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.

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.

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.

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.

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.

Staged surgical palliation in hypoplastic left heart syndrome and its variants.

BACKGROUND: Surgical options for infants with hypoplastic left heart syndrome (HLHS) and/or its variants are cardiac transplantation or the heart-preserving staged palliation with Norwood operation,followed by a two-staged Fontan procedure. We describe our 17-year experience with staged palliation of HLHS and/or its variants. METHODS: Between December 1989 and December 2006, 64 patients with HLHS and/or its variants underwent a Norwood procedure (mean age/weight, 11.8+/-2.5 days/3.4 kg). Forty-four patients had classical HLHS. Twenty-eight percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 25 patients underwent a bidirectional Glenn procedure (stage II) and 11 patients a modified Fontan procedure (stage III). Others await stage II and/or stage III. The follow-up was 143.2 patient-years. RESULTS: Including the learning curve, overall early mortality from 1989 to 1999 after the Norwood procedure was 39.06%. This decreased tremendously for the last seven years, and reduced to 12.8% in 2000 to 2003 until 0% in 2004 to 2006 (p < 0.005). The causes of mortality were sepsis, capillary leak,or heart failure. Three patients died between stages II and III. One patient underwent heart transplantation after the second stage because of heart failure. Among 34 Norwood survivors, four are slightly tachypneic from a mild pulmonary hyperperfusion; one presents symptoms of minimal brain disease. CONCLUSION: This report identified an outcome improvement after staged palliation of HLHS, attributed to an increase in experience and expertise gained over time. Lower operative weight, ascending aortic size, prolonged duration of cardiopulmonary bypass, and hypothermic circulatory arrest were identified to significantly influence early mortality after the Norwood procedure.

Partial anomalous pulmonary venous drainage in young pediatric patients: the role of magnetic resonance imaging.

Studies of larger patient groups for systematic assessment of the anatomical accuracy of magnetic resonance imaging (MRI) for partial anomalous pulmonary venous drainage (PAPVD) have been performed so far only in adults. This study was undertaken to evaluate whether MRI can precisely depict pulmonary venous anatomy in infants and young children. Data on 26 children under 10 years old that underwent MRI over the past 2 years for suspected PAPVD were assessed. The MRI protocol included shunt quantification by velocity-encoded cine as well as morphological and functional assessment by multislice multiphase and contrast-enhanced MR techniques. MRI was performed in the compliant patient in breath-hold (n = 8; age range, 4.6-9.5 years) and in the noncompliant patient in conscious-sedation free breathing (n = 18; age range, 0.4 to 7.5 years). In 22 patients, PAPVD was diagnosed with MRI and confirmed during surgery. In four patients with large atrial septal defects not accessible to percutaneous closure, normal pulmonary venous return was demonstrated by MRI and confirmed during surgery. MRI under conscious sedation accurately specifies the anatomy of pulmonary veins in infants and small children. Therefore, we suggest performing MRI in patients with inconclusive transthoracic echocardiographic results in the preoperative assessment of PAPVD.

Exercise capacity reflects ventricular function in patients having the Fontan circulation.

BACKGROUND: In this study we sought to determine, first, whether maximal exercise capacity reflects ventricular function, and second, whether the age of the patient, and the age of completion of the Fontan circulation, influence ventricular function and exercise performance. METHODS AND RESULTS: Cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed in 29 patients at a median time of 6.9 years after completion of the Fontan circulation. We divided the patients into 2 groups, the first 19 having their operation below the age of 18 years, and the second group, of 10 patients, having completion of the Fontan circulation when they were older than 18 years. Parameters for ventricular function and exercise were compared for both groups with controls. Compared to controls, the younger patients had normal end-diastolic ventricular volumes, but significantly impaired ventricular function, lower maximal work load and consumption of oxygen. The older patients had greater end-diastolic ventricular volumes, and significantly poorer ventricular function than both the younger patients and the controls. Maximal work load and consumption of oxygen were significantly lower in the older patients than in the younger ones and the controls. CONCLUSION: Patients with the Fontan circulation have an impaired systolic ventricular function, which correlates with maximal exercise capacity and uptake of oxygen. Those having completion of the Fontan circulation when younger than 18 years had significantly better ventricular function and exercise performance than those who had completion of the Fontan circulation at an older age. An early creation of the Fontan circulation may preserve cardiac function and exercise capacity.

Biventricular repair in children with complete atrioventricular septal defect and a small left ventricle.

OBJECTIVE: Biventricular repair of complete atrioventricular septal defect (CAVSD) with small left ventricle aims to restore the normal loading conditions of the left ventricle. This report retrospectively evaluates the outcome of biventricular repair in 19 children with CAVSD and a small left ventricle. METHODS: Our computer database was searched for all patients with CAVSD operated on between January 1988 and December 2005. Patients who underwent biventricular repair of CAVSD were considered for investigation if they had a preoperative left ventricle-to-right ventricle long axis ratio (LAR) of <1.1 as determined by cardiac catheterization. RESULTS: There were 259 patients who underwent surgical correction of complete atrioventricular septal defect. Nineteen (10.3%) of 184 patients who underwent biventricular repair had small left ventricle based on LAR measurements. These children had no other associated congenital heart anomalies and had no previous surgery. Nine of these children had associated Trisomy 21. There were 10 (52.6%) males and 9 (47.4%) females, with age of 1-210 (122+/-67) days and weight of 2.5-9.0 (5.26+/-1.7) kg at surgery. Mean LAR was 0.76+/-0.14. Two patients (10.5%) died on the 8th and 11th postoperative day, respectively. Both had very small left ventricle (LAR of 0.45 and 0.60, respectively) and received ECMO support for postoperative low output syndrome and intractable pulmonary hypertension. A patient with an LAR of 0.62 who had ECMO support for postoperative myocardial failure underwent successful heart transplantation on the 21st postoperative day. The long-term survivors (89.5%) with LAR>0.65 had an uncomplicated postoperative course, had undergone regular follow-up (65+/-36 months) with echocardiographic assessment of the left ventricle, and had good left ventricular function. There was no early reoperation for residual ventricular septal defect, left AV valve regurgitation, or left ventricular outflow tract obstruction. Late reoperation was performed in three patients (17.4%) who underwent mitral valve repair for significant regurgitation in the 18th, 59th, and 87th month postoperatively. CONCLUSIONS: Biventricular repair of CAVSD with small left ventricle in infants and children whose LAR is >0.65, although not without risks, improve patients' functional and clinical status even in long-term follow-up. Particular caution should be taken in patients with LAR of <0.65, since these are patients who may not be amenable to biventricular repair, but for whom univentricular palliation may be more suitable.

Mid-term follow-up in patients with diaphragmatic plication after surgery for congenital heart disease.

OBJECTIVE: Diaphragmatic palsy (DP) is a rare but severe complication after surgery for congenital heart disease. Transthoracic diaphragmatic plication is an effective means of treatment for those with respiratory impairment due to DP, but little is known about the mid-term effects of diaphragmatic plication. DESIGN: We performed a study in 24 patients with history of DP. Diaphragm movement was assessed using ultrasound. Patients with DP who were old enough were additionally followed-up with lung function and exercise testing. A group of patients with similar age, diagnoses and operations served as controls. RESULTS: Ultrasound showed that in the majority of cases with history of DP the paralysed diaphragm was static, independently of whether it was plicated or not. Patients with DP had a more restrictive lung function pattern (VC: 54.3 vs. 76.4% predicted, p<0.001; FEV(1): 58.4 vs. 86.2% predicted, p<0.001) and a lower exercise capacity compared with the control group (peak VO2: 24.5 vs. 31.3 ml/kg/min, p=0.03). Comparing patients with and without plication for DP, only a tendency towards lower lung function values in patients after diaphragmatic plication, but no differences regarding exercise capacity, could be found. CONCLUSIONS: Our results provide evidence that DP is a serious surgical complication with a reduction in lung function and exercise capacity, even at mid-term follow-up; however, diaphragmatic plication, a useful tool in treating post-surgical DP in children with respiratory impairment, seems to be without mid-term risk in terms of recovery of phrenic nerve function, lung function values, and exercise capacity.

Postoperative phrenic nerve palsy: early clinical implications and management.

OBJECTIVE: We examined the clinical impact of diaphragmatic palsy (DP) as a result of phrenic nerve injury following cardiothoracic surgery, specifically its effects on morbidity and mortality, early regeneration capacity of the phrenic nerve, and role of surgical diaphragmatic plication. METHODS: A retrospective case control study was performed in 74 children with DP and 74 matched controls after cardiothoracic surgery within the past 14 years. RESULTS: Following 5,128 surgical procedures in children (aged under 18 years) we found an incidence of DP of 1.4%. There were no differences in mortality between the groups, and the cause of death was not related to DP or plication in any of the patients. However, patients with diaphragm impairment had significantly longer duration of mechanical ventilation (median 3 days vs. 1), ICU stay (7 days vs. 3.5), duration of hospital stay (16 days vs. 12), and for antibiotic treatment (16 days vs. 7). Because of prolonged respiratory problems 40 children (54%) underwent surgical diaphragmatic plication to flatten the diaphragm in its inspiratory position. In children with DP younger age was a strong predictor for plication (median 3.8 months vs. 12.1). CONCLUSIONS: Especially in newborns and young infants with DP the length of mechanical ventilation, ICU stay, and hospital stay are prolonged. Early spontaneous recovery of the phrenic nerve is rare. In cases of respiratory impairment early transthoracic diaphragmatic plication is an effective means of treatment.

Mechanical cardiac support in the young with the Berlin Heart EXCOR pulsatile ventricular assist device: 15 years' experience.

The pediatric-size pneumatically driven pulsatile extracorporeal ventricular assist device (VAD) Berlin Heart EXCOR (Berlin Heart Mediprodukt GmbH, Berlin, Germany) was introduced into clinical practice by the German Heart Institute Berlin in 1992. Until July 1, 2005, Berlin Heart EXCOR systems have been used for circulatory support in 68 children up to 18 years of age with severe circulatory failure resistant to pharmacologic therapy. These were patients suffering from cardiomyopathy, fulminant myocarditis, end-stage congenital cardiac defects, and acute heart failure following congenital heart surgery. Mean VAD support time was 35 days (range, 0 to 420 days). Forty-two patients (62%) survived to transplantation or after weaning; 37 patients (54%), including eight infants, were discharged home. These results in patients with very advanced disease have improved significantly in recent years because of technical developments and growing experience in the treatment of patients on the device, in postoperative care and optimal timing for VAD implantation. Timely implantation of the Berlin Heart EXCOR in the course of progressive heart failure now appears to be justified because the system has undergone the necessary modifications and the accumulation of clinical knowledge has made its use highly reliable and safe.

Genome-wide array analysis of normal and malformed human hearts.

BACKGROUND: We present the first genome-wide cDNA array analysis of human congenitally malformed hearts and attempted to partially elucidate these complex phenotypes. Most congenital heart defects, which account for the largest number of birth defects in humans, represent complex genetic disorders. As a consequence of the malformation, abnormal hemodynamic features occur and cause an adaptation process of the heart. METHODS AND RESULTS: The statistical analysis of our data suggests distinct gene expression profiles associated with tetralogy of Fallot, ventricular septal defect, and right ventricular hypertrophy. Applying correspondence analysis, we could associate specific gene functions to specific phenotypes. Furthermore, our study design allows the suggestion that alterations associated with primary genetic abnormalities can be distinguished from those associated with the adaptive response of the heart to the malformation (right ventricular pressure overload hypertrophy). We provide evidence for the molecular transition of the hypertrophic right ventricle to normal left ventricular characteristics. Furthermore, we present data on chamber-specific gene expression. CONCLUSIONS: Our findings propose that array analysis of malformed human hearts opens a new window to understand the complex genetic network of cardiac development and adaptation. For detailed access, see the online-only Data Supplement.

Paralysis of the phrenic nerve as a risk factor for suboptimal Fontan hemodynamics.

OBJECTIVE: The introduction of the Fontan operation for single ventricle physiology was based on the dual principle of the pulmonary blood flow. It is postulated that normal breathing movements are necessary for passive blood flow into the lungs. We compared patients with and without palsy of the phrenic nerve regarding the sufficiency of Fontan hemodynamics. METHODS: We analyzed 85 consecutive patients, who were available for follow-up after completion of their total cavopulmonary connection (TCPC) between February 1992 and February 2003. The median age at TCPC completion was 4.3 (range 1.3-37) years. Sixty were operated on with an extracardiac conduit and 25 with a lateral tunnel. Fifty patients underwent postoperative heart catheterization with contrast angiography. The diagnosis of diaphragm paralysis was made using echocardiography, fluoroscopy and X-ray examination. Surgical diaphragm plication was performed in 13 patients (Four before and nine after Fontan operation) at a median of 2.2 years after the diagnosis. RESULTS: Twenty-one patients developed fixed palsy of the phrenic nerve during a total of 225 operations before and including completion of TCPC. There were no differences in the incidence of phrenic nerve paralysis between small children (aged <3 years) and older patients or between patients with the extracardiac and intracardiac Fontan procedures. There were no differences in the duration of mechanical ventilation. However, prolonged pleural effusions and a hospital stay of longer than 2 weeks were noted more frequently in patients with palsy (P<0.05). During the median follow-up of 4.6 (range: 0.7-11.4) years significantly more patients with phrenic nerve palsy developed chronic ascites compared to those without palsy (8 of 20 vs. 2 of 65; P<0.001). CONCLUSIONS: Phrenic nerve palsy was recognized as a risk factor for suboptimal Fontan hemodynamics due to the hindrance of passive venous blood flow. Patients with phrenic nerve palsy have a longer hospital stay and a higher incidence of prolonged pleural effusions and of chronic ascites, than those without. Early diaphragm plication may be favorable to optimize the Fontan circuit in these patients. Completion of the TCPC in patients with diaphragm paralysis should be viewed critically.

Consumption of blood products during mechanical circulatory support in children: comparison between ECMO and a pulsatile ventricular assist device.

OBJECTIVE: Different mechanical circulatory support (MCS) systems are used in children with intractable heart failure. However, the need for anticoagulation leads to hemorrhage with subsequent use of blood products. We compared the coagulation disorders and the need for blood products in children treated either with extracorporeal membrane oxygenation (ECMO) or with the Berlin Heart pulsatile pneumatic ventricular assist device. PATIENTS: We retrospectively reviewed the first 8-day course of 64 children who were on MCS for more than 2 days between 1990 and 2002. Thirty children (median age 7.4 years, weight 25.5 kg) received Berlin Heart support and 34 children (median age 1.8 years, weight 9.2 kg) ECMO. Anticoagulation was accomplished by continuous infusion of heparin. Red blood cell count, platelet count, aPTT, AT III, fibrinogen, and ACT were measured regularly. Depending on blood loss and the coagulation disorder, red blood cells, fresh frozen plasma, platelets, and AT III were substituted. RESULTS: There were no preoperative differences in hematological parameters between the two groups. In the Berlin Heart group platelet transfusion was 4.3 ml x kg x day vs 24.6 ml x kg x day in the ECMO group. Red blood cell substitution was 17.2 vs 60.3 ml.kg.day. Fresh frozen plasma substitution was 8.5 ml x kg x day vs 46.9 ml x kg x day (P<0.001). Even in the congenital heart defect subgroups, when MCS was implanted without recent cardiotomy, the differences were significant. Nevertheless, the mean daily values for hemoglobin, platelets, and fibrinogen were lower in the ECMO group. There was lower overall mortality in the Berlin Heart group. CONCLUSIONS: Compared to ECMO, use of the Berlin Heart in children results in less blood loss and lower consumption of red blood cells, platelets, and fresh frozen plasma.

Atrial switch operation: past, present, and future.

The atrial switch operation was developed by the efforts of many surgeons, with the most notable contributions made by Blalock, Hanlon, Albert, Baffes, Senning, and Mustard. The atrial switch operation was the first definitive repair for patients with transposition of great arteries and produced good results. Although it is rarely performed today, the atrial switch is not merely of historical interest as there remain a few important indications for this operation. A thorough understanding of the atrial switch is still required for surgeons dealing with complex congenital cardiac malformations. Herein we summarize the history, review long-term results, and discuss the future of the atrial switch operation.

Comparison of somatic development and status of conduit after extracardiac Fontan operation in young and older children.

OBJECTIVE: We set out to examine whether the extracardiac Fontan operation (ECFO) in young children is beneficial for the early postoperative course and whether it has a negative impact on the mid-term hemodynamics and growth of the children due to absent growth potential of the prosthetic conduit. Therefore we compared our medium-term experience with ECFO in children under 4 years of age to that in older children regarding the incidence of postoperative complications, somatomotoric development and conduit status. METHODS AND RESULTS: Between 11/95 and 12/02 ECFO was performed in 30 children under 4 years of age and 21 older children aged 4-13 years. There were no deaths in children under 4 years of age and two older children died. No prolonged support (>72 h) of suprarenin was required in small children compared to 4 older children. In twenty-seven children, who underwent postoperative heart catheterization no pulmonary artery or systemic vein distortion occurred. One re-operation and one transcatheter intervention were performed because of the partial conduit stenosis. During the median follow-up of 4.8 years a manifestly accelerated postoperative weight gain was observed in children operated on under 4 years of age, compared to that in older children (up to the 50 vs. 10th percentile, P<0.05). CONCLUSIONS: The ECFO could be performed in young children with low morbidity and mortality rates. In the majority of patients, implanted prosthetic grafts maintain stable form without the development of stenosis. Accelerated somatic development, especially in small children, is to be observed after completion of the Fontan circulation.

Extracorporeal Membrane Oxygenation as a Bridge to Cardiac Transplantation in Children.

The feasibility and efficacy of extracorporeal membrane oxygenation (ECMO) as a bridge to cardiac transplantation was examined in 6 pediatric patients who suffered irreversible myocardial failure after undergoing surgery for congenital heart defects. The mean time of ECMO support was 260.5 h, range, 101-402 h. Three patients underwent transplantation, 2 of whom are long-term survivors. Progressive hypotension as a result of capillary leak syndrome precluded further ECMO support in the other 3 patients. Overall, 2 of the 6 patients survived. Major complications were encountered in 4 patients including bleeding in 2, a seizure in 1, and renal failure in 3, 2 of whom recovered renal function after transplantation. Infection did not occur in any of the 6 patients. Exchanging ECMO components was performed with no difficulties; these exchanges included a centrifugal pump once for 2 patients and a membrane oxygenator once for 3 patients. Our results indicate that ECMO can safely keep critically ill pediatric transplant candidates alive for more than 1 week with a low incidence of multiple organ failure.

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.

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.