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A. Dodge-Khatami, MD, PhD Chief of Pediatric Cardiac Surgery

Klinik für Kinderherzchirurgie. Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency. A. Dodge-Khatami, MD, PhD Chief of Pediatric Cardiac Surgery Head of Program for Congenital Heart Disease University Heart Center – UHZ

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A. Dodge-Khatami, MD, PhD Chief of Pediatric Cardiac Surgery

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  1. Klinik für Kinderherzchirurgie Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency A. Dodge-Khatami, MD, PhD Chief of Pediatric Cardiac Surgery Head of Program for Congenital Heart Disease University Heart Center – UHZ University of Hamburg-Eppendorf School of Medicine Hamburg, Germany

  2. Klinik für Kinderherzchirurgie even rare congenital heart defects will be seen once in your careers (0.8% of all births); how should you react? most important objective: distinguish between a blue and pink patient with a murmurandunderstand why!

  3. Klinik für Kinderherzchirurgie Shunts: Location + Direction Intra or extra-cardiac? Which heart chambers are affected? Qp/Qs = pulmonary / systemic flow ratio Qp = VO2 / pulm Vv O2 – PA O2 Qs = VO2 / Vv O2 – Ao O2 In the absence of a shunt, Qp/Qs = 1

  4. Klinik für Kinderherzchirurgie Normal circulation Q = P/R Qp/Qs = 1

  5. Klinik für Kinderherzchirurgie Shunts: Direction Left >>> rightor Right >>> left? Which is more probable? Why? Left > right : PDA, ASD, VSD, AVSD, AP window, Truncus, PAPVD, TAPVD Right > left : right inflow or outflow obstruction + intra-cardiac shunt: Tricuspid atresia (TA)/Tricuspid Stenosis (TS), Pulmonary Atresia/Pulmonary stenosis, TOFallot

  6. Klinik für Kinderherzchirurgie Shunts: Direction Left >>> right : VSD Left >> right shunt Qp/Qs > 2 - 3 Pressure + Volume Overload

  7. Klinik für Kinderherzchirurgie Shunts: Physiology Left >>> right: LV volume overload Increased pulmonary flow, pulmonary infections Pulmonary Hypertension (PHN), severity and degree according to shunt size Bacterial endocarditis Right >>> left: RV pressure overload + strain Cyanosis Polyglobulia

  8. Klinik für Kinderherzchirurgie Shunts: Treatment Left >>> right: volume restriction, diuretics, inotropes, permissive hypercapnea ventilation (hypoventilation), shunt closure Right >>> left: hydration, (transfusion), hyperventilation, increase pulmonary blood flow +/- shunt closure

  9. Klinik für Kinderherzchirurgie Shunts: Operative Indications L >> R: Symptoms: tachycardia, tachypnea, hepatomegaly, sweating during feeds, failure to thrive Qp:Qs > 1.5 Aortic valve prolapse +/- insufficiency R >> L: cyanosis, RVH + strain

  10. Klinik für Kinderherzchirurgie 5 most common congenital heart defects?

  11. Klinik für Kinderherzchirurgie 5 most common congenital heart defects? Ventricular Septal Defect (VSD) 30% Patent Ductus Arteriosus (PDA) 10% Coarctation (coA) 5-8% Atrial Septal Defect (ASD) ~ 8% Tetralogy of Fallot (TOF) 5-10%

  12. Klinik für Kinderherzchirurgie case: blue child (10 years old) with a murmur (where?) auscultation: holosystolic murmur at precordium saturations: ?Cyanosis: centralorperipheral? Central: intracardiac shunt + obstruction to pulmonary blood flow Peripheral: Chronic Pneumonia, Chronic Interstitial Lung Disease, Pulmonary Neoplasia, Circulatory Collapse (+Peripheral Vasoconstriction) next step ? „Hippocratic fingers“- Clubbing

  13. Klinik für Kinderherzchirurgie x-ray: differential diagnosis?

  14. Klinik für Kinderherzchirurgie x-ray: prominent central pulmonary markings black peripheral lung fields next step ?

  15. Klinik für Kinderherzchirurgie echocardiography: Cardiomegaly, biventricular dilatation + hypertrophy Diagnosis ?

  16. Klinik für Kinderherzchirurgie echocardiography: Cardiomegaly, biventricular dilatation + hypertrophy VSD: why is the child blue?

  17. Klinik für Kinderherzchirurgie Right >> Left shunting = Cyanosis > increased cellularity (muscular and interstitial) >> fixed pulmonary vascular resistance = Eisenmenger syndrome

  18. Klinik für Kinderherzchirurgie Patent Ductus Arteriosus(PDA)

  19. Klinik für Kinderherzchirurgie Patent Ductus Arteriosus(PDA) • continuous „machinery“ murmur • LV hypertrophy + LA dilatation • Increased pulmonary vascular markings, interstitial pulmonary edema • failure to thrive • recurrent upper respiratory infections • fatigue with exertion • tachypnea, tachycardia, heart failure

  20. Klinik für Kinderherzchirurgie Portsmann, 1967 Patent Ductus Arteriosus(PDA) R. Gross, Boston, 1938

  21. Klinik für Kinderherzchirurgie Coarctation(coA)

  22. Klinik für Kinderherzchirurgie Coarctation(coA) • bi-modal presentation: • newborns in cardiovascular shock: ductal-dependent (PGE1) • vs. • „asymptomatic“ hypertensive children: headaches, epistaxis

  23. Klinik für Kinderherzchirurgie Coarctation(coA) • mid-systolic murmur in the back, systolic or continuous murmurs on the lateral chest walls (collaterals), diminished femoral pulses • Left Ventricular hypertrophy, myocardial infarction • circle of Willis aneurysms, aortic aneurysms, aortic dissection, aortic rupture • average age at death ~ 35 years if untreated : congestive heart failure (1/4), bacterial endocarditis (1/4), spontaneous rupture of the aorta (20%), intracranial hemorrhage (13%)

  24. Klinik für Kinderherzchirurgie Coarctation(coA) C. Crafoord, Stockholm, 1944 End-to-end anastomosis

  25. Klinik für Kinderherzchirurgie Voßschulte, 1957 Patch plasty Coarctation(coA) Gross, 1951 Interposition graft Waldhausen, 1966 Subclavian flap

  26. Klinik für Kinderherzchirurgie Coarctation(coA) : results • Mortality: 4-14%, age-dependent • Complications: • hypertension, chylothorax, recurrent nerve paresis (stridor) • recurrent coA ~ 10-15% if surgery in the newborn period, >> balloon dilatation • paraplegia • aneurysm

  27. Klinik für Kinderherzchirurgie Atrial Septal Defect(ASD) • systolic murmur, fixed split second heart sound (prolonged flow time on the right – delayed closure of the pulmonary valve) • Dilated right atrium + ventricle • Pulmonary hypertensionrecurrent upper respiratory infections • atrial arrhythmia (flutter, fibrillation) • congestive heart failure • no risk of bacterial endocarditis

  28. Klinik für Kinderherzchirurgie Atrial Septal Defect(ASD) King, 1976, device closure F.J. Lewis, Minneapolis, 1952, inflow occlusion

  29. Klinik für Kinderherzchirurgie Atrial Septal Defect(ASD) J. Gibbon Jr., Rochester, father of cardio-pulmonary bypass, 1934-53

  30. Klinik für Kinderherzchirurgie Atrial Septal Defect(ASD) J. Gibbon Jr., Rochester, 1953

  31. Klinik für Kinderherzchirurgie Atrial Septal Defect(ASD) : results • Gibbon (1953): first success, followed by 5 deaths, abandonned surgery and requested a 1 year moratorium on his bypass machine… • current: mortality ~ 0%

  32. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD)most frequent CHD ~ 30%

  33. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD) • Holosystolic murmur, increased pulmonary vascularity on x-ray, • Cardiomegaly, biventricular dilatation + hypertrophy. • Dyspnea, sweating during feeding, failure to thrive. • Recurrent upper respiratory tract infections.

  34. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD) • Untreated: • 25-40% spontaneous closure > 2-3 years • endocarditis (0.3% per year) • pulmonary hypertension > pulmonary arteriolar wall thickening • increased PVR, reversal of shunt • = Eisenmenger syndrome • cyanosis (by 1-2 years of age) • death

  35. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD)

  36. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD >>> VSD) • increased cellularity (muscular and interstitial) • increased reactivity • fixed contraction • vascular wall sclerosis • >> fixed pulmonary vascular resistance = Eisenmenger syndrome

  37. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD) Cross-circulation: father as oxygenator, but potentially 200% mortality… C.W. Lillehei, Minneapolis 1954: VSD „King of Hearts: the True Story of the Maverick Who Pioneered Open Heart Surgery “, G.W. Miller

  38. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD) C.W. Lillehei, Minneapolis 1954: VSD 28/47 patients survived:~ 40% mortality

  39. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD)

  40. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD): Resultsmortality ~ 1-2%heart block > pacemaker 1-2% long-term prognosis excellent!

  41. Klinik für Kinderherzchirurgie Ventricular Septal Defect(VSD): palliation • PA banding • multiple VSDs • small baby, failure to thrive Muller / Damman, 1952

  42. Klinik für Kinderherzchirurgie Tetralogy of Fallot (TOF)most frequent cyanotic CHD ~ 10%1. Overriding Aorta2. Ventricular Septal Defect3. Right ventricular hypertrophy4. Right Ventricular Outflow Tract Obstruction (RVOTO)

  43. Klinik für Kinderherzchirurgie Tetralogy of Fallot • systolic murmur • right aortic arch (25%), „boot shape“ heart • right ventricular hypertrophy • cyanosis, tet „spells“: dynamic RVOT contraction • clubbing (after 6 months), dyspnea, exercise intolerance • brain abscess • polycythemia > pulmonary + cerebral thrombosis

  44. Klinik für Kinderherzchirurgie Tetralogy of Fallot (TOF)Palliation H. Taussig A. Blalock Baltimore, 1944, classic Blalock-Taussig Shunt = „blue baby operation“ Modified BT shunt, 1976

  45. Klinik für Kinderherzchirurgie Tetralogy of Fallot (TOF)Right Ventricular Outflow Tract Obstruction(RVOTO):- Suprapulmonary (Pulmonary Arteries)- Pulmonary Valve - Subpulmonary (Right Ventricle)Central Importance of the Pulmonary Valvedistally: Pulmonary Artery growth proximally: protect the Right Ventricle

  46. Klinik für Kinderherzchirurgie Tetralogy of Fallot(TOF): complete repair C.W. Lillehei, Minneapolis 1955: Fallot correction

  47. Klinik für Kinderherzchirurgie Tetralogy of Fallot(TOF)

  48. Klinik für Kinderherzchirurgie Tetralogy of Fallot(TOF): results • Mortality 3-5 % • Heart Block < 3%, seldom requires a pacemaker • Post-operative arrhythmia frequent • Reoperations required for: • residual VSD (seldom) • residual pulmonary valve insufficiency • residual right outflow obstruction

  49. Klinik für Kinderherzchirurgie Tetralogy of Fallot(TOF): reoperations • residual pulmonary valve INSUFFICIENCY • right ventricular volume overload + dilatation + failure • arrhythmia • better growth of pulmonary arteries? • late REOPERATION • residual right outflow STENOSIS • right ventricular pressure overload • pulmonary artery stenosis/hypoplasia • late REOPERATION

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