**4. Summary**

Risk stratification for LQTSs is available with high correlation to positive genetic testing with 75% likelihood if the score is more than 4 points. Half of LQTS cases prove positive mutation. This is not the case with other channelopathies where paucity of positive mutations is the role. Beta blockers (propranolol and nadolol than metoprolol) are the first-line and easiest therapeutic choice for both LQT1 and LQT2. There is no scientific evidence favoring selective over non-selective beta blockers. It is always advised to keep beta blockers as adjunct treatment after ICD implants. In the current medical literature, there is controversy regarding the use of beta blockers in LQT3. Scientific evidence is suggesting significant therapeutic role of sodium channel blockers like ranolazine, mexiletine and flecainide in LQT3 treatment. Mexiletine was proved also of being an effective therapeutic option in LQT3 as well as LQT1 and LQT2. In the absence of concomitant gene mutations, epinephrine and isoproterenol were found to be effective in acquired LQTS. The implantation of an ICD is pivotal secondary prevention in LQTS and a reasonable primary prevention approach in selected cases. Surgical therapy in the form of left cardiac sympathetic denervation (LCSD) is a well-accepted treatment option in LQTS patients. It is an option in selected cases like LQT1 and LQT2 patients with no proper response to beta blockers, intolerance to beta blockers, or after ICD implant with recurrent arrhythmias. Aggressive management of febrile illnesses as well as avoidance of drugs inducing VT/VF is critical in BrS arrhythmia patients. Isoproterenol intravenously is used with success to control VF storms in BrS. ICD implant is a must for secondary prevention but is guarded in primary prevention especially in asymptomatic individuals. In case of frequent ICD shocks, quinidine can be used as adjunct treatment (up to 600 mg a day). Ablation of the anterior aspect of RVOT seems a promising and successful option in BrS patients. In PCVT, high doses of nadolol (3–5 mg/kg) may be necessary to suppress exertional ectopy. Because of the high risk of recurrent events and SCD on β-blockers, adjunctive ICD implantation is recommended in all PCVT symptomatic patients. Physicians must

**125**

prevent channelopathies in the human kind.

The author acknowledges his great parents the late Abdulrhman Alabdulgader and Mariam Almulhim (with a blessing for her for a long peaceful life) who taught

**Acknowledgements**

*Inherited Ventricular Arrhythmias, the Channelopathies and SCD: Current Knowledge…*

never rely on exercise test result for PCVT management. Flecainide (150–200 mg a day) is a promising first-line drug or as adjunct with beta blockers. Surgical option for PCVT represented by LCSD is an effective choice as a hybrid therapy to pharmaceutical agents. ICD, with primary termination, is the golden choice. Due to the role of emotional upset to induce attacks of VT/VF in PCVT, emotional management is of paramount importance. ER pattern was proven to be truly arrhythmogenic in 2008. Pathophysiological mechanism, as well as therapeutic approaches of ERS, is similar to that of BrS. Enhancing inward calcium current with β-adrenergic activation using isoproterenol is effective in suppressing ER arrhythmias in acute cases. Inhibiting cardiac transient outward potassium current (*Ito*) using quinidine is also effective to suppress ER arrhythmias and was of proven superiority in chronic cases. A combination of cilostazol and bepridil was found to suppress VF in ERS and BrS effectively. In idiopathic VF (IVF) secondary prevention; immediate ICD implantation is a must. Acute suppression of the VF can be achieved successfully with isoproterenol or quinidine. Verapamil was found to be also successful acutely. Ablation of the triggering PVC seems to be a very promising choice. For PCCD and SND with channelopathy elements, ICD with pacemaker capabilities is the standard choice. Toward the discovery of putative mutations and genes comes *variants of unknown significance (VUS)* (looking for functional significance of allele or gene variant). The delta T50 (a measure of the variability of ventricular repolarization at 50% of the T-wave downslope) is a new tool to improve our diagnostic accuracy of channelopathies. In the absence of identifiable point mutation*, copy number variation (CNV)* is a form of genetic abnormality, which may explain the genetic basis of channelopathies. The psychophysiological well-being associated with positive emotional state orchestrates the sympathovagal tone favorably. Cardiac coherence (CC) (where heart frequencies dominate in the range of 0.04–0.26 Hz) is a state of recruiting positive emotion by special training resulting in homogeneity between all body functions and systems. It is seen as sine wave appearance of HRV. Training ICD patients to self-regulate emotions with cardiac coherence can increase selfregulatory capacity, making them less vulnerable to depletions and fear of and consequently less rhythmic events. In view of the complexity of the different channelopathies and its variable responses to different treatment modalities, it is believed that comprehensive global perspective is highly needed. We adopted new universal perspective for diseases management extending from genes to galaxies. Experimental research on channelopathies is a model in this direction. Induced pluripotent stem cell–derived cardiomyocytes provide a new platform for studying arrhythmic disorders leading to sudden cardiac death. Cellular transfection models are able to mimic the expression of a single-ion channel. Both are amenable for the weak electromagnetic currents that are common between genes and cosmos. The rise of Schumann resonance power, solar radio flux and cosmic rays are all reflected in the increase of parasympathetic tone and HRV. The degree of effect of those energetic environmental stressors on human cardiovascular system affects different people differently depending on their health, sensitivity and self-regulation capabilities. The protective effect of simulated weak magnetic field in the range of the first Schumann resonance (7.8 Hz) is proven at cellular level. The future in this direction is promising to revolutionize our interventional capabilities to treat and

*DOI: http://dx.doi.org/10.5772/intechopen.92131*

#### *Inherited Ventricular Arrhythmias, the Channelopathies and SCD: Current Knowledge… DOI: http://dx.doi.org/10.5772/intechopen.92131*

never rely on exercise test result for PCVT management. Flecainide (150–200 mg a day) is a promising first-line drug or as adjunct with beta blockers. Surgical option for PCVT represented by LCSD is an effective choice as a hybrid therapy to pharmaceutical agents. ICD, with primary termination, is the golden choice. Due to the role of emotional upset to induce attacks of VT/VF in PCVT, emotional management is of paramount importance. ER pattern was proven to be truly arrhythmogenic in 2008. Pathophysiological mechanism, as well as therapeutic approaches of ERS, is similar to that of BrS. Enhancing inward calcium current with β-adrenergic activation using isoproterenol is effective in suppressing ER arrhythmias in acute cases. Inhibiting cardiac transient outward potassium current (*Ito*) using quinidine is also effective to suppress ER arrhythmias and was of proven superiority in chronic cases. A combination of cilostazol and bepridil was found to suppress VF in ERS and BrS effectively. In idiopathic VF (IVF) secondary prevention; immediate ICD implantation is a must. Acute suppression of the VF can be achieved successfully with isoproterenol or quinidine. Verapamil was found to be also successful acutely. Ablation of the triggering PVC seems to be a very promising choice. For PCCD and SND with channelopathy elements, ICD with pacemaker capabilities is the standard choice. Toward the discovery of putative mutations and genes comes *variants of unknown significance (VUS)* (looking for functional significance of allele or gene variant). The delta T50 (a measure of the variability of ventricular repolarization at 50% of the T-wave downslope) is a new tool to improve our diagnostic accuracy of channelopathies. In the absence of identifiable point mutation*, copy number variation (CNV)* is a form of genetic abnormality, which may explain the genetic basis of channelopathies. The psychophysiological well-being associated with positive emotional state orchestrates the sympathovagal tone favorably. Cardiac coherence (CC) (where heart frequencies dominate in the range of 0.04–0.26 Hz) is a state of recruiting positive emotion by special training resulting in homogeneity between all body functions and systems. It is seen as sine wave appearance of HRV. Training ICD patients to self-regulate emotions with cardiac coherence can increase selfregulatory capacity, making them less vulnerable to depletions and fear of and consequently less rhythmic events. In view of the complexity of the different channelopathies and its variable responses to different treatment modalities, it is believed that comprehensive global perspective is highly needed. We adopted new universal perspective for diseases management extending from genes to galaxies. Experimental research on channelopathies is a model in this direction. Induced pluripotent stem cell–derived cardiomyocytes provide a new platform for studying arrhythmic disorders leading to sudden cardiac death. Cellular transfection models are able to mimic the expression of a single-ion channel. Both are amenable for the weak electromagnetic currents that are common between genes and cosmos. The rise of Schumann resonance power, solar radio flux and cosmic rays are all reflected in the increase of parasympathetic tone and HRV. The degree of effect of those energetic environmental stressors on human cardiovascular system affects different people differently depending on their health, sensitivity and self-regulation capabilities. The protective effect of simulated weak magnetic field in the range of the first Schumann resonance (7.8 Hz) is proven at cellular level. The future in this direction is promising to revolutionize our interventional capabilities to treat and prevent channelopathies in the human kind.
