Congenital Heart Disease in the DR:

Tips from a Pediatric Cardiologist

Neonatal Resuscitation Program Course (VC1152)
Sarah Gelehrter, MD
University of Michigan
Ann Arbor, MI
Faculty Disclosure Information

I have no relevant financial relationships with the

manufacturer(s) of any commercial product(s) and/or
provider(s) of commercial services discussed in this CME
activity.

I do not intend to discuss an unapproved/investigative use

of a commercial product/device in my presentation.
Learning Objectives

At the conclusion of the presentation, participants should

be able to:
1. Discuss the expected delivery room course for
neonates with prenatally diagnosed CHD.
2. Start multidisciplinary conversations at their
institution around delivery planning for neonates with
prenatally diagnosed CHD.
Case #1
Term infant born via repeat c-section
DOL 2: Discharged home doing well
DOL 3: fast breathing and difficulty feeding
DOL 4:
Taken to local hospital in respiratory distress. Transferred to
regional medical center
Intubated for respiratory distress. Poor perfusion with absent
femoral pulses. Prostaglandin started. Umbilical lines placed.
Echo showed hypoplastic left heart syndrome. Transferred to
surgical center.
DOL 12: Underwent cardiac surgery (Norwood)
Newborn Pulse Oximetry Screening
 Timing of Closure of the Ductus Arteriosus
• Median closure time: 13.5hrs (range:7.7- 18.7 hrs)

• < 30 min: All PDAs were open; no baby had a small duct.

• 2-3hrs: 80% had a large PDA, 20% had a small PDA.

• 7-10 hrs: PDA had closed in 20%; 47% had a small PDA

• Iwashima; Early Human Development, 2018

• Jain, et al; Pediatrics, 2018
Case #2

• Prenatal diagnosis of
hypoplastic left heart
syndrome
• Induced delivery at full term
• Immediately taken from OB
by NICU team: PGE started,
umbilical lines placed,
admitted to cardiac ICU
Neonates with Ductal-Dependent CHD

Missed Diagnosis Prenatal Diagnosis

Undertreatment Overtreatment

Altered Parent-Infant
Neonatal Shock
Bonding
Prenatal Diagnosis of CHD
• Why does it seem scary to know that a
baby has ductal-dependent CHD?

• How can we safely risk-stratify delivery

room management of babies with CHD?
Delivery Planning for Babies with CHD

• How good is prenatal diagnosis of CHD?

• Why/when are babies with CHD sick in the delivery

room?
• Can we predict it?
• Cardiac vs non-cardiac risk factors
Accuracy of Screening of Fetal Hearts

• Obstetric Screening
• Dependent on method (3 vs 5 views)
• Sensitivity: 37-72%
• Specificity : 97-99%
• Fetal Echo
• Sensitivity: 90-97%
• Specificity : 98-100%
Bak et al, Detection of fetal cardiac anomalies: cost-
effectiveness of increased number of cardiac views.
Ultrasound Obstet Gynecol. 2020
What to Expect at Delivery: Most Babies with CHD

Most infants with CHD will do well in the first few hours
after birth
• Ductus arteriosus is wide open
• Patent foramen ovale is open
• Good cardiac function and output
• Pulmonary resistance still relatively high- does not allow for
too much shunting/ pulmonary blood flow
What to Expect at Delivery: The Unstable Exceptions

• Poor oxygenation
• d-transposition of the great arteries
• Pulmonary congestion/ edema
• Obstructed total anomalous pulmonary venous return
• Hypoplastic left heart syndrome with restrictive atrial septal defect
• Poor cardiac output
• Some arrhythmias, cardiomyopathies
• Airway compromise
• Tetralogy of Fallot with absent pulmonary valve
Babies with Congenital Heart Disease

All
Babies

Babies
with CHD

Critical CHD
Why are Babies with CHD Sick in the DR?

• Usual risk factors for 30%

Intubation or CPAP in First 2 Hours of Life
needing advanced
resuscitation apply to CHD 25%

babies as well 20%

24%

19%
• 300 term infants with 15% 18%
isolated ductal-dependent 10% 12%
CHD not anticipated to be
sick for cardiac reasons in 5%
2%
DR 0%
NRFHR Sepsis Risk Resp Distress Any Risk No Risk
Factor Risk Factor Factor Factor
 Risk Stratified
Delivery Planning
Decreasing the Stress Of Cardiac Deliveries
Why are Cardiac Deliveries so Anxiety Provoking?

• Unclear or absent communication

• Conflicting definitions
• Unclear expectations
• Worry about “doing the wrong thing”
• Overmedicalization
• Appropriate concern
• The sick ones can be really sick
• HALO events
 Pre-Delivery Communication
• Definitions
• Expected Course
• “Permission” to
de-medicalize

"Low hanging fruit" by Jason Rosenberg is licensed under CC BY-NC 2.0

What do we mean by “Critical” CHD?

• AAP/AHA, 2009
• CHD “requiring intervention within
the first year of life”
• 2016 Update
• 12+ types of CHD
Critical Congenital Heart Disease Definition

“ Critical forms of CHD have an additional element of

instability during the perinatal transition period and
often require emergent cardiac intervention in the first
hours after delivery for stabilization and survival.”
• Pruetz JD, et al. Delivery room emergencies in critical congenital heart diseases, Seminars in
Fetal and Neonatal Medicine, 2019
Standardized Wording in Prenatal Cardiology Notes

• Ductal Physiology • Acceptable Oxygen

• Delivery Location/Team Saturations
• Parental-Infant Bonding
 Standardized Prenatal Documentation
40%

35% p = 0.03

30%

25%
% of patients

20%
38%

15% p = 0.004
25%
*
10%
15%
13%
5% 10% *
* 5% 5%
4% 3% 4% 2% * 2%
0%
BBO2 PPV NC CPAP Intubation Any Respiratory
Support
Pre (N=289) Post (N=97)
Delivery Risk Stratification Schemes
 Published Delivery Risk Stratification Schemes
Predicted Risk Donofrio, Pruetz, Slodki, 2012 Berkley, Suggested Delivery Room
of Instability 2013 2014 2009 Delivery Plan Recommendations
Not Expected LOC 1 ENCI 1 Planned Care Plan 2 Routine Routine
CHD
Minimal LOC 2 ENCI 2 Severe Care Plan 3 Routine, Neonatologist in DR
Planned consider
CHD induction > 39
weeks
High LOC 3 ENCI 3 Severe Care Plan 4 Planned Neonatologist in DR,
Urgent CHD induction > 39 cardiology alerted
weeks
Very High LOC 4 ENCI 4 Severest Care Plan 5 Timed delivery Neonatology,
Heart to coordinate Cardiology and
Defect neonatal care Surgery/Intervention
teams in DR
Delivery Risk Stratification Schemes - Limitations

• Which level is higher risk for hemodynamic instability at

birth?
• LOC 1 or LOC 4?
• LOC 4, Care Plan 5 or ENCI 4?
• Is there a Care Plan 1?
• How does this apply to my institutional practice?
• Who? Where? How fast?
Creating a Delivery Risk Stratification Scheme
Risk Stratification
Delivery Risk Stratification
Sick at Sick in First Sick in First Newborn
Delivery? 2 hours of 2 days of Admission
life? life? Location
Ventricular Septal
Defect
Tetralogy of Fallot
Hypoplastic Left
Heart Syndrome
d- TGA
HLHS with restrictive
ASD
Delivery Risk Stratification
Sick at Sick in First Sick in First Newborn
Delivery? 2 hours of 2 days of Admission
life? life? Location
Ventricular Septal No No No Normal Newborn
Nursery
Defect
Tetralogy of Fallot No No Maybe TBD

Hypoplastic Left No No Yes Cardiac ICU

Heart Syndrome
d- TGA Maybe Maybe Yes Cardiac ICU

HLHS with restrictive Yes Yes Yes Cardiac ICU

ASD
Delivery Risk Stratification
Sick at Sick in First Sick in First Admission
Delivery? 2 hours? 2 days? Location
Ventricular Septal No No No Normal Newborn
Nursery
Defect
Tetralogy of Fallot No No Maybe TBD

Hypoplastic Left No No Yes Cardiac ICU

Heart Syndrome
d- TGA Maybe Maybe Yes Cardiac ICU

HLHS with restrictive Yes Yes Yes Cardiac ICU

ASD
 Michigan Fetal Congenital Heart Disease
Delivery Risk Stratification
Delivery Category – Risk of Neonatal Compromise in Delivery Newborn Newborn
First Hour of Life due to CHD Location Provider at Admission
Delivery Location

Grey - Comfort care/Palliative Care LDRP Peds (9th floor Mother-Baby See fetal cardiology, pediatric
- Previously established plan for comfort care after delivery due to CHD palliative care notes for plan
APP)
and/or other anomalies

Green – No additional Risk LDRP L&D RN Mother-Baby See fetal cardiology note for
- Minimal or no heart disease pre-discharge or outpatient
OR recommendations
- Heart disease not needing neonatal intervention

Yellow – Low Risk LDRP Peds TBD Evaluation in NEST per fetal
- Heart disease needing evaluation in first 3-12 hours cardiology note
(9th floor APP)
Orange – Moderate Risk LDRP Peds PCTU Transfer to NEST by 1hr of life
- Ductal dependent CHD and candidate for possible in-room delivery prior to PCTU admission
(9th floor APP)

Red – High Risk OR NICU PCTU See fetal cardiology notes for
- Ductal Dependent CHD potentially needing intervention in first hour anticipated postnatal care

Purple - Cardiac Delivery OR NICU PCTU See fetal cardiology notes for
- Severe congenital heart disease with anticipated need for immediate plan
transfer for urgent cath/surgical intervention
Risk Stratification:
Respiratory Support in first 2 hours
100%
90%
80%
70%
60%
50% 97%
40%
30% 56%
47%
20% 35%
10% 7%
19% 19%
0% 3% 0% 6% 12%
0%
Passed Orange Failed Orange Red
RA NC CPAP Intubation
Poll Question # 1
• Prenatal diagnosis of a large ventricular septal defect (VSD).
What kind of DR and initial newborn care does this term
baby need?
A) Standard delivery, newborn care in regular nursery
B) Standard delivery, needs cardiac evaluation in first 6 hours of life,
location of newborn admission TBD
C) Standard delivery, needs cardiac evaluation and treatment in first
few hours, admission to ICU in anticipation of neonatal cardiac
surgery
D) High risk delivery, may be sick from CHD in the DR, may need
urgent cardiac procedure in first few hours
Scenario #1: Large Ventricular Septal Defect

• Typical course:
• Murmur develops after PVR falls – takes days
• Symptoms of CHF at 4-6 weeks
• Elective surgical repair at 4-6 months

• Standard Delivery and Postnatal Care

• Echo and cardiology consult prior to discharge
to establish care
Poll Question # 2
• Prenatal diagnosis of “standard risk” hypoplastic left heart
syndrome. What kind of DR and initial newborn care does
this baby need?
A) Standard delivery, newborn care in regular nursery
B) Standard delivery, needs cardiac evaluation in first 6 hours of life,
location of newborn admission TBD
C) Standard delivery, needs cardiac evaluation and treatment in first
few hours, admission to ICU in anticipation of neonatal cardiac
surgery
D) High risk delivery, may be sick from CHD in the DR, may need
urgent cardiac procedure in first few hours
Scenario #2: Hypoplastic Left Heart Syndrome

• Ductal-dependent for systemic blood flow

• Shock develops with PDA closure
• PDA closure takes hours
• PVR is still high, so lungs don’t “flood”
• Neonatal surgical palliation in first week

• Standard Delivery, Initiation of Cardiac

Care in First Few Hours, ICU Admission
Scenario #2: Hypoplastic Left Heart Syndrome
• In the DR, PDA is open, PVR is still high
• Should be hemodynamically stable
• Goal O2 saturations > 75%
• It’s OK to give supplemental O2 if needed
• If baby is unwell, probably not cardiac
• If baby appears well, encourage parental-
infant bonding

• Without a prenatal diagnosis, these babies

often look well enough to discharge as
“healthy” newborns!
Poll Question # 3
• Prenatal diagnosis of d-transposition of the great arteries.
What kind of DR and initial newborn care does this baby
need?
A) Standard delivery, newborn care in regular nursery
B) Standard delivery, needs cardiac evaluation in first 6 hours of life,
location of newborn admission TBD
C) Standard delivery, needs cardiac evaluation and treatment in first
few hours, admission to ICU in anticipation of neonatal cardiac
surgery
D) High risk delivery, may be sick from CHD in the DR, may need
urgent cardiac procedure in first few hours
Scenario #3: d-Transposition of the Great Arteries

• Ductal-dependent for adequate mixing

• Atrial septal defect critical for adequate
mixing
• May need urgent cath for balloon atrial
septostomy
• Neonatal surgical repair in first week

• Critical CHD
Scenario #3: d-Transposition of the Great Arteries

• In the DR, PDA is still open

• May be severely hypoxic
• May need urgent cath for balloon atrial
septostomy to make ASD bigger
• Cannot predict that this won’t be needed prenatally
• Notify cardiologists when delivery is imminent
• Give supplemental oxygen and respiratory
support as needed
General Tips for the DR*:

• If the baby is sick, it’s probably not due to the heart

disease
• Resuscitation of babies with heart disease should look
like resuscitation of a baby without heart disease
• Follow NRP
• It’s OK to give oxygen to a “cardiac baby” if they need it.
• Keep the cardiologists out of the DR for non-critical
deliveries
* Adjust accordingly based on pre-delivery
communication
Changes you may wish to make in practice:

1. Standardize what is included in prenatal cardiology

notes to aid in delivery planning/DR care.
2. Implement an institution-specific risk stratification
scheme for delivery of neonates with CHD.
3. Regularly have conversation between neonatology
and pediatric cardiology regarding upcoming
deliveries.
References

For more information on this subject, see the following

publications:

• Sanapo et al. Fetal echocardiography for planning perinatal and delivery room
care of neonates with congenital heart disease. Echocardiography. 2017.
• Pruetz JD, et al. Delivery room emergencies in critical congenital heart diseases,
Seminars in Fetal and Neonatal Medicine, 2019
• Park’s Pediatric Cardiology for Practitioners. Ed. M. Park and M. Salamat.
Elsevier. 2020