Guidelines Pediatric Congenital Heart Disease SYNCOPE

Guidelines Pediatric Congenital Heart Disease SYNCOPE www.kinderkardiologie.org/dgpkleitlinien.shtm

Definition and Characteristics of Syncope temporary loss of consciousness and tonicity due to inadequate global cerebral perfusion realtively sudden short lasting self-terminating rapid recovery an aura may be present sudden loss of tonicity may cause injury symmetric or asymmetric subultus may occur syncope may be rare and singular or happen frequently in some individuals

Observed Frequency of Syncope ~15% of all children and adolescents receive at least one syncope >75% benign reflex syncope typical age: 12-19 years f > m <10 years breath holding spells typical age: 6 months- 5 years 2-5% of all children cardiac syncope

loss of consciouness <-> syncope Syncope is only one of many conditions that cause transient loss of consciousness cerebral seizure neurological disease psychogenic pseudo-syncope metabolical disease Pregnancy

Clinical Classification of Syncope in Children and Adolescents 1. reflex (neurally mediated) syncope breath holding spells 1. cyanotic 2. pallid adolsecents 1. vasodepressant 2. heart frequency inhibitory 3. mixed forms 4. postural tachycardia 2. orthostatic hypotension 3. cardiac syncope 1. arrhythmias 2. congenital heart defect

benign syncope <-> malign syncope Basic Diagnostics case history, medical history (congenital heart disease is a main risk factor for a cardiac syncope/sudden cardiac death) physical examination ECG blood pressure measurement (orthostatism [Schellong-] test) has the loss of consciousness been a syncope? is there adequate evidence to clear the cause? is the patient at risk for cardiac syncope?

benign syncope <-> malign syncope

Checklist Basic Diagnostics

Advanced Diagostics for Syncope Echocardiography Advanced ECG-monitoring Tilt table test Ergometry Coronary angiography (catheterization, MRI, CT-scan) EEG Cerebral MRI- or CT-scan

Advanced Diagostics for Syncope Evidence for cerebral seizure Echocardiography Advanced ECG-monitoring Tilt table test Ergometry Coronary angiography (catheterization, MRI, CT-scan) EEG Cerebral MRI- or CT-scan

Advanced Diagostics for Syncope not required if basic diagnostics provide clear evidence for preceding reflex syncope and no risk factors are present is recommended if basic diagnostics leave the cause of syncope unclear or if cardiac syncope is suspected cardiac syncope should be supposed in the presence of outflow tract obstruction, pericardial tamponade, pulmonary arterial hypertension, coronary anomalies, after cardiac surgery, after Kawasaki syndrom Echocardiography Advanced ECG-monitoring Tilt table test Ergometry Coronary angiography (catheterization, MRI, CT-scan)

Advanced Diagostics for Syncope 24h-Holter-ECG: on suspicion of arrhythmia if relapse of syncope is expected within days External event recorder: in patients with good compliance and frequent relapses Implantable loop recorder: betimes in serious cases Ajmalintest: http://www.brugadadrugs.org EPI: in patients with cardiac syncope rarely indicated in special cases (e.g. with cardiomyopathy) Echocardiography Advanced ECG-monitoring Tilt table test Ergometry Coronary angiography (catheterization, MRI, CT-scan)

Advanced Diagostics for Syncope Extensive, costly and patient wearing examination In individual cases if suspicion of reflex syncope or orthostatic hypotension is ambiguous If the patient is in a high risk situation for syncope (risk of injury, education, business) may be helpful to demonstrate a patient and/or his enviroment sensitivity for reflex syncope Echocardiography Advanced ECG-monitoring Tilt table test Ergometry available for children >10 years of age for adequate test protocol look at the DGPK website (AG Belastungsuntersuchung) Coronary angiography (catheterization, MRI, CT-scan)

Advanced Diagostics for Syncope after exertional syncope (suspicion of cardiac cause) non-invasive coronary ischemia diagnostic e.g. after Kawasaki syndrom Echocardiography Advanced ECG-monitoring Tilt table test Ergometry Coronary angiography (catheterization, MRI, CT-scan)

Advanced Diagostics for Syncope after exertional syncope (suspicion of cardiac cause) Non-invasive coronary ischemia diagnostic e.g. after Kawasaki syndrom anamnestic and clinical evidence for the presence of coronary disease/-anomaly Echocardiography Advanced ECG-monitoring Tilt table test Ergometry Coronary angiography (catheterization, MRI, CT-scan)

Prognosis of Reflex Syncope anticipation of freedom of symptoms in the long-term perspective is excellent anticipation of relapse after first reflex-syncope is realistic

Therapy of Reflex Syncope Reassurance and Education benign affection good prognosis for permanent freedom of syncope relapse does not deteriorate good prognosis avoidance of trigger situations rescue manoevers work out to strength the circulatory system drinking / good hydration enough sleep sleep in elevated position for the upper body (renin-angiotensin-aldosteron axis) resign heavy meals

Rescue Manoevers change of body position (to be seated or lay down) activation of leg muscle pump (venous drainage) isometric hands draft (acitivation of sympathetic nervous system) Rev Esp Cardiol. 2012;65:75

Drugs for Prevention of Relapses only in exceptional cases temporary limited no evidence for clear positive recommendations negative recommendations: beta-blockers, dihydroergotamin, etilefrin under consideration: alpha-agonists (e.g. midodrin) [side effect arterial hypertension], mineralo-corticosteroids, serotonin uptake-blocker [psychogenic side effects] for recurrent pallid breath holding spells, iron substitution for recurrent cyanotic breath holding spells

Pacemaker for Prevention of Cardioinibitory Syncope vascular component of reflex syncope may not be adressed recurrent syncope with PM is possible no direct vital compromise good prognosis in children and adolescents in exceptional cases of recurrent paalid breath holding spells with extended asystoly