Miguel Antonio Belaunzaran, Samuel Koenigsberg, Akash Mathavan, Akshay Mathavan
{"title":"部分肺静脉异常回流:进行性肺动脉高压患者的诊断挑战和成功纠正。","authors":"Miguel Antonio Belaunzaran, Samuel Koenigsberg, Akash Mathavan, Akshay Mathavan","doi":"10.1136/bcr-2024-264779","DOIUrl":null,"url":null,"abstract":"<p><p>Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous system, creating a left-to-right shunt and potentially causing right-sided volume overload. Frequently associated with atrial septal defects, PAPVR may present with non-specific symptoms such as exertional dyspnoea and is often misdiagnosed as pulmonary hypertension of other origins. We describe a woman with progressive exertional dyspnoea and lower extremity oedema in the setting of newly identified precapillary pulmonary hypertension. Imaging revealed severe right atrial enlargement and a left-to-right shunt from the right superior pulmonary vein draining into the superior vena cava, consistent with PAPVR. She underwent successful surgical correction with the Warden procedure resulting in functional recovery. This case highlights the importance of recognising unexplained right atrial enlargement as a clue to PAPVR and demonstrates the potential for symptomatic and physiological resolution following timely surgical intervention.</p>","PeriodicalId":9080,"journal":{"name":"BMJ Case Reports","volume":"18 7","pages":""},"PeriodicalIF":0.6000,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Partial anomalous pulmonary venous return: diagnostic challenges and successful correction in a patient with progressive pulmonary hypertension.\",\"authors\":\"Miguel Antonio Belaunzaran, Samuel Koenigsberg, Akash Mathavan, Akshay Mathavan\",\"doi\":\"10.1136/bcr-2024-264779\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous system, creating a left-to-right shunt and potentially causing right-sided volume overload. Frequently associated with atrial septal defects, PAPVR may present with non-specific symptoms such as exertional dyspnoea and is often misdiagnosed as pulmonary hypertension of other origins. We describe a woman with progressive exertional dyspnoea and lower extremity oedema in the setting of newly identified precapillary pulmonary hypertension. Imaging revealed severe right atrial enlargement and a left-to-right shunt from the right superior pulmonary vein draining into the superior vena cava, consistent with PAPVR. She underwent successful surgical correction with the Warden procedure resulting in functional recovery. This case highlights the importance of recognising unexplained right atrial enlargement as a clue to PAPVR and demonstrates the potential for symptomatic and physiological resolution following timely surgical intervention.</p>\",\"PeriodicalId\":9080,\"journal\":{\"name\":\"BMJ Case Reports\",\"volume\":\"18 7\",\"pages\":\"\"},\"PeriodicalIF\":0.6000,\"publicationDate\":\"2025-07-03\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"BMJ Case Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1136/bcr-2024-264779\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"BMJ Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1136/bcr-2024-264779","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
Partial anomalous pulmonary venous return: diagnostic challenges and successful correction in a patient with progressive pulmonary hypertension.
Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous system, creating a left-to-right shunt and potentially causing right-sided volume overload. Frequently associated with atrial septal defects, PAPVR may present with non-specific symptoms such as exertional dyspnoea and is often misdiagnosed as pulmonary hypertension of other origins. We describe a woman with progressive exertional dyspnoea and lower extremity oedema in the setting of newly identified precapillary pulmonary hypertension. Imaging revealed severe right atrial enlargement and a left-to-right shunt from the right superior pulmonary vein draining into the superior vena cava, consistent with PAPVR. She underwent successful surgical correction with the Warden procedure resulting in functional recovery. This case highlights the importance of recognising unexplained right atrial enlargement as a clue to PAPVR and demonstrates the potential for symptomatic and physiological resolution following timely surgical intervention.
期刊介绍:
BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.