MANAGEMENT OF CYANOTIC CHILD BIVIN

DR BIVIN WILSON DNB REGISTRAR CARDIOLOGY

Cyanosis -- Greek word meaning "dark blue")

Cyanosis is dependent upon the absolute concentration of the reduced hemoglobin and not on the ratio of reduced hemoglobin to oxyhemoglobin.

Central cyanosis is one, which is present throughout the body, including the mucous membranes and tongue. If cyanosis is limited to the extremities, it is called peripheral cyanosis

Differential cyanosis - PDA with reversal of shunt or severe left sided obstructive lesions

In Reverse differential cyanosis -TGA with PHT&PDA and TAPVC above the diaphragm with PDA.

General causes of Cyanosis

PulmonaryPulmonary CardiacCardiac OthersOthers

Airway diseaseAirway disease Intrapulmonary shunting

Intrapulmonary shunting

Intracardiac shunting

Intracardiac shunting

CyanosisCyanosis

CAUSES OF CENTRAL CYANOSIS

A. Congenital Heart Disease

1) Cyanosis with PBF 2) Cyanosis with PBF

a) TOF a) D-TGA b) Pulm. Atresia b) DORV c) Tricuspid Atresia c) TAPVC d) Critical PS d) Truncus

arteriosus

CAUSES OF CENTRAL CYANOSIS B) LUNG DISEASE D) CNS DEPRESSION a) RDS a) IVH b) Pneumonia b) Perinatal asphyxia c) Pneumothorax c) Heavy maternal sedation d) Pleural effusion e) Diaphragmatic hernia f) T.E.Fistula

C) PERSISTENT PULMONARY E) MISCELLANOUS HYPERTENSION a) shock & sepsis b) Hypoglycemia c) Methemoglobinemia d) Neuromuscular

conditions ( Werdnig – Hoffman)

STEPS IN THE MANAGEMENT OF CYANOTIC NEWBORNS ABG in room air: confirm or reject

central cyanosis. An elevated PCO 2 suggests

pulmonary or central nervous system problems.

A low pH may be seen in sepsis, circulatory shock, or severe hypoxemia

Hyperoxia test: To differentiate between Cardiac from Pulmonary cause. Oxygen should be administered through a plastic hood

for at least 10 minutes to replace the alveolar air completely with oxygen.

With pulmonary disease, arterial PO 2 usually rises to> 150 mm Hg.

When there is a significant intracardiac right-to-left shunt, the arterial PO 2 does not exceed 100 mm Hg, and the rise is not more than 10 to 30 mm Hg.

However in Persistent Pulmonary Hypertension of newborn with a normal heart) PaO 2 may not have a rise in arterial PO 2 to 100 mm Hg.

The purpose of positive hyperoxia test helps in ruling out significant cyanotic congenital heart defect but when negative does not differentiate between cyanotic heart and PPHN.

ECG may be helpful in cardiac origin of cyanosis

Chest x-ray films: may reveal pulmonary causes of cyanosis. They can also hint at the presence or absence of cardiac defects and the type of defect

2-D Echocardiography and a Doppler examination

RADIOLOGICAL FEATURES CXR may exclude non cardiac causes of cyanosis e.g. RDS. .

Meconium aspiration, Diaphgramatic hernia, PneumothoraxPulmonary Vascular Markings

Decreased Increased

Heart Size Heart Size

Normal Increased Increased( “Boot shaped”) (“ Wall-to-Wall”) TOF Ebstein (“ egg-on-end”) D-TGA Aortic Arch \ Mediastinum

Abdominal Situs

CLUES TO DIAGNOSIS

TRANSIENT CYANOSIS – ASD,Ebsteins anomaly, DORV with subaortic VSD without PS & Truncus arteriosus

INTERMITTENT CYANOSIS --Ebsteins anomaly, TAPVC unobstructed,Complete A-V canal defect & Eisenmengers with bidirectional shunt

PERSISTENT AND PROGRESSIVE CYANOSIS –TGA, Single ventricle,Hypoplastic left heart Syndrome ,Tricuspid atresia ,Pulmonary atresia &Ebsteins anomaly

DEFERRED CYANOSIS –TOF,TGA with VSD with PS, Corrected TGA with VSD with PS, DORV with VSD with PS & Single ventricle with VSD with PS

CYANOSIS WITH FAILURE

Congestive cardiac failure in a patient with cyanosis denotes cyanotic heart disease with increased pulmonary blood flow physiology.

1) Transposition of great vessels 2) Taussing Bing anomaly 3) Truncus arteriosus 4) Total anomalous Pulmonary venous

connection 5) Single ventricle with low PVR and no

pulmonary stenosis 6) Common atrium

CYANOSIS WITH SQUATTING

1) Tetralogy of Fallot 2) Tricuspid atresia 3) Pulmonary atresia 4) Double outlet right ventricle 5) Single ventricle 7) Eissenmengers syndrome

CYANOSIS WITH COLLAPSING PULSE --TOF with AR ,Truncus arteriosus TOF/PA with increased aorto-pulmonary collaterals , Following shunt surgeries & Cyanotic CHD with PDA

CYANOSIS WITH a WAVE IN JVP -Tricuspid atresia, Pulmonary atresia with intact IVS,TGA with intact IVS, Eissenmengers ASD &TOF

CYANOSIS WITH CONTINUOUS MURMUR - Pulmonary atresia with VSD ,Truncus arteriosus ,TOF with peripheral artery stenosis ,Post shunt surgery,& Hypoplastic left heart

CYANOSIS WITHOUT MURMUR--PAH / Eisenmenger,

CYANOSIS WITH LEFT AXIS DEVIATION IN ECG

Cyanotic heart disease with LAD in ECG are

1) Tricuspid atresia 2) Complete AV canal defect 3) Single ventricle –LV type 4) Large Pulmonary AV fistula

INTERVENTIONS

Ductus dependent lesions

Those with severe RVOT obstruction: Pulmonary atresia, TOF, Severe pulmonary stenosis.

Transposition of great arteries with intact ventricular septum.

Tricuspid atresia with pulmonary stenosis.

Hypoplastic left heart syndrome.

Interventions to maintain ductal patency

Prostaglandin infusion to maintain ductal patency.

Transcatheter intervention

Endo vascular stents

PROSTAGLANDINS :

PG E1 : 0.01 – 0.1 mcg / kg / min

PG E2 : 0.003 – 0.01 mcg / kg / min

Start low dose and increase at 15 – 30 mins. interval, if no response.

> 3 fold increase in dose – rarely needed.

Oral PG E 2 – 25 – 40 mcg / kg / h . go stepwise to 4 – 6 hourly for c/h use.

Balloon atrial septostomy

Introduced by Rashkind

Improves mixing of oxygenated and deoxygenated blood in pts with transposition physiology or in those requiring venting of an atrium with restricted outflow

Better inter - atrial mixing. Transposition of great arteries

Augmentation of L-R shunt Mitral atresia DORV with restrictive left A-V valve, Univentricular heart with restrictive left A-

V valve,

Augmentation of R-L shunt TAPVC Pulmonary atresia, tricuspid atresia Ebstein's anomaly

Blalock – Taussig shunt Allows partially desaturated systemic blood to

enter the pulmonary artery, thus increasing pulmonary blood flow, and hence, oxygenation.

To avoid the problems of growth retardation of the limb associated with the ligation of the subclavian artery, modification was introduced

Modification of the Blalock - Taussig shunt in which a Teflon prosthetic tube is anastomosed end to side to the undivided subclavian artery and end to side to the pulmonary artery was first described by Klinner and colleagues.

Cyanotic Heart DiseaseCyanotic Heart Disease

Tetralogy ofFallot

Tetralogy ofFallot

Transposition Of Great Arteries


TruncusArteriosusTruncus

Arteriosus

TricuspidAtresia

TricuspidAtresia

Total AnomalousPulmonary venous Return


Tetralogy of Fallot (TOF)1. RVOT

obstruction2. VSD3. Overriding

aorta4. RV

hypertrophy

Tetralogy of Fallot (TOF)1. RVOT

obstruction2. VSD3. Overriding

aorta4. RV

hypertrophy

Hypercyanotic spell

Primary problem is decreased pulmonary blood flow with increased R->L shunting

Typically occurs in morning of after sleep (SVR is low, and blood volume is low)

May be precipitated by activity or fright, but may also be spontaneous

Hypercyanotic spell

Cyanosis will be accompanied by Hyperpnea

Increased rate and depth of respirations Increased fussiness progressing to

decreased level of consciousness Increasing acidosis, can be fatal

Hypercyanotic spell

Theories Primary infundibular spasm (unlikely) Hyperpnea as a primary cause Circulating catecholamines Reduced SVR

Spells are an indication for need of surgical intervention

Tachycardia

Impaired RV filling

RVOT obstruction

Rt Lt shunt

Agitation

Hypovolemia

Age PVR

Hypercyanotic Spells ( TET spells)

Cyanosis

Syncope

FluidMorphine

Oxygen

Phenylepherine

Knee-chest

Cyanotic Spells Increase systemic vascular resistance Squat/Knee chest position Ketamine 1-2mg/kg IV Phenylephrine 0.02mg/kg IV Tachycardia Propranolol 0.1mg/ Kg IV Release of infundibular spasm Irritability Morphine 0.2mg/ Kg S.C or IM

Hypoxia Oxygen Dehydration Volume

Acidosis NaHco3 1mEq/ Kg IV

Surgical Palliation

Surgical Management

VSD closure transatrial access if possible Infundibular resection for visualization Patch closure

Relief of RVOT obstruction Infundibular resection vs. transannular

patch

Transposition of Great Areries (TGA)

Aorta originating from the right ventricle, and pulmonary artery originating from the left ventricle

Accounts for 5-7% of all congenital heart disease

TGA .. Acute Management

PGE-1 with no supplemental O2 Maintain ductus arteriosus patency, this

will increase the effective pulmonary blood flow, and thence increase the left atrial pressure, therefore inhance the left to right shunt at the atrial level

Balloon atrial septostomyLife saving procedure in the presence of

inadequate atrial septal defect

TGA .. Surgical Management

Arterial switch with re-implantation of the coronary artery

to the new aortic site.

Atrial switch : the old style surgery Redirecting the pulmonary and systemic

venous return to result in a physiologically normal state

The right ventricle remains the systemic ventricle

Rarely needed

Truncus Arteriosus

The presence of a common trunk that supply the systemic, pulmonary and coronary circulation

Almost always associated with VSD

1.2-2.5% of all congenital heart disease

Managment Acute management

Diuretics Afterload reduction to enhance systemic

blood flow

•Surgical management: complete repair with VSD closure and conduit placement between the right ventricle and pulmonary arteries•Long term problems :

–truncal valve dysfunction–RV conduit obstruction

Trcuspid Atresia

Complete absence of communication between the right atrium and right ventricle

About 3 % of congenital heart disease

Management

PBF

Decreased Increased

PGE-1, and minimal supplemental O2 to maintain ductal patency

Afterload reductionDiuretics

Surgical ManagementSingle ventricle paliation First stage : to establish a reliable source

of PBF Aorta to pulmonary artery shunt ( BT shunt) Pulmonary arterial banding in cases of

increased PBF

Second stage: Glenn Anastomosis ( superior vena cava to pulmonary artery

Third stage : Fontan anastomosis ( Inferior vena cava to pulmonary artery

TAPVC Treatment

Correct acidosis

Antifailure

Surgery: Anastomosis of Common Pulmonry Vein to the left

atrium

Cyanotic Heart DiseaseCyanotic Heart Disease

Tetralogy ofFallot

Tetralogy ofFallot



TruncusArteriosusTruncus

Arteriosus

TricuspidAtresia

TricuspidAtresia



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MANAGEMENT OF CYANOTIC CHILD BIVIN