Percutaneous Therapies for Severe Pulmonary Hypertension
Percutaneous Therapies for Severe Pulmonary Hypertension
Despite improvements in medical therapy, the overall prognosis of patients with severe pulmonary arterial hypertension remains poor. Heart-lung transplantation or bilateral lung transplantation is the final pathway for a minority. This paper describes both established and novel percutaneous interventional techniques that may palliate or bridge pulmonary hypertension patients to transplantation.
Pulmonary hypertension (PH) is a progressive and irreversible disorder eventually resulting in right ventricular (RV) failure and death. Despite advances in the treatment of PH, the overall prognosis remains poor, with an approximate annual mortality rate of 11.8% in patients with idiopathic pulmonary arterial hypertension (IPAH), and 16.6% in those diagnosed with scleroderma-related PH.
Heart-lung transplantation (HLTx) and bilateral lung transplantation (BLTx) remain the final options for patients with PH remaining in New York Heart Association (NYHA) functional class III/IV despite combination therapy. Patients in NYHA functional class IV or who remain in functional class III despite combination therapy should be referred early to a PH center of excellence or other tertiary center for transplantation assessment. Predictors of survival after lung transplantation include the 6-min walk distance (6MWD) and peak myocardial oxygen consumption, right atrial pressure (RAP) >15 mm Hg, and a cardiac index <2.0 l/min/m.
BLTx is the operation of choice for patients with IPAH and those with severe secondary PH, as single-lung transplant has an increased risk of perioperative allograft edema. In patients with congenital cardiac abnormalities, particularly Eisenmenger syndrome, and severe right and/or left heart dysfunction, HLTx provides survival advantages and may be considered the procedure of choice. In other etiologies of PAH, the choice of either HLTx or BLTx depends on individual center choice and policy and donor availability. Overall survival rates of BLTx and HLTx are similar; however, freedom from obliterative bronchiolitis–related death has been reported to be significantly greater in the latter. After transplantation, there is an immediate decrease in pulmonary artery pressure (PAP) and RV size and normalization of septal geometry. Unadjusted 3-month mortality is highest in patients with PH pre-transplantation. However, among those surviving at least 1 year, better conditional half-lives after transplantation occur in patients with PAH (10 years) compared with patients with other underlying diagnoses such as chronic obstructive pulmonary disease or idiopathic pulmonary fibrosis (6.8 years for both). Heart-lung transplant recipients with Eisenmenger syndrome and IPAH have significantly better overall survival than patients with other congenital abnormalities. After 1-year survival post-transplantation, long-term survival rates are relatively good, and ~50% of patients remain alive >9 years after transplantation.
Unfortunately, lung transplantation is only a viable option in approximately one-third of patients with PAH referred for lung transplantation. This paper describes both established and novel percutaneous interventional techniques that may palliate symptoms or serve as a bridge to transplantation in selected patients with PH.
Abstract and Introduction
Abstract
Despite improvements in medical therapy, the overall prognosis of patients with severe pulmonary arterial hypertension remains poor. Heart-lung transplantation or bilateral lung transplantation is the final pathway for a minority. This paper describes both established and novel percutaneous interventional techniques that may palliate or bridge pulmonary hypertension patients to transplantation.
Introduction
Pulmonary hypertension (PH) is a progressive and irreversible disorder eventually resulting in right ventricular (RV) failure and death. Despite advances in the treatment of PH, the overall prognosis remains poor, with an approximate annual mortality rate of 11.8% in patients with idiopathic pulmonary arterial hypertension (IPAH), and 16.6% in those diagnosed with scleroderma-related PH.
Heart-lung transplantation (HLTx) and bilateral lung transplantation (BLTx) remain the final options for patients with PH remaining in New York Heart Association (NYHA) functional class III/IV despite combination therapy. Patients in NYHA functional class IV or who remain in functional class III despite combination therapy should be referred early to a PH center of excellence or other tertiary center for transplantation assessment. Predictors of survival after lung transplantation include the 6-min walk distance (6MWD) and peak myocardial oxygen consumption, right atrial pressure (RAP) >15 mm Hg, and a cardiac index <2.0 l/min/m.
BLTx is the operation of choice for patients with IPAH and those with severe secondary PH, as single-lung transplant has an increased risk of perioperative allograft edema. In patients with congenital cardiac abnormalities, particularly Eisenmenger syndrome, and severe right and/or left heart dysfunction, HLTx provides survival advantages and may be considered the procedure of choice. In other etiologies of PAH, the choice of either HLTx or BLTx depends on individual center choice and policy and donor availability. Overall survival rates of BLTx and HLTx are similar; however, freedom from obliterative bronchiolitis–related death has been reported to be significantly greater in the latter. After transplantation, there is an immediate decrease in pulmonary artery pressure (PAP) and RV size and normalization of septal geometry. Unadjusted 3-month mortality is highest in patients with PH pre-transplantation. However, among those surviving at least 1 year, better conditional half-lives after transplantation occur in patients with PAH (10 years) compared with patients with other underlying diagnoses such as chronic obstructive pulmonary disease or idiopathic pulmonary fibrosis (6.8 years for both). Heart-lung transplant recipients with Eisenmenger syndrome and IPAH have significantly better overall survival than patients with other congenital abnormalities. After 1-year survival post-transplantation, long-term survival rates are relatively good, and ~50% of patients remain alive >9 years after transplantation.
Unfortunately, lung transplantation is only a viable option in approximately one-third of patients with PAH referred for lung transplantation. This paper describes both established and novel percutaneous interventional techniques that may palliate symptoms or serve as a bridge to transplantation in selected patients with PH.
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