Complete transposition of the great arteries - surgical results and prognosis

Objectives: The aim of this study was to analyze the surgical results and the prognosis in patients with complete transposition of the great arteries (TGA).Study design: A group of 64 children with complete TGA out of 272 patients with critical congenital heart disease (CCHD) from the region of North-East Bulgaria was retrospectively followed for a period of 25 years (1987-2011 year). Methods of investigations include: clinical examination, electrocardiography, conventional radiography, echocardiography and cardiac catheterization. Surgical results were based on a scale, elaborated by us.Results: Complete TGA is the most frequent CCHD in the region of North-East Bulgaria - 23%, and in 64.1% of the cases it was associated with other cardiac malformations. Surgical correction was performed in 41 of the children (64.1%) with TGA - an average 2.17 interventions on each patient. The overall postoperative mortality rate was 43.9% and it was highest if concomitant lesions were presented, such as pulmonary stenosis or atresia, criss-cross atrioventricular connection and others - up to 50-60%. The balloon atrioseptostomy was the most common initial palliative procedure, performed in 83% of the newborns at a mean age of 7.1days and a mortality rate - 8.8%. In 31 of the children (75.6%) a complete corrective intervention was made. The most frequent was arterial switch operation (ASO) - 16 children (51%). Six of the children died - 37.5%, but the mean age of which the surgery was performed was significantly higher - 32.2 days in contrast to 16.8 days for the whole group (p<0.05). The surgical results on the 5th year were assessed as good/very good in 4 of the children and satisfactory in 1 child. Senning procedure was performed in 8 of the patients (26%) with a mortality rate of 25%. Four of the children (13%) undergone Rastelli or REV procedure and 3 of them (10%) had one-chambered Fontan procedure.Conclusions : Complete TGA is the most frequent CCHD. The associated cardiac malformations have significant influence upon the prognosis and the surgical results. On different reasons still significantly high number of children with TGA remains without corrective interventions. The fundamental corrective procedure is ASO and the delayed intervention leads to unsatisfactory postoperative results

Therapeutic approach to idiopathic hypertrophic cardiomyopathy

Идиопатичната хипертрофична кардиомиопатия е рядко срещана в детска възраст с висок потенциален риск от фатален край. Основен отличителен белег е миокардната хипертрофия при липса на хемодинамична причина. От първия описан случай на експериментално лечение на кърмаче през 1971 г., бета-блокерите имат водеща роля в медикаментозното лечение с вариации в дозовите режими. По последни литературни данни и клинични проучвания употребата на високи дози бета-блокер неоспоримо показва значим процент на преживяемост в дългосрочен план.Представяме клиничен случай на 11-месечно кърмаче, което постъпва в нашата клиника с данни за сърдечна недостатъчност. От проведените образни изследвания - рентгенография на гръден кош и ехокардиография, се установи изразена симетрична хипертрофична необструктивна кардиомиопатия. Започна се медикаментозно лечение с пропранолол в постепенно покачваща се доза до 5мг/кг / 24часа. Няколко седмици по-късно при контролно ехокардиографско изследване се установи значително подобрение в диастолната функция на лява камера с известна регресия в хипертрофията на миокарда.Контролираното прилагане на високи дози бета-блокери би могло съществено да подобри прогнозата и дългосрочната преживяемост при пациентите с идиопатична хипертрофична кардиомиопатия.We`re presenting a clinical case of an 11-month old infant, who was admitted to our clinic with symptoms of congestive cardiac failure. The chest radiography and echocardiography results showed a severe symmetric hypertrophic cardiomyopathy without an obstruction in the left ventricular outflow tract. The treatment was started with Propranolol in a titrating dose until reaching the dose of 5mg/kg/24h. Several weeks later, echocardiography examination showed a significant improvement in the left ventricular diastolic function with a certain degree of regression of the myocardial hypertrophy.The use of high doses of beta blockers together with the monitoring of the clinical state could improve the prognosis and survival in patients with idiopathic hypertrophic cardiomyopathy.Idiopathic hypertrophic cardiomyopathy is a rare disease in childhood with a high probability of a lethal outcome. The hallmark of the disorder is myocardial hypertrophy that occurs in the absence of an obvious hemodynamic stimulus. Since the first case of an experimental treatment of an infant described in 1971, beta blockers have become one of the leading medication options with variations of the dose regimes. According to the current literature data and clinical trials, the use of beta blockers in high doses is consistent with a high percentage of survival

Diagnosis, management and prognosis of patients with critical coarctation of the aorta

The aim of this study is to analyze the clinical presentation, diagnosis, operative results and prognosis of the patients with critical coarctation of the aorta (CoA). Methods and materials: A group of 43 newborns and infants up to 3 months of age with critical CoA out of 272 patients with critical congenital heart diseases (CCHD) from the region of North-East Bulgaria were retrospectively followed for a period of 25 years (1987-2011). Methods of investigation included: clinical examination, electrocardiography, conventional radiography, echocardiography and cardiac catheterization. Corrective procedures was done in 31 of the children. Time of postoperative follow-up was an average of 6 years. Operating results are evaluated on a scale developed by us. Results: The CoA is the second most frequent CCHD after the transposition of the great arteries (TGA) - 16%, most often in conjunction with other cardiac abnormalities. The clinical presentation and the time of diagnosis of the patients were significantly delayed (ð<0,001). One third of newborns with CCHD, discharged from hospital without being diagnosed with cardiac malformation, turned out to be with CoA. Only 9.5% of our patients were diagnosed in neonatology department, versus 32.7% in other critical cardiopathies (p<0.001) and in one fourth of the children the diagnosis was incorrect or incomplete. Corrective surgical procedures were performed in 31 (72.1%) of the children - 1.6 intervention on each patient average. In 64.5% one-stage correction was performed with spontaneously recovery of the concomitant heart malformation. The preferable surgical technique was Amato procedure - resection and plastic `end-to-end`. Early postoperative mortality was 9.7%. Reoperations were performed in 6 children - 19.4 percent. The late operating results are often good or very good in 65.4% of patients. Conclusion: CoA is a common CCHD and the time of the clinical manifestation and the diagnosis are significantly delayed. In spite of the common concomitant heart anomalies in most of the patients it is possible a one-stage corrective procedure to be performed with good postoperative result

A CASE REPORT OF A CHILD WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS - A RARE BUT POTENTIALLY DANGEROUS DISEASE

Introduction:Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic juvenile idiopathic arthritis (sJIA) is a rare form of this group characterised by systemic autoinflammation. It contributes to about two-thirds of the total mortality rate of JIA and is frequently complicated by macrophage activation syndrome. Clinical manifestations of the disease can be non-specific in the beginning and as a result the diagnosis can be delayed.Materials and Methods:We present a 13-year-old boy hospitalized at the St. Marina University Hospital, Varna with complaints of a fever over 38oC accompanied by a rash on the trunk. After physical examination, hyperemic throat, erythematous rash and cervical lymphadenopathy were discovered. The laboratory tests showed leukocytes - 19.32x109/L and CRP - 203.78 mg/l. Echocardiography and computed tomography revealed pericardial effusion and splenomegaly. The patient was diagnosed with acute pericarditis and treated with antibiotics but his condition was not improving. After a month he developed symptoms such as dyspnea, cough and joint pains and was admitted urgently to the Hospital. Further examinations were performed showing hemoglobin- 114 g/l, leukocytes - 23.37x109/L and pleural effusion in addition of the previous objective symptoms.Results: Due to the clinical presentation, the patient was diagnosed with sJIA. Now he is successfully treated with tocilizumab and methotrexate. Conclusion: The clinicians should be aware of sJIA as a disease with various clinical features in the beginning and the resemblance to infectious or malignant diseases. Early diagnosis and effective treatment are essential to achieve the best outcomes for the patients and to prevent fatal complications

Abstracts Of The Proceedings And The Posters From The Third Scientific Session Of The Medical College Of Varna

October 2-3, 201