Lung transplantation in pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2520-2524
Author(s):  
Laurent Savale
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Medical Management ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Right Heart Failure ◽  
Optimal Timing ◽  
Optimal Medical Management ◽  
Pulmonary Arterial

Despite recent advances in medical management, lung and heart–lung transplantation remains an established treatment option in patients with end-stage pulmonary arterial hypertension (PAH) despite optimal medical therapy. The optimal timing of referral and listing patients for transplantation must take into consideration the co-morbidities, the aetiology of PAH, and the regular risk assessment based on a multidimensional approach. With the exception of PAH due to complex congenital heart disease, double-lung transplantation is the preferred option over combined heart–lung transplantation as cardiac function recovers after transplantation in most cases. The use of extracorporeal life support as a bridge to urgent transplantation may be considered in expert centres in selected patients with refractory right heart failure despite optimal medical management in an intensive care unit. With a survival rate at 5 years varying from 50% to 75%, transplantation in PAH can be considered as a life-saving procedure for patients with severe symptoms.

scholarly journals Severe thrombocytopenia in patients with idiopathic pulmonary arterial hypertension provided several strategies for lung transplantation

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096910
Author(s):  
Takayuki Kobayashi ◽  
Ayako Shigeta ◽  
Jiro Terada ◽  
Nobuhiro Tanabe ◽  
Toshihiko Sugiura ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Right Heart Failure ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Right Heart ◽  
Thrombocytopenic Purpura ◽  
Bilateral Lung Transplantation ◽  
Pulmonary Arterial ◽  
Bilateral Lung

While the prognosis of idiopathic pulmonary arterial hypertension has improved significantly due to newer medications, lung transplantation remains a critical therapeutic option for severe pulmonary arterial hypertension. Hence, it is essential for patients awaiting lung transplantation to avoid complications, including thrombocytopenia, which may affect their surgical outcomes. Herein we present the case of a 21-year-old woman diagnosed with idiopathic pulmonary arterial hypertension at the age of 15. She developed thrombocytopenia while awaiting lung transplantation. Her medication was switched from epoprostenol to treprostinil, suspecting possible drug-induced thrombocytopenia. Furthermore, she was administered thrombopoietin receptor agonists in view of the possibility of idiopathic thrombocytopenic purpura, along with maximum support for right heart failure. Subsequently, her platelet count increased to >70,000/µL, enabling her to successfully undergo bilateral lung transplantation. Post-bilateral lung transplantation, pulmonary arterial hypertension as well as thrombocytopenia appeared to have resolved. In this case, we suspected that thrombocytopenia could have resulted owing to a combination of pulmonary arterial hypertension, right heart failure, drug interactions, and idiopathic thrombocytopenic purpura. Thrombocytopenia is a very critical condition in patients with pulmonary arterial hypertension, especially those awaiting lung transplantation. Several approaches are known to improve intractable thrombocytopenia in patients with pulmonary arterial hypertension.

Optimal Timing for Lung Transplantation: Why Not Include RVEF As an Indicator?

2012 ◽  
Vol 11 (1) ◽  
pp. 47-48
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Lung Transplantation ◽  
Severity Of Illness ◽  
Right Atrial ◽  
Optimal Time ◽  
Optimal Timing ◽  
Predictors Of Outcome ◽  
Donor Organ ◽  
Pulmonary Arterial ◽  
Rv Function

One of the most challenging questions to answer in pulmonary arterial hypertension (PAH) is: “When is the optimal time to proceed with lung transplantation?” The current lung allocation scoring (LAS) system prioritizes donor organ resources based on severity of illness. Factors used to assign LAS do not account for known predictors of outcome for PAH patients—including determinants of right ventricular (RV) function. It has been recognized that the system places PAH patients at a distinct disadvantage, and concerted efforts are being made to correct this by considering variables that reflect RV function, specifically mean right atrial pressure (mRAP) and cardiac index (CI).

Navigating the Road to Transplant in Pulmonary Arterial Hypertension: A Road Less Taken

2016 ◽  
Vol 15 (1) ◽  
pp. 12-13
Author(s):  
Adaani E. Frost ◽  
Harrison W. Farber
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Small Cell ◽  
Small Cell Lung ◽  
Donor Organ ◽  
The Road ◽  
Lung Allocation Score ◽  
Pulmonary Arterial

Dramatic advances in therapy for pulmonary arterial hypertension (PAH) in the last 20 years have improved survival from a median of 2.5 years in the pretreatment era to 7.5 years currently. However, impressive as that may seem, it is important to note that a median survival of 7.5 years is equivalent to that of surgically resected non-small cell lung cancer, thus underscoring the importance of lung transplantation as a treatment option in patients with PAH. In this edition of Advances, Edelman has reviewed the pathway to transplantation for patients with PAH, detailing the recommendations for timing of referral, listing for lung transplantation, the role of the lung allocation score in allocating a donor organ, and the outcome of lung transplantation.

scholarly journals Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

scholarly journals Study of the effect of Occlutech Atrial Flow Regulator on symptoms, hemodynamics, and echocardiographic parameters in advanced pulmonary arterial hypertension

2021 ◽  
Vol 11 (1) ◽  
pp. 204589402198996
Author(s):  
Kothandam Sivakumar ◽  
Gopalavilasam R. Rohitraj ◽  
Monica Rajendran ◽  
Nithya Thivianathan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Right Atrial ◽  
Echocardiographic Parameters ◽  
Procedural Complications ◽  
Pulmonary Arterial ◽  
Flow Regulator ◽  
Risk Predictors

Optimal sized balloon atrial septostomy improves hemodynamics in advanced pulmonary arterial hypertension. Occlutech Atrial Flow Regulator is designed to provide an atrial septal fenestration diameter titrated according to the age and right atrial pressures. This observational study analyzed symptoms, exercise distance, oxygen saturations, hemodynamics and echocardiographic parameters after Atrial Flow Regulator implantation in patients with syncope or right-heart failure. Patients with high-risk predictors of mortality during septostomy were scrutinized. Thirty-nine patients (9 children) with syncope (34/39) or right-heart failure (27/39) underwent Atrial Flow Regulator implantation without procedural complications. Six-minute walk distance increased from 310 ± 158.2 to 376.4 ± 182.6 m, none developed syncope. Oxygen saturations reduced from 96.4 ± 6.4% to 92 ± 4.9% at rest and further to 80.3 ± 5.9% on exercise. Right atrial pressures reduced from 9.4 ± 5 (2–27) mmHg to 6.9 ± 2.6 (1–12) mmHg, while cardiac index increased from 2.4 ± 0.8 (0.98–4.3) to 3 ± 1 (1.1–5.3) L/min/m2 and systemic oxygen transport increased from 546.1 ± 157.9 (256.2–910.5) to 637.2 ± 191.1 (301.3–1020.2) ml/min. Echocardiographic improvement included significant reduction of pericardial effusion and inferior caval congestion at a median follow-up of 37 months. Overall survival improved except two early and one late deaths in high-risk patients. Five of seven patients with advanced disease and key hemodynamic predictors of mortality survived. Acute hemodynamic benefits in pulmonary arterial hypertension after Atrial Flow Regulator were improved cardiac output, systemic oxygen transport, and reduced right atrial pressures. Improvement of symptoms especially syncope, exercise duration, and right ventricular systolic function as well as device patency were sustained on mid-term follow-up. Implantation was safe in all including young children without procedural complications. Mortality was noted only in patients who had high-risk predictors and patients at advanced stage of the disease.

Sign in / Sign up

Export Citation Format

Share Document