Where on the Differential Is Eisenmenger Syndrome in a Patient Without Prior Cardiopulmonary Disease?

Pulmonary hypertension (PH) can occur in patients with undiagnosed congenital heart disease, like atrial septal defects (ASDs), causing chronic left-to-right shunting. This may ultimately result in Eisenmenger physiology or syndrome (ES), a reversal of left-to-right shunting, resulting in a right-to-left shunt, thereby causing deoxygenated blood to enter systemic circulation as it bypasses the lungs. Development of PH due to an ASD is uncommon, and the occurrence of ES is <1% as most ASDs are corrected early in life. We present a 28-year-old female presenting with new-onset dyspnea found to have an undiagnosed ASD with ES. A 28-year-old female without past medical history presented to the emergency department after a chest x-ray performed by her primary care physician (PCP) showed dilation of the pulmonary artery concerning PH. The patient reported a three-month history of progressively worsening intermittent palpitations and dyspnea, now unable to walk more than one block without becoming dyspneic. Further imaging studies revealed a 1.4 centimeters (cm) secundum ASD, 4.4 cm dilatation of the PA, a mean pulmonary artery pressure (PAPm) of 132 millimeters (mm) mercury (Hg), and Eisenmenger physiology. She was placed on pulmonary vasodilators and iron supplementation to address an underlying iron-deficiency anemia. The patient is stable on her current regimen and is undergoing evaluation for possible heart-lung transplantation at an outside hospital. Dyspnea is one of the top 10 most common indications for emergency room visits annually. The differential diagnosis for dyspnea is vast, with ES, affecting only 0.8 in 1 million, far down on the list of possibilities, thus requiring high clinical suspicion to prompt further evaluation. Ultimately, the condition is preventable with early identification of underlying structural abnormalities for which definitive treatment options exist and are readily available, dramatically improving the prognosis if implemented before ES develops.

Pulmonary Necrobiotic Nodules at Time of Diagnosis in a Patient with Ulcerative Colitis.

Inflammatory bowel disease (IBD) is an umbrella term encompassing intestinal conditions Crohn's disease and ulcerative colitis (UC), characterized by inflammation of intestinal wall, differing in location, depth, pathophysiology, and sequela. Extraintestinal manifestations (EIM) of IBD commonly affect the skin, joints, eyes, and in rare instances, the lungs. Pulmonary involvement affects the large and small airways, serosal surface, and vasculature leading to a range of pathology, including bronchiectasis, pleural effusions, and necrobiotic nodules. The presence of EIM is uncommon at the diagnosis, particularly in regard to pulmonary EIM, most commonly seen years later. We present the case of a 22-year-old African American female who was discovered to have pulmonary involvement of her UC at the time of her diagnosis complicating management of her appendicitis. A 22-year-old female with a history of UC was transferred from an outside hospital for the management of her appendicitis after imaging revealed numerous pulmonary nodules. The presence of multiple cavitary pulmonary nodules delayed surgical intervention leading to a ruptured appendix. The patient had no cardiopulmonary complaints, and review of prior imaging studies showed these nodules to be present six weeks prior, the time of her diagnosis with UC. After antibiotic management failed, the patient required a laparotomy appendectomy with omentectomy due to resulting appendiceal abscess and phlegmon. The nodules were determined to be EIM of UC after sarcoidosis, infectious, and malignant etiologies were ruled out. For the pulmonary nodules, she is following at an outside hospital for the management of her UC as treatment of her underlying UC will result in a decrease or resolution of the EIM. EIM of IBD may present in patients at any time, even before their initial diagnosis of an IBD. While pulmonary manifestations are rare EIM, the presence of pulmonary nodules at the time of initial diagnosis is exceedingly uncommon. Evaluation and management of these nodules, even if asymptomatic in nature, requires diligence and thorough documentation regarding their onset and etiology. In the event of a medical emergency, such as in the case of our patient with appendicitis, a lack of thorough documentation and evaluation of the nodules may result in unnecessary medical testing, invasive procedures, and delay in treatment of their current medical illness.