Fig. 3. Bronchoscopic findings revealed hemorrhagic lesion in left upper segments of inferior lobe.
CONCLUSION AND RESULTS
After stabilization, the patient was discharged on maintenance medications to control PAH e.g., diuretics, and was advised to follow up regularly with her pulmonologist. On a two-week follow-up, the patient remained asymptomatic. Lung examination was clear bilaterally and repeat CXR was consistent with the resolution of the lung opacities.
Discussion
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.