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Congenital Heart Defects Craig T. Carter, D.O. Assistant Professor – EM/Peds University of Kentucky.

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Presentation on theme: "Congenital Heart Defects Craig T. Carter, D.O. Assistant Professor – EM/Peds University of Kentucky."— Presentation transcript:

1 Congenital Heart Defects Craig T. Carter, D.O. Assistant Professor – EM/Peds University of Kentucky

2 n A nurse approaches you - Doctor, Doctor - a kid just checked in with a history of Hypoplastic Left Heart syndrome – What is that again?? You reply …..

3 n I need to go to the bathroom, as soon as I get back, I will tell you all about it....

4 So you sit and think…..

5 And think some more….

6 Back to Basics-Fetal Circulation n Four shunts of blood flow : placenta, ductus venosus, foramen ovale and ductus arteriosus

7 Back to Basics-Fetal Circulation

8 Changes after birth: n Shift of blood flow for gas exchange, from the placenta to the lungs. n Closure of ductus venosus :no placenta n increase in pulmonary blood flow n functional closure of foramen ovale (increased LA pressure) n Closure of ductus arteriosus (^O2)

9 Newborn Cardiac Exam n Vital signs: RR, HR, BP n respiratory effort n color n palpate point of maximal impulse (PMI) of heart n palpate pulses of all extremities n auscultate

10 Case 1: 2 day old infant n 2 day old baby girl is being examined for discharge physical. n Vitals: HR:120 RR:40 BP: r arm 75/40 n Gen : low hairline, webbed neck, edemetous dorsum of feet

11 Case 1: cardiac exam n Normal PMI, no thrill n No hepatosplenomegally n unable to palpate pulses in lower extremities n BPs all four extremities: both legs systolic BP lower 40/30

12 Case 1: Auscultation n S2 splits normally n ejection click n systolic ejection murmur III/VI at the URSB and LLSB n systolic murmur radiates to the back n early diastolic decrescendo murmur at 3LICS

13 Case 1: n What do you think is the congenital defect?

14 Case 1: n What further evaluation do you need?

15 Coarctation of Aorta (COA)

16 n Incidence: 8% of all congenital heart defects n Turners syndrome: 30% have COA n Preductal: 40% associated with other cardiac defects,symptomatic early in life n Postductal: less likely to have symptoms early

17 COA n EKG: LVH (but may be normal in 20%) n X-rays: heart size may be normal or slightly enlarged n Rib notching in older children n Echo: can see narrowing n Bicuspid aortic valve n Doppler:disturbed flow

18 COA complications n COA can cause CHF, HTN (intracranial bleeding,hypertensive encephalopathy) n Bicuspid aortic valve: stenosis or regurg n LV failure n infective endocarditis

19 Preductal COA in Newborn n 80% of infants with preductal COA develop CHF by 3 months of age! n Symptoms of CHF: poor feeding, poor weight gain, dyspnea in first 2-6 weeks of life.

20 Case 2: Two week old with murmur n Two week old infant, who you saw as a newborn with normal exam, now is noted to have a heart murmur. n PMI LSB, not hyperdynamic n pulses equal all extremities n No HSM

21 Case 2: Cardiac exam n Grade III/VI holosystolic murmur at LLSB

22 Case 2: What would you like to do next? n EKG: LVH,LAH n Blood pressures all four extremities n Xray: look for cardiomegally and increase in pulmonary vascularity n oxygenation n Echo: VSD

23 Ventricular Septal Defect (VSD) n The most common form of congenital heart disease: 20-25% n may be located in different anatomical locations n may be associated with many other cardiac defects ( in many cases essential for survival) n may be small or large (can cause CHF)

24 Case 3: One day old infant n One day old infant with tachypnea and cyanosis n Gen: cyanotic n RR:65, HR 140, BP 40/20 n pulse ox on RA: less than 80

25 Case 3: Respiratory and Cardiac n Lungs clear, no retractions, RR rapid n Cardiac: PMI at LSB n pulses palpable all extremities n S2 single and loud. No heart murmur.

26 What would you like to do? n ABG: before oxygenation n Give 100% O2 and then repeat ABG n EKG, 4 extremity BP n CXR

27 Results of ABG n Before O2: PH: 7.15,PaCO2 30 and Pa O2 40 n After 100% O2: PH: 7.12, PaCO2 25 and PaO2 50

28 Transposition of Great Arteries

29 n 5% of all congenital heart defects n Aorta arises anteriorly from RV, PA arises posteriorly from LV n defects (VSD,ASD,PDA) that permit mixing of the two circulations are necessary for survival

30 TGA: n ABG:hypoxemia is unresponsive to O2 n EKG: RVH n X-rays: egg-on-a- string silhouette n cardiomegally with increased pulmonary vascularity n Echo: PA from LV n associated anomalies:VSD,ASD,PDA

31 Immediate Treatment n Prostaglandin E1 to reopen PDA n Oxygen n cardiology/surgery referral

32 DDX of Cyanotic Heart Dz n Transposition of the Great Arteries n Tetralogy of Fallot n Total Anomalous Pulmonary Venous Return n Tricuspid Atresia n Pulmonary Atresia n Truncus Arteriosus

33 Other congenital cyanotic defects n Ebsteins anomaly n single ventricle n Double-outlet right ventricle (depends on associated defects…if cyanotic or not)

34 Tetralogy of Fallot

35 Tetralogy of Fallot (TOF) n Large VSD n RV outflow obstruction n right ventricular hypertrophy n overriding of the aorta

36 Tetralogy of Fallot n 10% of all congenital heart defects n The MOST COMMON CYANOTIC cardiac defect beyond infancy

37 TOF: physical exam n Varying degrees of cyanosis and clubbing n systolic thrill LSB n S2 single with ejection click n loud III-V/VI SEM LSB n continuous murmur of PDA

38 TOF n EKG: RAD, RVH n Xray: boot-shaped heart (hypoplastic MPA) n Echo: image of four defects associated

39 Tetralogy of Fallot

40 TOF complications n Hypoxic spells n growth retardation with severe cyanosis n brain abscess and CVA n infective endocarditis n polycythemia

41 Hypoxic Spell ( TET Spell n Paroxysm of hyperpnea (rapid and deep) n irritability and prolonged cry n increasing cyanosis n decreased intensity of heart murmur n (may lead to limpness, convulsion,CVA or death)

42 Treatment of TET Spell n Knee-chest (squat )position n morphine sulfate n treat acidosis n oxygenation

43 Total Anomalous Pulmonary Venous Return n One percent of all congenital heart defects n Defect: no direct communication between the pulmonary veins and the left atrium (they can drain: supracardiac,cardiac,infracardiac or mixed)

44 Total Anomalous Pulmonary Venous Return

45 TAPVR findings n S2 widely split and fixed n S3 or S4 gallop n SEM: III-IV/VI n middiastolic rumble at LLSB n Xrays: cardiomegally n Snowman n Echo can define anatomy n EKG:RAD

46 Tricuspid Atresia n 1-2% of all congenital heart disease in infancy n tricuspid valve is absent and RV is hypoplastic n associated defects of VSD,ASD or PDA are necessary for survival

47 Tricuspid Atresia

48 Tricuspid Atresia: findings n Exam: cyanosis n S2 single, often syst murmur of VSD, and occ of PDA present n early CHF

49 Tricuspid Atresia n EKG: superior QRS between O and -90 n LVH n Pulmonary vascularity is decreased n Echo: defines minimal RV, and large LV

50 Pulmonary Atresia n Less than 1% of congenital heart diseases n valve is atretic, RV cavity is hypoplastic n need other defects: ASD,PDA for survival

51 Pulmonary Atresia

52 Pulmonary Atresia: findings n PE: S2 is single n murmur of PDA n EKG: normal axis,LVH n Xray: decreased pulmonary vascularity n Echo:atretic pulmonary valve and hypoplastic RV

53 Pulmonary Atresia n Prostaglandin E1 n cardiac surgery

54 Truncus Arteriosus n Less than 1% of all congenital heart Dz n Only a single arterial trunk leaves the heart and gives rise to the pulmonary, systemic and coronary circulations n large VSD is always present

55 Truncus Arteriosus n PE: cyanosis n wide pulse pressure and bounding pulses n harsh VSD murmur LSB n EKG:normal axis,LAH n Xrays: cardiomegally and increased pulm vascularity n 50% R aortic arch n Echo:single great artery,VSD

56 Hypoplastic Left Heart

57 n Hypoplastic left heart syndrome refers to underdevelopment of the left side of the heart. n This syndrome may include: –Small aorta: This is the major blood vessel from the left ventricle to the body.

58 Hypoplastic Left Heart May Include: –Aortic valve atresia (absence): This valve normally opens and closes to let blood flow from the left ventricle to the aorta. When atresia is present, there is no connection between the left ventricle and aorta, and no forward blood flow. – –Mitral valve stenosis or atresia: This valve normally opens and closes to let blood flow between the left atrium and left ventricle. Stenosis causes little blood flow; atresia causes no blood flow. Either atresia or stenosis may be present.

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