Congenital Heart Defects Quiz

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Questions and Answers

What is the primary consequence of truncus arteriosus on blood flow in the body?

Increased blood flow to the aorta

Decreased oxygen saturation in systemic circulation (correct)

Increased blood flow to the lungs due to lower pressure pulmonary arteries (correct)

Equal distribution of blood to both ventricles

Which of the following treatments is NOT a standard procedure for cardiomegaly?

Palliative banding of pulmonary arteries (correct)

Balloon atrial septostomy

Prostaglandin administration

Surgical repair with cardiopulmonary bypass

Which symptom is most likely associated with Ebstein's anomaly?

Cyanosis with low oxygen saturation (correct)

Bradycardia in infants

High blood pressure

Increased oxygen saturation during exertion

What is a characteristic feature of Ebstein's anomaly?

Displacement of tricuspid valve leaflets and backflow into the atrium Signup and view all the answers

Which of the following statements about truncus arteriosus is false?

It only occurs in isolation without other congenital heart diseases. Signup and view all the answers

What is the primary consequence of hypoplastic left heart syndrome if untreated?

Complete failure of the left ventricle leading to mortality Signup and view all the answers

What is a common symptom associated with total anomalous pulmonary venous return?

Severe post-natal cyanosis or mild cyanosis Signup and view all the answers

In transposition of great arteries, which of the following best describes the blood circulation?

Oxygenated blood returns to the body while deoxygenated blood goes back to the lungs Signup and view all the answers

What surgical procedure is the first in the staged operations for hypoplastic left heart syndrome?

Norwood procedure Signup and view all the answers

What condition often accompanies total anomalous pulmonary venous return, facilitating blood circulation?

Atrial septal defect Signup and view all the answers

Which statement about preoperative laboratory studies is accurate?

Healthy children undergoing minor surgery may not require any lab tests. Signup and view all the answers

What is the recommended food/fluid restriction for infants less than 6 months before surgery?

Formula or breast milk until 4 hours before, then clear liquids until 2-3 hours prior. Signup and view all the answers

Which premedication is often administered to counteract hypotension and bradycardia?

Atropine Signup and view all the answers

What is a key feature of the Norwood procedure?

It repairs conditions where only one ventricle is functioning. Signup and view all the answers

What is the expected postoperative oxygen saturation (SaO2) level for patients after a Norwood procedure?

70% to 75% Signup and view all the answers

Which procedure usually involves the use of a balloon catheter to treat a stenotic valve?

Valvotomy/Valvuloplasty Signup and view all the answers

What type of replacement uses a part of a donor's aorta along with the aortic valve?

Aortic homograft Signup and view all the answers

What are common postoperative complications following cardiac surgical procedures?

Bleeding and dysrhythmias Signup and view all the answers

What are the primary characteristics of cardiogenic shock in children?

Increased preload, increased afterload, decreased contractility Signup and view all the answers

Which treatment is NOT commonly associated with managing cardiogenic shock in children?

ACE inhibitors for blood pressure regulation Signup and view all the answers

In hypertrophic cardiomyopathy, what is primarily affected by the thickening of heart mass?

Ventricular capacity which decreases filling efficiency Signup and view all the answers

What is a common preoperative consideration for children undergoing cardiac surgery?

Delaying surgery for children with non-viral respiratory infections Signup and view all the answers

What is the likely result of left ventricular failure in the context of cardiogenic shock?

Fluid backing up resulting in pulmonary edema Signup and view all the answers

What may cause the obstructive form of hypertrophic cardiomyopathy?

Asymmetrical thickening of the ventricular septum misaligning the mitral valve Signup and view all the answers

Which complication is most directly associated with hypothermia during postoperative management?

Systemic vascular resistance Signup and view all the answers

What potential consequence can arise from hypocalcemia in a post-surgical patient?

Seizures or myocardium depression Signup and view all the answers

What is a goal of respiratory and ventilatory management after surgery?

Optimize gas exchange and tissue perfusion Signup and view all the answers

What is a likely effect of renal dysfunction after cardiac surgery?

Increased risk for cardiac arrest due to hyperkalemia Signup and view all the answers

Which aspect is critical for neurological monitoring following cardiac surgery?

Avoiding hyperthermia and metabolic acidosis Signup and view all the answers

Which surgical procedure is primarily used to treat Coarctation of the Aorta (COA) by utilizing a Dacron patch to expand the lumen of the aorta?

Patch aortoplasty Signup and view all the answers

For a Ventricular Septal Defect (VSD), which surgery involves placing a constricting band around the pulmonary artery?

Pulmonary artery banding Signup and view all the answers

What procedure is performed to increase blood mixing by enlarging the foramen ovale during cardiac catheterization in Transposition of Great Arteries (TGA)?

Rashkind-ballon atrial septostomy Signup and view all the answers

Which procedure involves creating a conduit between the inferior vena cava and the pulmonary artery, thereby bypassing the right side of the heart?

Fontan Signup and view all the answers

In the context of Hypoplastic Left Heart Syndrome, which surgical procedure is designed to create a large atrial septal defect?

Norwood Signup and view all the answers

Which procedure involves the creation of an aortopulmonary conduit from the subclavian artery to the pulmonary artery in patients with Tetralogy of Fallot?

Blalock Taussig Shunt Signup and view all the answers

Which surgical intervention uses an umbrella or coil device to close a PDA during cardiac catheterization?

PDA closure Signup and view all the answers

What is the primary purpose of the Jatene arterial switch procedure in cases of Transposition of Great Arteries?

Reattach the aortic and pulmonary arteries to their opposite stumps Signup and view all the answers

During cardiac catheterization for Atrial Septal Defect (ASD), which procedure specifically involves closing the defect with a device like a septal occluder?

Transcatheter closure Signup and view all the answers

Which of the following conditions is treated with a Norwood procedure?

Hypoplastic Left Heart Syndrome Signup and view all the answers

What is the role of methylprednisolone in heart transplant procedures?

To suppress immune response prior to cross-clamp removal Signup and view all the answers

During the orthotopic heart transplant procedure, what portion of the old heart is typically preserved?

The posterior portion of the atria Signup and view all the answers

What complication is associated with the use of a larger donor heart during transplantation?

Compression of the lungs Signup and view all the answers

Which medication is typically administered before discontinuing cardiopulmonary bypass?

Isoproterenol Signup and view all the answers

What factor significantly increases the risk of laryngospasm in pediatric patients during surgery?

Environmental tobacco smoke exposure Signup and view all the answers

What is the primary purpose of using a right ventricular assist device (RVAD) post-transplant?

To support right ventricular function in case of failure Signup and view all the answers

How long should prostaglandin be continued postoperatively if used preoperatively in heart transplant patients?

1-2 days Signup and view all the answers

What technique is recommended to reduce the incidence of laryngospasm during extubation?

Awake extubation approach Signup and view all the answers

What is generally monitored in heart transplant patients to assess the risk of rejection?

Routine laboratory tests Signup and view all the answers

What is the common initial treatment for laryngospasm in pediatric patients?

Jaw thrust maneuver Signup and view all the answers

Study Notes

Hypoplastic Left Heart Syndrome (HLHS)

HLHS involves underdevelopment of the left ventricle and aortic atresia, impairing the heart's ability to pump blood.
Blood primarily flows from the left atrium to the right atrium due to the foramen ovale, with the descending aorta receiving blood via the ductus arteriosus.
Without surgical intervention, mortality rates approach 100%; with correction, rates drop to 25%.
Symptoms intensify post-ductus arteriosus closure, including increasing cyanosis and reduced cardiac output.
Surgical options include the Norwood procedure, Glenn procedure, and Fontan repair. Heart transplantation may be preferred but is limited by donor availability.

Total Anomalous Pulmonary Venous Return (TAPVR)

TAPVR is characterized by pulmonary veins connecting to the right atrium instead of the left, necessitating an atrial septal defect for blood shunting.
Patients may initially be asymptomatic if no obstruction occurs.
Symptoms manifest as heart murmur, post-natal cyanosis, dyspnea, low oxygen saturation, and cardiomegaly.
Surgical repair is often performed immediately after birth or can be delayed for 1-2 months.

Transposition of Great Arteries (TGA)

TGA presents with the aorta arising from the right ventricle and the pulmonary artery from the left, disrupting normal blood flow.
Symptoms include varying levels of cyanosis and signs of congestive heart failure.
An atrial septal defect and ductus arteriosus can allow some mixing of blood until closure.

Cardiomegaly

Cardiomegaly occurs postnatally, potentially indicating severe cardiac defects.

Treatment Options

Prostaglandins may be used to maintain ductus arteriosus and foramen ovale patency.
Balloon atrial septostomy can enlarge foramen ovale size.
Surgical repair often requires cardiopulmonary bypass and aortic cross-clamping.

Truncus Arteriosus

Truncus arteriosus involves one large artery arising from both ventricles, typically accompanied by a ventricular septal defect.
Symptoms include congestive heart failure, cyanosis, dyspnea, poor feeding, and increased risk for infections.
Treatment involves palliative banding or surgical repair to redirect blood flow.

Ebstein's Anomaly

Abnormal tricuspid valve positioning causes right atrium enlargement, potentially leading to deoxygenated blood mixing.
Symptoms can range from asymptomatic to life-threatening and include cyanosis and arrhythmias.
Treatment options include medications (ACE inhibitors, diuretics, digoxin) and potential surgical repair.

Cardiogenic Shock in Children

Causes include congenital heart disease and other disorders impacting heart function.
Characterized by increased preload and afterload with decreased contractibility leading to reduced cardiac output.
Treatment may involve mechanical ventilation, managing underlying conditions, and administering diuretics and inotropic agents.

Hypertrophic Cardiomyopathy

A genetic condition resulting in heart mass increase, particularly septal thickness, affecting ventricular efficiency.
Obstructive and non-obstructive forms exist, with symptoms worsening in adolescence.

Cardiac Surgery Preoperative Considerations

Preparation includes familiarizing children with equipment and managing anxiety.
Health concerns like viral infections may necessitate surgery delays.

Laboratory Review and Fluid Restrictions

Laboratory requirements differ by institution, with previous restrictions to prevent dehydration based on age.

Surgical Procedures for Cardiac Conditions

Multiple fixing surgeries exist, such as:
- Patch aortoplasty for coarctation of aorta.
- Transcatheter closure for atrial/ventricular septal defects.
- Jatene arterial switch for TGA.

Heart Transplantation

Involves cardiopulmonary bypass; techniques enable donor heart attachment and resumption of function.
Immunosuppressive therapy is crucial to prevent rejection, particularly in the first few months post-transplant.

Post-operative Management

Requires continuous monitoring of hemodynamics, respiratory management, and careful fluid and electrolyte balance to optimize recovery and minimize risks of complications.### Cardiopulmonary Bypass and Related Complications
Cardiopulmonary bypass may impair respiratory function, leading to pulmonary edema or hypertension.
Pulmonary hypertension is characterized by elevated pressure in pulmonary arteries, increasing cardiac workload.
Contributing factors to pulmonary hypertension include hypothermia and microemboli.

Neurological Monitoring

Essential to monitor for seizures and long-term neurological issues from deep hypothermic cardiac arrest (DHCA).
Prevention of hyperthermia and metabolic acidosis is crucial for nervous system protection.

Antimicrobial Prophylaxis

Broad-spectrum antibiotics are administered preoperatively until all invasive devices are removed to prevent infections.
Strict aseptic techniques and standard precautions are employed consistently.

Enteral Feedings

Postoperative enteral feedings provide essential nutrients to support immune function and healing.
Feeding amounts are calculated per kilogram of body weight for infants and children.
Transition to oral feedings should occur promptly in children to avoid complications.

Activity Guidelines

Activities should be increased gradually, incorporating sufficient rest periods.
Diversional activities and therapeutic play can aid in coping for children.
Avoid lifting infants and small children under the arms to prevent stress on surgical incisions.

Analgesia and Sedation

Analgesia/sedation is critical for pain management and stress reduction.
Pain monitoring may rely on physiological responses like cardiac rate and hypertension.
Use of opioids and benzodiazepines is common; all patients, including infants, require adequate analgesia.
Regional analgesia methods include peripheral nerve blocks, spinal anesthesia, and epidurals.
Patient-controlled analgesia (PCA) can be utilized for children aged six and above.

Electrolyte Imbalances

Sodium

Sodium ($Na$) is vital for fluid volume regulation, osmolality, and muscle and nerve function.
Normal sodium levels: Infants (133-144 mEq/L), Children (135-145 mEq/L).
Hyponatremia (below 135 mEq/L) can stem from diarrhea, vomiting, and certain illnesses, causing symptoms from irritability to seizures.
Hypernatremia may arise from fluid depletion or renal issues, presenting with symptoms such as confusion and muscle weakness.

Potassium

Potassium ($K$) is crucial for neuromuscular activity and maintains substantial intracellular concentration.
Normal potassium levels: Infants (4.1-5.3 mEq/L), Children (3.4-4.7 mEq/L).
Hypokalemia (below 3.4 mEq/L) can result from renal disease and dehydration, necessitating potassium replacement.

Calcium

Calcium is critical for nerve impulse transmission, muscle contraction, and blood coagulation.
Normal calcium levels: 11 days to 2 years (9-11 mg/dL), 3 to 12 years (8.8-10.8 mg/dL), 13 to 18 years (8.4-10.2 mg/dL).
Treatments for calcium imbalances include addressing underlying causes and various medications to manage potassium levels.

Hypocalcemia and Hypercalcemia

No specific details provided; further investigation may be necessary for full understanding.

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Description

Test your knowledge on congenital heart defects, including truncus arteriosus, Ebstein's anomaly, and hypoplastic left heart syndrome. This quiz covers consequences, symptoms, and treatment options related to these conditions. Perfect for medical students and healthcare professionals.