Evaluation of the congenital supravalvular aortic stenosis by different imaging modalities

A 36‐year‐old female was admitted to hospital exhibiting chest pain, dyspnea, and a heart murmur on the right upper sternal border, radiating to both carotid arteries. The blood pressure of the patient's right arm exceeded the pressure in the left by 25 mm Hg (Coanda effect). In spite of laboratory results that did not fall outside the expected range, the left ventricle was revealed to be hypertrophic following electrocardiography. Transthoracic echocardiography revealed a severe supravalvular aortic stenosis (SVAS) with a peak Doppler velocity of 6.04 cm/s and an estimated mean pressure gradient of 89 mm Hg, with moderate aortic and mitral regurgitation. Contrast‐enhanced computed tomography (CCT) indicated a partial hourglass‐shaped narrowing of the ascending aorta. Lesions associated with supravalvular stenosis of the pulmonary artery, patent ductus arteriosus, and aortic coarctation were ruled out by the CCT. Congenital SVAS is a rare heart condition, and three anatomically distinct forms have been described. The most common type is the “hourglass,” which produces a marked thickening and disorganization of the aortic tissue, producing a constricting annular ridge at the superior margin of the sinuses of Valsalva.     

A rare complication of mechanical aortic valve replacement: Separation in the region of the mitral‐aortic intervalvular fibrosa

A 28‐year‐old man was admitted to our emergency service with a shortness of breath and palpitation. On admission, his blood pressure was high and he was in hypertensive pulmonary edema. His physical examination showed rales in both lungs and pansystolic murmur at mitral focus. His medical history included aortic valve replacement (AVR) because of native aortic valve infective endocarditis. Transthoracic echocardiography (TTE) showed normal functional aortic valve. Color flow imaging demonstrated severe mitral regurgitation with posterior eccentric jet. To examine in detail, transesophageal echocardiography (TEE) and three‐dimensional (3D) echocardiography were performed. TEE disclosed a separation in the subaortic curtain leading to severe mitral regurgitation from the left ventricle to the left atrium. In addition to severe mitral regurgitation with posterior eccentric jet, 26‐mm‐long pouch was seen in mitral‐aortic intervalvular fibrosa (MAIVF) at 120° TEE view. This pouch was separated from the mitral anterior leaflet junction releasing the mitral anterior leaflet and causing prolapse and chorda rupture in the A2 scallop of the mitral anterior leaflet. The MAIVF connects the anterior mitral leaflet to the posterior portion of the aortic annulus. The separation of the MAIVF represents a complication of the aortic valve replacement.     

Sexual function may be impaired during pregnancy in adolescent women

Prevalence of sexual dysfunctions is reported as high as 70% during pregnancy. However, there are limited data about the burden of sexual dysfunction in pregnant adolescents. This cross-sectional study was conducted in the outpatient antenatal clinic of our institution between October and December 2014. The total female sexual function index scores and domain scores were compared between adolescent and adult pregnant women. A total of 121 pregnant women participated in the study. Of those women, 29 (24.0%) were 21 years old or younger (Group 1), whereas the remaining were older (Group 2). The sexual dysfunction ratios were 41.4% and 23.9% in Groups 1 and 2, respectively (p = 0.068). The desire (3.29 vs. 2.79, p = 0.024) and arousal (3.48 vs. 2.71, p = 0.008) domain scores of adolescent pregnant women were significantly higher whereas the remaining domain scores (i.e. lubrication, orgasm, satisfaction and pain) were not significantly different. The adolescent women seem to be more prone to sexual dysfunctions during the course of pregnancy, especially in the third trimester. The health care providers should consider discussing the sexual life during pregnancy as a part of routine antenatal visit to improve sexual function and quality of life, especially among adolescent pregnant population.     

Application of Rhenium-186 Radiosynovectomy in Elbow Diffuse Pigmented Villonodular Synovitis. - A Case Report with Multiple Joint Involvement

After surgical therapy of diffuse pigmented villonodular synovitis (DPVNS), recurrence is seen in almost half of the patients. The effectiveness of radiosynovectomy (RSV) in preventing recurrence and complaints of DPVNS is well known. Elbow involvement in DPVNS is a very rare condition; therefore, RSV in elbow hasn’t been experienced widely. The aim of this case report is to show the effectiveness of RSV with rhenium-186 (Re-186) sulfide colloid. We applied Re-186 sulfide colloid to the elbow joint of DPVNS patients six weeks after arthroscopic synovectomy. As a result, the patient did not have any complaints, and our findings are compatible with residue or recurrence on magnetic resonance imaging (MRI) in sixth and twentieth month controls after administration. We concluded that Re-186 is an effective adjuvant therapy for the prevention of recurrence and complaints.     

Development of amyloidosis in Behçet's syndrome with isolated mucocutaneous involvement

Development of secondary amyloidosis is an infrequent complication in patients with Behçet's syndrome (BS). It has been reported that multiple systemic involvement, long disease duration, and male gender are major clinical factors accompanying the development of amyloidosis in BS. We report a case of secondary amyloidosis in a patient diagnosed as having BS with a positive pathergy test 9 years previously and who had isolated mucocutaneous involvement. Regular use of colchicine since the diagnosis and somewhat mild progress of the disease could not prevent the development of secondary amyloidosis in this patient. He is alive and receives hemodialysis regularly.     

Adenocarcinoma arising from a strictureplasty site in Crohn's disease

The occurrence of small-bowel cancer in Crohn's disease (CD) is a rare event. The risk seems to be greatest in patients with long-standing disease. Strictureplasty has proved to be a valuable alternative in the management of Crohn's strictures of the small-bowel. Critics and proponents of strictureplasty for selected patients with smallbowel Crohn's disease have voiced their concerns about cancer risk in the strictured or strictureplasty site. To date, there has been no clear or detailed report of such an occurrence. The authors report the first case of small-bowel adenocarcinoma arising at the site of a previous strictureplasty. In this patient, biopsies of the strictures at the original operation confirmed CD and excluded both cancer and dysplasia. Malignancy occurred seven years later at a strictureplasty site. The main clinical sign associated with the adenocarcinoma was severe, persistent anemia. The authors conclude that the risk of adenocarcinoma developing at the site of a previous strictureplasty for CD, although small, is real.