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Transcript
Congenital Heart Disease Epidemiology Physiology Incidence 8-8/1000; >75% survive first few months; 15% have >1 cardiac abnormality Innocent murmurs Venous hum: high pitched continuous murmur over base of heart, neck, SCJ; varies with respiration and position of head; disappears on lying down Vibratory murmur: short, buzzing mid-systolic murmur at L sternal edge / apex; louder on lying down, softer on standing Pul flow murmur: soft blowing ejection SM over pul area; prominent with exercise, fever, anaemia Loud, pan-systolic / diastolic; assoc with symptoms or thrill; radiate; not brief Non-innocent murmurs Duct dependent lesions Duct dependent lesions: depend on flow through PDA to provide systemic / pul flow; discovered in 1st-3rd week of life (ie. Neonatal period) when closure of ductus arteriosus causes rapid decompensation with shock and CV collapse Acyanotic: Coarctation of aorta (bypassed by PDA) Critical aortic stenosis Hypoplastic L heart syndrome (RV provides flow via PDA) Cyanotic: TGA Pul atresia / stenosis Hypoplastic R heart syndrome Examination: check all BP’s, L and R hand SaO2; metabolic acidosis; maybe cyanosis Ix: blood gas; ECG and CXR to re-open PDA; Mng: O2 is vasodilator and decreases RL flow across PDA,Mng and with may PGE2: cause vasoC of PDA 0.05-0.15mcg/kg/min giving O2 may help if pul HTN or vasoC; however may cause pul vasc overcirculation / steal systemic blood flow if PDA and ductdependent flow worsen systemic perfusion. Only give O2 if signs and Sx of inadequate tissue perfusion, or if O2 sats significantly below their normal baseline. PGE1 0.1mcg/kg/min improvement of systemic flow in mins titrate down to lowest effective dose (usually 0.05mcg/kg/min) Acyanotic heart disease LR CCF Cyanotic heart disease RL Tetralogy of Fallot IVF 10ml/kg bolus to incr preload and CO NaHCO3 1-2mmol/kg for severe metabolic acidosis (pH <7) Maybe pressors (eg. Dopamine, dobutamine) Give empiric Abx as cannot exclude sepsis 75% of all congenital heart disease; presents after 1-3/12 (when pul resistances decreases and hence LR shunt increases pul overcirculation) with CCF; CXR tends to show cardiomegaly and incr lung markings Causes: ASD (PFO) – 10% congenital heart disease VSD – 25% congenital heart disease (most common) Patent ductus arteriosus – 10% congenital heart disease; normally closes at 15hrs closes completely by 3/52 ligamentum arteriosum Common AV canal – 3% congenital heart disease; strongly assoc with Downs Coarctation of aorta – pul HTN develops with closure of PDA CCF; weak pulses in legs; ash-grey colour Hypoplastic L heart syndrome – decr LV outflow; systemic blood flow based entirely on PDA; CV collapse with closure; single heart sound Aortic stenosis – 6% congenital heart disease; CCF if severe; harsh SM to neck Causes: LR shunt pul overcirculation incr preload (eg. VSD, AVSD, truncus arteriosus, PDA; will present in 1st 3/12) Acute L heart obstruction (eg. AS, COA incr afterload; will present in 1st 1/52) 1Y myocardial decr inotropic function (eg. Cardiomyopathy, myocarditis; can present at any age) Other (eg. Anaemia, metabolic, toxic; usually presents on D1, unlike other causes) Sx: SOB after feeding, poor feeding, sweating, incr RR, incr HR, cardiomegaly, gallop rhythm, thrill, enlarged liver / spleen, incr incidence chest infections, weak pulse, FTT, fatigue; pul rales; incr WOB; no peri oedema Mng: O2 (large LR shunt (eg. VSD, PDA) may worsen with O2 so aim SaO2 no more than 95%) Elevate head of bed; if give IVF, be careful Frusemide 1-2mg/kg IV PO frusemide and spironolactone Digoxin (for inotrope): 40mcg/kg if >1/12, 30mcg/kg if <1/12, 20mcg/kg if prem – give ½ dose as bolus ¼ 812hrs later ¼ 8-12hrs later Consider dopamine / dobutamine (5-10mcg/kg/min) Consider nitroprusside, Ca channel blockers in consultation with cardiologist Epidemiology: 25% of all congenital heart disease Causes: caused by mixing of oxygenated and deoxygenated blood Tetralogy of Fallot Transposition of great arteries (mixing occurs through concurrent ASD / VSD) Tricuspid problems (eg. Tricuspid atresia, Ebstein’s anomaly) Truncus arteriosus Total anomalous pul venous return Eisenmeger’s syndrome (large uncorrected ASD / VSD; occurs later as infant / child) Other: persistent fetal circulation (caused by structural heart disease, meconium aspiration, pneumonia, sepsis, pul HTN) blood shunts RL through PFO / ASD / VSD Sx: effortless incr RR, polycythaemia; presents in neonatal period; causes cyanotic spells; for cyanosis to be present, SaO2 70-80% Ix: hyperoxia test: measure PaO2 15min high flow O2 measure PaO2 should rise by 20mmHg, if not = cyanotic heart disease Epidemiology: 10% of all congenital heart disease; most common cause of cyanotic Pathophysiology: 1. Large VSD – subaortic perimembranous; R L shunt through this to aorta 2. Pul stenosis +/- hypoplastic pul artery RV outflow obstruction 3. Over-riding aorta (aortic root overlying septum and VSD) 4. RVH If assoc with ASD (in 10%) = pentalogy of Fallot; cyanosis due to infundibular spasm and decr pul blood flow Sx: onset of cyanosis in 1st few wks/mths of life; cyanosed after feeding, squatting after exercise (decr return of desaturated blood from legs, incr aortic p, incr afterload decr size of RL shunt); degree of cyanosis depends on extent of RV outflow obstruction and hence direction of shunt Tet spells: caused by RV outflow tract obstruction / changes in vascular resistance (may also occur in patients with pul stenosis) decr SVR and pul blood flow RL shunting through VSD; precipitated by crying, fever, dehydration, incr RR, incr HR, defecation, feeding, anaphylaxis, hypoxia (which causes pul vasoC), acidosis; present with period of inconsolable crying, incr RR, incr cyanosis, decr intensity of heart murmur, limpness, seizures; most common in 4-6/12; trt by decreasing SVR, HR, agitation, infundibular spasm; complications are due to prolonged hypoxia Examination: ejection click (due to aorta) ejection systolic murmur with thrill in pul area (turbulent flow at RV outflow tract) ejection systolic murmur at L sternal edge which radiates to back (obstruction of RV outflow) clubbing, cyanosis, RV heave, loud S2; normal pulses, S1, heart size, continuous PDA murmur Ix: ECG: ventricular arrhythmias in 40%; AF, flutter common; RAD; RAH; RVH; tall peaked T waves CXR: small pul arts (due to decr blood flow); boot shaped heart (due to RVH); oligaemic lung fields; no Cardiomegaly Mng: Of Tet spells: aim is to incr pul blood flow by increasing preload, provide pul vasoD, incr afterload (SVR) to reverse RL shunting, correct hypoxia, correct acidaemia 1. O2 100% (usually has little effect though) 1. knees bent posture (incr VR, incr SVR); rest; abdo compression; calm child 1. morphine 0.1-0.2mg/kg IV/IM (decr catecholamines, decr RR; but will also decr SVR) 2. IVF 10-20ml/kg (incr preload, decr dynamic outflow obstruction; give this before drugs that may cause hypotension) 3. HCO3 1-2mmol/kg IV (corrects acidosis, promotes pul vasoD) – repeat at 10-15mins 4. metaraminol 50mcg/kg IV over 10-15mins 0.25-1mcg/kg/min infusion (incr afterload decr RL shunt) 4. esmolol 500mcg/kg over 1min 50mcg/kg/min infusion (or propanolol 0.01-0.2mg/kg) (decr RV outflow obstruction via decr infundibular spasm, incr pul outflow) or propanolol 0.010.2mg/kg IV 5. ketamine, RSI Of TOF: corrective surgery (when v young, <1% surgical mortality in children; 90% 30yr survival with good levels of function) early palliative OT if severely ill (pul valvuloplasty to incr pul art blood flow) Complications: Tet spells, polycythaemia ( thrombosis), consumptive coagulopathy, endocarditis Transposition of great vessels Ebstein’s anomaly Epidemiology: 5-8% congenital heart disease; most common cyanotic lesion manifesting in newborn period Only compatible with life if mixing of R and L circulations – VSD (in 2040%), ASD, PDA (3) Sx: onset of severe cyanosis within hours unresponsive to O2 Examination: loud S2; no audible murmurs (SM if VSD) Ix: CXR: cardiomegaly; egg shaped heart; incr pul vasculature ECG: RAD, RVH Mng: urgent balloon atrial septoplasty will provide rapid improvement; arterial-switch operation (low mortality, excellent long-term outcome) Pathophysiology: leaflets of tricuspid valve displaced into RV, portion of RV located in RA tricuspid regurg (sometimes stenosis); ASD in 80% Sx: vary from very mild to very severe; cyanosis worsens with increased RL shunt through ASD Examination: wide split S1 and S2; S3 and S4; SM at lower L sternal edge (due to TR); hepatomegaly Ix: ECG: P pulmonale, RBBB, 1st deg HB; assoc with SVT CXR: cardiomegaly, RA enlargement, decr pul vasc Markings Mng: temporary arterial shunt from LR if severe to incr pul blood flow; repair 1-2% congenital heart disease; no tricuspd valve; development of RV and pul art wrong decr pul blood flow; needs mixing to survive (ASD, VSD, PDA) as RA needs RL shunt in order to empty CXR: slight cardiomegaly, pul oligaemia ECG: RAH, LAH, LVH, superior axis Triscuspid atresia <1% congenital heart disease Single artery arises from heart then branches into PA and aorta; pul blood flow may be incr ( CCF), normal or decreased (cyanosis) Mild cyanosis, CCF in newborn, bounding pulses CXR: cardiomegaly, pul plethora ECG: LVH and RVH Persistent truncus arteriosus Eisenmenger syndrome TAPVR CXR summary Pathophysiology: LR shunt incr pul blood flow pul HTN (incr arteriolar muscles) becomes RL shunt; occurs with VSD, ostium primum defect, transposition of great vessels with large shunt Sx: haemoptysis (20%), CVA (10%), paradoxical embolism, brain abcess (5%); Sx occur in adolescents / young adults Examination: clubbing, cyanosis; normal / high JVP; small vol pulse; RV heave; loud P2; R sided S4; murmur of original defect disappears when Eisenmenger syndrome develops; decrescendo diastolic murmur (due to pul regurg), pansystolic murmur (due to TR) Ix: ECG: AF, flutter; RAD; RVH; P pulmonale CXR: cardiomegaly; large pul art Mng: no surgical trt avalable; maintain intravascular vol; avoid hypoxia and vasoD 1% congenital heart disease; pul veins enter into RA instead of LA; survival depends on mixing of blood (ASD, PFO); more commonly presents with CCF than cyanosis CXR: Snowman sign, severe cardiomegaly, incr pul markings ECG: RAD, RVH CXR: cardiomegaly: TOF, TGV, Ebstein’s; Eisenmeger’s; ASD; VSD Pul. Plethora: LR shunt; TGV; patent truncus arteriosus Pul. Oligaemia: TOF, Ebstein’s Pul. Venous congestion: L heart obstruction Notes from: Dunn, TinTin