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Cardiovascular and hematological disease in children

Sickle Cell Anemia Etiology

An autosomal recessive disorder which inserts an abnormal amino acid into hemoglobin and causes a gel like hgB which sickles in shape and decreases the O2 carrying capacity of RBCs.

Vaso-occlusive crisis

Painful episode- most common non-life-threating crisis. Characterized by ischemia causing mild to severe pain that may last mins. to days. Localized or general pain, migratory pain w/ presence of low-grade fever w/out an exacerbation of anemia. Result in variety of skeletal problems, like hand foot syndrome.

What factors contribute to increased sickling in Sickle Cell Anemia?

Hypoxemia, Extreme temperatures, decreased pH, increased plasma solutes and decreased plasma volume.

Symptoms of sickle cell anemia

Present after 6 months of age, pallor, irritable, jaundice, growth delay, Pain r/t occlusion, stroke, hand-foot syndrome

Treatment for sickle cell anemia

Activity regulation, O2 administration, monitor intake and output to assure adequate hydration, fluid/electrolytes, NSAIDs to Opioids for pain, heat compresses, Infection prevention (B19 parvovirus)

Labs for sickle cell anemia

RBC, hct, hgb, liver function

Differences between child and adult hematology

Infants produce RBC in liver and spleen whereas adults produce in the bone marrow, infants store catabolized iron in liver and marrow which prevents anemia, infant's have higher WBC counts than adults, and RBCs are higher in infancy.

Preop cardiovascular surgery nursing cares

History and physical exam, educate on procedure, assess allergies (iodine), Mark lower extremity pulses, NPO 4-6 hours prior to Tx, VS and O2 baseline, Pre-sedate as needed.

Post op cardiovascular nursing cares

Include caregivers in cares to assure competency by d/c, monitor cardiac function and O2 sats, Skin color/temp, insertion sites for bleeding, Dressing care, Intake and output, monitor for hypoglycemia, clear oral intake and assist to void, straight positioning of affected extremity 4-8 hours post procedure, educate on fatigue, nutrition, wound care and meds.

Congenital Heart defects

Anomalies present at birth, may spontaneously resolve or require surgery, often accompanied by other anomalies.

Acquired hear disease

Develops after birth secondary to infection or trauma, and requires treatment for resolution.

Hypo-plastic left heart syndrome etiology

Atresia of the mitral or aortic valve causes a dysfunctional/small left heart which blocks left outflow. Patent ductus arteriosis is needed for circulation.

Hypo-plastic Left Heart symptoms

Present within hours of birth up to 1 week after, cardiac failure, hypotension, tachycardia, and tachypnea.

Hypo-plastic Left Heart Treatment

Transplant or staged surgeries, PDA maintained with PGE1 and O2<75% until fixed

Hypo-plastic Left Heart Surgery: Norwood Procedure

1st surgery to repair hypoplastic left heart, The aorta is replaced with pulmonary artery, a BT shunt or Sano shunt is placed, and the atrial septum is destroyed.

Hypo-plastic Left Heart Surgery: Glenn procedure

The superior vena cava is attached to pulmonary inlet at 4-6 months of age

Hypo-plastic Left Heart Surgery: Fontan procedure

The inferior vena cava is attached to pulmonary inlet at 2-3 years of age.

Tetralogy of Fallot etiology

VSD, pulmonary stenosis, Right ventricular hypertrophy and overriding aorta decrease pulmonary blood flow.

Tetralogy of Fallot Symptoms

Moderate systolic murmur, tet (cyanotic) spells, hyperpnea, acidosis, clubbed digits, growth delays, exertional dyspnea, polycythemia causes clotting.

Tetralogy of Fallot Nursing care

Knee chest position for tet spells, morphine sulfate to decrease agitation r/t tet spells, blood volume resuscitation to prevent clots, O2 admin, IV phenylephrine to increase systemic vascular resistance, BT shunt placed until surgery at 3-12 months of age.

Coarctation of the Aorta etiology

A narrowed area of the aorta (normally by the PDA), causes blood back up into the left ventricle. Affects 5xs more boys than girls. Increased pulmonary blood flow.

Coarctation of the Aorta Symptoms

Symptoms present when the ductus arteriosus closes and are classic CHF, Pulsus paradoxus (greater than 20 mm Hg difference between upper and lower), headache, nosebleeds, leg pain, left ventricle hypertrophy.

Coarctation of the Aorta Treatment

balloon dilation in cath lab or anastamosis between the ages of 3-5 to prevent recurrence associated with infancy anastomosis. HTN meds given post surgery to prevent bleeds and residual HTN.

Increased Pulmonary Blood Flow etiology

Left to right blood shunting, Classic CHF symptoms including tachypnea, tachycardia, diminished pulses, decreased urine output, exertional respiratory distress, hepatomegaly, murmurs, ACYANOTIC

Decreased Pulmonary Blood Flow etiology

Right to Left blood shunting, hypoxia, dyspnea, Polycythemia r/t increased RBC production, hct >65%, murmurs, CYANOTIC

Why do pulmonary blood flow defects occur?

Abnormal connections between the left and right sides of heart.

Ventricular Septal Defect Etiology

Ventricles are connected and cause left to right shunting leading to pulmonary congestion.

Ventricular Septal Defect Symptoms

Classic CHF sx, holosystolic murmur, left heart hypertrophy. Diagnosed with ECHO

Ventricular Septal Defect Treatment

Before surgery, treat CHF sx with digoxin, ACE inhibitors, and diuretics (monitor for dig toxicity and potassium decreases), assure nutrition with small meals and tube feedings as needed.

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