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Cardiac emergancies for m students 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. 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. 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 Case Study 1 Medication therapy Digitalis Digoxin ACE inhibitors (Angiotensin-converting enzyme inhibitors) captopril Diuretics: Lasix, , aldactone. 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. 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. Tetralogy of Fallot “Tet spell” Hyperpnea Worsening cyanosis Disappearance of murmur RBBB pattern on ECG Tetralogy of Fallot Small to N cardiac silhouette pulmonary vasculature SVR PVR Treatment of Tet Spell Quiet, calm environment Knee-chest or squatting position • Oxygen Morphine • increases afterload by promoting systemic vasoconstriction Crystalloids • to reduce spasm and/or sympathetic stimulation Phenylephrine • increases afterload thus increasing systemic resistance consider small volume challenge (5-10 cc/kg) to increase preload and SVR NaHCO3 • to treat acidosis Oxygenate to target saturation levels Squatting Common with unrepaired TOF Increases oxygen saturations Angulation and kinking of femoral arteries with increased SVR, decreasing the R->L shunt Hypercyanotic Spells ( TET spells) Syncope Morphine Hypovolemia Agitation Tachycardia Oxygen Cyanosis Knee-chest Rt Lt shunt PVR Fluid Impaired RV filling RVOT obstruction Phenylepherine Age Cyanotic Spells Increase systemic vascular resistance Squat/Knee chest position Ketamine 1-2mg/kg IV Neosynephrine 0.02mg/kg IV Tachycardia Release of infundibular spasm Propranolol 0.1mg/ Kg IV Irritability Morphine 0.2mg/ Kg S.C or IM Hypoxia Oxygen Dehydration Volume Acidosis NaHco3 1mEq/ Kg IV 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. 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. 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 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. 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) 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 . Case study 3 Neonates and infants with central cyanosis or cardiac failure are an emergency – irrespective of their clinical state. 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 Case Study 4 Tests : CBC, CRP, ESR EKG, ECHO Ultrasound of abdomen Case Study 4 • • • • On the 2nd 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 Kawasaki Disease She had an attack of syncope for 2 minutes in the 2nd day evening so transferred to ICU . She also complained from chest pain ,shortness of breath and weakness. She was very anxious. 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 • Kawasaki Disease 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. 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. 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. Qwaves in L2 &L3 and T wave inversion in V5 & V6 are noted. Case Study 6 Case Study 6 MYOCARDITIS MYOCARDITIS Symptomatic • o o o Correction of etiology Immunosupression • • o o o o • inotropes Diuretics afterload reduction Steroids Anti-virals Cyclosporin Interferon Transplantation 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. Three similar episodes; Preceded by palpitations ,one of them associated with exercise. Family History : Negative Case study 6 Now awake and alert; skin color is normal , normal breathing. normal appearance Vital signs: HR 70; RR 20; BP 90/60; T 37.7 C Wt 39 kg; O2 sat 99% Systemic examination ; normal Case study 6 What is your general impression of this patient? 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 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? Case study 6 • Stable Patient with syncope In no distress; normal exam Concerning/ominous history What are your initial management priorities? Diagnostic Studies Laboratory is often normal but may include: Electrolytes / Ca++, Mg++, PO4. 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 Diagnostic Studies ECG/Holter. Echocardiography Cardiac MRI Continuous cardiac monitoring EEG Genetic testing Stress ECG 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. Case study 6 Normal heart structure WPW syndrome. Long or short QT syndrome. Brugada syndrome CPVT Case Study 5 Long QT syndrome (Jervell-Nielson-Lange) QT (corrected) QTc= QT (msec) √R-R (sec) = 640/ 1.05 = 610 msec > 450 m sec is long Syncope in Children – transient loss of consciousness and postural tone due to generalized cerebral ischemia with rapid and spontaneous recovery Presyncope - no complete loss of consciousness occurs Syncope Syncope in Children Affects 15% of children between 8-18 Uncommon under age 7 therefore think about: Seizure disorders Breath holding Primary cardiac dysrhythmias Cardiovascular causes unusual but life-threatening anatomic abnormalities congenital malformations valvular disease electrical abnormalities Syncope in Children Vasovagal Events 32% to 50% of cases Decreased PVR Decreased venous return Decreased cardiac output Hypotension Bradycardia 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 Differential Diagnosis of Syncope: Seizures vs Hypotension Observation Onset Duration Jerks Headache Confusion after Incontinence Eye deviation Tongue biting Prodrome EEG Seizure Inadequate Perfusion Sudden More gradual Minutes Frequent Seconds Rare Frequent (after) Frequent Frequent Occasional (before) Rare Rare Horizontal Frequent Vertical (or none) Rare Aura Often abnormal Dizziness Usually normal Causes of True Syncope NeurallyMediated Orthostatic Cardiac Arrhythmia Structural CardioPulmonary 1 2 3 4 • Vasovagal • Carotid Sinus • Situational Cough PostMicturition • Drug-Induced • Autonomic Nervous System Failure • Brady Primary Secondary • Tachy SN Dysfunction AV Block VT SVT • Long QT Syndrome Unexplained Causes = Approximately 1/3 • Acute Myocardial Ischemia • Aortic Stenosis • HCM • Pulmonary Hypertension • Aortic Dissection Likely Causes In Children Vasovagal Situational Psychiatric Long QT* WPW syndrome RV dysplasia Hypertrophic cardiomyopathy Catecholaminergic VT Other genetic syndromes 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 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 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 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) 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 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 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 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 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 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 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 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 . Case study 7 What is your general impression of this patient? Case study 7 Laboratory: all normal Electrolytes CBC with differential Hepatic and renal profile. Head CT was postponed Case study 7 On exam by the 2nd 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. Case study 7 Laboratory Evaluation: CBC & ABG/VBG Ca, Magnesium & Phos Renal profile CXR & 12 lead ECG Hormonal assay Ultrasound of abdomen/ Echocardiography Urinary VMA 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 Case study 7 Case study 7 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. Coarctation of Aorta Case study 7 Management : Coarctation of Aorta 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 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