Massive hemoptysis in a patient with Eisenmenger syndrome, polysplenia and transverse liverMohsen Shafiepour M.D 1, Behnam Dalfardi M.D2, Ali Nemati M.D 3, Sina Bakhshaei M.D 4, Elahenaz Parsimood M.D 5Assistant Professor of Pulmonology; Afzalipour Hospital Research Center‚ Kerman University of Medical Sciences‚ Kerman‚ Iran.Email: [email protected] Thoracic Research Center, Tehran University of Medical Sciences, Tehran, IranEmail: [email protected] Professor of Hematology and oncology, Afzalipour Hospital Research Center‚ Kerman University of Medical Sciences‚ Kerman‚ Iran.Email: [email protected] medicine resident, UHS SoCal MEC, Temecula, California, USAEmail: [email protected] Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran (Corresponding author)Email: [email protected] : Hemoptysis is defined as blood-streaked sputum from the lower parts of the respiratory tract. Hemoptysis, even in small amounts, is a frightening and an alarm sign for possible underlying conditions such as infections, pulmonary diseases, neoplastic conditions, cardiovascular alterations, vasculitis, traumatic events, hematological derangements, and iatrogenic or drug-induced events. The initial step in the evaluation of hemoptysis is to determine the source of bleeding.Case presentation : Herein, we report an unusual case of massive hemoptysis in a young patient with polysplenia and Pulmonary Artery Hypertension (PAH) in the setting of Eisenmenger syndrome. Chest radiography was suggestive of multiple lung opacities bilaterally. Chest Computed Tomography (CT) revealed a non-heterogeneous mass-like lesion measuring 4 × 5.2 × 5.6 cm in the superior segment of the inferior lobe of the left lung, concerning for accessory spleen. The patient underwent Video bronchoscopy, which showed tracheomalacia and active bleeding in the left main bronchus. The bleeding was controlled by Argon Plasma Coagulation (APC) technique. Broncho-alveolar lavage (BAL) was negative for acid-fast bacilli on staining and on culture. After stabilization, the patient was discharged home on medical management for PAH. On two-week follow-up, imaging revealed resolution of the pulmonary mass-like lesion.Conclusion : Our report highlights the importance of bronchoscopy in determining the bleeding source in patients with hemoptysis and managing it via the APC technique.Keywords: Hemoptysis; Pulmonary Hypertension; Eisenmenger syndrome; Polysplenia; Transverse liverINTRODUCTIONHemoptysis is defined as blood-streaked sputum from the lower parts of the respiratory tract. The initial step in the evaluation of hemoptysis is to determine the source of bleeding. Pseudo hemoptysis, which refers to blood expectoration via a source other than the bronchial or pulmonary system, should be ruled out by history and physical examination (1).Hemoptysis, even in small amounts, is a frightening and alarm sign. The clinical spectrum ranges from minor blood-stained sputum to major bleeding causing respiratory failure and hemodynamic instability. Underlying causes may vary from benign self-limiting conditions to severe and potentially life-threatening conditions. In terms of severity, hemoptysis is considered scant when presents with bleeding <5 mL, mild when <20 mL, and moderate when >20 ml, while massive hemoptysis is defined as bleeding amount of 100 mL/24 hours or more (2).Hemoptysis may derive from multiple underlying conditions, such as infections, pulmonary diseases, neoplastic conditions, cardiovascular alterations, vasculitis, traumatic events, hematological derangements, and iatrogenic or drug-induced events (Table 1) (3).In adults, acute respiratory tract infections (e.g., bronchitis, pneumonia), bronchiectasis, asthma, chronic obstructive pulmonary disease, and malignancy are the most common etiologies. Tuberculosis (TB) is a major cause of hemoptysis in endemic regions and in developing countries (4). However, in industrialized areas, bronchial carcinoma and bronchiectasis are more common culprits.Pulmonary artery hypertension (PAH) is a serious condition causing progressive obstruction and obliteration of the pulmonary vascular bed. PAH is a rare cause of hemoptysis, which is responsible for 0.2- 4% of the cases. Although, hemoptysis is a relatively more common finding in patients with Eisenmenger syndrome (1).In the past, depending on the severity and etiology of hemoptysis, several management strategies were recommended including supportive care, surgical resection, and lung transplant. Currently, the more commonly used strategy is bronchial artery embolization (BAE). In this technique, a particulate material is injected into angiographically identified abnormal bronchial arteries, helping in hemostasis. BAE is usually well tolerated; however recurrent bleeding is commonly associated with the procedure (6).Herein, we report a case of hemoptysis in a patient with Eisenmenger syndrome and polysplenia. We aim to portray the importance of bronchoscopy as both a diagnostic and therapeutic tool, which helped us to identify the source of bleeding and manage it via the BAE technique.CASE DESCRIPTIONHistoryA 34-year-old female with past medical history of Eisenmenger syndrome in the setting of Ventricular Septal Defect (VSD), who presented with 10 days’ history of cough, hemoptysis (about 300 ml/day), pleuritic chest pain, and exertional dyspnea. She did not report any fever, gum bleeding, or epistaxis.ExaminationOn physical examination the patient was tachypneic, tachycardic, and hypoxic with oxygen saturation of 85% in room air. Chest auscultation revealed left upper lobe crackles and holosystolic murmur in the lower sternal border. Also, she was found to have clubbing in her hands bilaterally.METHODS (DIFFERENTIAL DIAGNOSIS, INVESTIGATIONS, AND TREATMENT)Laboratory findings were significant for microcytic anemia (Hemoglobin 10.6 gm/dl), elevated erythrocyte sedimentation rate (ESR) at 23 mm/hr., and negative sputum for both Acid Fast Bacilli (AFB) stain and geneXpert. Chest radiograph revealed non-homogenous opacities in both lungs (figure 1). Computed tomography (CT) Chest showed a non-heterogeneous mass like lesion measuring 4 × 5.2 × 5.6 cm in superior segment of inferior lobe of the left lung (figure 2), in addition to a transversus liver and multiple spleens were noted (Figure 3). Echocardiography was remarkable for mild Pulmonary valve insufficiency, Pulmonary artery pressure (PAP) of 80 mmHg, abnormal septal motion, large membranous VSD, severe right ventricular (RV) enlargement, and left ventricular Ejection fraction (LVEF) of 55%.