Lung Transplantation and Atrial Septostomy in Pulmonary Arterial Hypertension

Key points

Atrial septostomy

Atrial septostomy was first described by Rich and Lam⁸ in 1983 in a 22-year-old woman with refractory PAH⁸ based on the suggestion that patients with PH and a patent foramen ovale had better clinical function based on their right-to-left shunt off-loading a failing right heart. The procedure is performed percutaneously either by using a blade-tipped or a balloon-tipped catheter to create a hole in the right atrium.9, 10, 11 Increased systemic oxygen transport from improved cardiac output can

Lung and heart-lung transplantation

The first successful heart-lung transplantation (HLT) was performed in 1981 in a patient with PAH.²³ Lung transplantation and combined HLT remain important surgical options for these patients. Of all HLT procedures performed since 1982, 877 (27.6%) have been performed in patients with PAH, whereas 1064 lung transplant procedures worldwide (3.1% of the total) have been performed for PAH.²⁴ In the United States, patients with PAH made up 6.9% of the waiting list and accounted for 4.4% of lung

Waiting list outcomes in PAH

In the past, waiting list times among different transplant centers have been variable.²⁵ Until May 2005, priority for lung transplantation in the United States was based on the number of days of waiting time accrued since the patient’s placement on the lung transplant waiting list; a candidate who had been on the list for 30 days would have had higher priority than a candidate on the list for 2 days, regardless of clinical acuity.²⁶ In May 2005, the Organ Procurement and Transplantation Network

Outcomes after lung transplantation

Most single-center and registry studies show higher early mortality for patients with PAH undergoing lung transplantation compared with other transplant indications.26, 27 In Franke’s single-center study, survival at 30 days as well as at 1 year was significantly lower for patients with idiopathic PAH compared with patients with other subgroups of PH (ie, Eisenmenger and chronic thromboembolic pulmonary hypertension [CTEPH]).³¹ More recent data from one center’s experience with lung transplant

Type of transplant procedure

Discussion regarding optimal surgical treatment (single lung transplantation [SLT] vs bilateral lung transplantation [BLT] vs HLT) is ongoing and center dependent. In a 2001 review of transplant centers in the United States, Canada, Europe, and Israel, bilateral lung transplantation was preferred in the United States whereas HLT was preferred in Europe and Israel.²⁵ Franke and colleagues,³¹ compared outcomes in 63 patients with IPAH, secondary PH, CTEPH, and Eisenmenger who underwent

Recommendations for timing of referral and listing for lung transplantation

Box 4 presents criteria for referral for lung transplant evaluation and listing in patients with PH as established by the ISHLT in 2006.54, 55 Based on the authors’ clinical experience, we recommend referring patients with PAH for lung transplant evaluation once they meet one or more of the following criteria:

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  Patients receiving parenteral prostanoids regardless of symptoms or NYHA functional class

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  Patients with NYHA functional class III or IV symptoms despite escalating therapy

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  Patients with

Summary

Surgical options for PH to other than CTEPH include atrial septostomy and lung transplantation as well as combined HLT. Atrial septostomy should be considered for suitable patients with significant RV compromise as a palliative measure or bridge to transplantation. Transplant referral should be considered for any patient with high NYHA functional impairment or rapidly progressive disease. Given the high mortality that can be associated with the disease, referral for transplantation should be