Aortopulmonary Window Flashcards
(23 cards)
Definition
A communication between the ascending aorta and pulmonary trunk. There must be two distinct and separate semilunar valves. Other terms include aortopulmonary fenestration, aortopulmonary septal deft, and aorticopulmonary window.
Prevalence
less than 0.2 to 0.6% of all congenital heart defects
Embryology
- 50% have no associated cardiac defect
- failure of aortopulmonary separation
How many types
3
Type 1
- most common
- small defect midway between semilunar valves and pulmonary artery bifurcation
Type 2
Distal defect whose border is formed by pulmonary artery bifurcation
associated with aortic origin of right pulmonary artery
Type 3
- rare
- large defect that includes type 1 and 2
Pathology/ Physiology
- represented by a solitary opening between the left side of the aorta and the right side of the pulmonary trunk. The opening is usually wide and lies close to the right pulmonary artery
- similar to PDA, VSD, truncus arteriosus
-left to right shunt
Associated conditions
*PDA
*ToF
*Subaortic stenosis
*Pulmonary atresia
*Interrupted aortic arch (type A)
*Coarctation of aorta
*VSD
*Aortic origin of rt pul artery
*Aortic Atresia
*Tricuspid atresia
*DiGeorge Syndrome
*d-transposition of the great arteries
*Anomalous origin of rt coronary artery from the pulmonary artery and right aortic arch
History
Small shunt
- asymptomatic (Infrequent)
Mod to large Shunt
- CHF
-failure to thrive
-Tachypnea
- recurrent respiratory infection
-diaphoresis
- pulmonary hen
- cyanosis (rare)
Physical Examination
- Similar to PDA, VSD
-tachypnea (rapid breathing)
-wide pulse pressure (For example, someone with a SBP of 120 millimeters of mercury (mmHg) and a DBP of 80 mmHg would have a PP of 40 mmHg.) - bounding peripheral pulses
- prominent rv impulse
Cardiac Auscultation
- murmur varies from continuous to absent
- prominent S2 (pulm htn)
- systolic ejection chick at left sternal border
- machinery type murmur (Long rumbling continuous )
- harsh systolic ejection murmur best heard at left upper sternal border (mod to large defect)
- apical mid-diastolic murmur due to increased flow across the MV (mod to large defect)
Electrocardiogram
Small Shunt
- normal ekg
Mod to Large Shunt
- biventricular hypertrophy
- isolated RVH (very young infants and older patients with pulm htn)
Chest xray
- cardiomegaly (LA/LV enlargement)
-prominent pulmonary trunk - increased pulmonary vascularity
- small aortic knob
- right sided aortic arch (occasionally)
- pulmonary edema
Cardiac Catheterization
- angio of pulmonary artery or aorta demonstrates defect
- venous catheter may cross defect and enter aorta
- increased left atrial pressure
- pulmonary hypertension
-evaluate coronary artery anatomy
Natural history/ complications
- CHF
- Increased risk of infective endocarditis
- Pulmonary vascular occlusive disease
medical mangement
anticongestive medications
surgical management
- Transaortic closure of defect with Dacron Patch
-irreversible pulmonary occlusive disease in a contraindication
Views (echo)
- parasternal/subcostal short axis of the aortic valve
-parasternal long axis with lateral and superior tilt
- suprasternal long axis
M-mode/ 2D
- absence of echoes between ascending aorta and pulm artery
- T-artifact at outer edges of defect
-LAE/ LVE
-LV volume overload pattern (LVE with hyperkinesis)
- main pulm artery/ pulm artery branch dilation
-PV dilation
-determine diameter of defect in two orthogonal views at end diastole
-determine distance between proximal border of defect and the semilunar valves and coronary arteries
-determine distance between the distal border of the defect and the pulmonary artery branches
-Determine the presence of associated lesions (PDA, VSD, partial anomalous pulmonary venous return, ToF, Subaortic stenosis, bicuspid Aov, coarctation, IAA, Rt Ao arch)
PW/CW/Color flow doppler
- high velocity, turbulent, continuous flow across the defect
- turbulent flow between the ascending aorta and pulmonary artery
-demonstrates diastolic flow reversal in the abdominal aorta, DTAO, Ao Arch, Ascending Ao
- increased pulmonary venous return, MV flow velocities/integrals
-increased LVOT aortic valve velocities.integrals
-determine the presence and severity of MR/TR/PR
PostOP Responsibilities
- flat/paradoxical septal motion (normal post op)
-evaluate the integrity of the patch repair (residual shunting, patch aneurysm)
- evaluate atrial dimension
-determine LV dimensions, thickness, and systolic.diastolic function
- Determine SPAP/MPAP/PAEDP
It is important to distinguish aortopulmonary window from truncus arteriosus.
The AP window has 2 distinct semilunar valves, in truncus arteriosus there is only 1
