PLASTIC BRONCHITIS - IS IT A SYNDROMIC DISEASE?

Plastični ili odljevni bronhitis izrazito je rijetka i teška bolest koju se sve češće prikazuje kao kasnu komplikaciju kod djece nakon uspostave Fontanove cirkulacije. Ovdje prikazujemo 11-godišnjeg dječaka s inicijalnom dijagnozom sindroma hipoplastičnog lijevog srca (HLHS) kod kojeg se u dobi od 11 godina razvila slika plastičnog bronhitisa s tipičnim kliničkim, makroskopskim i mikroskopskim nalazom. Razmatraju se mogući etiološki čimbenici u nastanku bolesti, od nekih graničnih izvornih kriterija (hipoplazija velikih plućnih krvnih žila) do brojnih kirurških i interventnih postupaka. Neovisno o činjenici da ne postoji jedinstven etiološki čimbenik, u pozadini bolesti nalazi se poremećaj limfne drenaže. U radu se razmatraju mogući etiološki čimbenici s opsežnom analizom mogućeg poremećaja limfne drenaže u kardiopulmonalnom odnosu kod Fontanove cirkulacije. Kratko se opisuju i druge kasne komplikacije kod Fontanove cirkulacije, ali i opsežan i uspješan terapijski pristup sindromu odljevnog bronhitisa.Plastic or cast bronchitis is a very rare and severe condition that occurs as a late complication with increasing frequency in children after the establishment of the Fontan’s circulation. The case study presents an 11-year-old boy with an initial diagnosis of hypoplastic left heart syndrome (HLHS) who developed plastic bronchitis features with typical clinical, macroscopic and microscopic fi ndings. Possible etiological factors are considered in the disease pathogenesis, from some of the borderline original criteria (hypoplasia of large pulmonary blood vessels) to numerous operating and interventional procedures. Regardless of the fact that there is no unique etiological factor, the disease is marked by an underlying disorder in the lymphatic drainage. Possible etiological factors are evaluated in the paper, with extensive analysis of a possible lymphatic fl ow disorder in cardiopulmonary relationship with Fontan’s circulation. Furthermore, other possible late complications with Fontan’s circulation are presented, as well as a comprehensive and successful therapeutic approach to the plastic bronchitis syndrome

PLASTIC BRONCHITIS - IS IT A SYNDROMIC DISEASE?

Plastični ili odljevni bronhitis izrazito je rijetka i teška bolest koju se sve češće prikazuje kao kasnu komplikaciju kod djece nakon uspostave Fontanove cirkulacije. Ovdje prikazujemo 11-godišnjeg dječaka s inicijalnom dijagnozom sindroma hipoplastičnog lijevog srca (HLHS) kod kojeg se u dobi od 11 godina razvila slika plastičnog bronhitisa s tipičnim kliničkim, makroskopskim i mikroskopskim nalazom. Razmatraju se mogući etiološki čimbenici u nastanku bolesti, od nekih graničnih izvornih kriterija (hipoplazija velikih plućnih krvnih žila) do brojnih kirurških i interventnih postupaka. Neovisno o činjenici da ne postoji jedinstven etiološki čimbenik, u pozadini bolesti nalazi se poremećaj limfne drenaže. U radu se razmatraju mogući etiološki čimbenici s opsežnom analizom mogućeg poremećaja limfne drenaže u kardiopulmonalnom odnosu kod Fontanove cirkulacije. Kratko se opisuju i druge kasne komplikacije kod Fontanove cirkulacije, ali i opsežan i uspješan terapijski pristup sindromu odljevnog bronhitisa.Plastic or cast bronchitis is a very rare and severe condition that occurs as a late complication with increasing frequency in children after the establishment of the Fontan’s circulation. The case study presents an 11-year-old boy with an initial diagnosis of hypoplastic left heart syndrome (HLHS) who developed plastic bronchitis features with typical clinical, macroscopic and microscopic fi ndings. Possible etiological factors are considered in the disease pathogenesis, from some of the borderline original criteria (hypoplasia of large pulmonary blood vessels) to numerous operating and interventional procedures. Regardless of the fact that there is no unique etiological factor, the disease is marked by an underlying disorder in the lymphatic drainage. Possible etiological factors are evaluated in the paper, with extensive analysis of a possible lymphatic fl ow disorder in cardiopulmonary relationship with Fontan’s circulation. Furthermore, other possible late complications with Fontan’s circulation are presented, as well as a comprehensive and successful therapeutic approach to the plastic bronchitis syndrome

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

Double chambered right ventricle – concealed companion of ventricular septal defect

Cilj: Cilj je ovog rada prikazati kliničke karakteristike i rezultate kirurškog liječenja petero bolesnika s dvostrukom (pregrađenom) desnom klijetkom (engl. Double-Chambered Right Ventricle – DChRV ) kao mogućom posljedicom ventrikularnoga septalnog defekta (VSD). Rezultati istraživanja: U petero djece (4 djevojčice i 1 dječak) u dobi od 2,3 do 11,3 godine (srednja dob 6,3 godine) dijagnosticiran je intrakavitarni gradijent desne klijetke zbog pregradnje aberantnim mišićnim snopovima. Svi su bolesnici imali VSD s hemodinamski nevažnim lijevo-desnim pretokom (Qp/Qs od 10 do 31%). U troje djece DChRV razvio se s primarnim VSD-om, kod jednog s ostatnim defektom nakon zatvaranja perimembranoznog VSD-a zakrpom, a u jednog se djeteta desna klijetka pregradila približno 4 godine nakon primarno zatvorenog VSD-a. Intrakavitarni gradijent mjeren je s pomoću katetera end-hole u rasponu od 50 do 90 mmHg. Dva bolesnika nisu imala subjektivnih tegoba srčane naravi, a u troje se opisuje umor pri pojačanim naporima. Kod dva bolesnika VSD je bio smješten proksimalno od pregradnje (perimembranozni), a kod dva distalno (subaortalni- suprakristalni, konalni – doubly committed). Jedna je bolesnica imala dva VSD-a, jedan smješten proksimalno, a drugi distalno od pregradne stenoze. Dosad je grješka kardiokirurški korigirana u tri bolesnika patch-plastikom VSD-a i uklanjanjem zapreke u desnoj klijetki resekcijom aberantne muskulature, sve u dobi od 2 godine i 7 mjeseci do 6 godina i 3 mjeseca. Isti zahvat planiran je u još jednog bolesnika, dok se u drugoga planira izolirana resekcija aberantnog snopa. Postoperativnih komplikacija i subjektivnih simptoma srčane naravi u naših bolesnika nije bilo, a maksimalni intrakavitarni gradijent bio je 20 mmHg. Zaključak: Pregradnja desne klijetke vjerojatno je uzrokovana postojanjem VSD-a, neovisno o veličini i lokalizaciji, a može se razviti kod primarnog i rezidualnog VSD-a te nakon njegova uspješnog zatvaranja. Stoga pacijente s VSD-om, osobito perimembranoznim i suprakristalnim, valja dugotrajno kontrolirati u duljim vremenskim razmacima i nakon operacije. To je osobito važno zbog mogućnosti recidiva pregrađivanja, pojave subaortalne membrane i/ili insuficijencije aortalne valvule. Kirurško uklanjanje hipertrofičnog i aberantnog tkiva prikladan je oblik liječenja s relativno rijetkim teškim komplikacijama, što, s obzirom na očekivani progresivan tijek bolesti, opravdava operaciju pri znatnoj opstrukciji DChRV -a i odsutnosti simptoma.Objective: The primary objective is to present the diagnostic and treatment experiences with double chambered right ventricle (DChRV ) as a possible consequence of the ventricular septal defect (VSD). Results:The intracavitary gradient as a consequence of the remodeling with aberrant muscle bundles in the right ventricle was diagnosed in five children (four females) at the age of 2.3–11.3 years (mean age 6.3 years). All patients had VSD with hemodynamically nonsignificant L-R shunts (Qp/Qs 10–31%). In three children DChRV developed with primary VSD, in one with a residual defect after patch repair of perimembranous VSD, and in one four years after adequate VSD closure. Intracavitary gradient measured by an end-hole catheter was 50–90 mmHg. Two patients had no cardiac symptoms, while three of them had exercise intolerance.In two patients VSD was located proximally to the obstructive band (perimembranous) and in two patients distally (subaortic-supracristal, conal-doubly committed). One patient had two VSDs – one located proximally and one distally to the obstructive band. The defect was so far surgically corrected in three patients with VSD patch and resection of the obstructive aberrant muscle tissue at the age ranging from two years and seven months to the age of six years and three months. One patient waits for the same surgical procedure, and the other one for an isolated aberrant band resection. There were no postoperative complications, no postoperative symptoms were reported, and maximal intracavitary gradient was 20 mmHg. Conclusion: Remodeling of RV (DChRV ) is probably caused by the presence of VSD, independent of its size and location; it can develop in primary, residual VSD, as well as after successful VSD closure. Therefore, patients with VSD, especially with perimembranous and doubly committed VSD need a continuous follow up even after operation. Long-term follow up is necessary due to possible obstruction recurrence, subaortic membrane and/or aortic valve insufficiency appearance. Surgical removal of the hypertrophic and aberrant tissue is an adequate way of treatment with relatively rare serious complications. Regarding the expected progressive course of the disease, the operative treatment in significant DChRV obstruction even in the absence of symptoms is justified

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

Dvostruka (pregrađena) desna klijetka - prikriveni pratitelj ventrikularnoga septalnog defekta [Double chambered right ventricle – concealed companion of ventricular septal defect]

OBJECTIVE: The primary objective is to present the diagnostic and treatment experiences with double chambered right ventricle (DChRV ) as a possible consequence of the ventricular septal defect (VSD). ----- RESULTS:The intracavitary gradient as a consequence of the remodeling with aberrant muscle bundles in the right ventricle was diagnosed in five children (four females) at the age of 2.3–11.3 years (mean age 6.3 years). All patients had VSD with hemodynamically nonsignificant L-R shunts (Qp/Qs 10–31%). In three children DChRV developed with primary VSD, in one with a residual defect after patch repair of perimembranous VSD, and in one four years after adequate VSD closure. Intracavitary gradient measured by an end-hole catheter was 50–90 mmHg. Two patients had no cardiac symptoms, while three of them had exercise intolerance.In two patients VSD was located proximally to the obstructive band (perimembranous) and in two patients distally (subaortic-supracristal, conal-doubly committed). One patient had two VSDs – one located proximally and one distally to the obstructive band. The defect was so far surgically corrected in three patients with VSD patch and resection of the obstructive aberrant muscle tissue at the age ranging from two years and seven months to the age of six years and three months. One patient waits for the same surgical procedure, and the other one for an isolated aberrant band resection. There were no postoperative complications, no postoperative symptoms were reported, and maximal intracavitary gradient was 20 mmHg. -----CONCLUSION: Remodeling of RV (DChRV ) is probably caused by the presence of VSD, independent of its size and location; it can develop in primary, residual VSD, as well as after successful VSD closure. Therefore, patients with VSD, especially with perimembranous and doubly committed VSD need a continuous follow up even after operation. Long-term follow up is necessary due to possible obstruction recurrence, subaortic membrane and/or aortic valve insufficiency appearance. Surgical removal of the hypertrophic and aberrant tissue is an adequate way of treatment with relatively rare serious complications. Regarding the expected progressive course of the disease, the operative treatment in significant DChRV obstruction even in the absence of symptoms is justified

COARCTATION OF THE AORTA IN CHILDREN IN THE 10-YEAR EPIDEMIOLOGICAL STUDY: DIAGNOSTIC AND THERAPEUTIC CONSIDERATION

Naša je studija klinička epidemiološka retrospektivna analiza koarktacije aorte u vremenskom slijedu od 10 godina (2001. – 2011.). U studiju je uključeno 201 dijete, 72 (35,82%) djevojčice i 129 (64,18%) dječaka (1 : 1,6) prosječne dobi kod postavljanja dijagnoze 28,57 ± 49,37 mjeseci (0,1 – 204). Razvrstani su u 4 dobne skupine: novorođenačka dob ( 6 godina. Izoliranu koarktaciju imalo je 125 (62,19%) bolesnika, od čega 33 (16,42%) preduktalnu i 92 (45,77%) postduktalnu, a njih 76 (37,81%) imalo je koarktaciju s dodatnom srčanom grješkom; s ventrikularnim septalnim defektom 32 (15,92%), u okviru kompleksne srčane grješke 28 (13,93%), u sklopu Shoneova kompleksa 11 (5,47%) i s dilatacijskom kardiomiopatijom 5 (2,49%). U mlađim dobnim skupinama (novorođenačka i dojenačka dob) prevladava zamaranje, pojačano znojenje i dispnoičke tegobe, a kod starije djece klaudikacije, glavobolja i epistaksa. Kod čak 61 (30,35%) ispitanika dogodio se previd dijagnoze na prethodnome kardiološkom pregledu. Kod 20 (9,95%) bolesnika koarktacija se nalazi u okviru poznatih sindromnih bolesti (Turner, Noonan, Williams-Beuren, Ellis-van Creveld, parcijalna trisomija 18, Downov sindrom, fetalni valproatni sindrom). Ehokardiografija je učinjena u svih bolesnika, a kod 45 (22,38%) bila je i jedina slikovna dijagnostička pretraga. Učinjene su ukupno 123 kateterizacije srca, 38 kompjutoriziranih tomografija i 15 magnetskih rezonancija. Gradijent na mjestu koarktacije mjeren prije operacije ili interventnog liječenja izmjeren na kateterizaciji srca kod 132 (65,67%) bolesnika iznosio je 57,99 ± 18,68 mmHg (20 – 100 mmHg). Kod 82 (40,80%) bolesnika nađena je bikuspidalna aortalna valvula. Prosječna dob kod operacije bila je 27,92 ± 47,98 mjeseci (0,1 – 204). Kod 169 (84,07%) izvršena je kardiokirurška korekcija, od toga T-T-anastomoza kod 109 (54,23%), a kod 30 (14,92%) učinjena je terapijska kateterizacija (balonska dilatacija ili implantacija stenta). Smrtni ishod zabilježen je u četvero djece (1,99%), svi u novorođenačkoj ili dojenačkoj dobi.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: 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 diagnostic 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