Antithrombotic therapy in pediatric ventricular assist devices: Multicenter survey of the European EXCOR Pediatric Investigator Group.

OBJECTIVES: Mechanical circulatory support for pediatric heart failure patients with the Berlin Heart EXCOR ventricular assist system is the only approved and established bridging strategy for recovery or heart transplantation. In recent years, the burden of thromboembolic events has led to modifications of the recommended antithrombotic therapy. Therefore, we aimed to assess modifications of antithrombotic practice among the European EXCOR Pediatric Investigator Group members. METHODS: We sent a questionnaire assessing seven aspects of antithrombotic therapy to 18 European hospitals using the EXCOR device for children. Returned questionnaires were analyzed and identified antithrombotic strategies were descriptively compared to "Edmonton protocol" recommendations developed for the US EXCOR pediatric approval study. RESULTS: Analysis of 18 received surveys revealed substantial deviations from the Edmonton protocol, including earlier start of heparin therapy at 6-12 h postoperatively and in 50% of surveyed centers, monitoring of heparin effectiveness with aPTT assay, administering vitamin K antagonists before 12 months of age. About 39% of centers use higher international normalized ratio targets, and platelet inhibition is changed in 56% including the use of clopidogrel instead of dipyridamole. Significant inter-center variability with multiple deviations from the Edmonton protocol was discovered with only one center following the Edmonton protocol completely. CONCLUSION: Current antithrombotic practice among European EXCOR users representing the treatment of more than 600 pediatric patients has changed over time with a trend toward a more aggressive therapy. There is a need for systematic evidence-based evaluation and harmonization of developmentally adjusted antithrombotic management practices in prospective studies toward revised recommendations.

Melody transcatheter valve: Histopathology and clinical implications of nine explanted devices.

OBJECTIVES: We examined interventionally implanted valved Melody conduits after surgical explantation by means of histology and immunohistochemistry and matched these findings with clinical data in order to assess in vivo biocompatibility and to identify risk factors for graft failure. METHODS: 9 Melody valves had been implanted in 8 patients (pulmonary n = 7, tricuspid position n = 1). Indication for explantation included significant obstruction in 7 patients and valve insufficiency in 1 patient. 4 of 8 patients had suffered from endocarditis. Mean interval between implantation and explantation was 3.2 (1.8-5.2) years. All explants were worked up using a uniform protocol with fixation in formalin and embedding in methylmethacrylate. RESULTS: All but one valve of the explanted Melody grafts were thin and histologically intact without any pathological findings. Complete neo-endothelialization could be demonstrated by means of immunohistochemistry. All 4 Melody valves from patients with endocarditis showed dense granulocytic infiltrations, 3 of these showed thrombotic material within the valves. CONCLUSION: This report covers the first series of explanted Melody valves from humans applying a uniform protocol for histopathological examination. Good biocompatibility of the Melody valves could be demonstrated after a mid-term follow-up. Factors for graft failure included endocarditis, outgrowth, and residual stenosis. These findings may have significant implications for the implant procedure as well as care of the patients during long-term follow-up.

Muscular ventricular septal defect in a newborn with truncus arteriosus communis: perventricular transcatheter closure under echocardiographic guidance only, a case report.

We report a case of a 3.5-kg newborn presenting with a muscular ventricular septal defect (VSD) in the setting of truncus arteriosus communis (common arterial trunk). Reparative surgery using a hybrid approach included perventricular closure of the muscular VSD on the beating heart.

Successful management of neonatal myocardial infarction with ECMO and intracoronary r-tPA lysis.

Acute myocardial infarction (MI) is a life-threatening condition rarely encountered in neonates. The patients usually present with sudden cardiogenic shock. Clinical management in neonates is extremely challenging. If treatment is delayed, the prognosis is dismal. We report on a 4-day-old full-term male newborn presenting with acute MI and cardiogenic shock secondary to proximal thromboembolic occlusion of the left descending coronary artery. Hemodynamic stabilization could only be achieved after extracorporeal membrane oxygenation (ECMO) support. Coronary artery patency restoration was performed by selective intracoronary lysis with recombinant tissue plasminogen activator (r-tPA). ECMO support could be discontinued and myocardial function recovered within 6 weeks. We discuss the potential etiologies of acute perinatal MI and the role of ECMO support in the immediate post-MI period. Prompt recognition, timely referral to a cardiac center with availability of specialized advanced treatment options, and management in an orchestrated interdisciplinary approach are crucial for achieving a good outcome.

What are the obstacles to training in surgery for congenital heart disease in Germany?

In recent years, several publications from German institutions have highlighted the need for quality assessment, complexity and risk interpretation, and assurance of sustainable structures in congenital heart surgery. In addition, there is a severe shortage of congenital cardiac surgeons in Germany. To provide quality assurance, the German Society for Thoracic and Cardiovascular Surgery (DGTHG, Deutsche Gesellschaft für Thorax-, Herz- und Gefäßchirurgie) offers a Certificate for Congenital Cardiac Surgery, which is awarded with proof of a minimal number of congenital procedures. We outline that there are too many low-volume centers in Germany putting the cardiac surgeon into the role of a "soloist." In this situation, adequate training cannot be provided in the majority of the centers in Germany. Furthermore, a considerable reduction of the specifically required operations in the "Weiterbildungsordnung für die Ärzte" (WBO) will be paramount for the future. There should be a stronger consideration for the specific requirements for congenital heart surgeons, to make their training shorter and less cumbersome. Also, the DGTHG's Certificate for Congenital Cardiac Surgery should be tailored to realistic needs in the field of congenital surgery. An adjustment of both the WBO and the Certificate would be in line with the justified claim made in the recommendations of the DGTHG.

Reoperations on the pulmonary autograft and pulmonary homograft after the Ross procedure: An update on the German Dutch Ross Registry.

OBJECTIVES: Reinterventions after the Ross procedure are a concern for patients and treating physicians. The scope of the present report was to provide an update on the reinterventions observed in the large patient population of the German-Dutch Ross Registry. PATIENTS AND METHODS: From 1988 to 2011, 2023 patients (age, 39.05 ± 16.5 years; male patients, 1502; adults, 1642) underwent a Ross procedure in 13 centers. The mean follow-up was 7.1 ± 4.6 years (range, 0-22 years; 13,168 patient-years). RESULTS: In the adult population, 120 autograft reinterventions in 113 patients (1.03%/patient-year) and 76 homograft reinterventions in 67 patients (0.65%/patient-year) and, in the pediatric population, 14 autograft reinterventions in 13 patients (0.91%/patient-year) and 42 homograft reinterventions in 31 patients (2.72%/patient-year) were observed. Of the autograft and homograft reinterventions, 17.9% and 21.2% were performed because of endocarditis, respectively. The subcoronary technique in the adult population resulted in significantly superior autograft durability (freedom from autograft reintervention: 97% at 10 years and 91% at 12 years; P < .001). The root replacement technique without root reinforcement (hazard ratio, 2.4; 95% confidence interval, 1.4-4.1) and the presence of pure aortic insufficiency preoperatively (hazard ratio, 2.3; 95% confidence interval, 1.5-3.5) were statistically significant predictors for a shorter time to reoperation. The center volume had a significant influence on the long-term results. The freedom from homograft reoperation for the adults and pediatric population was 97% and 87% at 5 years and 93% and 79% at 12 years, respectively (P < .001), with younger recipient and donor age being significant predictors of a shorter time to homograft reoperation. CONCLUSIONS: The autograft principle remains a valid option for young patients requiring aortic valve replacement. The risk of reoperation depends largely on the surgical technique used and the preoperative hemodynamics. Center experience and expertise also influence the long-term results. Adequate endocarditis prophylaxis might further reduce the need for reoperation.

Major adverse cardiac and cerebrovascular events after the Ross procedure: a report from the German-Dutch Ross Registry.

BACKGROUND: The purpose of the study is to report major cardiac and cerebrovascular events after the Ross procedure in the large adult and pediatric population of the German-Dutch Ross registry. These data could provide an additional basis for discussions among physicians and a source of information for patients. METHODS AND RESULTS: One thousand six hundred twenty patients (1420 adults; 1211 male; mean age, 39.2±16.2 years) underwent a Ross procedure between 1988 and 2008. Follow-up was performed on an annual basis (median, 6.2 years; 10 747 patient-years). Early and late mortality were 1.2% (n=19) and 3.6% (n=58; 0.54%/patient-year), respectively. Ninety-three patients underwent 99 reinterventions on the autograft (0.92%/patient-year); 78 reinterventions in 63 patients on the pulmonary conduit were performed (0.73%/patient-year). Freedom from autograft or pulmonary conduit reoperation was 98.2%, 95.1%, and 89% at 1, 5, and 10 years, respectively. Preoperative aortic regurgitation and the root replacement technique without surgical autograft reinforcement were associated with a greater hazard for autograft reoperation. Major internal or external bleeding occurred in 17 (0.15%/patient-year), and a total of 38 patients had composite end point of thrombosis, embolism, or bleeding (0.35%/patient-year). Late endocarditis with medical (n=16) or surgical treatment (n=29) was observed in 38 patients (0.38%/patient-year). Freedom from any valve-related event was 94.9% at 1 year, 90.7% at 5 years, and 82.5% at 10 years. CONCLUSIONS: Although longer follow-up of patients who undergo Ross operation is needed, the present series confirms that the autograft procedure is a valid option to treat aortic valve disease in selected patients. The nonreinforced full root technique and preoperative aortic regurgitation are predictors for autograft failure and warrant further consideration. Clinical Trial Registration-URL: http://www.clinicaltrials.gov. Unique identifier: NCT00708409.

Autograft reinforcement to preserve autograft function after the ross procedure: a report from the german-dutch ross registry.

BACKGROUND: Autograft reinforcement interventions (R) during the Ross procedure are intended to preserve autograft function and improve durability. The aim of this study is to evaluate this hypothesis. METHODS AND RESULTS: 1335 adult patients (mean age:43.5+/-12.0 years) underwent a Ross procedure (subcoronary, SC, n=637; root replacement, Root, n=698). 592 patients received R of the annulus, sinotubular junction, or both. Regular clinical and echocardiographic follow-up was performed (mean:6.09+/-3.97, range:0.01 to 19.2 years). Longitudinal assessment of autograft function with time was performed using multilevel modeling techniques. The Root without R (Root-R) group was associated with a 6x increased reoperation rate compared to Root with R (Root+R), SC with R (SC+R), and without R (SC-R; 12.9% versus 2.3% versus 2.5%.versus 2.6%, respectively; P<0.001). SC and Root groups had similar rate of aortic regurgitation (AR) development over time. Root+R patients had no progression of AR, whereas Root-R had 6 times higher AR development compared to Root+R. In SC, R had no remarkable effect on the annual AR progression. The SC technique was associated with lower rates of autograft dilatation at all levels of the aortic root compared to the Root techniques. R did not influence autograft dilatation rates in the Root group. CONCLUSIONS: For the time period of the study surgical autograft stabilization techniques preserve autograft function and result in significantly lower reoperation rates. The nonreinforced Root was associated with significant adverse outcome. Therefore, surgical stabilization of the autograft is advisable to preserve long-term autograft function, especially in the Root Ross procedure.

Impact of placing a conduit from the right ventricle to the pulmonary arteries as the first stage of further palliation in the Norwood sequence for hypoplasia of the left heart.

OBJECTIVE: We describe the experience from a single institution with the Norwood sequence of palliation for hypoplasia of the left heart, emphasizing complications related to placement of a conduit from the right ventricle to the pulmonary arteries and their management. METHODS: Between November, 2002 and January, 2006, we palliated 32 patients with hypoplastic left heart syndrome or its variants by placing a conduit from the right ventricle to the pulmonary arteries. We reviewed retrospectively the charts and angiograms from these patients. RESULTS: Hospital survival after construction of the conduit was 90.6%. There were 3 interstage deaths, of which 2 were likely due severe obstruction of the conduit. Stents were implanted into the proximal or medial portions of the conduits of 3 patients. Early revision of the distal anastomosis, and shortening the conduit, was performed early postoperatively in 2 patients. So far, 24 out 26 survivors of the first stage underwent a bi-directional cavopulmonary anastomosis after a mean interval of 4.3 plus or minus 1.4 months. Of these, 3 required a semi-urgent second stage of palliation because of worsening cyanosis, with one patient dying after the second stage. Completion of the Fontan circulation by insertion of an extracardiac conduit was performed in 8 patients at the mean age of 19.8 plus or minus 2.2 months. We were able to achieve biventricular repair in 1 patient, with aortic atresia, hypoplastic arch and ventricular septal defect, 4.3 months after the initial palliative procedure. Overall survival of the whole cohort of 32 patients was 78.9%, plus or minus 7.8%, at 5 months, and 74.3%, plus or minus 8.6%, up to 25 months. CONCLUSIONS: The introduction of the conduit placed from the right ventricle to the pulmonary arteries has led to an improved outcome in the complex entity of hypoplastic left heart syndrome and its variants. Stenosis of the conduit, nonetheless, may account for significant interstage morbidity, and often requires intervention or early installation of the second stage of palliation.