Partial anomalous pulmonary venous drainage in patients presenting with suspected pulmonary hypertension: A series of 90 patients from the ASPIRE registry

Abstract

Background and objective There are limited data regarding patients with PAPVD with suspected and diagnosed PH. Methods Patients with PAPVD presenting to a large PH referral centre during 2007–2017 were identified from the ASPIRE registry. Results Ninety patients with PAPVD were identified; this was newly diagnosed at our unit in 71 patients (78%), despite 69% of these having previously undergone CT. Sixty‐seven percent had a single right superior and 23% a single left superior anomalous vein. Patients with an SV‐ASD had a significantly larger RV area, pulmonary artery and L‐R shunt and a higher % predicted DLCO (all P < 0.05). Sixty‐five patients were diagnosed with PH (defined as mPAP ≥ 25 mm Hg), which was post‐capillary in 24 (37%). No additional causes of PH were identified in 28 patients; 17 of these (26% of those patients with PH) had a PVR > 3 WU. Seven of these patients had isolated PAPVD, five of whom (8% of those patients with PH) had anomalous drainage of a single pulmonary vein. Conclusion Undiagnosed PAPVD with or without ASD may be present in patients with suspected PH; cross‐sectional imaging should therefore be specifically assessed whenever this diagnosis is considered. Radiological and physiological markers of L‐R shunt are higher in patients with an associated SV‐ASD. Although many patients with PAPVD and PH may have other potential causes of PH, a proportion of patients diagnosed with PAH have isolated PAPVD in the absence of other causative conditions.

Pattern of Vascular Anomalies Associated With Sinus Venosus Atrial Septal Defect

“…The significant clinical implication of persistent left SVC is that its diagnosis must be known before cannulation for open heart surgical repair or correction. In a study conducted by Lewis et al, out of 90 patients with PAPVR, 31 (34%) patients had associated SV-ASD [ 21 ]. In our patient population, the most common site of anomalous vein was seen draining into SVC and right atrial (RA) junction in 55 (76%) patients followed by nine (12.5%) in IVC and seven (9.7%) in upper and middle part of SVC, and in one (1.3%) patient, anomalous vein was seen draining directly into the RA.…”

Clinical–radiological–pathological correlation in pulmonary arterial hypertension

“…Furthermore, an increase in the septal angle has been shown to have high specificity for the presence of PH [ 32 ]. To exclude anomalous pulmonary venous drainage as a cause of RV dilatation, the insertion of left and right superior and inferior pulmonary veins into the LA should be confirmed [ 35 ]. The interatrial and interventricular septum should be inspected for the presence of an atrial or ventricular septal defect.…”

Three challenges of partial anomalous pulmonary venous drainage associated with pulmonary hypertension

“…There are three key challenges with partial anomalous pulmonary venous drainage that are highlighted in the recent article in Respirology by Lewis et al 1 . The challenges include finding the vascular abnormality, correctly characterizing the impact it is having and then determining how best to treat it.…”