Background To evaluate long-term outcome of tetralogy of Fallot repair analyzing an unbiased country-wide surgically treated population with tetralogy of Fallot. Methods and Results Retrospective analysis of consecutive patients aged <18 years who underwent tetralogy of Fallot repair at a single nationwide pediatric cardiac center. Death from any cause and need for surgical or catheter reintervention were the study end points. Cox regression analysis was used to identify related risk factors. A total of 917 patients (male, 56.3%) were analyzed. Staged repair was performed in 16.9%. Early mortality (24/917, 2.62% patients) was confined to the early surgical eras. Late mortality was 4.5% (40/893 patients). Survival probability was 95.1%, 93.8% and 91.9% at 10, 20 and 30 years after repair, respectively. Early surgical era (P=0.013) and surgical/catheter reinterventions (P<0.001) were multivariable predictors of late death. A total of 487 reinterventions were performed after initial repair in 253/917 patients (27.6%), with pulmonary artery revalvulation (196/917 patients, 21.4%) being most frequent. Probability of freedom from first reintervention was 89.0%, 73.3%, and 55.1% at 10, 20, and 30 years after primary repair, respectively. Transannular repair was associated with the need for pulmonary artery revalvulation (P<0.001). Patients who underwent staged repair were more likely to need reinterventions on pulmonary arteries (P<0.001). Conclusions In an unbiased nationwide cohort, tetralogy of Fallot repair carried a favorable survival of >90% at 30 years. Each reintervention significantly incrementally increased the risk of mortality. Type of initial repair predicted the need for specific surgical or catheter reinterventions.
- MeSH
- dítě MeSH
- Fallotova tetralogie * chirurgie MeSH
- kojenec MeSH
- lidé MeSH
- následné studie MeSH
- plicní chlopeň * chirurgie MeSH
- reoperace MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- svoboda MeSH
- výsledek terapie MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Severe aortic stenosis can be accompanied by various degrees of left ventricular underdevelopment. The assessment whether a borderline-sized left ventricle can or cannot support the systemic circulation is crucial. The decision-making still remains challenging. We present a case that illustrates that the development of haemodynamic parameters can be difficult to estimate, even in the long term. The patient went from biventricular to univentricular circulation and back and could finally be palliated by heart transplantation. Modern technology including long-term mechanical cardiac support as a bridge to candidacy and drug therapy for pulmonary hypertension were vital to successfully combat a previously lethal disease.
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.
- 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 MeSH
Aims: Right bundle branch block is associated with right ventricular (RV) electromechanical dyssynchrony, which may contribute to acute haemodynamic impairment after repair of tetralogy of Fallot (ToF). We sought to evaluate the effects of RV resynchronization on haemodynamics and tissue oxygenation during the first 24 h after surgery. Methods and results: Arterial pressures, cardiac output, and tissue oxygenation were measured in 28 consecutive patients (median age 10.1 months) during baseline sinus rhythm with right bundle branch block and after RV resynchronization by atrial-triggered RV free wall pacing in complete fusion with spontaneous activation. Studied variables were compared in a crossover design in four 5-min intervals (baseline rhythm and stimulation, 2x each). Resynchronization reduced the QRS complex duration from median 110 to 70 ms (P < 0.001), increased significantly median arterial systolic, mean and pulse pressure, cardiac index, left ventricular maximum +dP/dT and decreased central venous pressure (P < 0.001 for all). Both cerebral and renal oxygenation improved (P < 0.001). Eleven of the 28 patients showed a clinically highly significant resynchronization effect defined as an increase in arterial pulse pressure of ≥ 10%. The q-RV interval (expressed as % of QRS duration) at the RV pacing site during baseline rhythm was the only predictor of resynchronization effect. Conclusions: RV resynchronization carried short-term improvement of haemodynamics in children early after surgery for ToF and might be a useful non-pharmacologic adjunct to the management of haemodynamically compromised patients. Resynchronization effect was maximized when pacing from area of the latest RV activation.
- MeSH
- blokáda Tawarova raménka diagnóza patofyziologie terapie MeSH
- časové faktory MeSH
- Fallotova tetralogie diagnóza patofyziologie chirurgie MeSH
- funkce pravé komory srdeční * MeSH
- hemodynamika * MeSH
- kardiochirurgické výkony * MeSH
- kojenec MeSH
- lidé MeSH
- obnova funkce MeSH
- srdeční resynchronizační terapie * MeSH
- věkové faktory MeSH
- výsledek terapie MeSH
- Check Tag
- kojenec MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX. METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%). RESULTS: The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30 days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9 years (range 0-23.7 years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04). CONCLUSIONS: Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.
- MeSH
- časové faktory MeSH
- dítě MeSH
- dospělí MeSH
- Fontanova operace škodlivé účinky mortalita MeSH
- Kaplanův-Meierův odhad MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- neúspěšná terapie MeSH
- pooperační komplikace diagnóza mortalita patofyziologie chirurgie MeSH
- předškolní dítě MeSH
- proporcionální rizikové modely MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- transplantace srdce * škodlivé účinky mortalita MeSH
- vrozené srdeční vady diagnóza mortalita chirurgie MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- pozorovací studie MeSH
- Geografické názvy
- Evropa MeSH
