Pediatric Cardiology😊

Umbilical Cord Contents

• Contains: 1 vein & 2 arteries (70%)

Constituents	Function
2 umbilical arteries	Carry deoxygenated blood from fetus.
1 umbilical vein	Carries oxygenated blood to fetus. (SpO2 -80%)
Wharton's jelly	Gelatinous substance
Allantois	Early urine formation → becomes urachus
Vitello-intestinal duct	between yolk sac and midgut

• Single umbilical artery
• 5-10/1000 live births
• A/w ↑↑ risk of a renal anomaly in the baby

Order of Decreasing Saturation of O2

• Umbilical vein: 80%

• Inferior vena cava (IVC): 70%

• LV: 65% > RV: 55-60%

• Superior vena cava

Where would you place the pulse oximeter to measure preductal oxygen saturation in an infant who was born 3 minutes ago?

1. Lungs become the source of O2 instead of the placenta

2. Pulmonary vascular resistance decreases:
1. Blood flow to the lung increases
2. produces systolic murmur

3. Systemic vascular resistance ↑↑↑

4. Foramen ovale closes

Additional Notes on Remnants

• Septum primum → Fossa ovalis.

• Septum secundum → Limbus fossa ovalis.

Closure of Structures

• Anatomical: Order V (Veno) Are (Art) Onnikkunnu (Ovale)

• Anatomical Closure time
• Vein → close fast → 1 week
• Artery → 3 week
• Heart → 3 months

• Pathways coming after Placenta closes functionally immediately after its removal
• Like Foramen ovale and Ductus venosus

• Foramen ovale closure Process:
• Blood goes to lungs via pulmonary vein.
• LA pressure ↑↑ and RA pressure ↓↓
• Fusion of septum primum and secundum.
• Closure of foramen ovale.

Applied Anatomy

• Patent ductus arteriosus (PDA):
• Causes acyanotic heart disease.

• Probe patency of foramen ovale:
• Gap between septum primum and septum secundum (seen in 20% cases).
• Not a functional defect.

• Umbilical arteries → 2
• Obliterate before birth.
• Remnant:
• Proximal part → Superior vesical artery.
• Distal part → Obliterated umbilical artery → Medial umbilical ligament
• NOTE
• Lateral umbilical ligament
• Remnant of Inferior Epigastric vessels

• Umbilical vein (left) → 1
• Obliterates after closure of umbilical artery.
• Remnant: Ligamentum teres hepatis.

• Spiral septum never formed

• Sitting duck appearance

 

• X-ray shows
• “figure of 8”
• “Snowman”
• “Cottage loaf”

 

• MC causes mortality in 1st week of life

• Pulmonary plethora is seen.

• All 4 chambers of the heart have equal oxygen saturation

• Mnemonic: Tap (TAPVAC) dance in christmas (snowman)

• Associated with maternal diabetes DM

• Parallel Circulation

• The cardiac silhouette shows
• “Egg on the side”
• Egg on string appearance.

• Important
• Septum dependent heart ds
• Keep PDA Open
• ↑ pulmonary blood flow
• Narrow Pedicle

• Not compatible with life unless a/w ASD + PDA
• Keep PDA open up to surgery
• IV Prostaglandin E1
• To make ASD
• Atrial Septoplasty
• Rashkind's Balloon Atrial Septostomy immediately

• Definitive surgery - Jatene's arterial switch operation by two weeks of age

• Mnemonic: Thenga → TGA → Emergency Rush (Rashkind) to surgery, Jiitne keliye (Jatene)

• POST DUCTAL M/c

Clinical presentation

• Severe coarctation:
• Heart failure in neonates with
• B/L feeble or impalpable femoral pulses

• Moderate:
• Weak femoral pulses and hypertension

• Milder disease:
• Intermittent claudication of lower limbs

• Focal narrowing of aorta.

• Radiofemoral delay
• i.e. LLBP < ULBP.

• Associated with Turner's syndrome.

Auscultation

• Murmur in the shoulder region due to the collaterals

Chest X-ray

• Reverse 3 sign:
• Ba swallow

• Roesler’s sign & figure of 3 appearance
• CXR
• Notching of the inferior margin of 3rd - 9th ribs usually seen >3 years age
• Due to collateral between anterior and posterior intercoastal arteries
• along the lower border become prominent collaterals as compensation

• Due to teratogenic effect of lithium.

• The X-rays show cardiac shadow typically covering the entire thorax.
• Box-shaped heart

• Atrialization of the right ventricle
• Right atrium enlargement.

• Pulmonary oligemia.

• Tricuspid valve
• Downward displacement
• Functionally abnormal

• ECG shows
• Himalayan 'P' waves

• Treatment:
• Cone repair of tricuspid valve.
• Return valve to original position.

Einstein (ebstein) carrying a box (Box shaped heart) in Himalaya (Himalayan waves), with a wolf (WPW) near him

• Most common congenital cyanotic heart disease in children

• The most common congenital cyanotic heart disease beyond infancy

• 4 Components of TOF
1. Large non-restrictive VSD
2. Pulmonary infundibular stenosis
3. Overriding of aorta
4. Right ventricular hypertrophy

• NOTE:
• ASD is a component of the
• trilogy and pentalogy of Fallot
• Pentalogy = TOF + ASD
• NOT tetralogy of Fallot

Pathophysiology and Clinical Features

• Pulmonary artery stenosis
• ↓↓ blood pumped to the lungs

Clinical Features

• Cyanosis (central) due to hypoxia: spo2=75-85%

• Clubbing

• Polycythemia

• Cyanotic spells
• Small → knee chest position
• Older → squatting position

• Heart failure is NOT SEEN unless
• Anemia
• Infective endocarditis
• Myocarditis
• Systemic hypertension

• Shunt is Right-to-Left shunt through VSD

• Reversal of shunt is NEVER SEEN in TOF
• NO Eisenmenger syndrome
• Reason
• TOF = congenital right-to-left shunt from the beginning.
• Due to Right Ventricular Outflow Tract Obstruction (RVOTO) → ↑ Right Ventricular pressure > Left ventricle

• Auscultatory Findings
• Ejection systolic murmur in the pulmonary area
• Single S2 (P2 is soft and inaudible)

Severity of TOF

• Lesser blood goes across pulmonary valve

• Murmur is shorter and softer

• More severe cyanosis

Investigations

• Chest X-ray:
• Boot-Shaped Heart (or) 'Cor En Sabot' Appearance
• D/t RVH
• + Pulmonary Oligemia

Treatment

• Moist O2 inhalation (to reduce hypoxia)

• Inj. Sodium Bicarbonate (to neutralize acidosis)

• Morphine

• Ketamine (increases systemic vascular resistance)

• Alpha agonists (phenylephrine)

• Beta — blockers (Propranolol): decreases pulmonary infundibular spasm

• Cyanotic spells
• Squatting or knee-chest position

• PRBCs transfusion

Definitive (corrective) Sx

• VSD closure +

• Repair of pulmonary stenosis

Shunt (palliative) Sx

ACUTE RHEUMATIC FEVER

• Roomatic fever
• Vijayan → Jai Vigyan
• Marginal ds (Erythema marginatum)
• Carry and Oomb in Middle of room → Carey coomb murmur
• Give mouth like a fish in her button hole (Fish mouth/Button hole stenosis)
• Show ass everywhere (Aschoff bodies → All layers → max in myocardium), Apply some butter (bread and butter → fibrinous pericarditis)
• Do Per Rectal (Prolonged PR)
• Buy benz (Benzathine Penicillin) & Get 12 lakh every 3 weeks () (for adults)

Etiology and Pathogenesis

• Etiology:
• Post streptococcal disorder
• Group A β hemolytic streptococci (Strains: 1, 3, 5, 6, 18)

• Age: School-going children (5-15 years).

• Incidence: m = F.

Pathogenesis:

• Pharyngitis
• 10 days to few weeks

• Type II hypersensitivity reaction (antibody mediated).
• Streptococcal M-protein cross reacts with 
• glycoprotein in heart and joints
• molecular mimicry

Affected Valves

• Most common valve affected: 
• mitral valve.

• Least common valve affected: 
• Pulmonary valve.

• Mitral valve (F > m) > Aortic valve (m > F).

Presentations

• Acute RHD: 
• mitral regurgitation.

• Chronic RHD: 
• mitral stenosis.

LAE/Mitral stenosis:

• Straightening of left heart border (Image 1).

• Splaying of carinal angle.

• Double density sign/ double right heart border sign (Image 2).

• Walking man sign:
• lateral chest x-ray (Image 3).
• Left atrium cause elevation of left bronchus

• Third Mogul sign (Image 4):
• Prominent third Mogul
• because of left atrium.

• Dysphagia
• Left atrium → Esophageal compression (Image 5)
• Trans esophageal echocardiography (TEE) is done.

Chamber Enlargement Signs

• RVH: Apex up.

• LVH: Apex down and out.

Revised Jones Criteria

Major Criteria (J.O.N.E.S)

1. Joint Arthritis: 
• Monoarthritis / Polyarthritis / Polyarthralgia
• Migratory polyarthritis:
• Involving large joints.
• Non-erosive.
• Excellent response to aspirin.
• Complete recovery (No residual joint damage)
• DONT include MONOARTHRALGIA

2. Pancarditis:
• Clinical
• Subclinical (ECHO Findings+)

3. Subcutaneous nodules.
• Painless.

4. Erythema marginatum.
• Most common & earliest manifestation.
• Raised rash, sparing the face.
• Transient

5. Sydenham's chorea.
• DOC: Haloperidol

Minor Criteria

1. Clinical:
• Fever.
• Monoarthralgia

2. Laboratory:
• ↑ ESR.
• ↑ CRP.

3. ECG
• ↑ PR interval.

• CAFE Pal

Essential Criteria

1. ↑ ASO titre.

2. Throat swab positive for streptococci:
• Risk of false positive/negative → not preferred.

3. History of preceding sore throat (< 50%).

Diagnosis

• All cases require Essential criteria.

• First Episode:
• 2 major criteria OR
• 1 major + 2 minor criteria

• Recurrence:
• 2 major criteria OR
• 1 major + 2 minor criteria OR
• 3 minor criteria

• Mnemonic: Jones → JO → start from 2 major criteria

Pancarditis

• Early finding (within 2 weeks).

• Most serious: 
• Permanent damage of valves can occur.

• Most common feature of ARF 

Pericardium:

Fibrinous pericarditis

• Pericarditis: chest pain + frictional rub.

• Deposition of fibrinous exudate between the layers of pericardium.

• Appearance: Bread and butter appearance.

Myocardium: 

• Aschoff bodies.
• Pathognomonic of RHD.
• Seen in all layers.
• Maximum in myocardium.
• Components:
1. Anitschkow
• AKA caterpillar cells.
• Macrophages with slender wavy ribbon-like nuclei.
2. Fibrinoid necrosis.
3. Inflammatory cells.

Types of valvulitis:

Mitral valvulitis:

• Carey coombs murmur (delayed diastolic murmur).

• CC murmur → DD murmur

Mitral regurgitation:

• In acute RHD 
• Soft S₁
• Pan systolic murmur.

• Hemodynamic overload → LVF (morbidity/mortality in ARF).

Mitral Stenosis

• In chronic RHD 
• fibrosis & calcifications of MV.
• Loud S₁

• Fish mouth/Button hole stenosis 

Subendocardial Jets / McCallum Plaques

• Thickening of left atrial wall due to mitral regurgitation.

Mitral Valve Thickening / Commissural Fusion

• Shortening & thickening of chorda tendinae.

Vegetations

• Size: 1-4 mm.

• Appearance: Small, warty, verrucous, sterile lesions.

• Location: Along the lines of closure of valve leaflets.

Treatment & Prophylaxis

Treatment:

• Bed rest for 2 weeks.

Penicillin:

1. Benzathine Penicillin G:
• > 30 kg: 
• 1.2 million/ 12 lakh IU every 3 weeks.
• < 30 kg: 
• 6 lakh IU every 2 weeks.

2. Oral Penicillin V: 
• 250 mg BD.

3. If allergic to Penicillin:
• Erythromycin 250 mg BD.

Anti-inflammatory Medications

• For 12 weeks
1. Steroids:
• Preferred (especially in cardiac problems).
• Oral Prednisolone: (12 weeks - 3/9)
• 2 mg/kg/day (max 60 mg/day) x 3 weeks 
• → Taper over 9 weeks.
2. Aspirin: (12 weeks - 10/2)
• 90-120 mg/kg/day in 4 divided doses x 10 weeks 
• → Taper over next 2 weeks.

Duration of Prophylaxis