● Your healthcare provider will ask you about your child’s eating ● Children's umbilical hernias often close

close on their own in the

Overview habits. Then your child will get examined. Sometimes, first two years of life, though some remain open into the fifth
I. Care of Child with GIT problems providers can feel an olive-sized lump in your child’s belly. year or longer.
A. Pyloric Stenosis That’s the thickened pyloric muscle. ● Umbilical hernias that appear during adulthood are more
B. Umbilical Hernia ● Your provider may recommend a blood test as well. This test likely to need surgical repair.
C. Omphalocele and Gastroschisis can tell if your child is dehydrated or has an electrolyte
D. Congenital Diaphragmatic Hernia imbalance from vomiting. Electrolytes are minerals that keep
E. Inflammatory Bowel Diseases the body working the way it should.
II. Care of Child With Cardiac Problems
A. Review of Fetal Circulation Signs and Symptoms
B. Diagnostic Tests for Cardiac ● Pyloric stenosis symptoms usually start when the baby is 2 to
Problems/Anomalies 8 weeks old. But it can take up to five months for the
III. Congenital Cardiac Disorders symptoms to become apparent. If you notice symptoms, talk
IV. Disorders With Increased Pulmonary Blood Flow to your healthcare provider. It’s best to treat HPS before your
A. Disorders With Obstruction to Blood Flow baby becomes dehydrated and undernourished.
B. Disorders With Mixed Blood Flow ● Symptoms start when babies are around 2 to 8 weeks old.
V. Intraoperative Measures ● Infants with pyloric stenosis may eat well but have these
VI. Post-partal Care Measures symptoms:
○ Frequent projectile vomiting (forceful vomiting), usually
Care of Child with GIT problems within a half hour to an hour after eating.
■ Pyloric stenosis causes projectile vomiting and can
Pyloric Stenosis
lead to dehydration in babies.
● Pyloric stenosis is a condition that affects an infant's pylorus,
■ Symptoms of dehydration: Sunken eyes, no tears
a muscle at the end of the stomach.
while crying, sunken soft spot, less urination (brown
● When the pylorus thickens, food can’t pass through. Symptoms:
or dark yellow),
● Pyloric stenosis is a thickening or narrowing of the pylorus, a ● Pain
○ Abdominal (belly) pain.
muscle in the stomach. ● Vomiting
○ Dehydration.
● The full name of the condition is hypertrophic pyloric stenosis ● Constipation
○ Hunger after feedings.
(HPS). ● Strangulated intestine
○ Irritability.
● Hypertrophy means thickening.
○ Small stools.
Causes:
○ Wave-like stomach motion right after eating, just before
● During gestation, the umbilical cord passes through a small
vomiting starts. Occasionally a mass like a sausage can
OCCURRENCE opening in the baby's abdominal muscles. The opening
be felt in the stomach.
Pyloric stenosis affects 3 out of normally closes just after birth. If the muscles don't join
every 1,000 babies born. It’s the together completely in the midline of the abdominal wall, an
Management
most frequent condition requiring umbilical hernia may appear at birth or later in life.
● Surgery
surgery in infants. ● In adults, too much abdominal pressure contributes to
○ Pyloromyotomy
umbilical hernias. Causes of increased pressure in the
abdomen include:
○ Obesity
Umbilical Hernia
● Multiple pregnancies
● An umbilical hernia occurs when part of your intestine bulges
● Fluid in the abdominal cavity
through the opening in your abdominal muscles near your
Prevalence ● Previous abdominal surgery
belly button (navel).
● It’s the most frequent condition requiring surgery in infants. ● Long-term peritoneal dialysis to treat kidney failure
● Umbilical hernias are common and typically harmless.
● Babies usually aren’t born with pyloric stenosis. The ● Umbilical hernias are most common in infants, but they can
thickening of the pylorus starts to happen in the weeks after Risk Factors:
affect adults as well.
birth. ● Umbilical hernias are most common in infants — especially
● In an infant, an umbilical hernia may be especially evident
premature babies and those with low birth weights. In the
when the infant cries, causing the belly button to
Diagnosis United States, black infants appear to have a slightly
protrude.This is a classic sign of an umbilical hernia.
 increased risk of umbilical hernias. The condition affects boys
and girls equally.
● For adults, being overweight or having multiple pregnancies
may increase the risk of developing an umbilical hernia. This
type of hernia tends to be more common in women.
Symptoms:
● An umbilical hernia creates a soft swelling or bulge near the
navel. In babies who have an umbilical hernia, the bulge may
be visible only when they cry, cough or strain.
● Umbilical hernias in children are usually painless. Umbilical
hernias that appear during adulthood may cause abdominal
discomfort.
Complications:
● For children, complications of an umbilical hernia are rare.
Complications can occur when the protruding abdominal
tissue becomes trapped (incarcerated) and can no longer be
pushed back into the abdominal cavity. This reduces the
blood supply to the section of the trapped intestine and can
lead to abdominal pain and tissue damage.
● If the trapped portion of intestine is completely cut off from the
blood supply, it can lead to tissue death. Infection may spread
throughout the abdominal cavity, causing a life-threatening
situation.
● Adults with umbilical hernias are somewhat more likely to
experience a blockage of the intestines. Emergency surgery
is typically required to treat these complications.
Diagnosis
● An umbilical hernia is diagnosed during a physical exam.
Sometimes imaging studies — such as an abdominal
ultrasound or a CT scan — are used to screen for
complications.
Treatment:
● Most umbilical hernias in babies close on their own by age 1
or 2.Your doctor may even be able to push the bulge back
into the abdomen during a physical exam.
● Although some people claim a hernia can be fixed by taping a
coin down over the bulge, don't try this. Placing tape or an
object over the bulge doesn't help and germs may
accumulate under the tape, causing infection.
● For children, surgery is typically reserved for umbilical
hernias that:
○ Are painful
○ Are slightly larger than 1/4 to 3/4 inch (1 to 2
centimeters) in diameter
○ Are large and don't decrease in size over the first two
years of life
○ Don't disappear by age 5 Risk Factors:
○ Become trapped or block the intestine ● Young maternal age
● For adults, surgery is typically recommended to avoid ● Tobacco and alcohol intake during gestation
possible complications, especially if the umbilical hernia gets
bigger or becomes painful. Diagnosis (Before Birth)
● During surgery, a small incision is made near the belly button. ● Ultrasound/Ultrasonography
The herniated tissue is returned to the abdominal cavity, and
the opening in the abdominal wall is stitched closed. In
adults, surgeons often use mesh to help strengthen the Treatment:
abdominal wall. ● Surgery (Place organs back in, repair defect, may need IV
nutrients and antibiotics)
Omphalocele Vs. Gastroschisis
Omphalocele
● An omphalocele is caused by an opening (defect) in the
middle of the abdominal wall at the belly button (umbilicus)
Gastroschisis
● The skin, muscle, and fibrous tissue are missing
Gastro - Gastrointestinal Tract
● The intestines spill (herniate) out through the opening and are
Schisis - separation
covered by a thin sac. The umbilical cord is in the center of
● Abnormal opening of the abdominal wall
the defect
○ Anterior abdominal wall fails to close and remains open
● Involves herniation of abdominal viscera through an enlarged
throughout fetal development. This results in the
umbilical ring an fail to return from the umbilical cord during
abdominal organs, specifically the intestines, to protrude
fetal development
outside.
○ The viscera may include: liver, small and large intestine,
● In gastroschisis, the opening is near the belly button (usually
stomach, spleen gallbladder.
to the right) but not directly over it, like in omphalocele
● Covered by a amnion/peritoneal layer
● Before birth, because the intestines are not covered by a sac,
● An omphalocele commonly occurs along with other birth
they may be damaged by exposure to amniotic fluid, which
defects (such as heart defects and kidney defects) and with
causes inflammation.
specific genetic syndromes (such as Down syndrome,
○ The inflammation irritates the intestine, which can result
trisomy 18, trisomy 13, and Beckwith-Wiedemann syndrome).
in complications such as problems with movements of
the digestive system, scar tissue, and intestinal
Prevalence:
obstruction.
● 2.5/10000 births
● After delivery, the protruded organs are exposed to air as
● Approximately half of live-born infants with omphalocele have
there is no peritoneal layer.
chromosomal abnormalities.
● Causes are unknown.
● Thought to originate from genetic and environmental factors
Diagnosis (Before Birth)
● Ultrasound/Ultrasonography
● Doctors may suspect gastroschisis if the level of
alpha-fetoprotein (a protein produced by the fetus) in the
mother's blood is abnormally high during pregnancy.
● Both omphalocele and gastroschisis are usually diagnosed
before birth with routine prenatal ultrasonography. If not, the
defects are very obvious as soon as the infant is delivered.
Treatment:
● Surgery
● Once the infant is delivered, the exposed intestines are
covered with a sterile dressing to keep them moist and
protected and the infant is given fluids and antibiotics by vein.
● A long, thin tube is passed through the nose and placed in
the stomach or intestine (nasogastric tube) to drain digestive
fluid that collects in the stomach.
● Surgery is required to replace the intestines in the abdomen
and close the opening. If possible, surgery to repair the
defect is done soon after birth.
● However, the skin of the abdominal wall often must be
stretched for a few days before surgery so there is enough
tissue to cover the opening.
● If the defect is large, doctors may need to create skin flaps to
close it.
Congenital Diaphragmatic Hernia
● Congenital diaphragmatic hernia (CDH) is a rare condition
that happens in a baby before birth.
● It occurs early in pregnancy when a baby's diaphragm — the
muscle that separates the chest from the abdomen — fails to
close as it should.
● This leaves a hole in the diaphragm. The hole is called a
hernia
● This hernia in the muscle of the diaphragm creates an
opening between the abdomen and the chest.
● The intestines, stomach, liver and other abdominal organs
may move through the hole into the baby's chest.
● If the intestines are in the chest, they don't develop the typical
connections that hold them in place in the abdomen
(malrotation).
● They may twist on themselves, cutting off their blood supply
(volvulus).
● In addition, the lung is small on the side of the diaphragm
with the hernia, but the development of both lungs is affected.
● The air sacs (alveoli) inside the lungs don't develop as they
should. This results in problems with blood flow and
increased pressure inside the lung's blood vessels.
● The blood pressure in the lungs is higher than it should be,
which can make it hard for the baby to breathe after birth.
● Some infants may also have problems with heart
development
Symptoms
● Congenital diaphragmatic hernia ranges in severity. It may be
mild and have few or no effects on the baby, or it can be more
serious and affect the ability to bring oxygen to the rest of the
body.
● Babies born with CDH may have:
○ Severe trouble breathing due to small lungs that don't
work well (pulmonary hypoplasia)
○ A type of high blood pressure that affects the arteries in
the lungs and the right side of the heart (pulmonary
hypertension).
○ Problems with development of the heart.
○ Damage to the intestines, stomach, liver and other
abdominal organs if they move through the hernia into
the chest.
Causes:
● In most cases, the cause of congenital diaphragmatic hernia
is not known. In some cases, CDH can be linked to a genetic
disorder or random gene changes called mutations.
● In these cases, the baby may have more issues at birth, such
as problems with the heart, eyes, arms and legs, or stomach
and intestines
Complications
● Lung problems.
● Stomach, intestine and liver problems.
● Heart disease.
● Recurrent infections.
● Hearing loss.
● Changes in the shape of the chest and curve of the spine.
● Gastroesophageal reflux — stomach acid flowing back into
the tube called the esophagus, which connects the mouth
and stomach.
● Problems with growth and weight gain.
● Developmental delays and learning disabilities.
● Other problems present from birth.
Diagnosis
● Congenital diaphragmatic hernia is most often found during a
routine fetal ultrasound exam that's done before your baby is
born.
● A prenatal ultrasound exam uses sound waves to make
images of your uterus and baby.
● Occasionally, the diagnosis may not be made until after birth.
Rarely, CDH may not be diagnosed until childhood or later
○ This may be because there are no signs or symptoms or
because signs and symptoms such as respiratory and
intestinal problems are mild
● Prenatal Ultrasound
○ Most often, you have another ultrasound during months
four to six (second trimester) of your pregnancy. Your
health care provider checks the growth and development
of your baby. Your provider looks at the size and location
of your baby's lungs, heart and other organs.
○ If your baby shows signs of CDH, your provider may
have you get ultrasound exams more often. This can
show how severe CDH is and whether it's getting worse.
Other Tests
More tests may be done to assess the function of your baby's
organs. These may include:
● Fetal magnetic resonance imaging (MRI).
○ This is a medical imaging technique that uses a
magnetic field and computer-generated radio waves to
create detailed images of the organs and tissues in the
baby's body.
● Fetal echocardiogram.
○ An echocardiogram uses sound waves to produce
images of the baby's heart beating and pumping blood.
The images from an echocardiogram can identify
problems with the developing heart.
● Genetic tests.
○ Genetic testing can identify genetic syndromes or other
gene changes that are sometimes associated with CDH.
Genetic counseling can help you understand these test
results and give you more information about your baby's
condition
Treatment
Treatment of congenital diaphragmatic hernia depends on when
the condition is found and how serious it is.
● Care before delivery
○ Your health care team watches you closely before your
baby is born. You typically have ultrasounds and other
tests often to check your baby's health and development.
○ An emerging treatment for severe CDH now being
studied is called fetoscopic endoluminal tracheal
occlusion (FETO). This surgery is done on your baby
while you're still pregnant. The goal is to help the baby's
lungs grow as much as possible before birth.
○ FETO is done in two procedures:
— First procedure. The first procedure happens early in
the last few months (third trimester) of the pregnancy.
Your surgeon makes a small incision in your abdomen
 and uterus. The surgeon inserts a special tube with a Follow-up care to ensure the repair remains in place other organs) ● Toxic MegaColon
camera at the end, called a fetal endoscope, through usually includes chest X-rays ● Abscess - perianal ● Colorectal Cancer
your baby's mouth and into the windpipe (trachea). A ○ After leaving the hospital, your baby may need extra ● Strictures ● PSC (P primary sclerosing
small balloon is placed in your baby's trachea and support. This can include supplemental oxygen. Oxygen ● Small bowel cancer cholangitis) - inflammation
inflated. is delivered by thin plastic tubing with prongs that fit into ● Colorectal cancer of the biliary tract wall which
— The natural uterine fluid during pregnancy, called the nostrils or thin tubing connected to a mask worn over increases the cancer risk of
amniotic fluid, flows in and out of your baby's lungs the nose and mouth. Feeding support may also be the biliary tract called
through the mouth. Inflating the balloon keeps amniotic needed to help with growth and development. Medicine cholangiocarcinoma.
fluid in your baby's lungs. The fluid expands the lungs to may be given for conditions associated with CDH, such
help them develop as acid reflux or pulmonary hypertension Manifestations in both
— Second procedure. After about 4 to 6 weeks, you ● Uveitis (iritis)
have a second procedure. The balloon is removed so Inflammatory Bowel Disease ● Episcleritis
that your baby is ready to take air into the lungs after Ulcerative Colitis vs. Crohn’s Disease ● Red lesions (erythema nodosum)
birth ● Pyoderma gangrenosum
— A special delivery method may be used if labor starts CROHN’S ULCERATIVE ● Spondylitis - inflammation of vertebral column (meaning there
before the balloon has been removed and removal of the ● Crohn’s disease can occur ● Always involves the rectum is back pain)
balloon with an endoscope is not possible .This method anywhere in the GI tract but then spreads contiguously ● Sacroiliitis - inflammation of the sacroiliac joint (hip pain)
is called an ex utero intrapartum treatment (EXIT) the most common is the up through the sigmoid up Diagnosis
procedure. The delivery is done by C-section with ileum and colon to the distal colon ● Antibody Testing
placental support. This means that your baby continues (right/ascending). particularly the descending ○ Crohn’s
to get oxygen through the placenta before the umbilical ● Spares the rectum colon transverse colon and - Presence of anti-saccharomyces cerevisiae antibodies
cord is cut. Placental support continues until the balloon it can even involve the (+)
is out and a breathing tube is in place, allowing a ascending colon. - No presence of P-ANCA antibodies
machine to take over breathing ● Pag may involvement ng - Skip Lesions
FETO may not be the right choice for everyone. And there's no buong colon = pancolitis - cobblestoning sign
guarantee about the results of surgery. Your health care team ● - Creeping fat sign
evaluates you and your baby to see whether you may be ● Transmural Inflammation ● Only Involves the mucosa - String sign
candidates for this surgery. Talk to your team about the benefits involves mucosa, and submucosa - non-caseating granulomas
and possible complications for you and your baby. submucosa, muscularis
externa, serosa/adventitia ○ Ulcerative Colitis
● Care during delivery - Presence of P-ANCA antibodies
○ Usually, you can deliver your baby either vaginally or by - Continuous lesions
C-section. You and your health care provider decide - Lead pipe sign
which method is best for you - Crypt abscess — Crypt abscesses are the accumulation
○ After birth, the health care team helps you plan treatment of inflammatory cells within the crypts of the
that meets your baby's needs. Your baby will likely be gastrointestinal tract
cared for in the newborn intensive care unit (NICU). -
○ Your baby may need to have a breathing tube. The tube Treatment
is attached to a machine that helps your baby breathe. CROHN’S ULCERATIVE
This gives the lungs and heart time to grow and develop. ● Pain should be felt at the ● Tenderness will be at left ● sulfasalazine/mesalamine ● sulfasalazine/mesalamine
○ Babies who have life-threatening lung problems may right lower quadrant lower quadrant ● corticosteroids ● corticosteroids
need a treatment called extracorporeal membrane ● azathioprine ● azathioprine
Signs and Symptoms: Signs and Symptoms:
oxygenation (ECMO). This is also known as ● methotrexate ● methotrexate
● Malnourished ● Tenesmus = feeling that you
extracorporal life support (ECLS). The ECMO machine ● biologic agents ● cyclosporine
● Watery Diarrhea need to pass stools, even
does the work of your baby's heart and lungs, allowing ○ tnf alpha inhibitors ● biologic agents
● GallStones though your bowels are
these organs to rest and heal. ○ Infliximab ○ tnf alpha inhibitors
● Kidney Stones already empty
○ Most babies who have CDH have surgery to close the ○ Ustekinumab ○ Infliximab
● Bloody Diarrhea
hole in the diaphragm. When this surgery takes place ○ vedolizumab ○ vedolizumab
Complications: Complications:
depends on your baby's health and other factors. Surgical: Colectomy (Not Surgical: Colectomy (Curative)
● Fistula - connection (to ● Fulminant Colitis
 curative) defects.

Care of Child With Cardiac Problems ○ Medications

5 Areas for Listening To The Heart — Certain medications taken during pregnancy may cause
birth defects, including congenital heart defects.
— Medications known to increase the risk of congenital heart
defects include:
❖ thalidomide (Thalomid)
❖ angiotensin-converting enzyme (ACE) inhibitors
❖ statins
❖ the acne medication isotretinoin (Myorisan,
Zenatane, others)
❖ some epilepsy drugs and certain anxiety drugs
○ Drinking alcohol during pregnancy
SIMILARITIES — Drinking alcohol during pregnancy increases the risk of
● BOTH FORMS OF IBD congenital heart defects
● BOTH CAN CAUSE INFLAMMATION AND ULCERS ○ Smoking
● ETIOLOGY: UNKNOWN — If you smoke, quit. Smoking during pregnancy increases
● LINKED TO FAULTY IMMUNE SYSTEM the risk of a congenital heart defect in the baby.
● MAY BE TRIGGERED BY ENVIRONMENT AND GENETICS ○ Family History and Genetics
● FLARE UPS FOLLOWED BY REMISSION (NO — Congenital heart defects sometimes run in families (are
SYMPTOMS) inherited) and may be associated with a genetic syndrome.
● TYPES VARIES DEPENDING ON THE LOCATION — Many children with an extra 21st chromosome (Down
● INCREASED RISK OF COLON CANCER syndrome) have congenital heart defects.
● WOF FOODS THAT CAN CAUSE FLARE UPS Congenital Heart Defect — A missing piece (deletion) of genetic material on
● CONSUME LOW FIBER, HIGH IN PROTEIN, STAY ● A congenital heart defect is a problem with the structure of chromosome 22 also causes heart defects.
HYDRATED the heart that a child is born with.
● S/S: ABDOMINAL CRAMPING AND DIARRHEA ● Some congenital heart defects in children are simple and Symptoms
DIFFERENCES don't need treatment. Serious congenital heart defects usually are noticed soon after
CROHN’S ULCERATIVE ● Other congenital heart defects in children are more complex birth or during the first few months of life. Signs and symptoms
● AFFECTS WHOLE OF ● AFFECTS ONLY LARGE and may require several surgeries performed over a period of could include:
GIT(MOUTH TO ANUS) INTESTINE AND RECTUM. several years. ● Pale gray or blue lips, tongue or fingernails (cyanosis)
● MOST COMMON IN ● STARTS IN THE RECTUM ● Rapid breathing
TERMINAL ILEUM AND AND MIGRATE IN A Risk Factors ● Swelling in the legs, belly or areas around the eyes
START OF THE COLON CONTINUOUS FASHION ● Most congenital heart defects result from changes that occur ● Shortness of breath during feedings, leading to poor weight
● FOUND IN SCCATERED ● AFFECTS INNER LINING early as the baby's heart is developing before birth. gain
PATCHES (NOT OF THE INTESTINAL ● The exact cause of most congenital heart defects is Less-serious congenital heart defects may not be diagnosed until
CONTINUOUS WITH WALL (SUBMUCOSA AND unknown, but some risk factors have been identified. Risk later in childhood. Signs and symptoms of congenital heart
HEALTHY LINING NEXT MUCOSA) factors for congenital heart defects include: defects in older children may include:
TO DISEASED LINING) ● SURGERY: ○ Rubella (German Measles) ● Easily becoming short of breath during exercise or activity
● COBBLESTONE ○ COLLECTOMY WITH — Having rubella during pregnancy can cause problems in a ● Easily tiring during exercise or activity
APPEARANCE ILEOSTOMY baby's heart development. ● Fainting during exercise or activity
● AFFECTS ENTIRE LINING ○ CREATION OF — A blood test done before pregnancy can determine if ● Swelling in the hands, ankles or feet
OF INTESTINAL WALL TO ILEOANAL you're immune to rubella.
SEROSA ANASTOMOSIS OR ○ Diabetes How Congenital Heart Defects Develop
● NO CURE J-POUCH — Careful control of blood sugar before and during ● During the first six weeks of pregnancy, the baby's heart
● SURGERY HELPS pregnancy can reduce the risk of congenital heart defects in begins to form and starts beating.
QUALITY OF LIFE AND the baby. ● The major blood vessels that run to and from the heart also
SYMPTOMS — Diabetes that develops during pregnancy (gestational begin to develop during this critical time.
Bowel resection/ total colectomy diabetes) generally doesn't increase a baby's risk of heart ● It's at this point in a baby's development that congenital heart
 defects may begin to develop.
● Researchers aren't sure exactly what causes most of these
defects, but they think genetics, certain medical conditions,
some medications, and environmental or lifestyle factors,
such as smoking, may play a role.
● There are many different types of congenital heart defects.
They fall into the general categories:
1. Altered connections in the heart or blood vessels
— Altered connections allow blood to flow where it
usually wouldn't. Holes in the walls between heart
chambers are one example of this type of congenital
heart defect.
— An altered connection can cause oxygen-poor blood
to mix with oxygen-rich blood.
— This lowers the amount of oxygen sent through the
body.
— The change in blood flow forces the heart and lungs
to work harder.
I. Atrial septal defect
➢ is a hole between the upper heart chambers
(atria).
II. Ventricular Septal Defect
➢ is a hole in the wall between the right and left
lower heart chambers (ventricles)
III. Patent ductus arteriosus
➢ is a connection between the lung artery and the
body's main artery (aorta).
➢ It's open while a baby is growing in the womb,
and typically closes a few hours after birth. But
in some babies, it stays open, causing incorrect
blood flow between the two arteries
IV. Total or partial anomalous pulmonary venous
connection
➢ occurs when all or some of the blood vessels
from the lungs (pulmonary veins) attach to the
wrong area or areas of the heart.
2. Congenital heart valve problems
— Heart valves are like doorways between the heart
chambers and the blood vessels.
— Heart valves open and close to keep blood moving in
the proper direction. If the heart valves can't open and
close correctly, blood can't flow smoothly.
— Heart valve problems include valves that are
narrowed and don't open completely (stenosis) or valves
that don't close completely (regurgitation).
I. Aortic stenosis
➢ A baby may be born with an aortic valve that
has one or two valve flaps (cusps) instead of
three.
➢ This creates a small, narrowed opening for
blood to pass through.
➢ The heart must work harder to pump blood
through the valve.
➢ Eventually, this leads to enlargement of the
heart and thickening of the heart muscle.
Signs and Symptoms
● A heart murmur is the most common sign seen by a doctor
that shows a valve problem may be present
● Children with mild-to-moderate degrees of aortic valve
stenosis will have easily detectable heart murmurs. They
won’t have any symptoms at all.
● Symptoms occur with severe aortic stenosis. A newborn with
critical aortic valve stenosis develops heart failure in the first
days of life. This is an emergency that requires immediate
treatment.
● In an older child, severe aortic stenosis rarely causes heart
failure. The child may have chest pain, lightheadedness or
fainting spells with exercise. Severe aortic stenosis is a rare
cause of sudden death during sports activities.
Diagnosis
● The diagnosis of aortic stenosis is suspected because a
doctor hears a heart murmur or click.
● There is often a click sound when the thickened valve snaps
to its open position. These sounds can be found through
careful examination of the heart by a doctor.
● Other testing may show aortic stenosis. Tests may help
document how severe it is.
● An electrocardiogram is often done.
- The electrocardiogram is normal with mild-to-moderate
aortic stenosis. With severe aortic stenosis, the
electrocardiogram can show enlargement of the left
ventricle. It may show left ventricular strain
- The echocardiogram is the most important test to find
and evaluate aortic valve stenosis.
- The echocardiogram documents that the blockage is
present at the valve level. It can estimate the degree of
valve obstruction.
- The echocardiogram is also important in rejecting other
problems which may be associated with aortic stenosis.
○ Left ventricular failure
○ Coarctation of the aorta
○ Ventricular septal defect
○ Mitral valve stenosis.
● Cardiac catheterization is an invasive technique that allows
doctors to measure how much aortic stenosis is present.
- During cardiac catheterization, pressure measurements
are made above and below the valve.
- This measures the amount of blockage. Pictures are
taken to see the degree of valve obstruction.
- Echocardiography has replaced cardiac catheterization
in many cases to find and measure aortic stenosis.
- From time to time, it may be necessary to do a cardiac
catheterization to add to the information from the echo
studies.
- Cardiac catheterization can be combined with a balloon
dilation procedure.
Treatment
● Children with mild aortic stenosis rarely need treatment.
● Aortic stenosis may continue to develop. Children with mild
disease may need treatment later in life.
● All treatment for aortic valve stenosis is palliative (it does not
return the valve to a normal condition).
● Before and after treatment it is important that all children with
aortic stenosis follow up with a cardiologist for the rest of their
life.
● The type of treatment needed depends on the type of valve
defect. The most common defect is when the aortic valve, is
of normal size, is bicuspid (that is, two leaflets instead of
three), and has a degree of fusion along the commissures (or
lines of leaflet opening). This form of aortic valvar stenosis
responds well to balloon dilation procedures.
● Balloon dilation valvuloplasty is done at the time of cardiac
catheterization. It does not require open-heart surgery.
● In the newborn, it can be done through the umbilical artery.
● In older children the procedure is done through a femoral
artery. Patients are kept in the hospital overnight.
● Balloon valvuloplasty can be done as an outpatient
procedure in some children.
● Surgical valvotomy is an open-heart procedure where the
surgeon opens the valve. In many centers this open-heart
procedure has been replaced by the less invasive balloon
dilation valvuloplasty technique.
● Open-heart surgical procedures are needed for more
complex valves. These valves may be blocked by severe
calcium deposits in their leaflets. The valve ring may be small
and underdeveloped. For these conditions surgical aortic
valve replacement will be needed.
● The Ross procedure is an aortic valve replacement option
which may be best for young children.
● In the Ross procedure, the patient's own pulmonary valve is
transplanted to the aortic valve position.
● The pulmonary valve is replaced with a homograft (human
donor valve) from the right ventricle to pulmonary artery.
● It is a good option for young children because the replaced
aortic valve can grow with the child. It also does not need the
use of a blood thinner.
● A more traditional aortic valve replacement procedure
involves the implantation of a mechanical prosthesis in
the aortic valve position
● Anticoagulation therapy (blood thinner) is needed with any
 mechanical valve substitute.
● When the aortic valve is small (also known as a hypoplastic
valve annulus or ring), more involved surgical techniques
(like the Konno procedure) are required.
● The Konno procedure makes the aortic valve ring larger
with a cut into the wall between the two ventricles.
● The larger valve annulus can then accept a more normal size
prosthetic aortic valve or pulmonary valve auto transplant.
● A child with valvular aortic stenosis and a hypoplastic valve
ring might require a Ross-Konno procedure. This is a
combination of the Ross procedure plus a Konno procedure.
II. Pulmonary Stenosis
➢ A defect on or near the pulmonary valve
narrows the pulmonary valve opening and
slows the blood flow.
III.
➢ Diagnosis
➢ A whooshing sound, called a heart murmur,
may be heard. The sound is caused by
choppy blood flow across the narrowed
valve.
➢ Electrocardiogram (ECG or EKG). An ECG
can show how the heart is beating and may
reveal signs of heart muscle thickening
➢ Echocardiogram. An echocardiogram can
show the shape of the pulmonary valve.
The test can show how much of the valve
is narrowed.
➢ Cardiac catheterization. A provider can
determine the severity of pulmonary
stenosis by checking the difference in
pressure between the right lower heart
chamber and the lung artery.
➢ Other imaging tests. Magnetic resonance
imaging (MRI) and computed tomography
(CT) scans are sometimes used to confirm
the diagnosis of pulmonary valve stenosis.
➢ Treatment
■ If you have mild pulmonary valve stenosis
without symptoms, you may only need
occasional health checkups.
■ If you have moderate or severe pulmonary
valve stenosis, you may need a heart
procedure or heart surgery. The type of
procedure or surgery done depends on
your overall health and the appearance of
your pulmonary valve.
➢ Surgeries
■ Balloon valvuloplasty. Valvuloplasty may
improve blood flow through the heart and
reduce pulmonary valve stenosis
symptoms. But the valve may narrow
again. Some people need valve repair or
replacement in the future.
■ Pulmonary valve replacement. If balloon
valvuloplasty isn't an option, open-heart
surgery or a catheter procedure may be
done to replace the pulmonary valve. If
there are other heart problems, the
surgeon may repair those during the same
surgery. People who have had pulmonary
valve replacement need to take antibiotics
before certain dental procedures or
surgeries to prevent endocarditis.
Ebstein Anomaly
➢ The tricuspid valve — which is located between
the right upper heart chamber (atrium) and the
right lower chamber (ventricle) — is malformed
and often leaks
3. Combination of congenital heart defects
— Some infants are born with several congenital heart
defects that affect the structure and function of the heart.
— Very complex heart problems may cause significant
changes in blood flow or undeveloped heart chambers.
I. Tetralogy of Fallot
➢ a combination of four congenital heart defects:
VSD, Pulmonary Stenosis, Overriding of Aorta,
Right Ventricular Hypertrophy
➢ Boot shaped heart
➢ Diagnosis
■ diagnosed soon after birth
■ Cyanosis
■ abnormal whooshing sound (heart murmur)
when listening to the baby's heart with a
stethoscope
■ Oxygen level measurement (pulse
oximetry). A small sensor placed on a
finger or toe measures the amount of
oxygen in the blood.
■ Echocardiogram. An echocardiogram can
show the structure, placement and function
of the heart wall, heart chambers, heart
and pulmonary valves, and aorta.
■ Electrocardiogram (ECG or EKG). An
ECG can help determine if the heart
chambers are enlarged and if there's an
abnormal heartbeat (arrhythmia)
■ Chest X-Ray. A common sign of tetralogy
of Fallot on an X-ray is a boot-shaped
heart, because the right ventricle is
enlarged.
■ Cardiac catheterization. Doctors may use
this test to evaluate the structure of the
heart and plan surgical treatment.
➢ Treatment
■ All babies who have tetralogy of Fallot
need corrective surgery performed by a
heart (cardiovascular) surgeon.
■ Some children may need medicine while
waiting for surgery to maintain blood flow
from the heart to the lungs.
➢ Surgery
■ Surgery for tetralogy of Fallot involves
open-heart surgery to correct the defects
(intracardiac repair) or a temporary
procedure that uses a shunt. Most babies
and older children have intracardiac repair.
■ Intracardiac repair. usually done during
 the first year after birth and involves
several repairs. Adults with tetralogy of
Fallot rarely may undergo this procedure if
they didn't have surgical repair as children.
■ Temporary Shunt Surgery. Occasionally
babies need to undergo a temporary
(palliative) surgery before having
intracardiac repair in order to improve
blood flow to the lungs. This procedure
may be done if your baby was born
prematurely or has pulmonary arteries that
are undeveloped (hypoplastic)
II. Pulmonary Atresia
➢ The valve that lets blood out of the heart to go
to the lungs (pulmonary valve) isn't formed
correctly.
➢ Blood can't travel its usual route to get oxygen
from the lungs.
➢ Diagnosis
■ Chest X-ray.
■ Electrocardiogram (ECG or EKG).
■ Echocardiogram.
■ Cardiac catheterization
➢ Treatment
■ Medications. Medication may be given
through an IV to help prevent the closure of
the natural connection (ductus arteriosus)
between the pulmonary artery and the
aorta.
➢ Surgery. Sometimes, pulmonary atresia repairs
can be done using a long, thin tube (catheter)
inserted into a large vein in a baby's groin and
threaded up to the heart. Catheter-based
procedures for pulmonary atresia include:
■ Balloon atrial septostomy. A balloon is
used to enlarge the natural hole (foramen
ovale) in the wall between the upper two
chambers of the heart. This hole usually
closes shortly after birth. Making the hole
larger increases the amount of blood
available to travel to the lungs.
■ Stent placement. A health care provider
may place a rigid tube (stent) in the natural
connection between the aorta and
pulmonary artery (ductus arteriosus). This
opening also usually closes soon after
birth. Keeping it open allows blood to travel
to the lungs.
■ Shunting. Creating a bypass (shunt) from
the main blood vessel leading out of the
heart (aorta) to the pulmonary arteries
allows for adequate blood flow to the lungs.
However, babies usually outgrow this shunt
within a few months.
■ Glenn procedure. In this surgery, one of
the large veins that returns blood to the
heart is connected directly to the
pulmonary artery instead. Another large
vein continues to provide blood to the right
side of the heart, which pumps it through
the surgically repaired pulmonary valve.
This can help the right ventricle grow
larger.
■ Fontan procedure. If the right ventricle
remains too small to be useful, surgeons
may use this procedure to create a
pathway that allows most, if not all, of the
blood coming to the heart to flow directly
into the pulmonary artery.
■ Heart transplant. In some cases, the heart
is too damaged to repair and a heart
transplant may be necessary.
III. Tricuspid Atresia
➢ The tricuspid valve isn't formed. Instead, there's
solid tissue between the right upper heart
chamber (atrium) and the right lower chamber
(ventricle).
➢ This congenital heart defect restricts blood flow
and causes the right ventricle to be
underdeveloped.
IV. Transposition of Great Arteries
➢ In this serious, rare congenital heart defect, the
two main arteries leaving the heart are reversed
(transposed).
➢ There are two types. Complete transposition of
the great arteries is typically noticed during
pregnancy or soon after birth.
Levo-transposition of the great arteries (L-TGA)
is less common.
➢ Symptoms may not be noticed right away.
➢ Diagnosis
■ diagnosed after a baby is born
■ Fetal echocardiogram. the condition may
be seen before birth during a routine
pregnancy ultrasound
■ After birth, a health care provider may think
about a diagnosis of a TGA if the baby has
blue or gray skin, a weak pulse, or trouble
breathing. The care provider may hear a
heart sound, called a murmur, while
listening to the baby's heart
■ Echocardiogram.
■ Chest X-ray.
■ Electrocardiogram (ECG or EKG).
➢ Treatment
■ All infants with complete transposition of
the great arteries (D-TGA) need surgery to
correct the heart problem. Treatment for
congenitally corrected transposition
(L-TGA) depends on when the condition is
diagnosed and what other heart conditions
exist.
■ Before surgery is done to fix the switched
arteries, a medicine called alprostadil
(Caverject, Edex, others) may be given to
the baby. This medicine increases blood
flow. It helps oxygen-poor and oxygen-rich
blood better mix together.
➢ Surgery
■ usually done within the first days to weeks
after birth
■ Atrial septostomy. This treatment may be
done urgently as a temporary fix before
surgery. It helps mix oxygen-rich and
oxygen-poor blood, improving oxygen
levels in the baby's body.
■ Arterial switch operation. This is the most
common surgery used to correct
transposition of the great arteries. During
this surgery, the two main arteries leaving
the heart are moved to their correct
positions.
■ Rastelli procedure. This surgery may be
done if a baby with TGA also has a hole in
the heart called a ventricular septal defect.
The surgeon patches the hole and redirects
blood flow from the left lower heart
chamber to the aorta. This lets oxygen-rich
blood go to the body. An artificial valve
connects the right lower heart chamber to
the lung artery
■ Double switch procedure. This complex
surgery is used to treat congenitally
corrected transposition. It redirects blood
flow coming into the heart. It switches the
great artery connections so the left lower
heart chamber can pump oxygen-rich blood
to the aorta.
V. Hypoplastic Left Heart Syndrome
➢ A major part of the heart fails to develop
 properly.
➢ In hypoplastic left heart syndrome, the left side
of the heart hasn't developed enough to
effectively pump enough blood to the body.
➢ Treatment
■ Hypoplastic left heart syndrome is treated
through several surgical procedures or a
heart transplant.
■ Medication. The medication alprostadil
(Prostin VR Pediatric) helps widen the
blood vessels and keeps the ductus
arteriosus open.
■ Breathing assistance. Babies who have
trouble breathing might need help from a
breathing machine (ventilator).
■ Intravenous fluids. A baby might receive
fluids through a tube inserted into a vein.
■ Feeding tube. Babies who have trouble
feeding or who tire while feeding can be fed
through a feeding tube.
■ Atrial septostomy. This procedure creates
or enlarges the opening between the
heart's upper chambers to allow more
blood flow from the right atrium to the left
atrium. This is done if the foramen ovale
closes or is too small. Babies who already
have an opening (atrial septal defect) might
not need this procedure.
➢ Complications
■ Congestive heart failure. This serious
complication may develop in babies who
have a significant heart defect. Signs of
congestive heart failure include rapid
breathing, often with gasping breaths, and
poor weight gain.
■ Heart infections. Congenital heart defects
can increase the risk of infection of the
heart tissue (endocarditis), which can lead
to new heart valve problems.
■ Irregular heart rhythms (arrhythmias). A
congenital heart defect or scarring from
heart surgery may cause changes in the
heart's rhythm.
■ Slower growth and development
(developmental delays). Children with
more-serious congenital heart defects often
develop and grow more slowly than do
children who don't have heart defects. They
may be smaller than other children of the
same age. If the nervous system has been
affected, a child may learn to walk and talk
later than other children.
■ Stroke. Although uncommon, some
children with congenital heart defects are at
increased risk of stroke due to blood clots
traveling through a hole in the heart and on
to the brain.
■ Mental health disorders. Some children
with congenital heart defects may develop
anxiety or stress because of developmental
delays, activity restrictions or learning
difficulties.
Postpartum Care
Pregnancy changes your body in more ways than you might
expect. And it doesn't stop when the baby is born. Here's what to
expect physically and emotionally after a vaginal delivery.
● Vaginal Soreness
○ If you had a vaginal tear during delivery or your doctor
made an incision, the wound might hurt for a few weeks.
○ To ease discomfort while you're recovering:
— Sit on a pillow or padded ring.
— Cool the area with an ice pack, or place a chilled witch
hazel pad between a sanitary napkin and the area
between your vaginal opening and anus (perineum).
— Use a squeeze bottle to pour warm water over the
perineum as you're passing urine.
— Sit in a warm bath just deep enough to cover your
buttocks and hips for five minutes. Use cold water if you
find it more soothing.
— Take an over-the-counter pain reliever. Ask your
health care provider about a numbing spray or cream, if
needed.
● Vaginal Discharge
○ After delivery, you'll begin to shed the superficial mucous
membrane that lines your uterus during pregnancy.
○ The discharge will be red and heavy for the first few
days. Then it will taper, become increasingly watery and
change from pinkish brown to yellowish white.
● Contractions
○ You might feel occasional contractions, sometimes called
afterpains, during the first few days after delivery.
○ These contractions — which often resemble menstrual
cramps — help prevent excessive bleeding by
compressing the blood vessels in the uterus.
○ Afterpains are common during breastfeeding due to the
release of the hormone oxytocin.
● Incontinence
○ Pregnancy, labor and a vaginal delivery can stretch or
injure your pelvic floor muscles, which support the
uterus, bladder and rectum.
○ This might cause you to leak a few drops of urine while
sneezing, laughing or coughing.
○ In the meantime, wear sanitary pads and do pelvic floor
muscle exercises (Kegels) to help tone your pelvic floor
muscles and control your bladder.
● Hemorrhoids and bowel movements
○ If you notice pain during bowel movements and feel
swelling near your anus, you might have swollen veins in
the anus or lower rectum (hemorrhoids).
○ To ease discomfort while the hemorrhoids heal:
■ Apply an over-the-counter hemorrhoid cream or
suppository containing hydrocortisone.
■ Use pads containing witch hazel or a numbing
agent.
○ Soak your anal area in plain warm water for 10 to 15
minutes two to three times a day.
○ Eat foods high in fiber — including fruits, vegetables and
whole grains — and drink plenty of water.
● Tender Breasts
○ A few days after birth, you might experience full, firm,
tender breasts (engorgement). Frequent breastfeeding
on both breasts is recommended to avoid or minimize
engorgement.
○ If your breasts — including the dark circles of skin
around the nipples — are engorged, your baby might
have difficulty latching.
○ To help your baby latch, you might hand express or use
a breast pump to express a small amount of breast milk
before feeding your baby.
○ If you're not breastfeeding, wear a supportive bra, such
as a sports bra. Don't pump your breasts or express the
milk, which will cause your breasts to produce more milk.
● Hair loss and skin changes
○ During pregnancy, elevated hormone levels mean your
hair grows faster than it sheds.
○ The result is often an extra-lush head of hair — but now
it's payback time. After delivery, you'll experience hair
loss for up to five months.
○ Stretch marks won't disappear after delivery, but
eventually they'll fade from red to silver.
● Mood Changes
○ Childbirth triggers a jumble of powerful emotions. Many
new moms experience a period of feeling down or
anxious, sometimes called the baby blues.
○ Symptoms include mood swings, crying spells, anxiety
and difficulty sleeping.
○ The baby blues typically subside within two weeks.
○ If you experience severe mood swings, loss of appetite,
overwhelming fatigue and lack of joy in life shortly after
 childbirth, you might have postpartum depression.
● Weight Loss
○ After you give birth, you might look like you're still
pregnant. This is normal.
○ Most women lose 13 pounds (6 kilograms) during birth,
including the weight of the baby, placenta and amniotic
fluid.
○ In the days after delivery, you'll lose additional weight
from leftover fluids.
● The Postpartum checkup
○ The American College of Obstetricians and
Gynecologists recommends that postpartum care be an
ongoing process rather than just a single visit after your
delivery.
○ Within 12 weeks after delivery, see your health care
provider for a comprehensive postpartum evaluation.