Delivery Room Emergencies in Critical

Congenital Heart Diseases

Abstract
Transition from fetal to postnatal life is a complex process, particularly in neonates with congenital heart
disease (CHD). The resuscitation team must be well-prepared for significant interventions during delivery.
This paper reviews transitional circulation, focusing on altered hemodynamics in complex CHD, and
discusses preparing for high-risk deliveries. It also examines pathophysiology from cardiac structural
anomalies and delivery room management for specific critical congenital heart diseases.

Introduction
Understanding the normal fetal and transitional circulations is crucial for managing neonates with
congenital heart disease (CHD). Knowledge of these processes allows for anticipation of symptomatology
and effective management in the delivery room (DR). This paper provides an overview of normal fetal and
transitional circulations, altered hemodynamics in CHD, and perinatal management strategies for critical
congenital heart diseases (CCHD).

Introduction to the Hemodynamic Effects of Critical Congenital

Heart Defects at Birth
Fetal and Transitional Circulation
The normal fetal circulation involves oxygenated blood from the umbilical vein entering the portal venous
system, with about 50% bypassing the liver via the ductus venosus. The Eustachian valve directs this blood
flow toward the foramen ovale. Poorly oxygenated blood from the lower body and the superior vena cava
is directed toward the tricuspid valve. The most oxygenated blood enters the left side of the heart,
supplying the myocardium and brain. The right ventricle receives most systemic venous return, with 70-
80% bypassing the pulmonary vascular bed via the ductus arteriosus to the descending aorta. Both
ventricles contribute to systemic blood flow, with the right ventricle being dominant.

At birth, lung aeration reduces pulmonary vascular resistance, while systemic vascular resistance increases
due to the removal of placental circulation. This results in the reduction or cessation of ductus venosus
flow, reversal of ductus arteriosus flow, and functional closure of the foramen ovale.

In CHD, fetal circulation alterations vary with the structural defect, impacting fetal growth and postnatal
transition. For example, in hypoplastic left heart syndrome (HLHS), hypoplasia of the aortic and/or mitral
valve decreases forward flow, leading to left ventricular dysfunction and increased left atrial pressure,
potentially reversing foramen ovale shunt direction and compromising brain oxygenation.

Definition of Critical Congenital Heart Disease

Critical CHD includes lesions requiring emergent medical, surgical, or catheter-based interventions in the
first days to weeks of life. These lesions, such as obstructed total anomalous pulmonary venous return
(TAPVR) and HLHS with restrictive atrial septum (RAS), often necessitate immediate intervention for
stabilization and survival. Prenatal diagnosis allows for careful maternal care planning, optimizing delivery,
and targeted postnatal care.

Preparation for Resuscitation of Neonates with Critical CHD

Preparation for resuscitation includes reviewing prenatal findings, clear communication among medical
teams, and assembling the resuscitation team well in advance. Necessary equipment and supplies should
be prepared, including umbilical venous catheterization kits, saline syringes, and other relevant tools.

Perinatal Management Strategies to Optimize Postnatal Transition

Perinatal management action plans for critical CHD involve risk stratification and level of care (LOC)
assignments based on fetal echocardiography findings. These plans guide delivery, DR management, and
postnatal care. Examples of LOC management are detailed in Table 1.

Table 1: Emergent Neonatal Cardiac Intervention (ENCI) Classification System

Cardiology,
CT Surgery,
Mode Critical CTICU,
of NICU Care OR/Cath
ENCI Risk PGE Delivery Acuity Neonatologist Transport Lab on Examples
Level Dependent an Issue Level Present in DR Needed Standby of CHD

Low Risk No No Low No No No ASD, VSD,

mild PS

Intermediate No No Mid Possibly Possibly No CAVC,

Risk TOF/PS,
Truncus
Arteriosus

Moderate Possibly Possibly High Yes Yes Possibly HLHS,

Risk TOF/PA,
PA/IVS

High Risk Likely Yes High Yes Yes Yes D-

TGA/RAS,
HLHS/RAS,
obstructed
TAPVR

Overview of Critical CHD Requiring Emergent Intervention in

the DR
Critical CHD lesions can be grouped into categories based on the nature of hemodynamic compromise.
Specific management strategies for each category are provided in Table 2.

Table 2: Delivery Room Management Recommendations for ENCI Level 4 Cardiac Lesions

Cardiac Lesion DR Preparation DR Resuscitation

D-TGA with PGE available, IV and UV Initiate PGE, intubation, mechanical ventilation,
restrictive/intact atrial catheter setup consider inhaled nitric oxide, transfer to cath lab for
septum BAS

HLHS with restrictive/intact PGE available, IV and UV Initiate PGE, intubation, mechanical ventilation,
atrial septum catheter setup transfer to cath lab or OR for intervention
Cardiac Lesion DR Preparation DR Resuscitation

Severe Ebstein anomaly PGE available, IV and UV Initiate PGE, intubation, mechanical ventilation,
catheter setup consider inhaled nitric oxide, transfer to ICU

TOF/APV IV and UV catheter Prone positioning, intubation if necessary, transfer to

setup ICU

Obstructed TAPVR PGE available, IV and UV Initiate PGE, intubation, mechanical ventilation,
catheter setup consider ECMO, transfer to OR for surgical repair

Tachyarrhythmias Cardiac Monitor, IV and Cardioversion if unstable, administration of adenosine,

UV catheter setup transfer to cardiac center

CHB with HR < 55 bpm Cardiac Monitor, IV and Initiate chronotropic agent, transcutaneous pacing,
UV catheter setup intubation if needed, transfer to ICU or OR for pacing
wires

Other Considerations
Fetal Intervention Improving DR Management
Fetal interventions, such as balloon valvuloplasty for critical aortic stenosis and inter-atrial stent placement
for HLHS with RAS, can stabilize the fetus and improve neonatal outcomes. Emerging therapies, like
implantable fetal pacemakers, show potential for better postnatal clinical status.

Delayed Cord Clamping

Delayed cord clamping (DCC) offers benefits such as increased hematocrit and iron stores. While
controversial in critical CHD, recent studies suggest DCC is safe and feasible, potentially improving
outcomes.

Conclusions
Neonates with critical CHD require a well-coordinated perinatal plan and DR management strategy for
successful stabilization and transition to cardiac care. Advances in prenatal diagnostics and active perinatal
strategies hold promise for improving outcomes. Future research and innovative fetal therapies may
further enhance care for these high-risk neonates.

Learning Points
1. Understanding fetal and transitional circulation is essential for managing critical CHD in the DR.
2. Risk stratification and level of care management plans are effective for predicting postnatal care
needs.
3. Clear communication among medical teams and thorough prenatal assessment are crucial for
successful resuscitation.

Research Directions
1. Refining prenatal prediction of delivery room intervention needs in critical CHD.
2. Exploring fetal interventions to reduce the severity of CHD and the need for emergent postnatal
interventions.
References
Pruetz JD, Wang S, Noori S. Delivery room emergencies in critical congenital heart diseases. Seminars in
Fetal and Neonatal Medicine. 2019;24:101034.