GENERAL INDICATIONS
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Advanced lung disease with
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Chronic progressive respiratory failure refractory to maximal available medical treatment
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Absence of effective medical therapy for patients with advanced lung disease and poor prognosis
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Poor prognosis with estimated life expectancy of approximately 2 years
ABSOLUTE CONTRAINDICATIONS
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Malignancy in the last 2 years ,
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With the exception of cutaneous squamous and basal cell tumors, at least 2-year disease-free survival after definitive therapy for a malignancy is recommended but a 5-year disease-free interval is prudent, especially in the case of malignant melanoma and breast cancer.
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Advanced dysfunction of another major organ, although combined organ transplantation can be considered on an individual basis
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Systemic inflammatory disease such as sarcoidosis, amyloidosis, or progressive systemic sclerosis with primary lung involvement but also with extensive extrathoracic involvement
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Chronic extrapulmonary infection that cannot be treated and resolved
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Human immunodeficiency virus infection and disease have traditionally been considered to be an absolute contraindication to lung transplantation. However, increasing experience with transplantation of other solid organs in this patient population may render this a relative contraindication.
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Significant chest wall/spinal deformity
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Medical noncompliance
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Uncontrolled psychiatric condition that cannot be treated and resolved
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Lack of stable social support system
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Substance abuse within the last 6 months, including smoking and alcohol abuse
RELATIVE CONTRAINDICATIONS
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Age older than 65 years for single lung transplantation, 60 years of age for bilateral lung transplantation, and 55 years of age for heart-lung transplantation
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Acute critical clinical condition
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Poor functional status without potential for rehabilitation
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Colonization or active infection with resistant or highly virulent microorganisms including Burkholderia cepatia, molds, and atypical mycobacteria
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Obesity, with body mass index (BMI) greater than 30 kg/m .
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Advanced malnutrition with BMI less than 20 kg/m
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Severe or symptomatic osteoporosis.
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Mechanical ventilation for acute respiratory failure, although patients have successfully undergone transplantation surgery who have relatively stable chronic respiratory failure on noninvasive mechanical ventilation or on mechanical ventilation via tracheotomy
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Comorbidities that have not yet resulted in advanced organ dysfunction
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Chronic high-dose steroid therapy usually defined as the equivalent of more than 20 mg of prednisone a day
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Lack of adequate medical insurance coverage
REFERRAL FOR LUNG TRANSPLANTATION
In general, evaluation for lung transplantation is recommended for patients with advanced lung disease when
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2- to 3-year predicted survival is less than 50%
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Level of function at New York Heart Association (NYHA) class III or IV
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There is refractory hypoxemia
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There is development of cor pulmonale
DISEASE-SPECIFIC GUIDELINES
Chronic Obstructive Pulmonary Disease
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Chronic obstructive pulmonary disease (COPD) is currently the most common indication for lung transplantation.
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Very symptomatic COPD patients may have a relatively good prognosis, which makes identifying the appropriate timing for transplantation rather challenging.
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An acute exacerbation with hypercapnia and requiring hospitalization carries a poor prognosis of 49% 2-year survival.
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In general, the goal of thoracic organ transplantation in this patient population is improvement in quality of life rather than prolongation of survival and life expectancy.
When to Refer
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Continued deterioration despite optimal medical and surgical therapy including
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Smoking cessation
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Maximal bronchodilating treatment
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Pulmonary rehabilitation
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Long-term oxygen therapy
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Lung volume reduction, if indicated
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Decline in measures of health-related quality of life: BODE Index-the components include:
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B MI
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Degree of airflow o bstruction (percent predicted FEV )
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Degree of d yspnea (based on modified Medical Research Council dyspnea scale)
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E xercise capacity (based on 6-minute walk test [6-MWT])
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In a prospective study of 625 patients with COPD, a BODE index of 7 to 10 was associated with a median survival of about 3 years, which is less than would be expected after transplantation.
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Patients with a BODE score of 5 to 6 would less likely derive a survival benefit from transplantation but may be candidates for early referral.
When to Transplant
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Patients with a BODE index of 7 to 10 or at least 1 of the following:
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Hospitalization for exacerbation associated with acute hypercapnia (P CO 2 exceeding 50 mm Hg), which suggests a 49% 2-year survival
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Pulmonary hypertension or cor pulmonale, or both, despite oxygen therapy
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FEV 1 of less than 20% and either carbon monoxide diffusing capacity (DLCO) of less than 20% or homogenous distribution of emphysema
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Idiopathic Interstitial Pneumonia
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Idiopathic pulmonary fibrosis (IPF) is the most common of idiopathic interstitial pneumonia and carries the worst prognosis.
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IPF is the second most frequent indication for lung transplantation.
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Median survival time from diagnosis of IPF is 2.5 to 3.5 years.
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Patients with IPF have the highest mortality on the transplant waiting list.
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A subset of patients with fibrotic nonspecific interstitial pneumonia (NSIP) with severe functional impairment at diagnosis or a rapid decline in their functional indices over 6 to 12 months follow-up have a similar poor prognosis to IPF with a dismal 2-year survival, regardless of treatment. ,
When to Refer
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Histologic or radiographic evidence of usual interstitial pneumonia (UIP)
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Histologic evidence of fibrotic NSIP
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Other forms of interstitial lung disease such as desquamative interstitial pneumonia (DIP), chronic hypersensitivity pneumonitis or eosinophilic granulomatosis refractory to medical therapy
When to Transplant
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Histologic or radiographic evidence of UIP and any of the following:
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DLCO less than 39% predicted
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Decline in forced vital capacity (FVC) of 10% or greater during 6 months of follow-up
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Oxygen desaturations to less than 88% during 6-MWT
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Honeycombing on high-resolution computed tomography (HRCT) scan (fibrosis score of >2)
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Development of secondary pulmonary hypertension
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Development of hypercapnia
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Histologic evidence of fibrotic NSIP and any of the following:
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DLCO less than 35% predicted
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Decline in FVC of 10% or greater during 6 months of follow-up
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Decline in DLCO of 15% or greater during 6 months of follow-up
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Development of secondary pulmonary hypertension
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Development of hypercapnia
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Collagen Vascular Disease
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Introduction
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There has been limited experience with collagen vascular disease (CVD)
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By 2004, The International Society for Heart and Lung Transplantation registry had documented only 65 cases.
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Reluctance centered around the concern for an active systemic disease and extrapulmonary involvement that could complicate the post-transplant course.
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General considerations in such cases now include
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Quiescent status of systemic disease
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Failure of state-of-the-art medical therapeutic approaches
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Each candidate needs to be evaluated on an individual basis.
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Scleroderma
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Interstitial lung disease and pulmonary arterial hypertension are the major thoracic complications of scleroderma
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Severe lung disease with or without pulmonary arterial hypertension is a leading cause of death in scleroderma
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Previously, patients with scleroderma have been regarded as suboptimal candidates for lung transplantation.
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More recent data, however, are more encouraging.
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Data on 29 patients with scleroderma who underwent transplantation in the Johns Hopkins and University of Pittsburgh lung transplant programs were reported :
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The 2-year mortality rate for scleroderma patients was 38%, which is similar to that for IPF (33%) and idiopathic pulmonary arterial hypertension (IPAH; 37%)
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Early survival was less in scleroderma patients (69% at 6 months versus 80% and 79% for IPF and IPAH, respectively)
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Early deaths with scleroderma occurred in those with single lung transplants and PAH who developed primary graft dysfunction. Bacterial pneumonia was also a contributor
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In another analysis of 47 patients from 23 US centers, 1- and 3-year survival rates were 68% and 46%, respectively.
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Lung transplantation should be considered a viable option in appropriate candidates with scleroderma
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Pre- and post-transplant challenges include enhanced likelihood of renal dysfunction, hypertension (with risk of crisis), and aspiration from esophageal dysmotility
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Pulmonary Langerhans Cell Histiocytosis (Eosinophilic Granuloma)
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There is a variable natural history with 10% to 15% mortality rate from progressive pulmonary disease and respiratory failure. , Median survival overall is 13 years.
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In addition, pulmonary arterial hypertension, which is often severe and disproportionate to the degree of lung disease or hypoxemia can complicate the disease. The latter is thought to be due to a small vessel vasculopathy
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In patients referred for lung transplantation, mean pulmonary artery pressure was 59 mmHg.
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In a 2005 report, pulmonary Langerhans cell histiocytosis (PLCH) has accounted for only 0.2% of lung transplants.
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Considerations for lung transplantation include
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Progressive respiratory dysfunction: FEV 1 less than 40%; FVC less than 55%, and DLCO less than 40% of predicted.
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Significant PAH
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NYHA functional class III or IV
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Progressive decline in quality of life measures
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Lymphangioleiomyomatosis
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This is a rare disease affecting women in which proliferation of atypical smooth muscle produces thin-walled lung cysts. Angiomyolipomas of the kidney and other intra-abdominal and retroperitoneal structures also occur in 30% to 70% of patients.
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In a 2005 report, lymphangioleiomyomatosis (LAM) has accounted for only 1.1% of lung transplants.
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Considerations for lung transplantation include
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Progressive respiratory dysfunction despite optimal antiestrogen therapy: FEV 1 less than 30%; FVC less than 50%, and total lung capacity (TLC) less than 130% of predicted.
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Severe cystic lung disease and complications
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NYHA functional class III or IV
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Transplant and post-transplant considerations
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Double lung transplant suggested to avoid native lung complications (e.g., pneumothorax, chylothorax)
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Postoperative bleeding from lung explantation in the face of extensive pleural adhesions can pose a serious problem.
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Postoperative chylothorax, which may require thoracic duct ligation, can occur.
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LAM recurrence in transplant allografts reported but rare.
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Cystic Fibrosis
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Cystic fibrosis (CF) is the third most common indication for lung transplantation.
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CF patients are often chronically infected with multidrug-resistant organisms, increasing their potential risks for infection with post-transplant immune suppression.
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Despite increased risk of infection and comorbidities associated with CF, post-transplant survival of patients with CF is similar to or greater than in others undergoing lung transplantation.
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Bilateral lung transplantation is mandatory in patients with CF and others with chronic suppurative lung disease.
When to Refer
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FEV 1 greater than 30% predicted or a rapid decline in FEV 1
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Acute exacerbation requiring stay in intensive care unit
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Increasing frequency of exacerbations and requirement for antibiotic therapy
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Recurrent or refractory pneumothorax
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Recurrent hemoptysis despite embolization treatment
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Development of colonization or infection with increasingly resistant microorganisms
When to Transplant
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Oxygen-dependent respiratory failure
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Hypercapnia
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Pulmonary hypertension
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Decreasing exercise tolerance
Pulmonary Arterial Hypertension
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PAH is a disorder with varying etiologies, induced by increase in pulmonary vascular resistance that progressively worsens, leading to right-sided heart failure.
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Untreated idiopathic PAH carries a reported median survival rate of 2.8 years.
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In general, these patients are managed with bilateral lung transplantation, although successful unilateral lung transplantation has been reported by several centers. Heart-lung transplantation is reserved for those individuals who have very severe right ventricular dysfunction.
When to Refer
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Rapidly progressive disease refractory to medical therapy
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Intolerance to or severe adverse reaction attributed to available medical therapy
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NYHA functional class III or IV
When to Transplant
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NYHA class III or IV despite maximal medical therapy, including IV epoprostenol or its analogues
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6-MWT of less than 380 meters
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Declining 6-MWT
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Cardiac index of less than 2 L/min/m
Sarcoidosis
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Sarcoidosis currently accounts for 2.6% of indications for adult lung transplantation.
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Patient with sarcoidosis on lung transplant waiting list have a reported 30% to 50% mortality rate. ,
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Sarcoidosis has a variable course that makes finding the appropriate time for transplantation referral relatively difficult.
When to Refer
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NYHA functional class III or IV
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Failure to respond to medical therapy
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Progressive restriction on pulmonary function tests
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Stage III and IV disease, which is less likely to respond to steroid therapy
When to Transplant
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NYHA functional class III or IV and any of the following:
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Hypoxemia at rest
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Pulmonary hypertension
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Right atrial pressure greater than 15 mm Hg.
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