Chapter 30 Congenital Heart Defects v=KRy8gfmGSxg v=KRy8gfmGSxg.
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Transcript of Chapter 30 Congenital Heart Defects v=KRy8gfmGSxg v=KRy8gfmGSxg.
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Chapter 30Chapter 30Congenital Heart DefectsCongenital Heart Defects
•http://www.youtube.com/watch?v=KRy8gfmGSxg
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Cardiac Defects• Patent Ductus Arteriosus• Atrial Septal Defect• Ventricular Septal Defect• Tetralogy of Fallot• Transposition of the Great Arteries• Coarctation of the Aorta• Anomalous Venous Return• Truncus Arteriosus• Hypoplastic Left-Heart Syndrome
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Heart• Congenital heart disease (CHD) occurs in 1/125
live births.• Neonates may present with a variety of non-
specific findings, including: - tachypnea - cyanosis - pallor - lethargy - FTT - sweating with feeds
• More specific findings include: - pathological murmurs - hypertension - abnormal pulses - syncope
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Neonatal cardiac physiology
• The transformation from fetal to neonatal circulation involves two major changes:
1. A marked increase in systemic resistance.• caused by loss of the low-resistance placenta.
2. A marked decrease in pulmonary resistance.• caused by pulmonary artery dilation with the neonate’s first
breaths.
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•Fetal Circulation
•No circulation to lungs
•Foramen ovale
•Ductus arteriosum
•Circulation must go to placenta
•Umbilical aa., vv.
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Fetal cardiac physiologyFetal circulation:• Blood flows from the placenta
IVC RA through the PFO LA LV
• ascending aorta
• brain• returns via
the SVC
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Fetal cardiac physiologyFetal circulation:• From the SVC
RA RV
• pulm aa
• through the PDA• descending
aorta
• lower
extremities and
placenta
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Fetal cardiac physiologyFetal circulation: • Only a very small amount of
blood is directed through the right and left pulmonary aa’s to the lungs.
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Neonatal cardiac physiologyNeonate circulation:• The transformation to neonatal
circulation occurs with the first few breaths.
• The two remaining remnants of the fetal circulation are a patent foramen ovale...
• and ductus arteriosus.
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Congenital Heart Disease• Neonates with CHD often rely on a patent ductus
arteriosus and/or foramen ovale to sustain life.• Unfortunately for these neonates, both of these
passages begins to close following birth.– The ductus normally closes by 72hrs.– The foramen ovale normally closes by 3 months.– http://www.youtube.com/watch?v=FG-CNV501bc
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CHD
• That being said, in the presence of hypoxia or acidosis (generally present in ductus-dependent lesions), the ductus may remain open for a longer period of time.
• As a result, these patients often present to the ED during the first 1-3 weeks of life.
– i.e. as the ductus begins to close.
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Classifying CHD
• There are many different classification systems for CHD.
– None are particularly good.
• I will be discussing the Pink/Blue/Grey-Baby system:
1. Pink Baby – Left to right shunt2. Blue Baby – Right to left shunt3. Grey Baby – LV outflow tract
obstruction
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Pink Baby (L R shunt)
• L R shunts cause CHF and pulmonary hypertension.
• This leads to RV enlargement, RV failure, and cor pulmonale.
• These babies present with CHF and respiratory distress.
– They are not typically cyanotic.– http://www.youtube.com/watch?
v=46tmI2_RVuE&list=PLA81DD78BDE77FBFC
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Pink Baby (L R shunt)
•ASD
•VSD
• These lesions include (among others) ASD’s, VSD’s, and persistently patent ductus arteriosus.
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Pink Baby (L R shunt)
•Persistently patent ductus
arteriosus
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Pink Baby (L R shunt)• Diagnosing L R shunts depends on:
1. Examination findings:• Non-cyanotic infant in resp distress.• Crackles, widely-fixed second heart sound, elevated JVP, cor
pulmonale.2. CXR:
• Increased pulmonary vasculature (suggestive of CHF).• RA and/or RV enlargement.
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Pink Baby (L R shunt)• Initial management should be directed at
reducing the pulm edema.– Adminster Lasix 1mg/kg IV.
• Peds Cardiology/ PICU should be consulted urgently regarding use of:
– Morphine– Nitrates– Digoxin– Inotropes
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Ductus Arteriosus• Fetal Circulation Component
– Connects Pulmonary Artery to Aorta– Shunts blood away from lungs– Maintained patent by presence of prostaglandins
• Closure secondary to:– Increase in PaO2_
– Decrease in level of prostaglandins
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Patent Ductus Arteriosus
• 5-10% of all births (1 of 2000 live births)– 80% of premature babies
• 2-3 times more common in females than males.• 5th or 6th most common congenital cardiac defect.
– Often associated with other defects.– May be desirable with some defects.
• Morbidity/Mortality related to degree of blood flow through PDA.
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Pathophysiology - PDA
• With a drop in pulmonary arterial pressure (reduction in hypoxic pulmonary vascular constriction), blood will flow through PDA.– LEFT TO RIGHT SHUNT
• Increased pulmonary blood flow may lead to pulmonary edema.– Reduced blood flow to all postductal organs
• NEC
• If pulmonary artery pressure rises above Aortic pressure, blood will move in the other direction.– RIGHT TO LEFT SHUNT
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Diagnosis - PDA
• Loud grade I to grade III systolic murmur at left sternal border.– Washing machine
• Echocardiography
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Treatment - PDA
• Restrict fluids.• Diuretics• Prostaglandin Inhibitors - Indomethacin• Surgical closure (ligation).
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Atrial Septal Defect
• 6-10% of all births (1 of 1500 live births)• 2 times more common in females than males.• Types:
– Ostium Secundum (at or about the Foramen Ovale)– Sinus Venous
– In 1950 most children with ASD did not reach the first grade. Today, first year surgery facilitates normal growth and development.
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ASD: Pathophysiology and Diagnosis
• Pathophysiology– Left to Right Shunt
• Inefficient recirculation of good blood through pulmonary arteries.
– May not manifest symptoms and may be found later in life.
– If defect is significant, may cause problems later in life due to inefficiencies.
• Diagnosis– Murmur– Echocardiography
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Treatment - ASD• Surgical closure.• Non-Surgical closure via cardiac catheterization.
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Ventricular Septal Defect
• 1% of all births (2 to 4 of 1000 live births)– Vast majority the hole is small.
• In 1950, fatal. Today almost all VSD can be closed successfully, even in small babies. Lillehei was the first person in history to correct both ASD and VSD on 8/31/54.
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VSD: Pathophysiology & Diagnosis
• Pathophysiology– May be isolated or associated with other
congenital cardiac defects.– With normal PVR:
• LEFT TO RIGHT SHUNT
– With elevated PVR (RDS):• RIGHT TO LEFT SHUNT
• Diagnosis– Echocardiography
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Treatment - VSD
• Nothing if VSD is small.• With CHF or Failure to Thrive: Surgical closure.
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Blue Baby (R L shunt)• R L shunts cause hypoxia and central cyanosis.• Neither hypoxia or cyanosis tend to improve with
100% oxygen.• R L lesions include (among others):
– Tetralogy of Fallot (TOF)– Transposition of the Great Arteries (TGA)
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Blue Baby (R L shunt)
• Hypoxia and cyanosis (unresponsive to oxygen) in the neonatal period suggests a ductus-dependent lesion.
• Treatment is a prostaglandin-E1 (PGE1) infusion.– Dosing discussed momentarily
• This should obviously be accompanied by urgent Peds Cardiology and PICU consultation.
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Tetralogy of Fallot• Characterized by:
1. Pulmonary aa OTO2. RV hypertrophy3. VSD4. Over-riding aorta
• With severe pulmonary OTO...
•*•*
•*
•*• bloodflow to the
lungs may be highly ductus-dependent.
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Tetralogy of Fallot• The classic CXR finding in TOF is
the boot-shaped heart.
• Pulmonary vasculature is typically decreased.
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Tetralogy of Fallot• 1% of neonates.• Most common of the cyanoticcyanotic cardiac diseases.• Mortality increases with age (1 year-old has a 25%
mortality, 40 year-old has 95%).• In 1950, fatal. Today, less than 5% mortality with
children operated on in infancy, leading normal lives.Four Defects– Pulmonary Artery Stenosis (determinant factor related to
severity)– VSD (usually large)– Overriding Aorta– RV hypertrophy
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Tetralogy of Fallot: Diagnosis and Treatment• Tet Spells (Blue spells)
• CXR: Boot-shaped Heart• Diagnosed with echocardiography.• Surgical correction.
– Reparative or Palliative (Blalock-Taussig)
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Blalock-Taussig• Something the
Lord Made.– Vivien
Thomas
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Tetralogy of Fallot
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Transposition of the Great Arteries
• TGA is one of the most common cyanotic lesion presenting in the first week of life.
• Anatomically:– RV aorta– LV pulmonary aa
• To be compatible with life, mixing of the two circulations must occur via an ASD, VSD, or PDA.
• http://www.youtube.com/watch?v=O83cYwKOKtI&list=PLA81DD78BDE77FBFC
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Transposition of the Great Arteries
• The CXR findings in TGA are typically less dramatic than in TOF.
• Pulmonary vasculature is typically increased.
• http://www.youtube.com/watch?v=b-TkE_wygT4&list=PLA81DD78BDE77FBFC&index=1
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Complete Transposition of the Great Arteries
• Second most common form (5-7%) of congenital cardiac anomalies.
• Aorta arises from RV and Pulmonary Arteries from LV.
• Without an abnormality, life would not be possible.– ASD– VSD (30-40%)– PDA
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Transposition – Diagnosis and Treatment• Diagnosis
– Chest X-Ray: “Egg on a String”– Echocardiography– Cardiac Catheterization (?)
• Treatment– Balloon septostomy during
cardiac cath.• Rashkind’s Procedure• Reestablish Foramen Ovale
– Prostaglandin E1 to keep PDA open.
– Surgical Correction• Jantene Operation
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Grey Baby (LVOTO)
• Left-ventricular outflow tract obstructions (LVOTO’s) lead to cyanosis, acidosis, and shock early in the neonatal period.
• Complete obstruction is universally fatal unless shunting occurs through an ASD, VSD, or PDA.
• Examples of these lesions include:– Severe coarctation of the aorta– Hypoplastic left heart syndrome (HLHS)
• X
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Grey Baby (LVOTO)
• Treatment:– Any neonate presenting with shock unresponsive to
fluids +/- pressors has a LVOTO until proven otherwise.
– As with the Blue babies, appropriate management is an urgent PGE1 infusion and emergent consultation.
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Coarctation of the Aorta
• 7% of congenital cardiac defects.• Constriction of the aorta.
– Results in severely reduced blood flow.• Increased work on the heart leading to CHF
and cardiovascular collapse.• Location of narrowing determines the clinical
signs.• Usually associated with PDA, VSD and a
defective aortic valve.
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•http://www.youtube.com/watch?v=SiNJfvK_qeI
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Location of Coarctation
• Pre-Ductal– Less common but more serious– Associated with VSD, PDA, Transposition
• Post-Ductal– More common– Often associated with collateral circulation beyond
coarctation, which minimizes effect.– Diagnosed by a difference in blood pressure between
lower extremities and upper ones.• Pressure in upper extremities > lower
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Coarctation – Diagnosis and Treatment
• Diagnosis– Chest X-Ray – Echocardiography– Cardiac catheterization
• Treatment– Support with inotropic agents (Dopamine).– Prostaglandins to maintain PDA.– Surgical repair– http://www.youtube.com/watch?v=AGohu9fqKHg&list=PLF81322B9674D16CF
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Anomalous Venous Return
• Return of pulmonary venous blood to the right atrium instead of the left.– ASD is present to sustain life.– Can also be partial.
• Cyanosis usually present.• Diagnosed with echocardiography.• Surgical correction with reimplantation of
pulmonary veins.
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Truncus Arteriosus• Defect in which one large vessel arises from right and
left heart over a large VSD.• Cyanosis is often present.• CHF common.• Diagnosed with echocardiography and cardiac
catheterization.• Surgery:
– Separate pulmonary arteries from truncus.– Closure of VSD– Create valved connection between RV and Pulmonary
Artery– http://www.youtube.com/watch?v=HXlWeSGIR7A
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Repair of Truncus
Arteriosus
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Hypoplastic Left-Heart Syndrome• Several anomalies:
– Coarctation of the aorta– Hypoplastic left ventricle– Aortic and mitral valve stenosis or atresia.
• Cyanotic defect.• Right heart pumps blood to body through PDA.• Closure of PDA results in hypotension, shock, and
death.– Maintain hypoxemia with normalized CO2 levels.
• “40-40 Club”
http://www.youtube.com/watch?v=DcbiHP6zvus
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•
1 Patent foramen ovale
2 Coarctation of the aorta
3 Patent ductus arteriosus
4 Narrowed aorta5 Hypoplastic left
ventricle6 Aortic atresia
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Surgical Treatment of Hypoplastic Left Heart Syndrome
• Three separate surgeries.– Norwood procedure
• First few days after birth.– Glenn Shunt (Cavo
Pulmonary Connection)• 3-9 months of age
– Fontan Procedure • 2 years of age
– Less wait because of damage from pulmonary hypertension.
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Stage I - Norwood Procedure
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Stage II - Glenn Shunt
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Stage III – Fontan Procedure
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Prostaglandin-E1
• PGE1 promotes ductus arteriosus patency.
• Use an IV infusion at 0.05-0.1 ug/kg/min.• A response should be seen within 15 min.
– If ineffective, try doubling the dose.– If effective, try halving the dose.
• The lowest possible dose should be used– as adverse-effects of PGE1 can include:
- fever- flushing- diarrhea- periodic apnea (be ready to intubate)
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Remnants of Fetal Circulation
• Ligamentum teres = Round ligament– Remnant of the umbilical vein– Anterior abdominal wall
• Ligamentum venosum– Remnant of ductus venosum– On liver’s inferior surface
• Medial Umbilical Ligaments– Remnant of umbilical arteries– Anterior abdominal wall below navel– Also gives branch to urinary bladder
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Clinical Monitoring
• Blood pressure• Oxygen saturation• End tidal carbon dioxide
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Respiratory Care of Patients with Cardiac Anomalies
• Vascular resistance• Ventilator management• Inhaled nitric oxide• Subambient oxygen• Hypercarbia