BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

• Klíčová slova
• Adult congenital heart disease, Congestive heart failure, Heart transplantation, Mechanical circulatory support, Systemic right ventricle, Transposition of the great arteries,
• MeSH
• dítě MeSH
• dospělí MeSH
• dysfunkce pravé srdeční komory * komplikace   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• retrospektivní studie MeSH
• srdeční selhání * komplikace   MeSH
• transpozice velkých cév * komplikace   chirurgie   MeSH
• trikuspidální insuficience * komplikace   MeSH
• vrozeně korigovaná transpozice velkých tepen MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• Research Support, N.I.H., Extramural MeSH

BACKGROUND: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. OBJECTIVES: The authors aimed to determine factors associated with survival in a large cohort of such individuals. METHODS: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). RESULTS: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. CONCLUSIONS: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

• Klíčová slova
• QRS duration, atrial arrhythmia, congenital heart disease, congestive heart failure, systemic right ventricle, transposition of the great arteries,
• MeSH
• arterie MeSH
• dospělí MeSH
• korekce transpozice velkých arterií * škodlivé účinky   MeSH
• lidé MeSH
• následné studie MeSH
• retrospektivní studie MeSH
• srdeční selhání * epidemiologie   etiologie   MeSH
• transpozice velkých cév * chirurgie   MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• Research Support, N.I.H., Extramural MeSH

Background The aim of this study was to evaluate long-term survival and freedom from coronary artery reintervention after the arterial switch operation (ASO). Methods and Results This single-center nationwide retrospective study included consecutive children who underwent ASO between 1990 and 2016 (n=605). Long-term outcomes were obtained by cross-mapping individual data with the National Death Registry and the National Registry of Cardiovascular Interventions for adults. A control group was randomly retrieved at a 1:10 ratio from the National Birth and Death Registries. Early mortality was 3.3% and late mortality was 1.7% during a median follow-up of 10 (interquartile range, 5-16) years. The probability of overall survival at 20 years after ASO was 94.9% compared with 99.5% in the background population (hazard ratio [HR] 15.6; 95% CI, 8.9-27.5, P<0.001). Independent multivariable predictors of worse survival were an intramural coronary artery (HR, 5.2; 95% CI, 1.8-15.2, P=0.002) and period of ASO 1990 to 1999 (HR, 4.6; 95% CI, 1.5-13.6, P<0.001). Fourteen patients (2.3%) required 16 coronary artery reoperations. Freedom from coronary artery reintervention at 20 years after ASO was 96%. The only independent multivariable predictor associated with a higher hazard for coronary artery reintervention was an intramural coronary artery (HR, 33.9; 95% CI, 11.8-97.5, P<0.001). Conclusions Long-term survival after ASO is excellent. Coronary artery reinterventions are rare. An intramural coronary artery was an independent predictor associated with a higher risk for coronary artery reintervention and death, regardless of the surgical period.

• Klíčová slova
• congenital heart disease, coronary artery anomaly, transposition of great vessels,
• MeSH
• anomálie koronárních cév diagnostické zobrazování   epidemiologie   chirurgie   MeSH
• časové faktory MeSH
• dítě MeSH
• doba přežití bez progrese choroby MeSH
• dospělí MeSH
• hodnocení rizik MeSH
• kojenec MeSH
• korekce transpozice velkých arterií * škodlivé účinky   mortalita   MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• novorozenec MeSH
• pooperační komplikace mortalita   chirurgie   MeSH
• předškolní dítě MeSH
• přežívající MeSH
• registrace MeSH
• reoperace MeSH
• replantace * škodlivé účinky   mortalita   MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• transpozice velkých cév diagnostické zobrazování   epidemiologie   chirurgie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• srovnávací studie MeSH
• Geografické názvy
• Česká republika epidemiologie   MeSH

BACKGROUND: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type. METHODS: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n = 99) and DORV TGA-type (n = 12). Main indications for LSR were neoaortic valve insufficiency (n = 52 [47%]) and coronary artery problems (CAPs) (n = 21 [19%]). RESULTS: Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p = 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%]). CONCLUSIONS: Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required.

• MeSH
• aortální insuficience epidemiologie   etiologie   chirurgie   MeSH
• dítě MeSH
• dvojvýtoková pravá komora chirurgie   MeSH
• incidence MeSH
• kojenec MeSH
• korekce transpozice velkých arterií škodlivé účinky   MeSH
• lidé MeSH
• míra přežití trendy   MeSH
• mladiství MeSH
• následné studie MeSH
• pooperační komplikace epidemiologie   etiologie   chirurgie   MeSH
• předškolní dítě MeSH
• prognóza MeSH
• reoperace metody   MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• transpozice velkých cév chirurgie   MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

OBJECTIVE: The patients after Mustard and Senning corrections of transposition of the great arteries (TGA) are at an increased risk of unexpected death. The aim of this study was to identify markers allowing risk stratification of patients after atrial switch correction of TGA to provide them with optimum care. METHODS AND RESULTS: In this study, 87 patients were retrospectively evaluated after atrial switch correction of TGA followed-up between 2005 and 2015. The mortality during the follow-up was 9% (8 cardiac deaths). Markers significantly predictive of death using univariable Cox proportional hazard ratio survival analysis were: N-terminal pro-B-type natriuretic peptide (NT-proBNP), ejection fraction and end-diastolic dimension of the systemic right ventricle, mitral E, e', and s'. Surprisingly, the Doppler parameters of mitral valve in subpulmonary ventricle were more important for prognosis than those of systemic tricuspid valve. In multivariable analysis, the only independent predictors of mortality were NT-proBNP (P = .00048; AUC 0.97) and the velocity of early diastolic filling (mitral E) in subpulmonary ventricle (P = .01815; AUC 0.81). According to Kaplan-Meier survival analysis, patients with NT-proBNP > 1000 pg/ml are at high risk of death. Patients with mitral E < 68 cm/s are also at an increased risk of death. CONCLUSIONS: NT-proBNP is the most reliable prognostic mortality factor and should be measured regularly in TGA patients after Mustard or Senning correction. Diastolic filling velocity of the subpulmonary left ventricle (mitral E) may be more important for prognosis than systolic function of the systemic right ventricle.

• Klíčová slova
• Mustard and Senning operation, NT-proBNP, adult congenital heart disease, mitral E, mortality, transposition of the great arteries,
• MeSH
• biologické markery krev   MeSH
• časové faktory MeSH
• dopplerovská echokardiografie MeSH
• dospělí MeSH
• hodnocení rizik metody   MeSH
• kardiochirurgické výkony metody   MeSH
• lidé MeSH
• míra přežití trendy   MeSH
• mladý dospělý MeSH
• následné studie MeSH
• natriuretický peptid typu B krev   MeSH
• peptidové fragmenty krev   MeSH
• pooperační období MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• transpozice velkých cév krev   mortalita   chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika epidemiologie   MeSH
• Názvy látek
• biologické markery MeSH
• natriuretický peptid typu B MeSH
• peptidové fragmenty MeSH
• pro-brain natriuretic peptide (1-76) MeSH Prohlížeč

BACKGROUND: There is limited clinical experience with bridging to transplant with a left ventricular assist device (VAD) in patients with previously palliated transposition of great arteries. METHODS: Five adult patients presenting with systemic right ventricular failure 30 years after a Mustard operation were implanted with a HeartMate II VAD. The implant was completed using standard procedures with only minor modifications to accommodate right ventricular cannulation. RESULTS: All 5 patients were men, with a mean age of 31.5 ± 1.8 years and a median time since Mustard operation of 30 (range 28 to 32) years. All patients had sternal closure on Post-operative Day (POD) 1, and 2 patients required additional re-operation for bleeding. One patient required temporary support of the non-systemic ventricle. The mean duration of VAD support was 284 ± 177 days; 3 patients underwent heart transplant and 2 died on PODs 502 and 34, respectively. Both deaths were due to progressive heart failure and pump thrombosis. Comorbidities, anatomy and mediastinal scarring did not preclude implantation and heart failure symptoms improved in all patients. CONCLUSIONS: With the increased prevalence of late post-Mustard heart failure, bridge to transplant with a VAD may be a suitable treatment option for patients who are severely ill.

• Klíčová slova
• Mustard, VAD, bridge to transplant, congenital heart disease, systemic right ventricle, transposition,
• MeSH
• dospělí MeSH
• dysfunkce pravé srdeční komory chirurgie   MeSH
• korekce transpozice velkých arterií * MeSH
• lidé MeSH
• podpůrné srdeční systémy * MeSH
• transplantace srdce * mortalita   MeSH
• transpozice velkých cév chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• dospělí MeSH
• lidé MeSH
• počítačová rentgenová tomografie MeSH
• podpůrné srdeční systémy * MeSH
• srdeční komory diagnostické zobrazování   chirurgie   MeSH
• transpozice velkých cév chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH

• MeSH
• aneurysma diagnostické zobrazování   chirurgie   MeSH
• arteria pulmonalis diagnostické zobrazování   chirurgie   MeSH
• elektrokardiografie MeSH
• kinematografická angiografie MeSH
• lidé středního věku MeSH
• lidé MeSH
• obstrukce výtoku ze srdeční komory chirurgie   MeSH
• pooperační komplikace diagnostické zobrazování   chirurgie   MeSH
• transpozice velkých cév chirurgie   MeSH
• ultrasonografie MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

BACKGROUND: Most adolescents and young adults born with transposition of the great arteries and survivors of Mustard operation are alive today and are leading ordinary lives. How far is the quality of their life influenced by residual findings? METHODS: We have analysed the oldest group of 177 children operated on with the Mustard procedure during 1979-1984 from our total experience of 609 corrections of transposition of the great arteries until 1996. In the long-term follow-up 12 to 18 years after Mustard operation the clinical investigation, 24 hours ECG Holter monitoring, 2D and colour flow Doppler echocardiography, radionuclide angiocardiography and exercise testing were performed. RESULTS: Basic sinus rhythm had 61% of 137 survivors, sinus node dysfunction had 51%. All but 4 patients needed no antiarrhythmic therapy. There were 10 sudden deaths. Severe tricuspid valve regurgitation developed in 15.9% of children, and decreased to 6% after intensive medical treatment. The right ventricular systolic dysfunction was found on radionuclide ventriculography in 8% of children after surgery. The left ventricular ejection fraction was subnormal in 10% and left ventricular diastolic function expressed by rapid filling fraction was subnormal in 80% of patients and may cause inability to increase cardiac output at exercise The obstruction of caval veins was found in 9%. There were neurological complications in 13.3% (10% already preoperatively) but severe brain damage was found in 4.4% only. CONCLUSIONS: Exercise tolerance with the maximum oxygen consumption over 30 ml/kg/min in 96% of investigated children suggests the good ability and full efficiency for recreation sports. Most survivors (84%) are reported to lead ordinary lives and function in NYHA class I.

• MeSH
• časové faktory MeSH
• dítě MeSH
• dopplerovská echokardiografie MeSH
• dospělí MeSH
• elektrokardiografie ambulantní MeSH
• kardiochirurgické výkony škodlivé účinky   MeSH
• kojenec MeSH
• komorová dysfunkce diagnóza   etiologie   patofyziologie   MeSH
• kontrakce myokardu MeSH
• kvalita života * MeSH
• lidé MeSH
• míra přežití MeSH
• mladiství MeSH
• následné studie MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• prognóza MeSH
• radioisotopová ventrikulografie MeSH
• retrospektivní studie MeSH
• srdeční selhání etiologie   mortalita   patofyziologie   MeSH
• srdeční síně abnormality   chirurgie   MeSH
• tepový objem MeSH
• transpozice velkých cév diagnóza   chirurgie   MeSH
• trikuspidální insuficience diagnóza   etiologie   patofyziologie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH

Primary repair of transposition of the great arteries with interrupted aortic arch was performed in a 2 day old neonate weighing 2.1 kg. The repair consisted of a direct anastomosis between the ascending and the descending aorta and an arterial switch repair without Lecompte procedure because of the side by side position of the great arteries and an anomaly of the coronary arteries. A total of 28 months after the repair, the patient remains in excellent clinical condition.

• MeSH
• aorta thoracica abnormality   MeSH
• kardiovaskulární chirurgické výkony metody   MeSH
• lidé MeSH
• novorozenec MeSH
• transpozice velkých cév komplikace   chirurgie   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH