Tomas Tlaskal

Resynchronization pacing is a useful adjunct to the management of acute heart failure after surgery for congenital heart defects

The acute hemodynamic effect of atrioventricular (AV) and inter/intraventricular (IV) resynchronization accomplished by temporary pacing using multiple epicardial pacing wires was evaluated in 20 children (aged 3.4 months to 14.0 years) after surgery for congenital heart defects fulfilling the following criteria: (1) presence of AV and/or IV conduction delay, and (2) need for inotropic support. AV resynchronization (n = 13) was achieved by AV delay optimization during atrial synchronous right ventricular outflow tract pacing. IV resynchronization (n = 14) was accomplished by atrial synchronous pacing from the right ventricular lateral wall in 7 patients with right bundle branch block and normal AV conduction and by atrial synchronous multisite ventricular pacing in another 7 patients with previously performed AV resynchronization. Compared with baseline values, AV resynchronization resulted in an increase in arterial systolic, mean, and pulse pressures by 7.2 +/- 8.3% (p <0.01), 8.6 +/- 8.1% (p <0.005), and 6.9 +/- 13.5% (p = NS), respectively. IV resynchronization used either alone or added to previously performed AV resynchronization led to a pressure increase of 7.0 +/- 4.7%, 5.9 +/- 4.7%, and 9.4 +/- 7.8%, respectively (p <0.001 for all). The combined effect of AV and IV resynchronization resulted in a systolic, mean, and pulse pressure increase of 10.2 +/- 5.0% (range 4.0 to 19.1), 8.6 +/- 5.4% (range 0.8 to 14.8), and 15.2 +/- 8.5% (range 6.1 to 33.3), respectively (p <0.001 for all). The increase in systolic arterial pressure after IV resynchronization was positively correlated with the initial QRS duration (r = 0.62, p <0.05) and extent of QRS shortening (r = 0.66, p <0.05). In conclusion, resynchronization pacing led to a significant increase in arterial blood pressure and was a useful adjunct to the treatment of acute postoperative heart failure in patients with AV and/or IV conduction delay.

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. # Clinical Perspective {#article-title-44}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.

Hemodynamically optimized temporary cardiac pacing after surgery for congenital heart defects.

Disturbance of normal AV synchrony and dyssynchronous ventricular contraction may be deleterious in patients with otherwise compromised hemodynamics. This study evaluated the effect of hemodynamically optimized temporary dual chamber pacing in patients after surgery for congenital heart disease. Pacing was performed in 23 children aged 5 days to 7.7 years (median 7.3 months) with various postoperative dysrhythmias, low cardiac output, and/or high inotropic support and optimized to achieve the highest systolic and mean arterial pressures. The following four pacing modes were used: (1) AV synchronous or AV sequential pacing with individually optimized AV delay in 11 patients with first‐to third‐degree AV block; (2) AV sequential pacing using transesophageal atrial pacing in combination with a temporary DDD pacemaker for atrial tracking and ventricular pacing in three patients with third‐degree A V block and junctional ectopic tachycardia, respectively, who had poor signal and exit block on atrial epicardial pacing wires; (3) R wave synchronized atrial pacing in eight patients with junctional ectopic tachycardia and impaired antegrade AV conduction precluding the use of atrial overdrive pacing; (4) Atrio‐biventricular sequential pacing in two patients. Pressures measured during optimized pacing were compared to baseline values at underlying rhythm (13 patients with first‐degree AV block or junctional ectopic tachycardia) or during pacing modes commonly used in the given clinical situation: AAI pacing (1 patient with slow junctional rhythm and first‐degree AV block during atrial pacing), WI pacing (2 patients with third‐degree A V block and exit block and poor sensing on epicardial atrial pacing wires) and dual‐chamber pacing with AV delays set to 100 ms (atrial tracking) or 150 rns (AV sequential pacing) in 7 patients with second‐ to third‐degree A V block and functional atrial pacing wires. Optimized pacing led to a significant increase in arterial systolic (mean) pressure from 71.5 ± 12.5 (52.3 ± 9.0) to 80.5 ± 12.2 (59.7 ± 9.1) mmHg (P < 0.001 for both) and a decrease in central venous (left atrial) pressure from 12.3 ± 3.4 (10.5 ± 3.2) to 11.0 ± 3.0 (9.2 ± 2.7) mmHg (P < 0.001 and < 0.005, respectively). In conclusion, several techniques of individually optimized temporary dual chamber pacing leading to optimal AV synchrony and/or synchronous ventricular contraction were successfully used to improve hemodynamics in patients with heart failure and selected dysrhythmias after congenital heart surgery.

Results of primary and two-stage repair of interrupted aortic arch

OBJECTIVE Early results of primary and two-stage repair of interrupted aortic arch have improved. Experience with different surgical approaches should be analysed and compared. METHODS Forty neonates and infants with interrupted aortic arch underwent primary repair (19 patients) or palliative operation (21 patients). Twenty (50%) patients were followed-up for 5.1+/-4.3 years. All patients were regularly examined with the aim of determining clinical development, presence of residual lesions or complications and need for re-intervention. Aortic arch and the left ventricular outflow tract growth were assessed by echocardiographic examination. Data from hospital and outpatient department records were analysed. RESULTS The early mortality was 61.9% after palliative operations and 36.8% after the primary repair. Presence of complications (P < 0.001), earlier year of surgery (P < 0.01), bad clinical condition and acidosis (P < 0.05) represented statistically significant risk factors for death in the whole series. In seven (87.5%) out of eight early survivors, after the initial palliative operation, closure of ventricular septal defect and debanding were done, and in three (37.5%) patients, re-operation for aortic arch obstruction was also required. Out of 12 patients, after the primary repair, one required early re-operation for persistent left ventricular outflow tract obstruction and two needed late re-intervention for left bronchus obstruction. In three (25%) patients, after the primary repair, left ventricular outflow tract obstruction with a maximal systolic pressure gradient higher than 30 mmHg developed. At present, all 20 early survivors are alive. Five patients, after palliative operation, are in NYHA class 1, but in three patients, who are in class III or IV, the outcome is influenced by severe complications. All patients after the primary repair are in class I or II. CONCLUSIONS Our experience confirmed better results after the primary repair of interrupted aortic arch, which was associated with lower mortality, prevalence of severe complications and need for re-intervention. Higher prevalence of subaortic stenosis after primary repair could be explained by patient selection early in our experience. We recommend the primary repair of interrupted aortic arch and associated heart lesions in neonates, however, in unfavourable conditions an individualised surgical approach with initial palliative surgery should be considered.

Coagulation profile and liver function in 102 patients after total cavopulmonary connection at mid term follow up

Objective: To examine coagulation factors and liver function test abnormalities in patients after total cavopulmonary connection (TCPC). Design: Cross sectional study comprising clinical and echocardiographic evaluation, and biochemical and coagulation profile screening. Setting: Tertiary referral centre. Methods: 102 patients aged 4–24 years (median 10 years) at one to eight years (median five years) after TCPC were examined. All patients were maintained on a low dose of aspirin. 96% of patients were in a good clinical condition (New York Heart Association class I or II). No intracardiac thrombi were detected on echocardiography and ventricular function was good in 91% of patients. Results: Total bilirubin was increased in 27% and γ glutamyltransferase in 54% of patients. Serum total protein, albumin, and prealbumin were normal in almost in all patients. Compared with the control group, patients after TCPC had significantly lower fibrinogen, factor V, factor VII, and protein C concentrations, prolonged international normalised ratio, and increased antithrombin III concentration. Factor V concentration was abnormally decreased in 35%, factor VII in 16%, and protein C in 28% of patients. Antithrombin III was increased in 23% of patients. Factor VII, factor V, protein C, and antithrombin III correlated significantly with serum prealbumin. There was also a significant correlation between procoagulant factor VII and both anticoagulant protein C and antithrombin III. Conclusions: Almost half of patients after TCPC had laboratory signs of mild cholestasis. Decreased liver synthesis of procoagulant and anticoagulant factors was observed but overall coagulation homeostasis appeared to be in balance in this selected group of patients with a good clinical outcome.

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.

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.

Nitrogen balance, 3-methylhistidine excretion, and plasma amino acid profile in infants after cardiac operations for congenital heart defects: The effect of early nutritional support☆☆☆★★★

OBJECTIVE The objective of this study was to evaluate the effect of nutritional support on proteolysis and plasma amino acid profile in infants early after cardiac operations for congenital heart defects. METHODS Thirty-seven patients, 2 to 12 months old, were randomized on postoperative day 1 for 24-hour isocaloric metabolic study. Group STANDARD (18 patients) received glucose as the maintenance fluid, and group PN (19 patients) received glucose and crystalloid amino acid solution at a dosage of 0.8 +/- 0.1 gm/kg per day. The nonprotein caloric intake in the two groups was 25 +/- 15 and 33 +/- 9 kcal/kg, respectively (p = not significant). RESULTS The nitrogen balance was markedly less negative in group PN than in group STANDARD (-114 +/- 81 vs -244 +/- 86 mg/kg, respectively, p = 0.001). There was a highly significant inverse correlation between the nitrogen balance and urinary 3-methylhistidine excretion in both groups, but the muscle proteolysis was blunted more effectively in patients receiving amino acids. Concentrations of the plasmatic branched-chain amino acids, alanine, glycine, and proline, decreased significantly in group STANDARD but not in group PN on postoperative day 2. Glutamine and threonine levels declined significantly on postoperative day 2 in both groups. Low levels of arginine were observed in our patients before operation and in the early postoperative period. The amino acid concentrations normalized on postoperative day 7 in all patients. CONCLUSION Significant proteolysis and hypoaminoacidemia were observed in infants early after cardiac operations. This hypercatabolic response was blunted by parenteral nutritional support.

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.

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.