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Surgical pulmonary artery reconstruction in patients with arterial tortuosity syndrome has excellent outcomes. In this study, we report our late outcomes after more than a decade of experience with such complex interventions.We conducted a retrospective review of 33 arterial tortuosity syndrome patients who underwent pulmonary artery reconstruction. The mean preoperative right ventricular to left ventricular pressure ratio was 1.19 ± 0.2. Our surgical approach included either a single-stage complete repair through a median sternotomy (17 patients) or a two-stage repair through sternotomy/left thoracotomy (16 patients), depending on the degree of distal involvement in the left pulmonary artery.Median age was 36 months. All patients had distal segmental peripheral pulmonary artery stenosis. Thirty patients (90.1%) were symptomatic before surgery. There was one hospital death due to viral pneumonia 78 days after the surgery (in-hospital mortality 3%). The mean right ventricular to left ventricular pressure ratio decreased to 0.31 ± 0.07 early postoperatively (P0.001), representing a 74% reduction compared with preoperative values. Follow-up was 100% complete for all hospital survivors (32 of 33) with a mean follow-up of 70.42 ± 43.32 months (range, 2 to 143). There was no late mortality or need for reintervention (surgical or catheter based) after hospital discharge. In late postoperative catheterization, the mean right ventricular to left ventricular pressure ratio was 0.27 ± 0.05 (P = .003 compared with early postoperative value). All patients were asymptomatic on their most recent follow-up.A strategy of complete surgical reconstruction of all stenotic pulmonary artery segments in patients with arterial tortuosity syndrome is recommended for sustainable successful outcomes more than a decade later.

Background The presence of peripheral pulmonary artery stenosis (PPAS) involving lobar and/or segmental pulmonary artery (PA) branches might preclude proceeding to a single-ventricle pathway. We adopted a strict strategy for PA rehabilitation and surgical reconstruction in patients who are unable to progress to a single-ventricle pathway. Methods We conducted a retrospective review of 22 patients with single-ventricle physiology who underwent surgical reconstruction for PPAS from April 2008 to March 2020. Our surgical approach was single stage in 15 patients (68.2%) and 2 stage in 7 patients (31.8%) depending on the presence or absence of PA hypoplasia distal to the PPAS. Results The PPAS was type 3 (lobar) in 19 patients (86.4%) and type 4 (segmental) in 3 patients (13.6%). The mean number of PA angioplasties performed was 8 ± 5.6 per patient. There was no mortality in this series with a median of 52 months (range, 8-143) of follow-up. Twenty patients (90.9%) were able to progress in the single-ventricle pathway with 12 patients (54.5%) undergoing a Fontan operation and 8 patients (36.4%) achieving bidirectional Glenn shunt and awaiting a Fontan operation. Two patients (9.1%) were not suitable to progress to the single-ventricle pathway because of elevated PA pressure. All patients who achieved cavopulmonary connection were alive and asymptomatic with no signs of elevation of PA pressure. No patient required further reinterventions for the PA. Conclusions PPAS is not a contraindication for single-ventricle palliation. With careful planning and aggressive early surgical reconstruction, most of these patients can successfully progress to cavopulmonary connections.

The ideal management of peripheral pulmonary artery stenosis is still controversial. We adopted a primary surgical approach to this complex lesion with excellent early outcomes. In this study, we analyzed our late outcomes.We performed a retrospective review of 91 patients with biventricular anatomy who underwent peripheral pulmonary artery reconstruction from March 2008 to July 2020. Our surgical approach included either a single-stage complete repair through median sternotomy or a 2-stage repair through sternotomy/left thoracotomy, depending on the degree of distal involvement of the left pulmonary artery branches.Median age was 26 months. Syndromic etiology was established in 54 patients (59.3%) versus nonsyndromic etiology in 37 patients (40.7%). Single-stage repair was achieved in 68 patients (74.7%). There were 2 (2.2%) in-hospital mortalities. The mean right ventricular to aortic systolic pressure ratio decreased from 1.07 ± 0.20 preoperatively to 0.32 ± 0.07 immediately postoperatively (P .001), representing a 70.1% reduction. At 1-year postoperative catheterization, the mean right ventricular to aortic systolic pressure ratio was 0.28 ± 0.05 (P .001 compared with immediately postoperative value). With a median follow-up of 68 months (IQR, 39-117.5 months), there was no late mortality after discharge. All patients were active and asymptomatic on the most recent follow-up. There were no early or late reinterventions on pulmonary arteries.Late outcomes of surgical reconstruction of peripheral pulmonary arteries are excellent and durable in various pathologies (syndromic and nonsyndromic) with a significant reduction in right ventricular to aortic systolic pressure ratio, low mortality, and no reintervention.

Introduction The goal of this study is to describe (1) different types of isolation of arch branches and (2) to show the importance of careful evaluation of arch morphology using imaging tools such as echocardiography, cardiac computerized tomography (CT) and cardiac catheterization. Methodology Following IRB approval from KAIMRC with number RC17/298/R, we reviewed retrospectively a consecutive case series of pediatric patients who were diagnosed to have isolated innominate or subclavian arteries at King Abdul-Aziz and Prince Sultan Cardiac Centers by use of imaging techniques mentioned above. Data was collected, including demographic information, cardiac diagnosis, diagnostic modalities, and surgical interventions. Results There were a total of 11 patients who were diagnosed to have isolated arch branches. 9 of these patients (82%) had a right aortic arch (RAA) while 3 patients (27%) were found to have a left-sided aortic arch (LAA). Three patients from those with documented RAA had isolated left innominate artery with retrograde blood supply from the circle of Willis through the left vertebral artery and the isolated artery was connected to the pulmonary artery by patent ductus arteriousus or its remnant ligament; the remaining six patients with RAA presented with an isolated left subclavian artery. 3 patients were found to have LAA arch with an isolated left subclavian artery. Of the 11 patients included in this study, one patient had normal intracardiac anatomy, 3 patients a VSD, 2 patients an interrupted aortic arch type B, 2 patients had tetralogy of Fallot, one patient left isomerism, another one hypoplastic left heart syndrome, and the last patient presented with crossed PAs and LPA stenosis. Conclusion Isolation of arch branches is a rare condition, but if it is present a careful initial assessment of arch morphology during echocardiography examination is mandatory; however, the utilization of cardiac CT with expert people reading the results will improve the detecting rate of such a lesion significantly.