Alterations in Oxygenation

CONGENITAL HEART DISEASE

ACYANOTIC HEART DISEASE = INCREASED pulmonary blood flow

- Blood moves from ARTERIES to VEINS / LEFT to RIGHT shunts

Oxygenated to deoxygenated blood

- Due to a defect that permits the passage of blood from a higher pressure left cardiac chamber (LA / LV) to a lower pressure right
cardiac chamber (RA / RV)

- RESULT: Heart functions as an ineffective pump, thus making the child prone to heart failure.

1. VENTRAL SEPTAL DEFECT

: most common type of congenital heart disease
: there is an opening in the ventricular septum
: pressure in the LV is greater thus pushing blood to the RV
: impairs the effort of the heart

ASSESSMENT DIAGNOSIS
- Easy fatigue - Echocardiography with color flow
- Dyspnea - Doppler
- Swelling of extremities - ECG (reveals ventricular hypertrophy)
- Crackles - MRI (reveals RV hypertrophy & possible pulmonary artery dilatation from the ↑ blood flow)
- Sweating
- Low growth rate
- Loud, harsh pansytolic murmur on left sternal border, 3rd or 4th interspace
- Thrill (variation)

THERAPEUTIC MANAGEMENT
- Cardiac catheterization (to close in moderate size) - Digoxin
- Open heart surgery (if the opening is > 3mm) - Diuretics

2. ATRIAL SEPTAL DEFECT

: abnormal communication bet. the two atria bec. of an opening between them
: blood shifts from left to the right atrium
: more common in girls than boys
a. Ostium Premium (ASD1) – opening is at the lower end of the septum
b. Ostium Secundum (ASD2) – opening is near the center of the septum

ASSESSMENT
- Harsh systolic murmur over the 2nd or 3rd interspace (pulmonic area)
- --- d/t extra amount of shunted blood that crosses the pulmonic valve leading to the fixed splitting of second heart sound
- Enlarged right side of the heart via echocardiography DIAGNOSIS
- Increased pulmonary circulation - Echocardiography
- Separation in the atrial septum - Cardiac catheterization
- Increased SPO2 in the right atrium - Doppler

THERAPEUTIC MANAGEMENT
- Surgery to close the defect (edges of the septum are approximated & sutured with CCath technique if the defect is small)
- Open heart surgery & CP bypass for large defects (a Silastic or Dacron patch may be sutured into place to occlude the space)
 ** Done between 1 and 3 years of age.

3. PATENT (open) DUCTUS ARTERIOSUS

: DA is an accessory fetal structure that connects the pulmonary artery to the aorta
: closing begins with the first breath & is completed at 7-14 days of age
: full closure occurs until 3 months
: more common in girls than boys
** If it fails to close at birth, O2 blood will escape from the aorta to the
pulmonary artery (deoxygenated blood) bec. of increased pressure in the aorta.
** The shunted blood returns to the PA – Lungs – LA – LV – Aorta – PA & the cycle
continues.
** Remains open IU d/t the stimulation of prostaglandin (PGE1) from the placenta & low oxygen level of fetal blood.
** After birth, when the PGE1 level falls & the oxygen level increases, the ductus arteriosus is stimulated to close.
EFFECT: Increased pressure in the pulmo. Circu. from the extra shunted blood = RV hypertrophy & Ineffective heart action
Extra blood to lungs stresses & adds pressure to the lungs overtime = Damages & narrows lung arteries = Pulmo. HTN
RISK FOR: Decreased O2 Lung infection
Heart failure Endocarditis (d/t increased pressure damages the heart’s lining & increases risk of bacteria)

ASSESSMENT
- Wide pulse pressure / the difference bet. systolic and diastolic
- Low diastolic pressure d/t the shunt / run off of blood that reduces resistance
- Normal ECG but may show ventricle enlargement if the shunt is large
- Machinery murmur heard at the upper left sterna border or under the clavicle in older children
C ardiac (machinery murmur, endocaritis, ↑HR, ↓O2, wide pulse pressure, low diastolic p., heart failure, crackles)
A ctivity intolerance
L ungs (risk for infections & feeding problems)
L oss of weight (body burns calories in order to breath)

DIAGNOSIS
- Echocardiography = provides good visualization of patent ductus arteriosus

THERAPEUTIC MANAGEMENT
- Ibuprofen (drug of choice) / Indomethacin via IV --- if does not close spontaneously
- Assess the following: reduced glomerular filtration, impaired platelet aggregation, diminished GI & cerebral blood flow
- Prophylaxis for preterm infants
- Prostaglandin inhibitors
- Insertion of Dacron (coated stainless steel coils inserted by CCath – for 6 mts. to 1 y/o) --- if medical management fails
- Ductal ligation --- major surgery of opening the chest (thoracotomy) & manipulating the great vessels
CYANOTIC HEART DISEASE = DECREASED pulmonary blood flow

- Blood moves from VEINS to ARTERIES / RIGHT to LEFT = deoxygenated blood invades the systemic circulation
Deoxygenated to oxygenated blood

- Systemic venous return (unoxygenated blood) bypasses the lungs & enter general circulation directly
Obstruction in the pulmonary artery

1. TRICUSPID ATRESIA
: tricuspid valve is completely closed, thus blood cannot flow from the RA to the RV
: blood then crosses the patent foramen ovale into the LA, bypassing the lungs
: closed TV causes extreme cyanosis, tachycardia, & dyspnea in infant

Management
- IV infusion of PGE1 --- to ensure that ductus remains open
- Fontan procedure / Glenn Shunt baffle --- construction of a vena cava to the pulmo. artery to deflect more blood to the lungs

2. TETRALOGY OF FALLOT
: 4 anomalies are present = VSD
Hypertrophy of RV --- d/t the extra effort to force blood thru the stenosed/narrowed pulmo. artery
Dextroposition (overriding) of the aorta
Pulmonary stenosis --- pressure builds up in the right side of the heart
--- blood shunts bec. of increased pressure into the LV & overrides the aorta
Assessment
- Skin is cyanotic
- Polycythemia --- body attempts to provide enough RBC to supply O2 to all body parts
- Thickening of blood => Clot formation => Thrombophlebitis, Embolism, Cerebrovascular accident
- Severe dyspnea
- Growth restriction
- Clubbing of the fingers
- Syncope (fainting)
- Hypercyanotic episodes (spells) --- caused by decreased blood and oxygen supply to the brain
- Cognitive challenge
- Loud, harsh, widely transmitted murmur or…
- Soft, scratchy localized systolic murmur in the left 2nd, 3rd, or 4th parasternal interspace
** The child assumes a SQUATTING / KNEE CHEST position when resting.
** Squatting gives physiologic relief to an overstressed heart by trapping blood in the lower extremities.

Diagnosis
- Echocardiography shows the enlarged chamber of the right side of the heart
- Decrease in the size of the pulmonary artery
- Reduced blood flow through the lungs
- Cardiac catheterization permits a definitive evaluation of the extent of the defect, esp. pulmonary stenosis & VSD
Laboratory findings: Polycythemia Total RBC count
Increased Hgb Oxygen saturation
Hematocrit
Management
- Place the baby in a knee chest position --- to trap blood in the lower extremities & keep the heart from being overwhelmed
- Surgery --- to connect the heart defects done at 1-2 y/o
- Administer oxygen
- Administer morphine sulfate
- Oral Propanolol, ex. Inderal – a beta blocker --- to aid pulmonary artery dilation
- Blalock - Taussig procedure
- = creates a shunt bet. the aorta & pulmonary artery (creating a ductus arteriosus)
- = will allow blood to leave the aorta
- = will allow blood to enter the PA, oxygenate the lungs, & return to the left side of the heart > aorta > body
- Brock procedure
- = a repair that relieves pulmonary stenosis, VSD, & overriding aorta

POSTOPERATIVELY:
- Observe arrhythmias --- may result from any ventricular septal repair, edema, or conduction interference.

NOTE:
- No palpable pulse in the right arm because the subclavian artery is used.
- No taking of BP and venipuncture in the affected arm.
OBSTRUCTION OF BLOOD FLOW

- A vessel or valve becomes narrower than usual

- Blood flow pressure increases PRIOR to the narrowing & decreases AFTER the narrowing
- Prohibits enough blood from reaching the lungs & the rest of the body
- Threatens / overwhelms the heart because of back pressure

1. PULMONARY STENOSIS
: narrowing of the distal portion of the PA /PV (distal to the valve)
: leads to RV hypertrophy which disables the RV to evacuate blood by way of the PA
: 10% of congenital anomalies
: can expect a normal life span

ASSESSMENT
- Cyanosis (if narrowing is severe) - Thrill
--- d/t inability of the blood to reach the lungs for oxygenation - Widely split of the 2nd heart sound
/ R-L shunting across the foramen ovale => happens bec.
of increased pressure at the right side of the heart
- Systolic ejection murmur (grade IV or V / crescendo to decresendo in quality)
--- loudest at the upper left sterna border radiating to the suprasternal border
--- children may have residual heart murmur

DIAGNOSIS
- ECG = reveal ventricular hypertrophy
- Cardiac catheterization = used for interventional enlargement of the stenosed valve

THERAPEUTIC MANAGEMENT
- Balloon angioplasty = procedure of choice
--- a catheter with an uninflated balloon at its tip is inserted and passed through the heart into the stenosed valve
--- as the balloon is inflated, it breaks the valve adhesions and relieves the stenosis

2. AORTIC STENOSIS
: stricture of the aortic valve that prevents blood from passing from the LV > Aorta
: heart cannot force blood through the narrowed valve => Increased pressure & LV hypertrophy
: if there is acute LV pressure => Increased pressure in LA, Back pressure in PV, Pulmonary edema

ASSESSMENT
- Asymptomatic
- Murmur --- can be transmitted to the right shoulder, clavicle, up the vessels of the neck, heart apex
- Rough systolic sound --- heard loudest in the 2nd right interspace (aortic space)
- Thrill --- at supra sterna notch
- Decreased cardiac output
- Faint pulses
- Hypotension
- Tachycardia
- Inability to suck for long periods
- Chest pain similar to angina --- bec. the coronary arteries receive inadequate amount of oxygen needed by the heart muscle
on exertion far exceeds what is available

DIAGNOSIS
- ECG = reveal left ventricular hypertrophy
 THERAPEUTIC MANAGEMENT
- Balloon valvuloplasty (treatment of choice)
- Beta-blocker / alcium channel blocker --- to reduce cardiac hypertrophy before the defect is corrected
- Dividing the stenotic valve / dilating an accompanying constrictive aortic ring
- If prosthetic valve is used --- continue to anticoagulation / antiplatelet therapy and antibiotic prophylaxis against endocarditis

** May lead to aortic valve insufficiency later in life & further surgery may be needed.
** Some children will need artificial valve replacement for correction,
** Children need exercise testing before participating in competitive sports if an artificial valve is in place.

3. COARCTATION OF AORTA
: narrowing of the lumen of the aorta due to constricting band making it difficult for blood to pass
: occurs most frequently in boys than in girls
: leading cause of congestive heart failure in the first few months of life
Preductal –constriction is bet. the subclavian artery & ductus arteriosus
Postductal – constriction is distal to the ductus arteriosus

RESULT: BP increases proximal to the coarctation & decreases distally

↑ BP in the heart & upper portions of the body as pressure in the subclavian artery increases
Headache, Vertigo, Epistaxis (nose bleed), Cerebrovascular accident

ASSESSMENT
- Pulse is weak, delayed, or absent
- Pulse in the upper extremities are rapid & bounding
- Absent palpable femoral pulses --- d/t slight coarctation
- Absent brachial pulses --- if there is an obstruction proximal to the left subclavian artery
- Lower BP in lower extremities --- d/t diminished blood supply to lower extremities
- Higher BP in upper extremities --- d/t the pull of gravity
- Headache
- Epistaxis
- Cold feet
- Muscle spasms
- Leg pain on exertion
- Collateral arteries enlargement
- Seen on the ribs as obvious nodules as the child grows older
- Soft, moderately loud systolic murmur --- from the base of the heart => left interscapular area
** Always include evaluation of femoral pulses in all initial newborn assessment and admission inspections.

DIAGNOSIS
- BP in the arms will be at least 20 mmHg higher than the legs
- ECG = reveal left-sided heart enlargement from back pressure & notching of the ribs from the enlarge collateral vessels
- MRI
- X-ray

THERAPEUTIC MANAGEMENT
- Interventional angiography (balloon catheter) / Surgery
= narrowed portion of aorta is removed & new ends of aorta are anastomosed (connecting healthy sections of tubular
structures after the diseased portion has been surgically removed)
= a graft of transplanted subclavian artery may be necessary if the narrowed section is so extensive than an anastomosis
cannot be accomplished readily
- Digoxin is given before surgery
- Diuretics will reduce the severity of congestive heart failure from hypertension

PLANNING IS IMPORTANT:

It would be ideal if children could achieve the greater part of their adult height before surgical correction, preventing strain on
the incision site as they grow.

In terms of self-image – correction is best done before children begin to think of themselves as chronically ill or before they
develop complications such as chronic hypertension.

Girls must have the defect repaired before childbearing age – or the extra blood volume during pregnancy can cause heart
failure.

Surgical repair is scheduled by 2 years of age.

After operation – abdominal vessels receive more blood resulting to abdominal pain or generalized abdominal discomfort.

Some may have elevated upper body hypertension after the repair.

Need continual treatment with antihypertensive agents.

Some may require repeat balloon angioplasty at adolescence to re-enlarge the aortic lumens and help reduce this upper body
hypertension.
MIXED BLOOD FLOW

- Mixing of blood from the pulmonary & systemic circulation in the heart chambers
- Results in a relative deoxygenation of systemic blood flow, although a cyanosis is most always visible

1. TRANSPOSITION OF GREAT ARTERIES

: aorta arises from the RV instead of the left & pulmonary artery arises from the LV instead of the right
: blood enters the heart from the VC => RA => RV => Aorta => Body => Completely deoxygenated blood returns to VC
: a secondary source of blood enters the heart from the PV => LA => LV => PA => Lungs => Oxygenated blood returns to LA
: atrial & ventricular septal defects occur in connection with transposition
: occur in large newborns (9-10lbs); most often in boys

ASSESSMENT
- Cyanotic from birth
- Enlarged heart
- Heart changes
- Low oxygen saturation

DIAGNOSIS
- Echocardiography – reveals enlarge heart
- ElectroCG – reveals heart changes
- Cardiac catheterization – reveals low oxygen saturation

THERAPEUTIC MANAGEMENT
PGE1 / Prostaglandin
= if no septal defect; to keep the ductus arteriosus patent
Balloon Atrial Septal Pull-through Operation
= deflated balloon catheter is passed from the RA => Foramen ovale => LA
= balloon is inflated & the catheter is drawn back into the RA to enlarge the opening of the FO & create an artificial ASD
= done at 1 week to 3 months of age
= involves an arterial switch procedure in which the major vessels are switched in position
= survival rate is 95%

2. TOTAL ANOMALOUS PULMONARY VENOUS RETURN

: PV returns blood to the RA / SVC instead to the LA
: for blood to reach the systemic circulation, it must shunt across a patent / open foramen ovale or a ductus arteriosus

ASSESSMENT THERAPEUTIC MANAGEMENT

- Absent spleen - Surgery to reimplant the pulmonary veins into the left atrium
- Mildly cyanotic - Balloon Atrial Septal Pull-through
- Easily gets tired - Maintain on a continuous IV infusion of PGE1 to help keep the ductus arteriosus open

3. HYPOPLASTIC LEFT HEART SYNDROME

: LV is nonfunctional = it lacks adequate strength to pump blood into the systemic circulation
: causes the RV to hypertrophy as it tries to maintain the entire heart action
: may have accompanying mitral / aortic valve atresia

ASSESSMENT
- Mild to moderate cyanosis
- Deoxygenated blood is shunted across the foramen ovale d/t greater pressure on the right
THERAPEUTIC MANAGEMENT
- Ultrasound (prenatally) - Prostaglandin therapy --- to maintain a PDA & increase blood supply to the aorta
- Echocardiography - Heart transplant --- to prolonged the child’s life