Karen Corlett, RN, MSN, CPNP-AC/PC Pediatric Nurse Practitioner Congenital Heart Surgery Unit Pediatric Cardiac Intensivists of North Texas Medical

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1 Karen Corlett, RN, MSN, CPNP-AC/PC Pediatric Nurse Practitioner Congenital Heart Surgery Unit Pediatric Cardiac Intensivists of North Texas Medical City Children s Hospital, Dallas

2 Hypoxia Shortage of oxygen for the tissues (body) Hypoxemia Shortage of oxygen in the blood Desaturation Decreased from normal vs. decreased from patient s baseline

3 Visible cyanosis > 3 gm% (3gm per 100mL) desaturated hemoglobin More visible in polycythemic patients Hgb 25gm; 3% desaturated at SaO2 88% Hgb 16gm; 3% desaturated at SaO2 85% Hgb 8 gm; 3% desaturated at SaO2 63% Cyanosis blanches with pressure Measured cyanosis Pulse oximetry Saturation on arterial blood gas

4 Central cyanosis Includes mucous membranes and tongue Acrocyanosis (aka peripheral) Often normal in the newly born Don t ignore Differential cyanosis Pink upper, blue lower Preductal vs postductal saturations Right to left shunt via PDA reverse differential cyanosis Classic for Transposition of the Great Arteries (TGA)

5 Age in minutes PaO2 SaO % % % %

6 Need for immediate support of Circulation Bradycardia, poor perfusion, hypotension Airway Gasping, choking, stridor, no air movement Breathing Apnea, bradypnea, poor respiratory effort

7 Maternal History Drug use/abuse, diabetes, prenatal infections Obstetrical History Resuscitation required, Apgar scores Meconium Gestational age Hydrops Fever or prolonged membrane rupture Prenatal screening results

8 Presenting symptoms Duration of symptoms Improving or deteriorating Exacerbating/relieving events Feeding Treatments and results Coexistent conditions Acidosis, hypoglycemia, anemia, sepsis

9 CAB s Structural abnormalities, syndromic features Other pearls Scaphoid abdomen with congenital diaphragmatic hernia (CDH) Abdominal distention Hypotonia: sepsis, metabolic disorders, CNS disturbances Hypertonia with narcotic withdrawal On to the specifics

10 Circulation Airway Breathing Rare causes Methemoglobinemia Metabolic disorders

11 History, physical exam Narrows differential diagnosis Hyperoxia Test Right radial arterial blood gas on room air, and again after 15 min in 100% oxygen Should see significant rise in PaO2 and SaO2 if parenchymal lung disease Cyanotic CHD see minimal to no change Often not helpful in PPHN

12 Congenital malformations Pierre Robin syndrome Tumors or masses Tracheoesophageal fistula (TEF)/esophageal atresia Choanal atresia Laryngotracheomalacia Subglottic stenosis, vocal cord paralysis

13 Physical Exam findings Suprasternal retractions, stridor, gasping, difficulty handling secretions Congenital anomalies Atretic nasal passages, micrognathia, TEF, esophageal atresia, macroglossia History of intubation or resuscitation Subglottic stenosis, vocal cord paresis Cyanosis and hypercarbia

14 Initial Stabilization Airway adjuncts Nasopharyngeal airways Oral airways LMA s Endotracheal tubes Positive pressure ventilation Invasive or noninvasive routes

15 Parenchymal lung disease Hyaline membrane disease Aspiration Pulmonary hemmorhage Pulmonary edema Pulmonary hypoplasia Pulmonary lymphangiectasia Nonparenchymal lung disease TEF, CDH Congenital cystic adenomatoid malformation (CCAM) Pneumothorax, pneumomediastinum Pleural effusion Laryngeal web Lobar emphysema

16 Physical Exam Findings Respiratory distress Tachypnea, substernal and subcostal retractions, grunting, flaring, rales, rhonchi, crackles Apnea Unequal chest rise, differential breath sounds Abnormal chest size or configuration Radiologic findings Improvement with supplemental oxygen Newborns may have murmur

17 Initial stabilization Supplemental oxygen NOT anymore in DR!! Positive pressure ventilation Invasive or non invasive Relief of restrictive physiology Abdominal decompression Decompression of tension pneumothorax or pleural effusion

18 Persistent Pulmonary Hypertension (PPHN) Congenital Heart Disease (CHD) Low Cardiac Output

19 Restricted pulmonary flow Right to left intracardiac shunt

20 Physical exam findings Central cyanosis +/- respiratory distress May have murmur Chest radiograph Cardiomegaly on CXR Not true in total anomalous pulmonary venous return Dark lung fields in restricted pulmonary blood flow +/- pulmonary edema in R-->L intracardiac shunt Cardiac output may be compromised Failure to thrive in the not newly born

21 Initial stabilization Much easier decisions if ECHO available Maintain cardiac output Prostaglandin E 1 infusion Ductal dependent pulmonary blood flow Tetralogy of Fallot Pulmonary Atresia Ductal dependent systemic blood flow Hypoplastic Left Heart Syndrome (HLHS) Interrupted Aortic Arch, critical coarctation of the Aorta

22 Does Oxygen help or hurt?? Know the physiology Physical Exam!! Follow cardiac output Pulses/perfusion Urine output Lactate Cerebral NIRS

23 Restricted pulmonary flow Right to left intracardiac shunt

24 Elevated pulmonary artery pressures High RV pressures, decreased flow to lungs Minimal blood flow available for LV to eject LOW cardiac output Desaturation Tachycardia then Bradycardia Arrest

25 VSD and PA hypertension Persistent pulmonary hypertension (PPHN)

26 Physical Exam Findings Central cyanosis PPHN may have differential cyanosis +/- respiratory distress Murmur present Loud S2 Good cardiac output

27 Initial stabilization Control as much as you can Sedation Maneuvers to decrease PAH High FiO2 ino Alkalinize without compromising cerebral perfusion Normal to high ph Low normal paco2 Correct any identifiable causes & times of risk

28 Impacts mixed venous oxygen saturations NIRS as surrogate of mixed venous saturation

29 Physical Exam Findings Increased heart rate Poor pulses and perfusion Consider end organ function +/- decrease in systemic blood pressure Elevated core temperature Cool extremities

30 Initial Stabilization Optimize cardiac output Heart rate and rhythm Sedation and pain relief for tachyarrhythmias Preload Volume resuscitation or repletion Contractility Pressors Relief of external compression (tamponade states)

31 Initial stabilization cont d Optimize cardiac output Optimize afterload Poor function often improved with decreased afterload Profound vasodilation needs vasoconstriction Warm shock, neurogenic shock Decrease metabolic demands Remove work of breathing Sedation and pain relief Avoid hyperthermia, avoid hypothermia with shivering

32 Targeted history General survey CAB s, obvious problems Physical exam Respiratory distress? Radiologic exams Hyperoxia test Differential diagnosis Stabilization Will oxygen hurt them? Definitive diagnosis and care? Transport required

33 Aly, H. Respiratory disorders in the newborn: identification and diagnosis. Pediatrics in Review : McConnell ME, Elixson EM. The neonate with suspected congenital heart disease. Critical Care Nursing Quarterly : Oliver Jr TK, Demia JA, Bates GD. Serial blood-gas tensions and acid base balance during first hour of life in human infants. Acta Paediatrica : Sasidharan, P. An approach to diagnosis and management of cyanosis and tachypnea in term infants. Pediatric Clinics of North America :

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