Cardiac

Question 1

dDx breathless child

Answer 1

Left to right shunt
ASD
VSD
PDA

Question 2

Features ASD

Answer 2

Secundum (most common, central defect) and partial AVSD- present similarly with different anatomy
Common asymptomatic

Question 3

ASD secundum presentation

Answer 3

May present as recurrent chest infection/wheeze, arrythmias in 4th decade

Question 4

Ix ASD secundum

Answer 4

Ejection systolic murmur best heard at upper left sternal edge
Fixed and widely split second heart sound
Xray showed cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings
ECG shows partial right BBB and right axis deviation

Question 5

Mx ASD secundum

Answer 5

Treatment required if large enough to cause right ventricle dilation
Cardiac catheterization and insertion of an occlusion device

Question 6

Ix partial AVSD

Answer 6

Asymptomatic pansystolic murmur from AV valve regurgitation

ECG shows QRS negative in AvF

Question 7

Mx partial AVSD

Answer 7

Surgical correction between 3-5y

Question 8

Features small VSD

Answer 8

<3mm and asymptomatic
Normal X Ray and ECG
Loud pansystolic murmur at left lower sternal edge
Usually self resolve
Require good dental hygiene prevent endocarditis

Question 9

Features large VSD

Answer 9

More prominent presentation 
Heart failure with breathlessness and faltering growth after 1 week old
Recurrent chest infection
Soft pansystolic murmur
Atypical mid-diastolic murmur
Loud pulmonary second sound

Question 10

Ix large VSD

Answer 10

Chest Xray shows cardiomegaly, enlarged pulmonary artery, increased pulmonary vascular markings and pulmonary edema
ECG shows ventricular hypertrophy by 2 months of age

Question 11

Mx large VSD

Answer 11

Drug therapy for heart failure with diuretics combine with captopril
Additional calorie input required
Surgery performed at 3-6 months to prevent permanent lung damage from pulmonary hypertension and high blood flow

Question 12

Cx VSD uncorrected

Answer 12

Eisenmenger syndrome

Question 13

Features Eisenmenger syndrome

Answer 13

Long term complications of untreated left right shunting
Pulmonary arteries become thick walled with high resistance
Features develop after 1 year of uncorrected shunt defect
Progressive presentation and death by right sided failure
Palliation or full heart lung transplantation (rare)

Question 14

Features PDA

Answer 14

PDA failure to close within first months due to a defect in the contractile mechanism
Resorts with continuous machinery murmur under the left clavicle continuing into diastole
Pulse pressure decreases with a collapsing bounding pulse
Large ducts exhibit heart failure and pulmonary hypotension

Question 15

Ix PDA

Answer 15

Normal chest Xray and ECG uless duct is large

In large duct ECG and X Ray exhibit signs of VSD

Question 16

Mx PDA

Answer 16

Closure to prevent endocarditis and pulmonary vascular disease
Closure with coil or occlusion device via a cardiac catheter 1 year of age
Prostaglandin inhibitors may be effective in term infants .e.g. Indomethacin
Preterm infants less responsive to prostaglandin inhibitors

Question 17

dDx blue baby

Answer 17

Right to left shunt
Tetralogy of fallot
Transposition of the great arteries
Eisenmenger syndrome

Question 18

Four features of tetralogy of fallot

Answer 18

Large VSD
Overriding of aorta with respect to ventricular septum
Subpulmonary stenosis causing right ventricular outflow obstruction
Right ventricular hypertrophy

Question 19

Exam and Ix in tetralogy of fallot

Answer 19

Loud ejection systolic murmur
Clubbing of fingers and toes develops in older childhood
Chest Xray features: relatively small heart, uptilted apex, pulmonary artery bay, concavity of the left heart border, decreased pulmonary vascular markings
ECG develops signs of right ventricular hypertrophy

Question 20

Mx tetralogy

Answer 20

Definitive correction at 6 months of age: closure of VSD, relieve RV outflow tract obstruction
Shunt via artificial tube between the subclavian and pulmonary OR balloon dilation of RV outflow obstruction
Hypercapnic episodes lasting >15 minutes related with analgesia, V propranolol, IV fluids, bicarbonate, artificial ventilation

Question 21

Features transposition of great arteries

Answer 21

Aorta connect to the RV and pulmonary artery connected to the LV
Without mixing, this is fatal
Cyanosis is predominant feature; profound and life threatening
Presentation typically follows ductal closure
Second heartland is often loud and single- no murmur

Question 22

Xray features transposition

Answer 22

egg on side appearance

increased vascular markings

Question 23

Mx transposition

Answer 23

Maintain patency of ductus arteriosus with prostaglandin infusion
Balloon atrial septostomy via the atrial septum
All patients require surgery in the neonatal period to switch vessels

Question 24

dDx blue and breathless

Answer 24

Common mixing
Complete AVSD
Tricuspid atresia

Question 25

Features complete AVSD

Answer 25

Associated with down's syndrome Defect in the middle of the heart producing a small fine leaflet valve stretch across whole AV junction Diagnosis by antenatal US Cyanosis or heart failure at 2 weeks to 3 months No associated murmur

Question 26

Mx complete AVSD

Answer 26

Routine echo in all patients with down syndrome | Initially medical treatment with full repair at 3-6 months

Question 27

Features tricuspid atresia

Answer 27

Only Left ventricle is effective: right ventricle is small and non-functional Cyanosis in newborn which is duct dependant

Question 28

Mx tricuspid atresia

Answer 28

Early palliation to maintain pulmonary blood supply low pressure: Blalock-Taussig shunt (subclavian to pulmonary artery) Corrective surgery rarely possible due to single ventricle Further palliative surgery: SVC to pulmonary at 6 months; IVC to pulmonary at 3-5 years

Question 29

Features coarctation of the aorta

Answer 29

Collapsed child with shock Outflow obstruction Atrial duct tissue overrides the aorta at the point of insertion of duct Present with collapse on day 2 when duct closes Very sick baby with heart failure Cardiomegaly Severe metabolic acidosis

Question 30

Features hypoplastic left heart

Answer 30

Underdevelopment of the entire left side of the heart: Mitral valve small and atretic LV diminished Small descending aorta Antenatally detected No flow in L side causing profound acidosis and cardiovascular collapse Weakness or absence of peripheral pulses

Question 31

Mx hypoplastic left heart syndrome

Answer 31

Norwood procedure

Question 32

Features aortic stenosis

Answer 32

Aortic valve leaflets are partially fused giving restrictive outflow Associated with mitral valve stenosis and coarctation of the aorta Must have asymptomatic murmur: ejection systolic maximal in upper right sternal edge radiating to the neck Reduced exercise tolerance, chest pain on exertion and syncope Small volume, slow rising pulse, carotid thrill, apical ejection click, delayed soft aortic second sound

Question 33

Ix Aortic stenosis

Answer 33

Prominent LV with post stenotic dilation of ascending aorta visible on chest X Ray LV hypertrophy on ECG

Question 34

Mx aortic stenosis

Answer 34

Regular clinic and echo assessment | Balloon valvotomy with symptoms on exercise, pressure gradient >64mmHg

Question 35

Features pulmonary stenosis

Answer 35

Pulmonary valve leaflets are partially fused Mostly asymptomatic Ejection systolic murmur on left sternal edge Ejection click on upper left sternal edge Thrill and heave present in severe disease

Question 36

Ix pulmonary stenosis

Answer 36

Post-stenotic dilation of pulmonary artery | ECG shows evidence of RV hypertrophy

Question 37

Mx pulmonary stenosis

Answer 37

Transcatheter dilation once pressure gradient exceeds 64mmHg

Question 38

Features supraventricular tachycardia

Answer 38

Rapid heart rate above 250-300 beats/min Poor cardiac output Pulmonary edema and heart failure Hydrops fetalis and IUD if in utero ECG shows narrow complex tachycardia with P wave following QRS Wolff-Parkinson white accessory pathway associated with a delta wave

Question 39

Mx supraventricular tachycardia

Answer 39

Restore sinus rhythm with PPV, vagal maneuvers, adenosine, electrical cardioversion

Question 40

Features congenital complete heart block

Answer 40

Rare condition with anti-Rho and anti-La antibodies in maternal serum Mother with connective tissue disorders: including latent and active Antibody prevents normal electrical conduction development Heart develops with atrophy and fibrosis of AVN Can cause death in utero and neonatal heart failure May be asymptomatic with some episodes of syncope or presyncope

Question 41

Mx congenital complete heart block

Answer 41

Endocardial pacemaker in symptomatic children