Pediatric Heart

Question 1

of pediatric heart txp performed every year

Answer 1

450

Question 2

Etiology for disease in the majority of pediatric heart transplants

Answer 2

CHD + Cardiomyopathy

Question 3

By age who are the largest portion of pediatric heart transplants

Answer 3

Infants 25%

Question 4

Most common indication for heart transplant in infants

Answer 4

> 60% CHD

30% cardiomyopathy

Question 5

Most common indication for OHT in older children

Answer 5

60% cardiomyopathy

Question 6

Percent of OHT in children is for re-transplant

Answer 6

5%

Question 7

Most common cardiomyopathy as an indication for OHT in children

Answer 7

DCM

Question 8

five year Transplant free survival for children with DCM

Answer 8

50%

Question 9

Hypertrophic cardiomyopathy
% of all CM,
% of all transplantation

Answer 9

Hypertrophic cardiomyopathy is 25-42% of all CM in children but

Question 10

Overall prognosis of restrictive CM in children

Answer 10

Extremely poor

transplant free survival at 5 years is is

Question 11

Left ventricular Noncompaction

Answer 11

Left Ventricular Noncompaction (LVN)

1. cardiomyopathy in children with and without CHD
2. Dialated CM with dialated and poorly functioning ventricle

Question 12

Arrhythmogenic right ventricular cardiomyopathy

Answer 12

characterized by:

1. loss of myocytes
2. Fibrofatty infiltration of the right and left ventricle

Question 13

Median survival after pediatric heart transplant

a. when is the most dangerous period

Answer 13

11-18 years

- first year is traditionally the most dangerous

Question 14

One year survival for pedi OHT patients with early primary graft failure

Answer 14

53%

Question 15

1. First drug to demonstrate improved survival in Adult patients with symptomatic heart failure ?
   a. Does it work in Kids?

Answer 15

1. ACE inhibitors were the first agents to demonstrate improved survival in adults w/ sx chf.
   a. In children, yet to demonstrate an improvment in survival, but hemodynamic improvements have been shown.

Question 16

PRA activity considered signficiant

Answer 16

Pannel reactive antibody

10% is signficiant

Question 17

Importence of elevated PRA

Answer 17

increased risk of acute rejection and early graft failure

Question 18

Impact on weight on pediatric heart donor / recipient

Answer 18

should be within 20% of weight

some studies have not noted a difference in survival based on size discrepancy

some particularly advocate for larger hearts, particularly in patients with PHTN
- there is an increased risk in post op lung collapse

Question 19

Age as an evaluation of donor heart

Answer 19

Corelates with graft survival in older children, less so with younger

Question 20

General schema for post operative imunosupression

Answer 20

1. corticostroid
2. calcinurin hinhibitor (cycclosprine or tacrolims)
3. antiproliferative (azathiprine or MMF)