Can be anywhere from moderate to severe.

3-4th week of gestation is the development of the heart and circulatory system.

By the 8th week it's completely formed.

By the 28th day around the heart starts beating.

Heart starts as a simple tube that just recieves blood from placenta and pumps out to body. Transforms into 4 chambers.

It's different than newborn circulation.

Lungs are nonfunctional.

Gets O2 through placenta. Liver needs minimal amount of blood as well.

Soul source of O2.

At birth switch occurs. Structures close and normal circulation happens.

Any problems in the first initial 8 weeks can lead to defects.

Any failure of structures to close can cause defects.

Maternal drug/alcohol use

Advanced maternal age (35+)

Maternal condition (lupus/diabetes)

Familial history

Some defects are life threatening, taken immediately to surgery. Some you can wait.

Allow blood flow from high pressure left side of heart to lower pressure right side of heart.

Causes increased pulmonary blood flow usually causes CHF.

3 major defects:

VSD, ASD PDA

Results from abnormal opening in septum b/w left and right ventricle. Can be tiny/large. 

Tiny-asymptomatic. 

Large-loud, audible murmur, hear the blood. S/SX: of dyspnea, of CHF, more prone to resp infections

DX: by listening, EKG, CXR TX: some close on own, srugery before child is 2, suture/patch

Opening in the septum between the left and right atria.

The formen ovalie stays open (usually the cause).

Blood flows from L atria to R atria and back to lungs. can lead to ventricularhypertrophy.

Sometimes asymptomatic or very soft heart murmur. As child gets older they get fatigued, delayed growth, and prone to CHF.

DX: EKG TX: suture/patch

Closes within 15hours of birth normally.

When ductus arteriosus remains patent, blood continues to shunt from aorta to the pulmonary artery.

S/SX: machine like murmur. Loud and audible, wide pulse pressure, bounding pulses, tired and fatigue, not growing, risk for CHF.

DX: hear murmur, EKC, cardiac cath

TX:meds, indomethacin prostaglandin inhibitor helps close a patent ductus. Surgery.

blood exiting heart meets an anatomic narrowing causing obstruction to blood flow.

2 major defects:

coarctation of the aorta

aortic stenosis (narrowing)

narrowing of the aorta specifically at the arch.

heart is working harder to pus hblood through.

Leads to CHF over time.

Causes increased pressure in upper extremities and decreased pressure in lower extremities.

Depends on the degree of defect.

Dyspnea, tachycardia, CHF,

BP and pulse increased in upper extremities, diminished in lower extremities

C/O cramping legs, H/A because of circulation

If not treated can result in HTN, aortic aneurysm

Stroke, cardiac failure

Surgery done by age 2.

Try to widen it or remove it and graft.

Decreased Pulmonary Blood Flow

(Cyanotic Defect)

Obstruction of pulmonary blood flow and anatomic defect b/w right and left side of heart. Septel defect.

Blood has difficulty leaving heart through pulmonary artery so right sided pressure increases.

Destaurated blood is shunted right to left resulting in hypoexemia.

Most common defect: Tetralogy of Fallot.

Most Common Defect of decreased pulmonary blood flow.

4 Seperate combined defects.

Pulmonary Stenosis-narrowing of pulmonary valve

VSD-ventricular septal defect. (hole)

Overriding aorta-aorta comes off both ventricles

Right ventricular hypertrophy.

The blood has a hard time getting to the lungs.

Defect in septum

Right ventricular hypertrophy

Overriding aorta

Pulmonary valve stenosis

(Tetralogy of Fallot)

S/SX of Decreased Pulmonary Blood Flow

(Tetralogy of Fallot)

Some will be acutely cyanotic at birth.

Does vary in degree. Can slowly progress over first year.

Murmur is often audible.

Feeding difficulties, poor wt gain. Slowed growth and development

Prone to tet spells/blue spells. Dyspnea, gasping respir, increased cyanosis that can lead to loss of consciousness and possible convulsions. They seem to be stresssed induced. 

Some older kids will drop into squatting postion (knees to chest) in attempt to get O2 into body.

They develop polycythemia (Increase in RBCs) in attempt to get more O2 to bind.

DX decreased pulmonary blood flow

(tetralogy of fallot)

Confirm with echocardiogram

EKG

TX decreased pulmonary blood flow

(tetralogy of fallot)

Usually repaired by the time the child is two.

Surgery.

The more the severe it is the earlier they'll do it.

They widen the valve to fix the stenosis or possibly replace the vave.

They close VSD, which also resolves the overriding aorta.

Overtime the hypertrophy will gradually disappear.

Mixed Blood Flow Defect

Cyanotic

Saturated Systemic blood and desaturated pulmonary blood mix in heart, aorta, and pulmonary vessels.

results in desaturation of systemic blood.

Most common defect: transposition of the great arteries

Most common defect of mixed blood flow.

Aorta comes off right ventricle and pulmonary artery leaves left ventricle. They've switched places.

Problem: there's no O2!!!

TX Mixed Blood Flow Defect

(Transposition of the Great Arteries)

Only thing that can save baby is a defect in the septum to let blood mix or a patent ductus. Otherwise they need immediate surgery!!!

If you give O2 to child it doesn't help. Usually this can be picked up on ultrasound.

Prostaglandin EI given IV maintains pantency of ductus arteriosis so blood can still mix.

Septostomy (balloon) inserted to make defect larger to allow more blood to mix.

By 1 week they NEED to have surgery to switch vessels and close septal defect as well.

S/SX: Mixed Blood Flow Defect

(Transposition of the Great Arteries)

Cyanosis doesn't improve with O2

Hypoxemia, CHF, Increased respiratory rate

Tachypnea, they need very freq rest periods

Mixed Blood Flow Defect 

(Transposition of the Great Arteries)

Prone to....

Acquired Heart Diseases

CHF

manifestations similiar to adults, but symptoms may be different in children.

S/SX: *tachycardia, crackles, edema, fatigue with feeding, tachypnea, flaring retractions, grunting, poor wt gain.

TX: diuretics, Digoxin (strengthen heart), promote oxygenation

Acquired Heart Disease

Kawasaki Disease

Acute systemic inflammatory illness

Also termed mucocutaneous lymph node syndrome.

S/SX present in 3 phases acute, subacute, convalescent.

Cause: unknown. Often seen later winter, early spring.

Can lead to cardiac complications affecting heart muscles and cardiac blood vessels.

Lasts 6-8weeks.

Acute: High fever, irritable, inflamm of conjuctiva, red throat, strawberry colored tongue, rash, edema in hands & feet, enlarged cervical lymph nodes. (lasts several weeks)

Subacute: Skin on lips, hands, and feet all slough off. C/O joint pain, serious cardiac complications can occur during this phase such as thrombosis, aneurysms of coronary arteries, and MI.

Convalescent: Symptoms gradually diminish. Energy & appetite returns.

Based on fever and 4 of these symptoms

-elevation of WBC and ESR (erthrocyte sed rate)

-conjuctivitis

-cervical lymphadenopathy

-rash

-changes in extremities & lymphadenopathy

EKG

TX of Kawasaki Disease

(acquired heart disease)

IV immunoglobin to reduce fever and imflammatory response.

ASA to reduce fever and it's an antiplatlet.

Close observation & comfort measures

Nutritional support clear liquid diet

ROM, minimize strain on heart.

Acquired Heart Diseases

Rheumatic Fever

Inflammatory Disease

Usually follows group A beta Strep trigged by autoiummune response.

Potential damage to heart, joints, CNS, and skin.

Most significant complication: cardiac valve damage

S/SX of Rheumatic Fever

acquired heart disease

Can be anywhere from few days to 6 weeks

Painful, inflammed joints, rash, lethargy, anorexia

Chorea- uncontrollable muscle tremors and emotional instability. Lasts a few weeks to 2 years. Primarily affects face and extremities. Can be mild-severe.

Cardiac effects-tachycardia & murmurs. Cardititis, major cause of permanent mitral and aortic valve damage.

DX & TX Rheumatic Fever

(acquired heart disease)

DX: Jones criteria along with lab tests

TX: aimed at preventing heart disease.

Bedrest to decrease cardiac workload

Steroids

Antibiotics to treat strep.

Bleeding disorder with hereditary link. Result is delayed coagulation of blood.

2 main forms: Factor VIII and Factor IX

X linked recessive disorder. Mom carries gene. Men usually get the disease.

Factor VIII: Hemophilia A. More common.

Factor IX: Hemophilia B/Christmas disease. Rare.

Bleeding into soft tissue or during invasive procedure.

Not seen initiatially.

Bleeding into joints (hemathrosis). Will be painful and edemitis. If not treated can cause severe deformitites.

Bruising easily. Epistaxis. Hematuria.

Bleeds into brain/tissue around airway.

PTT (partial thromboplastin time)

HX, assessment

Reveals low factor VIII or IX

Factor VIII or IX needed to form thromboplastin.

Classified as mild, moderate or severe. It's based on levels.

Factor VIII/IX replacement. Given IV.

Teach parent how to do it once child is past 2 years old. They need it throughout life.

Synthetic vasopressin (DDAVP) helps to stimulate factor VIII/IX. Used only for mild cases. 

Corticosterioids for hemoarthrosis.

NSAIDs rarely b/c they inhibit platlets.

NO ASA b/c of bleeding.

Narcotics occasionally for joint pain.

Ice packs, immobilization. NO HEAT!

Regular exercise to build muscle tone around joints.

Teach safety measures.

Unknown cause. Possible autoimmune response. Often follows viral infection.

2 forms: acute, chronic (6months/longer)

Decrease in number of platlets, rash because blood is leaking into skin.

S/SX: purpura, bleeding, hematuria, epistaxis, petichia.

DX: less than 2,000 platlet count

TX: varies with s/sx. can include corticosterioids, IV immunoglobin, platlets, splenectomy.

Spleen destroys the platlets. Remove if severe enough.

AVOID ASA and NSAIDs.

Hereditary Disorder where normal Hgb is replaced by Hgb S.

Causes the RBCs to take on sickle shape. You have trait or anemia.

If they get gene from 1 parent they have the trait. They'll have normal Hgb and no symptoms, but can pass it onto their children.

If both parents have trait there's a 25% chance with each pregnancy that child will develop anemia.

It primarily affects african americans, indians, mediterrean countries.

Asymptomatic for 4-6 months because of prescence of Hgb F. Prevents cells from getting into abnormal shape.

When symptoms do occur: Result of obstruction of vessels by sickled cells and increase RBC destruction/anemia.

Often triggered by periods of stress. Such as rapid growth spurt, illness, dehydration, increased exercise.

More sickle celled shapes get released into blood stream. Hypoxia, ischemia, and anemia occurs.

Abd pain, muscle spasms (b/c bloods not flowing there), joint pain and swelling, fever and nausea, pallor, coolness of skin.

Will impact the organs.

Heart-cardiomegaly, murmurs.

Lungs-pulmonary insufficiency, infection.

Kidneys-hematuria, inability to concentrate urine.

Liver- hepatomegaly, cirrhosis

Spleen-enlargement, fibrosis, infection

Extremities-Infection, degeneration of bones, leg ulcers, osteomyelitis

CNS-stroke, seizures, h/a, visual disturbances, aphasia, hemiparesis

Sickledex screen for Hgb S.

Fingerstick/heelstick.

If positive will do hemoglobin electrophoresis to confirm if trait or disease. It's done on newborns.

Prevent Crisis:

Promote hydration, minimize tissue hypoxia, freq rest periods, avoid highly strenous activities, avoid high altitudes, avoid infection, vaccinations, prophlatics antibiotics sometimes.

If have crisis:

Bedrest, hydration and electrolytes, analgesics, oxygen, blood transfusions. 

It's a chronic, life threatening disease. No cure, but can be managed!

Inherited disorder caused by abnormal hemoglobin synthesis.

Mediterrean descent. Both parents have to be carriers.

Results in destruction of RBC and leading to anemia.

Two types: Alpha and Beta (most common)

Anemia, H/A, fatigue, pallor, irritability, anorexia, bone pain, fx

Exercise is not tolerated well.

Liver and spleen may be enlarged. Gallbladder and pancreas can also be affected and can have heart failure.

Skin color changes due to deposits of hemosiderin. It gets deposited in the tissue. Give bronze tone.

Some will have structural changes in face and skull.

Genetic testing.

Symptoms.

Confirmed by Hgb electrophoresis

Management. Not curative.

Transfusions every 2-4 weeks.

Need chelating meds to prevent iron overload from transfusions.

Meds to prevent heart failure. Splenectomy (if enlarged significantly).

Often has slowed growth and delayed sexual maturity.

It's a chronic condition. They die from heart failure usually.

Acute Lymphoblastic Leukemia (ALL): lymphoblastic can be replaced with lymphocytic, lymphoid, or lymphoblastoid. They're all the same.

Acute Myeloid Leukemia (AML): Also known as myelogenous leukemia

Acute Leukemia

Acute Lymphoblastic Leukemia ALL

Lymphocytes divide rapidly but do not mature so they have no function. They release from the bone marrow.

Numbers increase and impact normal WBCs, RBCs, and platlets. They are still prone to infection.

It's the more common type. Has the best cure rate.

Acute Leukemia

Acute Myeloid Leukemia AML

Also known as acute myelogenous leukemia.

Rapid growth of abnormal WBCs that accumulate in bone marrow.

Interferes with production of normal WBCs, RBCs, and platlets.

Lower cure rate.

Diagnosed at age 2-6. Males have slightly higher incidence.

Based on hx, labwork.

Confirmed with bone marrow aspiration.

Symptoms are a result of anemia, infection, bleeding.

Fever, pallor, fatigue, anorexia, hemmorhage, bone and joint pain, *enlarged lymph nodes and spleen and liver, petichiae, bleeding gums, bruising, abd pain, distention, N/V.

Chemo therapy done in phases.

Induction: to achieve resmission. Give steroids.

Sanctuary: Keep abnormal cells from invading CNS. Intrathecal chemo. Do a lumbar puncture and put chemo right into cerebrospinal fluid.

Maintence: To maintain state of remission. Steriods. Phase lasts 2-3 years.

If relapse occurs additional therapy is needed. With each relapse it makes the prognosis worse.

Infection Prevention

Monitor for bruising and bleeding

Monitor kidney function

Watch fluid and nutritional status

Pain relief & emotional support.

ALL: try to hold off on bone marrow if relapse. AML: might choose to do bone marrow transplant if relapse.