Impact of Echocardiography in Diagnosis Heart Lesion after Rheumatic Fever Disease in Kosovar Children

Background: Acute rheumatic fever and its sequels, rheumatic heart diseases, remain major unsolved preventable health problems in Kosovo population, particularly among the disadvantages indigenous Albanian and Egyptians people. In Kosovo, despite of performing secondary prophylaxis with benzathine penicillin, acute rheumatic fever hospitalization rates have remained essentially unchanged for the last 20 years. The role of echocardiography in the diagnosis of acute rheumatic carditis was established over the last 20 years. Aims In this study we aimed to determine the prevalence of rheumatic heart disease in children from Kosovo population with the first attack of acute rheumatic fever. Also, we presented that echocardiography examination detects a greater prevalence of rheumatic heart disease than other diagnostic procedures. We aimed to compare the sensitivity and specificity of cardiac auscultation, ECG record, lab analysis to echocardiography and to determine the feasibility of specific age in this setting. MethodsTo optimize accurate diagnosis of rheumatic fever and rheumatic heart disease, we utilized two group models. In the first group of 388 children who were hospitalized and treated before 1999, diagnosis of rheumatic fever was decided basing on the clinical and laboratory findingswhereas in second group (221 children treated from1999 to 2010), clinical and lab diagnosis were amplified also on the detection by echocardiography. ConclusionIn second group, using echocardiography as a method of diagnosis and assessment children with rheumatic fever, we found high rates of undetected rheumatic heart disease in this high-risk group population. Echocardiographic examination of children with rheumatic fever for rheumatic heart disease may over-diagnose rheumatic heart disease unless congenital mitral valve anomalies and physiological regurgitation are excluded.Keywords: rheumatic fever, rheumatic heart disease, chorea minor, echocardiography

Prenatally Diagnosis and Outcome of Fetuses with Cardiac Rhabdomyoma - Single Centre Experience

BACKGROUND: Cardiac rhabdomyoma (CRs) are the most common primary tumour of the heart in infants and children. Usually are multiple and, basing on the location can cause a haemodynamic disturbance, dysrhythmias or heart failure during the fetal and early postnatal period. CRs have a natural history of spontaneous regression and are closely associated with tuberous sclerosis complex (TSC). It has an association with tuberous sclerosis (TS), and in those, the tumour may regress and disappear completely, or remain consistent in size. AIM: We aimed to evaluate the prenatal diagnosis, clinical presentation and outcome of CRs and their association with TSC in a single centre. The median follow-up period was three years (range: 6 months - 5 years). MATERIAL AND METHODS: We reviewed medical records of all fetuses diagnosed prenatally with cardiac rhabdomyoma covering the period January 2010 to December 2016 which had undergone detailed ultrasound evaluation at a single centre with limited technical resources. RESULTS: Twelve fetuses were included in the study; mostly had multiple tumours and a total of 53 tumours were identified in all patients - the maximum was one fetus with16 tumours. All patients were diagnosed prenatally by fetal echocardiography. In two patient's haemodynamic disturbances during the fetal period was noted and pregnancies have been terminated. After long consultation termination of pregnancy was chosen by the parents in totally 8 cases. In four continuing pregnancies during the first year of live tumours regressed. TSC was diagnosed in all patients during the follow-up. CONCLUSIONS: Cardiac rhabdomyoma are benign from the cardiovascular standpoint in most affected fetuses. An early prenatal diagnosis may help for an adequate planning of perinatal monitoring and treatment with the involvement of a multidisciplinary team. Large tumour size, the number of tumours and localisation may cause hydrops, and they are significantly associated with poor neonatal outcome

Holt–Oram Syndrome Associated with Complex Congenital Heart Disease: A Rare Case Presentation and Literature Review

BACKGROUND: First described by Holt and Oram in 1960 in a four-generation family with atrial septal defects and thumb abnormalities, is an inherited disorder characterized by abnormalities of the upper limbs and heart. This syndrome is characterized by upper extremity malformations involving radial, thenar, or carpal bones. An abnormal carpal bone is present in all affected individuals and may be the only evidence of disease. About 75% of individuals with Holt–Oram syndrome (HOS) have a congenital heart malformation which may include an atrial or ventricular septal defect or heart block. In rare cases, the syndrome can affect other organs and systems which can be life threatening. CASE REPORT: Here, we present a newborn with clinical and radiologic features of HOS consisting of bilateral asymmetric hypoplastic thumbs, generalized brachydactyly, limited bilateral supination due to radioulnar synostosis, and associated with complex heart disease and hypoplastic tricuspid valve. CONCLUSION: In our case HOS is associated with complex congenital heart defects including atrial septal defect, ventricular septal defect with hypoplastic tricuspid valve. Based on the listed literature we didn’t find any other case where tricuspid valve was affected

Klippel – Feil Syndrome Associated with Congential Heart Disease Presentaion of Cases and a Review of the Curent Literature

First time described in 1912, from Maurice Klippel and Andre Feil independently, Klippel-Feil syndrome (synonyms: cervical vertebra fusion syndrome, Klippel-Feil deformity, Klippel-Feil sequence disorder) is a bone disorder characterized by the abnormal joining (fusion) of two or more spinal bones in the neck (cervical vertebrae), which is present from birth. Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. Most affected people have one or two of these characteristic features. Less than half of all individuals with Klippel-Feil syndrome have all three classic features of this condition.Since first classification from Feil in three categories (I – III) other classification systems have been advocated to describe the anomalies, predict the potential problems, and guide treatment decisions. Patients with Klippel-Feil syndrome usually present with the disease during childhood, but may present later in life. The challenge to the clinician is to recognize the associated anomalies that can occur with Klippel-Feil syndrome and to perform the appropriate workup for diagnosis

Echocardiography as a Predicting Method in Diagnosis, Evaluation and Assessment of Children with Subvalvar Aortic Stenosis

BACKGROUND: Obstruction to the left ventricular outflow of the heart may be above the aortic valve (5%), at the valve (74%), or in the subvalvar region (23%). These anomalies represent 3 to 6% of all patients with congenital heart defects (CHD), and it occurs more often in males (male-female ratio of 4:1).AIM: The purpose of this study was to determine the sensitivity and specificity of transthoracic echocardiography in diagnosis of discrete subaortic membrane, to determine convenient time for surgical intervention, and for identifying involvement of the aortic valve by subaortic shelf.MATERIAL AND METHODS: A retrospective review of the medical records and echocardiograms of 18 patients [14 male (77%) and 4 female (23%)] with discrete subaortic membrane, aged 11 month to 12 years, with mean age of 5 years and 3 month, diagnosed at the Pediatric Clinic in Prishtina, during the period September, 1999 and December, 2010 were done.RESULTS: Four patients, in neonatal age were operated from critical coarctation of the aorta and, initial signs of congestive heart failure were presented. 2 of them were operated in Belgrade, Serbia and 2 in Lausanne, Switzerland.CONCLUSION: In all presented patients bicuspid aortic valve was noted, but none of them subaortic membrane was registered

Clinical Characteristics of Patients with Acute Rheumatic Fever and Rheumatic Heart Disease in Kosovo, Evaluation of the Long-Term Results

Background. Rheumatic fever, also known as acute rheumatic fever (ARF), is an inflammatory disease that can involve the heart, joints, skin, and brain.[1] The disease typically develops two to four weeks after a throat infection.[2] Signs and symptoms include fever, multiple painful joints, involuntary muscle movements, and a characteristic but uncommon nonitchy rash known as erythema marginatum. Acute rheumatic fever and its sequels, rheumatic heart diseases, remain major unsolved preventable health problems in Kosovo population, particularly among the disadvantages indigenous Albanian and Egyptians people. In Kosovo, despite performing secondary prophylaxis with benzathine penicillin, acute rheumatic fever hospitalization rates have remained essentially unchanged for the last 20 years. The role of echocardiography in the diagnosis of acute rheumatic carditis was established over the last 20 years. Aims. Our study aimed to determine the prevalence of rheumatic heart disease in children from Kosovo population with the first attack of acute rheumatic fever. Also, we presented that echocardiography examination detects a greater prevalence of rheumatic heart disease than other diagnostic procedures. We aimed to compare the sensitivity and specificity of cardiac auscultation, ECG record, lab analysis to echocardiography and to determine the feasibility of specific age in this setting. Methods. To optimize accurate diagnosis of rheumatic fever and rheumatic heart disease, we utilized two group models. In the first group of 388 children who were hospitalized and treated before 1999, diagnosis of rheumatic fever was decided to base on the clinical and laboratory findings whereas in the second group (221 children treated from1999 to 2010), clinical and lab diagnosis was amplified also on the detection by echocardiography. Conclusion. In the second group, using echocardiography as a method of diagnosis and assessment children with rheumatic fever, we found high rates of undetected rheumatic heart disease in this high-risk group population. Echocardiographic examination of children with rheumatic fever for rheumatic heart disease may over-diagnose rheumatic heart disease unless congenital mitral valve anomalies and physiological regurgitation are excluded.Keywords: rheumatic fever, rheumatic heart disease, chorea minor, echocardiography

Kosovo’s Experience for Children with Feeding Difficulties after Cardiac Surgery for Congenital Heart Defect

BACKGROUND: A feeding disorder in infancy and during childhood is a complex condition involving different symptoms such as food refusal and faddiest, both leading to a decreased food intake.AIM: We aimed to assess the prevalence and predictor factors of feeding difficulties in children who underwent cardiac open heart surgery in neonatal period and infancy. We address selected nutritional and caloric requirements for children after cardiac surgery and explore nutritional interdependence with other system functions. METHODS: This was a retrospective study in a tertiary referral hospital, and prior approval from the institutional ethics committee was obtained. Information for 78 children (42 male and 36 female) was taken from patients charts. Data were analysed with descriptive statistics and logistic regression.RESULTS: From a cohort of analysed children with feeding problems we have occurred in 23% of such cases. At the time of the study, refusal to eat or poor appetite was reported as a significant problem in 19 children and subnormal height and weight were recorded in 11 children. Early neonatal intervention and reoperation were identified as risk factors for latter feeding difficulties or inadequate intake. Children with feeding problems also tended to eat less than children without feeding problems. There was a trend towards more feeding problems in patients with chromosomal abnormalities or other associated anomalies.CONCLUSION: Feeding disorder is often and a frequent long-term sequel in children after neonatal or early infancy heart surgery. Patients with chromosomal and associated anomalies who underwent multiple cardiac surgeries are at risk of developing feeding difficulties

EEG as Prognostic Argument in First Afebril Seizures

Cerebral disorder crises are sudden disorder and transition of brain function that are characterized by the appearance of motor, sensitive, vegetative or psychological symptoms depending on the area in which are discharge and tend to repeat. Clinically manifested of focal crisis, generalized tonic, clonic, floppy, with sensibilities disorder or vegetative symptomatology such as nausea, sweating, pallor, tachycardia, salivation. (4,6,7,10). The mention groups of cerebral crisis may be manifested as single or combined. Cerebral crisis manifested as seizures for practical aim are split in 1. Provoked seizures, 2.Unprovoked seizures (6,7).Keywords: cerebral disorder crisis, epileptic and nonepileptic crisis patient‟s history, cardio respiratory system, etc.

The Attitude of Parents of Children with the Crisis of Consciousness

Every disease that affects the child's health, especially those with negative prognosis has a detrimental affect on the parents and extended family. Children consciousness crises have particular impact considering, on the one hand, the nature of children and variety of the disease and on the other hand, the actual attitude that exists in our society.Keywords: Consciousness attacks, convulsions, paroxysmal attacks