Pediatric Cardiac Emergencies

1. Pediatric Cardiac Emergencies Gavin Greenfield Peggy Thomsen

2. 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

3. 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

4. Infant Cardiac Disease Leading to ER Presentation • Congenital • Acquired • Cardiomyopathy • Myocarditis (usually with CHF) • Dysrhythmias

5. 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

6. 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

7. 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 • myocarditis, pericarditis with tamponade • cardiomyopathy (dilated or hypertrophic) • Kawasaki syndrome (early phase) • metabolic: electrolyte, hypothyroid • myocardial contusion • toxins: dig, calcium channel blockers, beta blockers • Dysrhythmia

8. 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

9. 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

10. 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

11. infectious viral bacterial TB fungal parasitic Connective tissue RA Rheumatic fever SLE Metabolic / Endocrine uremia hypothyroid Hematology / Oncology bleeding diathesis malignancy Trauma Iatrogenic Etiology

12. 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

13. 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. 14 year old male collapses at school while in class • non-responsive for one minute • feels fine in the department • Approach?

16. 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

17. Cardiac Syncope • Dysrhythmias • tachy • brady • Outflow obstruction • Myocardial Dysfunction • cardiac syncope often precedes future sudden cardiac death

18. Sudden Cardiac Death • includes those causes that directly relate to cardiovascular dysfunction • one third of all sudden deaths

19. 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

20. 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

21. 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-Lange-Nielsen

22. Other dysrhythmias • WPW and other SVT’s • AV block • usually acquired, rarely congenital • Sick sinus syndrome

23. 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

24. 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

25. 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”:

26. 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

27. 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%

28. Congenital Heart Disease • Fetal to Neonatal Circulation

30. 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 Anatomic Classification; 4 groups

31. 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

32. CHD Classified as Cyanotic vs. Acyanotic • Acyanotic (L to R shunts, stenotic lesions) • ASD • VSD • PDA • aortic valve stenosis • pulmonic valve stenosis • aortic coarctation

33. 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

34. Congenital Heart Disease • History • feeding difficulties • tachypnea • diaphoresis • syncope • cyanotic episodes • failure to thrive

35. 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

36. Cyanotic Congenital Heart Disease • R to L shunts • mixing lesions

37. RV outflow obstruction RVH VSD overriding aorta CXR reveals boot shaped heart with decreased pulmonary blood flow Tetralogy of Fallot – the classic cyanotic lesion

39. 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

41. 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

42. 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

43. Acyanotic Congenital Heart Disease • L to R shunts • stenotic lesions

44. VSD • Most common congenital lesion • Large VSD’s 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

45. 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”:

46. 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

47. LV Outflow Obstruction

48. 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

49. 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

50. 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