OBJECTIVES: We evaluated long-term results of two different repair strategies of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) in two German surgical centres. METHODS: We performed a retrospective review of patients who underwent ALCAPA repair between November 1980 and October 2012. Ventricular function was assessed by standardized transthoracic echocardiographic studies. RESULTS: A total of 30 patients with a median age of 0.4 years (range 0.05–37.6 years) underwent ALCAPA repair. Nineteen patients received coronary transfer, 9 underwent Takeuchi repair (creation of an intrapulmonary tunnel) and 2 were treated by closure of the ALCAPA. There was one concomitant mitral valve repair. The total follow-up was 244.8 years with a mean follow-up of 8.16 ± 6.7 years (range 0.06–24.3 years). There was no late mortality. None of the patients treated with coronary transfer or Takeuchi repair died. One moribund patient who received coronary ligation in the 1980s died perioperatively. The 10-year rate of freedom from reoperation was 94.1% for patients treated with coronary transfer. For patients with Takeuchi repair, the 10-year rate of freedom from reoperation was 71.1% (P = 0.146). At last followup, left ventricular end-diastolic diameter (LVEDD) and function measured by fractional shortening was normal in all patients. The mean shortening fraction improved from 20.25 ± 10.86% to 35.7 ± 4.8%, with the most remarkable change taking place in the first year, and the mean LVEDD indexed to normal improved from 42 ± 34% above normal values preoperatively to 3 ± 12% at the last follow-up (P < 0.01). The number of patients with moderate-to-severe mitral regurgitation (MR) improved from 50% to 6.7% (2 of 30) at the last followup. In all children receiving ALCAPA repair under the age of 6 months, MR was absent or trivial at the last follow-up. Eight years after Takeuchi repair, 79.9% of these patients suffered from at least moderate pulmonary regurgitation, in contrast to 0% after coronary transfer (P < 0.001). CONCLUSIONS: Survival rates and long-term left ventricular function were excellent for both surgical strategies. Mitral regurgitation resolved in all patients operated during their first year of life independent of the type of repair. Takeuchi repair, however, led to significant pulmonary regurgitation.

dantly secreted trophic factors, such as VEGF, MCP-1, and bFGF.This effect was synergistically enhanced in the presence of leukocytes, which secreted a cocktail of pro-and anti-inflammatory factors in response to the elastomeric scaffold (e.g.TNFa, IL-10).Surprisingly, leukocytes in the scaffold were found to express a-SMA and contribute directly to ECM production (mainly sGAG and collagen III), depending on the level of strain.In-vivo, we observed active remodeling of the valve scaffold in-situ.The long-term invivo effect of BMC preseeding on this regenerative process is currently investigated with our clinical partner.Conclusions: Our results illustrate that preseeded BMCs secrete paracrine factors, important to the in-situ tissue engineering process, in synergy with the inflammatory milieu as set by the circulating leukocytes.These findings contribute to the development of completely cell-free synthetic heart valve grafts, which would be advantageous for clinical translation.

OBJECTIVES: The choice of valve prosthesis for aortic valve replacement (AVR) in young patients is challenging. Decellularized pulmonary homografts (DPHs) have shown excellent results in pulmonary position. Here, we report our early clinical results using decellularized aortic valve homografts (DAHs) for AVR in children and mainly young adults.METHODS: This prospective observational study included all 69 patients (44 males) operated from February 2008 to September 2015, with a mean age of 19.7 +/- 14.6 years (range 0.2-65.3 years). In 18 patients, a long DAH was used for simultaneous replacement of a dilated ascending aorta as an extended aortic root replacement (EARR). Four patients received simultaneous pulmonary valve replacement with DPH.RESULTS: Thirty-nine patients (57%) had a total of 62 previous operations. The mean aortic cross-clamp time in isolated cases was 129 +/- 41 min. There was 1 conduit-unrelated death. The mean DAH diameter was 22.4 +/- 3.7 mm (range, 10-29 mm), the average peak gradient was 14 +/- 15 mmHg and the mean aortic regurgitation grade (0.5 = trace, 1 = mild) was 0.6 +/- 0.5. The mean effective orifice area (EOA) of 25 mm diameter DAH was 3.07 +/- 0.7 cm(2). DAH annulus z-values were 1.1 +/- 1.1 at implantation and 0.7 +/- 1.3 at the last follow-up. The last mean left ventricle ejection fraction and left ventricle end diastolic volume index was 63 +/- 7% and 78 +/- 16 ml/m(2) body surface area, respectively. To date, no dilatation has been observed at any level of the graft during follow-up; however, the observational time is short (140.4 years in total, mean 2.0 +/- 1.8 years, maximum 7.6 years). One small DAH (10 mm at implantation) had to be explanted due to subvalvular stenosis and developing regurgitation after 4.5 years and was replaced with a 17 mm DAH without complication. No calcification of the explanted graft was noticed intraoperatively and after histological analysis, which revealed extensive recellularization without inflammation.CONCLUSIONS: DAHs withstand systemic circulation, provide outstanding EOA and appear as an alternative to conventional grafts for AVR in young patients. EARR using DAH is a further option in aortic valve disease associated with aorta ascendens dilatation as it avoids the use of any prosthetic material.

External pacemakers (PM) via temporary epicardial leads are routinely applied to infants and children during heart surgery, which usually, after an uneventful post surgical course, can be removed without complications. We report about two infants with complex congenital heart defects after cardiac surgery (arterial switch and Mustard operation for Transposition of the great arteries). Intraoperative these patients received temporary epicardial PM wires. Thirteen and 18 days post surgery, respectively, the PM wires were removed under electrocardiogram (ECG) monitoring. The patients showed acute ECG changes in terms of significant ST elevation during and after removing their pacing wires. Clinically, patients were stable and subsequent echocardiographic examination showed no evidence of myocardial dysfunction or pericardial effusion. In the course of time, patients showed no signs of arrhythmia or abnormal ECG changes. The decision to place temporary pacing wires during the cardiac surgery in patients with congenital heart defects should be considered carefully and their removal should occur under ECG monitoring as soon as the situation of the patient allows. It should be taken into consideration that a complication like this case may be related to delayed removal of temporary PM's leads.

OBJECTIVES:The longevity of homografts is determined by the activation of the recipients' immune system resulting from allogenic antigen exposition. Fresh decellularized pulmonary homografts (DPH) have shown promising early results in pulmonary valve replacement in children and young adults and could potentially avoid significant activation of the immune system, as more than 99% of the donor DNA is removed during the decellularization process. While the humoral immune response to decellularized allografts has been studied, detailed information on the more significant cellular immune response is currently lacking.METHODS AND RESULTS:Peripheral blood samples were obtained from patients undergoing pulmonary valve replacement with DPH before, after, and for approximately 3 years after implantation. Absolute counts and percentages of mature T- (CD3(+)), B- (CD19(+)), and natural killer- (CD16(+)/CD56(+)) cells, as well as T helper- (CD4(+)) and cytotoxic T-cell- (CD8(+)) subsets, were determined by fluorescence-activated cell sorting (FACS). Between May 2009 and September 2013, 199 blood samples taken from 47 patients with a mean age at DPH implantation of 16.6±10.8 years were analyzed. The hemodynamic performance of DPH was excellent in all but one patient, and no valve-related deaths or conduit explantations were observed. The short-term follow up revealed a significant postoperative decrease in cell counts of most subtypes with reconstitution after 3 months. Continued assessment did not show any significant deviations in cell counts from their baseline values.CONCLUSION:The absence of cellular immune response in patients receiving DPH supports the concept that decellularization can provide a basis for autologous regeneration.

Background: The use of fresh, non-seeded, decellularized valved allografts shows promising results in replacement of the pulmonary valve. Here, we report about our initial clinical results of aortic valve replacement using decellularized aortic homografts (DAH) in children and adults.

BACKGROUND:This study we evaluate our results for surgical treatment of thoracic aortic aneurysms in patients with congenital heart disease.PATIENTS AND METHODS:Fifty patients aged between 12 and 71 years were treated for 51 thoracic aortic aneurysms. Forty-four of the patients developed aneurysms in the ascending aorta and seven developed aneurysms in the descending aorta. The underlying diseases for ascending aortic aneurysms included 16 congenital aortic stenosis, 16 bicuspid aortic valves, 4 transposition of the great arteries, 3 tetralogy of Fallot, 2 truncus arteriosus communis, and 3 other diseases. Patients with connective tissue disorders were excluded. All descending aortic aneurysms developed late after coarctation repair. The time interval between the correction and aneurysm operations was 15 years in ascending aneurysms and 28 years in descending aneurysms.RESULTS:The operative procedures for 44 ascending aortic aneurysms included 38 ascending aortic replacements, with or without aortic valve replacement (including 31 conduits), and 6 David operations. Graft replacement was undertaken for the seven descending aortic aneurysms. There were two hospital deaths. Forty-eight hospital survivors were in New York Heart Association functional class II or less at follow-up, which was conducted up to a maximum of 8 years after the procedure. All patients were free from reoperation and thromboembolic events. Aortic valve function was good in all six patients after the valve-sparing operation.CONCLUSION:Thoracic aortic aneurysms in patients with congenital heart disease is highly associated with bicuspid aortic valve and aortic coarctation. For ascending aortic aneurysms, conduit replacement is the method of choice. The David procedure provides good results in selected patients. For descending aortic aneurysms, graft replacement is the preferred procedure. The relatively late development of thoracic aortic aneurysms indicates that long-term follow-up in patients with congenital heart disease, especially in patients with bicuspid aortic valve and aortic coarctation, is essential.

We describe a patient in whom severe neoaortic (anatomic pulmonary) valve regurgitation developed late after Damus-Kaye-Stansel anastomosis and Fontan operation. The valve was replaced with a fresh decellularized homograft, which we developed and applied in the normal pulmonary and aortic position in more than 100 patients. During follow-up of more than 2 years, the valve function is excellent, and no infectious or thromboembolic complications were seen. The decellularized homograft seems to be an ideal material in this situation. (C) 2014 by The Society of Thoracic Surgeons

This article puts forward a modified technique of Konno aortoventriculoplasty for repeat procedures. After incision of the ventricular septum, this approach involves aortic valve replacement using a mechanical valved conduit, reimplantation of the coronary arteries, and graft replacement of the ascending aorta. This modification allows the removal of the diseased ascending aortic wall caused by a previous patch enlargement or poststenotic dilation.

Ectopia cordis (EC) is characterized by a complete or partial malposition of the heart outside the thorax. Despite the interdisciplinary treatment, the repair of EC is still very difficult and offers new surgical challenges because of its complexity and various combinations with other anomalies. We report the successful outcome after using a pedicled latissimus dorsi flap in reconstructive surgery in the setting of chronic wound dehiscence in an 8-month-old female infant born with a thoracic EC and omphalocele.

Partial anomalous pulmonary venous connection (PAPVC) causes a left-to-right shunt from the anomalous pulmonary vein (PV) to a systemic vein. We report an uncommon adult case of PAPVC, in which the left upper PV drained into both the innominate vein and the left atrium (LA), demonstrating retrograde shunting from the LA to the innominate vein. The anomaly was surgically repaired.

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.

Mild to moderate neoaortic root dilatation late after arterial switch operation (ASO) is a well-documented morbidity, but rarely needs surgical replacement. Here, we report on a 22-year-old man, who developed marked dilatation of neoaortic root and needed an aortic root replacement with mechanical conduit. His aortic valve was bicuspid. Only three cases have been reported for surgical replacement of aortic root after ASO, and two of them had bicuspid neoaortic valves. We suspect that a bicuspid neoaortic valve may be a risk factor for marked dilatation of the aortic root late after ASO.

Background— Degeneration of xenografts or homografts is a major cause for reoperation in young patients after pulmonary valve replacement. We present the early results of fresh decellularized pulmonary homografts (DPH) implantation compared with glutaraldehyde-fixed bovine jugular vein (BJV) and cryopreserved homografts (CH). Methods and Results— Thirty-eight patients with DPH in pulmonary position were consecutively evaluated during the follow-up (up to 5 years) including medical examination, echocardiography, and MRI. These patients were matched according to age and pathology and compared with BJV (n=38) and CH (n=38) recipients. In contrast to BJV and CH groups, echocardiography revealed no increase of transvalvular gradient, cusp thickening, or aneurysmatic dilatation in DPH patients. Over time, DPH valve annulus diameters converge toward normal z -values. Five-year freedom from explantation was 100% for DPH and 86±8% and 88±7% for BJV and CH conduits, respectively. Additionally, MRI investigations in 17 DPH patients with follow-up time >2 years were compared with MRI data of 20 BJV recipients. Both patient groups (DPH and BJV) were at comparable ages (mean, 12.7±6.1 versus 13.0±3.0 years) and have comparable follow-up time (3.7±1.0 versus 2.7±0.9 years). In DPH patients, the mean transvalvular gradient was significantly ( P =0.001) lower (11 mm Hg) compared with the BJV group (23.2 mm Hg). Regurgitation fraction was 14±3% and 4±5% in DPH and BJV groups, respectively. In 3 DPH recipients, moderate regurgitation was documented after surgery and remained unchanged in follow-up. Conclusions— In contrast to conventional homografts and xenografts, decellularized fresh allograft valves showed improved freedom from explantation, provided low gradients in follow-up, and exhibited adaptive growth.

Abstract We present a case of sternal steel strut dislodgement and migration in a patient undergoing Ravitch repair for pectus excavatum (PE) 37 years ago. Broken struts perforated the right ventricle and right ventricular outflow tract (RVOT) and additionally migrated into the left upper lobar bronchus. Dislodged sternal struts represent rare complications after surgical repair of patients suffering from pectus excavatum. Reviewing the literature, only five cases of intrapericardial migration of dislodged sternal struts or wires have been reported so far. In our case, the first strut was removed from the airways through a left antero-lateral thoracotomy. Using cardiopulmonary bypass, a second strut was removed via ventriculotomy. These life-threatening sequelae underscore the importance of postoperative follow-up and early removal of osteosynthetic materials used in open PE repair. Accurate preoperative localization of migrated materials and availability of CPB support are crucial for successful surgical removal. Introduction The migration of dislodged sternal steel struts or wires into the pericardium and cardiac cavities is a rare but life-threatening complication of open pectus excavatum (PE) repair 1. Removal of these materials poses a challenge for cardiothoracic surgeons. Herein, the authors report a case of migration of dislodged steel struts through the right ventricle and right ventricular outflow tract (RVOT) into the left upper lobar bronchus in a patient who underwent Ravitch repair 37 years ago.

Total anomalous pulmonary venous connection is occasionally associated with a functional single ventricle and is thought to be a risk for the staged Fontan approach. We report a patient with a very rare presentation of mitral atresia and intact atrial septum with physiologically total anomalous pulmonary venous drainage by a connecting vein between the left atrium and the superior vena cava. A surgical atrioseptectomy as a functional repair of total anomalous pulmonary venous connection and pulmonary artery banding was successfully performed as the first palliation for Fontan completion.