Congenital heart DZ

Question 1

What is a patent ductus arteriosus?

Answer 1

• opening between the artery to the aorta
• normally closes a few hours after birth
• delayed closure is related to RDS and prematurity
• Meds that affect PDA
  
  • Prostaglandin (alprostadil)
  • Indomethacin
    *

Question 2

what is a Atrial Septal Defect?

Answer 2

• opening between left and right atrium
• usually benign at first, as it results in small left to right shunt
• over time can lead to atrial dilation and atrial arrhythmias
• 4 types of ASD
  
  • Primum
  • secundum
  • superior sinus venosus
  • inferior sinus venosus

Question 3

What is Ventricular septal defect?

Answer 3

• Most common form of CHD (21% of all cases)
• Large defect characterized by a significant L to R shunt
  
  • Can lead to excessive pulmonary blood flow that can result in pulmonary edema, CHF, and tachypnea
  • Surgically repaired by patch repair

Question 4

What is Hypoplastic Left Heart Syndrome?

Answer 4

• Characterized by:
  
  • Hypoplasia or atresia of mitral valve
  • Hypoplasia of left ventricle
  • Aortic Stenosis or atresia
  • Corarctation of aorta
• Treatment
  
  • Pre-Surgical treatment
  • Three stage surgical palliation
  • Hospice Care
• Pre-surgical treatment
  
  • Prostaglandin E1 to maintain ductal patency
  • Sub-ambient oxygen therapy if pulmonary over circulation is present
  • Avoid oxygen
  • Goal SpO2 70-80%

Question 5

What is Tetralogy of Fallot?

Answer 5

• Consists of four defects
  
  • Overriding aorta
  • Pulmonary stenosis
  • VSD
  • Right ventricular hypertrophy
• Mild cyanosis at birth
• Cyanosis worsens as PDA closes
• Prostaglandin E1 infusion to maintain ductal patency

Question 6

What is a blue TET vs Pink TET?

Answer 6

• Degree of cyanosis depends on the degree of pulmonary stenosis
• Mild pulmonary stenosis results in L to R shunt in the ventricles (Pink TET)
• Significant pulmonary stenosis results in R to L Shunt in ventricles (Blue TET)
  
  • Ductal dependent pulmonary blood flow

Question 7

What is a TET spell?

Answer 7

• TET patients typically have equal pressures in R and L Ventricle due to large VSD
• Decrease in SVR will lead to progressive R to L shunt and progressive cyanosis
• Decreased PaO2 will stimulate baby to increase minute ventilation which increases venous return to right side of heart
  
  • Lead to vicious cycle or continued R to L shunt
• Knee to Chest Position will increase SVR and force blood to the lungs

Question 8

What is Transposition of the Great Vessels?

Answer 8

• Aorta arises from right ventricle
• Pulmonary artery arises from left ventricle
• Usually there is an associated ASD, VSD, or PFO.
  
  • If the septum is intact or restrictive the patient will be profoundly cyanotic
  • Patient will go directly to cath lab for emergency balloon septostomy
• Definitive repair is a Arterial Switch.

Question 9

What is Truncus Arteriosus?

Answer 9

• Aorta and pulmonary artery leave the heart as a common vessel (Truncus Arteriosus)
• Large VSD which the Truncus overrides
• Intracardiac mixing which leads to systemic cyanosis
• Pre-surgical treatment
  
  • Reduce pulmonary blood flow
    
    • Sub-ambient oxygen therapy
• Surgical treatment
  
  • Closure of VSD
  • RV to PA Conduit

Question 10

What is Total Anomalous Pulmonary Venous Return (TAPVR)?

Answer 10

• Pulmonary veins carry blood back to venous circulation instead of the left atrium
• Systemic blood flow is dependent on ASD
• Only heart defect that may need surgical repair on same day of birth.

Question 11

What is Coarctation of Aorta?

Answer 11

• Narrowing of the lumen of Aorta
• Results in higher blood pressure proximal to obstruction and lower blood pressure distal to the obstruction
  
  • Distal organ perfusion can be compromised
• Ductal closure can lead to LV failure and pulmonary hypertension
  
  • PgE1 Administration to prevent ductal closure is necessary
• Surgical repair
  
  • End to End Anastamosis