Sherpath- Congenital heart disease

How can a nurse distinguish between a patient with hypoplastic left heart syndrome and truncus arteriosus?

Oxygenated blood flows to left atrium in hypoplastic left heart syndrome, whereas oxygenated blood flows into left ventricle where blood mixing occurs in truncus arteriosus. These findings will help a nurse distinguish patient with hypoplastic left heart syndrome from one with truncus arteriosus. In hypoplastic heart syndrome, most of oxygenated blood cannot leave aorta and is instead shunted to right side back in to pulmonary circulation.

What is the benefit of maintaining a patent ductus arteriosus (PDA) for a child with tetralogy of Fallot?

PDA will help to maintain the pulmonary blood flow. Tetralogy of Fallot is associated with pulmonary stenosis and right ventricular outflow tract obstruction. This leads to a right-to-left shunt. Maintaining the PDA will create an alternative pathway for blood to take to the lungs and therefore help to oxygenate blood and reduce hypoxemia experienced by the child.

A neonate has cyanosis and increased pulmonary blood flow. The family is confused about how increased blood flow to the lungs would decrease oxygen levels in the body. What can the nurse say to explain this pathophysiology?

Anatomical features present in this child prevent blood from becoming fully loaded with oxygen in the lungs before going out to the body. The body appears cyanotic due to the lower than normal oxygen levels. Truncus arteriosus, hypoplastic left heart syndrome, and transposition of the great arteries are three examples of congenital heart disease (CHD) that have cyanosis with increased pulmonary blood flow. Mixing of blood from the right and left sides prevents fully saturated blood from entering systemic circulation, therefore cyanosis is present.

Which statement explains the development of pulmonary hypertension in an infant with a large left-to-right shunting defect?

Any congenital shunt can increase blood volume in the heart affecting both the pulmonary vein and pulmonary artery in delivering blood to the lungs and from the lungs to the heart respectively. The pulmonary vasculature responds to this increased load by vasoconstriction leading to pulmonary hypertension. Any congenital heart defect that leads to fluid overload in the ventricles can affect the pulmonary vasculature. If more work is required by both the pulmonary artery and pulmonary vein, then the pulmonary vasculature will respond by vasoconstricting leading to pulmonary hypertension. This can further damage the alveoli and cause the respiratory problems often seen in children with congenital heart defects.

The nurse is caring for a patient suffering from a cyanotic heart lesion and has just inserted an IV line. Moments later the child appears to be in moderate distress and action is required. Which clinical manifestation should require the most immediate action?

Changes in neurologic status Any child with a right-to-left shunt is at increased risk of arterial and venous blood mixing. If air is introduced to an IV line, the venous blood obtaining this air can enter the arterial system and send an air embolus to the brain causing a cerebrovascular accident. This child will require immediate attention since changes in neurologic status can be evidence of a stroke.

How should the nurse explain the reversal of a cardiac shunt due to pulmonary hypertension that went untreated?

Changes in the pulmonary blood vessels led to significantly increased pulmonary pressures. The pressure differences changed the direction of the shunt and now less blood is pumped to the lungs for adequate oxygenation therefore causing cyanotic spells in the child. Left-to-right shunts cause increased pressure in the pulmonary vein thereby leading to pulmonary hypertension in the child. This creates a thickened right ventricle therefore impeding blood flow in the left-to-right direction. The flow therefore reverses through the shunt bypassing adequate oxygenation.

Closure of the ductus arteriosus occurs shortly after birth. Children born with right-to left shunts begin to experience an increase in cyanosis with this closure. Which explanation describes the pathophysiology of this clinical manifestation?

Closure of ductus arteriosus decreases the volume of blood going to the lungs for oxygenation. In a right-to-left shunt, deoxygenated blood mixes with the oxygenated blood on the left side of the heart. This can decrease blood flow through the pulmonary trunk. When there is a PDA, mixed blood from the left side can go to the lungs for oxygenation. When the ductus arteriosus closes, this alternate pathway for oxygenation is lost.

A child has been diagnosed with hypoplastic left heart syndrome. Which action by the nurse will be a priority for providing long term management of this child?

Contact provider as surgical consult is needed This child will need a surgical consult in order to be assessed for a 3 step surgical procedure called "staged palliation". This procedure is for long-term management and is meant to correct the blood flow pattern in and out of the heart and help to improve the child's blood oxygenation.

A mother is concerned that her child suffering from heart failure (HF) has started to experience abdominal pain. What explanation by the nurse can be provided to the mother to help her understand this coexisting condition?

Fluid overload causing congestion can lead to abdominal pain. Since many children with heart defects experience right-sided heart failure, they can experience congestion in the abdomen due to inadequate delivery of blood back to the heart.

During development, pulmonary veins may aberrantly attach to the superior vena cava. What are the possible outcomes or clinical manifestations of this anomaly?

Cyanosis Cyanosis will occur because oxygenated blood is not sent to systemic circulation. Tachycardia Tachycardia occurs in an effort to increase cardiac output (CO) and increase oxygen delivery. Peripheral edema There will be decreasing peripheral pulses that would not promote edema. Mixed blood will be delivered to systemic circulation Pulmonary arteries deliver oxygenated blood to the deoxygenated right side of the heart. This causes a mixing of blood. There must be an open PDA to allow blood to flow to left side of the heart and distribute mixed blood to systemic circulation.

What physiological changes are augmented by strenuous exercise and high altitudes in the patient with pulmonary arterial hypertension (PAH)?

Cyanosis Patients with PAH can develop cyanosis with strenuous exercise and high altitudes as pulmonary blood flow rate may not be adequate. Peripheral edema Peripheral edema is possible as the right side of the heart is volume- and pressure- overloaded. Right-sided heart hypertrophy The right side of the heart can develop hypertrophy due to increased pressure within pulmonary arteries.

Which statement explains the appearance of cyanosis in an individual with polycythemia secondary to congenital heart disease (CHD)?

Cyanosis will appear in the presence of higher oxygen saturation levels (less desaturated) than normal. In children with polycythemia, cyanosis will appear when hemoglobin is less desaturated.

Pulmonary artery stenosis can precipitate regurgitation and prevent the closure of the foramen ovale. Which manifestation can occur if the foramen ovale remains patent in a patient with pulmonary stenosis?

Decreased oxygenation of blood entering systemic circulation The patent foramen ovale allows the blood to bypass the pulmonary circulation. Blood contains lower oxygen saturation levels when entering systemic circulation.

A child with aortic stenosis may experience symptoms of myocardial infarction (MI). How can these symptoms occur in a patient without vascular disease?

Decreased pressure in the aorta results in decreased elastic recoil. Subsequently, less blood is delivered to the coronary arteries. Aortic stenosis results in high left ventricular pressures as the blood has difficulty leaving the ventricle through the stenosis. This prevents a normal increase in aortic stretch due to high volume and pressure. During ventricular diastole, less elastic recoil of the aorta results in less blood entering the coronary arteries, resulting in symptoms of MI.

In doing the respiratory assessment in a patient with heart failure (HF), which assessment findings should the nurse expect to see?

Distinctive cough Patients suffering from heart failure may have an accumulation of fluid in the lungs, and a distinctive cough may be heard in doing a respiratory assessment in these patients. Abnormal lung sounds If there is congestion in the lungs, this may be heard by the nurse while performing the respiratory assessment on the child. Deep breaths with activity A child with heart failure will often need to take deep breaths with any sort of exertion, and this would be an expected finding in the respiratory assessment.

What are some of the clinical manifestations associated with left-sided obstructive lesions?

Exercise intolerance The left side of the heart supplies blood to systemic circulation. A left-side obstruction would decrease blood delivery and decrease exercise tolerance. Pulmonary hypertension Increased pressure in left ventricle due to left-side heart obstruction can cause backflow into lungs and hypertension. Correct Left ventricular hypertrophy Increased pressure in left ventricle causes hypertrophy.

Pulmonary arterial hypertension (PAH) management is designed to treat the symptoms of heart failure (HF). How does treating HF symptoms facilitate the management of PAH?

Improving right-sided heart function and fluid management will decrease the symptoms of PAH. The right side of the heart cannot match the afterload of the PAH and begins to undergo fibrosis and chamber dilation. Increasing contractility will help match afterload and decrease EDV. Decreasing blood volume will also improve right-side function. Increasing afterload will force the heart to increase contractility and therefore decrease PAH. PAH is the cause of increased afterload. Drugs targeting pulmonary circulation to decrease afterload can be useful.

Tachycardia, though initially beneficial, potentiates heart failure in the long-term. What is the relationship between tachycardia and heart failure?

Increased heart rate (HR) increases metabolic demand and decreases filling and resting time for the heart. These changes lead to heart failure. Tachycardia, although beneficial to compensate for early HF, increases myocardial oxygen consumption, decreases diastolic filling time and resting phase of the heart, and decreases coronary artery perfusion. Over time, this will lead to heart failure.

What is the relationship between right-sided heart failure and pulmonary artery stenosis?

Increased pressure developed in the right ventricle can cause hypertrophy and eventual failure of the right side of the heart. Development of hypertrophy and the increased workload of the right ventricle are caused by the stenotic PA. The RV has to increase its contraction efforts to overcome the resistance from the PA, and this can lead to right sided heart failure.

What should the nurse expect to see regarding pulmonary blood flow if an infant had a ventricular left-to-right shunt?

Increased pulmonary blood flow Increased pulmonary blood flow would be expected in a patient with left-to-right shunt. Pulmonary arterial hypertension (PAH) PAH is an expected finding in a patient with left-to-right shunt. Pulmonary artery thickening Due to increased pressure, pulmonary artery thickening would be an expected finding in a patient with left-to-right shunt.

The parent of a patient with aortic stenosis would like more information about a new diagnosis of pulmonary venous hypertension. What information should the nurse provide regarding treatment and outcomes?

Keep regular follow-up appointments for observation. Keep regular appointments for observation of the aortic stenosis and progression of PAH. Changes in symptoms may require medical intervention. Athletes, depending on the severity of the stenosis and PAH, may need to limit or abstain from competitive sports. Interventional correction of the stenotic valve will improve cardiac output and decrease the pulmonary hypertension. Surgical valvuloplasty decreases the stenosis and improves cardiac output.

The mother of a child with tetralogy of Fallot states that over the past two weeks the child has been experiencing periodic episodes of increased cyanosis, irritability, and moments of deep breathing in the morning. After child is assessed and vitals appear stable what is the most important action for the nurse to take next?

Left-to-right shunting will cause volume overload on the heart. Contact the providers as the child is in need of a surgery consult. Left-to-right shunting will increase the volume on the right side of the heart, resulting in both volume and pressure overload. This child is experiencing "tet spells" that include episodes of increased cyanosis with crying, deep respirations, and other abnormal breathing patterns, and increased pulmonary venous return. This evidence of increased hypoxia leaves the child at risk for cerebrovascular accident. Surgical consult is necessary.

What is a major concern associated with transposition of the great vessels when the patient has patent ductus arteriosus (PDA) but no additional septal defect?

Limited areas for mixing of blood Patient relies on the mixing of blood through the fetal structures (and a septal defect), if present.

A newborn infant has pulmonary atresia with intact ventricular septum. The parents want to know why the health care provider said it was important to keep fetal structures open. How can the nurse explain the rationale for maintaining fetal structures in the newborn infant?

Maintaining open fetal structures will allow blood to make its way to the lungs. This will allow for oxygenation of the blood for the baby. In the fetus, blood bypasses nonfunctioning lungs using ductus arteriosus and foramen ovale. Foramen ovale will continue to let deoxygenated blood move to left side of the heart. Ductus arteriosus will now have reversed flow; blood will move from aorta into ductus arteriosus and to pulmonary circulation for oxygenation of blood. Then, mixed blood can be pumped to body.

What is the relationship between the fetal cardiac anatomical features and the survivability of complex cardiac lesions in the early neonatal stage?

Patent fetal structures maintain pathways for the movement of blood and allow for mixing of oxygenated and deoxygenated blood. Fetal structures (foramen ovale and ductus arteriosus) that can be maintained after birth can improve survivability of patients with heart defects. Theses maintained pathways allow for the movement of blood around possible obstructions or due to pressure gradients. They also allow for the mixing of oxygenated and deoxygenated blood which can reduce hypoxia and therefore improve survivability in children with heart defects.

What conclusions can be drawn regarding clinical manifestations for a patient with a left-to-right ventricular shunt and decreased pulmonary blood flow?

Patient may have polycythemia. Polycythemia results from decreased systemic oxygenation. The kidney responds by increased RBC production. Patient may be hypoxemic, resulting in cyanosis. Decreased pulmonary blood flow and mixing of blood in the ventricles can result in hypoxemia and cyanosis.

Which statement helps to explain why a patient with Down syndrome might have a marked increase in pulmonary blood flow?

Patients with Down syndrome often have cardiac defects associated with the genetic syndrome. The patient may have an atrioventricular septal defect (AVSD). AVSD (atrioventricular septal defect) is often associated with genetic syndromes such as Down syndrome. In AVSD there is abnormal development of both septa and AV valves. Left-to-right shunting increases pulmonary blood flow.

A nurse is measuring blood pressure (BP) in a patient with a stenotic lesion. What should the nurse expect when comparing blood pressures proximal and distal to the lesion?

Proximal to the lesion, pressure will be high; distal to the lesion, pressure will be decreased or absent. Pressure increases proximal to the lesion (obstruction/stenosis). Distal to the lesion, pressures are decreased. The force of BP pushes against the obstruction. After the obstruction, no additional force is generated.

A child presents to the clinic suffering from Truncus Ateriosus. After the nurse administers the diuretic, which physiologic change can the nurse expect to see in the patient over the course of treatment?

Reduction in volume overload thereby increasing cardiac output (CO) With diuretic therapy the nurse will expect to see a reduction in volume overload in the heart, thereby improving cardiac output and lowering the risk for heart failure.

In the patient with heart failure, many organ systems will be altered. Which affected organ systems have the greatest impact on potentiating the volume overload causing heart failure (HF)?

Renal system The renal system maintains fluid and electrolyte balance for body. When cardiac output (CO) is decreased, glomerular filtration rate (GFR) can decrease, leading to activation of renin-angiotensin-aldosterone system (RAAS). Pulmonary system Increased volumes and pressures in the pulmonary system can lead to pulmonary hypertension and edema.

Atrial septal defects are conservatively treated as many spontaneously close. What assumptions can be made regarding the possible outcomes if the defect does not close?

Right-sided pressures will increase. Volume from the high pressure, left side of the heart will create increased pressures on right side of the heart. Right side of heart will be volume overloaded. Volume from the high pressure, left side of the heart will create a volume overload of right side of the heart. there will be increased pulmonary blood flow. Volume from the high pressure, left side of the heart will cause an increase in pulmonary blood flow. There will be increased oxygen saturation on right side of the heart. Volume from highly saturated, left side of the heart will mix with deoxygenated blood on the right side. This will increase oxygen saturation on right side of the heart.

In response to the pathophysiology of heart failure (HF), there is activation of the sympathetic nervous system and the release of hormones in an effort to maintain cardiac output (CO). How do these two systems synergistically increase cardiac output?

Sympathetic nervous system activity increases heart rate. Sympathetic outflow increases heart rate which increases CO. Sympathetic nervous system activity increases stroke volume. Sympathetic outflow increases stroke volume which increases CO. Endocrine function of the hormones of renin-angiotensin-aldosterone-system (RAAS) leads to increased intravascular volume. In response to low blood volume and low blood pressure, the RAAS system works to increase sodium and water reabsorption in the kidney to increase blood volume and increase CO.

The nurse notes that QP/QS ratio (pulmonary-to-systemic ratio) is normal, however the right side of the heart has increased saturation. What conclusion can be drawn from this data?

There is a right-to-left shunt. When pulmonary-to-systemic ratio is norma, equal amounts of blood are being pumped by both sides of the heart. A right-to- left shunt would decrease oxygen saturation on the left side of the heart.

What is the most important rationale for monitoring nutritional intake in a child with heart failure (HF)?

To determine if child is obtaining enough nutrients to support increased energy demands of the heart. If less nutrition is consumed while more energy is expended, resulting in fewer calories being consumed, the child will develop failure to thrive (FTT).

In patients with coarctation of the aorta, infusion of prostaglandin E1 may be used to keep the ductus arteriosus open. What is the rationale for facilitating patent ductus arteriosus (PDA) when the patient has coarctation of the aorta?

To increase blood flow to descending aorta by allowing deoxygenated blood from pulmonary trunk to mix with blood distal to lesion Coarctation of the aorta is an obstructive lesion that decreases blood supply to abdominal organs and lower periphery. Maintaining PDA will increase blood flow in the descending aorta.

What is the rationale for administering potent vasoconstriction agents to a child experiencing a hypercyanotic episode?

To increase systemic vascular resistance Vasoconstriction will increase systemic vascular resistance and help maintain BP. This increase in pressure will help limit shunting of blood, thereby helping to improve movement of blood out of right ventricle into pulmonary artery and to lungs for oxygenation. To decrease the degree of right-to-left shunting Vasoconstriction can help to limit amount of blood that enters left ventricle through ventricular septal defects. This will help to limit amount of deoxygenated blood entering left ventricle and the aorta into systemic circulation. To increase blood flow into the pulmonary circulation Potent vasoconstrictors can help to limit amount of deoxygenated blood that enters aorta and help to increase amount of blood entering pulmonary artery and lungs for proper oxygenation.

When caring for a child with a right-to-left shunt, what precaution is essential when obtaining IV access?

Use meticulous attention to avoid introducing air bubbles in tubing of IV line. There is an increased risk of air embol in patients with right-to-left shunts, which can cause stroke or heart attack.

What is the relationship between left-to-right shunt congenital heart defects and increased pulmonary blood flow?

Volume overload results in the right side of the heart and this increases pulmonary blood flow. Volume shunts from the left to the right side of the heart resulting in right-sided volume overload. The increase in volume leads to an increase in pulmonary blood flow.