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Common Cardiac Emergencies

Common Cardiac Emergencies. Agustin E. Rubio, MD Sibley Heart Center Cardiology Children’s Healthcare of Atlanta Emory School of Medicine. Topics. Cyanosis & Ductal Dependence Emergency Room Diagnoses: Tetralogy of Fallot Hypoplastic Left Heart Syndrome Coarctation of Aorta SVT

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Common Cardiac Emergencies

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  1. Common Cardiac Emergencies Agustin E. Rubio, MD Sibley Heart Center Cardiology Children’s Healthcare of Atlanta Emory School of Medicine

  2. Topics • Cyanosis & Ductal Dependence • Emergency Room Diagnoses: • Tetralogy of Fallot • Hypoplastic Left Heart Syndrome • Coarctation of Aorta • SVT • Shunt Dependent vs Non-shunt Dependent

  3. Epidemiology • Cardiac malformations • 10% of infant mortality • Incidence: • 4-6/1000 live births • Most common lethal diagnosis: • Left ventricular outflow tract obstruction • Hypoplastic left heart syndrome • Coarctation of aorta • Aortic stenosis

  4. Circulatory Transitions • Conversion from right sided (placental oxygenation) to left sided circulation (pulmonary oxygenation) • Progression is secondary: • Decreasing PVR • Closure of ductal shunts • Clinical presentations: • Cyanosis • Respiratory failure • Shock

  5. Cyanosis • Typically, 2 g/dL of reduced hemoglobin • 5g/dL of reduced Hb  clinical cyanosis • The higher the Hb the less likely to have severe cyanosis

  6. Ductal Dependent Lesions Cyanosis CHF/Shock • Lt Ventricular Outflow Tract Obstruction: • HLHS • Coarctation of Aorta/ AS • Truncus arteriosus • TGA with VSD • TAPVR • Rt to Lt shunting: • Tricuspid atresia • TOF/ Pulm atresia • Ebstein’s anomaly

  7. Left Ventricular Outflow Tract Obstruction • Major source of neonatal M&M from CHD • Accounts for ~ 12% of congenital cardiac disease in infancy • ~ 75% discharged from hospital w/o diagnosis • ~ 65% - normal newborn screen examination • 6% died before diagnosis • 96% symptoms by 3 wks of life

  8. Symptoms in Real-Time Timeline of Clinical Diagnosis Week #1 HLHS Coarctation of aorta TAPVR - obstucted Week #2-6 Transposition of Great Arteries Total Anomalous Venous Return Truncus arteriosus

  9. Tetralogy of Fallot

  10. Tetralogy of Fallot • Prevalence: • - 10% of CHD • Most common cyanotic heart defect beyond infancy

  11. Tetralogy of Fallot • +/- Cyanosis • Small to Nl cardiac silhouette • pulmonary vasculature

  12. Tetralogy of Fallot • “Tet spell” • Hyperpnea • Worsening cyanosis • Disappearance of murmur • RBBB pattern on ECG

  13. Tetralogy of Fallot • “Tet spell” • Treatment objectives: • Reverse the right-to-left shunt • systemic vascular resistance (SVR) • Correct potential acidosis with NaHCO3 & volume • Consider peripheral vasoconstriction (phenylephrine – 0.02 mg/kg IV) • Ketamine • increase SVR and sedates 2 mg/kg over 1 min • Morphine sulphate • Oxygen

  14. Tetralogy of FallotSurgical Options • Blalock-Taussig shunt • Delayed repair • Trans-annular patch • VSD closure

  15. Tetralogy of FallotPost-operative Concerns • Post-pericardiotomy syndrome • ~ 4 weeks post-op (25-30% of open heart pts) • Fever, elevated ESR and CRP • Increased work of breathing (? pericardial effusion) • Cardiomegaly, pleural effusions • ECG – persistent ST segment elevation with flat or inverted T waves in limb & left lateral limb leads • Pericardiocentesis – performed when tamponade physiology present

  16. Tetralogy of FallotPost-operative Concerns • Endocarditis • Dx after >2 BCx or echo evidence • Residual VSD • Arrhythmias • AV block, ventricular arrhythmias • Remember: • Any incision in the ventricle produces a RBBB pattern (rSR’ in V1; wide complex QRS)

  17. Tetralogy of FallotPost-operative Concerns • Arrhythmias • TOF - 40% increased incidence of lethal arrhythmias • Syncopal events- lethal ventricular arrhythmias ??

  18. Hypoplastic Left Heart Syndrome

  19. HLHS

  20. HLHS • Uncommon form of cyanotic heart disease • Most common cause of death in the first month of life • Critically ill infant within the first 7 days with low O2 saturations

  21. HLHS • Clinically: • Progressive cyanosis and hypoxemia • Hx of poor feeding, tachypnea and poor weight gain • Cardiovascular shock • Severe acidosis • Congestive heart failure

  22. Consequences and Complications • Polycythemia (erythrocytosis) • Clubbing (>6 mos of age) • Hypoxic spells • CNS • Cyanotic heart disease accounts for 5-10% of brain abscesses • Cerebral venous thrombosis - <2 yrs, cyanotic and microcytic anemia • Dyscrasias

  23. HLHSPre-operative Resuscitation • Medical management: • Intubation • Ventilate and oxygen • Intravenous access • Central/ umbilical/ intra-osseos • Glucose • Na HCO3 • PGE1(get that PDA open!!) • PGE1 0.05 mcg/kg/min • Volume – NS/ 5% Albumin/ PRBC’s • NIRS probe

  24. HLHSNorwood/ Blalock-Taussig Shunt • Post-operative changes • Uncontrolled PBF • Re-constructed aortic outflow tract • Fluid balance sensitive • Widened pulse pressures • Tenuous coronary circulation • Single ventricle for all circulation

  25. HLHSNorwood/ Sano shunt • Post-operative changes • Direct PA communication with RV • Uncontrolled PBF • Neo-aortic reconstruction • Higher diastolic pressures • Better coronary perfusion

  26. HLHSPost-Operative Resuscitation • Limit oxygen (remember: relative uncontrolled PBF) • Hemoglobin • Auscultate for murmur: • Continuous murmur at RUSB (? BT shunt) • Systolic murmur at RLSB/ LUSB (Sano shunt) • Fluid balance: • Palpate liver • +/- rales and CXR to evaluate for CHF • Reverse dehydration • Reverse acidosis

  27. Coarctation of Aorta

  28. Coarctation of Aorta • Common cause of left sided heart failure • 95% located in juxtaductal region • Associated with other congenital anomalies • May be short segments or long segments

  29. Coarctation of Aorta • Associations: • HLHS • Aortic stenosis • TOF • Truncus arteriosus • VSD • DORV • Turner’s syndrome

  30. Coarctation of Aorta • Clinical • Poor feeding, dyspnea & poor weight gain • Upper arm vs lower extremity BP discrepancy • >10-20 mmHg systolic upper vs. lower • 20-30% develop CHF by 2-3 months • Hx of lower extremity weakness or pain after exercise • 50% will have no murmur

  31. Coarctation of Aorta • Acute clinical presentation: • Cardiovascular shock • Somnolent & lethargic • Poor po intake/ dehydrated, poor U/O • Cold, clammy & diaphoretic • Poor pulses • +/- organomegaly • Bradycardia/ tachycardia

  32. Coarctation of Aorta • Laboratory Evaluation: • CBC & ABG/VBG • CMP, Magnesium & Phos • Lactate • BNP level • CXR & 12 lead ECG • Blood cultures • NIRS probe

  33. Coarctation of Aorta • Neonatal Coarctation • rSR’ in the right precordial leads (V1 & V2) • Deep S waves in the lateral leads • RAD

  34. Coarctation of Aorta • Infant Coarctation • LVH apparent (left lateral leads) • Deep S waves in the right chest • Large R waves in lateral leads

  35. Coarctation of AortaSurgical repairs

  36. Coarctation of AortaPost-operative State • Re-coarctation • Occurs most commonly within the first 12 months • Evaluated by 4 extremity BP’s • Physical examination of upper & lower extremity pulses

  37. Tachyarrhythmia:Sinus Tach vs. SVT

  38. Clinical Signs of Tachyarrhythmia

  39. Neonate: Sudden onset of irritability& sudden relief Poor po intake & somnolence Inconsolable “Rapid heart beat”– felt by parents Older Child: Stops activity abruptly “Palpitations”/ “feels funny” Sudden relief with vasovagal manuever Chest pain - rare Symptoms from History

  40. ECG Findings Sinus Tach Sinus Tach

  41. Rhythms SVT • Regular rhythm, narrow QRS, HR >200, p buried in T wave Sinus Tach • Regular rhythm <200, distinct p waves, nl intervals

  42. Sinus Tachycardia vs. SVT

  43. SVT – Hemodynamically Stable

  44. SVT – Hemodynamically Unstable ** Cardioversion should be performed in a location which can provide for continuous monitoring and potential complications of sedation.

  45. Medications for SVT

  46. Laboratory Evaluation • Electrolytes • Calcium, Magnesium & Phosphorus • CBC with diff • CXR & 12 lead EKG • looking for pre-excitation – WPW

  47. Shunt Dependent vs. Non-dependent What’s the big deal !!!

  48. The Difference • Shunt Dependent • The only source of PBF = SHUNT • Non-Dependent • Two sources of PBF = Shunt + some antegrade flow through diminuitive PV

  49. Shunt Dependent • Oxygen therapy • Limit O2 therapy for cyanosis • Maintain sats 75-85% • Sats can drop significantly and quickly • If sats >85%: • PVR  PBF  Pulmonary edema and circulatory shock • Use blended O2 with range of up to FiO2 0.4

  50. Non-Dependent • Oxygen therapy • Two sources of PBF: • One with fixed obstruction and the other is uncontrolled • If BT shunt present: • Limit O2 • O2 saturations should not drop as far nor as quickly

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