Congenital Heart Disease Study Guide

The first concept to understand and trace are the changes that take place during the transition from intrauterine life to after birth. FETAL CIRCULATION -Blood enters Rt. Atrium -Most of blood flows to left side thru foramen ovale -To Lt. Ventricle -To Aorta -From Aorta to head and upper extremities -After circulation there blood returns to Rt. Atrium thru SVC -1/3rd of blood entering Rt. Atrium does not flow thru foramen ovale; instead stays in the right side of heart eventually flowing into PA

POST DELIVERY - Ductus Arteriosus begins to wither and close off with larger amts of oxygenated blood - Lung circulation increases blood flow to LA - Increased pressure causes Foramen Ovale to close and blood to circulate normally

Ductus Venosus: becomes the Ligamentum teres. Foramen ovale: should close off completely

Patent Ductus Arteriosus: should close off and become Ligamentum arteriosus when level of oxygenation increases

CON NE

REMEMBER BASIC HEMODYNAMICS -Resistance in the pulmonary circulation is lower than in the systemic circulation. -Therefore, pressures in right side of the heart are normally lower than in the left side of the heart

-Remember that the blood flow will take the path of least resistance and will flow from high pressure areas to low pressure areas.

-85% of Congenital Heart Disease is multifactorial in cause - 10%-12% due to Chromosomal/genetic abnormalities - 1-2% are due to maternal or environmental exposures Ie: Fetal alcohol syndrome-50% have CHD Maternal Illness: Rubella exposure in 1st 7 wks of pregnancy CMV, Toxoplasmosis, other viral illnesses cause cardiac defects. Diabetic Mother: 10% risk of CHD (VSD, cardiomyopathy, TGA most common).

Cardiac dysfunction>>hypoxia Maslows hierarchy: Hypoxia>>poor feeding, poor growth, poor development Infants with cardiac dysfunction will take longer to feed and use up extra calories working to breathe and suck at the same time. Nursing goal is to limit length of time for feeding to 30 minutes; and either feed more frequently or increase calories/oz. of formula or breast milk. Prenatal Hx: Preterm birth; Maternal Diabetes Mellitus ; Intrauterine growth retardation, more likely to have congenital anomalies than general population Family Hx: Freq fetal loss; sudden infant deaths; sudden deaths in adults>>Heart disease Hx of Congenital heart defects

Congenital Heart Disease Classifications

Increased Pulmonary Blood Flow Decrease Pulmonary Blood Flow Outlet Obstruction

Mixed Blood Flow

Characterized by Blood Flow Pattern within the Heart

Right side of heart is normally low pressure and left side is normally high pressure. In PDA (patent ductus arteriosus) the ductus connects the pulmonary artery to the aorta, bypassing the lungs.

Atrial Septal Defect

Opening between the atria Left Pressure is higher than R atrial pressure. Blood flows from L to R. Heart can become dilated, muscle can become weak. Because there is low pulmonary vascular resistance, blood backs up into the pulmonary vessels and the R ventricle becomes distended as well. But because it is under low pressure, this is often tolerated very well and the child may be asymptomatic. Rarely see CHF in uncomplicated ASD. Minimal symptoms of Pulmonary vascular changes until several decades of unrepaired ASD. Risk for atrial dysrhythmias and emboli formation in later life if unrepaired. TX: may be closed in cardiac catheterization procedure; surgical repair w/ patch usually before age 6.

Ventricular Septal Defect

Defect in ventricular septumError in early fetal development ; 20-25% of all CHDs are VSD ManifestationsDepends on size of VSD and degree of shunting. Can be pinhole size to absence of entire septum.Sm to moderated defects may close spontaneously within first yr of life. Pressure is higher in L ventricle than in the R ventricle and systemic arterial circulation offers more resistance than the pulmonary circulation, blood flows through the defect and into the pulmonary artery. R Ventricle becomes enlarged (hypertrophied); over time the R atria may also become distended. Symptoms: Characteristic Murmur; CHF is common; risk of Bacterial Endocarditis; risk of pulmonary vascular obstructive disease .REPAIRS: Cardiac Catheter repair common. Surgical repair w/ bypass; Pulmonary artery banding (if not too large) or patch

Patent Ductus Arterosus

Ductus SHOULD close by about age 15 hours after birth. Often corrects self within first 2 months 5-10% of all CHDs are PDA.

DUCTUS closes due to increased in arterial oxygen concentration that follows initiation of pulmonary function. DA allows blood to flow from left to right and X pulmonary blood flow Blood pumped out by LV>>some of it flows back into the pulmonary artery due to lower pressure .^ pressure in the pul art over time Manifestations: Small = asymptomatic Symptoms Large = may be CHF w/ tachypnea, dyspnea, and hoarse cry.

BOUNDING Peripheral pulses. Widened Pulse Pressure (>25)

Murmur (machinery murmur) at upper left sternal border or in L infraclavicular area Murmur audible throughout cardiac cycle DEFINITIVE DX: ECHO Management of PDA:

Preterm kids= INDOMETHICIN to close PDAs; surgical ligation if meds fail. Placed on prophylactic antibiotics to prevent bacterial endocarditis Surgery>> between age 1-2 yrs

Atrioventricular Canal

Is a combination of ASD and VSD that can encompass and open the entire cardiac septum. If the defect is large there is mixing of large amounts of oxygenated and unoxygenated blood. This causes increased blood volume on right side of heart, increased pulmonary blood flow, decreased systemic blood flow. This predisposes the child to cyanosis, CHF. Most common defect in Trisomy 21 (downs syndrome) population

Cya

D
Pulmonary blood flow is obstructed + have defect of ASD or VSD. >>Blood backs up in right side of heart. Causes desaturated blood to shunt to the left and into systemic circulation. Usually hypoxemic and usually cyanotic. Most common defects are TET & tricuspid atresia.

Tetralogy of Fallot

-Decreased pulmonary blood flow -Cyanotic (o2 sats 75-85%) -Must have four anomalies to meet criteria 1. Overriding Aorta 2. Pulmonary Stenosis 3. VSD 4. Right Ventricular Hypertrophy

- Suffer from TET Spells due to transient increase in resistance to blood flow to lungs. Causes increasingly desaturated blood flow to body. Leads to marked cyanosis/syncope/brain hypoxia/death.

Sometimes, babies with tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying, feeding, having a bowel movement, or kicking his or her legs upon awakening. These episodes are called "Tet spells" and are caused by a rapid drop in the amount of oxygen in the blood. Toddlers or older children may instinctively squat when they are short of breath. Squatting increases blood flow to the lungs. Tet spells are more common in young infants, around 2 to 4 months old.

Tricuspid Atresia
Tricuspid atresia is a heart defect present at birth (congenital) in which one of the valves (tricuspid valve) between two of the heart's chambers isn't formed. Instead, there's solid tissue between the chambers. If a baby is born with tricuspid atresia, blood can't flow from the lungs to the right atria and into the right ventricle and into the lungs to pick up oxygen as it normally would. The result is the lungs can't supply the rest of the baby's body with the oxygen it needs. Babies with tricuspid atresia tire easily, are often dyspneic and cyanotic. Blood instead flows from the upper right chamber to the upper left chamber through a hole in the wall between the septum. This hole is either a heart defect (atrial septal defect) or an enlarged natural opening (foramen ovale) that is supposed to close soon after birth. If a baby with tricuspid atresia doesn't have an atrial septal defect, the baby may need a procedure to create this opening emergently in order for any oxygenated blood to get to the systemic circulation. Tricuspid atresia is treated with surgery to place a valve (either human or pig donor). Most babies with tricuspid atresia who have surgery will live well into adulthood, though follow-up surgeries are often needed as the child grows.

- Transposition of Great Arteries

Transposition of the great arteries is a rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). Transposition of the great arteries changes the way blood circulates through the body, leaving a shortage of oxygen in blood flowing from the heart to the rest of the body. Without an adequate supply of oxygen-rich blood, the body can't function properly. Transposition of the great arteries is usually detected within the first few weeks of life. Corrective surgery soon after birth is the usual treatment for transposition of the great arteries. Having a baby with transposition of the great arteries is naturally worrisome to you as a parent, but with proper treatment, the outlook is promising. VSD is needed to mix blood. If no VSD is present one will be surgically enlarged. Child will be less cyanotic as oxygenated blood is introduced.

- Total Anomalous Pulmonary Venous Return

Total Anomalous Pulmonary Venous Return (TAPVR) is a rare congenital malformation in which all four pulmonary veins do not connect normally to the left atrium. Instead all four pulmonary veins drain abnormally to the right atrium by way of an abnormal (anomalous) connection. All types of Total Anomalous Pulmonary Venous Return have to have an atrial septal defect (ASD). Because none of the pulmonary veins connect normally to the left side of the heart (and thus out to the body) blood is shunted from the right atrium across the atrial septal defect. Absence of an atrial septal defect in Total Anomalous Pulmonary Venous Return is not compatible with survival. Surgical repair is performed emergently in the newborn period for newborns with Total Anomalous Pulmonary Venous Return and obstructed pulmonary veins. Some of these children will actually require extracorporeal life support (ECMO) prior to surgery because of their marked hemodynamic instability.

- Truncus Arteriosus

Normally there are two main blood vessels leaving the heart: the aorta carrying blood to the body and the pulmonary artery that branches immediately to carry blood to each lung. Instead of having a separate pulmonary artery and aorta, each with their own three-leafed valves, a baby with truncus arteriosus has only one great blood vessel or trunk leaving the heart, which then branches into blood vessels that go to the lungs and the body. This great vessel usually has one large valve which may have between two and five leaflets. Usually this great vessel sits over both the left and right ventricle. The upper portion of the wall between these two chambers is missing resulting in what is known as a ventricular septal defect (VSD). In rare cases, the ventricular septal defect is absent. Initial treatment begins with stabilizing the infant. Medications to control congestive heart failure such as diuretics are often begun. Ensuring good nutrition may require the use of a feeding tube or intravenous hyperalimentation. Surgical correction is typically carried out in the first few weeks of life after the infant is maximally stabilized. The surgical repair of truncus arteriosus requires the use of heart-lung bypass machine support. It involves three major components: 1) Separating the pulmonary arteries from the main truncus (the truncus will remain as the first part of the aorta);2) closure of the ventricular septal defect using a patch; 3) creating a connection between the right ventricle and the pulmonary arteries using a valved conduit, usually a homograft pulmonary artery.

- Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a congenital heart defect that occurs when the left side of the heart does not develop normally. The left ventricle, which normally pumps oxygen rich blood to the body, and the opening of the aorta, the vessel that carries that blood, are too small. The aortic and mitral valves that control the flow of blood into the left ventricle and out to the aorta are narrowed or completely closed. Treatment for hypoplastic left heart syndrome begins with rapid stabilization immediately after a child is born. Stabilization measures include the insertion of catheters and use of medications to keep open the communication between the left and right atria and optimally balance the flow of blood through the babys heart, lungs and the rest of the body. Treatment options would then include a series of operations, usually three, to reconfigure the childs cardiovascular system to be as efficient as possible despite the lack of an adequate left ventricle. A heart transplant is a less common treatment option. For a baby with HLHS and RAS, immediate treatment would include placing a catheter in the babys heart to create or enlarge the internal communication between the left and right atria and facilitate the flow of blood out of the lungs. This procedure is known as a catheter-based atrial septoplasty.

Mortality is high among infants who have atrial septoplasty after birth. These infants are usually very unstable, with severe hypoxemia and acidosis complicating treatment. Even with successful placement of the catheter, mortality ranges from 48 to 64 percent, with many dying while awaiting reconstructive surgery or a heart transplant.

Obstructive Defects
-Related to anatomic narrowing causing obstruction to blood flow. -Pressure before obstruction is increased -Pressure after obstruction is decreased or absent -Coarctation of the aorta

The aorta is narrowed near the insertion of the ductus arteriosus. Causes high blood pressure (above narowing) proximal to the defect. Causes high BP & bounding pulses in arms; weak or absent femoral pulses, and cool lower extremities with low BP. *KEY FINDING - ^ bp hi arms, Low bp legs Signs of CHF in infants. Condition can deteriorate rapidly particularly after closure of PDA Older kids may c/o dizziness, h/a, fainting and epistaxis from hypertension.

Pt at risk for ruptured aorta, aortic aneurysm, or stroke TX: Non-surgical= balloon angioplasty. Usually effective Surgical: Does not require bypass since defect is outside pericardium. Post-op complication is hypertension. Usually done before age 2 yrs.

-Aortic Stenosis

Aortic Stenosis refers to a condition that causes obstruction to blood flow between the left ventricle and the aorta. There are a variety of causes, including muscular obstruction below the aortic valve, obstruction at the valve itself, or aortic narrowing immediately above the valve. The most common form of aortic stenosis is obstruction at the valve itself, referred to as aortic valvular stenosis. The normal aortic valve consists of three thin and pliable valve leaflets. When the left ventricle ejects blood into the aorta, normal aortic valve leaflets spread apart easily and cause no obstruction to outflow of the blood from the heart. Aortic stenosis occurs when abnormalities of the aortic valve lead to narrowing and obstruction between the left ventricle and the aorta. The most common abnormality occurs when the aortic valve has only two (instead of three) leaflets. This is called a bicuspid aortic valve (or BAV). Often the valve leaflets are thickened and less pliable than normal, and the lines of separation between them (or "commissures") are fused together to a variable degree. When the aortic valve does not open freely, the left ventricle must work harder to eject blood into the aorta. To compensate for this additional workload, the muscle of the left ventricle gradually thickens to provide additional strength to left ventricular ejection. The increased left ventricular muscle, also known as hypertrophy, is rarely a problem itself. Instead, hypertrophy is an indication of significant valve obstruction.

When the aortic valve is severely obstructed, the left ventricular muscle may not be able to compensate satisfactorily. In these very severe, but fortunately rare, instances the left ventricle may fail to pump blood effectively and the patient may develop cardiac failure. If this occurs it is almost always in a newborn infant with very severe valve obstruction. Heart failure rarely occurs later in childhood.

-Pulmonic Stenosis
Pulmonary stenosis is a condition characterized by obstruction to blood flow from the right ventricle to the pulmonary artery.This obstruction is caused by narrowing or stenosis at one or more of several points from the right ventricle to the pulmonary artery. It includes obstruction from thickened muscle below the pulmonary valve, narrowing of the valve itself, or narrowing of the pulmonary artery above the valve.The most common form of pulmonary stenosis is obstruction at the valve itself, referred to as pulmonary valvular stenosis. The normal pulmonary valve consists of three thin and pliable valve leaflets. When the right ventricle ejects blood into the pulmonary artery, the normal pulmonary valve leaflets spread apart easily and cause no obstruction (blockage) to outflow of blood from the heart. Pulmonary valve stenosis occurs when abnormalities of the pulmonary valve lead to narrowing and obstruction between the right ventricle and the pulmonary artery. the pulmonary valve leaflets are thickened and fused together along their separation lines (commissures). leaflets become less pliable than normal, which contributes to the obstruction. At times, the diameter of the pulmonary valve itself is small or hypoplastic. right ventricle must work harder to eject blood into the pulmonary artery. To compensate for this additional workload, the muscle of the right ventricle (the myocardium) gradually thickens to provide additional strength to right ventricular ejection. Increased RV hypertrophy rarely a problem itself; more an indication that significant valve obstruction exists. When the pulmonary valve is severely obstructed right ventricle cannot eject sufficient volume of blood flow into the pulmonary artery. Blue blood bypasses the right ventricle flowing from the right atrium to left atrium, through the foramen ovale, a communication or "hole" between these two chambers that is normally present in newborns. Newborns with critical pulmonary stenosis therefore will have cyanosis (blue discoloration of the lips and nailbeds) due to lower oxygen levels in their blood. Treatment: Children with mild pulmonary valve stenosis rarely require treatment. Patients with mild pulmonary valve stenosis are healthy, can participate in all types of physical activities and sporting events, and lead normal lives. Mild pulmonary valve stenosis in childhood rarely progresses after the first year of life. However, mild pulmonary stenosis in a young infant may progress to more severe degrees and requires careful follow-up. With moderate-to-severe degrees of pulmonary stenosis require treatment, the timing of which is often elective.

Type of treatment required depends on the type of valve abnormality present. Most commonly, the stenotic pulmonary valve is of normal size, and the obstruction is due to fusion along the commissures or lines of valve leaflet opening. "Typical" form of pulmonary valve stenosis responds very nicely to balloon dilation. Balloon dilation valvuloplasty is performed at the time of cardiac catheterization and does not require open-heart surgery. In the newborn, balloon dilation for critical pulmonary valve stenosis can be a technically challenging procedure as these newborns are often critically ill and unstable. Older children; procedure is performed electively on an outpatient basis.

Clinica Cya
Polycythemia:
Severe heart or lung disease causes secondary polycythemia that limits oxygen delivery to tissues

Polycythemia

The body overproduces the hormone erythropoietin. Erythropoietin is in charge of regulating the bodys supply of red blood cells. When overproduced, erythropoietin can cause thickening of the blood, leading to the symptoms of polycythemia. Symptoms of polycythemia include symptoms related to excessive thickening of the blood, such as reddened face, bleeding of the gums, dizziness, and itchiness. Polycythemia can also affect the eyes and ears, leading to blurred vision or tinnitus. Polycythemia is treated by thinning the blood to keep clots from forming. This can be performed by periodic blood draws to reduce red blood cell count. Medications may be administered to suppress the bone marrow and reduce blood cell counts, including hydroxyurea and interferon. Aspirin may also be used to prevent blood clots, although this is less common due to an increased risk of stomach bleeding.

Clubbing:

Common symptoms of clubbing:

The nail beds soften. The nails may seem to "float" instead of being firmly attached. The angle that the nail makes with its cuticle increases. The last part of the finger may seem large or bulging. It may also be warm and red. The nail curves downward, similar to the shape of the round part of an upside-down spoon.

Clubbing can develop quickly, often within weeks. It also can go away quickly when its cause is treated.

CONGEST
Signs and Symptoms of CHF in the child: Tachypnea Tachycardia Dyspnea Retractions Activity/Feeding Intolerance Weight Gain

THE INAB PUMP AN BLOOD AT

 Hepatomegaly

CONGEST

IN CHILDRE STRUCTUR CARDIOMY ELECTROL

CONGEST

CHILDREN LEFT AND

CONGENI

DECREASE BLOOD FLO CAUSES RE OBSTRUC H2 O LESIONS

Treatmen

Medical Management of CHF:

Sodium and Fluid Restriction

Improve card Remove accu CONGEST Decrease card THERAPEU Improve tissu

IMPROVE

DIGOXI

 Digoxin: Increases cardiac output, left ventricular squeeze Decreases heart size, venous pressure, edema, heart rate
Dosing of digoxin is done by weight. Very important to educate parents in proper dose and administration and signs/sx of toxicity including nausea, vomiting, bradycardia.

Treatmen

Improve card Remove accu

CONGEST
*Daily weights will assess for increased fluid retention

NURSING C

I&O AND BED RES

PRIORIT

ASSESS F AND HYP OBSERVE

Review questions 1. Understand changes between fetal circulation and changes after birth. 2. Understand each anomaly. Is it intra-cardiac or extracardiac? 3. Is there more than one anomaly required to have the disease? 4. Differentiate between abnormalities that cause increased pulmonary blood flow and those that dont. 5. Differentiate between cyanotic and acyanotic disorders. 6. Which anomalies are obstructive and why? 7. What are the primary signs/sx of Coarctation found on the physical exam? 8. Understand cause, signs/sx, and treatment in pediatric CHF. 9. Digoxin; what is it used for, how is it dosed, what are signs/sx of overdose, what does the nurse teach the parent?
TET/Coarctation/ CHF pediatric signs and symptoms/ nursing management Use/signs and symptoms of overdose of Digoxin