Hemoptysis in Pulmonary Arterial Hypertension (PAH): A Life-Threatening Complication.

Jaïs, Xavier; Savale, Laurent; Sitbon, Olivier; Montani, David; Price, LC; Ioos, Vincent; Sztrymf, Benjamin; Tchérakian, Colas; Hamid, Am; Humbert, Marc; Simonneau, G

doi:10.1164/ajrccm-conference.2009.179.1_meetingabstracts.a2667

Cited by 5 publications

(9 citation statements)

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“…Mortality was 25% at 30 days for the BAE group and 63% at 30 days for the conservatively managed group. 3 These results were not statistically different and very similar to our findings of a large absolute mortality differences between the two groups that did not meet statistical significance.…”

Section: Discussionsupporting

confidence: 83%

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Clinical implications of haemoptysis in patients with pulmonary arterial hypertension

Cantu¹,

Wang²,

Safdar³

2012

International Journal of Clinical Practice

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Introduction Pulmonary arterial hypertension (PAH) is a disabling disease that may result in haemoptysis. Patients with congenital heart disease associated PAH (CHD-APAH) may have a survival advantage when compared with patients with other types of PAH presenting with haemoptysis. The effects of aetiology and sub-sequent management choice of haemoptysis in PAH patients is not well-defined. Methods We conducted outcome analysis in CHD-APAH vs. all other subtypes of PAH patients presenting with haemoptysis to The Methodist Hospital. Twenty-one patients were identified, thirteen patients in the CHD-APAH group and eight patients in the non-CHD group. We evaluated outcomes related to treatment (bronchial artery embolization vs. conservative management), hospital length of stay, mortality rates and survival in this cohort. Results The CHD-APAH and non-CHD groups had similar baseline demographic, haemodynamic and laboratory values except BMI was higher in the non-CHD group and haematocrit was higher in the CHD-APAH group. Twenty-eight-day mortality (0% vs. 31%) and 1-year mortality (0% vs. 54%) was lower in the CHD-APAH patients as compared with non-CHD group. A statistically significant difference was found in the survival rate in favour of CHD-APAH group for the total follow-up period (p = 0.02). Although not statistically significant, patients treated with BAE had shorter length of stay (4.0 days ± 4.0 vs. 13.7 days ± 22.5; p = 0.26). There was recurrent haemoptysis in 43% of patients treated with BAE. Conclusion Haemoptysis in PAH patients is a serious event with a high mortality rate. CHD-APAH seems to confer a survival advantage, independent of therapy utilised. Termination of haemoptysis with BAE is rapid with relatively few complications except for frequent re-bleeding episodes. Further studies are needed to determine the risk factors that may predispose PAH patients to excessive mortality from haemoptysis and to identify an optimal therapeutic modality.

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Section: Discussionsupporting

confidence: 83%

“…2 Previously published retrospective case reports highlight hemoptysis in PAH patients as a seminal event with excessive mortality. 3, 4 …”

Section: Introductionmentioning

confidence: 99%

Clinical implications of haemoptysis in patients with pulmonary arterial hypertension

Cantu¹,

Wang²,

Safdar³

2012

International Journal of Clinical Practice

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“…97 According to the French national reference center experience, in contrast to PAH associated with congenital heart disease, idiopathic and heritable PAH hemoptysis represents a rare complication, with 20 cases reported over a 10-year period. 98 In chronic thromboembolic pulmonary hypertension, hemoptysis occurs more often and may be recurrent, as a result of dilated and hypertrophied bronchial collateral circulation. 99 This complication can be life-threatening, with cumulative blood volumes averaging 79 mL (20 -300 mL).…”

Section: Hemoptysismentioning

confidence: 99%

“…Survival rates of 60%, 43%, and 36% at 1, 3, and 12 months, respectively, were documented, according to the French national reference center experience. 98 Since many PAH patients are treated with anticoagulants, therapeutic dilemma may ensue in case of concomitant hemoptysis.…”

Section: Hemoptysismentioning

confidence: 99%

Complications Leading to Sudden Cardiac Death in Pulmonary Arterial Hypertension

Demerouti

Manginas²,

Athanassopoulos

et al. 2012

Respir Care

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SummaryPulmonary arterial hypertension (PAH) is a disease of small pulmonary arteries, characterized by vascular proliferation and remodeling. Progressive increase in pulmonary vascular resistance ultimately leads to right ventricular heart failure and death. PAH-specific drug therapy has improved clinical outcomes and survival. While the survival is better, progression of pulmonary vasculopathy contributes to pulmonary artery dilatation. Left main compression syndrome, pulmonary artery dissection, pulmonary artery rupture, and severe hemoptysis are reported as complications leading to sudden cardiac death, an event encountered more often in PAH patients. The advent of PAHtargeted drug therapy has reduced referral for lung transplantation; however, severe complications require rapid diagnosis, decision making, and possible registration on a lung transplantation waiting list. PAH referral centers provide multidisciplinary emergency care and specific therapeutic management, contributing to improved quality of life and survival for PAH patients. We review the complications leading to sudden death in PAH. Key words: pulmonary hypertension; pulmonary artery dissection; pulmonary artery rupture; left main compression syndrome. [Respir Care 2013;58(7):1246 -1254.

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“…According to the French national reference centre experience, haemoptysis is a rare complication in idiopathic and familial PAH, as opposed to PAH associated with congenital heart disease, with 20 cases reported over a 10-yr period [30]. However, it can be life-threatening, with cumulative volumes averaging 79 mL (range 20-300 mL) and survival rates of 60%, 43% and 36% at 1, 3 and 12 months, respectively.…”

Section: Haemoptysismentioning

confidence: 99%

Optimising the management of pulmonary arterial hypertension patients: emergency treatments

Delcroix¹,

Naeije²

2010

Eur Respir Rev

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Pulmonary arterial hypertension (PAH) is a rare and potentially fatal disease whose management is usually restricted to a few specialised centres. As patients do not necessarily live in the neighbourhood of these centres, daily care and emergencies have to be delegated to first and second lines. Treatment guidelines do not usually provide recommendations for acute emergency situations as evidence is scarce. This short review provides a description of our therapeutic protocols based on available data.A model of transmural organisation of care for PAH patients, currently applied in Belgium, is described. Thereafter, based on an analysis of the reasons of death in the PAH population, a review of the main emergencies is provided. Cardiac arrest and resuscitation, decompensated right heart failure, respiratory failure, arrhythmia, pericardial effusion, haemoptysis, surgery and drug-related adverse events will be discussed successively.Case reports showing the precariousness of PAH patients will enforce our thesis of the need for optimal patient management organisation.

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Hemoptysis in Pulmonary Arterial Hypertension (PAH): A Life-Threatening Complication.

Cited by 5 publications

References 0 publications

Clinical implications of haemoptysis in patients with pulmonary arterial hypertension

Clinical implications of haemoptysis in patients with pulmonary arterial hypertension

Complications Leading to Sudden Cardiac Death in Pulmonary Arterial Hypertension

Optimising the management of pulmonary arterial hypertension patients: emergency treatments

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