Which of the following diseases is appropriately treated with combined heart-lung transplantation?

Many causes of end-stage lung disease have been appropriately treated with lung transplantation. Whether one lung or both lungs are replaced at the time of transplantation depends on recipient factors. Patients with restrictive processes like primary pulmonary fibrosis do well with a single lung transplant. For patients with primary pulmonary hypertension, unloading of the right ventricle with single lung transplantation has been adequate and replacement of both lungs has not been necessary in most cases. Cystic fibrosis patients do well after lung transplantation but double lung transplant is frequently necessary because of chronic infections. Secondary pulmonary hypertension is due to left ventricular failure with concomitant increases in pulmonary pressures secondary to increases in left ventricular end-diastolic pressures. Reactive secondary pulmonary hypertension is best treated with heart transplantation. Long-standing secondary pulmonary hypertension that is chiefly fixed is best treated with combined heart-lung transplantation.