Management of systemic to pulmonary shunts and elevated pulmonary vascular resistance

Background Repair of systemic to pulmonary shunts is timed to prevent the development of irreversible pulmonary vascular disease, including in patients with other factors contributing to pulmonary hypertension. This study assessed outcomes of an individualised strategy for managing patients with mild-moderately elevated pulmonary vascular resistance (PVR) deemed borderline eligible for repair. Methods A retrospective chart review was conducted of patients with systemic to pulmonary shunts and baseline indexed PVR (PVRi) >= 3 WU center dot m2 treated at a single centre from 1 January 2005 to 30 September 2019. Data included demographics, World Health Organization functional class (WHO FC), medications and haemodynamic data at baseline and serial follow-up. Results 30 patients (18 females) met criteria for inclusion. Median age at diagnosis of pulmonary arterial hypertension was 1.3 years (range 0.03-54 years) and at surgery was 4.1 years (range 0.73-56 years). Median follow-up time was 5.8 years (range 0.2-14.6 years) after repair. Most patients received at least one targeted pulmonary arterial therapy prior to repair and the majority (80%) underwent fenestrated shunt closure. There was a significant decrease in mean pulmonary arterial pressure (mPAP) ( p<0.01), PVRi ( p=0.0001) and PVR/systemic vascular resistance ( p<0.01) between baseline and preoperative catheterisation and a decrease in PVRi ( p<0.005), mPAP ( p=0.0001) and pulmonary to systemic flow ratio ( p<0.03) from baseline to most recent catheterisation. WHO FC improved from FC II-III at baseline to FC I post repair in most patients (p<0.003). Conclusions In carefully selected patients with systemic to pulmonary shunts and elevated PVR considered borderline for operability, the use of preoperative targeted therapy in conjunction with fenestrated or partial closure of intracardiac shunts is associated with improvement in WHO FC and clinical outcomes.

Automated summary

This study assessed the outcomes of an individualized strategy for managing patients with mildly to moderately elevated pulmonary vascular resistance (PVR) deemed borderline eligible for repair. The results showed that the use of preoperative targeted therapy in conjunction with fenestrated or partial closure of intracardiac shunts improved the clinical outcomes and WHO functional class (FC) in these patients.