Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review

Abstract

Background: Isolated unilateral absence of pulmonary artery (UAPA) in adulthood is a rare congenital anomaly. Although some case reports exist, the clinical symptomatology, lung parenchymal features, collateral circulation and therapeutic approaches in adult patients with isolated UAPA remain unknown. The objectives of this study are to investigate the clinical characteristics, elucidate the correlation between clinical symptomatology and radiology, and summarize treatment of adult patients with isolated UAPA. Methods: Cases of adult patients with isolated UAPA who had been diagnosed at our hospital and identified from PubMed, EMBASE and Web of Science from 1990 to 2016 were analyzed. Results: Hemoptysis was present in 41.5% of patients, exertional dyspnea in 41.5%, and recurrent respiratory infection in 35.4%. Lung parenchymal abnormalities were found on chest computed tomography (CT) scan, including bronchiectasis, which occurred in 30.2% of the patients, interstitial changes in 14.0%, and multiple bullae in 14.0% of the patients. Exertional dyspnea was more frequent in patients with pulmonary hypertension than in those without pulmonary hypertension (P<0.001). Recurrent respiratory infection were more frequent in patients with bronchiectasis than in those without bronchiectasis (P<0.001).Hypertrophic bronchial, phrenic, internal thoracic and intercostal arteries were found in 71.9%, 46.9%, 43.8%, and 43.8% of the patients, respectively. Pneumonectomy reduced hemoptysis in seven cases. Oral phosphodiesterase inhibitors or endothelin receptor antagonist improved exertional dyspnea in three cases with pulmonary hypertension. Conclusions: Clinicians should be aware of undiagnosed cases of isolated UAPA in adults with unexplained hemoptysis or exertional dyspnea. Early recognition and management of isolated UAPA in adult patients are crucial to avoid the devastating effect of massive hemoptysis or severe pulmonary hypertension (PHT) in the long term.

Left lung cancer in a patient with congenital unilateral absence of the left pulmonary artery: a case report and literature review

“…To date, no consensus was reached on the management of patients with UAPA. Pneumonectomy and surgical revascularization, selective embolization of systemic arteries, and pharmacological treatment for pulmonary hypertension are optional treatments for UAPA [5]. Surgical treatment for UAPA is considered for patients in whom associated anomalies are present or for those who are symptomatic.…”

“…Presented symptoms may include dyspnea on exertion, recurrent pulmonary infections, hemoptysis, chest pain, and pleural effusion. Approximately 13 to 15% of patients with unilateral pulmonary artery agenesis remain asymptomatic and are diagnosed incidentally [1,4,5]. Digital subtraction angiography (DSA) has ever been the gold standard for establishing a diagnosis of pulmonary artery agenesis, and now been replaced by non-invasive methods like CTA.…”

Unilateral pulmonary artery atresia in an adult: A case report

“…Adult patients with unilateral pulmonary atresia typically present with hemoptysis or exertional dyspnea, but can also have recurrent respiratory infections, chest pain, or even a propensity for high altitude pulmonary edema. The lung parenchyma on the affected side can have bronchiectasis (particularly in patients with recurrent infections), interstitial changes, or multiple bullae [6].…”

Right Pulmonary Artery Agenesis and Right Pulmonary Hypoplasia, presenting with Bronchiolitis Symptoms

“…Etyolojisinde kromozomal defektler, A vitamini eksikliği, intrauterin enfeksiyonlar ve çevresel faktörler rol oynadığı düşünülmektedir (5). UPAA olgularında genellikle pulmoner vasküler yatak normaldir, bunun nedeni vasküler yatağın dorsal aortanın ventral dallarından köken almasıdır (6). Distal intrapulmoner dallar; bronşiyal, interkostal, internal mamarian, subdifragmatik, subklaviyan ve hatta koroner arterlerden kollateraller alarak beslenirler.…”

“…Toraks MRI da yararlı bilgiler verebilir. Ancak UPAA tanısında altın standart yöntem pulmoner anjiografidir, pulmoner arterin yokluğunu gösterebildiği gibi, kollateral dolaşımı göstermede ve hemoptizi tedavisinde embolizasyon amaçlı da kullanılabilmektedir (6,15). Ayrıcı tanıda; Masif pulmoner emboli, Swyer-James-Macleod sendromu, çocukluk çağında bronşiolit ile giden viral ve mikoplazma enfeksiyonları akılda bulundurulmalıdır.…”