Truncus arteriosus occurs when the aorta and pulmonary artery arise as one common trunk from both ventricles, often accompanied by a VSD and cyanosis. Treatment involves surgically restructuring the vessels. Ebstein anomaly is a rare congenital heart defect characterized by downward displacement of the tricuspid valve, causing right heart enlargement and cyanosis. Hypoplastic left heart syndrome involves severe underdevelopment of the left heart structures. Pulmonary stenosis can be valvular, subvalvular, or supravalvular and causes obstruction of blood flow from the right ventricle to the lungs. Congestive cardiac failure results from various heart defects or diseases impairing the heart's ability to pump

1. Truncus Arteriosus
Truncus arteriosus occurs when both aorta and pulmonary artery
arise as one trunk which arises from both right and left
ventricles. There is usually an accompanying high VSD & mild
to moderate cyanosis and H.F.
Treatment involves restructuring the common trunk to create
separate vessels.
Ebstein Anomaly
"E.A."
E.A. is a rare form of neonatal cyanosis, characterized by
downward displacement of the abnormal tricuspid valve into the
right ventricle (atrialization of the upper part of the right
ventricle) making the right atrium to be hugely dilated.
Right ventricle is also dysplastic,and shows some degrees of
outflow obstruction resulting in right to left shunt through ASD
or foramen ovale and various degrees of cyanosis.
Mild forms of E.A. may be asymptomatic.
Neonates with severe E.A. show; deep cyanosis, huge
cardiomegaly, C.C.F. and a pan-systolic murmur.
X.ray shows a hugely enlarged box shaped heart.
ECG shows characteristic changes of RBBB, P- pulmonale, P-
mitrale and prolonged P-R with or without WPW.
Echo, catheterization and angiography are confirmatory.
Prognosis in severe forms is very bad as there are very few
surgical options.
PGE1 and O2 may help.
Hypoplastic left heart syndrome
It is also called aortic atresia.
This rare form of C.H.D. is characterized by severe hypoplasia
of mitral and aortic valves, left ventricle and the aortic arch, a
PDA is the only source of systemic blood flow.
Very few survive the 1st week of life.
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2. Pulmonary stenosis
"P.S"
P.S may be valvular, subvalvular (infandibular) or supravalvular
(main pulmonary artery, the right or left branches).
Most cases are mild, asymptomatic and diagnosed accidentally.
Severe forms may have symptoms like dyspnea on effort and
cyanosis.
Ejection systolic murmur at ULSB is heared. X.ray in severe
forms shows cardiomegaly, oligemic lungs and prominent
pulmonary artery.
Treatment is mainly by balloon pulmonary valvuloplasty if the
right ventricular pressure is more than 75% of the systemic
systolic pressure.
Congestive Cardiac Failure
"CCF"
CCF means failure of the heart to maintain an output necessary
for the individual's body.
Causes:
1. C.H.D: e.g VSD, PDA, TGA, TAPVD, truncus arteriosus,
coarctation of aorta, severe stenosis of A.V, P.V or M.V,
hypoplastic left heart, anomalous left coronary origin,
arteriovenous malformation; etc.
2. Myocardial diseases:
-Viral myocarditis; more common during the 1st two
months of life.
-Primary myocardial disorders as:
*Glycogen storage disease.
*Endocardial fibroelastosis.
*Ischemic and other cardiomyopathies.
-Drugs as Doxorubicin(a chemotherapeutic agent).
-Hyperthyroidism.
3.Others:-Severe anemia(less than 5 gm/dl).
-Arrhythmia as SVT.
-Severe ARI.
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3. -Chronic upper airway obstruction.
-Hypertension.
-Overhydration by overloading the circulation.
Clinical Picture: This varies to some extent according to the age
and whether the failure is right sided, left sided or both.
In general features common in left failure include:
-Tachypnea and feeding difficulty
-Tachcardia
-Cough
-Wheezing
-Rhonchi and basal cripitation.
Right features include:
-Hepatomegaly
-Facial oedema
-Jagular venous engorgement (difficult to be detected in
infants)
-Leg oedema.
In addition the following are seen in failure of either side:
-Cardiomegaly
-Gallop rhythm (S3)
-Peripheral cyanosis
-Small volume pulse
-Failure to gain weight
-Irritability and excessive sweating
-Cyanosis and shock are late manifestations.
Investigations:
1-CXR helps in
-Cardiac size assessment and presence or absence of
pulmonary vascularity.
-Excluding pulmonary cause.
-Evidence of some C.H.D.
2-ECG e.g non specific ST and T changes, general amplitude,
arrhythmia, P- pulmonale, etc.
3-Echo in determining functional capacity of the heart and in
diagnosis of infective endocarditis.
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4. Management:
A-General:
1-Bed rest in "propped up" position at an angle of 45°.
2-O2 by nasal catheter, head box or in plastic tent.
3-Sedation ; using Morphine, Pethidine, Phenobarbital,
Chloral hydrate, Promethazine or Diazepam.
4-Antibiotics in presence of infection.
5-Diet ; low salt.
B-Anti failure:
1-Digoxin; increases myocardial efficiency and decreases
tachycardia. Digitalization is made during 1st 24 hours, at a
dose of: Neonates 0.04 mg/kg
1-12 months 0.08 mg/kg
1-3 years 0.06 mg/kg
> 3 years 0.04 mg/kg
Half the total dose initially, the next half of the dose is divided
into two and given with 8 hours apart. This is the oral dosage.
The parentral dose is 2/3 of the oral dose.
Maintenance is given from the 2nd day at a dose of 1/4 to 1/3 of
the digitalization dose.
2-Diuretics: specially Frusimide at a dose of 1-3 mg/kg orally
or 0.5-1.5 mg/kg I.V± Spironolactone (a potassium sparing
mildly effective diuretic) 1-4 mg/kg/day in two divided doses.
3-Angiotensin Converting Enzyme Inhibitors (ACEI); as
Captopril reduce work of the heart by causing peripheral
vasodilatation and may reduce left to right shunt.
ACEI is a potassium sparing drug, hence no need for potassium
supplements.
Both Digoxin and ACEI should be avoided in left heart outflow
obstruction.
4-Potassium supplements especially if Digoxin and
Frusimide are given.
5-Correction of the underlying cause e.g anemia, arrhythmia,
chest infection, circulatory overload, thyrotoxicosis, infective
endocarditis, hypertension, etc.
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5. 6-In refractory CCF; vasodilators as Nitroprusside, inotropic
(Dopamine, especially in presence of cardiogenic shock), or
Betablockers may be tried.
7- Heart transplant or artificial heart is another form of
treating intractable HF.
Rheumatic Fever
"R.F"
R.F. is an immunological manifestation which follows U.R.I. by
specific subtypes of group -A- beta-haemolytic streptococci, in
genetically predisposed individuals. R.F has become very rare
nowadays if compared to several decades ago.
Streptococcal skin infections do not cause R.F.
5-15 year age group is most commonly affected.
There is a latent period of 1-3 weeks between sore throat and
R.F
There is probably some antigenic similarity between
streptococcal antigens and those of joint, heart, skin etc…
Diagnosis of R.F. depends upon modified Jones criteria which
include:
Major criteria:
-Carditis
-Arthritis
-Chorea
-Subcutaneous nodules
-Erythema marginatum.
Minor criteria:
-Fever; rarely exceeds 39 Cº
-Arthralgia (only joint pain, no objective signs of arthritis)
-Elivated E.S.R, C.R.P
-Prolonged P-R interval
-Previous rheumatic fever.
R.F is diagnosed when two major or one major and two minor
criteria are present in addition to an evidence of streptococcal
infection as positive culture, increased A.S.O.T or residual
manifestations of recent scarlet fever.
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6. In cases when arthritis is taken as a major criteria arthralgia can
not be taken as a minor minor one.
As chorea is a late manifestation; other features of R.F may not
be present. Thus in absence of other causes of chorea it can
(even in absence of supportive evidences of previous
streptococcal infection) be considered as rheumatic chorea.
Similarly insidious or late onset of carditis can be considered as
rheumatic carditis provided that other causes are excluded.
-Arthritis: affects 75%, clinically migratory (flitting)
polyarthritis, mainly affecting large joints and commonly
sparing temporomandibular, sternoclavicular, interphalengeal
and spine.
The affected joint is painful, tender, swallen, shows
limitations of movements with very little if any sterile
effusion. After 2-5 days the pain and swelling may resolve,
only to appear in another joint, without any residual damage.
It is very rare to have more than one joint affected at the same
time and is also rare to have affection of only one joint.
-Carditis: occurs in 50%. It is usually pancarditis (endo, myo
and pericarditis).
Carditis is the major cause of mortality in R.F. and is the only
acute manifestation which may leave squelae.
Features of endocarditis include valvular affection by
regurgitation, most commonly mitral, less commonly aortic and
least commonly tricuspid and extremely rarely pulmonary.
In a long term even calcification and stenosis of the affected
valves may occur.
A pansystolic and sometimes a diastolic (Carey comb murmur)
are heard.
Myocarditis results in soft heart sounds and C.C.F.
Pericarditis manifests by chest pain, pericardial rub and mild
effusion.
Generally if carditis does not appear during the 1st two weeks of
R.F. it is unlikely to appear later.
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7. -Chorea (Sydenham's chorea"S.C"): is a late manifestation
occuring about three months after the onset of R.F.
S.C is 3-4 times more common in girls.
Features of S.C include purposeless, jerky movement resulting
in deranged speech, falling things, disturbed handwriting,
awkward gait, weakness, emotional liability and sometimes
depression.
Clinical tests used to detect S.C. include:
– Finger-nose test.
– Buttoning the clothes test.
– Dinner fork hand positioning.
– Pronator test.
– Milk maid grip.
– Darting tongue.
– Audible click during speech.
– Clumsy writing.
– Sustained hung-up or double knee jerk.
S.C is a self limited condition and disappears during 2-6 weeks
even without any treatment, but chorea patients are predisposed
to have carditis late in life, hence they should be given
prophylaxis to avoid further streptococcal infection.
Other causes of chorea are: choreoathetoid C.P., drugs and
Huntington's chorea.
-Subcutaneous nodules: occur over bony prominences (elbow,
shin, occiput and spine). They vary in size from few mm. to 1-2
cm. They are firm, non tender and subcutaneous in location,
remain for weeks-months and are found in < 5%.They appear
3-6 weeks after the onset of R.F and when present, they almost
indicate presence of carditis.
-Erythema marginatum: an early major skin sign which appears
very rarely and is usually undetectable in dark skinned. They are
evanescent, non pruretic, non-blanching with raised margin and
pale center. They mainly occur over trunk.
As subcutaneous nodules these rashes are also common in
patients with carditis.
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8. Prognosis of R.F.: depends on the severity of R.F. and especially
the presence of carditis or later development of infective
endocarditis.
Management of R.F:
– Bed rest; for 2-3 weeks in all, and larger period and strictly
in presence of carditis.
– 10 days antibiotics (especially penicillin) to eradicate any
streptococcal infection.
– Treatment of H.F if present.
– Anti-inflammatory drugs (the corner stone of R.F
treatment).
Aspirin and steroids are the only two anti-inflammatory
agents known to suppress rheumatic activity.
In absence of carditis Aspirin alone while in presence of
carditis use of steroid (Prednisolone) is mandatory.
Anti-inflammatory drugs are given for 12 weeks, initially at
high doses e.g. 100 mg/kg/day for Aspirin and 2 mg/kg/day
for Prednisolone and then gradually reduced.
Chorea (in presence of rheumatic activity) when mild needs
no drug treatment apart from reassurance.
Severe forms can be treated by drugs as; Phenobarbitone,
Diazepam, Chlorepromazine, Phenothiazide, Haloperidol and
sometimes steroids.
Prophylaxis: This is extremely important to prevent recurrence
of R.F.
This is provided by using long acting Penicillin which is better
to be continued for life.
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9. Infective Endocarditis
"I.E"
I.E is a form of infection (commonly caused by bacteria,
rarely fungal or others) of the endothelial surface of the heart
and forming vegetations (made of fibrin, inflammatory cells
and microorganisms).
I.E. predominantly occurs in a diseased heart (congenital or
rheumatic valvular diseases or prosthetic valves) or rarely in
an otherwise normal heart, especially during septicemic
illnesses. I.E may occur after heart surgery, especially shunt
operations.
I.E. of normal heart is usually acute, and results in acute H.F,
while I.E. of a diseased heart is subacute.
Predisposing causes include infection anywhere in the body
like boils, gingival infection, tooth abscess, ear infection,
UTI, osteomylitis, interventions as dental procedures (even
tooth brushing), cardiac catheterization, G.U procedures,
bronchoscopy and I.V. canulation.
Drug addicts using I.V. route are also prone.
The commonest organism in the past has been Streptococcus
viridance, recently Staph. aurius has also become common,
gram negatives and fungi are rare causes.
Clinical picture: any fever for more than 7 days in a patient with
a known heart disease raises the question of S.A.B.E.
Clinical manifestations can be grouped into:
1-Septic signs: fever, chills, rigors, night sweats, general
malaise, anorexia, weight loss, anemia, splenomegaly and
clubbing.
Abscesses may affect brain, kidney or bones. Right side I.E.
results in lung abscesses.
2-Cardiovascular signs: left, right or biventricular failure, a
new or changing of a pre-existing murmur, embolic
phenomena as stroke from CNS embolism, hematuria from
renal infarction, left flank pain from splenic infarction, G.I.
hemorrhage from mesenteric embolism.
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10. Evidence of vegitations and rapidly developing regurgitation
lesions of the aoric, mitral or rarely tricuspid valves.
3-Features of immune complex deposition: these present as
vasculitis, e.g:
-Microscopic hematuria, glomerulonephritis and even
acute R.F.
-Osler nodes (tender erythematous nodules over the pulp
of the finger tips).
-Roth spots (boat-shaped retinal hemorrhage with pale
center).
-Splinter hemorrhage under the nails.
-Janeway lesions are non tender erythematous patches
over the palms and the soles.
Investigations:
-Blood culture; several samples with 30 min. apart are taken for
aerobic, anaerobic and fungal culture.
-C.B.C. and E.S.R: increased total W.B.C and total neutrophils
(especially in the acute form of I.E.)
Decreased platelet, increased ESR and normocytic
normochromic anemia.
-Positive C.R.P.
-Microscopic hematuria ± albominurea.
-Hypergammaglobulinemia.
-Positive rheumatoid factor in 50%.
-Echo; shows vegitations of > 2 mm. and may detect
complications as ruptured chordae tendinae or valve cusp
perforation and mycotic aneurism.
Management of I.E:
Large doses of parentral (I.V.) antibiotics should be started in
the form of Penicillin and Gentamycin for 4-6 weeks.
Culture and sensitivity results determines the proper
combination. Complications may need emergency cardiac
surgery.
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11. Prophylaxis:
All patients with congenital or acquired heart lesions need
antibiotic prophylaxis before any surgical procedures that are
likely to initiate bacteremia.
Amoxil 50 mg/kg one hour before and 25 mg/kg 6 hours after
the procedure.
In highly risky patients I.V. Ampicillin + Gentamycin is given
before and after the procedure.
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