Approach to the neonatal cyanosis

Approach to the neonatal cyanosis Topic presentation

By Ext. Sripada Kriangkhajorn

Faculty of Medicine, Srinakharinwirot University

Objective

• Definition, presentation, and abnormalities in cyanotic newborn

• Approach cause of neonatal cyanosis; include history, risk, and initial evaluation

• Initial management in neonatal cyanosis disease; pulmonary, cardiac cause

Cyanosis

• A physical sign causing bluish discoloration of the skin and mucous membranes

• Three factor causes cyanosis are

• Total amount of Hb in blood

• The degree of Hb saturation

• State of circulation.

Central cyanosis

• Increase in arterial deoxyhemoglobin, associated with decreased PaO2 and Hb oxygen saturation (SaO2)

• Present when deoxyhemoglobin in blood reaches 3-5 g/dL.

Peripheral cyanosis

• Discoloration of skin but sparing in mucous membrane, tongue. Usually normal in PaO2.

• Peripheral vascular instability, cold exposure are common cause in the cyanosis

Differential Cyanosis

• Asymmetrical cyanosis between upper and lower extremities, usually lower limbs more than the upper limbs.

• This finding suggested of Rt.-to-Lt. Shunt from PDA

Differential Cyanosis Cause1

-PPHN with PDA

-PDA with severe pulmonary hypertension (Eisenmenger Syndrome)

-Interrupted aortic arch

-Severe coarchtation of aorta with VSD and PDA

Differential Cyanosis

• Usually lower limbs more cyanosis than the upper limbs.

Reversed differential cyanosis

• Cyanosis that appear in upper limbs more than lower limbs.

• Found in complete TGA with severe pulmonary hypertension, D-TGA with VSD ,or Interrupted aortic arch or severe coarctation of aorta

http://journal.frontiersin.org/article/10.3389/fphar.2013.00070/full

APPROACH TO A CYANOTIC NEONATE

1. Identify kind of cyanosis

Cyanosis

Peripheral Cyanosis

Central Cyanosis

• Pink tongue, conjunctiva • Normal PaO2 • Cold peripheral Ext. • Cap. Refill > 2 sec.

• Discoloration all skin and mucous

• Decrease PaO2 ,SpO2 • Capillary refill <2 sec.

2. Identify possible cause

• Three common causes of central cyanosis are Cardiac disease,Respiratory disease, Central nervous system

• Another cause is hematologic cause such as methemoglobinemia

Central Cyanosis

CNS

Pulmonary disease

Cardiac disease

CNS

Causes and clinical finding of Central Cyanosis5

System Causes Clinical Findings

CNS depression

Perinatal asphyxia Hypoventilation Heavy maternal sedation Intrauterine fetal distress

Shallow irregular respiration Poor muscle tone Cyanosis resolved when given oxygen or stimulated the patient

Pulmonary System


System

Causes Clinical Findings

Pulmonary disease

Parenchymal lung diseases Pneumonia Pneumothorax or pleural effusion Congenital lung abnormalities Persistent pulmonary hypertention

Tachypnea, respiratory distress with chest retraction, or expiratory grunting Crackles,or decreased breath sound X-ray films may show some lung abnormalities Oxygen giving may improved cyanosis

Cardiac System


System Causes Clinical Findings

Cardiac disease

Cyanosis CHD with right to left shunt (5 ‘T’s)

Tachypnea, but not respiration effort Normal breath sound unless severe CHF A continuous murmur may present. X-ray may shows cardiomegaly, increased or decreased lung markings. Little or no improved in oxygen giving.

3. History & risks evaluation Risk assessment in initial evaluation5,6

History Possible risks

Maternal Diabetic Heart disease GBS and infectious screening PROM

CHD, sepsis, fetal asphyxia, pneumonia

Oligohydramnios Hypoplastic lung disease

Polyhydramnios Airway, esophageal, neurological disorder

Perinatal Difficult delivery Neurological cause; birth trauma, ICH, phrenic nerve paralysis

Postnatal Polycythemia Hypoglycemia

Hypoventilation

4. Physical Examination

• Complete examination but also pay focus on pulmonary, cardiac, and neurologic system

• Growth curve for SGA, LGA which are prone for polycythemia, and associated congenital anomaly

Cardiac Examination

• In cardiac auscultation, focus on S2, which will be loud and single (or narrowly split) in PPHN, TGA, PA

• Heart murmurs is often not helpful to detect serious lesions such as TGA

• Loud murmurs are frequently benign lesion such as a small VSD.

5. Special Tests

• Hyperoxia test

• Hyperoxia-hyperventilation Test

• Pre-/postductal PaO2 Test

• Echocardiography

Hyperoxia test

• Perform by given 100% oxygen for 5-10 minutes, then measure the before and after oxygen saturation

Changes after performed Hyperoxia Test1

PaO2 SpO2

Pulmonary disease Pneumonia RDS, Hyaline membrane disease etc,.

> 150-200 mmHg

Up to 99%

Rt.-to-Lt. Shunt Disease Congenital cyanosis heart disease PPHN Pulmonary AV Fistula

< 50-150 mmHg

< 80%

Hyperoxia-hyperventilation Test

• Given the 100% O2 through ET-tube or oxygen bag,

• Perform the patient hyperventilation, start at 100 times/min then keep monitor PaCO2 at 20-30 mmHg

Changes after performed Hyperoxia-Hyperventilation Test

Rt.-to-Lt. Shunt PaO2 SpO2

Congenital cyanosis heart disease

< 50-150 mmHg

< 80%, no improve

PPHN > 100 mmHg Up to 95%

Pre-/postductal PaO2 Test

Pre-/postductal PaO2 Test

Pre-/postductal PPHN CHD

PaO2 difference > 15-2o mmHg <15mmHg

SpO2 difference > 10% <10%

6. Investigation

• Chest X-ray

• Help differentiate lung parenchymal diseases, some congenital anomaly, and some congenital heart diseases

• EKG

• Useful to detect cardiac arrhythmias, but is not useful to detected serious neonatal cardiac condition such as TGA

Identify possible cause3 Central Cyanosis

CNS

Cardiac disease

Cyanosis not improve when crying No respiratory effort +/- murmurs +SpO2, PaO2 do not improve after O2 support Abnormal S2 sound +/- CRX abnormal +/- EKG annormal

Cyanosis improve when crying Respiratory effort; grunting, chest wall retraction, RR>60/min Normal cardiac examination +SpO2, PaO2 do improve after O2 support Normal CRX, EKG

Perinatal asphyxia Hypoventilation Heavy maternal sedation Intrauterine fetal distress Difficult delivery

Pulmonary disease

TREATMENT BY SPECIFIC CAUSE

Respiratory distress in the neonates

Common abnormalities in the Neonatal Respiratory Distress2

Initial management Specific treatment

RDS On O2 support, with

CPAP , or ET-tube Keep normal BT at

36.5-37.5c

Correct metabolic disturbance

IV fluid support

Surfactant,mechanical ventilation

MAS

Keep PaO2 60-80 mmHg, adequate O2,

ventilation

Sepsis/Pneumonia Antibiotic

Pneumothorax Pleural tapping

Congenital diaphragmatic hernia

Retain OG tube, definitive surgery

Airway obstruction Definitive surgery

Cardiac cause of neonatal CHD

Congenital Heart Disease7,1

Pulmonary BloodFlow Cyanotic Acyanotic

High flow

TA TGA TAPVR Common Atrium Common Ventricle

ASD VSD PDA AVC

Low flow

TOF Tricuspid atresia Ebstein’s anomaly Pulmonary stenosis

Normal -

Coarctation of Aorta Aortic stenosis

CYANOTIC CONGENITAL HEART DISEASE

Fetal and neonatal circulation • Structure and Function: The Heart Before and After Birth

Source aviva.co.uk

ทมา ภาวะวกฤตทางหวใจในเดก; มลนธเพอสนบสนนการผาตดหวใจในเดก; กรกฎาคม 2551

Cyanotic Congenital heart disease

Common finding cyanotic

congenital heart disease

ทมา ภาวะวกฤตทางหวใจในเดก; มลนธเพอสนบสนนการผาตดหวใจในเดก; กรกฎาคม 2551

Ductal Dependent Cardiac Lesions

• Congenital cardiac abnormality that need the remain opening ductus arteriosus to maintain vital circulation.

• Must be considered in any neonate (<28day) with sudden onset shock should be treated as having ductal dependent lesions until proved otherwise

• PGE1 infusion, by maintaining patency of ductus arteriosus is life-saving in infants

Ductal Dependent Cardiac Lesions

• Lt. to Rt. shunt pulmonary vascular resistance is lower than systemic vascular resistance.

• Rt. to Lt. shunt pulmonary vascular resistance is suprasystemic.

Patent Ductus Arteiosus8,7,1,9

Dependent non-Dependent Initial Management

Pulmonary Systemic Confirm cardiac cause of cyanosis. Initial resuscitation; ABC’s, but limit O2 support in preterm Identify whether it is ductal dependent lesion if possible Medication PGE1 IV continuous drip 0.05-0.1mcg, prefer start with 0.1mcg, then taper down Side effect; apnea, flushing, diarrhea. Intubation may be used in some patient Correct metabolic disturbance(acidosis)

PA e IVS

TGA e IVS

TOF e PA

Critical PS

TA e PS/PA

Severe Ebstein’s anomaly

Univentricular Heart

Coarctation of aorta

Critical AS

HLHS

IAA

TAPVR If can’t rule out the non-ductal dependent lesion, and the patient became more deteriorate, PGE1 may be used

Truncus arterosus

TOF • Most common cyanosis CHD, 14% of all CHD

• PE; Loud single S2, systolic ejection murmur Lt. mid-upper sternal border, clubbing of finger

• Cyanosis with decrease pulmonary blood flow, no CHF

• TOF with PA has early onset

of cyanosis, ductal dependent

lesion, no murmur

Source Swatchz’s principal of surgery edition 10th

TOF

TGA

• 3rd common cyanotic heart disease, associated with another CHD


TGA • A. The heart is enlarged with a narrow "pedicle" giving the so

called "egg on a string" appearance.

• The superior mediastinum appears narrow due to the antero-posterior relationship of the transposed great vessels and "radiologic-absence of the thymus".

Ebstein anomaly

• an uncommon congenital cardiac anomaly, characterised by a variable developmental anomaly of the tricuspid valve

TAPVR(Total anomalous pulmonary venous return)

• Mixing blood circulation with increase pulmonary blood flow

• Pulmonary vein obstruction is key to determine severity of disease

TAPVR

TAPVR (Supracardiac)

Finding mild cardiomegaly, increased pulmonary vascular markings and "snowman" appearance

TAPVR(infradiaphragmatic-obstructed)

• The heart is normal sized with increased pulmonary venous pattern preferentially in the right upper lobe

https://www.bcm.edu/radiology/cases/pediatric/text/2e-desc.htm

Truncus arteriosus

• Single great vessel exit from heart, mixing blood circulation, rare, 0.9% of all cyanotic CHD

• Present with late cyanosis, with congestive heart failure, or URI on top



• Group of abnormality such as; tricuspid atresia, pulmonary atresia, HPLS, single ventricle

• 2nd most common with 10% of all CHD

• Mixing blood lesion, variable severity

Tricuspid atresia

• Complete absence of the communication between the right atrium and ventricle. This lesion occurs in approximately 1:15,000 live births

HPLS

• Most severe cyanotic CHD


• Pulmonary atresia


Cyanosis neonate in cardiac disease • Most common cyanosis CHD in neonate is TOF

• Most of early cyanosis in newborn <1wk is cyanosis CHD with ductal dependent lesion

• Subacute cyanosis with CHF usually come from decrease pulmonary vascular resistant present at > 2wk of life

• Hypercyanotic spell from TOF mostly present late, at 2mo.-6mo.

Before refer to specialist

• If possible, identify ductal dependent lesion

• Resuscitation; ABC’s, but limit O2 support in preterm

• Medication;

• PGE1 IV continuous drip 0.05-0.1mcg, prefer start with 0.1mcg, then taper down

• Side effect; apnea, flushing, diarrhea

• Correct metabolic disturbance(acidosis)

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References

1.ภาวะวกฤตทางหวใจในเดก; มลนธเพอสนบสนนการผาตดหวใจในเดก; กรกฎาคม 2551 2.Cyanosis in neonate; คมอกมารเวชศาสตรฉกเฉน; คณะแพทยศาสตร รามาธบด กนยายน 2554 3.Fetal Asphyxia; คมอทารกแรกเกด; คณะแพทยศาสตร มหาวทยาลยของแกน 4.Evaluation and management of the cyanotic neonate;Clin Pediatr Emerg Med. ;Pubmed;Author manuscript; PMC 2009 Sep 1. 5.Pediatric cardiology; 6.Swatchz’s principal of surgery edition 10th 7.โรคหวใจตงแตก าเนด; ต ารากมารเวชศาสตร; คณะแพทยศาสตร มหาวทยาลยศรนครทรวโรฒ 8.Identification and Management of Ductal Dependant Cardiac Defects in the Transport Setting - Robyn Neely Funk, RN, BS/BSN, PHRN, CMTE 9.Ductal-dependent cardiac lesions/Hyperplastic left heart syndrome; Atlas of pediatric emergency medicine; McGrawHill; second edition 10.Patent Ductus Arteriosus Aortopulmonary Window; George A. Gibson9 11.https://www.bcm.edu/radiology/cases/pediatric/text/2e-desc.htm

Education

Approach to the neonatal cyanosis