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Journal of perinatology fetal cardiology changing the definition of critical heart disease in the newborn
1. State-of-the-Art
Journal of Perinatology (2016) 36, 575–580; doi:10.1038/jp.2016.20; published online 10 March 2016
Fetal cardiology: changing the definition of critical heart disease in the newborn
M Słodki1,2, M Respondek-Liberska1,3, J D Pruetz4,5,6 and M T Donofrio7
1Department of Prenatal Cardiology, Polish Mother’s Memorial Hospital Research Institute, Łódż, Poland
2
Institute of Health Sciences, The State School of Higher Professional Education, Płock, Poland
3Department of Diagnoses and Prevention Fetal Malformations, Medical University, Łódż, Poland
4
Division of Pediatric Cardiology, Children’s Hospital Los Angeles, Los Angeles, CA, USA
5Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
6Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
7Division of Cardiology and Fetal Medicine, Children’s National Medical Center, Washington, DC, USA
Correspondence: Professor M Słodki, Department of Prenatal Cardiology, Polish Mother Memorial Hospital Research Institute, ul. Rzgowska 281/289, 93-338 Łódż, Poland. E-mail:
maciejslodki@op.pl
Received 24 November 2015; Revised 25 January 2016; Accepted 27 January 2016
Advance online publication 10 March 2016
Abstract
Infants born with congenital heart disease (CHD) may require emergent treatment in the newborn period. These
infants are likely to benefit the most from a prenatal diagnosis, which allows for optimal perinatal planning. Several
cardiac centers have created guidelines for the management of these high-risk patients with CHD. This paper will
review and compare several prenatal CHD classification systems with a particular focus on the most critical forms of
CHD in the fetus and newborn. A contemporary definition of critical CHD is one which requires urgent intervention
in the first 24 h of life to prevent death. Such cardiac interventions may be not only life saving for the infant but also
decrease subsequent morbidity. Critical CHD cases may require delivery at specialized centers that can provide
perinatal, obstetric, cardiology and cardiothoracic surgery care. Fetuses diagnosed in mid-gestation require detailed
fetal diagnostics and serial monitoring during the prenatal period, in order to assess for ongoing changes and
identify progression to a more severe cardiac status. Critical CHD may progress in utero and there is still much to be
learned about how to best predict those who will require urgent neonatal interventions. Despite improved
therapeutic capabilities, newborns with critical CHD continue to have significant morbidity and mortality due to
compromise that begins in the delivery room. Fetal echocardiography is the best way to predict the need for
specialized care at birth to improve outcome. Once the diagnosis is made of critical CHD, delivery at the proper time
and in appropriate institution with specific care protocols should be initiated. More work needs to be done to better
delineate the risk factors for progression of critical CHD and to determine which newborns will require specialized
care. The most frequently described forms of critical CHD requiring immediate intervention include hypoplastic left
heart syndrome with intact or severely restricted atrial septum, obstructed total anomalous pulmonary venous
return and transposition of the great arteries with restrictive atrial septum.
Introduction
Infants born with congenital heart disease (CHD) may require emergent treatment in the newborn period. These infants are likely to
benefit the most from a prenatal diagnosis, which allows for optimal perinatal planning. Several cardiac centers have created guidelines
for the management of these high-risk patients with CHD.1, 2, 3, 4, 5, 6, 7 This paper will review and compare several prenatal CHD
classification systems with a particular focus on the most critical forms of CHD in the fetus and newborn.
With the technologic advances seen in prenatal diagnostic ultrasound over the past decade, the detection rate for CHD has steadily
increased. Furthermore, improved earlier diagnosis capabilities in pregnancy has resulted in the observation that certain forms of CHD
can progress in utero.2, 3, 8, 9, 10, 11, 12, 13 The explanation for why certain CHD undergo progression during pregnancy is still unknown.
However, with proper monitoring perinatal planning may be optimized even in cases requiring emergent neonatal intervention.1, 2, 3, 4 A
detailed understanding of the specific features of these forms of CHD is being explored, and the criteria for predicting progression of
critical CHD is still being established. Specific fetal echocardiographic parameters may help predict the progression of critical CHD in
utero and can be monitored to determine which fetuses/newborns will require specialized care.
A contemporary definition of critical CHD is one which requires urgent intervention/treatment in the first 24 h of life to prevent death.
Such cardiac intervention may be not only life saving for the infant but also decrease subsequent morbidity.3, 6 Critical CHD cases may
require delivery at specialized centers that can provide perinatal, obstetric, cardiology and cardiothoracic surgery care. Fetuses
diagnosed in mid-gestation require detailed fetal diagnostics and serial monitoring during the prenatal period, in order to assess for
ongoing changes and identify progression to a more severe cardiac status.
The normal physiological transition that occurs at birth may result in the need for urgent neonatal intervention in the newborn with
CHD. Owing to the difference in the circulatory states of the fetus and the neonate the echocardiographic findings seen in utero for
certain critical CHD may not always accurately reflect or predict the severity of the infant’s condition after delivery. Another goal of this
review is to highlight the importance of echocardiographic examination of the fetus during the late third trimester in order to determine
the severity of critical CHD before delivery.5
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2. The in utero progression of CHD
Heart defects are the most common congenital anomalies seen in both fetuses and infants. They are responsible for a third of perinatal
deaths.14, 15 Advances in fetal echocardiography have led to increased prenatal detection rates of CHD.3 Prenatal progression of certain
forms of CHD has been described in recent literature.2, 3, 8, 9, 10, 11, 12, 13 However, the underlying cause for disease progression in many
cases remains unknown. Yamamoto and Hornberger9 suggest that further prospective multicenter investigations into the natural history
of fetal outflow tract obstruction are necessary to establish the pathogenic mechanisms and pathophysiological consequences of outflow
tract obstruction at different gestational ages, and to identify better predictors of clinical outcome critical for prenatal counseling,
development of fetal interventional strategies and perinatal management planning. Further study is needed to increase our
understanding of CHD progression in utero. This is vital to improving the management of fetuses with forms of progressive CHD that
result in critical CHD at birth.1, 2, 3, 4 For instance, progressive atrial obstruction can occur in up to 64% of fetuses with hypoplastic left
heart syndrome (HLHS) and up to 83% of fetuses with transposition of the great arteries (d-TGA) sometimes only in late third
trimester.3 Neonates with critical CHD requiring emergent neonatal cardiac intervention have higher rates of morbidity and mortality,
and so far the impact of prenatal diagnosis on the short-term outcomes for this high-risk group remains unclear.4
Proposal of new fetal classification system for critical CHD
The classic descriptions of CHD may not always accurately reflect the severity or prognosis of the condition in the fetus. For example, d-
TGA may require immediate intervention in the first hours of life after birth, or may need only medications such as prostaglandin E1
with a plan for surgery in first week.1, 2, 3, 4 The definition of critical CHD in pediatric cardiology typically includes those defects with
dependency on the ductus arteriosus after birth.16, 17 This is understandable, particularly in the case of no prenatal diagnosis, as an
infant with a ductal-dependent defect that does not receive prostaglandin E (PGE) will be in a life-threatening situation. However, the
prognosis changes markedly and stops being critical, when the defect is diagnosed prenatally and the infant is given PGE as planned
after birth. More important, for those diagnosed prenatally, critical CHD includes defects, which cannot be stabilized with a PGE
infusion alone and instead requires an intervention in the first hours of the infant’s life.1, 2, 3, 4 This is a relatively small group of
newborns with CHD that has become better defined with advancements in fetal echocardiography. The echocardiography parameters for
evaluating these critical forms of CHD are still under intense study with the goal of optimizing the perinatal and postnatal management
including choice of medical center for delivery, appropriate mode of delivery, level of perinatology and neonatology services, and
immediate access to cardiology and cardiothoracic surgery care.3, 18, 19, 20 This subset of neonates with critical CHD diagnosed
prenatally cannot be stabilized with standard medical treatment, and necessitate emergent neonatal cardiac intervention or other
specialized care within hours of life to survive.1, 2, 3, 4
These observations have led to the creation of a new classification of the CHD, which takes into account the cardiovascular changes that
occur during the transition period, which starts with cord clamping. Recently, the approach to the management of critical CHD in the
delivery room and perinatal period in neonates diagnosed prenatally has been reported by several centers with classification of fetuses
into groups based on expected compromise at delivery.1, 2, 3, 4
In the United States, Donofrio et al.3, 6 created a system for risk stratification of CHD and anticipated level of care in 2004 and
prospectively used this approach over an 8-year period (Table 1).
Table 1 - Classification presented by Donofrio et al. 3.
Full table
This system was expanded and reported by the American Heart Association in the first Scientific Statement paper for the diagnosis and
management of fetal cardiovascular disease to include palliative care and the management of CHD requiring cardiac transplantation
soon after birth.21
In Poland, Respondek-Liberska and Slodki defined four groups of fetuses with CHD and tested it retrospectively (Table 2).
Table 2 - Classification presented by Respondek-Liberska and Słodki.
Full table
Similar classifications have been reported by Berkley et al.1 and Pruetz et al.4 Berkley et al.1 evaluate the success of fetal
echocardiography in guiding delivery management in pregnancies complicate with CHD. They proposed five care plans (Table 3).
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3. Table 3 - Classification presented by Berkley et al. 1.
Full table
Pruetz et al.4 studied the outcome of CHD requiring emergent neonatal cardiac intervention with and without prenatal diagnosis.
Patients were divided into four groups of CHD (Table 4).
Table 4 - Classification presented by Pruetz et al. 4.
Full table
What is important to note is that all the classifications were designed based on regional practice patterns and available resources, and yet
they are all very similar. What these classification systems all have in common is that they identify a group of patients that need
specialized treatment in the delivery room.1, 2, 3, 4 These are critical heart defects, which need to be treated in the first hours of the
infant’s life, usually with an interventional procedure or very specialized care.1, 2, 3, 4 These patients will need carefully planned
deliveries in a high-level medical center with access to not only excellent perinatology and neonatology services but also cardiology and
cardiothoracic surgery specialties.1, 2, 3, 4 The optimal fetal echocardiographic parameters to define which heart defects meet these
criteria have been described, however in certain lesions, are still under study. The most frequently described forms of critical CHD
requiring immediate intervention include HLHS with intact or severely restricted atrial septum, obstructed total anomalous pulmonary
venous return (TAPVR) and d-TGA with restrictive atrial septum. Without early diagnosis and intervention, these infants are at high risk
of mortality and severe morbidity, including cardiac arrest, end organ failure and brain damage. Below is a description of each of these
lesions.
Hypoplastic left heart syndrome
This form of critical CHD is dependent on adequate shunting at the level of the foramen ovale (FO), which ensures not only the correct
blood circulation in the pulmonary circulation but also proper development of the pulmonary vessels. HLHS with FO restriction occurs
in frequency of 6 to 20% among fetuses.2, 18, 22, 23 Despite good postnatal cardiac care, many of these infants do not survive to undergo
the Norwood operation or die in the first 24 h after surgery.2, 24 The initial presentation in the delivery room of the infant with HLHS
and restrictive or closed FO immediately after birth may not show the severe hemodynamic abnormality that is present, resulting in
decreased vigilance of the medical team. Unfortunately, without intervention rapid clinical deterioration usually in the first few hours
after delivery then occurs. Attempts to improve outcomes for infants with HLHS and restrictive or closed FO, have used the strategy of
emergent creation of an atrial communication immediately after birth.2, 3, 4, 22 Prenatal selection of this group of fetuses is crucial in
order to coordinate the labor such that the intervention can be performed in the first few minutes to hours of life. This requires a very
well-organized plan that calls for close cooperation between the obstetrician and neonatologist for a prompt and effective initial
resuscitation with rapid transfer to the care of the cardiac surgeon or invasive pediatric cardiologist to open the atrial septum.
Echocardiographic examination late in the third trimester allows for assessment of the FO just before labor. Traditionally, the
assessment of the FO included a measure of the width of the communication, which is not necessarily predictive of postnatal status.25, 26
However, more recently it has been shown that an increased A wave (reversed wave during atrial systole) in the pulmonary veins reflects
an increased pressure in the left atrium in fetuses with HLHS. Multiple studies have shown that Doppler flow patterns in the pulmonary
veins are very helpful in determining restriction at the FO in the setting of HLHS.25, 27 The definition of ‘severe urgent’ or level of care 4
for HLHS cases reflects the current research published by several authors.28, 29, 30 Of all the Doppler parameters evaluated, a forward to
reverse velocity time integral ratio (VTIf/VTIr) of pulmonary vein flow <5 is a sensitive predictor of need for emergent postnatal atrial
septectomy.29 Further study showed that the sensitivity can be increased to predict the need of urgent intervention postnatally, if a
forward/reverse pulmonary vein VTI ratio of <3 is used.30 On the basis of these findings, it has been suggested that a classification
algorithm for HLHS fetuses be based on this Doppler data: low-risk group for infants with a pulmonary VTIf/VTIr ratio >5; medium-risk
group for the fetuses with VTIf/VTIr ratio between 3 and 5; and high-risk group with need for urgent intervention after birth for fetuses
with VTIf/VTIr ratio <3, echocardiographic. Owing to the potential for disease progression in HLHS fetuses, pregnant women carrying a
fetus with HLHS should have serial echocardiographic examinations with the final study done late in the third trimester 2 to 3 weeks
before delivery to assess the pulmonary vein flows as well changes in right ventricular function and the presence or increase of tricuspid
insufficiency.19, 29, 31 Of note is that the treatment options for fetuses with restrictive FO in HLHS are improving with the advent of fetal
cardiac intervention.32 This is important because the neonatal survival with HLHS and prenatally detected restriction of FO is 67%, and
without FO restriction is 88% (P=0.2).2
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4. Transposition of the great artery
Another important form of critical CHD is d-TGA. Differentiating d-TGA with nonrestrictive atrial septum from critical d-TGA with
restrictive atrial septum can be challenging. Several studies have suggested that the assessment of the FO and ductus arteriosus shunting
patterns in the fetus can be useful.33, 34 Investigators have suggested a highly detailed evaluation of the FO structure, movement and
excursion in to the left atrium is of the greatest benefit.35 FO flow patterns can vary during gestation and then change markedly at
birth.3, 20, 33 It may be that the gradual increase of pulmonary flow during late pregnancy initiates the process of early closure of the
FO.36 Therefore, a single assessment of the FO and the ductus arteriosus at mid-gestation may be inadequate to accurately predict the
postnatal course in d-TGA. In addition, assessment of the ductus arteriosus is important as it has been noted to be smaller and may have
abnormal flow in some fetuses with d-TGA.33 In comparison to normal fetal cardiac physiology in which 30 to 40% of total cardiac
output (right ventricular ejection) crosses the ductus arteriosus, in fetuses with d-TGA the quantity of cardiac output across the ductus
arteriosus (left ventricular ejection) is decreased to ~20%.37, 38 The single evaluation of the ductus arteriosus flow at twentieth week of
pregnancy may not predict the same physiological condition in the third trimester. For this reason, it is important to perform serial
echocardiography in the fetus with the d-TGA, with the study done late in the third trimester 2 to 3 weeks before delivery. This will help
determine which babies with d-TGA will need urgent intervention after labor or within the first hours of life. Missing a restrictive FO
could result in delivering a critical newborn that rapidly decompensates before receiving the appropriate cardiac intervention. Even
when a proper antenatal diagnosis of severe urgent d-TGA is made, not all of these infants have good outcomes despite immediate
actions including (PGE and Rashkind procedure).20 It has also been suggested that fetal echocardiogram examination be done a few
days before labor to best determine which babies will need intervenetion.2, 20
Creating a special delivery room within the treating children’s hospital may provide more rapid access to treatment and result in less
morbidity and mortality in these critical patients.3, 6 Pruetz et al. reported that need for an atrial septostomy procedure was suspected in
one of five patients (20%) diagnosed prenatally with d-TGA. In the retrospective study presented by Słodki,2 of 29 fetuses with d-TGA, 4
cases were classified as severe urgent defects and 25 cases as severe planned defects. In the postnatal analysis, 4 newborns were
additionally found that needed urgent intervention, which were classified prenatally as severe planned defects. These cases were
considered false-negative diagnosis for prediction of the condition of the newborns.2 Donofrio also reported the inability to accurately
predict the need for immediate intervention in d-TGA newborns using current criteria. After several missed cases, their protocol was
changed such that all patients with d-TGA are now treated as if an immediate cardiac intervention after birth is required.6
It may be that one of the reasons for the misclassification of the need for atrial septostomy is due to a large time gap between the last
examination and the labor. In the study from Poland, the last echocardiographic examination of the four fetuses that were not identified
prenatally to need septostomy was performed on average in the thirty-third week of pregnancy, and the labors took place at the fortieth
week of pregnancy. For this reason, it was suggested that to plan for a suitable course of treatment for the infant after labor, it is
important to monitor the defect as well as to assess the cardiovascular system’s hemodynamics accurately just before labor.2, 20 Among
fetuses with d-TGA, it is vital to confirm in the late third trimester, the patency of the ductus arteriosus and the anatomy and flow at the
FO.
Of note is that the recommendations from the American Heart Association Fetal Cardiology Scientific Statement given that there is
currently no good fetal measure to determine postnatal FO closure with a high specificity and sensitivity, is that all babies with d-TGA
should be treated as if the FO will close at deliver, and for this reason, all fetuses with this diagnosis should be delivered in a location
where there are cardiologists available to perform the septostomy, if it is needed.21, 39
Obstructed total abnormal pulmonary venous return
TAPVR with obstruction is a critical CHD with high mortality, and is still very difficult to diagnose in the fetus. In neonates, if the
diagnosis is not known, respiratory distress and decompensation may ensue rapidly after delivery, if there is substantial obstruction to
pulmonary venous return.40 Obstruction of a pulmonary venous connection results in elevated pulmonary pressures and decreased
systemic oxygen delivery. Early detection along with appropriate stabilization and early surgical repair usually results in a good long-
term prognosis,41, 42, 43 but the high morbidity encountered when not recognized until after birth makes prenatal diagnosis of this
condition particularly important.40 In some cases, such as obstructed TAPVR, immediate surgery may be required to reconnect the
pulmonary veins and decompress the pulmonary circulation. Obstruction occurs in about 42 to 48% of TAPVR cases.40, 44 The prenatal
detection of isolated obstructed TAPVR reported by Pruetz et al. is 13%.4 In Pruetz study there were no deaths in the prenatally
diagnosed patients with isolated obstructed TAPVR, but there were two deaths in those diagnosed postnatally.4 The prenatal diagnosis
of TAPVR is challenging given that often the four chambers of the heart appear normal and the pulmonary veins are often difficult to
visualize. Ganesan et al.40 recently reported that the assessment of the pulmonary vein Doppler may be beneficial in detecting this
potentially very critical heart defect.
Conclusions
Several studies have shown that prenatal diagnosis of a CHD results in significant improvement in neonatal outcome.45, 46 However, the
growing number of fetuses with CHD diagnosed prenatally sets new challenges for prenatal cardiologists, including identifying which
infants with CHD will need urgent cardiac intervention. For those diagnosed prenatally, ‘urgent’ refers to any newborn in which
intervention or specialized care in the first or second hour of life is required. When urgent intervention is expected, labor should take
place in a tertiary-care center with the appropriate specialists including maternal-fetal medicine, neonatology, pediatric cardiology,
interventional cardiology and cardiothoracic surgery, as well as the appropriate equipment (for example, incubator and catheterization
laboratory). Subspecialist availability often requires complex coordination. The most frequent CHDs requiring urgent intervention are
HLHS, d-TGA and obstructed TAPVR.1, 2, 3, 4
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5. Critical CHD may progress in utero and there is still much to be learned about prediction in the most urgent neonatal interventions.
Donofrio et al.3 showed that 53% of fetuses required emergent intervention, but standard protocols were not predictive in all cases (two
with HLHS and six with d-TGA). Pruetz et al. showed that the overall prenatal detection rate for CHD requiring emergent neonatal
cardiac intervention was low at only 38%. There may be potential for improved detection by simulating the cardiopulmonary changes
that occur at birth using maternal hyperoxygenation in the third trimester to detect hemodynamic abnormalities that may occur at
delivery in specific forms of critical CHD.47, 48, 49, 50 This hypothesis requires further evaluation.
Newborns with critical CHD continue to have high mortality rate due to compromise that begins in the delivery room.3 Fetal
echocardiography is the best way to predict the need for specialized care in the delivery room to improve outcome.1, 2, 3, 4 Once the
diagnosis is made of critical CHD, delivery at the proper time and in appropriate institution with specific care protocols should be
initiated.3 More work needs to be done to better delineate the risk factors for progression of critical CHD and to determine which
newborns will require specialized care. The fetus should be considered a patient from the time of first encounter, and care should include
comprehensive planning for delivery and postnatal management based on the specific in utero CHD diagnosis and the local resources
available.1, 2, 3, 4
Conflict of interest
The authors declare no conflict of interest.
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