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.

Introduction Hemoptysis is an uncommon complication in patients with pulmonary arterial hypertension (PAH). Although the mechanism of hemoptysis is unknown, treatment with bronchial artery embolization (BAE) is proposed as a safe and reliable method of treatment. We report Baylor PH Center experience in treating PAH patients presenting with acute hemoptysis that required multiple BAEs. Results Three female and one male PAH patients ages 45±9 years (mean±SD) presented with acute hemoptysis. Right ventricular systolic pressure and cardiac index at the time of first episode of hemoptysis was 85±17 mm Hg and 2.7±.7 L/min/m2 respectively. Two of the four patients had recurrent episode of hemoptysis requiring multiple BAEs. All four were on intravenous prostacyclin analogue. None were receiving warfarin or endothelin receptor antagonist at the time of the episode. During each episode of hemoptysis INR was 1.09 ±0.11 units and platelet count was 124,000±47,000 per microliter. Each episode of hemoptysis was acutely terminated with BAE. In majority of cases, patients had multiple aberrant bronchial arteries embolized and an average of 2.3 arteries was embolized per session (1–4 embolized arteries). Each BAE was performed utilizing polyvinyl alcohol particles ranging from 250–500 microns. There were no reported complications of the 14 BAE procedures performed. Conclusion Although the incidence of hemoptysis is unknown and likely underreported, we report our experience where recurrent hemoptysis was treated with multiple BAE procedures. This report emphasizes the efficacy and safety of BAE in terminating episodes of recurrent hemoptysis in patients with severe PAH.

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