In patients with congenital systemic to pulmonary shunts, there is a window of operability before irreversible sheer stress related pulmonary vascular disease develops. For those with significantly elevated pulmonary vascular resistance, complete repair can be catastrophic. Although not well studied, with the availability of targeted PAH medications, a “treat and repair” strategy may be utilized in select PAH-CHD patients. We hypothesize that in selected patients, use of pre and/or post-operative targeted PAH treatment and partial closure of large shunts may halt disease progression, and improve outcomes and describe our early experience using this approach for PAH-CHD with elevated PVR.
A retrospective chart review of patients with CHD and PAH (n=28; Jan2005-Sept2018) and a baseline PVR > 3 WU*m2 who were treated with targeted PAH therapy preoperatively or started on PAH targeted therapy after fenestrated closure of shunts was conducted. Data collected included demographics, WHO functional class (WHO-FC), echocardiographic and hemodynamic data at baseline and on serial follow-up.
The median age of patients (9ASD, 2VSD, 4PDA, 13 complex shunts) at diagnosis of PH was 1.2y (2mo–54 y) and at surgery was 10.2 y, range (2–63y). Median follow up time was 4.3y (0.5–13y). With use of targeted PAH therapy, there was a significant improvement in mPAP and PVR with preserved CI from baseline to pre-OP state which was sustained at latest follow-up and improvement in WHO-FC (Table). All patients were alive at the time of last follow up.
In carefully selected PAH-CHD patients with borderline elevated PVR, the use of targeted PAH therapy pre-OP followed by fenestrated/partial closure of shunts to permit pop-off when needed, is associated with improvement in pre-operative PVR and mPAP, and post-operative WHO-FC.