Introduction Pulmonary artery aneurysm (PAA) is a common condition among pulmonary arterial hypertension (PAH) patients (38%). A pulmonary artery diameter ≥48 mm is an independent risk factor for sudden cardiac death in PAH patients. In the past years, the diagnosis of potentially lethal complications related to PAA is increasing. These complications include pulmonary artery dissection, bronchial compression and left main coronary artery compression (LMCAC). Our aim is to compare the survival and cause of death between PAA patients with a diagnosed and treated LMCAC and the rest of our PAA population. Methods Among our PAH population, we selected those patients who had a PAA diagnosis, defined as main pulmonary artery dilatation >40 mm. All the information was gathered from our institutional database. Patients with a suspected LMCAC based on computed tomography findings were evaluated with coronary angiography and treated surgically or percutaneously when LMCAC was confirmed. Survival from PAH diagnosis was estimated by the Kaplan–Meier method and compared by log-rank test between groups. Results 86 patients in our cohort were diagnosed with a PAA (24% male, 49.8±12.5 years). In 10 (12%) patients, a LMCAC was diagnosed, 9 of them were treated with a percutaneous coronary intervention and the remaining one underwent PAA resection surgery due to severe pulmonary regurgitation. Among patients with LMCAC, only 3 presented chest pain and 2 had signs of ischemia in cardiopulmonary stress testing. In the other 5 patients, LMCAC was suspected after performing a thoracic computed tomography for different reasons: chronic cough in 1 patient, congenital heart disease evaluation in 2 patients and asymptomatic PAA follow-up in 2 patients. None of the patients presented complications secondary to revascularization. There were no differences between patients with and without LMCAC neither in age (49.5±12.9 vs. 49.6±12.4 years; p=0.982) nor in PAH hemodynamic severity (mean pulmonary artery pressure: 63.1±27.6 vs. 56.6±15.0 mmHg; p=0.486). Patients with LMCAC had larger median pulmonary artery diameter (52.5 [47–60.3] vs 43 [46–50] mm; p=0.024). Among LMCAC patients, 1 (10%) died during follow-up due to neoplastic disease. Among patients without LMCAC, 18 (24%) died (5 [6%] sudden cardiac death; 8 [11%] heart failure; 5 [6%] neoplastic or infectious diseases). Despite LMCAC diagnosis, there were no differences between both groups in time from PAH diagnosis to death (log rank test p=0.38). Conclusion LMCAC is a common complication among PAH patients with a PAA. Its early identification and treatment might reduce the rate of sudden cardiac death among PAH patients. Since LMCAC is often asymptomatic regular screening with non-invasive tests should be incorporated to PAH follow-up protocols.

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With the advent of balloon pulmonary angioplasty (BPA) for non-surgical chronic thromboembolic pulmonary hypertension (CTEPH) patients, there is renewed interest in the pulmonary angiography technique. This technique is still the standard imaging modality to confirm CTEPH, which, in addition, helps to determine the most appropriate treatment. Furthermore, learning this technique fulfills two main purposes: to identify BPA candidates and to provide the operator with the catheter handling needed to perform BPA. Operators interested in performing BPA must learn not only the pulmonary arteries’ anatomy, but also which are the best angiographic projections and the most suitable catheters to canalize and display each segmental branch. Unfortunately, this information is scarce in the literature. With this goal, learning the diagnostic pulmonary angiography technique can be a first step on the way to perform BPA. Although there are descriptions on how to perform a pulmonary angiography with balloon-tipped catheters and the digital subtraction technique, this technique does not provide operators with the catheter knowledge and manual skill needed to cannulate each segmental branch. In contrast, learning the conventional selective segmental pulmonary angiography (SSPA) technique provides the operator with this knowledge and skills. In this review, based on the experience of the authors, we describe the pulmonary arteries’ anatomy and detail the practical aspects of the SSPA procedure, with the aim of providing operators with the anatomical and technical knowledge needed to perform BPA. We also summarize the contemporary complications of SSPA in CTEPH patients at a reference center.

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Background/Introduction Pulmonary artery aneurysm (PAA), defined as a pulmonary artery (PA) diameter >40 mm, is a common finding among pulmonary arterial hypertension (PAH) patients. Although often asymptomatic, PAA may lead to life-threatening complications such as left main coronary artery compression or PA dissection. Transthoracic echocardiography (TTE) is regularly employed for risk assessment in PAH patients. However, TTE accuracy for PA measurement has not been evaluated, and current practice guidelines lack formal recommendations for PAA screening and follow-up. We aim to determine whether TTE is an appropriate tool for PA diameter measurement and determine an optimal cut-off point to diagnose a PAA through TTE. Methods We retrospectively analyzed a cohort of 657 PAH patients followed up at a national referral centre. For this analysis, we selected those patients who had undergone at least one TTE and one computed tomography (CT) or magnetic resonance (MR) within six months before or after the TTE. We performed an agreement analysis between CT/MR-based and TTE-based PA diameter using the Passing–Bablok method. Furthermore, we calculated the area under the curve for the identification of a PAA with a TTE (compared to CT/MR). Results We analyzed 281 simultaneous CT/MR and TTE of a total of 178 PAH patients (71% women). Median age at diagnosis was 42.1 (32.2–58.0) years. PAH etiology was idiopathic or familial in 67 (38%), associated with congenital heart disease in 28 (16%) and associated with connective tissue disease in 36 (20%) patients. In 46 (26%) patients PAH was associated with other entities, such as human immunodeficiency virus, pulmonary veno-occlusive disease, drugs or portal hypertension. We found a significant correlation between PA diameter measured in TTE and CT/CMR (Lin's concordance correlation coefficient = 0.851) (Figure 1). The area under the curve for the detection of PAA was 0.91 (95% CI 0.88–0.95, p=0.018) (Figure 2). We selected a TTE-based PA diameter 37 mm as the optimal cut-off point for PAA identification. This diameter correctly classified 85.4% of measurements with a sensitivity and a specificity of 83.2% and 87.2%, respectively. Conclusion Our study demonstrates that TTE is an adequate tool for PA diameter quantification with a strong correlation with CT/MR. This good correlation makes TTE an excellent tool for PAA screening among PAH patients, avoiding unnecessary CT or MR scan and helping to identify those patients in whom close follow-up is advisable. Based on these results, we recommend the inclusion of PA diameter measurement in TTE acquisition protocols for PAH patients. FUNDunding Acknowledgement Type of funding sources: None. Figure 1. Passing–Bablok regression line Figure 2. ROC curve for PAA detection with TTE