; Which part of the heart will be affected
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Which part of the heart will be affected


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									                    Alyaa Kotby,
              Professor of Pediatrics,
            Head of Pediatric Cardiology,
                 Pediatric Hospital
               Ain Shams University


Which part of the heart will be affected?
 Any part
      Please don’t under estimate anemia

      Wh ?

      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
                                           *Coronary artery
      Growth hormone def                   Congenital (ALCAPA)
      Hypo, hyperthyroidism
                                           Acquired : Kawasaki
      Pheochromocytoma                      *Familial
 Inborn errors of metabolism                  hypercholesterolemia
        Storage disease                      *End stage of HCM *Structural
 Infiltration                                 HD
 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.
  C S
FACIES                               LL edema

 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.

Pulmonary hypertension
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 disease?
Heart Failure (acute or due to cardiomyopathy)
Pericardial infiltration
Leukemia, lymphoma & neuroblastoma
Pericardial tumors
        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
malignant disease.
Dyspnea, fatigue, or asthenia may be the initial
Symptomatic pericardial effusions are often
preterminal .
General Considerations:
 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.
 Light-headedness, syncope
 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
of arrhythmias.
Electrical alternans, (beat-to-beat) variation
in QRS amplitude, occurs with excessive
motion of the heart within the fluid-filled
pericardial space.
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
right atrium.
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
Physical signs

   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
                           non Hodgkin
 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
                                    p      y
symptoms occur.
 Because of this accompanying respiratory
component SVCS in children differs from the
adult syndrome and constitutes a serious
medical emergency.

a) Chest x-ray
         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
symptoms                            hematuria
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
caval syndrome.
Diagnosis should be considered in patients
with a cardiac mass sometimes with
pericardial effusion. It is confirmed using
transthoracic echocardiography and
magnetic resonance

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
Sinus tachycardia
Heart block
Premature ventricular contractions
Ventricular tachycardia.
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
sudden death.

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
      AD, AR
      X linked
        li k d                             Familial      l               l
                                           F ili l muscular or neuromuscular
      Mitochondrial                          disorders
 Post-infectiousviral, bacterial, fungal         Muscular dystrophies
  EndocrineGrowth hormone def
      Hypo, hyperthyroidism                Intrinsic cardiac diseases
                                             *Coronary artery
      Hypocalcemia, DM,
                                             Congenital (ALCAPA)
      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
                                             *Systemic hypertension
       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
Profuse sweating
      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
           C S O

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