Congenital 12

All of the following are criteria targeted after PA banding EXCEPT:

A 2 day old infant was worked up for cyanosis and found to have transposition of the great vessels, VSD and pulmonary stenosis. Which surgical procedure(s) should be done to correct these lesions?

A 30 year old male presented to the ED with progressive shortness of breath on exertion. Workup with echocardiogram revealed a coronary sinus ASD with a Qp:Qs ratio 2:1. What is this lesion associated with?

Which of the following is often considered a contraindication to ventricular septal defect (VSD) closure in adults with congenital heart disease?

A 25-year-old female presents to the emergency department with progressive shortness of breath and occasional palpitations with increasing frequency. She states that she was “born with a hole in my heart” and had heart surgery as a baby when she was 6 months old, and again when she was 5 years old, but she does not know why. In the emergency department, her vital signs are as follows: Tmax 98.7, HR 89, BP 103/76, RR 20, O2 90% on room air. X-ray shows bilateral interstitial pulmonary edema with mild bilateral pleural effusions. What is the next best step in the diagnosis and work-up of this patient?

In the modern era, what percentage of children born with congenital heart disease may be expected to survive into adulthood, depending on the severity of their underlying lesion(s).

A 65 kg, 16-year-old boy with history of recent viral respiratory illness presents with severe fatigue and dyspnea. Upon admission to the inpatient ward, he acutely decompensates and requires emergent cannulation for VA ECMO for cardiogenic shock. His ventricular function demonstrates little recovery over the next week. What is the best course of action?

A 12 kg, 4-year-old girl with known dilated cardiomyopathy who has recently completed transplant evaluation is transferred to the ICU for initiation of inotropes and is listed for transplantation. Despite this, her condition fails to improve, and she begins to develop a metabolic acidosis with signs of renal and liver dysfunction. Care should be escalated with:

A 3-month-old boy undergoing repair of complete AV canal defect fails to wean off cardiopulmonary bypass due to significant myocardial dysfunction. His operative course is further complicated by surgical site bleeding and acquired coagulopathy. Pulmonary function is intact. How should the surgeon proceed?

Which of the following is NOT a risk when performing an unroofing procedure to AAOLCA?

Which of the following is NOT a potential disadvantage of coronary artery bypass grafting to treat AAOLCA?

A 6 week old infant has been having difficulty with feedings and now presents with signs of heart failure and dilated cardiomyopathy on transthoracic echocardiography. Which of the following must be ruled-out during the clinical work-up?

Aortopulmonary window may be distinguished from Truncus Arteriosus by:

Berry syndrome consists of which of the following lesions

Which of the following is true of a cardiac (left to right) shunt:

Which of the following is nearly always present in patients with truncus arteriosus?

Which of the following is least likely to be present in truncus arteriosus without arch anomalies?

You go on bypass to repair a patient with standard Type 1 truncus arteriosus. Your perfusionist complains that mean arterial pressures are persistently low and the heart remains full. You cross clamp and give cardioplegia. The heart is less full now but regains electrical activity after a few minutes. What crucial intervention have you failed to perform?

A 2-year-old child with tricuspid atresia underwent a non-fenestrated extracardiac Fontan procedure. On postoperative day 1, the right lung is noted to be very hazy while the left lung is clear. There is no right pleural effusion. Cardiac catheterization shows wide patency of the Fontan pathway, cavopulmonary anastomosis, and branch pulmonary arteries. Most of the pulmonary blood flow appears directed to the left lung and there is delayed return from the right lung on the levophase angiogram. What is the best next step?

Which of the following is correct regarding the bidirectional cavopulmonary shunt?