Lung Transplantation For Pulmonary Hypertension Research Articles

Single Lung Transplant for Secondary Pulmonary Hypertension: The Right Option for the Right Patient.

Introduction: The optimal treatment for Secondary Pulmonary Hypertension from End-Stage Lung Disease remains controversial. Double Lung Transplantation is widely regarded as the treatment of choice as it eliminates all diseased parenchyma and introduces a large volume of physiologically normal allograft. By comparison, the role of single lung transplantation for pulmonary hypertension (PAH) is less clear. The remaining diseased lung will limit clinical improvements and permit downstream sequelae; including residual cough, recurrent infection, and continued pulmonary hypertension. But not every patient can undergo DLT. Advanced age, frailty, co-morbid conditions, and limited availability of organs will all affect surgical candidacy and can offset the benefits of double lung procedures. Studies that compare SLT and DLT do not commonly explore the utility of single lung procedures even though multiple theoretical advantages exist; including reduced waiting times, less waitlist mortality, fewer surgical complications, and lower operative mortality. Worse, multiple forms of publication and selection bias may favor DLT in registry-based studies. In this review, we present the prevailing literature on single and double lung transplants in patients with secondary pulmonary hypertension and clarify the potential utility of these procedures. Materials and Methods: A PubMed search for English-language articles exploring single and double lung transplants in the setting of secondary pulmonary hypertension was conducted from 1990 to 2023. Key words included "single lung transplant", "double lung transplant", "pulmonary hypertension", "rejection", "complications", "extracorporeal membranous oxygenation", "death", and all appropriate Boolean operators. We prioritized research from retrospective studies that evaluated clinical outcomes from single centers. Conclusions: The question is not whether DLT is better at resolving lung disease; instead, we must ask if SLT is an acceptable form of therapy in a select group of high-risk patients. Further research should focus on how best to identify recipients that may benefit from each type of procedure, and the clinical utility of perioperative VA ECMO.

Living-donor lobar lung transplantation for pulmonary hypertension: living on the edge?

Journal Article Accepted manuscript Living-donor lobar lung transplantation for pulmonary hypertension: living on the edge? Get access Dirk Van Raemdonck, MD, PhD, Dirk Van Raemdonck, MD, PhD Departments of Thoracic Surgery, University Hospitals LeuvenDepartment of Chronic Diseases and Metabolism, Katholieke Universiteit Leuven, Leuven, Belgium Corresponding Author Contact Information: Dirk Van Raemdonck, MD, PHD, Department of Thoracic Surgery, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium. E-mail: dirk.vanraemdonck@uzleuven.be Phone:+32 16 34 68 23, Facsimile:+32 16 34 68 24 https://orcid.org/0000-0003-1261-0992 Search for other works by this author on: Oxford Academic PubMed Google Scholar Robin Vos, MD, PhD, Robin Vos, MD, PhD Departments of Respiratory Diseases, University Hospitals LeuvenDepartment of Chronic Diseases and Metabolism, Katholieke Universiteit Leuven, Leuven, Belgium https://orcid.org/0000-0002-3468-9251 Search for other works by this author on: Oxford Academic PubMed Google Scholar Geert M Verleden, MD, PhD, Geert M Verleden, MD, PhD Departments of Respiratory Diseases, University Hospitals LeuvenDepartment of Chronic Diseases and Metabolism, Katholieke Universiteit Leuven, Leuven, Belgium https://orcid.org/0000-0003-3048-2429 Search for other works by this author on: Oxford Academic PubMed Google Scholar Laurens J Ceulemans, MD, PhD Laurens J Ceulemans, MD, PhD Departments of Thoracic Surgery, University Hospitals LeuvenDepartment of Chronic Diseases and Metabolism, Katholieke Universiteit Leuven, Leuven, Belgium https://orcid.org/0000-0002-4261-7100 Search for other works by this author on: Oxford Academic PubMed Google Scholar European Journal of Cardio-Thoracic Surgery, ezad097, https://doi.org/10.1093/ejcts/ezad097 Published: 23 March 2023 Article history Received: 03 March 2023 Accepted: 13 March 2023 Published: 23 March 2023

Comparison of living-donor lobar lung transplantation and cadaveric lung transplantation for pulmonary hypertension.

Living-donor lobar lung transplantation (LDLLT) is a life-saving procedure for critically ill patients with various lung diseases, including pulmonary hypertension (PH). However, there are concerns regarding the development of heart failure with pulmonary oedema after LDLLT in which only 1 or 2 lobes are implanted. This study aimed to compare the preoperative conditions and postoperative outcomes of LDLLT with those of cadaveric lung transplantation (CLT) in PH patients. Between 2008 and 2021, 34 lung transplants for PH, including 12 LDLLTs (5 single and 7 bilateral) and 22 bilateral CLTs, were performed. Preoperative variables and postoperative outcomes were retrospectively compared between the 2 procedures. Based on the preoperative variables of less ambulatory ability (41.7% vs 100%, P < 0.001), a higher proportion of World Health Organization class 4 (83.3% vs 18.2%, P < 0.001) and higher mean pulmonary artery pressure (74.4 vs 57.3 mmHg, P = 0.040), LDLLT patients were more debilitated than CLT patients. Nevertheless, hospital death was similar between the 2 groups (8.3% vs 9.1%, P > 0.99, respectively). Furthermore, the 5-year overall survival rate was similar between the 2 groups (90.0% vs 76.3%, P = 0.489). Although LDLLT patients with PH had worse preoperative conditions and received smaller grafts than CLT patients, LDLLT patients demonstrated similar perioperative outcomes and prognoses as CLT patients. LDLLT is a viable treatment option for patients with PH.

Advanced Circulatory Support and Lung Transplantation in Pulmonary Hypertension.

Pulmonary arterial hypertension (PAH) is a progressive fatal disease. Although medical therapies have improved the outlook for these patients, there still exists a cohort of patients with PAH who are refractory to these therapies. Lung transplantation (LT), and in certain cases heart-lung transplantation (HLT), is a therapeutic option for patients with severe PAH who are receiving optimal therapy yet declining. ECMO may serve as a bridge to transplant or recovery in appropriate patients. Although, the mortality within the first 3 months after transplant is higher in PAH recipients than the other indications for LT, and the long-term survival after LT is excellent for this group of individuals. In this review, we discuss the indications for LT in PAH patients, when to refer and list patients for LT, the indications for double lung transplant (DLT) versus HLT for PAH patients, types of advanced circulatory support for severe PAH, and short and long-term outcomes in transplant recipients with PAH.

Bilateral lung transplant for pulmonary hypertension with pulmonary artery aneurysm.

Bilateral lung transplant for pulmonary hypertension with pulmonary artery aneurysm.

New strategy to resume and taper epoprostenol after lung transplant for pulmonary hypertension

The perioperative outcome of lung transplantation (LTx) for patients with severe pulmonary hypertension (PH) remains poor due to the occurrence of primary graft dysfunction (PGD) from left ventricular failure. We hypothesized that tapering pretransplant use of epoprostenol rather than abrupt discontinuation after transplantation might improve perioperative outcomes. We performed 23 LTxs for patients with severe PH who received epoprostenol therapy from 2008 until 2021. In the discontinued group (n = 6), epoprostenol was discontinued after the establishment of extracorporeal circulation. In the tapered group (n = 17), epoprostenol was discontinued and resumed after reperfusion, and then gradually tapered over the following 2weeks. We assessed survival, bleeding, blood transfusion, re-opening of the chest, oxygenation, PGD score, extracorporeal membrane oxygenation (ECMO) requirement for recovery after transplantation, and duration of mechanical ventilation. The PGD score was significantly lower in the tapered group than in the discontinued group at 0h, 24h, and 48h after LTx. In addition, the discontinued group required longer mechanical ventilation than the tapered group. Delayed chest closure and post-transplant ECMO use for recovery occurred significantly more frequently in the discontinued group. To resume and taper epoprostenol administration after reperfusion in patients with severe PH may be a valuable new strategy associated with better perioperative outcomes.

Meticulous closure of collateral vessels in the perihilar mediastinal pleura to control intraoperative bleeding during lung transplantation for pulmonary hypertension.

BackgroundMassive blood transfusion compensating hemorrhage during lung transplantation (LT) results in primary graft dysfunction (PGD) and worse outcomes after LT. Collateral vessels in the perihilar mediastinal pleura could be the source of hemorrhage during LT in patients with pulmonary hypertension (PH). The purpose of this study was to examine the effect of closure with hemoclips of the vessels in the perihilar mediastinal pleura on the risk of intraoperative hemorrhage and outcomes after LT in patients with PH.MethodsWe retrospectively reviewed 80 patients who underwent LT, including 13 patients with primary PH, 29 patients with secondary PH, and 38 patients with non-PH.ResultsThe median number of hemoclips was significantly higher in the primary PH group than in the non-PH group (P=0.0045) or secondary PH group (P=0.0060). The intraoperative blood loss, transfusion volume, maximum PGD grade, and the 30-day and 90-day mortality rates in the primary PH group were equivalent to those in the other two groups.ConclusionsMeticulous closure of collateral vessels in the perihilar mediastinal pleura during LT in patients with primary PH allowed intraoperative hemorrhage to be controlled and might be associated with acceptable mortality rate in these patients similar to that of LT in patients with other diseases.

Pneumonia With Scedosporium apiospermum and Lomentospora prolificans in a Patient After Bilateral Lung Transplantation for Pulmonary Hypertension: A Case Report

Infections caused by the Scedosporium genus have become recognized as a fatal complication after lung transplantation in Europe and Australia, but the reports have been rare from Asian countries including Japan. We present a case of pneumonia caused by a mixed infection of Scedosporium apiospermum (SA) and Lomentospora prolificans (LP) that developed after augmentation of immunosuppression for chronic lung allograft dysfunction (CLAD) after lung transplantation. A 13-year-old man underwent bilateral lung transplantation for pulmonary hypertension. One year after surgery, he was treated with a series of augmented immunosuppressive therapy for severe acute rejection and subsequent CLAD. Three months following the first steroid pulse therapy, his serum β-D-glucan elevated without any sign of fungal infection by other tests. The serum β-D-glucan once returned to a normal level by empirical administration of micafungin; however, the patient's condition worsened again by discontinuation of it. He did not recover by restarting micafungin, and computed tomography (CT) scans eventually demonstrated new infiltrates in his lung field 6 weeks after the elevation of serum β-D-glucan. Microscopic findings of transbronchial lung biopsy specimens showed filamentous fungi, and the culture of bronchoalveolar lavage fluid revealed the growth of SA and LP. Despite subsequent voriconazole administration, he died 14 days after the start of voriconazole. Early and aggressive inspection including bronchoscopy should be performed for the diagnosis of Scedosporium infection in immunocompromised patients, even if CT scans and sputum culture show no evidence of infection.

Midterm outcomes of the Potts shunt for pediatric pulmonary hypertension, with comparison to lung transplant

ObjectiveFor children with severe pulmonary hypertension, addition of Potts shunt to a comprehensive palliation strategy might improve the outcomes afforded by medications and delay lung transplantation. MethodsA prospective analysis was conducted of all children undergoing Potts shunt (first performed in 2013) or bilateral lung transplant for pulmonary hypertension from 1995 to present. ResultsA total of 23 children underwent Potts shunt (20 surgical, 3 transcatheter), and 31 children underwent lung transplant. All children with Potts shunt had suprasystemic right ventricle pressures despite maximal medical treatment. In the majority of patients, the Potts shunt was performed through a left thoracotomy approach (90%, 18/20), by direct anastomosis (65%, 13/20), and without the use of extracorporeal support (65%, 13/20). Perioperative outcomes after Potts shunt were superior to lung transplant including mechanical ventilation time (1.3 vs 10.2 days, P = .019), median hospital length of stay (9.8 vs 34 days, P = .012), and overall complication rate (35% [7/20] vs 81% [25/31], P = .003). Risk factors for operative mortality after Potts shunt (20%, 4/20; compared with 6%, 2/31 for lung transplant, P = .195) included preoperative extracorporeal membrane oxygenation and significant right ventricle dysfunction. In midterm follow-up (median 1.8, maximum 6.1 years), patients with Potts shunt had durable equalization of right ventricle/left ventricle pressures and improved functional status. There was no significant survival difference in patients with Potts shunt and patients with lung transplant (P = .258). ConclusionsPotts shunt is an effective palliation for children with suprasystemic pulmonary hypertension that may become part of a strategy to maximize longevity and functional status for these challenging patients.

Lung transplantation for pulmonary hypertension with giant pulmonary artery aneurysm

BackgroundAneurysm of the main pulmonary artery trunk (PAA) is a rare but severe complicating factor in patients suffering from pulmonary arterial hypertension (PAH). Many centers consider PAA an indication for a heart-lung transplantation. We aimed to summarize our institutional experience with a lung-only strategy in this complex group of patients. MethodsWe performed a retrospective single-center analysis of patients with PAH and a severe PAA who underwent lung transplantation between January 1996 and November 2018. ResultsA total of 127 patients with PAH underwent lung transplantation during the study period. Seven patients presented with severe PAA (mean diameter, 70.4 mm). Donor lungs were procured together with the main pulmonary artery (PA). In the recipient, cardiopulmonary bypass with bicaval cannulation was established, and bilateral pneumonectomy together with resection of the entire PA trunk was performed. The right donor lung was implanted, and the attached PA trunk was pulled through behind the superior vena cava and ascending aorta. Anastomosis was performed just above the level of the pulmonary valve. Thereafter, the left lung was implanted by reconnecting the left PA to the main PA trunk. All but 1 patient, who died from sepsis on postoperative day 13, were successfully discharged. ConclusionsTo the best of our knowledge, this is the largest published experience of patients with PAH and severe PAA who underwent lung transplantation. We show that these patients are eligible for double lung transplantation and do not require heart-lung transplantation.

A New Strategy after Lung Transplantation for Pulmonary Hypertension: Tapering Epoprostenol Administration

Purpose The perioperative survival of lung transplantation (LTx) for patients with idiopathic pulmonary arterial hypertension is known to be worse than for those with other underlying diseases because of difficulties in managing perioperative haemodynamic instability. High dose epoprostenol (PGI2) is frequently used for patients with end-stage pulmonary hypertension (PH) before transplantation, however, it is generally discontinued at the time of transplantation. We hypothesized that tapering epoprostenol rather than abrupt discontinuation after reperfusion would improve perioperative outcomes. Methods We performed 193 LTxs since 2008 and 16 PH patients were on high dose epoprostenol (10-200 ng/kg/min) therapy. Epoprostenol was discontinued at the time of lung transplantation in 6 patients and it was tapered after reperfusion in ten patients. In the Discontinued group, epoprostenol was discontinued intraoperatively during extracorporeal membrane oxygenation (ECMO) or cardiopulmonary bypass and was not resumed postoperatively. In the Tapered group, epoprostenol was discontinued during extracorporeal support and was resumed at the quarter of preoperative dose after reperfusion. It was then gradually tapered typically over the following two weeks according to the heart function on echocardiography. Results One patient in the Discontinued group died on day 125 due to multiple organ failure, in contrast, no operative death was encountered in the Tapered group. Delayed chest closure and postoperative ECMO were more frequently required in the Discontinued group than the Tapered group (p = 0.0076, respectively). Primary graft dysfunction score was significantly lower in the Tapered group than the Discontinued group (0.6 ± 1.3 vs 2.2 ± 1.3 at 0h, p = 0.031; 0.4 ± 0.8 vs 2.0 ± 1.5 at 24h, p = 0.028; 0.3 ± 0.7 and 1.8 ± 1.5 at 48h, p = 0.033). Furthermore, the Tapered group required significantly shorter duration of postoperative mechanical ventilation (15.1 ± 6.8 vs 41.5 ± 41.9 days, p = 0.039). Conclusion Tapering epoprostenol administration after reperfusion for PH may be a valuable new strategy which is associated with better perioperative outcomes.

Intensive care, right ventricular support and lung transplantation in patients with pulmonary hypertension.

Intensive care of patients with pulmonary hypertension (PH) and right-sided heart failure includes treatment of factors causing or contributing to heart failure, careful fluid management, and strategies to reduce ventricular afterload and improve cardiac function. Extracorporeal membrane oxygenation (ECMO) should be considered in distinct situations, especially in candidates for lung transplantation (bridge to transplant) or, occasionally, in patients with a reversible cause of right-sided heart failure (bridge to recovery). ECMO should not be used in patients with end-stage disease without a realistic chance for recovery or for transplantation. For patients with refractory disease, lung transplantation remains an important treatment option. Patients should be referred to a transplant centre when they remain in an intermediate- or high-risk category despite receiving optimised pulmonary arterial hypertension therapy. Meticulous peri-operative management including the intra-operative and post-operative use of ECMO effectively prevents graft failure. In experienced centres, the 1-year survival rates after lung transplantation for PH now exceed 90%.

Central versus Peripheral Cannulation of Extracorporeal Membrane Oxygenation Support during Double Lung Transplantation for Pulmonary Hypertension

Central versus Peripheral Cannulation of Extracorporeal Membrane Oxygenation Support during Double Lung Transplantation for Pulmonary Hypertension

Lobar Lung Transplantation from Brain-dead Donors in Pulmonary Hypertension

Lobar lung transplantation is known as an alternative for higher Donor/Receiver mismatch in cystic fibrosis or idiopathic pulmonary fibrosis patients. Using lobar lung transplantation for Pulmonary Hypertension remains questionable because of the higher risk of primary graft dysfunction. We thought to determine wether lobar lung transplantation from brain-dead donors is an option for Pulmonary Hypertension.

Lung Transplantation in Pulmonary Hypertension: A Multidisciplinary Unit's Management Experience

BackgroundPrognosis assessment of pulmonary hypertension (PH) is multifactorial and placement of patients on the lung transplantation (LT) waiting list requires the weighing of a complex set of criteria. The aim of this retrospective cohort study was to analyze a series of patients treated in our unit at the moment of their inclusion on the LT waiting list and long-term survival after LT. Material and MethodsBaseline characteristics, LT outcomes, and survival were evaluated in all patients diagnosed with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) who were included on the LT waiting list in 2011–2016. ResultsThirty-three patients were listed with a diagnosis of PAH or PVOD. Patients had an average age of 43 ± 12 years and 71% were female. The median time between PAH diagnosis and inclusion on the LT waiting list was 62.5 months (interquartile range [IQR], 6–93.3 months). Twenty-eight patients (84%) underwent double LT. The difference between the waiting time in urgent cases (1.5 months; IQR, 0.4–4.2 months) and in elective cases (7.4 months; IQR, 2.7–16.2 months) was significant (P < .049). The 28 patients with PAH/PVOD in our hospital had a 95% short-term survival after LT both at 1 and at 3 months, without variance between urgent and elective LT. Longer-term survival rate was 84% both at 12 and 36 months. ConclusionsThere is great complexity in determining the appropriate time for transplantation referral and inclusion on the waiting list for patients with PAH/PVOD so that LT can be more realistically incorporated into the treatment algorithm for PAH. LT offers a good short- and long-term survival in patients with PAH/PVOD.

Central versus peripheral cannulation of extracorporeal membrane oxygenation support during double lung transplant for pulmonary hypertension.

Extracorporeal membrane oxygenation (ECMO) has become the standard of cardiopulmonary support during a sequential double lung transplant for pulmonary hypertension. Whether central or peripheral cannulation is the best strategy for these patients remains unknown. Our goal was to determine which is the best strategy by comparing 2 populations of patients. We performed a single-centre retrospective study based on an institutional prospective lung transplant database. Between January 2011 and November 2016, 103 patients underwent double lung transplant for pulmonary hypertension. We compared 54 patients who had central ECMO (cECMO group) to 49 patients who had peripheral ECMO (pECMO group). The pECMO group had significantly more bridged patients who received emergency transplants (31% vs 6%, P = 0.001). The incidence of Grade 3 primary graft dysfunction requiring ECMO (14% vs 11%, P = not significant) and of in-hospital mortality (11% vs 14%, P = not significant) was similar between the groups. Groin infections (16% vs 4%, P = 0.031), deep vein thrombosis (27% vs 11%, P = 0.044) and lower limb ischaemia (12% vs 2%, P = 0.031) occurred significantly more often in the pECMO group. The number of chest reopenings for bleeding or infection was similar between the groups. The 3-month, 1-year and 5-year survival rates did not differ between the groups (P = 0.94). Central or peripheral ECMO produced similar results during double lung transplant for pulmonary hypertension in terms of in-hospital deaths and long-term survival rates. Central ECMO provided satisfactory results without major bleeding provided the patient was weaned from ECMO at the end of the procedure. Despite the rate of groin and lower limb complications, peripheral cannulation remained the preferred solution to bridge the patient to transplant or for postoperative support.

Lung Transplantation for Pulmonary Hypertension and Strategies to Bridge to Transplant.

AbstractDespite an expanded armamentarium of medical therapies, pulmonary arterial hypertension (PAH) remains a progressive disease associated with significant morbidity and premature mortality. Lung transplantation (LT) is the last safety net for patients failing medical therapy, providing excellent overall long-term survival and health-related quality-of-life outcomes in line with more common parenchymal lung disease indications. Waitlist mortality remains disproportionally elevated, however, reflecting an inability of the lung allocation score to completely capture PAH disease severity, and a propensity for PAH to deteriorate rapidly without warning, even in patients who appear externally “well.” Early referral to a LT center can mitigate these risks and facilitate rapid listing if necessary. Several bridging therapies are available to support severely unwell patients to LT, such as extracorporeal life support (ECLS) and atrial septostomy. Unique perioperative considerations include higher rates of primary graft dysfunction and dynamic right ventricular outflow obstruction which may, at least in part, reflect rapid afterload reduction in the face of a conditioned right ventricle. Extending ECLS into the perioperative period may ameliorate these risks by allowing more gradual adaptation of both ventricles to their new loading conditions. Chronic lung allograft dysfunction, particularly bronchiolitis obliterans syndrome, remains a major cause of long-term morbidity and mortality, and complications from corticosteroid and immunosuppressive therapy are common. Nevertheless, the morbidity, mortality, and burden of disease management after LT continue to improve and compare favorably to that of refractory PAH in carefully selected patients.

(1248) - Left Atrial Pressure Continuous Monitoring Improves Early Postoperative Outcomes After Double Lung Transplantation for Pulmonary Hypertension

(1248) - Left Atrial Pressure Continuous Monitoring Improves Early Postoperative Outcomes After Double Lung Transplantation for Pulmonary Hypertension

Multidetector computed tomography shows reverse cardiac remodeling after double lung transplantation for pulmonary hypertension

ObjectiveTo use multidetector computed tomography (MDCT) to evaluate the structural changes in the right heart and pulmonary arteries that occur in patients with severe pulmonary hypertension treated by double lung transplantation. Material and methodsThis was a retrospective study of 21 consecutive patients diagnosed with severe pulmonary hypertension who underwent double lung transplantation at our center between 2010 and 2014. We analyzed the last MDCT study done before lung transplantation and the first MDCT study done after lung transplantation. We recorded the following variables: diameter of the pulmonary artery trunk, ratio of the diameter of the pulmonary artery trunk to the diameter of the ascending aorta, diameter of the right ventricle, ratio of the diameter of the left ventricle to the diameter of the right ventricle, and eccentricity index. Statistical analysis consisted of the comparison of the means of the variables recorded. ResultsIn all cases analyzed, the MDCT study done a mean of 24±14 days after double lung transplantation showed a significant reduction in the size of the right heart chambers, with improved indices of ventricular interdependency index, and reduction in the size of the pulmonary artery trunk (p<0.001 for all the variables analyzed). ConclusionPatients with pulmonary hypertension treated by double lung transplantation present early reverse remodeling of the changes in the structures of the right heart and pulmonary arterial tree. MDCT is useful for detecting these changes.

Lung transplantation for pulmonary hypertension: management of pulmonary hypertension on the waiting list

Despite the apparent improvement in outcomes for pulmonary arterial hypertension (PAH) with current medical therapy, for many patients, lung transplantation remains as an important therapeutic option. Such patients are at high risk for clinical deterioration while awaiting lung transplantation. Thus, the medical management is crucial to keep these tenuous patients acceptable on the waitlist. This includes optimization of PAH-targeted therapy and adjunctive therapies. For select patients, atrial septostomy may further expand the transplant window. Clinical deterioration of PAH, usually in the form of decompensated right-sided heart failure, often requires careful critical care management. Care of such patients is limited by high in-hospital mortality and a dearth of guiding evidence. Extracorporeal life support (ECLS) has been increasingly utilized to bridge select decompensated and otherwise moribund patients to lung transplantation. Overall, careful pre-transplant management may be contributing to the reduced waitlist mortality in recent years despite higher disease acuity.