Case Report: Young Girl Presents Dyspnea and Unilateral Interlobular Septal Thickening

In this case, a 12-year-old girl arrived at the hospital with exercise intolerance and radiographic abnormalities. Her five-year history included exercise-induced shortness of breath; two months before admission, she reported fever and right chest pain. A CT scan presented a small right hemithorax, interlobular septal thickening, and ground-glass opacity of the right lung; she was diagnosed with pneumonia and received antibiotics. Her fever subsided but shortness of breath during exercise persisted. A contrast-enhanced CT scan of the chest revealed interlobular septal thickening in the right lung and a smaller right pulmonary artery compared to the left. She had no systemic symptoms. Regarding family history, her grandparents were cousins and her father had congenital dysarthria. Upon admission the patient had no fever and was in overall healthy condition. A chest exam revealed decreased breath sounds over the right posterior lung field but no rales or rhonchi. The patient was ultimately diagnosed with isolated unilateral pulmonary vein atresia (IUPVA). IUPVA is a rare condition with a high risk for morbidity and mortality. It refers to right- or left-sided pulmonary vein atresia not in the presence of congenial heart disease. Symptoms typically manifest in the first three years of life. There are fewer than 60 reported cases of IUPVA in the medical literature. Read more