Baby is blue
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1) A newborn with cyanosis of the extremities alone is initially assessed for common causes like hypothermia, but peripheral cyanosis can also indicate serious conditions like sepsis and should not be ignored until ruled out. 2) Differential or harlequin cyanosis, where one half of the body is cyanotic while the other is pink, can indicate a PDA or vasomotor instability. 3) In an infant presenting with cyanosis, lethargy and cold extremities on day 4 of life, the physician would perform a full examination, order diagnostic tests like a chest x-ray and echocardiogram, and consider treatments for potential cardiac or pulmonary causes while closely monitoring the
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Baby is blue
- 1. Baby is blue .. What to do ? Approach to a cyanotic newborn Dr Bharathi
- 2. Case Scenario 1 • B/o X • Term / male /AGA born to primi mother by vaginal delivery . • Baby cried immediately after birth and is otherwise well . • There is cyanosis of peripheries alone…. Acrocyanosis
- 3. Peripheral cyanosis • Physiological • Pathological Shock Polycythemia Hypothermia Hypoglycemia
- 4. Although peripheral cyanosis is seen in conditions in which the infant is exposed to a cold environment, it could also be the presenting sign of serious conditions such as • sepsis, • hypoglycemia, • hypoplastic left-heart syndrome Peripheral cyanosis should not be ignored unless other conditions have been ruled out.
- 5. Case scenario 2 • A concerned mother brings her newborn to you “ One side of my baby is blue and the other side is red . What is this ?”
- 6. Harlequin skin change • One quadrant or one half of the body may become cyanotic or pale while the rest of the body remains pink. • Hands and feet remain warm. • Exact reason not known • Thought to be - vasomotor instability
- 7. What would you call it if the upper half is pink and the lower half is blue ? • Differential cyanosis • Seen in PDA with R – L shunting
- 8. Case scenario 3 • Term / AGA baby born to primi mother by SVD . Cried well at birth . Was well for 1st 3 days. • On day 4 child develops lethargy , poor feeding , cold extremities and is blue over face , tongue , hands and feet . • How will you approach ?
- 9. Cyanosis • Cyanosis (from the Greek word meaning ‘‘dark blue’’) is a blue to dusky hue in the newborn • Colour of the reduced hemoglobin gives rise to the colour seen in cyanosis. • Lees reported that cyanosis would be visible if the deoxygenated hemoglobin content is greater than 3 g% (3 g per 100 mL). Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatr. Clin. North Am. 2004 Aug;51(4):999–1021, ix.
- 10. Cyanosis is evident in polycythemic babies at higher oxygen saturation Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatr. Clin. North Am. 2004 Aug;51(4):999–1021, ix.
- 11. Why cyanosis is deleterious in newborn ?
- 12. Physiology of oxygen transport • Variables for gas exchange at the alveolar level • Concentration of oxygen in the gas entering the alveolus • Concentration of oxygen in the mixed venous blood entering the capillaries • Quantities of the gas flow and blood flow
- 13. Causes of cyanosis - pathophysiology • Hypoventilation • Significant right-to-left intracardiac or intrapulmonary shunting • Ventilation perfusion unevenness • Inadequate transport of oxygen by the hemoglobin. Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatr. Clin. North Am. 2004 Aug;51(4):999–1021, ix.
- 14. Aetiology of cyanosis PULMONARY CAUSES PPHN CARDIAC DEFECTS MISCELLANEOUS CNS, Others Neonate with Cyanosis
- 15. Aetiology of cyanosis Parenchymal • Transient tachypnea of newborn (TTN) • Hyaline membrane disease (HMD) • Aspiration—meconium, blood, mucus, or milk • Pneumonia • Pulmonary hemorrhage • Pulmonary edema • Pulmonary hypoplasia • Pulmonary lymphangiectasia Nonparenchymal • Tracheo esophageal fistula (TEF) • Choanal atresia • Laryngeal web • congenital diaphragmatic hernia (CDH) • Congenital cystic adenomatoid malformation (CCAM) • Pulmonary sequestration • Pneumothorax, pneumomediastinum • Pleural effusion • Lobar emphysema Pulmonary Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatr. Clin. North Am. 2004 Aug;51(4):999–1021, ix.
- 16. CYANOTIC CHD IN NEONATE CRITICAL NON-CRITICAL ↓PBF ↑PBF TOF TA-PS SV-PS DORV-PS TRUNCUS ARTERIOSUS SINGLE VENTRICLE TRI ATRESIA TAPVC(NO OBSTR) DDSC TGATAPVC OBST DDPC
- 17. Duct Dependent pulmonary circulation:Duct Dependent pulmonary circulation: Clinical ConditionsClinical Conditions ANATOMIC OBSTRUCTIONANATOMIC OBSTRUCTION • Pulmonary Atresia :Pulmonary Atresia : 1.1. Intact ventricular septum (hypoplastic right heart)Intact ventricular septum (hypoplastic right heart) 2.2. With VSD (TOF-PA)With VSD (TOF-PA) 3.3. With Tricuspid AtresiaWith Tricuspid Atresia 4.4. With Single VentricleWith Single Ventricle • Severe Tetralogy of FallotSevere Tetralogy of Fallot • Critical pulmonary stenosisCritical pulmonary stenosis FUNCTIONAL OBSTRUCTIONFUNCTIONAL OBSTRUCTION • Ebstein’s anomalyEbstein’s anomaly
- 18. Duct Dependent Systemic circulation:Duct Dependent Systemic circulation: Clinical ConditionsClinical Conditions • Critical aortic stenosis • Coarctation of aorta • Interrupted aortic arch • Hypoplastic left heart Manual of neonatalogy – John P Cloherty – 17 th edition
- 19. Aetiology of cyanosis Central nervous system (CNS) • Cerebral edema • Hemorrhage • Infection • Hypoventilation • Vocal cord paralysis or paresis Miscellaneous • Methemoglobinemia • Hemoglobin M • Metabolic acidosis • Sulfhemoglobinemia • Hypoglycemia • Sepsis • Polycythemia • Associated with feeding, gastroesophogeal reflux (GER)
- 20. Initial assessment of infants who have cyanosis •History •Physical examination •Chest radiograph •CBC with differential count •Blood glucose, calcium •Arterial blood gas (ABG)/hyperoxia test •Blood culture/sepsis screen •EKG and echocardiogram
- 21. History Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatr. Clin. North Am. 2004 Aug;51(4):999–1021, ix.
- 22. Physical examination • Examined in neutral thermal environment • Away from blue phototherapy lights • Asses capillary refill time- <3 sec • Temperature , pallor, polycythemia, femoral pulses, BP of all 4 limbs • Barrel shaped chest –post term-MAS • Bell shaped thorax – neurologic abnormalities • Scaphoid abdomen-CDH • Look for nasal flaring,grunting & retractions • Airway – choanal atresia. • Pulse oximetry. Sasidharan P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatr. Clin. North Am. 2004 Aug;51(4):999–1021, ix.
- 23. CARDIAC PULMONARY Early < 4 hours > 4 hours presentation Resp distress > cyanosis Cyanosis > Resp distress Type of cyanosis CentralCentral/diff complicated labor More likelyunlikely CLINICAL DIFFERNCES: CARDIAC VS PULMONARY ONSET
- 24. CCHD in Newborns:CCHD in Newborns: Clues based on presentationClues based on presentation Cyanosis No Resp Distress Cyanosis + Resp Distress Shock Differential cyanosis TGA DDPC TAPVC obstructed DDSC
- 25. CCHD in Newborns:CCHD in Newborns: Clues Based on S2 splitClues Based on S2 split S2 single fixed DDPC TGA DDSC TAPVC
- 26. CCHD in Newborns:CCHD in Newborns: Clues based on Onset of CyanosisClues based on Onset of Cyanosis 0-6 days TGA HLHS TOF COA VSD others 7- 13 days COA VSD HLHS TGA TOF Others 14-28 days VSD COA TOF TGA PDA Manual of neonatalogy – John P Cloherty – 17 th edition
- 27. Chest xray • Situs – locate stomach , liver, heart • Cardiac position • Chamber enlargement • Arch sidedness • Lung vasculature • Lung parenchyma • Bony cage and diaphragm
- 28. • Small heart- hypovolemia, adrenal insufficiency, pulmonary interstitial emphysema, congenital lobar emphysema. • Egg-on-end - transposition of the great vessels • Snowman sign – TAPVC • Boot-shaped heart - tetralogy of Fallot. • Severe cardiomegaly -Ebstein’s anomaly • Moderate cardiomegaly - Infants of diabetic mothers (hyperinsulinemia), cardiomyopathy (caused by infections,metabolic disorders or asphyxia)
- 30. 2 DAY OLD NEONATE WITH SAO2 OF 40% WITH SINGLE S2 AND SOFT 2/6 ESM TRANSPOSITION OF GREAT ARTERIES
- 31. CYANOTIC NEONATE WTH SIGNS OF RESPIRATORY DISTRESS AND CHF WITH SEVERE PAH OBSTRUCTED TAPVC
- 32. 3 day neonate with profound cyanosis , ECG shows slurred upslope of qrs NEONATAL EBSTEIN S
- 33. Hyperoxia test 100% O2 via hood ~15 min.. Take ABG – pO2 / Transcutaneous po2 PO2 < 100 mmHg CHD likely PO2 >250mmHg, CHD unlikely 100 to 250 ± < 70 mmHg CHD very likely Manual of neonatalogy – John P Cloherty – 17 th edition
- 34. ECG • Most conditions cause right axis deviationMost conditions cause right axis deviation with RVHwith RVH • Cyanotic infant with left axis deviation andCyanotic infant with left axis deviation and LVH:LVH: 1.Tricuspid atresia1.Tricuspid atresia 2 Pulmonary atresia with intact ventricular2 Pulmonary atresia with intact ventricular septum.septum. • ArrhythmiaArrhythmia
- 36. Management • Thermo-neutral environmentThermo-neutral environment • Airway , breathing , circulationAirway , breathing , circulation • Correct hypovolemia, metabolicCorrect hypovolemia, metabolic derangements, treat sepsis.derangements, treat sepsis. • Particular care of IV lines and infusions –Particular care of IV lines and infusions – avoid flushing of air bubbles into IV lines.avoid flushing of air bubbles into IV lines. • Feeding may be withheld in neonates withFeeding may be withheld in neonates with duct dependent states if prostaglandin isduct dependent states if prostaglandin is anticipated.anticipated.
- 37. PGE 1 • Neonate failing hyperoxia test • Shock within 1st 3 weeks of life • Start at 0.05 µg/kg/mt – 0.1mcg/kg/mt • Anticipate apnea • Can worsen TAPVC • Available as 500 μg vialAvailable as 500 μg vial Manual of neonatalogy – John P Cloherty – 17 th edition
- 38. Oxygen TherapyOxygen Therapy • Can be dangerous in certain statesCan be dangerous in certain states • Ductus dependent systemic lesionsDuctus dependent systemic lesions • Pulmonary blood flow can increase at thePulmonary blood flow can increase at the cost of systemic blood flow(DDSC)cost of systemic blood flow(DDSC) • Tailor the FiO2, aTailor the FiO2, a SaO2 of 80-85% maySaO2 of 80-85% may be adequate to balance systemic andbe adequate to balance systemic and pulmonary circulationspulmonary circulations • Cyanosis not associated with acidosisCyanosis not associated with acidosis need not be correctedneed not be corrected Manual of neonatalogy – John P Cloherty – 17 th edition
- 39. DiagnDiagn osisosis ClinicalClinical presentationpresentation CXRCXR ImmeImme diatediate TrtTrt DefinitiveDefinitive TrtTrt DDPCDDPC (Pulm(Pulm Atresia)Atresia) Cyanosis D2-7Cyanosis D2-7 No distressNo distress S2 single; Short ESMS2 single; Short ESM OligemicOligemic LungLung fieldsfields PGE1PGE1 BT shunt/BT shunt/ PDAPDA stentingstenting TGATGA Cyanosis D1Cyanosis D1 Min. DistressMin. Distress S2 single; Short ESMS2 single; Short ESM Egg onEgg on sideside PlethoraPlethora BAS +BAS + PGE1PGE1 ASOASO TAPVCTAPVC (obstr)(obstr) Cyanosis variableCyanosis variable Marked distressMarked distress Fixed split S2; noFixed split S2; no murmurmurmur GroundGround glassglass lunglung fieldsfields No PGE1No PGE1 surgerysurgery DDSCDDSC Shock withShock with diff.cyanosisdiff.cyanosis S2 single;S2 single; PlethoraPlethora CE +CE + PGE1PGE1 Repair/Repair/ NorwoodNorwood
- 40. Strategies to manage PPHN • Avoid hyperoxemia (spo2 90 – 98) • HFV • iNO • ECMO • Sedation • Hemodynamic support • Correct acidosis ,polycythemia • Drugs – sildenafil, mag sulph , adenosine,CCB, tolazoline, prostacycline,inhaled ethyl nitrite Manual of neonatalogy – John P Cloherty – 17 th edition
- 41. Met hemoglobinemia • Iron molecule in hemoglobin- normally in the ferrous state (Fe2. • The ferric (Fe3+) form combines with water producing methemoglobin (MetHb). • The predominant intracellular mechanism for the reduction of MetHb is cytochrome 5b. • MetHb may be increased in the red cell owing to exposure to toxic substances or to absence of reductive pathways Nelson textbook of pediatrics – 19 th edition
- 42. Treatment • Methylene blue given IV (1-2 mg/kg initially) is used to treat toxic methemoglobinemia. • An oral dose can be administered (100- 300 mg PO per day) as maintenance therapy. Nelson textbook of pediatrics – 19 th edition
- 43. Goldsmith ‘s assisted ventilation of neonate- 5th edition
- 44. Goldsmith ‘s assisted ventilation of neonate- 5th edition
- 45. • Thank You
Editor's Notes
- Capillary blood has an oxygen content that is between the venous and arterial blood oxygen contents, but in states of poor perfusion there will be large arteriovenous oxygen content difference. In such circumstances, it is possible to see Fig. 1. peripheral cyanosis, because of the higher amount of deoxyhemoglobin in the capillary blood, even though the arterial blood oxygen content may be normal [5,6]. Arterial blood oxygen content will be normal in peripheral cyanosis, whereas in central cyanosis there is decreased oxygen content in the arterial blood. Peripheral cyanosis is likely to be seen with exposure to cold, polycythemia, and hypoplastic left-heart syndrome (decreased peripheral perfusion)
- When the degree of oxygenation or percentage of saturation of hemoglobin with oxygen is plotted against the partial pressure of oxygen, it is referred to as oxyhemoglobin dissociation curve. The changing oxygen affinity of hemoglobin with oxygenation results in a sigmoid curve. The shape of its midportion is the midportion of the saturation (ie, 50% saturation of the hemoglobin) and its corresponding PO2 is called the P50. The adult hemoglobin has a P50 value of 27, which means that at 50% saturation the PO2 is 27 mm Hg. The P50 in thenewborn is 22; that is, at 50% oxygen saturation the PO2 is 22 mm Hg. Thus, if an infant is cyanotic, he or she may have a significantly lower PO2 value and requires immediate attention compared with an older child. Because PO2 is the driving force in oxygen exchange, a low PO2 will adversely affect the oxygen delivery to the tissues. Oxygen delivery is also dependent upon the amount of hemoglobin available to carry oxygen and the blood flow.
- In the normal lung, the average ventilation-to-perfusion ratio is in the range of 0.8 to 1.0. The concentration of oxygen in the gas entering the alveolus, the concentration of oxygen in the mixed venous blood entering the capillaries, and the respective quantities of the gas flow and blood flow are the main variables for gas exchange at the alveolar level [9]. Although diffusion abnormalities exist in adults, they are not commonly seen in the newborn. In the normal physiologic state, the pulmonary blood flow (QP) should be equal to systemic blood flow (QS). In other words, the volume of blood entering the lungs should be equal to the volume of blood leaving the left ventricle. This is given in the equation QP = QS. The volume of blood that participates in gas exchange, however, may not be equal to the volume of blood entering the pulmonary circulation. The volume of blood that participates in actual gas exchange is called effective P. Sasidharan / Pediatr Clin N Am 51 (2004) 999–1021 1003 pulmonary blood flow (QeP). In an ideal circumstance, the QP should be equal to QeP; the total volume of blood that enters the lungs participates in gas exchange. This does not take place normally, however. When there is a shunt between the systemic venous return to the pulmonary veins, or the left ventricle, it is called right-to-left shunt. In this case QP (QeP) is less than QS. This gives rise to decreased oxygen content in arterial blood (Fig. 2) [17]. Conversely, with a shunt from the left ventricle or aorta to the right ventricle or pulmonary artery (left-toright shunt), there is increase in pulmonary blood flow, and hence QP is greater than QS. In this condition, the infant will not develop cyanosis until the formation of pulmonary congestion and pulmonary edema. Intrapulmonary shunting takes place when nonventilated portions of the lungs are perfused, because the blood return from those areas does not take part in gas exchange. Intrapulmonary shunting or low ventilation to perfusion (V &lt; Q) is seen in pulmonary edema, atelectasis, or pneumonia. During the newborn period, the total percentage of shunting from right to left is approximately 2% to 5%, but this does not give rise to hypoxia.