ABSTRACT
Chronic thromboembolic pulmonary hypertension(CTEPH)is a complication of pulmonary embolism. It is emphasized that the need for a multidisciplinary team involving diagnosis and treatment. The understanding and management of CTEPH have been updated on the 6 th World Symposium on Pulmonary Hypertension(WSPH)in 2018. Key diagnostic steps in early identification and accurate diagnosis are emphasised. An updated treatment algorithm is proposed. Combined with clinical practice, the article will give an interpretation of diagnosis and treatment of CTEPH based on this consensus opinions.
ABSTRACT
<p><b>BACKGROUND</b>Inoperable chronic thromboembolic pulmonary hypertension (CTEPH) is a severe clinical syndrome characterized by right cardiac failure and possibly subsequent liver dysfunction. However, whether serum markers of liver dysfunction can predict prognosis in inoperable CTEPH patients has not been determined. Our study aimed to evaluate the potential role of liver function markers (such as serum levels of transaminase, bilirubin, and gamma-glutamyl transpeptidase [GGT]) combined with 6-min walk test in the prediction of prognosis in patients with inoperable CTEPH.</p><p><b>METHODS</b>From June 2005 to May 2013, 77 consecutive patients with inoperable CTEPH without confounding co-morbidities were recruited for this prospective cohort study. Baseline clinical characteristics and 6-min walk distance (6MWD) results were collected. Serum biomarkers of liver function, including levels of aspartate aminotransferase, alanine aminotransferase, GGT, uric acid, and serum bilirubin, were also determined at enrollment. All-cause mortality was recorded during the follow-up period.</p><p><b>RESULTS</b>During the follow-up, 22 patients (29%) died. Cox regression analyses demonstrated that increased serum concentration of total bilirubin (hazard ratio [HR] = 7.755, P < 0.001), elevated N-terminal of the prohormone brain natriuretic peptide (HR = 1.001, P = 0.001), decreased 6MWD (HR = 0.990, P < 0.001), increased central venous pressure (HR = 1.074, P = 0.040), and higher pulmonary vascular resistance (HR = 1.001, P = 0.018) were associated with an increased risk of mortality. Serum concentrations of total bilirubin (HR = 4.755, P = 0.007) and 6MWD (HR = 0.994, P = 0.017) were independent prognostic predictors for CTEPH patients. Patients with hyperbilirubinemia (≥23.7 μmol/L) had markedly worse survival than those with normobilirubinemia.</p><p><b>CONCLUSION</b>Elevated serum bilirubin and decreased 6MWD are potential predictors for poor prognosis in inoperable CTEPH.</p>
Subject(s)
Aged , Female , Humans , Male , Middle Aged , Antihypertensive Agents , Therapeutic Uses , Bilirubin , Blood , Exercise Test , Hypertension, Pulmonary , Blood , Drug Therapy , Pathology , Prognosis , Prospective StudiesABSTRACT
<p><b>BACKGROUND</b>Pulmonary capillary hemangiomatosis (PCH) is a rare disease and no Chinese case has been reported yet. The disease is often misdiagnosed and its clinical characteristics are incompletely described. The aim of this study was to describe two Chinese cases and to clarify the clinical and radiographic parameters of patients with PCH.</p><p><b>METHODS</b>Two PCH cases were presented and other cases were searched from the English literature. All available clinical and radiographic data were collected from 62 literature reported PCH cases. A pooled analysis of total 64 cases was made.</p><p><b>RESULTS</b>Dyspnea and hemoptysis were the most common clinical symptoms of PCH. Pulmonary hypertension (PH) was found in 78% of the reported cases. PCH typically showed characteristic diffuse or patchy ground-glass opacities (GGOs) and/or multiple ill-defined centrilobular nodules in the computed tomography.</p><p><b>CONCLUSIONS</b>The diagnosis of PCH requires a high clinical suspicion. However, both clinical presentations and radiographic studies often provide clues to the diagnosis, which may prompt early lung biopsy for a definite diagnosis.</p>
Subject(s)
Adolescent , Adult , Aged , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Hemangioma, Capillary , Diagnosis , Lung Neoplasms , Diagnosis , Tomography, X-Ray ComputedABSTRACT
<p><b>BACKGROUND</b>Acute exacerbation of chronic obstructive pulmonary disease (COPD) is always associated with a high incidence and mortality. Because of the presence of some concomitant risk factors such as immobilization, bronchial superinfection, patients who are admitted for acute exacerbations of COPD are generally considered to be at moderate risk for the development of venous thromboembolism. In this study, we investigated the prevalence and the clinical manifestations of deep venous thrombosis (DVT) in patients with acute exacerbation of COPD.</p><p><b>METHODS</b>From March 2007 to March 2009, 520 consecutive patients were included in this study. On admission, color Doppler ultrasound of lower extremities in all cases was performed for diagnosing DVT. Patients with DVT were compared with those without DVT from such aspects as demographics, symptoms, physical signs and risk factors.</p><p><b>RESULTS</b>Among the 520 patients, DVT was found in 46 cases (9.7%). In patients with DVT, the duration of hospitalization was longer (P = 0.01), and the mechanical ventilation requirement increased (P < 0.001). Other indicators for patients with more possibility of DVT were immobility exceeding 3 days (P < 0.001); pneumonia as concomitance (P = 0.01); respiratory failure type II (P = 0.013); current smoking (P = 0.001). Lower extremity pain was more common in DVT cases in comparison to those without DVT (34.8% vs. 15.2%, P = 0.01).</p><p><b>CONCLUSIONS</b>The acute exacerbation of COPD patients, who were immobilized for over 3 days, complicated by pneumonia and had respiratory failure type II, had a higher risk of DVT. In addition, DVT detection awareness should be increased in cases that had a lower extremity pain.</p>