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

most important objective: 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 murmur and understand why!

Shunts: Location + Direction 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

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

Shunts: Direction Klinik für Kinderherzchirurgie 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

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

Shunts: Physiology Klinik für Kinderherzchirurgie 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

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

Shunts: Operative Indications 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

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

5 most common congenital heart defects? 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%

Klinik für Kinderherzchirurgie 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 ? „Hippocratic fingers“- Clubbing

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

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

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

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

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

Patent Ductus Arteriosus (PDA) Klinik für Kinderherzchirurgie Patent Ductus Arteriosus (PDA)

Patent Ductus Arteriosus (PDA) 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

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

Klinik für Kinderherzchirurgie Coarctation (coA)

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

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%)

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

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

Coarctation (coA) : results 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

Atrial Septal Defect (ASD) 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 hypertension recurrent upper respiratory infections atrial arrhythmia (flutter, fibrillation) congestive heart failure no risk of bacterial endocarditis

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

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

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

Atrial Septal Defect (ASD) : results 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%

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

Ventricular Septal Defect (VSD) 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.

Ventricular Septal Defect (VSD) 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

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

Ventricular Septal Defect (VSD >>> VSD) 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

Ventricular Septal Defect (VSD) 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

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

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

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

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

Klinik für Kinderherzchirurgie 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 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

Tetralogy of Fallot (TOF) Palliation 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

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 Valve distally: Pulmonary Artery growth proximally: protect the Right Ventricle

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

Tetralogy of Fallot (TOF) Klinik für Kinderherzchirurgie Tetralogy of Fallot (TOF)

Tetralogy of Fallot (TOF): results 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

Tetralogy of Fallot (TOF): reoperations 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