Heart-lung transplantation for Eisenmenger syndrome: early and long-term results

doi:10.1016/S0003-4975(01)03099-5

The Annals of Thoracic Surgery

Volume 72, Issue 6, December 2001, Pages 1887-1891

https://doi.org/10.1016/S0003-4975(01)03099-5 Get rights and content

Abstract

Background. Heart-lung transplantation (HLT) for Eisenmenger syndrome (ES) provides superior early and intermediate survival when compared with other forms of transplantation. The early risk factors and long-term outcome of HLT for ES are less well defined.

Methods. We analyzed 263 patients who had undergone HLT at our institution during more than 15 years. Fifty-one consecutive patients with ES who underwent HLT, 33 (65%) of which had simple anatomy, were compared with 212 cases having HLT for other indications (non-ES).

Results. Female sex and previous thoracotomy were more prevalent in the ES group. Patients with ES had greater postoperative blood loss and returned more frequently to the operating room for control of bleeding. There were 8 (16%) early deaths in the ES group compared with 27 (13%) in non-ES (p = 0.65). One-, 5-, and 10-year survival rates for ES were 72.6%, 51.3%, and 27.6%, respectively, compared with non-ES of 74.1%, 48.1%, and 26.0%, respectively, and there was no difference in survival overall (p = 0.54). Among ES patients, previous thoracotomy was a risk factor for hospital death. A subgroup analysis based on simple versus complex type of ES did not show statistically significant differences in terms of postoperative course or early or late survival.

Conclusions. Heart-lung transplantation is a successful procedure for ES. Despite a greater frequency of risk factors and a more difficult operative course, early and late outcome with HLT is comparable to non-ES recipients.

Section snippets

Patients and methods

A retrospective study of HLT was performed comparing the outcome of patients with ES with those receiving transplants for other indications. There have been no cases of bilateral-lung transplantation or single-lung transplantation for ES in our institution. The records of all patients who underwent HLT at Papworth Hospital between July 1984 and August 1999 were reviewed. Fifty-one patients with the clinical and operative diagnosis of ES were identified. A group formed by all 212 non-Eisenmenger

Results

The demographic characteristics and operative variables of the two groups are listed in Table 1. The cardiac anatomy of the ES patients is shown in Table 2. Patients with ES had similar age to non-ES at the time of transplantation (34.1 versus 32.8 years, p = 0.47). In the ES group, female sex (69% versus 46%, p = 0.005) and previous cardiothoracic operation (24% versus 11%, p = 0.012) were more prevalent.

There was no statistically significant difference in allograft ischemic times and

Comment

For many decades physicians caring for patients with ES have been frustrated by their inability to offer more than inadequate palliation. Even those cases followed up until recently in centers specializing in congenital heart disease had a life expectancy that rarely exceeded 40 years [1]. With the development of HLT, the outlook for these patients changed significantly, although with time it has been increasingly recognized that the benefits of transplantation for this group of patients are

Acknowledgments

Doctors Stoica and Satchithananda are supported by grants from the Garfield Weston Research Trust.

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Cited by (68)

Prognosis factors in heart–lung transplantation: Thirty years experience in a reference center
2024, Cirugia Espanola
El trasplante cardiopulmonar (TCP) ha presentado una disminución progresiva en el número de procedimientos. En nuestro país existe poca información al respecto, siendo el objetivo de este estudio analizar la experiencia de un hospital de referencia.
Estudio observacional unicéntrico de una cohorte histórica en el periodo entre 1990 y 2021. Las asociaciones entre categorías se evaluaron mediante la prueba de X² o la f de Fisher. La supervivencia se analizó a través del método de Kaplan-Meier. Las diferencias se evaluaron con el estudio de log-rank y el análisis multivariante con el método de Cox.
Se observó una reducción del número de procedimientos realizados en el último decenio (2000-2009: 19 [44,2%]; 2010-2021: 15 [34,8%]). La mortalidad posoperatoria precoz fue de 23,3%, reduciéndose a 13,3% a partir del 2010. La intrahospitalaria fue de 41%, disminuyendo a 33% en 2010. Los factores asociados a la mortalidad fueron cirugía torácica previa, corticoterapia, circulación extracorpórea (CEC) mayor a 200 min, tiempo de isquemia mayor a 300 min y dehiscencia traqueal (p < 0,005). La supervivencia global a uno, cinco y 10 años fue de 58, 44,7 y 36,1%, respectivamente. Los factores asociados a menores tasas de supervivencia fueron cirugía torácica previa, donante masculino, CEC mayor 200 min, tiempo de isquemia mayor a 300 min, dehiscencia traqueal y diferencia de pesos (p < 0,005).
Existe una disminución en el número de procedimientos, siendo más evidente en la última década, pero evidenciando una mejora tanto de la mortalidad posoperatoria y supervivencia.
Heart–lung transplantation has shown a progressive decrease in the number of procedures. There is a lack of information about this field in Spain. The main goal of this study is to analyze the experience of a national reference hospital.
We performed a retrospective study of a historical cohort of heart–lung transplanted patients in a single center, during a 30 years period (from 1990 to 2021). The associations between variables were evaluated using the χ² test or Fisher's exact test. Survival was analyzed using the Kaplan–Meier method. Differences were evaluated using the log-rank test and multivariate analysis with the Cox method.
A decrease in the number of procedures performed in the last decade was observed [2000–2009: 19 procedures (44.2%); 2010–2021: 15 procedures (34.8%)]. Early postoperative mortality was 23.3%, falling to 13.3% from 2010. In-hospital mortality was 41%, falling to 33% from 2010.
Main factors related to higher mortality: previous thoracic surgery, corticosteroid therapy, extracorporeal circulation (ECLS) greater than 200 min, ischemia time greater than 300 min, and tracheal dehiscence (p < 0.005).
Overall survival at one, five, and ten years was 58%, 44.7%, and 36.1%, respectively. Factors associated with lower survival rates: previous thoracic surgery, male donor, extracorporeal circulation greater than 200 min, ischemia time greater than 300 min, tracheal dehiscence and weight difference (p < 0.005).
There has been a progressive decrease in the number of heart–lung transplantations, being more evident in the last decade, but showing an improvement in both mortality and survival.
Pulmonary Hypertension in Adults with Congenital Heart Disease
2022, Cardiology Clinics
Citation Excerpt :
Discussion of the intricacies involved in the decision for lung transplant with congenital defect repair versus dual organ transplant is outside of the scope of this review. Early post-transplant mortality is higher among patients with PAH-CHD compared with other transplant recipients, but after the immediate post-transplant period PAH-CHD patients experience excellent clinical outcomes.56 Palliative care is an important pillar of the management of patients with PAH-CHD and should be considered in patients with symptom burden such that their quality of life is affected.
Waitlist and post-transplant outcomes for eisenmenger syndrome: A comparison of transplant strategies
2021, Journal of Heart and Lung Transplantation
End-stage Eisenmenger syndrome (ES) due to unrepaired atrial septal defect (ASD) or ventricular septal defect (VSD) is an indication for lung transplantation (LTx) or heart-lung transplantation (HLTx). Limited evidence exists as to the optimal transplant strategy for this unique population.
To describe waitlist characteristics and post-transplant outcomes in patients with ES-ASD or ES-VSD.
Using the ISHLT Registry, data were extracted for all ES-ASD or ES-VSD patients who underwent transplantation between 1987 and 2018. Additional data were sought for patients listed for LTx or HLTx in the OPTN Registry during the same period. Early era was defined as 1987-2004, and current era was defined as 2005-2018.
In the current era, patients with ES-ASD or ES-VSD represented a lessening proportion of all LTx and HLTx. Compared to LTx for other indications, the odds of transplantation were significantly less for both ES-ASD 0.18 [0.07-0.50] and ES-VSD 0.03 [0.004-0.22]. In the early era, an equivalent survival was observed for ES-ASD who underwent HLTx versus LTx (p = 0.47), and superior survival for ES-VSD (p = 0.015). In contrast, ES-ASD patients who underwent LTx from the current era displayed better survival compared with HLTx, 10-year survival 52% vs 30% p = 0.036. Similar survival were observed for ES-VSD for both transplant strategies (p = 0.68).
LTx shows superior survival outcomes in the current era for ES ASD patients, and equivalent outcomes for ES-VSD. In the current era, ES-ASD or ES-VSD patients were less likely to be transplanted than other candidates for LTx.
A Review of Heart Transplantation for Adults With Congenital Heart Disease
2021, Journal of Cardiothoracic and Vascular Anesthesia
Citation Excerpt :
Despite higher blood loss and a more frequent return to the operating room for bleeding, early mortality for HLT in ES is similar to that for other indications, with a tendency toward better long-term mortality. New York Heart Association class IV and previous thoracotomy appear to be risks for perioperative death.54,55 The predominant causes of mortality after the perioperative period include infection, heart failure, and bronchiolitis obliterans.52
As techniques for corrective and palliative surgery in congenital heart disease improve, the number of patients surviving to become adults with congenital heart disease (ACHD) has increased. A significant proportion of these patients will progress to develop advanced heart failure, the symptoms of which vary, complicating prediction of life expectancy. Unlike acquired heart failure, there is a lack of evidence-based treatments with which to relieve symptoms and prolong survival. As a result, a number of ACHD patients will proceed to heart transplantation. Referral for transplantation should be considered early, given the difficulties with prognostication, and should take place in a center with surgical and medical expertise in the management of ACHD patients and transplantation. In addition to assessing contraindications to heart transplantation for the general population, factors specific to ACHD should be considered. These include pulmonary hypertension, cyanosis, liver disease, previous surgeries, and the degree of allosensitization. Once listed for transplantation, ACHD patients spend longer on the waitlist, and are more likely to die or be delisted than their non-ACHD counterparts. Mechanical circulatory support is used less commonly as a bridge to transplantation given the difficulties with implantation and unfamiliarity of use. Recent evidence suggests that with increased experience and early consideration, mechanical circulatory support can be used successfully as a bridging therapy. Despite a higher early mortality, long-term survival is greater for ACHD patients after transplant due to younger age and relative lack of comorbidities. With early referral, careful assessment of each individual's unique anatomy and physiology, and care in a center experienced with ACHD patients and transplantation, outcomes will continue to improve.
Not All Septal Defects Are Equal: Outcomes of Bilateral Lung Transplant With Cardiac Defect Repair vs Combined Heart-Lung Transplant in Patients With Eisenmenger Syndrome in the United States
2020, Chest
Thoracic transplantation is considered for patients with Eisenmenger syndrome (ES) who have refractory right ventricular failure despite optimal therapy for pulmonary arterial hypertension. This study compared the outcomes of bilateral lung transplantation (BLT) with cardiac defect repair vs combined heart-lung transplantation (HLT).
This study presents an updated analysis using a US national registry to evaluate the outcomes of patients diagnosed with ES who underwent HLT or BLT with repair of cardiac defects.
This study identified patients with ES who underwent thoracic transplantation from 1987 to 2018 from the United Network for Organ Sharing database. Survival curves were estimated by using the Kaplan-Meier method and were compared by using the log-rank test.
During the study period, 442 adults with ES underwent thoracic transplantation (316 HLTs and 126 BLTs). Following BLT, overall survival 1, 5, and 10 years’ posttransplant was 63.1%, 38.5%, and 30.2%, respectively. Following HLT, overall survival 1, 5, and 10 years’ posttransplant was 68.0%, 47.3%, and 30.5% (P = .6). When survival analysis was stratified according to type of defect, patients with an atrial septal defect had better survival following BLT than following HLT (88.3% vs 63.2% 1 year posttransplant, P < .01; 71.1% vs 49.8% 3 years’ posttransplant, P < .01; and 37.4% vs 29.9% 10 years’ posttransplant, P = .08). Patients with a ventricular septal defect (VSD) exhibited better survival following HLT than following BLT (78.2% vs 49.6% 1 year posttransplant, P < .01; 55.6% vs 34.3% 5 years’ posttransplant, P < .01; and 35.7% vs 26.5% 10 years’ posttransplant, P = .03). The most common cause of mortality in patients with VSD undergoing BLT was cardiac ventricular failure.
This study suggests that the best transplant option for patients with VSD remains HLT, which prevents subsequent development of ventricular failure. BLT with cardiac defect repair should be considered as the first-line treatment option in patients with ES due to an uncorrected atrial septal defect. These patients can be considered to have isolated and reversible right ventricular failure akin to patients with advanced pulmonary arterial hypertension.
Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology
2019, Canadian Journal of Cardiology
Eisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease. A large nonrestrictive systemic left-to-right shunt triggers the development of pulmonary vascular disease, progressive pulmonary arterial hypertension, and increasing pulmonary vascular resistance at the systemic level, which ultimately results in shunt reversal. Herein, we review the changing epidemiological patterns and pathophysiology of Eisenmenger syndrome. Multiorgan disease is an integral manifestation of Eisenmenger syndrome and includes involvement of the cardiac, hematological, neurological, respiratory, gastrointestinal, urinary, immunological, musculoskeletal, and endocrinological systems. Standardized practical guidelines for the assessment, management, risk stratification, and follow-up of this very fragile and vulnerable population are discussed. Multidisciplinary care is the best clinical practice. An approach to the prevention and management of a broad spectrum of complications is provided. Relevant therapeutic questions are discussed, including anticoagulation, noncardiac surgery, physical activity, transplantation, and advanced-care planning (palliative care). Advanced pulmonary arterial hypertension therapies are indicated in patients with Eisenmenger syndrome and World Health Organization functional class II or higher symptoms to improve functional capacity, quality of life, and—less well documented—survival. Specific recommendations regarding monotherapy or combination therapy are provided according to functional class and clinical response. The ultimate challenge for all care providers remains early detection and management of intracardiac and extracardiac shunts, considering that Eisenmenger syndrome is a preventable condition.
Le syndrome d'Eisenmenger est le phénotype le plus sévère et extrême de l'hypertension artérielle pulmonaire associée aux cardiopathies congénitales. Un important shunt gauche-droit systémique non restrictif engendre une maladie vasculaire pulmonaire, l'apparition d'une hypertension artérielle pulmonaire et la majoration des résistances vasculaires pulmonaires; les pressions pulmonaires atteignent un seuil systémique à l'origine du renversement du shunt. Cet article discute des bases physiopathologiques et du changement épidémiologique du syndrome d'Eisenmenger. L'atteinte multiviscérale est caractéristique du syndrome d'Eisenmenger impliquant le système cardio-vasculaire, hématologique, neurologique, respiratoire, gastro-intestinal, urinaire, immunologique, musculo-squelettique et endocrinien. Nous proposons des lignes directrices pratiques pour l’évaluation, la prise en charge, la stratification des risques et le suivi de cette population fragile et vulnérable. Les pratiques cliniques exemplaires recommandent des soins multidisciplinaires. Nous présentons aussi une approche pour la prévention et la prise en charge d'un grand éventail de complications et nous abordons des questions thérapeutiques pertinentes, notamment l'anticoagulothérapie, la chirurgie non cardiaque, l'activité physique, la greffe et la planification préalable des soins (soins palliatifs). Les traitements spécifiques de l'hypertension artérielle pulmonaire sont indiqués chez les patients atteints de syndrome d'Eisenmenger avec une classe fonctionnelle de II ou plus de l'Organisation mondiale de la Santé afin d'améliorer la capacité fonctionnelle, la qualité de vie, et bien que moins documentée la survie. Enfin, nous formulons des recommandations spécifiques concernant le recours à la monothérapie ou à l'association thérapeutique selon la classe fonctionnelle et la réponse clinique. Le diagnostic précoce et la prise en charge optimale des shunts intracardiaques et extracardiaques sont les défis ultimes des équipes traitantes médicales pour prévenir le syndrome d'Eisenmenger.

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Original article: general thoracicHeart-lung transplantation for Eisenmenger syndrome: early and long-term results

Abstract

Section snippets

Patients and methods

Results

Comment

Acknowledgments

Am J Cardiol

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Heart Lung Transplant

J Thorac Cardiovasc Surg

J Heart Lung Transplant

Ann Thorac Surg

Eisenmenger syndrome. Factors relating to deterioration and death

Eur Heart J

Lung or heart-lung transplantation for Eisenmenger’s syndromeanalysis of the ISHLT/UNOS Joint Thoracic Registry

J Heart Lung Transplant

Original article: general thoracic
Heart-lung transplantation for Eisenmenger syndrome: early and long-term results