[Clinical features of post-splenectomy pulmonary hypertension and effects of its target therapy].

Objective: To analyze the clinical features of post-splenectomy pulmonary hypertension and effects of its target therapy, and improve the diagnosis and treatment of the disease. Methods: Clinical data of 18 patients with post-splenectomy pulmonary hypertension admitted to Fuwai Hospital from October 2006 to March 2017 were systematically reviewed and analyzed. Results: Among the 18 patients with a mean age of (41±11) years old, 11 were women and 7 were men. The interval between splenectomy and the diagnosis of pulmonary hypertension was (11±6) years. Shortness of breath (14/18) and chest distress (12/18) were the most common clinical manifestations. Nine patients were in World Health Organization functional class (WHO-FC) Ⅲ-Ⅳ, and two patients were combined with pulmonary embolism. According to the cause of splenectomy, the pulmonary hypertension patients were divided into hepatocirrhosis portal hypertension group (10/18) and non-hepatocirrhosis portal hypertension group (8/18). In hepatocirrhosis portal hypertension group, patients were combined with chronic liver abnormalities presentation, and no pulmonary embolism was found in these patients. Cardiac output and cardiac index were significantly higher ( P =0.007 and 0.011, respectively) and pulmonary vascular resistance was significantly lower ( P =0.013) in hepatocirrhosis portal hypertension group as compared with non-hepatocirrhosis portal hypertension group. There was no difference in WHO-FC and mean pulmonary arterial pressure between two groups ( P =0.448 and 0.379, respectively). After 3 months of target treatment in 8 patients, WHO-FC of 3 patients improved, systolic pulmonary artery measured by ultrasonic cardiogram was significantly decreased[(84±10) vs (71±12) mmHg(1 mmHg=0.133 kPa), P =0.005]and right ventricular end diastolic diameter/left ventricular end diastolic diameter was significantly reduced[(0.78±0.17) vs (0.62±0.16), P =0.010]compared to the baseline. The level of NT-proBNP also tended to be decreased, although not to a significant extent. Conclusions: Post splenectomy pulmonary hypertension has various clinical features. Post splenectomy pulmonary hypertension resulted from hepatocirrhosis portal hypertension has better hemodynamics. Target treatment is effective for the patients with post splenectomy pulmonary hypertension.