34 research outputs found
PLASTIC BRONCHITIS - IS IT A SYNDROMIC DISEASE?
PlastiÄni ili odljevni bronhitis izrazito je rijetka i teÅ”ka bolest koju se sve ÄeÅ”Äe prikazuje kao kasnu komplikaciju kod djece nakon uspostave Fontanove cirkulacije. Ovdje prikazujemo 11-godiÅ”njeg djeÄaka s inicijalnom dijagnozom sindroma hipoplastiÄnog lijevog srca (HLHS) kod kojeg se u dobi od 11 godina razvila slika plastiÄnog bronhitisa s tipiÄnim kliniÄkim, makroskopskim i mikroskopskim nalazom. Razmatraju se moguÄi etioloÅ”ki Äimbenici u nastanku bolesti, od nekih graniÄnih izvornih kriterija (hipoplazija velikih pluÄnih krvnih žila) do brojnih kirurÅ”kih i interventnih postupaka. Neovisno o Äinjenici da ne postoji jedinstven etioloÅ”ki Äimbenik, u pozadini bolesti nalazi se poremeÄaj limfne drenaže. U radu se razmatraju moguÄi etioloÅ”ki Äimbenici s opsežnom analizom moguÄeg poremeÄaja limfne drenaže u kardiopulmonalnom odnosu kod Fontanove cirkulacije. Kratko se opisuju i druge kasne komplikacije kod Fontanove cirkulacije, ali i opsežan i uspjeÅ”an 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 Fontanās 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 Fontanās circulation. Furthermore, other possible late complications with Fontanās circulation are presented, as well as a comprehensive and successful therapeutic approach to the plastic bronchitis syndrome
PLASTIC BRONCHITIS - IS IT A SYNDROMIC DISEASE?
PlastiÄni ili odljevni bronhitis izrazito je rijetka i teÅ”ka bolest koju se sve ÄeÅ”Äe prikazuje kao kasnu komplikaciju kod djece nakon uspostave Fontanove cirkulacije. Ovdje prikazujemo 11-godiÅ”njeg djeÄaka s inicijalnom dijagnozom sindroma hipoplastiÄnog lijevog srca (HLHS) kod kojeg se u dobi od 11 godina razvila slika plastiÄnog bronhitisa s tipiÄnim kliniÄkim, makroskopskim i mikroskopskim nalazom. Razmatraju se moguÄi etioloÅ”ki Äimbenici u nastanku bolesti, od nekih graniÄnih izvornih kriterija (hipoplazija velikih pluÄnih krvnih žila) do brojnih kirurÅ”kih i interventnih postupaka. Neovisno o Äinjenici da ne postoji jedinstven etioloÅ”ki Äimbenik, u pozadini bolesti nalazi se poremeÄaj limfne drenaže. U radu se razmatraju moguÄi etioloÅ”ki Äimbenici s opsežnom analizom moguÄeg poremeÄaja limfne drenaže u kardiopulmonalnom odnosu kod Fontanove cirkulacije. Kratko se opisuju i druge kasne komplikacije kod Fontanove cirkulacije, ali i opsežan i uspjeÅ”an 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 Fontanās 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 Fontanās circulation. Furthermore, other possible late complications with Fontanās circulation are presented, as well as a comprehensive and successful therapeutic approach to the plastic bronchitis syndrome
Bikuspidalna aortalna valvula i grjeÅ”ke izlaznog trakta lijeve klijetke u djece ā 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 ā SYNDROME OF BICUSPID AORTOPATHY?
Bikuspidalna aortalna valvula (BAV) epidemioloÅ”ki se ne uvrÅ”tava u studije priroÄenih srÄanih grjeÅ”aka (PSG) u djece iako se u odrasloj dobi smatra najÄeÅ”Äim PSG-om, s prevalencijom od 0,5 do 2%. PoremeÄaj aortalne valvulogeneze posljedica je genski uvjetovanih promjena u izlaznom dijelu lijeve klijetke (LVOT) u koje spadaju: sindrom hipoplastiÄnoga lijevog srca (HLHS), aortalna stenoza (AS) i insuficijencija (AI), dilatacija uzlazne aorte (DAA), koarktacija aorte (CoA), Shoneov sindrom (SS), a vjerojatno i neki drugi poremeÄaji. Mi smo zapazili da su uz BAV vezane znatne patomorfoloÅ”ke promjene veÄ u djeÄjoj dobi. U retrospektivnu studiju dugu 11 godina (od 2002. do 2012.) ukljuÄeno je 229-ero djece s BAV-om, s predominacijom muÅ”kih (1,7). NajÄeÅ”Äa grjeÅ”ka 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 oÄituju kao aortalna stenoza i/ili insuficijencija. AS je veÄinom progresivan i postaje veÄ u djeÄjoj dobi hemodinamski važan u dijela bolesnika, a AI je veÄinom blag i rijetko hemodinamski važan.Velik broj bolesnika imao je izolirani AS uz DAA (21 ili 14,7%), a najveÄi broj imao je kombinaciju AS-a i AI (29 ili 20,3%). Zbog morfoloÅ”kih promjena na samoj valvuli i pridruženim grjeÅ”kama uÄinjeni su brojni intervencijski i kardiokirurÅ”ki zahvati. Njihov broj rastao je s dobi, sukladno oÄekivanoj progresiji patoloÅ”kih 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 grjeÅ”kama LVOT-a. Uz osnovnu dijagnozu BAV-a veÄ u djeÄjoj dobi uÄinjene 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%), mehaniÄka aortalna valvula 3 bolesnika (1,3%), potkljuÄni režanj (subclavian flap) 3 bolesnika (1,3%), bioloÅ”ka 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 Äimbenik (navjeÅ”ÄivaÄ) progresivnog razvoja morfoloÅ”kih promjena u razliÄitim dijelovima LVOT-a, s potrebom za uklanjanjem hemodinamskih reperkusija u dijela bolesnika veÄ u djeÄjoj dobi. ZakljuÄak: Pojam valvularna aortopatija, odnosno sindrom bikuspidalne aortalne valvule trebalo bi rabiti veÄ u djeÄjoj dobi, a anomaliju valja ukljuÄiti u epidemioloÅ”ka 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), Shoneās 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%); Ā»subclavian flapĀ« 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 ā SYNDROME OF BICUSPID AORTOPATHY?
Bikuspidalna aortalna valvula (BAV) epidemioloÅ”ki se ne uvrÅ”tava u studije priroÄenih srÄanih grjeÅ”aka (PSG) u djece iako se u odrasloj dobi smatra najÄeÅ”Äim PSG-om, s prevalencijom od 0,5 do 2%. PoremeÄaj aortalne valvulogeneze posljedica je genski uvjetovanih promjena u izlaznom dijelu lijeve klijetke (LVOT) u koje spadaju: sindrom hipoplastiÄnoga lijevog srca (HLHS), aortalna stenoza (AS) i insuficijencija (AI), dilatacija uzlazne aorte (DAA), koarktacija aorte (CoA), Shoneov sindrom (SS), a vjerojatno i neki drugi poremeÄaji. Mi smo zapazili da su uz BAV vezane znatne patomorfoloÅ”ke promjene veÄ u djeÄjoj dobi. U retrospektivnu studiju dugu 11 godina (od 2002. do 2012.) ukljuÄeno je 229-ero djece s BAV-om, s predominacijom muÅ”kih (1,7). NajÄeÅ”Äa grjeÅ”ka 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 oÄituju kao aortalna stenoza i/ili insuficijencija. AS je veÄinom progresivan i postaje veÄ u djeÄjoj dobi hemodinamski važan u dijela bolesnika, a AI je veÄinom blag i rijetko hemodinamski važan.Velik broj bolesnika imao je izolirani AS uz DAA (21 ili 14,7%), a najveÄi broj imao je kombinaciju AS-a i AI (29 ili 20,3%). Zbog morfoloÅ”kih promjena na samoj valvuli i pridruženim grjeÅ”kama uÄinjeni su brojni intervencijski i kardiokirurÅ”ki zahvati. Njihov broj rastao je s dobi, sukladno oÄekivanoj progresiji patoloÅ”kih 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 grjeÅ”kama LVOT-a. Uz osnovnu dijagnozu BAV-a veÄ u djeÄjoj dobi uÄinjene 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%), mehaniÄka aortalna valvula 3 bolesnika (1,3%), potkljuÄni režanj (subclavian flap) 3 bolesnika (1,3%), bioloÅ”ka 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 Äimbenik (navjeÅ”ÄivaÄ) progresivnog razvoja morfoloÅ”kih promjena u razliÄitim dijelovima LVOT-a, s potrebom za uklanjanjem hemodinamskih reperkusija u dijela bolesnika veÄ u djeÄjoj dobi. ZakljuÄak: Pojam valvularna aortopatija, odnosno sindrom bikuspidalne aortalne valvule trebalo bi rabiti veÄ u djeÄjoj dobi, a anomaliju valja ukljuÄiti u epidemioloÅ”ka 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), Shoneās 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%); Ā»subclavian flapĀ« 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 nekrotizirajuÄa upala krvnih
žila. Posebnu skupinu Äine granulomatozni vaskulitisi,
izrazito rijetko opisivani u djeÄjoj 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 teŔke
simptome opstrukcijskih lezija aortnih ogranaka, osobito
teÅ”ku stenozu koronarnih arterija i okluziju lijeve potkljuÄne
arterije te stenozu torakalne aorte ispod istmusa. Bolest
je dijagnosticirana u akutnoj fazi, lijeÄena je opsežno medikamentozno
(glukokortikoidi, citostatici, metotreksat) i
složenim kardiokirurŔkim pristupom, a zbog recidiva koriŔtena
je i bioloŔka (rituksimab) terapija. Druga je bolesnica
otkrivena zbog simptomatske arterijske hipertenzije, s izostankom pulseva na donjim udovima, a razlog tome
naÄen je u teÅ”kom suženju aorte od dijafragme do bifurkacije
femoralnih arterija (mid aortic sindrom). U trenutku
dijagnoze sama bolest nije bila u aktivnoj fazi. LijeÄena je
osobitim kardiokirurŔkim pristupom i polimedikamentno
zbog recidiva. Obje su bolesnice u adolescentnoj dobi i
uspjeÅ”no se lijeÄe uz zadovoljavajuÄu kvalitetu života. Tip
II. a s dodatnom okluzijom koronarnih krvnih žila nije
prikazan u dostupnoj literaturi. Opisani vaskulitisi joÅ” uvijek
snažno povezuju pedijatrijsku kardiologiju i reumatologiju,
a prikaz svjedoÄi 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 ā concealed companion of ventricular septal defect
Cilj: Cilj je ovog rada prikazati kliniÄke karakteristike i rezultate kirurÅ”kog lijeÄenja petero bolesnika s dvostrukom (pregraÄenom) desnom klijetkom (engl. Double-Chambered Right Ventricle ā DChRV ) kao moguÄom posljedicom ventrikularnoga septalnog defekta (VSD). Rezultati istraživanja: U petero djece (4 djevojÄice i 1 djeÄak) u dobi od 2,3 do 11,3 godine (srednja dob 6,3 godine) dijagnosticiran je intrakavitarni gradijent desne klijetke zbog pregradnje aberantnim miÅ”iÄnim 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 pomoÄu katetera end-hole u rasponu od 50 do 90 mmHg. Dva bolesnika nisu imala subjektivnih tegoba srÄane naravi, a u troje se opisuje umor pri pojaÄanim naporima. Kod dva bolesnika VSD je bio smjeÅ”ten proksimalno od pregradnje (perimembranozni), a kod dva distalno (subaortalni- suprakristalni, konalni ā doubly committed). Jedna je bolesnica imala dva VSD-a, jedan smjeÅ”ten proksimalno, a drugi distalno od pregradne stenoze. Dosad je grjeÅ”ka kardiokirurÅ”ki 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 joÅ” jednog bolesnika, dok se u drugoga planira izolirana resekcija aberantnog snopa. Postoperativnih komplikacija i subjektivnih simptoma srÄane naravi u naÅ”ih bolesnika nije bilo, a maksimalni intrakavitarni gradijent bio je 20 mmHg. ZakljuÄak: Pregradnja desne klijetke vjerojatno je uzrokovana postojanjem VSD-a, neovisno o veliÄini i lokalizaciji, a može se razviti kod primarnog i rezidualnog VSD-a te nakon njegova uspjeÅ”nog 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 moguÄnosti recidiva pregraÄivanja, pojave subaortalne membrane i/ili insuficijencije aortalne valvule. KirurÅ”ko uklanjanje hipertrofiÄnog i aberantnog tkiva prikladan je oblik lijeÄenja s relativno rijetkim teÅ”kim komplikacijama, Å”to, s obzirom na oÄekivani 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 2.3ā11.3 years (mean age 6.3 years). All patients had VSD with hemodynamically nonsignificant L-R shunts (Qp/Qs 10ā31%). 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 50ā90 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 ā 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 nekrotizirajuÄa upala krvnih
žila. Posebnu skupinu Äine granulomatozni vaskulitisi,
izrazito rijetko opisivani u djeÄjoj 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 teŔke
simptome opstrukcijskih lezija aortnih ogranaka, osobito
teÅ”ku stenozu koronarnih arterija i okluziju lijeve potkljuÄne
arterije te stenozu torakalne aorte ispod istmusa. Bolest
je dijagnosticirana u akutnoj fazi, lijeÄena je opsežno medikamentozno
(glukokortikoidi, citostatici, metotreksat) i
složenim kardiokirurŔkim pristupom, a zbog recidiva koriŔtena
je i bioloŔka (rituksimab) terapija. Druga je bolesnica
otkrivena zbog simptomatske arterijske hipertenzije, s izostankom pulseva na donjim udovima, a razlog tome
naÄen je u teÅ”kom suženju aorte od dijafragme do bifurkacije
femoralnih arterija (mid aortic sindrom). U trenutku
dijagnoze sama bolest nije bila u aktivnoj fazi. LijeÄena je
osobitim kardiokirurŔkim pristupom i polimedikamentno
zbog recidiva. Obje su bolesnice u adolescentnoj dobi i
uspjeÅ”no se lijeÄe uz zadovoljavajuÄu kvalitetu života. Tip
II. a s dodatnom okluzijom koronarnih krvnih žila nije
prikazan u dostupnoj literaturi. Opisani vaskulitisi joÅ” uvijek
snažno povezuju pedijatrijsku kardiologiju i reumatologiju,
a prikaz svjedoÄi 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 (pregraÄena) desna klijetka - prikriveni pratitelj ventrikularnoga septalnog defekta [Double chambered right ventricle ā 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 2.3ā11.3 years (mean age 6.3 years). All patients had VSD with hemodynamically
nonsignificant L-R shunts (Qp/Qs 10ā31%). 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 50ā90 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
ā 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
NaÅ”a je studija kliniÄka epidemioloÅ”ka retrospektivna analiza koarktacije aorte u vremenskom slijedu od 10 godina (2001. ā 2011.). U studiju je ukljuÄeno 201 dijete, 72 (35,82%) djevojÄice i 129 (64,18%) djeÄaka (1 : 1,6) prosjeÄne dobi kod postavljanja dijagnoze 28,57 Ā± 49,37 mjeseci (0,1 ā 204). Razvrstani su u 4 dobne skupine: novoroÄenaÄka dob ( 6 godina. Izoliranu koarktaciju imalo je 125 (62,19%) bolesnika, od Äega 33 (16,42%) preduktalnu i 92 (45,77%) postduktalnu, a njih 76 (37,81%) imalo je koarktaciju s dodatnom srÄanom grjeÅ”kom; s ventrikularnim septalnim defektom 32 (15,92%), u okviru kompleksne srÄane grjeÅ”ke 28 (13,93%), u sklopu Shoneova kompleksa 11 (5,47%) i s dilatacijskom kardiomiopatijom 5 (2,49%). U mlaÄim dobnim skupinama (novoroÄenaÄka i dojenaÄka dob) prevladava zamaranje, pojaÄano znojenje i dispnoiÄke tegobe, a kod starije djece klaudikacije, glavobolja i epistaksa. Kod Äak 61 (30,35%) ispitanika dogodio se previd dijagnoze na prethodnome kardioloÅ”kom 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 uÄinjena u svih bolesnika, a kod 45 (22,38%) bila je i jedina slikovna dijagnostiÄka pretraga. UÄinjene su ukupno 123 kateterizacije srca, 38 kompjutoriziranih tomografija i 15 magnetskih rezonancija. Gradijent na mjestu koarktacije mjeren prije operacije ili interventnog lijeÄenja izmjeren na kateterizaciji srca kod 132 (65,67%) bolesnika iznosio je 57,99 Ā± 18,68 mmHg (20 ā 100 mmHg). Kod 82 (40,80%) bolesnika naÄena je bikuspidalna aortalna valvula. ProsjeÄna dob kod operacije bila je 27,92 Ā± 47,98 mjeseci (0,1 ā 204). Kod 169 (84,07%) izvrÅ”ena je kardiokirurÅ”ka korekcija, od toga T-T-anastomoza kod 109 (54,23%), a kod 30 (14,92%) uÄinjena je terapijska kateterizacija (balonska dilatacija ili implantacija stenta). Smrtni ishod zabilježen je u Äetvero djece (1,99%), svi u novoroÄenaÄkoj ili dojenaÄkoj dobi.Our study is a clinical epidemiological retrospective analysis of coarctation of the aorta in a 10-year follow-up (2001ā2011). The study includes 201 children, 72 (35.82%) girls and 129 (64.18%) boys (1:1,6), with an average age of 28.57Ā±49.37 mo (0.1 ā 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 57.99Ā±18.68 mmHg (20ā100 mmHg). In 82 (40.80%) patients a bicuspid aortic valve was found. Average age at the time of surgery was 27.92Ā±47.98 months (0.1ā204 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