Genetic and Developmental Disorders: Fellow Case Report Poster - Genetic and Developmental Disorders |

Limited at Birth: Complications of Congenital Pulmonary Anomalies FREE TO VIEW

Navitha Ramesh, MD; Jaime Deseda, MD; Mariana Mercader, MD; Jason Filopei, MD; Roxana Sulica, MD
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Mount Sinai Beth Israel, New York, NY

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

Chest. 2016;150(4_S):639A. doi:10.1016/j.chest.2016.08.732
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SESSION TITLE: Fellow Case Report Poster - Genetic and Developmental Disorders

SESSION TYPE: Affiliate Case Report Poster

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

INTRODUCTION: Mild congential pulmonary anamolies are usually well tolerated by patients well into their adulthood. However, most major congenital pulmonary anamolies become evident in infancy. We present a rare case of unilateral lung and pulmonary artery hypoplasia, which was diagnosed in the sixth decade of life.

CASE PRESENTATION: A 62-year-old African American woman presented to the pulmonary clinic with complaints of worsening shortness of breath. Her physical examination revealed HR 103 bpm and saturation of 82% on 5L NC, decreased breath sounds without ronchi or crackles and moderate lower extremity edema. Her past medical history was significant for morbid obesity, severe scoliosis and obstructive sleep apnea(uncontrolled). Her chest X ray did not show any focal abnormality, however it was limited by her body habitus(BMI 50). Her pulmonary function tests showed restrictive defect with very low diffusing capacity Give numbers. Her echocardiogram showed elevated pulmonary pressure and right heart catheterization confirmed severe pulmonary hypertension with RV dysfucntion. Pulmonary hypertension work-up including a CTA of the chest revealed significant hypoplasia of left lung and left pulmonary artery. The patient was not aware of this diagnosis until this point. She had always attributed her dyspnea to her body habitus and group 3 pulmonary hypertension due to her sleep apnea.

DISCUSSION: Pulmonary hypoplasia is a rare congenital anomaly, typically diagnosed in infancy and childhood. This type of congenital anomaly can develop between the 4th and 24th week of gestation. Although congenital pulmonary hypoplasia leads to decreased number or size of airways, vessels and alveoli, the gross morphology of the lung is essentially normal. However, the pulmonary reserve is tenuous when there is a combination with other cardiopulmonary comorbidities.

CONCLUSIONS: Timely diagnosis and management of complications of congenital pulmonary defects is pivotal. Pulmonary diseases in adulthood is often complicated by several comorbidites and hence congenital anamolies may get ignored until the patient cannot compensate anymore.

Reference #1: Georgescu A, Nuta C, Bondari S. 3D Imaging in Unilateral Primary Pulmonary Hypoplasia in an Adult: A Case Report. Case Rep Radiol. 2011;2011:659586.

Reference #2: Katsenos S, Antonogiannaki EM, Tsintiris K. Unilateral primary lung hypoplasia diagnosed in adulthood. Respir Care. 2014;59(4):e47-50.

DISCLOSURE: The following authors have nothing to disclose: Navitha Ramesh, Jaime Deseda, Mariana Mercader, Jason Filopei, Roxana Sulica

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