cardiology Flashcards
talk about congenital heart diseases
30% of all congenital anomalies
Incidence of 8/1000 live births
8 common lesions account for 80% cases of congenital heart disease
what are 8 if the commonest lesions in congenital heart disease?
- Ventricular Septal Defect (VSD)
- Patent Ductus Arteriosus (PDA)
- Atrial Septal Defect (ASD)
- Pulmonary Stenosis
- Aortic Stenosis
- Coarctation of the Aorta
- Transposition of Great Arteries
- Tetralogy of Fallot
what are the two tings affecting aetiology of congenital heart disease?
- Genetic Susceptibility -Environmental Hazard
- Teratogenic Insult - 18 to 60 days post conception
what are some environmental factors that affect congenital heart disease?
- DRUGS - Alcohol, Amphetamines, Cocaine, Ecstasy, Phenytoin, Lithium
- INFECTIONS - TORCH and others (Toxoplasma, Rubella, CMV, Herpes)
- MATERNAL - Diabetes Mellitus, Systemic Lupus Erythematosus
what is the relationship between chromosomal abnormalities and CHD
- 6 - 10% of all CHD have underlying chromosomal problem
- 30% of chromosomal abnormalities have CHD
what are the most common chromosomal abnormalities connected with CHD?
- Trisomy 13 90%
- Trisomy 18 80%
- Trisomy 21 40%
specifically AVSD (Atrio-Ventricular
Septal Defect) but ASD and VSD
more common
what are other syndromes connected to CHD?
- Turner : Co-arctation of aorta
- Noonan : Pulmonary Stenosis (other ventricular outflow tract problems)
- Williams : Supravalvular aortic stenosis
- 22q11 deletion syndrome
(Digeorge – cleft palate, hypoparathyroidism and hypocalcaemia
Hyperplasia of thymus with T cell defect)
what symptoms would be suggestive of heart problems?
- Feeding (might get breathless or start sweating during feeds), Weight (won’t put on weight well - compensating for HF) and Development –> faltering growth
- Cyanosis (Right to left shunt causes central cyanosis)
- Dyspnoea
- Tachypnoea (HF) (pulmonary congestion)
- Tachycardia (HF)
- Hepatomegaly (HF)
- Exercise Tolerance
- Chest Pain
- Syncope
- Palpitation
- Joint Problems
- Murmurs (but most are innocent heart murmur)
what findings in the examination would be suggestive of heart problems?
Weight and Height Dysmorphic features Cyanosis Clubbing Tachy-/Dyspnoea Pulses/Apex (femoral pulses!) Heart Sounds (clicks, split, 3rd and 4th) Murmurs
what investigations would you do for heart disease?
Blood Pressure O2 saturation, arterial BGA ECG (12 lead, 24hrs, event monitor) CXR Echocardiogram Catheter Angiography MRI/A Exercise testing (ECG, sO2)
what are the treatment principles in heart disease?
- If you can fix it -> fix it
- If you can’t fix it -> improve the situation :
- medication
- palliative procedure, e.g.
BT shunt, balloon valvoplasty,
Prostaglandin infusion, pulmonary banding - If you can do neither ->replace it
what are the characterisations of murmurs?
- Timing in Cardiac Cycle
- Systole / Diastole / Continuous - Duration
- Early / Mid / Late- Ejection / Holo- or Pan Systolic
- Pitch / Quality
- Harsh or Mixed Frequency (Turbulence)
- Soft or Indeterminate
- Vibratory / Pure Frequency (Laminar Flow)
talk about the cardiac cycle
…..
what are common murmur sites?
- Upper Right Sternal Border
- UL SB
- LL SB
Apex
talk about innocent murmurs
- 70-80% of murmurs
- NOT A DIAGNOSIS OF EXCLUSION
Specific Features - 4 Types
what are the 4 types of innocent murmurs?
- still’s murmur (LV outflow murmur)
- pulmonary outflow murmur
- Carotid/Brachiocephalic Arterial Bruits
- Venous Hum
what are some common features of innocent murmurs?
- Systolic murmur (continuous in venous hum)
- No other signs of cardiac disease
- Soft murmur, grade 1/6 or 2/6
- Vibratory, musical
- Localised
- Varies with position, respiration, exercise
Talk about the Still’s murmur (LV outflow murmur)
- Age 2-7 years
- Soft systolic; vibratory, musical,”twangy”
- Apex,left sternal border
Increases in supine position and with exercise
Talk about Pulmonary Outflow Murmurs
- Age 8-10 years
- Soft systolic; vibratory
- Upper left sternal border, well localised, not radiating to back
- Increases in supine position, with exercise
- Often children with narrow chest
Talk about Carotid/Brachiocephalic Arterial Bruits
- Age 2-10 years
- 1/6-2/6 systolic; harsh
- Supraclavicular, radiates to neck
- Increases with exercise, decreases on turning head or extending neck
Talk about venous hums
- Age 3-8 years
- Soft, indistinct
- Continuous murmur, sometimes with diastolic accentuation
- Supraclavicular
- Only in upright position, disappears on lying down or when turning head
what is a ventricular septal defect (VSD)?
talk about 3 types
- 3 main types :
- subaortic
- perimembranous
- muscular
- L to R shunt (means you are acynatonic)-
- Bigger the hole the worse it is
- Higher towards the values the more trouble
what is the clinical presentation of VSD?
- Pansystolic murmur lower left sternal edge, sometimes with thrill
- In very small VSDs, early systolic murmur
- In very large VSDs diastolic rumble due to relative mitral stenosis
- Signs of cardiac failure in large VSDs, eventually leading to biventricular hypertrophy and pulmonary hypertension
what is eisenmenger syndrome?
Due to relation of ventricles to each other, the blood flow is going straight up in PA
PA gets most of shunt volume so right ventricle doesn’t have much bother from it, but left ventricle does have bother
so get left ventricular hypertrophy
get fluid overloads and hyperaemia in pulmonary circulation - pulmonary arteries are not happy so lay down fibroblasts in cell wall and get stiffer
RV has to pump harder as resistance inc
RV hypertrophies
Pulmonary arterial pressure inc
and this keeps going - vicious cycle
- need to close it
- might need to have combined heart and lung transplant to overcome it
how would you treat a VSD?
Close the hole -
- amplatzer or other occlusion device, trans-catheter
- Patch closure, open heart surgery
Talk about atrial septal defects (ASD)
- Few clinical signs in early childhood, good chance of spontaneous closure
- Sometimes detected in adulthood with atrial fibrillation, heart failure or pulmonary hypertension
- Wide fixed splitting of 2nd heart sound (late sign) , pulmonary flow murmur
- L–> R Shunt
Talk about Atriovetriculo-septal defect or endocardial cushion defect or AV canal defect
- Associated with Trisomy 21
- Singular AV valve with ostium primum ASD and high VSD
what happens in complete
Atrio-Ventricular Septal Defect?
- Mitral valve and tricuspid valve are fused - single valve
- The clefts in this valve determine where the blood flow goes
- if you have this v likely to have trisomy 21
talk about pulmonary stenosis
- Asymptomatic in mild stenosis, in moderate and severe exertional dyspnoea and fatigue
- Ejection systolic murmur - upper left sternal border with radiation to back
what happens in balloon valvoplasty?
- Put balloon in pulmonary trunk which will break and rupture adhesions in periphery and break annulus to make the valve bigger - to inc the pulmonary circulation
- can get significant pulmonary regurgitation as have damaged the leaflet of the valve
- can replace the valve once you have gone through puberty
- but try to push getting a replacement valve as much as possible as the only last about 15 years and the more you need the harder it becomes
Talk about aortic stenosis
- Mostly asymptomatic; if severe, reduced exercise tolerance, exertional chest pain, syncope
- Ejection systolic murmur upper right sternal border, radiation into carotids
What are the changes in the Fetal Circulation at Birth?
- Pulmonary Vascular Resistance Falls
- Pulmonary Blood Flow Rises
- Systemic Vascular Resistance is increased
- Ductus Arteriosus Closes (within mins of surgery)
- Foramen Ovale Closes
- Ductus Venosus Closes
How would you treat Patent Ductus arteriosus (Botalli)
- Very common in pre-term infants, treatment with:- fluid restriction/diuretics, prostaglandin inhibitors
(Indomethacin, Ibuprofen), surgical ligation - In term babies good chance of spontaneous closure, not prostaglandin sensitive
- if you cant hear it but can find in echo then leave it alone
- If you can hear and find it in echo then close it
what investigations would you do for co-arctation of aorta?
3D MRI Scan
what is the management for co-arctation of the aorta?
- Re-open DA with Prostaglandin E1 or E2
- Resection with end-to-end anastomosis
- Subclavian patch repair
- Balloon Aortoplasty (when other complications present - to stabilise baby - then go for definitive surgery later)