1. Approach to the Cyanotic Infant Billie Parsley

2. Goals Recognition of a cyanotic infant Recognition of a cyanotic infant What to do in any setting from ICU to rural community pediatric practice What to do in any setting from ICU to rural community pediatric practice What questions to ask What questions to ask What to look for What to look for What imaging to order What imaging to order What test to order What test to order

3. Case 39 week male born at home in uncomplicated vaginal delivery 39 week male born at home in uncomplicated vaginal delivery Presented at ACH ED 24 hours old due to temp of 35.1 rectal Presented at ACH ED 24 hours old due to temp of 35.1 rectal Sats in 50s, HR 100, RR 80, BP 76/59 Sats in 50s, HR 100, RR 80, BP 76/59 Cyanotic on general appearance Cyanotic on general appearance

4. What is cyanosis? Cyanosis is a bluish discoloration of the tissue that results when the absolute level of reduced hemoglobin (when not combined with oxygen) in the capillary bed exceeds 3 g/dL. The appearance of cyanosis depends upon the total amount of reduced hemoglobin rather than the ratio of reduced to oxygenated hemoglobin.

5. Cyanosis Two mechanisms result in cyanosis: Two mechanisms result in cyanosis: Peripheral cyanosis Peripheral cyanosis Normal systemic arterial oxygen saturation and increased tissue oxygen extraction that leads to a widened systemic arteriovenous oxygen difference of >60% (normal 40%) resulting in an increased concentration of reduced hemoglobin on the venous side of the capillary bed. Normal systemic arterial oxygen saturation and increased tissue oxygen extraction that leads to a widened systemic arteriovenous oxygen difference of >60% (normal 40%) resulting in an increased concentration of reduced hemoglobin on the venous side of the capillary bed. Central cyanosis caused by systemic arterial oxygen desaturation Central cyanosis caused by systemic arterial oxygen desaturation Don’t confuse central cyanosis with acrocyanosis!!!! Don’t confuse central cyanosis with acrocyanosis!!!! Acrocyanosis-common physical finding in newborns as a result of peripheral vasoconstriction Acrocyanosis-common physical finding in newborns as a result of peripheral vasoconstriction

6. Cyanosis Best indicator is the tongue-due to free of pigmentation and has rich vascular supply. Best indicator is the tongue-due to free of pigmentation and has rich vascular supply. Also seen in lips, nails, earlobes, mucous membranes Also seen in lips, nails, earlobes, mucous membranes

7. Cyanosis Depends on multiple factors Depends on multiple factors Hemoglobin Hemoglobin Fetal hemoglobin Fetal hemoglobin Skin Pigment Skin Pigment Factors affecting the hemoglobin dissociation curve Factors affecting the hemoglobin dissociation curve

10. Cause Alveolar hypoventilation Alveolar hypoventilation Nml response is hypercarbia but can also cause hypoxemia Nml response is hypercarbia but can also cause hypoxemia Ventilation Perfusion mismatch Ventilation Perfusion mismatch Nml is for areas with decreased ventilation to have decreased blood flow, but alterations in this cause hypoxemia Nml is for areas with decreased ventilation to have decreased blood flow, but alterations in this cause hypoxemia Diffusion Impairment Diffusion Impairment Oxygen molecules must diffuse from alveoli to pulmonary capillary to oxygenate hemoglobin Oxygen molecules must diffuse from alveoli to pulmonary capillary to oxygenate hemoglobin Right to Left Shunt Right to Left Shunt Systemic venous blood bypasses ventilated alveoli and returns to the left heart without being oxygenated Systemic venous blood bypasses ventilated alveoli and returns to the left heart without being oxygenated Disturbed Hemoglobin Disturbed Hemoglobin Decreased oxygen affinity Decreased oxygen affinity

13. History Pregnancy Pregnancy Maternal Diabetes-associated with cyanotic heart dz. Maternal Diabetes-associated with cyanotic heart dz. Polyhydramnios-associated with fetal airway, esophageal, neurologic abnormalities Polyhydramnios-associated with fetal airway, esophageal, neurologic abnormalities Oligohydramnios-associated with renal defects and pulmonary hypoplasia Oligohydramnios-associated with renal defects and pulmonary hypoplasia Drug Use during pregnancy Drug Use during pregnancy Maternal Serology Maternal Serology US results US results Previous complications with pregnancy (stillborn/multiple spontaneous abortions) Previous complications with pregnancy (stillborn/multiple spontaneous abortions) Medication Use during pregnancy Medication Use during pregnancy

14. History Perinatal/Peripartum Perinatal/Peripartum Gestational Age Gestational Age Prolonged/Premature Rupture of Membranes Prolonged/Premature Rupture of Membranes What/How treatment given for illness during pregnancy (GBS) What/How treatment given for illness during pregnancy (GBS) Maternal fever Maternal fever Birth Trauma Birth Trauma Neonatal polycythemia Neonatal polycythemia Hypoglycemia Hypoglycemia Meconium Staining-associated with Meconium Aspiration syndrome and P-HTN Meconium Staining-associated with Meconium Aspiration syndrome and P-HTN Hospital course post delivery Hospital course post delivery

15. History HPI HPI Fever Fever Exposure Exposure Trauma Trauma Well water Well water Seizure Seizure Poor Feeding Poor Feeding Decreased urine output Decreased urine output Medications Medications Different Temperatures between extremities Different Temperatures between extremities Cough/Congestion Cough/Congestion Rashes Changes in skin Rashes Changes in skin “birth marks” “birth marks” Abnormal breathing-pauses in breathing Abnormal breathing-pauses in breathing Weight loss or gain Weight loss or gain Edema Edema

16. History Diet Diet What (could mom be taking in meds that get into breastmilk) What (could mom be taking in meds that get into breastmilk) How long does it take? How long does it take? Perspiration with feeds Perspiration with feeds Coughing/Gagging/Gurgling with feeds Coughing/Gagging/Gurgling with feeds Medications Medications Family History Family History Hemoglobinopathies Hemoglobinopathies Congenital Cardiac Abnormalities Congenital Cardiac Abnormalities Cystic Fibrosis Cystic Fibrosis Neuromusclar Disorders Neuromusclar Disorders

17. Physical Exam *The presence and degree of respiratory distress are very important factors in differentiating your 2 main causes of Cyanosis-Cardiac vs. Respiratory *The presence and degree of respiratory distress are very important factors in differentiating your 2 main causes of Cyanosis-Cardiac vs. Respiratory General exam: Comfort, WOB, LOC, Color, General exam: Comfort, WOB, LOC, Color, Head: Fontanel, Shape of Head, eccymosis, lacerations, Pupils, nose shape and location, shape of chin and size, mouth size, moisture, cleft palate, Head: Fontanel, Shape of Head, eccymosis, lacerations, Pupils, nose shape and location, shape of chin and size, mouth size, moisture, cleft palate, Neck: size of vessels, abnormal masses Neck: size of vessels, abnormal masses Abdomen: HSM, ascities, Abdomen: HSM, ascities, Skin: Rashes, birthmarks, mottled, slate gray color associated with methemolglobinemia Skin: Rashes, birthmarks, mottled, slate gray color associated with methemolglobinemia Limb abnormalities Limb abnormalities

18. Physical Exam Respiratory Exam Respiratory Exam Respiratory Rate (newborn nml 40-60) Respiratory Rate (newborn nml 40-60) Low RR-alveolar hypoventilation Low RR-alveolar hypoventilation Tachypnea-Cardiac or Pulmonary cause Tachypnea-Cardiac or Pulmonary cause Regularity of RR Regularity of RR apnea events apnea events Grunting (prevents end expiratory alveolar collapse) Grunting (prevents end expiratory alveolar collapse) Nasal flare (reduces nasal resistance) Nasal flare (reduces nasal resistance) Stridor Stridor Tracheal Deviation Tracheal Deviation Chest wall movement Chest wall movement Retractions Retractions Accessory muscle use Accessory muscle use Auscultation Auscultation

19. Physical Exam Cardiac Exam-not as severe respiratory distress Cardiac Exam-not as severe respiratory distress Heart Rate Heart Rate Pulses Pulses Perfusion Perfusion Auscultation Auscultation Four Extremity Blood Pressure Four Extremity Blood Pressure Pre/Post Ductal oxygen Saturation Pre/Post Ductal oxygen Saturation Point of Maximal Impulse Point of Maximal Impulse

20. Labs ABG-for arterial oxygenation ABG-for arterial oxygenation Painful and can cause increasing agitation Painful and can cause increasing agitation Methemoglobinemia oxygen saturation will be low but measured PO2 will be normal Methemoglobinemia oxygen saturation will be low but measured PO2 will be normal Elevated arterial PCO2 suggest pulmonary cause Elevated arterial PCO2 suggest pulmonary cause Metabolic acidosis indicates poor perfusion-possible due to poor cardiac output or oxygen delivery or shock. Metabolic acidosis indicates poor perfusion-possible due to poor cardiac output or oxygen delivery or shock. CBC CBC Can show high hgb/hct in polycythemia Can show high hgb/hct in polycythemia I/T ratio I/T ratio Blood glucose Blood glucose Blood Culture Blood Culture

21. Imaging Trauma Trauma Airway Airway Situs of heart, stomach, liver-any abnormalities in location point towards cardiac disease Situs of heart, stomach, liver-any abnormalities in location point towards cardiac disease Heart size and shape Heart size and shape Lung fields Lung fields Vascular Markings Vascular Markings Decreased vascular markings can be seen in cyanotic cardiac lesions and pulmonary hypertension Decreased vascular markings can be seen in cyanotic cardiac lesions and pulmonary hypertension

27. Other Test Hyperoxia testing—Right radial artery PaO2 (preductal) measured on RA and after 10 minutes on 100%FiO2 Hyperoxia testing—Right radial artery PaO2 (preductal) measured on RA and after 10 minutes on 100%FiO2 if right radial arterial PaO2 on 100% FiO2 is less than 150mmHg, severe congenital heart disease is likely. The infant is presumed to have ductal dependant congenital heart disease and the low PaO2 is attributed to the obligatory mixing of oxygenated with deoxygenated blood within the circulatory system. if right radial arterial PaO2 on 100% FiO2 is less than 150mmHg, severe congenital heart disease is likely. The infant is presumed to have ductal dependant congenital heart disease and the low PaO2 is attributed to the obligatory mixing of oxygenated with deoxygenated blood within the circulatory system.

29. Other Test Echo Echo Transposition Transposition Tetralogy of Fallot Tetralogy of Fallot Truncus arteriosus Truncus arteriosus Total anomalous pulmonary venous Return Total anomalous pulmonary venous Return Tricuspid Tricuspid “Tons” of others “Tons” of others EKG EKG

32. Managment ABCs ABCs Access-venous and arterial if possible Access-venous and arterial if possible Ventilation as needed Ventilation as needed Cautious fluid resuscitation Cautious fluid resuscitation Antibiotics Antibiotics Prostaglandin Infusion Prostaglandin Infusion

33. Prostaglandin Goal is to prevent closure of the PDA if this is where mixing is occurring. Goal is to prevent closure of the PDA if this is where mixing is occurring. Improvement normally seen within 1-2 hours Improvement normally seen within 1-2 hours Side effects: APNEA and peripheral vasodilatation with hypotension Side effects: APNEA and peripheral vasodilatation with hypotension

34. Case Review Mom GBS + treated with oral clindamycin Mom GBS + treated with oral clindamycin Baby with severe Pulmonary HTN with near systemic pressures Baby with severe Pulmonary HTN with near systemic pressures By 48 hours of life on ECMO on way to Edmonton By 48 hours of life on ECMO on way to Edmonton