COMPression of Left Main coRonary artEry in patientS With Pulmonary Arterial Hypertension aSymptomatIc fOr aNgina

Study Overview
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Brief Summary
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The prevalence of critical ab extrinsic compression of left main coronary artery (LMCA) is very high in patients with pulmonary arterial hypertension (PAH) symptomatic for angina (up to 40% according to a recent study of 121 patients with PAH). The element that most of all correlates with the degree of coronary stenosis is the diameter of the pulmonary artery (PA). In particular, a diameter ≥ 40 mm has a sensitivity of 83% and a specificity of 70% in patients with angina. Critical stenosis of LMCA is a risk factor for sudden death and in these condition percutaneous coronary angioplasty with stent implantation has proven to be a safe and effective long-term procedure. Preliminary data from a retrospective analysis of the registry of patients with PAH in Bologna (ARCA registry, 109/2016/U/Oss) highlights that even in PAH patients asymptomatic for angina, compression of LMCA can occur in up to 13% of patients and the main predictive parameter of compression was found to be a diameter ≥ 42 mm (with a sensitivity of 87% and a specificity of 77%). Performing a screening test by coronary-CT scan in all subjects suffering of PAH with a PA diameter ≥ 40 mm even if asymptomatic for angina could therefore help to identify patients with PAH at increased risk for sudden death at an early stage.

Official Title
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COMPression of Left Main coRonary artEry in patientS With Pulmonary Arterial Hypertension aSymptomatIc fOr aNgina

Conditions
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Pulmonary Arterial Hypertension

Intervention / Treatment
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* Radiation: Coronary CT angiography


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with PAH (group 1 of World Health Organization pulmonary hypertension classification) who have undergone at least one pulmonary CT angiography with a PA trunk diameter ≥ 4 cm Age ≥18 years Obtaining informed consent Exclusion Criteria: Patients with angina pectoris Severe chronic kidney disease [Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) estimated glomerular filtration rate <30 ml/min) or need for dialysis Allergy to iodinated contrast agent Intolerance or allergy to acetylsalicylic acid or clopidogrel History of stroke or transient ischemic attack in the last 6 months or a history of intracranial haemorrhage Known cerebral arteriovenous malformation or aneurysm Oral anticoagulant therapy which cannot be suspended for the duration of the study Known moderate or severe hepatic insufficiency (Child Pugh B or C) Thrombocytopenia (<100.000/μL) or anemia (hemoglobin <10 g/dL) Active bleeding or factors which, in the investigator's judgment, significantly increase the risk of bleeding Major surgery in the past 30 days Cancer in the active phase Pregnancy or breastfeeding Patient prognosis <1 year in the opinion of the investigator Any condition that increases the risk of non-compliance or of being lost to follow-up Patients who have already undergone a LMCA angioplasty

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Coronary-CT<br>Patients with PAH, asymptomatic for angina, with a PA trunk diameter ≥ 4 cm that undergo a coronary-CT scan examination | Radiation: Coronary CT angiography<br>* A coronary CT angiography will be used to study the relationship between the PA and the LMCA and 4 radiological patterns will be considered: Normal: minimum distance between the two vessels> 1 mm; Proximity: distance between the two vessels ≤1 mm without displacement or stenosis of the LMCA; Dislocation: dislocation of the LMCA by the main branch of the PA with a take-off angle <60 ° (the take-off angle is defined by the angle formed by the perpendicular to the aortic valve ring and the longitudinal axis of the LMCA); Compression: stenosis of the LMCA ≥50% due to extrinsic compression by the PA.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Incidence of extrinsic compression of the LMCA | To evaluate the incidence of extrinsic compression of the LMCA in patients with PAH and a PA diameter of at least 4 cm, asymptomatic for angina pectoris, subjected to a screening test by coronary CT angiography | Baseline | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Incidence of extrinsic compression of the LMCA by radiological pattern | To evaluate the incidence of extrinsic compression of LMCA in the different possible radiological patterns described by coronary CT angiography (compression, dislocation, contiguity) | Baseline | 
| Safety of LMCA angioplasty | To evaluate the safety of the LMCA angioplasty in patients with LMCA critical ab extrinsic compression by evaluating the incidence of in-hospital complications [death, myocardial infarction, transient ischemic attack (TIA) or stroke, re-angioplasty, or acute stent thrombosis, vascular complications, acute kidney injury] and at 1 year [death, myocardial infarction, TIA or stroke, restenosis, stent thrombosis and bleeding whose severity will be assessed according to the Bleeding Academic Research Consortium (BARC) classification] | Baseline, 1 year | 
| Six minute walking test (6MWT) | In patients who will undergo LMCA angioplasty for LMCA critical ab extrinsic compression the change from baseline in the six-minute walk test (6MWT) after the procedure will be evaluated | Baseline, 6 months |