[Heterotaxia syndrome without congenital cardiac defects in dilated cardiomyopathy]. / Heterotaxie-Syndrom ohne kongenitalen Herzfehler bei dilatativer Kardiomyopathie.

UNLABELLED: 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. INVESTIGATION: 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. CONCLUSION: 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.

Anticoagulation with the low-molecular-weight heparin dalteparin (Fragmin) in atrial fibrillation and TEE-guided cardioversion.

BACKGROUND: Cardioversion (CV) in atrial fibrillation can cause arterial embolism. Effective anticoagulation clearly reduces the risk. In practice, in every third case anticoagulation is not in line with the recommendations. Simplification can be achieved, and time gained, by transesophageal echocardiography (TEE) due to the shorter anticoagulation period prior to CV, and by use of low-molecular-weight heparin (LMWH) for anticoagulation. As yet little data is available on LMWH in cardioversion. The aim of this cohort study was to investigate the administration of a LMWH in this indication under everyday clinical conditions. METHODS: 125 patients treated as inpatients for atrial fibrillation or -flutter received the LMWH Fragmin (dalteparin 2 x 100 anti-Xa units/kg, maximum dosage 2 x 10,000 anti-Xa units subcutaneously). In the presence of a relevant indication, TEE-guided CV was performed. The application of dalteparin was terminated as soon as effective anticoagulation had been achieved from phenprocoumon or once anticoagulation was no longer indicated. RESULTS: 125 patients with atrial fibrillation or -flutter received dalteparin for a median of 11 days (range of 3-41 days). TEE was performed in 39 patients. Five patients revealed a thrombus in the left atrial appendage in the TEE, and one patient died from suspected cerebral embolism over the further course. In the remaining 124 patients, no thromboembolic event was established. Successfully cardioverted were 26 of 34 patients (76%) who had no thrombus in the TEE. Serious adverse effects did not ensue. CONCLUSION: Simple, well tolerated and effective anticoagulation is possible with dalteparin in TEE-guided CV. Due to the methodic limitations of a cohort study and the low incidence of emboli, the efficacy of dalteparin in this indication needs to be further confirmed by prospective and randomized studies.