Massive hemoptysis in a patient with Eisenmenger syndrome,
polysplenia and transverse liver
Mohsen Shafiepour M.D 1, Behnam Dalfardi M.D2, Ali Nemati M.D 3, Sina Bakhshaei
M.D 4, Elahenaz Parsimood M.D 5
Assistant Professor of Pulmonology; Afzalipour Hospital Research
Center‚ Kerman University of Medical Sciences‚ Kerman‚ Iran.
Email: m.shafiepour@kmu.ac.ir
Advanced Thoracic Research Center, Tehran University of Medical
Sciences, Tehran, Iran
Email: B.dalfardi@kmu.ac.ir
Assistant Professor of Hematology and oncology, Afzalipour Hospital
Research Center‚ Kerman University of Medical Sciences‚ Kerman‚ Iran.
Email:
ali_nemati802002@kmu.ac.ir
Internal medicine resident, UHS SoCal MEC, Temecula, California, USA
Email:
sina.bakhshaei@uhsinc.com
cardiovascular Research Center, Institute of Basic and Clinical
Physiology Sciences, Kerman University of Medical Sciences, Kerman,
Iran (Corresponding author)
Email: eliprs5@gmail.com
Abstract
Introduction : 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 liver
INTRODUCTION
Hemoptysis 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 DESCRIPTION
History
A 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.
Examination
On 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%.