D R J O S E M N U Ñ E Z

M BBS M R C P

PEDIATRIC CARDIOLOGY

• • •
 Table of Contents

Congestive Heart Failure 1

CHF occurs when oxygen delivery by the heart is insufficient to meet the metabolic
and circulatory demands of the body. 1

Ventricular Septal Defect 2

An opening in the ventricular septum in one of 4 locations: 2

Epidemiology 2

Atrial Septal Defect 4

4 types 4

Epidemiology 4

Pathophysiology 4

Patent Ductus Arteriosus 6

The ductus arteriosus is a blood vessel that is present in all babies while still in the
womb that allows blood to bypass the pathway to the lungs; it allows blood to flow
from the pulmonary artery to the aorta. After birth the ductus arteriosus usually
closes within the first hours of life. On occasion, however, the ductus arteriosus does
not close on its own and this is referred to as a PDA. 6

Epidemiology 6

Aortic Stenosis 8

Obstruction of the left ventricular outflow tract leads to a systolic pressure gradient
between the left ventricle and the aorta 8

Pathophysiology 8
O r g a n i z a t i o n N a m e P r o p o s a l Ti t l e

i
2. Aortic stenosis with thickened and fused valve leaflets preventing full
opening during systole. 9

Pulmonary Stenosis 10

Coarctation of the Aorta 12

Narrowing of the aorta, most commonly just beyond the origin of the left subclavian
artery 12

Tetralogy of Fallot 14

A form of cyanotic CHD resulting from a malaligned infundibular septum and char-
acterized by: 14

Transposition of the Great Arteries 16

A form of cyanotic CHD whereby the aorta arises from the RV and the pulmonary
artery arises from the LV. 16

Kawasaki Disease 18

An acute febrile vasculitis of childhood defined by 5 days of fever and four of the
following: conjunctivitis, mucous membrane changes, peripheral extremity changes,
polymorphous rash, cervical adenopathy. 18

Rheumatic Fever 20

Post -Infectious sequelae of group A streptococcal pharyngitis(GAS). Diagnosis is

based on the Jones criteria - 2 major OR 1 major and 2 minor criteria PLUS evidence
of prior GAS infection. 20

O r g a n i z a t i o n N a m e P r o p o s a l Ti t l e

ii
 Congestive Heart Failure
CHF occurs when oxygen delivery by the heart is insufficient to meet the metabolic and circulatory demands of the
body.

Etiology

• Congenital cardiac causes of CHF: AVSD, Coarctation of aorta, critical AS or PS, PDA, TGA, tricuspid atresia, hy-
poplastic left heart syndrome, truncus arteriosus, VSD, TAPVR.

• Acquired cardiac causes of CHF: arrhythmias, Kawasaki disease, viral myocarditis, rheumatic heart disease, meta-
bolic disorder, muscular dystrophy, chemotherapy, dilated cardiomyopathy

• Non-cardiac causes of CHF: acute hypertension, anemia, hyperthyroidism, obstructive sleep apnoea

Clinical manifestations

• Infants: failure to thrive, increased work of breathing, feeding difficulties, excessive sweating

• Children and adolescents: shortness of breath, reduced exercise tolerance, peripheral oedema, cough, orthopnea

• On examination: tachypnoea, tachycardia, hepatomegaly, gallop rhythm, puffy eyelids, swollen feet, crepitations

Diagnosis

• Chest xray: helps assess degree of cardiomegaly and pulmonary oedema

• ECG: may demonstrate cardiomegaly or rhythm disturbance

• Echocardiography: helps define congenital heart defects, ventricular size, ventricular function, and fractional
shortening (normal 28 to 40%)

Management

• General measures: oxygen, treatment of precipitating factors such as anemia, fever, infection, hypertension and
arrhythmias; nurse in cardiac position

• Diuretics: loop diuretics e.g.. frusemide are considered first line therapy for CHF. Electrolyte abnormalities are
common ( hypokalemia, hypochloremia)

• thiazide diuretics e.g.. chlorothiazide work at the distal tubules and are used to complement loop diuretics

• spironolactone is potassium sparing and is often used in combination with above diuretics

• Digoxin increases cardiac contractility; toxicities include bradycardia, heart block and ventricular arrhythmias.

• Afterload reducing agents: reduction in afterload results in increased stroke volume and improved cardiac output;
ACE inhibitors e.g. Enalapril, reduce peripheral vascular resistance, may also have an effect on myocardial remod-
elling

• intravenous inotropic agents along with positive pressure ventilation are reserved for ICU patient

P e d i a t r i c C a r d i o l o g y

1
 Ventricular Septal Defect
An opening in the ventricular septum in one of 4 locations:

1. Perimembranous: a defect involving the membranous septum beneath the aortic valve; 70% of VSDs.

2. Muscular (or trabecular): a defect within the muscular septum between the the felt and right ventricles; often in-
volves multiple, small defects which may be difficult to repair surgically; 5 to 20% of VSDs.

3. Outlet (supracristal, subpulmonary, conoseptal, subarterial): a defect beneath the pulmonic valve which communi-
cates with the RV outflow tract; 5 to 7% of VSDs.

4. Inlet: located posteriorly and inferiorly to perimembranous VSDs; 5 to 8% of VSDs; often associated with endo-
cardial cushion defects.

VSDs are classified as tiny, small, moderate, large or very large; small VSDs are less than 3mm, large VSDs are about
the same size as the aortic valve annulus (10mm in a newborn).

Epidemiology

• Two to six per 1000 live births; 25% of congenital heart disease

• Most common form of CHD

Pathophysiology

• small defects (restrictive) are not usually hemodynamically significant.

• large defects (unrestricted) allow significant left to right shunting( more than 2:1) which may lead to pulmonary
overcirculation, compromise of systemic cardiac output and CHF.

• Large unrepaired defects often lead to pulmonary vascular obstructive disease and Eisenmenger’s syndrome.

• Complications include pulmonary hypertension, RV outflow tract obstruction, aortic regurgitation and endocardi-
tis.

Clinical Features

• may be asymptomatic

• symptomatic VSDs often present at 4 to 6 weeks of age as pulmonary vascular resistance decreases after birth

• may present with feeding difficulties, poor growth, dyspnoea excess sweating especially when feeding, fatigue,
CHF

• VSDs are often silent in the newborn period when pulmonary vascular resistance is similar to systemic and there-
fore with little shunting

• Physical exam findings vary depending on size and location of the VSD as well as the degree of pulmonary vascu-
lar resistance

P e d i a t r i c C a r d i o l o g y

2
• may include a loud, harsh, blowing pan systolic murmur at the lower left sternal border (LLSB), palpable thrill at
LLSB with parasternal lift and apical thrust

• small defects may be associated with loud murmurs

Diagnosis

• chest X-ray may be normal or may reveal cardiomegaly and increased pulmonary vascular markings

• ECG may be normal or may reveal evidence of LVH, LAH and biventricular hypertrophy

• Echocardiography reveals the size and location of the VSD

• Cardiac catheterization may be performed in complicated cases.

Management

Medical

• small VSDs are often well tolerated

• Small muscular VSDS are most likely to close spontaneously

• If signs of CHF, consider diuretics, ACE inhibitors and digoxin

• Endocarditis prophylaxis recommended.

Surgical

• Indications for surgery include uncontrolled CHF, failure to thrive despite optimum medical therapy and signifi-
cant left to right shunting ( pulmonary: systemic flow ratio , Qp:qS, greater than 2:1.

• direct surgical repair under cardiopulmonary bypass may incorporate a Dacron patch

• pulmonary artery banding is usually reserved for complicated cases and premature infants as a palliative proce-
dure

• surgical complications include cardiac dysrhythmias (especially RBBB), residual shunt, myocardial dysfunction,
respiratory complications and CNS complications.

• VSD

P e d i a t r i c C a r d i o l o g y

3
 Atrial Septal Defect
4 types

1. Ostium secundum: located at the site of the foramen ovale; 50 to 70% of all ASDs

2. Ostium primum: located ;low in the septum, associated with a cleft in mitral valve

3. Sinus venosus: located high in the septum near the SVC, associated with anomalous pulmonary venous return

4. ASD of the IVC; least common.

Epidemiology

• 5 to 10% of CHD

• female: male ratio, 2:1

• 1 in 1500 live births

Pathophysiology

• small defects may close spontaneously often before 3 years of age

• magnitude of left to right shunt depends on size of defect and pulmonary and systemic vascular resistance

• may led to right atrial and right ventricular volume overload with RV dysfunction; rarely pulmonary hypertension
develops.

Clinical Features

• Often asymptomatic; may present until adulthood

• may present as heart murmur in 3 to 5 year old patients

• occasionally presents in childhood with fatigue, dyspnoea, recurrent respiratory infections and CHF

• CHF is more common if the ASD is associated with another defect

• Atrial arrhythmia including fibrillation and SVT are more common in adults

• PHT is more common in adults

• paradoxical emboli may occur

• on examination: S1 is loud or normal, S2 is widely split and fixed, prominent right ventricular cardiac impulse,
ejection systolic murmur at the left upper sternal border (LUSB), diastolic murmur at LLSB may represent signifi-
cant flow across the tricuspid valve if shunt is greater than 2:1.

Diagnosis

• Chest X-ray may show right atrial enlargement, RVH, dilated pulmonary artery and increased pulmonary vascular
markings

P e d i a t r i c C a r d i o l o g y

4
• ECG may show right axis deviation, RVH and partial RBBB (RSr1 in V1, V2)

• echocardiography reveals location, size and any associated anomalies

• Cardiac catheterization may be used to confirm presence of the defect and determine Qp:Qs ratio

• TOE or bubble test may be useful.

Management

Medical

• CHF is managed with digoxin and diuretics

• occasional arrhythmia may require medical management

• secundum ASDs close spontaneously in ~40% of patients

Surgical

• usually recommended if Qp:Qs ratio is greater than 2:1, patient is symptomatic or ASD is moderate to large in size

• uncomplicated ASDs are often closed between 2 to 4 years of age

• traditionally the defect is sutured or a patch is applied under cardiopulmonary bypass

• recently, a variety of closure devices that are deployed by transvenous catheterization have been used.

ASD
3 delivery catheter
4 Amplatzer ASD occluder
5 right atrial side deployed
6 device deployed on atrial septum

P e d i a t r i c C a r d i o l o g y

5
 Patent Ductus Arteriosus
The ductus arteriosus is a blood vessel that is present in all babies while still in the womb that allows blood to by-
pass the pathway to the lungs; it allows blood to flow from the pulmonary artery to the aorta. After birth the ductus
arteriosus usually closes within the first hours of life. On occasion, however, the ductus arteriosus does not close on
its own and this is referred to as a PDA.

Epidemiology

While this condition is much more often seen in premature babies, it may also appear in term infants.

Pathophysiology

• The symptoms of a PDA depend on the size of the ductus and how much blood flow it carries. After birth, the
pressures and resistance are much tighter in the aorta than the pulmonary artery, so if a ductus arteriosus is pre-
sent, blood will flow from the aorta into the pulmonary artery. This extra blood flow into the lungs can overload
the lungs and put an additional burden on the heart to pump this extra blood.

• This situation may not be well tolerated in a premature baby who already has problems related to immaturity of
the lungs themselves. These babies may need more support from the ventilator and have symptoms of congestive
heart failure.

Clinical Features

• A newborn with a patent ductus arteriosus, may have fast breathing, an increase in the work of breathing, more
frequent respiratory infections, tiring more easily, or poor growth.

• However, if the patent ductus arteriosus is not large, it may cause absolutely no symptoms at all and may be de-
tected only upon further evaluation of a heart murmur.

• A PDA typically causes a continuos, machinery type murmur best heard at the left infra clavicular area. It is a as-
sociated with a wide pulse pressure, a collapsing pulse and easily palpable peripheral pulses.

• Even in the absence of symptoms, the turbulent flow of blood through the patent ductus arteriosus puts a person
at a higher risk for endocarditis.

Diagnosis

• The presence of the characteristic murmur along with symptoms of heart failure in a premature infant most fre-
quently leads to the diagnosis of patent ductus arteriosus.

• The chest x-ray will show an enlarged heart and evidence of an excessive amount of blood flow to the lungs.

• An echocardiogram is performed to confirm the diagnosis. This will demonstrate the size of the ductus arteriousus
and will demonstrate if the heart chambers have become enlarged due to the extra blood flow.

• In older children, though, the chest X-ray is typically normal. An echo will demonstrate the flow of blood through
the patent ductus arteriosus and will typically be performed to confirm the diagnosis.

P e d i a t r i c C a r d i o l o g y

6
Management

• In a newborn, the PDA still has the potential to close on its own without intervention. Thus additional time may be
allowed for the PDA to close on its own if the heart failure can be easily managed. If symptoms are severe, such as
in a premature infant, or if it is felt unlikely to close on its own, however, medical or surgical closure is pursued.

• If a PDA is still present beyond the newborn period, it will generally never close on its own. Closure is recom-
mended in such cases to prevent the future risk of endocarditis.

• In newborns, a medication such as indomethacin or ibuprofen can be given. These medications can constrict the
muscle in the wall of the PDA and promote closure. These drugs do have side effects, however, such as kidney in-
jury or bleeding, so not all babies can receive them. Because of the potential side effects, the baby must have lab
values checked before medications can be given. If the lab values are not normal or if the medications do not work,
surgery can be performed and the PDA tied off (ligated).

• Medications are generally only successful in newborn patients. In older infants and children, options for closure
include surgery or closure in the cardiac catheterization laboratory with a device or coil.

• During the cardiac catheterization procedure, 2 methods can be used to close the ductus. If it is small, a coil may be
placed within the vessel which will expand to block the blood flow. If the ductus is larger, a flexible device can be
placed within the ductus as a "plug".

• The ductus arteriosus may also be closed with surgery,. For surgery, a small incision is made between the ribs on
the left side and the ductus arteriosus is ligated and cut. Surgical closure of the patent ductus arteriosus can be per-
formed at any age, and is specifically recommended in some situations such as a very large patent ductus arterio-
sus or other unusual anatomy.

• The risk of complications with any of these treatments is low, determined mostly by how ill the child is prior to
treatment.

2 PDA ligated
1 PDA

P e d i a t r i c C a r d i o l o g y

7
 Aortic Stenosis
Obstruction of the left ventricular outflow tract leads to a systolic pressure gradient between the left ventricle and
the aorta

• Aortic valve stenosis: most common form of aortic stenosis; frequently due to bicuspid aortic valve; identified in up
to 2% of adults

• Subvalvular stenosis: due to fibro-muscular ring or shelf below the aortic valve; may be associated with malaligned
VSD or aortic coarctation

• Supravalvular stenosis: least common form of AS; may be localized or diffuse; may be associated with William’s
syndrome.

Epidemiology

• 3 to 6% of CHD; male:female ratio 4:1

Pathophysiology

• Critical AS: high pressure gradient across the aortic valve may result in LV failure, low cardiac output and pulmo-
nary edema

• As cardiac output increases with growth during childhood, the pressure gradient may increase

• Abnormal diastolic filling is due to LV hypertrophy

Clinical Features

• often asymptomatic in infancy

• symptoms depend on severity and location of obstruction

• may present with irritability, paleness, tachycardia, tachypnea, retraction, rales

• CHF is most common on neonates with critical disease or in adults with untreated disease

• valve calcifications may lead to worsening diseases in adults

• occasional ventricular arrhytymias and sudden death may occur

• on exam: early ejection click at the apex, harsh ESM at the base radiates to the neck, palpable LV lift, precordial
systolic thrill at base

Diagnosis

• Chest x-ray may show evidence of LV hypertrophy

• ECG may show LVH or LV strain in severe disease

• Echo may define anatomy and hemodynamic severity of the lesion

P e d i a t r i c C a r d i o l o g y

8
• Cardiac catheterization is occasionally used to establish severity and measure pressure gradient across aortic valve

Management

Medical

• Prostaglandin E1 dilates the PDA to increase systemic circulation in severely ill neonates

• digoxin: consider for patients with poor cardiac output or LV hypertrophy

• Antibiotic prophylaxis for endocarditis is necessary during invasive procedures

• Exercise avoidance is mandatory in severe disease

Surgical

• Aortic valve stenosis: balloon valvuloplasty, surgical valvotomy, aortic valve replacement with prosthetic valve,
homograft or pulmonary autograft (Ross procedure)

• Sub-aortic stenosis: surgical resection of fibromuscular shelf or ring

• Supravalvular: prosthesis to widen or repair stenotic segment

2. Aortic stenosis with thickened and fused valve leaflets preventing full opening during systole.
3. Fusion of a commissure in a malformed, bicuspid aortic valve

P e d i a t r i c C a r d i o l o g y

9
 Pulmonary Stenosis
Pulmonary stenosis is a condition characterized by obstruction to blood flow from the right ventricle to the pulmo-
nary artery.

• This obstruction is caused by narrowing or stenosis at one or more of several points from the right ventricle to the
pulmonary artery.

• It includes obstruction from thickened muscle below the pulmonary valve, narrowing of the valve itself, or narrow-
ing of the pulmonary artery above the valve.

• The most common form of pulmonary stenosis is obstruction at the valve itself, referred to as pulmonary valvar
stenosis.

• Most commonly with pulmonary valvar stenosis, the pulmonary valve leaflets are thickened and fused together
along their separation lines (commissures).

Epidemiology

Pathophysiology

• When the pulmonary valve is obstructed, the right ventricle must work harder to eject blood into the pulmonary
artery leading to RVH.

• RVH, is rarely a problem in itself but instead is an indication that significant valve obstruction exists.

• When the pulmonary valve is severely obstructed, especially in newborns with critical degrees of pulmonary
stenosis, the right ventricle cannot eject sufficient volume of blood flow into the pulmonary artery resulting in right
to left shunting at the atrial level and the baby being cyanosed.

• Right ventricular failure rarely occurs with pulmonary valve stenosis.

Clinical Features

• Children with pulmonary valvar stenosis are usually asymptomatic and in normal health.

• An ESM best heard at the LUSB, left upper sternal border with radiation to the lung fields. There is often an associ-
ated click when the thickened valve snaps to its open position.

• Symptoms occur only with severe pulmonary valve stenosis.

• A newborn with critical pulmonary valve stenosis develops cyanosis in the first few days of life. This is due to di-
minished volume of blood flow into the lungs, together with a shunt of blue blood from right to left atrium.

• A newborn with critical pulmonary stenosis presents an emergency situation that requires immediate treatment,
either balloon dilation of the valve or surgery.

• In an older child, severe pulmonary valve stenosis may cause easy fatigue or shortness of breath with physical ex-
ertion. Severe pulmonary valve stenosis rarely results in right ventricular failure or sudden death.

•
P e d i a t r i c C a r d i o l o g y

10
Diagnosis

• The ECG is typically normal in the presence of mild pulmonary stenosis. With moderate-to-severe pulmonary
stenosis the ECG may show RVH.

• The Echocardiogram accurately documents that the obstruction is present at the valve level and Doppler studies
are used to estimate the degree of obstruction.

• The echocardiogram is also important to exclude other problems that may be associated with pulmonary stenosis,
such as an ASD or VSD.

• Cardiac catheterization enables accurate measurement of the degree of pulmonary stenosis that is present. During
cardiac catheterization, pressure measurements are made above and below the valve to define the amount of ob-
struction and motion pictures are taken to visualize the pulmonary valve.

• Cardiac catheterization is rarely needed to make the diagnosis but, instead, is typically done to perform a balloon
dilation procedure described below.

Management

• Children with mild pulmonary valve stenosis rarely require treatment. Patients with mild pulmonary valve steno-
sis are healthy, can participate in all types of physical activities and sporting events, and lead normal lives.

• Mild pulmonary valve stenosis in childhood rarely progresses after the first year of life. However, mild pulmonary
stenosis in a young infant may progress to more severe degrees and requires careful follow-up.

• Children with moderate-to-severe degrees of pulmonary stenosis require treatment, the timing of which is often
elective.

• The type of treatment required depends on the type of valve abnormality present. Most commonly, the stenotic
pulmonary valve is of normal size, and the obstruction is due to fusion along the commissures or lines of valve
leaflet opening.

• This "typical" form of pulmonary valve stenosis responds very nicely to balloon dilation. Balloon dilation valvulo-
plasty is performed at the time of cardiac catheterization and does not require open heart surgery.

• In the newborn, balloon dilation for critical pulmonary valve stenosis can be a technically challenging procedure as
these newborns are often critically ill and unstable.

• More typically, in older children the procedure is performed electively on an outpatient basis.

• Open heart surgery is required for more complex valves, where balloon dilation is not sufficient therapy. These
valves may be obstructed by thick and dysplastic leaflet tissue (such as in patients with Noonan syndrome), and
the diameter of the valve itself may be small in some cases.

• For these conditions surgical pulmonary valvotomy , partial valvectomy and possibly a transannular patch (patch
from the right ventricle to pulmonary artery) may be required during the open-heart surgery repair.

P e d i a t r i c C a r d i o l o g y

11
 Coarctation of the Aorta
Narrowing of the aorta, most commonly just beyond the origin of the left subclavian artery

Epidemiology

• 5 to 8% of CHD, M:F ratio 2:1

• often associated with Turner syndrome, XO

• may occur in isolation or in association with other CHD defects

Pathophysiology

• degree of symptoms depends on severity of coarctation

• LV outflow tract obstruction leads to LV hypertrophy and increased systolic pressure

• decreased blood flow to lower extremities may occur especially after closure of PDA

• in severe disease, LV dysfunction may lead to pulmonary edema and low output cardiac failure

• an extensive collateral circulation may develop in later life

Clinical Features

• neonates may present in the first 3 weeks of life with tachypnea, poor feeding, diaphoresis, CHF, cardiogenic shock
and decreased or absent femoral pulses

• older children may have upper extremity hypertension and claudication

• on exam: decreased or absent femoral pulses, systolic murmur at left sternal border between 3rd and 4th intercostal
space radiating to the left infra-scapular area, ejection click if bicuspid aortic valve present

Diagnosis

• Four extremity BPs show differential of more than 10mm Hg between upper and lower extremities

• Chest x-ray may show rib notching in children over 6 years of age

• ECG may show LVH and possible LAH in older children, RVH in neonates

• Echo reveals segment of coarctation and any associated anomalies

• Cardiac catheterization may be performed to delineate affected segment and identify collateral circulation

• MRI or CT scan may help define lesion and identify collateral vessels, useful for serial follow-up

Management

• Medical

• in severely affected neonates, PGE1 maintains patency of ductus to help provide distal perfusion
P e d i a t r i c C a r d i o l o g y

12
• Digoxin and diuretics as necessary for CHF

• rebound hypertension is common in the immediate post-op period and may require antihypertensive medication

Surgical

• timing depends on age of diagnosis, severity of disease and related defects

• multiple approaches include balloon angioplasty, end-to-end anastomosis, subclavian flap repair, prosthetic patch,
bypass graft and endovascular stent.

1 coarctation
2 coarctation repair

P e d i a t r i c C a r d i o l o g y

13
 Tetralogy of Fallot
A form of cyanotic CHD resulting from a malaligned infundibular septum and characterized by:

1. overriding aorta

2. right ventricular outflow tract obstruction

3. malaligned VSD

4. right ventricular hypertrophy

Epidemiology

• 5 to 7% of CHD , incidence 1 in 2700

• 15% of patients with TOF have DiGeorge syndrome; 50% of DiGeorge syndrome have TOF

Pathophysiology

• Pulmonary valve annulus has variable size and helps determine degree of RVOTO

• Severity of symptoms is determined by degree of ROTO related to subvalvular pulmonary stenosis and right to left
shunting; degree of shunt depends on pulmonary vascular resistance, systemic vascular resistance and presence of
PDA

• VSD is usually large and unrestrictive

• mild cases may have imperceptible cyanosis ( “pink tet”)

Clinical Features

• cyanosis in most patients by 1 year of age, dyspnea with exertion, clubbing

• tendency to assume knee to chest or squatting position

• paroxysmal hypercyanotic attacks or TET spells

• on exam: RV impulse and systolic thrill palpable along left sternal border, harsh ESM at left sternal border, single
2nd heart sound

Diagnosis

• Chest x-ray shows “boot-shaped” heart, clear lung fields, possible right aortic arch

• ECG shows right axis deviation, RVH, dominant R wave or RSR’ pattern in precordial leads

• Echo shows overriding and enlarged aorta, aortic-septal discontinuity, narrowed RVOT, hypoplastic pulmonary
arteries, VSD

• Cardiac catheterization is often necessary to assess magnitude of right to left shunt and to define anatomy of coro-
nary arteries

P e d i a t r i c C a r d i o l o g y

14
Paroxysmal hypercyanotic attacks (TET spells)

• Characterized by the sudden onset of increased cyanosis, dyspnea and change in mental status ( often with irrita-
bility)

• due to sudden increased ratio of pulmonary to systemic vascular resistance resulting in increased right to left
shunting and reduction in pulmonary blood flow

• may lead to severe hypoxemia, metabolic acidosis and death

• onset generally between 2 and 9 months of age

Management

TET spells

• remove restrictive clothing, calm patient and place in knee-chest position

• oxygen

• morphine

• IV beta blocker or Bicarbonate may be required

Medical

• in neonates, avoid stressors such as cold and monitor glucose levels

• if RVOTO is severe, infants may depend on PDA; consider PGE1

• oral propranolol may decrease frequency and severity of TET spells

Surgical

• palliative surgery: modified BT shunt can augment pulmonary blood flow and allow pulmonary arteries to grow

• total surgical correction often done during infancy

1 RVOTO 2 RVH 3 OVERRIDING AORTA

4 VSD
7 TRANS-ANNULAR PATCH REPAIR

P e d i a t r i c C a r d i o l o g y

15
 Transposition of the Great Arteries
A form of cyanotic CHD whereby the aorta arises from the RV and the pulmonary artery arises from the LV.

• The aorta is anterior and to the right of the pulmonary artery

• in type 1 TGA, the ventricular septum is intact

• in type 2 TGA, a VSD exists

Epidemiology

• 5% of CHD, M;F ratio 3;1

• more common in infants of diabetic mothers

Pathophysiology

• TGA results in 2 parallel circuit

• to sustain life, mixing of blood must occur through an associated PDA, VSD or ASD.

• If untreated, TGA is usually fatal in the neonatal period

Clinical features

• Dyspnea and cyanosis from birth, progressive respiratory distress, feeding difficulties, CHF

• On exam: no murmur is appreciated or murmur of VSD is noted, single, loud S1

Diagnosis

• Hyperoxia test: 100% oxygen is administered for 10 minutes. If PaO2 increases above 100mm Hg, lung disease is
suspected whereas a PaO2 of less than 50 indicates cyanotic CHD. Alternatively, a PaO2 increase of less than 10 to
30 mmHg indicates CHD.

• Chest x-ray shows mild cardiomegaly, “egg-on-a-string” appearance of cardiac silhouette, pulmonary vascular
congestion

• ECG shows right axis deviation, RVH or RAH

• Echo confirms anatomy and any associated defects, helps determine degree of mixing

• Cardiac catheterization confirms defect, may be used to define coronary artery anatomy, may be accompanied by
balloon atrial septostomy to help blood mixing at atrial level

Management

Medical

• PGE1 dilates PDA in order to improve mixing

P e d i a t r i c C a r d i o l o g y

16
• sodium bicarbonate for severe metabolic acidosis

• oxygen

• digoxin

• diuretics

• iron if anaemic

Surgical

• ballon atrial septostomy (Rashkind procedure) can increase interatrial mixing, may stabilize the ill neonate before
definitive treatment

• Arterial switch operation restores left ventricle as systemic pump

• Atrial switch operation or venous switch (Mustard or Senning technique) has risk of late RV failure.

1. Aorta arising from the right ventricle. Poorly oxygenated blood is delivered to the body.
2. Pulmonary artery arising form the left ventricle. Well oxygenated blood delivered back to the lungs.
3. Coronary arteries mobilized from base of aorta in preparation for reimplantation onto pulmonary artery ("neo-
aorta").
4. The PDA has been divided and the pulmonary arteries are moved in front of the aorta for reconstruction.
5. Pericardial patch used for pulmonary artery reconstruction at the site where the coronary arteries used to be.
6. Close-up view of pulmonary artery reconstruction.
7. Close-up view of aortic reconstruction showing coronary arteries reimplanted.

P e d i a t r i c C a r d i o l o g y

17
 Kawasaki Disease
An acute febrile vasculitis of childhood defined by 5 days of fever and four of the following: conjunctivitis, mucous
membrane changes, peripheral extremity changes, polymorphous rash, cervical adenopathy.

Atypical (or incomplete) KD may present with fewer than four criteria and is more common in infants where the in-
cidence of coronary artery aneurysms can be as high as 40%.

Epidemiology

• Incidence: USA 12 per 100,000; Japan 112 per 100,000

• 80% of cases less than 5 years of age

Differential Diagnosis

• Viral; measles, EBV, adenovirus, enterovirus, parvovirus B19

• Bacterial: scarlet fever, staphylococcal scalded skin syndrome, toxic shock syndrome, Yersinia pseudotuberculosis,
typhoid fever

• Allergic: drug reaction, serum sickness, Stevens-Johnson syndrome

• Rheumatologic: systemic JRA, polyarteritis nodosa, Reiter syndrome

• Toxic: mercury poisoning

Pathophysiology

• Etiology unknown

• Vasculitis of medium-sized arteries including coronary arteries and arterioles, capillaries and venules.

• Oedema of endothelial and smooth muscle cells; inflammatory infiltration of vascular wall.

Clinical Manifestations

• Three phases: acute febrile phase (7 to 14 days), subacute phase (10 to 24 days) and convalescent phase( >24 days)

• Fever: high and spiking

• Conjunctival injection: bilateral, bulbar, non-suppurative

• Peripheral extremity changes: oedema and erythema of palms and soles, desquamation of fingers and toes after
first week

• Mucous membranes changes: injected pharynx, dry and cracked lips, strawberry tongue

• Polymorphous rash: variable, not petechial, may be macula-papular, scarlatiniform, erythema multiforme, promi-
nent in groin area

• Cervical adenopathy: 1.5cm or greater, generally unilateral, not common in Trinidad

P e d i a t r i c C a r d i o l o g y

18
• Coronary artery aneurysm (CAA): most worrisome sequelae, develops in 15 to 25% of patients not treated within
10 days of onset but in less than 5% of treated patients, more common in infants

• Other manifestations: extreme irritability, aseptic meningitis, cranial nerve palsy, pancarditis, gallbladder hydrops,
hepatitis, hepato-splenomegaly, arthralgia, arthritis, uveitis.

Diagnosis

• Elevated ESR and CRP

• CBC: WBC normal or leukocytosis (WBC >15,000 in 50%), normocytic anemia, thrombocytosis after first week

• Mildly elevated ALT, AST, GGT, Bilirubin, alkaline phosphatase

• Urinalysis: sterile pyuria in 70%

• CSF: pleocytosis with lymphocyte predominance in 25 to 50% of patients who undergo LP

• Chest-xray: may show pneumonitis, cardiomegaly

• ECG: may show arrhythmia, ischemia, low voltage

• Echocardiogram: may show coronary artery ectasia or aneurysm, pericardial effusion, valvular abnormalities, di-
minished ventricular function

• Slit-lamp examination: anterior uveitis in majority of patients(85%)

Management

• Intravenous Immunoglobulin (IVIG): 2 G/kg over 10 to 12 hours (bottles come in 6G), if still febrile after 48 hours
consider a repeat dose

• Aspirin: 80-100mg/kg/day divided into four doses; when afebrile and ESR down, decrease dose to 3-5mg/kg/
day. Discontinue aspirin if follow-up echo at 6 weeks and ESR are normal

• Steroids: consider for persistent fever after 2 doses of IVIG

• Disposition: close follow-up with pediatric cardiology; follow-up echo at 6 weeks and then 6 months if abnormal.

P e d i a t r i c C a r d i o l o g y

19
 Rheumatic Fever
Post -Infectious sequelae of group A streptococcal pharyngitis(GAS). Diagnosis is based on the Jones criteria - 2
major OR 1 major and 2 minor criteria PLUS evidence of prior GAS infection.

• Major criteria: carditis, migratory polyarthritis, chorea, erythema marginatum, subcutaneous nodules

• Minor criteria: fever, arthralgia, prolonged PR interval, elevated ESR, CRP

• Evidence of prior GAS infection: positive throat culture, positive rapid strep test or increasing antistreptolysin-O or
anti-DNAse B titers

• Exceptions to Jones criteria: chorea as sole manifestation, indolent carditis, recurrence of RF

• Should be distinguished from post-streptococcal reactive arthritis.

Epidemiology

• Most common in 5 to 15 year olds

• Carditis more common in younger children, arthritis more common in young adults

Etiology

• Usually develops 2 to 3 weeks following an untreated GAS pharyngitis

• Not caused by streptococcal skin infections

Pathophysiology

• Immune complexes may cause non-destructive synovitis and reversible reactions in basal ganglia that cause chorea

• Extracellular GAS toxin may target organs such as the heart and brain

• Autoimmunity and cell-mediated cytotoxicity may cause valvular inflammation

Clinical Manifestations

• Arthritis: migratory, typically affects knees, ankles, elbows; joints may be swollen, warm, tender with limited range
of movement; joint involvement more common and more severe in teenagers; does not cause chronic joint disease

• Subcutaneous nodules: firm and painless; over extensor surfaces of joints, occipital region, thoracic or lumbar
spinous processes

• Erythema marginatum: pink/ red blanching rash with raised borders, central clearing, not pruritic or indurated,
facial sparing, common at height of fever

• heart disease in 40 to 80% of patients: affects mitral and aortic valve more commonly; valves scar with typical “fish
mouth: abnormality or calcified tissue; early involvement can manifest as unexplained tachycardia; pericarditis
gives rubs

P e d i a t r i c C a r d i o l o g y

20
• Chorea: involuntary, purposeless movements, often unilateral; associated with muscle weakness and emotional
lability, usually disappears over weeks to months, rarely recurs

Diagnosis

• Evidence of prior GAS infection

• ESR and CRP usually elevated

• CBC: normocytic, normochromic anemia

• Joint fluid: sterile, WBC count may be in the septic range

• X-rays of affected joints; normal or effusion present

• Chest-xray: may show cardiomegaly

• ECG: may show prolonged PR interval ( 1st degree heart block), cardiomegaly

• Echocardiography: may show mitral and/ or aortic valve regurgitation, pericardial effusion

Management

• GAS infection: Penicillin 10 days oral therapy or single IM Benzathine penicillin

• Arthritis: Aspirin 90-120mg/kg/day

• Carditis: Aspirin plus steroids are indicated if heart failure is present, 2.5mg/kg/day for 2 to 3 weeks then tapering

• Bed Rest for carditis, at risk of mitral valve apparatus rupture

• Chorea: haloperidol or diazepam

• Secondary prophylaxis: Penadur IM monthly for 5 years post ARF or lifetime for rheumatic heart disease.

P e d i a t r i c C a r d i o l o g y

21