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Congenital Heart Disease

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by

Ehab Eshtaya

on 21 March 2013

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Transcript of Congenital Heart Disease

Cyanosis High Pulmonary flow Truncus Arteriosus TGA TAPVR Low / Normal Pulmonary flow Tricuspid Atresia Ebstein's Anomaly Critical PS
PA with intact IVS Tetralogy of Fallot Congestive Heart Failure Cardiogenic Shock HLHS Aortic Coarctation Interrupted Aortic Arch Elevated Preload PDA VSD ASD AVSD Ventricular dysfunction Inflammatory Primary Cardiomyopathy Shock Cardiogenic Hypovolemic Distributive Cyanosis Concerning features in the history: Neonates/Infants Failure to Thrive

Feeding intolerance

Cyanosis with feeding

Tachypnea post feeds

Hx Perinatal complications

Malformation Older Children Chest Pain

Syncope

Exertional dyspnea

Exercise intolerance

FH of sudden death

FH of Marfans When should you consider cardiac disease in the differential? History Clincial Picture Heart Failure Cardiac disease should be considered when a patient presents in one of the following: So you think it's Cardiac? Now what's your approach? You've considered the possiblity of cardiac disease ... now what? Organized Cardiac Exam Ancillary investigations Non Cardiogeic Elevated Afterload Aortic Stenosis
Pulmonary Stenosis
Coarctation Severe Anemia
Hypoglycemia
Hypocalcemia
Metabolic
Malnutrition Step 1: Inspection / Vitals Step 3: Auscultation Step 2: Palpation/Pulses Circulation Breathing Appearance Identify Dysmorphic Features Precordial Palpation Precordial activity Should be done supine

If increased - often abnormal finding.
Implies elevated RV/LV Stroke Volume.
Often caused by ASD, VSD, PDA.

Can be a normal finding in anemia, hyperthyroidism and anxiety. Feel for Thrills Always an abnormal finding Pulses Feel brachial Feel Femoral Compare Right brachial to femoral Assess for volume and quality If equal - coarctation is unlikely.
Not a specific finding Pressure and oxygen saturation 4-limb Blood Pressure 4-limb Pulse Oxymetry Heart Sounds Murmurs S1 S2 S3 S4 Key Features Split S1? Is it obscured by another sound? Always an abnormal finding Often an abnormal finding.
Implies 'Click' or mistaken for S4 Key Features Key Features Key Features Electrocardiogram Chest X-ray Blood-work Non-splitting and loud? Always an abnormal finding Always Split? Always an abnormal finding Loud and doesn't vary with position? Likely an abnormal finding Common normal finding if it varies with position Due to the relatively hyperdynamic, high cardiac output state of pediatric heart Aknowledgments Dr Bill Sevcik Dr Lindsay Ryerson Pediatrics Emergency Medicine
Adult Emergency Medicine Pediatrics Cardiology
Pediatrics Critical Care ? Always an abnormal finding Pathologic Murmurs Functional Murmurs Grade Timing Character Key Features Key Features Key Features Grade III or higher / high pitch Grade I - Barely audible
Grade II - Soft murmur
Grade III - Loud murmur
Grade IV - Associated with Thrill
Grade V - Heard with stethoscope partially off the chest
Grade VI - Heard without a stethoscope Holosystolic Always abnormal Always abnormal Diastolic Murmur Abnormal but must make the distingtion from venous hum which is a normal finding. Harsh Murmur Machine like Murmur Abnormal - often associated with VSD / AS / PS Always abnormal - suggestive of PDA Volume Status Assess for pulmonary Edema Peripheral Edema Hepatomegaly / Ascites Blowing Murmur Abnormal - often associated with MR/TR*
Can also be heard with ASD / OFTO Continuous Always abnormal Functional murmur and the differential Functional murmur
vs ASD murmur Functional Murmur Defined as a low pitch, low grade crescendo-decrescendo systolic murmur associated with otherwise normal heart sounds. Aortic/Pulmonary
Stenosis Mitral/Tricuspic
Insufficiency Ventricular Septal
Defect Atrial Septal defect Significant lesions are difficult to confuse due to a high grade murmur (3 or greater). Different location, high pitch and associated with abnormal S1 (click). Regurgitant/blowing murmur in a different location. Significant lesions are not confused with functional murmur Often a holosystolic harsh murmur; rarely confused with a functional murmur Most difficult distinction to make. Many ASD murmurs are mistaken for functional murmur. Discussed next. When to refer? Symptomatic + Abnormality on exam? Cardiology consult +/- Echocardiogram in ED Asymptomatic + abnormality on exam? Cardiology consult +/- Echocardiogram as outpatient* Asymptomatic + functional murmur? Follow up with Family Practitioner Malformation + Abnormality on exam? Cardiology consult +/- Echocardiogram in ED The Diagnosis is Right! Cardiac Non-Cardiac CK
Troponin
BNP Glucose
Blood Gas
Extended electrolytes
CBC-D
Panculture (+/- LP)
+/- TSH
+/- Toxicology screen Severe Acidosis
Sepsis
Toxins / Drugs
Volume overload*
Renal Failure Emergency Departement Approach to Pediatric Cardiac Disease Ehab Eshtaya, MD
Emergency Medicine - R3 January 31, 2012 Presents with severe cyanosis within hours Poor Diffusion Cardiogenic Decreased FiO2 V/Q Mistmatch Hypoventilation Hyperoxia Test Echocardiogram Look for ...

1) Rate/Rhythm
2) Conduction delays/Blocks
3) Axis deviation
4) Strain patter
5) Ischemia Look for ...

1) Cardiac Silhouette / Situs
2) Increased vascular marking
3) Pleural effusion
4) Pneumothorax Look for ...

1) Cardiac markers of ischemia
2) Cardiac markers of volume overload*
3) Surrogate markers of perfusion
4) Sepsis workup
5) Metabolic and hematologic
6) Tox screen - if suspected from history Available in the Stollery ED for unstable patient
Urgency depends on the clinical scenario
Adult Echocardiography is not appropriate* Check pre-ductal and post-ductal PaO2 in room air
Give 100% O2 for 20 mins and repeat PaO2 If no respiratory or CV disease PaO2 will increase to > 500mmHg If lung disease* PaO2 will increase to > 150mmHg If obstructed pulmonary blood flow If intracardiac mixing lesion Minimal change in PaO2 PaO2 may increase by 10-20 mmHg (<100 mmHg) Sepsis
Neurogenic
Endocrine / Metabolic
Anaphylactic Bleed (external / Internal)
Fluid loss (Gastric / Renal)
Poor intake Shock is a dynamic and unstable pathophysiologic state characterized by inadequate tissue perfusion Compensated Shock Decompensated Shock Diaphoretic
Irritable
Normal tone
Tachypnea (or normal)
Tachycardia (Normal BP)
Oliguria Acrocyanosis/Pallor
Cold Extremities
Lethargic / Coma
Decreased Tone
Respiratory Failure
Bradycardia/ Hypotension
Abdominal Distension / Vomiting
Anuria Central Cyanosis is a physical sign that implies low Hgb saturation Slate blue discolouration of the mucous membranes, nail beds or skin
40-50 g/L of deoxygenated Hgb required for the sign Hypoxemia is defined as low oxygen tension in blood Conditions such as methemoglobinemia cause cyanosis but not hypoxemia* Hypoxia, or more appropriately, tissue hypoxia, is defined by low oxygen availability to specific tissue Myocardial infarction causes myocardial hypoxia but not necessarily systemic hypoxemia Cardiogenic Congestive heart failure is defined as the inability of the heart to meet the body’s metabolic demands Multiple definitions exist - poor consensus in pediatrics literature. The word 'Congestive' is also poorly defined but it relates to the body's neurohormonal response to retain fluid to maintain an 'effective circulating volume'.* The Diagnosis is Right! The full list of cyanotic cardiac disorders goes beyond the scope of this talk. Recall that cyanosis is a physical sign. Conditions listed under "congestive heart failure" and under "Shock" can cause cyanosis and vice versa. The differential listed here generally* present under the age of 6 weeks. Almost all are dependent on the ductus arteriosus for survival and/or oxygenation.* Disclaimer Transposition of the Great Arteries Total Anomalous Pulmonary Venous Return Duct Dependant Lesions The full list of cardiac disorders that cause shock goes beyond the scope of this talk. Conditions listed under "congestive heart failure" and under "Cyanosis" can result in shock if left untreated. The differential listed here generally* present under the age of 6 weeks. All are dependent on the ductus arteriosus for perfusion.* Most of these lesions present to ED when the duct is closing/closed. Disclaimer Hypoplastic left heart syndrome Multiple forms exist as dictated by associated leasions Rate / Rhythm Congenital Heart Block Tachyarrhythmia Ventricular Dilatation Abnormal Hgb Vital Signs Severe
Hypertension Pulmonary HTN* Difficult to categorize but presents with cyanosis, eventual RVH and failure, may cause cardiogenic shock Ventricular Hypertrophy Left-to-Right shunt Aortic Insufficiency Mitral Stenosis* Mitral Insufficiency Valvular Disease Bradyarrhythmia Dilated Cardiomyopathy Hypertrophic Cardiomyopathy Restrictive
Cardiomyopathy Ischemia ALCAPA Myocarditis Immunologic Vasculitis Abnormal
Coronary Secondary Cardiomyopathy 11 month 1-week 15 Year 9 Year 3 Year
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