Congenital heart disease Flashcards
What are the two main causes of cyanotic heart disease?
Tetralogy of Fallot
Transposition of the great arteries
Also tricuspid atresia and truncus arteriosus
What is the Tetralogy of Fallot and why is it a cyanotic heart disease?
1/2000
4x deficits = VSD, right ventricular hypertrophy, overriding aorta (receives blood from L+R sides of the heart) and pulmonary stenosis
The stenosis is what causes the cyanosis (as means that less bloody being oxygenated and thus blood leaving the aorta will be less than 97-100% saturated)
What are some other symptoms of Tetralogy of Fallot?
Difficulty feeding, failure to thrive, delayed development, exertional dyspnoea, digital clubbing
‘Tet’ spells = SOB + cyanosis + agitation + loss of consciousness - squatting during the process will increase systemic vascular resistance and temporarily reduce/reverse the R-L shunt
What murmur will you hear in Tetralogy of Fallot?
Harsh ejection systolic murmur at the upper left sternal border - murmur made by the pulmonary stenosis
What is the transposition of the great arteries, why is it a cyanotic heart disease and how is it managed?
Aorta and pulmonary arteries are swapped over
Is incompatible with life - but picked up on antenatal scans
Cyanotic because systemic circulation will never receive oxygenated blood
The link between both circulations - the ductus arteriosus - is kept open with IV prostaglandins, allowing newborn to circulate own blood prior to surgery once child is 6kg; duct can then be shut with NSAIDs and an arterial switch surgery performed
When should you consider heart disease as a cause of cyanosis?
When its central (peripheral may just be due to cold for example)
When it is uncorrected by giving oxygen
What are some common causes of acyanotic heart disease?
Anything that involves L-R shunting will be acyanotic e.g. PDA, VSD, ASD, coarctation, aortic stenosis - because blood leaving the aorta in these conditions will still be maximally saturated with O2
What are the two important ejection systolic murmurs and how do you differentiate them?
Aortic stenosis - loudest URSB, peripherally cyanotic, also possible coarctation (CHECK FOR)
Pulmonary stenosis = loudest ULSB, centrally cyanotic
What are some important systolic murmurs and how do you differentiate them?
VSD = most common, loudest at LLSB
Mitral regurg = uncommon in UK because less rheumatic heart disease, loudest 4th/mid + radiating ot axilla
Tricuspid regurg = associated with RH failure - palpable liver?
ASD - ejection systolic loudest in pulmonary area, fixed splitting of 2nd heart sound (made by hearing closure of aortic and pulmonary valves separately instead of together - very characteristic)
What are the diastolic and late diastolic murmurs?
Diastolic = aortic regurg + pulmonary regurg (rare in paeds)
Late diastolic = mitral stenosis + pulmonary stenosis
What is a continuous murmur and its cause?
Persistent throughout systole and diastole
PDA - loudest at the left clavicle
What are some signs a murmur is innocent?
Localised (non-radiating) No signs or symptoms Normal sats Normal echo High output cardiac state (= flow murmur)
How common is congenital heart disease?
up to 8/1000 babies
What are some risk factors for developing congenital heart disease?
Down syndrome (1/2 of cases, AVSD most common); also Turner’s (bicuspid aortic valve, coarctation), Noonan
Maternal infection e.g. rubella, influenza; especially in first trimester
Maternal drugs - statins, benzodiazepines, ibuprofen (at >30wks); smoking, drinking
Poorly controlled maternal DM -T1 or T2
Also VACTERL association: Vertebral anomalies -hypoplastic/small Anal atresia Cardiovascular anomalies - VSD, tetralogy of fallot Tracheoosophageal fistula Esophageal fistula Renal and/or radial abnormalities - hypoplastic thumb, polydactyly Limb defects
How is congenital heart disease usually picked up?
Routine scans e.g. 20wk scan
If detected or strong FHx - specialist fotal echo between 18-20wks can confirm Dx
