A gray zone in the literature: indications, options, and outcomes for surgical repair of pulmonary artery aneurysms

Pulmonary artery aneurysms are rare. They are characterised by an aneurysmatic dilatation of the pulmonary vascular bed, including the main pulmonary artery or the accompanying pulmonary artery branches. Increases in pulmonary flow and pulmonary artery pressure increase the risk of rupture: when these conditions are detected, surgical intervention is necessary. This study is a retrospective analysis of 33 patients treated in our paediatric cardiac surgery clinic from 2012 to 2020. Aneurysms and pseudoaneurysms in patients who were patched for right ventricular outflow tract reconstruction and corrected with a conduit were excluded from the study. Seventeen (51.5%) of the patients included in the study were female and 16 (48.5%) were male. The patients were aged between 23 and 61 years (mean 30.66 ± 12.72 years). Graft interpositions were performed in 10 patients (30.3%) and pulmonary artery plications were performed in 23 patients (69.7%) to repair aneurysms. There was no significant difference in mortality between the two groups (p > 0.05). Pulmonary artery aneurysm interventions are safe, life-saving treatments that prevent fatal complications such as ruptures, but at present there is no clear guidance regarding surgical timing or treatment strategies. Pulmonary artery interventions should be performed in symptomatic patients with dilations ≥5 cm or asymptomatic patients with dilations ≥8 cm; pulmonary artery pressure, right ventricular systolic pressure, and pulmonary artery aneurysm diameter must be considered when planning surgeries, their timing, and making decisions regarding indications. Experienced surgical teams can achieve satisfactory results using one of the following surgical techniques: reduction pulmonary arterioplasty, plication, or graft replacement.

Usefulness of transthoracic echocardiography for pulmonary artery aneurysm screening in patients with pulmonary arterial hypertension

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

Pulmonary Artery Aneurysm Repair with Alpha-1 Anti-trypsin Deficiency

A 62-year-old woman with pulmonary hypertension due to alpha-1 antitrypsin deficiency and known congenital pulmonic valve stenosis presented with palpitations, chest pressure and bradycardia and was found to have a 6 cm pulmonary artery aneurysm on work-up. The patient underwent surgical pulmonary artery aneurysm repair and recovered from operation without complications.