Clasificacion Cardioaptias Ductus Dependientes
Clasificacion Cardioaptias Ductus Dependientes
Clasificacion Cardioaptias Ductus Dependientes
PAEDIATRICS AND CHILD HEALTH 32:9 332 Ó 2022 Elsevier Ltd. All rights reserved.
SYMPOSIUM: NEONATOLOGY
Adapted from Singh Y, Tissot C. Echocardiographic Evaluation of Transitional Circulation for the Neonatologists. Front Pediatr 2018; 6:140.
AS - aortic stenosis; ASD - atrial septal defect; APW - aorto-pulmonary window; AVSD -atrio-ventricular septal defect; CHD - congenital heart disease; COA - coarctation of
aorta; DORV - double outlet right ventricle; HLHS - hypoplastic left heart disease; IAA - interrupted aortic arch; IVS - intact ventricular septum; PA - pulmonary atresia; PDA
- patent ductus arteriosus; PS - pulmonary stenosis; SV - single ventricle; TA - tricuspid atresia; TAC - truncus arteriosus communis; TAPVC - total anomalous pulmonary
venous connection; TGA - transposition of great arteries; TOF - Tetralogy of Fallot; VSD - ventricular septal defect.
Table 1
Acyanotic congenital heart defects acute collapse or even death. The latter is more common in
Acyanotic CHDs form a spectrum of congenital heart lesions with critical CHD. Common critical acyanotic CHDs are left heart
variable presentation depending upon the underlying anatomical obstructive duct-dependent lesions such as Hypoplastic left heart
and physiological abnormality. Infants may be completely syndrome (HLHS), Critical aortic stenosis (AS), Severe coarcta-
asymptomatic presenting with a heart murmur alone or suffer tion of aorta (CoA), Interrupted aortic arch (IAA), and Single
ventricle (SV) with severe AS or CoA, which may present with
acute deterioration when ductus arteriosus closes.
Cyanotic CHDs presenting in neonatal period
5 Ts in cyanotic CHDs Clinical presentation of infant with critical CHD
The timing of presentation and severity of the presentation de-
C Tetralogy of Fallot (TOF) and double outlet right ventricle (DORV) -
pends upon:
most common cyanotic CHD (Figure 1)
1. The nature and severity of underlying defect.
C Transposition of great arteries (TGA) - most common CHD pre-
2. The alteration in cardiovascular physiology secondary to the
senting in neonatal period (Figure 2)
effect of the transitional circulation, i.e.
C Truncus arteriosus
C Total anomalous pulmonary venous connection (TAPVC) A. Closure of ductus arteriosus (DA)
C Tricuspid atresia B. Restriction of patent foramen ovale (PFO)
C Pulmonary atresia with no VSD (severe spectrum of TOF ) C. Fall in pulmonary vascular resistance (PVR)
C Ebstein anomaly (rare with variable presentation depending upon
the severity of the lesion) Critical cyanotic CHD
Cyanosis results from the presence of more than 50g/L of
Box 2 deoxygenated haemoglobin. It follows that the ability to
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SYMPOSIUM: NEONATOLOGY
Figure 1 Anatomy in tetralogy of Fallot tetralogy e the most common cyanotic congenital heart defect. Image a and b: show bigger right side of
the heart with caudally deviated interventricular septum, mal-aligned ventricular septal defect (VSD) leading to over-riding of aorta. Image c
showing VSD position at 10 O’clock with right ventricular outflow obstruction in parasternal short axis view. Image d shows similar anatomy but
with more than 50% over-riding of aorta, hence classified as double outlet right ventricle (DORV). The central image shows the flow pattern. RV e
right ventricle, LV - left ventricle, Ao e aorta (ascending aorta). Copyright Yogen Singh.
recognize cyanosis is related to the level of haemoglobin. diseases (for hyperoxia test details - see below in approach to
Cyanosis can be recognized more easily in babies with higher management).
haemoglobin concentrations. Measuring preductal saturation (right arm) and post-ductal
saturation (right leg) may show a significant difference be-
Cyanosis in an infant may be due to one of four mechanisms: tween pre- and post-ductal PaO2 indicating that the blood is
(1) shunting of deoxygenated blood from the right side of the shunted at the level of PDA from pulmonary to systemic circu-
heart to the left side systemic circulation leading to intracar- lation as it happens in cases of severe PPHN or CHDs with right
diac shunt secondary to cyanotic CHD to left ductal shunt (such as in interrupted arch of aorta).
(2) extrapulmonary right-to-left shunts at PDA or PFO in pulmo-
nary hypertension Critical acyanotic congenital heart diseases
(3) incomplete oxygenation process of blood passing through the Patients with acyanotic heart diseases may present acutely in a
lung due to lung pathology e.g. intrapulmonary shunt or critical condition either because of left-sided obstructive lesion or
(4) abnormal haemoglobin that cannot bind to oxygen. due to heart failure.
The first two mechanisms occur in cyanotic infants due to CHDs Critical acyanotic ductal-dependent heart diseases
(central mixing such as in tetralogy of Fallot’s or across PDA in presenting with shock
pulmonary atresia without VSD). When deoxygenated blood is During the fetal and transitional circulation ductus arteriosus
mixed with oxygenated blood, the resultant blood has a lower (DA) maintains the blood flow to systemic circulation bypassing
oxygen saturation than normal. This may lead to significant central the area of stenosis or interruption. When DA closes the flow to
cyanosis. This type of shunt is called an intracardiac shunt. descending aorta, in duct dependent left heart obstructive le-
One of the clinical bedside tests that can help in differenti- sions, will be comprised leading to tissue hypoperfusion and
ating intrapulmonary from intracardiac shunt is by giving 100% tissue hypoxia. This subsequently leads to lactic acidosis and
O2 through oxygen hood to the affected neonate (the hyperoxia shock. The most common acyanotic critical CHD is neonatal
test). The peripheral saturation usually improves in cases of lung coarctation of the aorta (Figure 3). Other critical CHDs lesions in
diseases associated with intrapulmonary shunt; while on the this category include other left-sided obstructive cardiac lesions
other hand, the cyanosis, O2 saturation, and PaO2 do not such as interrupted aortic arch (IAA), critical aortic stenosis (AS),
improve in cases of intracardiac shunt due to congenital heart aortic valve atresia, mitral atresia and HLHS.
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SYMPOSIUM: NEONATOLOGY
Figure 2 Transposition of great arteries (TGA) e most common cyanotic congenital heart defect presenting in the neonatal period. Image a
showing anatomy in 2 D and image b with color Doppler - pulmonary artery originating from posterior ventricle (left ventricle) and dividing soon
after origin while ascending aorta originating from anterior (right) ventricle. Copyright Yogen Singh.
Without a timely and appropriate intervention, an infant with hemodynamic stabilization and initiation of appropriate dose of
critical coarctation or severe left-sided obstruction may deterio- prostaglandin E1 infusion (see below) as soon as possible. When
rate rapidly with possible demise. Clinicians should have a low there is a clinical suspicion of acyanotic critical CHD, there should
index of suspicion of critical acyanotic CHD in infants presenting not be any delay in starting prostaglandin E1 infusion and clinicians
with shock during the first couple of weeks after birth. These should not wait for a confirmatory echocardiography or cardiologist
infants may mimic the clinical picture of septic or hypovolemic opinion to start the drug, although they should be sought urgently.
shock e with early non-specific signs and symptoms and then
sudden deterioration leading to shock. Acyanotic heart diseases presenting with heart failure
Management of infants with suspected critical acyanotic CHD This category of CHDs compromise infants with significant left to
including ductal-dependent acyanotic heart lesion should include right shunt resulting in increased pulmonary blood flow and thus
Figure 3 Critical coarctation of aorta (CoA). Image a and b show dilated right side of the heart with right ventricle (RV) being big and rounded in
image b. There is a muscular VSD seen in image b. Image c showing critical coarctation of aorta with severe narrowing at the isthmus, junction
between transverse arch and descending aorta. Image d shows classical diastolic decay seen in infants with coarctation of aorta e there is
increased velocity in systole and diastolic ‘tail’ which remains high during diastole. Copyright Yogen Singh.
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SYMPOSIUM: NEONATOLOGY
leading to heart failure. The common heart lesions include Total anomalous pulmonary venous connection (TAPVC)
ventricle septal defect (VSD), atrioventricular septal defect 2) To relieve pulmonary congestion in
(AVSD), PDA or combination of lesions. As the PVR drops after Hypoplastic left heart syndrome/hypoplastic left ventricle
birth, there is increased left (systemic) to right (pulmonary) Mitral stenosis/atresia
shunt leading to congestion of lungs from increased pulmonary 3) To permit adequate mixing of deoxygenated and oxygenated
blood flow. They often present with signs and symptoms of heart blood as in TGA with intact ventricular septum.
failure (breathlessness, tachypnoea, tachycardiac, poor feeding, These infants deteriorate when blood flow across the foramen
hepatomegaly and cardiomegaly) around 4e6 weeks after birth. ovale is restricted, despite a patent ductus arteriosus. They need
Any infant presenting with heart failure during the first 1 urgent intervention (atrial septostomy or atrial septectomy) to
e2 weeks after should be suspected to have more serious critical facilitate unrestricted blood flow via foramen ovale.
CHD such as left heart obstructive lesions.
Obstructed total anomalous pulmonary venous
An approach to understand cardiovascular physiology in connection
infants presenting as neonatal emergency Obstructed TAPVC, usually infracardiac type, presents as true
As discussed above, both acyanotic and cyanotic CHDs can time critical emergency and clinical picture mimics severe PPHN.
present as neonatal emergencies. These cardiac emergencies can However, these infants get worse with fall in PVR as compared to
be broadly divided into 3 categories: duct-dependent CHDs, cir- persistent pulmonary hypertension of the newborn (PPHN), and
culation dependent upon mixing of blood centrally via foramen they often deteriorate after starting nitric oxide to manage PPHN
ovale, and obstructed total anomalous pulmonary venous like clinical picture. Drop in PVR leads to increased pulmonary
connection (TAPVC). blood flow but it increases pulmonary congestion because
drainage in obstructed TAPVC is blocked.
Ductal dependent CHDs An urgent pediatric cardiology consultation is warranted
In neonates with duct dependent critical CHDs, closure or when a critical CHD is suspected, and a comprehensive echo-
constriction of ductus arteriosus may be associated with pro- cardiography would provide a definitive diagnosis.
found circulatory compromise. Duct dependent circulation can
be categorized in the following three categories: A simplified approach to evaluation and initial
1) To maintain adequate pulmonary blood flow in right sided management of infants with suspected congenital heart
obstructive lesions (cyanotic CHDs) disease
Pulmonary atresia with intact ventricular septum
Pulmonary atresia with ventricular septal defect The clinical presentation of CHD in the neonatal period can be
Critical pulmonary stenosis (PS) non-specific mimicking other more common conditions in in-
Tricuspid atresia with pulmonary atresia or critical (PS) fancy such as sepsis, respiratory, hypovolemia or metabolic
Univentricular heart with pulmonary atresia condition. Therefore, having a high index of suspicion and a
Severe Ebstein’s anomaly systematic approach is vitally important for the timely diagnosis
2) To maintain adequate systemic blood flow in left sided and management. The initial evaluation of an infant with sus-
obstructive lesions (Acyanotic CHDs) pected CHD should include a detailed history including obstetric
Hypoplastic left heart syndrome (HLHS) history and family history, meticulous physical examination
Critical aortic stenosis/aortic atresia including palpation of peripheral pulses and measuring pre- and
Severe or critical coarctation of aorta post-ducal saturations, and hyperoxia test. The presence of
Interrupted aortic arch (IAA) dysmorphic features and other congenital anomalies may help in
Single ventricle with severe aortic stenosis/coarctation suspecting underlying CHD.
3) To ensure adequate central mixing in conditions with parallel During the fetal period the shunt direction across ductus
circulation arteriosus is right-to-left shunt (from pulmonary artery to
Transposition of great vessels (TGA) without VSD aorta) because of high PVR and low SVR. This leads to higher
The circulation in infants with duct dependent CHDs require pre-ductal saturation than post-ductal saturation, which is
patent ductus arteriosus to keep them stable until a definitive often seen soon after birth during early transitional circulation
procedure/intervention is performed. The dose and indications and infants with persistently high PVR (such in infants with
vary depending upon timing of diagnosis and clinical pre- severe Persistent Pulmonary Hypertension of Newborn). Right
sentations as discussed in the section below. to left shunt can also be seen in certain critical CHDs such as
interrupted arch of aorta and critical aortic stenosis/aortic
Circulation dependent upon central mixing at foramen atresia.
ovale As discussed above, hyperoxia test can be useful in differ-
Certain congenital heart conditions require patent foramen ovale entiating between the cardiac and respiratory causes of
to maintain hemodynamic stability in the newborn. The cyanosis in newborns infants. It works on the assumption that
congenital heart conditions requiring unrestricted intra-atrial in infants with cyanotic CHDs or those with right to left shunt,
communication can be categorized in two sub-groups: regardless of the level of alveolar oxygenation, the desaturating
1) To maintain systemic blood flow effect of the lesion/shunt will not alter. Oxygen is administered
Tricuspid atresia through a plastic hood for at least 10 minutes in order to fill the
Pulmonary atresia with intact septum alveolar spaces completely with oxygen and arterial partial
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SYMPOSIUM: NEONATOLOGY
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SYMPOSIUM: NEONATOLOGY
time critical emergency needing urgent discussion with a pe- Park MK. Paediatric cardiology for practitioners. Philadelphia: Mosby
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The definitive diagnosis of CHD is made by diagnostic echocar-
CD011912. https://doi.org/10.1002/14651858.CD011912.pub2.
diography which should be performed by a pediatric cardiologist
Published 2018 Mar 1.
or clinician trained in performing echocardiography for congen-
Singh Y, Lakshminrusimha S. Perinatal cardiovascular physiology and
ital heart defects such Pediatrician (or Neonatologist) with
recognition of critical congenital heart defects. Clin Perinatology
Expertize in Cardiology (PEC). If a CHD is suspected on neona-
2021; 48: 573e94.
tologist performed echocardiography (NPE) or targeted neonatal
Singh Y, Mikrou P. Use of prostaglandins in duct dependent
echocardiography (TNE), even when NPE/TNE trained clinician,
congenital heart conditions. Arch Dis Child Educ Pract Ed, 2017;
the infant should be referred to the specialist pediatric cardiolo-
1e4. https://doi.org/10.1136/archdischild-2017-313654. 0.
gist. All infants with suspected critical CHDs should be discussed
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urgently with the pediatric cardiologist.
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atrics 2018; 6: 79.
Conclusion
Singh Y. Cardiovascular physiology in preterm and term infants. In: da
Infants with CHDs, even those with critical CHDs, may remain Cruz EM, Ivy D, Hraska V, Jaggers J, eds. Paediatric and congenital
completely asymptomatic during the transitional period when cardiology, cardiac surgery and intensive care. London: Springer,
fetal shunts are open and often present with non-specific signs 2021. https://doi.org/10.1007/978-1-4471-4999-6_254-1.
and symptoms. Clinicians should keep a low index of suspicion Singh Y. Evaluation of a child with suspected congenital heart disease.
of critical CHDs in infants presenting with shock or cyanosis Paediatrics Child Health 2018; 28: 556e61.
during the neonatal period. Neonatologists and acute pediatri-
cians need to have a good understanding of cardiovascular
physiology and the varied presentations of undiagnosed critical Key practice points in infective endocarditis in
CHDs. Timely and the appropriate dose of prostaglandin E1 children
infusion can be lifesaving in ductal-dependent CHDs. There
should not be any delay in starting prostaglandin E1 infusion in
C The incidence of CHD is around 8e10 per 1000 live births, and
infants with suspected critical CHD while waiting for the echo- around 25e33% of these lesions are critical lesions needing ur-
gent surgery or intervention.
cardiography or cardiology consultation. A
C The signs and symptoms of critical congenital heart defects are
often non-specific soon after birth.
C A delay in diagnosis of critical CHDs leads to poor outcome with
FURTHER READING
higher mortality and morbidities.
Bonnet D, Coltri A, Butera G, et al. Prenatal diagnosis of transposition C A high degree of suspicion is warranted in critical CHDs in infants
of great vessels reduces neonatal morbidity and mortality. Arch Mal
presenting with shock or hypoxia.
Coeur Vaiss 1999; 92: 637e40. C Understanding perinatal cardiovascular physiology is essential for
Hooper SB, Te Pas AB, Lang J, et al. Cardiovascular transition at birth:
timely diagnosis and initiate appropriate management for infants
a physiological sequence. Pediatr Res 2015; 77: 608e14. https://
with critical CHDs.
doi.org/10.1038/pr.2015.21. C Timely recognition and therapy with prostaglandin E1 infusion
Levey A, Glickstein JS, Kleinman CS, et al. The impact of prenatal
can be lifesaving in duct dependent neonatal cardiac
diagnosis of complex congenital heart disease on neonatal out-
emergencies.
comes. Pediatr Cardiol 2010; 31: 587e97.
PAEDIATRICS AND CHILD HEALTH 32:9 338 Ó 2022 Elsevier Ltd. All rights reserved.