience sheds lights in LQTS studies in developing countries. Disclosure of Interest: None Declared PM025 A Severe Type of Long QT Syndrome and Cardiovascular Anomaly in a Homozygous Carrier of a Novel KCNH2 Mutation and Findings in Family Screening Khalfan S. Al-Senaidi* 1 , Guoliang Wang 2 , Li Zhang 3 , Dominik A. Beer 4 , Abdullah M. Al-Farqani 5 , Salim N. Al-Maskaryi 5 , Daniel J. Penny 2 , Peter R. Kowey 3 , Yuxin Fan 2 1 Cardiology, Sultan Qaboos Univ. Hospital, Muscat, Oman, 2 Section of Cardiology, Department of Pediatrics, John Welsh Cardiovascular Diagnostic Laboratory, Baylor College of Medicine, Houston, 3 Cardiology, Lankenau Medical Center, Lankenau Institute for Medical Research, Jefferson Medical College, 4 Philadelphia College of Osteopathic Medicine, Philadelphia, United States, 5 Cardiology, Royal Hospital, Muscat, Oman Introduction: Patients with inherited long QT syndrome (LQTS), especially if carrying homozygous mutations, are prone to torsade de pointes (TdP) and sudden death (SD). Identifying individuals with LQTS is of particular importance for SD prevention. Table 1. CPET results during LBBB vs NIVC CPET measure LBBBvsNIVC atRest Pvalue LBBBvsNIVCatPeak ofexercise Pvalue Heart Rate 73.9+/-8.6 vs 73.1+/-7.9 P¼0.820 176.7+/-4.02 vs 176.3+/-3.7 P¼0.798 SBP 128.1+/-2.3 vs 128.2 +/-2.3 P¼0.915 182.2+/-2.3 vs 221.7+/-2.3 P¼0.000 DBP 83.18+/-2.3 vs 81.07 +/-1.9 P¼0.019 102.7+/-4.2 vs 91.4+/-3.3 P¼0.000 VO2 ml/Kg/min 8.3+/-1.9 vs 8.2+/-1.8 P¼0.863 50.3+/-9.06 vs 50.1+/-8.05 P¼0.954 O2 pulse ml/beat 6.01+/-1.3 vs 5.87+/-1.4 P¼0.802 13.06+/-2.3 vs 12.8+/-2.4 P¼0.790 PWL 166.8+/-2.3 vs 167.1+/-2.3 P¼0.749 e66 GHEART Vol 9/1S/2014 j March, 2014 j POSTER/2014 WCC Posters POSTER ABSTRACTS

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Page 1: PM024 The Common Genotypes of Long Qt Syndrome And The Role Of Electrocardiogram Prediction---The China Experience

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Introduction: Up to now there are not studies on comparative response to cardiopul-monary exercise testing (CPET) during transient left bundle branch block (LBBB) andnormal intra-ventricular conduction (NIVC) in female athletes.Objectives: The aim of this study is to fill the gap existing to date for comparative studieson the response to CPET during transient LBBB and NIVC in female athletes.Methods: From January 2004 to April 2013 two cases of transient LBBB were detected on10107 (0.02%) consecutive female athletes referred for cardiac preventive screening tocompetitive sports activity,. According to protocol both athletes, respectively a 25 years oldroller skater and a 27 years old water-polo player, performed a CPET, and were invited to comeback every sixmonths for control.Havebeenmade twenty-sixCPET, respectively twelveduringLBBB and fourteen during NIVC. The rest and peak oxygen uptake (VO2), oxygen pulse (O2pulse), heart rate (HR), blood pressure, and the peak work load (PWL) sustained, measuredduring CPET performed in LBBB and NIVC were compared using the primer software.Results: The CPET data collected during LBBB and normal conduction expressed as meanplus/minus standard deviation (SD), and the P value were shown on the table. During thefollow up the LBBB spontaneously disappear after fifty-four months in both females.

Table 1. CPET results during LBBB vs NIVC

CPET measure LBBBvsNIVC atRest Pvalue LBBBvsNIVCatPeak ofexercise Pvalue

Heart Rate 73.9+/-8.6 vs 73.1+/-7.9 P¼0.820 176.7+/-4.02 vs 176.3+/-3.7 P¼0.798

SBP 128.1+/-2.3 vs 128.2 +/-2.3 P¼0.915 182.2+/-2.3 vs 221.7+/-2.3 P¼0.000

DBP 83.18+/-2.3 vs 81.07 +/-1.9 P¼0.019 102.7+/-4.2 vs 91.4+/-3.3 P¼0.000

VO2 ml/Kg/min 8.3+/-1.9 vs 8.2+/-1.8 P¼0.863 50.3+/-9.06 vs 50.1+/-8.05 P¼0.954

O2 pulse ml/beat 6.01+/-1.3 vs 5.87+/-1.4 P¼0.802 13.06+/-2.3 vs 12.8+/-2.4 P¼0.790

PWL 166.8+/-2.3 vs 167.1+/-2.3 P¼0.749

Conclusion: This study is the first to compare the CPET response in female athletes withtransient LBBB during the transitory conduction disturbance and NIVC. The above resultssupport the notion that except the minor increase in peak SBP, the peak HR as well as theoverall functional capacity expressed as peak VO2 achieved and PWL sustained in NIVCremain unchanged during LBBB.Disclosure of Interest: None Declared

PM023

Aerobic Exercise Altered Electrocardiogram And Cardiac Biomarkers In CachecticTumor-Bearing Rats

Aline Toneto1, Luiz Alberto F. Ramos*1, Emiliane Salomão1, Miguel A. Areas1,Maria Cristina C. Gomes-Marcondes11Biology Structural and Physiology, STATE UNIVERSITY OF CAMPINAS - UNICAMP,Campinas, Brazil

Introduction: Cancer-cachexia state promotes intense involuntary weight loss, wastingskeletal muscle protein due to higher proteolysis and/or decreased protein synthesis.Physical exercise can delay the onset of anorexia and cachexia, inhibiting tumor growth andmaintain the body sources associated to less fatigue and skeletal muscle mass, being analternative of co-adjuvant cancer treatment1.Objectives: This work evaluated exercise effects on electrocardiogram (ECG) and cardiacbiomarkers in the cachectic rats bearing Walker-256 tumor (W) subjected to mild aerobicexercise.Methods: Adult rats were distributed into 4 groups: C–control rats; W- tumor-bearing;EC–exercised rats (swimming for 9 weeks) and EW–exercised tumor-bearing rats (5 ani-mals per group). The electrocardiogram was evaluated after anesthesia (ketamine 100mg/Kg. plus xelazine 7mg/Kg intramuscular) during pre-agonic state assayed by LabChartsoftware. Cardiac biomarkers (MPO, TIMP, PAI-total) were evaluated in heart musclehomogenate analysed by multiplex assay (Millipore-Luminex). Difference among groupsassayed using one-way ANOVA followed by Bonferroni’s test (P<0.05).Results: ECG parameters: Heart rate decrease in W and WE groups. QT-c interval tendedtoincreased only in W rats (P¼0.109); recovering in EW. T wave amplitude increased inEW group.Cardiac biomarkers: MPO and TIMP increasedespecially in W group; PAI-totalincreased in both tumor-bearing groups, especially in EW rats.

e66

Conclusion: Aerobic exercise has benefic effects in Walker tumor-bearing rats partlyrestoring the ventricular function (similar QT-c), but did not prevent the increased cardiacbiomarkers related to thrombogenesis processes.Reference

1. SALOMÃO et al, Nutrition and Cancer, 2010, 62(8), 1095.

Disclosure of Interest: None Declared

PM024

The Common Genotypes of Long Qt Syndrome And The Role Of ElectrocardiogramPrediction—The China Experience

Cuilan Li1, Yuanfeng Gao2, Wenling Liu*2, Li Zhang3, Dayi Hu2, and For ChineseChannelopathy Register Investigators1Heart Center, 2Peking University People’s Hospital, Beijing, China, 3Lankenau Medical Center,Lankenau Institute for Medical Research, Jefferson Medical College, Philadelphia, United States

Introduction: LQTS is an inherited cardiac disorder characterized by QT prolongation andincreased risks of sudden death. Mutations in 13 genes have been identified in hereditaryLQTS. However, only 60% to 70% of the individuals affected with LQTS could be detectedto have mutations in these genes. Approximately 90% of known mutations were onLQTS1-3 causing genes.Objectives: Since the vast majority of mutation carriers of LQT1-3 presented with geno-type-specific ECG patterns, in this study we tested its utility in targeted genotyping in alarge cohort of Chinese patients with LQTS.Methods: The ECG-pattern-guided genotyping was conducted in 74.3% (107/144) pro-bands with LQTS enrolled in the Chinese Channelopathy Register Study. Probands withatypical ECG patterns were excluded from this study (n¼37). The initial 12-lead ECG ofeach patient was evaluated. Individuals were considered affected if they have a prolongedQT interval (QTc > 450ms in male and > 470 ms in females). Patients were predicted aspossible LQT1, 2 or 3 based on the presence of ECG patterns typical to each genotype.Based on the ECG predictions, mutational screening were performed using PCR and directDNA sequence analysis. DNA samples from 50 healthy Han Chinese served as controls.Results: A total of 67 mutations, including 29 novel mutations, were identified. Of which 26were KCNQ1, 39 KCNH2 and 2 SCN5Amutations, respectively. Among the 107 patients, theECG-diagnosis results showed that there were 38 LQT1, 65 LQT2 and 4 LQT3, while genescreening results showed that 26, 48 and 2 patients could be diagnosed genetically as LQT1,LQT2 and LQT3, respectively. The mutational positive rate was 71.0% (76/107) for allpatients. To be specific, the ECG predicted genotype matched the mutation results in 68.4%(26/38), 73.8% (48/65) and 50.0% (2/4) for LQT1, LQT2 and LQT3, respectively.Conclusion: This study shows thatmost mutations harbored by LQTS patients are on LQT1-3 causing genes while LQT2 the most common in Chinese. ECG prediction-guidedgenotyping is proven time and economically efficient, except for a low predictable rate of ECG-diagnosis in LQT3. Our experience sheds lights in LQTS studies in developing countries.Disclosure of Interest: None Declared

PM025

A Severe Type of Long QT Syndrome and Cardiovascular Anomaly in a HomozygousCarrier of a Novel KCNH2 Mutation and Findings in Family Screening

Khalfan S. Al-Senaidi*1, Guoliang Wang2, Li Zhang3, Dominik A. Beer4,Abdullah M. Al-Farqani5, Salim N. Al-Maskaryi5, Daniel J. Penny2, Peter R. Kowey3,Yuxin Fan21Cardiology, Sultan Qaboos Univ. Hospital, Muscat, Oman, 2Section of Cardiology, Departmentof Pediatrics, John Welsh Cardiovascular Diagnostic Laboratory, Baylor College of Medicine,Houston, 3Cardiology, Lankenau Medical Center, Lankenau Institute for Medical Research,Jefferson Medical College, 4Philadelphia College of Osteopathic Medicine, Philadelphia, UnitedStates, 5Cardiology, Royal Hospital, Muscat, Oman

Introduction: Patients with inherited long QT syndrome (LQTS), especially if carryinghomozygous mutations, are prone to torsade de pointes (TdP) and sudden death (SD).Identifying individuals with LQTS is of particular importance for SD prevention.

GHEART Vol 9/1S/2014 j March, 2014 j POSTER/2014 WCC Posters