Acute right ventricular resynchronization improves haemodynamics in children after surgical repair of tetralogy of Fallot.

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.

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.

Permanent epicardial pacing in children: long-term results and factors modifying outcome.

AIMS: To evaluate the results of permanent epicardial pacing in children. METHODS AND RESULTS: All consecutive patients from one country (n = 119, period 1977-2009) undergoing permanent epicardial pacemaker implantation at <18 years of age (median 1.8 years, inter-quartile range 0.3-6.4 years) were studied retrospectively. Median patient follow up was 6.4 years (inter-quartile range 2.9-11.1 years); 207 generators, 89 atrial and 153 ventricular pacing leads were implanted. The probability of absence of any pacing system dysfunction was 70.1 and 47.2% at 5 and 10 years after implantation, respectively. Overall probability of continued epicardial pacing was 92.8 and 76.1% at 5 and 10 years, respectively, and increased in the recent implantation era (post-2000, P = 0.04). The use of steroid-eluting leads decreased the risk of exit block with a hazard ratio (HR) of 0.20 [95% confidence interval (CI) 0.09-0.44, P < 0.001)]. The use of bipolar Medtronic 4968 leads reduced the risk of surgical reintervention because of fracture, insulation break, outgrowth or exit block in comparison to the unipolar 4965 lead design (HR 0.12, 95% CI 0.04-0.40, P < 0.001). The AutoCapture™ feature (HR 0.08, 95% CI 0.02-0.36, P < 0.001) and steroid-eluting leads (HR 0.30, 95% CI 0.11-0.84, P = 0.021) decreased the risk of battery depletion. CONCLUSION: The probability of continued epicardial pacing in children was 76% at 10 years after implantation, increased for implantation in recent years, and allowed transvenous pacing to be deferred to a significantly greater age. The use of bipolar steroid-eluting leads and of a beat-to-beat capture tracking feature significantly increased pacing system longevity and decreased the need for surgical reinterventions.

Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study.

BACKGROUND: Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. METHODS AND RESULTS: From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. CONCLUSIONS: The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

How many types of circulation can a boy have during his life? A case of aortic stenosis with a borderline left ventricle.

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.

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.

Long-term (up to 20 years) results of percutaneous balloon angioplasty of recurrent aortic coarctation without use of stents.

AIMS: To assess the efficacy, safety, and long-term results of the balloon angioplasty of recoarctation. METHODS AND RESULTS: The angioplasty was performed in 99 consecutive patients aged 36 days to 32.6 years (median 268 days). Recoarctation to descending aorta diameter ratio increased from 0.44 (0.35/0.50) to 0.66 (0.57/0.77), P < 0.001. Systolic gradient was reduced from 34.0 (26.0/44.75) to 15.0 (8.25/27.0) mmHg, P < 0.001. In seven patients (7.1%) the procedure was ineffective. One patient (1%) with heart failure died within 24 h after a successful angioplasty and in another (1%) an intimal abruption necessitated surgical revision. The follow-up ranged up to 20.7 years (median 8.1 years). Actuarial probability of survival 20.7 years after the procedure was 0.91, and of reintervention-free survival was 0.44. Older age at the angioplasty was associated with a higher incidence of reinterventions (hazard ratio 1.057; 95% confidence interval 1.012-1.103; P = 0.010). The type of surgery and the recoarctation anatomy did not influence the outcome. In 69 patients aneurysm formation was studied by high-sensitive methods with only one positive finding per 462 patient-years. CONCLUSION: Angioplasty is safe and effective regardless of the type of surgery used and the recoarctation anatomy. Older age at the angioplasty is associated with a higher incidence of reinterventions.

Haemodynamic changes due to delayed sternal closure in newborns after surgery for congenital cardiac malformations.

BACKGROUND: Delayed sternal closure is used to prevent low cardiac output syndrome in selected newborns shortly after cardiac surgery for congenital cardiac defects. Sternal closure itself often causes haemodynamic and ventilatory instability that cannot be entirely assessed by standard monitoring means. Therefore, we used transpulmonary thermodilution technique for an exact evaluation of the haemodynamic changes. PATIENTS AND METHODS: Between April, 2006, and December, 2008, 23 neonates aged from 1 to 30 days, with a median of 7 days, and weighing from 1.9 to 4.2 kilograms, with a median of 3.25 kilograms, were studied after biventricular corrections. Residual intracardiac shunts were excluded by echocardiography. Haemodynamic and ventilatory parameters, along with those obtained by the transpulmonary thermodilution technique, were recorded before and immediately after the sternal closure, and then at 0.5, 1, 2, 6, 12, 24, and 48 hours. RESULTS: Chest closure caused significant decrease in systolic arterial pressure from 80.04 +/- 11.48 to 69.48 +/- 9.63 mmHg (p < 0.001), cardiac index from [median (25th/75th centile)] 2.640 (2.355/2.950) to 2.070 (1.860/2.420) l/min/m2 (p < 0.001), stroke volume index from 18.50 (16.00/20.00) to 14.00 (11.00/17.00) ml/m2 (p < 0.001), and dynamic lung compliance from 2.45 (2.31/3.00) to 2.30 (2.14/2.77) ml/cmH2O (p = 0.007). Stroke volume variation increased from 14.00 (9.25/16.75) to 18.00 (15.00/21.00) % (p < 0.001). The oxygenation index transitorily increased from 2.50 (2.14/3.15) to 3.36 (2.63/4.29) (p < 0.001). Serum lactate decreased from 1.40 (1.12/2.27) to 1.0 (0.8/1.3)mmol/l, p < 0.001 in coincidence with a haemodynamic stabilisation at a later time after chest closure. Cardiopulmonary instability caused by the sternal closure necessitated therapeutic intervention in 18 of 23 patients (78.3%). CONCLUSION: Delayed sternal closure causes a significant transitory decrease in stroke volume, cardiac output and arterial blood pressure. Also lung compliance and blood oxygenation are temporarily significantly compromised.

Surgical options after Fontan failure.

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.

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.

Experience with the surgical treatment of atrioventricular septal defect with left ventricular outflow tract obstruction.

OBJECTIVES: We sought to determine the prevalence, morphology, surgical methods and results of surgery for left ventricular outflow tract obstruction (LVOTO) associated with atrioventricular septal defect (AVSD). METHODS: Correction of AVSD was performed in 615 patients. Twenty-three (3.7%) patients with LVOTO were identified. Sixteen (70%) of them had partial and 7 (30%) had complete AVSD. Surgery for AVSD was performed at a median of 0.6 years (mean 2.1 ± 3.0 years), and surgery for LVOTO at a median of 3.4 years (mean 4.7 ± 3.5 years). The point and period prevalence of LVOTO in AVSD were determined. Detailed morphological study, individualized repair of AVSD with LVOTO and long-term follow-up were performed. Early and long-term results were analysed. RESULTS: The point prevalence of LVOTO at the time of AVSD repair was 1.3%. The period prevalence of LVOTO was 3.7% in course of 8.3 ± 6.0 (0-18.4) years and 191.4 patient-years following AVSD repair. Causes of LVOTO were fibromuscular membrane (n = 17), septal hypertrophy (n = 17), abnormal atrioventricular (AV) valve (n = 9), muscular bands (n = 3), fibrous strands (n = 4) and stenotic aortic valve (n = 2). Usually, a combination of several obstructive lesions was present. LVOTO was present at the time of AVSD repair in 8 patients (35%) and developed after repair in 15 (65%) patients. Membrane excision (n = 17), myectomy (n = 17), excision of abnormal AV valvar tissue (n = 8), excision of muscular bands and fibrous strands (n = 6), AV valve replacement (n = 2) and aortic valvotomy (n = 2) were required. There was 1 (4%) early and 1 (4%) late death. Six (29%) survivors required reoperation for recurrence of LVOTO at an average interval of 6.3 ± 3.2 years after surgery. The actuarial survival at 1 and 10 years was 96 and 88%, respectively. The actuarial freedom from reoperation for LVOTO was 80, 40 and 20% at 6, 10 and 15 years after surgery, respectively. Eighteen (78%) patients remain in good condition at mean 6.0 ± 5.5 years after surgery. CONCLUSIONS: The point prevalence of LVOTO at the time of AVSD repair was 1.3%, and period prevalence 3.7%. Fibromuscular membrane, septal hypertrophy and valvar attachments represent the most common causes of LVOTO. Usually, more structures are involved. The repair must be individualized. The presence of LVOTO increases the need for reoperation.

Pediatric coronary artery revascularization: a European multicenter study.

BACKGROUND: We sought to evaluate the hospital and midterm results of different surgical revascularization techniques in pediatric patients within the European Congenital Heart Surgeons Association. METHODS: From 1973 to 2011, 80 patients from 13 European Congenital Heart Surgeons Association centers underwent 65 pediatric coronary artery bypass grafting (PCABG) and 27 other coronary artery procedures (OCAP; 12 patients had combined PCABG and other coronary artery procedures). Excluded were patients with Kawasaki disease. Median age at the time of coronary procedure was 2.3 years (range, 2 days to 16.9 years); 33 patients (41.2%) were younger then 12 months. An emergency procedure was necessary in 34 patients (42.5%). RESULTS: Twelve patients (15%) died in the hospital; age at surgery (p=0.02) and the need for an emergent procedure (p=0.0004) were related to hospital mortality. Median follow-up time was 7.6 years (range, 0.9 to 23 years). There were 3 late cardiac deaths, all after a median time of 4 years (range, 9 months to 8.8 years) after PCABG. Fourteen patients (20.5%) presented with symptoms, including congestive heart failure (n=10) and angina (n=4), that were significantly associated with a low ejection fraction (p<0.001) and the presence of moderate or severe mitral valve regurgitation (p=0.0003). Six patients underwent a reintervention for impaired myocardial perfusion; all of them had a stenotic or atretic PCABG (p=0.001), and the majority were symptomatic (5 of 6 patients; 83.3%; p=0.001). CONCLUSIONS: Both PCABG and other coronary artery procedures are suitable surgical options in pediatric patients with impaired myocardial perfusion, which increases operative and midterm survival. Such population of patients needs to be followed for life to prevent and treat any possible cause of further myocardial ischemia.

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.

Slide plasty of trachea and right main bronchus in a newborn.

Congenital stenosis of airways is a rare and possibly life-threatening congenital defect with difficult treatment and uncertain prognosis. In our report, we describe a rare case of a 1-month-old newborn with concomitant stenosis of trachea and main bronchus and its successful treatment using slide plasty.

Corrective surgery of congenital heart defects in neonates: the Prague experience.

AIM: To assess the outcome and mortality trend in newborns undergoing corrective surgery for congenital heart defect. METHOD: We reviewed the hospital records of 1,033 neonates under 30 days of life, who had congenital heart defects operated on at the Kardiocentrum, Motol University Hospital in Prague, Czech Republic, during 1977-2001. Early and late mortality and reoperation rates were analyzed. RESULTS: A total of 1,156 operations were performed in 1,033 neonates. Obstructive lesions were surgically treated in 56%, left-to-right shunts in 21%, and complex conotruncal lesions in 23% of the cases. Total correction has been achieved in 62% of the neonates. Most operations (75%) were performed in the first two weeks of neonate s life. Early 30-day hospital mortality was 13%. Late mortality, after the discharge from the hospital, was 10%. In the last three years, the hospital mortality rate decreased to about 2%. Out of 590 reinterventions in 379 neonates, with the mortality of 6%, 229 were secondary staged corrections and 190 further palliative procedures aimed mostly toward Fontan or Rastelli type of circulation. Residual or recurrent defects were solved in 62 neonates. There were 30 valve replacements, with 18 mechanical valves and 12 pulmonary valve autotransplantations (the Ross procedure). The homograft valved conduit was used in 38 children. CONCLUSION: Most newborns with critical congenital heart defects can undergo corrective operation under acceptable risk. Due to complex improvements in perioperative, anaesthetic, surgical, and postoperative care, contemporary hospital mortality can be reduced to 1-3%. Palliative procedures still play an important role in the staged treatment of severe complex heart defects in neonates.