Ebrahimi Pouya, Mandegar Mohammad Hossein, Jafari Fesharaki Mehrdad, Ghasemloo Negar, Ramezani Pedram, Akbari Tooba, Naderi Fatemeh
Non-Communicable Diseases Research Center, Endocrinology and Metabolism Population Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran.
Department of Cardiovascular Surgery, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Int J Emerg Med. 2025 Jan 7;18(1):6. doi: 10.1186/s12245-025-00809-3.
Partial anomalous pulmonary vein connections (PAPVC) are rare congenital abnormalities in which one or more pulmonary veins drain into the right atrium. This pathological condition may present in various ways, such as chest pain and dyspnea, or it may be diagnosed incidentally. Consequently, missed or late diagnoses are common, highlighting the importance of optimal diagnostic modalities. This study presents a case of PAPVC that remained undiagnosed despite two years of symptomatic evaluations.
The patient was a 58-year-old woman who presented with chest pain and dyspnea, New York Heart Association Functional Class (NYHA FC) II, which had started 2 years before. She had been evaluated with a transthoracic echocardiogram, coronary angiography, and chest imaging, none of which resulted in a diagnosis or effective treatment. After being referred for evaluation by a cardiac imaging specialist, the diagnosis of PAPVC was revealed. The patient underwent corrective surgery, resulting in complete recovery and symptom improvement. Post-surgical cardiac overload caused pleural effusion and dyspnea, which were managed with diuretics. Four- and six-month follow-ups showed no abnormal findings on examination and imaging, and the patient reported no new complaints.
The findings in this structural cardiac abnormality are nonspecific and can be missed, and misdiagnosis is relatively common. However, patients with symptoms such as chest pain, dyspnea, increased pulmonary artery pressure, and right ventricle dilation should undergo surgical treatment to avoid more serious complications, such as heart failure.
Detection of PAPVC is not straightforward in many cases and often requires evaluation with various imaging techniques for accurate diagnosis. Therefore, physicians encountering undefined causes of chest pain or dyspnea should consider multiple imaging modalities. Follow-up is also important, as certain groups of patients may require surgical treatment to prevent complications.
部分性肺静脉异位连接（PAPVC）是一种罕见的先天性异常，其中一条或多条肺静脉引流至右心房。这种病理状况可能以多种方式出现，如胸痛和呼吸困难，也可能是偶然被诊断出来。因此，漏诊或误诊很常见，凸显了最佳诊断方式的重要性。本研究报告了一例尽管经过两年的症状评估仍未被诊断出的PAPVC病例。
患者为一名58岁女性，出现胸痛和呼吸困难，纽约心脏协会心功能分级（NYHA FC）为II级，症状始于两年前。她接受了经胸超声心动图、冠状动脉造影和胸部影像学检查，但均未得出诊断结果或有效治疗方案。在被转诊给心脏影像专家进行评估后，确诊为PAPVC。患者接受了矫正手术，完全康复且症状改善。术后心脏负荷过重导致胸腔积液和呼吸困难，通过利尿剂进行了处理。术后四个月和六个月的随访显示，检查和影像学检查均未发现异常，患者也未报告新的不适。
这种心脏结构异常的表现不具有特异性，可能会被漏诊，误诊也相对常见。然而，出现胸痛、呼吸困难、肺动脉压升高和右心室扩张等症状的患者应接受手术治疗，以避免出现更严重的并发症，如心力衰竭。
在许多情况下，PAPVC的检测并不简单，通常需要采用各种成像技术进行评估才能准确诊断。因此，遇到胸痛或呼吸困难原因不明的医生应考虑多种成像方式。随访也很重要，因为某些患者群体可能需要手术治疗以预防并发症。
