INTRODUCTION:Pulmonary arterial hypertension (PAH) and morbid obesity both dramatically impair functional capacity. New therapies have emerged for both conditions, including pharmaceutical agents for the former and bariatric surgery for the latter. The presence of both conditions simultaneously, however, may limit the use of these therapies.
CASE PRESENTATION:A 54 year old morbidly obese man was diagnosed with idiopathic pulmonary arterial hypertension (PAH) 5 years ago. PAH was suggested by echocardiography and confirmed by right heart catheterization (RHC), demonstrating a pulmonary artery (PA) pressure of 74/24, mean 47 mmHg, normal pulmonary capillary wedge pressure and cardiac output, and a pulmonary vascular resistance of 6.3 Wood Units. Obstructive sleep apnea had been diagnosed years earlier, and continued to be treated successfully with positive airway pressure. The patient was functional class III but repeatedly declined therapy with intravenous epoprostenol. Bosentan and daytime supplemental oxygen were begun 3 and 4 years later, respectively.At the time of PAH diagnosis the patient weighed 145.9 kg and had a body mass index (BMI) of 46.3 kg/m2. Despite weight loss attempts by conventional means, he gained weight and clinically deteriorated. Repeat RHC one year after initiating bosentan demonstrated worsening PAH. The patient again declined epoprostenol, but inquired about bariatric surgery. After evaluation by the bariatric center he was thought a candidate if his hemodynamics could be optimized. To this end, Sildenafil was added with improvement in his hemodynamics. Inhaled iloprost was added two months prior to surgery and RHC 48 hours prior to surgery demonstrated further improvement in hemodynamics. The patient underwent uncomplicated laparoscopic Roux-en-Y gastric bypass. PAH therapies were continued peri-operatively, and he was discharged home on post-operative day 4 with instructions to continue all 3 agents. The patient, however, discontinued iloprost 3 days after discharge, while continuing bosentan and sildenafil. On the day prior to surgery the patient’s weight was 157.3 kg, his BMI was 49.9 kg/m2 and his distance achieved on 6 minute walk test (6MWT) was 257m. Over the first 7 months following surgery, his weight decreased to 106.8 kg and BMI dropped to 33.7 kg/m2 with a dramatic increase in 6MWT distance (to 410m). He is now in functional class II and no longer requires daytime supplemental oxygen.
DISCUSSIONS:We report dramatic improvement following bariatric surgery in a morbidly obese patient with severe PAH. Surgery was performed after his hemodynamics were optimized with aggressive combination PAH therapy. Surgical and PAH clinicians closely collaborated in his peri-operative care. The patient’s postoperative functional improvement as measured by increase in 6MWT distance (+ 153 m), far exceeds the mean values reported in studies of pharmacological therapies for patients with PAH (typically + 30 to + 70 m).
CONCLUSION:Traditionally, “elective” procedures like bariatric surgery were withheld from patients with advanced PAH for fear of excessive peri-operative morbidity. However, the increasing number of PAH therapies may allow for sufficient hemodynamic improvement in these patients to permit such procedures to be performed safely. Furthermore, an argument can be made that bariatric surgery for morbidly obese patients with severe PAH offers the best chance for real functional improvement and, perhaps, extended survival.
DISCLOSURE:Gautam Ramani, No Product/Research Disclosure Information; Grant monies (from industry related sources) Dr. Mathier: Grant support from Actelion, Modest; Shareholder Dr.Cadaret is a (minor) share holder in Pfizer, Johnson and Johnson, and Monicare; Consultant fee, speaker bureau, advisory committee, etc. Dr. Mathier: Consulting fees from Actelion, Gilead and United Therapeutics Dr. Mathier: Speaker bureau for Actelion, Gilead, GlaxoSmithKline and United Therapeutics Dr. Cadaret: Speaker bureau for Actelion