CYANOTIC CONGENITAL HEART DISEASES WITH DECREASED BLOOD FLOW

Dr. Veerabhadrudu. K

MBBS, DNB Paediatrics--III

Cyanosis is a bluish or purplish tinge of the skin and mucous membranes.

Clinically detectable only when reduced Hb

is >5g% or saturation is <85%.

Cyanosis is recognized at a higher level of oxygen saturation in patients with polycythemia and at a lower level of oxygen saturation in patients with anemia.

Pulmonary RDS Pneumothorax Pneumonia MAS CDH TTNB PPHN

• CVS

– Cyanotic HD

• Pulm blood flow

• Pulm blood flow

• With CHF

– CHF alone

• CNS– Maternal

meds

– Asphyxia

– ICH

– NMD

• Metabolic– Hypoglycemia

– Adrenogenital syndrome

• Hematologic– Acute blood loss

– Chronic blood loss

– Polycythemia

– Methemoglobinemia

• Infectious– SEPSIS

Central – cyanotic CHD Peripheral – hypothermia, CCF Mixed Cyanosis – CHD in Shock

Differential cyanosis – PDA with reversal Reversed differential cyanosis – TGA with PDA

with reversal

Intermittent Cyanosis – Ebstein’s anomaly

Circum oral cyanosis

Cyclical cyanosis – Bilateral choanal atresia

Pseudo cyanosis : a bluish tinge to the skin and/or

mucous membranes that is not associated with

either hypoxemia or peripheral vasoconstriction.

Most causes are related to metals (e.g., silver

nitrate, silver iodide, silver, lead) or drugs (e.g.,

Phenothiazines, amiodarone, chloroquine

hydrochloride).

True Cyanosis

Associated with

clubbing

ABG - confirms

Cyanosis like

conditions

Not associated

with clubbing

Lab estimation of

Meth Hb and Sulph

Hb Confirms

Acrocyanosis Central cyanosis

Involves peripheries tongue, nail beds and

mucosal membranes

Peripheries Cold Warm

CRT Delayed Normal

Crying Decreases Increases

Part of normal

transition

May last upto 72 hrs

Cardiac Respiratory

At rest Comfortable Distress

Crying Increases Decreases

Resting PaO2 <60 >100

PaCO2 Normal Abnormal

On giving 100% O2 PaO2 not increased PaO2 increases

SpO2 <95% >95%

CVS Murmur No murmur

ECG Abnormal Normal

Echo Abnormal Normal

Most sensitive and specific tool for

evaluation of a neonate with suspected CHD

especially in the absence of ECHO.

Helps to differentiate the cardiac and non

cardiac causes of cyanosis.

Pulse oximetry is not reliable, but in resource limited settings, it can be used.

Both pre and post ductal sites are used.

Rt radial ABG in air after administration

of 100% O2 for 5-10min.

Rt radial ABG in air after 10 min 100% O2

paO2 > 250mmHg - excludes CCHD

paO2 > 150 to 250 - CCHD unlikely (mixing lesions)

paO2 < 150 - CCHD likely (shunting lesions)

< 150 mmHg most likely duct dependent lesions, so PGE1 can be started until anatomic lesion is defined

Interpretation of hyperoxia test: From Harriet Lane Handbook

FiO2= 0.21 PaO2 FiO2 =1.00 PaO2 PaCO2

(%sats) (%sats)

Normal 70 (95%) >200 (100%) 35

Pulmonary Dz 50 (85%) >150 (100%) 50

Neurologic Dz 50 (85%) >150 (100%) 50

Methemoglobinemia 70 (95%) >200 (100%) 35

Cardiac Dz

Separate circulation

(TGA no VSD) <40 (<75%) <50 (85%) 35

Restricted PBF

( TA +PS, PA, PS + no VSD, TOF) < 40 (<75%) <50 (<85) 35

Complete mix no restricted PBF 50 (85%) <150 (<100%) 35 (Truncus, TAPVR, Single Vent,

TGA +VSD, TA no PA or PS)

PPHN Preductal Post ductal

PFO no R->L shunt 70 (95%) <40 (<75%) Variable 35-50

PFO + R->L shunt <40 (<75%) <40 (75%) Variable 35-50

Clubbing

Cyanotic spell

Depressed IQ

Infective endocarditis

Polycythemia

Embolic phenomenon

Cyanotic Congenital Heart Disease

Increased

Pulmonary Blood

Flow

Cyanosis, Clubbing, Polycythemia

Decreased

Pulmonary Blood

Flow

Transposition of Great arteries

(3-5%)

Truncus Arteriosus (1-2%)

Single Ventricle (1-2%)

TAPVC (1-2%)

HLHS (1-3%)

Tetralogy of Fallot

(7-10%)

Tricuspid Atersia

Ebstein’s Anomaly

Pulmonary Atersia

% given for 100

CHDs

Clinical sub types:

Tetrology of Fallot

Tricuspid atresia with PS

DORV with VSD and PS

Single ventricle with PS

TGA and VSD with PS

AVSD with PS

In 1888, Fallot described the anatomy of TOF

Incidence 7-10 % of all forms of congenital

heart disease

The most common cardiac malformation

responsible for cyanosis after 1 year of age.

The four components

of TOF are

VSD

RVOT

Overriding aorta

RVH

Only two abnormalities are required A VSD large enough to equalize pressures in both

ventricles

A right ventricular outflow tact obstruction

RVH is secondary to right ventricular outflow tract obstruction (RVOT) and VSD

Over riding of aorta varies

VSD is perimembranous defect with extension into the subpulmonary region

VSD is non restrictive and large

RVOT obstruction can be in the form of

Infundibular stenosis - 45%

Pulmonary valve stenosis - 10%

Combination - 30%

Pulmonary atresia - 15%

Pulmonary annulus and MPA are hypoplastic

Right aortic arch in 25% of TOF

Abnormal coronary arteries in about 5% of

TOF patients

Enlarged, overriding aorta and hypoplasia of

pulmonary flow results from septation of the

conus and truncus arteriosus with anterior

deviation of the conus into the RV outflow

Experimental lesions in specific loci of

ectodermal tissue that migrate to the conus

can reproduce the defects seen in TOF

* PS increased RV

pressure, RVH

*pressures in both RV&LV ---

EQUAL

*RT LT shunt occurs

*flow across PS results in ESM

*the more severe the PS the

more the

RT LT shunt, so, more

cyanosis.

HAEMODYNAMICS:

Appearance of cyanosis

After neonatal period

Exception TOF with Pulmonary atresia

Hypoxemic Spells

Low birth weight or development delay or

easy fatigability

Cyanosis

Clubbing

Polycythemia

Squatting position

Tachypnea

Increased activity

Increased respiration

Increased venous return

Fixed pulmonary blood flow

Increased (RV) to (LV) shunt

Increased cyanosis

Decreased venous return

Increased systemic vascular resistance

Increased pulmonary blood flow

Decreased cyanosis

Squating Equivalent – Knee Chest position,

child sitting with flexed limbs, mother

carrying the child with folded limbs and

others

RV tap in left sternal borderSystolic thrill in upper and mid left

sternal bordersEjection click which originates from aortaS2 is single due to absent pulmonary

component A loud ejection type systolic murmur

heard at the mid and upper left sternal border

This murmur originates from the Pulmonary stenosis and may be confused with the pansystolic murmur of VSD

Intensity of the murmur depends of the severity of RVOT obstruction

More severe the obstruction, shorter and softer murmur will be heard

In Pulmonary atresia, murmur is either absent or very soft

Auscultation of back is important to find the presence of MAPCAs ( Major AortoPulmonary Collateral Arteries)

Acyanotic or pink TOF – RVOT obstruction is mild, so clinical picture resembles VSD

Pentalogy of Fallot – TOF with ASD

Tetralogy of Fallot with Pulmonary atresia

Tetralogy of Fallot with Absent Pulmonary Valve

Tetralogy of Fallot with absence of branch pulmonary artery

Fetal hydantoin syndrome

Fetal carbamazepine syndrome

Fetal alcohol syndrome

Maternal phenylketonuria (PKU)

CATCH 22 – Cardiac defects, abnormal facies,

thymic hypoplasia, cleft palate,

hypocalcemia

Anemia

Infective Endocarditis

Valvular Regurgitation

Surgically created or naturally occurring

large left to right shunts

Systemic hypertension

Unrelated or coincidental myocardial disease

Hematology Polycythemia secondary to cyanosis (hematocrit

>65%)

Anemia – due to relative iron deficiency

Electrocardiography

X-ray

Echocardiography

Angiogram

Right axis deviation, Right ventricular hypertrophy

Normal size heart

Pulmonary vascular markings are decreased

Concave main pulmonary artery segment with an

upturned apex – BOOT shaped heart or coeur en

sabot

Right atrial enlargement (25%)

Right aortic arch (25%)

Typical - Fallot CXR

Pulmonary bay

RV apex

Pulmonary

oligaemia

TOF-boot ,no cardiomegaly, oligaemia

Hyper cyanotic or Tet or cyanotic or hypoxic spell

Peak incidence 2 - 4 months

Usually occurs in morning after crying, feeding or defecation

Severe spell may lead to limpness, convulsions, CVA or even death

Secondary to infundibular spasm and/or

decreased SVR with increased right-to-left

shunting at the VSD, resulting in diminished

pulmonary blood flow

Spasm of decrease SVR crying/feeding/defaecation

RVOT

Increase R…..L shunting

Increase systemic venous return DecreaseO2 Increase CO2 Decrease pH

Tachypneea

Sudden onset of cyanosis or deepening of

cyanosis

Sudden onset of dyspnea

Alterations in consciousness, encompassing a

spectrum from irritability to syncope

Decrease in intensity or even disappearance

of systolic murmur

Central cyanosis & anoxic spell (hyper cyanotic):

Knee chest position or squatting –decreases systemic venous return and increases systemic vascular resistance at femoral arteries

Morphine sulphate – 0.1 - 0.2mg/kg sc or im, suppresses the respiratory centre and abolishes hyperpnoea

Oxygen has little effect of arterial oxygen saturation

Acidosis should be treated with sodium bicarbonate 1mEq/kg administered intravenously

Preceding treatment, patient becomes less cyanotic, and heart murmur become louder

Indicates increased amount of blood flowing through stenotic right ventricular outflow tract

If Hypoxemic spell not fully respond Vasoconstrictor: Phenylephrine 0.02 mg/kg IV

Propranolol 0.01 to 0.25 mg/kg slow IV push, reduces the heart rate and may reverse the spell

Ketamine 1 – 3 mg/kg over 60 secs, increases systemic vascular resistance and sedates the patient

Prevention of Hypoxemic spell Oral Propranolol 0.5 to 1.5 mg/kg every 6 hours

Relative IDA should be detected and treated since anemic children are more susceptible to CVA

Maintenance of good dental hygiene and infective endocarditis prophylaxis

Hct has to maintained <65%, Phlebotomy may be needed to manage polycythemia

Neonates with TOF and pulmonary atresia

Infants with hypoplastic pulmonary annulus, which requires a transannular patch for complete repair

Children with hypoplastic pulmonary arteries

Severely cyanotic infants younger than 3 months of age

Infants younger than 3 to 4 months old who have medically unmanageable hypoxic spells

Systemic – Pulmonary Shunt

Blalock-Taussig: between subclavian artery and ipsilateral PA, preformed in infants older than 3 months

Gore-Tex Interposition shunt (modified BT): between subclavian and ipsilateral PA, done even in small infants younger than 3 months

Waterston: between ascending aorta right PA, no longer performed

Potts: between descending aorta and left PA, no longer performed

Symptomatic infants with favourable anatomy of the RVOT and PA

Asymptomatic and minimally cyanotic children may have repair between 3 and 24 months depending on the degree of annular and PA hypoplasia

Mildly cyanotic who had previous shunt surgery may have total repair 1 to 2 years after shunt operation

Asymptomatic and acyanotic children have the operation at 1 to 2 years of age

Asymptomatic children with coronary artery anomalies may have repair at 3 to 4 years of age

Patch closure of VSD

Widening of RVOT by resection of the

infundibular tissue and placement of a fabric

patch

Takedown of prior shunt (if done)

Congestive heart failure (right or left),

residual outflow obstruction, VSD, and/or

pulmonic regurgitation

Atrial flutter, ventricular arrhythmias, RBBB,

or left anterior hemiblock

Infective endocarditis

Erythrocytosis

Brain abscess

Acute gouty arthritis

Infective endocarditis

Cerebrovascular thrombosis

Delayed puberty

Scoliosis

Pulmonary Atresia

- With VSD - Extreme form of TOF

- Without VSD - No egress of blood from Rt vent.

- Shunt through foramen ovale to Lt atrium

Left ventricle

systemic circulation

Aorta

pulmonic circulation

- Hypoplastic right ventricle (PDA)

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Clinical Manifestation- Cyanosis at birth- Respiratory distress- Single 2nd heart sound- No murmur

Diagnosis- CXR - concave pulmonary artery segment

and apex tilted upward- ECG- Echocardiography

Treatment - PGE1

- Surgery

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PA with IVS – LAD with RAE and LVH

Tricuspid atresia- No outlet from RA to RV

- Systemic venous return

Rt atrium

Lt atrium

Left ventricule

systemic Pulmonic

(VSD, PDA)

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Clinical Manifestation

- Cyanosis at birth

- Polycythemia

- Easily fatiguability

- Exertional dyspnea

Diagnosis

- CXR

- ECG – “superior” QRS axis with RAE and LVH

- Echocardiography

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Pulmonary vascularity reduced.

Pulmonary artery segment – inconspicuous.

Heart size – normal.

LAO – Humped appearance of right cardiac

border and a prominent left cardiac

silhouette

Inferior border – flat or receding owing to

absence of RV.

TA - ECG – “superior” QRS axis with RAE and LVH

Presence of an

imperforate linear

echo density in the

location of normal TV.

Confirm the presence

and size of the

interatrial

communication and

VSD.

Limited role at present.

Therapeutic role for balloon atrial

septostomy.

Prior to a Fontan procedure for determining

pulmonary vascular resistance.

- PGE1, balloon atrial septostomy

- Surgery - Aortico - pulmonary Shunt

- Bidirectional Glenn shunt

- Modified Fontan operation

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Displacement of abnormal tricuspid valve into right ventricle

Anterior cusp retains some attachment to the valve ring

Other leaflets are adherent to the valve of the right ventricle

Right atrium is huge -Atrialisation of Right Ventricle

Tricuspid valve is regurgitant

Clinical Manifestations- Easly fatiguability- Intermittent Cyanosis- Dysrhythmia- Rt to Lt shunt through formen ovale- Holosystolic Murmur at tricuspid area- Heart failure

- Multiple Clicks

Diagnosis- CXR - box shaped heart- ECG - Right BBB

- Superior axis deviationTreatment

- PGE1

- Surgery

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Ebstein’s anomaly –BOX -heart

RBBBRAE

Ebstein’s with WPW Syndrome

TGA with VSD and PS; single ventricle with PS or

DORV with PS will have TOF picture

Thank you…