Professor of Pediatrics,
Head of Pediatric Cardiology,
Ain Shams University
Which part of the heart will be affected?
Please don’t under estimate anemia
Acute anemia causes heart failure.
Chronic anemia causes myocardial affection.
DD of Dilated Cardiomyopathy : Etiology
Idiopathic :50% Connective tissue disorders
Familial 5-10% Drugs & toxins
AD, AR Familial muscular or neuromuscular
X linked disorders
Mitochondrial Muscular dystrophies
- viral, bacterial, fungal
Intrinsic cardiac diseases
E d i
Growth hormone def Congenital (ALCAPA)
Acquired : Kawasaki
Inborn errors of metabolism hypercholesterolemia
Storage disease *End stage of HCM *Structural
Nutritional deficiency *Systemic hypertension
Selenium, carnitine, thiamine ,
Vitamin D Peripartum
Fe deficiency Anemia (no heart abnormality)
Hb of 7gm/dl for 3 months = no cardiac
h b h
changes by echo
Hb <6gm/dl for 3months = cardiac changes
appeared & were reversed by Fe intake
What about 6 months of Hb of 7gm/dl ?
How does Fe deficiency anemia affect the
It affects the cardiac performance through:
- Hb= increases the COP
- Inhibits the heme dependent cytochrome
for th iditi
enzymes necessary f the oxiditive
metabolism of the cardiac myocyte.
This leads to cardiac dysfunction from
compromised cardiac metabolism
Acute hemolytic anemia: the heart can’t stand
the anemia if prolonged
Chronic hemolytic anemia
Β Thalassemia major
Cardiac manifestations of anemia alone are seen
h 1 k f hemolysis
in the1st attacks of h l
Later the effect of iron overload on the heart
Why does the heart have a central role in the
h l fF l d?
pathology of Fe overload?
Because of its complex array of cells & structure
the heart has to function in coordination.
FACIES LL edema
SICKLE CELL ANEMIA
The ti t is ll di d in i i
Th patient i usually diagnosed i a crisis.
Patients with sickle cell have a Hb 7-9gm/dl
There is early cardiomegaly, hyperdynamic
heart & increased ejection fraction.
The heart cant stand this high state of activity
It declines with time & diastolic dysfunction develops.
The patient with PVCs
Can present for the first time with
pulmonary hypertension & right side heart
Does anemia per se affect the heart?
Pure Red Cell Aplasia
Patients develop heart failure in early infancy.
What are the symptoms & signs?
What happens if the patient has an underlying
Heart Failure (acute or due to cardiomyopathy)
Leukemia, lymphoma & neuroblastoma
hl h h
Mesothilioma, teratoma, pheochromocytoma
Myocardial tumors irritating the pericarduim
A i t d i l diti
Associated viral myocarditis
Malignant PE occurs in up to 21% of cancer patients
and is frequently not suspected until clinical signs or
symptoms of pericardial tamponade develop.
Two thirds of patients have subclinical PE with no
overt cardiovascular signs or symptoms.
g y p
Pericardial effusion may initially present with
symptoms of cardiac tamponade.
Pericardial effusion may be the first sign of
Dyspnea, fatigue, or asthenia may be the initial
Symptomatic pericardial effusions are often
Slow accumulation of large amounts of fluid
may collect with no clinical symptoms.
Rapid accumulation of fluid is tolerated
poorly with nonspecific symptoms of
dyspnea, chest or shoulder pain, or
Dyspnea , Orthopnea
Chest pain, discomfort
Ch d f
Characteristically, pericardial pain may be
relieved by sitting up and leaning forward and
is intensified by lying supine.
Respiratory - Cough, hoarseness
Gastrointestinal - Hiccoughs
Neurologic - Anxiety, confusion
Classic Beck triad of pericardial tamponade
(hypotension, distant heart sounds, jugular venous
(h t i di t t h t d j l
Pulsus paradoxus: decrease in systolic BP>than 10
mm Hg with inspiration, signaling falling cardiac
output during inspiration.
Pericardial friction rub
Widened pulse pressure
Hepatomegaly & Hepatojugular reflux:
Tachypnea: Ewart sign - Dullness to percussion
beneath the angle of left scapula from compression
of the left lung by pericardial fluid
Changes are nonspecific but include low
voltage QRS complexes, ST segment
elevation, T wave inversion and development
Electrical alternans, (beat-to-beat) variation
in QRS amplitude, occurs with excessive
motion of the heart within the fluid-filled
SVCS is an array of symptoms caused by the
impairment of blood flow through the SVC to the
i i f bl d fl h h h h
Superior mediastinal syndrome (SMS) refers to
the i t
th respiratory component t
Symptoms : dyspnea, coughing, and swelling of
the face, neck, upper trunk, and extremities. In
rare instances, patients may complain of
hoarseness, chest pain, d
h h h i
i dysphagia, andd
Neck vein distention,
Thoracic vein distention,
Edema of the face or upper extremities,
Pl th d tachypnea.
Plethora, and t h
Rarely, cyanosis, Horner syndrome, and a
paralyzed vocal cord may also be present
SVCS appears at presentation in 12% of
pediatric patients with malignant mediastinal
In children the most frequent malignant
cause of the syndrome is non-Hodgkin
A frequent nonmalignant cause is thrombosis
from catheterization for venous access.
In children the trachea & right main bronchus
are soft structures making them more
susceptible to compression.
In dditi the l ti l ll intraluminal
I addition, th relatively smaller i t l i l
diameters of a child’s trachea and bronchus
can tolerate little edema before respiratory
Because of this accompanying respiratory
component SVCS in children differs from the
adult syndrome and constitutes a serious
a) Chest x-ray
Anterior mediastinal mass - lymphoma
b) Simultaneous bilateral arm venogram
· Defines obstruction and collateral circulation
· Identifies thrombus
c) Computerized axial tomography
· Assessment of mediastinum
· Determine patency of jugular veins
· Directed needle biopsy
Direct extension from the inferior vena cava to the
right atrium can occur in patients with Wilms tumor,
g p ,
renal myosarcoma, leiomyoma, and
Wilms tumor: symptoms of right heart obstruction
or failure can develop before presenting signs and
s mptoms of an abdominal mass or hemat ria .
Ultrasound & echocardiography are diagnostic.
Atrial Wilms tumors can mimic atrial myxomas by
their to-and-fro motion across atrioventricular
Primary tumors include : myxomas (most
common), rhabdomyomas , fibromas ,
lipomas, sarcomas etc
Secondary cardiac tumors are more
frequently observed than primary tumors.
The most common secondary tumors in
pediatric patients are non-Hodgkin
lymphoma, leukemia, and neuroblastoma
A mass can be seen anywhere in the heart.
Hypertrophied papillary muscle
Lymphoma of the heart can be primary or
Primary cardiac lymphoma presents with
constitutional symptoms or superior vena
Diagnosis should be considered in patients
with a cardiac mass sometimes with
pericardial effusion. It is confirmed using
transthoracic echocardiography and
Cardiac infiltration varies from microscopic
foci, to grossly identifiable tumor deposits
destroying and replacing normal heart
Infiltration affects the
Conduction system with various forms of arrhythmia
Myocardium with myocardial dysfunction
Pericardium with effusion
Endocardium & valves
Premature ventricular contractions
Conduction system tumors are most likely
congenital rests of endodermal origin, can be
associated with other congenital anomalies,
and often cause symptoms of heart block and
Tumors of the atrioventricular nodal region
were studied. Sudden death occurred in 14
DD of Dilated Cardiomyopathy : Etiology
Idiopathic :50% Connective tissue disorders
Familial 5-10% & toxins
Drugs Drugs & Toxins
li k d Familial l l
F ili l muscular or neuromuscular
Post-infectiousviral, bacterial, fungal Muscular dystrophies
EndocrineGrowth hormone def
Hypo, hyperthyroidism Intrinsic cardiac diseases
Pheochromocytoma Acquired : Kawasaki
Inborn errors of metabolism
Storage disease hypercholesterolemia
Nutritional d fi i
N ii l deficiency S l i
*End stage of HCM *Structural
carnitine, thiamine , Vit D
Infiltration Systemic Hypertension
Patient ill for 2 years.
Presented with an echo diagnosis of dilated
d h h d fdl d
O/E developed typical attacks of
l ti ti t
A pulsating patient.
BP 180/110 & later became 210/140
G p gy
Gross pathology p
Intramyocardial teratomas have
Occurs in the newborn period or in the
first 6 years of life.
Most patients are symptomatic and present
with congestive heart failure;
Rarely, a patient may be asymptomatic, or
sudden death may occur
Don’t underestimate anemia
D ’t d ti t i
Follow up dyspnea (?refusal of feeding)
Pericardial effusion can be fatal if massive
Mild pericardial effusion is easily missed
Intervention in the right time can save the
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