Cardio - 1 - Presentations/left-to-right shunts

Question 1

What are the presenting features of cardiac failure?

Answer 1

Breathlessness
Sweating
Poor feeding
Recurrent chest infections
Wheezing

Faltering growth
Tachypnoea without recession!
Tachycardia
Heart murmur/Gallop rhythm
Hepatomegaly
Cool peripheries

Question 2

What are the features of innocent murmur?

Answer 2

5 S (InnoSent)
Soft
Systolic only
aSymptomatic
left Sternal edge

Also, there is:
no parasternal thrills
no radiation

More common in <6 months

Question 3

When are innocent murmurs more common in children?

Answer 3

During febrile illness or anaemia (increased cardiac output)

Question 4

List the epidemiology of acyanotic heart diseases (VSD, ASD, AS, PS)

Answer 4

ASD:
Secundum ASD (80%)
Partial atrioventricular septal defect (pAVSD)

Question 5

What are the features of ASD?

Answer 5

Primum and secundum are similar, except that secundum involves centre of septum with foramen ovale and a regurgitant left AV valve.

Commonly no symptoms
Recurrent chest infections
Arrhythmias (4th decade onwards)

Question 6

What are the physical signs of ASD?

Answer 6

Increased flow through pulmonary valve leads to Ejection systolic murmur (upper left sternal edge)

Apical pansystolic murmur in pAVSD from atrioventricular valve regurgitation

Question 7

What are the presenting features of ASD on CXR?

Answer 7

Enlarged heart
Enlarged pulmonary arteries
Increased pulmonary vascular markings

Question 8

What are the features of ASD on ECG?

Answer 8

secundum ASD:
Right axis deviation (due to right ventricular enlargement)
Right BBB

pAVSD:
Superior axis (negative deflection in lead AVF) because middle part of heart, where the AV node is, is displaced.

Question 9

What is the mainstay of ASD diagnosis?

Answer 9

Echocardiogram

Delineates anatomy

Question 10

How is ASD managed in children?

Answer 10

If significant (ie right ventricular enlargement)

Secundum ASD:
Cardiac catheterisation, inserting an occlusion device. (plug or patch)

partial AVSD:
surgical correction

At ca. 3-5 years of age to prevent RHF and arrhythmias in later life

Question 11

What is the prevalence of different types of left-to-right shunts (breathless) in congenital heart disease?

Answer 11

VSD - 30%
Persistent arterial duct - 12%
Atrial septal defect - 7%

Question 12

What are the causes for cardiac failure in neonates?

Answer 12

there is obstructed left ventricle and thus (duct-dependent) systemic circulation. Right-to-left flow via arterial duct

Hypoplastic left heart syndrome.
Critical aortic valve stenosis.
Severe coarctation of the aorta.
Interruption of the aortic arch.

Question 13

What are the causes of heart failure in infants (>1week old)

Answer 13

VSD
ASD
large PDA

Question 14

What are the causes of heart failure in older children and adolescents?

Answer 14

Eisenmenger syndrome (RHF)
Rheumatic heart disease
Cardiomyopathy

Question 15

What are the symptoms, auscultation signs and ECG results of small VSD?
Management?

Answer 15

<3mm diameter.
Asymptomatic.
ECG normal.
CXR normal.

Loud pansystolic murmur at lower sternal edge!
Quiet pulmonary second sound (P2).

Lesion will close spontaneously. Good dental hygiene to prevent bacterial endocarditis.

Question 16

What are the symptoms and physical signs of large VSDs?

Answer 16

Same size or bigger than aortic valve.
Heart failure with breathlessness and failure to thrive after 1 week old.
Recurrent chest infections.

Physical
Tachypnoea, tachycardia and hepatomegaly.
Soft pansystolic murmur or no murmur (implying large defect)!
Apical mid-diastolic murmur (increased flow across mitral valve).
Loud pulmonary 2nd sound (P2) from raised pulmonary arterial pressure

Question 17

Which congenital heart disease present without cyanosis?

Answer 17

VSD (32%)
PDA (12%)
Pulmonary Stenosis (80%)
ASD (6%)
Coarctation of the aorta (6%)
Aortic stenosis (5%)

Question 18

Which congenital heart diseases present with cyanosis?

Answer 18

Tetralogy of Fallot

Transposition of greater arteries

Question 19

Which CHD is associated with Down’s syndrome?

Answer 19

Atrioventricular septal defect (30%)

VSD

Question 20

Which CHD are associated with Turner syndrome? (45XO)

Answer 20

Aortic valve stenosis
Coarctation of the aorta
15%

Question 21

What are the ECG, CXR, and echo findigns of large VSDs?

Answer 21

ECG:
Biventricular hypertrophy by 2 months.

CXR:
Cardiomegaly
Increased pulmonary arteries.
Increased pulmonary vascular markings.
Pulmonary oedema.

Echo:
Demonstrates anatomy, haemodynamic effects and pulmonary HTN

Question 22

What are the medical and surgical treatment options of small and large VSD?

Answer 22

None in small VSD.

Diuretics, captopril and calories in large. Plus surgery at 3-6 months (to prevent lung damage from pulmonary hypertension and manage heart failure).

Question 23

What is the ductus arteriosus and what happens during development and at birth?

Answer 23

Connects pulmonary artery to aorta, just after it branches off to supply upper extremities and brain. As a fetus, oxygenated blood comes from the placenta and flows from RA though the foramen ovale directly into the LA. Any blood that flows into RV goes to aorta instead of the lungs, since it is not yet matured. Kept open by PGE2.

Ductus arteriosus closes at birth due to change in lung which releases bradykinin, higher blood oxygenation, reduced PGE2.

In premature babies, there is physiological reason for failure to close.

Question 24

What are the presenting features of patent ductus arteriosus?

Answer 24

Failed to close 1 month after birth

Continuous murmur
Collapsing pulse
Sick neonate

Can present with Eisenmenger syndrome (pulmonary HTN leading to Right-to-Left shunt and cyanosis), but only lower extremities because of the position of the duct.
Quiet rare, mostly if the duct is very big.

Question 25

What is the management of PDA?

Answer 25

Closure recommended due to risk of bacterial endocarditis and pulmonary vascular disease

Can be closed by Indomethacin (NSAID which inhibits prostaglandin E2)

Coil or occlusion device via cardiac catheter at ca. 1 year of age.
Occasionally, surgical ligation

Question 26

Which CHD are associated with Noonan syndrome? (PTPN11 mutation and others)

Answer 26

Hypertrophic cardiomyopathy
ASD
Pulmonary valve stenosis

Question 27

Which CHD are associated with Marfan’s syndrome

Answer 27

Aortic enlargement
Aortic tear/rupture
Mitral valve prolapse/leak

Later in life: aortic aneurysm

Question 28

What is the anatomy of atrioventricular septal defect?

Answer 28

Ranges from pAVSD (involving primum atrial septum) to complete AVSD (presence of common atrio-ventricular valve)

Question 29

What are the presenting features of cAVSD?

Answer 29

Cyanosis at birth
OR
Breathless at 2-3 weeks of life. (pulmonary oedema and heart failure)

No murmur.
Usually detected on routine echo in babies with Down’s syndrome

Question 30

What are the ECG and CXR findings in AVSD?

Answer 30

complete:
Always superior axis on ECG

CXR:
Signs of heart failure and pulmonary oedema (cardiomyopathy, increased lung vasculature, fluid)