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Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency A. Dodge-Khatami, MD, PhD Chief of Pediatric Cardiac Surgery.

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Presentation on theme: "Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency A. Dodge-Khatami, MD, PhD Chief of Pediatric Cardiac Surgery."— Presentation transcript:

1 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 Klinik für Kinderherzchirurgie

2 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 murmur and understand why! Klinik für Kinderherzchirurgie

3 Shunts: Location + Direction Intra or extra-cardiac? Which heart chambers are affected? Qp/Qs = pulmonary / systemic flow ratio Qp = VO 2 / pulm Vv O 2 – PA O 2 Qs = VO 2 / Vv O 2 – Ao O 2 In the absence of a shunt, Qp/Qs = 1 Klinik für Kinderherzchirurgie

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

5 Shunts: Direction Left >>> right or 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 Klinik für Kinderherzchirurgie

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

7 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 Klinik für Kinderherzchirurgie

8 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 Klinik für Kinderherzchirurgie

9 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 Klinik für Kinderherzchirurgie

10 5 most common congenital heart defects? Klinik für Kinderherzchirurgie

11 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% Klinik für Kinderherzchirurgie

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

13 x-ray: differential diagnosis? Klinik für Kinderherzchirurgie

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

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

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

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

18 Patent Ductus Arteriosus (PDA) Klinik für Kinderherzchirurgie

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

21 Coarctation (coA) Klinik für Kinderherzchirurgie

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

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

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

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

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

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

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

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

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

33 Ventricular Septal Defect (VSD) Klinik für Kinderherzchirurgie 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 Ventricular Septal Defect (VSD) Klinik für Kinderherzchirurgie 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 Ventricular Septal Defect (VSD) Klinik für Kinderherzchirurgie

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

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

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

39 Ventricular Septal Defect (VSD) Klinik für Kinderherzchirurgie

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

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

42 Tetralogy of Fallot (TOF) most frequent cyanotic CHD ~ 10% 1. Overriding Aorta 2. Ventricular Septal Defect 3. Right ventricular hypertrophy 4. Right Ventricular Outflow Tract Obstruction (RVOTO) Klinik für Kinderherzchirurgie

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

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

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

47 Tetralogy of Fallot (TOF) Klinik für Kinderherzchirurgie

48 Tetralogy of Fallot (TOF): results Klinik für Kinderherzchirurgie 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 Tetralogy of Fallot (TOF): reoperations Klinik für Kinderherzchirurgie 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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