Kalaydzhiev Petar, Partenova Anelia, Ilieva Radostina, Genova Kamelia, Kinova Elena
Cardiology Department, University Hospital "Tsaritsa Yoanna-ISUL", 1000 Sofia, Bulgaria.
Emergency Department, Medical University, 1000 Sofia, Bulgaria.
Reports (MDPI). 2024 Jun 20;7(2):48. doi: 10.3390/reports7020048.
Congenital absence of pericardium is a rare cardiac disorder with a reported incidence of less than 1 in 10,000. Although most of the cases are of little clinical significance, some of them are associated with serious complications, including risk of herniation and strangulation or coronary artery compression. Detailed Case Description: We report a case of a 36-year-old male referred for routine cardiovascular examination. He had a medical history of a heart murmur since childhood. Electrocardiogram (ECG) revealed sinus rhythm, normal axis, poor R-wave progression in the precordial leads and repolarization abnormalities with negative T waves in leads V1-V4. On 2D transthoracic echocardiography (TTE), an unusual heart position was noted with poor image quality from the standard acoustic windows. The parasternal long axis view gave the impression of right ventricular dilatation. The findings raised the suspicion of left to right shunt and possible atrial septal defect. For further evaluation, the patient was referred for cardiac magnetic resonance which demonstrated complete left-sided absence of the pericardium.
Due to indistinct and atypical symptoms and lack of clinical awareness, pericardial congenital absence is frequently misdiagnosed. Patients may complain of atypical chest pain. Patient's history and physical examination are often nonspecific. In cases with complete pericardial absence, ECG findings may include right axis deviation, right bundle block and sinus bradycardia. Echocardiography findings are also not characteristic, but some may raise the clinical suspicion of this diagnosis. The imaging modalities of choice are computed tomography and cardiac magnetic resonance. Treatment depends on the type of defect and clinical symptoms.
先天性心包缺如是一种罕见的心脏疾病，报告发病率低于万分之一。虽然大多数病例临床意义不大，但其中一些与严重并发症相关，包括疝形成、绞窄风险或冠状动脉受压。详细病例描述：我们报告一例36岁男性因常规心血管检查前来就诊。他自幼有心脏杂音病史。心电图（ECG）显示窦性心律、电轴正常、胸前导联R波进展不良以及V1-V4导联T波倒置的复极异常。在二维经胸超声心动图（TTE）检查中，发现心脏位置异常，标准声学窗图像质量差。胸骨旁长轴视图显示右心室扩张。这些发现引发了对左向右分流和可能的房间隔缺损的怀疑。为进一步评估，患者被转诊进行心脏磁共振检查，结果显示左侧心包完全缺失。
由于症状不明确且不典型以及临床意识不足，先天性心包缺失常被误诊。患者可能主诉非典型胸痛。患者的病史和体格检查通常不具有特异性。在完全心包缺失的病例中，心电图表现可能包括电轴右偏、右束支传导阻滞和窦性心动过缓。超声心动图表现也不具有特征性，但有些表现可能会引起临床对此诊断的怀疑。首选的影像学检查方法是计算机断层扫描和心脏磁共振成像。治疗取决于缺损类型和临床症状。
