Cooccurrence of Postural Orthostatic Tachycardia Syndrome with Two Different Clinical Entities

Postural orthostatic tachycardia syndrome (POTS) is an abnormal heart rate response to a positional change. Several potential mechanisms for pathophysiology of POTS are defined. This syndrome can coexist with different clinical situations. In our report, the first case was a 13-year-old female who has been followed up for diagnosis of homocystinuria. She was admitted to our outpatient clinic with complaints of dizziness after suddenly moving from supine to upright position and chest pain after exercise. Tilt table test was performed to evaluate dizziness. According to the tilt table test the patient was diagnosed with POTS. The second case was a 17-year-old female who had been evaluated in different centers with the complaints of fainting, bruising, redness, and swelling on the hands and feet after moving from supine position to upright position during the last 4 years. Postural orthostatic tachycardia syndrome was diagnosed by tilt table test and ivabradine was started. Herein, we aimed to point out the cooccurrence of different clinical entities and POTS

Coming from behind to win - A Qualitative research about psychological conditions of adolescents who have undergone open-heart surgery for single ventricle between the ages 0-5

Early recognition of congenital cardiac pathologies and their treatment by means of palliative or corrective surgery at birth or infancy has vital importance. Successful repair of congenital cardiac defects by surgical methods has gained importance especially during the last twenty years. As the scope of the surveillance increased so did the interest in the outcomes of these treatments when the patients had reached puberty and adulthood. The purpose of our research was to study the psychological framework of the adolescents who had experienced these surgeries by listening both the children and the parents talk about their feelings and experiences. Our data was accumulated through interviews with 17 adolescents and their families, using qualitative methods. The main theme at the end of the analysis was "to be strong and resistive". We reached the conclusion that this condition was not a pathological build up but an attitude of coping, as it did not cause loss of functionality. The defensive psychological mechanisms used by these adolescents consisted of repression, compensation and reaction formation. We believe that this information is important to understand the real meaning of the manners displayed when these adolescents and their families pursue their daily lives, communicate and make relationships with their environment and especially professionals in the health services

Clinically asymptomatic myocardial bridging in a child with familial subaortic stenosis

Myocardial bridging is usually seen in the setting of hypertrophic cardiomyopathy or left ventricular hypertrophy. It is rarely reported in an asymptomatic patient with an otherwise structurally normal heart. Familial subaortic stenosis is also a rare entity, and its mode of inheritance is still unknown. Here, we described the case of a 13-year-old asymptomatic girl with a positive family history of sudden cardiac death and subaortic stenosis who was diagnosed with severe myocardial bridging concomitant with familial subaortic stenosis

Generalised lymphangiomatosis in an 8-year-old girl who presented with cardiomegaly

Generalised lymphangiomatosis is a rare disease that is characterized by widespread bony and soft tissue involvement of lymphangioma. Radiological evaluation is crucial because the site and extent of the lymphangioma are important prognostic factors. The computed tomography, ultrasonography and magnetic resonance images showed sharply defined, non-enhanced cystic lesions involving the mediastinum, bones, spleen, lung, and lower neck. We report here a case that was referred to us for investigation of marked cardiomegaly

Clinically asymptomatic myocardial bridging in a child with familial subaortic stenosis

Myocardial bridging is usually seen in the setting of hypertrophic cardiomyopathy or left ventricular hypertrophy. It is rarely reported in an asymptomatic patient with an otherwise structurally normal heart. Familial subaortic stenosis is also a rare entity, and its mode of inheritance is still unknown. Here, we described the case of a 13-year-old asymptomatic girl with a positive family history of sudden cardiac death and subaortic stenosis who was diagnosed with severe myocardial bridging concomitant with familial subaortic stenosis

Modified myocardial performance index and its prognostic significance for adverse perinatal outcome in early and late onset fetal growth restriction

© 2018, © 2018 Informa UK Limited, trading as Taylor & Francis Group.Objective: To compare the fetal modified myocardial performance index (Mod-MPI) in appropriately grown, early (EO) and late onset (LO) fetal growth restricted (FGR) fetuses and to assess its prognostic significance for adverse perinatal outcome. Study design: In a prospective case-control study, Mod-MPI was performed in 22 and 51 fetuses with EO and LO-FGR fetuses, respectively. Mod-MPI values of FGR fetuses were compared against gestation-matched controls (34 for EO-, and 32 for LO-FGR, respectively). Correlation testing related with poor perinatal outcomes were performed. Results: Incidences of pathologic uterine artery Doppler rate, cesarean section rate, 5-min Apgar score < 7, neonatal intensive care unit (NICU) admission and perinatal mortality were significantly higher in the EO-FGR group (p <.001). There was a decrease in Mod-MPI with gestational age in the normal (Pearson’s r = 0.401, p <.001), and growth-restricted fetuses (Pearson’s r = 0.248, p =.034). Mean Mod-MPI values were significantly higher in both EO- and LO-FGR group than gestation-matched controls (p <.001). There was no significant correlation between Mod-MPI values and perinatal deaths (Pearson’s r = 0.004, p =.987) and 5-min Apgar score < 7 (Pearson’s r = 0.391, p =.088) in the EO-FGR fetuses. There was a significant negative correlation between Mod-MPI values and cerebroplacental ratio (CPR) values (Pearson’s r = −0.288, p =.041); however no significant correlation between Mod-MPI values and 5-min Apgar score< 7, and fetal distress during labor (Pearson’s r = 0.149, p = 0.297) in the LO-FGR fetuses was noted. Conclusion: EO and LO-FGR fetuses have significantly higher Mod-MPI values, demonstrating prenatal cardiac dysfunction. Evaluating Mod-MPI is not so effective in predicting poor perinatal outcome in both EO and LO-FGR fetuses

Heart rate variability in patients with atrial septal defect and healthy children

Objective: Heart rate variability (HRV) measures are altered in various cardiac and non-cardiac situations in children. The autonomic nervous system is assumed to have a role in the pathophysiology of atrial septal defect (ASD). In this study, we evaluated the autonomic system by measuring HRV in children with ASD

Aneurysmal formation in the setting of muscular ventricular septal defects

Objective: To emphasize the formation of fibrous aneurysmal structures in the setting of muscular ventricular septal defects Methods: Among patients with muscular ventricular septal defects followed in our institute, we described six who were diagnosed with formation of fibrous aneurysms. Of the patients, one was female and others were male. The age at diagnosis ranged from eight days to 16 years. In one, the defect opened between the ventricular inlets, but in the others the defects occupied the middle part of the apical trabecular septum. Results: The sizes of defects at initial echocardiographic evaluation ranged from 2.9 to 8 millimetres. The period of follow up for four patients was from 5 months to 7 years, one patient could not be followed up, and the other had already been diagnosed when first seen in our Institution. In our second and third patients, no aneurysm was seen during the initial echocardiographic evaluation, and it was recognized during follow-up. In these two patients, sequential echocardiographic examinations showed that the formation of the aneurysm made the defect smaller, with it almost closing in one. Conclusion: Although seen most frequently in the setting of perimembranous defects, fibrous aneurysms can also form adjacent to, and reduce the size of, muscular ventricular septal defects