Diagnosis and management of the double orifice mitral valve: three case reports

Double orifice mitral valve (DOMV) is an uncommon congenital heart defect. The isolated occurrence of this anomaly is very rare and, more often, is associated with another congenital malformation, dominated by atrioventricular canal defects (AVCD). Mitral insufficiency and/or stenosis may complicate this malformation. Treatment may be summarized as abstention, surgical valve repair, or valve replacement. In here, we report three cases with DOMV. The first patient was an 18-month-old boy who was operated for severe mitral valve stenosis and mild insufficiency (in 1980s), the second was a 47-year-old female, who was operated recently for mitral valve replacement (MVR) under cardiopulmonary bypass due to severe mitral valve insufficiency. This patient had been operated due to the secundum-type atrial septal defect in our institution previously. The last one, a 3-year-old boy, with DOMV and three papillary muscles, was on clinical follow-up because he had no symptoms.     

Body fat composition: a predictive factor for obstructive sleep apnea

OBJECTIVE: To investigate body fat composition, measured by bioelectrical impedance assay (BIA), for predicting the presence and severity of obstructive sleep apnea-hypopnea syndrome (OSAHS). Body fat composition was also compared with other well-known OSAHS predictors such as body mass index (BMI), neck circumference, and abdominal visceral fat. STUDY DESIGN: A prospective study was designed. Fifty-one patients (41 male, 10 female), who were referred to Hacettepe University Faculty of Medicine, Department of Otorhinolaryngology, Head and Neck Surgery with suspected OSAHS, between April 2003 and June 2004, were included in the study. METHODS: All patients underwent polysomnography (PSG) and were classified according to their apnea-hypopnea index (AHI) into four groups. The cross-sectional area of abdominal visceral fat was measured by computed tomography (CT) scanning in 33 of the patients. Neck circumference and BMI was measured for all patients. BIA was performed to determine body fat composition. The groups were compared, and correlation of the variables with AHI was investigated. RESULTS: Of the variables, BMI and percentage of body fat (determined by BIA) were found to be significantly correlated with AHI (r = 0.782, r = 0.647). CT of cross-sectional area of abdominal visceral fat provided 100% sensitivity and specificity (P < .001) in differentiating simple snorers from OSAHS patients. By combining percentage of body fat and body fat mass, higher levels of sensitivity (95%) and specificity (100%) were achieved for diagnosis of OSAHS. CONCLUSION: It was concluded that the BIA could be an inexpensive and practical alternative to prePSG screening tests and should be included in the evaluation of OSAHS patients.     

Concurrent Poland anomaly and idiopathic hirsutism

Poland syndrome is characterized by congenital and unilateral absence of the pectoralis major muscle and ipsilateral upper limb anomalies. Identified patients also may include other disorders. We report a 15-year-old Caucasian woman with a unilateral hypoplasia of the breast and nipple, ipsilateral chest wall depression deformity, pectoralis major muscle agenesis, and severe hirsutism (Ferriman-Gallwey score: 21) without extremity anomaly. She had regular menses, and no hormonal abnormality and family history of hirsutism. Therefore, she was considered as a case of idiopathic hirsutism. This is the first case report of hirsutism in a patient with Poland syndrome.     

Effects of diazeniumdiolates (NONOates) and methylene blue on the reduction of postoperative adhesion in rats

We investigated the effects of spermine NONOate (SPER/NO), diethylenetriamine NONOate (DETA/NO) and methylene blue for preventing postoperative adhesion in a rat uterine horn model. Before operations, rats were randomly assigned into 6 groups, each composed of 12 rats. These were the sham, control, normal saline, SPER/NO, DETA/NO and methylene blue groups. Each rat was anesthetized with ketamine hydrochloride (40 mg/kg i.v.). The abdominal wall was shaved; the surgical site was scrubbed with povidone iodine and rinsed with sterile saline 3 times before surgery. Under sterile conditions, a 3-cm vertical midline incision was made in all groups. In the sham group, we closed the abdominal wall without any procedure. In the other groups, a 2-cm segment of each uterine horn was injured in 10 spots on the antimesenteric surface using unipolar cautery. Before the final abdominal closure, no adjuvant therapy was administered intraperitoneally to the rats in the control group; 2 ml of normal saline solution, 1% methylene blue solution, SPER/NO (0.5 mg/ml) and DETA/NO (0.1 mg/ml) were instilled into the uterine horns of the rats in the respective groups. The incision was closed with a running 4-0 monofilament delayed absorbable suture in a single layer of muscle and fascia in a running pattern, excluding the peritoneum, and in the covering layer of skin in an interrupted pattern, in all groups. Two weeks after the surgery, all animals were killed, second laparotomies were performed, and the extent and severity of adhesions were determined by a blinded examiner. The adhesion scores of the sham group were significantly lower than those of the other groups (p < 0.05). In the methylene blue, SPER/NO and DETA/NO groups, adhesion scores were significantly lower than in the normal saline and control groups (p < 0.05). However, there were no significant differences related to the extent and severity scores of adhesions between the methylene blue, SPER/NO and DETA/NO groups (p > 0.05). This study showed that SPER/NO, DETA/NO and methylene blue administered at the end of surgery reduced the adhesion formation in a rat uterine horn model.     

Transcatheter Embolization of a Coronary Fistula Originating from the Left Anterior Descending Artery by Using N-Butyl 2-Cyanoacrylate

In this report, we describe a successful percutaneous transcatheter n-butyl 2-cyanoacrylate embolization of a coronary fistula originating from the left anterior descending artery in an adolescent with unexpected recurrent attacks of myocardial ischemia.     

Immune function in children with classical phenylketonuria and tetrahydrobiopterin deficiencies

BACKGROUND: An increased susceptibility to infections has been observed in some patients with phenylketonuria (PKU), which is not well known whether it is due to alterations of plasma essential amino acid concentrations or to some other factors. OBJECTIVE: This study is designed to establish B cell and T cell functions in 44 children with classical PKU and tetrahydrobiopterin (BH4) deficiencies and the effects of too high plasma phenylalanine (PA) concentrations (16.53 to 30.54 mg/dL) on the same parameters. DESIGN: B and T cell functions of 33 children with classical PKU (divided into two groups based on fasting mean plasma PA concentrations: Group-I = 20.9 +/- 3.7 mg/dL, Group-II = 3.8 +/- 1.02 mg/dL), and 11 children with BH4 deficiencies (Group III) were studied. The results were compared between the groups and referenced with previously reported values from healthy controls. RESULT: Delayed type skin hypersensitivity responses to purified protein derivative (PPD) in Group I and phytohaemagglutinin (PHA) in Group I, III were lower than the other groups and healthy controls. Plasma IgG and IgM concentrations of Group I was lower than the reference values. Although mean serum zinc and iron levels of all patients were lower than published values of healthy children, zinc and iron deficiencies in Group I, III were much more prominent as compared to Group II. CONCLUSION: The somewhat low plasma IgG concentrations in Group I may be related to the very high plasma PA levels, however the role of zinc deficiency as a causal factor can not be ruled out. BH4 metabolism defects do not appear to affect the same parameters. Impaired delayed skin hypersensitivity responses in Group I and III can be explained by severe serum zinc deficiency. In the light of this study, we conclude that in order to establish a causal relationship between PKU and immune functions, further studies need to be conducted after the correction of micro-nutrient status of such children.     

Double-chambered right ventricle mimicking asymmetric septal hypertrophy

Double-chambered right ventricle (DCRV) is an uncommon congenital heart disease. Anomalous muscle bundles in the right ventricle divide the right ventricle into two portions and cause intracavitary obstruction of the right ventricle. We report a patient with DCRV mimicking asymmetric septal hypertrophy. We did clinical, echocardiographic and angiographic evaluation. Surgical intervention was successfully managed.     

Increased P-wave dispersion a risk for atrial fibrillation in adolescents with anorexia nervosa

ABSTRACT

Studies have shown that a prolonged P-wave dispersion is a risk factor for the development of atrial fibrillation. The aim of this study was to evaluate P-wave dispersion in adolescents with anorexia nervosa at diagnosis. We evaluated electrocardiographic findings, particularly the P-wave dispersion, at initial assessment in 47 adolescents with anorexia nervosa. Comparison of P-wave dispersion between adolescents with anorexia nervosa and controls showed a statistically significant higher P-wave dispersion in patients with anorexia nervosa (72 ± 16.3 msec) when compared to the control group (43.8 ± 9.5 msec). Percent of body weight lost, lower body mass index, and higher weight loss rate in the patients with anorexia nervosa had no effect on P-wave dispersion. Due to the fact that anorexia nervosa has a high mortality rate we believe that cardiac pathologies such as atrial fibrillation must also be considered in the medical evaluation.