Pulmonary thromboendarterectomy in Portugal: Initial experience.

INTRODUCTION: Surgical treatment for chronic thromboembolic pulmonary hypertension (CTEPH) is challenging. Most Portuguese patients with CTEPH have been referred to foreign institutions for treatment, with significant social and economic costs. To meet this emerging need, the cardiothoracic surgery department of Hospital de Santa Marta, Lisbon, has developed a dedicated program for pulmonary thromboendarterectomy (PTE). We hereby present the results for the first 19 patients treated. METHODS: We conducted a retrospective analysis of all 19 patients who underwent PTE at Hospital de Santa Marta between 2008 and April 2019. RESULTS: Since 2008, a total of 19 patients have undergone PTE in our department. The procedure was performed with good outcomes in both survival and functional recovery. At the very beginning of the series two patients died perioperatively, before all the team underwent formal training at the Royal Papworth Hospital, UK, with no early deaths since. Postoperative complications were similar to other published series. During 11 years of follow-up, there were three late deaths, all in patients with residual pulmonary arterial hypertension. At the latest follow-up (October 2019), all surviving patients showed significant functional recovery, all in NYHA class I or II, with only one patient on vasodilator therapy with sildenafil (the first in the series, operated in 2008). CONCLUSIONS: PTE is a demanding procedure, in which outcomes are related to volume and accumulated experience, however it can be performed safely and with reproducible results by a properly prepared dedicated team with a well-controlled learning curve. More patients and multidisciplinary experience will be needed to further improve and streamline results.

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

OBJECTIVES: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. METHODS: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. RESULTS: We collected 156 surgical patients (median age 39.5 years, interquartile range 15-53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1-5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan-Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. CONCLUSIONS: Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study.

BACKGROUND: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. METHODS: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. RESULTS: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p < 0.001). Coronary unroofing was the most frequent procedure (56.4%). Operative mortality was 1.3% (2 patients with preoperative severe heart failure). At a median follow up of 18 months (range 0.1-23 years), 89.9% of survivors are in NYHA ≤ II, while only 3 elderly surgical patients died late. Return to sport activity was significantly higher in Surgical patients (48.1% vs 18.2%, p < 0.001). CONCLUSIONS: Surgery for AAOCA is safe and with low morbidity. When compared to Medical patients, who remain on exercise restriction and medical therapy, surgical patients have a benefit in terms of symptoms and return to normal life. Since the long term-risk of sudden cardiac death is still unknown, we currently recommend accurate long term surveillance in all patients with AAOCA.