Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension

Abstract

The etiology of pulmonary arterial hypertension (PAH) is complex, especially the investigation of rare pathogeny is difficult. Congenital portosystemic venous shunt (CPSS) is a rare congenital anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt, and drains directly into the inferior vena cava (IVC) (Howard and Davenport in J Pediatr Surg 32:494–497, 1997).CPSS is an uncommon cause of PAH (Christiane et al. in J Pediatr Gastroenterol Nutr 56:675–681, 2013), and often covered by other pathogenic factors. The clinical manifestations of CPSS-associated PAH are not specific, thus making it difficult to distinguish from PAH caused by other pathogenetic factors based on clinical presentations alone. This is a retrospective analysis of data from six patients with CPSS at a single center. Of these, five were diagnosed as PAH: four were also associated with other predisposing factors of pulmonary hypertension (PH). All patients had high serum bile concentration and high cardiac output. The aim of this retrospective study was to investigate the clinical recognition of PAH secondary to CPSS. The concentration of serum bile acid and cardiac output can be used as two important non-invasive indicators in clinical practice. Thus far, few studies have reported the clinical outcomes of CPSS-associated PAH specifically (Anna et al. in Hepatology 71:658–669, 2020;Franchi-Abella et al. in J Pediatr Gastroenterol Nutr 51:322–330, 2010;Uike et al. in Pediatr Pulmonol 53:505–511, 2018;). In the current study, such patients carried a poor prognosis if left untreated, or treated with pulmonary vasodilators alone.

Prediction of clinical risk assessment and survival in chronic obstructive pulmonary disease with pulmonary hypertension

“…The development of online risk assessment nomogram software will provide a clinical tool for long‐term management of patients with COPD and COPD–PH in primary hospitals or community centres. In addition, in contrast to previous single‐centre studies, 18,23,24 our study included a larger sample of patients with COPD–PH, and used a multicentre study design and internal and external validation. These processes contributed to improving the reliability and generalisability of our research findings.…”

“…These processes contributed to improving the reliability and generalisability of our research findings. Unlike previous studies, 18,23,24 this study adheres to the latest diagnostic criteria from the European Society of Cardiology guidelines. Moreover, we used RHC to show PH in patients with COPD, which is more accurate than other studies that used Doppler echocardiography to diagnose PH 23,24 .…”

Pulmonary hypertension in chronic obstructive pulmonary disease: current understanding, knowledge gaps and future directions

“…Despite the above-mentioned limitations for PH diagnosis, the study identified clear increases in 5-year mortality (32% vs. 13%, P < 0.001) when comorbid PH was identified. In addition, a FVC/DLCO threshold of >0.44 l/mmol/min/kPa was found to predict PH in COPD patients [7]. In a recent study utilizing patients who underwent right heart catheterization (RHC), Kovacs and colleagues aimed to identify predictors of severe PH-COPD, which was defined as a mPAP !35 mmHg.…”

“…It is important to note that most studies have failed to consistently show a relationship between the severity of COPD (i.e., GOLD stage or FEV1) and the severity of PH although most cite increasing incidence of PH as COPD progresses. This discrepancy, and the identification of severe PH in WHO group 3 patients with relatively preserved pulmonary function, was highlighted in recent articles where the term 'pulmonary vascular phenotype' in WHO group 3 PH was described [7][8][9][10]. Thus, there remain considerable uncertainties with regards to the risk factors for development of PH-COPD requiring the clinician to keep a high index of suspicion in the COPD patient with worsening dyspnea.…”

Bile Acid Metabolism Analysis Insight into the Vascular Endothelial Injury in High Salt-Induced Hypertensive Rats

“…Bile acids are the main component of human bile and the nal product of cholesterol after liver action. Bile acids are widely present in plasma and regulate a variety of physiological functions through enterohepatic circulation, such as reducing blood fat, which can prevent atherosclerosis and protect blood vessels [9,10] . Bile salts are sodium or potassium salts formed by the combination of bile acids secreted with sodium or potassium ions.…”