Repair of Congenital Right Atrial Aneurysm Associated With Wolff-Parkinson-White Syndrome in a 5-Year-Old Girl.

This report describes our experience with a 5-year-old girl who had an extremely rare presentation of a right atrial aneurysm and associated Wolff-Parkinson-White syndrome. Before being referred to our department, she underwent an ineffective radiofrequency ablation for repeated episodes of paroxysmal supraventricular tachycardia that were causing dizziness, palpitations, and chest discomfort. We resected the aneurysm with good results; she was doing well and was in normal sinus rhythm at the time of her 1-year follow-up visit.

Presence of reduced regional left ventricular function even in the absence of left ventricular wall scar tissue in the long term after repair of an anomalous left coronary artery from the pulmonary artery.

BACKGROUND: We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery. METHODS: A total of 20 patients aged 12.8±7.4 years were assessed 10 (0.5-17) years after the repair of an anomalous origin of the left coronary artery from the pulmonary artery; of them, 10 (50%) patients showed left ventricular wall scar tissue on current cardiac MRI. Left ventricular regional function was assessed by two-dimensional speckle-tracking echocardiography in 10 patients with scar tissue and 10 patients without scar tissue and in 10 age-matched controls. RESULTS: In patients with scar tissue, MRI-derived left ventricular ejection fraction was significantly reduced compared with that in patients without scar tissue (51 versus 61%, p<0.05), and echocardiography-derived longitudinal strain was significantly reduced in five of six left ventricular areas compared with that in healthy controls (average relative reduction, 46%; p<0.05). In patients without scar tissue, longitudinal strain was significantly reduced in two of six left ventricular areas (average relative reduction, 23%; p<0.05) and circumferential strain was reduced in one of six left ventricular areas (relative reduction, 56%; p<0.05) compared with that in healthy controls. CONCLUSIONS: Regional left ventricular function is reduced even in patients without left ventricular wall scar tissue late after successful repair of an anomalous origin of the left coronary artery from the pulmonary artery. This highlights the need for meticulous lifelong follow-up in all patients with a repaired anomalous origin of the left coronary artery from the pulmonary artery.

Myocardial perfusion, scarring, and function in anomalous left coronary artery from the pulmonary artery syndrome: a long-term analysis using magnetic resonance imaging.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. We aimed to examine the role of cardiac magnetic resonance imaging (MRI) in the long-term surveillance of repaired ALCAPA with regard to myocardial scarring, wall motion abnormalities, perfusion deficits, and myocardial function. METHODS: Twenty-one patients after direct reimplantation of ALCAPA (median age at operation, 2.8 years) were examined after a median 10.6 years by MRI at rest and under dobutamine stress conditions, echocardiography, and ergometry. Results were compared with preoperative, immediately postoperative (5 days), and intermediate-term (5.8 years) echocardiography. RESULTS: No early or late deaths occurred. Improvements in indexed left ventricular end-diastolic dimension, ejection fraction, and mitral valve function were observed in all patients. However, MRI at rest showed wall motion abnormalities in 67% and perfusion deficits in 28%. Myocardial scars were seen in 67%. Dobutamine stress MRI detected wall motion abnormalities in 19% and perfusion deficits in 14%, which were not seen on MRI at rest. Exercise testing did not reflect cardiac dysfunction. CONCLUSIONS: Although long-term follow-up showed global left ventricular function had improved after ALCAPA repair, MRI showed left ventricular wall motion abnormalities, perfusion deficits, and myocardial scarring were seen in many patients. Dobutamine stress MRI identified deficits that were not evident on MRI at rest, and can therefore be considered a valuable surveillance tool. These results suggest the need for lifelong surveillance of repaired ALCAPA.

Rare and private variations in neural crest, apoptosis and sarcomere genes define the polygenic background of isolated Tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Its genetic basis is demonstrated by an increased recurrence risk in siblings and familial cases. However, the majority of TOF are sporadic, isolated cases of undefined origin and it had been postulated that rare and private autosomal variations in concert define its genetic basis. To elucidate this hypothesis, we performed a multilevel study using targeted re-sequencing and whole-transcriptome profiling. We developed a novel concept based on a gene's mutation frequency to unravel the polygenic origin of TOF. We show that isolated TOF is caused by a combination of deleterious private and rare mutations in genes essential for apoptosis and cell growth, the assembly of the sarcomere as well as for the neural crest and secondary heart field, the cellular basis of the right ventricle and its outflow tract. Affected genes coincide in an interaction network with significant disturbances in expression shared by cases with a mutually affected TOF gene. The majority of genes show continuous expression during adulthood, which opens a new route to understand the diversity in the long-term clinical outcome of TOF cases. Our findings demonstrate that TOF has a polygenic origin and that understanding the genetic basis can lead to novel diagnostic and therapeutic routes. Moreover, the novel concept of the gene mutation frequency is a versatile measure and can be applied to other open genetic disorders.

Outcome of surgical correction of congenital supravalvular aortic stenosis with two- and three-sinus reconstruction techniques.

BACKGROUND: Several surgical techniques for the treatment of congenital supravalvular aortic stenosis have been developed, yet there is no consensus about the optimal approach. We reviewed our institutional experience with 2- and 3-sinus reconstruction techniques. METHODS: Thirty-eight patients operated on for supravalvular aortic stenosis between 1987 and 2012 in our institution were analyzed retrospectively. Eight patients (21%) were infants and in 5 (13.2%) diffuse stenosis was present. Mean peak pressure gradient was 86.1±28.7 mm Hg preoperatively. Surgical procedures included single-patch enlargement (McGoon, n=3), inverted bifurcated-patch aortoplasty (Doty, n=22), 3-sinus patch augmentation (Brom, n=8), and autologous slide aortoplasty (n=5). Major concomitant procedures were performed in 10 patients (26.3%). RESULTS: Early mortality was 2.6%. Follow-up continued for a median of 7.5 years (range 3 weeks to 22 years). Overall survival estimates were 94% and 90% and overall freedom from reoperation was 83% at 5 and 20 years, respectively. No differences were found between surgical techniques in respect to survival, clinical course, hemodynamic outcome, or freedom from reoperation rates. A significantly worse outcome in regard to survival and reoperation rates was observed in infants. CONCLUSIONS: Our study demonstrates equally good results for the repair of supravalvular aortic stenosis with both 2- and 3-sinus reconstruction. No evidence of a superior outcome for 3-sinus reconstruction techniques was found. Operation in infancy is an important factor associated with unfavorable outcome.

Altered right ventricular function in the long-term follow-up evaluation of patients after delayed aortic reimplantation of the anomalous left coronary artery from the pulmonary artery.

This study aimed to evaluate regional and global ventricular functions in the long term after aortic reimplantation of the anomalous left coronary artery from the pulmonary artery (ALCAPA) and to assess whether the time of surgical repair influences ventricular performance.The study examined 20 patients with a median age of 15 years (range 3-37 years) who had a corrected ALCAPA and 20 age-matched control subjects using echocardiography and tissue Doppler imaging (TDI). The median follow-up period after corrective surgery was 6 years (range 2.6-15 years). Seven patients underwent surgery before the age of 3 years (early-surgery group), whereas 13 patients had surgery after that age (late-surgery group). The TDI-derived myocardial strain of the interventricular septum (IVS), lateral wall of the left ventricle (LV), and lateral wall of the right ventricle (RV) in the basal and mid regions were examined, and a mean was calculated. The pulsed Doppler-derived Tei index was used to assess global left ventricular function. No significant differences were found between the early-surgery group and the control group regarding the regional myocardial strain or the Tei index. Compared with the early-surgery group, the late-surgery group had a significantly higher Tei index (mean 0.37; range 0.31-0.42 vs. mean 0.52; range 0.39-0.69; p < 0.005), a lower strain percentage of the lateral wall of the LV (mean 29; range 17-30 vs. mean 9; range 7-23), IVS (mean 23; range 21-31 vs. mean 19; range 13-25), and lateral wall of the RV (mean 23; range 21-31 vs. mean 19; range 13-25). The age at operation correlated significantly with the Tei index (r = 0.84, p < 0.001) and inversely with the mean strain of the lateral wall of the LV (r = -0.53, p = 0.028), IVS (r = -0.68, p = 0.003), and lateral wall of the RV (r = -0.68, p = 0.003). At the midterm follow-up evaluation after corrective surgery of ALCAPA, not only the left but also the right ventricular function seemed to be affected in patients with delayed diagnosis and late surgical repair but preserved among the younger patients with early diagnosis and corrective surgery.

Surgery impacts right atrial function in tetralogy of Fallot.

OBJECTIVE: To analyze the impact of surgery and pericardial integrity on right atrial function and total heart volume variation in the setting of pulmonary valve insufficiency. METHODS: Right atrial function and total heart volume variation were analyzed in 2 subgroups of patients with pulmonary valve insufficiency compared with healthy controls: group 1 with surgically repaired tetralogy of Fallot (n = 20 patients) and group 2 after balloon angioplasty of pulmonary valve stenosis in patients with isolated valve disease without surgery (n = 7 patients). Volumetric analysis of magnetic resonance imaging data revealed parameters of atrial function (reservoir, conduit, and pump functions and cyclic volume change) and of total heart volume (end-diastolic and end-systolic total heart volume and the variation). Statistical analysis included uncorrected and corrected pairwise comparisons and the calculation of groupwise Pearson correlation coefficients. RESULTS: In group 1 with a pulmonary regurgitation fraction of 31.0% ± 14.9%, right atrial function was clearly impaired, with reduced reservoir and elevated conduit function, and total heart volume variation was elevated to 13.9% ± 3.4%. In group 2 with a pulmonary regurgitation fraction of 22.8% ± 6.9%, the values were close to normal, with unaffected atrial function and a total heart volume variation of 9.9% ± 3.3%. CONCLUSIONS: The hydrodynamic effect of pulmonary valve insufficiency alone is likely not the only reason for impaired right atrial function and elevated total heart volume variation in patients with tetralogy of Fallot; it is rather the scar in the right atrium, the injured pericardium, and the disease itself that are responsible for the energetically unfavorable alterations.

Analysis of the risk factors for early failure after extracardiac Fontan operation.

BACKGROUND: We analyzed risks for severe morbidity in the early period after extracardiac Fontan operation. METHODS: Between November 1995 and May 2011, 140 patients (median age, 3.8 years) underwent extracardiac Fontan operation. We assumed as preoperative risk factors systemic right ventricle (n=51), heterotaxia (n=25), arterial oxygen saturation less than 75% (n=22), and adult age (>16 years, n=20) at time of surgery. Prolonged cardiopulmonary bypass time of longer than 120 minutes (n=30) and use of cardioplegia (n=26) were analyzed as intraoperative risks. RESULTS: Heterotaxia was revealed as a risk factor for postoperative prolonged inotropic support, acute renal failure, prolonged mechanical ventilation, prolonged pleural effusions, and tachyarrhythmias. With the exception of pleural effusions, the same held true for right ventricle morphology. Low preoperative arterial oxygen saturation was found to be associated with an increased risk of prolonged inotropic support, acute renal failure, and prolonged mechanical ventilation. Adult age was identified as a risk factor for acute renal failure. Of the intraoperative factors, prolonged cardiopulmonary bypass time longer than 120 minutes was a risk factor for acute renal failure and prolonged pleural effusions, whereas use of cardioplegia was associated with an increased risk of prolonged inotropic support, prolonged mechanical ventilation, acute renal failure, and tachyarrhythmias. Multivariate analysis demonstrated heterotaxia, right ventricular morphology, and low preoperative arterial oxygen saturation to be independent risk factors for postoperative prolonged inotropic support and prolonged mechanical ventilation. CONCLUSIONS: Patients with heterotaxia, systemic right ventricle, and low preoperative arterial oxygen saturation are still at high risk for early Fontan failure after extracardiac Fontan operation and require special management for optimal outcome.

Improved early postoperative outcome for extracardiac Fontan operation without cardiopulmonary bypass: a single-centre experience.

OBJECTIVES: The use of modified extracardiac Fontan operation (ECFO) for total cavo-pulmonary connection allows cardiopulmonary bypass (CPB) to be avoided and seems to improve early postoperative results. We evaluated our experience with the off-pump technique for ECFO. METHODS: Since 2009, the last 17 consecutive patients of 137 (median age 3.2 years, median weight 14.5 kg) in whom no intracardiac surgery was necessary underwent ECFO without CPB. The non-fenestrated graft was connected end-to-side to the pulmonary artery without bypass; subsequently temporary passive inferior vena cava (IVC)-to-atrial bypass was used for the anastomosis between IVC and graft. The perioperative and postoperative course was compared between consecutive paediatric patients operated on using the CPB vs off-pump technique. RESULTS: There was no mortality in the off-pump group, with a total early mortality of 3.0%. Overall operation time for the Fontan operation using the off-pump technique was significantly reduced (160 vs 200 min, P < 0.001). The median Fontan pressure 24 and 48 h postoperatively was significantly lower in the off-pump group (P = 0.002/0.042). Duration of mechanical ventilation (9 vs 14 h, P = 0.016), pleural effusions (4 vs 8 days, P < 0.001) as well as the median intensive care unit (2 vs 4 days, P = 0.013) and hospital stay (median 10 vs 15 days, P < 0.001) was significantly shorter in patients who underwent the off-pump Fontan operation. The necessity of blood transfusions was significantly reduced with the off-pump in comparison with the on-pump technique (14 of 17 vs 34 of 84 patients, P = 0.003). CONCLUSIONS: The ECFO without CPB is an established low-risk surgical procedure that improves the early postoperative course and significantly reduces the use of blood products and the duration of pleural effusions in selected patients.

Risk factors for distal Contegra stenosis: results of a prospective European multicentre study.

Objectives The EUCon study was designed to identify risk factors for distal anastomotic stenosis after bovine jugular vein (Contegra) implantation in children. Methods Between March 2006 and August 2008, 104 devices were implanted in nine European centers. Preoperative, intraoperative, and follow-up data (at discharge, 6, 12, 24 months) including standardized echocardiography were prospectively registered, source data verified and collected in a central database. Main endpoint was distal stenosis (either postvalvular gradient of ≥50 mm Hg or need for intervention for distal stenosis). Eight potential risk factors (age <2 years, diagnosis, running suture, use of glue, flapless anastomosis, oversizing less than + 2 z, anticoagulation, implantation site) were investigated. Cox regression, decision tree analyses, and "Clustering by Response" were applied. Results Patient age ranged from 0 to18 years, mean 6.0 ± 6.1, median 3.2 years. Implantation reasons: 88% congenital malformations, 12% Ross operations. Follow-up was 88.3% complete. Durability (freedom from death, reoperation, degeneration, endocarditis, and explantation) compared well to corresponding homograft literature. Sixteen patients reached study endpoints. Age <2 years was the only invariably significant risk factor (p = 0.044); "Clustering By Response" found young anticoagulated patients with oversized conduits to be at a higher risk than the others (p = 0.018, OR = 3.2). Conclusion Patient age is the main risk factor for development of distal anastomosis stenosis after Contegra implantation. The influence of the other investigated factors is too small to be proven in 104 patients after 2 years, or other risk factors must be taken into consideration to explain outcome differences among recipients under 2 years.

Suturing of penetrating wounds to the heart in the nineteenth century: the beginnings of heart surgery.

The beginnings of cardiac surgery go back to the 19th century. This article describes the history of the first attempts to operate on the heart. In 1882, Dr Block from Danzig, and in 1895, Simplicio Del Vecchio, published reports of animal experiments showing that the suturing of heart wounds is possible. After unsuccessful attempts by Axel Cappelen in Norway and Guido Farina in Italy, it was Ludwig Rehn of Germany who first sutured a laceration of the right ventricle of a human heart. Shortly afterward, Antonio Parrozzani successfully sutured a stab wound of the left ventricle. Following cardiac surgery back to its very beginnings, it is striking that the first attempts in the 19th century to repair the injured heart were regarded with great skepticism, and that heart suturing only slowly became an established method of treatment. Once the concept of cardiac surgery had become accepted, however, many kinds of operations were developed, paving the way for an explosion in the number of cardiac operations, as we well know, in the century that followed.

Adaptive growth and remodeling of transplanted hearts in children.

OBJECTIVE: We aimed to evaluate the adaptive growth and remodeling behavior of the transplanted heart in pediatric heart-transplant recipients by comparing donor body surface area (BSA) and cardiac dimensions during transplantation with the corresponding parameters of the recipient over a period of time. METHODS: A retrospective review of medical and echocardiographic records of 167 children (8.65 ± 5.98, median 9; range 0-17 years) who underwent orthotopic heart transplantation between 1987 and March 2010 was done. RESULTS: In the first 30 days post-transplantation, right- and left-ventricular end-diastolic diameters, volumes, and myocardial mass were found to be significantly increased (z score 3.96, p < 0.000) in relation to the recipients' BSA. Within the first year of post-transplantation, there was a significant reduction in the right-ventricular diameter (z score, -1.0 to +1.6, p = 0.000), left-ventricular diameter (z score -1.0 to +1.9, p = 0.000), right-ventricular end-diastolic volume (z score -1.3 to +1.9, p = 0.000) and left-ventricular end-diastolic volume (z score -1.3 to +1.8, p = 0.000), right-ventricular mass (z score, -1.4 to +1.7, p = 0.000) and left-ventricular mass (z score, -1.4 to +1.8, p = 0.000). During subsequent follow-up periods of 2-5 and 6-10 years, the aforementioned cardiac dimensions and volumes increased appropriately in accordance to the BSA (p = 0.000). In all the cardiac dimensions and volumes measured, donor-recipient mismatch did not influence the continuous growth of the measured parameters, which was in accordance to the recipients' BSA over time. Kaplan-Meier survival analysis showed a survival rate of 61.7% at 10 years. There is no statistically significant difference in survival rate among patients with varying donor-recipient weight ratios and donor-recipient BSA ratios (p = 0.53). CONCLUSIONS: This study demonstrates that the transplanted heart undergoes remodeling processes and grows adaptively, in accordance to the BSA, over a period of time.

Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children.

OBJECTIVE: Although mortality after direct aortic reimplantation for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has significantly decreased, many questions remain unanswered. METHODS: Between 1986 and June 2010, we operated on 27 consecutive pediatric patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). All patients underwent reestablishment of a dual coronary system with direct aortic reimplantation of the left coronary artery into the aorta. Postoperative extracorporeal mechanical circulatory support was necessary in 7 cases. In all 7 patients, hemodynamic stability was achieved after 4 to 10 days of support. Mitral valve repair was performed in 9 patients with severe mitral valve incompetence and resulted in stable mitral valve function during follow-up as long as 19 years. RESULTS: There were no early or late deaths. During follow-up (3 months-17.5 years), both early and late improvement of myocardial function was observed in all patients. Reduced left ventricular regional function late after successful surgical correction of ALCAPA was related to the presence of left ventricular myocardial scar tissue, as detected by magnetic resonance imaging. CONCLUSIONS: Despite the absence of early and late mortality, the long-term prognosis for patients after reimplantation of ALCAPA into the aorta is not clear. Scars and perfusion deficits of the left ventricle may not be detected by standard echocardiographic evaluation of global left ventricular function and therefore may be underestimated. We therefore recommend lifelong surveillance of these patients, including magnetic resonance imaging.

Pediatric heart transplantation: 23-year single-center experience.

OBJECTIVE: Early and late mortality have significantly improved during recent decades in pediatric patients after heart transplantation (HTx). Nevertheless early and late morbidity and mortality are influenced by acute rejection, cardiac allograft vasculopathy (CAV), malignancy, renal failure, and graft failure. METHODS: We evaluated our results after HTx in children under the age of 18 years with 23 years of follow-up. Perioperative characteristics, probability of survival, and time-related morbidity were retrospectively analyzed. RESULTS: We included 169 pediatric HTx recipients, transplanted between 05/1986 and 05/2010. One hundred and one were males with a median age of 8.7 (0.02-23.2) years at the time of HTx. Main preoperative diagnoses were cardiomyopathy (CMP) (n = 139) with a median survival of 7.0 (0-23.2) years and congenital heart disease (CHD) (n = 30), median survival 11.3 (0-19.9) years. Overall survival at 1, 5, 10, and 15 years was 87%, 76%, 68%, and 50%, respectively. Patient survival was significantly reduced in patients with 0-1 year at the time of HTx versus 1-10 and 11-18 years: 2.3 (0-13.2) years versus 1-10 years = 8.6 (0-23.2) years; 11-18 years = 5.9 (0.003-18.5) years. Fifty-one patients were on mechanical circulatory support as a bridge-to-HTx with increased early but not late mortality. Ten patients underwent retransplant due to acute or chronic graft failure after a median posttransplant time of 12.25 (0.3-17.45) years. Late mortality was influenced by rejection, infection, posttransplant lymphoproliferative disease (PTLD) (11.8%), or CAV with an incidence of 25% at 5 years, 50% at 10 years, and approximately 75% at 15 years. CONCLUSIONS: Pediatric HTx is a safe and effective treatment for terminal heart failure. In our experience, there is no adverse effect of previous cardiac assist device implantation in long-term follow-up. Virtually all anatomic malformations are amenable to orthotopic HTx. Significant progress has been achieved in controlling rejection through improved immunosuppression and noninvasive rejection monitoring.

Rescue extracorporeal membrane oxygenation in children with refractory cardiac arrest.

We describe our experience with extracorporeal cardiopulmonary resuscitation (CPR) using extracorporeal membrane oxygenation (ECMO) in children with refractory cardiac arrest, and determine predictors for mortality. ECMO support was instituted on 42 children, median age 0.7 years (1 day-17.8 years), median weight 7.05 (range 2.7-80) kg who suffered refractory cardiac arrest (1992-2008). Patients were postcardiotomy (n=27), or had uncorrected congenital heart diseases (n=3), cardiomyopathy (n=3), myocarditis (n=2), respiratory failure (n=3), or had trauma (n=4). Cannulation site was the chest in all except for three neonates who were cannulated through the neck vessels and two children who had femoral cannulation. ECMO was successfully discontinued in 17 patients. Primary cause of mortality was neurological injury. Pre-ECMO CPR duration for survivors against those who died was a mean of 35±1.3 min vs. a mean of 46±4.2 min. Age, weight, sex, anatomic diagnosis, etiology (surgical vs. medical) were not significant predictors of poor outcome. Prolonged CPR and high-dose inotropes are significant predictors of mortality. Rescue ECMO support in children with refractory cardiac arrest can achieve acceptable survival and neurological outcomes.

Single-center experience with treatment of cardiogenic shock in children by pediatric ventricular assist devices.

BACKGROUND: Pediatric ventricular assist devices may be superior to extracorporeal membrane oxygenation in some respects, especially for medium- and long-term cardiac support. We present our nearly 20-year experience with pediatric ventricular assist devices. METHODS: Between 1990 and April 2009, Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) was implanted in 94 children. Patients were compared according to period of treatment: group I, implantation between 1990 and 2001 (n = 45), and group II, implantation since 2002 (n = 49). RESULTS: Preoperative serum creatinine (1.2 vs 0.7 mg/dL, P = .002) and bilirubin (1.5 vs 1 mg/dL, P = .002) were lower in period II, and fewer patients were artificially ventilated before surgery (26 vs 13, P = .002). In period I, more patients were supported with biventricular assist devices (64% vs 22.5%, P < .001). Median time on support was shorter in period I (10 vs 40 days, P < .001). Success (weaning from system or heart transplant) was achieved in 49% and 69%, respectively (P = .043). Whereas in period I 17% of children younger than 1 year were discharged home after transplant or weaning, rate during period II was 93% (P < .001), in particular because of improvement in discharge rate of patients with postcardiotomy heart failure (13% vs 50%). Rates of pump exchange for thrombus formation were 0.029/d in period I and 0.014/d in period II (P = 0.003). CONCLUSIONS: Recent results show significant improvements in survival and discharge rate, especially for children younger than 1 year. Pediatric Berlin Heart EXCOR ventricular assist device may provide a safe mechanical support strategy in children with cardiogenic shock.

Predictors for the use of left ventricular assist devices in infants with anomalous left coronary artery from the pulmonary artery.

BACKGROUND: Left ventricular (LV) mechanical circulatory support (MCS) may be necessary after repair of anomalous left coronary artery from the pulmonary artery. We evaluated LV function parameters for their ability to predict postoperative need for MCS. METHODS: Fourteen infants (median age, 3.6; range, 2.3 to 12 months) underwent direct aortic reimplantation of the left coronary artery. We compared preoperative LV end-diastolic diameter, end-diastolic pressure, ejection fraction, and fraction of shortening of 8 patients with successful weaning from cardiopulmonary bypass (group 1) and 6 patients with unsuccessful weaning from cardiopulmonary bypass and temporary MCS support (group 2). RESULTS: No perioperative or late deaths occurred. All patients at follow-up were free of reoperation (median follow-up, 10.4 years [range, 1.4 to 17 years]). Median preoperative LV end-diastolic diameter (47 [range, 41 to 60 mm] vs 32 mm [range, 21 to 36 mm]) and LV end-diastolic pressure (20 [range, 18 to 25 mm Hg] vs 12 mm Hg [range, 7 to 20 mm Hg]) were significantly higher in group 2 than in group 1 (p = 0.002 and p = 0.048). LV ejection fraction (0.28 [range, 0.19 to 0.37] vs 0.43 [range, 0.23 to 0.76]) and LV fraction of shortening (9% [range, 7% to 15%] vs 22% [range 13% to 30%]) were significantly lower in group 2 than in group 1 (p = 0.035 and p = 0.002). MCS support duration ranged from 4 to 12 days. There were no significant differences in LV function parameters at discharge or during follow-up between the groups. CONCLUSIONS: A preoperative LV end-diastolic diameter above 40 mm is the strongest predictor for postoperative temporary MCS after anomalous left coronary artery from the pulmonary artery repair in infancy. However, even with temporary MCS, direct aortic reimplantation for anomalous left coronary artery from the pulmonary artery can be performed with no mortality and excellent LV recovery.

The first closure of the persistent ductus arteriosus.

Congenital heart disease began to be a treatable condition when, in 1938, Robert Edward Gross first successfully ligated a persistent ductus arteriosus. This overview traces the historical development from Munro's first idea of how to close a patent ductus, presented in 1907, to the clinical ligation or division of the ductus. Surgical treatment of the infected ductus began with an unsuccessful attempt by Strieder, but it was not until Tubbs' and Touroff's successful operations that it was actually accomplished.

Extracorporeal membrane oxygenation for intraoperative cardiac support in children with congenital heart disease.

OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) is commonly used in children to allow recovery from ischemic injury or cardiac surgery, to support the circulation in case of end-stage cardiomyopathy, as bridge-to-bridge therapy and as bridge to transplantation as well. It has achieved success in providing cardiac support for these kind of patients with expected mortality due to severe myocardial dysfunction. In this modern era, ECMO support should be considered an important option for children with cardiopulmonary failure refractory to medical therapy or resuscitation. We report our experience in pediatric patients supported by ECMO for intraoperative cardiac failure between November 1991 and December 2006. METHODS AND RESULTS: Sixty-six patients with a mean age of 5.2+/-4 years (range: 1 day-17 years) and mean weight of 14.3+/-11 kg (range: 2.8-69 kg) had intraoperative ECMO support for failure to wean off cardiopulmonary bypass (n=46, 69.7%), low cardiac output syndrome (n=8, 12.1%), isolated right ventricular failure (n=6, 9.1%), isolated left ventricular failure (n=3, 4.5%), malignant arrhythmia (n=1, 1.5%) and pulmonary hypertension (n=2, 3.1%). Mean duration of ECMO support was 5.1+/-3 days. Overall 30 (45.4%) patients were successfully weaned off ECMO and survived to decannulation. Overall 6 (9.1%) patients were successfully bridged to heart transplantation while on ECMO support. Thirty patients died (54.4%) (16 while on ECMO and 14 after decannulation) because of multi-factorial complications, i.e. cerebral hemorrhage, pulmonary failure, consumption coagulopathy and therapy-resistant myocardial insufficiency, leding to an overall hospital mortality rate of 45.4%. Mean survival time after decannulation was 28+/-16 h. Overall survival rate on ECMO as bridge to recovery and transplantation has been 54.5% with successful hospital discharge of patients. CONCLUSIONS: Our experience shows that ECMO support can be offered intraoperatively to any children after palliative or corrective surgery for congenital heart disease with potentially reversible pulmonary, cardiac or cardiopulmonary failure. In the majority of patients who did not survive late after weaning from ECMO support, significant myocardial dysfunction persisted or pulmonary hypertensive events. Nevertheless, an acceptable proportion of patients who were successfully weaned from ECMO ultimately survived to leave the hospital.

Long-term cardiopulmonary exercise capacity after modified Fontan operation.

OBJECTIVE: Early circuit separation enhances the long-term success of Fontan haemodynamics. To test this hypothesis, we analysed the postoperative cardiopulmonary capacity in children and adults. PATIENTS: Spiroergometry was performed at least twice in 43 patients with a median age of 14 (range: 7-43) years, with a median time interval of 4.6 (1.1-10.4) years between early and late testing. Twenty-eight patients had been operated on in childhood and 15 as adults. The exercise capacity (W(max)) and oxygen consumption capacity (VO(2max)) were compared between children and adults. RESULTS: The VO(2max) in children early postoperatively was better than in adults (median 27.9 vs 22.9, p=0.032). Both VO(2max) (median 30.1 ml min(-1) kg(-1) vs 16.9 ml min(-1)kg(-1), p<0.001), and W(max) (median 2.2 W kg(-1) vs 1.4 W kg(-1), p<0.001) were significantly better in children late after surgery. In the patient group as a whole, there was a significant decrease of VO(2max) between early and later testing (median 26.5 l min(-1) kg(-1) vs 20.7 l min(-1) kg(-1), p<0.001). CONCLUSIONS: Fontan palliation in early childhood results in better cardiopulmonary capacity during long-term follow-up. Regular surveillance of the physical capacity by spiroergometry is indispensable for the supervision of patients with Fontan haemodynamics.