14 research outputs found
Aritmogena kardiomiopatija desne klijetke ā jesmo li suvremenim dijagnostiÄkim i terapijskim pristupom izbjegli tragediju u obitelji? [Right ventricular arrhytmogenic cardiomyopathy ā have we avoided a family tragedy by applying contemporary diagnostic and treatment approach?]
Introduction: Arrhytmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized
by fi brofatty replacement, dominantly in the right, but often also in the left ventricle. It is a signifi cant cause of
sudden death in children and adolescents. A thorough family history and contemporary diagnostic and treatment approach
are prerequisites for the prevention of the sudden death syndrome. The diagnosis is more often established in adults than in
children. Patients: We present a family with four members affected and diagnosed with ARVC already in childhood/adolescence.
The average age of symptom presentation was 12 years (10ā14 years). The importance of family tree isemphasized
and contemporary diagnostic and treatment methods, with the aim of sudden death prevention, are described. Conclusions:
Family history is essential for the early ARVC diagnosis. There is a need for revision of current diagnostic criteria in
pediatric population. In the future progression of fi brous tissue proportion in relation to age and the stage of the disease
should be taken in account. Furthermore, the repolarisation abnormality is inappropriate as a criterion in children less than
14 years old, so in that age group less of present diagnostic criteria can be applied. A combination of modern implantable
cardioverter defi brillator and radiofrequency ablation assures an excellent life quality in our patients. However, the longterm
prognosis for our patients, because of a progressive course of the disease, remains uncertain
Primary malignant fibrous histiocytoma of the heart with skeletal muscles metastases
Malignant fibrous histiocytoma is an extremely rare primary malignant tumor of the heart. It is usually diagnosed when it is locally aggressive or has already metastasized. The prognosis is poor with an average survival time of one year. We report a case of recurrent left atrial malignant fibrous histiocytoma initially misdiagnosed as myxoma. The patient underwent repeated surgical resections followed by chemotherapy. Despite adjuvant chemotherapy, 18 months after initial diagnosis, definitive tumor relapse in left atrium was diagnosed. This is the 48th case of primary cardiac fibrous malignant histiocytoma reported in the literature
RIGHT VENTRICULAR ARRHYTMOGENIC CARDIOMYOPATHY ā HAVE WE AVOIDED A FAMILY TRAGEDY BY APPLYING CONTEMPORARY DIAGNOSTIC AND TREATMENT APPROACH?
Uvod: Aritmogena kardiomiopatija desne klijetke (engl. Arrhythmogenic right ventricular cardiomyopathy ā ARVC) nasljedna je kardiomiopatija karakterizirana pretvorbom miocita u masno i vezivno tkivo, pretežno desne, no Ānerijetko i lijeve klijetke. Važan je uzrok iznenadne smrti djece i adolescenata. Temeljita obiteljska anamneza i suvremeni dijagnostiÄki i terapijski pristup preduvjeti su za spreÄavanje sindroma iznenadne smrti. ÄeÅ”Äe se dijagnoza postavlja u odraslih nego u djece. Prikaz bolesnika: Prikazujemo obitelj u kojoj je u Äetvero Älanova postavljena dijagnoza ARVC-a veÄ u djeÄjoj/adolescentnoj dobi. ProsjeÄna dob pojave simptoma kod tih bolesnika bila je 12 godina (10 ā 14). IstiÄe se važnost obiteljskog stabla i opisuju se suvremene metode dijagnosticiranja i terapijskog pristupa, radi prevencije iznenadne smrti. ZakljuÄujemo da je obiteljska anamneza temelj za rano postavljanje dijagnoze ARVC-a. Nazire se potreba revizije postojeÄih dijagnostiÄkih kriterija ARVC-a za pedijatrijsku populaciju. Valjalo bi uzeti u obzir progresivno poveÄanje Āudjela veziva u odnosu prema miocitima u ovisnosti o dobi i stadiju bolesti. S obzirom na neprimjenjivost kriterija smetnja repolarizacije kod djece mlaÄe od 14 godina, treba razmotriti smanjenje broja kriterija potrebnih za postavljanje dijagnoze u toj dobnoj skupini. Implantabilni elektrokardioverter defibrilator (ICD) i radiofrekventna ablacija u kombinaciji kratkoroÄno osiguravaju odliÄnu kvalitetu života kod naÅ”eg bolesnika s teÅ”kim oblikom ARVC-a, dok su dugoroÄni rezultati s obzirom na progresivni tijek bolesti neizvjesni.Introduction: Arrhytmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by fibrofatty replacement, dominantly in the right, but often also in the left ventricle. It is a significant cause of sudden death in children and adolescents. A thorough family history and contemporary diagnostic and treatment approach are prerequisites for the prevention of the sudden death syndrome. The diagnosis is more often established in adults than in children. Patients: We present a family with four members affected and diagnosed with ARVC already in childhood/adolescence. The average age of symptom presentation was 12 years (10ā14 years). The importance of family tree isemphasized and contemporary diagnostic and treatment methods, with the aim of sudden death prevention, are described. Conclusions: Family history is essential for the early ARVC diagnosis. There is a need for revision of current diagnostic criteria in pediatric population. In the future progression of fibrous tissue proportion in relation to age and the stage of the disease should be taken in account. Furthermore, the repolarisation abnormality is inappropriate as a criterion in children less than 14 years old, so in that age group less of present diagnostic criteria can be applied. A combination of modern implantable cardioverter defibrillator and radiofrequency ablation assures an excellent life quality in our patients. However, the longterm prognosis for our patients, because of a progressive course of the disease, remains uncertain
Amyloidosis of the Aortic Root in a Patient with Polyarteritis Nodosa
Amyloidosis results from the deposition of insoluble fibrillar proteins in various tissues. Cardiac symptoms, when present, are usually related to congestive heart failure or arrhythmias. Amyloid involvement of the aortic root is exceedingly rare. We describe a case of aortic root amyloidosis in a patient with a long-standing history of polyarteritis nodosa
TESTICULAR CAPILLARY HEMANGIOMA, REPORT A TWELVE YEAR PATIENT
Kapilarni hemangiom testisa iznimno je rijedak tumor. Prikazujemo sluÄaj testikularnog hemangioma u namjeri da skrenemo pozornost na rijetke benigne neoplazme i poÅ”tednu kirurgiju testisa zahvaÄenog tumorom. Bolesnik je dvanaestogodiÅ”nji djeÄak, koji se prezentirao vodenom kilom i palpabilnim tumorom lijevog testisa. Ultrazvuk skrotuma i testisa pokazuje varikokelu II. stupnja, manji izljev u lijevom skrotumu te hipoehogenu zonu lijevog testisa veliÄine 5 mm u promjeru. Razina tumorskih markera u serumu (alfa-fetoproteina i beta-korionskoga gonadotropina) bila je u granicama referentnih vrijednosti. Intraoperativno je naÄinjena biopsija, a brza histoloÅ”ka analiza (na smrznutom preparatu) pokazuje kapilarni hemangiom. Potom je tumor potpuno odstranjen, a testis saÄuvan. KliniÄki, ultrazvuÄnim nalazom i nalazom magnetske rezonancije hemangiomi se ne razlikuju od malignih solidnih tumora testisa. Intraoperacijska brza patohistoloÅ”ka dijagnoza odreÄuje opsežnost kirurÅ”kog zahvata. Enukleacija hemangioma u zdravo tkivo testisa siguran je i opravdan kirurÅ”ki zahvat.Capillary hemangiomas of the testis are extremly rare tumors. We reported a case of intratesticular hemangioma, unusually localisation of this vascular benign neoplasm. The patient was 12 year old boy, with hydrocele and a palpabile testicular mass. Scrotal sonography revealed varicocele and hydrocele in the left scrotal sac. There was a solid hypoechogenic zone 5 mm in diameter in the left testis. Tumor markers like serum level of b human chorionic gonadotropin and a fetoprotein were normal. The patient underwent surgery, and intraoperativ frozen section showed a capillary hemangioma. The lesion was completly removed, but testis was preserved. Accordingly tu the literature, tumors of vascular origin are extremly rare. Capillary hemangiomas of the testis are similarity to malignant testicular solid tumors during physical examination, ultrasound examination and MR imaging. Before operation, itās hardly differentiate tumors of testis, but intraoperativ frozen section cuold be helpfull to differentiate a hemangioma from other testicular mass. Hemangioma is benign, but lesion must be complete removed to avoid recurence. In cases capillarx hemangimas, tumor enucleation with preservation tissue of the testis is possible if intraoperative frozen section examination can be performed
Kardiomiopatije u djece - DanaŔnja stajaliŔta i naŔa iskustva Hrvatska retrospektivna epidemioloŔka studija 1988. - 2016. [Cardiomyopathies in children - Current opinions and our experiences Croatian retrospective epidemiological study 1988-2016]
INTRODUCTION: Cardiomyopathies (CM) account for 3-5% of patients in the care of pediatric cardiologists.
They are found in all age groups, from fetal to adolescent age, and along with cardiology, teams from several
other pediatric subspecialties (neurology, metabolism, genetics) are also included. New findings have led to a
high survival rate. ----- GOAL: The primary goal is to present CM as an important part in the work of pediatric cardiologist
through an elaborate epidemiological study, current classifications, the latest diagnostic methods and treatments,
as well as the intertwining with other subspecialties. The secondary goal is to show that CM are no longer
āuncommon, insignificant and terminalā, but are common, significant and treatable diseases. ----- RESULTS: From January
1988 to December 2016 (28 years) in the Referral Center for Pediatric Cardiology, Department of Pediatrics,
Clinical Hospital Centre, 315 patients were diagnosed with cardiomyopathy,183 males (58.1%) and 132 females
(41.9%). In three different periods (10 , 12 and six years) a classification from 1996 was used (10). All three
periods have features of a population study, whereas the latter two also have features of an epidemiological study.
In all three periods there was a predominance of dilated cardiomyopathies (DCM) (42.5%) , followed by hypertrophic
cardiomyopathies (HCM) (37.1%) and restrictive cardiomyopathies (RCM) (6.7%) . Their relative relations
were in constant balance. A significant increase of some entity forms, arrhythmogenic right ventricular cardiomyopathies
(ARVCM) and non-compaction cardimyopathies (NCCM) has been observed, from 5.8% (1988-1998) to
16.2% (2010-2016). Owing to advances in diagnostic methods, number of unclassified CM has been decreasing significantly. The cause remained unknown in only 24.4% of DCM patients, and in 18.8% of HCM patients. In the
last 18 years the mortality rate of 7.4% (14/194) has been recorded, 50% (7/14) due to DCM. That is the result of
teamwork, targeted medical therapy, electrotherapy (electrical stimulation - ES, cardiac resynchronisation - CRS,
implantable cardioverter defibrilator - ICD) and surgical therapy (Morrow, pulmonary artery banding - PAB),
including heart transplantaton since 2011 (8 patients). Conclusion: Cardiomyopathies are after congenital heart
defects the most severe diseases under care of pediatric cardiologists. They require fluent teamwork of several
expert groups, and mastering of numerous diagnostic and therapeutic methods. Dilated cardiomyopathies are the
most common cause of death and the indication for heart transplatation in children
Cardiomyopathies in children ā Current opinions and our experiences Croatian retrospective epidemiological study 1988-2016
Uvod: Na kardiomiopatije (KM) otpada 3 ā 5% svih bolesnika za koje se skrbe pedijatrijski kardiolozi. Nalaze se u svim dobnim skupinama, od fetalne do adolescentne dobi, a u timskom radu obuhvaÄaju uz kardiologiju viÅ”e pedijatrijskih supspecijalnosti (neurologija, metabolizam, genetika). Zbog novih spoznaja i moguÄnosti lijeÄenja stupanj preživljenja sve je viÅ”i. Cilj: Primarni je cilj prikazati KM-e opsežnom retrospektivnom epidemioloÅ”kom studijom prema suvremenoj klasifikaciji, primjenom najnovijih dijagnostiÄkih metoda i terapijskih pristupa te prožimanjem s ostalim supspecijalnostima. Sekundarni je cilj pokazati da kardiomiopatije viÅ”e nisu ārijetke,
nevažne i neizljeÄiveā, nego su Äeste, važne i ljeÄive. Rezultati studije: U razdoblju od sijeÄnja 1988. do prosinca 2016. (28 godina) u Referentnom centru za pedijatrijsku kardiologiju Klinike za pedijatriju KBC-a Zagreb hospitalizirano je 315 bolesnika s dijagnozom kardiomiopatije, 183 muÅ”kog (58,1%) i 132 ženskog (41,9%) spola. U tri razliÄita razdoblja (10 godina, 12 godina, 6 godina) služili smo se klasifikacijom KM-a iz 1996. godine. Sva tri razdoblja imaju obilježja populacijske, a posljednja dva imaju obilježja i kliniÄke epidemioloÅ”ke studije. U sva tri razdoblja najÄeÅ”Äa je dilatacijska kardiomiopatija (DKM) (42,5%), a slijede hipertrofiÄna (HKM) (37,1%) i restrikcijska (RKM) (6,7%). Njihovi su relativni odnosi u stalnoj ravnoteži. UoÄava se znatan porast entitetskih oblika: aritmogena desnoventrikularna kardiomiopatija (ADVKM) i nekompaktna kardiomiopatija (NKKM), i to od 5,8% (1988. ā 1998.) na 16,2% (2010. ā 2016.). Smanjen je broj KM-a nepoznata uzroka: kod DKM-a 24,4%, a kod HKM-a tek 18,8%. U posljednjih 18 godina zabilježili smo smrtnost od 7,4% (14/194), od Äega 50% (7/14) otpada na DKM. Rezultat je to timskog rada, ciljane medikamentne terapije, elektroterapije (elektrostimulator ā ES, srÄana resinkronizacija ā CRS, implantabilni kardioverterski defibrilator ā ICD) i kardiokirurÅ”ke terapije (operacija prema Morrowu, zaomÄavanje (engl. banding) pluÄne arterije ā PAB), ukljuÄujuÄi i presadbu srca od 2011. godine (8 bolesnika). ZakljuÄak: Kardiomiopatije su nakon priroÄenih srÄanih grjeÅ”aka najteže bolesti u skrbi pedijatrijskog kardiologa, nalažu skladan timski rad viÅ”e skupina struÄnjaka te svladavanje brojnih dijagnostiÄkih i terapijskih metoda. Dilatacijske kardiomiopatije najÄeÅ”Äi su uzrok smrti i najÄeÅ”Äa indikacija za presadbu srca u djece.Introduction: Cardiomyopathies (CM) account for 3-5% of patients in the care of pediatric cardiologists. They are found in all age groups, from fetal to adolescent age, and along with cardiology, teams from several other pediatric subspecialties (neurology, metabolism, genetics) are also included. New findings have led to a high survival rate. Goal: The primary goal is to present CM as an important part in the work of pediatric cardiologist through an elaborate epidemiological study, current classifications, the latest diagnostic methods and treatments,
as well as the intertwining with other subspecialties. The secondary goal is to show that CM are no longer āuncommon, insignificant and terminalā, but are common, significant and treatable diseases. Results: From January 1988 to December 2016 (28 years) in the Referral Center for Pediatric Cardiology, Department of Pediatrics, Clinical Hospital Centre, 315 patients were diagnosed with cardiomyopathy,183 males (58.1%) and 132 females (41.9%). In three different periods (10 , 12 and six years) a classification from 1996 was used (10). All three periods have features of a population study, whereas the latter two also have features of an epidemiological study. In all three periods there was a predominance of dilated cardiomyopathies (DCM) (42.5%) , followed by hypertrophic cardiomyopathies (HCM) (37.1%) and restrictive cardiomyopathies (RCM) (6.7%) . Their relative relations were in constant balance. A significant increase of some entity forms, arrhythmogenic right ventricular cardiomyopathies (ARVCM) and non-compaction cardimyopathies (NCCM) has been observed, from 5.8% (1988-1998) to 16.2% (2010-2016). Owing to advances in diagnostic methods, number of unclassified CM has been decreasing significantly. The cause remained unknown in only 24.4% of DCM patients, and in 18.8% of HCM patients. In the last 18 years the mortality rate of 7.4% (14/194) has been recorded, 50% (7/14) due to DCM. That is the result of teamwork, targeted medical therapy, electrotherapy (electrical stimulation - ES, cardiac resynchronisation - CRS, implantable cardioverter defibrilator - ICD) and surgical therapy (Morrow, pulmonary artery banding - PAB), including heart transplantaton since 2011 (8 patients). Conclusion: Cardiomyopathies are after congenital heart defects the most severe diseases under care of pediatric cardiologists. They require fluent teamwork of several expert groups, and mastering of numerous diagnostic and therapeutic methods. Dilated cardiomyopathies are the most common cause of death and the indication for heart transplatation in children
Amyloidosis of the aortic root in a patient with polyarteritis nodosa [Amiloidoza korijenja aorte u bolesnika s nodoznim poliarteritisom]
Amyloidosis results from the deposition of insoluble fibrillar proteins in various tissues. Cardiac symptoms, when present, are usually related to congestive heart failure or arrhythmias. Amyloid involvement of the aortic root is exceedingly rare. We describe a case of aortic root amyloidosis in a patient with a long-standing history ofpolyarteritis nodosa
Kapilarni hemangiom testisa, prikaz dvanaestogodiŔnjeg bolesnika [Testicular capillary hemangioma, report a twelve year patient]
Capillary hemangiomas of the testis are extremly rare tumors. We reported a case of intratesticular hemangioma, unusually localisation of this vascular benign neoplasm. The patient was 12 year old boy, with hydrocele and a palpabile testicular mass. Scrotal sonography revealed varicocele and hydrocele in the left scrotal sac. There was a solid hypoechogenic zone 5 mm in diameter in the left testis. Tumor markers like serum level of beta human chorionic gonadotropin and alpha fetoprotein were normal. The patient underwent surgery, and intraoperativ frozen section showed a capillary hemangioma. The lesion was completly removed, but testis was preserved. Accordingly tu the literature, tumors of vascular origin are extremly rare. Capillary hemangiomas of the testis are similarity to malignant testicular solid tumors during physical examination, ultrasound examination and MR imaging. Before operation, it's hardly differentiate tumors of testis, but intraoperativ frozen section cuold be helpfull to differentiate a hemangioma from other testicular mass. Hemangioma is benign, but lesion must be complete removed to avoid recurence. In cases capillarx hemangimas, tumor enucleation with preservation tissue of the testis is possible if intraoperative frozen section examination can be performed