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10/18/2012

CONGENITAL HEART DISEASE

Touching the Future of Children


October, 2012

Case Scenario #1
 A 16 day old infant is transferred to a Level IV NICU
 Hx: NSVD at 34 weeks, features of Trisomy 21 noted and
confirmed by karyotype, remained hospitalized due to
poor feeding, murmur noted on DOL #6
 Current: Tachypneic, hypotonic, poor weight gain

 Exam: Weight 15% below birthweight, RR= 90, mild-


mod retractions, pale, diaphoretic with crying

 What issue(s) might you suspect?


Case Scenario #2
 A term infant is delivered with prenatal diagnosis of
CHD
 Fetal echo report: Transposition of the great vessels with
a small-moderate inlet VSD. Foramen ovale appeared
non-restricted at time of echo
 Infant arrives to NICU at 17 minutes of age in RA, color is
slightly dusky with sat probe on left foot reading 71%.
Resp effort easy, good tone

 What are priorities of care at this time??

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10/18/2012

Case Scenario #3
 A 3 day old male infant is found limp and
cyanotic in Mom’s room on postpartum unit
 Hx: Poor PNC, C/S for breech, fed OK on DOL 1 & 2
but very sleepy today

 Taken to NBN and transferred to NICU

 Infant
tachypneic, no retractions, pale, gray color,
femoral pulses barely detected, Sats = 66% despite
100% BBO2

 What do you suspect to be the diagnosis?

Congenital Heart Disease

 Incidence
 6-8/ 1000 live births
 40,000children diagnosed per year
 25% considered “critical CHD”

 Classification: based on physiology of the


defect

Fetal Development

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Embryology
 Development occurs Day 18 through 12th week of
fetal life
 Heart beat detectable Day 21-25
 Stages:
 Cardiac tube
 Septation
 Valve formation
 Great vessel development

Teratogenesis
 85% attributed to multifactorial causes
 Genetic predisposition coupled with a causative factor

 Genetic factors
 Environmental factors
 AEDs: phenytoin, carbamazepine,
 Anticoagulants

 Antineoplastics

 Lithium

Teratogenesis
 Environmental factors
 Retinoic
acid
 Alcohol
– FAS
 Amphetamines

 Maternal disease
 Diabetes: 5 x greater risk with IDDM
 Maternal lupus
 Rubella

 CMV

 Maternal obesity

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10/18/2012

Cardiac assessment
 Color
 Heart rate and rhythm
 Resp rate and effort
 Blood pressures: 4 extremities
 Peripheral pulses
 Perfusion: central and peripheral
 Precordial activity
 Pulse oximetry
 Response to oxygen

CHD Screening with Pulse Ox


 Late detection of CCHD increases morbidity and
mortality
 2010: HHS recommends universal screening pulse
oximetry screening
 2011: Endorsed by AAP
 Directed at detection of specific lesions:
 HHLS, Pulmonary atresia, TOF, TAPVR, TGA, Tricuspid
atresia, Truncus arteriosus
 Will not detect all CHD: acyanotic, some left heart
defects

CHD Screening with Pulse Ox


 Must establish acceptable ranges and abnormal
threshold
 Perform after 24 hrs of life
 Late as possible with early D/C
 Use a motion-tolerant pulse oximeter
 Site: right hand and either foot (pre & post ductal)
 Simultaneous or sequential
 Performed by qualified personnel
 Timely evaluation of infants with abnormal screen

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10/18/2012

Nurse-driven algorithm

Hines (2012) Advances in Neonatal Care Vol. 12, No. 3

CHD Presentation:
 CHF
 Cyanosis
 Shock; decreased CO

 Murmur
 Tachypnea
 +/- resp. distress
 Dysrhythmia
 Abnormal heart size, shape, location

Terminology
 Murmur: Sound produced by turbulent blood flow

 May be: Nonpathological


Pathological

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10/18/2012

Murmurs
 Timing
 Systolic: between S1 and S2 of same beat
 Diastolic: between S2 and S1 of next beat
 Continuous: Starts in systole and ends in diastole

 Intensity: Grade 1 - 6
 I: barely audible
 II: soft, but audible
 III: moderate, but no thrill
 IV: loud and/or associated with thrill
 V: stethoscope barely touching chest
 VI: rare in countries with organized health care

Murmurs
 Nonpathologic / benign
 Common in first week of life
 Shortsystolic murmurs
 Grade I-II

 No associated s/s

 Normal CXR

Murmurs

 Pathological
≥ Grade 3
 Harsh quality
 Diastolic or continuous murmurs

 Abnormal S2

 Associated symptoms

 CXR
 Abnormal size or shape of heart
 Increased or decreased pulmonary vascular markings

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10/18/2012

Murmurs
 However…..
 ~20% of neonates with CHD do not have a murmur!

Physiology of absent murmur:


- Low turbulence
- Decreased ventricular function
- Elevated pulmonary vascular
resistance limits flow

Terminology
 Murmur: A sound produced by turbulent blood flow
 Nonpathological

 Pathological

 Shunt:
 via defect or persistent fetal structure
 Which direction?

Normal Heart

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10/18/2012

Shunt: Left to Right


 From left heart (or aorta) to right heart (or PA)
 A systemic to pulmonary shunt
 Oxygenated blood recirculated to right side of the heart

Shunt: Right to Left


 From right heart (or PA)
into left heart (or aorta)

 Deoxygenated blood
mixing into oxygenated
blood

Shunt: Right to Left


 A pulmonary to
systemic shunt

 Results in systemic
desaturation and
cyanosis

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10/18/2012

Most Common CHD

 VSD: Ventricular Septal Defect


 ASD: Atrial Septal Defect
 CoA: Coarctation of the Aorta
 PS : Pulmonary Stenosis
 AS: Aortic Stenosis
 TOF: Tetrology of Fallot
 TGV: Transposition of Great Vessels

Physiology of CHD
 Acyanotic
 Issue: Increased pulmonary blood flow

 Cyanotic
 Issue: Central cyanosis with low arterial saturation
 Not ductal dependent
 Ductal dependent

 Left outflow tract obstruction CHD


 Obstruction to systemic blood flow
 Issue: circulatory collapse / shock

CHD

18%

Acyanotic

Cyanotic

Left Heart Obstruction


24% 57%

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Physiology of CHD
 Acyanotic
 Issue: Increased pulmonary blood flow

 Cyanotic
 Central cyanosis with low arterial saturation

 Left outflow tract obstruction CHD


 Obstruction to systemic blood flow

Acyanotic CHD

 Left to right shunt

 Oxygenated blood re-circulated to pulmonary bed


 Volume and pressure overload

 S/S of pulmonary overload / CHF

 Long term risk of pulmonary hypertension

Ventricular Septal Defect

 Incomplete division of R
& L ventricles
 MOST common CHD
 Incidence 1-5:1,000

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VSD - types

 Perimembraneous
 Supracristal,
infundibular, conal, or
subpulmonary
 Inlet
 Muscular

Endocardial Cushion Defect


(AV Canal)
 Malformations in
development of
endocardial cushion
 Abnormal central heart:
 ASD
 VSD
 Tricuspid valve

 Mitral valve

Endocardial Cushion Defect


AV Canal
 Potential for mixing among
all 4 chambers

 NB period: PVR increased


 Balanced shunting or mild
cyanosis possible

 When PVR drops (2-4 wks)


 LR shunt dominates
 Pulmonary overload, CHF,

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10/18/2012

Acyanotic CHD: Clinical Presentation


 Varies with spectrum of defect
 Active precordium
 Resp. distress
 Diaphoresis
 Activity intolerance
 Resp infections
 Growth failure
 Murmur
 Hepatomegaly
 CXR: Possible cardiomegaly, RA, RV
enlargement, Increased pulmonary
markings

Acyanotic CHD: Management


 Monitor closely  Nutrition:
 NG feeds if fatigued
 Manage CHF:  High calorie formula
 Fluid restriction
 Inotropes: Digoxin,  SBE prophylaxis
Dobutamine  Immunizations
 Diuretics
 Oxygen

Palliative Surgery

 PA banding procedure

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10/18/2012

Acyanotic CHD: Repair

 Surgical repair
 Patch closure
 Valvuloplasty

 Timing varies

Categories of CHD
 Acyanotic
 Issue: Increased pulmonary blood flow

 Cyanotic
 Central cyanosis with low arterial saturation

 Left outflow tract obstruction CHD


 Obstruction to systemic blood flow

Cyanotic CHD
 Common Defects:
 Transposition of Great Vessels (TGV)
 Tetrology of Fallot (TOF)
 Truncus arteriosus
 TAPVR

 Require immediate intervention

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10/18/2012

Cyanotic CHD
 Cyanosis due to:
 Mixing of deoxygenated blood into oxygenated
blood

? shunt

OR

 Obstruction of pulmonary blood flow

Case Scenario #3
 Previously well newborn infant presenting with
cyanosis:

What is the differential diagnosis?

Previously Well NB

 Fetal shunts
 Ductus Arteriosus
 Foramen Ovale

 May allow compensation in


first days of life

Ductus Arteriosus

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10/18/2012

Fetal Shunts

 Significant deterioration
when shunts close

 Screening for CHD with


pulse ox before
discharge from NBN
 Endorsed by HHS and AAP

Foramen Ovale

Transposition of Great Vessels

 Aorta arises from RV


 Pulmonary artery from LV

 Parallel circulations

 Some shunt (VSD, PFO,


PDA) essential for survival

TGV

TGV with IVS TGV with VSD

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10/18/2012

TGV
 Presentation:
 Cyanosis

 Tachypnea

 Murmur

 CXR:
 Cardiomegaly

 “Egg on string”
 Increased PVM
 Narrow mediastinum

Transposition
 Diagnosis
 Echocardiogram

 Stabilization
 Prostaglandin E (PGE,
Alprostadil) to open ductus
arteriosus
 Maintain sats >75%

 Palliation
 Balloon septostomy
 If foramen is restricted
 Increases mixing

 Repair
 Arterial switch procedure

Tetrology of Fallot (TOF)


1. VSD
2. RV hypertrophy
3. Over-riding aorta
4. RV outflow tract
obstruction
(Spectrum of minimal >> severe
pulmonary stenosis / atresia)

“Pink Tet” or “Blue Tet” ??

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10/18/2012

Tetrology of Fallot (TOF)


 Presentation:
 Variable cyanosis

 Murmur

 CXR:
 Boot shaped heart
 Decreased PVM if pulmonary
outlet obstruction severe

“Pink Tet” or “Blue Tet” ??

Pulmonary Atresia
Severe Pulmonary Stenosis
 Pulmonary valve
obstruction
 RV hypoplasia
 Most have ASD or
Patent Foramen Ovale

 Pulmonary blood flow


via ductus arteriosus

Pulmonary atresia
Severe pulmonary stenosis
 Presentation
 Variable cyanosis at
birth
 Murmur

 Single S2

 Critically ill as ductus


arteriosus closes

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10/18/2012

Pulmonary atresia / TOF with PS

 Newborn Stabilization
 PGE (Alprostadil) to open ductus and provide
pulmonary blood flow
 Creates a __________ shunt through DA
 Maintain sats >75%
 Treat acidosis

 Respiratory support

Prostaglandin

 Prostaglandin E
 PGE1
 Prostin VR Pediatric®
 Alprostadil

PGE / Prostaglandin

 Maintains patency of ductus arteriosus


 Action: Causes vasodilation by direct
action on vascular and ductal smooth
muscle

 Half life 5-10 minutes: Continuous IV infusion


 Critical thinking???
 Metabolism: lungs → active metabolite
 Elimination: renal

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Administration

 Initial dose – 0.05 – 0.1 mcg/kg/min


 Assess closely for response (max effect ~30 min)
 Increased oxygenation
 Decreased metabolic acidosis
 Titrate dose to lowest effective dose:
 0.01 – 0.05 mcg/kg/min

Side effects
 Apnea
 Fever
 Flushing
 Rash
 Irritability
 Hypotension
 Bradycardia
 Muscle twitching
 Diarrhea
 Hypoglycemia
 Inhibits platelet aggregation

P. Atresia / TOF with P. atresia


 Palliation
 Blalock-Taussig shunt
 Modified BT shunt

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Categories of CHD
 Acyanotic
 Issue: Increased pulmonary blood flow

 Cyanotic
 Central cyanosis with low arterial saturation

 Left outflow tract obstruction CHD


 Obstruction to systemic blood flow
 Issue: circulatory collapse / shock

Left Heart Obstructive CHD


 Includes: CoA, Critical AS, Interrupted arch, HLHS

 Obstruction to aortic blood flow

 May initially look well

 Deterioration; CV collapse when ductus closes


 Newborn appears GRAY

 Ductal dependent for systemic blood flow


 Creates Rt to left shunt through DA

Coarctation of the Aorta

 Narrowing of aorta

 Common at area of DA

 May include
hypoplastic aortic arch,
other CHD

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10/18/2012

Coarctation – Presentation

 BP higher in upper
extremities
 Pulses greater in upper
extremities
 Decreased perfusion to
GI organs, kidneys

Coarctation - Surgery
 Left subclavian flap

Coarctation - Surgery
 Oblique resection

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10/18/2012

Aortic stenosis

 Obstructed aortic valve

 May have LV
hypoplasia and poor LV
function

 Ductal dependent for


systemic blood flow and
what else? ________

Hypoplastic left heart syndrome


(HLHS)
 Spectrum of conditions:
 small LV
 aorta & mitral valve atresia or
stenosis
 LA smaller than normal
 hypoplasia of ascending aorta &
arch
 Obstruction to systemic blood
flow
 LV output almost nil
 Ductal dependent CHD

Left Heart Obstruction


 Goals:
 Improve systemic
perfusion
 Reverse effects of
shock, acidosis
 Treat CHF
 Balance PVR and SVR

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Left Heart Obstruction


 Balance PVR and SVR
 Avoid pulmonary
vasodilation
 Cautious administration of O2
 Vigilant fluid balance
 Avoid systemic
vasoconstriction
 Avoid inotropes with alpha
effect
 May use afterload reduction
 Milrinone, Nipride

 Vigilant fluid balance


 Monitor renal and GI status

Left Heart Obstruction


 Stabilization:
 Ventilation
 PGE infusion
 Maintain sats 75-85%
 Treat acidosis
 Cautious volume
resuscitation
 Inotropes for poor
myocardial fxn
 Dobutamine
 Milrinone

Categories of CHD
 Acyanotic
 Issue: Increased pulmonary blood flow

 Cyanotic
 Central cyanosis with low arterial saturation

 Left outflow tract obstruction CHD


 Obstruction to systemic blood flow

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10/18/2012

General Nursing Care


 Understand patient’s defect
 Blood flow patterns
 Expected issues: CHF?, Cyanosis?
 Plan of care: palliation?, staged repair, full repair?

 Know baseline vital signs


 Normal heart rate, BP for status
 Baseline O2 saturation, blood gas values

General Nursing Care


 IV Access
 Unrepaired Cyanotic or Obstructive Defects:
NO AIR IN IV LINES!
Risk of air embolus

 Oxygen administration
 Understand physiology and possible effects
 Discuss plan for acute desaturation

General Nursing Care

 Correct metabolic acidosis

 Ensure adequate fluid balance

 Monitor urine output, renal function

 Prevent cold stress in infants

 Involve and support parents

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Summary: CHD
 Acyanotic
 Often with increased pulmonary blood flow

 Cyanotic:
 Mixing – OR - decreased pulmonary blood flow

 Left outflow tract obstruction CHD


 Obstruction to systemic blood flow

Case Scenario #1
 A 16 day old infant is transferred HMC from another
NICU.
 Hx: NSVD at 34 weeks, features of Trisomy 21 noted and
confirmed by karyotype, remained hospitalized due to
poor feeding, murmur noted on DOL #6
 Current: Tachypneic, hypotonic, poor weight gain

 Exam: Weight 15% below birthweight, RR= 90, mild-


mod retractions, pale, diaphoretic with crying

 What issue(s) might you suspect?


Case Scenario #1
 A 16 day old infant is transferred HMC from another
NICU.
 Differential diagnosis:

 Care:

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10/18/2012

Case Scenario #2
 A term infant is delivered with prenatal diagnosis of
CHD
 Fetal echo report: Transposition of the great vessels with
a small-moderate inlet VSD. Foramen ovale appears
non-restricted at time of echo
 Infant arrives to NICU in RA, color sl dusky with sats =
71% in RA. Resp effort easy, good tone

 What are priorities of care at this time??

Case Scenario #2
 A term infant is delivered with prenatal diagnosis of
TGV
 What are priorities of care at this time??

Case Scenario #3
 A 3 day old male infant is found limp and
cyanotic in Mom’s room on WH unit
 Hx: Poor PNC, C/S for breech, fed OK on DOL 1 & 2
but sleepy today

 Taken to NBN and transferred to NICU

 Infant
tachypneic, no retractions, pale, gray color,
femoral pulses barely detected, Sats = 69% despite
100% BBO2

 What do you suspect to be the diagnosis?

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10/18/2012

Case Scenario #3
 A 3 day old male infant is found limp and
cyanotic in Mom’s room on WH unit

 Thank you!

References
 Kaplan et al.. 2005. Effect of Prenatal Diagnosis on Outcome in Patients With Congenital Heart
Disease. Neoreviews; 6: 326-331.
 Karlsen, K. & Tani, L. STABLE Program: Cardiac Module: Recognition and stabilization of
neonates with severe CHD. 2003. The Stable Program, Park City, UT.
 Khoo, N.S. et al. 2008. Effectiveness of Prenatal Diagnosis of Congenital Heart Defects in South
Australia: A Population Analysis 1999-2003. Australian and New Zealand Journal of Obstetrics
and Gynaecology (Volume 48 Issue 6, December 2008)
 Khoshnood, B. et al. 2005. Trends in Prenatal Diagnosis, Pregnancy Termination, and Perinatal
Mortality of Newborns With Congenital Heart Disease in France, 1983–2000: A Population-
Based Evaluation PEDIATRICS Vol. 115 No. 1 January 2005, 95-101.
 Knight, S. & Washington, R. 2006. Cardiovascular Diseases and Surgical Interventions. In In
Handbook of Neonatal Intensive Care. 6th edition. Merenstein & Gardner, eds. Mosby Elsevier.
 Meckler GD, Lowe C. To intubate or not to intubate? Transporting infants on prostaglandin E1.
Pediatrics. 2009;123:e25—e30.
 Sadowski, S. 2010. Cardiovascular Disorders. In Core Curriculum for Neonatal Intensive Care.
4th edition. Verklan & Walden, eds. Saunders Elsevier.
 Sendelbach DM, Jackson GL, Lai SS, Fixler DE, Stehel EK, Engle WD. Pulse oximetry screening at
four hours of age to detect critical congenital heart disease. Pediatrics. 2008;122:e815–e820.

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