Proximal interruption of the right pulmonary artery: A rare anomaly.

We report a case of a 21-year-old woman with cyanosis where computed tomography (CT) angiography revealed a complete absence of the mediastinal part of the right pulmonary artery with the normal intrapulmonary vascular network, receiving supply from multiple collateral vessels. The case also highlights the various differential diagnoses and the role of CT angiography in arriving at the correct diagnosis.

Pneumonectomy for Unilateral Proximal Interruption of Pulmonary Artery: A Case Series from the Literature.

BACKGROUND: Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature. The occasional necessity for pneumonectomy is still supported by limited evidence. METHODS: A systematic review of the literature was conducted using the PubMed search engine, focusing on UPIPA cases that received pneumonectomy. Thirty-one pertinent articles were selected and included in the analysis. A case reported from our institution was included in the analysis. RESULTS: We found 25 adults and seven children affected by UPIPA who received an indication for pneumonectomy, plus an additional case that was reported by our institution. Among adult patients, the predominant indication was hemoptysis (57%), followed by suspected or confirmed lung cancer (23%). Approximately 46% of surgical procedures were classified as urgent or emergent. Postoperative complications were observed in 36% of cases, with no recorded mortality. In pediatric cases, pneumonectomy was primarily a life-saving intervention, performed urgently or emergently in 75% of instances. A possible late complication in pediatric patients involves a mediastinal shift leading to respiratory distress, which may be mitigated using an inflatable prosthesis. CONCLUSIONS: Pneumonectomy achieves complete resolution of UPIPA symptoms. In the adult population, its primary indication is hemoptysis, with procedures conducted in both elective and urgent/emergent settings. Despite a mortality rate of zero, a notable proportion of patients may experience postoperative complications. In pediatric cases, the clinical presentation varies more extensively, and pneumonectomy is typically reserved for life-threatening situations, emphasizing the need for careful patient selection.

Proximal Interruption of Pulmonary Artery: Spectrum of Radiological Findings With Emphasis on Chest Radiograph and Contrast-Enhanced Computed Tomography (CECT).

Introduction Proximal interruption of pulmonary artery (PIPA) is a congenital anomaly presenting with aberrant termination of the pulmonary artery at the hilum. It results in a variety of radiological and clinical manifestations. Clinically, isolated PIPA can be asymptomatic till late adulthood or can present with dyspnoea, chest discomfort, hemoptysis and recurrent infections. PIPA can be associated with multiple cardiovascular anomalies such as tetralogy of Fallot (TOF), ventricular septal defects (VSD), and scimitar syndrome. We present a spectrum of cases with both isolated proximal interruption of the pulmonary artery and cases associated with other cardiovascular abnormalities. Typical chest radiographs and chest contrast-enhanced computed tomography (CECT) findings are discussed and demonstrated in detail. Proper and early diagnosis is a crucial step as it can significantly modify the treatment choice, thereby reducing morbidity. Objective To document the different presentations of the proximal arrest of pulmonary arteries, to document associations with cardiovascular and pulmonary manifestations, and to elaborate on and demonstrate the various radiological imaging findings. Material and methods All the cases that were reported with proximal interruption of pulmonary artery on the CECT studies conducted between 2019 and 2022 at a tertiary care hospital in Telangana, India. The demographic data, clinical presentation, chest radiographs, and chest CECT were collected retrospectively. Data analysis was done using Microsoft Excel 2019 to calculate descriptive statistics. A total of 22 cases were identified of which three cases were excluded of as they were previously operated and 19 cases were taken as the study population. Results Nineteen patients were included in the study. Demographic details, clinical history, CECT, and chest radiographs were collected wherever available. The majority of the cases belonged to the ≤ 10 yrs age group with the most common clinical presentation being a previous diagnosis of tuberculosis or recurrent upper respiratory tract infections. The predominant findings on chest radiographs were deviation of the trachea to the affected side, volume loss in the ipsilateral lung field, and compensatory hyperinflation of the contralateral lung field. On the CECT chest, the main findings were interrupted pulmonary artery, hypoplastic lung fields with bronchiectasis, or ground glassing. Associated cardiovascular and pulmonary malformations were identified with notable cases: TOF, right-sided aortic arch and scimitar syndrome. Their typical imaging findings have been elucidated and discussed in detail. Conclusions Patients with recurrent respiratory infections or hemoptysis having hypoplastic lung field with hyperinflation of the contralateral lung on chest radiographs should be evaluated for pulmonary artery interruptions. Chest CECT allows evaluation of the bronchial tree and lung parenchyma at the same time which helps distinguish pulmonary interruption from conditions such as Swyer-James-Macleod syndrome, pulmonary hypoplasia, thromboembolism and arteritis. Cases with PIPA can also be associated with cardiovascular and pulmonary anomalies such as TOF, partial anomalous pulmonary venous connection (PAPVC), and VSD. The knowledge of these associations is essential as they can influence the mode of treatment and can help reduce the long-term morbidity and mortality associated with the condition.

Unilateral proximal interruption of pulmonary artery with ipsilateral interstitial lung disease - A rare case report.

Unilateral proximal interruption of pulmonary artery with ipsilateral occurrence of lung fibrosis is a very rare entity. This case report is about a 27 year old male who had complaints of progressive dysponea since 1 year. He had past history of recurrent lower respiratory tract infections. On auscultation, velcro crackles are heard on right side. Pulmonary function test showed restrictive pattern. Chest Radiography, High Resolution Computed Tomography and CT Pulmonary angiography were performed.