Acquired hepatocerebral degeneration is a chronic encephalopathy which occurs in ∼1% of patients with liver cirrhosis and seems related to portosystemic shunts. Its is characterized by a combination of parkinsonism and cerebellar signs. MRI pallidal and extrapallidal lesions are seen in most patients, probably reflecting intracerebral deposits of manganese. Liver transplant did not improve the neurological signs in our patients, perhaps because of the persistence of portosystemic shunts.
A 52-year-old man was referred to our hospital to be evaluated for a heart transplant. He had a history of multiple admissions due to heart failure. His physical examination revealed: jugular distension, hepatomegaly, and peripheral edema. The electrocardiogram showed sinus rhythm with right axis deviation. A chest x-ray revealed cardiomegaly. Transthoracic echocardiography showed an aneurysmal dilatation of the right atrial appendage (RAA) with intracavity thrombus (Fig. 1). The right ventricular ejection fraction was impaired, with moderate tricuspid regurgitation, and pulmonary hypertension. A nuclear resonance image confirmed a giant appendage aneurysm with the intracavity thrombus and right ventricular dysfunction (Figs. 2 and 3). A 64-slice multidetector computed tomography ruled out chronic pulmonary Figure 1. Four-chamber echocardiography: RA = right atrial; RV = right ventricle.thromboembolism, and an aneurysmatic cavity was three-dimensionally reconstructed (Fig. 4). A cardiac catheterization was performed. The pulmonary arterial systolic pressure was 73 mmHg, the diastolic pressure was 28 mmHg, and the mean pressure was 42 mmHg. The left ventricle end-diastolic pressure was 25 mmHg. The cardiac index was 2.9 liter/min per meter square.Approximately 100 cases of right atrium abnormalities have been reported in the literature. 1 Giant RAA is a very rare anomaly discovered accidentally by an enlarged silhouette on a chest x-ray; an associated atrial septal defect has been reported. 2,3 Slightly less than half of the cases have symptomatic arrhythmias. The optimal treatment is controversial; a conservative approach is recommended in the absence of symptoms, leaving surgical treatment to symptomatic cases. Figure 2. Steady-state free precession (SSFP-b) images in systole, four-chamber view: RA = right atrial; RV = right ventricle; and T = thrombus. 95
A 2-year-old boy was referred for evaluation of a systolic heart murmur. Two-dimensional Doppler echocardiogram showed an abnormal flow through the interventricular septum, directed upward and toward the posterior wall of the main pulmonary artery. Left coronary angiogram showed a normal distribution of the anterior descending and circumflex arteries. The right coronary artery (RCA) was fully filled through collaterals from the left coronary system, and arising from the main pulmonary artery. Successful surgical reimplantation of the RCA was undertaken. Although uncommon, it is important to recognize the anomalous origin of the RCA arising from the pulmonary artery since it can be associated with serious adverse cardiac events.
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