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A 29-Year-Old Woman With Severe Sepsis and Hemoptysis FREE TO VIEW

Allison Aripoli, MD; Lucas Meek, MD; Steven Lemons, MD; Leslie Spikes, MD
Author and Funding Information

aDepartment of Radiology, University of Kansas Medical Center, Kansas City, KS

bDepartment of Pulmonary and Critical Care Medicine, University of Kansas Medical Center, Kansas City, KS

CORRESPONDENCE TO: Allison Aripoli, MD, University of Kansas Medical Center, Department of Radiology, 3901 Rainbow Blvd, Mail Stop 4032, Kansas City, KS 66160


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(2):e53-e57. doi:10.1016/j.chest.2016.02.681
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Published online

A 29-year-old woman presented with a 1-week history of fever, weakness, anorexia, darkened urine, and mild cough. The patient described her cough as nonproductive and without hemoptysis. She had no chest pain. The patient’s medical history was significant for x-linked hypophosphatemia, renal stones, migraine headaches, and chronic back pain managed on prescribed oral opiates for some time. She reported regular cigarette smoking, but denied illicit or IV drug use or any recent travel or sick contacts. The patient also had no known pertinent family history.

Figures in this Article

On arrival, the patient was febrile with a temperature of 40.9°C and heart rate of 159 beats/min, systolic BP 70 mm Hg, respiratory rate 20 breaths/min, and oxygen saturation 95% on room air. She was alert and oriented, and appeared fatigued but in no distress. Pulmonary examination was pertinent for bilateral rales; and cardiac examination was significant for tachycardia with regular rate, no appreciable murmur. Bilateral upper extremity ecchymosis was present. There was no clubbing, cyanosis, or edema, and no rashes or nail lesions.

CBC revealed leukocytosis, anemia, and thrombocytopenia. Hemoglobin level was 8.5 g/dL (normal, 12.0-15.0 g/dL), WBC count was 19,600 cells/μL (normal, 4,500-11,000 cells/μL), and platelets were 69,000/μL (normal, 150,000-400,000/μL). BUN and creatinine were normal. Procalcitonin was 46.09 ng/mL (normal, < 0.10 ng/mL) and troponin was 0.04 ng/mL (normal). Urine drug screen was positive for amphetamines, benzodiazepines, and opiates.

Admission contrast-enhanced CT of the chest, abdomen, and pelvis showed multiple bilateral pulmonary nodular opacities, some with central cavitation, and small pleural effusions (Fig 1).

Figure 1
Figure Jump LinkFigure 1 Contrast-enhanced CT of the chest shows bilateral pleural effusions. Areas of central lucency are identified within central and peripheral nodular consolidation.Grahic Jump Location

Blood cultures grew methicillin-sensitive Staphylococcus aureus. Transthoracic echocardiogram demonstrated a small mobile tricuspid valve vegetation and mild tricuspid regurgitation. After an episode of small volume frank hemoptysis, repeat contrast-enhanced CT of the chest showed development of a 5.8-cm enhancing mass in the left lower lobe and a smaller 3.4-cm right middle lobe fusiform enhancing mass (Fig 2).

Figure 2
Figure Jump LinkFigure 2 Contrast-enhanced CT of the chest shows increased bilateral pleural fluid and large homogeneously enhancing fusiform and saccular mass in the left lower lobe. A smaller, fusiform mass is present in the right middle lobe.Grahic Jump Location

What is the diagnosis?

Diagnosis: Mycotic pulmonary artery pseudoaneurysm formation in the setting of infectious right-sided endocarditis.

An aneurysm is defined by dilation of the vessel wall, whereas a pseudoaneurysm occurs when there is destruction of the two inner vessel wall layers with preservation of the tunica adventitia. If destruction of the entire wall occurs, it is prone to rupture as the surrounding thrombus lyses. Aneurysms of any type affecting the pulmonary arteries are rare compared with aortic, intracranial, or other major vascular locations. Mycotic pseudoaneurysms are caused by infectious involvement of the vessel wall.

The proposed pathologic mechanism of mycotic pulmonary artery pseudoaneurysm formation in the setting of right-sided endocarditis is by direct extension of intraluminal septic thromboemboli into vessel walls by endovascular seeding resulting in weakening of the walls and subsequent dilation. Mycotic aneurysms from septic pulmonary emboli most often arise in segmental pulmonary arteries and are usually multiple and bilateral because of their embolic nature. Spontaneous resolution of mycotic pseudoaneurysms with antibiotic therapy for endocarditis has been reported, and as such, the true prevalence of pulmonary artery pseudoaneurysm is unknown. The most common complication of mycotic pulmonary artery pseudoaneurysm is hemoptysis, which occurs when the integrity of the pulmonary arterial wall is compromised. Hemoptysis can be massive and reportedly leads to death in nearly 50% of patients. Other infectious causes leading to the formation of pulmonary artery pseudoaneurysms include tuberculosis (Rasmussen aneurysm), necrotizing pneumonia, syphilis, and mucormycosis. Noninfectious central pulmonary artery aneurysms and pseudoaneurysms are associated with altered pulmonary artery flow dynamics in pulmonary hypertension and in structural cardiac abnormalities such as patent ductus arteriosus. Central or peripheral pulmonary artery aneurysms and pseudoaneurysms are associated with weakened or inflamed vessel walls in vasculitis and connective tissue disease, primary lung neoplasm or pulmonary metastasis eroding into pulmonary arteries, and in penetrating trauma to pulmonary arteries. The most commonly described cause of iatrogenic pulmonary artery aneurysm, pseudoaneurysm, or pulmonary artery rupture in the literature is malpositioning of balloon-tipped pulmonary artery (Swan-Ganz) catheters too far into a pulmonary arterial branch and resultant endovascular injury (reported incidences < 1% for pulmonary artery pseudoaneurysm and 0.02% to 0.4% for pulmonary artery rupture).

Imaging findings of pulmonary artery pseudoaneurysm on unenhanced chest CT are nonspecific and may show a mass with associated atelectasis. In the presence of IV contrast, an enhancing nodule or mass in close proximity to a pulmonary artery with the same attenuation as the enhancing vessels suggests origin from a pulmonary artery, especially if connection with the adjacent pulmonary artery is visualized. When present, surrounding ground-glass opacities are consistent with bleeding from the aneurysm. Definitive diagnosis is made by pulmonary arteriogram and typically shows slow emptying of contrast material from the pseudoaneurysm resulting from inelastic properties of the aneurysm wall (Fig 3). Rapid growth of aneurysm diameter within 24 hours indicates an unstable vessel wall with high risk of life-threatening hemorrhage.

Figure 3
Figure Jump LinkFigure 3 Left pulmonary arteriogram shows the large left lower lobe pulmonary artery pseudoaneurysm.Grahic Jump Location

Experience in management of pulmonary artery pseudoaneurysm is limited because of its rarity, and treatment is not clearly established. Conservative management consisting of intravenous antibiotics and follow-up imaging is usually chosen in stable patients when there is no evidence of acute hemoptysis, chest pain, or dyspnea. However, some sources recommend early endovascular or surgical intervention for asymptomatic pseudoaneurysms because of the inability to predict rupture and associated massive hemorrhage, though no published criteria for treatment exist based on pulmonary artery pseudoaneurysm maximum size or interval growth in asymptomatic patients.

Endovascular embolization and surgery are the most commonly performed treatment options for pulmonary artery pseudoaneurysms with active hemorrhage, regardless of cause (infectious, trauma from Swan-Ganz catheter, vasculitis, or erosion by necrotic tumor). Therapeutic intervention literature is largely focused on controlling active pulmonary artery bleeding once hemoptysis has already ensued. Pseudoaneurysm embolization by an interventional radiologist is often the selected management option for active hemoptysis because it is less invasive and associated with lower morbidity and mortality compared with surgical options. Small published case series and individual case reports of patients with either pulmonary artery aneurysms or pseudoaneurysms and massive hemoptysis document high embolization technical success rates (up to 100%) and low long-term reintervention rate. The choice of embolic material is largely based on the interventional radiologist’s experience and preference, although detachable balloon and steel coils have successfully been used to treat mycotic pulmonary artery pseudoaneurysms. Peripheral mycotic pulmonary artery pseudoaneurysm is amenable to embolization. The preferred approach is embolization of the feeding pulmonary artery. Placing coils within a pseudoaneurysm (intrasaccular embolization) carries a potential risk of rupture because mycotic pseudoaneurysm walls are not stable, though this approach potentially preserves pulmonary artery branches distal to the pseudoaneurysm.

Clinical Course

CT findings and hemoptysis in the setting of infective endocarditis led to the diagnosis of mycotic pulmonary artery formation in this case. The patient underwent pulmonary arteriogram to show pathologic anatomy and provide a roadmap for possible therapeutic embolization. The larger left lower lobe pulmonary artery pseudoaneurysm was initially selected for treatment because of the higher risk of catastrophic hemorrhage compared with the smaller right middle lobe lesion. The feeding vessel was identified and embolized using detachable coils. Additionally, an Amplatzer plug was deployed at the origin of the pseudoaneurysm. The patient experienced no further hemoptysis, so it was presumed that the larger pseudoaneurysm was the source of hemoptysis, and, in the absence of symptoms, antibiotic management with imaging monitoring was elected for the smaller pseudoaneurysm. If the patient had continued to have hemoptysis, therapeutic embolization of the right middle lobe pseudoaneurysm would have been feasible. Her hospital course was prolonged by hematuria and proteinuria suspected because of glomerulonephritis in the setting of endocarditis. In addition, she suffered symptomatic heart failure from severe tricuspid regurgitation. The patient was discharged on hospital day 53 to complete a 6-week course of IV antibiotic therapy. Her history of hypophosphatemia was thought unrelated to the development of endocarditis.

Although the patient denied history of IV drug use, the constellation of what appeared to be IV drug use marks on the upper extremities, right-sided endocarditis, and positive urine drug screen raised the concern for IV drug use as the likely source of bacteremia. Follow-up CT pulmonary arteriogram 1 month after embolization demonstrated a stable embolized large left lower lobe pulmonary artery pseudoaneurysm without evidence of residual contrast opacification. The smaller right middle lobe pulmonary artery pseudoaneurysm showed peripheral thrombosis within the pseudoaneurysm sac and overall decreased aneurysm sac size. The patient was scheduled for possible outpatient tricuspid valve replacement; however, a repeat echocardiogram showed near complete recovery of tricuspid valve function.

  • 1.

    Mycotic aneurysms refer to any infectious process involving the arterial wall. Typical infectious causes include right-sided endocarditis, necrotizing pneumonia, tuberculosis (Rasmussen aneurysm), syphilis, and mucormycosis.

  • 2.

    The onset of hemoptysis in a patient with right-sided endocarditis should prompt further investigation of the pulmonary arteries to evaluate for mycotic pseudoaneurysm formation.

  • 3.

    Treatment of pulmonary artery pseudoaneurysms by percutaneous embolization is a less invasive alternative to surgery. With selective intrasaccular aneurysmal embolization, preservation of distal pulmonary artery branches is possible sparing pulmonary function distal to the pseudoaneurysm.

Financial/nonfinancial disclosures: None declared.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.


Figures

Figure Jump LinkFigure 1 Contrast-enhanced CT of the chest shows bilateral pleural effusions. Areas of central lucency are identified within central and peripheral nodular consolidation.Grahic Jump Location
Figure Jump LinkFigure 2 Contrast-enhanced CT of the chest shows increased bilateral pleural fluid and large homogeneously enhancing fusiform and saccular mass in the left lower lobe. A smaller, fusiform mass is present in the right middle lobe.Grahic Jump Location
Figure Jump LinkFigure 3 Left pulmonary arteriogram shows the large left lower lobe pulmonary artery pseudoaneurysm.Grahic Jump Location

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