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.

scholarly journals Occult catheter rupture causing episodic symptoms in a patient treated with epoprostenol

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321774805 ◽  
Author(s):  
Barbara L. LeVarge ◽  
Anica C. Law ◽  
Blanche Murphy
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Long Term Outcomes ◽  
Pulmonary Arterial

Infection, thrombosis, and catheter dislodgment are well-recognized potential complications of chronic intravenous prostanoid therapy for pulmonary arterial hypertension. As long-term outcomes of pulmonary hypertension patients improve, novel adverse events are likely to arise. We describe the sudden development of unexplained hypotension and lightheadedness in a patient receiving intravenous epoprostenol for several years, ultimately determined to be due to an unusual catheter complication, not previously described in this population.

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.

scholarly journals Challenges in the Patient With Pulmonary Hypertension and Atrial Septal Defect: Understanding When and How to Close the Defect

2019 ◽  
Vol 18 (1) ◽  
pp. 10-13 ◽  
Author(s):  
Mary P. Mullen
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Defects ◽  
Defect Closure ◽  
Long Term Outcomes ◽  
Septal Defects ◽  
Pulmonary Arterial

Atrial septal defects (ASDs) are common congenital heart defects in children and adults. Pulmonary arterial hypertension (PAH) is found in subsets of both pediatric and adult patients with atrial defects under varied clinical contexts. The pulmonary hypertension specialist is often faced with questions surrounding timing and method of defect closure, which may have significant impact on procedural and long-term morbidity and survival. This review highlights important differences in management between children and adults with ASDs associated with PAH, highlighting indications for closure, operability, types of closure, and long-term outcomes.

Long-Term Outcomes in Adult Patients With Pulmonary Hypertension After Percutaneous Closure of Atrial Septal Defects

2021 ◽  
Author(s):  
Selai Akseer ◽  
Lusine Abrahamyan ◽  
Douglas S. Lee ◽  
Ella Huszti ◽  
Lukas M. Meier ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Long Term Outcomes ◽  
Septal Defects ◽  
Mean Pulmonary Arterial Pressure ◽  
Cerebrovascular Events ◽  
Pulmonary Arterial ◽  
Asd Closure

Background: Pulmonary hypertension (PH), recently redefined as mean pulmonary arterial pressure >20 mm Hg (PH 20 ), may be observed in patients with atrial septal defects (ASD). We aimed to determine the effect of preprocedural PH 20 status on outcomes among patients undergoing ASD closure. Methods: Study population was selected from a retrospective registry of adult patients who underwent percutaneous ASD closure from 1998 to 2016 from a single center and had right heart catheterizations during the procedure. The clinical registry was linked to administrative databases to capture short- and long-term outcomes. Results: We included a total of 632 ASD closure patients of whom 359 (56.8%) had PH 20 . The mean follow-up length was 7.6±4.6 years. Patients with PH 20 were older (mean age 56.5 versus 43.1 years, P <0.001) and a higher prevalence of comorbidities including hypertension (54.3% versus 21.6%, P <0.001) and diabetes (18.1% versus 5.9%, P <0.001) than those without PH. In a Cox proportional hazards model after covariate adjustment, patients with PH had a significantly higher risk of developing major adverse cardiac and cerebrovascular events (heart failure, stroke, myocardial infarction, or cardiovascular mortality), with hazards ratio 2.45 (95% CI, 1.4–4.4). When applying the prior, mean pulmonary arterial pressure ≥25 mm Hg (PH 25 ) cutoff, a significantly higher hazard of developing major adverse cardiac and cerebrovascular events was observed in PH versus non-PH patients. Conclusions: ASD patients with PH undergoing closure suffer from more comorbidities and worse long-term major adverse cardiac and cerebrovascular events outcomes, compared with patients without PH. The use of the new PH 20 definition potentially dilutes the effect of this serious condition on outcomes in this population.

Temperate approach to ASD closure in pulmonary arterial hypertension: the fenestrated patch technique

2021 ◽  
pp. 1-5
Author(s):  
Ergin Arslanoglu ◽  
Nihat Cine ◽  
Kenan Abdurrahman Kara ◽  
Abdullah Arif Yılmaz ◽  
Fatih Tomrukcu ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Nyha Classification ◽  
Pulmonary Arterial ◽  
Patch Technique ◽  
Asd Closure ◽  
Safe Approach

Abstract The most common CHD in the adult patient population is an atrial septal defect due to its asymptomatic nature in early life. However, when diagnosis and treatment are delayed, pulmonary arterial hypertension may develop as a long-term complication, sometimes before adulthood. The presence of PAH adversely affects the results of surgical treatment and may even eliminate the feasibility of surgery in some patients. In such patients who have high pulmonary artery pressure and pulmonary vascular resistance at the margin of inoperability, the response to the acute vasoreactivity test determines the treatment modality. In our retrospective study, a total of 906 patients who underwent ASD closure between January, 2011 and November, 2020, seven of which had undergone the fenestrated patch procedure after they were identified to have high PAP, but positive AVT test response were included. Short-term follow-up of patients with fenestrated ASD patches revealed decreased pulmonary pressure, regression in NYHA classification, and improvement in symptoms. The fenestrated patch technique should be kept in mind as an option to expand the surgical spectrum in the treatment of patients with high pulmonary pressures, where complete closure is risky. It appears that the fenestrated patch technique is a safe approach in the management of pulmonary hypertension in patients with ASD who have pulmonary hypertension, according to short- and midterm follow-up findings.

scholarly journals Long-term outcomes of pregnant women with pulmonary hypertension diagnosed by echocardiography: a retrospective cohort study in a single center from China

2021 ◽  
Vol 11 (1) ◽  
pp. 204589402096687
Author(s):  
Weisi Lai ◽  
Yiling Ding ◽  
Lieming Wen
Keyword(s):  
Pulmonary Hypertension ◽  
Cohort Study ◽  
Arterial Pressure ◽  
Pregnant Women ◽  
Retrospective Cohort ◽  
Systolic Arterial Pressure ◽  
Severe Pulmonary Hypertension ◽  
Long Term Outcomes

Recent studies suggest that pregnancy may not be absolutely contraindicated in women with moderate pulmonary hypertension. We aimed to evaluate the long-term outcomes of pregnant women with pulmonary hypertension diagnosed by echocardiography in our clinical department. Pregnant women with pulmonary hypertension, diagnosed by a pulmonary systolic arterial pressure > 30 mmHg via echocardiography, who were admitted in our department for termination of pregnancy or delivery between 2004 and 2016 were included in this retrospective cohort study. Demographic characteristics, clinical histories, perinatal outcomes, and follow-up outcomes after discharge were reported. The primary outcome was survival of the pregnant women after discharge. A total of 88 pregnant women with pulmonary hypertension were included in this cohort study. The women were categorized into severe and moderate pulmonary hypertension groups according to their pulmonary systolic arterial pressure at admission. Women with severe pulmonary hypertension were significantly more likely to have deteriorated cardiac function and higher incidence of neonatal complications during the perinatal periods (p < 0.05). During a median follow-up of 26 months, the mortality rate was significantly higher in women with severe pulmonary hypertension (p < 0.05). However, the accumulated survival rate was >90% for women with moderate pulmonary hypertension within the follow-up period. Multivariate Cox regression analyses showed that poor cardiac function before pregnancy, irregular antenatal care, and hyperuricemia were independent mortality risk factors for women with pulmonary hypertension after discharge. In conclusion, the long-term survival of pregnant women with moderate pulmonary hypertension diagnosed by echocardiography was considered acceptable in this cohort. Our findings suggest that pregnancy might not be absolutely contraindicated in women with moderate pulmonary hypertension.

scholarly journals Long-term outcomes of dasatinib-induced pulmonary arterial hypertension: a population-based study

2017 ◽  
Vol 50 (1) ◽  
pp. 1700217 ◽  
Author(s):  
Jason Weatherald ◽  
Marie-Camille Chaumais ◽  
Laurent Savale ◽  
Xavier Jaïs ◽  
Andrei Seferian ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Heart Association ◽  
Functional Class ◽  
Class Iii ◽  
Long Term Outcomes ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial

This study aimed to describe the long-term outcomes of pulmonary arterial hypertension (PAH) induced by dasatinib.21 incident, right heart catheterisation-confirmed cases of dasatinib-induced PAH were identified from the French Pulmonary Hypertension Registry. Clinical and haemodynamic variables were compared from baseline to last follow-up (median (range) 24 (1–81) months).Median age was 52 years and 15 patients were female (71%). 19 patients received dasatinib for chronic myelogenous leukaemia for a median (range) duration of 42 (8–74) months before PAH diagnosis. No bone morphogenic protein receptor-2 (BMPR2) mutations were found in the 10 patients tested. Dasatinib was uniformly discontinued and 11 patients received PAH medications. Four patients died during follow-up. New York Heart Association functional class improved from 76% in class III/IV to 90% in class I/II (p<0.01). Median (range) 6-min walk distance improved from 306 (0–660) to 430 (165–635) m (p<0.01). Median (range) mean pulmonary arterial pressure improved from 45 (30–70) to 26 (17–50) mmHg (p<0.01) and pulmonary vascular resistance from 6.1 (3.2–27.3) to 2.6 (1.2–5.9) Wood units (p<0.01). Patients treated with PAH medications had worse baseline haemodynamics but similar long-term outcomes to untreated patients. PAH persisted in 37% of patients.Dasatinib-induced PAH frequently improves after discontinuation but persisted in over one-third of patients, therefore systematic follow-up is essential.

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