Pediatric Cardiac Emergencies Gavin Greenfield Peggy Thomsen 4 year old female presents with fast breathing, grunting, cough x 5 days seen 2 days earlier and started on steroids and bronchodilators initial vitals: HR 150, BP 100/85, RR 36, T 37.5
1 month old with irritability, poor feeding (fatigues), failure to thrive, fast breathing no fever or runny nose physical exam: HR 160, RR 60 with minimal respiratory distress, gallop rhythm, rales Infant Cardiac Disease Leading to ER Presentation Congenital
Acquired Cardiomyopathy Myocarditis (usually with CHF) Dysrhythmias Congestive Heart Failure the physiologic state in which cardiac output is unable to meet tissue metabolic demands (Rosen) CO = HR x SV
SV dependent upon preload, afterload, contractility CHF - Presentation infants: irritable, poor feeding (early fatigue), failure to thrive, respiratory symptoms always consider in patients with respiratory symptoms often misdiagnosed as respiratory illness / infection
CHF - Etiology Increased Preload L to R shunts (VSD, PDA, AV fistula) severe anemia Increased Afterload
HTN Congenital (aortic stenosis, coarctation of aorta) Decreased Contractility
Dysrhythmia CHF - Etiology presents immediately at birth anemia, acidosis, hypoxia, hypoglycemia, hypocalcemia, sepsis presents at 1 day (congenital) PDA in premature infants presents in first month (congenital)
HPLV, aortic stenosis, coarctation, VSD presents later presents later (acquired) myocarditis, cardiomyopathy (dilated or hypertrophic), SVT, severe anemia, rheumatic fever Myocarditis leading cause of dilated cardiomyopathy and one of the most common causes of CHF in children etiology: idiopathic, viral, bacterial, parasitic
hallmark is CHF failure to respond to bronchodilators in wheezing child treatment includes inotropes, afterload reduction, diuretics, antibiotics, antivirals Pericarditis sharp stabbing precordial pain worse with supine and better leaning forward no sensory innervation of the pericardium
pain referred from diaphragmatic and pleural irritation Etiology infectious
Pericarditis usually a benign course virulent bacteria (H. flu, E. coli) can cause constrictive pericarditis and subsequent tamponade may need urgent pericardiocentesis uncomplicated pericarditis usually responds to rest and anti-inflammatories Chest Pain 4% of children will have a cardiac origin
remainder: MSK, pulmonic (asthma, bronchitis, pneumonia), GI Cardiac causes: myocarditis, pericarditis, structural abnormalities such as congenital heart disease or hypertrophic cardiomyopathy 14 year old male collapses at school while in class non-responsive for one minute feels fine in the department
Approach? Syncope 20-50% of adolescents experience at least one episode of syncope most cases benign Pathophysiology vascular orthostatic, hypovolemia neurally mediated
hypoxia: PE, CNS depression from OD, CO cardiac Cardiac Syncope Dysrhythmias tachy brady Outflow obstruction Myocardial Dysfunction cardiac syncope often precedes future
sudden cardiac death Sudden Cardiac Death includes those causes that directly relate to cardiovascular dysfunction one third of all sudden deaths Sudden Cardiac Death Etiology
myocarditis cardiomyopathy (hypertrophic)
cyanotic and noncyanotic congenital heart disease valvular heart disease congenital complete heart block WPW long QT syndrome Marfan syndrome coronary artery disease anomalous coronary arteries Risk Factors for Serious Cause of Syncope
history of cardiac disease in patient FH of sudden death, cardiac disease, or deafness recurrent episodes recumbent episode exertional prolonged loss of consciousness associated chest pain or palpitations medications that can alter cardiac conduction What to look for in the Department:
EKG Long QT syndrome congenital or acquired get paroxysmal v tach with torsades de pointes congenital long QT associated with hypertrophic cardiomyopathy long QT defined as corrected QT longer than 0.44 s T wave alternans sometimes present can have normal ECG in the department two clinical syndromes not associated with structural heart disease: Romano-Ward and Jervell-LangeNielsen
Other dysrhythmias WPW and other SVTs AV block usually acquired, rarely congenital Sick sinus syndrome Idiopathic Hypertrophic Cardiomyopathy aka IHSS
both a fixed and dynamic subvalvular obstruction characterized by ventricular hypertrophy with principle involvement of the ventricular septum associated with long QT autosomal dominant often presents with exertional syncope 10 year mortality is 50% for children diagnosed by age 14 Other structural cardiac diseases
dilated cardiomyopathy usually secondary to myocarditis syncope and death secondary to ventricular dysrhythmias or severe myocardial dysfunction arrhythmogenic RV dysplasia congenital cyanotic and non-cyanotic heart disease valvular diseases aortic stenosis coronary artery anomalies
exertional syncope or sudden death aberrant artery passes between aorta and pulmonary artery 2 week old infant brought in by parents with difficulty breathing HR 180, BP 50/P, RR 80, T 37.5 history and physical investigations repeat vitals: HR 30, no BP, RR 12 definitive treatment:
4 year old male presents with 2 weeks history of cough, fast breathing, fatigue, decreased exercise tolerance, puffy eyes On exam: tachypneic, moderate respiratory distress, O2 sats 92%, bilateral crackles 6 month male presents with failure to thrive, fast breathing, blue lips On exam tachypnea but no respiratory distress, lips and extremities blue, oxygen
saturations 70% Congenital Heart Disease Fetal to Neonatal Circulation Anatomic Classification; 4 groups Right to Left Shunt Tetralogy of Fallot Transposition of
the Great Arteries Tricuspid Atresia Left to Right Shunt ASD VSD PDA Stenotic Aortic valve stenosis
Pulmonic valve stenosis Aortic coarctation Mixing Truncus Total Anomalous Pulmonary Venous Return Hypoplastic left heart syndrome
CHD Classified as Cyanotic vs. Acyanotic Cyanotic (R to L shunt and mixing lesions) tetralogy of Fallot transposition of great vessels tricuspid atresia total anomalous pulmonary venous return truncus arteriosus hypoplastic left heart syndrome
CHD Classified as Cyanotic vs. Acyanotic Acyanotic (L to R shunts, stenotic lesions) ASD VSD PDA aortic valve stenosis pulmonic valve stenosis aortic coarctation
Cyanosis Classified as central or peripheral Central cyanosis (always abnormal) mucous membranes, trunk, extremities classified as cardiac (R to L shunt) or pulmonary Peripheral cyanosis (acrocyanosis)
no involvement of mucous membranes involves hands, feet, circumoral area common in neonates from vasomotor instability CHF, PVD, shock, cold extremities Congenital Heart Disease History feeding difficulties tachypnea diaphoresis
syncope cyanotic episodes failure to thrive Congenital Heart Disease Physical Examination colour: pink, blue, gray vitals: tachypnea, tachycardia, BP symptoms suggestive of infection palpation and auscultation of precordium chest auscultation
survey for organomegaly pulses in all extremities Cyanotic Congenital Heart Disease R to L shunts mixing lesions Tetralogy of Fallot the classic cyanotic lesion RV outflow
obstruction RVH VSD overriding aorta CXR reveals boot shaped heart with decreased pulmonary blood flow 2 month old female with known tetralogy of Fallot brought in with 24 hour history of
vomiting and diarrhea On exam: moderate dehydration during IV attempts patient becomes irritable and cyanotic Treatment of Tet Spell quiet, calm environment knee-chest or squatting position increases afterload thus decreasing R to L shunting Oxygen
Morphine to treat hyperpnea and decrease systemic catecholamines Phenylephrine increases afterload thereby decreasing R to L shunt Manual external aortic compression below level of renal arteries Propranolol to block beta receptors in infundibulum therefore lessening RV outflow obstruction
Consider consider small volume challenge (5-10 cc/ kg) to increase preload and reduce dynamic outflow obstruction ?NaHCO3 for correction of acidosis may need general anesthesia if severe and/or prolonged spell interim prophylactic treatment with propranolol while awaiting surgery
Acyanotic Congenital Heart Disease L to R shunts stenotic lesions VSD Most common congenital lesion Large VSDs may be silent and become symptomatic in first few weeks as pulmonary resistance SOB and diaphoresis w feeds
Poor weight gain Systolic murmur CXR demonstrates CHF 2 week old infant brought in by parents with difficulty breathing HR 180, BP 50/P, RR 80, T 37.5 history and physical investigations repeat vitals: HR 30, no BP, RR 12 definitive treatment:
Differential Dx of Infant Shock infection (septic shock/ meningitis) bacterial: GBS, E. coli, S. aureus virus: enteroviruses, H. simplex metabolic: amino/organic acidopathies, urea cycle defect hypoxic shock: eg. RSV, C.N.S. depression heart disease: congenital or acquired
LV Outflow Obstruction LV Outflow Obstruction Aortic coarctation Hypoplastic left heart syndrome Aortic stenosis (presents later) Rosen: any neonate in shock that does not respond to fluids or pressors has LV outflow obstruction until proven otherwise
complete obstruction incompatible with life unless there is shunting Coarctation of the aorta Most often distal to L subclavian Can be diagnosed anytime Neonates present as acutely ill, gray shocky (from DA closure) Systolic murmur at the back Hepatomegaly Diminished femoral pulses
BP difference b/t arms and legs CXR demonstrates CHF Treatment of CHF Prostaglandin E1 Duct Dependant Lesions Duct needed to perfuse lungs or periphery Lungs Tetralogy of Fallot, transposition of great arteries, tricuspid or pulmonary atresia a patent ductus arteriosus results in preserved
pulmonary blood flow Periphery Aortic coarctation (severe) and Hypoplastic left heart Treatment of acute decline in patients with ductal dependant lesions Open the closed duct
Prostaglandin E1 0.1 ug/kg/min infusion reduce dosage as perfusion and colour return Rosen: any infant in the first week of life with decreased perfusion, hypotension, or acidosis should be considered a candidate for PGE1 administration What do you need to know about PGE ? it functions by dilating vascular smooth
muscle, both systemically and in the pulmonary vascular bed its use in CHD pts is to maintain patency of the PDA, whether to maintain PBF or to maintain systemic blood flow past a Classification Review pink child in respiratory distress suggests acyanotic chd (L to R shunt, coarct, aortic stenosis) blue cyanotic child in little respiratory
distress suggests R to L shunt or mixing lesions gray, shocky baby suggests outflow tract obstruction Bradyarrhythmias Etiology hypoxia, acidosis, hypoglycemia excess vagal stimulation (ex. intubation) Treatment
Epinephrine Atropine if known vagally mediated or heart block Congenital Bradyarrhythmias complete AV block autoimmune injury to fetal conduction system secondary to maternal autoimmune disease atropine, isoproteronol, epinephrine may be tried temporarily prior to pacing
Tachyarrhythmias Supraventricular Tachycardia re-entrant with accessory pathway (AV nodal or WPW) re-entrant without accessory pathway (reentry occurs within sinus node or within atrium) ectopic nonspecific presentations in infants Murmurs
Areas aortic: R 2nd intercostal space pulmonic: L 2nd intercostal space mitral: apex tricuspid and VSD: L lower sternal border Pathologic diastolic, holosystolic, late systolic, continuous Innocent Heart Murmurs History
normal growth and development, normal exercise tolerance no history of cyanosis Physical Examination Grade II or less, localized
varies with position (decreased with upright posture) normal precordium normal pulses Lab normal EKG, normal CXR 3 innocent murmurs Stills short ejection systolic murmur musical or vibratory quality
heard best between apex and left sternal border physiologic pulmonary flow murmur harsh, located at pulmonic area peripheral arterial stenosis low-intensity systolic ejection murmur best heard in axilla and back 8 year old male presents with fever, arthralgias
mother mentions that he had a sore throat 3 weeks ago for a few days with spontaneous resolution a throat swab was done and positive for GAS but patient better so did not take the prescribed antibiotics Acute Rheumatic Fever school aged children associated with certain strains of Group A betahemolytic streptococcal infections the streptococcal organism stimulated antibody
production to host tissues CT of heart, joints, CNS, subcutaneous tissues, skin carditis is an endomyocarditis with valvulitis involving mitral and aortic valves 2 to 6 weeks post streptococcal pharyngitis Jones Criteria Major carditis new or changing
erythema marginatum subcutaneous nodules Minor fever arthralgia history of previous ARF elevated ESR, CRP prolonged PR on EKG Rising titer of
antistreptococcal antibodies ER Treatment management of complicating features of carditis (CHF) significant carditis or CHF managed with glucocorticoids high-dose ASA 75-100 mg/kg/day pencillin
long term management of rheumatic heart disease Pediatric EKGs General Principles RV Dominance at birth; gradually changes to LV dominance axis up to +180 in normal newborn T waves negative in right precordial leads until adolescence (except they are upright in first week of life)
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