We examined the etiology of a long-term postoperative complication, pulmonary arteriovenous malformation (PAVM), in a patient who underwent total cavopulmonary shunt (TCPS). The patient underwent TCPS at the age of 3 years and 1 month, and a PAVM developed. At the age of 10 years, TCPS was switched to total cavopulmonary connection to facilitate circulation of hepatic venous blood to the pulmonary artery. As a result, oxygen saturation increased. These findings suggest that the absence of pulmonary circulation of hepatic venous blood is involved in the development of PAVM.
We present two patients with thromboembolic pulmonary hypertension associated with unusual complications probably caused by disseminated fibromuscular dysplasia (FMD) or FMD-like vascular lesions. Intimal fibroplasia, which is typical of the vascular lesions associated with FMD, was observed in both patients. The presence of such intimal lesions suggests that there was a systemic factor that caused the formation of recurrent thrombi in the systemic vessels in these patients. These cases are the first ones reported in which an association between FMD and pulmonary hypertension has been observed. The pathogenesis of the thrombi in our patients was thought to be recurrent pulmonary thromboembolisms resulting from FMD.
We report on an 8 month old infant with Kawasaki disease associated with giant coronary aneurysms and transient thrombocytopenia. The patient's platelet count decreased to 24000/mm3 on the 31st day of illness and fibrin degradation product was 5 μg/mL. Platelet count increased to the normal level (357000/mm3) on the 35th day of illness. On the 27th day of illness, dilatation of the distal abdominal aorta adjacent to the bifurcation of the iliac arteries was observed by B‐mode and color Doppler ultrasonography. It gradually returned to a normal size by the 45th day of illness. Aspirin administered from the 3rd to the 26th day of illness was replaced with flubioprophen because of liver dysfunction. Although we can not eliminate aspirin allergy as the cause of the transient thrombocytopenia, we think that the thrombocytopenia may have been related to the regression of the abdominal aorta.
Two-dimensional echocardiographic features in a case of transposition of the great arteries (TGA) with the posterior aorta are described. The unusual arrangement of the great arteries and the presence of bilateral conuses and aortic-mitral fibrous continuity were clearly demonstrated by angiocardiography and then confirmed at surgery.
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