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Heart disease and its associations in children (and adults) Dr. Andrew Sands Cardiology Department RBHSC 08/10/21Heart Disease in Children  CONGENITAL (almost 1% of births) and acquired  Cyanotic and acyanotic  Presentation (symptoms and signs)  Heart rhythm problems  Management of heart disease  Associated concernsCongenital Heart Disease Acyanotic Cyanotic • TGA • VSD • ASD • Tetralogy of Fallot • TAPVD • PDA • PS • Truncus Arteriosus• AS • Tricuspid Atresia • Coarctation • HLHS3 Day Old Infant Presents with Pallor, Poor Feeding and Respiratory Distress  Typical presentation of coarctation. Typically day 2-4 of life as duct closesDuct dependent left heart obstruction • Commence prostaglandin early • dobutamine) circulation with inotropes if necessary (eg • In HLHS - maintain mild respiratory acidosis • Early palliation/correctionNewborn Infant presents to primary care and is thought to be “dusky” • Initial action? • Differentials? • How to tell between cardiac and lung disease? • Further management? • Definitive diagnosis and therapy?4 Year Old Boy  Systolic murmur heard when he presents with tonsillitis  Differentials?  Anything else you want to know?  Investigation?  Advice for parents?Innocent Murmurs • Systolic only (note venous hum) • Grade 3 or less • No clicks and normal heart sounds • Alter with position • Generally well children (may be pyrexial) • Normal investigationsHeart rate/rhythm  Too fast (especially SVT) – typically 220-300/min  Too slow (SAN/AVN problems)  JUST RIGHTCongenital Heart Disease Acyanotic Cyanotic • TGA • VSD • ASD • Tetralogy of Fallot • TAPVD • PDA • PS • Truncus Arteriosus • AS • Tricuspid Atresia • Coarctation • HLHSSymptoms/signs and treatment of infants with VSD  Small VSDs are well tolerated  Larger VSDs cause dyspnoea, poor feeding, slow weight gain  Signs – tachypnoea, tachycardia and hepatic enlargenment.  Medications such as diuretics, ACEI can help. Nutrition is vital. Surgical/catheter repairCongenital Versus Acquired • Kawasaki disease • Structural CHD • Congenital rhythm and other arteritis disease • Rheumatic heart disease • Congenital muscle disease • Myocarditis/endo carditis • Miscellaneous • MiscellaneousCongenital Heart Disease – not just a paediatric condition • ASD • Coarctation of aorta • Valvar lesionsThanks Dr. But……. Can he/she play football?Miscellaneous Concerns  Syncope and pre-syncope  Palpitations  Blue lips  Dyspnoea  Screening  Chest painChest Pain IN CHILDREN/ YOUNG ADULTSChest pain to most people means- heart disease. The true incidence in the young is 75% throughout childhoodoccur in 20- The most common identifiable cause in children is costochondritis (20-30%) 20-45% are idiopathic “Cardiac causes” account for <5% In one study 1/200 had a tenuous link to the heart (MVP).Causes of Chest Pain 1 Thoracic cage Abdominal  Costochondritis  Oesophagitis/gastritis  Muscle strain/trauma  Mallory-Weiss tear  Mastalgia  Cholecystitis  Skeletal abnormality  Bornholm/Zoster Non organic/psychogenic  Especially if a family Respiratory  Cough history of angina or chronic pain  Asthma  Effusion  Infection  PneumothoraxCauses of Chest Pain 2 Cardiac Structural lesions  AS/PS/HOCM  Pulmonary vascular obstructive disease  MVP ?  Anomalous coronary Inflammatory  Pericarditis  Kawasaki  Others Arrhythmia Diagnostic approach Thorough history and exam .  Often no investigation Summary  ECG  Chest pain is common in the  CXR young  Cardiac chest pain is  ECHO exceptionally rare in the young  Holter etc.  A good history and exam will  EST often be enough  Thallium  Parental anxiety is often  Angiography prominentSudden Cardiac Death (in the young)  Congenital heart disease  Hypertrophic cardiomyopathy  Other cardiomyopathy  Coronary disease, congenital/acquired  Channelopathies (LQT, Brugada, CPVT)  ARVC  WPW  Bradycardias  Pulmonary hypertension  OthersIncidence of SCD in the “young”  1 in 100,000-300,000 of high school age athletes/year  1 in 15,000-18,000 of older athletes/year Males more affected than females (2-5 times more likely to die) Sudexercise induced (89%)in athletes is mainly In non-athletes only 9% of sudden, unexpected deaths occur during exercise,Causes of Sudden death (leisure sports) – in general < 30 years >30 years  HCM - around 30%  Coronary disease 50%  ARVC – around 20%  HCM 20%  Coronary disease 15%  Dilated CM 11%  Others including channelopathy, LVHSudden Cardiac Death in Young Athletes  HCM 26.4%  Commotio cordis 20%  Coronary anomaly 14%  LVH 8%  Myocarditis 5%  Channelopathies, AS, asthma, heat stroke, drugs < 3% each  Adapted from Maron: N Engl Journal Med. 2003Sudden Death in Athletes and Non-athletes (<35) in Veneto, Italy Athletes Non-athletes  ARVC  Atherosclerotic coronary disease  Atherosclerotic coronary disease  Mitral valve prolapse  Anomalous origin of coronary Disease of conduction system  Disease of conduction system Myocarditis  Mitral valve prolapse  ARVC  HCM  HCM  Myocarditis  Dissecting aortic aneurysm  Myocardial bridge  Others account for almost 30% Adapted from Corrado etal. N Eng J Med 1998What about N. Ireland?  Limited data suggest incidence of SCD in 1-35 years of 1.4/100,000/year  Limited population  Media aware  Small population with relatively large number of related individualsCardiomyopathy  Mainly hypertrophic (up to 1 in 500 prevalence)  May be dominantly inherited  Very variable condition  Note other causes of LVH eg Fabry’s disease, Friedrich’s etc. LVH with no cause  What about “athletic hearts” Others include dilated cardiomyopathy/myocarditis/restrictive CMCoronary anomalies  Atherosclerosis is common in older patients  Kawasaki disease, Williams’ syndrome, Takyasu disease  Congenital (0.3% autopsy) Anomalous L or RCA from contralateral sinus especially LCA from right passing between the aorta and pulmonary trunk – causing ischaemia and arrhythmia with exercise  Others Adapted from Taylor et al JACC 1992Channelopathies  LQT  Brugada  CPVT  Short QT  Idiopathic VF  Others such as short coupled VTLong QT (congenital/drug induced)  Up to 1/500  Role of gene testing – 10 plus genes – KCNQ1, KCNH2, SCN5A  May cause polymorphic ventricular tachycardia, presenting with collapse  Before gene testing around 40%presented with SCDLQTCPVT  Very rare, but may be familial - at least one NI family identified  40 – 50% mortality by 30 years  Up to 25% mortality even on beta blockers  RyR2 gene increasingly identified, may also be other genes involvedProblems with screening for SCD  Who to screen and when?  How to screen?  How often?  When to stop?Problems with screening 2  40% of athletes have abnormal ecg (Maron: Circ 2006)  30-50% have IRBBB, 10-80% have LVH, 40% have Wenkebach on Holter (Foote et al)  Some sports seem to be more hazardous than others eg endurance cycling. Some studies suggest long-term damage to myocardium of RV (Heidbuchel EHJ 2003)  Screening would cost $3.7M per life saved according to AHA Screening for SCD needs to be focused – usually based on history +/- symptomsSyncope  Syncope may affect 3-4% of the general population and  15% of 8-18 y/o will have at least one syncopal episode.  More common in females than males.  It tends to be recurrent and its exact cause is not found in up to 40%.Key Points  History is crucial  In particular ask about diurnal variation, meals, activities, associated symptoms.  The eye witness account.  Family history.  Access to prescribed/illicit drugs.Beware  Syncope with loud noise/fright  Syncope with exercise  Syncope while supine  Syncope with abnormal movements  Bad family history  “Odd” historyDifferential Diagnosis  Neurally mediated  Other vagal  Hypoglycaemia  Neuropsychiatric  Cardiac syncopeNeurally Mediated Syncope • Reflex syncope (neurocardiogenic) • Postural orthostatic tachycardia syndrome (POTS) • Pure autonomic failure • Multiple system atrophy. This group of conditions can occur at any age but are most common in toddlers and adolescents. Common reflex syncope occurs when blood pools, emptying heart. Heart contracts very vigorously. “C” fibres are stimulated which the brain interprets as hypertension causing decreased sympathetic tone. Tonic seizure may follow. A variant is cerebral vasoconstrictive syncope.Investigation of Syncope • ECG: Probably the most important investigation. Look at QT, pre-excitation, heart block and ventricular hypertrophy • EST: Important especially if symptoms associated with exercise or LQT suspected • Holter: Usually done but often unproductive. Even event monitors give limited yield. • ECHO: Often done usually normal unless signs suggest otherwise • EEG/CT/MRI: Usually unhelpful but often done. Will be part of the assessment where there is definite seizure activity or the history suggests possible water immersion induced symptoms etc. • Blood investigations: Usually of no use unless for example, significant hypoglycaemia is suspected • Tilt testingTherapeutic Options • Certain conditions require specific treatment . For the majority- • Education and reassurance • Adequate hydration • Increase salt intake • Fludrocortisone. Some suggest should be first line for reflex syncope/POTS and other dysautonomia • Midodrine A direct alpha agonist • B blocker Aims to blunt adrenergic stimuli of the Bezold-Jarish reflex • SSRIs Down regulate post synaptic 5-HT receptors therefore reducing the serotonin effect on sympathetic activity. • Postural training and exerciseEvaluation of palpitations  History all important  ? Exercise / swimming / anxiety  Onset / cessation & duration  Associated symptoms: Presyncope / syncope +/- SOB / chest pain  Medications: B2 agonists / decongestants / ADHD  Caffeine: coffee / tea / drinks / chocolate  FMH: Sudden death / CMP/ LQTS  Examination  Full cardiac exam - special attention to pulse  Look for signs of other cause: anaemia, hyperthyroid etc  12 lead ECGCauses Cardiac  Isolated:  Fever  PACs & PVCs  Anxiety  Arrhythmia  Hyperventilation / Panic attack  Exercise  SVT  VT  Anaemia  VF  Hyperthyroidism  AV block  Phaeochromocytoma  Sinus arrest  Muscle spasmWhat we tend to do  Full Hx & examination  12 lead ECG  +/- 24 hour tape  Occasionally EST (if indicated by history)  If documented arrhythmia – Echo  We will treat if we diagnose an arrhythmia or the patient has LQTS / cardiomyopathy -not solely on the basis of palpitations.But – Can he play football doctor?A Suggested Approach  Take a good history  Chest pain is rarely cardiac in young people  Palpitations are often benign but SVT is fairly common (1/250 healthy children)  Syncope is usually entirely benign but beware odd history and worrying family history  Don’t place limitations on exercise unless significant likelihood of major pathology