CYANOSIS

Bluish purple discoloration of the skin and/ or mucus membrane due to lack of oxygen in the blood to supply to the tissue

Cyanosis

True cyanosis

pseudocyanosis

Methemoglobinemia

Hemoglobin cyanosis

Central cyanosis

Peripheral cyanosis

PSEUDOCYANOSIS
bluish discoloration of the skin and mucous membranes, caused neither by hypoxemia nor by Peripheral vasoconstriction. It is due to skin pigmentation or deposits of exogenic substances, e. g., metals (silver nitrate,silver iodide, silver, lead) or drugs (amiodarone, chloroquine, phenothiazines).

Methemoglobin

Altered state of hemoglobin in which the ferrous (Fe2+) irons of heme are oxidized to the ferric (Fe3+) state. ferric hemes has lost its capacity to transport oxygen. Cyanosis becomesclinically visible when methemoglobin concentration exceeds 1.5 g/dL. Hereditary methemoglobinemia- Pronounced cyanosis is typically already present at birth or shortly after birth NADPH Methemoglobin Reductase Deficiencyautosomal recessive hereditary disorder. Deficiency in NADPH methemoglobin reductase increases the amount of methemoglobin in the blood

Central cyanosis
Concentration of deoxyHb >5g/dl

Peripheral cyanosis
Reduced blood supply

Presentation: Tongue
1. Arterial O2 Saturation 2. Polycythaemia 3. Hb abnormalities(rare)

Presentation: Extremities( Hands and feet)

1. Causes of central cyanosis 2.Exposure to cold 3. Cardiac output 4.Arterial/Venous obstruction With central cyanosis: consider CVS & Respiratory Without central cyanosis: VasoC, Obstruction( Arterial & Venous), Cardiac output.

CVS & Respiratory system

Hb

content: 15g/L O 2 saturation: 79%

Hb

content: 9g/L O 2 saturation: 65% Other manifestation of hypoxemia(respiratory symptoms, mental status change)
Hb

content: <9g/L Succumb from hypoxemia before cyanosis become evident

Therefore,

cyanosis is less pronounced in anaemic patients.

CARDIAC CAUSES OF CYANOSIS

How cardiac problems can cause cyanosis ?

Obstruction to pulmonary blood flow Right to left shunt Total mixing of systemic and pulmonary venous blood

Cyanotic

congenital heart diseases are the 5 TS

Cardiac Problems

Tetralogy

of Fallot Transposition of great vessels Tricuspid valve atresia Total anomalous pulmonary venous return Truncus arteriosus Hypoplastic of left ventricle

Tetralogy Of Fallot
Most

common type of (CCHD) , 10 % Most common who do survive the neonatal period Clinical feature : - large VSD - aorta that overrides the right and left ventricles - pulmonary Stenosis - right ventricular hypertrophy Obstruction of right vent. Outflow tract
Blood is shunted R to L, thru the VSD Cyanosed

Symptoms & Signs

Symptom : - hypercyanotic spell

( Hypoxic episodes on exertion ) - Patients have deep cyanosis and possibbly syncope when crying n feeding - retarded growth

Sign : - clubbing and polycythaemic obvious

after 12 m/o - loud harsh ejection systolic murmur - single 2nd heart sound

Investigations
1) Chest x-ray - Boot shaped heart 2) ECG - usually normal at birth - Right ventricular hypertrophy at older child 3) Echocardiography - demonstrate all cardinal feature, level of stenosis degree of stenosis.

CXR

Treatment :
Squatting position / knee chest position to increase the venous return O2 administration ( min effect ) Phenylephrine to increase systemic vascular resistance Complete surgical repair - VSD closure n removal of pulmonary stenosis

Transposition of Great Vessels

Represent only 5% of CCHD Occur in 2-3/ 10 000 live birth M: F is 2 :1 blood circulate parallel circulation 50 % a/w VSD

TGA- Clinical features

May

present within few hours of birth as cyanotic baby, but 90 % present a few days later. Cyanosis depends on the amount of mixing Severe cyanosis from birth
closure of DA reduced mixing of blood cyanotic If there is mixing (associated anomaly), less severe cyanosis Without mixing , death occur quickly Mixing can occur at atrial ( patent foramen ovale, ASD ) at ventricular ( vsd ) or PDA

 Quiet

tachypnea Single S2 If ventricular septum intact- no murmur Transposition n VSD

Improved

intracardiac mixing Less cyanosis Signs of heart failure Hyperdynamic heart Palpable L n R Ventricular impulse Loud VSD murmur S2 is single

Investigation n treatment
Investigations

- 2D echocardiography, identify the connection btwn great vessels and the cardiac chambers, detect ass. VSD,ASD,PDA Treatment - Prostaglandin (E 1) , maintain d PDA - Rashkind balloon atrial septostomy - Arterial switch operation, first 2 wks of life

Total Anomalous Pulmonary Venous Return

1

% of CHD Distruption of the development of normal pulmonary venous drainage ( 3rd gest ) the four pulmonary veins are not connected to the left atrium n return abnormally via R. side of the heart. Atrial level communication need for survival n syst. Cardiac output.

Tricuspid atresia

Truncus arteriosus

Hypoplastic left heart syndrome

CYANOSIS IN RESPIRATORY SYSTEM

Hypoxemic respiratory failure Hypercabic respiratory failure

ventilation perfusion mismatch when the perfusion of lung not adequately ventilated

inadequate alveolar ventilation 2ndry to increase in dead space ventilation.

High ratio due to low perfusion,such as pulmonary embolism /low ratio due to lowventilation such as in asthma and pulmonary oedema

Hypoxemic respiratory failure is commmon in hypercabic respiratory failure.

ACUTE BRONCHIOLITIS
First time wheezing with viral respiratory infection for less 2 years child. respiratory tract inflammation with airway obstruction due to swelling of small bronchioles cause inadequate expiratory airflow. Severe in infant because of smaller airways and immature immune system.. Respiratory syncytial virus is the most common cause. Early present with common cold,cough,rapid breathing and low grade fever. Differentiate from asthma by age presentation,presence of fever,no family history of asthma and for asthma has recurrent epsodes. Cause hyperexpansion of the lung

ASTHMA
Inflammation due to hyperresponsive airways that cause bronchospasm which is reversible obstruction of airways in response to allergens,irritants,viral infection,humidity and exercise. mucus secretion and it is chronic respiratory disorder Symptoms - coughing,rapid breathing,chest tightness,nocturnal cough and morning exacerbation. Sign-resp distress,hyperinflated chest and hyperresonance on percussion. For asthma patient,they have prolong expiratory phase,wheezing and tachypnea. When the shortness of breath continue,it can cause cyanosis and unable to speak in full sentences and can cause hypoxemic respiratory failure.

ASTHMA
. Atopic disease such as eczema, allergic rhinitis and allergic conjuctivitis can be seen in asthma patient.(Type 1 hypersensitivity) In FBC,eosinophils will increase Family history of asthma and allergic history are very important for the diagnosis of asthma. Usually,give Salbutamol nebulizer to reduce the shortness of breath and some asthma patient need inhaler for the sudden attack of asthma Ventolin evohaler(blue) when asthma attack and Budesonide aerosol(brown) twice a day.

PNEUMONIA
Infection of lung parenchyma Lobar pneu effect one or more lobes and bronchopneu like patches consolidation on CXR. Viral pneumonia is the most common and bacteria is common in older children. Severe pneumonia can cause pleural efffusion that will reduce lung compliance,so cause rapid breathing that can lead to decrease in exchange of gas. Newborn-bacteria from mother(group b haemolytic,E.coli.Klebsiella) Infant mostly from RSV ,if bacteria,Strep pneumonia.H.infuenza Older children-Mycoplasma pneumoniae and Strep pneumoniae

PNEUMONIA
Syptoms-fever,cough,runny nose,restless,breathlessness,lethargy,pleuritic chest pain. If pneumonia is severe,it can cause convulsion and poor feeding. In FBC, increase WBC may suggest bact cause and if normal or decrease may suggest viral cause For viral pneumonia,it do not require antibiotic. Supportive treatment is very important to maintain the body fluid,temperature and oxygen monitiring. There is also aspirate pneumonia

Antenatal hx(alcoholic,high age pregnancy,DM,) Any siblings with Cyanotic HD. Dyspnoea on exertion Failure to thrive Irritability and inconsolable crying, due to hypoxia and breathlessness. Squat after exertion to prevent syncope Symptoms of paediatric heart failure (BP,HR,dyspnoea,tachypnoea,difficulty feeding,cardiac asthma[wheezing],hepatosplenomegaly,ascites,pe dal edema,)

Dysmorphism(Downs,DiGeorge) Digital clubbing Parasternal heave due to right ventricular hypertrophy(TOF,TAPVR) Heart murmur (varied) Fixed split S2 heart sound S3 gallop.

Asthma history Fever Chesty Cough Noisy breathing Rhinorrhea Neck or Abdominal pain Lethargic

Vital signs Respiratory distress signs Hyperinflation Rhonci Bronchial breath sound? Crepitation Consolidation