The Blue Baby Network Stabilisation of the Term Infant Study Day 15 th March 2017 Joanna Behrsin
Session Structure Definitions and assessment of cyanosis Causes of blue baby Structured approach to assessing and managing blue baby
Cyanosis Cyanosis is visible when there is an excess of deoxygenated haemoglobin in the blood It may be less visible if the infant is anaemic Central - mucous membranes inside mouth Peripheral extremities Result of inadequate functioning of either the heart or lungs Primary pathology Secondary to other problems such as sepsis
Early Effective Intervention Recognising the sick infant and need for intervention Timely and effective stabilisation and referral for definitive care Slowing the progression of any illness Avoiding iatrogenic harm
Delay in intervention may lead to further clinical compromise and increased morbidity or mortality Death
CNS diseases Intra / periventricular haemorrhage Meningitis Seizures causing hypoventilation Other: Polycythaemia Sepsis Shock Methaemoglobinaemia Respiratory: Respiratory distress syndrome (rare in term babies) Pneumonia Meconium Aspiration Syndrome Persistent Pulmonary Hypertension of the Newborn Pneumothorax Congenital Anomalies (e.g. Congenital diaphragmatic hernia or cystic adenomatous malformation) Potential causes of cyanosis Cardiac: Cyanotic heart disease Transposition of the great arteries Total anomalous pulmonary venous drainage Tricuspid atresia Tetralogy of fallot Truncus arteriosus Critical pulmonary stenosis Critical obstruction to the systemic circulation will cause pallor / greyness aortic stenosis hypoplastic left heart
Assessing the infant with Cyanosis Structured approach is key ABCD Identify life-threatening features requiring urgent intervention Make a diagnosis & definitive management plan
Airway and Breathing Assess the infants airway and breathing Signs of airway obstruction Effort of breathing Apnoeas Equal air entry Added sounds Administer oxygen ECG, saturation monitoring (pre & post ductal) Check for pneumothorax and if present clinically, consider the need for cold light examination, needle aspiration or chest drain insertion Blood gas
Management of pneumothorax Needle Thoracocentesis -Butterfly needle attached to three way tap and syringe -2 nd intercostal space -Mid clavicular line -Aspirate air until BP / saturations improve -Follow up with a definitive chest drain Chest Drain - See workshops
Recognition of respiratory distress in term babies Decide whether infant requires respiratory support and initiate Order a CXR early (don t wait until 4 hours of age) If PPHN is suspected special measures
Respiratory support in term babies Options: Supplemental oxygen (headbox, incubator, nasal cannulae NCPAP HHFNC oxygen Mechanical Ventilation Maintaining oxygenation, avoiding excessive work of breathing, strategies to avoid lung trauma Term babies are at risk of pneumothorax and PPHN
Circulation Is there a murmur? Are the pulses normal? Cyanosis associated with a murmur or abnormal pulses make a cardiac leasion more likely If pulses are diminished consider: Critically obstructed systemic circulation Severe sepsis Consider a formal cardiac assessment including cardiac ECHO, liaise with cardiology team Measure pre and post ductal saturations and perform a blood gas if not already done Consider starting prostaglandin
Prostaglandin There should be a low threshold for starting prostaglandin in an unwell infant. Consider starting if: There is a murmur and cyanosis If the infant has reduced pulses If the patient is in extremis
Disability Are there signs of neurological compromise? What is the blood sugar? Is the baby having seizures?
Factors to help discriminate between respiratory and cardiac pathology History Respiratory Prolonged ruptured membranes, maternal sepsis, meconium, difficult delivery Cardiac Antenatal scans, family history of congenital heart disease Examination Respiratory distress Cardiac signs (murmur, parasternal heaves) Relative lack of respiratory distress and signs on auscultation CXR Evidence of lung pathology: RDS, meconium aspiration, infection, CDH ECG Normal Abnormal Blood gases and response to increased FiO 2 High PaCO 2 Increasing oxygen improves saturations Decreased pulmonary vascular markings, cardiomegaly, abnormal cardiac shadow Low or normal PaCO 2 Increasing oxygen has little effect on saturations
Persistent Pulmonary Hypotension Failure of adaptation to ex-utero life Persistence of high pulmonary vascular resistance after birth leading to cyanosis from right to left shunting at ductal and atrial levels The heart is structurally normal
Causes Mechanisms Pulmonary vasoconstriction secondary to factors secondary to hypoxia and acidosis Decreased size of pulmonary vascular bed associated with pulmonary hypoplasia Chronic hypertrophy of pulmonary vascular smooth muscle inutero PPHN is associated with a number of conditions Meconium Aspiration Sepsis (Especially GBS) Birth asphyxia Respiratory distress syndrome Pulmonary hypoplasia Congenital diaphragmatic hernia
Clinical presentation Respiratory distress Difficulty in achieving oxygenation may be out of proportion to relative ease of maintaining normal PaCO2 Difference of >5% between pre and post ductal saturation measurements (pre=higher) Significantly higher pre ductal PaO2 (right radial artery) compared with post ductal PaO2 Chest X-ray may demonstrate a primary respiratory pathology
Goals of management Strategies aimed to reduce pulmonary vascular resistance: Optimise oxygenation FiO2 Ventilatory strategies Sedation and muscle relaxation Avoid and correct acidosis Systemic BP > pulmonary BP Inotropes Aim for a BP 5-10 greater than estimated pulmonary pressures Normal glucose, calcium and magnesium Correct polycythaemia Thermoregulation (refractory PPHN indication to rewarm in HIE) Consider inhaled nitric oxide
ECMO Criteria Neonates requiring ECMO treatment need to meet the following criteria: Inclusion Criteria: Exclusion Criteria: Oxygenation Index * (OI) >40 Gestational age >35 weeks Weight >2kgs Reversible lung disease Significant coagulopathy or uncontrollable bleeding Major (>grade 1) intracranial haemorrhage Irreversible lung injury Major congenital / chromosomal anomalies or severe encephalopathy Major cardiac malformation Mechanical ventilation 10-12 days Cardiac arrest other than immediately at birth * OI = (Mean Airway Pressure [cmh20] x FiO2 [in percentage]) divided by (post ductal PaO2 [mmhg])
Duct dependent congenital heart Antenatal diagnosis Clinical signs disease Common presenting features Difficulty feeding secondary to increased breathlessness Cyanosis unresponsive to supplemental oxygen Acute cardiorespiratory collapse with shock
Diagnostic algorithm Blue Femoral pulses Y Murmur Y Fallot AVSD Tricuspid atresia Pulmonary atresia Critical Pulmonary stenosis N Transposition of the great arteries PPHN Total anomalous pulmonary venous circulation Pink Femoral pulses N Co-arctation of the aorta Hypoplastic left heart Critical aortic stenosis Y VSD / ASD/ Aortic stenosis / pulmonary stenosis
Management Airway and Breathing Intubate if indicated Pre-post ductal saturations Administer supplemental oxygen target saturations 75-85% Indications for intubation Apnoea Shock Respiratory failure
Management Circulation IV access consider UVC Treat hypotension (volume & dopamine) Prostin to maintain ductal patency 5ng/kg/min can be increased 4 limb BP ECHO ECG Side effects of prostin: Hypotension hypoglycaemia Apnoea (more common >10ng/kg/min) Fever Higher doses may be needed to open a closed duct following cardiology advice
Summary Structured approach is key - the initial goal should be to Identify life-threatening features requiring urgent intervention followed by diagnosis and definitive management Low threshold for mechanical ventilation rather than ncpap or NCHHFO 2 if respiratory support needed Term infants with respiratory disorders are at risk of PPHN which has a specific management strategy Think could the cause of cyanosis be cardiac