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.

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.

INDICATIONS

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

CARDIOVASCULAR

One of the most feared complications of complex congenital heart disease is Eisenmenger syndrome, described by the triad of system-to-pulmonary communication, pulmonary artery hypertension (HTN), and cyanosis. This subset of patients may have undergone repair of a congenital heart defect in the past, so it remains crucial to obtain history of any surgeries in the past. Initial management is aimed at decreasing pulmonary vascular resistance, with special attention to avoiding systemic volume depletion, and sudden decreases in systemic vascular resistance. Transplantation is indicated in only severely symptomatic patients, because overall survival with medical management has shown good results.³

Previously, idiopathic pulmonary hypertension (IPAH) used to be the most common indication for HLT.⁶ However, this trend has changed since it has been shown that the RV failure can be reversed after double lung transplant.^7-9 The optimal procedure for patients with IPAH is still controversial because recent single-center data have not shown any significant difference in survival.^10-12

In patients with acquired heart disease, such as valvular pathology or cardiomyopathy, HLT is rarely performed. According to the ISHLT registry, only 5.5% of HLT reported between 1982-2014 had acquired heart disease as the primary indication. In these cases, the indication is fixed pulmonary vascular resistance (described as pulmonary vascular resistance greater than 5 Woods units [320 dynes-s/cm⁵], or transpulmonary gradient >15 to 20 mm Hg). If pulmonary artery pressures can be reduced to acceptable levels with vasodilator challenge while maintaining a systolic systemic blood pressure over 88 mm Hg, an isolated heart transplant may be performed.⁴

PULMONARY

Longstanding lung parenchymal disease can lead to severe RV dysfunction, as well as left ventricular (LV) failure. Conditions such as cystic fibrosis (CF), idiopathic pulmonary fibrosis, as well as chronic obstructive pulmonary disease may be the underlying pathology. As mentioned earlier, in cases where RV failure is reversible, a single or double lung transplant may be performed. The increasing trend toward this practice is also evidenced by the decreasing numbers of HLT performed for CF.

CONTRAINDICATIONS

Absolute and relative contraindications for HLT are similar to those for isolated heart or lung transplantation (Table 42-2).