scholarly journals Targeted therapy in pulmonary veno-occlusive disease: time for a rethink?

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Qin Luo ◽  
Qi Jin ◽  
Zhihui Zhao ◽  
Qing Zhao ◽  
Xue Yu ◽  
...  
Keyword(s):  
Targeted Therapy ◽  
Pulmonary Edema ◽  
Exercise Capacity ◽  
Targeted Therapies ◽  
Occlusive Disease ◽  
Heart Catheterization ◽  
Long Term Outcome ◽  
Pulmonary Arterial

Abstract Background Pulmonary veno-occlusive disease (PVOD) is a rare condition with poor prognosis, and lung transplantation is recommended as the only curative therapy. The role of pulmonary arterial hypertension targeted therapy in PVOD remains controversial, and long-term effects of targeted therapy have been rarely reported. This study aims to retrospectively evaluate the role of targeted therapy in PVOD patients and the long-term outcome. Methods PVOD patients with good responses to targeted therapies were analyzed, and data pre- and post- targeted therapies were compared. An overview of the effects of targeted therapies on PVOD patients was also conducted. Results Five genetically or histologically confirmed PVOD patients received targeted therapies and showed good responses. Their mean pulmonary arterial pressure by right heart catheterization was 62.0 ± 11.7 mmHg. Two receiving monotherapy got stabilized, and three receiving sequential combination therapy got improved, cardiac function and exercise capacity significantly improved after treatments. No pulmonary edema occurred. The mean time from the first targeted therapy to the last follow up was 39.3 months, and the longest was 9 years. A systematic review regarding the effects of targeted therapies on PVOD patients indicated majorities of patients got hemodynamics or 6-min walk distance improved, and 26.7% patients developed pulmonary edema. The interval from targeted drugs use to death ranged from 71 min to over 4 years. Conclusions Cautious use of targeted therapy could safely and effectively improve or stabilize hemodynamics and exercise capacity of some patients without any complications. PVOD patients could live longer than expected.

Venookklusive Erkrankung: Eine gezielte vasoaktive Therapie als Brücke zur Lungentransplantation

2020 ◽  
Vol 8 (3) ◽  
pp. 142-143
Author(s):  
Simon-Dominik Herkenrath
Keyword(s):  
Targeted Therapy ◽  
Pulmonary Edema ◽  
Exercise Capacity ◽  
Targeted Therapies ◽  
Heart Catheterization ◽  
Long Term Effects ◽  
Long Term Outcome ◽  
Pulmonary Arterial

Background: Pulmonary veno-occlusive disease (PVOD) is a rare condition with poor prognosis, and lung transplantation is recommended as the only curative therapy. The role of pulmonary arterial hypertension targeted therapy in PVOD remains controversial, and long-term effects of targeted therapy have been rarely reported. This study aims to retrospectively evaluate the role of targeted therapy in PVOD patients and the long-term outcome. Methods: PVOD patients with good responses to targeted therapies were analyzed, and data pre- and post-targeted therapies were compared. An overview of the effects of targeted therapies on PVOD patients was also conducted. Results: Five genetically or histologically confirmed PVOD patients received targeted therapies and showed good responses. Their mean pulmonary arterial pressure by right heart catheterization was 62.0 ± 11.7 mmHg. Two receiving monotherapy got stabilized, and three receiving sequential combination therapy got improved, cardiac function and exercise capacity significantly improved after treatments. No pulmonary edema occurred. The mean time from the first targeted therapy to the last follow up was 39.3 months, and the longest was 9 years. A systematic review regarding the effects of targeted therapies on PVOD patients indicated majorities of patients got hemodynamics or 6-min walk distance improved, and 26.7% patients developed pulmonary edema. The interval from targeted drugs use to death ranged from 71 min to over 4 years. Conclusions: Cautious use of targeted therapy could safely and effectively improve or stabilize hemodynamics and exercise capacity of some patients without any complications. PVOD patients could live longer than expected.

scholarly journals Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension

2017 ◽  
Vol 50 (4) ◽  
pp. 1700465 ◽  
Author(s):  
Athénaïs Boucly ◽  
Vincent Cottin ◽  
Hilario Nunes ◽  
Xavier Jaïs ◽  
Abdelatif Tazi ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Targeted Therapy ◽  
Immunosuppressive Therapy ◽  
Exercise Capacity ◽  
Stage Iv ◽  
Long Term Outcomes ◽  
Hypertension Diagnosis ◽  
Pulmonary Arterial

Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited.Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP) >35 mmHg or mPAP 25–35 mmHg with cardiac index <2.5 L·min−1·m−2) were collected from the French Pulmonary Hypertension Registry between 2004 and 2015.Data from 126 patients with severe S-APH were analysed (mean±sd age 57.5±11.6 years, 74% radiological stage IV). 97 patients (77%) received PAH-targeted therapy and immunosuppressive therapy was initiated or escalated in 33 patients at the time of pulmonary hypertension diagnosis. Four months after PAH-targeted therapy initiation, mean±sd pulmonary vascular resistance decreased from 9.7±4.4 to 6.9±3.0 Wood units (p<0.001), without significant improvement in exercise capacity. Among the 11 patients treated only with immunosuppressive therapy, a haemodynamic improvement was observed in four patients, including two with compressive lymph nodes. After a median follow-up of 28 months, 39 patients needed PAH-targeted therapy escalation, nine underwent lung transplantation and 42 had died. Survival at 1, 3 and 5 years was 93%, 74% and 55%, respectively.PAH-targeted therapy improved short-term pulmonary haemodynamics in severe S-APH without change in exercise capacity. Immunosuppressive therapy improved haemodynamics in selected patients. Pulmonary hypertension in sarcoidosis remains associated with a poor prognosis.

Depression: Beyond Description As an Important Comorbidity in Pulmonary Arterial Hypertension—The Next Steps

2012 ◽  
Vol 10 (4) ◽  
pp. 227-232 ◽  
Author(s):  
Deborah H. McCollister ◽  
Philippe Weintraub ◽  
David B. Badesch
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Important Determinant ◽  
Provider Education ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Broad Array ◽  
Pulmonary Arterial

The recent identification of depression as an important comorbidity in pulmonary arterial hypertension (PAH)12 is leading to a broad array of efforts to further refine our understanding of this disorder, enhance patient and provider education about it, and encourage prompt recognition, appropriate diagnosis, and treatment of affected individuals. We will provide an update on the nature and extent of the problem, and describe ongoing and future efforts to address this very important determinant of quality of life and possible long-term outcome in patients with PAH.

The Role of ECT in Schizophrenia

2002 ◽  
Vol 10 (4) ◽  
pp. 385-388 ◽  
Author(s):  
Richard Keuneman ◽  
Rajiv Weerasundera ◽  
David Castle
Keyword(s):  
Acute Onset ◽  
Clinical Settings ◽  
Limited Data ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Affective Symptoms ◽  
Maintenance Ect ◽  
The Subject

Objective: To review the place of electroconvulsive therapy (ECT) in the treatment of schizophrenia. Conclusions: ECT is as effective, if not more so, than the antipsychotic drugs in certain clinical settings. It can be rapidly effective in acute episodes. When used alone, antipsychotics have comparable or superior efficacy to ECT alone in the short term. However, ECT possibly confers better long-term outcome. Combination treatment with antipsychotic medications and ECT is superior to either treatment alone, and is safe and effective, notably in medication resistant schizophrenia. Benefits of acute courses of ECT may be short-lived unless maintenance ECT is instituted, although there are limited data on the subject. Clinically, patients with acute onset, shorter episodes are more likely to respond to ECT. Catatonia, preoccupation with delusions and hallucinations, and a relative absence of premorbid schizoid and paranoid personality traits, are other clinical factors less strongly predictive of positive response. The presence of affective symptoms is often thought to be predictive of clinical response. However, there is little research evidence for this. While medications remain the mainstay of treatment in schizophrenia, ECT does have a clear and increasingly recognised role which requires further evaluation.

scholarly journals Tubercular meningitis with hydrocephalus with HIV co-infection: role of cerebrospinal fluid diversion procedures

2015 ◽  
Vol 122 (5) ◽  
pp. 1087-1095 ◽  
Author(s):  
Raman Mohan Sharma ◽  
Nupur Pruthi ◽  
Arivazhagan Arimappamagan ◽  
Sampath Somanna ◽  
Bhagavathula Indira Devi ◽  
...  
Keyword(s):  
Hiv Positive ◽  
Short Term ◽  
Tubercular Meningitis ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Shunt Placement ◽  
Poor Outcome ◽  
Hiv Negative

OBJECT Hydrocephalus is one of the commonest complications of tubercular meningitis (TBM), and its incidence is increasing with the HIV epidemic. Literature evaluating the role of ventriculoperitoneal shunts in HIV-positive patients with TBM and their long-term prognosis is scarce. METHODS Between June 2002 and October 2012, 30 HIV-positive patients with TBM and hydrocephalus underwent ventriculoperitoneal shunt placement. Thirty age-, sex-, and grade-matched HIV-negative patients with TBM and hydrocephalus were randomly selected as the control group. Outcome was analyzed at discharge (short-term outcome) and at follow-up (long-term outcome). Univariate and multivariate analyses were performed to look for predictors of outcome; p < 0.05 was considered significant. RESULTS There were no differences in the clinical, radiological, or biochemical parameters between the 2 groups. Short-term outcome was better in the HIV-negative group (76.7% improvement) than in the HIV-positive group (70%). However, the long-term outcome in HIV-positive patients was very poor (66.7% mortality and 76.2% poor outcome) compared with HIV-negative patients (30.8% mortality and 34.6% poor outcome). Seropositivity for HIV is an independent predictor of poor outcome both in univariate and multivariate analyses (p = 0.038). However, in contrast to previous reports, of 5 patients with TBM in good Palur grades among the HIV-positive patients, 4 (80%) had good outcome following shunt placement. CONCLUSIONS The authors recommend that shunt treatment should not be performed in HIV-positive patients in poor Palur grade with hydrocephalus. A trial of external ventricular drainage should be undertaken in such patients, and shunt treatment should be performed only if there is any improvement. However, HIV-positive patients in good Palur grades should undergo VP shunt placement, as these patients have better outcomes than previously reported.

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