Download - cijanoza novorodj uvod
-
7/27/2019 cijanoza novorodj uvod
1/21
Assessment of cyanosis in the newbornOverview
Summary
Aetiology
Emergencies
Urgent considerations
DiagnosisStep-by-step
Differential diagnosis
Guidelines
Resources
References
Images
Patient leaflets
Credits
Email
Print
Feedback
ShareAdd to Portfolio
Bookmark
Add notes
SummaryTachypnoea and cyanosis are frequently encountered in the neonatal period. The prevalence of respiratory
distress in newborns ranges from 2.9% to 7.6%. Cyanosis can result from a range of disorders, including
cardiac, metabolic, neurological, and parenchymal/non-parenchymal pulmonary disorders. In all, 4.3% of
newborns may require supplemental oxygen therapy because of cyanosis.[1] [2] [3] Cyanosis is dependent on
the absolute concentration of the reduced haemoglobin and not on the ratio of reduced haemoglobin to
oxyhaemoglobin. Cyanosis is classified into central and peripheral cyanosis. When present throughout the body,
including the mucous membranes and tongue, the condition is termed central cyanosis. When limited to the
extremities, it is termed peripheral cyanosis or acrocyanosis.
AetiologyAny of the following 5 mechanisms may give rise to arterial oxygendesaturation:
Hypoventilation
Significant right-to-left intracardiac or intrapulmonary shunting
Ventilation perfusion unevenness
Diffusion impairment
Inadequate transport of oxygen by the haemoglobin.
http://bestpractice.bmj.com/best-practice/monograph/768/overview.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/overview/summary.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/overview/aetiology.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/emergencies.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/emergencies/urgent-considerations.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/step-by-step.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/guidelines.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/references.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/patient-leaflets.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/credits.htmlhttp://bestpractice.bmj.com/best-practice/emailfriend/768/overview/summary.htmlhttp://bestpractice.bmj.com/best-practice/feedback/768/overview/summary.htmlhttp://bestpractice.bmj.com/best-practice/share/768/overview/summary.htmlhttp://portfolio.bmj.com/portfolio/add-to-portfolio.html?u=%3C;url%3Ehttp://bestpractice.bmj.com/best-practice/mybp/mybpSave.html?category=bookmark&dataKey=Cyanosis+in+the+newborn+(Assessment+of)+-+Summary&dataValue=%2Fbest-practice%2Fmonograph%2F768.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-2http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-2http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-3http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-3http://bestpractice.bmj.com/best-practice/monograph/768/overview.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/overview/summary.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/overview/aetiology.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/emergencies.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/emergencies/urgent-considerations.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/step-by-step.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/guidelines.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/references.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/patient-leaflets.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/credits.htmlhttp://bestpractice.bmj.com/best-practice/emailfriend/768/overview/summary.htmlhttp://bestpractice.bmj.com/best-practice/feedback/768/overview/summary.htmlhttp://bestpractice.bmj.com/best-practice/share/768/overview/summary.htmlhttp://portfolio.bmj.com/portfolio/add-to-portfolio.html?u=%3C;url%3Ehttp://bestpractice.bmj.com/best-practice/mybp/mybpSave.html?category=bookmark&dataKey=Cyanosis+in+the+newborn+(Assessment+of)+-+Summary&dataValue=%2Fbest-practice%2Fmonograph%2F768.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-1http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-2http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-3 -
7/27/2019 cijanoza novorodj uvod
2/21
During assessment, it is helpful to break down the aetiology into differentsystems. Cyanosis may be due to:
Cardiac causes (memorised as the 5Ts mnemonic):
Transposition of great arteries: The great arteries (the aorta and the pulmonary artery) have their origins
transposed so that the aorta originates from the right ventricle and the pulmonary artery originates from the left
ventricle. Thus, deoxygenated blood is in the aorta, giving rise to severe cyanosis. Generally, these infants have
either an intra-cardiac shunt, such as a ventricular septal defect (VSD) or an atrial septal defect (ASD). It
becomes an emergency if there is no intra-cardiac shunt.
Tetralogy of Fallot: The 4 common problems are: right ventricular hypertrophy, infundibular pulmonary
stenosis, overriding of the aorta, and a VSD. Most infants will be cyanotic at birth but a few of them will have
adequate oxygen saturation depending on the pulmonary blood flow and shunt.
Total anomalous pulmonary venous return: The pulmonary veins drain into the right atrium (instead of
the left atrium), and this condition leads to cyanosis and pulmonary congestion. The veins may also drain into
the superior vena cava, inferior vena cava, or hepatic veins. The condition is generally associated with an ASD
for intra-cardiac shunting. The condition can be partial or total, depending on whether all 4 pulmonary veins drain
into the right side or not.
Truncus arteriosus: 1 large vessel originates from both ventricles, instead of 2 vessels (the aorta and the
pulmonary artery). It is associated with a VSD. This gives rise to cyanosis and increased pulmonary blood flow.
Tricuspid atresia: right atrial blood is forced to shunt through foramen ovale or ASD to the left atrium;
most patients also have a VSD. This condition is usually associated with a hypoplastic right ventricle.
Other less common cardiac causes:
Pulmonary atresia: generally associated with a VSD but may occur with intact ventricular septum, in
which case pulmonary blood flow depends on the patent ductus arteriosus (PDA).
Ebstein's anomaly: the tricuspid valve is abnormal with 2 of its leaflets displaced towards the right
ventricle with subsequent atrialization of the right ventricle. The right atrium is significantly enlarged and there is
regurgitation of the tricuspid valve; shunt takes place from right atrium through foramen ovale to left atrium.
Left-to-right shunt with pulmonary oedema: generally this takes place if there is a VSD or PDA. Cyanosis
is less severe than in conditions where there is right to left shunt but they have more severe respiratory distress.
Single ventricle states (hypoplastic left or right heart)
Low cardiac output states - CHF.
-
7/27/2019 cijanoza novorodj uvod
3/21
Pulmonary causes:[4]
Parenchymal
o Respiratory distress syndrome due to surfactant deficiency in pre-term and surfactant protein B
deficiency in full-term infantsView image
o
Transient tachypnoea of the newborn due to delayed clearance of fetal pulmonary fluidViewimage
o Aspiration (meconium,View image blood, mucus, or milk) can give rise to atelectasis or chemical
pneumonitis
o Pneumonia
o Pulmonary haemorrhage (seen in coagulopathy, asphyxia, or left to right shunts with pulmonary
oedema)
o Pulmonary oedema
o Pulmonary hypoplasia (history of oligohydramnios, or congenital diaphragmatic hernia)
o Pulmonary lymphangiectasia: a rare congenital condition with pulmonary subpleural, interlobar,
perivascular and peribronchial lymphatic dilatation, leading to significant respiratory distress at birth.
Non-parenchymal
o Tracheo-oesophageal fistula/oesophageal atresiaView image
o Congenital diaphragmatic herniaView image
o Congenital cystic adenomatoid malformationView image
o PneumothoraxView image
o Pleural effusionView image
o Upper airway obstruction (choanal atresia, laryngeal web, laryngomalacia, subglottic stenosis,
and vocal cord paralysis)
o Lobar emphysema
o Persistent pulmonary hypertension of the newborn.
Other important conditions to consider include:
Methaemoglobinaemia
Sepsis giving rise to apnoea. Pulmonary haemorrhage may be seen in severe cases, in the presence of
disseminated intravascular coagulation
Hypoglycaemia leading to apnoea or seizures
http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/2.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/2.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/3.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/6.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/4.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/5.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/7.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/10.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/2.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/2.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/3.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/6.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/4.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/5.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/7.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/10.html -
7/27/2019 cijanoza novorodj uvod
4/21
Polycythaemia, usually does not result in central cyanosis, but may give rise to increased pulmonary
vascular resistance and delayed clearance of fetal pulmonary fluid; acrocyanosis is frequently seen
Asphyxia leading to cerebral oedema and intra-cranial hemorrhage. Cyanosis is a result of the
subsequent hypoventilation
Arteriovenous malformation leading to (high output) CHF.
Other disorders of haemoglobin (haemoglobin M and sulfhaemoglobinaemia)should be considered in rarer instances. Cyanosis can also result in anyseverely ill infant when a range of other features are present (e.g., metabolicacidosis, seizures).
Urgent considerationsSee Differential Diagnosis for more details
All cases of newborn cyanosis persisting beyond the first 5 minutes postpartum require immediate attention.
Most cases of persisting newborn cyanosis are due to cardiopulmonary causes that require rapid intervention
with a goal of maintaining oxygen saturation >90%.
Oxygen administration should occur prior to evaluation of the underlying cause.
Oxygen saturation should be maintained >90%.
If hyperoxia test is positive (i.e., a rise in arterial PO2 after 15 minutes exposure to 100% oxygen),
assisted ventilation (nasal CPAP or intubation and ventilation) may be required for infants with severe cyanosis.
Vascular access should be established for infusion of drugs and/or fluids:
Fluids and vasopressors (dopamine, dobutamine, or epinephrine) should be given for hypotension and
shock.
Antibiotics should be given if there is evidence of sepsis or pneumonia.
Prostaglandins for patency of ductus arteriosus in suspected ductal-dependent congenital cardiac
conditions.
Administration of alprostadil (prostaglandin E1) as an IV infusion is life-saving in infants with ductal-dependent
cyanotic cardiac lesions. Treatment should therefore be initiated without delay if this cause is suspected, keeping
in mind that apnoea results from alprostadil administration.
Cyanotic infants should be transferred to a tertiary care centre immediately for further management where
balloon septostomy and/or surgery may be necessary. Symptomatic hypoglycaemia and hypocalcaemia should
http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html -
7/27/2019 cijanoza novorodj uvod
5/21
be corrected with IV administration of glucose or calcium, as these conditions can give rise to apnoea (with or
without seizures).
Red flags
Transposition of great arteries (TGA)
Congenital diaphragmatic hernia (CDH)
Upper airway obstruction
Asphyxia
Hypoglycaemia
Neonatal sepsis
Total anomalous pulmonary venous return (TAPVR)
Hypoplastic left heart syndrome or single ventricle physiology states
Tricuspid atresia
Pulmonary haemorrhage
Pleural effusion
Arteriovenous malformation
Step-by-step diagnostic approachInitial assessment of infants with cyanosis should include:[4]
History
Physical examination
CXR
FBC with differential count
Blood glucose
Calcium (hypocalcaemia associated with CNS irritability/cyanotic heart disease)
Pulse oximetry
ABG/hyperoxia test
Blood culture/sepsis screen
ECG and echocardiogram.
Maternal history
http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-1http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-20http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-22http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-25http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-27http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-28http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-3http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-4http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-5http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-14http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-23http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-621708http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-1http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-20http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-22http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-25http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-27http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-28http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-3http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-4http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-5http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-14http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-23http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html#expsec-621708http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4 -
7/27/2019 cijanoza novorodj uvod
6/21
Important features of maternal history predisposing to paediatric disordersinclude:
Diabetes
o Transient tachypnoea of newborn
o Respiratory distress syndrome
o Hypoglycaemia
o Large for gestational age
Asthma
o Transient tachypnoea of newborn
Use of opiates
o Respiratory depression from opiates
Pregnancy-induced HTN
o Intrauterine growth retardation
o Polycythaemia
o Hypoglycaemia
Polyhydramnios
o Tracheo-oesophageal fistula/oesophageal atresia
Delivery of previous sibling with respiratory distress syndrome
o Surfactant protein B deficiency
o Group B streptococcal pneumonia
Oligohydramnios
o Pulmonary hypoplasia.
Factors relating to labour and delivery
-
7/27/2019 cijanoza novorodj uvod
7/21
Premature and prolonged rupture of membranes
o Sepsis
o Pneumonia
Epidural anaesthesia
o Fever
Anaesthesia/analgesia
o Respiratory depression
o Apnoea
o Cyanosis
Asphyxia
o Cerebral oedema
o Metabolic acidosis
Chorioamnionitis
o Sepsis
Caesarean section without labour
o Transient tachypnoea of the newborn
o Respiratory distress syndrome
o Persistent pulmonary hypertension of the newborn
Breech delivery (trauma)
o Erb's palsy with phrenic nerve palsy.
Physical examination: overviewCyanosis can develop immediately or several hours after birth.
Immediate:
-
7/27/2019 cijanoza novorodj uvod
8/21
Transient tachypnoea of newborn
Respiratory distress syndrome
Pneumothorax or air leak
Meconium aspiration syndrome
Congenital diaphragmatic hernia
Congenital cystic adenomatoid malformation.
Onset hours after birth:
Cyanotic congenital heart disease
Aspiration
Tracheo-oesophageal fistula.
General examinationIn a cold environment, acrocyanosis is common. Examination should beperformed under a radiant warmer in a well-lit room. Prolonged capillary refilltime is often evident when peripheral perfusion is suboptimal in:
Hypoplastic left heart syndrome
Sepsis/pneumonia
Polycythaemia
Acidosis
Hypovolaemic states
Hypothermia.
Respiratory examinationTachypnoea, retractions, nasal flaring, and grunting generally indicate apulmonary cause. However, in cardiac conditions with significant left-to-rightshunt these symptoms may also occur. In cyanotic heart disease andmethaemoglobinaemia, the respiratory rate may be normal. Apnoea andcyanosis may be due to sepsis, asphyxia, or seizures.
-
7/27/2019 cijanoza novorodj uvod
9/21
Upper airway obstruction may be evident from stridorous breathing due to:
Laryngotracheomalacia
Subglottic stenosis
Vocal cord paralysis
Glossoptosis with micrognathia (Pierre-Robin syndrome).
Upper airway obstruction can also give rise to supraclavicular,submandibular, and suprasternal retractions. Bilateral choanal atresiagenerally gives rise to significant retractions at birth, but the symptoms arerelieved by an oral airway. Cyanosis due to parenchymal lung disease isassociated with intercostal and subcostal retractions.
Absent/poor aeration of one side of chest on auscultation may be due to:
Pneumothorax
Pleural effusion
Atelectasis
Congenital diaphragmatic hernia (CDH).
Cardiovascular examinationNormal heart rate in full-term newborns is approximately 120 beats perminute (bpm), with a range of 100 to 140 bpm. During quiet states it may beas low as 90 bpm. Heart rates >160 bpm during quiet state are abnormal. Insupraventricular tachycardia, heart rates >200 bpm are usually seen. Heartrate variability is also important: in severe sepsis and asphyxia, the beat-to-beat variability will be lost. S2 is loud and narrowly split in pulmonary HTN.
A single S2 is generally indicative of:
Severe pulmonary stenosis
Pulmonary atresia
Abnormal single valve position (transposition of great arteries)
A large semilunar valve, as in truncus arteriosus.
-
7/27/2019 cijanoza novorodj uvod
10/21
It is important to record the type and quality of murmurs. The location ofapical impulse should be noted to rule out dextrocardia. Precordial thrillindicates significant murmur of grade >3/6. Precordial hyperactivity isgenerally due to increased ventricular activity. It should be remembered thatnot all heart murmurs are pathological and that many cardiac conditions do
not cause murmurs.
Abdominal examinationThe abdomen may appear scaphoid in congenital diaphragmatic hernia.
Abdominal distension due to bowel obstruction or ascites can give rise torespiratory distress. Hepatosplenomegaly with ascites and hydrops fetalis, asseen in cases of severe haemolytic disease of the newborn, can lead tosevere respiratory distress. Hepatomegaly can co-exist with pulmonarycongestion in conditions such as total anomalous pulmonary venous return.
Absence of bowel sounds indicates ileus and is associated with sepsis,gangrenous bowel, or peritonitis.
CNS examinationHypotonia is one of the earliest signs of:
Sepsis
Asphyxia
Metabolic disorders.
Hypertonia is often seen in narcotic withdrawal. Phrenic nerveparesis/paralysis can precipitate respiratory distress and is associated withErb's palsy following traumatic vaginal delivery. Excessive traction on theneck or vaginal breech extraction are the common aetiologies.
CXR
CXR is integral to initial assessment of respiratory distress.[4] [5] [6] Thelocation of stomach, liver, and heart should be ascertained to rule outdextrocardia and situs inversus.
The size and shape of the heart may yield some clues to the diagnosis:
A small heart may be due to hypovolaemia, adrenal insufficiency from asphyxia or adrenal
haemorrhage, significant pulmonary interstitial emphysema, and congenital lobar emphysema.
http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-5http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-5http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-6http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-6http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-4http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-5http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-6 -
7/27/2019 cijanoza novorodj uvod
11/21
Severe cardiomegaly is present in Ebstein's anomaly. Moderate cardiomegaly is seen in infants with
diabetic mothers (hyperinsulinaemia) and in cardiomyopathy (infections, metabolic disorders, or asphyxia) and
congestive cardiac failure. CHF without intrinsic cardiac anomaly is seen in coarctation of the aorta and
arteriovenous malformation.
The commonly described cardiac silhouettes in congenital heart disease are:
o "Egg on end" appearance of transposition of the great vessels
o "Snowman" sign (a rounded, figure-of-eight-like cardiac contour) of total anomalous pulmonary
venous return
o Boot-shaped heart of tetralogy of Fallot.
Increased pulmonary vascular markings and pulmonary congestion are
indicative of left-to-right shunt, while decreased pulmonary vascular markings(oligaemic lung fields) indicate pulmonary stenosis or pulmonary atresia withinadequate ductal shunting. Decreased pulmonary vascular markings mayalso occur in persistent pulmonary hypertension of the newborn.
The expansion of lungs (lung volume) on both sides should be checked.Normal inspiratory films should have 8 intercostal spaces of lung fields onboth sides. Diaphragmatic paralysis (more commonly seen on the right side)is manifested by elevation of the right hemidiaphragm by more than 2intercostal spaces compared to the left side. This may simulate right lower
lobe atelectasis. Hyperinflated lung fields are seen occasionally in lobaremphysema or cystic lesions of lungs. The prevalence of spontaneous airleaks giving rise to pneumothorax View image and pneumomediastinum ViewimageView image in full-term newborns is approximately 1% to 2%.
Some characteristic pulmonary findings on CXR are associated with specificpathologies:
Transient tachypnoea of the newborn: lung fields may appear hazy with normal lung volume and
increased parahilar markings, frequently with fluid in the horizontal fissure.View image
Respiratory distress syndrome (RDS): CXR may not be reliable in the initial stages. As RDS worsens,
the characteristic reticular granular pattern and air bronchograms become evident. Lung volume is reduced
significantly in severe RDS.View image
Meconium aspiration syndrome: fluffy infiltrates, patchy areas of atelectasis, and areas of hyperinflation
due to air trapping may be evident.View image
http://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/7.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/9.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/9.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/8.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/2.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/3.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/3.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/7.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/9.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/9.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/8.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/2.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/1.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/3.html -
7/27/2019 cijanoza novorodj uvod
12/21
Pleural effusion (due to chylothorax or other causes): visible on lateral aspect of lung fields as a linear
opacity. In large effusions, a whole lobe may appear opaque, with mediastinal shift to the contralateral side.View
image
Lobar atelectasis: mediastinal shift towards the ipsilateral side is seen. In pneumonic consolidation,
which may simulate atelectasis, no mediastinal shift is present.
Congenital diaphragmatic hernia: soon after birth, a large area of opacity may be the only finding instead
of the classical finding of bowel gas in the thorax.View image This should be differentiated from congenital cystic
adenomatoid malformation.View image
It is important to check the bony thoracic cage. In asphyxiating thoracicdystrophy, the thoracic cage will be small and narrow. The thorax may have abell-shaped appearance in infants with severe hypotonia. Fractures of theribs, humerus, or clavicles should be looked for following difficult vaginaldeliveries, as infants may develop respiratory distress from the pain and
splinting of the chest.
UltrasoundUltrasound examination of diaphragmatic motion during spontaneousbreathing can detect paradoxical motion in diaphragmatic paralysis. Pleuraleffusion, eventration of the diaphragm, and size/location of the liver/spleencan also be determined.
Other imagingCT scan of the chest is helpful if diagnosis is not clear, and it can identifycongenital abnormalities and tumours of the mediastinum, lungs, and heart.Feeding-associated cyanosis may be due to incoordination of sucking andswallowing, vocal cord paralysis, laryngeal cleft, or severe birth asphyxia.Upper GI contrast studies should be obtained to rule out severe gastro-oesophageal reflux and oesophagitis.
Pulse oximetryPulse oximetry monitoring is recommended for all infants with respiratorydistress and cyanosis, since clinician assessment of colour at birth is wellrecognised to vary.[7] It is an accurate, reliable, and non-invasive method formonitoring oxygen saturation in infants.[8] [9][10] [11] In severe cyanosiswith respiratory distress, both preductal and postductal oxygen saturationsshould be monitored to detect the gradient across the ductus arteriosus. Forthis procedure, the pulse oximeter probes should be placed over the righthand and a lower extremity. Pulse oximetry screening has been shown to be
http://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/10.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/10.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/4.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/5.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-7http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-7http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-7http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-8http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-8http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-8http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-9http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-9http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-10http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-10http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-11http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-11http://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/10.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/10.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/4.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/images/print/5.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-7http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-8http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-9http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-10http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-11 -
7/27/2019 cijanoza novorodj uvod
13/21
helpful in early detection of congenital cardiovascular malformations in thenewborn nursery.[12]Acrocyanosis is a common finding in the first fewminutes after birth. This generally resolves spontaneously. If a low-pulseoximeter reading persists, it may be appropriate to proceed to a hyperoxiatest.
Pulse oximetry screening is helpful in the early detection of congenitalcardiovascular malformations in the newborn nursery, and may detect life-threatening malformations before symptoms develop.[12] [13] There is asignificant body of evidence suggesting that early detection of congenitalcardiovascular heart disease (CCHD) through pulse oximetry monitoring is aneffective strategy for reducing morbidity and mortality rates in young children.[13]A working group has identified strategies and made recommendations forhospitals and birthing centres to implement the use of pulse oximetry forscreening for CCHD in newborns, which should complement physical
examinations.[13] ]
Hyperoxia testThe hyperoxia test is a further clinical tool to differentiate between cardiacand pulmonary aetiologies. It is indicated if the pulse oximeter reading is
-
7/27/2019 cijanoza novorodj uvod
14/21
Laboratory testsABG can help in determining the oxygenation, ventilation, and acid-basestatus of the infant. In methaemoglobinaemia, the PO2 is normal even withcyanosis.
FBC with differential count is an important test to rule out polycythaemia,anaemia, neutropenia, leukopenia, abnormal I:T (immature to total neutrophilcount) ratio, and thrombocytopenia as signs of sepsis.
If sepsis is suspected, a blood culture should be obtained and spinal tapperformed before antibiotic therapy is started.
Significant metabolic acidosis may indicate cardiac failure, sepsis, asphyxia,or metabolic disorders. Closure of the ductus arteriosus in an infant with a
ductal-dependent cardiac lesion can lead to shock and severe metabolicacidosis with cyanosis and respiratory distress.
Calcium and magnesium levels should be obtained where other causes havebeen ruled out. Hypocalcaemia and hypomagnesaemia are both associatedwith CNS irritability and seizures.
Metabolic screening of urine and drug screening of urine/meconium shouldbe performed as clinically indicated.
ECGAn ECG is an important test, but of limited value except in certain specificconditions.[15] [16]Normally, there is right ventricular predominance in thenewborn, and many cases of cyanotic congenital heart disease (CHD) willhave similar findings. Left axis deviation with left ventricular dominance isseen in tricuspid atresia or pulmonary atresia with an intact ventricularseptum. Left axis deviation is frequently associated with right ventricularhypertrophy as seen in arteriovenous canal malformation. ECG is importantin the diagnosis of arrhythmias.
EchocardiogramEchocardiogram is the definitive test in the diagnosis of congenital cardiaclesions and pulmonary HTN. A technician who is trained in performingechocardiograms in newborns and a paediatric cardiologist are required forthis procedure.
http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-15http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-15http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-15http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-16http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-16http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-15http://bestpractice.bmj.com/best-practice/monograph/768/resources/references.html#ref-16 -
7/27/2019 cijanoza novorodj uvod
15/21
Differential diagnosisSort by: common/uncommonorcategory
Commonhide allTransposition of great arteries (TGA)
see our comprehensive coverage of Overview of congenital heart disease
History Exam 1st test
incidence is higher in male infants and
infants of diabetic mothers; cyanosis
appears within the first 24 hours,
sometimes with no respiratory distress; if
TGA is present with an intact ventricular
septum, the infant will be very cyanotic,
requiring immediate intervention
prominent right ventricular heave
and a single second heart sound (a
loud A2) are usually present; a
systolic murmur due to increased
pulmonary blood flow may be
heard in a few cases
echocardiogram: definitive diagno
reveals abnormal position of the ao
pulmonary arteries, abnormal cardi
and function
CXR: "egg on a string" appearance
cardiomegaly and increased pulmo
markings
Tetralogy of Fallot (TOF)
see our comprehensive coverage of Tetralogy of Fallot
History Exam 1st test
cyanosis depends on the degree of right ventricular outflow
tract obstruction, which ranges from very mild to severe; the
age of symptom onset is variable; VACTERL (vertebral
anomalies, imperforate anus, cardiac lesions, tracheo-
oesophageal fistula, renal and limb anomalies) are seen in
approximately 15% of infants who have TOF; infants may be
referred for mild cyanosis or presence of a murmur at birth
prominent right
ventricular heave and
systolic ejection
murmur at left sternal
border are usually
present
CXR: boot-shaped heart
echocardiogram: diagno
revealing characteristic c
and function
Pulmonary atresiasee our comprehensive coverage of Overview of congenital heart disease
History Exam 1st test
variable presentation depends on the presence
of ventricular septal defect (VSD) and ASD
for adequate mixing of blood at the atrial or
ventricular level; mild to moderate cyanosis
becomes worse when ductus arteriosus closes
murmur of patent
ductus arteriosus is
the only common
finding
CXR: may reveal decreased pulmonary
vascular markings
echocardiogram: definitive diagnostic test t
reveals pulmonary atresia, abnormal cardiac
anatomy and function
Respiratory distress syndrome (RDS)
see our comprehensive coverage of Acute respiratory distress syndrome
History Exam 1st test
generally occurs in preterm infants due to surfactant deficiency;
antenatal history may reveal immature lung profile in the amniotic
fluid; higher incidence has been noted in infants of diabetic
mothers; full-term infants with RDS have been reported to have
surfactant protein B deficiency; majority of preterm infants with
tachypnoea, nasal flaring,
grunting, and retractions with
cyanosis; in severe cases,
diminished air entry is present
on chest auscultation
CXR: reticular g
bronchograms;
cases, decrease
volumeMore
http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis/by-category.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/701.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/374.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis/by-category.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/701.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/374.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html -
7/27/2019 cijanoza novorodj uvod
16/21
RDS are born to mothers who did not receive antenatal
corticosteroids
Transient tachypnoea of the newborn (TTN)
History Exam 1st test
higher incidence among full-term newborns born by
elective caesarean section; tachypnoea is the presentingsymptom with increased FiO2 requirement; self-limiting
problem; generally resolves within the first 3 days;
cyanosis may occur in severe cases
tachypnoeic infant with
reasonable air entrybilaterally upon
auscultation
CXR: good lung volume wit
markings along with fissura
markings on the right horizo
fissureMore
Persistent pulmonary hypertension of the newborn (PPHN)
History Exam 1st test
occurs in full-term and post-term infants commonly;
aetiology is variable; risk factors include history of
asphyxia, meconium aspiration syndrome, sepsis,
congenital diaphragmatic hernia, pulmonary hypoplasia
loud S2 and right
ventricular heave
CXR: depends on the precipitatin
aspiration syndrome, sepsis/pneu
diaphragmatic hernia
echocardiogram: definitive diag
pulmonary artery pressure, tricus
ventricular size and function
Pneumothorax
see our comprehensive coverage of Pneumothorax
History Exam 1st test
can occur spontaneously at birth in full-term newborns or
following resuscitation with positive pressure ventilation;
occurs frequently in preterm infants with respiratory
distress syndrome; assisted ventilation and CPAP
contribute to the development of pneumothorax; higher
incidence in meconium aspiration syndrome, pulmonary
hypoplasia, and congenital diaphragmatic hernia
tachypnoea and cyanosis are usually present;
depending on severity (proportionate to the amount
of free air in the pleural space), breath sounds and
heart sounds may be faint/distant; mediastinal shift
to the contralateral side may occur; transillumination
of the chest will be positive in most cases
CX
co
vis
ma
Aspiration pneumonia
see our comprehensive coverage of Aspiration pneumonia
History Exam 1st t
may be due to meconium aspiration syndrome, blood, milk aspiration, or amniotic
fluid; meconium aspiration is commonly seen in post-term infants and those who
develop fetal distress; a hx of thick meconium-stained amniotic fluid at the time of
rupture of membranes is characteristic; most infants become symptomatic with
respiratory distress at birth; in the case of blood aspiration, there may be a hx of
antepartum haemorrhage; in the case of milk aspiration, emesis following feeding
and subsequent development of respiratory distress is usually present
tachypnoea, grunting, retractions,
and cyanosis are present; on
auscultation diminished air entry,
rales, and rhonchi may be evident
Pneumonia
see our comprehensive coverage of Overview of pneumonia
History Exam 1st test
http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/504.htmlhttp://bestpractice.bmj.com/best-practice/monograph/21.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1113.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/504.htmlhttp://bestpractice.bmj.com/best-practice/monograph/21.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1113.html -
7/27/2019 cijanoza novorodj uvod
17/21
risk factors include a hx of prolonged rupture of
membranes, chorioamnionitis, or positive maternal
group B beta streptococcus (GBS) screen; congenital
pneumonia due to viruses or bacteria is rare; early-onset
GBS sepsis may present as GBS pneumonia with
apnoea and circulatory collapse within the first week of
life
respiratory distress with tachypnoea,
retractions, and grunting may be present;
auscultation often reveals rales, rhonchi,
and diminished air entry in a few cases;
apnoea may be the only presenting
symptom in some cases
CXR: diagnostic
GBS sepsis may
distress syndrom
FBC: abnormal;
thrombocytopen
amounts of imma
abnormal I:T (im
ratio (>0.2) is pre
blood culture: m
Pulmonary oedema
History Exam 1st test Othe
generally due to congestive cardiac
failure; underlying cardiac disease,
AV malformation, or severe anaemia
may be present
tachypnoea, tachycardia, and
hepatomegaly are usually present;
severe pallor or signs of AV
malformation present (bruit)
CXR: fluffy infiltrates or hazy
lung fields and associated
cardiomegaly
Congenital cystic adenomatoid malformation (CCAM)
History Exam 1st test
most of these cases are diagnosed by antenatal ultrasound; the
severe forms may lead to hydrops in the fetus; milder forms
may have mild respiratory distress or no symptoms at birth;
many infants are diagnosed in childhood when they present
with recurrent infections
tachypnoea and mild cyanosis are
present in many infants;
diminished air entry on the
affected side will be present
CXR: multiple c
opaque areas in
lungMore
Congenital diaphragmatic hernia (CDH)History Exam 1st test Othe
most cases are diagnosed by antenatal
ultrasound; more common on the left
side; most cases are symptomatic at
birth with severe cyanosis; some have
minimal symptoms and are diagnosed
later in the neonatal period or infancy
scaphoid abdomen at birth,
diminished air entry on the left side
of chest, with cyanosis at birth are
typical features; heart sounds may
not be heard on the left side of
chest due to mediastinal shift
CXR: diagnostic with intestinal
gas pattern in the left
hemithorax with mediastinal
shiftMore
ABG: severe hypoxia and
hypercarbia
Upper airway obstruction
see our comprehensive coverage of Central airway obstruction
History Exam 1st test Other t
most become symptomatic shortly after birth
with no significant cyanosis; symptoms worsen
during feeding, with stridorous breathing; those
with vocal cord paralysis will have a weak or
non-existent cry; cyanosis may develop; risk of
submandibular, suprasternal, and
supraclavicular retractions are
characteristic of upper airway
obstruction; infants with choanal
atresia or stenosis may have mild to
CXR and lateral
view of
neck:sometimes
the airway calibre
http://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1069.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1069.html -
7/27/2019 cijanoza novorodj uvod
18/21
aspiration during feeding; infants with vocal
cord paralysis may have CNS conditions, such
as meningomyelocele with Arnold-Chiari
malformation and hydrocephalus
moderate symptoms depending on
whether unilateral or bilateral;
inability to pass nasogastric tube is
diagnostic
can be
demonstrated
Polycythaemia
History Exam 1st te
polycythaemia is common in insulin-dependent
diabetes mellitus, small for gestational age
infants, and twin-to-twin transfusion; maternal hx
of diabetes and HTN during pregnancy are
frequent; slightly higher incidence of transienttachypnoea of the newborn may be present
either large for gestational age or small for gestational age infants
who may appear cyanotic but have normal ABGs; some infants may
have transient tachypnoea of the newborn or mild persistent
pulmonary HTN of newborn; infants appear ruddy and plethoric, and
have a higher incidence of acrocyanosis; clinical examination may benormal or findings of transient tachypnoea of the newborn may be
present
Asphyxia
History Exam 1st test
hx of fetal distress with perinatal asphyxia and low
Apgar scores requiring vigorous resuscitation; hx of
seizures or apnoea may be the presenting symptom;
may lead to hypoxic ischaemic encephalopathy
neurologically depressed, hypotonia, subtle
seizure activity, with good aeration to both
lung fields; apnoea or hypoventilation
(bradypnoea) is the cause for cyanosis
CXR: normal
serum
creatinine: elev
liver function
tests: raised AL
Methaemoglobinaemia (met-Hb)
History Exam 1st test
usually well in spite of characteristic skin
discoloration, termed pseudocyanosis; condition may
worsen with SOB and CNS features including
seizures; may be congenital or acquired; acquired
form may result from exposure to drugs/toxins
known to cause met-Hb
characteristic blue colour, dyspnoea, mental
state changes; arterial blood with elevated
methaemoglobin levels has a characteristic
chocolate-brown colour; pulse oximetry is
unreliable
ABG: normal
PaO2More
Hypoglycaemia
see our comprehensive coverage of Non-diabetic hypoglycaemia
History Exam 1st test
either a small for gestational age or
large for gestational age infant born
to a diabetic or hypertensive woman
may be jittery or may have seizures; clinical examination may
be normal or may reveal hypo- or hypertonia; cyanosis is
usually due to apnoea or transient persistent pulmonary
hypertension of newborn
blood sugar: s
hypoglycaemic
(
-
7/27/2019 cijanoza novorodj uvod
19/21
Neonatal sepsis
see our comprehensive coverage of Sepsis
History Exam 1st test
hx of premature rupture of membranes,
chorioamnionitis, or intrapartum maternal
fever may be present; may present with
apnoea and cyanosis or with circulatory shock;
history of lethargy, poor feeding, or emesis,
temperature instability and hypo- or
hyperglycaemia may be present
infants may be hypotonic with normal or
poor peripheral perfusion and hypotension;
cyanosis in most cases is due to
hypoventilation or apnoea and in some cases
associated persistent pulmonary HTN of
newborn is also present; chest examination
is normal
FBC: leukopenia,
neutropenia, elevated
ratio, thrombocytopen
ESR: elevated
blood culture: pathog
organism cultured
Uncommonhide allTotal anomalous pulmonary venous return (TAPVR)
see our comprehensive coverage of Overview of congenital heart disease
History Exam 1st test
clinical condition depends on the
presence of pulmonary venous
obstruction; pulmonary congestion
(pulmonary oedema) with respiratory
distress and cyanosis can be mistaken
for interstitial pneumonitis
prominent right ventricular heave; widely
split S2; systolic ejection murmur at the left
upper sternal border may occur;
hepatomegaly is common; response to
alprostadil (prostaglandin E1) infusion is
generally ineffective to minimal
CXR: cardiomegaly; "snowma
congestionMore
echocardiogram: definitive d
reveals pulmonary venous dra
characteristic cardiac anatom
Hypoplastic left heart syndrome or single ventricle physiology states
see our comprehensive coverage of Overview of congenital heart disease
History Exam 1st test
most cases are diagnosed prenatally; more common in boys; if
unrecognised at birth, infants become symptomatic at the time of the
closure of the ductus arteriosus; when the ductus arteriosus closes,
respiratory distress develops, shock and cyanosis with poor
peripheral perfusion generally occur within the first 3 days of life;
severe cyanosis can occur at birth when the foramen ovale is closed
or restrictive
right ventricular
heave and single
S2 are present
CXR: cardiomegaly with s
pulmonary congestion
echocardiogram: definitiv
that reveals abnormal card
function
Tricuspid atresia
see our comprehensive coverage of Overview of congenital heart diseaseHistory Exam 1st test
most cases are diagnosed antenatally by fetal echo;
symptoms depend on the presence of ventricular septal
defect (VSD); if there is a large shunt through the VSD,
many infants may have mild cyanosis or none in the
neonatal period; many infants present with CHF later; if
the VSD is very restrictive, severe cyanosis may develop
systolic ejection
murmur at the left upper
sternal border and
prominent left
ventricular impulse
CXR: depends on the degree
blood flow and may present w
congestion
echocardiogram: definitive d
that reveals tricuspid atresia;
http://bestpractice.bmj.com/best-practice/monograph/245.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/245.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.html -
7/27/2019 cijanoza novorodj uvod
20/21
when the ductus arteriosus closes atrium and dilated right ventri
Truncus arteriosus (TA)
see our comprehensive coverage of Overview of congenital heart disease
History Exam 1st test
mild to moderate cyanosis with
or without respiratory distress
may present in first week of
life; velocardiofacial syndrome
(22q deletion) is present in 30%
to 50% of TA cases
hyperdynamic precordium with loud single
S2; loud systolic and diastolic murmur with
wide pulse pressure and bounding pulses;
respiratory distress depends on the degree of
pulmonary congestion and worsens when
pulmonary vascular resistance drops after the
first 2 to 3 days; CHF may develop as a result
CXR: cardiomegaly and increased
pulmonary blood flow
echocardiogram: definitive diagn
may reveal abnormal morphology
functional derangement of the trun
Pulmonary haemorrhage
History Exam 1st test
most cases occurring in full-term infants will
give a hx of severe asphyxia or coagulopathy;
in preterm infants who have
haemodynamically significant patent ductus
arteriosus with left-to-right shunt, pulmonary
haemorrhage may develop; most of these
infants are ventilated post-surfactant
instillation
sudden appearance of cyanosis or
increased requirement of oxygen when
infant is being ventilated is the earliest
sign; bloody secretions from the trachea
may be suctioned from the endotracheal
tube; rales or diminished air entry found
on the affected side, but condition is
generally bilateral
CXR: fluffy infiltrates
bilaterally
ABG: severe hypoxia a
hypercarbia
FBC: normal but in som
cases thrombocytopen
may be present
DIC screen: prolonged
and PTT; low fibrinogen
and low platelet count w
increase in D-dimers
Pulmonary hypoplasia
History Exam 1st test Oth
hx of oligohydramnios and respiratory
distress soon after birth are present;
higher incidence of pneumothorax in
these cases
tachypnoea, cyanosis, and fair to poor
aeration of lungs present; S2 is loud
with associated pulmonary
hypertension
CXR: decreased lung
volume
ABG: hypoxia and
hypercarbia
Pulmonary lymphangiectasia
History Exam 1st test Other tests
respiratory distress
and cyanosis in the
newborn
tachypnoea and cyanosis in a full-term
newborn; air entry will not be diminished;
occasionally rales may be heard
CXR: diffuse reticular
opacities bilaterally
CT
inv
lun
lym
inte
Tracheo-oesophageal fistula (TOF)/oesophageal atresia (OA)
http://bestpractice.bmj.com/best-practice/monograph/1056.htmlhttp://bestpractice.bmj.com/best-practice/monograph/1056.html -
7/27/2019 cijanoza novorodj uvod
21/21
see our comprehensive coverage of Tracheo-oesophageal fistula
History Exam 1st test
hx of polyhydramnios and large amount
of pharyngeal secretions present after
birth; inability to pass an orogastric or
nasogastric tube is diagnostic; OA
associated with VACTERL (vertebral
anomalies, imperforate anus, cardiac
lesions, TOF, renal and limb anomalies)
respiratory distress with frothy secretions
at the mouth following birth is usually
present; conducted upper airway sounds
will be heard on auscultation; most forms
of TOF have OA with/without tracheo-
oesophageal fistula; respiratory distress is
due to secretions in the upper airway and
aspiration
CXR: diagnostic with an
indwelling orogastric tube
curled in the upper
oesophageal pouchMore
Congenital lobar emphysema
History Exam 1st test
generally asymptomatic at birth;
respiratory distress becomes
progressively worse over the next
few days
tachypnoea and cyanosis are present;
diminished air entry over the affected
side (commonly over left upper chest)
CXR: hyperinflation of the affected lob
present; in severe cases, there may be
herniation to the opposite side
ABG: hypoxia and hypercarbia
Pleural effusion
see our comprehensive coverage of Pleural effusion
History Exam 1st test
most pleural effusions present at birth are seen
in infants with hydrops fetalis and are
diagnosed by antenatal ultrasound; in severe
cases it will be difficult to ventilate unless
needle aspiration or chest tube insertion occursto remove the fluid; minor degrees of pleural
effusion are seen in pneumonitis, meconium
aspiration syndrome, and persistent pulmonary
HTN of newborn
in severe cases, diminished air
entry will be present on the
affected side; in bilateral
effusions, as seen in hydrops
fetalis, it will be difficult toventilate the lungs unless the
pleural fluid is evacuated
CXR: opacity on the lateral borde
with blunting of the cardiophrenic
unilateral effusions; in severe cas
mediastinal shift to contralateral s
effusion is unilateral; in severe bi
effusions, the whole chest is opaq
indistinguishable cardiac borderM
Arteriovenous malformation
History Exam 1st test Other tests
CHF, large head due to
hydrocephalus, seizures
bruit may be heard over the
cranium on auscultation
CXR: may show
cardiomegaly
angiogram
angiogram
1
http://bestpractice.bmj.com/best-practice/monograph/760.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/287.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/760.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.htmlhttp://bestpractice.bmj.com/best-practice/monograph/287.htmlhttp://bestpractice.bmj.com/best-practice/monograph/768/diagnosis/differential-diagnosis.html