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B L U E B A B Y
S Y N D R O M E
BIKRANT ROY
BLUE BABY
SYNDROME
Refers to a number
of conditions that
results in blueness
of the skin in babies
WHAT HAPPENS???
It’s characterized by an overall skin color
with a blue or purple tinge, called cyanosis.
This bluish
appearance is most
noticeable where
the skin is thin, such
as the lips,
earlobes, and nail
beds.
Other potential symptoms of blue baby syndrom
include:
 Difficulty in breathing
 Vomiting
 Diarrhea
 Lethargy
 Increased salivation
 Loss of consciousness
 Seizures
In severe cases, blue baby syndrome can even
cause death.
AT HAPPENS INSIDE???
BASICALLY,
WHAT HAPPENS
IS
When there’s a
problem with the
heart, lungs, or
blood, blood may
not be oxygenated
properly.
This causes the skin
to take on a blue
color.
ETIOLOGY
Cyanotic heart
disease occurring due
to a congenital heart
defect
Methemoglobinemiap
otentially caused
by nitrates in drinking
water
LET’S GO TO THE DEPTH OF THE
BLUES
CONGENITAL
HEART
DEFECTS
TETRALOGY OF FALLOT
Classically there are four defects :
1. pulmonary stenosis, narrowing of the exit from the right
ventricle
2. a ventricular septal defect, a hole between the two ventricles
3. right ventricular hypertrophy, thickening of the right
ventricular muscle
4. an overriding aorta, which allows blood from both ventricles
to enter the aorta
Overriding Aorta
Pulmonary
Stenosis
VSD
Right
Ventricular
Hypertrophy
Normal Heart Tetralogy Of Fallot
PATENT TRUNCUS ARTERIOSUS
Persistent truncus arteriosus (or patent truncus
arteriosus or common arterial trunk), is a rare form
of congenital heart disease that presents at birth. In this
condition, the embryological structure known as the truncus
arteriosus fails to properly divide into the pulmonary
trunk and aorta. This results in one arterial trunk arising from
the heart and providing mixed blood to the coronary
arteries, pulmonary arteries, and systemic circulation.
RA. Right Atrium SVC. Superior Vena Cava TV. Tricuspid Valve
RV. Right Ventricle IVC. Inferior Vena Cava MV. Mitral Valve
LV. Left Ventricle AO. Aorta
Truncus Arteriosus
TRICUSPID ATRESIA
Tricuspid atresia is a form of congenital heart
disease whereby there is a complete absence of the tricuspid
valve. Therefore, there is an absence of right atrioventricular
connection. This leads to a hypo plastic (undersized) or
absent right ventricle.
TRICUSPID ATRESIA
Superior Vena Cava
Pulmonary
Artery
Pulmonary
Vein
Inferior Vena Cava
Rudimentary
Right
Ventricle
Atresia
Tricuspid
Valve
Aorta
TRANSPOSITION OF THE
GREAT VESSELS
Transposition of the great vessels (TGV) is a group
of congenital heart defects involving an abnormal spatial
arrangement of any of the great
vessels: superior and/or inferior venae cavae, pulmonary
artery, pulmonary veins, and aorta. Congenital heart diseases
involving only the primary arteries (pulmonary artery and aorta)
belong to a sub-group called transposition of the great
arteries.
Anomalous pulmonary
venous connection
Anomalous pulmonary venous
connection (or anomalous pulmonary venous
drainage or anomalous pulmonary venous return)
is a congenital defect of the pulmonary veins.
NOBEL
NITRATES
Blue baby syndrome can also be caused
by nitrates in drinking water leading
to methemoglobinemia.
Dr. Comly
In 1987, the Journal of the American Medical
Association reprinted a case study originally published
in 1945.
The author of the original paper, Hunter Comly, was a
pediatric resident in Iowa City when he described a
previously unrecognized blood condition and skin
coloration in infants.
But what was the nature of the
difference between the hospital
and my farm house water?????
After the third episode of admission
and treatment with methylene blue for
one child, her father demanded to
know exactly what “poison” in his well
was responsible. Analyses of the
water showed high levels of nitrate.
AFTER ANALYSIS OF
THE DRINKING
WATER SOURCES
OF OTHER
NEIGHBOURING
CASES SHOWED
THE PRESENCE OF
NITRATES IN
ABUNDANCE
Dr. Comly
how biologically
inert nitrate was
responsible for
oxidizing
hemoglobin to
methemoglobin???
The most
plausible
candidate for the
oxidizing agent
was nitrite, which
was known to be
a potent
generator of
methemoglobin.
Many common
organisms found in
the bowel were
known to convert
nitrate to nitrite; the
nitrite could then
be readily
absorbed into the
blood to do its
damage.
GOTCHA!!!
That led to a
consideration of the
second-most common
finding in affected infants,
namely evidence of
gastrointestinal upset in
the form of vomiting or
diarrhea.
According to WHO’s latest
recommendation , 5 mg/l of
nitrate is the standard for
normal drinking water
supplies.
In river water, it can go up to
17 mg/l, which is also
normal.
METHEMOGLOBINEMIA
TREATMENT
CONGENITAL HEART DEFECTS
SURGERY
Blalock–Thomas–Taussig shunt
Commonly called the Blalock–Taussig shunt is a
surgical procedure used to increase pulmonary
blood flow in cyanotic heart defects which are
common causes of blue baby syndrome.
In modern surgery, this procedure is temporarily used to direct blood
flow to the lungs and relieve cyanosis while the infant is waiting for
corrective or definitive surgery.
Complete Intracardiac Repair
Surgery to repair Tetralogy of Fallot is done to
improve blood flow to the lungs and to make
sure that oxygen-rich and oxygen-poor blood
flows to the right places. The surgeon will:
1. Widen the narrowed pulmonary blood
vessels.
2. Close the ventricular septal defect (VSD).
Fixing these two defects resolves problems caused by the
other two defects.
When the right ventricle no longer has to work so hard to pump
blood to the lungs, it will return to a normal thickness.
The incision (cut) that the surgeon makes to reach the heart
usually heals in about 6 weeks.
The surgeon or a hospital staff member will explain when it's
okay to give your baby a bath, pick him or her up under the
arms, and take your baby for his or her regular shots
(immunizations).
METHEMOGLOBINEMIA
For babies with methemoglobinemia MEDICATION
is recommended.
The condition can be reversed by taking a drug
called methylene blue, which can provide oxygen to
the blood. This drug needs a prescription and is
usually delivered via a needle inserted into a vein.
OUTLOOK
The number of cases fell off steadily through the early
1950s. Today the disease has all but disappeared.
Only two cases have been reported since the mid-1960s
and none since 2000.
Within 10 years the epidemic had waned as suddenly as it
had appeared, without any preventive action having
knowingly been taken.
Whether it was because of public awareness, a massive
improvement in rural drinking water quality, a trend toward
breastfeeding or other factors may never be known.
THANK
YOU

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Blue Baby Syndrome - A Hope Under THE BLUE

  • 1.
  • 2. Let's see…… B L U E B A B Y S Y N D R O M E BIKRANT ROY
  • 3. BLUE BABY SYNDROME Refers to a number of conditions that results in blueness of the skin in babies
  • 5. It’s characterized by an overall skin color with a blue or purple tinge, called cyanosis.
  • 6. This bluish appearance is most noticeable where the skin is thin, such as the lips, earlobes, and nail beds.
  • 7.
  • 8. Other potential symptoms of blue baby syndrom include:  Difficulty in breathing  Vomiting  Diarrhea  Lethargy  Increased salivation  Loss of consciousness  Seizures In severe cases, blue baby syndrome can even cause death.
  • 10. BASICALLY, WHAT HAPPENS IS When there’s a problem with the heart, lungs, or blood, blood may not be oxygenated properly. This causes the skin to take on a blue color.
  • 12. Cyanotic heart disease occurring due to a congenital heart defect
  • 14. LET’S GO TO THE DEPTH OF THE BLUES
  • 16. TETRALOGY OF FALLOT Classically there are four defects : 1. pulmonary stenosis, narrowing of the exit from the right ventricle 2. a ventricular septal defect, a hole between the two ventricles 3. right ventricular hypertrophy, thickening of the right ventricular muscle 4. an overriding aorta, which allows blood from both ventricles to enter the aorta
  • 18. PATENT TRUNCUS ARTERIOSUS Persistent truncus arteriosus (or patent truncus arteriosus or common arterial trunk), is a rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. This results in one arterial trunk arising from the heart and providing mixed blood to the coronary arteries, pulmonary arteries, and systemic circulation.
  • 19. RA. Right Atrium SVC. Superior Vena Cava TV. Tricuspid Valve RV. Right Ventricle IVC. Inferior Vena Cava MV. Mitral Valve LV. Left Ventricle AO. Aorta Truncus Arteriosus
  • 20. TRICUSPID ATRESIA Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypo plastic (undersized) or absent right ventricle.
  • 21. TRICUSPID ATRESIA Superior Vena Cava Pulmonary Artery Pulmonary Vein Inferior Vena Cava Rudimentary Right Ventricle Atresia Tricuspid Valve Aorta
  • 22. TRANSPOSITION OF THE GREAT VESSELS Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenital heart diseases involving only the primary arteries (pulmonary artery and aorta) belong to a sub-group called transposition of the great arteries.
  • 23.
  • 24. Anomalous pulmonary venous connection Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital defect of the pulmonary veins.
  • 25.
  • 27. Blue baby syndrome can also be caused by nitrates in drinking water leading to methemoglobinemia.
  • 28. Dr. Comly In 1987, the Journal of the American Medical Association reprinted a case study originally published in 1945. The author of the original paper, Hunter Comly, was a pediatric resident in Iowa City when he described a previously unrecognized blood condition and skin coloration in infants.
  • 29. But what was the nature of the difference between the hospital and my farm house water????? After the third episode of admission and treatment with methylene blue for one child, her father demanded to know exactly what “poison” in his well was responsible. Analyses of the water showed high levels of nitrate.
  • 30. AFTER ANALYSIS OF THE DRINKING WATER SOURCES OF OTHER NEIGHBOURING CASES SHOWED THE PRESENCE OF NITRATES IN ABUNDANCE
  • 31. Dr. Comly how biologically inert nitrate was responsible for oxidizing hemoglobin to methemoglobin???
  • 32. The most plausible candidate for the oxidizing agent was nitrite, which was known to be a potent generator of methemoglobin.
  • 33. Many common organisms found in the bowel were known to convert nitrate to nitrite; the nitrite could then be readily absorbed into the blood to do its damage. GOTCHA!!!
  • 34. That led to a consideration of the second-most common finding in affected infants, namely evidence of gastrointestinal upset in the form of vomiting or diarrhea.
  • 35. According to WHO’s latest recommendation , 5 mg/l of nitrate is the standard for normal drinking water supplies. In river water, it can go up to 17 mg/l, which is also normal.
  • 38. CONGENITAL HEART DEFECTS SURGERY Blalock–Thomas–Taussig shunt Commonly called the Blalock–Taussig shunt is a surgical procedure used to increase pulmonary blood flow in cyanotic heart defects which are common causes of blue baby syndrome.
  • 39. In modern surgery, this procedure is temporarily used to direct blood flow to the lungs and relieve cyanosis while the infant is waiting for corrective or definitive surgery.
  • 40. Complete Intracardiac Repair Surgery to repair Tetralogy of Fallot is done to improve blood flow to the lungs and to make sure that oxygen-rich and oxygen-poor blood flows to the right places. The surgeon will: 1. Widen the narrowed pulmonary blood vessels. 2. Close the ventricular septal defect (VSD).
  • 41. Fixing these two defects resolves problems caused by the other two defects. When the right ventricle no longer has to work so hard to pump blood to the lungs, it will return to a normal thickness. The incision (cut) that the surgeon makes to reach the heart usually heals in about 6 weeks. The surgeon or a hospital staff member will explain when it's okay to give your baby a bath, pick him or her up under the arms, and take your baby for his or her regular shots (immunizations).
  • 42. METHEMOGLOBINEMIA For babies with methemoglobinemia MEDICATION is recommended. The condition can be reversed by taking a drug called methylene blue, which can provide oxygen to the blood. This drug needs a prescription and is usually delivered via a needle inserted into a vein.
  • 44. The number of cases fell off steadily through the early 1950s. Today the disease has all but disappeared. Only two cases have been reported since the mid-1960s and none since 2000. Within 10 years the epidemic had waned as suddenly as it had appeared, without any preventive action having knowingly been taken. Whether it was because of public awareness, a massive improvement in rural drinking water quality, a trend toward breastfeeding or other factors may never be known.

Editor's Notes

  1. The lack of oxygenation can occur for several reasons.
  2. An atrial septal defect (ASD) and a ventricular septal defect (VSD) must both be present to maintain blood flow-from the right atrium, the blood must flow through the ASD to the left atrium to the left ventricle and through the VSD to the right ventricle to allow access to the lungs
  3. Both congenital heart disease and methemoglobinemia tend to result in hypoxia and peripheral vasodilation, which may intensify the cyanosis.
  4. He recognized that “the only significant change in the infant’s environment from hospital to farm home was in the water that was used to prepare the formula.”
  5. Simple laboratory tests are available to identify and quantify methemoglobin in blood, and infants with high levels of it respond rapidly to intravenous methylene blue—this blue dye turns the blue baby pink.