Fetal Perfusion The Placenta:

The placenta is the means of metabolic and nutrient exchange between the fetal
and maternal circulatory systems.

The main functions of the placenta are fetal respiration, fetal nutrition and
excretion.

In the fully developed placenta, fetal blood flows through the 2 umbilical arteries
to the capillaries of the villi, becomes oxygen enriched and then flows back through the single umbilical vein into the fetus. Fetal Circulation:

 Most of the blood supply bypasses the fetal lungs- the placenta carries out gas
exchange in the fetus. Normal Fetal Blood Flow: Placenta Umbilical Vein Ductus Venosus (supplies blood to the liver) Superior and Inferior Vena Cava Right Atria (most goes to the Left Atria through the Foramen Ovale) Tricuspid Valve Right Ventricle Pulmonic Valve Pulmonary Artery Lungs Pulmonary Vein Left Atria Mitral Valve Left Ventricle Aortic Valve Aorta Systemic Circulation (some blood returns to the Pulmonary Artery through the Ductus Arteriosus to the Aorta and then to systemic circulation) Umbilical Arteries Placenta Mommy Dearest Fetal Shunts Ductus Venosus: The vascular channel in the fetus passing

through the liver and joining the umbilical vein with the inferior vena cava. Before birth it carries highly oxygenated blood from the placenta to the fetal circulation. It closes shortly after birth as pulmonary circulation is established and as the vessels in the umbilical cord collapse and become occluded.
Foramen Ovale: An opening in the septum between the right and the left atria in the fetal heart. This opening provides a bypass for blood that would otherwise flow to the fetal lungs. Most of the blood from the inferior vena cava in the fetus flows through the foramen ovale into the left atrium. After birth the foramen ovale functionally closes when the newborn takes the first breath and full circulation through the lungs begins. Ductus Arteriosus: a vascular channel in the fetus that joins the

pulmonary artery directly to the descending aorta. It normally closes after birth.

Conversion From Fetal to Neonatal Circulation: With the first breath, mechanical inflation of the lungs and an increase in oxygen saturation bring about a dramatic fall in pulmonary vascular resistance and, consequently, increased pulmonary blood flow. This leads to beginning constriction of the ductus arteriosus.

 As the pressures within the heart become relatively higher on the left side and lower on the right, the foramen ovale closes. The ductus venosus also constricts and eventually closes however the exact mechanism for this change is unkown.

Congenital Heart Defects That Increase Pulmonary Blood Flow Patent Ductus Arteriosus (PDA) PDA is a condition in which the normal fetal circulation conduit between the pulmonary artery and the aorta fails to close and results in increased pulmonary blood flow (left-toright shunt) PATHOPHYSIOLOGY: Patent ductus arteriosus (PDA) is the persistent patency of the ductus arteriosus after birth, which results in the shunting of blood directly from the aorta (higher

pressure) into the pulmonary artery (lower pressure). This left-to-right shunting causes the recirculation of increased amounts of oxygenated blood in the lungs, which raises demands on the left side of the heart. The additional effort required of the left ventricle to meet this increased demand leads to progressive dilation and left atrial hypertension. The cumulative cardiac effects cause increased pressure in the pulmonary veins and capillaries, which results in pulmonary edema. The pulmonary edema leads to decreased diffusion of oxygen and hypoxia, with progressive constriction of the arterioles in the lungs. Pulmonary hypertension and failure of the right side of the heart ensue if the condition is not corrected through medical or surgical treatment. Closure of the PDA depends primarily on the constrictor response of the ductus to the oxygen tension in the blood. Other factors affecting ductus closure include the action of prostaglandins, pulmonary and systemic vascular resistances, the size of the ductus, and the condition of the infant (premature or full-term). PDA occurs more frequently in premature infants; it is also less well tolerated in these infants, because their cardiac compensatory mechanisms are not as well developed and left-to-right shunts tend to be larger. CLINICAL MANIFESTATIONS: Patients may be asymptomatic or show signs of CHF. There is a characteristic machinery-like murmur. A widened pulse pressure and full bounding pulses Tachypnea Tachycardia Poor weight gain Diaphoresis Periorbital edema Frequent respiratory infections.

LAB/DIAGNOSTIC TESTS: Cardiac catheterization: Patent ductus and right ventricular hypertrophy evident. Chest Radiograph and ECG: Signs of ventricular hypertrophy noted. Echocardiogram: Septal defects and ventricular hypertrophy noted. MEDICAL MANAGEMENT: Surgical ligation of PDA is the treatment of choice Transcatheter closure by obstructive device is attempted in some older children Intravenous Indomethacin may stimulate the close of the ductus arteriosus in premature infants but cannot be used if CHF is present. Prophylactic antibiotics to prevent infective endocarditis are required until the PDA is closed. NURSING MANAGEMENT: Full cardiac and respiratory assessment Vital signs monitoring Cardiac output (color, pulses, perfusion, capillary refill, renal function, and neurologic function) Keep accurate record of intake and output. Weigh child or infant on same scale at same time of day Administer medications on schedule. Assess effectiveness of medications and any side effects noted. Be aware of drug-drug and drug-food interactions. Prevent fatigue during feeding; offer small frequent feedings to infant's or child's tolerance. Organize nursing care to allow child/infant uninterrupted rest. Promote activities that do not cause fatigue.

PATIENT EDUCATION: Provide explanation of diagnostic tests and why each test is performed because many of them are invasive procedures. Use simple words or pictures to describe procedures, and answer all questions the child and family may have. Educate child and family about care such as medication preparation and administration. Educate child and family about when to notify their physician of any clinical changes ATRIAL SEPTAL DEFECT (ASD) DESCRIPTION/PATHOPHYSIOLOGY: ASD is when there is an abnormal opening between the atria, allowing blood from the higher-pressure left atrium to flow into the lower-pressure right atrium (left to right shunt) 3 types of Atrial Septal Defects: Primum ASD: opening at lower end of septum; may be associated with mitral valve abnormalities Secundum ASD: opening near center of septum Sinus venosus defect: opening near junction of superior vena cava and right atrium; may be associated with partial anomalous pulmonary venous connection Because left atrial pressure slightly exceeds right atrial pressure, blood flows from the left to the right atrium, causing an increased flow of oxygenated blood into the right side of the heart. Although there is right atrial and ventricular enlargement, cardiac failure is unusual in an uncomplicated ASD. CHF may develop in adults who have had an undetected ASD for years. CLINICAL MANIFESTATIONS:

 Infants and young children typically have no symptoms CHF, easy tiring and poor growth can occur with a large ASD A soft systolic ejection murmur in the pulmonic area with wide fixed splitting of S2 through all phases of respiration. Tachypnea Tachycardia Poor weight gain Diaphoresis Periorbital edema Frequent respiratory infections. Enlarged right atrium LAB/DIAGNOSTIC TESTS: Echocardiogram identifies a dilated right ventricle due to blood overload and shunt size Chest radiograph and ECG reveal little information unless the ASD is large, has excessive shunting and right ventricular hypertrophy is present. MEDICAL MANAGEMENT: Spontaneous closure of some ASDs occurs within the first 4 years of life Surgery to close or patch the ASD is performed when significant increased pulmonary blood flow causes CHF or when spontaneous closure has not occurred by 4 years of age. Sucundum ASDs may be closed by a septal occluder during cardiac catheterization Smaller defects that have a rim around them for attachment of the device can be closed with a device; large, irregular defects without a rim require surgical closure.

 Successful closure in appropriately selected patients yields results similar to surgery, but involves shorter hospital stays and fewer complications. Surgical closure is still considered the standard therapy as long-term outcomes following device closure are yet to be defined. NURSING MANAGEMENT: Full cardiac and respiratory assessment Vital signs monitoring Cardiac output (color, pulses, perfusion, capillary refill, renal function, and neurologic function) Keep accurate record of intake and output. Weigh child or infant on same scale at same time of day Administer medications on schedule. Assess effectiveness of medications and any side effects noted. Be aware of drug-drug and drug-food interactions. Prevent fatigue during feeding; offer small frequent feedings to infant's or child's tolerance. Organize nursing care to allow child/infant uninterrupted rest. Promote activities that do not cause fatigue

PATIENT EDUCATION: Provide explanation of diagnostic tests and why each test is performed because many of them are invasive procedures. Explain any invasive or surgical procedures that will be done to the patient and family. Use simple words or pictures to describe procedures, and answer all questions the child and family may have.

 Educate child and family about care such as medication preparation and administration. Educate child and family about when to notify their physician of any clinical changes VENTRICULAR SEPTAL DEFECT (VSD) DESCRIPTION/PATHOPHYSIOLOGY: VSD is an abnormal opening between the right and left ventricles. May be classified according to location: membranous or muscular. May vary in size from a small pinhole to absence of the septum, which results in a common ventricle. Ventricular septal defects (VSDs) are frequently associated with other defects, such as pulmonary stenosis, transposition of the great vessels, patent ductus arteriosus, atrial defects, and coarctation of the aorta. Many VSDs will close spontaneously. Spontaneous closure is most likely to occur during the first year of life in children having small or moderate defects. A left-to-right shunt is caused by the flow of blood from the higher-pressure left ventricle to the lower-pressure right ventricle. Because of the higher pressure within the left ventricle and because the systemic arterial circulation offers more resistance than the pulmonary circulation, blood flows through the defect into the pulmonary artery. The increased blood volume is pumped into the lungs, which may eventually result in increased pulmonary vascular resistance. Increased pressure in the right ventricle as a result of left-toright shunting and pulmonary resistance causes the muscle to hypertrophy. If the right ventricle is unable to accommodate the increased workload, the right atrium may also enlarge as it attempts to overcome the resistance offered by incomplete right ventricular emptying. Defects left unrepaired will most likely cause pulmonary vascular disease. CLINICAL MANIFESTATIONS:

 A systolic murmur (blowing sound) is auscultated at the third or fourth left intercostal space at the sternal border. Failure to thrive Tachypnea Tachycardia Poor weight gain Diaphoresis Periorbital edema Frequent respiratory infections. LAB/DIAGNOSTIC TESTS: Chest radiograph and ECG reveal little when the VSD is small. Large VSDs with shunting show an enlarged heart and pulmonary vascular markings. Right and left ventricular hypertrophy may be seen on ECG. Echocardiogram will show shunting (if present) Cardiac Catheterization: Increased oxygen in the right ventricle and increased systolic pressure in the right ventricle and pulmonary artery. MEDICAL MANAGEMENT: Most small VSDs close spontaneously within the first 6 months of life Treatment is conservative when no signs of CHF or pulmonary artery hypertension are present. Surgical patching of the VSD is performed when poor growth is evident. Device closure of VSD may be performed on some patients during cardiac catheterization. NURSING MANAGEMENT (same as PAD and ASD)

PATIENT EDUCATION (same as PAD and ASD)

TETRALOGY OF FALLOT DESCRIPTION/PATHOPHYSIOLOGY: The classic form includes four defects: ventricular septal defect (VSD, pulmonic stenosis, overriding aorta, and right ventricular hypertrophy. Some children have a fifth defect- ASD. The alteration in hemodynamics varies widely, depending primarily on the degree of pulmonary stenosis, but also on the size of the VSD and the pulmonary and systemic resistance to flow. Because the VSD is usually large, pressures may be equal in the right and left ventricles. Therefore the shunt direction depends on the difference between pulmonary and systemic vascular resistance. If pulmonary vascular resistance is higher than systemic resistance, the shunt is from right to left. If systemic resistance is higher than pulmonary resistance, the shunt is from left to right. Pulmonic stenosis decreases blood flow to the lungs and, consequently, the amount of oxygenated blood that returns to the left side of the heart. Depending on the position of the aorta, blood from both ventricles may be distributed systemically. CLINICAL MANIFESTATIONS: As the ductus arteriosus closes, the infant becomes hypoxic and cyanotic with the degree of pulmonary stenosis determining the severity of symptoms. A systolic murmur is heard in the pulmonic area and transmitted to the suprasternal notch. A thrill may be palpated in the pulmonic area Polycythemia, hypercyanotic spells, metabolic acidosis, poor growth, clubbing and exercise intolerance may develop.

 Toddlers with uncorrected defects instinctively squat (assume a knee-chest position) to decrease the return of systemic venous blood to the heart. LAB/DIAGNOSTIC TESTS: Chest X-ray shows a boot shaped heart due to the large right ventricle, decreased pulmonary vascular markings and a prominent aorta The ECG show right ventricular hypertrophy The echocardiogram shows the VSD, obstruction of pulmonary outflow, an overriding aorta and the size of the pulmonary arteries. Blood tests reveal and elevated HcT and HgB and an increased clotting time. MEDICAL MANAGEMENT: Management of cyanotic spells Monitoring for acidosis Palliative surgery to delay total correction surgery in some patients. Total repair is often performed before 6 months of age when the infant has a hypercyanotic epidsode. NURSING MANAGEMENT: Vital signs monitoring Respiratory assessment Cardiac assessment- Cardiac output (color, pulses, perfusion, capillary refill, renal function, and neurologic function), Heart sounds, Dysrhythmias Monitor for any neurologic changes. Monitor all intravenous lines for air. No air can be allowed in any IV line. Keep accurate record of intake and output.

 Monitor for adequate hydration. Weigh child or infant on same scale at same time of day. Following shunt placement: Monitor shunt patency by auscultating over the shunt and hearing the shunt murmur. Monitor for increased cyanosis. Monitor platelet levels. PATIENT EDUCATION: Teach the family signs and symptoms of dehydration. Teach the family signs and symptoms of hypercyanotic spells. Teach the family how to manage a hypercyanotic spell. Knee-chest position Notify the child's physician immediately. Educate child and family about care such as medication preparation and administration. Discuss SBE (subacute bacterial endocarditis) prophylaxis Provide explanation of diagnostic tests and why each test is performed because many of them are invasive procedures. Explain any invasive or surgical procedures that will be done to the patient and family. Use simple words or pictures to describe procedures, and answer all questions the child and family may have.