Pulmonary Vascular Disease |

Right Pulmonary Artery Agenesis With Unilateral Hypoplastic Lung: A Case Report FREE TO VIEW

Shopana Ganpath, MD; Camelia Arsene, MD; John Haapaniemi, DO; Thomas Piskorowski, DO
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Internal Medicine, Detroit Medical Center, Sinai Grace Hospital, Detroit, MI

Chest. 2014;146(4_MeetingAbstracts):883A. doi:10.1378/chest.1992245
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SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Unilateral pulmonary artery agenesis is a rare congenital anomaly accompanied by cardiovascular abnormalities such as pulmonary hypertension, Eisenmenger's syndrome, Tetralogy of Fallot and history of recurrent lower respiratory tract infections in childhood. We report a case of right pulmonary artery agenesis diagnosed in adulthood.

CASE PRESENTATION: A 38 year-old African-American male presented with acute respiratory distress requiring emergent intubation. The patient had recurrent lower respiratory tract infection history, without prior intubations. He was a smoker with history of asthma and diffuse rhonchi on the left side and diminished breath sounds on the right on physical examination. Chest X-ray showed left-sided pneumonia and hypoplastic right lung. Subsequent CT scan revealed absent right pulmonary artery. Secondary to shunt with profound hypoxemia, patient required over two weeks of pressure control ventilation with high FIO2. Patient was treated for pneumonia with eventual tracheostomy and PEG placement. Laboratory studies showed leukocytosis and elevated troponins. Echocardiogram and cardiac catheterization showed non-ischemic cardiomyopathy and pulmonary hypertension with 46mmHg mean pulmonary artery pressure. He was eventually discharged to subacute rehab.

DISCUSSION: Unilateral absence of pulmonary artery was first documented in the 1860s. There is also an entity where the pumonary artery is simply "hidden". Cardiac abnormalities are usually more common with the left sided agenesis. The history is typical for multiple recurrent pulmonary infections. Radiographic findings are usually hypoplasia of the lung of the affected side and elevation of that hemidiaphragm. CT or MRI can actually confirm the diagnosis; however angiography may be required if surgical intervention is considered. Our patient had moderate pulmonary artery Hypertension and shunt related to Paten Foramen Ovale. Treatment typically is directed at controlling symptoms, in case of recurrent severe pulmonary infections or hemoptysis , pneumonectomy or lobectomy can be performed. "Hidden" pulmonary arteries can be revascularised but require close follow up.

CONCLUSIONS: Our patient presented in acute hypoxic respiratory failure. Review of 10 years of chest X-rays revealed hypoplastic right lung, prompting CT evaluation, revealing absent right pulmonary artery. Swyer-James syndrome was initially suspected due to hypoplastic right lung and recurrent pulmonary infection history. This individual survived into adulthood without confirmed diagnosis and significant complications till this catastrophic event.

Reference #1: Fraser RS, Muller NL, Colman N, Pare PD. Developmental anomalies affecting the pulmonary vessels. In: Fraser RS, Pare PD, Eds. Fraser and Pares Diagnosis of Disease of the Chest. Fourth Edition. Philadelphia. WB Saunders Company 1999: 637-675

Reference #2: Putov, NV, Shiriaeva, KF, Libov, SL. Isolated unilateral agenesis of the pulomonary artery. Grudin Khir1978; 41-47

DISCLOSURE: The following authors have nothing to disclose: Shopana Ganpath, Camelia Arsene, John Haapaniemi, Thomas Piskorowski

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