Congenital Pulmonary Vein Stenosis Flashcards
(15 cards)
Definition
Characterized by discrete narrowing of one or more pulmonary veins at or near their junctions with the left atrium or by generalized hypoplasia of most, if not all, of a pulmonary veins
Pathology/Physiology
-Characterized by hypoplasia of involved veins or by a fibrous intimal lesion at the pulmonary vein-left atrium junction
-one or more of the pulmonary veins may be affected
Associated Conditions
ASD
corrected transposition of the great arteries
Atrioventricular septal defect
Ebsteins anomaly
History
-dyspnea
-fatigue
-Poor weight gain
-hemoptysis
-frequent lower respiratory tract infections
Physical Exam
-tachypnea
-central cyanosis
-PHTN
-pulmonary edema
-hepatomegaly
-RV lift
Cardiac Auscultation
-Loud S2 (P2 component) due to PHTN
-Pulmonary systolic click
-short systolic click
EKG
-RVH
-Right axis deviation
-RAE
Chest Xray
- Cardiomegaly due to RVH
-Symmetric/asymmetric pulmonary venous congestion
-Prominent central pulmonary arteries
-Pulmonary edema
-Kerley B lines
-Absence of LAE
Cardiac Cath
-normal left atrial pressure
-elevated pulmonary wedge pressure
-increased RV and pulmonary artery pressures
-Right to left shunt with PFO, ASD, PDA
-Pulmonary angiography may visualize stenosis
-arterial desaturation due to pulmonary edema
Natural History/complications
Death - average age 4 years, range four month to 5 years
Medical Management
-restrict physical activity
-oxygen therapy
-relief of pulmonary edema (digitalis, diuretics)
surgical management
-difficult to remedy
-pericardial patch angioplasty
-balloon angio
stent placement
echo views
-suprasternal notch “crab view”
parasternal long axis (left pulmonary vein), 4 ch (right and left upper pulm veins), subcostal
Mmode/2D
RVH/RVE
PHTN
RAE
Determine the presence of associated lesions (congenital MS, cor triatriatum, complete transposition, Ebsteins, mustard/senning repair)
PW/CW/color doppler
- turbulent, high velocity (greater than 2.0 m/s), continuous flow pattern and pressure gradient across pulmonary vein stenosis
-color flow demonstrates turbulence at he point of entry of the veins into the left atrium
-right to left PFO/ASD, VSD, PDA
