26
Specific Conditions
Table 11. Isolated Shunt Lesions and Pulmonary Arterial
Hypertension
PAH-ACHD Subtype
Patient Characteristics
Resting Hypoxemia/Cyanosis Typical Defect Size
A. Eisenmenger syndrome Usually Large
B. Prevalent systemic
to pulmonary shunts
(left-to-right shunts
with elevated PVR, yet
not meeting criteria for
Eisenmenger syndrome)
No Moderate to large
C. Coincidental defects with
elevated PVR
Rare Small
D. Postcorrection defects
with elevated PVR
Rare (with small residual shunts
and severe pulmonary vascular
disease)
Corrected
* No validated score available.
†
See Section 4.4.6 for details on the order of PAH-directed drug initiation.
‡
For patients in groups B, C, and D, the 3- and 4-strata models have been applied, and
evidence for use has included enrollment of patients in this category; however, validation for
this specific population in isolation from other forms of PAH has not been directly studied.
§
Trials that included patients with PAH aer congenital defect correction.
6MWD indicates 6-minute walk distance; ACHD, adult congenital heart disease; BNP, B-type
natriuretic peptide; N/A, not applicable; PAH, pulmonary arterial hypertension; and PVR,
pulmonary vascular resistance.
4.1. Shunt Lesions
