Fig. 3. Bronchoscopic findings revealed hemorrhagic lesion in left upper
segments of inferior lobe.
Hemoptysis is a serious complication of PAH that is rarely reported in
end-stage patients. The incidence of hemoptysis in PAH patients remains
uncertain (7). In 75% of the patients, CT chest shows an evidence of
bronchial artery hypertrophy. The number of dilated bronchial arteries
correlates with the severity of PAH. The presence of hypertrophied
bronchial artery increases the risk of hemoptysis in PAH patients (8).
Diagnosis of pulmonary hypertension can be established by Right Heart
Catheterization (RHC) and revealing elevated mean pulmonary arterial
pressure (mean PAP) ≥25 mmHg at rest. Recommended pharmacologic
treatment for PAH includes vasodilators, prostanoids, nitric oxide,
phosphodiesterase inhibitors, endothelin receptor antagonists, and
anti-coagulants treatment (9). Bronchial artery embolization is
suggested as an immediate emergency procedure in patients with PAH and
severe hemoptysis or as an elective intervention in patients with
frequent episodes of mild-to-moderate hemoptysis. Anticoagulant therapy
in patients with PAH and hemoptysis should be postponed. Interventional
Bronchoscopy and APC are useful tools to control the bleeding. APC is an
electrosurgical technique similar to laser or electrocautery, which is
used during bronchoscopy procedures to ablate malignant airway tumors,
control hemoptysis, remove granulation tissue from stents or
anastomoses, and treat a variety of benign disorders (10).
Our case is the first report of hemoptysis in a patient with PAH and
multiple spleens. Nugraha et al in 2021 reported a 25-year-old woman
with past history of PAH that presented with hemoptysis (400ml) and
shortness of breath. Her examination revealed jugular venous dilatation,
right ventricular heaving, accentuated 2nd heart sound, a grade 3/6
pansystolic murmur with best heard at the left lower sternal border.
Further workups showed elevated PAP at 98 mmHg. A non-reactive O2 test,
observation of hemoptysis suspected to be associated with pulmonary
hypertension, suspected Hospital Acquired Pneumonia (HAP), and
hypokalemia. The treatments included sildenafil, digoxin, furosemide,
iloprost nebulizer, Aspar K, ceftazidime and ciprofloxacin (9).
Our patient had an atypical presentation of hemoptysis and pleuritic
chest pain, and it was challenging to pinpoint the cause bleeding,
especially in the setting of concomitant polysplenia. Hemoptysis is
usually an end-stage presentation of PAH and has a poor prognosis. Given
the severity of this condition, prompt and aggressive management is
crucial to achieve the best outcome. Take away point from our report is
to highlight the importance of bronchoscopy in determining the bleeding
source in patients with hemoptysis and to manage the bleeding in a
timely manner via the APC technique.
AUTHOR CONTRIBUTIONS
MS, AN managed the patient, MS, EP and BD drafted the paper, SB, AN and
MS finalized the paper. All authors read and approved final version of
the paper.
ACKNOWLEDGMENTS
The authors declare that there is no conflict of interest regarding the
publication of this paper.
FUNDING INFORMATION
None.
CONFLICT OF INTEREST STATEMENT
None.
CONSENT
Written informed consent was obtained from the patient to publish this
report in accordance with the journal’s patient consent policy.
DATA AVAILABILITY STATEMENT
The data are not publicly available due to privacy or ethical
restrictions.