Long-Term Response to Calcium Channel Blockers in Idiopathic Pulmonary Arterial Hypertension

Abstract

Background-Characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) who benefit from long-term calcium channel blockers (CCB) are unknown. Methods and Results-Acute pulmonary vasodilator testing with epoprostenol or nitric oxide was performed in 557 IPAH patients. Acute responders, defined by a fall in both mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) Ͼ20%, received long-term oral CCB. Patients who benefit from long-term CCB were defined as those being in New York Heart Association (NYHA) functional class I or II after at least 1 year on CCB monotherapy. Among the 70 patients who displayed acute pulmonary vasoreactivity (12.6%; 95% CI, 9.8% to 15.3%) and received CCB therapy, only 38 showed long-term improvement (6.8%; 95% CI, 4.7% to 8.9%). Long-term CCB responders had less severe disease at baseline than patients who failed. During acute vasodilator testing, long-term CCB responders displayed a more pronounced fall in mean PAP (Ϫ39Ϯ11% versus Ϫ26Ϯ7%; PϽ0.0001), reaching an absolute value of mean PAP lower than that measured in patients who failed (33Ϯ8 versus 46Ϯ10 mm Hg; PϽ0.0001). After 7.0Ϯ4.1 years, all but 1 long-term CCB responders were alive in NYHA class I or II, with a sustained hemodynamic improvement. In the group of patients who failed on CCB, the 5-year survival rate was 48%. Conclusions-Long-term

Pediatric Pulmonary Hypertension

“…In children with IPAH or familial PAH (isolated PVHD), the result is used to define the likelihood of response to long-term November 24, 2015 treatment with CCB therapy and for prognosis. There are 2 definitions of responding to AVT in IPAH or isolated PHVD: (1) a decrease in mPAP of at least 10 mm Hg to <40 mm Hg with a normal or increase in CO [102][103][104] and (2) a decrease in mPAP ≥20%, an increase or no change in cardiac index, and a decrease or no change in PVR/SVR ratio. 1 There is controversy about the frequency of acute responders in pediatrics.…”

“…The current definition for an acute response in an adult PAH patient is defined as a decrease in mPAP >10 to <40 mm Hg with no change or an increase in cardiac output; this definition appears to predict long-term response to CCB therapy in adult IPAH/HPAH. 103 In REVEAL, when the adaptation of the conventional pediatric definition was used, 36 of 102 children (35%) with IPAH/ HPAH were acute responders compared with 9 of 60 patients (15%) with PAH associated with CHD (P=0.006); thus, patients with repaired CHD are less likely to be responsive to AVT than those with IPAH. 1,295 For acute responders with IPAH treated with CCB, survival was 97%, 97%, and 81% at 1, 5, and 10 years, respectively, and sustained treatment success was 84%, 68%, and 47%, respectively.…”

Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome

“…The 6-min walk test (6MWT) and pulmonary function tests were performed according to recommendations [24,25]. RHC was performed as described [26]. Date of diagnosis was defined as the date of RHC, and all data (symptoms, 6MWT, pulmonary function and echocardiography) were obtained at the time of the RHC.…”

Mitomycin-Induced Pulmonary Veno-Occlusive Disease

“…Acute vasodilator testing was performed during right heart catheterization by using inhaled nitric oxide, as previously described. 10 Routine evaluation at baseline included medical history, physical examination, echocardiography, high-resolution CT of the chest, ventilation/perfusion lung scan, abdominal ultrasound, autoimmunity screening, and human immunodeficiency virus (HIV) serology. Age at diagnosis, modified New York Heart Association functional class, and 6-minute walk distance were also recorded.…”