Appearance of noncompaction-like remodeling of the anatomical right ventricle in a middle-aged subject with modified transposition of the great arteries who did not undergo surgery.

We describe noncompaction-like remodeling of the anatomical right ventricle (ARV) in a middle-aged subject with modified transposition of the great arteries (TGA). A 54 year-old male had been diagnosed with modified TGA at age 40, but no surgery was performed. Enhanced multislice CT revealed the ascending aorta coursing left of the anterior pulmonary trunk. Furthermore, the myocardium of the ARV appeared thickened, but contrast material could be observed in the ARV myocardium, which resembled noncompaction of the left ventricle (LV). We speculated trabecular development of the ARV, such as a Chiari network, and with TGA, the ARV provided systemic circulation through the aorta. The ARV wall may have thickened due to systemic pressure load, resulting in reduction of wall motion of the ARV on transthoracic echocardiogram. Recent advances in multislice CT imaging have revolutionized the exploration of RV anatomy, especially for depicting the three-dimensional appearance of noncompaction-like remodeling of the ARV in modified TGA in addition to transposition of the great arteries.

[Social concern and independence in adults with congenital heart disease].

OBJECTIVES: Recent advances in medical and surgical treatment have led to the survival of increasing numbers of adults with congenital heart disease (CHD). However, the social status of these patients remains unknown. This survey investigated the social prospects for adults with CHD, and the limiting factors for social independence. METHODS: A written questionnaire on patient characteristics, education, employability, marital status and insurability was designed to define the characteristics of social independence in adults with CHD. Randomly selected adults with CHD were enrolled: 13 patients with cyanotic unrepaired CHD (4 males, 9 females, mean age: 29.8 +/- 10 years, range: 18-56 years) and 102 patients with other CHDs (48 males, 54 females, mean age: 29.5 +/- 10 years, range: 18-74 years). RESULTS: University of California at Los Angeles functional class I-II was found in 94% of patients, medication in 46%, and hospitalization in 51%. Compared with the data from Japanese general population, study patients had a lower ratio of high school graduates (86% vs 94%), life insurability (51% vs 71%), marital status (31% vs 32%) and employability (82% vs 80%). Patients with unrepaired cyanotic CHD had significantly lower ratio than those with other CHDs (marital status 15%, p = 0.19; employability 40%, p = 0.0003; high school graduates 69%, p = 0.06; life insurability 18%, p = 0.02, respectively). CONCLUSIONS: Factors affecting social independence in adults with CHD were severity of disease, continuing medication, lower level of education, lower self-esteem, and unknown natural history of CHD. To improve social independence in these patients, further development of medical and surgical therapy and more detailed knowledge of the patients, caretakers and society in this field are needed.