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PEDIATRIC HEART DISEASE Fetal Heart Sounds Powered By Docstoc
					                   PEDIATRIC HEART DISEASE
   1% of population has congenital heart dz
   Pathophysiology: review fetal circulation
   Note that newborn heart is stiff and functions like diastolic dysfunction (rapid rates
     decrease diastolic filling time)
   Congenital heart disease often misdiagnosed as respiratory illness but be aware that
     they often can co-occur: congenital heart dz that is exacerbated by the respiratory

   Congenital
                    Congenital Heart Disease
                    Cardiomyopathy
                    Congenital arrythmias (Accessory pathway —> SVT; Congenital heart
     Acquired
                    Myocarditis
                    Pericarditis
                    Cardiomyopathy
                    Cardiac tumors
                    Toxicologic
                    Traumatic
                    Volume overload
                    Infectious: Kawasaki’s
                    Ischemic
                    Acquired arrythmia: HB

   History
              Lethargy
              Poor feeding
              Dyspnea during feeding (marker of increased pulmonary venous
              Distress with feeding (exercise)
              Irritability
              Failure to thrive/poor weight gain
              Respiratory distress (CVS MUST BE ON DDX)
              Excessive sweating
              Hemoptysis (pulmonary congestion)
              Syncope in older kids

     Physical Examination
                  Color
                    -       Pink: acyanotic heart dz, left to right shunts
                    -       Blue: cyanotic heart dz, right to left shunts
                    -       Gray: shocky, LVOTO
                  Vitals: Tachypnea in absence of lung disease, Tacchycardia, Hypoxia,
                    Diaphoresis, hypotension
                  CVS: central cyanosis, murmurs, gallop rhythm, single heart sound,
                    overactive precordium,
                  Chest: crackles and wheezing (MUST be on ddx of wheeze)
                  General: periorbital edema common (ask about puffy face)
                  Poor peripheral pulses, pulse difference b/w upper and lower extremities
                  Abd: hepatomegaly common (ddx is hyperinflation with asthma or
                    bronchiolitis or infiltrative liver disease; may be absent early);
                    splenomegaly uncommon with CHF and more indicative of infection
                  NOTE: NO ankle edema or increased JVP in infants
    Cyanosis
                  Bluish color of skin, mucous membranes, and nail bed from deoxygenated
                  Central: mucous membranes, tongue, nail bed (not lips);
                  Peripheral:
                  Remember fetal Hb pushes curve to the left thus Pa02 of 40 - 50 may be
                    enough to maintain sats at 95%

                     EXAM FOR ? CONGENITAL HEART DZ
                     Cardiac murmurs
                     Look for hepatomegaly
                     Decreased pulses to legs

   Cap gas, BW (Hb, pH)
   CXR
                 Cardiomegaly (cardiothoracic ratio > 0.6)
                 Cardiomegaly almost always present: absence essentially r/o CHF
                 Absence of lung pathology
                 Increased vascular markings
                 Bootshaped heart = Tetrology of Fallot
                 Look for RA, LA, LV, and pulmonary artery hypertrophy
   Hyperoxia Test
                 Put infant on 100% Fi02
                 Improvement suggest respiratory etiology, none suggests cardiac
                 Pa02 increase to > 150: pulmn cause
                 Pa02 b/w 100 - 150: likely cardiac
                 Pa02 < 100: cardiac cause
   ECG
                 Look for atrial and ventricular hypertrophy signs
                 Look for abnormal axis
                 Arrythmias
                 Ischemic changes: ST wave changes are not good markers of ischemia;
                   look for ST elevation, prolonged Qtc
   Troponin
                    Very little data in ED patients
                    Normal ranges are essentially unknown
     Echo is best initial diagnositic test
     Angiography sometimes required

   Features
               PINK
               Acyanotic
               Resp Distress re congestive heart failure
               Relatively well perfused
               Left to Right Shunts
   Causes = AVAPCA
               ASD
               VSD
               AVSD
               PDA
               Coarctation
               Aortic Stenosis
   ASD
               Left to right shunting causes CHF
               Severity depends on the location of shunt and the amount of shunting
               A small ASD has minimal shunting and minimal or no symptoms
               Presents later with pulmonary hypertension
               Murmur = pulmonic flow murmur
               HS = wide, fixed split S2
   VSD
               Larger pressure gradients than ASD thus larger amount of shunting
               Small VSD: minimal shunt and usually asymptomatic
               Larger VSD: loud murmur, may have chf and biventrictulra failure
               Eventually leads to elevated right sided pressure, and shunt reverses thus
                 becoming right to left and cyanotic (Eisenmenger’s)
   AVSD
               Endocardial cushion defect
               Associated with Down’s syndrome
               ECG with northwest axis
               Risk of Eisenmenger’s syndrome
   Coarctation
               Definition = constriction of the aortic arch
               95% are beyond the take off of the subclavian
               Slow closure of ductus, incomplete obstruction of aorta presents with CHF
                 or hypertension etc (Acute, complete obstruction presents as Gray baby)
               Associations: bicuspid ao valve, fhx, Turners, VSD
               Complications: CHF, HTN, RF, ICH, Endocarditis, AoDissection,
               Late presentations
                          -      Hypertension
                          -      Poor leg pulses
                          -      Leg claudication
                           -        Headaches
                           -        ICH
                           -        Ao Dissection
                           -        Renal failure from HTN, poor circulation
                           -        Epistaxis
                           -        Gradual onset CHF
                  EXAM: SBP > 140 in infant, poor leg pulses, BP diff b/w arms, SEM over
                    back, no pulsox in toes
                  Labs: renal failure
                  CXR: notched ribs, big heart, chf
                  ECG: LVH
                  Dx: echo, suprasternal doppler
    PDA
                  Usually closes 2-3 wks
                  May close up to 5-6 months
                  Mumur is machinery continous murmur left ICS 2
                  Large: presents early with CHF, PHTN
                  Small: presents late with endocarditis, murmur
                  Mx: indomethicin, cath, scope closure
                  closure of Less severe coarctations present with hypertension, poor
                    peripheral pulses and acyanotic heart disease (chf secondary to
                    increased afterload from coarct)
                  Coarctation can be preductal or post ductal
                  Severe coarctations present as LVOTO as duct closes

   Features
                 Cyanosis with No or minimal respiratory distress
                 Relatively well perfused
                 Right to left shunt
   Causes = the 5 T
                 Tetrology of Fallot
                 Tricuspid atresia
                 Truncus arteriosus
                 Transposition of the great vessels
                 Totally anomalous venous return
   Compare to pulmonary related cyanosis
            Dyspneic                             Minimal dyspnea
            Appear ill                           Minimal distress
            Improves with oxygen                 Doesn’t improve with oxygen

    Tetrology of Fallot
                   Features
                            -      VSD
                            -      RVH
                            -      Subpulmonic stenosis
                            -      Overriding aorta
                   Pathophysiology
                            -      Right to left shunt through the VSD
                              -       The patent ductus arteriosus is main source of pulmonary
                                      blood flow (aorta -> PDA –> lungs)
                              -       Closing of ductus can lead to worsening
                     Features
                              -       CXR with bootshaped heart (note that thymic involution
                                      can mimic this appearance)
                     Tet Spells
                              -       Hypercyanotic
                              -       Results from an abrupt increase in pulmonary resistance
                                      thus decrease pulmonary flow and increased right to left
                              -       Can occur for many reasons but agitation, pain are
                              -       Management is aimed to increase systemic pressure to
                                      increase the flow through the lungs

                  MANAGEMENT OF TET SPELL
                  K   Knee to chest (increases systemic pressure) (fist to
                      abdomen can also be done)
                  N   Normal saline bolus 10 ml/kg
                  O   Oxygen
                  P   Phenylephrine 10 ug/kg iv bolus then 2 - 5 ug/kg/min
                  E   Esmolol 500 ug/kg iv bolus then 100 ug/kg/min

                  M       Morphine 0.1 mg/kg for pulmonary vasodilation ???
                  B       Bicarb 1 mEq/kg iv bolus

    Features
                  Looks GRAY because of shock
                  Presents early in neonatal period
                  Cyanotic with very poor perfusion
    Causes
                  Hypoplastic left heart (Mitral + Aortic atresia)
                  Severe Coarctation
                  Severe Aortic Stenosis
    Pathophysiology
                  The patent ductus is the life - line as blood flows through RV/PA into the
                    ductus and then into the systemic circulation
                  When the ductus closes, there is no means to perfuse the systemic
                    circulation and the rapid onset of shock, cyanosis develops
    Coarctation
                  Coarcts present as LVOTO when they are severe enough to have
                    significant limitation on the outflow from the LV
                  They present when the ductus closes
                 Sudden, complete obstruction of aortic flow presents with Gray baby

  General Mx
                 Oxygen
                 Intubate/ventilate
                 Volume: avoid unless hypotensive: WORSENING WITH FLUID IS A CLUE
                 Early cardiac referral: discuss case, pink/blue/gray and get
                   recommendation on initial managment
                 Worsening with ventolin is a CLUE (won’t help cardiac wheeze and may
                   make worse b/c of tachycardia)
   Lasix
                 Dose 1 mg/kg
                 Watch K+
   Digoxin
                 Good for primary myocardial disease
                 Loading: 20 ug/kg (premies) - 40 ug/kg (infants) iv over 24 hrs
                 Maintenance: 5 ug/kg (premies) - 10 ug/kg (infants) iv over 24hrs
                 Dig toxic if levels ? 2 ng/ml
                 Watch for dig toxic ECG changes
   ACE-I
                 Afterload reduction, decrease MV02, increase renal blood flow
                 Watch for hypotension, increase in Scr and K+
                 Captopril
   Ionotropes
                 Dopamine
                 Dobutamine
   Prostaglandin E1
                 Bolus: 0.1 ug/kg/min
                 Infusion: 0.05 - 0.1 ug/kg/min
                 MOA: opens ductus arteriosus by smooth muscle relaxation in pulmonary
                   vascuar bed and systemic circulation
                 Side - effects: apnea, hypotension, fever, bradycardia, seizure
                 Contraindications = NONE
                 General Indication: any infant in first week with decreased perfusion,
                   hypotension, or acidosis should be considered candidate for PGE1 infusion
                 Systemic Duct dependant lesions (Gray baby)
                           -        Hypoplastic left heart (allows systemic circulation)
                           -        Severe coarctation (allows systemic circulation)
                           -        Severe aortic stenosis
                 Pulmonic Duct dependant lesion (Blue baby)
                           -        Hypoplastic right heart (tricuspid and pulmonic atresia)
                           -        Tricuspid atresia
                           -        Pulmonic atresia
                           -        Severe pulmonic stenosis
                           -        Tetrology of fallot with decreased pulmn flow
                         Resp Distress re failure
                         Relatively well perfused
                          (Left to Right Shunts)

Coarctation (mild)
Aortic Stenosis (mild)

                         No/minimal resp distress
                         Relatively well perfused
                          (Right to Left Shunts)
THE 5 Ts
Tetrology of Fallot
Tricuspid Atresia
Totally anomolous PVR
Transposition of GA
Truncus Arteriosus

(Hypoplastic right heart = tricuspid and pulmonic atresia)

                                     Poor perfusion

Coarctation (severe)
Hypoplastic Left Heart
Aortic stenosis (severe)
                         PEDIATRIC SYNCOPE
   50% of adolescents have at least one syncopal episode
   Cardiac
   Resp
   Neurologic
   GI
   Psychogenic
   Not syncope: Seizure, hypoglycemia, breath-holding

   By far the MCC in pediatric syncope
   Preceding pallor, diaphoresis, nausea, visual changes
   ECG is normal
   BHCG if reproductive age
   No further work up required
   Tilt table testing if recurrent
   Occasionally put on florinef

   Sudden, no warning, no presyncopal symptoms
   Syncope at rest or with exertion is worrisome
   MCC: HOCM, AS, arrythmias, cardiac tumors

   General
                  Abnormally long repolarization thus second impulse can hit during relative
                    refractory period and stimulate reentrant pathway
                  Torsades often, but not always, occurs in setting of prolonged QT which is
                    a marker of abnormal ventricular repolarization
                  Anything that increases QT is contraindicated: Ia, Ic, TCA, haldol, etc
                  Definition is UNKNOWN; Qtc > 440 is suspicious, > 460 usually
                    considered diagnostic; risk of torsades doesn’t increase that much until >
     Pause - dependant Torsades (Majority)
                  Precipitated by SLOW heart rate
                  Etiology
                            -       Lytes: Magnesium, hypokalemia, hypocalcemia
                            -       Drugs: Ia, Ic, TCA, Haldol
                            -       Severe brady or AV block
                            -       CVA
                            -       Myocardial ischemia
                            -       Hypothyroid
                            -       Contrast
                  Treatment
                          -       Goals = correcting underlying metabolic/lyte problems then
                                   increase HR to shorten the ventricular repolarization
                                  (HR>100 - 120)
                           -      Increase HR to prevent recurrence: overdrive pacing,
                           -      Mg 2 - 4 gm iv: prevent or terminate
                           -      Lido/phenytoin: little evidence, shortens repolarization
   Adrenergic Dependant Torsades
                 Rare, younger
                 Precipitated by catecholamine excess
                 Congenital: Romano-Ward (not deaf), Lang-Neilson (deaf), sporadic, MVP
                 Acquired: SAH, CVA, autonomic surgery (vagotomy, carotid endart, neck
                 Prevention: BB decreases HR to prevent sympathetic surges;
                   ganglionectomyies or sympathectomies have been done
                 ICDs may be needed
                 Acute treatment: magnesium, defibrillation, NO amiodarone
   Long QT syndromes
                 Romano-Ward: normal hearing
                 Lange-Nielsen: deaf
                 Arrythmia precipitants: stress, emotions
   Acquired long QT
                 Anorexics can be malnourished enough to be Mg depleted

                       RED FLAGS FOR PEDIATRIC SYNCOPE
                      Exertional chest pain
                      Exertional syncope
                      Syncope at rest or recumbency
                      Absence of warning symptoms
                      Fhx of sudden cardiac death
                      Fhx of cardiac disease
                      Prolonged QT
                      Abnormal cardiac exam
   HOCM - MCC in athletes
   Heat stroke - 2nd MCC in athletes
   Myocarditis
   Dysrhythmogenic right ventricular dysplasia: fibrosis of RV, risk of Vfib/Vtach
   Coronary artery anomalies: obstruction of coronary flow during exertion; warning is
     exertional chest pain or syncopal episodes
   Prolonged QT syndromes
   WPW
   Cardiac concussion (Commotio cordis)
   Miscellaneous congenital heart dz
   Idiopathic LVH
   Toxicology: cocaine, amphetemines

    Exertional chest pain and syncope are warning symptoms
    Palpitations
    ECG
                   Normal variants: sinus brady, 1st and 2nd degree AV blocks
                   Signs of LVH
                   RBBB, LBBB, RVH, prolonged QT
                   Flipped T waves are not predictive
    Investigations
                   CXR
                   ECG
                   Echo
                   24hr monitor
                   Stress thallium
                        PEDIATRIC CHEST PAIN
     4% of pediatric chest pain has a cardiac cause
     Cardiac causes
                    Pericarditis
                    Myocardititis
                    Cardiac ischemia
                    HOCM
                    Congential heart disease
                    Anomalous coronary arteries
     Non-cardiac
                    MSK and chest wall (costochondritis)
                    Pulmonary: pneumonia, pleuritis, asthma, bronchitis
                    GI
     Concerning history
                    Exertional chest pain
                    Chest pain with syncope

  See adult notes
  Children of all ages
  MCC of dilated CM
  Coxsachie, echovirus, influenza, varicella, EBC etc
  Fever, chest pain, dyspnea, wheezing
  Wheezing that doesn’t respond to bronchodilators is a clue
  Younger kids: poor feeding, sweating, grunting, lethargy
  Can be in fulminant CHF and cardiogenic shock
  Cardiac enzymes often elevated
  ECG, CXR, Enzyme, Echo all required
  Biopsy required for definitive dz
  Mx of chf: lasix, ACE-I, discuss dig with cardiologist
  Mx of cardiogenic shock: intubation, dopamine

   DCM post myocarditis is the most common
   HOCM is the second most common: see adult notes

   Present to ED with infections, graft rejection, medication problems, chf
   Rejection: lethargy, poor feeding, fever, CHF, arrythmias
   Infections are opportunistic infections (as per adults)
   Same ddx as adults but post viral is the MCC by far
   Significant effusions are rare but CAN occur and produce tamponade
   Usually self limited course and resolve with antiinflammatories

   Usually in kids with congenital heart disease
   Presents like viral syndrome: fever, arthralgias, myalgias, weakness
   Skin findings are not common in kids
   Think about prophylaxing them for procedure in the ED
                   Prosthetic valves
                   Prior endocarditis
                   Complex cyanotic malforamtion
                   Surgical systemopulmonary shunts
                   HOCM
                   MVP with regurg
                   Rheumatic or acquired valvular dysfunction
                   Most other complex congenital malformations

      Prophylaxis NOT recommended
                    Isolated ASD
                    Repaired ASD, VSD, PDA without problems after 6 months
                    Innocent murmurs
                    Previous kawasakies or rheumatic fever without valve dysfunction
                    Pacemakers and ICDs
      Procedures requiring prophylaxis
                    Dental procedures with any bleeding
                    T&A
                    Surgery on intestine or respiratory mucosa
                    Rigid Bronchoscopy
                    Cystoscopy
                    Urethral catheterization (mc in ED)
                    Vaginal delivery with infection

  Acute vasculitis primarily of infants and children
  Majority occur < 5 yo; 1-2 yo is most common; RARE in adults
  Myocarditis is the most common cause of death
  Clinical phases
                   Phase I: acute phase (1-2wks); fever, myocarditis, pericaridal effusions
                   Phase II: subacute phase (1-4 wks): fever/rahs/lymph nodes resolve and
                     conjucntivitis persist; desquamation/arthritis/myocardial dysfunction occurs
                   Phase III: convalescent phase; clinical findings resolve, continues until ESR is
                     normal 6-8 weeks after onset
  CDC Diagnostic Criteria = Unexplained fever for 5 days + 4/5 criteria
                                    C        -       Conjunctivitis: bilateral w/o exudate
                                    R        -       Rash
                                   E        -       Extremity changes (red, swollen, desquamating)
                                   A        -       Adenopathy (cervical ln, at least one > 1.5 cm)
                                   M        -       Mucous membrane lesions (injected
   MCC of pediatric acquired heart dz:
                  Myocarditis
                  Coronary artery aneurysms: occurs in 20% of untreated cases; prone to MI or
                    coronary rupture and tamponade
                  Coronary vasculitis
                  Pericarditis
                  CHF
                  Pericardial effusions
                  Valve dz
                  Arrtyhmiasose < 6 months were diagnosis is more difficult
   Investigations
                  Admit for echo (all need): actually can see aneuryms
                  Do ECG, may need coronary angiogram
                  CBC, lytes, creatinine, ESR, CXR, blood culture, urinalysis, ASOT titer, throat
                    swab, LP if signs of aseptic meningitis
   Managment
                  IV gamaglobulin: 2 g/kg over 12 hours (may need further treatments)
                  High dose ASA: 100 mg/kg/day divided into q6hr dosing
                  Therapy decreases incidence of coronary aneurysm
   Introduction
                  Sporatic cases of Strep pharyngitis rarely cause rheumatic fever (RF)
                  Most cases occur with epidemics of Strep pharyngitis
                  Children 4yo - 8yo are at greatest risk
   Clinical Features
                  Many will not remember preceeding pharyngitis
                  RF occurs after latency of 1 - 5 weeks (average 2weeks) post pharyngitis
                  Various combinations of migratory polyarthritis, fever, chorea, nodules,
   Jone’s Criteria
                  Requires evidence of preceeding Streptococcal infection (+ve throat
                    cultures or ASOT) and two major or one major + two minor criteria
                  MAJOR CRITERIA
                            -        Subcutaneous nodules
                            -        Pancarditis
                            -        Arthritis (polyarthritis)
                            -        Chorea
                            -        Erythema marginatum
                  MINOR CRITERIA
                            -        Arthralgias
                            -        CRP elevation
                            -        ESR elevation
                            -        PR prolongation
                            -        Fever
   Carditis
                  40% with pancarditis
                   Heart murumur, cardiomegaly, pericardial effusion, occasional CHF, mitral
                     valve is most common,
                   MR most common
                   AR is 2nd most common
     Laboratory
                   Elevated ESR and CRP nonspecific
                   Antistrep antibody (ASOT) +ve for 4-6 wks
                   Throat cultures often negative by the time of rheumatic fever
                   Urine with protein/casts in 50%
                   ECG: PR prolongation
     Treatment
                   Penicillin
                   NSAIDs
                   Manage CHF
                   Corticosteriods for carditis (not arthritis)
                   Penicillin V or erythromycin po bid X 5 year for prophylaxis

                        PEDIATRIC MURMURS
   Levine’s grading
                      1       quiet
                      2       barely heard
                      3       loud
                      4       loud + thrill
                      5       stethoscope on edge
                      6       stethoscope off chest
   Many innocent murmurs disappear when child sits up

    LLSB and apex
    Buzzing or vibrating quality
    Decreases in sitting position
    Usually grade 1,2 but can be grade 3
    LV tendons or bands is a common normal variant that can cause it

   2nd left ICS close to sternum
   Short mid-sydtolic murmur
   Associated with straight back syndrome and pectus excavatum
   Decreases in sitting position

   Sounds like aortic stenosis
   No ejection click
     Disappears when shoulders are hyperextended

   Upper sternal borders
   Can be loud (3/6)
   Very dependant on patient position
   Pressure over the jugular vein on that side decreases the murmur

   Heard over the breast
   Increase blood flow to breast in pregnancy

                                                  FEATURES OF INNOCENT HEART
                                                    Early systolic
                                                    Not very loud
  Early systolic
                                                    Normal CXR
  Not very loud
                                                    Normal ECG
  Normal CXR
                                                    Normal child
  Normal ECG
                                                    S3 or S4 gallop
  Normal child
                                                    Varies with position

                      Late systolic
                      Pansystolic murmurs (VSD, MR)
                      Loud murmurs with thrills
                      Diastolic murmurs
                      Continuous murmurs (other than
                        venous, mammary)
                      Abnormal CXR, ECG, child
                      S3 or S4
                      Doesn’t vary with position

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Description: PEDIATRIC HEART DISEASE Fetal Heart Sounds