Survival and Freedom From Reinterventions in Patients With Repaired Tetralogy of Fallot: Up to 42-Year Follow-Up of 917 Patients.

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.

Long-Term Survival and Freedom From Coronary Artery Reintervention After Arterial Switch Operation for Transposition of the Great Arteries: A Population-Based Nationwide Study.

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.

[Czech paediatric cardiac surgery - history and presence]. / Ceská detská kardiochirurgie - historie a soucasnost.

The beginnings of the Paediatric Cardiac Surgery in the Czech Republic date back to the period immediately after the end of World War II. Its protagonists were Prof. Emerich Polák from the Surgical Clinic in Prague, Vinohrady, Prof. Jan Bedrna from Surgical Clinic in Hradec Kralove, Prof. Vladislav Rapant from Surgical Clinic in Olomouc and Prof. Václav Kafka from the Second Surgical Clinic in Prague. They started with operations of the patent ductus arteriosus, the Blalock-Taussig shunt in cyanotic heart defects and resection of coarctation of the aorta. Operations of congenital heart defects, on the open heart were elaborated namely by cardiosurgeons in Brno, under the leadership of Professor Jan Navrátil. On the extension of those methods participated Professor Jaroslav Procházka in Hradec Kralove and Prof. Václav Kafka at the newly opened department of Paediatric surgery in Prague. In the next period, attention of paediatric cardiac surgery was directed at operations of critical congenital heart defects in the smallest children. Palliative operations of the critical heart defects in newborns and infants were first introduced at the clinic of paediatric surgery of the Paediatric University Hospital in Prague. Radical operations of infants and newborns with extra-corporal circulation were elaborated in the Children's heart centre in Prague, Motol. Initiative in the further development of paediatric cardiac surgery was taken over by the Children's heart centre in Prague since its founding in 1977. There was concentrated all medical care of children born with a congenital heart defect in the Czech Republic. This concentration of specialized care at one institution allowed to accumulate extremely large experience with the diagnostics and surgical treatment of congenital heart defects in all age groups with the decrease of patients mortality after operations to 1% even for the smallest children and enabled continuously monitor the quality of life of patients treated for congenital heart defect up to adulthood. It was followed by a system of care for adults with congenital heart defect in cooperation with cardiac surgery departments for adults. Excellent level of paediatric cardiac surgery in the Czech Republic is currently confirmed by comparison of the results in the global database operations of congenital heart defects, led by the European Association for Cardiothoracic Surgery (EACTS).

Aggressive tumoral calcinosis in an infant thoracotomy scar.

The authors report a case of tumoral calcinosis (TC) in a six-month-old infant, which developed within the thoracotomy scar from previous aortic coarctation repair. After initial resection of the lesion, the child returned with a large mass of TC restricting movement of the left shoulder. Repeated total resection was successful with no recurrence in 12 months' follow-up. This is the first report of TC that developed in a postoperative scar and is unusual in its recurrence and aggressive growth. Pathogenesis, diagnosis and treatment of this rare event is discussed.

Improved results after the primary repair of interrupted aortic arch: impact of a new management protocol with isolated cerebral perfusion.

OBJECTIVE: The aim of this retrospective study was to analyse results and risk factors of death after the repair of the interrupted aortic arch, and to compare results obtained with the use of deep hypothermic circulatory arrest versus isolated cerebral perfusion. METHODS: The primary repair of the interrupted aortic arch and associated heart lesions was performed in 50 consecutive patients. The median age was 5 days and the mean weight was 3.1+/-0.6 kg. The interrupted aortic arch was of type A in 12 (24%) patients, type B in 37 (74%) and type C in one (2%) patient. Ventricular septal defect was present in 48 (96%) patients, subaortic stenosis in 15 (30%), truncus arteriosus in 14 (28%), transposition of the great arteries in two (4%), aortopulmonary window in two (4%) and double-outlet right ventricle in one (2%). The surgery consisted of reconstruction of the aortic arch by direct anastomosis and repair of associated heart lesions. In 25 (50%) patients, aortic arch reconstruction was performed using hypothermic circulatory arrest (group I) and in 25 by isolated cerebral perfusion (group II). The duration of cardiopulmonary bypass, aortic cross-clamping and circulatory arrest or isolated cerebral perfusion was 191+/-46 min, 90+/-24 min and 40+/-14 min, respectively, in group I; and 194+/-39 min, 74+/-20 min and 31+/-6 min, respectively, in group II. RESULTS: There were 10 (20%) deaths in this series, eight (32%) in group I and two (8%) in group II. Out of 12 patients operated before 1995, seven (58%) patients died; and out of 38 patients operated between 1995 and 2009, three (8%) patients died (p=0.008). By Cox multifactorial analysis, the earlier date of operation represented the only risk factor of death (p=0.037). Twelve (71%) survivors in group I and five (22%) survivors in group II required re-intervention, most often for subaortic stenosis, aortic arch obstruction or conduit obstruction. All patients remain in the New York Heart Association (NYHA) class I or II at median 12.6 years in group I, and 1.7 years in group II, respectively, after surgery. CONCLUSIONS: Interrupted aortic arch can be repaired in neonates with a mortality of 5-10%. The results depend on experience. Isolated cerebral perfusion was joined with decreased mortality but it did not influence the occurrence of neurological complications.

Long-term results after correction of persistent truncus arteriosus in 83 patients.

OBJECTIVE: This study aims to analyse long-term results after correction of truncus arteriosus in all patients operated in one institution over 28 years. METHODS: Between 1981 and 2009, 83 patients, median age 54 days, underwent repair of truncus arteriosus. Interrupted aortic arch was present in 14 (17%), severe truncal valve insufficiency in 10 (12%) and non-confluent pulmonary arteries in five (6%) patients. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 80, and other methods in three patients. At the same time, correction of interrupted aortic arch was done in 14 and truncal valve repair in eight patients. Survivors were repeatedly examined echocardiographically for assessment of residual heart lesions. RESULTS: The early mortality was 19 (23%). Out of 35 patients operated between 1981 and 1996, 17 (46%) died, and out of 48 patients operated between 1997 and 2009, two (4%) died. Operation before 1997 (p=0.001) and aortic cross-clamping time >90min (p=0.009) were found to be risk factors of death. Eight (10%) patients died late, a median of 68 days after surgery. Fifty-seven (69%) patients were followed for 10.9 + or - 6.7 years. Three (4%) patients were lost. Twenty-five (30%) patients are alive with their original conduit 7.5 + or - 5.2 years after correction. Twenty-eight patients required 41 re-operations for conduit dysfunction with first replacement at mean 5.8 + or - 4.1 (range 0.1-14.1 years) years after correction. Nine (11%) patients required 12 truncal valve replacements. Eleven (13%) patients required balloon dilatation or stent for conduit obstruction, pulmonary branch stenosis, aortic arch obstruction or stenosis of vena cava. Recent clinical examination was undertaken in 53 (64%) patients and 49 (59% or 77% of early survivors) are in good/very good condition. CONCLUSIONS: Truncus arteriosus remains a challenging heart disease. With growing experience, the early mortality decreased to 4%, but numerous re-interventions for conduit obstruction, pulmonary branch stenosis and truncal valve insufficiency are required. Surgery before 1997 and prolonged cross-clamping were risk factors of death. Pulmonary homografts had the best re-intervention-free survival. Statistically, however, the difference between conduits was not significant. Dysplastic valve and truncal valve insufficiency represent risk factors presenting the need for truncal valve replacement.

Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.

Cardiac resynchronization therapy: a novel adjunct to the treatment and prevention of systemic right ventricular failure.

OBJECTIVES: This study aimed to evaluate the technical feasibility and hemodynamic benefit of cardiac resynchronization therapy (CRT) in patients with systemic right ventricle (RV). BACKGROUND: Patients with a systemic RV are at high risk of developing heart failure. Cardiac resynchronization therapy may improve RV function in those with electromechanical dyssynchrony. METHODS: Eight patients (age 6.9 to 29.2 years) with a systemic RV and right bundle-branch block (n = 2) or pacing from the left ventricle (LV) (n = 6) with a QRS interval of 161 +/- 21 ms underwent CRT (associated with cardiac surgery aimed at decrease in tricuspid regurgitation in 3 of 8 patients) and were followed-up for a median of 17.4 months. RESULTS: Change from baseline rhythm to CRT was accompanied by a decrease in QRS interval (-28.0%, p = 0.002) and interventricular mechanical delay (-16.7%, p = 0.047) and immediate improvement in the RV filling time (+10.9%, p = 0.002), Tei index (-7.7%, p = 0.008), estimated RV maximum +dP/dt(+45.9%, p = 0.007), aortic velocity-time integral (+7.0%, p = 0.028), and RV ejection fraction by radionuclide ventriculography (+9.6%, p = 0.04). The RV fractional area of change increased from a median of 18.1% before resynchronization to 29.5% at last follow-up (p = 0.008) without a significant change in the end-diastolic area (+4.0%, p = NS). CONCLUSIONS: The CRT yielded improvement in systemic RV function in patients with spontaneous or LV pacing-induced electromechanical dyssynchrony and seems to be a promising adjunct to the treatment and prevention of systemic RV failure.