A 37-year-old woman is admitted to a hospital with progressive dyspnea on minimal effort, with significant worsening in the past 6 months. She states that she was diagnosed with heart murmur when she was 5 years old, but has had no treatment for it. She describes breathlessness, fatigue, and occasional presyncopal episodes. On examination she has dyspnea on exertion, 3+ bilateral lower extremity edema, ascites, and orthopnea. She has clubbing, tachypnea, mild right upper quadrant tenderness, and some bruising on her extremities. Chest x-ray demonstrates an enlarged pulmonary artery silhouette. Transthoracic echocardiogram demonstrates a large ventricular septal defect with right to left shunt, right ventricular (RV) enlargement, and an elevated estimated RV systolic pressure.
Cardiac catheterization was performed, which showed suprasystemic RV as well as pulmonary artery pressures. Pulmonary vascular resistance was measured at 8 Woods units. There was minimal change in hemodynamics with inhaled oxygen or vasodilator challenge.
The first heart-lung transplantation (HLT) was performed in 1981 at Stanford University, for a 45-year-old woman with pulmonary hypertension.1 According to the International Society for Heart and Lung Transplantation Registry, 3820 heart-lung transplants have been reported through June 2014 (Figure 42-1). After a spike in the late 1980s, the number of transplants performed in the last decade has plateaued to 64 to 65 transplants per year. Heart-lung transplantation remains the only viable option for patients with concomitant end-stage heart and respiratory failure.
Number of adult heart-lung transplants by year (1982-2013). This figure includes only the adult heart-lung transplants that were reported to the International Society for Heart and Lung Transplantation Registry and does not represent the number of adult heart-lung transplants performed worldwide. (Reproduced with permission from Yusen RD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: Thirty-second Official Adult Lung and Heart-Lung Transplantation Report—2015; Focus Theme: Early Graft Failure. J Heart Lung Transplant. 2015;34(10):1264-1277. DOI: 10.1016/j.healun.2015.08.014.)
Management of these patients is complex, and usually involves a multidisciplinary team consisting of, but not limited to, pulmonologists, cardiologists, transplant surgeons, and pharmacists. In this chapter, we will attempt to concisely elucidate the indications, management, and prognosis of this special group of patients.
Combined heart-lung transplantation should be considered for patients with end-stage heart failure (HF), along with end-stage lung disease. On the basis of pathophysiology, these can be divided into 2 major subgroups, with either HF or pulmonary failure as the primary disease process. Table 42-1 summarizes the primary indications for HLT.5
Table 42-1Primary Indications