CONGENITAL HEART DISEASE

Question 1

Approach to the Critically Ill Congenital Heart Disease

Answer 1

FOR SUSPECTED CONGENITAL HEART DISEASE:

ASSESS

Cyanosis

Auscultate for murmurs: harsh, 3+ murmur, pansystolic, abnormal S2

Hepatomegaly

Pre-ductal (RUE) pulse Ox

Post-ductal (LE) pulse Ox

4 extremity Blood Pressure: >20 mm Hg difference between RUE and LE

AND

Hyperoxia Test:
100% 02 at bedside for 10-15 min

If no response, then consider cyanotic heart disease

PGE1: 0.1 μg/kg/min IV initially;
THEN
0.01-0.05 μg/kg/min.

AIRWAY
Beware intubation in preload dependent lesions -> increased intrathoracic pressure.

BREATHING
Beware apnea with PGE1

Beware pulmonary vasodilation -> systemic steal with oxygen in cyanotic ductal dependent lesions

CIRCULATION
5-10 ml / kg NS bolus
Beware bradycardia and hypotension with PGE1
Epi 0.1-1 ug / kg / min IV / IO ***
Dopamine 2-10 ug / kg / min IV / IO
Dobutamine 2-20 ug / kg / min IV / IO

Significant acidosis 1-2 mEq/kg if ph < 7

DISABILITY
POC Glucose

Question 2

DDx: Ductal Dependent Cyanotic Lesions

Answer 2

Truncus Arteriosus
Transposition of the Great Arteries
Tricuspid Anomalies (atresia or Epstein’s anomaly)
Tetralogy of Fallot
TAPVR

Question 3

DDx: Ductal Dependent Lesions causing Shock