CHD

Patent Ductus Arteriosus

Normally, the ductus arteriosus closes before the 4th week of life
Failure of closure of the ductus is more common in females




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PDA: Pathophysiology

Pressure gradient between the aorta & PA occurs throughout the cardiac cycle
This leads to continuous flow of blood from the aorta to PA


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Adult life

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Fetal Life

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Adult with PDA

Adult
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PDA: Pathophysiology

The shunt involves PA, LA, LV, & aorta
The RV and RA are not involved in the shunt



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PDA: Pathophysiology

The magnitude of the shunt depends on the size of the communication

Usually there is LV volume overload and increased flow through the mitral valve

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PDA: Clinical Presentation

Exertional dyspnea
Recurrent chest infections
Congestive heart failure
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PDA: Physical Findings

Large volume pulse
Left ventricular dilatation (displaced apex beat)
Systolic & diastolic thrills in the pulmonary area
Palpable systolic expansion of the pulmonary artery

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PDA: Physical Findings

Left parasternal heave (RVH due to PHT)
Auscultation: continuous murmur in the pulmonary area (machinery murmur)

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PDA: Clinical Presentation

Eisenmenger syndrome with differential cyanosis:
Cyanosis of the toes but not the fingers
The right to left shunt bypasses the cerebral and upper limb vessels & flows directly into the aorta




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Moccetti F et al. Eur Heart J 2014;35:1410

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com

PDA: Investigations: CXR

LV & LA dilatation
Prominent main pulmonary artery
Plethoric lungs (increased pulmonary vascular markings: arterial and venous)
Prominent aorta
In older patients: the ductus may be calcified


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PDA: Investigations

ECG: LVH
tall R waves in chest leads V5 & V6
Deep S waves in chest leads V1 & V2

Echocardiography & Doppler: Demonstrates the site of the ductus and the continuous flow by Doppler

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PDA: Management

Closure of the ductus whatever the size
Usually closed through catheterization
Large ducts are closed surgically
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Valvular Pulmonary Stenosis

Increased resistance for RV ejection
Pressure gradient between RV & PA
This leads to right ventricular hypertrophy
The forceful jet into the PA leads to dilatation of the main pulmonary artery
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Valvular PS: Clinical Picture

Raised JVP
RVH: left parasternal heave
Systolic thrill in the pulmonary area
Faint pulmonary second sound
Systolic ejection murmur at the pulmonary area
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Valvular PS: Investigations

ECG:
RVH: tall R waves in V1 & V2


CXR:
Oligemic lungs: reduced pulmonary blood flow
Prominent main pulmonary artery (post-stenotic dilatation)
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ECG in PS

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Valvular PS: Investigations

Echocardiography:
Reduced pulmonary valve motion
Doppler: estimation of the gradient across the pulmonary valve


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Valvular PS: Treatment

Transcatheter dilatation of the stenotic pulmonary valve using a balloon inflated at the pulmonary valve
If this fails: surgery
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Coarctation of the Aorta

Narrowing of the aorta just distal to the origin of the left SCA


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Coarctation of the Aorta

With age, collaterals form around the narrowing to bridge the proximal & distal parts




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Coarctation of the Aorta

Usually associated with bicuspid aortic valve
More common in males



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Coarctation of the Aorta: Clinical

Co A is an important cause of congestive heart failure in neonates
Often undetected during physical examination


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Coarctation of the Aorta: Clinical
The hallmark is radio-femoral delay of the pulse


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Hypertension may cause headache

Leg cramps: reduced circulation in the lower limbs




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Coarctation of the Aorta: Clinical

BP is raised in the arms, normal in the legs
Auscultation:
systolic murmur over the coarctation (heard over the back)
Systolic ejection click (dilated aorta, bicuspid aortic valve)
Continuous murmur from flow into collaterals: heard best over the spine


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Coarctation of the Aorta: Investigations

CXR:
Rib notching: from enlarged collaterals
Prestenotic and post-stenotic dilatation form the “3 sign” of the descending aorta
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Coarctation of the Aorta: Investigations

Echocardiography:
Shows the site of stenosis & pressure gradient across it

CT & MRI: can show the entire extent of the aorta

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Coarctation of the Aorta: treatment

Early relief of obstruction: by surgery or catheter-based intervention
BP returns to normal
If intervention is delayed, HT may persist


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Tetralogy of Fallot (TOF)

The four components of TOF are:
Ventricular septal defect
Over-riding aorta
Pulmonary stenosis, RV outflow tract obstruction
Right ventricular hypertrophy
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TOF: Clinical Picture

Cyanosis & clubbing
Squatting: sudden assumption of the sitting position in the upright patient:
This increases the LV afterload and reduces the magnitude of right -to-left shunt
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TOF: Clinical Picture

Cyanotic spells: sudden lethargy and weakness with increased depth of cyanosis:
Fever
Crying
Exertion
Cause: Reduced pulmonary blood flow & increased shunting of blood to the left side

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TOF: Physical findings

Clubbing
Cyanosis
Prominent RV impulse
No thrill (≠ valvular PS): reduced pulmonary blood flow
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TOF: Physical findings
Auscultation:
single second heart sound
Faint systolic ejection murmur at the pulmonary area

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TOF: investigation

ECG:
Right ventricular hypertrophy

Chest X ray:

Right ventricular enlargement
Pulmonary bay (underdeveloped pulmonary artery)
The combination of these findings gives the “boot-shaped heart”
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TOF: Treatment

Surgical correction
If the PA is very small & underdeveloped: the aorta is anastomosed to the pulmonary arteries to enhance their growth before total correction ( Blalock-Taussig shunt)
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