P16 Identifying partial anomalous pulmonary venous drainage (PAPVD) in patients presenting with suspected pulmonary hypertension (PH)

Abstract

Introduction There are few published data regarding patients with PH and PAPVD. Aims To review all patients seen at a large PH referral centre with suspected PH, in whom PAPVD was identified. Methods Hospital databases were interrogated to identify all patients with PAPVD seen between 2007 and 2017. Baseline demographics, haemodynamics and radiological investigations were recorded. Results Ninety-two patients with PAPVD were identified. Clinical characteristics are shown in the table 1. 79% were not previously known to have PAPVD, while 54% had isolated PAPVD. All cases of PAPVD were identified at CTPA or cardiac MRI while 33% of patients not previously known to have PAPVD also had an atrial septal defect visible on cross-sectional radiology. Of the 50 patients with isolated PAPVD, 47 underwent right heart catheterisation (RHC), mean values: mean pulmonary arterial pressure (mPAP) 36.5 (±15) mmHg, pulmonary arterial wedge pressure (PAWP) 14.2 (±6.9) mmHg, cardiac output 5.8 (±2) l/min, pulmonary vascular resistance 4.35 (±3.64) Woods Units. Qp:Qs analysis for intra-cardiac shunt – assessed by cardiac MR (performed in 24/50) was 1.53:1 (±0.49). Additional possible triggers for PH were present in 50% of those with isolated PAPVD: 14% emphysema on CT, 6% interstitial lung disease on CT, 4% portal hypertension, 18% possible left heart disease (elevated PAWP) and 8% chronic thromboembolic disease. Conclusions The presence of PAPVD should be considered in patients with suspected PH and pulmonary venous anatomy should be specifically assessed on cross-sectional imaging. Approximately half of all patients with isolated PAPVD at a large PH referral centre had other possible triggers for the development of PH.