Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis

Abstract

Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved.

Clinical and imaging features of presumptive congenital pulmonary underdevelopment with complications in two dogs

“…In these cases, especially, thoracic radiography is readily available at a low cost for long‐term follow‐up. However, radiographic features, such as mediastinal shift, collapsed affected lung lobe and hyperlucent contralateral lung lobe in previous veterinary reports and humans with congenital pulmonary underdevelopment are difficult to diagnose as these features mimic other chronic lung diseases 7,10,11,18,19,24 . In both cases in this report, lung parenchymal atelectasis was considered for the affected lung lobes on thoracic radiographs, but CT scans confirmed congenital pulmonary lesions without alveolar structure.…”

“…Nevertheless, in some affected adults, serious complications, such as uncontrolled recurrent lung infections, life‐threatening pulmonary haemorrhage or pulmonary hypertension, may require medical treatment or emergency surgery 7–9,18 . Therefore, an early diagnosis through advanced imaging modality and a long‐term follow‐up in adult patients are extremely important, as they enable consequent timely detection and treatment of serious complications 16,19,25 . For these reasons, in human medicine, patients with congenital pulmonary underdevelopment require a multidisciplinary approach for patient care, involving subspecialists for expected complications and prognosis 16,25 .…”

“…Thus, the diagnosis of congenital pulmonary underdevelopment only by radiographs, without CT scans, which provide a detailed description of the bronchial tree, parenchyma and vasculature, was limited. Among the complications of congenital pulmonary underdevelopment, the management of pulmonary hypertension plays an essential role in prolonging the survival period in human medicine 6,17,19,26,27 . Follow‐up with yearly echocardiography evaluation should be considered for timely detection and treatment of complications, especially pulmonary hypertension secondary to congenital pulmonary underdevelopment 6,25 .…”

“…The commonly used histological methods are the lung weight to bodyweight ratio and the radial alveolar count defined as the number of alveoli crossing a line drawn from a respiratory bronchiole centre to the nearest and definitive connective tissue septum 16 . These histopathologic methods are related to the functional parenchymal volume of the lung, and based on this principle, in human medicine, congenital pulmonary hypoplasia can be diagnosed through indirect measurement of the affected lung parenchymal volume through CT, ultrasound, MRI and comparison with normal contralateral lung lobes 6,9,17–21 . Even in veterinary medicine, there is a report of presumptive diagnosis through the CT scan without performing a histopathologic examination 10 …”

“…The hypoplastic lung lesions identified in these cases can be considered congenital lesions; the acquired lung diseases may also be able to develop pulmonary hypoplasia. In human medicine, adult diagnosis of congenital pulmonary hypoplasia is challenging to differentiate from acquired pulmonary diseases such as pseudo‐hypoplasia, which is caused by pulmonary consolidation or fibrosis due to chronic lung inflammation 9,18,19 . Because histological examination in adult patients is clinically limited, the tentative diagnosis of congenital pulmonary hypoplasia can be based on the absence of the history of lung disease diagnosed during infancy, the fact that it was asymptomatic until adulthood and the imaging features of chest CT 18,19 .…”