Paediatric Cardiology

Question 1

What is ventricular septal defect?

Answer 1

VSD - MC congenital heart disease.
spontaneously happen in 50% pts.

Question 2

Aetiology of Ventricular septal defect

Answer 2

congenital VSD association with chromosomal disorders:
- downs syndrome
- edwards syndrome
- patau syndrome
- cri-du chat syndrome

congenital infections

acquired causes:
- post-myocardial infarction

Question 3

can VSD be detected in utero and how?

Answer 3

yes during 20week scan.

Question 4

tell me some post natal presentations of ventricular septal defect

Answer 4

failure to thrive
features of HF: hepatomegaly, tachypnoea, tachycardia, pallor

classically - pan-systolic murmur - louder in smaller defects

Question 5

how would you manage a ventricular septal defect?

Answer 5

if small VSD asymptomatic : they often close spontaneously and simple require monitoring

moderate to large VSD : result in degree of HF in 1st few months:
nutritional support
med for HF: diuretics
surgical closure of the defect

Question 6

complications of ventricular septal defects

Answer 6

aortic regurgitation : due to poorly supported right coronary cusp results in cusp prolapse.

infective endocarditis
right HF
pulmonary HTN: pregnancy CI in women with pulmonary HTN - 30-50% mortality

eisenmenger’s complex - due to prolonged pulmonary htn from left to right shunt = RV hypertrophy = increase RV pressure. = exceeds LV pressure = reversal of blood flow. = clubbing and cyanosis.

Question 7

what is the eisenmengers complex an indication for ?

Answer 7

heart-lung transplant

Question 8

what is an atrial septal defect?

Answer 8

most likely congenital found in adulthood.

50% pts dead by 50.

2 types:
ostium secundum - MC
ostium primum

Question 9

features of atrial septal defects

Answer 9

ejection systolic murmur, fixed splitting of s2

embolism might pass from venous system to left side of heart = stroke

Question 10

tell me about ostium secundum - 70% of ASDs

ecg finding

Answer 10

associated with holt-oram syndrome - (tri-phalangeal thumbs)

ecg: RBBB with Right axis deviation

Question 11

tell me about ostium primum

Answer 11

presents earlier than ostium secundum defects

associated with abnormal AV valves

ECG: RBBB with left axis deviation , prolonged PR interval

Question 12

what is tetralogy of fallot?

Answer 12

mc cyanotic congenital heart disease.

presents around 1-2 mths - could not be picked up by 6 mths.

Question 13

why does TOF happen.

Answer 13

anterior malalignment of the aorticopulmonary septum

Question 14

4 characteristic features of tetralogy of fallot

Answer 14

ventricular septal defect
RV hypertrophy
RV outflow tract obstruction, pulmonary stenosis
overriding aorta

Question 15

in TOF what determines the degree of cyanosis and clinical severity?

Answer 15

the severity of the right ventricular outflow tract obstruction (pulmonary stenosis)

Question 16

other general features of TOF

Answer 16

cyanosis : unrepaired TOF infants could get episodic hyper cyanotic “tet” spells due to near occlusion of RV outflow tract.

right to left shunt
ejection systolic murmur at left sternal edge - due to pulmonary stenosis (VSD doesnt usually cause murmur)

right sided aortic arch - 25% pts

cxr : “boot-shaped” heart,
ECG: RV hypertrophy

Question 17

tell me a little bit about tet spell - TOF

Answer 17

in unrepaired TOF infants

hypercyanotic spell due to near occlusion of RV outflow tract.

features :
tachypnoea
severe cyanosis could result in loss of conciousness

typically when:
infant upset
in pain
fever

Question 18

how would you manage TOF?

Answer 18

surgical repair in 2 parts

cyanotic episodes : beta blockers to reduce infundibular spasm

Question 19

at birth what is the most common lesion in patients with TOF?

Answer 19

transposition of the great arteries.

Question 20

what is heart failure?

cause?

Answer 20

heart unable to pump enough blood to meet met needs of body.

structural or functional heart disease.

Question 21

classifying heart disease by ejection fraction

Answer 21

either normal LVEF or abnormal LVEF.

measure using echocardiography

reduced LVEF = <35%-40%. - HF-rEF - 50% of HF pts.

other 50% : HF-pEF - preserved LVEF.

Question 22

systolic vs diastolic dysfunction - HF

Answer 22

HF-rEF : systolic dysfunction - impaired myocardial contraction during systole

HF-pEF: diastolic dysfunction : impaired ventricular filling during diastole.

Question 23

can you give me some causes of systolic dysfunction?

Answer 23

ischaemic heart disease
dilated cardiomyopathy
myocarditis
arrhythmias

Question 24

can you give me some causes of diastolic dysfunction?

Answer 24

hypertrophic obstructive cardiomyopathy
restrictive cardiomyopathy
cardiac tamponade
constrictive pericarditis

Question 25

categorising HF by time

Answer 25

acute : acute exacerbation of chronic HF. urgent sx due to LV failure = pulmonary oedema.

Question 26

categorising HF by left/right

Answer 26

HF-rEF and HF-pEF most develop left HF. due to : increased ventricular afterload (arterial htn/aortic stenosis) backflow to LV right HF : caused by increased RV afterload (pulmonary htn) or increased RV preload (tricuspid regurgitation)

Question 27

what signs does lv failure typically result in?

Answer 27

pulmonary oedema : - dyspnoea -orthopnoea - paroxysmal noctural dyspnoea - bibasal fine crackles

Question 28

what signs does rv failure typically result in ?

Answer 28

peripheral oedema - ankle/sacral odema raised jugular venous pressure hepatomegaly weight gain bc of fluid retention anorexia (cardiac cachexia)

Question 29

what does it mean by high-output HF?

Answer 29

where normal heart cant pump enough blood to meet the metabolic needs of body

Question 30

causes of high output HF

Answer 30

anaemia pagets disease arteriovenous malformation pregnancy thyrotoxicosis thiamine deficiency (wet beri-beri)

Question 31

different between afterload and preload - hf?

Answer 31

preload - amount of blood in the ventricle at the end of diastole (before contraction) afterload - resistance the ventricle must overcome to pump blood out during systole

Question 32

features of chronic heart failure

Answer 32

dyspnoea cough: possible worse at night : pink/frothy sputum orthopnoea paroxysmal noctural dyspnoea wheeze : cardiac wheeze bibasal crackles on auscultation signs of right sided HF: raised JVP , ankle oedema and hepatomegaly weight loss : cardiac cachexia : 15% pts - could be hidden by weight gained secondary to edema

Question 33

diagnosing chronic heart failure? 1st line what to do ?

Answer 33

1st line: N-terminal pro-B-type natriuretic peptide (NT-proBNP) blood test if high : specialist assessment (transthoracic echocardiography too) within 2 weeks if raised : within 6 weeks

Question 34

what is bnp and what are high levels?

Answer 34

b-type natriuretic peptide - produced by LV myocardium responding to strain. - very high levels = poor prognosis high levels are: bnp : >400 pg/ml (116 pmol/l) NTproBNP: >2000 pg/ml (236 pmmol/l) raised levels are : bnp : 100-400 pg/ml (29-116 pmol/l) NTproBNP: 400- 2000 pg/mol (47-236 pmol/l) normal levels: bnp: <100 pg/ml (29 pmol/l) NTproBNP: <400 pg/ml (47 pmol/l)

Question 35

name some factors that can increase BNP

Answer 35

lv hypertrophy sepsis copd diabetes age over 70 liver cirrhosis hypoxemia - includes pulmonary embolism rv overload tachycardia ischaemia

Question 36

factors decreasing bnp levels

Answer 36

obesity diuretics acei beta-blockers aldosterone antagonists angiotensin 2 receptor blockers

Question 37

what is the classification used to classify severity of chronic heart failure?

Answer 37

NYHA - new york heart association class 1 -4 1: no sx. no limitation : ordinary physical exercise doesnt cause undue fatigue, dyspnoea or palpitations 2: mild sx. slight limitation of physical activity. comfortable at rest- ordinary activity = fatigue, palpitations/dyspnoea 3. moderate sx. marked limitation of physical activity. comfortable at rest - less than ordinary activity = sx. 4. severe sx. cant do physical activity without discomfort. hf sx present at rest - increased discomfort with any physical activity.

Question 38

How to manage chronic heart failure?

Answer 38

1st line: ace-inhibitor and beta blocker. start 1 drug at a time. clinical judgement on which one 2nd line: 3rd line: