Extracorporeal life support in pediatric patients with congenital heart diseases: outcome of a single centre.

AIM: In pediatric patients with congenital heart disease low cardiac output (LCO) is the principal complication after corrective heart surgery. In LCO refractory to all therapeutic options, mechanical circulatory support is the final method to keep these patients alive. In this present study the authors reviewed the outcome of pediatric patients who required mechanical circulatory support after corrective surgery with extracorporeal membrane oxygenation or ventricle assisted devices (VAD). METHODS: A retrospective single centre consecutive cohort study was carried out in children who required different mechanical circulatory support indicated by postcardiotomy low output syndrome between 1991 and 2004. A total of 20 patients received extracorporeal life support. The indications for surgery were: 12 transposition of great arteries, 1 Bland-White-Garland syndrome, 3 tetralogy of Fallot, 1 hypoplasia of aortic arch, 1 total anomalous pulmonary vein connection, and 2 ventricle septum defect. RESULTS: Mean age was 1.29 years. Mean duration of assist was 8.87 days. Seven patients out of 20 survived, six could be discharged after myocardial recovery from LCO and one could be discharged after successful heart transplantation. The overall mortality in patients with extracorporeal life support was 65%. The causes of death were multiorgan failure and bleeding in one case was a VAD related complication. CONCLUSION: The use of extracorporeal life support (ECLS) shows a high mortality rate. However, ECLS can still help to keep some of those patients alive. Mechanical support devices are the ultimate chance to save time, to increase survival and to bridge the time until heart transplantation.

Heat shock protein 27 modification is increased in the human diabetic failing heart.

Chronic conditions like diabetes mellitus (DM) leading to altered metabolism might cause cardiac dysfunction. Hyperglycemia plays an important role in the pathogenesis of diabetic complications including accumulation of methylglyoxal (MG), a highly reactive alpha-dicarbonyl metabolite of glucose degradation pathways and increased generation of advanced glycation endproducts (AGEs). The aim of this investigation was to study the extent of the MG-modification argpyrimidine in human diabetic heart and in rat cardiomyoblasts grown under hyperglycemic conditions. Left ventricular myocardial samples from explanted hearts of patients with cardiomyopathy with (n=8) or without DM (n=8) as well as nonfailing donor organs (n=6), and rat cardiac myoblasts H9c2 treated with glucose were screened for the MG-modification argpyrimidine. The small heat shock protein 27 (Hsp27) revealed to be the major argpyrimidine containing protein in cardiac tissue. Additionally, the modification of arginine leading to argpyrimidine and the phosphorylation of Hsp27 are increased in the myocardium of patients with DM. In H9c2 cells hyperglycemia leads to a decrease of the Hsp27-expression and an increase in argpyrimidine content and phosphorylation of Hsp27, which was accompanied by the induction of oxidative stress and apoptosis. This study shows an association between diabetes and increased argpyrimidine-modification of myocardial Hsp27, a protein which is involved in apoptosis, oxidative stress, and cytoskeleton stabilization.

Preoperative haemostasis testing does not predict requirement of blood products in cardiac surgery.

OBJECTIVE: Standard haemostasis screening tests are performed to reveal unknown congenital or acquired disturbances of plasma and/or platelet haemostasis. Since their diagnostic efficacy is often low, routinely performed haemostasis testing has been questioned. We investigated whether preoperatively assessed haemostasis testing can be used to predict the requirement of blood products. METHODS: We retrospectively assessed haemostasis parameters including platelet function testing by PFA 100 as well as the numbers of red blood cell (RBC) concentrates, fresh frozen plasmas (FFPs), and platelet concentrates (PCs) that were given peri-operatively and during the first two postoperative days in 2,831 cardiac surgery patients. Logistic regression analyses were used to select those parameters, which could predict blood product requirement. RESULTS: Of our study cohort, 56.5% needed RBCs, 15% FFPs, and 5% PCs. The need for RBCs was associated with significantly altered pre-operative values of most haemostasis parameters. However, by the use of logistic regression analysis fibrinogen was the only haemostasis parameter that was independently associated with the use of RBCs (odds ratio 1.56; 95% CI: 1.27-1.91; P <0.001). The predictive value of other parameters such as age, body weight, haemoglobin, and haematocrit was however much higher in comparison to fibrinogen (odds ratios: 1.92-3.50; P <0.001). It was not possible to develop a score based on haemostasis parameters to accurately identify patients at risk for RBC use. Moreover, we were unable to estimate the need for FFPs and PCs using preoperative haemostasis testing. CONCLUSIONS: Our data demonstrate that preoperatively performed haemostasis testing is not predictive in estimating the need for blood products in cardiac surgery patients.

Cardiac retransplantation in adults: an evidence-based systematic review.

BACKGROUND: It remains a matter of dispute whether cardiac retransplantation should be performed. We aimed to systematically review the available evidence regarding cardiac retransplantation in adults. MATERIAL AND METHODS: In PubMed, we searched for original reports on cardiac retransplantation in adults. The evidence level of individual studies was assessed. RESULTS: Twenty-two studies met our selection criteria. The cumulative incidence was 3% (range: 1% to 15%). The incidence rate was 164/10,000 person-years (range: 145 to 318/10,000 person-years). The main indications for retransplantation were cardiac allograft vasculopathy (55%), acute rejection (19%), and primary graft failure (17%). The early mortality rate was 16% (range: 5% to 38%). Refractory acute rejection and primary graft failure, female donor, shorter transplant interval, initial diagnosis of ischemic cardiomyopathy, need for mechanical circulatory support, a center volume of less than 9 transplantations/year, older recipient age, requirement of pretransplant ventilator and intensive care, and ischemic time were associated with poorer outcomes, while cardiac allograft vasculopathy as the cause of allograft failure, employment, and later transplant period were associated with improved survival. CONCLUSION: This systematic review shows that the results of cardiac retransplantation in adults are inconclusive. Retransplantation for cardiac allograft vasculopathy is associated with satisfactory outcomes.

Cardiac retransplantation: a 15-year single-center clinical experience.

BACKGROUND: Cardiac retransplantation is a controversial therapy because of the shortage of donor hearts. We retrospectively reviewed the short-term and long-term outcomes after cardiac retransplantation. METHODS AND RESULTS: Twenty-eight cases (18 males, 7 females; mean age, 50.3 +/- 13.5 years) underwent cardiac retransplantation: 25 first retransplantations and 3 second retransplantations. The indications for retransplantation were primary graft failure (PGF) in 11 patients (39.3%), intractable acute cardiac rejection (IACR) in 4 patients (14.3%), and coronary allograft vasculopathy (CAV) in 13 patients (46.4%). The patients had been supported as follows: prolonged cardiopulmonary bypass (CPB; n = 3), intra-aortic balloon pumping (IABP; n = 1), intravenous inotropic support (n = 7), extracorporeal membranoxygenator (ECMO; n = 3), ventricular assist device (VAD; n = 4), and no inotropic support (n = 10). There were 8 deaths within 30 days after retransplantation (28.6%). The overall 1-, 5-, 10-, and 15-year survival rates were 46.4%, 40.6%, 32.5%, and 32.5%, respectively. Acute cardiac rejection was the most common cause of death (43.8%). Thirty-day and 1-year survival rates of IACR, PGF, and CAV were 50.0%/0%, 63.6%/45.5%, and 84.6%/68.4%, respectively. CONCLUSIONS: Long-term survival after retransplantation was acceptable for patients with CAV and PGF; however, we must select patients for retransplantation carefully if the indication is IACR, because of the poor outcome.

[A telemetrically-guided program for weight reduction in overweight subjects (the SMART study)]. / Telemedizinisch betreutes Programm zur Gewichtsreduktion bei Ubergewichtigen (SMART-Studie).

BACKGROUND AND OBJECTIVE: Compliance with weight reducing programs can be improved by intensive care and control. We tested a telemetrically-guided weight reduction program in overweight and obese persons. PATIENTS AND METHODS: 200 outpatients (62 males) with a mean body mass index of 34 kg/m (2) and a mean age of 47 years participated in a prospective study for one year. During the first six months, telemetrical support (weight-transmission via Bluetooth (short range)-technology, 20-minutes telephone consultation with a nutritionist) was given weekly. After six months, participants were randomly assigned either to a group with further telemonitoring support (telemetric group) or to a group without contact to our clinic (control group). At baseline, and after six and twelve months, body weight, body composition (bioelectrical impedance analysis), and parameters of the metabolic syndrome were assessed at our clinic. RESULTS: 16 participants terminated the study prematurely during the first 6 months and 19 participants (10 from the telemetric group and 9 from the control group) during the second 6 months. According to the intention-to-treat principle, mean weight loss was 6.7 kg (p < 0,001), mean loss of body fat was 5.1 kg (p < 0,001), and mean loss of fat-free mass was 1.6 kg (p < 0,001) within the first six months. Moreover, metabolic and cardiovascular risk markers such as waist circumference, blood pressure, serum triglycerides and blood glucose declined significantly (p < 0,001). Prevalence of the metabolic syndrome fell from 49.5% to 42.0 % (p < 0,05). During the second six months body fat content, waist circumference, and blood glucose increased again in the control group but not in the telemetric group (p < 0,05-0,001). CONCLUSION: The telemetrically-guided weight loss program was a more efficacious measure than the less intensive support without telemonitoring.

Prolapsing left atrial myxoma: preoperative diagnosis using a multimodal imaging approach with magnetic resonance imaging and real-time three-dimensional echocardiography.

Real-time three-dimensional echocardiography (RT3DE) is a new promising technique for the evaluation of intracardiac masses. We present the diagnostic work-up using a multimodal-imaging approach in a 74-year-old patient with a prolapsing tumour in the left atrium suggestive of a myxoma, causing severe congestive heart failure attributable to dynamic left ventricular inflow obstruction, and mimicking severe mitral valve stenosis. Real-time three-dimensional echocardiography allowed to accurately image the entire volume of the myxoma, and to analyse the dynamic left ventricular inflow obstruction. The size of the lobulated mass as assessed by RT3DE was 65 x 25 x 22 mm. The mass was surgically removed, histology was diagnostic for myxoma, and the patient had an uneventful recovery. Real-time three-dimensional echocardiography images the entire volume of a mass allowing for accurate measurements in multiple planes, and allowing for real-time evaluation of obstructive effects on ventricular in- or outflow. This case shows how RT3DE and other non-invasive imaging modalities may be used as complementary techniques for evaluation of intracardiac masses.

[Constrictive pericarditis or restrictive cardiomyopathy? Echocardiographic tissue Doppler analysis]. / Konstriktive Perikarditis oder restriktive Kardiomyopathie? Die echokardiografische Gewebedoppler-Analyse.

BACKGROUND AND OBJECTIVE: Echocardiographic tissue Doppler imaging (TDI) has been proposed for differentiating between constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM). The aim of this retrospective study was to analyse TDI in patients with severe diastolic dysfunction associated with proven constrictive pericarditis or restrictive cardiomyopathy. PATIENTS AND METHODS: The cohort included 34 consecutive patients (24 men. 10 women; mean age 58 12 years), 20 of whom had proven CP (pericardectomy) and 14 had RCM due to amyloidosis (proven by biopsy). Tissue Doppler Imaging was performed online by pulsed-wave TDI at the lateral and septal mitral annulus in the four-chamber view. Filling pressures were measured invasively. RESULTS: 20 of the 34 patients (60%) were in NYHA class III. 19 of the 34 patients were in sinus rhythm (56 %) and 15 had atrial fibrillation. Left ventricular systolic function was normal in all patients with CP. Eight patients with RCM had normal, 3 patients near normal and 3 patients slightly impaired left ventricular contractile function (EF 50-55% and EF 40%, respectively). Respiratory variation of the transmitral inflow was increased in 10 of 12 patients with CP and sinus rhythm. TDI of the early diastolic velocity across the mitral annulus E} was significantly higher in patients with CP than in those with RCM at the septal and at the lateral mitral annulus (13.8 4.2 cm/s vs. 4.0 1.2 cm/s; p < 0.01 and 11.4 3.4 cm/s vs. 4.4 1.7 cm/s; p < 0.01, respectively). A cut-off value 8 cm/s for the diagnosis of RCM showed a sensitivity of 100% and a specificity of 90% (septal) and 80% (lateral), respectively. The E/E}ratio also was significantly different between both groups (septal: 11.2 8.8 vs. 25.1 8.7; p < 0.01). CONCLUSION: TDI of the early diastolic velocity of the mitral annulus E} makes it possible to differentiate between constrictive pericarditis and restrictive cardiomyopathy and should be part of the echocardiographic work-up in clinical routine.

Heart transplantation at the Heart Center North Rhine-Westfalia.

At the Heart Center North Rhine-Westfalia, Germany, more than 1,500 adult cardiac transplantations and more than 100 pediatric cardiac transplantations have been performed since the transplant program was initiated in 1989. Each year, we take care of 800 cardiac transplant recipients and 1,700 patients with heart failure who are in a long-term program for cardiac transplantation or on the Eurotransplant waiting list for cardiac transplantation. We have experience with ventricular assist device implantation as bridge to transplant in more than 300 patients. In total, our clinical know-how with cardiac transplant recipients is based on 10,800 patient-years of observation. In 2006, we transplanted the first donor heart worldwide with the Organ Care System, a technology capable of maintaining human organs in a functioning state ex-vivo. Usually, our transplant recipients have more preoperative risk factors than cardiac transplant recipients at other German heart centers. Our postoperative patient care is individualized with respect to immunosuppression and the performance of myocardial biopsies and coronary angiography. Since 1989, we have performed 31 cardiac retransplantations.

Implantation of CardioWest total artificial heart for irreversible acute myocardial infarction shock.

Patients who develop cardiogenic shock after acute myocardial infarction have a very high mortality rate despite early reperfusion therapy. Hemodynamic stabilization can often only be achieved by implanting a mechanical circulatory support system. When, in cases representing expansive myocardial impairment without any chance of recovery, pharmacological therapy and the use of percutaneous assist devices have failed, the implantation of a total artificial heart is indicated. We report our first experiences with this extensive and innovative method of managing irreversible cardiogenic shock patients. The CardioWest total artificial heart was implanted in 5 patients (male; mean age, 50 years). All patients were in irreversible cardiogenic shock despite maximum dosages of catecholamines, an intra-aortic balloon pump and/or a femoro-femoral bypass. In all patients early reperfusion therapy was performed. After implantation of the Cardio West system, all dysfunctional organ systems rapidly recovered in all patients. Four of 5 patients underwent successful heart transplantation after a mean support time of 156 days. One patient died because of enterocolic necroses caused by an embolic event after termination of dicumarol therapy. In summary, our first experiences justify this extensive management in young patients who would otherwise have died within a few hours.

The MiniACcor: constructive redesign of an implantable total artificial heart, initial laboratory testing and further steps.

The Aachen Total Artificial Heart (ACcor) has been under development at the Helmholtz Institute in Aachen over the last decade. It may serve as a bridge to transplant or as a long-term replacement of the natural heart. Based upon previous in vivo experiments with the ACcor total artificial heart, it was decided to optimize and redesign the pump unit. Smaller dimensions, passive filling and separability into three components were the three main design goals. The new design is called the MiniACcor, which is about 20% smaller than its predecessor, and weighs only 470 grams. Also its external driver/control unit was miniaturized and a new microcontroller was selected. To validate the design, it was extensively tested in laboratory mock loops. The MiniACcor was able to pump between 4.5 and 7 l/min at different pump rates against normal physiological pressures. Several requirements for the future compliance chamber and transcutaneous energy transmission (TET) system were also measured in the same mock loop. Further optimization and validation are being performed in cooperation with the Heart and Diabetes Centre North Rhine-Westphalia.

Papillary fibroelastoma of the ascending aorta presenting with cardiogenic shock.

We describe an uncommon case of a 58-year-old woman who presented with cardiogenic shock. The echocardiography examination revealed a papillary fibroadenoma located in the ascending aorta which was subsequently surgically treated.

Mechanical valve replacement of the systemic atrioventricular valve in children.

In children with systemic atrioventricular valve disease (SAVVD), reconstructive surgery is the primary goal. However, in cases with severely dysplastic valves or failed repair, valve replacement is the only option. The purpose of this study was to assess the early and late outcome following mechanical valve replacement in SAVVD. Between 1989 and 2003, 31 children underwent mechanical valve replacement (19 St. Jude Medical, 12 Carbomedics) in SAVVD (27 mitral, 3 tricuspid in corrected transposition of the great arteries, 1 common in an univentricular heart) at our institution. The ages ranged from 3 months to 15 years (mean 4 years) and body weight varied between 4.2 and 57 kg (mean 13.3 kg). The size of prostheses ranged between 16 and 31 mm (mean 23.9 mm). The main indication for valve replacement was severe insufficiency of left atrioventricular valve (84%); 84% of the patients had had a previous cardiac operation. The overall hospital mortality was 6.5% The mean follow up was 7.7 years (range 2-13 years). Ninety percent of children represent sinus rhythm, 87% are in NYHA class I. All patients were placed on a regimen of Phenprocoumon aiming to maintain the international normalized ratio (INR) between 3.0 and 4.0. Since 1994, INR self-management of oral anticoagulation was performed either by the patient or his or her parents. There was no anticoagulation-related complication in this patient group. Mechanical valve replacement in left atrioventricular valve disease carries a low operative risk across the spectrum of pediatric age despite previous operations in most cases. Long-term survival and quality of life are good in nearly all cases. The rate of anticoagulation-related complications is very low, especially when INR self management is performed.

Acute myocardial infarction in a young adult: a case report and literature review.

Sudden cardiac death related to sports in young patients can have many causes. Hypertrophic cardiomyopathy, congenital coronary abnormalities, and myocarditis make up about half of the causes of sudden cardiac death after sports. Screening for all athletes is important to prevent such episodes. This involves yearly examinations including clinical examinations, stress echocardiograms, echocardiography, and laboratory investigations. Also, behavioral follow up should be addressed, as cocaine administration and doping can both lead to cardiac problems and sudden cardiac death after sports. We present a case of a 17-year-old boy who collapsed after an ice hockey competition as a result of an acute myocardial infarction, which was first represented by ventricular fibrillation. We also review the main causes of sudden cardiac death in such young athletes and the main investigations that have to be performed to reach the proper diagnosis and etiology of the condition.

Unusual case of an 18-year-old heart transplant recipient with endocardial fibroelastosis.

An 18-year-old female Japanese patient who suffered from heart failure and severe pulmonary hypertension was referred to our clinic. The etiology of her cardiomyopathy was unclear. Inhaled prostacyclin therapy resulted in an improvement of pulmonary arterial pressure and allowed us to avoid lung transplantation. Heart transplantation resulted in a complete remission of her respiratory function. Autopsies of the explanted heart revealed massive endomyocardial fibroelastosis. We concluded that endomyocardial fibroelastosis has to be considered a cause of heart failure in young adults with unclear cardiomyopathy.

Heart transplantation in children after mechanical circulatory support: comparison of heart transplantation with ventricular assist devices and elective heart transplantation.

Heart transplantation (HTx) is an ultimate treatment for children with end-stage heart failure or inoperable congenital heart disease. The supply of hearts is inadequate; therefore, different mechanical support systems must be used as bridge to HTx in pediatric patients with postoperative low output. The use of ventricular assist devices (VADs) as bridge to HTx in children is limited because of size differences. The purpose of this study was to evaluate the overall long-term outcome of pediatric circulatory support before pediatric HTx. From 1989 through 2004, 91 pediatric patients underwent isolated HTx. Seven of them required mechanical support before transplantation. We reviewed retrospectively the course of 91 children (mean age 14.7 years) who underwent HTx. Group A consisted of elective HTx patients who were treated as outpatients before HTx, whereas group B was the VAD-HTx bridging group (n=7; mean age 12.31 +/- 2.8 years). Mean duration of VAD support was 108 +/- 98 days (minimum 1 day, maximum 258 days). Overall survival rate after HTx was 80% at 1 year without significant differences between groups. Five of seven patients survived and could be discharged after successful HTx, for a survival rate of 77%. The mean follow-up period was 16.76 +/- 10.6 months. No differences in posttransplantation long-term survival and rejection episodes occurred between patients transplanted with or without VAD. VAD therapy can keep pediatric patients with end-stage heart failure alive until successful HTx, and bridge to HTx is a safe procedure in pediatric patients. After HTx, survival rates of these children are similar to those of patients awaiting elective HTx.

Mechanical valve replacement in congenital heart defects in the era of international normalized ratio self-management.

As the number of patients with congenital heart defects requiring heart valve replacement increases, the need for durable valve substitutes with good hemodynamic performance and a low incidence of complications becomes more apparent. The use of porcine xenografts is burdened with early fibrocalcific degeneration, whereas the use of mechanical heart valves led to an increased number of thromboembolic events, especially when implanted in the right side of the heart. We report on our experiences implanting bileaflet heart valves in congenital heart defects since the introduction of international normalized ratio (INR) self-management. The data of 68 long-term survivors (33 males, 35 females) who underwent mechanical heart valve replacement in congenital heart defect were reviewed. Patient age at the time of valve replacement ranged from 5 months to 61 years (mean 21 years). Underlying diagnoses were tetralogy of Fallot (n=33), morbus Ebstein (n=4), atrioventricular canal (n=13), truncus arteriosus communis (n=5), transposition of the great arteries (n=10), and congenitally corrected transposition of the great arteries (n=3). In all patients, bileaflet valves were implanted (St. Jude Medical n=40, Carbomedics n=28). Anticoagulation was performed using dicumarol (Marcumar) and INR self-management in all cases. The mean follow-up was 72 months (range 6-132 months; 409 patient-years). Valve thrombosis developed in 3 of 68 patients (4.4%, all three had tetralogy of Fallot, mean age 9.8 years) after a mean follow-up of 3.5 years. In two of these three patients, re-pulmonary valve replacement was necessary, whereas the third patient was treated by thrombolysis. From our experience, we conclude that mechanical heart valve replacement is a good therapy option with a low complication rate for patients with congenital heart defects requiring valve replacement, especially when INR self-management is performed.