The standing of percutaneous pulmonary valve implantation compared to surgery in a non-preselected cohort with dysfunctional right ventricular outflow tract - Reasons for failure and contraindications.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) numbers are rising but are still minor compared to surgery due to several contraindications. We sought to analyze the impact of PPVI compared to standard surgery in an unselected cohort with dysfunctional right ventricular outflow tract (RVOT). Reasons for PPVI failure and possible contraindications were explored. METHODS: Between 2010 and 2015 all consecutive patients who underwent surgery or intervention for a dysfunctional RVOT were investigated. RESULTS: A total of 382 cases was identified - 246 patients underwent successful valve insertion: 166 surgeries (166/246=67.4%) with 55/166 homografts (33.1%), 106 Contegra® grafts (63.8%), 5 Hancock valves (3.0%). Overall, 70/246 patients presented a priori with an enlarged RVOT>28mm (28.5%) not appropriate for PPVI and 14/246 (5.7%) had additional defects necessitating surgery. Some 31/246 patients had surgery for initial repair of congenital defects or were too small (<20kg) for PPVI (12.6%). 80 underwent successful PPVI (32.5% of 246 valves implanted) [51 Edwards Sapien® valves (63.7%), 29 Melody valves (36.3%)]. The RVOT was too large for PPVI in 22/246 patients (8.9%). A total 20/246 patients (8.1%) showed coronary compression after balloon interrogation. In 4/246 patients PPVI was not possible due to technical issues (1.4%). CONCLUSION: PPVI could be performed successfully in 80/382 patients (20.9%). An enlarged RVOT, small patient size, and coronary compression were the major obstacles for interventional management. Future developments for larger RVOTs and smaller body weight may expand the indication for PPVI.

High proportion of genetic cases in patients with advanced cardiomyopathy including a novel homozygous Plakophilin 2-gene mutation.

Cardiomyopathies might lead to end-stage heart disease with the requirement of drastic treatments like bridging up to transplant or heart transplantation. A not precisely known proportion of these diseases are genetically determined. We genotyped 43 index-patients (30 DCM, 10 ARVC, 3 RCM) with advanced or end stage cardiomyopathy using a gene panel which covered 46 known cardiomyopathy disease genes. Fifty-three variants with possible impact on disease in 33 patients were identified. Of these 27 (51%) were classified as likely pathogenic or pathogenic in the MYH7, MYL2, MYL3, NEXN, TNNC1, TNNI3, DES, LMNA, PKP2, PLN, RBM20, TTN, and CRYAB genes. Fifty-six percent (n = 24) of index-patients carried a likely pathogenic or pathogenic mutation. Of these 75% (n = 18) were familial and 25% (n = 6) sporadic cases. However, severe cardiomyopathy seemed to be not characterized by a specific mutation profile. Remarkably, we identified a novel homozygous PKP2-missense variant in a large consanguineous family with sudden death in early childhood and several members with heart transplantation in adolescent age.

Bovine Jugular Veins versus Homografts in the Pulmonary Position: An Analysis across Two Centers and 711 Patients-Conventional Comparisons and Time Status Graphs as a New Approach.

BACKGROUND: Various diseases and diversity in implantation ages, together with evolving diagnostic and therapeutic options, hinder comparative evaluations of long-term outcomes for valved conduits used for reconstruction of the right ventricular outflow tract (RVOT). We combined two common evaluation methods to optimally use information obtained by pooling the raw data from two high volume centers, each with very regular follow-up procedures, with the aim of analyzing durability differences between conventional homografts and bovine jugular veins. PATIENTS AND METHODS: In the period 1985 to 2012, a total of 444 bovine jugular veins and 267 homografts were implanted, and 6,738 postoperative examinations took place. Evaluations included age-stratified Kaplan-Meier analyses, Cox regression models, and time status graphs, the third showing age-group stratified, time-related frequencies of intact, insufficient, stenotic, both insufficient and stenotic, and postinterventional conduits below the freedom from explantation curve. They take into account interventions, explantations, and the nonterminal character of echocardiographic findings. RESULTS: The durability of intact bovine jugular veins in children and young adults is not inferior to that of homografts. Averaged over the first 12 years after implantation, the age groups < 25 years in fact showed advantages for bovine jugular vein recipients. The average fraction of patients younger than 25 years whose conduits were not explanted, postinterventional, stenotic, insufficient, or stenotic and insufficient was at least 10% higher in recipients of bovine jugular veins than in homograft recipients. CONCLUSION: According to the time status graphs, the use of bovine jugular veins for RVOT in patients younger than 25 years appears to lead to superior results when compared with cryopreserved homografts.

"Intraoperative hybrid stenting of recurrent coarctation and arch hypoplasia with large stents in patients with univentricular hearts".

BACKGROUND: Obstruction of the reconstructed aortic arch, tubular hypoplasia and recurrent coarctation (RC) is an important risk factor in univentricular physiology. For the past two years we have adopted the concept of intraoperative hybrid stenting of RC and arch hypoplasia with large stents in patients with univentricular hearts as standard care procedure. METHOD/RESULT: Retrospective analysis of the anatomy and procedural outcome of 14 patients was scheduled for intraoperative stenting of the aortic arch (12 during surgery for BCPS, 2 during Fontan completion). The median age was 5.3 months, weight 5.5 kg, height 62 cm. Five patients had tubular hypoplasia and 9 patients had distal stenosis of the aortic arch. Nine patients had a previous balloon dilatation. The mean diameter of the distal arch was 11.0mm, at the coarctation 5.1mm, at the level of the diaphragm 8.2mm (CoA-index 0.62). Intraoperative stenting was performed in 13/14 patients. Stents were implanted with a mean balloon diameter of 10.8mm (SD 3.4mm). The achieved final mean diameter was 9.8mm (mean, SD 2.8mm) with an oversized Coa-index of 1.2. There was no re-coarctation at a mean follow-up of 7.3 months (range 3 to 24), the maximum flow velocity of 2m/s across the stented lesion assessed by ECHO. CONCLUSION: This hybrid approach is an easy and safe concept to manage recurrent aortic arch hypoplasia and stenosis. The use of large stents allows redilatation to adult size diameters later on.

Stenting of the right ventricular outflow tract in symptomatic neonatal tetralogy of Fallot.

There is ongoing debate regarding the initial management of symptomatic neonates with tetralogy of Fallot. Although neonatal repair can be performed with low mortality, it is associated with increased morbidity and long-term impact on right ventricular performance. Traditionally, the modified Blalock-Taussig shunt remains the palliative procedure of choice. Differential pulmonary artery flow may occur and subsequently result in underdevelopment and distortion of pulmonary vessels. Transcatheter therapy was previously limited to balloon valvulotomy when the obstruction is predominantly at the pulmonary valve level. Stenting of the right ventricular outflow tract can enable adequate forward flow; however, pulmonary regurgitation may impact on right ventricular performance and cardiac output. Stenting of the right ventricular outflow tract with valve sparing placement of the stent thus treating the underlying pathophysiology of the hypercyanotic spells provides a safe and effective management strategy, improving arterial oxygen saturation, avoiding pulmonary regurgitation and encouraging pulmonary artery growth.

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.

Safety of Long-Term Mechanical Support With Berlin Heart EXCOR in Pediatric Patients.

Background. The Berlin Heart EXCOR Pediatrics is utilized at our center as a bridge to transplantation or bridge to recovery. This retrospective study reviews our results regarding the safety of long-term support and outcome. Methods. Between January 2008 and December 2010, 12 patients (6 females and 6 males) underwent implantation of a ventricular assist device. The median weight was 14.2 kg (range 4.2-51.6 kg) and the median age was 4.12 years (range 0.25-11.83 years). All patients were on inotropes, five patients required mechanical ventilation and three patients experienced cardiopulmonary resuscitation. Results. Eight patients received a left ventricular assist device and four patients received a biventricular assist device. Of the 12 patients, 8 were bridge to heart transplantation, in 2 patients explantation was possible, and 1 patient died on support. The median support time for these 11 patients was 151 days (range 4-488 days), with 2124 days of cardiac support. One patient is on support. Survival rate was 91.6%. Seven patients had a blood pump change once. Four patients had local signs of infection. There was no mediastinitis and thromboembolism. One patient had intracerebral hemorrhage. There was no death after heart transplantation or after explantation of the device. Conclusions. The Berlin Heart EXCOR is effective in bridging children of almost all ages and sizes to cardiac transplantation or myocardial recovery. Our experience proved that long-term support is possible with a low rate of adverse events.

Infective endocarditis in infancy after complete atrioventricular septal defect repair.

We report a rare case of infective endocarditis after repair of a complete atrioventricular (AV) septal defect in a four-month-old patient. Perforation of a valve leaflet with progressive severe left AV valve regurgitation required surgical intervention. Valve reconstruction using fresh autologous pericardium was successfully accomplished. This reconstruction in association with prolonged antibiotic therapy resulted in complete recovery of the patient.

Successful correction of a total anomalous venous connection in a 63-year-old male--case report and review of the literature.

Total anomalous pulmonary venous connection is a rare variant of cyanotic congenital heart disease and usually requires surgical correction within the first few months of life. We report midterm results of a 63-year-old male with intracardiac total anomalous venous return into the coronary sinus who presented with congestive predominantly right heart failure and underwent corrective surgery with unroofing of the coronary sinus and patch closure of the secundum atrial septal defect.

Reoperations in adults after correction of tetralogy of Fallot.

Adults with congenital heart diseases are an increasing patient population. Since the first successful palliative procedure in 1945 (Blalock and Tausing, JAMA 128: 189-194, 1945) and first corrective operation in 1955 (Lillehei et al., Ann Surg 142: 418, 1955) patients with Tetralogy of Fallot, which is the most common cyanotic CHD, have had excellent long term results and good quality of life. Over the long term, these patients are under risk of sudden death because of arrhythmias and congestive heart disease caused by tricuspid regurgitation, pulmonary regurgitation, ventricular septal defect, right ventricular outflow tract obstruction.

Scimitar syndrome presenting in adults.

Scimitar syndrome is a rare and complex congenital anomaly, which is characterized by the image of a Turkish sword on the chest X-ray. Very few cases in adults are reported in the literature. The long-term results of scimitar syndrome after surgical correction remain disappointing. We report an adult patient with scimitar syndrome undergoing surgery at our center.

Heart transplantation after Fontan procedure in adults.

Complex congenital heart disease is the cause for 1.5%-2.4% of heart transplantations in adults. Indications for heart transplantation are ventricular dysfunction, failed Fontan physiology, and cyanosis. We present a 43-year-old man, 20 years after a Fontan operation by an univentricular heart, with a tricuspid atresia, L-Transposition of the Great Arteries, and high-grade subpulmonary stenosis. Persistent bleeding after successful transplantation necessitated major blood transfusions and reoperation. We reconstructed superior vena cava and vena brachiocephalica using a pericardial patch, and because of postoperative low output syndrome, he was connected to extracorporeal circulation through cannulation of femoral artery and explanted after hemodynamic stability. Heart transplantation can be successful in patients with end-stage congenital heart diseases after Fontan procedure, because of improved surgical and medical management. Challenges include limited ability to assess hemodynamics, complex anatomy, increased risk of bleeding caused by multiple prior procedures, and unique underlying pathologic states. Careful patient selection and surgical timing are essential to increase long-term survival.

Surgical repair of congenital supravalvular aortic stenosis in adult.

Supravalvular aortic stenosis is a rare congenital cardiac anomaly occurring mainly as a part of Williams-Beuren syndrome. Aortic narrowing above the level of the aortic valve causes obstruction of the left ventricular outflow tract, and a pressure gradient between the left ventricle and the aorta causes left ventricle hypertrophy. We report here a case of a 22-year-old man who underwent extended patch aortoplasty because of supravalvular aortic stenosis accompanying Williams-Beuren syndrome. He was in New York Heart Association functional class III with localized hourglass type supravalvular aortic stenosis. Related to arterial hypertension he was in a cardiac decompensation. Mean pressure gradient was 73 mm Hg and maximum gradient 104 mm Hg. Electrocardiography indicated left ventricle hypertrophy, which was also seen in x-ray, as heart enlargement. We successfully treated this patient with extended patch aortoplasty and immediate postoperative echocardiography showed reduction of gradient. Good surgical outcome of congenital supravalvular aortic stenosis in adults can be achieved with this treatment. This technique provides symmetric reconstruction of the aorta with good postoperative results and no gradient across aortic valve and aortic valve insufficiency remains, providing excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence.

Heart transplantation in children: clinical outcomes in a single center.

BACKGROUND: Despite being accepted as the best treatment for end-stage heart diseases, the long-term benefit of heart transplantation in children remains a matter of controversial debate. This study aimed to evaluate our clinical experience with heart transplantation in children. METHODS: From March 1989 to December 1999, 93 consecutive orthotopic heart transplantations in children (less than 18 years of age) were performed at the Department of Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW in Bad Oeynhausen, Germany. Clinical data were retrieved from a computerized database. Follow-up information was 100% completed. RESULTS: The main indications for heart transplantation were dilated cardiomyopathy (68%) and congenital heart disease (31%). Early mortality risk was 14% +/- 3.6%. Primary graft failure (39%) was the main cause of early death. Total follow-up time was 694 patient-years (mean, 104.1 +/- 42.8 months). Twenty-three patients died during follow-up, resulting in 33 of 1,000 patient-years of late mortality rate. Acute rejection (43%) and allograft vasculopathy (26%) were attributed to late mortality. The 1-, 5-, 10-, and 15-year survival was 83%, 74%, 63%, and 50%, respectively. Recipient age less than one year (p = 0.02) and ischemia time greater than 300 minutes (p = 0.04) were associated with decreased survival. Social activities at the end of follow-up were school (69%), working (19%), and at home (12%). CONCLUSIONS: Heart transplantation is a rational and durable treatment option for children with end-stage heart diseases. The long-term outcomes and quality of life after heart transplantation in children are encouraging.

Heart transplantation in adults with congenital heart disease: experience with 15 patients.

End-stage congenital heart disease (CHD) is an important indication for pediatric heart transplantation (HTx) as well as transplantation in adult populations. The purpose of this retrospective analysis was to compare the survival rate of adults who underwent HTx for end-stage CHD with those who underwent HTx for other causes. To find out whether HTx is a viable therapeutic option for adult patients with preoperated CHD, data from 15 adult patients with different forms of CHD, who had previously undergone different corrective and palliative procedures, were retrospectively analyzed and compared with the HTx data of 1400 adult patients (>15 years old) whose indications for HTx were other than end-stage CHD. From 1989 to 2005, 15 adult patients (eight men/seven women) were given transplantation for end-stage CHD. Ten patients had been preoperated once, five patients twice. Their mean age was 34.06 +/- 3.9 years. In five cases, patients had development of acute renal failure. One female patient died 40 days after surgery, after having a cerebral infarction; one male patient died 4 years after HTx for OKT 3 monoclonal antibody-resistant rejection; and two patients died as the result of multiple organ failure at 4 days and 30 days after HTx, respectively; 11 patients are still alive. The cumulative survival rate at 1 year is 80% versus 80% in patients given transplantation for noncongenital indications. Heart transplantation in adults with end-stage CHD can be performed with a good long-term prognosis. Previous palliative operations do not affect outcome after HTx.

Mechanical ventricular circulatory support in children; Bad Oeynhausen experience.

INTRODUCTION: Recently various mechanical circulatory support systems are being used all over the world, nevertheless the size of the devices limits the implantation in pediatric cases. Accordingly we report our experiences with assist devices applied for pediatric patients. PATIENTS AND METHODS: Twenty-nine children underwent mechanical circulatory support implantation operation. The diagnoses of preoperation were dilated cardiomyopathy in 16, congenital heart disease in 12 and allograft dysfunction in 1. RESULTS: From November 1987 to January 2004 we implanted 7 LVAD, 11 BVAD and 11 ECMO in pediatric patients. The 29 patients were supported from 11 to 231 days (mean 32.3 days). Three children were supported by Thoratec LVAD. Biventricular Thoratec VAD was used in 3 children. Three children were supported by Medos-HIA system LVAD, and 8 children by biventricular VAD using the Medos-HIA system. One child was supported by Novacor LVAD. Fourteen children were supported by ECMO. We succeeded in heart transplantation in 10 cases, but lost 16 children during the support. Bleeding occurred in 7 cases, thrombosis occurred in 6 cases, infection occurred in 8 cases, pneumothorax occurred in 3 cases and neurological deficit occurred in 2 cases. CONCLUSION: The development of assist device for children which has long durability and small in size as a future subject is desired. Further clinical and experimental research and application of those assist devices for children are in progress.

Long-term results of pediatric heart transplantation.

OBJECTIVE: We retrospectively reviewed 104 consecutive patients who underwent orthotopic heart transplantation between November 1989 and February 2004. PATIENTS AND METHODS: From November 1989 to February 2004, the total number of heart transplantations were 1,340 cases at our institute. One hundred four (7.8%) of these 1,340 patients were pediatrics. Average age was 6.2 years, ranging from 4 months to 16 years. The cause of heart disease before transplantation was: idiopathic dilated cardiomyopathy (DCM) in 74 patients (71%), and congenital heart disease (CHD) in 30 (29%). RESULTS: Hospital mortality rate was 14.4% (15 patients). Late complications were rejection in 23 (22.1%), infection in 11 (10.6%), gingival hyperplasia in 28 (26.9%), hypertention in 22 (21.1%), coronary artery disease (CAD) in 12 (12.5%), graft failure in 11(10.5%), and malignancy in 2 (1.9%). Late mortality occurred in 11 (10.6%) patients. Causes of death were sudden death in 2 (1.9%), CAD in 3 (2.8%), graft failure in 1 (1.0%), acute rejection in 4 (4.6%), and infection in 1 (1.0%). Actuarial survival rates in pediatrics at 1, 5, 10 years were 82%, 80%, and 78%, respectively. On the other hand, actuarial survival rates in adults at 1, 5, 10 years were 78%, 75%, 59%, respectively. CONCLUSION: Heart transplantation for pediatrics is an effective therapy with acceptable morbidity and mortality. The long-term survival results in pediatrics are comparable to those of adult heart transplantations. However, the actuarial survival rate in pediatrics after 10 years is significantly better than in adults' cases. Renal function in pediatric heart transplantation recipients treated with cyclosporine remains stable during long-term follow-up.

Atrial septal defects in pediatric patients: noninvasive sizing with cardiovascular MR imaging.

PURPOSE: To evaluate phase-contrast magnetic resonance (MR) imaging for sizing of secundum atrial septal defects (ASDs) and inflow MR angiography for detection of associated venous anomalies in pediatric patients with inconclusive transthoracic echocardiographic (TTE) results. MATERIALS AND METHODS: Sixty-five children (mean age, 5.4 years +/- 2.7 [SD]) with ASD and inconclusive TTE results underwent phase-contrast MR imaging. Defect size and rim distances measured on MR imaging sections obtained in the ASD plane and from the defect to the venae cavae, aortic root, and atrioventricular valves were compared with transesophageal echocardiographic (TEE) findings (n = 30) during transcatheter closure or surgical measurements (n = 40) by using Bland-Altman analysis. Inflow MR angiography was compared with invasive cine angiocardiography for detection of associated venous anomalies. RESULTS: For ASD size, mean differences were less than 1 mm between MR imaging and TEE measurements (with upper and lower limits of agreement between 2.3 and -3.3 mm) and were between 1.2 and -1.6 mm between MR imaging and surgical measurements (with upper and lower limits of agreement between 4.7 and -5.2 mm). Septal rim measurements at MR imaging agreed fairly well with TEE and surgical results. Septal length was overestimated at MR imaging versus TEE (mean difference, 3.0 mm; upper and lower limits of agreement, between 8.0 and -2.8 mm), but MR imaging septal length measurements agreed with surgical results. Rim distance to coronary sinus was difficult to assess. MR imaging enabled referral of 25 of 30 patients for successful transcatheter closure; five patients were found to have too large defects after balloon sizing. Multiple ASDs and/or associated vascular anomalies in 17 of 65 patients were clearly identified at MR imaging, compared with results of TEE, surgery, and cardiac catheterization. CONCLUSION: In children with ASD and inconclusive TTE results, MR imaging can enable determination of defect size, rim distances to adjacent structures, and venous connections.

Results of staged reconstruction for hypoplasia of the left heart: an experience of 12 years from one institution.

BACKGROUND: We reviewed our 12-year experience with staged reconstruction for hypoplasia of the left heart, examining the results of each surgical step and the impact of the year of the Norwood operation on survival. We compared survival of patients with hypoplasia of the left heart subsequent to completion of the Fontan circulation to survival of patients with a dominant left ventricle undergoing a Fontan procedure. PATIENTS: Between 1989 and 2001, we performed a first stage procedure in 89 patients. Their median age was 9 days, with a range from 2 to 140 days, and the median weight was 3.4 kg, with a range from 2.4 to 5.4 kg. RESULTS: Survival at 1, 4, and 10 years was 55%, 49%, and 49%, respectively. We experienced 23 early deaths (26%), and 12 deaths between the stages of the Norwood cascade. Of our patients, 42 underwent the second stage, and 30 the third stage. Prior to the first stage, symptoms of necrotising enterocolitis, and of obstructed pulmonary venous return, influenced survival significantly. The latter was eliminated as risk factor when surgery was performed within the first week of life. During the later part of our experience, survival at the first stage operation improved significantly, with survival at 3 years increasing from 42% to 75% for the patients at standard-risk (p = 0.017), and from 17% to 42% for those deemed to be at high-risk (p = 0.1). No deaths occurred in 23 patients older than 3 years of age, all of whom had proceeded through the third stage. After completion of the Fontan circulation, the survival of the patients with hypoplasia of the left heart at 4 years was comparable to the survival of patients undergoing the Fontan procedure with a dominant left ventricle (88% versus 90%, p = 0.8). CONCLUSIONS: Early and intermediate survival has improved significantly over the period of 12 years. Late death has been uncommon, and none of our patients are listed for cardiac transplantation.