scholarly journals Surgical management of congenital heart defects associated with heterotaxy syndrome☆

2010 ◽  
Vol 38 (6) ◽  
pp. 721-727 ◽  
Author(s):  
Alain Serraf ◽  
Nawel Bensari ◽  
Lucile Houyel ◽  
André Capderou ◽  
Régine Roussin ◽  
...  
Keyword(s):  
Surgical Management ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Heterotaxy Syndrome

Surgical management of congenital heart defects in adolescent and adult patients, between years 2001–2008

2009 ◽  
Vol 150 (37) ◽  
pp. 1739-1743 ◽  
Author(s):  
István Hartyánszky ◽  
Andrea Székely ◽  
László Király ◽  
Zsolt Prodán ◽  
Sándor Mihályi ◽  
...  
Keyword(s):  
Surgical Management ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Adult Patients

A felnőttkorban operált veleszületett szívhibák között vezetnek az I. rekonstrukciós beavatkozások: a) frissen felismert betegségek, b) megelőzően inoperábilisnak ítélt kórképek, c) pulmonalis hypertonia, jobbkamra-elégtelenség miatt „elkésett” műtétek. Növekszik a II. REDO műtétek száma: a) residuumok korrigálása, b) kinőtt, diszfunkciós homograftok cseréje, c) műtéti/intervenciós korrigálás utáni recoarctatio (aneurysma, dissectio) sebészete, d) aorta valvulotomia/valvuloplastica, illetve társvitiumok (TGA) korrigálásának következményeként Ross-műtét, műbillentyű-beültetés . Betegek, eredmények: A 2001–2008 között végzett 4496 műtét közül 166 volt fiatal-felnőtt korú (16–52, átlagéletkor: 28 év) (Ia: 77, Ib: 15, Ic: 4, IIa: 11, IIb: 22, IIc: 9, IId: 28). Műtéti mortalitás nem volt, 1 beteg pulmonalis hypertoniás krízisben, 1 jobbkamra-elégtelenség miatti malignus ritmuszavarban, 2 többszerv-elégtelenségben halt meg. Konklúzió: A rizikófaktorokat a pulmonalis hypertonia és a jobbkamra-elégtelenség jelenti. A bonyolult sebészi megoldások a „congenitalis szívsebész” számára nem jelentenek problémát, de koraibb diagnózisok, terápiában az extracorporalis membránoxigenátor használata az eredményeket javíthatja.

scholarly journals Genetic syndromes and congenital heart defects: how is surgical management affected?

2009 ◽  
Vol 35 (4) ◽  
pp. 606-614 ◽  
Author(s):  
Roberto Formigari ◽  
Guido Michielon ◽  
Maria Cristina Digilio ◽  
Gerardo Piacentini ◽  
Adriano Carotti ◽  
...  
Keyword(s):  
Surgical Management ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Genetic Syndromes

scholarly journals Postmortem Assessment of Isolated Congenital Heart Defects Remains Essential Following Termination of Pregnancy

2021 ◽  
pp. 109352662110161
Author(s):  
Camilla Struksnæs ◽  
Harm-Gerd K Blaas ◽  
Sturla H Eik-Nes ◽  
Eva Tegnander ◽  
Christina Vogt
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Main Diagnosis ◽  
Heterotaxy Syndrome ◽  
Autopsy Findings ◽  
Tertiary Center ◽  
The Us ◽  
Icd 10 ◽  
Left Heart Syndrome

Objectives To investigate the correlation between prenatal ultrasound (US) and autopsy findings in pregnancies terminated due to isolated congenital heart defects (CHDs), including CHDs associated with heterotaxy syndrome. Materials and methods The material consists of 67 fetuses with prenatally detected isolated CHDs or CHDs associated with heterotaxy syndrome at a tertiary center in Norway between 1985 and 2014. The main CHDs were categorized into subdiagnoses of CHDs in accordance with ICD-10. The US and autopsy findings were categorized according to degree of concordance. Results Gestational age at termination was 12 + 0–22 + 6 weeks. Hypoplastic left heart syndrome was the most common main diagnosis among the 67 fetuses (32.8%). There was full agreement between US and autopsy findings in 97.4% (222/228) of all subdiagnoses. The discrepant findings in three fetuses had no influence on the decision to terminate the pregnancy. Conclusions The correlation was high between prenatal US and postmortem findings in fetuses with isolated CHDs. Meticulous assessment of cardiac anatomy is particularly necessary when the decision to terminate relies on isolated CHDs. The trend of earlier termination challenges verification of diagnoses at autopsy. Consequently, the fetus should be examined at a tertiary center with fetal medicine specialists, pediatric cardiologists and perinatal pathologists.

scholarly journals Szemléletváltozás a Down-szindrómás szívbetegek szívsebészeti kezelésében, 1974–2016

2016 ◽  
Vol 157 (40) ◽  
pp. 1601-1603
Author(s):  
István Hartyánszky ◽  
Gábor Bogáts
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Life Expectancy ◽  
Surgical Management ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Cardiac Surgeons ◽  
The Impact

Introduction: Congenital heart defects are frequently present in patients with Down syndrome. Aim: The authors analyzed the impact of changing approach in surgical management of congenital heart defect on the life expectancy of patients with Down syndrome. Method: Between 1974 and 1997 the data of 359 children with Down syndrome were collected. Among them 255 patients had no surgery and the mortality in this group was 25.9%, whereas the mortality in the group of 104 patients who underwent palliative surgery was 8.6%. Results: Surgical management of congenital heart defects provides the same life expectancy for these patients as compared to Down patients without cardiac defects. Primary reconstruction is the preferable surgical procedure in infancy that provides good results. Nowadays the number of the operated grown-up congenital heart disease patients with Down syndrome is increasing. During the last three years 82 grown-up congenital heart disease patients, including 4 patients with Down syndrome (aged between 24 and 60 years) were reconstructed successfully. Conclusions: Due to the successful surgery in infancy the population of grown-up congenital heart disease patients with Down syndrome is increasing. The cardiac surgeons are ready to do everything for the optimal life expectancy of these patients. However, management of special problems (indication and necessity of reoperation, optimal age) in patients with Down syndrome poses a great challenge for cardiologists and cardiac surgeons. Orv. Hetil., 2016, 157(40), 1601–1603.

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