Sherpath Peds- Congenital Heart Disease

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 the patent ductus arteriosus (PDA) decreases the right-to-left shunting. Closure of the ductus arteriosus causes the development of pulmonary hypertension. Closure of ductus arteriosus decreases the volume of blood going to the lungs for oxygenation. Closure of the ductus arteriosus increases the volume of blood going to the lungs. Increased blood flow causes decreased oxygenation.

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 Ensure adequate weight gain through proper feeding Administer diuretics as ordered to help control fluid overload Administer digoxin as ordered and prepare to administer on long term basis

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.

What physiological changes are augmented by strenuous exercise and high altitudes in the patient with pulmonary arterial hypertension (PAH)? Select all that apply. Cyanosis Peripheral edema Pulmonary edema Right-sided heart hypertrophy Increased pulmonary venous pressure

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.

In doing the respiratory assessment in a patient with heart failure (HF), which assessment findings should the nurse expect to see? Select all that apply. Distinctive cough Abnormal lung sounds Deep breaths with activity Distinctive odor on the breath Increased number of respirations

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? Select all that apply. Peripheral edema Exercise intolerance Right ventricle atresia Pulmonary hypertension Left ventricular hypertrophy

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. Left ventricular hypertrophy Increased pressure in left ventricle causes hypertrophy.

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? Heart failure causes ischemia leading to abdominal pain. Fluid overload causing congestion can lead to abdominal pain. H. pylori infections often occur in heart failure patients causing abdominal pain. Heart failure can lead to increased secretion of abdominal acids therefore causing stomach pain in the child.

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.

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? Select all that apply. Improving right-sided heart function and fluid management will decrease the symptoms of PAH. Increasing afterload will force the heart to increase contractility and therefore decrease PAH. Improving left-side heart functions will increase cardiac output to systemic circulation, therefore decreasing pulmonary arterial hypertension. Increasing preload will increase ventricular stretch and improve contractility (Frank-Starling Mechanism) which will overcome the afterload of the 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.

What is the relationship between right-sided heart failure and pulmonary artery stenosis? Decreased pressures on the right side of the heart cause backflow, peripheral edema and failure. Increased blood flow to the pulmonary circulation can lead to right-sided heart failure due to increased workload. Increased pressure developed in the right ventricle can cause hypertrophy and eventual failure of the right side of the heart. Decreased venous return limits the preload on the right side of the heart. This leads to the development of 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.

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? Select all that apply. Keep regular follow-up appointments for observation. Increase water consumption to increase blood volume. Use of vasodilators will improve both the aortic stenosis and the pulmonary venous hypertension. Interventional correction of the stenotic valve will improve cardiac output and decrease the pulmonary hypertension. Pay close attention to symptoms such as exercise intolerance, chest pain, dizziness, syncope, and changes in breathing patterns.

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.

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 bypass lungs. This will allow for use of mechanical ventilation. 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. Maintaining open fetal structures will shift pressure in the ventricles. High pressure generated on right side of the heart will force pulmonary blood flow. Maintaining open fetal structures will allow for reversal of blood flow in the heart and body. This will allow for oxygenation of blood from left side of the heart and right side of the heart will pump blood to body.

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.

How can a nurse distinguish between a patient with hypoplastic left heart syndrome and truncus arteriosus? Oxygenated blood flows to left ventricle in hypoplastic left heart syndrome, whereas deoxygenated blood flows into left heart in truncus arteriosus. Oxygenated blood flows to right atrium in hypoplastic left heart syndrome, whereas deoxygenated blood flows into left heart in truncus arteriosus. Deoxygenated blood flows to the left atrium in hypoplastic left heart syndrome, whereas deoxygenated blood flows into the left heart in 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.

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 relationship between the fetal cardiac anatomical features and the survivability of complex cardiac lesions in the early neonatal stage? Fetal structures can prevent the formation of pressure gradients and reduce shunting. Patent fetal structures can be removed during surgery and used during the surgical repair. Fetal structures close quickly to prevent aberrant flow. This prevents the mixing of oxygenated and deoxygenated blood. Patent fetal structures maintain pathways for the movement of blood and allow for mixing of oxygenated and deoxygenated blood.

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? Select all that apply. Patient may have polycythemia. Patient may be hypoxemic, resulting in cyanosis. Patient may have increased pulmonary pressures. Patient may have increased oxygen saturation on left side of heart. Patient may have increased cardiac workload and ventricular strain.

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. Patient may have increased cardiac workload and ventricular strain. Cardiac workload and ventricular strain would increase due to shunting blood and lesion or malformation that limits pulmonary blood flow.

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? Select all that apply. Right-sided pressures will increase. There will be increased systemic pressures. Right side of heart will be volume overloaded. There will be increased pulmonary blood flow. There will be increased oxygen saturation on right side of the heart.

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? Select all that apply. Sympathetic nervous system activity increases heart rate. Sympathetic nervous system activity increases stroke volume. Sympathetic nervous system activity initiates vasodilation in most of the peripheral vasculature. Endocrine function of hormones from the heart, such as atrial naturietic peptide, work to increase blood volume. Endocrine function of the hormones of renin-angiotensin-aldosterone-system (RAAS) leads to increased intravascular volume.

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. There is a left-to-right shunt. There is increased blood flow out of the right ventricle. There is decreased pulmonary blood flow leading to pulmonary hypotension.

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.

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 oxygenation of blood entering descending aorta To increase pressures in left ventricle and force perfusion of aorta To decrease blood flow distal to lesion and increase blood flow to pulmonary circulation. PDA maintains flow from aorta to lungs To increase blood flow to descending aorta by allowing deoxygenated blood from pulmonary trunk to mix with blood distal to lesion

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? Select all that apply. To decrease the afterload To increase stroke volume To increase systemic vascular resistance To decrease the degree of right-to-left shunting To increase blood flow into the pulmonary circulation

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? Carefully inspect tubing to ensure adequate pressure in the vein. Use meticulous attention to avoid introducing air bubbles in tubing of IV line. Use careful attention to the access site; placement in the forearm will limit accidental removal. Ensure that the patient will be able to walk to limit deep vein thrombosis. Placement of the IV into a vein of the upper extremity is preferred.

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.