Duct Dependent Lesions - PPSX

Duct dependent
Dr. Sinduja selvam

lesions
Fetal circulation
PDA
Briefly about various duct dependent lesions

Fetal circulation
Anatomy - shunts
Physiology : flow
Foramen ovale • ~15% blood goes to
lungs
Right atrium to left atrium
• ~85% of blood is
Ductus arteriosus shunted
Pulmonary artery to descending

• 60% RV to PDA to
aorta body
• 40% RA to PFO to
Ductus venosus
LA to LV to Aorta
Umbilical vein to IVC to body
Transition to normal
circulation
Patent Ductus arterious
(PDA)
PDA
Pre term infants : ductus tissue has
Lower intrinsic tone
Less smooth muscle fibre
Few sub endothelial cushions
Incidence in LBW - 15- 40%
Extremely premature babies <28 weeks - 50-65%

Ductal steal phenomenon
left - right shunt causes significant retrograde flow from

thoracic to abdominal aorta to pulmonary circulation
Systemic hypo perfusion - renal and mesenteric

circulation
Present throughout the cardiac cycle
Best seen in diastole during an ECHO
Low diastolic aortic pressure compromises the

coronary circulation - causes myocardial dysfunction
Clinical signs of PDA
Secondary to hyper dynamic circulation
Bounding pulses
Wide pulse pressure >25 mmHg
Hyperactive precordium
Systolic murmur
Persistent tachycardia
Biochemical markers -
PDA
Atrial natriuretic peptide - ANP
Cardiac troponin T - TnT
Brain natriuretic peptide - BNP (sensitivity -

88%, specificity - 92%)
ECHO is the gold standard for diagnosis and

assessing ductal shunting
Closure of PDA
About 90% of PDA in >= 30 weeks of gestation
65% of PDA in < 30 weeks of gestation

PGE1
causes vasodilation (direct effect on vasculature

and smooth muscle of ductus arteriosus)
4 receptors
EP2 , EP4 mediate PGE1 action through cAMP
Dose : 0.025 - 0.2 mcg/kg/min
Ductus opens in about 30 minutes - 2 hours

Duct dependent lesions
1. Severe restriction of pulmonary 2. Severe restriction of systemic
circulation circulation
Pulmonary atresia / stenosis Severe aortic stenosis
Complete AV canal defect Coarctation of aorta
Tricuspid atresia Interrupted aortic arch
TOF with pulmonary atresia

Hypoplastic left ventricle
syndrome
Double outlet right ventricle
Hypoplastic aortic arch
3. Heart defects
Total anomalous pulmonary
Transposition of great arteries
venous return
Critical aortic stenosis :
classification
Gradien
Valve Velocit
t
cm2 y m/s
mmHg
Mild >1.5 2-2.9 <20
Modera
1-1.5 3-3.9 20-39
te
Severe <1 >4 >40

Critical aortic stenosis :
physical exam
Vitals : tachycardia, tachypnoea, decreased O2 saturation
General : cyanosis
Cardiovascular : hyperactive pericardium, poor pulses
Murmur +, but may often be absent due to poor cardiac

output
Pulmonary : edema
Extremities : distal pulses - poor / unappreciable

Critical aortic stenosis:
management
Medical :
Prostaglandin and Dopamine
Intubation and Transfer to ICU
Catheterization (first line )
Angiography to confirm diagnosis
Intervention to dilate valve with balloon.
Surgery (second line )
Valvotomy
Hypoplastc left heart
syndrome
HLHS
Left ventricle is underdeveloped and small
Mitral valves is not formed / very small
Aortic valve is not formed / very small
Ascending portion of aorta is underdeveloped /

too small
HLHS
Predominant in male
a/w Turner syndrome, Noonan syndrome, Holt-

Oram syndrome, trisomies
Systemic and coronary perfusion are decreased -

tissue hypoxia - metabolic acidosis - vascular
shock and death
HLHS
Respiratory distress with tachypnoea and mild

cyanosis, shock, severe cyanosis
Some children may survive if they develop

pulmonary hypertension
Definite Treatment is surgical correction with

Norwood procedure or transplant
Coarctation of Aorta
Coarctation of aorta
Neonates with severe condition
Pale skin
Irritability
Heavy sweating
Difficulty breathing
Older children :
Pulses and blood pressure are weaker in the lower limbs
Murmur
Coarctation of the aorta
Definite treatment : balloon angioplasty with or

without stunting
Interrupted aortic arch
Absence or discontinuation of part of the arch
Survival is >90%, in the paediatric population - Post

correction
Interrupted aortic arch -
signs
Signs and symptoms of poor perfusion
Congestive cardiac failure
weakness/fatigue
Poor feeding
Tachypnoea, tachycardia
Low saturation levels in the blood
Shock : pale, mottled and cool, decreased urine output and poor
pulses (more in the lower extremities)
Transposition of great
arteries
Signs and symptoms
Cyanosis / poor saturation
Comfortably tachypnoeic - babies are not

working hard to breathe
Signs of CCF
TGA - treatment
Rashkind Atrial balloon septo stomy
Jatene Arterial switch is performed -with re

allocation of the coronary arteries
Total anomalous
pulmonary venous return
(TAPVR)
Abnormal drainage of the pulmonary veins
Supra cardiac :
drain into the RA via SVC (pulomnary veins to vertical vein to

innominate vein to SVC
Cardiac :
drain into coronary sinus and then RA
Infra cardiac :
drain to RA via hepatic and IVC; blood passes through the hepatic system
before draining into the RA
TAPVR - signs
Severely cyanotic
Respiratory problems : tachypnoea, grunting,

retraction
Signs are more if there is obstruction

Pulmonary atresia with
intact Intraventricular
septum
Complete absence of communication b/w RV and pulmonary

arteries = no integrate blood flow through the RVOT
Uncommon - 1-3 % of total CHDs
a/w Rv to coronary artery fistulas, Ebstein malformation and

right ventricle dependent coronary circulation
PA/IVS
RV hypoplasia
mild (more than two-thirds of normal),
moderate (one-third to two-thirds of normal),
severe (less than one-third of normal), as well

as accurate assessment of the TV annular
diameter
Pulmonary stenosis
Valvular
Supravalular
Infundibular
Double outlet right
ventricle (DORV)
Both aorta and

pulmonary
artery arise
form RV
a/w VSD and

pulmonary
valve stenosis
Complete Av canal defect
Defect in the centre of the

heart - septum of the atria
and ventricles are defective
with abnormal formation of
the AV valves
Aka atrioventricular septal

defects
Management of systemic
duct dependent lesions
2 principles :
1. Keep PDA
2. After ductus is opened, flow balance between

the systemic and pulmonic circulation should be
monitored
Ventilatory setting should aim to increases

pulmonary vascular resistance, avoid respiratory
alkalosis and systemic saturation around 80%
Systemic vascular resistance can also be lowered
using vasodilators
If low cardiac output persists : adequacy of

prostaglandin infusion, intravascular volume
and anaemia should be assessed.
Morphine if the child remains tachycardic

Management of
pulmonary circulatory
lesions
Airway management is primary concern.
PGE1 infusion
References
Aiims protocol 2nd edition
Congenital heart disease in newborn requiring

early intervention : Sin Weon Yun (korean
journal of paediatrics)
cinncinatichildren.org
Thank you

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Duct dependent

Dr. Sinduja selvam

Briefly about various duct dependent lesions

Pulmonary artery to descending

Pre term infants : ductus tissue has

Lower intrinsic tone

Less smooth muscle fibre

Few sub endothelial cushions

Incidence in LBW - 15- 40%

Extremely premature babies <28 weeks - 50-65%

left - right shunt causes significant retrograde flow from

Systemic hypo perfusion - renal and mesenteric

Present throughout the cardiac cycle

Best seen in diastole during an ECHO

Low diastolic aortic pressure compromises the

Secondary to hyper dynamic circulation

Wide pulse pressure >25 mmHg

Atrial natriuretic peptide - ANP

Cardiac troponin T - TnT

Brain natriuretic peptide - BNP (sensitivity -

ECHO is the gold standard for diagnosis and

About 90% of PDA in >= 30 weeks of gestation

65% of PDA in < 30 weeks of gestation

causes vasodilation (direct effect on vasculature

EP2 , EP4 mediate PGE1 action through cAMP

Dose : 0.025 - 0.2 mcg/kg/min

Ductus opens in about 30 minutes - 2 hours

Pulmonary atresia / stenosis Severe aortic stenosis

Complete AV canal defect Coarctation of aorta

Tricuspid atresia Interrupted aortic arch

TOF with pulmonary atresia

Mild >1.5 2-2.9 <20

Severe <1 >4 >40

Cardiovascular : hyperactive pericardium, poor pulses

Murmur +, but may often be absent due to poor cardiac

Extremities : distal pulses - poor / unappreciable

Prostaglandin and Dopamine

Intubation and Transfer to ICU

Catheterization (first line )

Angiography to confirm diagnosis

Intervention to dilate valve with balloon.

Surgery (second line )

Mitral valves is not formed / very small

Aortic valve is not formed / very small

Ascending portion of aorta is underdeveloped /

a/w Turner syndrome, Noonan syndrome, Holt-

Systemic and coronary perfusion are decreased -

Respiratory distress with tachypnoea and mild

Some children may survive if they develop

Definite Treatment is surgical correction with

Neonates with severe condition

Pulses and blood pressure are weaker in the lower limbs

Definite treatment : balloon angioplasty with or

Absence or discontinuation of part of the arch

Survival is >90%, in the paediatric population - Post

Congestive cardiac failure

Low saturation levels in the blood

Cyanosis / poor saturation