Early postoperative interventional ASD-closure for severe atrial right to left shunt in a neonate with common arterial trunk

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

Uhl\u27s anomaly- a rare cause of congestive heart failure

INTRODUCTION Uhl’s anomaly is characterized by absence of the right ventricular myocardial layer. Although it appears to be of congenital origin, etiology is still uncertain. Patients usually present with congestive heart failure. Diagnosis is generally suggested by echocardiography. Medication treatment is only palliative, but surgical methods offer etiological cure

DISTRIBUTION OF CONGENITAL HEART DISEASE IN CROATIA AND OUTCOME ANALYSIS A Croatian epidemiological study (2002–2007)

prikazati populacijski i hospitalni registar prirođenih srčanih grješaka za Hrvatsku. Metode: Tijekom 5 godina ispunjavali smo upitnik s pitanjima o populacijskom kretanju prirođenih srčanih grješaka (prema preporukama BWIS i EUROCAT study) i hospitalni registar kojim smo pratili liječenje i ishod liječenja u djece koja su u spomenutom razdoblju rođena u Hrvatskoj. Rezultati: Od 1. 10. 2002 do 1. 10. 2007. u Hrvatskoj je rođeno 205917-ero djece, od čega je 1480-ero djece imalo prirođenu srčanu grješku (7,2 promila). Najčešće postavljena dijagnoza je VSD (34,6%). Na kardiokirurško liječenje upućeno je 430-ero djece. Na njima je učinjeno 556 operacija, od čega 202 u Hrvatskoj, ostatak u inozemstvu. Ukupni mortalitet nakon kardiokirurškog liječenja iznosi 5%. Razlike u mortalitetu i morbiditetu nakon kardiokirurških operacija izvedenih u Hrvatskoj i onih u inozemstvu uočavaju se nakon analize ishoda liječenja prema dogovorenim metodologijama. Zaključak: Registar prirođenih srčanih grješaka čini osnovu za epidemiološka istraživanja, no i za razvoj smjernica u struci.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 205 051 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

DISTRIBUTION OF CONGENITAL HEART DISEASE IN CROATIA AND OUTCOME ANALYSIS A Croatian epidemiological study (2002–2007)

prikazati populacijski i hospitalni registar prirođenih srčanih grješaka za Hrvatsku. Metode: Tijekom 5 godina ispunjavali smo upitnik s pitanjima o populacijskom kretanju prirođenih srčanih grješaka (prema preporukama BWIS i EUROCAT study) i hospitalni registar kojim smo pratili liječenje i ishod liječenja u djece koja su u spomenutom razdoblju rođena u Hrvatskoj. Rezultati: Od 1. 10. 2002 do 1. 10. 2007. u Hrvatskoj je rođeno 205917-ero djece, od čega je 1480-ero djece imalo prirođenu srčanu grješku (7,2 promila). Najčešće postavljena dijagnoza je VSD (34,6%). Na kardiokirurško liječenje upućeno je 430-ero djece. Na njima je učinjeno 556 operacija, od čega 202 u Hrvatskoj, ostatak u inozemstvu. Ukupni mortalitet nakon kardiokirurškog liječenja iznosi 5%. Razlike u mortalitetu i morbiditetu nakon kardiokirurških operacija izvedenih u Hrvatskoj i onih u inozemstvu uočavaju se nakon analize ishoda liječenja prema dogovorenim metodologijama. Zaključak: Registar prirođenih srčanih grješaka čini osnovu za epidemiološka istraživanja, no i za razvoj smjernica u struci.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 205 051 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

Successful surgical management of total anomalous pulmonary ve-nous return in a pediatric patient

Total Anomalous Pulmonary Venous Return (TAPVR) is an infrequent congenital heart de-fect, occurring in about 1 in 15,000 live births. The condition involves an abnormal con-nection where pulmonary veins, rather than attaching to the left atrium, drain into the right atrium or systemic venous circulation

Referentne vrijednosti kardiopulmonalnog testa opterećenja u djece i adolescenata u sjeverozapadnoj Hrvatskoj

The already published reference values for cardiopulmonary exercise test (CPET) might not be representative for our population. Our aim is to create reference values for CPET in children and adolescents in northwest Croatia. Exercise testing according to modifi ed Bruce treadmill protocol was performed in 164 healthy children aged 11-17 years. Results are presented as mean and standard deviation. Mann-Whitney test was used for continuous variables without normal distribution. Boys reached signifi cantly higher peak oxygen consumption (VO2) at anaerobic threshold (AT) with mean 37.13±8.9 mL/kg/min compared with 31.95±6.6 mL/kg/ min in girls. The same fi nding was recorded for VO2 at peak exercise (peak VO2) where boys reached signifi cantly higher peak VO2 of 51.3±8.9 as compared with 41.49±6.51 mL/kg/min in girls. During incremental work, systolic pressure increased from mean 109.86±14.2 mm Hg to 145.43±11.9 mm Hg, with no signifi cant diff erence between girls and boys. During incremental work, together with linear increase in heart rate and systolic pressure, oxygen pulse that represents cardiac output, showed linear increase with signifi cantly higher values in boys. Compared with girls, boys had signifi cantly higher tidal volume at rest and reached signifi - cantly higher peak minute ventilation. This study has comprehensively provided a reference set of data for the most important cardiopulmonary variables in a population of healthy children and adolescents in northwest Croatia. Our fi ndings showed strong correlation with previous reports and gave a basis for further research.Referentne vrijednosti kardiopulmonalnog odgovora dobivenog spiroergometrijskim testiranjem djece i mladih ranije su objavili inozemni autori i vrijednosti vježbanja su tipične za određenu populaciju. Naš cilj je izraditi referentne vrijednosti kardiopulmonalnog odgovora u tijeku vježbanja u populaciji sjeverozapadnog dijela Hrvatske. Spiroergometrijsko testiranje prema modifi ciranom Bruce protokolu na traci u 164-ero ispitanika u dobi od 11 do17 godina. Rezultati su izneseni kao srednje vrijednosti sa standardnim devijacijama. Mann-Whitneyjev test primijenjen u ocjeni varijabli koje nisu imale normalnu distribuciju. Dječaci su postigli značajno višu potrošnju kisika (VO2) u točki anaerobnog praga (AT) sa srednjom vrijednošću 37,13 ± 8,9 ml/kg/min u odnosu na djevojčice koje su postigle 31,95 ± 6,6 ml/kg/min. Jednako tako dječaci su postigli značajno višu vršnu potrošnju kisika 51,3 ± 8,9 u odnosu na 41,49 ± 6,51 ml/kg/min kod djevojčica. Tijekom porasta rada sistolički tlak je rastao sa 109,86 ± 14,2 mmHg do 145,43 ± 11,9 mmHg u vršnom vježbanju, bez značajnih razlika među spolom. Porastom opterećenja, zajedno s linearnim porastom srčane frekvencije i sistoličkog tlaka, bilježi se i linearan porast srčanog izbačaja dobivenog neinvazivnim mjerenjem temeljenim na potrošnji kisika i Fickovom načelu. Postignute vršne vrijednosti srčanog izbačaja značajno su više kod dječaka. U odnosu na djevojčice dječaci su imali značajno veći volumen udisaja u mirovanju te su postigli značajno veću minutnu ventilaciju u vršnom vježbanju. Prikazani su parametri kardiopulmonalnog i metaboličkog odgovora u tijeku spiroergometrijskog testiranja i iznesene referentne vrijednosti. Dobivene vrijednosti ne razlikuju se značajno u odnosu na druge studije i temelj su za daljnja istraživanja

Bikuspidalna aortalna valvula i grješke izlaznog trakta lijeve klijetke u djece – sindrom bikuspidalne aortopatije? [Bicuspid aortic valve and left ventricular outflow tract defects in children - syndrome of bicuspid aortopathy?]

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

BICUSPID AORTIC VALVE AND LEFT VENTRICULAR OUTFLOW TRACT DEFECTS IN CHILDREN – SYNDROME OF BICUSPID AORTOPATHY?

Bikuspidalna aortalna valvula (BAV) epidemiološki se ne uvrštava u studije prirođenih srčanih grješaka (PSG) u djece iako se u odrasloj dobi smatra najčešćim PSG-om, s prevalencijom od 0,5 do 2%. Poremećaj aortalne valvulogeneze posljedica je genski uvjetovanih promjena u izlaznom dijelu lijeve klijetke (LVOT) u koje spadaju: sindrom hipoplastičnoga lijevog srca (HLHS), aortalna stenoza (AS) i insuficijencija (AI), dilatacija uzlazne aorte (DAA), koarktacija aorte (CoA), Shoneov sindrom (SS), a vjerojatno i neki drugi poremećaji. Mi smo zapazili da su uz BAV vezane znatne patomorfološke promjene već u dječjoj dobi. U retrospektivnu studiju dugu 11 godina (od 2002. do 2012.) uključeno je 229-ero djece s BAV-om, s predominacijom muških (1,7). Najčešća grješka pridružena BAV-u bila je CoA (75 bolesnika ili 32,6%). Od djece s BAV-om njih 62,4% (143 : 229) imalo je hemodinamske promjene na aortalnoj valvuli koje se očituju kao aortalna stenoza i/ili insuficijencija. AS je većinom progresivan i postaje već u dječjoj dobi hemodinamski važan u dijela bolesnika, a AI je većinom blag i rijetko hemodinamski važan.Velik broj bolesnika imao je izolirani AS uz DAA (21 ili 14,7%), a najveći broj imao je kombinaciju AS-a i AI (29 ili 20,3%). Zbog morfoloških promjena na samoj valvuli i pridruženim grješkama učinjeni su brojni intervencijski i kardiokirurški zahvati. Njihov broj rastao je s dobi, sukladno očekivanoj progresiji patoloških promjena na samoj valvuli (AS, AI) ili na aorti (DAA). Kod djece s BAV-om nalazimo DAA u 76 (33,2%) bolesnika, i to u raznim kombinacijama s drugim grješkama LVOT-a. Uz osnovnu dijagnozu BAV-a već u dječjoj dobi učinjene su ove operacije: resekcija CoA s T-T-anastomozom 56 bolesnika (24,5%), balonska dilatacija BAV-a 28 bolesnika (12,3%), komisurotomija 19 bolesnika (8,3%), balonska dilatacija CoA 15 bolesnika (6,5%), resekcija subaortalne membrane 11 bolesnika (4,8%), operacija prema Rossu 8 bolesnika (3,5%), resekcija CoA s rekonstrukcijom 8 bolesnika (3,5%), valvuloplastika 6 bolesnika (2,6%), plastika ascendentne aorte 5 bolesnika (2,2%), mehanička aortalna valvula 3 bolesnika (1,3%), potključni režanj (subclavian flap) 3 bolesnika (1,3%), biološka aortalna valvula 2 bolesnika (0,9%), operacija prema Bentallu 1 bolesnik (0,4%), operacija prema Davidu 1 bolesnik (0,4%). Doprinos studije: Nalaz BAV-a kod djece prediktivni je čimbenik (navješćivač) progresivnog razvoja morfoloških promjena u različitim dijelovima LVOT-a, s potrebom za uklanjanjem hemodinamskih reperkusija u dijela bolesnika već u dječjoj dobi. Zaključak: Pojam valvularna aortopatija, odnosno sindrom bikuspidalne aortalne valvule trebalo bi rabiti već u dječjoj dobi, a anomaliju valja uključiti u epidemiološka istraživanja PSG-a.. 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), coarctation 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 of the 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

BICUSPID AORTIC VALVE AND LEFT VENTRICULAR OUTFLOW TRACT DEFECTS IN CHILDREN – SYNDROME OF BICUSPID AORTOPATHY?

Bikuspidalna aortalna valvula (BAV) epidemiološki se ne uvrštava u studije prirođenih srčanih grješaka (PSG) u djece iako se u odrasloj dobi smatra najčešćim PSG-om, s prevalencijom od 0,5 do 2%. Poremećaj aortalne valvulogeneze posljedica je genski uvjetovanih promjena u izlaznom dijelu lijeve klijetke (LVOT) u koje spadaju: sindrom hipoplastičnoga lijevog srca (HLHS), aortalna stenoza (AS) i insuficijencija (AI), dilatacija uzlazne aorte (DAA), koarktacija aorte (CoA), Shoneov sindrom (SS), a vjerojatno i neki drugi poremećaji. Mi smo zapazili da su uz BAV vezane znatne patomorfološke promjene već u dječjoj dobi. U retrospektivnu studiju dugu 11 godina (od 2002. do 2012.) uključeno je 229-ero djece s BAV-om, s predominacijom muških (1,7). Najčešća grješka pridružena BAV-u bila je CoA (75 bolesnika ili 32,6%). Od djece s BAV-om njih 62,4% (143 : 229) imalo je hemodinamske promjene na aortalnoj valvuli koje se očituju kao aortalna stenoza i/ili insuficijencija. AS je većinom progresivan i postaje već u dječjoj dobi hemodinamski važan u dijela bolesnika, a AI je većinom blag i rijetko hemodinamski važan.Velik broj bolesnika imao je izolirani AS uz DAA (21 ili 14,7%), a najveći broj imao je kombinaciju AS-a i AI (29 ili 20,3%). Zbog morfoloških promjena na samoj valvuli i pridruženim grješkama učinjeni su brojni intervencijski i kardiokirurški zahvati. Njihov broj rastao je s dobi, sukladno očekivanoj progresiji patoloških promjena na samoj valvuli (AS, AI) ili na aorti (DAA). Kod djece s BAV-om nalazimo DAA u 76 (33,2%) bolesnika, i to u raznim kombinacijama s drugim grješkama LVOT-a. Uz osnovnu dijagnozu BAV-a već u dječjoj dobi učinjene su ove operacije: resekcija CoA s T-T-anastomozom 56 bolesnika (24,5%), balonska dilatacija BAV-a 28 bolesnika (12,3%), komisurotomija 19 bolesnika (8,3%), balonska dilatacija CoA 15 bolesnika (6,5%), resekcija subaortalne membrane 11 bolesnika (4,8%), operacija prema Rossu 8 bolesnika (3,5%), resekcija CoA s rekonstrukcijom 8 bolesnika (3,5%), valvuloplastika 6 bolesnika (2,6%), plastika ascendentne aorte 5 bolesnika (2,2%), mehanička aortalna valvula 3 bolesnika (1,3%), potključni režanj (subclavian flap) 3 bolesnika (1,3%), biološka aortalna valvula 2 bolesnika (0,9%), operacija prema Bentallu 1 bolesnik (0,4%), operacija prema Davidu 1 bolesnik (0,4%). Doprinos studije: Nalaz BAV-a kod djece prediktivni je čimbenik (navješćivač) progresivnog razvoja morfoloških promjena u različitim dijelovima LVOT-a, s potrebom za uklanjanjem hemodinamskih reperkusija u dijela bolesnika već u dječjoj dobi. Zaključak: Pojam valvularna aortopatija, odnosno sindrom bikuspidalne aortalne valvule trebalo bi rabiti već u dječjoj dobi, a anomaliju valja uključiti u epidemiološka istraživanja PSG-a.. 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), coarctation 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 of the 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

TAKAYASU ARTERITIS AND POSSIBLE CARDIOLOGY REPERCUSSIONS IN THE CHILDHOOD

Vaskulitisi su rijetke reumatske bolesti nepoznate etiologije kojima je osnovno obilježje nekrotizirajuća upala krvnih žila. Posebnu skupinu čine granulomatozni vaskulitisi, izrazito rijetko opisivani u dječjoj dobi. Prikazujemo dvije bolesnice s Takayasuovim arteritisom (TA) kao entitetske oblike rijetkih reumatskih bolesti. Jedna je bolesnica imala TA tip II. a, a druga tip IV. U prve bolesnice nalazimo teške simptome opstrukcijskih lezija aortnih ogranaka, osobito tešku stenozu koronarnih arterija i okluziju lijeve potključne arterije te stenozu torakalne aorte ispod istmusa. Bolest je dijagnosticirana u akutnoj fazi, liječena je opsežno medikamentozno (glukokortikoidi, citostatici, metotreksat) i složenim kardiokirurškim pristupom, a zbog recidiva korištena je i biološka (rituksimab) terapija. Druga je bolesnica otkrivena zbog simptomatske arterijske hipertenzije, s izostankom pulseva na donjim udovima, a razlog tome nađen je u teškom suženju aorte od dijafragme do bifurkacije femoralnih arterija (mid aortic sindrom). U trenutku dijagnoze sama bolest nije bila u aktivnoj fazi. Liječena je osobitim kardiokirurškim pristupom i polimedikamentno zbog recidiva. Obje su bolesnice u adolescentnoj dobi i uspješno se liječe uz zadovoljavajuću kvalitetu života. Tip II. a s dodatnom okluzijom koronarnih krvnih žila nije prikazan u dostupnoj literaturi. Opisani vaskulitisi još uvijek snažno povezuju pedijatrijsku kardiologiju i reumatologiju, a prikaz svjedoči o važnosti timskog rada pedijatrijskih kardiologa i reumatologa.Vasculitides are rare rheumatic diseases of unknown etiology whose main characteristic is a necrotizing infl ammation 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 fi rst 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. Th e 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. Th e 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). Th e disease was not active when diagnosis was made. Th e 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