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

Echocardiographic follow-up of congenital aortic valvular stenosis

We investigated the morphology of the stenotic aortic valve, the progression of the stenosis, and the onset and progression of aortic regurgitation (AR) in patients with congenital aortic valvular stenosis (AVS). The medical records of 278 patients with AVS were reviewed, with the patients with concomitant lesions besides AR excluded. Very mild aortic stenosis was defined as a transvalvular Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25-49 mmHg, moderate stenosis as 50-75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 4.9 +/- 4.3 years (range, 3 days to 15 years), and 203 (73%) were male. The number of the cusps was determined with two-dimensional echocardiography in 266 patients (95%): unicuspid in 3 patients (1%), bicuspid in 127 patients (48%), and tricuspid in 136 patients (51%). A total of 192 of all patients were followed for 2 months to 14.6 years (mean 4.2 +/- 3.3 years) with medical treatment alone. Among 72 patients with very mild stenosis at initial echocardiographic examination, 20% had mild, 3% moderate, and 1% severe stenosis after a mean period of 3.7 years. In 70 patients with mild stenosis at initial echocardiographic examination, 28% had moderate and 9% severe stenosis after a mean period of 5 years. Among 44 patients with moderate stenosis at initial echocardiographic examination, 36% had severe stenosis after a mean period of 3.7 years. Among 192 patients, 40% had AR (3% trivial, 28% mild, and 9% moderate) at initial echocardiographic examination. After a mean period of 4.2 years, 58% of the patients had AR (13 % trivial, 25% mild, 16% moderate, and 4% severe). There was not statistically significant difference between catheterization peak systolic gradients (47 +/- 16 mmHg) and Doppler estimated mean gradients (45 +/- 9 mmHg) (p = 0.53), whereas Doppler PSIGs (74.9 +/- 15.7 mmHg) were higher than catheterization peak systolic gradients (p < 0.0001) in 25 patients who were studied in the catheterization lab. Patients with very mild stenosis may be followed with a noninvasive approach every 1 or 2 years, and an annual follow-up is suggested for patients with mild stenosis. Nearly one-third of patients with moderate stenosis at initial echocardiographic examination had severe stenosis after a mean period of 3.7 years. Therefore, we recommend, that patients with moderate stenosis undergo noninvasive evaluation every 6 months. Doppler estimated mean gradient is very useful in predicting the need for intervention in children with AVS

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

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

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