244. Major Vascular Malformations

Question 1

What are the three distinct entities focused on in the topic of major vascular malformations?

Answer 1

Coarctation of the aorta, patent ductus arteriosus (PDA), vascular rings

Question 2

What is the incidence of coarctation of the aorta?

Answer 2

1.7 to 4 in 10,000 live births

Question 3

What is the incidence of patent ductus arteriosus (PDA)?

Answer 3

1 in 1600 term live births

Question 4

What type of shunt is associated with patent ductus arteriosus (PDA)?

Answer 4

Left-to-right shunt

Question 5

What are the presenting symptoms of coarctation of the aorta in neonates?

Answer 5

• Congestive heart failure
• Shock (when ductus arteriosus closes)

Question 6

What are the presenting symptoms of patent ductus arteriosus (PDA)?

Answer 6

• Congestive heart failure
• Pulmonary overcirculation
• Poor feeding
• Failure to thrive
• Recurrent upper respiratory tract infections
• Fatigue with exertion
• Asymptomatic (small shunt)

Question 7

What are the symptoms of vascular rings in neonates?

Answer 7

• Respiratory distress
• Stridor
• Barky cough
• Recurrent upper respiratory tract infections
• Apnea episodes
• Dysphagia

Question 8

What is the management for coarctation of the aorta?

Answer 8

• Surgical repair (left lateral thoracotomy)
• Balloon dilation ± stenting

Question 9

What is the management for patent ductus arteriosus (PDA)?

Answer 9

• NSAIDs (indomethacin, ibuprofen)
• Acetaminophen
• Percutaneous device closure
• PDA ligation (thoracotomy)
• Video-assisted thoracoscopic clip closure

Question 10

What is a common surgical approach for treating coarctation of the aorta?

Answer 10

Left lateral thoracotomy

Question 11

What is the primary pathophysiology of coarctation of the aorta?

Answer 11

Left-sided obstruction leading to left ventricular pressure overload and hypertrophy

Question 12

What is the anatomical location of the aortic isthmus?

Answer 12

Located at the distal part of the aortic arch, proximal to the transition to the descending aorta

Question 13

What are the anesthetic considerations for neonates with high-grade obstruction of coarctation of the aorta?

Answer 13

• Operative repair via left lateral thoracotomy
• Continuous infusion of prostaglandin E1
• Inotropic agents may be required

Question 14

What are the postoperative complications of aortic coarctation repair?

Answer 14

• Perioperative hemorrhage
• Spinal cord injury from hypoperfusion
• Rebound hypertension
• Recurrent laryngeal nerve injury
• Phrenic nerve injury
• Thoracic duct injury
• Re-coarctation

Question 15

What is the function of the ductus arteriosus (DA) during fetal development?

Answer 15

Allows blood to bypass pulmonary circulation as the lungs are not engaged in gas exchange

Question 16

What initiates the closure of the ductus arteriosus after birth?

Answer 16

Smooth muscle contraction triggered by an increase in PaO2 and a decrease in circulating prostaglandins

Question 17

What is the primary diagnostic test for vascular rings?

Answer 17

Computed tomography angiogram (CTA)

Question 18

True or False: Most patients with vascular rings present with respiratory symptoms in infancy.

Answer 18

True

Question 19

Fill in the blank: The first attempt at closing a PDA in neonates typically uses _______.

Answer 19

nonsteroidal anti-inflammatory drugs (NSAIDs)

Question 20

What is the purpose of using a right upper extremity arterial cannula during coarctation surgery?

Answer 20

Provides perioperative blood pressure monitoring and allows continued blood pressure monitoring during the clamp period

Question 21

What type of anesthesia is generally used for surgical and catheter-based closure of PDA?

Answer 21

General endotracheal anesthesia

Question 22

What should be avoided during surgical ligation of PDA to prevent increased left-to-right shunting?

Answer 22

• Hypoxia
• Acidosis
• Hypercarbia
• Hypothermia
• Sympathetic stimulation
• Atelectasis
• High airway pressure