Imaging: Student/Resident Case Report Poster - Imaging |

Left Pulmonary Artery Interruption: A Typical Presentation of an Uncommon Condition FREE TO VIEW

Srikanth Yandrapalli, MD; Deepthi Gandhiraj, MD; Lakshmi Asritha Gollapudi, MD; Gregg Lanier, MD; Ramin Malekan, MD
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Department of Medicine, New York Medical College at Westchester Medical Center, Valhalla, NY

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

Chest. 2016;150(4_S):664A. doi:10.1016/j.chest.2016.08.758
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SESSION TITLE: Student/Resident Case Report Poster - Imaging

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Unilateral absence of pulmonary artery (UAPA) or proximal interruption of pulmonary artery (PA) is a rare developmental anomaly due to a malformation of the sixth aortic arch. It is usually associated with other congenital heart defects or can present as an isolated lesion.

CASE PRESENTATION: A 36-year-old previously healthy male presented with worsening hemoptysis and exertional dyspnea of 2-week duration. Physical exam showed mild lip cyanosis and fixed splitting of second heart sound. A chest radiograph showed a left lung upper lobe(LUL) opacity and a prominent PA. Chest CAT scan showed a cavitary mass in the LUL, atretic left PA truncated at the hilum, many small left bronchial arteries arising from the aorta, enlarged right PA and pulmonary trunk, and mild aortic coarctation. A V/Q scan showed severely decreased perfusion of the left lung that correlated with atretic left PA. An echocardiogram and a cardiac MRI showed a 14mm secundum type atrial septal defec t(ASD), moderate tricuspid regurgitation, dilated right heart, normal biventricular function, and PA hypertension (PAH). Right heart catheterization showed moderate WHO group-1 PAH. Bronchoscopy guided transbronchial biopsy specimens grew Aspergillus fumigatus consistent with a diagnosis of an aspergilloma. Cultures were negative for mycobacteria. He was started on sildenafil, and voriconazole and underwent ASD closure with tricuspid annuloplasty, and LUL resection. During the procedure, large collaterals from the chest wall to entire left lung parencyma were noted.

DISCUSSION: Though UAPA is more common on the right side, congenital cardiovascular anomalies are more frequent with left sided UAPA. Depending on the presence of other coexisting anomalies, it can either present in infancy with congestive heart failure and pulmonary hypertension (PH) or remain asymptomatic until adulthood. Recurrent pulmonary infections, limited exercise tolerance, hemoptysis, or incidental discovery during chest radiography are the common presentations in adults. PH was present in 19-44% of reported cases. Collaterals to the affected lung usually arise from the bronchial arteries, but can arise from other arteries including the intercostals. Our patient presented with the most common symptoms associated with UAPA, had other congenital heart defects, PH, and a pulmonary infection. Extensive collateral circulation might have allowed him to remain asymptomatic until adulthood. Worsening hemoptysis could have been from the extensive collateral circulation or the aspergilloma, and this warranted LUL lobectomy.

CONCLUSIONS: Physicians should consider UAPA in the setting of appropriate presentation or abnormal chest radiograph findings. Workup for coexisting cardiovascular conditions is important as early diagnosis might prevent complications of PH.

Reference #1: Kruzliak P et al. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis. 2013;106:448-54.

DISCLOSURE: Gregg Lanier: Consultant fee, speaker bureau, advisory committee, etc.: Speaker bureau for Bayer, Gilead, Actelion, United Therapeutics, The following authors have nothing to disclose: Srikanth Yandrapalli, Deepthi Gandhiraj, Lakshmi Asritha Gollapudi, Ramin Malekan

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