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Congenital Heart DiseaseEpidemiologyIncidence 8-8/1000; >75% survive first few months; 15% have >1 cardiac abnormalityPhysiologyInnocent murmursVenous hum: high pitched continuous murmur over base of heart, neck, SCJ; varies with respiration and position of head; disappears on lying downVibratory murmur: short, buzzing mid-systolic murmur at L sternal edge / apex; louder on lying down, softer on standingPul flow murmur: soft blowing ejection SM over pul area; prominent with exercise, fever, anaemiaNon-innocent murmursLoud, pan-systolic / diastolic; assoc with symptoms or thrill; radiate; not briefDuct dependent lesions2908300-6778625Duct 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 Mng with PGE2: to re-open PDA; 0.05-0.15mcg/kg/min0Duct 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 Mng with PGE2: to re-open PDA; 0.05-0.15mcg/kg/minExamination: check all BP’s, L and R hand SaO2; metabolic acidosis; maybe cyanosisIx: blood gas; ECG and CXRMng: O2 is vasodilator and decreases RL flow across PDA, and may cause vasoC of PDA giving O2 may help if pul HTN or vasoC; however may cause pul vasc overcirculation / steal systemic blood flow if PDA and duct- dependent 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) 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 sepsisAcyanotic heart diseaseLR75% 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 markingsCauses: 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 neckCCFCauses: 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 oedemaMng: 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 ? dose as bolus ? 8- 12hrs later ? 8-12hrs later Consider dopamine / dobutamine (5-10mcg/kg/min) Consider nitroprusside, Ca channel blockers in consultation with cardiologistCyanotic heart diseaseRLEpidemiology: 25% of all congenital heart diseaseCauses: 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 / VSDSx: 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 diseaseTetralogy of Fallot3351530-2286635Epidemiology: 10% of all congenital heart disease; most common cause of cyanoticPathophysiology: 1. Large VSD – subaortic perimembranous; R L shunt through this to aorta2. Pul stenosis +/- hypoplastic pul artery RV outflow obstruction3. Over-riding aorta (aortic root overlying septum and VSD)4. RVHIf assoc with ASD (in 10%) = pentalogy of Fallot; cyanosis due to infundibular spasm and decr pul blood flow0Epidemiology: 10% of all congenital heart disease; most common cause of cyanoticPathophysiology: 1. Large VSD – subaortic perimembranous; R L shunt through this to aorta2. Pul stenosis +/- hypoplastic pul artery RV outflow obstruction3. Over-riding aorta (aortic root overlying septum and VSD)4. RVHIf assoc with ASD (in 10%) = pentalogy of Fallot; cyanosis due to infundibular spasm and decr pul blood flowSx: 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 shuntTet 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 hypoxiaExamination: 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 murmurIx: 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 CardiomegalyMng: 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 0.01- 0.2mg/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, endocarditisTransposition of great vessels2108200-6350Epidemiology: 5-8% congenital heart disease; most common cyanotic lesion manifesting in newborn periodOnly compatible with life if mixing of R and L circulations – VSD (in 20-40%), ASD, PDA (3)Sx: onset of severe cyanosis within hours unresponsive to O2Examination: loud S2; no audible murmurs (SM if VSD)Ix: CXR: cardiomegaly; egg shaped heart; incr pul vasculature ECG: RAD, RVHMng: urgent balloon atrial septoplasty will provide rapid improvement; arterial-switch operation (low mortality, excellent long-term outcome)Epidemiology: 5-8% congenital heart disease; most common cyanotic lesion manifesting in newborn periodOnly compatible with life if mixing of R and L circulations – VSD (in 20-40%), ASD, PDA (3)Sx: onset of severe cyanosis within hours unresponsive to O2Examination: loud S2; no audible murmurs (SM if VSD)Ix: CXR: cardiomegaly; egg shaped heart; incr pul vasculature ECG: RAD, RVHMng: urgent balloon atrial septoplasty will provide rapid improvement; arterial-switch operation (low mortality, excellent long-term outcome)Ebstein’s anomaly267970049530Pathophysiology: 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 ASDExamination: wide split S1 and S2; S3 and S4; SM at lower L sternal edge (due to TR); hepatomegalyIx: ECG: P pulmonale, RBBB, 1st deg HB; assoc with SVT CXR: cardiomegaly, RA enlargement, decr pul vasc MarkingsMng: temporary arterial shunt from LR if severe to incr pul blood flow; repairPathophysiology: 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 ASDExamination: wide split S1 and S2; S3 and S4; SM at lower L sternal edge (due to TR); hepatomegalyIx: ECG: P pulmonale, RBBB, 1st deg HB; assoc with SVT CXR: cardiomegaly, RA enlargement, decr pul vasc MarkingsMng: temporary arterial shunt from LR if severe to incr pul blood flow; repairTriscuspid atresia1-2% 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 emptyCXR: slight cardiomegaly, pul oligaemiaECG: RAH, LAH, LVH, superior axisPersistent truncus arteriosus267970053340<1% congenital heart diseaseSingle 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 pulsesCXR: cardiomegaly, pul plethoraECG: LVH and RVH<1% congenital heart diseaseSingle 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 pulsesCXR: cardiomegaly, pul plethoraECG: LVH and RVHEisenmenger syndromePathophysiology: 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 shuntSx: haemoptysis (20%), CVA (10%), paradoxical embolism, brain abcess (5%); Sx occur in adolescents / young adultsExamination: 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 artMng: no surgical trt avalable; maintain intravascular vol; avoid hypoxia and vasoDTAPVR1% 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 cyanosisCXR: Snowman sign, severe cardiomegaly, incr pul markingsECG: RAD, RVHCXR summaryCXR: 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 obstructionNotes from: Dunn, TinTin ................
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