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1.
Front Cardiovasc Med ; 11: 1391434, 2024.
Article in English | MEDLINE | ID: mdl-38836067

ABSTRACT

Introduction: In children, congenital heart defects represent the primary cause of increased serum troponin I. The elimination process of cardiac troponin I from the bloodstream and the factors influencing this process remain unknown. The objective of this study was to explore the role of troponin I as an indicator of cardiac damage in children both in serum and urine, a concept previously investigated in adults. Methods: Our prospective study involved 70 children under 24 months of age. The first group underwent ventricular septal defect repair, while the second group involved children who had undergone partial cavopulmonary anastomosis. For these groups, urine and serum troponin I were assessed on four occasions. The third group, consisting of healthy children, underwent a single measurement of urine troponin I. Results: Serum troponin I values exhibited an expected elevation in the early postoperative period, followed by a return to lower levels. Significantly higher concentrations of serum troponin I were observed in the first group of children (p < 0.05). A positive correlation was found between troponin I in the first three measurements and cardiopulmonary bypass and aortic cross-clamping time. There was no discernible increase in urine troponin I directly related to myocardial damage; troponin I couldn't be detected in most urine samples. Discussion: The inability to detect troponin I in urine remains unexplained. Potential explanatory factors may include the isoelectric point of troponin I, elevated urinary concentrations of salts and urea, variations in urine acidity (different pH levels), and a relatively low protein concentration in urine.

2.
Perfusion ; 38(5): 1002-1011, 2023 07.
Article in English | MEDLINE | ID: mdl-35543369

ABSTRACT

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) is an important treatment option for organ support in respiratory insufficiency, cardiac failure, or as an advanced tool for cardiopulmonary resuscitation. Reports on pediatric ECMO use in our region are lacking. METHODS: This study is a retrospective review of all pediatric cases that underwent a veno-arterial (VA) or veno-venous (VV) ECMO protocol between November 2009 and August 2020 at the Department of Pediatrics, University Hospital Center Zagreb, Croatia. RESULTS: Fifty-two ECMO runs identified over the period; data were complete for 45 cases, of which 23 (51%) were female, and median age was 8 months. Thirty-eight (84%) patients were treated using the VA-and 7 (16%) using VV-ECMO. The overall survival rate was 51%. Circulatory failure was the most common indication for ECMO (N = 38, 84%), and in 17 patients ECMO was started after cardiopulmonary resuscitation (E-CPR). Among survivors, 74% had no or minor neurological sequelae. Variables associated with poor outcome were renal failure with renal replacement therapy (p < .001) and intracranial injury (p < .001). CONCLUSION: Overall survival rate in our cohort is comparable to the data published in the literature. The use of hemodialysis was shown to be associated with higher mortality. High rates of full neurological recovery among survivors are a strong case for further ECMO program development in our institution.


Subject(s)
Extracorporeal Membrane Oxygenation , Heart Failure , Humans , Child , Female , Infant , Male , Extracorporeal Membrane Oxygenation/methods , Croatia , Retrospective Studies , Survival Rate
3.
Jpn J Radiol ; 41(5): 477-487, 2023 May.
Article in English | MEDLINE | ID: mdl-36495370

ABSTRACT

Cardiac tumors are neoplasms arising from or located in the heart or the pericardium. Although rare, primary cardiac tumors in children require an accurate and timely diagnosis. Most pediatric primary cardiac tumors are benign (around 90%). Echocardiography is the first imaging modality used due to its availability, noninvasiveness, inexpensiveness, and absence of ionizing radiation. Computed tomography (CT) and magnetic resonance imaging (MRI) offer better soft tissue visualization as well as better visualization of extracardiac structures. A great advantage of MRI is the possibility of measuring cardiac function and blood flow, which can be important for obstructing cardiac tumors. In this article, we will offer a brief review of clinical, echocardiographic, CT, and MRI features of cardiac rhabdomyomas, fibromas, teratomas, and lipomas providing their differential diagnosis.


Subject(s)
Heart Neoplasms , Child , Humans , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/pathology , Echocardiography , Tomography, X-Ray Computed , Magnetic Resonance Imaging , Radiologists
4.
JTCVS Open ; 11: 373-387, 2022 Sep.
Article in English | MEDLINE | ID: mdl-36172411

ABSTRACT

Objectives: Severe hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS. Methods: The medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed. Results: Of 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (<90 days) with PA hypoplasia (n = 3), mediastinitis/pneumonia (n = 3), pulmonary venous obstruction (n = 2), ventricular dysfunction (n = 1), and recurrent pneumothorax (n = 1). Four patients experienced hospital deaths. Six patients died after discharge, 10 achieved Fontan completion, and 6 were alive and waiting for Fontan. Overall survival after partial takedown was 54% at 3 years. The pulmonary venous obstruction (P = .041) and genetic/extracardiac anomalies (P = .085) were identified as risks for mortality after partial takedown. Conclusions: The partial takedown resulted in a 3-year survival rate of more than 50%. Of these patients, a significant number underwent successful Fontan completion who would exhibit potential early death with conservative treatment.

5.
Semin Thorac Cardiovasc Surg ; 34(4): 1300-1310, 2022.
Article in English | MEDLINE | ID: mdl-34838954

ABSTRACT

The study objective was to compare the results after Norwood procedure between modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery conduit (RVPAC) according to Sano in patients with hypoplastic left heart syndrome (HLHS) and aortic atresia (AA). A total of 146 neonates with HLHS and AA who underwent the Norwood procedure at our institution between 2001 and 2020 were divided into 2 groups according to shunt type (MBTS or RVPAC). Survival after the Norwood procedure was compared between the groups. Longitudinal right ventricular and tricuspid valve function in each group were evaluated using cubic splines method. RVPAC was performed in 103 patients and MBTS in 43 according to surgeon preference. There were no differences in the 30-day mortality rates (16.5% vs 16.3%, P  = 0.973). Survival at 0.5, 1 and 3 years was 79.6%, 74.6%, and 68.9% in RVPAC and 66.8%, 64.3%, and 58.5% in MBTS (P  =  0.293). Among 23 patients undergoing tricuspid valve procedure, different mechanisms of tricuspid regurgitation were observed between the groups. Longitudinal analysis revealed greater prevalence of late right ventricular dysfunction in RVPAC patients. In 77 patients who completed Fontan procedure, the postoperative N-terminal pro B-type natriuretic peptide value was significantly higher in RVPAC vs MBTS (554 vs 276 ng/L, P  =  0.007). No survival advantage of RVPAC over MBTS was observed in neonates with HLHS and AA undergoing the Norwood procedure. Longitudinal analysis demonstrated a greater prevalence of right ventricular dysfunction and higher N-terminal pro B-type natriuretic peptide values during late follow-up in patients with RVPAC.


Subject(s)
Aortic Diseases , Blalock-Taussig Procedure , Hypoplastic Left Heart Syndrome , Norwood Procedures , Ventricular Dysfunction, Right , Infant, Newborn , Humans , Natriuretic Peptide, Brain , Treatment Outcome , Hypoplastic Left Heart Syndrome/diagnostic imaging , Hypoplastic Left Heart Syndrome/surgery , Blalock-Taussig Procedure/adverse effects , Pulmonary Artery/diagnostic imaging , Pulmonary Artery/surgery , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery
6.
J Pediatr Endocrinol Metab ; 34(3): 389-393, 2021 Mar 26.
Article in English | MEDLINE | ID: mdl-33180048

ABSTRACT

OBJECTIVES: Hyperammonemia in a newborn is a serious condition, which requires prompt intervention as it can lead to severe neurological impairment and death if left untreated. The most common causes of hyperammonemia in a newborn are acute liver failure and inherited metabolic disorders. Several mitochondrial disorders have been described as a cause of severe neonatal hyperammonemia. CASE PRESENTATION: Here we describe a new case of adenosine-triphosphate (ATP) synthase deficiency due to m.8528T>C mutation as a novel cause of severe neonatal hyperammonemia. So far six patients with this mutation have been described but none of them was reported to need hemodialysis in the first days of life. CONCLUSION: This broadens the so far known differential diagnosis of severe neonatal hyperammonemia requiring hemodialysis.


Subject(s)
Hyperammonemia/genetics , Mutation , Proton-Translocating ATPases/genetics , Renal Dialysis , Diagnosis, Differential , Female , Humans , Hyperammonemia/diagnosis , Hyperammonemia/therapy , Infant, Newborn , Proton-Translocating ATPases/deficiency
7.
Perfusion ; 36(3): 305-307, 2021 04.
Article in English | MEDLINE | ID: mdl-32762308

ABSTRACT

A 2-week-old male newborn with a double inlet left ventricle developed a cardiac arrest following modified Blalock-Taussig anastomosis in pediatric intensive care unit. Probable causes of the arrest were hemodynamic instability and thrombosed shunt, which was later recanalized on extracorporeal membrane oxygenation therapy, which was successfully used with a pump flow lower than recommended in these patients-without the shunt clip, but without any complications.


Subject(s)
Blalock-Taussig Procedure , Cardiopulmonary Resuscitation , Extracorporeal Membrane Oxygenation , Heart Arrest , Anastomosis, Surgical , Blalock-Taussig Procedure/adverse effects , Child , Heart Arrest/etiology , Heart Arrest/therapy , Humans , Infant, Newborn , Male
8.
Cardiol Young ; 29(6): 837-839, 2019 Jun.
Article in English | MEDLINE | ID: mdl-31169113

ABSTRACT

A neonate presented with signs of heart failure early after a Norwood procedure, due to increase of aortic and mitral valve regurgitation. Because repeated surgery was considered risky, we closed the aortic valve by catheter intervention with an Amplatzer Duct Occluder. Aortic regurgitation was abolished almost completely and the child improved. Unfortunately, 2 weeks after discharge, the patient died suddenly.


Subject(s)
Aortic Valve Insufficiency/surgery , Aortic Valve/surgery , Heart Valve Prosthesis Implantation/methods , Hypoplastic Left Heart Syndrome/surgery , Norwood Procedures/adverse effects , Postoperative Complications , Aortic Valve/diagnostic imaging , Aortic Valve Insufficiency/diagnosis , Aortic Valve Insufficiency/etiology , Echocardiography , Fatal Outcome , Follow-Up Studies , Humans , Infant, Newborn , Male , Reoperation , Time Factors
9.
Cardiol Young ; 28(11): 1359-1360, 2018 Nov.
Article in English | MEDLINE | ID: mdl-30064531

ABSTRACT

We report a case of primary hyperparathyroidism with hypercalcaemia in a 16-year-old boy who had spontaneous attack of ventricular tachycardia. To our knowledge, presentation of ventricular tachycardia caused by primary hyperparathyroidism in such a young patient has not been reported in medical literature so far.


Subject(s)
Electrocardiography , Hypercalcemia/complications , Hyperparathyroidism, Primary/complications , Tachycardia, Ventricular/etiology , Adolescent , Adrenergic beta-Agonists/therapeutic use , Calcium/blood , Humans , Hypercalcemia/blood , Hypercalcemia/diagnosis , Hyperparathyroidism, Primary/diagnosis , Hyperparathyroidism, Primary/surgery , Isoproterenol/therapeutic use , Male , Parathyroid Glands/diagnostic imaging , Parathyroidectomy , Tachycardia, Ventricular/drug therapy , Tachycardia, Ventricular/physiopathology , Ultrasonography
10.
Pediatr Pulmonol ; 51(12): 1320-1329, 2016 12.
Article in English | MEDLINE | ID: mdl-27228382

ABSTRACT

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia. CDH survivors may have pulmonary morbidity that can decrease cardiopulmonary exercise. We aimed to examine whether cardiopulmonary exercise testing (CPET) results differ in CDH survivors versus healthy age-matched controls and whether CPET results among CDH survivors differ according to self-reported daily activity. METHODS: In one medical center in Croatia, CDH survivors-patients with surgically corrected CDH who were alive at age 5 years-were invited to participate in spirometry and CPET. Values were compared with those of controls matched 2:1 by age and sex for each CDH survivor aged 7 years or older. RESULTS: Among 27 CDH survivors aged 5-20 years, 13 (48%) had continued symptoms or spirometric evidence of pulmonary disease. Compared with controls (n = 44), survivors (n = 22) had lower peak oxygen consumption (V˙O2 mean [SD], 35.7 [6.9] vs. 45.3 [8.2] ml/kg per min; P < 0.001). At peak exercise, V˙O2/heart rate (P < 0.001), tidal volume (P = 0.005), and minute ventilation (P < 0.001) were lower in survivors, but the maximal respiratory rate was not different (P = 0.72). Among survivors, mean (SD) V˙O2peak (ml/kg per min) differed by self-reported activity level: athletic, 40.3 (5.0); normal, 35.8 (6.5); and sedentary, 32.1 (6.8) (by ANOVA, P = 0.10 across three groups and P = 0.04 athletic vs. sedentary). CONCLUSION: More than half of CDH survivors continue to have chronic pulmonary disease. CDH survivors had lower aerobic exercise capacity than controls. Self-reporting information on daily activities may identify CDH patients with low V˙O2max who may benefit from physical training. Pediatr Pulmonol. 2016;51:1320-1329. © 2016 Wiley Periodicals, Inc.


Subject(s)
Exercise Tolerance/physiology , Hernias, Diaphragmatic, Congenital/physiopathology , Lung Diseases/physiopathology , Lung/physiopathology , Oxygen Consumption/physiology , Survivors , Adolescent , Case-Control Studies , Child , Child, Preschool , Chronic Disease , Croatia , Exercise , Exercise Test , Female , Heart Rate , Hernias, Diaphragmatic, Congenital/complications , Hernias, Diaphragmatic, Congenital/surgery , Humans , Lung Diseases/etiology , Male , Spirometry , Young Adult
11.
Lijec Vjesn ; 137(1-2): 9-17, 2015.
Article in Croatian | MEDLINE | ID: mdl-25906542

ABSTRACT

Our study is a clinical epidemiological retrospective analysis of coarctation of the aorta in a 10-year follow-up (2001-2011). The study includes 201 children, 72 (35.82%) girls and 129 (64.18%) boys (1:1,6), with an average age of 28.57-49.37 mo (0.1-204 mo). They are categorized in 4 age groups: <1 month, 1 month - 1 year, 1 year - 6 years, >6 years. Isolated coarctation of the aorta was found in 125 (62.19%) patients; 33 (16.42%) preductal and 92 (45.77%) postductal. Coarctation of the aorta with an additional heart defect was found in 76 (37.81%) patients; 32 (15.32%) with ventricular septal defect, 28 (13.93%) within a complex heart defect, 11 (5.47%) within the Shone syndrome, and 5 (2.49%) with a dilated cardiomyopathy. Tiredness, intense tachypneic and dyspneic difficulties are dominant features in lower age groups (newborns and infants), while claudications, headaches and epistaxis are typical in older children. In the case of as many as 61 (30.35%) patients diagnosis was missed on the previous cardiological examination. In 20 (9.95%) patients coarctation is found within the known syndromes (Turner, Noonan, Williams Beuren, Ellis van Creveld, Down, partial trisomy 18, fetal valproate syndrome). Echocardiography was performed in all patients, and in 45 (22.38%) it was the only diagnos tic procedure. Altogether, 123 heart catheterizations, 38 multislice computed tomography and 15 magnetic resonance imagings were performed. The gradient on the place of coarctation before surgery or emergency procedures measured by catheter in 132 (65.67%) patients was 57.99 +/- 18.68 mmHg (20-100 mmHg). In 82 (40.80%) patients a bicuspid aortic valve was found. Average age at the time of surgery was 27.92 +/- 47.98 months (0.1-204 mo.). In 169 (84.07%) patients a cardiosurgical intervention was performed; 109 (54.23%) T-T anastomoses, 30 (14.29%) therapeutic catheterisations (balloon dilatation or stent implantation). Fatal outcome occurred in 4 (1.99%) children, all newborns or infants.


Subject(s)
Aortic Coarctation/diagnosis , Aortic Coarctation/surgery , Aortic Coarctation/epidemiology , Aortic Valve/abnormalities , Bicuspid Aortic Valve Disease , Cardiac Catheterization , Child , Child, Preschool , Comorbidity , Epidemiologic Studies , Female , Follow-Up Studies , Heart Defects, Congenital/epidemiology , Heart Valve Diseases/epidemiology , Humans , Infant , Infant, Newborn , Male , Retrospective Studies , Treatment Outcome
12.
Cardiol Young ; 25(2): 274-80, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25647467

ABSTRACT

OBJECTIVE: By employing the widely used and accepted methodologies of case-mix complexity adjustment in congenital cardiac surgery, we tried to evaluate our performance and use the ABC scores for a case complexity selection that may have different outcomes in various centres. METHODS: We analysed outcomes of cardiac surgical procedures - with or without cardiopulmonary bypass - performed in our institution between January, 2008 and December, 2011. Data were collected from the European Association for Cardio-Thoracic Surgery database. Together with prospective collection of these data, the data of all patients sent abroad to foreign cardiosurgical centres were recorded. RESULTS: During the period of study, 634 operations were performed; among them, 60% were performed in Croatia and 40% in foreign cardiosurgical centres. The number of operations performed in Croatia showed a linear increase: 55, 78, 121, and 126 operations performed in the years 2008, 2009, 2010, and 2011, respectively. Early mortality rates were 1.82%, 5.41%, 3.64%, and 3.48% in 2008, 2009, 2010, and 2011, respectively. The increase in the number of operations was followed by a satisfactory low average mortality rate of 3.85%. The mean ABC score complexity for operations performed in Croatia was 5.77. We determined a linear correlation between ABC score and early mortality, especially for the more complex operations. CONCLUSION: The use of standardised risk scores allows selection of complex cardiac diseases, which may have very different outcomes in various centres. In our case, those with higher ABC scores were correctly identified and referred for treatment abroad. In this way, we allowed gradual progress of the cardiosurgical model in Croatia and maintained an enviably low mortality rate.


Subject(s)
Cardiac Surgical Procedures/mortality , Developing Countries , Heart Defects, Congenital/surgery , Adolescent , Child , Child, Preschool , Croatia/epidemiology , Female , Humans , Infant , Infant, Newborn , Male , Outcome Assessment, Health Care , Risk Adjustment , Risk Assessment
13.
Lijec Vjesn ; 137(11-12): 348-56, 2015.
Article in Croatian | MEDLINE | ID: mdl-26975063

ABSTRACT

AIM: 1. To present an epidemiological (population and clinical) study of congenital heart defects (CHD) in Croatia in a 16-year period (1995-2011). 2. To analyze outcomes of surgical procedures for all patients in a five-year period (2002-2007) and to compare the results between Croatian and foreign centers. 3. To present the progress in surgical care of CHD in Croatia while acknowledging the requirement of achieving postsurgical mortality rate of below 5%. 4. To evaluate the projection of positive development of CHD management in Croatia in cooperation with major cardiac surgical centers in the neighboring countries. METHODS: Population study includes all children born from 1995 to 2000 and from 2002 to 2007 included in a database modeled by EUROCAT and BWIS. Outcome analysis was made using two models (ABC and RACHS-1) with early mortality rating and prolonged length of stay. Results: Based on two separate studies, mean value of CHD prevalence in Croatia is 7.6 per thousand. Outcome analysis according to the two mentioned evaluation models for the 2002-2007 period shows that children operated on in Croatia had a lower level of complexity compared to the complexity of those sent to foreign centers, but early mortality was below the assigned margin of 5% and there were also no differences in prolonged length of stay. As much as 63% of surgeries in the period were performed abroad, while the remaining 37% were performed in Croatia (351:202). In the following four-year period (2008-2011) there was a significant increase in the number of surgeries performed in Croatia when compared to those performed abroad (59:4% or 380:264). Independent EACTS analysis points to a positive trend of gradual increase in the number of surgeries and acceptance of higher complexity level of surgeries performed in Croatia, while maintaining the assigned margin (early mortality below 5%). Contribution: Proper selection of patients according to the complexity of cardiac surgical procedure is a prerequisite for both low mortality and fewer postsurgical complications. Professional advance relying on close cooperation with foreign centers is much faster and more acceptable than by "learning curves". CONCLUSION: Pediatric cardiac surgery in the developing countries must rely on the experiences of developed cardiac surgical centers in the neighboring countries due to complexity of congenital heart defects. Pediatric cardiology is inherently a public health problem, but the problem exacerbates with the appearance of a large number of adults with congenital heart defects (GUCH patients).


Subject(s)
Cardiac Surgical Procedures/statistics & numerical data , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/surgery , Patient Outcome Assessment , Adolescent , Child , Child, Preschool , Croatia/epidemiology , Heart Defects, Congenital/etiology , Heart Defects, Congenital/mortality , Humans , Infant , Infant, Newborn , Prevalence
14.
Reumatizam ; 62(2): 22-6, 2015.
Article in Croatian | MEDLINE | ID: mdl-26882800

ABSTRACT

Vasculitides are rare rheumatic diseases of unknown etiology whose main characteristic is a necrotizing inflammation of blood vessels. We are presenting two patients with Takayasu arteritis (TA) as entity forms of rare rheumatic diseases. One patient had TA type IIa and the other type IV. In the first patient we found severe symptoms of obstructive lesions of aortic branches, particularly severe coronary artery stenosis and complete occlusion of the left subclavian artery, and thoracic artery stenosis below the isthmus. The disease was diagnosed in the acute phase, treated extensively with medicaments (glucocorticoids, cytostatics, methotrexate) and a complex cardiac surgical procedure, and due to relapse the biological (Rituximab) therapy was used. The second patient was detected following symptomatic arterial hypertension, with absent pulses of lower limbs, whose cause was found in severe narrowing of the aorta from diaphragm to femoral arteries bifurcation (mid-aortic syndrome). The disease was not active when diagnosis was made. The patient was treated with a particular cardiac surgical procedure and with multiple medicaments due to a relapse. Both patients have reached adolescent age and are successfully treated with a satisfying quality of life. Type IIa with an additional occlusion of coronary arteries is not described in the available literature. Forementioned vasculitides emphasize the importance of pediatric cardiologists and rheumatologists teamwork.


Subject(s)
Takayasu Arteritis/complications , Adolescent , Humans , Quality of Life , Takayasu Arteritis/diagnosis , Takayasu Arteritis/surgery
15.
Lijec Vjesn ; 137(9-10): 267-75, 2015.
Article in Croatian | MEDLINE | ID: mdl-26749948

ABSTRACT

UNLABELLED: Although bicuspid aortic valve (BAV) is considered the most common congenital heart defect (CHD) in adult age, with the 0.5-2% prevalence, BAV is not part of epidemiological studies of congenital heart defects (CHD) in children. Aortic valvulogenesis disorder is part of the left ventricular outflow tract (LVOT) genetic disorders which include: hypoplastic left heart syndrome (HLHS), aortic stenosis (AS) and insufficiency (AI), dilatation of the ascending aorta (DAA), coaretation of the aorta (CoA), Shone's syndrome (SS), and probably some other disorders. Our observations indicate that BAV related significant pathologic and hemodynamic changes occur in children already. In an 11-year long retrospective study (2000-2011) we have found 229 BAV patients, predominantly males (1.7). The most common BAV associated disorder was CoA (75 patients, 32.6%). Of all the children with BAV, 62.4% (143:229) had hemodynamic alterations on the aortic valve which manifested themselves as aortic stenosis and/or insufficiency. AS is mostly progressive and becomes hemodynamically relevant in childhood age already, while AI is mostly mild and rarely hemodynamically relevant. A large proportion of patients had isolated AS with DAA (21 or 14.7%), while most patients had combined AS and AI (29 or 20.3%). Due to morphological changes on the valve itself and on the adjoining defects, numerous interventional and cardiosurgical procedures have been performed. Their number has been growing with age, in accordance with the expected progression of pathological changes on the valve (AS, AI) or on the aorta (DAA). DAA in children with BAV was found in 76 (33.2%) patients, in various combinations with other associated LVOT anomalies. Already in childhood have the following surgical procedures on children with primary BAV diagnosis been performed : resection of CoA with T-T anastomosis was performed in 56 patients (24.5%); balloon aortic valvuloplasty in 28 patients (12.3%); commissurotomy in 19 patients (8.3%); balloon dilatation of CoA in 15 patients (6.5%); subaortic membrane resection in 11 patients (4.8%); Ross procedure in 8 patients (3.5%); resection of CoA with reconstruction in 8 patients (3.5%); valvuloplasty in 6 patients (2.6%); ascending aortoplasty in 5 patients (2.2%); mechanical valve replacement in 3 patients (1.3%); "subclavian flap" in 3 patients (1.3%); biological aortic valve replacement in 2 patients (0.9%); Bentall procedure in 1 patient (0.4%); David procedure in 1 patient (0.4%). CONTRIBUTION OFTHE STUDY: A BAV finding in children is a predictive factor for a progressive development of morphological changes in various LVOT parts, requiring that in some patients hemodynamic repercussions be removed already in childhood. CONCLUSION: The term valvular aortopathy, that is bicuspid aortic valve syndrome, should be in use already for children, and the anomaly should be included in epidemiological CHD research.


Subject(s)
Aortic Valve/abnormalities , Heart Valve Diseases/epidemiology , Ventricular Outflow Obstruction/epidemiology , Bicuspid Aortic Valve Disease , Child , Disease Progression , Global Health , Heart Valve Diseases/diagnosis , Humans , Prevalence , Syndrome , Ventricular Outflow Obstruction/diagnosis
16.
Lijec Vjesn ; 136(9-10): 261-8, 2014.
Article in Croatian | MEDLINE | ID: mdl-25632771

ABSTRACT

A coronary artery fistula is a link between one or more coronary arteries with another heart cavity or a segment of systemic or pulmonary circulation. Arterial blood from a coronary vessel enters another segment via myocardial capillary bed. These are very rare anomalies which constitute approximately 0.2 - 0.4% of all congenital heart defects. Still, they are clinically significant if they are of medium or large size and are manifested with a series of clinical symptoms such as angina pectoris, arrhythmias, myocardial infarction, endocarditis, progressive dilatation, heart failure and cardiomyopathy, pulmonary hypertension, thrombosis of the fistula and formation of aneurysms with possible ruptures. We present six patients with a coronary arterial fistula, their history, diagnostic procedures and outcomes. Therapeutic closure of coronary artery fistulas is recommended in all symptomatic, but also in asymptomatic patients, if there are significant roentgenographic, electrocardiographic and other abnormalities. In recent times transcatheter closure of coronary fistulas has become a possible alternative to surgery and is becoming increasingly used thanks to improved diagnostic possibilities and technology. If possible, interventional closure of fistulas is precisely the method preferred in pediatric patients. The choice of method depends on the anatomy of the fistula, presence or absence of additional defects, and on the experience of an interventional cardiologist or a heart surgeon. If performed well, the effects of both methods are good. This paper presents two children with a fistula between the right coronary artery and the right ventricle (RV), one child with a fistula between LAD and RV, one child with a fistula between the main tree of the left coronary artery (LCA) and RV, one child with a fistula between LCA and the right ventricular outflow tract (RVOT), and one child with a fistula between LCA and the right atrium (RA). The last one (LCA-RA) is not described in the latest classification of anomalies of coronary blood vessels in children based on MSCT coronarography, so we consider our presentation to be a contribution to the new classification. Along with the descriptions of fistulas and presentations of interventional and cardiosurgical interventions, we are also presenting a rare case of spontaneous closing of the fistula within the first six months and of a reopening of the fistula between the right coronary artery and the right ventricle after six years.


Subject(s)
Cardiovascular Surgical Procedures/methods , Coronary Vessel Anomalies , Coronary Vessels/pathology , Embolization, Therapeutic/methods , Heart Atria/diagnostic imaging , Heart Ventricles/diagnostic imaging , Vascular Fistula , Child , Child, Preschool , Coronary Angiography/methods , Coronary Vessel Anomalies/complications , Coronary Vessel Anomalies/diagnosis , Coronary Vessel Anomalies/physiopathology , Coronary Vessel Anomalies/surgery , Electrocardiography/methods , Female , Humans , Infant , Infant, Newborn , Male , Outcome Assessment, Health Care , Ultrasonography , Vascular Fistula/congenital , Vascular Fistula/diagnosis , Vascular Fistula/physiopathology , Vascular Fistula/surgery
18.
Croat Med J ; 54(4): 394-6, 2013 Aug.
Article in English | MEDLINE | ID: mdl-23986282

ABSTRACT

Although closure of an atrial septal defect (ASD II) with an occluding device in the first year of life is not a routine procedure, it is a feasible treatment, even in neonates. Case reports on the off-label use of Amplatzer devices have been repeatedly published, but there are no reports on using the Amplatzer Duct Occluder (ADO) to close an atrial septal defect in a neonate. We report on a successful catheter closure of an ASD II with ADO in a severely cyanotic neonate, seven days after surgical repair of common arterial trunk. Due to progressive cyanosis and clinical signs of right ventricular failure, which developed after common arterial trunk repair, the neonate underwent cardiac catheterization. Diastolic filling impairment of the right ventricle (right ventricle hypertrophy, pulmonary regurgitation, and residual right ventricle outflow tract obstruction) was thought to be the cause of impaired right ventricle diastolic filling, resulting in the right-to-left shunt at the atrial level. Under transesophageal echocardiographic guidance, ADO was delivered through a 5 French sheath into the atrial septal defect. Amplatzer duct occluder closed the defect and proved to be stable in position after disconnection. During the procedure, the child was stable and then transferred to the intensive care unit with significantly improved oxygen saturation. This is the first report on placing a duct occluder in the atrial septal position, which is a novel procedure for-small neonates.


Subject(s)
Cardiac Catheterization , Heart Septal Defects, Atrial/surgery , Septal Occluder Device , Truncus Arteriosus, Persistent/surgery , Echocardiography, Transesophageal , Female , Heart Atria/diagnostic imaging , Heart Atria/surgery , Heart Failure/diagnostic imaging , Heart Failure/surgery , Heart Septal Defects, Atrial/diagnostic imaging , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery , Humans , Infant, Newborn , Off-Label Use , Postoperative Period , Truncus Arteriosus, Persistent/diagnostic imaging
19.
Lijec Vjesn ; 133(3-4): 81-8, 2011.
Article in Croatian | MEDLINE | ID: mdl-21612102

ABSTRACT

OBJECTIVE: The aim of our paper is to show results of population and hospital registry of congenital heart disease in Croatia. METHODS: Information on patients born during the five-year study and with diagnosis of congenital heart defects, obtained all across the country, were collected in the population and hospital registry set up according to the EUROCAT and BWIS registries principles. RESULTS: Between October 1, 2002, and October 1 2007, there were 205051 live births in Croatia, of which 1480 patients were diagnosed with congenital heart defects, accounting for 0.72% of the live-born children. The most common diagnosis was ventricular septal defect with percentage of 34.6%. Among 1480 children, 430 needed an operation. Among 553 cardiac surgeries performed, 202 were done in Croatia and others were done in institutions abroad. Mortality rate after surgery was 5%. Only after adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications between Croatia and centers abroad. CONCLUSION: The importance of the registry of congenital heart disease relies in the field of epidemiological research but can be used as a tool for future planning of health services.


Subject(s)
Heart Defects, Congenital/epidemiology , Child , Croatia/epidemiology , Female , Heart Defects, Congenital/surgery , Humans , Infant , Male , Prevalence
20.
Cardiol Young ; 20(4): 433-40, 2010 Aug.
Article in English | MEDLINE | ID: mdl-20307335

ABSTRACT

OBJECTIVE: The Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were developed and used to compare outcomes of congenital cardiac surgery. Both methods were used to compare results of procedures performed on our patients in Croatian cardiosurgical centres and results of procedures were taken abroad. METHODS: The study population consisted of all patients with congenital cardiac disease born to Croatian residents between 1 October, 2002 and 1 October, 2007 undergoing a cardiovascular operation during this period. RESULTS: Of the 556 operations, the Aristotle basic complexity score could be assigned to 553 operations and the risk adjustment in congenital cardiac surgery-1 method to 536 operations. Procedures were performed in two institutions in Croatia and seven institutions abroad. The average complexity for cardiac procedures performed in Croatia was significantly lower. With both systems, along with the increase in complexity, there is also an increase in mortality before discharge and postoperative length of stay. Only after the adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications. CONCLUSION: Both, the Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were predictive of in-hospital mortality as well as prolonged postoperative length to stay, and can be used as a tool in our country to evaluate a cardiosurgical model and recognise potential problems.


Subject(s)
Cardiac Surgical Procedures , Heart Defects, Congenital/surgery , Risk Adjustment/methods , Cardiac Surgical Procedures/adverse effects , Cardiac Surgical Procedures/mortality , Child, Preschool , Cohort Studies , Croatia , Female , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Hospital Mortality , Humans , Infant , Length of Stay , Male , Models, Cardiovascular , Outcome Assessment, Health Care , Reproducibility of Results , Retrospective Studies , Severity of Illness Index
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