Hildebrand H, Gunzenhauser D, Weber K, Frese W, Fröber R, Wetter D
Herz-Zentrum Bodensee, Departement für Kardiologie, D-Konstanz und CH-Kreuzlingen.
Dtsch Med Wochenschr. 2007 Apr 27;132(17):931-7. doi: 10.1055/s-2007-979360.
HISTORY AND OUTPATIENT INVESTIGATION: A 61-year-old woman presented with a six-month history of chest pain and shortness of breath on normal activity. The past medical history included hypertension treated with ACE inhibitors. While still an outpatient a diagnosis was made of cardiomyopathy with left atrial and ventricular dilatation, systolic and diastolic heart failure, moderate mitral valve regurgitation and newly documented atrial fibrillation.
Right heart catheterization was carried out via the right femoral vein. The inferior vena cava was found to be on the left, none on the right. Venous inflow was via a dilated hemiazygos vein, a persistent left superior vena cava and a markedly dilated coronary sinus into the right atrium. The hepatic veins were also directly connected to the right atrium, as was shown by retrograde perfusion during a venogram. Coronary heart disease was excluded by angiography, but a right heart catheterization was not possible because of the atypical venous connections. Ultrasound examination revealed abdominal situs inversus and polysplenia. Magnetic resonance imaging of the thorax demonstrated bilateral bilobar lungs and bilateral hyparterial bronchi.
DIAGNOSIS, TREATMENT AND COURSE: Heterotaxia with anomalous systemic veins and visceral defects was revealed during a diagnostic work-up, which was indicated by the finding of a dilated cardiomyopathy with chronic atrial fibrillation, moderate mitral valve regurgitation and arterial hypertension. Arterial hypertension and heart failure were successfully treated by medication. Attempts at rhythm control were unsuccessful. Safety measures were established to prevent thromboembolic complications and endocarditis.
Congenital anomalies of the systemic veins in adults are often discovered incidentally, because they are usually asymptomatic. They may cause diagnostic and therapeutic difficulties in cardiology, phlebology and surgery. These anomalies may increase the risk of thrombotic and thrombembolic events. Together with polysplenia and situs inversus they are phenotypical components of heterotaxia. They may have various clinical consequences and may occur spontaneously or may be familial.
病史与门诊检查：一名61岁女性因正常活动时胸痛和气短6个月前来就诊。既往病史包括用血管紧张素转换酶抑制剂治疗的高血压。仍为门诊患者时，诊断为扩张型心肌病伴左心房和心室扩大、收缩性和舒张性心力衰竭、中度二尖瓣反流以及新发现的心房颤动。
经右股静脉进行右心导管检查。发现下腔静脉位于左侧，右侧无。静脉血流经扩张的半奇静脉、持续存在的左上腔静脉和明显扩张的冠状窦进入右心房。肝静脉也直接与右心房相连，静脉造影时逆行灌注显示了这一点。血管造影排除了冠心病，但由于非典型静脉连接无法进行右心导管检查。超声检查显示内脏反位和多脾。胸部磁共振成像显示双侧双叶肺和双侧动脉下支气管。
诊断、治疗及病程：诊断检查发现存在内脏异位伴异常体静脉和内脏缺陷，表现为扩张型心肌病伴慢性心房颤动、中度二尖瓣反流和动脉高血压。动脉高血压和心力衰竭通过药物治疗成功控制。心律控制尝试未成功。采取了安全措施以预防血栓栓塞并发症和心内膜炎。
成人系统性静脉先天性异常常因无症状而偶然发现。它们可能在心脏病学、静脉学和外科手术中造成诊断和治疗困难。这些异常可能增加血栓形成和血栓栓塞事件的风险。与多脾和内脏反位一起，它们是内脏异位的表型组成部分。它们可能有各种临床后果，可能自发出现或具有家族性。
