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Neonatal Prostaglandin Use In

Rural Communities

William Waltz, MD, PhD, Pediatric Cardiology&

Joan Nold, MD, Neonatology

Sanford Childrens SymposiumWednesday, 17 October 2008


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PLAN

What are the odds?

The Patent Ductus Arteriosus

Defining ductal dependent heart disease Differentiating from non-ductal dependent heart

disease

Differentiating from non-cardiac disease

Evaluation of the newborn Prostin


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PLAN

What are the odds?



disease




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What Are The Odds?

Congenital Heart Disease8/1000 live births

Critical CHD3/1000 live births

In the USA:~ 32,000 children born/year with CHD~ 11,000/year with Critical CHD


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PLAN

What are the odds?



disease




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PDA Anatomy


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Fetal

Circulation


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Right to leftshunt

Left to rightshunt

Direction of shunt

depends onpressures


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PLAN

What are the odds?



disease




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Ductal-dependent Heart Disease

ie. Inadequate systemic oxgenation due toheart disease

Inadequate pulmonary blood flow

Inadequate systemic delivery of oxygenated

blood Inadequate mixing


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Normal


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Right sided obstruction


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Right sided obstruction


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Inadequate Mixing

Survival Depends UponMixing Between

Systemic and PulmonaryCircuits


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Left sided obstruction


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Cyanotic Congenital Heart Disease

Severe Cyanosis PaO2 40 mmHg

Decreased pulmonary blood flow

Tricuspid atresia, intact ventricular septum

Pulmonary atresia (with or without other lesion) Critical pulmonary stenosis

Ebsteins anomaly

Tetralogy of Fallot*

Chest X-Ray

Decreased pulmonary vascular markings

Huge cardiac silhouette in Ebsteins

anomaly


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MIXING LESIONS PaO2 > 40 and 100 mmHg

Clinical Characteristics

Double outlet right ventricle

Double inlet left ventricle

Tetralogy of Fallot* Transposition/ VSD

Total anomalous pulmonary venous return

Truncus arteriosus

Arterial oxygen content depends upon the relative amounts ofpulmonary and systemic blood flow



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Clinical Characteristics

Can present during newborn period when ductusarteriosus closes if severe

If left-sided obstruction coexists with a left-to-right shunt

or mixing lesion, can cause earlier symptoms Can present due to murmur, pulse abnormality, blood

pressure abnormality, or shock

Too much oxygen can hurt

steals blood flow from systemic to pulmonaryHigh sats low blood pressureLower sats better blood pressure



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Pulmonary vein stenosis (rare) Cor triatriatum

Supravalve mitral ring

Mitral valve stenosis

Subaortic stenosis

Aortic valve stenosis/aortic valve atresia

Supravalvar aortic stenosis (Williams Syndrome)

Coarctation of the aorta Interrupted aortic arch

Hypoplastic left heart syndrome

Cyanotic Congenital Heart DiseaseLeft sided obstruction


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Physical examination findings Hypoplastic left heart syndrome has diffusely

diminished pulses

Coarctation has decreased and delayed pulsesin lower extremities. Findings may not beapparent until complete closure andconstriction of PDA



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Initial Stabilization and treatment

PGE1 to maintain systemic flow

Intubation, ventilation

Aggressive treatment of metabolic acidosis

HLHS

ABG pH 7.40, pCO2 40, pO2 40

Avoid oxygen, agents with -adrenergic effect



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d-Transposition of the Great Arteries


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Pathophysiology Cyanosis due to failure of delivery of pulmonaryvenous blood to the systemic circulation

Two parallel circulations with no mixing

Open atrial septum (fossa ovalis) allows someleft-to-right shunt, enhanced by a left-to-rightductus arteriosus shunt

Presence of ventricular septal defect facilitatesmixing



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Chest film Oval-shaped heart

Narrow mediastinum

Normal or increased pulmonary vascularmarkings



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D-Transposition of the Great Arteries

3 neonates with TGA/IVS with no significant PDA at 1 day of age


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D-Transposition of the Great

Arteries Medical Management - Stabilization

Upon suspicion, PGE1 infusion to maintain

ductal patency and enhance atrial shunting Transfer to a tertiary care center with

suitable cardiac catheterization facility

Careful surveillance for signs of pulmonarycongestion


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D-Transposition of the Great

Arteries Balloon Atrial Septostomy

used for stabilization to insure adequate

atrial mixing in anticipation of surgery

Acyanotic Congenital Heart Disease


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VSD, AV canal

PDA, Aortopulmonary window, AV malformation

Usually present with a murmur which might not be present atbirth

Symptoms develop when pulmonary vascular resistance fallsat 1-3 months of age

Oxygen administration can cause significant fall in pulmonaryvascular resistance at any age, causing worsening symptoms,pulmonary edema, pulmonary hemorrhage

Acyanotic Congenital Heart DiseaseLeft to right shunts


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PLAN

What are the odds?



disease




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Non-Cardiac Causes of Newborn Cyanosis

Primary lung disease

RDS, pneumonia, hypoplasia, CCAM, lymphangectasia, lobar Emphysema

Mechanical interference with lung function

Congenital diaphragmatic hernia, pneumothorax,pneumomediastinum, chylothorax, choanal atresia, vascular ring,tracheal esophageal atresia, anomalies of the airway/larynx,Pierre-Robin/ micrognathia

Pulmonary hypertension Meconium aspiration, pneumothorax, RDS, sepsis, pneumonia


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CENTRAL NERVOUS SYSTEM

Seizure, meningitis, encephalitis, severe IVH, subduralor subarachnoid hemorrhage,

Severe hypermagnesemia

Shock

Sepsis, hypovolemia, adrenal crisis

Severe hypoglycemia (IDM, SGA babies)

Congenital neuromuscular condition (Werdnig-Hoffman)

- METHEMOGLOBINEMIA

Non-Cardiac Causes of Newborn Cyanosis


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PLAN

What are the odds?



disease




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CHD Presenting in the Neonatal Period

0-6 days 7-13 days 14-28 days(n=1603) (n=311) (n=306)

TGA (15%) Coarct (20%) VSD (18%)HLHS (12%) VSD (14%) TOF (17%)TOF (8%) HLHS (9%) Coarct (12%)Coarct (7%) TGA (8%) TGA (10%)

VSD (6%) TOF (7%) PDA (5%)Other (52%) Other (42%) Other (38%)


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Clinical Presentation of CHD in the Neonate

Fetal Diagnosis

Cyanosis

CHF/Shock/Circulatory Collapse

Arrhythmia

Asymptomatic Heart Murmur


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Clinical Presentation of CHD in the Neonate

Timing and Symptoms depend on

(1) anatomic defect

(2) in utero effects (if any)(3) physiologic changes - transitional circulation

closure of the ductus arteriosusfall in pulmonary vascular resistance


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Newborn Presentation of CHD

Cyanosis- Usually minimal symptoms- First 24-72 hours of life- Duct-dependent pulmonary blood flow

CHF/Circulatory Collapse/Shock- First 2 weeks of life- Ductal-dependent systemic blood flow

Secondary end-organ dysfunction- Heart, Brain, Kidneys, GI

Recognition of Cyanosis


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Cyanosis = blue color of skin and mucous membranes

caused by reduced oxygen content

oxygen content of blood depends upon:

Hgb level

oxygen saturation

blood flow

cyanosis usually noted when Sats


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Central cyanosis

noted in the trunk, tongue, mucous membranes

due to reduced oxygen saturation

Peripheral cyanosis

noted in the hands and feet, around mouth

due to reduced local blood flow




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all babies blue at birth

by 10 minutes, should have pink bodies,

but hands and feet still blue

crying may cause cyanosis

examine baby in white light while quiet




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Differential cyanosis

1. pink upper, blue lower

CoA, IAA, Pulm Htn

2. blue upper, pink lower

d-TGA with pulm Htn

*indicates serious underlying cardiac or lung disease*



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Evaluation of the Cyanotic Neonate

Detecting cyanosis:

ambient lighting

skin color

hemoglobin

if Hg is 20 gm/dl; 4 gm desaturated-visible cyanosis if Hg is 10 gm/dl; 2 gm desaturated-not cyanotic

Check Saturation

Hyperoxia Testto Determine Intrapulmonary vs. IntracardiacShunt


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Hyperoxia Test

1. On room air (if tolerated)-measure pO2 by blood gas

2. On 100% FIO2 - blow-by, mask, ETT

-repeat measurement of pO2

Must note site of measurement

Pulse oximetry not acceptable

DANGERS: Pulmonary overcirculationClosing the PDADeath


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Hyperoxia Test - Interpretation

pO2 < 100; cyanotic CHD likely

pO2 100-150; cyanotic CHD possible

pO2 > 150; cyanotic CHD unlikely

A failed hyperoxia test is a neonatal emergencyrequiring intervention.

Pulse Oximetry


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Pulse Oximetry

easy to use, harmless when done correctly

accuracy of 2% in the range of 70 to 100%

consider cyanotic when Sat


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CHD in the Neonate Cyanosis

Chest Radiograph may be helpful:

Decreased pulmonary vascular markings withintracardiac right to left shunting

Normal/increased pulmonary vascular markings withintracardiac mixing of pulmonary and systemic venousreturn


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Congestive Heart Failure

Clinical Syndrome marked by inability of the heart tomeet the metabolic demands of the body

After the first 24-48 hours of life, the neonate withCHF/shock has duct-dependent, left-sided heart

disease until proven otherwise


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Evaluation for and treatment ofpresumptive sepsis should be undertakensimultaneously with evaluation for cardiac

and pulmonary disease.


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PLAN

What are the odds?


Defining ductal dependent heart disease Differentiating from non-ductal dependent heartdisease



H t Di Lik l


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Heart Disease Likely

PGE1 0.05-0.1 mcg/kg/min

Sat monitor, heart rate monitor, frequent vitals

Observe 20-30 minutes

Watch sats

Watch perfusion

Repeat ABG and VS

Umbilical lines

Reach out and touch someone


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Prostaglandin E1

Apnea

Vasodilation/Hypotension

Fever

Seizures (rare)

May unmask CHD with obstruction to PV return

d-TGA-intact atrial septum

TAPVR

Mitral atresia with restrictive atrial septum

Sid Eff t f PGE 1 B Bi th W i ht


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Side Effects of PGE-1 By Birth Weight

2

KG KG_____________________________________

CV 37% 17%CNS 16 16Respiratory 42 10Metabolic 5 2Infectious 11 2

GI 11 3Hematologic 5 2Renal 0 2

D li ith Sid Eff t f PGE 1


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Apnea caffeine, intubate Hypotension volume, pressors

Seizures anti-seizure meds

Dealing with Side Effects of PGE-1

Alprostadil Standard of Care?


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Alprostadil Standard of Care?

S.T.A.B.L.E. cardiac module

PALS

Al t dil


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AlprostadilCost & Availability

Available in boxes of 5 vials/ampules

Cost per vial $25.19 $33.42

500 mcg drug in one ml vial dilute with 49 cc D5 for

standard concentration 10 mcg/ml (NEOFAX) 0.05-0.2 mcg/kg/min IV

For a non-syringe pump, make more dilute

Establishing standardized concentrations for non-

syringe pump

Alprostadil


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AlprostadilCost & Availability

Sanford Health affiliates may obtain single vials fromcentral pharmacy

Shelf Life 2 years

Vendor visits affiliate hospitals twice yearly to takeback outdated drugs. Return credit given.

S l t l O2 i C iti l CHD


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Supplemental O2 in Critical CHD

An oxygen saturation of ~75-85% usually provides

adequate oxygen delivery to prevent metabolic acidosis

Titrate supplemental O2 to saturation ~ 80%


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SUMMARY

Institutions that might deliver a babyshould have Prostin available for use

Call Sanford Childrens Neonatologist forany concerns about a newborn thatmight require a higher level of care

605-328-3099 to reach neonatologist

605-333-4447 Communication Center

Prenatal diagnosis

waltz neonatal pros tag land in

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