Recurrent Hemoptysis: An Emerging Life-Threatening Complication in Idiopathic Pulmonary Arterial Hypertension

doi:10.1378/chest.10-1134

Chest

Volume 139, Issue 3, March 2011, Pages 690-693

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Hemoptysis is a rarely reported complication of idiopathic pulmonary arterial hypertension (IPAH). We present the case of a 27-year-old woman with IPAH, who suffered from recurrent hemoptysis and who was treated with sitaxsentan and treprostinil and remained stable in World Health Organization functional class II. During several episodes of active hemoptysis, the patient underwent bronchial artery embolization (BAE), always with good immediate results. She developed severe respiratory insufficiency and died of electromechanical dissociation 2 days after another episode, despite effective bleeding control. Recurrent hemoptysis in patients with IPAH emerges as a potential indication for urgent placement on the lung transplant list, independent from the classic prognostic factors of functional class and indices of right-sided ventricular function. Repeated BAE should not be considered as a definitive treatment in patients with pulmonary arterial hypertension with recurrent bleeding, although it may help in bridging patients to lung transplant.

Section snippets

Case Report

A 27-year-old female patient with a several month history of increasing dyspnea on exercise and lower-limb edema was hospitalized in September 2005 after her second preterm childbirth. Pulmonary embolism was suspected, and treatment with low-molecular-weight heparin and a vitamin K antagonist was started. After several days of treatment, the patient experienced massive hemoptysis (defined as 300 mL of blood loss in <24 h), which stopped after administration of vitamin K. The patient was

Discussion

Complications of PAH unrelated to right-sided ventricular failure are becoming more clinically relevant, especially in patients who reach satisfactory hemodynamic goals on contemporary medical therapy and therefore would be expected to have improved survival.² Hemoptysis is a recognized problem in Eisenmenger syndrome.¹ However, little is known about its prevalence, clinical significance, or optimal management in other subtypes of PAH. In a review of 21 published studies involving 1,844

Conclusions

The management of hemoptysis in patients with PAH not associated with Eisenmenger syndrome poses a real challenge and may require BAE and, in some patients, early listing for lung transplantation. No simple or uniform solution is currently available. Each patient should be treated on a case-by-case basis and within the sphere of the available treatment options and experience of the medical team.

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Cited by (32)

Hypercoagulability in Pulmonary Hypertension
2018, Clinics in Chest Medicine
Citation Excerpt :
Although there are no data to guide this clinical dilemma, the authors’ own practice is to avoid anticoagulation in congenital heart disease APAH. Hemoptysis is also a known complication of patients with IPAH and CTEPH, with prevalence varying from 1% to 6%,72 and it can limit the use of anticoagulation. Because many patients with portopulmonary hypertension have coagulopathy and thrombocytopenia, they have an elevated bleeding risk; anticoagulation is not recommended in these patients, although it has not been studied.
Riociguat for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Results from a phase II long-term extension study
2017, Respiratory Medicine
Citation Excerpt :
The potentially increased risk of respiratory tract bleeding AEs with riociguat is reflected as a warning in the riociguat label [20,21]. Hemoptysis and pulmonary hemorrhage are known complications of pulmonary hypertension [22,23], and the incidence of hemoptysis in the current study was comparable to previous reports. For example, in a previous study of 210 patients with non-Eisenmenger PAH, moderate-to-severe hemoptysis was observed in 12 (6%) patients seen over a period of 10 years [23], while in another study of 79 patients with CTEPH, five (6%) patients suffered from moderate-to-severe hemoptysis requiring medical intervention [24].
Riociguat was well tolerated and improved exercise and functional capacity in patients with pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH) in a 12-week Phase II trial. We present final data from the long-term extension phase of this study.
During this multicenter, open-label, uncontrolled long-term extension study, riociguat dose could be changed at the physician's discretion (range 0.5–2.5 mg three times daily). The primary outcome was long-term safety and tolerability of riociguat; secondary outcomes included 6-minute walking distance, World Health Organization functional class, survival, and clinical worsening-free survival.
Sixty-eight patients (inoperable CTEPH, n = 41; PAH, n = 27) entered the long-term extension. Median treatment duration at the final data cut-off was 77 months. The most common adverse events were nasopharyngitis (57%) and peripheral edema (37%). Three patients (4%) experienced serious adverse events of hemoptysis: two moderate, one severe, none fatal or considered drug-related. At Month 48, 6-minute walking distance increased from baseline by 69 ± 105 m, and World Health Organization functional class improved/stabilized/worsened versus baseline in 50/45/5% of patients. Three-year survival and clinical worsening-free survival were 91% and 49%, respectively (with patients censored if they withdrew without experiencing an event). Starting a new PAH treatment was the most frequent clinical worsening event.
Improvements in exercise and functional capacity were maintained at 4 years in patients remaining on treatment, with no new safety signals identified. These data support riociguat as a long-term treatment option for PAH and inoperable CTEPH.
ClinicalTrials.gov.
NCT00454558.
ESC/ERS 2015 guidelines for the diagnosis and treatment of pulmonary hypertension
2016, Revista Espanola de Cardiologia
Frequency and prognostic significance of hemoptysis in pediatric pulmonary arterial hypertension
2013, American Journal of Cardiology
Citation Excerpt :
Hemoptysis in adult PAH is well known in Eisenmenger syndrome, in which the reported occurrence rate varies from 10% to ≥50% depending on the length of follow-up time in different studies.2,13,14 In contrast, in adults with non-Eisenmenger PAH, hemoptysis has been reported to occur in only 4% to 6% of the patients, but its occurrence is less well-defined in relation to follow-up time.15,16 Data from the present study suggest that the risk for hemoptysis in PAH increases with a longer time since PAH diagnosis also in children and equally in both types of PAH.
Data concerning the prevalence, risk factors, and prognostic significance of hemoptysis in pediatric pulmonary arterial hypertension (PAH) are scarce. A Dutch national cohort of 74 children with either idiopathic or heritable PAH (IPAH/HPAH, n = 43) or PAH associated with congenital heart disease (PAH-CHD, n = 31) were followed from 1993 to 2012. During a median follow-up of 3.5 years (range 0.1 to 19.2), hemoptysis occurred in 13 children (17.6%). The hemoptysis event rate was 9.9 per 100 patient-years, equally divided between IPAH/HPAH and PAH-CHD (p = 0.824). The median age at first hemoptysis was 12.5 years, and the median time since PAH diagnosis to first hemoptysis was 6.1 years. Patients with hemoptysis had longer time since PAH diagnosis (p = 0.001) and more frequently used anticoagulant therapy (p = 0.006). Univariate Cox regression analysis indicated that older age (hazard ratio [HR] 1.15, 95% confidence interval [CI] 1.01 to 1.30, p = 0.031), World Health Organization functional class IV (HR 0.28, 95% CI 0.08 to 0.95, p = 0.042), higher mean pulmonary arterial pressure (HR 1.04, 95% CI 1.00 to 1.07, p = 0.028), and higher indexed pulmonary vascular resistance (HR 1.08, 95% CI 1.02 to 1.15, p = 0.009), all at the time of PAH diagnosis, were associated with increased risk of hemoptysis during follow-up. Ten of 13 patients with hemoptysis died or underwent (heart-) lung transplantation; in 6 patients, this was directly related to hemoptysis. In conclusion, the occurrence of hemoptysis in pediatric IPAH/HPAH and PAH-CHD increases with time since diagnosis, is a serious condition, and is, in case of life-threatening hemoptysis, associated with poor outcome.
Pulmonary artery dilatation correlates with the risk of unexpected death in chronic arterial or thromboembolic pulmonary hypertension
2012, Chest
Citation Excerpt :
However, with growing experience of referral centers, new potentially life-threatening complications are being recognized. Supraventricular arrhythmias,16 recurrent haemoptysis,17 angina due to compression of the main left coronary artery,4 and management of PAH in pregnancy and labor18 exemplify new challenges faced by clinical teams caring for patients with PAH. Moreover, some otherwise clinically stable patients with PH die unexpectedly outside the hospital.
Right ventricular failure does not explain all cases of death in patients with chronic pulmonary hypertension. Searching for alternative explanations, we evaluated the prognostic significance of main pulmonary artery (PA) dilatation in patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH).
A retrospective outcome analysis was made of 264 patients (aged 46 ± 17 years; women, 69%; PAH, 82%) who underwent both CT scan measurement of the PA and right-sided heart catheterization (mean PA pressure, 57.6 ± 16.5 mm Hg) at initial evaluation.
The diameter of the PA ranged from 28 to 120 mm (mean, 39 ± 8.6 mm; median, 38 mm) and was largest in patients with unrepaired congenital defects (42.6 ± 7.6 mm). Pulmonary pulse pressure (P = .04), lower age (P = .03), and duration of symptoms (P < .001) were independently but weakly related to PA diameter. During follow-up (median, 38 months), 99 patients (37%) died. Of these 99 deaths, 73 (74%) were due to heart failure or comorbidities, and 26 (26%) were unexpected deaths (UE-Ds). PA diameter (hazard ratio [HR], 1.06 per 1 mm; 95% CI, 1.03-1.08), heart rate (HR, 1.30 per 10 beats/min; 95% CI, 1.01-1.66), and systolic pulmonary arterial pressure (HR, 1.02 per 1 mm Hg; 95% CI, 1.01-1.04) were the only independent predictors of UE-D and differed from the usual predictors found in the study group for all-cause mortality. PA diameter ≥ 48 mm had 95% specificity and 39% sensitivity and carried 7.5 times higher risk of UE-D (95% CI, 3.4-16.5; P < .0001) during follow-up.
PA dilatation emerges as an independent risk factor for death unexplained by right ventricular failure or comorbidities in patients with PAH and CTEPH. The possible mechanisms include, but are not limited to, PA compression of the left main coronary artery, PA rupture, or dissection with cardiac tamponade.
Medical Emergencies in Pulmonary Hypertension
2023, Seminars in Respiratory and Critical Care Medicine

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Selected ReportsRecurrent Hemoptysis: An Emerging Life-Threatening Complication in Idiopathic Pulmonary Arterial Hypertension

Section snippets

Case Report

Discussion

Conclusions

Chest

Am J Cardiol

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Selected Reports
Recurrent Hemoptysis: An Emerging Life-Threatening Complication in Idiopathic Pulmonary Arterial Hypertension