Cardiovascular System I

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Cardiovascular System I
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Objectives

• Present the clinical features and emergency
  management of cardiovascular disorders,
  including
• Recognize congenital and acquired heart disease.
• Outline management of ductal dependent lesions.
• Identify patients with myocarditis.

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Congenital Heart Disease Recognition and
Stabilization

• Rapid cardiopulmonary assessment to recognize and
  manage life-threatening illness caused by heart
  disease
• Understand the physiology of different conditions
  to optimize treatment plans.

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Critical Concepts

• Dysrhythmias can cause serious cardiovascular
  compromise.
• Structural congenital heart disease can present
  in many different ways at many different ages.
• Acquired heart disease can be subtle yet
  life-threatening.

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Case Study 1Rapid Breathing

• 10-day-old infant is brought to ED by mother for
  rapid breathing and not eating well.
• Product of normal spontaneous vaginal delivery
• Spent 2 days with mother in hospital
• Uneventful course, including circumcision
• Birth weight 3.2 kg

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Case Study 1 (continued)

• Slow to breastfeed since birth
• Would gasp and cry after sucking for a short
  time. Difficulty feeding.
• 3 to 4 wet diapers per day
• No congestion, no fever
• No vomiting with feedings
• 2 yellow seedy stools since passing meconium
  after birth

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Initial Assessment (1 of 2)

• PAT
• Abnormal appearance, abnormal breathing, abnormal
  circulation
• Vital signs
• HR 170, RR 70, BP 82/40, T 37C (rectal), Wt 3.4
  kg, O2 sat 90 on room air

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Initial Assessment (2 of 2)

• A No evidence of obstruction
• B Elevated RR and labored
• C Pale, diaphoretic, tachycardia, weak pulse,
  cyanosis
• D GCS grossly normal but in distress and
  inconsolable
• E No signs of head injury, fractures, or
  bruising

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Detailed Physical Exam

• Lung sounds equal bilaterally with rales in both
  bases
• Hyperactive precordium with a gallop rhythm
• Pulses weak in distal and lower extremities
• Distended abdomen with liver palpable 4 cm below
  right costal margin

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Question

• What is your general impression of this patient?

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General Impression

• Impending cardiopulmonary failure (compensated
  shock)
• Cyanosis, diaphoresis
• Pale, tachycardia
• What are your initial management priorities?

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Management Priorities (1 of 3)

• ABCs
• Give 15L O2 by nonrebreather mask or 100 O2 by
  BMV, or perform endotracheal intubation.
• Start IV, obtain blood glucose.
• ECG and monitor rhythm on cardiac monitor
• CXR
• Administer fluid challenge 10 cc/kg NS

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Management Priorities (2 of 3)

• Administer prostaglandin E1 (PGE1)
• 0.05 to 0.1 mcg/kg/min
• Intubate to protect against apnea and relieve
  stress from work of breathing.
• Consider furosemide (0.5 to 1 mg/kg).
• Sepsis work-up and then antibiotics
• Defer lumbar puncture.

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Management Priorities (3 of 3)

• Cardiology consultation or transfer to pediatric
  cardiology center emergently
• Echocardiogram
• If blood pressure and perfusion do not improve,
  add inotropic agent
• Dobutamine 2 to 20 mcg/kg/min
• Epinephrine 0.1 to 1.5 mcg/kg/min

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Case Discussion (1 of 2)

• This infant is in CHF.
• Poor feeding and easy fatigability
• Gallop rhythm and enlarged liver
• Diminished pulses
• Shock
• Altered mental status, compensated shock
  (tachycardia, diaphoresis, respiratory distress,
  normal BP in upper extremities)

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Case Discussion (2 of 2)

• Possible ductal dependent lesion
• Right age for presentation of shock triggered by
  closure of the ductus arteriosus
• Measure blood pressure in four extremities
• Assess oxygenation response to supplemental oxygen

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Case Progression Version 1

• BP differential noted in lower extremities.
• Oxygenation improves to 99 with supplemental
  oxygen.
• CXR shows cardiomegaly and pulmonary edema.
• Echocardiogram demonstrates coarctation of the
  aorta.
• Infant improves with PGE1 infusion, diuretics,
  and inotropes.

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Case Progression Version 2

• Oxygenation fails to improve with supplemental
  oxygen (remains 90).
• Oxygenation declines further to lt80.
• CXR is nonspecific.
• Echocardiogram demonstrates transposition of the
  great vessels.
• Infant improves with PGE1 infusion.
• Surgical intervention is scheduled.

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Background Structural Congenital Heart Disease

• Congenital heart disease 5 to 8 cases per 1,000
  live births
• Child with congenital anomaly usually does not
  show cardiovascular problems in utero.
• Changes at birth place great stress on infants
  cardiovascular system.
• Some cyanotic heart conditions are highly
  dependent on shunting through ductus arteriosus.
  Closure can be terminal event.

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Clinical Features Your First Clue

• Age
• Progressive deterioration (mild) followed by
  suddenly progressing to critical condition
• Cyanosis
• Congestive Heart Failure (CHF)
• Consider concurrent sepsis

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Diagnostic Studies (1 of 3)

• Radiology
• Pulmonary hypoperfusion pulmonic stenosis, TOF,
  TA
• CHF (if large VSD present to allow high-output
  failure, e.g., increased right-sided flow)
• Some classic CXR appearances (more classic if
  condition is permitted to worsen)
• TGA Egg on side
• TAPVR Snowman
• TOF Boot shaped

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Diagnostic Studies (2 of 3)

• ECG
• Right axis (RVH) Normal for newborns
• Left axis Hypoplastic right heart, tricuspid
  atresia, endocardial cushion defect (AV canal)
• ST-T changes, strain, ischemia
• Dysrhythmia
• Prolonged QT
• Low voltage

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Diagnostic Studies (3 of 3)

• Laboratory
• Glucose Any child in distress needs to have
  hypoglycemia excluded.
• CBC Look for anemia, signs of sepsis.
• Electrolytes Congenital adrenal hyperplasia,
  salt-wasting form
• Arterial blood gas Hyperoxia text

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Fetal Circulation (1 of 2)

• Placenta oxygenates blood and returns to right
  atrium (RA) via IVC.
• Preferentially shunts across FO to LA.
• LV ejects most oxygenated blood to carotids and
  coronaries.

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Fetal Circulation (2 of 2)

• Superior vena cava (SVC) returns deoxygenated
  blood to RA where it mixes with oxygenated blood
  from the placenta.
• Preferentially enters RV.
• RV ejects into PA.
• No pulmonary capillary flow, so PA is shunted
  into the descending aorta via the ductus
  arteriosus.

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Coarctation of the Aorta (1 of 2)
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Coarctation of the Aorta (2 of 2)
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Transposition of the Great Arteries
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Differential Diagnosis What Else? (1 of 2)

• Other cyanotic and acyanotic congenital
  structural heart disease
• Ductal dependent coarctation
• Hypothermia
• Sepsis
• TORCH

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Differential Diagnosis What Else? (2 of 2)

• Congenital adrenal hyperplasia (CAH)
• Hypoglycemia
• Shaken baby syndrome/intracranial lesion
• Catastrophic gastrointestinal process, e.g.,
  volvulus

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Normal CV System Function

• Represented by vital signs (O2 sat included)
• Factors affecting cardiac output (perfusion)
• Heart rate
• Stroke volume
• Contractility
• Vascular resistance
• Children lt8 years predominantly increase their HR
  to increase cardiac output (unable to increase
  stroke volume until gt10 years).

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Normal Vital Signs For Age
HR RR BP (systolic) Newborn
90-180 40-60 60-90 1 month 110-180 30-50 70-10
4 3 months 110-180 30-45 70-104 6
months 110-180 25-35 72-110 1 year
80-160 20-30 72-110 2 years
80-140 20-28 74-110 4 years
80-120 20-26 78-112 6 years
75-115 18-24 82-115 8 years
70-110 18-22 86-118 10 years
70-110 16-20 90-121 12 years
60-110 16-20 90-126 14 years
60-110 16-20 92-130
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Transition from Fetal Circulation

• Placental circulation is interrupted at birth
• Increase in systemic arterial blood pressure
• Spontaneous respirations
• Decreased pulmonary vascular resistance,
  increasing pulmonary blood flow
• Foramen ovale closes.
• Ductus arteriosus closes.
• This initial rapid change slows down over first
  24 hours of life.

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Cyanotic Heart Disease (CHD)

• Cyanotic Refractory to oxygen
• Right to left shunting
• Some lesions (e.g., TGA) are highly dependent on
  a shunt (VSD, PDA)
• Cyanosis usually presents shortly after birth.

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Cyanotic CHD

• 5 Ts
• Truncus arteriosus
• Tetralogy of Fallot (TOF)
• Transposition of the great arteries (TGA)
• Tricuspid atresia
• Total anomalous pulmonary venous return (TAPVR)
• Severe aortic stenosis
• Hypoplastic left heart
• Severe coarctation of the aorta

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Tetralogy of Fallot (TOF)

• Pulmonic stenosis
• Aortic override
• VSD
• RVH
• Right-to-left shunting through VSD dependent on
  severity of pulmonic stenosis

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Tricuspid Atresia

• RV is hypoplastic.
• Right-to-left shunt through VSD

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Total Anomalous Pulmonary Venous Return (TAPVR)
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Cyanosis

• Respiratory disorder
• Hemoglobin disorder
• Acrocyanosis (normal newborns) Cold stress and
  peripheral vasoconstriction
• Generalized or central cyanosis often due to
  cyanotic congenital heart disease. Often worsened
  by crying.

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Central Cyanosis vs. Acrocyanosis
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Hyperoxia Test

• Administer 100 oxygen.
• Significant increase in PaO2 seen with pulmonary
  and hemoglobin disorders.
• In CHD, PaO2 will not increase or it will
  increase slightly.
• Deoxygenated blood bypasses lungs and goes
  directly to left side of heart, diluting the
  fully oxygenated blood coming from lungs with
  deoxygenated blood.

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CHD

• Increased pulmonary vascularity
• Total anomalous pulmonary venous return
• Truncus arteriosus
• Transposition of the great arteries
• Other complex lesions without pulmonic stenosis
• Decreased pulmonary vascularity
• Tetralogy of Fallot
• Ebsteins anomaly
• Hypoplastic right heart, tricuspid atresia
• Complex lesions with pulmonic stenosis

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Prostaglandin E1

• Keeps the ductus open
• 0.05 to 0.1 mcg/kg/min with an increase to 0.2
  mcg/kg/min over several minutes
• Side effects Apnea, pulmonary congestion, fever,
  hypotension, seizures, and diarrhea
• Consider elective intubation.

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Noncyanotic CHD

• May present with CHF or heart murmurs heard
  during physical exam
• Left-to-right shunts
• Excess pulmonary vascularity
• ASD, VSD, PDA
• Obstructive lesions
• Aortic stenosis, coarctation of the aorta, mitral
  stenosis, pulmonic stenosis

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Clinical Features

• CHF Tachypnea, tachycardia, diaphoresis,
  decreased feeding, hepatomegaly, murmurs, gallop
  rhythms, pulmonary edema
• Decreased activity or poor sleeping with
  respiratory distress

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Diagnostic Studies

• CXR Cardiomegaly, pulmonary vascular congestion
• ECG Abnormal axis, ST segment changes
• Echocardiogram Definitive anatomic diagnosis,
  degree of congestive heart failure (chamber
  sizes, contractility)

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Management of CHF

• Give oxygen, assisted ventilation if needed.
• Elevate head and shoulders 45 degrees.
• Monitors, pulse oximetry
• Obtain IV access.
• Send laboratories.
• CXR and ECG
• Furosemide, nitroglycerin, digoxin
• Inotropes (dobutamine) for signs of shock

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Case Study 2Chest Pain, SOB

• 10-year-old boy presents with chief complaint of
  chest pain and shortness of breath.
• 5 days of cold and cough symptoms
• He has been lying around a lot and has missed 1
  week of school.
• Usually a very active child but complains that he
  is just too tired to play

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Initial Assessment

• PAT
• Abnormal appearance, abnormal breathing, abnormal
  circulation
• Vital signs
• HR 130, RR 44, BP 90/65, T 37.8C, O2 sat 90 on
  room air, increases to 100 on O2

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Initial Assessment

• A Patent
• B Intermittently shallow and deep rapid
  respiratory rate
• C Pale pulse rapid, thready, and weak
• D No focal deficits, GCS 15
• E No signs of injury

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Focused History

• O Chest hurts for several days.
• P Provoked by cough and exertion short of
  breath whenever he gets up and walks
• Q Burning, pressure
• R Substernal, some radiation to shoulders
• S 3 to 8 out of 10
• T Pressure and SOB last almost all day,
  exacerbations with exertion last 15 to 30 min.

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Detailed Physical Exam

• Neck Jugular venous distention supine
• Lungs Diminished breath sounds with occasional
  end expiratory wheeze with deep breaths
• Cardiac Distant heart sounds, no murmurs, S3
  gallop rhythm
• Abdomen Distended with palpable spleen and liver
• Neuro No focal deficits

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Question

• What is your general impression of this patient?
• What are your initial management priorities?

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General Impression

• Child is in respiratory distress and in
  cardiogenic shock.
• Demonstrates abnormal appearance with increased
  work of breathing and signs of shock.

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Management Priorities

• ABCs
• Give O2 by nonrebreather mask.
• Obtain IV access.
• Check rhythm on cardiac monitor.
• Obtain blood glucose, lab studies.
• Consider reducing preload and afterload with
  nitrates.
• Consider diuretic therapy.
• May need inotropic support.

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Case DiscussionDifferential Diagnosis

• Acquired cardiac problem
• Respiratory illness during winter months causing
  secondary myocarditis
• Congenital heart lesion that had been
  asymptomatic until this illness
• Anomalous coronary artery or valvular disease
• Pericarditis

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Clinical Features Your First Clue

• Consider myocarditis in any child with
• Weakness
• SOB
• Chest pain
• Especially if associated with preceding prodromal
  viral illness
• Distant heart sounds Silent Chest
• Enlarged heart on CXR

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Diagnostic Studies Myocarditis

• Radiology
• CXR will reveal cardiomegaly and prominent
  vasculature, perhaps even pulmonary edema
• Laboratory
• May not add much
• Not specific

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Differential Diagnosis What Else?

• Pericarditis
• Hypertensive crisis
• Anomalous coronary artery and myocardial
  ischemia/infarction
• Valvular disease
• Structural cardiac disease (e.g., VSD, ASD)
• Renal failure (e.g., glomerulonephritis)
• Rheumatic fever

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Management Myocarditis

• Gentle diuretic therapy
• Afterload reduction
• Possibly inotropic support
• Echocardiogram
• Intrinsic cardiac lesion?
• Muscle hypertrophy?
• Pericardial effusion?
• Decreased contractility?

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Case Progression Version 1

• CXR Cardiomegaly
• Echocardiogram Poor cardiac contractility
• Diagnosis Myocarditis
• Maintained on inotropes and pressor agents
• Recovered to a point that he could be discharged
  2 weeks later
• Will be followed closely to assess the degree to
  which he regains cardiac function

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Case Progression Version 2

• CXR Cardiomegaly
• Echocardiogram Poor cardiac contractility
• Diagnosis Myocarditis
• Deteriorates in ED
• Progressive shock
• Requires inotropic support
• Develops V-tach and V-fib
• Extracorporeal membrane oxygenation (ECMO)

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Myocarditis

• Inflammatory disease of the myocardium
• Direct infection of the myocardium (e.g., viral
  myocarditis)
• Toxin production (e.g., diphtheria)
• Immune response as a delayed sequela of an
  infection (postviral or postinfectious
  myocarditis)
• A common type of myocarditis is acute rheumatic
  fever (ARF).

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Acute Rheumatic FeverJones Criteria

• Major criteria
• Carditis Most commonly valvulitis
• Migratory polyarthritis
• Chorea, erythema marginatum, subcutaneous nodules
• Minor criteria
• Fever, elevated CRP or ESR, prolonged PR
  interval, arthralgia

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Pericarditis

• Pericardial inflammation
• Viral versus bacterial
• Bacterial causes include pneumococcus, S. aureus,
  H. influenzae type B
• Cardiac tamponade possibly requiring
  pericardiocentesis

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Pericarditis Clinical Features

• Chest pain
• Respiratory distress, CHF, or tamponade
• Precordial "knock" or rub (like the sound of
  shoes walking on snow)
• The classic signs include exercise intolerance,
  fatigue, jugular distension, lower extremity
  edema, hepatomegaly, poor distal pulses,
  diminished heart tones, and pulsus paradoxus.

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Endocarditis

• An infection of the endothelial surface of the
  heart, with a propensity for the valves
• Increased risk in children with artificial valves
  and patches, and patients with central lines
• 90 of cases are caused by gram-positive cocci.
• Alpha strep, Staph aureus, pneumococcus, group A
  ß hemolytic streptococci

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Endocarditis Clinical Features

• Fever
• Tachycardia, CHF, dysrhythmia, cardiogenic shock
• History of recent cardiac surgery or indwelling
  vascular catheter
• Heart murmur
• Petechiae, septic emboli, or splenomegaly

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Kawasaki Disease

• Vasculitis Propensity for coronary aneurysms
• Aneurysms may subsequently scar, resulting in
  coronary stenosis (early onset coronary artery
  disease).
• Coronary artery thrombosis and myocardial
  infarction
• Myocarditis, dysrhythmia

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Kawasaki Disease Clinical Features

• High fever
• Conjunctivitis
• Cervical lymphadenopathy
• Gingivostomatitis
• Polymorphous rash
• Swelling of the hands with erythema of the palms

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The Bottom Line

• Assessment of congenital heart disease can be
  challenging however, applying assessment skills
  with an understanding of normal physiology as
  well as pathophysiology of cardiovascular
  disorders in children will assist the clinician
  in management.

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