1. Cardiac emergancies for m students

2. Case Study 1  Ali is a 7 month old with Down Syndrome. His birth weight was 3.5 kg; a week ago he weighed 5.5 kg.  He is breathing 72 bpm with a HR of 190 and a sat of 91%.  He is diaphoretic and is not taking his usual 3 oz formula every 3-4 hours. He has had 4 moderately wet diapers in the past 24 hrs.

3. case study 1  Identify abnormal assessment data for ali  What other data would you collect? (Hx and examination)  What is your priority medical action?  What investigations' would you do for him?  What other medical therapy& interventions would you do for him.

4. Case Study 1  Air way support/management  Oxygen  fluids, as indicated( in CHF, fluids may be restricted).  increased calories or concentrated formula(prescribed)  rest and spacing of activity/rest periods

9. Case Study 1  Medication therapy u Digitalis  Digoxin u ACE inhibitors (Angiotensin-converting enzyme inhibitors)  captopril u Diuretics: Lasix,, aldactone.

10. Case Study 1  surgery during infancy to prevent PHT  patches placed over septal defects; mitral valve replacement  arrhythmias and mitral valve insufficiency occur postoperatively.  no difference between short term survival rates in infants with or without Down’s syndrome.

13. Case Study 2  You have responded for a 3 month old male infant because the parent’s state he is having episodes of excessive crying followed by limpness, cyanosis and passing out. He was born at 41 weeks of gestation by C-section because of failure to progress. Apgar scores of 7 and 8 at 1 and 5 minutes, respectively. His cyanosis increases with crying.  On Exam you note that he is alert and active in respiratory distress, with visible cyanosis. His lungs are clear. He has normal peripheral pulses with cyanotic nail beds and mucous membranes. VS: T 37 (98.6), P164, RR 64, oxygen saturation 83% on blow-by oxygen.

14. Tetralogy of Fallot  “Tet spell” u Hyperpnea u Worsening cyanosis u Disappearance of murmur u RBBB pattern on ECG

15. Tetralogy of Fallot  Small to N cardiac silhouette  pulmonary vasculature

17. SVR

18. PVR

19. Treatment of Tet Spell  Quiet, calm environment  Knee-chest or squatting position increases afterload thus increasing systemic resistance  Oxygen  Morphine to reduce spasm and/or sympathetic stimulation  Phenylephrine increases afterload by promoting systemic vasoconstriction  Crystalloids consider small volume challenge (5-10 cc/kg) to increase preload and SVR  NaHCO3 to treat acidosis to treat acidosis Oxygenate to target saturation levels

22. Squatting  Common with unrepaired TOF  Increases oxygen saturations  Angulation and kinking of femoral arteries with increased SVR, decreasing the R->L shunt

23. Tachycardia Impaired RV filling  RVOT obstruction  Rt  Lt shunt  Agitation Hypovolemia  Age  PVR Hypercyanotic Spells ( TET spells)  Cyanosis Syncope Fluid Morphine Oxygen Phenylepherine Knee-chest

24. Cyanotic Spells  Increase systemic vascular resistance Squat/Knee chest position Ketamine 1-2mg/kg IV Neosynephrine 0.02mg/kg IV  Tachycardia Propranolol 0.1mg/ Kg IV Release of infundibular spasm  Irritability Morphine 0.2mg/ Kg S.C or IM  Hypoxia Oxygen  Dehydration Volume  Acidosis NaHco3 1mEq/ Kg IV

27. Case study 3  A 5 hour old male newborn infant was born at 39 weeks gestation via normal vaginal delivery to a 23 year old G2 P2 O+ mother with unremarkable prenatal serology studies. Apgar scores were 8 and 9 at 1 and 5 minutes. His initial physical exam was normal. He stayed in mother's room and breastfed shortly after delivery. At 5 hours of age, with the second feeding, the baby appears tachypneic and cyanotic, and he is therefore taken to the nursery for further evaluation.

28. Case study 3  Exam: VS T 37.0, HR 145, RR 78, BP 67/38, oxygen saturation 82% in room air. Length 53 cm (50%ile), weight 3.7 kg (50%ile), HC 34 cm (50%ile). He is an alert active, nondysmorphic and mildly cyanotic term male. Tachypnea and mild nasal flaring are present. His heart is regular with a grade 2/6 soft systolic murmur at the upper left sternal border. The precordium is quiet. Lungs are clear bilaterally. No hepatosplenomegaly is noted.

29. Case study 3  He is placed in an oxygen hood with a fraction of inspired oxygen (FiO2) of 0.7 (70%) with no appreciable rise in his oxygen saturation. A radial arterial blood gas shows pH 7.44, pCO2 35, paO2 39, bicarb 22 in FiO2 0.7 (70%) by hood.  A chest radiograph : cardiomegaly, narrow mediastinum and increased pulmonary vasculature. ECG: RAD and RVH

30. Case study 3  Echocardiography reveals D-transposition of the great vessels with a 5mm ventricular septal defect and patent ductus arteriosus. A prostaglandin E1 infusion is started. The infant is mechanically ventilated and subsequently transported to a pediatric cardiac surgical specialty center. An arterial switch procedure is performed successfully. He is discharged home 3 weeks later.

31. Initial Stabilization  ABC’s: Volume resuscitation, ionotorpic support, correction of metabolic acidosis, r/o sepsis  Intubate if needed, titrate Fi02 to keep Sp02 80%-85% to prevent pulmonary overcirculation  Placement of umbilical lines  Infants who present in shock within the first 3 weeks of life, consider ductal dependent lesions  Use of PGE1 (0.025 to 0.1mcg/kg/min)

32. Stabilization for Transport  Reliable vascular access  Intubation if on PGE1, OG placement  Oxygen delivery, Sp02  Monitor HR, tissue perfusion, blood pressure, temp and acid-base status  Calcium and glucose status.

33. Case study 3  Neonates and infants with central cyanosis or cardiac failure are an emergency – irrespective of their clinical state.

35. Case study 4  6 years old girl presented with fever for 8 days,skin rash for 4 days associated with red strawberry tongue.  She was seen at private clinic 3 days earlier and diagnosed as scarlet fever, treated with ospen since then but without any improvement.  On examination : normal vital signs apart from fever Enlarged right anterior cervical lymph nodes, strawberry tongue. Maculopapular skin rash over the chest and back Normal systemic examination

38. Case Study 4  Tests :  CBC, CRP, ESR  EKG, ECHO  Ultrasound of abdomen

39. Case Study 4  On the 2 nd day after admission to hospital she had peeling of the fingers,nausea, vomiting, diaphoresis, pallor and irritability.  Examination: HR: 120.RR :42.BP: 100/60. Oxygen sat: 92% She is irritable,sick looking with respiratory distress Enlarged right anterior cervical lymph nodes. peeling of fingers

41. Kawasaki Disease  She had an attack of syncope for 2 minutes in the 2 nd day evening so transferred to ICU.  She also complained from chest pain,shortness of breath and weakness. She was very anxious.

42. Kawasaki Disease  ECG: o Q waves (>3 mm) In lead III, aVF o ST segment changes of >2 mm ST depression in lead V5, V6 with T wave inversion  Troponin Level was high

43. Kawasaki Disease

45. Case Study 5  You have responded for a 5 month old female who has been lethargic, not feeding well, and appears to be in respiratory distress. She was sick 2 weeks ago. At that time she had a fever, cough, and a runny nose. She was also seen 2 days ago in private clinic for respiratory distress,diagnosed as bronchiolitis and started on steroids and bronchodilators.  She is the product of a G3P2, full term, uncomplicated pregnancy. Delivery was unremarkable except for meconium stained fluid. She did well at delivery and in the nursery. Her pediatric follow-up has been poor.

46. Case Study 5  On Exam you note that she is an acyanotic infant who is pale, lethargic, and tachypneic, with mild to moderate subcostal and intercostal retractions. VS T 36.8, RR 72, HR 160, BP 72/48, with capillary refill of 4 to 5 seconds.. Oxygen saturation in room air is 94%. Her lungs have scattered crackles with slightly decreased aeration in the left lower lobe.

47. Case Study 5  Her heart is of regular rate and rhythm, with a normal S1 and S2. An S4 gallop is noted at the cardiac apex.  A 12 lead electrocardiogram shows a sinus tachycardia, low voltage QRS complexes. Q- waves in L2 &L3 and T wave inversion in V5 & V6 are noted.

49. Case Study 6

51. MYOCARDITIS

52. Symptomatic o inotropes o Diuretics o afterload reduction Correction of etiology Immunosupression o Steroids o Anti-virals o Cyclosporin o Interferon Transplantation

54. Case study 6  11-year-old girl passed out during reading; awoke after 3 min.  She was stiff with eyes rolled back ~ approx. 3 min. u Three similar episodes; Preceded by palpitations,one of them associated with exercise. u Family History : Negative

55. Case study 6 u Now awake and alert; skin color is normal, normal breathing. normal appearance u Vital signs: HR 70; RR 20; BP 90/60; T 37.7  C Wt 39 kg; O 2 sat 99% u Systemic examination ; normal

56. Case study 6  What is your general impression of this patient?

57. Clinical Features: Your First Clue  Loss of consciousness  Lasted only a few minutes  Minimal or no postictal state  No stigmata of seizure: Urinary incontinence, bitten tongue, witnessed tonic-clonic activity

58. Syncope: Key questions to address with initial evaluation  Is the loss of consciousness attributable to syncope or not?  Is heart disease present or absent?  Are there important clinical features in the history that suggest the diagnosis?

59. Case study 6 Stable  Patient with syncope  In no distress; normal exam  Concerning/ominous history What are your initial management priorities?

60. Diagnostic Studies  Laboratory is often normal but may include:  Electrolytes / Ca ++, Mg ++, PO 4. Blood glucose  CBC with differential  cardiac enzyme  Radiology:  CXR offers little.  CT or MRI of the brain and neck may be indicated if considering seizures or injury

61. Diagnostic Studies  ECG/Holter.  Echocardiography  Cardiac MRI  Continuous cardiac monitoring  EEG  Genetic testing  Stress ECG

62. Case study 6 Differntial diagnosis :  Structural heart defect : Known Congenital heart disease (Ebstein’s anomaly,LTGA,ASD) Hypertrophic cardiomyopathy Anomalous origin of the LCA Myocarditis Arrhythmogenic RV dysplasia Coronary artery disease Primary or secondary pulmonary hypertension.

63. Case study 6  Normal heart structure  WPW syndrome.  Long or short QT syndrome.  Brugada syndrome  CPVT

64. Case Study 5

65. QT (corrected) QTc= QT (msec) √R-R (sec) = 640/ 1.05 = 610 msec > 450 m sec is long Long QT syndrome (Jervell-Nielson-Lange)

66. Syncope in Children  Syncope – transient loss of consciousness and postural tone due to generalized cerebral ischemia with rapid and spontaneous recovery  Presyncope - no complete loss of consciousness occurs

67. Syncope in Children  Affects 15% of children between 8-18  Uncommon under age 7 therefore think about: u Seizure disorders u Breath holding u Primary cardiac dysrhythmias  Cardiovascular causes unusual but life-threatening u anatomic abnormalities u congenital malformations u valvular disease u electrical abnormalities

68. Syncope in Children  Vasovagal Events u 32% to 50% of cases u Decreased PVR u Decreased venous return u Decreased cardiac output u Hypotension u Bradycardia

69. Syncope Mimics  Disorders without impairment of consciousness Falls Drop attacks Cataplexy Psychogenic pseudo-syncope Transient ischemic attacks  Disorders with loss of consciousness Metabolic disorders Epilepsy Intoxications Vertebrobasilar transient ischemic attacks

70. Differential Diagnosis of Syncope: Seizures vs HypotensionObservationSeizure Inadequate Perfusion OnsetSudden More gradual DurationMinutesSeconds JerksFrequentRare Headache Frequent (after) Occasional (before) Confusion after FrequentRare IncontinenceFrequentRare Eye deviation Horizontal Vertical (or none) Tongue biting FrequentRare ProdromeAuraDizziness EEG Often abnormal Usually normal

71. Causes of True Syncope Orthostatic Cardiac Arrhythmia Structural Cardio- Pulmonary 1 Vasovagal Carotid Sinus Situational  Cough  Post- Micturition 2 Drug-Induced Autonomic Nervous System Failure  Primary  Secondary 3 Brady  SN Dysfunction  AV Block Tachy  VT  SVT Long QT Syndrome 4 Acute Myocardial Ischemia Aortic Stenosis HCM Pulmonary Hypertension Aortic Dissection Neurally- Mediated Unexplained Causes = Approximately 1/3

72. Likely Causes In Children  Vasovagal  Situational  Psychiatric  Long QT*  WPW syndrome  RV dysplasia  Hypertrophic cardiomyopathy  Catecholaminergic VT  Other genetic syndromes

73. 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

74. Syncope: Important Historical Features Questions about circumstances just prior to attack  Position (supine, sitting, standing)  Activity (rest, change in posture, during or immediately after exercise, during or immediately after urination, defecation or swallowing)  Predisposing factors (crowded or warm place, prolonged standing post-prandial period) and of precipitating events (fear, intense pain, neck movements) Questions about onset of the attack  Nausea, vomiting, feeling cold, sweating, pain in chest

75. Syncope: Important Historical Features Questions about attack (eye witness)  Skin color (pallor, cyanotic)  Duration of loss of consciousness  Movements ( tonic-clonic, etc.)  Tongue biting Questions about the end of the attack  Nausea, vomiting, diaphoresis, feeling cold, muscle aches, confusion, skin color, wounds

76. Syncope: Important Historical Feature Questions about background  Number and duration of syncope spells  Family history of arrhythmic disease or sudden death  Presence of cardiac disease  Neurological disease  Medications (Hypotensive, negative chronotropic and antidepressant agents )

77. Clinical Features Suggesting Specific Cause of Syncope Neurally-Mediated Syncope  Absence of cardiac disease  Long history of syncope  After sudden unexpected, unpleasant sensation  Prolonged standing in crowded, hot places  Nausea vomiting associated with syncope  During or after a meal  With head rotation or pressure on carotid sinus  After exertion

78. Clinical Features Suggesting Specific Cause of Syncope Syncope due to orthostatic hypotension  After standing up  Temporal relationship to taking a medication that can cause hypotension  Prolonged standing  Presence of autonomic neuropathy  After exertion

79. Clinical Features Suggestion Cause of Syncope Cardiac Syncope  Presence of structural heart disease  With exertion or supine  Preceded by palpitations  Family history of sudden death

80. Initial Exam: Thorough Physical  Vital signs  Heart rate  Orthostatic blood pressure change  Cardiovascular exam: Is heart disease present?  ECG: Long QT, pre-excitation, conduction system disease  Echo: LV function, valve status, HCM  Neurological exam

81. Orthostatic Measurements  Classically, abnormal if systolic BP decreases by more than 20 points and/or pulse increases in pulse rate of more than 20 beats per minute after a change from supine to standing  If there is only a pulse increase but no drop in blood pressure, the test is less significant

82. Diagnostic Objectives  Distinguish true syncope from syncope mimics  Determine presence of heart disease and risk for sudden death  Establish the cause of syncope with sufficient certainty to: Assess prognosis confidently Initiate effective preventive treatment

84. Case study 7  12 year old girls presented to ED with history of sever headache for last 2 weeks. The headache was intermittent,diffuse and bilateral.  She was seen in different private clinic and treated with regular analgesics but without effect.  Not regular in the school. no menses yet

85. Case study 7  On exam: T 37, HR 86, RR 20, BP 90/60, oxygen saturation 99% in room air. height 159 cm (50%ile), weight 43.7 kg (50%ile). She is alert,no signs of respiratory distress, pallor, cyanosis.  Normal E.N.T,chest,CNS,abdomen and skin examination.  Cardiac exam: normal heart sounds,short systolic murmur grade 2/6 at ULSB, good volume pulses.  Normal puberty stages.

86. Case study 7  What is your general impression of this patient?

87. Case study 7  Laboratory: all normal u Electrolytes u CBC with differential u Hepatic and renal profile.  Head CT was postponed

88. Case study 7  On exam by the 2 nd shift in ER: T 37, HR 80, RR 18, BP in RA 130/90, oxygen saturation 100% in room air. She is alert,no signs of respiratory distress, pallor, cyanosis.  Normal neurological examination.  Normal E.N.T,chest,abdomen and skin examination.  Cardiac exam: normal heart sounds,short systolic murmur grade 3/6 at ULSB with radiation to the back between the scapulae.  Extremities: Femoral pulses are slightly diminished to palpation; no peripheral edema, clubbing or cyanosis of the nail beds.  Normal puberty stages, normal female genitalia.

90. Case study 7  Laboratory Evaluation: u CBC & ABG/VBG u Ca, Magnesium & Phos u Renal profile u CXR & 12 lead ECG u Hormonal assay u Ultrasound of abdomen/ Echocardiography u Urinary VMA

91. Case study 7  Chest radiograph : cardiac/thoracic ratio of 0.55, normal cardiac configuration, and normal pulmonary vasculature and notching at lower margins of upper ribs  ECG:  sinus tachycardia  LVH apparent (left lateral leads)  Deep S waves in the right chest  Large R waves in lateral leads

92. Case study 7

93.  The echocardiogram demonstrates narrowing of the distal aortic arch with increased velocities on pulsed and color Doppler and bicuspid aortic valve  She was diagonesd as Coarctation of the aorta, bicuspid aortic valve.

94. Coarctation of Aorta

95. Case study 7  Management :

96. Coarctation of Aorta u Upper arm vs lower extremity BP discrepancy  >10-20 mmHg systolic upper vs. lower  20-30% develop CHF by 2-3 months u Hx of lower extremity weakness or pain after exercise u 50% will have no murmur

97. Hypertension  1-3% of the pediatric population  unknown cause = essential or primary HTN  underlying kidney or cardiac disease=secondary HTN  children’s BP in >90th at increase risk for adult HTN  HTN in adolescent correlates with obesity and elevated serum lipid level