108 research outputs found
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 Uhlās 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 (2002ā2007)
prikazati populacijski i hospitalni registar priroÄenih srÄanih grjeÅ”aka za Hrvatsku. Metode: Tijekom 5 godina ispunjavali smo upitnik s pitanjima o populacijskom kretanju priroÄenih srÄanih grjeÅ”aka (prema preporukama BWIS i EUROCAT study) i hospitalni registar kojim smo pratili lijeÄenje i ishod lijeÄenja u djece koja su u spomenutom razdoblju roÄena u Hrvatskoj. Rezultati: Od 1. 10. 2002 do 1. 10. 2007. u Hrvatskoj je roÄeno 205917-ero djece, od Äega je 1480-ero djece imalo priroÄenu srÄanu grjeÅ”ku (7,2 promila). NajÄeÅ”Äe postavljena dijagnoza je VSD (34,6%). Na kardiokirurÅ”ko lijeÄenje upuÄeno je 430-ero djece. Na njima je uÄinjeno 556 operacija, od Äega 202 u Hrvatskoj, ostatak u inozemstvu. Ukupni mortalitet nakon kardiokirurÅ”kog lijeÄenja iznosi 5%. Razlike u mortalitetu i morbiditetu nakon kardiokirurÅ”kih operacija izvedenih u Hrvatskoj i onih u inozemstvu uoÄavaju se nakon analize ishoda lijeÄenja prema dogovorenim metodologijama. ZakljuÄak: Registar priroÄenih srÄanih grjeÅ”aka Äini osnovu za epidemioloÅ”ka 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 205ā
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 (2002ā2007)
prikazati populacijski i hospitalni registar priroÄenih srÄanih grjeÅ”aka za Hrvatsku. Metode: Tijekom 5 godina ispunjavali smo upitnik s pitanjima o populacijskom kretanju priroÄenih srÄanih grjeÅ”aka (prema preporukama BWIS i EUROCAT study) i hospitalni registar kojim smo pratili lijeÄenje i ishod lijeÄenja u djece koja su u spomenutom razdoblju roÄena u Hrvatskoj. Rezultati: Od 1. 10. 2002 do 1. 10. 2007. u Hrvatskoj je roÄeno 205917-ero djece, od Äega je 1480-ero djece imalo priroÄenu srÄanu grjeÅ”ku (7,2 promila). NajÄeÅ”Äe postavljena dijagnoza je VSD (34,6%). Na kardiokirurÅ”ko lijeÄenje upuÄeno je 430-ero djece. Na njima je uÄinjeno 556 operacija, od Äega 202 u Hrvatskoj, ostatak u inozemstvu. Ukupni mortalitet nakon kardiokirurÅ”kog lijeÄenja iznosi 5%. Razlike u mortalitetu i morbiditetu nakon kardiokirurÅ”kih operacija izvedenih u Hrvatskoj i onih u inozemstvu uoÄavaju se nakon analize ishoda lijeÄenja prema dogovorenim metodologijama. ZakljuÄak: Registar priroÄenih srÄanih grjeÅ”aka Äini osnovu za epidemioloÅ”ka 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 205ā
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
Referentne vrijednosti kardiopulmonalnog testa optereÄenja 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 37.13Ā±8.9 mL/kg/min compared with 31.95Ā±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
51.3Ā±8.9 as compared with 41.49Ā±6.51 mL/kg/min in girls. During incremental work, systolic pressure increased from mean
109.86Ā±14.2 mm Hg to 145.43Ā±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 tipiÄne za odreÄenu populaciju. NaÅ” 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. DjeÄaci su postigli znaÄajno
viÅ”u potroÅ”nju kisika (VO2) u toÄki anaerobnog praga (AT) sa srednjom vrijednoÅ”Äu 37,13 Ā± 8,9 ml/kg/min u odnosu na djevojÄice
koje su postigle 31,95 Ā± 6,6 ml/kg/min. Jednako tako djeÄaci su postigli znaÄajno viÅ”u vrÅ”nu potroÅ”nju kisika 51,3 Ā± 8,9 u odnosu na
41,49 Ā± 6,51 ml/kg/min kod djevojÄica. Tijekom porasta rada sistoliÄki tlak je rastao sa 109,86 Ā± 14,2 mmHg do 145,43 Ā± 11,9 mmHg
u vrÅ”nom vježbanju, bez znaÄajnih razlika meÄu spolom. Porastom optereÄenja, zajedno s linearnim porastom srÄane frekvencije i
sistoliÄkog tlaka, bilježi se i linearan porast srÄanog izbaÄaja dobivenog neinvazivnim mjerenjem temeljenim na potroÅ”nji kisika i
Fickovom naÄelu. Postignute vrÅ”ne vrijednosti srÄanog izbaÄaja znaÄajno su viÅ”e kod djeÄaka. U odnosu na djevojÄice djeÄaci su
imali znaÄajno veÄi volumen udisaja u mirovanju te su postigli znaÄajno veÄu minutnu ventilaciju u vrÅ”nom vježbanju. Prikazani su
parametri kardiopulmonalnog i metaboliÄkog odgovora u tijeku spiroergometrijskog testiranja i iznesene referentne vrijednosti.
Dobivene vrijednosti ne razlikuju se znaÄajno u odnosu na druge studije i temelj su za daljnja istraživanja
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
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
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