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