RNC cardiac reviewElizabeth Rex, MS, NNP-BC
Outline
Transition
Embryology
Review Cardiac Basics
Review of Cardiac Assessment: Look, Listen, Feel Blood Pressure
Outline
Pathophysiology Shock
Cardiac Tamponade
Congestive Heart Failure
Outline
Congenital Heart Defects Overview
Diagnosis
Outline
Congenital Heart Defects Defects
• AV Canal
• Coarctation of the Aorta
• HLHS
• Pulmonary stenosis/Atresia
• TOF
• TGA
• TAPVR
Outline
Other Cardiac Content Lines
NCC Cardiac Content
♥ Transition to extrauterine life
♥ PDA
♥ CV AssessmentBPCVPEKGMonitoring Lines
♥ Cyanosis Central / PeripheralCardiac / Pulmonary
♥ Arrhythmias
♥ Congestive Heart Failure
♥ Hypertension
♥ Shock
♥ Cardiac Tamponade
♥ Anomalies (Cyanotic / Acyanotic)
AV Canal
Coarctation of aorta
HLHS
Pulmonary stenosis/atresia
TOF
TGA TAPVR
Fetal Circulation: Quick Overview
Gas exchange is liquid to liquid Organ of respiration is placenta High flow, low resistance
Fetal lungs Low flow, high resistance
PA’s constricted High right heart and lung pressures Low left heart pressures 3 Open fetal shunts
Placenta
Site for gas exchange in fetus (liquid to liquid)
Very vascular, large surface area
High flow/low resistance circuit
Responsible for delivering nutrients to and carrying waste products away from fetus
Fetal Lungs
Fluid-filled in utero
Low-flow, high-resistance circuit
Only 10% cardiac output flows to lungs
Only need blood flow for growth
Fetal Pulmonary Vessels
Greater amount of smooth muscle compared to adults Increases tone of vessels, increases
resistance to flow
Constrictor response (reactivity) of smooth muscle is great (hypoxia)
Number of vessels increases during fetal life Decreases resistance to flow
Open Fetal Shunts
There are three shunts present in utero:
Ductus Venosus
Foramen Ovale
Ductus Arteriosus
Purpose: shunt blood away from lungs
Fetal Circulation in Detail
Ductus Venosus
Majority of blood passes through DV (first of the 3 shunts)
From Umbilical Vein to IVC
Blood then flows into Right Atrium
Fetal Circulation in Detail Foramen Ovale
Majority of blood (> 50%) enters RA & flows through FO to LA (second of 3 shunts)
Bypasses the lungs (2o to high PVR) so oxygenated blood gets to upper body through LV and aorta
Pressure relationships keep FO open
Fetal Circulation in Detail
Ductus ArteriosusBlood that does go from RA to RV to PA goes through DA to aorta (third shunt)
Again, bypasses the lungs (2o to high PVR) so oxygenated blood gets out to body through aorta
Pressure relationships, prostaglandins keep DA open
Lung Fluid
Approximately 100 mL/kg/day secreted
Becomes part of amniotic fluid
Production slows late pregnancy
Absorption during early labor
35% original volume at birth
First Breath
Has to occur for other steps to follow Two important stimuli for infant to breathe
Cold Chemoreceptor response
to brief asphyxia Respiratory muscles contract, decrease
intrathoracic pressures• Air is pulled into lungs
Establish FRC
Volume of air retained in lung at end expiration (40% of fully expanded volume)
Initial opening breath requires high pressure for expansion (40 - 60 cm H2O)
Next breath requires much less pressure, better inflation (need surfactant)
Surfactant
Spreads easily and rapidly over inside of lung: oily liquid
Stabilizes alveolar surface during expiration so FRC is maintained
Reduces surface tension, facilitates lung expansion with lower pressure
Maintains patency of small airways
Works as antimicrobial agent
Circulation Changes
First breath causes rise in pO2
Pulmonary vessels dilate
PVR, increased flow Pulm vasculature becoming high-flow,
low-pressure circuit
R heart pressures
Circulation Changes
Removal of low resistance placenta Increases systemic pressure/LV pressure Increases volume (venous return from lungs)
left side of heart L heart pressures > R heart pressures Functional closure of the Foramen Ovale
within minutes to hours after birth (anatomical closure takes longer)
Circulation Changes
Pressure in aorta becomes > pressure in PA’s: reverse flow through DuctusArteriosus
Increase in oxygen level, smooth muscle constricts to close the ductus
PGE removed by lungs aids in closure
Cardiovascular Transition
♥ 10 min = PaO2 50 mm hg
♥ 1 hr = PaO2 62 mm hg
♥ 2 days PaO2 75-85 mm hg
24 hours after birth:
♥ Oxygen consumption triples
♥ Significant increase in cardiac output
♥ Left ventricle must remodel and hypertrophy
Embryonic Development
Embryonic Development
Cardiac septation: begins middle of the 4th week and complete by end of 5th week Defects arising from problems in
septation: VSD, ASD, endocardialcushion defect (AV canal), malformation of tricuspid and mitral valves
Great Vessel Development
Happens simultaneously with septation
Defects that occur with great vessel development:
Truncus Arteriosus
TOF
Pulmunary and Aortic valve malformations
Transposition
DORV
Review of Cardiac Basics
Cardiac CycleSystole & Diastole
Systole is contraction of the ventricles
Diastole is the relaxation and filling of the ventricles followed by a small atrial contraction
Adult
Cardiac Output The volume of blood pumped by the left ventricle in 1 min 120 -200 ml/kg/min
CO = stroke volume x HR
Cardiac Output
Influenced by changes in HR, pulmonary vascular resistance, and systemic vascular resistance to flow
Also influenced by the amount of blood returning to the heart
Stroke Volume Relatively fixed at 1.5 ml/kg
Factors that affect SV
♥ Preload
♥ Afterload
♥ Contractility
Preload
The volume of blood in the ventricle before contraction (or at the end of diastole – aka end diastolic pressure)
Clinically, a measure of pressure rather than volume
Dependent upon venous return to the heart
An ↑↓ in preload can significantly affect CO in the neonate’s non compliant heart
Preload Changes
↓ ♥ Hypovolemia
♥ Intrapartum blood loss
♥ Peripheral pooling secondary to bacterial sepsis
↑ ♥ Fluid overload
♥ Left to right shunt thru PDA, VSD, PFO
Contractility
Speed of ventricular contraction (systole) -
Intrinsic pumping ability
Neonate’s heart has a limited
capacity to increase contractility
Cannot be clinically measured
ContractilityIncreased by:
exogenous catecholamines(Dopamine and Dobutamine)
Decreased by:
Acidosis
Hypoxia
Hypocalcemia
Hypoglycemia
Hypercarbia
Myocarditis
Dependent on the systemic vascular resistance and pulmonary vascular resistance (increase in SVR or PVR = afterload)
After-load can be reduced by IV infusions of vasodilators
Resistance to blood leaving the ventricles
Conduction System
Conduction System
Continues to develop after birth!
Thus, HR variability and benign dysrhythmias can be normal in newborn PVCs
PACs
Bradycardia (Low resting HR)
Conduction System
♥ Normal rate 120-160 (may range 80-200)
♥ Normal sinus rhythm
Abnormalities of the Conduction System
Supraventricular tachycardia (SVT) Most common tachydysrhythmia in newborn
period
200-220 bpm sustained, regular
Associated with Ebstein’s anomaly
Treatment: vagal maneuvers, adenosine, cardioversion
SVT 70% chance of recurrence within 48 hours
WPW syndrome: propranolol
Heart Block
No communication between SA node and AV nodes
Depolarize independent of one another
Ventricular rate is slow; 60 bpm
If asymptomatic, do nothing
If symptomatic, may need a pacemaker
Strong association between heart block and mom’s with Lupus
Review of Cardiac Assessment
Look, Listen, Feel
Assessment - Observation
♥ Skin: color, temperature, diaphoresis, edema
♥ Precordium: quiet, visible, heave, thrill
PMI - LLSB 5th intercostal spacePMI shifted to the right:
DextrocardiaTension pneumothoraxDiaphragmatic hernia
PMI shifted to the leftTension right pneumothorax
Cyanosis - Peripheral
♥ Acrocyanosis Bluish discoloration
hands and feet No mucous
membrane involvement
Often resolves by 48 hours of age
Rule out hypothermia
Results from sluggish movement of blood to the extremities and increased tissue oxygen extraction
Cyanosis - Peripheral
♥ Circumoral cyanosis Bluish discoloration around the mouth Often associated with feeding R/O central cyanosis
Cyanosis - Central
♥ Bluish discoloration of tongue and mucous membranes
♥ Caused by desaturation of arterial blood. Hemoglobin carrying no O2 appears purple.
♥ At least 5g of desaturated hemoglobin/dl is necessary before you can observe cyanosis.
♥ Also influenced by presence of anemia or polycythemia
♥ Indicates cardiac or respiratory dysfunction
= deoxygenated blood leaving the
Cyanosis - Pulmonary / Cardiac
Pulmonary Cardiac
Cyanosis Yes Yes
Respiratory Rate Increased Increased – often tachypneic no GFR Infant looks comfortable if no CHF
Work of breathing Increased Easy effort unless CHF – then GFR
Acid/Base Balance Increased PCO2 Decreased PCO2 with tachypneaRespiratory acidosis Metabolic acidosis
Mixed resp/metabolic if pulmonarydisease
CXR Asymmetric pattern of Increased or decreasedinfiltrates or other pulmonary vasculature pulmonary disease
Heart silhouette normal abnormal Size/shape/location
O2 Challenge test PO2> 150 PO2 < 150 for cyanotic CHD
Heart Auscultation
♥ First heart sound – S1 Closure of mitral and
tricuspid valves
End of atrial systole
Heart Auscultation
Second heart sound – S2 Closure of aortic and pulmonic valves
End of ventricular systole
Split S2 is a normal finding and just reflects the aortic valve closing before the pulmonic valve.
S2 First 48 hours
Normal to hear single S2 in first two days of life because of increased PVR. If you hear a split S2 at birth, could indicate abnormalities of P or A valves or alterations in PVR and SVR.
• After 48 hours• S2 split elongated
• ASD, TAPVR, TOF, Ebstein’s anomaly
• Absent split• Aortic stenosis, PPHN, TGA, TA
S3 & S4 S3: Only heard in left to right shunts
and mitral valve insufficiency
S4: Should not be heard in newborn. If so, indicates decreased ventricular compliance
Ejection clicks: Abnormal after 24 HOL and heard after S1. Associated with dilation of great vessels or malformation of PV and AV
Heart Murmur
Sound caused by turbulent blood flow
♥ Blood forced through narrowed areas
♥ Regurgitation through incompetent or abnormal valves
♥ Increased flow across normal structures
Heart Murmur
Murmurs Location
Transmission
Intensity
Timing
Quality
Grading I - VI
Heart Murmur Intensity
Grade I - barely audibleGrade II - soft but audibleGrade III - moderately loud, no thrillGrade IV - loud, assoc. with thrillGrade V - audible with stethoscope barely
touching chestGrade VI - audible with stethoscope not
touching chest
Heart Murmur Timing
♥ Systolic Heard between S1 and S2 of same beat
S1 (murmur) S2 S1 (murmur) S2
♥ Diastolic Heard between S2 and S1 of next beat
S1, S2 (murmur) S1, S2 (murmur)
♥ Continuous Starts in systole and extends into diastole
Normal/Innocent Murmurs
♥ Continuous systolic/Crescendo systolic murmur ( L R flow through PDA)
♥ Early soft midsystolic ejection murmur aka Peripheral pulmonic stenosis (PPS) Grade I-II/VI (no thrill) upper left sternal border, radiates to axilla and back
♥ Systolic ejection murmur (turbulence of blood flow across pulmonary valve)
Grade I-II/VI may be heard 1st week of life as PVR decreases and PDA closes
Up to 50% of neonates can have a murmur in the first 48 hours of life
Pathologic Heart Murmur
DIASTOLIC• Aortic or pulmonic valve regurgitation
Continuous murmurPDA, AVM, aortopulmonary window
SYSTOLIC• Pan systolic murmur
• Mitral or tricuspid regurgitation• VSD
♥ > Grade 3 murmur within hours of birth
Pathologic Heart Murmur
♥ Central cyanosis
♥ Respiratory distress
♥ Abnormal heart silhouette
♥ ↑ or ↓ pulmonary vascularity on CXR
♥ Gallop CHF
Assessment - Palpation
♥ Pulses: compare upper to lower extremities and side to side (if not equal, could mean LOTO)
R Brachial and femoral equal in strength (R brachial = R subclavian = pre-ductal)
Pedal pulses palpable Weak: LOTO, myocardial failure or shock Bounding (=“aortic runoff”): PDA, aortic
insufficiency, systemic to pulmonary shunt
♥ CFT: press for 5 seconds, release
< 3 seconds normal
Blood Pressure
Blood pressure
Methods for measuring
Arterial:
♥ Umbilical artery
♥ PAL Radial
♥ PAL Posterior tibialis
Discrepancies in upper and lower BPs
Coarctation of the aorta
Any type of arch abnormality
PDA
Hypertension
♥ Systolic or mean arterial BP > 95th percentile for birth weight, gest age, and post-natal age
95th percentile for systolic BP = 65 mmHg at 24 wks.
95th percentile for BP = 90/60 mmHg at 40 wks post conception.
Renal abnormalities most common cause
Hypertension Treatment
♥ Varies with cause of hypertension
♥ Tx etiology if possible
♥ Anti-hypertensive only if hypertension immediately life threatening
CVP
♥ In most cases the trend in CVP is more helpful than absolute value
♥ CVP may be difficult to interpret because it is affected by several factors: hypervolemia myocardial failure excessive ventilatory pressures grunting respirations tension pneumothorax pleural effusion UVC tip in portal system
Diagnostics
Diagnostics: EKGcardiac depolarization is the result of an
electrical discharge across the myocardial cell
cardiac depolarization is measured by the EKG
reflects abnormal hemodynamic burdens placed on the heart
right ventricular prominence normal after birth
major tool to evaluate arrhythmias and the impact of electrical imbalances
Diagnostics: EKG
Tall, peaked P waves are seen in RIGHTsided heart failure
Diagnostics: EKG
Wide, notched P waves seen in LEFTsided heart failure
Diagnostics: Blood Gas
Hyperoxygen Test
1. ABG @ RA
2. 100% FiO2 x 10 mins
3. @ 10 mins, draw ABG
4. If significant bump in PaO2, likely respiratory. If not,
Diagnostics: X-ray
Cardiomegaly: Cardiothoracic ratio > 65%
“Egg on a string” = TGA
Diagnostics: X-ray
TOF = “boot-shaped” heart
Diagnostics: X-ray
TAPVR = “snowman” appearance
Echocardiogram
The Gold Standard
Provides rapid, non-invasive, and painless evaluation of the anatomy and flow by the use of ultrasonic waves
Cardiac Catheterization
♥ Invasive procedure to obtain data for a definitive diagnosis, intervene, or to prepare for surgery
DiagnosticEvaluate hemodynamicsSelective angiography
InterventionalBalloon atrial septostomy (Rashkind)Balloon valvuloplasty Balloon angioplasty
Pressure Values from Cardiac Catheterization
Systemic Arterial Pressure 60 – 90/20 – 60 mm Hg (birth to 5 days)
R atrial pressure 3 mm Hg
R vent. Pressure 30/3 mm Hg
Pulmonary Wedge Pressure 6 – 10 mmHg
L atrial pressure 8 mm Hg
L ventricular pressure 100/6 mm Hg
Pathophysiology
Shock
Shock
♥ An acute state in which circulatory function is inadequate to supply sufficient amounts of O2 and nutrients to meet metabolic demands
♥ In most cases, cardiac output is low
♥ In early shock, compensatory regional vasoconstriction may temporarily maintain normal BP
Hypotension
♥ Late sign - cardiac decompensation
♥ Treatment of shock based on more than BP:
Evaluate history
Physical exam
Labs
Shock
♥ As shock progresses, compensatory mechanisms fail and there is widespread cellular damage
♥ Insufficient delivery of O2 results in anaerobic metabolism and lactic acidosis.
Shock
♥ If shock persists, irreversible injury to vital organs occurs, death ensues despite vigorous treatment that may temporarily return cardiovascular measurements to normal
Shock Etiology
♥ Hypovolemia (Hypovolemic shock)
♥ Asphyxia (Cardiogenic shock)
♥ Cardiogenic causes
♥ Sepsis (Distributive shock)
Hypovolemia• Blood loss (intrapartum
or postnatal)
• Inadequate placenta transfusion
• Severe dehydration
• Third spacing
• Skin integrity losses (gastroschisis)
• Pleural effusions (hydrops)
Asphyxia
Antepartum
Intrapartum
Respiratory failure
Impaired O2 transport due to severeanemia or hemoglobinopathy
Cardiogenic
Cardiomyopathy
Dysrhythmias
Congenital malformation
Hypocalcemia
Severe hypoglycemia
Sepsis
Especially early onset group B beta-hemolytic Streptococcal
Drugs
1. Maximize cardiac output1. epinephrine
2. dopamine
3. dobutamine
4. Milrinone
5. Isoproterenol (increases HR and contractility)
Shock Presentation
Cardiovascular
Systemic arterial hypotension
Narrow pulse pressure
Central venous hypotension, althoughCVP may be elevated with cardiomyopathy
Tachycardia
Shock Presentation
Respiratory
Tachypnea
Retractions
Grunting
Apnea
Other signs
Prolonged capillary fill time
Oliguria
Hypothermia
Metabolic acidemia
Cardiac Tamponade
Cardiac Tamponade –a medical emergency
♥ The hemodynamic result of fluid accumulation in the potential space surrounding the heart or pericardium.
♥ Excessive fluid accumulation results in ↑ pericardial pressure, causing ↓ ventricular filling, ↓ cardiac output and hypotension.
♥ The rapidity of fluid accumulation influences the hemodynamic effect.
♥ Beck’s triad (jugular venous distension, hypotension, and muffled heart sounds).
Congestive Heart Failure
Congestive Heart Failure
♥ Myocardial dysfunction in which the heart is unable to pump enough blood to meet its needs, to dispose of venous return adequately, or a combination of the two
♥ May result from CHD or acquired heart diseases with volume or pressure overload or from myocardial insufficiency
Potential Causes
♥ Structural cardiac defects
♥ Cardiomyopathy
♥ Cardiac arrhythmias
♥ AV malformations
♥ Multiple hemangiomas
♥ Asphyxia
♥ Bronchopulmonary dysplasia
♥ Sepsis
♥ Hypoglycemia
♥ Hypocalcemia
♥ Severe anemia
♥ Polycythemia
♥ Fluid overload
♥ Renal Failure
♥ Adrenal insufficiency
♥ Hyperthyroidism
CHF - fetal causes
♥ SVT
♥ Severe bradycardia d/t complete heart block
♥ Anemia
♥ Ebstein’s anomaly
♥ Myocarditis
Presentation:
Hydrops
Treatment:
Digoxin
CHF - occurring the first day of life
♥ Asphyxia
♥ HLHS
♥ Hypoglycemia
♥ Severe tricuspid or pulmonary regurgitation
♥ Hypocalcemia
♥ Sepsis
♥ Systemic AVM
CHF - occurring the first week of life
♥ PDA
♥ TGA
♥ Adrenal Insufficiency
♥ TAPVR
♥ Closure of PDA with ductal dependent
lesion:
Coarctation of the aorta
Hypoplastic left heart syndrome
Interrupted aortic arch
CHF - occurring beyond the second week of life
♥ VSD most common reason
♥ Truncus arteriosus
CHF –pulmonary presentation
♥ Poor weight gain ***
♥ Poor feeding of recent onset ***
♥ Feeding intolerance
♥ Tachypnea, dyspnea that worsens during feeding ***
♥ Increased WOB:
Grunting, flaring, and retracting
Head bobbing
♥ Rales, rhonchi, wheezing ***
♥ Irritability, lethargy
♥ Pulmonary infiltrates on CXR
Pulmonary venous engorgement (aka Pulmonary Edema)
Cause is blood backing up in the pulmonary system Leakage of fluid into the
pulmonary interstitium
• Interferes with gas exchange
• LV dysfunction &/or overload may increase LV end diastolic pressure
CHF – CV presentation♥ Cold sweat on forehead - diaphoresis♥ Puffy eyelids, dependent edema♥ Pallor, mottling, cyanosis♥ Increased precordial activity
♥ Peripheral pulses initially full but decrease in end stage, prolonged CFT
♥ Tachycardia ***, gallop rhythm, BP changes
♥ Decrease in urine output (<0.5 ml/kg/hr), increase in specific gravity
♥ Sudden weight gain in end stages♥ Hepatomegaly ***♥ Cardiomegaly on CXR ***
Systemic venous engorgement
Blood backing up in the systemic system
Leakage of fluid into the periphery interstitium and liver
Hepatomegaly
RV dysfunction &/or overload my increase RV end diastolic pressure
CHF – SNS compensatory mechanisms
♥ ↑ HR
♥ ↑ cardiac contractility
♥ ↑ arterial BP
CHF – SNS compensatory mechanisms
Catecholamine release↑ venous tone ↑ blood return to the heart
↓ circulation to skin, kidneys, extremities, & splanchnic bed
↓ Renal blood flow stimulates release of renin angiotensin, and aldosterone
which triggers retention of Na and fluid, resulting in increased circulating blood volume
↑ Blood volume puts additional work load on the heart
CHF – mechanical compensatory mechanisms
Cardiac muscle thickens to ↑ myocardial pressure
Hypertrophy effective in early stages
as muscle mass ↑ compliance ↓
↓ compliance requires ↑ filling pressure for CO
CHF – mechanical compensatory mechanisms
Hypertrophied heart eventually becomes ischemic
Ventricular dilation occurs to accommodate volume
Initially the heart tries an increased force of contraction but soon fails
CHF - management
♥ Prone position
♥ Decrease oxygen consumption NTE Minimize stimulation Provide sedation Assisted ventilation Supplemental O2 Correct acidosis and electrolyte imbalances
CHF - management
Fluids and nutrition Fluid restriction limiting PO feeding increased calorie feedings
Medications for CHF
Diuretics:
Furosemide (Lasix)
Hypochloremia and hypokalemia can lead to metabolic alkalosis. Can also cause hypocalcemia .
Spironolactone (Aldactone)
K+ sparing, but can cause hyperkalemia
Chlorothiazide (Diuril)
K+ and Ca sparing; not as powerful as a diuretic
Medications for CHF
Digoxin:
positive inotropic ↑ cardiac contractility
negative chronotropic ↓ HR
Observe for bradycardia, arrythmias, hypokalemia
Inotropes: Dobutamine, dopamine
Congenital Heart Defects
Overview
Congenital Heart Defects
85% multifactorial
10% chromosomal
2% genetic
2% maternal or environmental teratogens
Chromosomal abnormalities
You will often see cardiac defects with: Trisomies (esp. 18 and 13)
Turner syndrome
Noonan’s
DiGeorge
22q11
Environmental Factors and Teratogens
Thalidomide
Anticonvulsants (Phenytoin, Carbamazepine, Valporic Acid, Pentobarbital, Trimethadione)
Anticoagulants (Warfarin, Heparin)
Lithium
Alcohol
Amphetamine
Maternal Disease and Viral Infections
Diabetes: 5 x’s the risk of CHD (VSD, DORV, TGA, TA and coarctation)
Lupus (heart block)
Rubella and CMV (PDA, PS, VSD, ASD)
Obesity
Congenital Heart Disease
Increased Pulmonary Flow:
PDA - Patent Ductus Arteriosus
VSD – Ventricular septal defect
AV Canal – Endocardial Cushion partial or complete
30% are infant’s with trisomy 21
ASD – Atrial Septal Defect
Congenital Heart Disease
Obstructive Lesions that cause Pulmonary Venous Congestion
Coarctation of the aorta
Aortic Stenosis
Congenital Heart Disease
Ductal dependent lesions that decrease pulmonary blood flow:
TOF - Tetralogy of Fallot with severe PS or PA
PA - Pulmonary Atresia
PS - Pulmonary Stenosis
TA - Tricuspid Atresia
Congenital Heart Disease
Mixed lesions:
TGA -Transposition of the great arteries
TAPVR - Total anomalous pulmonary venous return
HLHS - Hypoplastic left heart syndrome
TA -Truncus arteriosus
Ductal Dependent Defects
♥ Need to have PDA open for systemic or pulmonary circulation
♥ PGE1 continuous infusion to open and maintain ductal patency
PDA
Shunts right to left flow in utero
Persistent PDA will shunt left to right as PVR decreases
PGE used to open PDA:
If you need left to right flow for pulmonary perfusion
If you need right to left flow for systemic perfusion
Pathologic PDA
Symptoms: Increased pulmonary vasculature and
cardiomegaly on CXR
Bounding peripheral pulses
Active precordium
Widened pulse pressure
Low diastolic BP
Indomethacin
Prostoglandin inhibitor
Complications Decreased renal blood flow = oliguria
Increased incidence of GI bleed
Thrombocytopenia = increased chance of ICH
AV Canal
♥ Endocardial Cushion Defect or Atrioventricular Septal Defect
♥ Partial involves the atria
♥ Complete involves atria and ventricles
♥ Left to right shunt
AV canal Risk Factors
Trisomy 21
Rubella or other viral illness during early pregnancy
Alcohol consumption
Poorly controlled diabetes
Smoking
Parent with a CHD
AV canal complications♥ Cardiomegaly
♥ On auscultation, thrill at the LLSB. Loud pansystolicmurmur at LLSB radiating to back
♥ Pulmonary Hypertension
♥ Respiratory Tract Infections
♥ Congestive Heart Failure
Complications after correction of AV canal
♥ Regurgitant heart valves
♥ Stenosis of the heart valves
♥ Arrythmias
♥ PPHN
♥ Breathing difficulties associated with lung damage
COA – Coarctation of the aorta
♥ Juxtaductal narrowing of aorta
♥ Frequently associated bicuspid aortic valve
♥ Often associated with hypoplastic transverse arch
♥ Mild left heart hypoplasia
COA - Coarctation of the aorta
♥ As the ductus arteriosus closes, the area of coarctation narrows
♥ The result is decreased systemic blood flow, pulmonary venous congestion
♥ In severe cases, CHF results from decreased left ventricular function
♥ If severe, with ductal closure, shock and tissue hypoxia
COA Male dominance 2:1
Associated with Turner Syndrome
Presentation:• Decreased or absent LE pulses • Higher blood pressure in UE; BPs may wax
and wane with bidirectional flow through PDA• “systolic thrill @ suprasternal notch”• Cardiomegaly + increased pulmonary vascular
markings on CXR
COA Management
Treat/Manage CHF
PGE
Surgery
TOF – Tetralogy of Fallot
♥ Most common CYANOTIC heart defect
4 anomalies Large VSD
Right ventricular outflow tract obstruction
Overriding aorta
Right ventricular hypertrophy
♥ Symptom severity depends on the degree of right ventricular outflow tract obstruction
TOF
TOF Presentation
Harsh systolic murmur with thrill @ ULSB
Boot shaped heart on CXR
TOF - Management
♥ Supplemental FiO2 to treat cyanosis
♥ The majority are not ductal dependent
♥ If saturation still below 75% on oxygen may have significant pulmonary stenosis or atresia and need PGE
TET Spell Hypoxic Spell
♥ Cyanotic or hypercyanotic spell Progressive hypoxia
Hyperpneic
Pale, flaccid
Immediate treatment needed
Organ damage if severe hypoxia and acidosis
Eventual loss of consciousness
TET Spell Management
Calm infant
Increase systemic vascular resistance to decrease the right to left shunt at the VSD
Knees to chest
May need to be sedated
FiO2 but will only improve oxygenation once right to left shunt is decreased
May need intubation and correction of metabolic acidosis
Pulmonary stenosis
♥ Stenosis is a narrowing
♥ Varying degrees of narrowing; severity of symptoms depends on degree of narrowing
♥ Most common form is valvular
Mild Pulmonary Stenosis
Loud systolic murmur at LUSB is the only clinical finding
Severe Pulmonary Stenosis
♥ If critical PS, the right ventricle cannot eject sufficient blood flow to the pulmonary artery to maintain normal oxygen saturations.
♥ If critical, PGE is required to allow blood to shunt left to right to perfuse the lungs
♥ A newborn with critical pulmonary stenosis and intact VSD presents an emergency situation that requires immediate treatment, either balloon dilation of the valve or surgery.
Pulmonary Atresia with VSD
PA Pulmonary Atresia with intact ventricular septum
♥ Atresia means not formed, so absence
♥ Atretic pulmonary valve
♥ Hypertrophied right ventricle
♥ Hypoplastic left ventricle
♥ Blood flow R to L across PFO
♥ Pulmonary blood flow is dependent on the PDA♥
HLHS – hypoplastic left heart syndrome
♥ Hypoplasia of left ventricle
♥ Severe mitral valve stenosis or atresia or severe aortic valve stenosis or atresia
♥ Hypoplastic ascending aorta and transverse arch
♥ Coarctation is a frequent finding
HLHS
Ductal dependent blood flow right to left to perfuse all regions of the body
Management: HLHS
♥ To open PDA and to improve systemic perfusion
♥ Intubation and ventilation to reduce work of breathing and strain on the heart
♥ Avoid hyperoxia and hypocarbia (both will decrease PVR at the expense of systemic blood flow)
♥ Inotropic support may improve myocardial function
♥ Follow arm and leg BPs
TGA – Transposition of the Great Arteries
Great arteries are transposed relative to the ventricles
TGA
♥ Circulation pattern is parallel
♥ The majority of the blood from each ventricle is circulated back to the same ventricle
♥ Mixing must occur:
Best to have a VSD
and a PFO/ASD that is not restrictive
Use PGE to open the PDA
♥ If no VSD and a restrictive PFO/ASD a Rashkind/Balloon septostomy may be needed
There needs to be adequate mixing at 2 sites!
TAPVR – Total Anomalous Pulmonary Venous Return
TAPVR Types
♥ Supracardiac emptying into the left vertical vein (most common type 80-90%) which then drains into the SVC
♥ Cardiac emptying into the coronary sinus or right atrium
♥ Infradiaphragmatic emptying into the vertical vein that descends through the diaphragm into the portal vein or IVC
Infracardiac Obstructed TAPVR
♥ Oxygenated pulmonary blood mixes with deoxygenated systemic blood and returns to the right atrium to shunt right to left across the PFO/ASD to get to the left atrium and eventually to the body
♥ Severe hypoxia and profoundly ill after birth
Management Infracardiac Obstructed TAPVR
- Intubation 100% FiO2
- Treat acid /base disturbance
- Treat hypotension, hypothermia
- Emergency corrective surgery is required to anastomose the pulmonary veins to the heart
CXR Cardiac or supracardiac
CXR Obstructed TAPVRCXR can be confused with RDS or PNA
Truncus Arteriosus One great vessel
arising from both ventricles, VSD
One vessel supplying systemic and pulmonary circulation
As PVR decreases, shunting occurs L to R across VSD overloading lungs and LV (unless PS)
Sample Cardiac Questions
Normal values for pulmonary artery pressures in a term infant would be:
A. RAP 7-10 mm Hg, LAP 5-7 mm Hg and PAOP 4 mm Hg
B. RAP 3 mm Hg, LAP 8 mm Hg and PAOP 6-10 mm Hg
C. RAP 10-20 mm Hg, LAP 12-14 mm Hg and PAOP 10-14 mm Hg
The type of murmur commonly heard in patients with tricuspid atresia is:
A. A diastolic murmur
B. A systolic murmur
C. A holosystolic murmur
An ASD results in:
A. No damage to the septum
B. A left-to-right shunt with volume overload
C. A diastolic murmur at the USB
Neonates with cyanotic heart defects are at particular risk for:
A. Intrarenal kidney injury
B. CHF
C. Polycythemia
A mechanism by which a newborn can respond to increased volume is via:
A. A baroreceptor stretch
B. An increased heart rate
C. A decreased PVR
During your assessment, you note that the R brachial is stronger than the L. You conclude:
A. Cardiac tamponade
B. Thoracic anuerysm
C. Supravalvular aortic stenosis
Ventricular Septal Defect
VSDs are most common form of all CHDs
Presentation and management will vary according to size of VSD
50% - 75% of small defects will close spontaneously; 20% of large defects will close independently
VSD PVR
SVR
Left Right Shunt
Pulmonary Blood Flow
Pulmonary Edema
Pulmonary artery hypertrophy pulmonary artery stenosis
VSD
Small VSD
Asymptomatic
High-pitched pansystolicmurmur @ LSB 4 -10 DOL
Moderate VSD• Fatigue with feeding• Recurrent respiratory
infections
Large VSD• Loud, blowing pansystolic
murmur @ LLSB• CXR: increased
pulmonary vascular markings, cardiomegaly
• @ 1 -2 MOL: CHF, hepatomegaly, recurrent respiratory infections
Atrial Septal Defect
Female to Male 2:1
Spontaneous closure around 20%
Defect can be in ostium primum or secundum (most common)
PFO ASD
ASD
Symptoms present in 50% of patients IF ID’d in infancy (most go unrecognized)
Failure to thrive
Recurrent respiratory infections
Systolic murmur @ 2nd ICS LSB, persistent split S2 if defect is large, diastolic murmur @ LLSB
Aortic Stenosis
4 x’s more likely in males
3 types: Valvar is the most common
If AS is severe in utero, fetus will develop LV hypoplasia and L-sided syndrome
Aortic stenosis
Valvular aortic stenosis has a bicuspid aortic valve
LV is hypertrophied
Aortic Stenosis Presentation
If not diagnosed in utero and not severe, asymptomatic at birth , then progresses to CHF
Harsh systolic murmur in URSB that radiates
CXR: cardiomegaly but normal pulmonary vascular markings
Aortic Stenosis: Treatment
Balloon Valvuloplasty
Tricuspid Atresia
Agenesis of the tricuspid valve
VSD often present
Hypoplastic RV
Pulmonary valve atresia or stenosis
TGA in 30% of cases of tricuspid atresia
Truncus Arteriosus
Bounding pulses, widened pulse pressure
Severe cyanosis if TA with PS
Harsh systolic murmur LLSB, systolic ejection click, single S2
Management: Treat CHF (medications include diuretics, digoxin and ACE inhibitors)
Surgical repair