Heart-lung Transplantation Research Articles

Outcomes of heart-lung transplantation in Eisenmenger syndrome compared to primary pulmonary hypertension.

Heart-Lung Transplantation (HLTX) is required both in primary pulmonary hypertension (PPH) and Eisenmenger syndrome (ES) when there is associated end-stage heart disease. Although PPH is associated with an otherwise structurally normal heart, ES is associated with congenital heart defects, which may increase the complexity of the operation. This study analyzes if the diagnosis (PPH vs. ES) is related to short-term outcomes after HLTX. Patients ≥18 years of age with PPH and ES who underwent HLTX were identified in the United Network for Organ Sharing database from 2005 to 2021. Patients were propensity score matched on heart and lung listing status at the time of transplant. Univariable, multivariable, and Kaplan-Meir survival analyses were performed. The unmatched cohort had 128 PPH and 44 ES patients, and the matched cohort had 44 patients in each group. PPH patients had lower waitlist times and PA pressures but higher FEV1, heart, and lung listing status and ECMO bridge. There were no differences in immediate postoperative outcomes such as dialysis, stroke, and airway dehiscence. PPH patients had a higher treatment rejection in the first year. The 30-day, 1-year, and 3-year survival were better in the PPH group. However, a landmark analysis excluding deaths within 30 days eliminated differences in survival between the groups. Post-transplant dialysis and postoperative mechanical ventilation >5 days were risk factors for 1-year mortality in ES. The short-term outcomes of HLTX are inferior in ES compared to PPH and much of the attrition in ES occurs in the immediate postoperative period.

Extended criteria donor organ use for heart-lung transplantation in the modern era

BackgroundDemand for donor hearts and lungs exceeds their supply. Extended Criteria Donor (ECD) organs are used to help meet this demand, but their impact on heart-lung transplantation outcomes is poorly characterized. Methods and results: The United Network for Organ Sharing was queried for data on adult heart-lung transplantation recipients (n = 447) from 2005‒2021. Recipients were stratified based on whether they received ECD hearts and/or lungs. Morbidity was analyzed using Kruskal-Wallis, chi-square, and Fisher's exact tests. Mortality was analyzed using Kaplan-Meier estimation, log-rank tests and Cox regression. Sixty-five (14.5%) patients received two ECD organs, 134 (30.0%) received only an ECD lung, and 65 (14.5%) only an ECD heart. Recipients of two ECD organs were older, more likely to have diabetes, and more likely transplanted from 2015‒2021 (p < 0.05). Groups did not differ by pre-transplant diagnosis, intensive care unit disposition, life support use, or hemodynamics. Group five-year survival rates ranged from 54.5% to 63.2% (p = 0.428). Groups did not differ by 30-day mortality, strokes, graft rejection, or hospital length of stay. Conclusions: Using ECD hearts and/or lungs for heart-lung transplantation is not associated with increased mortality and is a safe strategy for increasing donor organ supply in this complex patient population.

Management and outcome of obstructive airway complications after lung transplantation - a 12-year retrospective cohort study.

Obstructive airway complications (OACs) represent a significant problem after lung transplantation (LTx). Bilateral OACs after double lung transplantation are infrequently reported. The aim of this study was to investigate management and outcome of OAC. Retrospective single-center cohort study. Adult patients with bilateral LTx performed between 2010 and 2021 were included. Patients with follow-ups of less than 3 months and after heart-lung transplantation were excluded. OAC was defined either as the need for stenting, surgical revision, or balloon dilatation. Outcome parameters included graft survival, graft function, quality of life, and management. During the study period, 1,170 patients were included. Hundred thirty-five (11.5%) patients developed OAC. Forty-six (4.4%) patients had significant bilateral OAC. Thirty-seven (80%) bilateral OAC patients were treated by stent insertion; in 34 patients, biodegradable stents were used. The median number of bronchoscopies in bilateral OAC was 26 during the first postoperative year compared with nine in controls (p < 0.001). Fourteen OAC patients (n = 10 bilateral) underwent surgical revision including six re-do transplantations. Graft loss occurred significantly more frequently in patients with bilateral OAC with a graft survival of 63% and 50% in these after 3 and 5 years compared with 83% and 73% in controls without OAC (p < 0.001). Baseline forced expiratory volume in 1 s (FEV1) in patients with bilateral OAC was median 58% predicted in comparison with 90% in controls (p < 0.001). Quality of life was significantly reduced. Bilateral OACs impose a high burden of disease on patients after lung transplantation and were associated with early and late graft loss. Affected patients' OAC demonstrated reduced graft function and impaired quality of life. Most OACs were managed by bronchoscopy preferably by non-permanent stenting. Surgery including re-do transplantation was used in selected cases.

State of the art - Extracorporeal membrane oxygenation as a bridge to thoracic transplantation.

Extracorporeal membrane oxygenation (ECMO) has revolutionized the treatment of refractory cardiac and respiratory failure, and its use continues to increase, particularly in adults. However, ECMO-related morbidity and mortality remain high. In this review, we investigate and expand upon the current state of the art in thoracic transplant and extracorporeal life support (ELS). In particular, we examine recent increase in incidence of heart transplant in patients supported by ECMO; the potential changes in patient care and selection for transplant in the years prior to updated United Network for Organ Sharing (UNOS) organ allocation guidelines versus those in the years following, particularly where these guidelines pertain to ECMO; and the newly revived practice of heart-lung block transplants (HLT) and the prevalence and utility of ECMO support in patients listed for HLT. Our findings highlight encouraging outcomes in patients bridged to transplant with ECMO, considerable changes in treatment surrounding the updated UNOS guidelines, and complex, diverse outcomes among different centers in their care for increasingly ill patients listed for thoracic transplant.

1801. LVAD antimicrobial prophylaxis and infections, a 12-year experience

Abstract Background The incidence of end-stage heart failure necessitating advanced cardiac therapy continues to increase in the United states. Implantable left ventricular assist devices (LVADs) represent an important modality that is utilized both as a bridge to heart transplant or as destination therapy. LVAD-associated infections, ranging from driveline exit site or pocket infections to endovascular infections, are a major complication with high mortality. Antibiotic prophylaxis at time of LVAD implantation is therefore an important strategy to mitigate infections. However, a clear surgical infection prophylaxis regimen and associated outcomes have not been reported. Methods We performed a single center, retrospective cohort study evaluating the impact of antimicrobial prophylaxis on risk of infection in patients who underwent LVAD implantation between February 2007 and June 2019 at Mayo Clinic. LVAD-specific and related infections were defined according to the International Society of Heart Lung Transplantation criteria. Single drug prophylaxis (SDP) included cefazolin only, vancomycin only, or cefazolin and vancomycin; multidrug (MDP) regimen includes either of the SDP regimens plus another antibiotic. We compared incidence of infection within 90-day and within one-year of implantation, as well as risk of C. difficile infections between the SDP and MDP cohorts. Results We reviewed 403 patients who received LVAD implantation (Table 1), 402 of had information on surgical prophylaxis. 307 (76%) patients received SDP and 95 patients (24%) received MDP. 14 patients developed an infection within 1 year of implantation (Table 2). There was no difference in the incidence of infections between SDP and MDP arms both at 90 days and 1-year post-implantation (p=0.11). The median time of infection since LVAD implantation was similar between SDP and MDP (p=0.37) arms. Incidence of C. difficile infection was not different between groups (p=0.10) (Table 3). Conclusion There was no significant difference in incidence or time to first infection, within 90-day and 1-year of implantation, between single- and multidrug antibiotic surgical prophylaxis regimens administered at the time of LVAD implantation. Future prospective trials are needed to develop a clear LVAD antibiotic prophylaxis protocol. Disclosures John C. O'Horo, Sr., MD, MPH, Bates college: Advisor/Consultant|MITRE corporation: Grant/Research Support|nferenec, Inc: Grant/Research Support.

Modern outcomes of heart-lung transplantation: assessing the impact of the updated US allocation system.

In 2018, the United Network for Organ Sharing modified their heart allocation policy to reduce waitlist mortality and prioritize patients with the greatest acuity. Considering declining rates of combined heart-lung transplantation, this study sought to investigate the impact of the new allocation system on waitlist and post-transplant outcomes of patients listed for combined heart-lung transplantation. Adult patients listed for combined heart-lung transplant between 2012 and 2021 were included. Patients were stratified according to listing era. Competing risk regression was used to assess waitlist outcomes. Cox proportional hazards regression was used to establish risk factors for post-transplant mortality. A total of 511 patients were included, of whom 295 (57.8%) were listed in era 1 and 216 (42.2%) in era 2. Era 2 was associated with increased likelihood of transplant (adjusted standard hazard ratio (aSHR): 1.60 [1.23-2.07]; P < 0.01) and decreased waitlist mortality (aSHR: 0.43 [0.25-0.73]; P < 0.01). Despite longer ischaemic times and increased use of preoperative veno-arterial extracorporeal membrane oxygenation (ECMO) in era 2, early post-transplant survival was equivalent. Predicted heart mass ratio <0.8 (Hazard ratio (HR); 3.24; P = 0.01), ventilator support (HR: 3.83; P < 0.01) and greater ischaemic times (HR: 1.80; P < 0.01) independently predicted the mortality. Procedures at high centre volumes (HR: 0.36; P = 0.04) were associated with decreased mortality. Use of ECMO was not predictive of mortality in the modern era. The allocation policy change has led to improvements in waitlist outcomes in patients listed for heart-lung transplantation. Despite increased ischaemic times and use of ECMO, early post-transplant survival was equivalent.

A newer era of heart-lung transplantation?

A newer era of heart-lung transplantation?

ICU Outcomes and Quality of Life in Lung Transplant Patients Readmitted to Intensive care

Admission to the ICU is relatively common in lung transplant recipients. The ICU mortality rate has been reported to be around 37% per admission. The aim of our study was to assess patient characteristics, ICU outcomes and QOL of lung transplant patients who required ICU readmission. ICU data from the first ICU readmission were collected retrospectively. QOL was assessed using SF36v2 tool prospectively. Cardiopulmonary transplant centre Participants: 63 lung transplant recipients who were readmitted to ICU formed the study group. 66 patients selected by computer random number generator software who underwent transplant at the same time but were not readmitted to ICU after transplant formed the control group. The response rate to the questionnaire was 70%. Mean ICU LOS was 4.53 (SD 4.68) days. Mean duration of mechanical ventilation was 3.83 (SD 4.03) days. Overall mortality was 21% in the study group and 9% in the control group (p = 0.08). SF36v2 scores were lower in the study population compared to Australian norms. The mean PCS summary scores for study group and control groups were 42.21 (SD 12.81) and 45.32 (SD 11.28) respectively (p = 0.267) and that for the mean MCS were 45.68 (SD 12.37) and 47.79 (SD 9.25) respectively (p= 0.410). Lung transplant patients requiring ICU readmission had higher overall mortality (p= 0.08). Those patients who survived had good ICU outcomes and similar QOL scores compared to those who did not require ICU readmission.

Heart and Lung Transplant for Mixed Connective Tissue Disease: A Case Report and Implications for Pretransplant Testing

We present a case of end-stage lung disease secondary to mixed connective tissue disease (MCTD) with concomitant myocarditis found on explant at time of transplant. The patient is a 37-year-old man who was first diagnosed with interstitial lung disease secondary to MCTD at 30 years of age. He underwent en bloc heart-lung transplant for progressive decline in left ventricular ejection fraction and severe pulmonary fibrosis despite immunosuppression with hydroxychloroquine, mycophenolate, and azathioprine. Cardiac MRI failed to demonstrate findings suggestive of myocarditis; however, explant demonstrated significant lymphocytic infiltrate with myocyte damage and areas of fibrosis with myocyte hypertrophy. In patients presenting with unexplained systolic dysfunction in the setting of MCTD, fluorodeoxyglucose-positron emission tomography may be a screening tool and if myocardial inflammation is noted, there may be a role for increased immunosuppression. While this strategy was not employed in our patient, his improvement in left ventricular ejection fraction while on mycophenolate mofetil as compared with HCQ and explant histology suggests a process that may have been further responsive to escalation of immunosuppression.

A bilateral lung transplant and giant pulmonary artery aneurysm reconstruction with an aortic graft in a woman with pulmonary hypertension.

An aneurysm of the pulmonary artery is a rare but severe complication in patients suffering from pulmonary arterial hypertension. For these patients, a lung or heart-lung transplant is the only definitive therapy available. Our goal was to show a reconstruction strategy in a patient with a mismatch between the pulmonary artery and the donor aorta.

Evolutionary Description of Heart Transplantation and Heart-Lung Transplantation in Congenital Heart Disease

Currently, a high percentage of patients with congenital heart disease (CHD) reach adulthood. The consequence is that more and more patients will require a heart transplant (HTx) or heart-lung transplant (HLTx). The objective of the study was to analyze the evolution and temporary trend of the number of HTxs and HLTxs in patients with and without CHD. We performed a retrospective analysis of all HTxs and HTLxs from a Spanish transplant hospital. Retransplant and other combined transplants were excluded. HTx and HLTx were divided into 2 groups (CHD or non-CHD). The number of procedures of each modality was grouped in 5 years. A total of 930 HTxs were analyzed between 1987 and 2020; 36 were CHD (18 HTxs and 18 HLTxs). HTx and HLTx in CHD showed a growing progressive trend, probably because of the greater number of these patients who reach adulthood and finally develop advanced heart failure. HTx in patients without CHD showed a very high rise in the first decade, reaching the maximum peak around the year 2000, with a poststabilization trend or even progressive reduction in the number of procedures. HLTx in patients without CHD showed a marked ascent during the first decade with a peak around 2005 and subsequent significant decline in recent years practically in disuse, probably because of the possibility of circulatory assistance in the case of right ventricular failure. The number of HTxs and HLTxs in CHD has a progressive rise. The number of HTx in patients without CHD remains relatively stable. HLTx in patients without CHD shows a marked decrease.

Evolving Indications for Heart-Lung Transplant in Spain

The outcomes of heart-lung transplant (HLT) are worse than those of heart transplant (HT) and lung transplant alone; this and the availability of mechanical assistance have meant that the indications for HLT have been changing. This study aims to analyze the evolution of indications for HLT in a country of 47 million inhabitants. We performed a retrospective observational study of all HLTs performed in Spain (performed in 2 centers) from 1990 to 2020. The total number of patients included was 1751 (HT 1673 and HLT 78). After clinical adjustment, overall survival was compared between the 2 groups. Seven etiological subgroups were considered within the HLT group: (1) cardiomyopathy with pulmonary hypertension (CM+PH);, (2) Eisenmenger syndrome, (3) congenital heart disease without Eisenmenger syndrome, (4) idiopathic pulmonary arterial hypertension (IPAH), (5) cystic fibrosis, (6) chronic obstructive pulmonary disease (COPD) and/or emphysema), and (7) diffuse interstitial lung disease. There were a large number of differences between patients with HLT vs HT. HLT had a 2.69-fold increased probability of death in the first year compared with HT. The indications for HLT have changed over the years. In the recent period the indications are mainly congenital heart disease and Eisenmenger syndrome, with some cases of CM+PH. Other indications for HLT have virtually disappeared, mainly lung diseases (IPAH, COPD, cystic fibrosis). Median survival was low in CM+PH (18 days), diffuse interstitial lung disease (29 days), and ischemic heart disease (114 days); intermediate in Eisenmenger syndrome (600 days); and longer in IPAH, COPD and/or emphysema, and cystic fibrosis. HLT is a procedure with high mortality. This and mechanical assists mean that the indications have changed over the years. Etiological analysis is of utmost interest to take advantage of organs and improve survival.

Comprehensive Review of Pulmonary Hypertension and Treatment Options in the Paediatric Population

Pulmonary hypertension (PH) is a complex condition that can occur as a result of a wide range of disorders, including left heart disease, lung disease, and chronic pulmonary thromboembolism. Multiple improvements have been made in the diagnosis and treatment of pulmonary arterial hypertension (PAH)including a greater understanding of the involvement of extrapulmonary vascular organ systems, validated point of care, clinical assessment tools, and a focus on the initial exposure of numerous pharmacotherapeutics in the appropriate level of care. To achieve a minimal symptom burden, improve the patient's biochemical, hemodynamic, and functional profile, and reduce adverse impact, early diagnosis of PAH is a key objective today. The preferred method of management for thromboembolic PH, which is chronic, is pulmonary endarterectomysince the majority of affected patients are operable. The timing of pulmonary endarterectomy should never be delayed for medical reasons, and risk stratification can enable us to select patients who have a high chance of success. Patients who are not qualified for endarterectomy should be referred for drug trials. Even though there are more effective ways to guarantee a sufficient, long-lasting septostomy, atrial septostomy is promising but undervalued. The procedure's indications remain the sameand needto be taken into account more frequently. Class III or IV patients who are not improving need to be consulted at a transplant centre as soon as possible as they may be candidates for potential recipients of bilateral sequential lung or heart-lung transplants, which is a significant choice for some people. PH is rarely linked to other conditions like connective tissue or thromboembolic disease. It is either idiopathic or linked to congenital heart disease. Infants and children with PH are more frequently recognised in conjunction with a congenital diaphragmatic hernia and developmental lung diseases like bronchopulmonary dysplasia. Although the underlying disease has not yet been treated and advanced structural changes have not yet been reversed, the value of natural life and survival have suggestively increased. Children's haemodynamic and functional outcomes have improved as a result of endothelin receptor antagonists, prostacyclin analogues, and phosphodiesterase type 5 inhibitors, which are examples of targeted pulmonary vasodilator therapies. The health maintenance of paediatric PH is still difficult because treatment decisions are largely based on the findings of adult studies that have been supported by evidence and the clinical expertise of paediatric specialists.

Should there be an age limit for advanced therapies?

Combined heart lung transplant has become a rare procedure. However, there is a significant number of patients potentially benefitting from replacement of both heart and lungs. This represents a quite diverse patient population. Decisions in patient selection have to be adjusted to individual needs and distinct constellation of the patient. Age may be a risk factor, but should be carefully integrated into the evaluation of perioperative and long term risks.

Risk of recipient age on 1-year mortality after simultaneous heart-lung transplantation.

Heart-lung transplantation (HLTx) is relatively uncommon, and there is a paucity of literature to suggest an age at which older recipients may be exposed to excess risk for mortality. This analysis aimed to identify a threshold of age that predicts adverse outcomes after HLTx. The United Network of Organ Sharing registry was used to identify adult patients undergoing HLTx from 2005 to 2021. The primary outcome was 1-year mortality. Threshold regression was used to identify the threshold at which age impacts 1-year mortality. Kaplan-Meier analysis was used to model survival, and Cox proportional hazards modeling was used for risk-adjustment. We identified 453 patients undergoing HLTx. Threshold analysis identified that the risk for 1-year mortality was significantly elevated beyond an age of 58 years, and 47 (10.38%) patients were older than this threshold. On Kaplan-Meier analysis, 1-year survival was significantly lower in patients > 58 years compared to younger recipients (64.7% vs. 82.0%, p = .007). After risk adjustment, the hazard ratio for 1-year mortality in recipients older than 58 years was 2.27 (95% confidence interval [1.21-4.28], p = .011). A threshold for recipient age of 58 years of age may avoid excess 1-year mortality after HLTx. However, patients older than this threshold demonstrate acceptable early and midterm survival, and the majority survive to 1 year. Advanced age should be considered in patient selection for HLTx, but may not be a contraindication for candidacy particularly in the absence of other risk factors.

Fungi between Immunostimulatory and Immunosuppressive

Microbes in general and fungi especially are promising biotechnological tools that are used for green synthesis of numerous products. Fungi in particular are potent producers of many industrially important compounds. β-Glucans are a group of biologically-active fibres or polysaccharides from natural sources with proven medical significance. β-Glucans are known to have antitumor, anti-inflammatory, anti-obesity, anti-allergic, anti-osteoporotic, and immunomodulating activities. On the other hand, cyclosporin is a cyclic undecapeptide with a variety of biological activities including immunosuppressive, antiinflammatory, antifungal, and antiparasitic properties. It is an extremely powerful immunosuppressant and is approved for the use in organ transplantation to prevent graft rejection in kidney, liver, heart, lung, and combined heart–lung transplants. Hence, this review aims to focus on β-glucan as immunostimulatory and Cyclosporine A as Immunosuppressants

Double-lung versus heart-lung transplantation for end-stage cardiopulmonary disease: a systematic review and meta-analysis.

We compared posttransplant outcomes following double-lung transplantation (DLTx) and heart-lung transplantation (HLTx), based on a search of PubMed, Cochrane Library, and Embase, from inception to March 8, 2022, for studies that report outcomes of these procedures. We then performed a meta-analysis of baseline characteristics and posttransplant outcomes. Subgroup analyses were implemented according to indication, publication year, and center. This study was registered on PROSPERO (number CRD42020223493). Ten studies were included in this meta-analysis, involving 1230 DLTx patients and 1022 HLTx patients. The DLTx group was characterized by older donors (P = 0.04) and a longer allograft ischemia time (P < 0.001) than the HLTx group. The two groups had comparable 1-year, 3-year, 5-year, 10-year survival rates (all P > 0.05), with similar results identified in subgroup analyses. We found no significant differences in 1-year, 5-year, and 10-year chronic lung allograft dysfunction (CLAD)-free survival, length of intensive care unit stay and hospital stay, length of postoperative ventilation, in-hospital mortality, or surgical complications between the groups (all P > 0.05). Thus, DLTx provides similar posttransplant survival to HLTx for end-stage cardiopulmonary disease. These two procedures have a comparable risk of CLAD and other posttransplant outcomes.

Severe pediatric pulmonary arterial hypertension: Long-term outcomes of reverse Potts shunt and transplantation

Reverse Potts shunt (RPS) and lung or heart-lung transplantation are life extending surgical interventions for pediatric patients with severe pulmonary arterial hypertension (PAH). Robust criteria for identifying patients who will benefit from these procedures remain elusive. Based on our 30 years of experience, we sought to refine the surgical indications of RPS. This single-center retrospective cohort study included 61 consecutive pediatric patients with PAH managed by RPS (2004–2020) or transplantation (1988–2020). Their long-term outcomes were assessed. Compared to the 20 patients managed by RPS, the 41 transplant waitlist patients, of whom 28 were transplanted, were older (14.9 versus 8.0 years, P = 0.0001) and had worse right-ventricular impairment (tricuspid annular plane systolic excursion, 12.5 mm versus 18.0 mm, P = 0.03). After implementation of a high-priority allocation program in 2007, waitlist mortality decreased from 52.6% to 13.6% ( P = 0.02) and survival raised from 57.1% to 74.7% after RPS and 55.6% to 77.2 after transplantation. At a median follow-up of 8.6 years after RPS and 5.9 years after transplantation, functional capacity had improved significantly, and PAH specific drug requirements had diminished markedly. The cumulative transplantation incidence after RPS was 0% and 25.3% at 5 and 10 years, respectively ( Fig. 1 ). RPS was associated with functional capacity improvements, decreased pharmacotherapy needs, and delayed need for transplantation over the long-term in selected children with suprasystemic PAH, preserved right ventricular function, and suitable anatomy.

Prognostic value of hemodynamic parameters in pulmonary arterial hypertension associated to congenital heart diseases

Limited data are available on the prognostic value of haemodynamic parameters in pulmonary arterial hypertension associated to congenital heart disease (PAH-CHD). The benefit of repeated invasive hemodynamic measurements in PAH-CHD patients remains controversial. The main objective of this study was to assess the prognostic value of haemodynamic variables in PAH-CHD. This bi-centre retrospective study included 109 patients with a median age of 47 [36-38] year-old with PAH-CHD (female, 69%). A multiparametric evaluation was performed, including clinical, hemodynamical, using a robust standardized approach based on Fick principle and oxygen consumption measurement, and cardiac magnetic resonance parameters ( n = 44). Sixty-four (59%) patients had a pre-tricuspid defect. Eisenmenger syndrome was presented in 66 patients (60%). Fifty-three (49%) patients were in WHO/NYHA function class III or IV, heart rate was 80 beats.min [73–88], six-minutes walking-distance was 406 m [355–470] and oxygen saturation at rest was 91% [84–96]. Pulmonary vascular resistance (PVR) was 10 Wood Units (WU) |5–16] and mean pulmonary artery pressure (mPAP) 53 mmHg [41–69]. Eighty-four (77%) patients received PAH-specific drug therapy. Median follow-up was 17 months [10–32]. Primary outcome (death or transplantation) occurred in 29 (26%) (16 deaths, 8 underwent double-lung transplant, two cases with later atrial septal defect percutaneous closure, and 5 underwent heart-lung transplant). In multivariate analysis, right ventricular (RV) failure signs (HR 3.1; CI 95% 1.2–7.7; P = 0.01), heart rate (HR 1.1; CI 95% 1.01–1.1; P < 0.001), tricuspid annular plane excursion (TAPSE) (HR 0.84; IC 95% 0.73–0.96; P = 0.01) and PVR (HR 1.1; CI 95% 1.1–1.2; P < 0.001) remained significant predictors of death or transplantation ( Fig. 1 ). Invasive assessment of haemodynamics in patients with PAH-CHD remains the cornerstone of their prognostic assessment. PVR seems to be a strong and independent predictor of mortality and transplant in PAH-CHD patients.

Long-term outcomes of Transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension

Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge ( P < 0.001) and stayed better at the last visit ( P < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional status across follow-up was significant ( P < 0.001). There was no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The Median follow-up was 62.7 months (IQR, 32.2–96.5). Survival was 100% at 1 year and 92.3% at 5 years. Freedom from reinterventions was 77% at 1 year and 17% at 5 years. Eight (61.5%) patients had stent reinterventions at a median of 22 months (IQR, 8.8–49.8) postoperative. Balloon dilatation and restenting were performed in 46.1% and 38.5% of patients, respectively. Stent malfunctioning was present in 46.2% of patients at the last follow-up. One patient is listed for heart-lung transplantation. Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Five-year survival is however satisfactory.