Cyanotic Congenital Heart Defects

Truncus Arteriosus

100

• There is a ____ shunt involved in a persistent truncus arteriosus.

• Right to left

200

What is truncus arteriosus associated with?

DiGeorge’s syndrome (failure of 3rd and 4th pharyngeal arches to develop)

300

• A 5-day-old boy is brought to the emergency department by his mother because of a 2-day history of difficulty feeding and multiple episodes of his lips turning blue. He was born at home via spontaneous vaginal delivery and Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. Physical examination shows grunting and moderate intercostal and subcostal retractions. Echocardiography shows a single vessel exiting from the heart. Which of the following is the most likely underlying cause of this patient's condition?

Failure of neural crest cell migration

Transposition of Great Vessels

100

• What characterizes transposition of the great vessels?

• Aorta arising from right atrium and pulmonary artery arising from the left ventricle

200

• Transposition of great vessels is due to the failure of the ___to spiral during fetal development.

• aorticopulmonary septum

300

• A 3-hour-old female infant becomes cyanotic and tachypneic after her first feeding. Her temperature is 37°C (98.6°F), pulse is 110/min, respirations are 65/min, and blood pressure is 80/50 mm Hg. Physical examination shows central cyanosis, but is otherwise normal. Pulse oximetry on room air shows an oxygen saturation of 80% on room air. Arterial blood gas analysis on room air shows PO2 of 30 mm Hg. Her airway is stable, and an echocardiogram was obtained and is shown below. Which of the following is the most appropriate first step in the management of this patient's condition?
• A. Administration of intravenous prostaglandin E1 
• B. Arterial switch procedure
• C. Dobutamine and empiric antibiotic therapy
• D. Emergent cardiac catheterization and atrial septostomy
• E. Observation pending resolution of hypoxemia

A

400

• A 23-hour-old infant delivered vaginally at 38 weeks gestation was born with progressive cyanosis in absence of respiratory distress. Physical examination shows central cyanosis, but is otherwise normal. The patient's delivery was smooth without any complications. Subcostal echocardiogram shows failure of the aorticopulmonary septum to spiral.  Which of the following conditions is most likely seen in the newborn's mother and is associated with this defect?

Diabetes Mellitus 

Tricuspid Atresia

100

• Tricuspid atresia is a cardiac malformation that involves which of the following septa?

• Atrioventricular (defects in both the atrial and ventricular septa must be present in order to maintain blood flow)

200

• Which two chambers of the heart are no longer connected?
• 
  

• Right Atrium and Ventricle

300

• What happens to the right ventricle?

• Becomes hypoplastic/small

Tetralogy of Fallot

100

A  _____ shaped heart is seen on chest X-ray in a patient with tetralogy of Fallot.

Boot

200

• Name the four abnormalities associated with this.

• Pulmonary stenosis, right ventricular hypertrophy, overriding aorta, ventricular septal defect

300

What decreases the cyanosis associated with Tetralogy of Fallot and why?

• Squatting because increases the afterload by putting pressure on the femoral arteries, this means there is less resistance in pulmonary circulation and increases the amount of blood that goes to the lungs

400

Which of the following heart sounds is most likely to be auscultated in this patient due to his condition?

• Wide S2 splitting-lengthening of right ventricular ejection time due to pulmonary stenosis

500

A 2-year-old boy comes to the pediatrician with this mother because of concerns that he occasionally "turns blue," especially when he plays with his older brother. She describes episodes in which the boy would be playing, and within a few minutes, he begins to develop shortness of breath and his lips become blueish; both of which are relieved upon squatting. He is otherwise healthy and has achieved all developmental milestones. A systolic ejection murmur is heard on auscultation at the left upper sternal border. An echocardiogram is ordered which shows multiple lesions consistent with a congenital cardiac defect. Which of the following findings is the most likely primary determinant of the severity of symptoms

• Pulmonary stenosis

Misc.

100

What is the fifth T that did not get its own category?

Total Anomalous Pulmonary Venous Return

200

What is the most common cyanotic congenital heart disease?

Tetralogy of Fallot

300

Name the three most common CHD that can be associated with Eisenmenger Syndrome.

VSD, ASD, Patent Ductus Arteriosus

400

• How soon after birth does the foramen ovale close? (A) 1–2 months (B) 1–2 weeks (C) 1–2 days (D) 1–2 hours (E) Immediately

• The foramen ovale functionally closes almost immediately after birth as pressure in the right atrium decreases and pressure in the left atrium increases, thereby pushing the septum primum against the septum secundum. Anatomical fusion occurs much later in life; more than 25% of the population has probe patency of the foramen ovale, in which anatomical fusion does not occur

500

• A 3-day-old boy delivered at 32 weeks of gestation is experiencing respiratory distress syndrome. The physician detects a heart murmur characteristic of a patent ductus arteriosus, a diagnosis that is confirmed with an echocardiogram. Which embryonic structure is involved in this diagnosis? (A) Left third aortic arch (B) Right third aortic arch (C) Left sixth aortic arch (D) Umbilical arteries (E) Vitelline arteries

C