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         LWBK340-c27_ pp638-654.qxd  6/30/09  12:01 AM  Page 641 Aptara Inc.
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                                                              C HAPTER 2 7 / Sudden Cardiac Death and Cardiac Arrest  641
                   ventricular arrhythmias that develop during physical activity in  blocking agents can be helpful in treating this condition. The first
                   the presence of a normal resting ECG. 20,23         episode often occurs during childhood. The arrhythmia is charac-
                                                                       terized by bidirectional and polymorphic VT. The condition is
                   Congenital Long QT Syndrome                         rare, and is related to a genetic disorder. CPVT is caused by mu-
                   Patients with the classic long QT intervals corrected for heart rate  tations involving the cardiac ryanodine receptor, and calse-
                                                                                                  20,23,31
                   (QTc interval) are at increased risk for torsades. 20,23,24  Congeni-  questrin, a calcium-buffering protein.
                   tal long QT is hereditary; mutations in eight genes have been
                   identified.  The most common mutations are LQT1 (42%),
                   LQT2 (45%), and LQT3 (8%). Most mutations affect the cardiac  MANAGEMENT OF SCA
                   potassium-channel genes KCNQ1 and KCNH2 and cause the
                   most frequent forms of long QT. An impaired sodium channel  The outcome of cardiac arrest is determined by how promptly
                   gene, SCN5A, is the cause for LQT3. However, the mutation  treatment is initiated with advanced cardiac life support (ACLS).
                   LQT4 has been identified as a mutation from a membrane adap-  To improve outcome from SCA, the following must occur as rap-
                   tor protein, and not an ion channel. 12,23  QT interval duration was  idly as possible: (1) early recognition of warning signs, (2) early
                   identified as the strongest predictor of SCD, even before genetic  activation of the emergency medical system, (3) early basic car-
                   mutation identification. A QTc greater than 500 ms in a patient  diopulmonary resuscitation (CPR), (4) early defibrillation, and
                   identified with an affected gene carries the highest risk of syncope  (5) early ACLS. These events have been described as “links in a
                   or SCD by the age of 40. 20  Two patterns of long QT have been  chain of survival,” because they are all connected and indispensa-
                   reported: the Romano Ward syndrome, which is an autosomal  ble to the overall success of emergency cardiac care. 32,33
                   dominant syndrome, and the Jervell and Lange-Nielsen syn-  Although this section summarizes ACLS recommendations for
                   drome. The latter is autosomal recessive, more severe, and often  the adult patient, it is not a complete reference. For each cardiac
                   associated with congenital deafness. 20,25          nurse, participation in an ACLS provider course by the American
                     In individuals with long QT, ventricular arrhythmias often oc-  Heart Association (AHA) is strongly recommended. In addition,
                   cur in the setting of stress and activity, but also can occur during  the most current version of Emergency Cardiac Care, Basic Life
                   rest and sleep. Exercise, particularly swimming, is often a trigger  Support for Healthcare Providers, and The Textbook of Advanced
                   for arrhythmias in the LQT1 patients. LQT2 patients have ar-  Cardiac Life Support should be used as definitive references.
                   rhythmias that are triggered during both rest and emotion, often
                   associated with acoustic stimuli. The LQT3 patients are more
                   prone to having arrhythmias at rest and during sleep. 20,26  Avoid-  Adult Advanced Cardiac Life Support
                   ance of competitive sports is recommended for LQT1 and LQT2  ACLS teaches the appropriate skills and knowledge, as deter-
                   patients, but not for LQT3 patients. 22
                                                                       mined by leaders in emergency cardiovascular care (ECC), to im-
                                                                       prove survival from SCA and acute life-threatening cardiopul-
                   Brugada Syndrome                                    monary events. ACLS includes early recognition of prearrest,
                   Brugada syndrome is an inherited disease that is associated with a  basic life support, the use of airway and circulation adjuncts, car-
                   high incidence of sudden death. A mutation of cardiac sodium-  diac monitoring, and defibrillation and other arrhythmia control
                   channel gene SCN5A, has been identified in about 20% of per-  techniques. ACLS also includes establishment of intravenous ac-
                   sons with the syndrome, and occurs more often in men. The Bru-  cess, drug therapy, and postresuscitation care. This section focuses
                   gada syndrome is confirmed with ST-segment elevation in the  on defibrillation and ACLS management of pulseless cardiac ar-
                   precordial leads, right bundle-branch block (RBBB) conduction  rest. Postresuscitation management of SCA survivors is also in-
                   pattern, and history of SCD. The ECG changes may be present  cluded. For discussions of basic and complex arrhythmias, con-
                   all the time or elicited when antiarrhythmic drugs are given that  duction disturbances, electrophysiology studies, hemodynamic
                   block sodium channels (Chapter 15). 20,27,28  Fever has been re-  monitoring, acute coronary syndrome, pacemakers, and im-
                   ported to unmask ECG changes of Brugada syndrome and elicit  plantable defibrillators, respectively, refer to Chapters 15, 16, 18,
                   an electrical VT storm. 29  Globally, the syndrome is more preva-  21, 22, and 28, respectively.
                   lent in areas of southeast Asia. 23                   In 2005, the AHA updated the  guidelines for CPR and
                                                                           32
                                                                       ECC. The guidelines are based on the 2005 International Con-
                   Wolff–Parkinson–White Syndrome                      sensus Conference on Cardiopulmonary Resuscitation and Emer-
                   Wolff–Parkinson–White (WPW) is associated with an accessory  gency Cardiovascular Care Science with Treatment Recommenda-
                   pathway that allows for conduction between the atria and ventri-  tions. The classes of recommendation for ACLS management are
                   cles. Normally, WPW is associated with nonlethal arrhythmias.  based on evidence evaluation (Display 27-3).
                   However, if atrial fibrillation develops and conduction is rapid
                   over the accessory pathway, the ventricular rate can become so fast  Electrical Therapy of Malignant
                   that the rhythm degenerates into VF. SCD in the setting of WPW  Arrhythmias
                   is quite low, and has been reported at 0.39% per year. 23,30  Defibrillation and cardioversion are a delivery of electrical energy
                                                                       that totally depolarizes and stuns the myocardium, which allows
                   Catecholaminergic Polymorphic                       the sinus node to resume its function as the pacemaker for the
                   Ventricular Tachycardia                             heart. Defibrillation is, by definition, the therapy for VF. Car-
                   CVPT is a catecholamine-dependent arrhythmia that occurs in  dioversion, which is a synchronized shock, is the electrical therapy
                   the absence of a long QT interval. The arrhythmia often occurs dur-  for all other tachyarrhythmias. Transcutaneous and transvenous
                   ing physical activity or emotional distress; therefore,  -adrenoceptor-  pacing are additional types of electrical therapy used in ACLS
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