CASE NUMBER 11

Click here for a video debrief of the EMBRYOLOGY relevant to this case. (click here for accompanying slide deck)

Clinical History (continued): An echocardiogram reveals a large ventricular septal defect with overriding aorta and right ventricular outflow obstruction. Surgery is scheduled when the child is one year old. During that time, she has frequent cyanotic episodes that improve when she squats. Unfortunately, at the time of surgery, there are complications and the patient dies.

Image Gallery:

(Summary of Gross Findings) The specimen shows a heart with attached lungs. There is a subvalvular ventricular septal defect. The aorta is overriding the defect.

(Review Heart Anatomy) Norm No. 24 Lung NetID login required (click here if not already logged in)

Gross image questions:

• Take a screenshot and annotate the lungs, the aorta and ventricular septal defect.

11-2. Why did the child’s cyanosis decrease when she squatted?

1. Decreased effort due to resting
2. Decreased visceral blood flow
3. Improved ventilation due to pressure on diaphragm
4. Increased right ventricular pressure
5. Increased peripheral vascular resistance

ANSWER

11-3. Which of the following is the most common long-term complication of this disease?

1. Eisenmenger syndrome
2. Hypocoagulability
3. Polycythemia
4. Right ventricular failure
5. Upper extremity hypertension

ANSWER