cyanosis or congestive heart failure in children: murmurs of shunts, stenosis, and insufficiency a....

Cyanosis or Congestive Heart Failure in Children:

Murmurs of Shunts, Stenosis, and Insufficiency

A. Dodge-Khatami, MD, PhDChief of Pediatric Cardiac SurgeryHead of Program for Congenital Heart DiseaseUniversity Heart Center – UHZUniversity of Hamburg-Eppendorf School of MedicineHamburg, Germany

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

• 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

Normal circulation

Qp/Qs = 1Q = P/R

Shunts: Direction

• Left >>> right or Right >>> left?

Which is more probable? Why?

• Left > right : PDA, ASD, VSD, AVSD, AP window, Truncus, PAPVD,

• Right > left : right inflow or outflow obstruction + intra-cardiac

shunt: Tricuspid atresia (TA)/Tricuspid Stenosis (TS), Pulmonary

Atresia/Pulmonary stenosis, TOFallot

Shunts: Direction

• Left >>> right : VSD

Left >> right shuntQp/Qs > 2 - 3

Pressure + Volume Overload

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

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

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?

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%

• case: blue child (10 years old) with a murmur (where?)auscultation: holosystolic murmur at precordiumsaturations: ? 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?

x-ray: • prominent central pulmonary markings• black peripheral lung fields

next step ?

echocardiography:

Cardiomegaly, biventricular dilatation + hypertrophy

Diagnosis ?

echocardiography:

Cardiomegaly, biventricular dilatation + hypertrophy

VSD: why is the child blue?

Right >> Left shunting = Cyanosis

increased cellularity (muscular and interstitial)>> fixed pulmonary vascular resistance = Eisenmenger syndrome

Patent Ductus Arteriosus

• 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

R. Gross, Boston, 1938

Portsmann, 1967

Coarctation

• bi-modal presentation:

newborns in cardiovascular shock: ductal-dependent (PGE1)

„asymptomatic“ hypertensive children: headaches, epistaxis

Coarctation

• 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%)

Coarctation

C. Crafoord, Stockholm, 1944

End-to-end anastomosis

Coarctation

Voßschulte, 1957Patch plasty

Gross, 1951Interposition graft

Waldhausen, 1966Subclavian flap

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

• 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

F.J. Lewis, Minneapolis, 1952, inflow occlusion

King, 1976, device closure

J. Gibbon Jr., Rochester, father of cardio-pulmonary bypass, 1934-53

J. Gibbon Jr., Rochester, 1953

(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

most frequent CHD ~ 30%

• Holosystolic murmur, increased pulmonary vascularity on x-ray,

• Cardiomegaly, biventricular dilatation + hypertrophy.

• Dyspnea, sweating during feeding, failure to thrive.

• Recurrent upper respiratory tract infections.

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

(VSD >>> VSD)

• increased cellularity (muscular and interstitial)

• increased reactivity• fixed contraction• vascular wall sclerosis

>> fixed pulmonary vascular resistance = Eisenmenger syndrome

C.W. Lillehei, Minneapolis1954: 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…

C.W. Lillehei, Minneapolis1954: VSD28/47 patients survived:~ 40% mortality

(VSD): Results

mortality ~ 1-2%

heart block > pacemaker 1-2%

long-term prognosisexcellent!

(VSD): palliation

PA banding• multiple VSDs

• small baby, failure to thrive

Muller / Damman, 1952

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)

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

H. Taussig A. Blalock

Baltimore, 1944, classic Blalock-Taussig Shunt = „blue baby operation“

Modified BT shunt, 1976

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

C.W. Lillehei, Minneapolis1955: Fallot correction

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

• residual pulmonary valve INSUFFICIENCY

right ventricular volume overload + dilatation + failurearrhythmiabetter growth of pulmonary arteries?late REOPERATION

• residual right outflow STENOSIS

right ventricular pressure overloadpulmonary artery stenosis/hypoplasialate REOPERATION

cyanosis or congestive heart failure in children: murmurs of shunts, stenosis, and insufficiency a....

epistaxis slide

pr slide

heart failure slide

volume overload slide

subclavian flap slide

endtoend anastomosis

pulmonary flow

right shunt qpqs

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