Corkscrew appearance in wedged pulmonary angiography: a distinct phenotype and prognostic marker in idiopathic pulmonary arterial hypertension

Background

Idiopathic pulmonary arterial hypertension (IPAH) is a progressive disorder characterised by elevated pulmonary vascular resistance (PVR); however, treatment responsiveness is not uniform, and a more detailed classification of phenotypes is required. Detailed evaluation of pulmonary vascular morphology may help phenotypic stratification of IPAH. Wedged pulmonary angiography (wPAG) enables the visualisation of peripheral pulmonary arteries that are not readily detected by computed tomography or magnetic resonance imaging. The clinical significance of peripheral tortuous pulmonary arteries detected using wPAG remains unclear.

Purpose

To investigate the clinical characteristics, haemodynamic features, and prognostic significance of peripheral tortuous pulmonary arteries on wPAG in newly diagnosed IPAH and explore their association with the genetic background.

Methods

We retrospectively analysed 75 consecutive patients with newly diagnosed IPAH who underwent wPAG between January 2011 and April 2025. Baseline characteristics, haemodynamics, treatment response, and outcomes were compared between patients with and without peripheral tortuous pulmonary arteries, termed corkscrew appearance. Genetic testing for PAH-associated genes, including BMPR2, was performed when available. The primary endpoint was clinical worsening, defined as all-cause death, lung transplantation, or disease progression requiring hospitalisation and escalation of therapy. Cox proportional hazards models were used to identify the predictors.

Results

Corkscrew appearance was observed in 17 patients (22.7%). These patients were younger (29.3 vs. 40.5 years, p<0.01), more frequently male (47.1% vs. 17.2%, p=0.03), and had a higher baseline mean pulmonary arterial pressure (mPAP) (57.4 vs. 48.7 mmHg, p=0.04). The reduction in mPAP during acute vasoreactivity testing was smaller (−6.2% vs. +0.5%, p=0.03). BMPR2 variants were more frequent in the corkscrew group (69.2% vs. 17.2%, p=0.04).

At follow-up right heart catheterisation (median 5.6 years), mPAP and PVR remained higher in the corkscrew group (42.2 vs. 28.6 mmHg, p<0.01; 6.9 vs. 4.6 Wood units, p<0.01). The percentage reduction in mPAP from baseline was smaller (−22.8% vs. −39.5%, p=0.02) despite similar combination therapy.

Over 6.3 years of follow-up, corkscrew appearance was associated with a higher incidence of clinical worsening (log-rank p<0.01) and independently predicted events after adjustment for age and sex (hazard ratio 3.12, p=0.03) and for age and baseline mPAP (hazard ratio: 4.34, p<0.01).

Conclusions

Corkscrew appearance on wPAG identifies a distinct IPAH phenotype characterised by more severe haemodynamics, reduced therapeutic response, and increased risk of clinical worsening. The higher frequency of BMPR2 variants suggests a possible association with the genetic background. Corkscrew morphology may serve as an imaging marker for risk stratification in patients with IPAH.Representative wedged pulmonary angiographyFor image description, please refer to the figure legend and surrounding text.Kaplan–Meier analysis of clinical events.For image description, please refer to the figure legend and surrounding text.