Pregnancy outcomes in pulmonary arterial hypertension in the modern management era

Jaïs, Xavier; Olsson, Karen M.; Barberà, Joan Albert; Blanco, Isabel; Torbicki, Adam; Peacock, Andrew J.; Vizza, Carmine Dario; Macdonald, P.; Humbert, Marc; Hoeper, Marius M.

doi:10.1183/09031936.00141211

Cited by 228 publications

(204 citation statements)

References 33 publications

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“…However, a recent report indicates that the outcome of pregnancies in PAH has improved, at least when PAH is well controlled, and particularly in long-term responders to CCBs [160]. During a 3-year period, the 13 participating centres reported 26 pregnancies.…”

Section: Pregnancy Birth Control and Post-menopausal Hormonal Therapymentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

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Section: Pregnancy Birth Control and Post-menopausal Hormonal Therapymentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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“…Th e physiologic changes induced by pregnancy impose a marked hemodynamic stress in women with IPAH, leading to a previously estimated 30% to 50% mortality rate. 71 , 72 More recent data indicate that the outcome of pregnancy in PAH has improved (a 12% maternal mortality rate was reported in a recent survey 73 ), at least when PAH is well controlled. However, pregnancy remains associated with a substantial mortality risk.…”

Section: Addition Of a Longmentioning

confidence: 99%

Pharmacologic Therapy for Pulmonary Arterial Hypertension in Adults

Taichman

Ornelas

Chung

et al. 2014

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OBJECTIVE:Choices of pharmacologic therapies for pulmonary arterial hypertension (PAH) are ideally guided by high-level evidence. Th e objective of this guideline is to provide clinicians advice regarding pharmacologic therapy for adult patients with PAH as informed by available evidence. METHODS:Th is guideline was based on systematic reviews of English language evidence published between 1990 and November 2013, identified using the MEDLINE and Cochrane Library databases. Th e strength of available evidence was graded using the Grades of Recommendations, Assessment, Development, and Evaluation methodology. Guideline recommendations, or consensus statements when available evidence was insufficient to support recommendations, were developed using a modifi ed Delphi technique to achieve consensus. RESULTS:Available evidence is limited in its ability to support high-level recommendations. Th erefore, we draft ed consensus statements to address many clinical questions regarding pharmacotherapy for patients with PAH. A total of 79 recommendations or consensus statements were adopted and graded.CONCLUSIONS: Clinical decisions regarding pharmacotherapy for PAH should be guided by high-level recommendations when suffi cient evidence is available. Absent higher level evidence, consensus statements based upon available information must be used. Further studies are needed to address the gaps in available knowledge regarding optimal pharmacotherapy for PAH. ABBREVIATIONS : 6MWD 5 6-min walk distance ; AHRQ 5 Agency for Healthcare Research and Quality ; ARIES 5 Ambrisentan in Pulmonary Arterial Hypertension, Randomized Double-Blind, PlaceboControlled, Multicenter, Efficacy Study ; BNP 5 brain natriuretic peptide ; CB 5 consensus-based ; CCB 5 calcium channel blocker ; CO 5 cardiac output ; COI 5 conflict of interest ; CTEPH 5 chronic thromboembolic pulmonary hypertension ; EPC 5 Evidence-Based Practice Center ; ETRA 5 endothelin receptor antagonist ; FC 5 functional class ; FDA 5 US Food and Drug Administration ; GOC 5 Guidelines Oversight Committee ; GRADE 5 Grades of Recommendations, Assessment , Development , and Evaluation ; HR 5 hazard ratio ; IOM 5 Institute of Medicine ; IPAH 5 idiopathic pulmonary arterial hypertension ; mPAP 5 mean pulmonary artery pressure ; PAH 5 pulmonary arterial hypertension ; PDE5 5 phosphodiesterase-5 ; PH 5 pulmonary hypertension ; PVR 5 pulmonary vascular resistance ; RCT 5 randomized controlled trial ; WHO 5 World Health Organization For treatment naive PAH patients with WHOFC I symptoms, we suggest continued monitoring for the development of symptoms that would signal disease progression and warrant the initiation of pharmacotherapy (Grade CB) . 5. We suggest that patients at risk for the development of PAH (eg, patients with systemic sclerosis or the presence of a known mutation placing the patient at risk for PAH) be monitored for the development of symptoms of PAH (Grade CB) . 6. We suggest also that contributing causes of PH (eg, sleep apnea and systemic hypertension) in patients wi...

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“…This was a prospective, multicentre, international, noninterventional survey of surgery or pregnancy in patients with PAH (the results from the pregnancy part of the study have already been published elsewhere [17]). The project was investigator-initiated.…”

Section: Methodsmentioning

confidence: 99%

Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey

et al. 2012

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We conducted an international, prospective, 3-year questionnaire-based survey among 11 pulmonary hypertension centres to assemble data from patients with pulmonary arterial hypertension (PAH) undergoing noncardiac and nonobstetric surgery.Data were collected between July 2007 and June 2010 from 114 patients with PAH (70% female, mean age 57 years) who underwent major surgery. At the time of surgery, 43% were in functional class III/IV. 82% of the interventions were performed under general anaesthesia and 18% under spinal anaesthesia. Major complications occurred in seven (6.1%) of the patients, of whom four died, resulting in an overall perioperative mortality rate of 3.5%. The mortality rate was 15% (two out of 13) in emergency procedures, compared with 2% (two out of 101) in nonemergency procedures (p50.01). Risk factors for major complications were an elevated right atrial pressure (OR 1.1, 95% CI 1.0-1.3; p50.01), a 6-min walking distance ,399 m at the last preoperative assessment (OR 2.2, 95% CI 1.1-3.7; p50.04), the perioperative use of vasopressors (OR 1.5, 95% CI 1.2-2.7; p50.03) and the need for emergency surgery (OR 2.4, 95% CI 1.4-3.6; p50.01).Major surgery in patients with PAH continues to be a high-risk procedure, particularly when emergency interventions are needed.

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Pregnancy outcomes in pulmonary arterial hypertension in the modern management era

Cited by 228 publications

References 33 publications

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Pharmacologic Therapy for Pulmonary Arterial Hypertension in Adults

Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey

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