CVS

Question 1

most common cause of cyanotic congenital heart disease?

Answer 1

tetralogy of Fallot:
overriding aorta with respect to ventricular septum
VSD
subpulmonary stenosis causing RV outflow tract obstruction
RV hypertrophy

Question 2

heart murmur detected in 1st 2 mnths of life that may be seen with tetralogy of Fallot?

Answer 2

loud harsh ejection systolic murmur at left sternal edge from day 1 of life:
due to RV outflow tract obstruction
as this increases, murmur will shorten and cyanosis will increase.

Question 3

CXR findings in tetralogy of Fallot?

Answer 3

relatively small heart
pulmonary artery ‘bay’=concavity on L heart border where convex shaped main PA and RV outflow tract would normally be profiled
decreased pulm vascular markings (oligaemia) due to reduced pulmonary blood flow.

Question 4

ECG findings in tetralogy of Fallot?

Answer 4

RV hypertropy-tall R waves V1 and V2, when child is older, and R axis deviation

Question 5

how is tetralogy of Fallot managed?

Answer 5

initial management=medical-morphine, beta blockers, phenylephrine-increase systemic vasc resistance to force more blood to lungs
if emergency tment required, can do shunt from a systemic artery e.g. SCA to PA to increase pulmonary blood flow (Blalock-Taussig shunt)
definitive tment=surgery, usually around 6mnths of age, with aim of closing VSD and relieving RV outflow tract obstruction, sometimes with an artificial patch (often pericardial)-although increased risk of pulm regurg and RV dilatation, which extends across pulm valve.
hypercyanotic spells-if more than 15min, treat with sedation and analgesia-morphine, IV propranolol, IV vol administration, IV bicarb for acidosis, artificial ventilation to reduce metabolic O2 demand.

Question 6

indication for urgent surgical intervention in tetralogy of Fallot?

Answer 6

hypercyanotic spells-rapid increase in cyanosis, usually assoc. with irritability or inconsolable crying due to severe hypoxia, and breathlessness and pallor as tissue acidosis, short murmur on auscultation.
risk of MI, brain ischaemia and death

Question 7

role of beta blockers in tetralogy of Fallot?

Answer 7

relax contracted infundibulum (where pulm trunk arises from RV) and allow more time for RV filling, improving pulmonary blood flow

Question 8

lifelong risk with PDA?

Answer 8

bacterial endocarditis

pulm vascular disease and heart failure

Question 9

why might neonates with tetralogy of Fallot be given PGs?

Answer 9

e.g. alprostadil, if severely limited pulm blood flow causing profound cyanosis, to maintain DA patency so that an alternative source of pulmonary blood flow can be provided.

Question 10

features o/e of child with tetralogy of Fallot?

Answer 10

clubbing of fingers and toes
loud harsh ejection systolic murmur at left sternal edge
cyanosis at 3mnths of age-may not initially be cyanotic, ? as dependent on RV hypertrophy which if not present means L sided pressures greater so L to R shunt across VSD?**

Question 11

define patent ductus arteriosus

Answer 11

remnant of 6th aortic arch connecting PA and aorta has failed to close by 1 mnth after expected date of delivery due to defect in duct constrictor mechanism.
presence in preterm infant is not from congenital heart disease but from prematurity.

allows a L to R acyanotic shunt unless pulmonary HTN occurs

defined as persistent patency of ductus arteriosus if remains open after 3 mnths in preterm infant and 1 year in full term as spontaneous closure after these times is very low.

normal closure is functionally within 12-18hrs, and anatomically within 2-3 wks

Question 12

management of PDA?

Answer 12

need to close to minimise lifelong risk of bacterial endocarditis and pulm vascular disease
medical management in term infants limited to decongestive measures e.g. diuretics, if features of HF-?due to LV overload following increased pulmonary b.flow
must close if symptomatic, or if features of LV overload
at about 1 yr of age, closure with coil or occlusion device via cardiac catheter: if asymptomatic infants, wait until 1 yr with regular ECHO r/v to see if spontaenous closure, if not then endovascular closure.
surgical ligation occasionally required-e.g. if HF or pulm HTN infants-duct ligated and divided through left posterolateral thoracotomy without cardiopulmonary bypass.

may give prophylactic indometacin or ibuprofen in preterm neonates

Question 13

clinical features of persistent ductus arteriosus?

Answer 13

small shunts usually asymptomatic, but large shunts may LRTIs and feeding difficulty, and poor growth during infancy with failure to thrive due to heart failure.
continuous murmur beneath left clavicle (after 3 mnths), pansystolic in neonates
increased pulse pressure-BOUNDING or collapsing pulse?-distal pulses will be easily palpable e.g. DP-normally difficult to palpate in baby*-due to pulmonary circulation overload, run off here drops diastolic pressure-also causes tachycardia and tachypnoea
HF and pulm HTN when large duct with increased pulm blood flow, can be LVH and RVH-forceful apex beat and L parasternal heave.

Question 14

ECG findings if large VSD?

Answer 14

biventricular hypertrophy-tall R waves throughout chest leads, apparent by 2mnths of age.

Question 15

why is surgery required in a large VSD?

Answer 15

usually performed at 3-6mnths of age to manage HF and failure to thrive, and prevent permanent lung damage from pulm HTN and high blood flow.

small VSDs (smaller than aortic valve in diameter, maybe up to 3mm) will close spontaneously.

Question 16

characteristics of innocent heart murmurs?

Answer 16

systolic
musical quality
no radiation
vary in intensity with posture and respiration
asymptomatic
normal peripheral pulses

Question 17

characteristics of ASD murmur?

Answer 17

soft systolic murmur in 2nd left IC space due to high flow across normal pulmonary valve, wide fixed splitting of S2*-due to R sided fixed volume overload.

Question 18

PDA murmur?

Answer 18

pansystolic in neonates

continuous murmur after 3mnths

Question 19

clinical features of an ASD?

Answer 19

systolic murmur in 2nd left ICS, and widely split fixed S2
occasionally may be SOB, tiredness on exertion or recurrent chest infections

if moderate or large defect, close with occluding device placed by cardiac catheterisation or open heart surgery.

Question 20

commonest congenital heart lesion?

Answer 20

VSD

Question 21

clinical features of large VSD?

Answer 21

SOB on feeding and crying
failure to thrive
recurrent chest infections
harsh pansystolic murmur at lower L sternal border
murmur may radiate over whole chest and cause thrill

CXR if large defect-cardiomegaly and large PAs

Question 22

initial management of large VSD?

Answer 22

medical-aimed at controlling heart failure:
diuretics, and high energy feeds
ACEIs-reduce afterload on heart so aid flow from LV out into aorta rather than across defect into RV
digoxin-inotropic effects.

Question 23

VSD complications?

Answer 23

bacterial endocarditis
pulmonary vascular disease and shunt reversal with eisenmenger’s syndrome development
perimembranous defects can devlop aortic valve prolapse and eventual regurge
can be muscular obstruction in RV or LV outflow tract
RBBB can be caused by operative trauma, and occasionally complete heart block.

Question 24

what considerations need to be made with regards to contraceptive advice in women with congenital heart disease?

Answer 24

COCP CI in any pt with pulmonary HTN due to thrombogenic effect of oestrogen
and pregnancy can be life threatening in those with pulmonary HTN

Question 25

which is the only congenital heart defect for which strenuous activity should be avoided?

Answer 25

aortic stenosis as at risk of sudden death

manage with balloon valvuloplasty, open heart surgery if this is unsuccessful.

Question 26

what do serious complications develop due to in patients with untreated coarctation of the aorta?

Answer 26

HTN

Question 27

clinical features of coarctation of the aorta?

Answer 27

this is a localised aortic constriction in region of the ductus arteriosus origin
RF delay
upper limb HTN, but note L arm BP may be normal or low if coarctation involves origin of L SCA
in severe cases, baby may present with collapse after 1st wk of life when DA, which was maintaining systemic blood flow, closes.
differential cyanosis-upper body pink, lower body blue
systolic murmur in left infraclavicular area
ejection click may suggest assoc. bicuspid aortic valve=most common congenital heart defect in adults

Question 28

what is the concern if pt with coarctation of the aorta presents with sudden onset thunderclap headache?

Answer 28

SA haemorrhage due to berry aneurysm rupture assoc. with coarctation of aorta

Question 29

presentation of tetralogy of fallot?

Answer 29

most either diagnosed antenatally, present with low SpO2 or diagnosed following assessment for a heart murmur
minority will present with:
severe cyanosis at birth if pulm atresia
low BW, growth restriction
poor feeding, SOB, agitation
SOBOE
squatting to rest whilst exercising-characteristic of R to L shunt in older child

Question 30

what is transposition of the great vessels?

Answer 30

PA originates from LV and aorta from RV, so deoxygenated blood passes out of RV into systemic circulation, causing cyanosis in babies
ductus arteriosus between PA and aorta allows mixing of venous and arterial blood so oxygentated blood from LV can pass into the aorta and cause less cyanosis, mixing may also occur though septal defect which may accompany condition.

PG infusion to keep pda open
Arterial switch procedure to treat

Question 31

RFs for a patent ductus arteriosus?

Answer 31

genetic factors
alongside critically timed environmental exposure e.g. asphyxia at birth, rubella infection during pregnancy, or unknown viral infections or chemicals
trisomy 21
valproic acid exposure during pregnancy
birth at high altitude-exposure to low oxygen tension

Question 32

investigation findings other than ECHO in PDA?

Answer 32

ECG-often normal, may be LV hypertrophy-tall R waves V5 and V6
if large, may be biventricular hypertrophy, and RV hypertrophy in pulmonary HTN

CXR-if large shunt, then PAs, PVs, LA and LV enlargement

Question 33

when should PDA be suspected in the preterm infant?

Answer 33

if resp distress due to hyaline membrane disease (surfactant deficiency) worsens or does not improve after initial improvement and baby cannot be weaned off the ventilator.

Question 34

complications of surgical duct ligation for a PDA?

Answer 34

pneumothorax
VC palsy
hypotension

Question 35

what specific investigation in girls must be performed if coarctation of aorta present?

Answer 35

karyotype-for Turner syndrome-45 X

Question 36

how are heart murmurs graded?

Answer 36

1-6
1-murmur heard by an expert in optimum conditions
2-murmur heard by a non-expert in optimum conditions
3-easily heard murmur, no thrill
4-palpable thrill, loud murmur
5-palpable thrill, very loud murmur often heard over wide area
6-heard without stethoscope, very loud

Question 37

causes of a continuous heart murmur?

Answer 37

PDA
AV fistulae-systemic, pulmonary or coronary
systemic venous collaterals

Question 38

most common congenital heart defect to be found in adulthood?

Answer 38

ASD

Question 39

ASD risk factors?

Answer 39

Smoking
Down’s syndrome
Maternal diabetes
Feral alcohol syndrome