With the development of effective drug treatment in the last 2 decades, lung transplantation has become the final option in the management of pulmonary arterial hypertension (PAH). Its main advantage is the curative aspect with recovery of cardiopulmonary capacity. Scarcity of donor organs and chronic graft rejection, however, remain serious limitations to short- and long-term success, and emphasize the need for judicial patient selection. Timely presentation of the patient to the transplant center is of critical importance.
Guidelines have been presented by the International Society for Heart and Lung Transplantation (ISHLT), the American Thoracic Society (ATS) and the American Society of Transplant Physicians (ASTP) in the year 1998. Selection criteria are clinical status (NYHA [New York Heart Association] > or = III and progressing, right heart insufficiency symptoms), hemodynamic data (mean pulmonary arterial pressure > 55 mmHg, systolic arterial pressure < 120 mmHg, cardiac index < 2 l/min/m2, central venous pressure > 15 mmHg, right heart function on echocardiography), and functional parameters (peak oxygen uptake < 10-12 ml/kg/min, 6-min walk test [MWT] < 332 m). Functional parameters have been shown to correlate with a 1-year mortality of 40% with a 6-MWT < 332 m, of 50% with a peak oxygen uptake < 10.4 ml/kg/min, and of 70% with a systolic arterial pressure < 120 mmHg. Combinations of the above lead to a 1-year survival of 23%. Mean survival of patients with primary pulmonary hypertension (PPH) is 2.8 years and is reduced to 6 months, when NYHA IV is reached (National Institutes of Health-PPH Registry 2002). These data underline the potential survival benefit from transplantation for patients with advanced lung failure due to PAH. TRANSPLANTATION: Single lung, bilateral lung and heart-lung transplantation are the transplantation procedures available. Even though heart-lung transplantation was the first procedure to be performed in 1981, bilateral lung transplantation is the procedure most commonly performed at present. Choice of transplantation procedure should be made after thorough evaluation, taking the potential reversibility of right ventricular dysfunction after the operation into account (Figure 1). Hemodynamic instability from right and/or left ventricular failure, however, may complicate early postoperative management. Heart and lung transplantation should only be considered, if structural heart damage is present. Lung function improves immediately postoperatively and increases in the following 6 months. Most patients have normal function studies 1 year after transplantation.
The Registry of the ISHLT (01/1982-06/2003) reports PAH as primary diagnosis in 4.2% of all lung transplantations (457/10,959) and 24.3% (550/2,263) of all heart-lung transplantations. This corresponds to 1.1% of all single (66/5,793) and 7.6% of all bilateral lung transplantations (391/5,166). Single lung transplantation is associated with shorter ischemia, cardiopulmonary bypass and operation time. The procedure, however, is accompanied by ventilation/perfusion mismatch, a higher likelihood of reperfusion injury and less functional reserve in case of progressive chronic rejection. The advantages of bilateral lung transplantation are under discussion, particularly as 5-year survival seems to be higher in some centers. Heart and lung transplantation is reported to have similar results as bilateral lung transplantation (Table 1), showing survival advantages only in patients with Eisenmenger's syndrome or ventricular septal defect. Bilateral lung transplantation, therefore, is the procedure of choice at the Essen Transplant Center, provided donor organs are available.
Thoracic transplantation has become a feasible therapeutic option in terminal PAH patients. Judicious patient selection, choice and timing of procedure are critical to a successful outcome.
[Show abstract][Hide abstract] ABSTRACT: Orthotopic heart transplantation (HTX) is nowadays the worldwide accepted gold standard for the treatment of terminal heart failure. The main indications for HTX are non-ischemic dilatative (54%) and ischemic (37%) heart failure. In the acute phase after HTX the survival rate is approximately 90%. Good short and long-term results with survival rates ranging from 81% after 1 year to more than 50% after 11 years demonstrate that there is currently no real treatment alternative to HTX for treatment of end-stage heart failure. In the case of irreversible pulmonary hypertension in combination with end-stage heart failure or complex congenital heart syndromes, a combined heart and lung transplantation (HLTX) is necessary. Compared with HTX the short-term survival of HLTX is reduced, mostly for technical reasons. Improved long-term results after HTX and HLTX are a result of highly specialized transplantation units and effective immunosuppression. However, a major problem is the shortage of organ donors in Germany and the resulting long waiting times for patients with frequently occurring blood groups of up to 10 months for transplantation. The consequence of the latter is the ever increasing number of implanted cardiac assist devices in patients not only as a bridge to transplant but also as destination therapy.
[Show abstract][Hide abstract] ABSTRACT: PAH associated with connective tissue diseases is associated with significant functional impairment and morbidity, and carries with it a poor prognosis. The mortality is as high as 10% to 15% in the first year after diagnosis; making it a devastating disease. The availability of ever-increasing numbers of treatment options in the recent era have improved survival in this patient population and have made early and accurate diagnosis a more important goal. According to the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL), 1-year, 3-year, 5-year, and 7-year survival rates from time of diagnostic right-sided heart catheterization in patients with PAH were found to be 85%, 68%, 57%, and 49%, respectively, which is a considerable improvement since the National Institutes of Health registry 2 decades previously. In a study by Condliffe and colleagues, survival rates in patients with SSC-associated PAH have improved to 78%at 1 year and 47% at 3 years. Patients with SLE-related PAH have a much higher survival rate of up to 75% at 3 years. Proper screening, early diagnosis, and early treatment can have a significant impact in reducing morbidity and mortality. A small study to assess outcomes in patients with asymptomatic CTD found to have exercise induced PAH suggest that bosentan may be safe and effective in improving the hemodynamics and outcomes in these patients. This study included only 10 patients, and additional randomized trials with larger numbers of subjects are needed to affirm this hypothesis. Studies are under way to find additional therapeutic modalities in the form of PDGF receptor blockers, VEGF blockers, tyrosine kinase inhibitors, endothelial dysfunction inhibitors, multikinase inhibitor of Raf-1, serotonin receptor antagonists,and rho kinase inhibitors. Despite these, clinical suspicion, early diagnosis, early
Rheumatic diseases clinics of North America 02/2014; 40(1):103-124. DOI:10.1016/j.rdc.2013.10.001 · 2.69 Impact Factor
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