Acyanotic Congenital Heart Diseases & Coarctation of Aorta

PAEDIATRIC CARDIOLOGY

Acyanotic Congenital Heart Diseases

with Focus on Coarctation of Aorta

Presented by

Dr. Ajinkya Kale

MBBS, MD Paediatrics

Neonatal Fellow (UK)

Lactation Consultant (BPNI, AIIMS Nagpur)

2026

What is Cyanosis?

Recognizing clinical signs & presentation

Acyanotic CHD — Classification & Examples

Left-to-right shunts & major subtypes

Coarctation of Aorta — Deep Dive

Pathophysiology, hemodynamics & diagnosis

Duct-Dependent Lesions

Prostaglandin use & critical stabilization

Emergency Management

Acute care & initial stabilization protocols

Definitive Surgical Management

Repair timing & operative approaches

India Statistics & Burden

Epidemiology & regional healthcare challenges

Key Pearls, Viva Q&A & Closing

Important takeaways & interactive review

FUNDAMENTALS

What is Cyanosis?

Cyanosis is the bluish-purple discolouration of the skin and mucous membranes caused by increased concentration of deoxygenated haemoglobin (>5 g/dL) in capillary blood.

CENTRAL CYANOSIS

Affects tongue, lips, mucous membranes. Indicates systemic desaturation. SpO2 typically <85%.

PERIPHERAL CYANOSIS

Affects extremities only. Can be normal in newborns (acrocyanosis).

Cyanosis is NOT always pathological in newborns — acrocyanosis in first 24-48 hrs is normal!

Acyanotic Congenital Heart Diseases

Left-to-Right Shunts & Obstructive Lesions

Left-to-Right Shunts

VSD (Ventricular Septal Defect)

Most common CHD, ~32%

ASD (Atrial Septal Defect)

Ostium secundum most common

PDA (Patent Ductus Arteriosus)

Common in premature infants

AVSD (Atrioventricular Septal Defect)

Associated with Down syndrome

PAPVR

Partial Anomalous Pulmonary Venous Return

Obstructive Lesions

Today's Focus

Coarctation of Aorta (CoA)

Narrowing of the descending aorta

Pulmonary Stenosis (PS)

Obstruction of right ventricular outflow tract

Aortic Stenosis (AS)

Obstruction of left ventricular outflow tract

Hypoplastic Left Heart Syndrome (HLHS)

Severe underdevelopment of left heart structures

📌 Acyanotic = No right-to-left shunt. May become cyanotic if Eisenmenger develops.

COARCTATION OF AORTA

Narrowing That Can Kill

Coarctation of the Aorta (CoA) is a discrete narrowing of the aortic lumen, most commonly at or just distal to the ductus arteriosus (juxtaductal), causing obstruction to blood flow.

Juxtaductal (near ligamentum arteriosum) — just distal to origin of left subclavian artery

Duct-Dependent Lesions

When the ductus closes — the baby crashes

What is the Ductus?

The ductus arteriosus connects pulmonary artery to descending aorta

Normally closes within 24-72 hours after birth

Mediated by rise in O₂, fall in prostaglandins

Duct-Dependent Systemic Flow

Lesions where systemic flow DEPENDS on PDA:

Coarctation of Aorta (critical)

Interrupted Aortic Arch

Hypoplastic Left Heart Syndrome (HLHS)

Critical Aortic Stenosis

These babies present with SHOCK when PDA closes

Duct-Dependent Pulmonary Flow

Lesions where pulmonary flow DEPENDS on PDA:

Pulmonary Atresia

Tricuspid Atresia

Critical Pulmonary Stenosis

Tetralogy of Fallot (severe)

These babies present with SEVERE CYANOSIS when PDA closes

GOLDEN RULE: Any neonate presenting in shock or severe cyanosis in first week of life = THINK DUCT-DEPENDENT LESION → Start PGE1 IMMEDIATELY

Definitive Surgical Management

Coarctation of Aorta — Timing & Techniques

Surgical Options

RESECTION & END-TO-END ANASTOMOSIS

<b>Gold standard in neonates and infants.</b> Resect the coarcted segment + extended anastomosis. Low recurrence rate. <b>Approach:</b> Left posterolateral thoracotomy.

SUBCLAVIAN FLAP AORTOPLASTY

Uses left subclavian artery as a flap to widen the coarctation. Useful in very small neonates. <b>Disadvantage:</b> May compromise left arm blood flow.

PATCH AORTOPLASTY

Dacron/Gore-Tex patch to widen segment. Higher risk of aneurysm formation — less preferred.

BALLOON ANGIOPLASTY ± STENTING

Preferred in older infants, children, adolescents and <b>RECOARCTATION</b>. Stents in children >25kg. <b>Risks:</b> Aneurysm, dissection.

Timing of Surgery

<b>Symptomatic neonate:</b> URGENT surgery (within 24-48h of stabilization)

<b>Asymptomatic infant:</b> Elective at 3-6 months

<b>Older child/adolescent:</b> At diagnosis

Complications to Watch

Recoarctation (10-15% in neonates)

Paradoxical hypertension post-op

Spinal cord ischemia (rare)

Recurrent laryngeal nerve injury

Chylothorax

Long-term Follow-up

Monitor BP in all 4 limbs annually

Echocardiography every 1-2 years

MRI aorta in adulthood

Lifelong cardiology follow-up

CHD in India — The Numbers

A Crisis We Can No Longer Ignore

240,000

New babies born with CHD every year in India

1 in 100

Newborns affected by congenital heart disease

25-30%

Of affected children die before age 1 without intervention

9.8%

Of ALL infant deaths in India attributed to Critical CHD (2018-2021)

<20%

Of children with CHD actually receive any form of cardiac care

28%

of all birth defect-related deaths in India are due to CHD — making it the #1 killer among birth defects

The Problem

Only ~50 paediatric cardiac surgery centres exist in a country of 1.4 billion people

The Hope

With timely PGE1 + referral, survival rates can exceed 90% post-surgery

PAEDIATRIC CARDIOLOGY

Saving a Life — It's Simpler Than You Think

The Chain of Survival in Duct-Dependent CHD

You — the first doctor who examines this baby — are the most important link in this chain.

WITHOUT RECOGNITION:

Baby dies of refractory shock within hours to days

WITH PGE1 + TIMELY SURGERY:

Child grows up, plays sports, lives a full life

RECOGNISE

Weak femoral pulses, shock, pre/post-ductal SpO₂ difference, sick neonate day 2-5

START PGE1

0.05 mcg/kg/min IV — Within minutes, perfusion is restored

CALL & TRANSFER

Contact nearest cardiac centre. Stable baby on PGE1 travels safely

SURGERY

Resection & end-to-end anastomosis. Median operating time: 2-3 hours

SURVIVOR

90%+ survival post-surgery. Normal life expectancy with follow-up

Key Clinical Pearls

Things That Will Save Lives & Ace Your Exams

💎

Always Check ALL FOUR LIMB BPs

Upper limb hypertension + lower limb hypotension = CoA until proven otherwise

💡

Pre vs Post-ductal SpO₂

Difference of >3% between right hand (pre-ductal) and foot (post-ductal) = significant right-to-left shunting at PDA level

💎

Don't Give High O₂ in Duct-Dependent Systemic Lesions

O₂ is a potent ductal constrictor. Can worsen CoA/HLHS by closing the PDA

💡

Radio-femoral Delay

Classic clinical sign of CoA. Feel radial and femoral pulse simultaneously — delay in femoral = CoA

💎

Rib Notching — Only in Older Children

Appears after age 5-6 years due to collateral vessels eroding rib undersurfaces. NOT seen in neonates/infants

💡

HLHS = Worst Duct-Dependent Lesion

Single ventricle physiology. Requires Norwood procedure in 3 staged surgeries. High mortality.

💎

VSD — Murmur Appears After Week 1

As pulmonary vascular resistance falls, L→R shunt increases and murmur becomes audible. Not always heard at birth!

💡

Eisenmenger Syndrome — Point of No Return

Uncorrected L→R shunt → pulmonary hypertension → reversal to R→L → inoperable. Early surgery prevents this.

Exam Preparation

High-Yield Points for MD/DNB Paediatrics

Must-Know Facts

Important Associations

ECG & CXR Findings

🎯 In exams: Always correlate ANATOMY → PHYSIOLOGY → CLINICAL FEATURES → INVESTIGATIONS → MANAGEMENT

Viva Questions — Be Ready!

Frequently Asked in MD/DNB/MRCPCH Viva Examinations

Q1: What is the most common site of coarctation?

Juxtaductal — just distal to origin of left subclavian artery, at the level of ductus arteriosus

Q2: How will you clinically diagnose CoA at bedside?

Radio-femoral delay + upper limb hypertension + absent/weak femoral pulses + 4-limb BP difference >10 mmHg

Q3: What is the emergency treatment for a neonate in shock with suspected CoA?

Prostaglandin E1 (0.05-0.1 mcg/kg/min IV) to reopen PDA + supportive care + urgent cardiac referral

Q4: Why should you NOT give high-flow oxygen in a neonate with duct-dependent CoA?

Oxygen causes ductal constriction, closing the PDA which is the only source of lower body blood flow — this will worsen shock

Q5: What CHD is associated with Turner syndrome?

Coarctation of Aorta (most common), also Bicuspid Aortic Valve

Q6: What is Eisenmenger syndrome and when does it occur?

Reversal of left-to-right shunt to right-to-left due to severe pulmonary hypertension from uncorrected large L→R shunt. Lesion becomes inoperable.

Q7: What is the surgical treatment of choice for CoA in a neonate?

Resection with extended end-to-end anastomosis via left posterolateral thoracotomy

Q8: What is the significance of pre vs post-ductal SpO2 difference?

Difference >3% suggests right-to-left shunt at PDA level — seen in duct-dependent systemic lesions

CLOSING STATEMENT

"Every baby born with a heart defect deserves a fighting chance."

Congenital heart disease is not a death sentence — it is a challenge we are fully equipped to meet. As paediatricians and neonatologists, we are often the FIRST and most CRITICAL link in the chain of survival. Knowing when to suspect, when to act, and when to refer is all it takes to give a child their entire lifetime.

✅ Check femoral pulses in EVERY newborn

✅ Know your duct-dependent lesions

✅ Keep PGE1 stocked and ready

✅ Refer early — Don't wait for deterioration

Dr. Ajinkya Kale | MBBS, MD Paediatrics | Neonatal Fellow (UK) | Lactation Consultant (BPNI, AIIMS Nagpur)

Thank You | Questions Welcome 🙏