Pulmonary Artery Reconstruction Research Articles

Total pulmonary arterial reconstruction in a patient with arterial tortuosity syndrome affecting the pulmonary artery: a case report and review of the literature

BackgroundArterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events.Case presentationA 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg.ConclusionATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

Total pulmonary arterial reconstruction in a patient with arterial tortuosity syndrome affecting the pulmonary artery: a case report and review of the literature

BackgroundArterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events.Case presentationA 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg.ConclusionATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

A novel bilateral lobar lung transplantation: End-to-side pulmonary artery reconstruction in left-to-right inverted split-lobe lung transplantation

A novel bilateral lobar lung transplantation: End-to-side pulmonary artery reconstruction in left-to-right inverted split-lobe lung transplantation

Outcomes of heart transplantation in children with heterotaxy syndrome.

End-stage congenital heart disease in children with heterotaxy syndrome might necessitate heart transplantation. Heart transplantation in heterotaxy patients can be associated with several technical (e.g. redo, systemic/pulmonary-venous/situs anomalies, pulmonary artery reconstruction), and extra-cardiac (e.g. ciliary dyskinesia, infections, gastrointestinal) challenges. We aim to examine if heterotaxy syndrome is associated with increased early or late transplantation risks. The United Network for Organ Sharing transplantation database was merged with the Paediatric Health Information System administrative database to identify children with heterotaxy who received heart transplantation. Characteristics and outcomes were compared between children with heterotaxy and contemporaneous non-heterotaxy congenital and non-congenital cardiomyopathy control groups. After merger, our cohort of 1122 patients was divided to three groups: group heterotaxy (n = 143), group non-heterotaxy congenital (n = 428) and group cardiomyopathy (n = 551). There were differences in the characteristics between the 3 groups, with heterotaxy being comparable to non-heterotaxy congenital group. The waitlist duration was longer for heterotaxy than non-heterotaxy congenital and cardiomyopathy groups (91 vs 63 vs 56 days, p < 0.001). Early post-transplant complications were similar for all groups except for operative mortality that was 1% for cardiomyopathy and 4% for heterotaxy and non-heterotaxy congenital, p < 0.001. Post-transplant stay was shorter for cardiomyopathy (57) compared to non-heterotaxy congenital (99) and heterotaxy (89) days, p < 0.001. While rejection prior to discharge was comparable between heterotaxy and CHD groups, it was higher at 1-year for heterotaxy (22%), than non-heterotaxy congenital (19%) and cardiomyopathy (13%), p < 0.001. Survival at 5 years was superior for cardiomyopathy (87%) compared to heterotaxy (69%) and non-heterotaxy congenital (78%), p < 0.001. For the heterotaxy group, no risk factors affecting survival were identified on multivariable analysis. Despite complexity, heart transplantation in selected children with heterotaxy is associated with good mid-term outcomes. Despite early results that are comparable to other patients with congenital heart disease, the increasing rejection rate at one year and the relatively accelerated attrition at mid-term warrant further follow-up. Due to database limitations in defining morphologic and surgical details, further work is warranted to delineate anatomic and surgical variables that could affect survival.

Bridge to transplant in single-ventricle anatomy: subpulmonary support with EXCOR ventricular assist device associated with pulmonary artery reconstruction.

Patients with a single ventricle circulation continue to be a challenge for a heart transplant. The patients in this cohort, often in poor clinical condition with frequent hepatic and renal impairment having undergone previous multiple surgical procedures, are often allosensitive and consequently have an increased risk of post-transplant complications. Although the most recently published series results are improved, this group of patients, with preserved ventricular function, continues to have a higher mortality rate. Moreover, the operation can be complicated by anatomical differences among the pulmonary arteries. This case report presents a child with tricuspid atresia and pulmonary vascular resistance contraindicating Fontan surgery, unfavourable anatomy of the central pulmonary arteries and in poor clinical condition. An EXCOR ventricular assist device (Berlin Heart) was used for sub- pulmonary mechanical circulatory support and pulmonary bifurcation reconstruction, as a bridge to a transplant.

(1271) - Bilateral Lung Transplantation Combining Pulmonary Artery Reconstruction for Giant Pulmonary Artery Aneurysm with Severe Pulmonary Artery Hypertension

(1271) - Bilateral Lung Transplantation Combining Pulmonary Artery Reconstruction for Giant Pulmonary Artery Aneurysm with Severe Pulmonary Artery Hypertension

Chronic left pulmonary artery occlusion caused by a distal aortic arch aneurysm: Simultaneous Frozen Elephant Trunk and left sided pulmonary endarterectomy after livesaving TEVAR for aortic rupture

We present a case of a 62-year-old male patient with chronic distal aortic arch aneurysm causing recurrent nerve palsy and prolonged total occlusion of the left main pulmonary artery (PA). During diagnostic work up, the aneurysm ruptured requiring cardio-pulmonary resuscitation (CPR) and emergency retrograde stenting. After circulatory stabilization the patient was treated with controlled hypothermia for 24 h, and successive extubation without neurological deficit.Postinterventional imaging revealed a reversed perfusion of the aneurysmal sac through the PA, prompting open surgical treatment. Surgery consisted of reconstruction of the left PA by pulmonary endarterectomy (PEA), xenopericardial patchplasty of the left PA and frozen elephant trunk (FET) repair after removal of the bare springs of the previously inserted Gore Tag stent graft. Pre-discharge computed tomography angiography (CTA) demonstrated a fully recanalized left PA system and a completely excluded aneurysm. At ten-year follow-up the patient presented physically unimpaired (NYHA I). CTA confirmed a fully intact thoracic aorta and improved pulmonary function despite persisting left recurrent nerve palsy.

Major vascular reconstructions in thoracic oncological surgery.

The replacement of the superior vena cava and thoracic outlet vessels for thoracic malignancies often becomes necessary for radical oncological surgery. The pulmonary artery can be directly infiltrated by the tumor or affected by metastatic hilar lymph nodes. In some cases, it must be resected and reconstructed to achieve oncological radicality and/or avoid pneumonectomy. This study reflects a single-surgeon, retrospective experience spanning 6years (2017-2023). We reviewed data from patients undergoing early anticoagulant therapy after superior vena cava or thoracic outlet vessels bypass and from patients undergoing early antiaggregation therapy following pulmonary artery reconstruction or resection. This series comprises 41 patients treated by a single surgeon. Fourteen patients underwent superior vena cava and thoracic outlet vessel procedures. Among these, eight patients received superior vena cava replacement (six for thymic malignancies and two for lung cancer), and six patients underwent jugular and subclavian artery/vein resection or replacement (all six had sarcomas). There was one death due to respiratory failure, not associated with bleeding or bypass closure. Additionally, there was one graft closure in a patient with severe coagulopathy and three instances of hemothorax (two patients had undiagnosed complex coagulopathies not evident in pre-operative routine blood tests). Following bleeding incidents, anticoagulation was initiated the next day in one case and based on hematological indications in the two coagulopathic patients. In the pulmonary artery series, 27 patients were involved: 20 underwent direct suture after tangential resection, and 7 received pericardial patch reconstruction. Only one case experienced bleeding necessitating redo-surgery. All these patients received early and chronic antiaggregation therapy after pulmonary artery reconstruction. We conclude that major thoracic oncological vascular surgery is safe and feasible with appropriate technical skills. However, achieving optimal results requires integration with correct early anticoagulant therapy or antiaggregation to maintain the patency of bypasses/grafts and prevent life-threatening risks associated with closure of the "new vessels."

Growth capacity of a Wharton's Jelly derived mesenchymal stromal cells tissue engineered vascular graft used for main pulmonary artery reconstruction in piglets.

Background: Surgical treatment of congenital heart defects affecting the right ventricular outflow tract (RVOT) often requires complex reconstruction and multiple reoperations due to structural degeneration and lack of growth of currently available materials. Hence, alternative approaches for RVOT reconstruction, which meet the requirements of biocompatibility and long-term durability of an ideal scaffold, are needed. Through this full scale pre-clinical study, we demonstrated the growth capacity of a Wharton's Jelly derived mesenchymal stromal cells (WJ-MSC) tissue engineered vascular graft used in reconstructing the main pulmonary artery in piglets, providing proof of biocompatibility and efficacy. Methods: Sixteen four-week-old Landrace pigs were randomized to undergo supravalvar Main Pulmonary Artery (MPA) replacement with either unseeded or WJ-MSCs-seeded Small Intestinal Submucosa-derived grafts. Animals were followed up for 6months by clinical examinations and cardiac imaging. At termination, sections of MPAs were assessed by macroscopic inspection, histology and fluorescent immunohistochemistry. Results: Data collected at 6months follow up showed no sign of graft thrombosis or calcification. The explanted main pulmonary arteries demonstrated a significantly higher degree of cellular organization and elastin content in the WJ-MSCs seeded grafts compared to the acellular counterparts. Transthoracic echocardiography and cardiovascular magnetic resonance confirmed the superior growth and remodelling of the WJ-MSCs seeded conduit compared to the unseeded. Conclusion: Our findings indicate that the addition of WJ-MSCs to the acellular scaffold can upgrade the material, converting it into a biologically active tissue, with the potential to grow, repair and remodel the RVOT.

Right ventricle to pulmonary artery conduit: a comparison of long-term graft-related events between bovine jugular vein conduit, aortic homograft, and porcine-valved conduits.

The optimal conduit for right ventricle to pulmonary artery (RV-PA) reconstruction does not exist. Reinterventions are common due to conduit stenosis and endocarditis. Tailoring conduit choice according to patients' characteristics could improve the outcomes. The study aimed to compare graft-related events (infective endocarditis, transcatheter pulmonary valve replacement (PVR), transcatheter conduit dilatation, surgical conduit replacement, and transcatheter pulmonary branch intervention for RV-PA reconstruction using bovine jugular vein, aortic homograft, and porcine-valved conduits. In a retrospective cohort study, 155 patients with 193 procedures that were done in King Faisal Specialist Hospital and Research Center-Jeddah (KFSHRC JED) for implanting RV-PA conduits from 1999 to 2021 were included. The patients were grouped according to the type of conduit into 3 groups; Group 1 (n=153) received bovine jugular vein (BJVs) grafts, Group 2 (n=29) received aortic homografts, Group 3 (n=11) received porcine-valved conduits. Factors associated with graft-related events were evaluated using stepwise Cox regression analysis. Patients with BJVs were significantly younger than those with porcine-valved conduits 3 (P=0.009). The weight of BJVs patients was significantly lower than homografts (P=0.002) and porcine-valved conduits patients 3 (P<0.001). The conduit size was as expected significantly lower in BJVs patients than patients who received porcine-valved conduits (P<0.001) and patients who received aortic homografts (P<0.001). There was no difference between Group 2 and 3 (P=0.084). Operative mortality occurred in 13 patients: 12 (7.84%) with BJV conduit and 1 (9.09%) with porcine valved conduit (P=0.351). Male gender [odds ratio (OR): 10.04; 95% confidence interval (CI): 1.28-78.86; P=0.028] and smaller conduit size (OR: 0.78; 95% CI: 0.61-0.99; P=0.048) were associated with increased operative mortality. Freedom from graft-related events at 5 and 10 years was 67% and 52% in BJVs patients, 74% and 36% in patients who received aortic homografts, and 53% in patients who received porcine-valved conduits. Factors associated with increased graft-related events were male gender (HR: 1.58; 95% CI: 1.004-2.50, P=0.048) and younger age (HR: 0.995; 95% CI: 0.991-0.999, P=0.041). RV-PA reconstruction was associated with low mortality, unrelated to the conduit type. Reinterventions for graft-related events were common. The durability and graft-related events might be comparable among BJV grafts, aortic homografts, and porcine-valved conduits. Factors associated with increased graft-related events in this study were male gender and younger age.

Modified Starnes Procedure With Patch Occlusion of the Main Pulmonary Artery and Other Technical Modifications to Facilitate Subsequent Biventricular Repair of Ebstein Anomaly

The Starnes procedure, introduced in 1991, has been a valuable advancement in the surgical treatment of critically ill neonates with Ebstein's anomaly (EA). However, it used to be followed by the single ventricle pathway, which presents long-term outcome limitations. In 2019, this management concept was modified by taking down the Starnes procedure and performing the Cone repair of the tricuspid valve, which resulted in a biventricular reconstruction. This approach rehabilitates the right ventricle, likely improving the patients' long-term outcomes. However, it requires modifications in the classical Starnes operation and observing certain surgical details. In cases of circular shunting, we use a patch to occlude the pulmonary artery instead of dividing and oversewing it, simplifying the succeeding pulmonary artery reconstruction. We use a PTFE fenestrated patch placed in a supra-valvar position, preserving the underneath tricuspid valve integrity, facilitating the tricuspid valve repair, and decreasing the possibility of heart block.Central message: In the current era of biventricular repair after the Starnes procedure, using a non-stick supravalvar patch to exclude the tricuspid valve and preserving the pulmonary valve structure by patch occlusion of the main pulmonary artery instead of division and oversewing it are valuable modifications of the classic Starnes operation to optimize a future biventricular repair.

Towards Zero Phrenic Nerve Injury in Reoperative Pediatric Cardiac Surgery: The Value of Intraoperative Phrenic Nerve Stimulation.

Phrenic nerve injury is a devastating complication that results in significant morbidity and mortality. We developed a novel technique to localize the phrenic nerve and evaluate its success. Two groups of children underwent repeat sternotomy for a variety of indications. Group I (69 patients, nerve stimulator) and Group II (78 patients, no nerve stimulator). There was no significant difference in the mean age and weight between the two groups: (6.4 ± 6.5 years vs. 5.6 ± 6.4 years; p = 0.65) and (25.2 ± 24.1 vs. 22.6 ± 22.1; p = 0.69), respectively. The two groups were comparable in the following procedures: pulmonary conduit replacement, bidirectional cavopulmonary anastomosis, aortic arch repair, and Fontan, while Group I had more pulmonary arterial branch reconstruction (p = 0.009) and Group II had more heart transplant patients (p = 0.001). There was no phrenic nerve injury in Group I, while there were 13 patients who suffered phrenic nerve injury in Group II (p < 0.001). No early mortality in Group I, while five patients died prior to discharge in Group II. Eleven patients underwent diaphragm plication in Group II (p = 0.001). The mean number of hours on the ventilator was significantly higher in Group II (137.3 ± 324.9) compared to Group I (17 ± 66.9), p < 0.001. Group II had a significantly longer length of ICU and hospital stays compared to Group I (p = 0.007 and p = 0.006 respectively). Phrenic nerve injury in children continues to be associated with significant morbidities and increased length of stay. The use of intraoperative phrenic nerve stimulator can be an effective way to localize the phrenic nerve and avoid its injury.

Novel mutation in ELN gene causes cardiac abnormalities and inguinal hernia: case report

BackgroundElastin-driven genetic diseases are a group of complex diseases driven by elastin protein insufficiency and dominant-negative production of aberrant protein, including supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Here, a Chinese boy with a novel nonsense mutation in the ELN gene is reported.Case presentationWe report a 1-year-old boy who presented with exercise intolerance, weight growth restriction with age, a 1-year history of heart murmur, and inguinal hernia. Gene sequencing revealed a novel nonsense mutation in the ELN gene (c.757 C > T (p.Gln253Ter), NM_000501.4). Due to severe branch pulmonary artery stenosis, the reconstruction of the branch pulmonary artery with autologous pericardium was performed. The inguinal hernia repair was performed 3 months postoperatively. After six months of outpatient follow-up, the child recovered well, gained weight with age, and had no special clinical symptoms.ConclusionWe identified a de novo nonsense mutation in the ELN gene leading to mild SVAS and severe branch pulmonary artery stenosis. A new phenotype of inguinal hernia was also needed to be considered for possible association with the ELN gene. Still, further confirmation will be necessary.

Modified Aortic Arch Reconstruction Combined With Pulmonary Artery Reconstruction in the Comprehensive Stage II Operation Following a Hybrid Stage I for Hypoplastic Left Heart Syndrome

The hybrid approach for hypoplastic left heart syndrome is a well-accepted alternative strategy to the conventional staged surgical approach. However, in the typical comprehensive stage II operation, bilateral branch pulmonary artery stenosis is a risk factor for postoperative catheter and/or surgical interventions. In addition to addressing the stenosis from the bands, the branch pulmonary arteries are at risk of compression from the dilated main pulmonary trunk and the resultant large neo-aorta. Here, we present a new aortic arch reconstruction technique combined with the pulmonary artery reconstruction in the comprehensive stage II operation to address these issues. In our experience of 11 patients who underwent hybrid strategy, Fontan completion was achieved in 8 cases (73%) during a median follow-up duration of 84 months (range; 45-161 months), and the 3-year, 5-year, and 10-year survival rates were 73% each. While 6 of 8 cases (75%) required catheter interventions to the branch pulmonary arteries, the Nakata Index was 204±11 before the stage II and 185±19 before Fontan completion, indicating that good pulmonary artery growth. Our modified comprehensive stage II technique optimizes the pulmonary arteries for the Fontan and beyond by addressing both anatomic stenoses and external compression for future growth.

Arterial Sleeve Lobectomy: Does Pulmonary Artery Reconstruction Type Impact Lung Function?

The aim of this single-center retrospective cohort study was to assess the impact of arterial reconstruction technique on lung perfusion. The second objective was to ascertain the functional validity of arterial sleeve lobectomy. Between January 2001 and December 2020, a total of 81 patients underwent lobectomy with pulmonary artery (PA) reconstruction for lung cancer at the University Hospital of Montpellier. After excluding patients with an incomplete postoperative pulmonary function test, we conducted a comparative analysis of the preoperative and postoperative functional outcomes (FEV1) of 48 patients, as well as the preoperative and postoperative Technetium99m scintigraphic pulmonary perfusion results of 28 patients. Then, we analyzed postoperative perfusion results according to the pulmonary artery reconstruction techniques use. PA reconstruction types were as follows: 9 direct angioplasties (19%), 14 patch angioplasties (29%), 7 end-to-end anastomoses (15%), 6 prosthetic bypasses (12%), 11 arterial allograft bypasses (23%), and 1 custom-made xenopericardial conduit bypass. Regardless of the type of vascular reconstruction performed, the comparative analysis of lung perfusion revealed no significant difference between the preoperative and postoperative perfusion ratio of the remaining parenchyma (median = 29.5% versus 32.5%, respectively; p = 0.47). Regarding the pulmonary functional test, postoperative predicted FEV1 significantly underestimated the actual postoperative measured FEV1 by about 260 mL (11.4%) of the preoperative value. The patency rate was 96% and the 5-year overall survival was 49% for a mean follow up period of 34 months. Lobectomy with PA reconstruction is a valid parenchymal-sparing technique in terms of perfusion and respiratory function.

Cardiac hydatidosis complicated by vascular involvement: a case report

BackgroundHydatid disease is a parasitic infection which is endemic in multiple regions across the globe including Indian subcontinent. Although it commonly affects the liver and other solid organs, isolated involvement of the heart is relatively uncommon. Cardiac hydatidosis can be confidently diagnosed on echocardiography, but cross-sectional imaging by computed tomography (CT) or magnetic resonance imaging (MRI) provides accurate information about its location, extent, and multiplicity. Multiple case studies of cardiac hydatid disease have been reported in the literature. However, cardiac hydatidosis with extension into pulmonary vasculature has not been reported commonly. This case report highlights this rare association, with stress upon the altered surgical management.Case presentationA 50-year-old patient presented with atypical chest pain to the cardiology department of a tertiary care hospital where he underwent multiple blood tests as well as electrocardiography (ECG) and echocardiography as part of the routine examination. Echocardiography revealed a large, multicystic mass invading the posterolateral wall of left ventricle (LV) with no internal vascularity and was provisionally diagnosed as hydatid disease. Subsequent CT study confirmed a large multicystic lesion with the presence of another smaller lesion invading the left atrial appendage (LAA). In order to evaluate the vascular and mediastinal invasion, cardiac MRI was then performed which revealed invasion of right pulmonary artery (PA), right superior pulmonary vein (PV) as well as compression of superior vena cava (SVC) by the smaller lesion. A final diagnosis of cardiac hydatidosis with invasion into right PA and right superior PV was made. Treatment plan for the patient changed, who then underwent an additional reconstruction of right PA and right superior PV after cyst removal.ConclusionsCardiac hydatid disease is a rare but potentially lethal condition, which tends to show slow growth and local invasion. The diversity of findings in such rare cases makes individual surgical approach extremely valuable with preoperative radiological imaging becoming indispensable for correct localization and appropriate planning. Our case report highlights rare association of cardiac hydatidosis with invasion of both pulmonary artery and vein, which eventually altered the surgical management of patient.

A Case of Right Ventricular Outflow Tract and Pulmonary Artery Reconstruction Using a Composite Graft in a Patient with Pulmonary Artery Intimal Sarcoma

A Case of Right Ventricular Outflow Tract and Pulmonary Artery Reconstruction Using a Composite Graft in a Patient with Pulmonary Artery Intimal Sarcoma

20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries

BackgroundWe have followed a consistent, albeit evolving, strategy for the management of patients with pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) that aims to achieve complete repair with low right ventricular pressure by completely incorporating blood supply and relieving stenoses to all lung segments. ObjectivesThe purpose of this study was to characterize our 20-year institutional experience managing patients with MAPCAs. MethodsWe reviewed all patients who underwent surgery for MAPCAs and biventricular heart disease from November 2001 through December 2021. ResultsDuring the study period, 780 unique patients underwent surgery. The number of new patients undergoing surgery annually was relatively steady during the first 15 years, then increased substantially thereafter. Surgery before referral had been performed in almost 40% of patients, more often in our recent experience than earlier. Complete repair was achieved in 704 patients (90%), 521 (67%) during the first surgery at our center, with a median right ventricular to aortic pressure ratio of 0.34 (25th, 75th percentiles: 0.28, 0.40). The cumulative incidence of mortality was 15% (95% CI: 12%-19%) at 10 years, with no difference according to era of surgery (P = 0.53). On multivariable Cox regression, Alagille syndrome (HR: 2.8; 95% CI: 1.4-5.7; P = 0.004), preoperative respiratory support (HR: 2.0; 95% CI: 1.2-3.3; P = 0.008), and palliative first surgery at our center (HR: 3.5; 95% CI: 2.3-5.4; P < 0.001) were associated with higher risk of death. ConclusionsIn a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis and MAPCAs have continued to improve.

Validity of Customized Branched Tissue Engineered Vascular Graft in a Porcine Model

BackgroundPatient-specific, 3-dimensional printed, tissue engineered vascular grafts (3DTEVGs) are manufactured to optimize hemodynamic performance and to accommodate growth. We evaluate growth outcomes of 3DTEVGs compared with standard grafts for pulmonary artery reconstruction in porcine models. MethodsMagnetic resonance imaging (MRI) with 4-dimensional flow data was acquired in porcine models (n = 8). 3DTEVGs guided in design by computational flow dynamics were implanted (n = 4), with polytetrafluorethylene grafts used as controls (n = 4). Postoperative MRI and histologic features of explanted grafts were evaluated after 10 weeks. ResultsAll pigs survived, with evidence of patent grafts on postoperative MRI. Graft inner diameter changes were 0.47 ± 2.31 mm in 3DTEVGs and −4.61 ± 2.15 mm in controls (P = .018). Mean main pulmonary artery wall shear stress was significantly lower in 3DTEVGs (7.12 ± 4.21 Pa) than in controls (18.15 ± 8.37 Pa; P = .0396). Histologic evaluation of 3DTEVGs showed a single layer of endothelial cells, an organized smooth muscle layer, and collagen deposition with a remaining scaffold area of 21.37% ± 20.46%. ConclusionsOur patient-specific 3DTEVGs demonstrated optimal anatomic fit while maintaining ideal flow dynamics and promoting appropriate neovessel formation.

Bilateral lung transplant with pulmonary artery reconstruction using donor aorta for pulmonary hypertension with a giant pulmonary arterial aneurysm.

Standard bilateral lung transplantation (BLT) is not feasible for patients with pulmonary arterial hypertension (PAH) complicated with a giant pulmonary arterial aneurysm (PAA). This study aimed to describe the outcomes of BLT with pulmonary artery reconstruction (PAR) using donor aorta for such patients. This is a retrospective single-centre study reviewing PAH patients with a PAA who received BLT with PAR using donor aorta from January 2010 through December 2020. We compared the characteristics and short- and long-term outcomes of recipients receiving PAR (PAR group) with those who had no PAA and received standard BLT (non-PAR group). Nineteen adult PAH patients underwent cadaveric lung transplantation during the study period. Among them, 5 patients with a giant PAA (median pulmonary artery trunk diameter, 69.9 mm) underwent BLT with PAR using donor aorta and the others received standard BLT. Although the operation time tended to be longer in the PAR group compared with the non-PAR group (1239 vs 958 mins, P = 0.087), 90-day mortality (PAR group: 0% vs non-PAR group: 14.3%, P > 0.99), and 5-year survival rate (PAR group: 100% vs non-PAR group: 85.7%, P = 0.74) was comparable between the groups. No dilatation, constriction or infection of the aortic grafts were recorded during the study period with a median follow-up time of 94 months in the PAR group. Lung transplantation with PAR using donor aorta is a valid surgical option for PAH patients complicated with a giant PAA.