Heart transplantation surgery in children and young adults with congenital heart disease.

BACKGROUND: Pediatric cardiac transplantation remains a surgical challenge as a variety of cardiac and vessel malformation are present in patients with congenital heart disease (CHD). Despite limited availability and acceptability of donor hearts, the number of heart transplantations remains on a stable level with improved survival and quality of life. OBSERVATION: As treatment options for CHD continue to improve and the chances of survival increase, more adult CHD patients are listed for transplantation. This review focuses on the clinical challenges and modified techniques of pediatric heart transplantations. CONCLUSION: Not only knowledge of the exact anatomy, but above all careful planning, interdisciplinary cooperation and surgical experience are prerequisites for surgical success.

Postoperative Complications and Long-Term Results after Primary Cardiac Sarcoma Resection.

BACKGROUND: Primary malignant cardiac tumors rarely occur in clinical care. Little is known about the impact of a parameter on postoperative survival. METHODS: From May 1991 to May 2014, a total of 24 patients underwent surgical treatment of a primary cardiac sarcoma in our center. We analyzed our clinical database retrospectively for information on patient characteristics and treatment data. The follow-up could be completed to 91.7%. RESULTS: Angiosarcoma and non-otherwise-specified sarcoma were the most common tumor entities. R0 resection was achieved in most cases. Postoperative mortality within the first 30 days was 20.8% (n = 5). In four of these five cases, postoperative low-output cardiac failure was the leading cause of death. The cumulative survival rate was 77.3% after 30 days, 68.2% after 3 months, 50.0% after 6 months, 45.0% after 12 months, and 18.0% after 24 months. The mean survival time in the whole group was 47.0 months. A low tumor differentiation was associated with low mean survival, but this was not statistically significant. Mean survival of sarcoma was higher after R0 resection. There was no significant rate of survival difference regarding the adjuvant therapy concept. CONCLUSION: Extended surgery alone or in combination with chemo- and/or radiotherapy may be successful in certain cases and may offer a satisfactory quality of life. The establishment of a multicenter heart tumor register in Germany is necessary to increase the number of cases in studies, get more remarkable study results, and standardize the diagnosis and therapy.

Differentiation of malignant and benign cardiac tumors using 18F-FDG PET/CT.

UNLABELLED: In the diagnostic algorithm of cardiac tumors, the noninvasive determination of malignancy and metastatic spread is of major interest to stratify patients and to select and monitor therapies. In the diagnostic work-up, morphologic imaging modalities such as echocardiography or magnetic resonance tomography offer information on, for example, size, invasiveness, and vascularization. However, preoperative assessment of malignancy may be unsatisfactory. The aim of this study was to evaluate the diagnostic value of (18)F-FDG PET and the incremental diagnostic value of an optimized CT score in this clinical scenario. METHODS: (18)F-FDG PET/CT scans (whole-body imaging with low-dose CT) of 24 consecutive patients with newly diagnosed cardiac tumors were analyzed (11 men, 13 women; mean age ± SD, 59 ± 13 y). The maximum standardized uptake values (SUV(max)) of the tumors were measured. Patients were divided into 2 groups: benign cardiac tumors (n = 7) and malignant cardiac tumors (n = 17) (cardiac primaries [n = 8] and metastases [n = 9]). SUV(max) was compared between the 2 groups. Results were compared with contrast-enhanced CT, using standardized criteria of malignancy. Histology served as ground truth. RESULTS: Mean SUV(max) was 2.8 ± 0.6 in benign cardiac tumors and significantly higher both in malignant primary and in secondary cardiac tumors (8.0 ± 2.1 and 10.8 ± 4.9, P < 0.01). Malignancy was determined with a sensitivity of 100% and specificity of 86% (accuracy, 96%), after a cutoff with high sensitivity (SUV(max) of 3.5) was chosen to avoid false-negatives. Morphologic imaging reached a sensitivity of 82% and a specificity of 86% (accuracy, 83%). Both false-positive and false-negative decisions in morphology could be corrected in all but 1 case using a metabolic threshold with an SUV(max) of 3.5. In addition, extracardiac tumor manifestations were detected in 4 patients by whole-body (18)F-FDG PET/CT. CONCLUSION: (18)F-FDG PET/CT can aid the noninvasive preoperative determination of malignancy and may be helpful in detecting metastases of malignant cardiac tumors.

Treatment of chronic ascites due to right-sided heart failure by transjugular intrahepatic portosystemic shunt stent-implantation after orthotopic heart transplant.

Long-term outcome after orthotopic heart transplant is often critical in patients with long-persisting right-sided heart failure and consecutive ascites. Transjugular intrahepatic portosystemic stent shunt is an effective treatment for chronic ascites. However, a case of transjugular intrahepatic portosystemic stent shunt implantation after orthotopic heart transplant and left ventricular assist device bridging has not been previously reported.

PITX2c is expressed in the adult left atrium, and reducing Pitx2c expression promotes atrial fibrillation inducibility and complex changes in gene expression.

BACKGROUND: Intergenic variations on chromosome 4q25, close to the PITX2 transcription factor gene, are associated with atrial fibrillation (AF). We therefore tested whether adult hearts express PITX2 and whether variation in expression affects cardiac function. METHODS AND RESULTS: mRNA for PITX2 isoform c was expressed in left atria of human and mouse, with levels in right atrium and left and right ventricles being 100-fold lower. In mice heterozygous for Pitx2c (Pitx2c(+/-)), left atrial Pitx2c expression was 60% of wild-type and cardiac morphology and function were not altered, except for slightly elevated pulmonary flow velocity. Isolated Pitx2c(+/-) hearts were susceptible to AF during programmed stimulation. At short paced cycle lengths, atrial action potential durations were shorter in Pitx2c(+/-) than in wild-type. Perfusion with the ß-receptor agonist orciprenaline abolished inducibility of AF and reduced the effect on action potential duration. Spontaneous heart rates, atrial conduction velocities, and activation patterns were not affected in Pitx2c(+/-) hearts, suggesting that action potential duration shortening caused wave length reduction and inducibility of AF. Expression array analyses comparing Pitx2c(+/-) with wild-type, for left atrial and right atrial tissue separately, identified genes related to calcium ion binding, gap and tight junctions, ion channels, and melanogenesis as being affected by the reduced expression of Pitx2c. CONCLUSIONS: These findings demonstrate a physiological role for PITX2 in the adult heart and support the hypothesis that dysregulation of PITX2 expression can be responsible for susceptibility to AF.

Do the age of patients with tetralogy of fallot at the time of surgery and the applied surgical technique influence the reoperation rate? a single-center experience.

BACKGROUND AND PURPOSE: Primary repair of tetralogy of Fallot (TOF) has been favored in many centers for years now and results and advantages of this management seem to verify this procedure. The authors wanted to know, if the age at the time of surgery and the surgical techniques had an influence on the long-term results. PATIENTS AND METHODS: Between 1992 and 2003, 124 patients underwent complete repair of TOF at the University Hospital Münster, Germany. Patients were subdivided into two groups based on their age (< 1 year and > 1 year of age). Patients in whom a transannular patch (TAP) was used were compared with those without (NTAP), or in whom a conduit was used. RESULTS: Overall mortality was 8%, with an average age of death of 9.53 years (range 0.06-19.77 years). The patients' age at the time of surgery affected their survival as only two cases of death were reported among the group of children < 1 year of age (3.2%) whereas eight patients were older (12.9%; p = 0.0483). Six patients died within the first 30 days post surgery. Reoperation had to be performed in 21 cases, 13 (61.9%) of these patients were < 1 year of age at the time of surgery, eight were older (38.1%). A TAP, NTAP or conduit treatment did not show significant differences in long-term survival or freedom from reoperation. CONCLUSION: Early repair of TOF within the 1st year of life can be recommended, because mortality is lower than in patients treated at a higher age. There seems no significant difference in the reintervention rate between patients treated within the 1st year of life or later.

Influence of inflow cannula length in axial-flow pumps on neurologic adverse event rate: results from a multi-center analysis.

BACKGROUND: The application of axial-flow pumps in patients with end-stage heart failure reveals a significantly reduced infectious complication rate as compared with rates observed with pulsatile devices. The remaining adverse event rate relates mainly to thromboembolic complications with neurologic consequences. We investigated the dependence of the neurologic adverse event rate on the length of the inflow cannula. METHODS: A total of 216 consecutive patients with an axial-flow pump (INCOR; Berlin Heart GmbH, Berlin, Germany) were included in a retrospective multi-center analysis. In 138 patients, a short inflow cannula (24-mm tip length into the left ventricle), and in 78 patients a long inflow cannula (tip length 34 mm) was applied. RESULTS: Patients with a long inflow cannula (LC) demonstrated a better survival rate than those with a short inflow cannula (SC) at the end of the observation period (LC, 63.4%; SC, 52.9%; p = 0.05). The thromboembolic adverse event rate was also significantly lower. Only 3 of the 78 patients (3.8%) with an LC had a thromboembolic adverse event (thromboembolic events per patient-year = 0.11) as compared with 32 (23.2%) of SC patients (thromboembolic events per patient-year = 0.50, p < 0.001). CONCLUSIONS: Patients with a long inflow cannula had a better survival rate and a lower incidence of cerebrovascular adverse events than patients with a short inflow cannula.

Explantation of implantable defibrillator leads using open heart surgery or percutaneous techniques.

BACKGROUND: To remove failing or infected implantable cardioverter defibrillator leads, percutaneous techniques and open heart surgery are two common approaches. However, well-defined indications for either technique are not available. We summarize our experience with implantable cardioverter defibrillator system explantation using open heart surgery and percutaneous lead removal. METHODS: A total of 1,391 transvenously introduced implantable cardioverter defibrillator systems were implanted during the analyzed time interval from January 1995 to June 2005 in our institution. In 21 patients (1.5%), open heart surgery for implantable cardioverter defibrillator lead and generator explantation was applied (group A), and in 53 patients (3.8%), a percutaneous lead removal was possible (group B). The log-rank test was used to calculate differences in survival between both patient groups, and the Student's t test was applied for differences in nonlethal complications. RESULTS: The 30-day, 6-month, 12-month, and 5-year survival rates were 91%, 91%, 81%, and 71%, respectively, for group A patients, and 100%, 100%, 94%, and 78%, respectively, for group B patients, which was not statistically different (p = 0.11). After open heart surgery, survival was comparable for cases with lead removal because of lead infection and those with lead malfunction (p = 0.28); however, patients with open heart surgery had a longer hospital stay (p = 0.03). Student's t test revealed no statistical difference in nonlethal complications between both patient groups (p = 0.37). CONCLUSIONS: As open heart surgery yielded similar results with regard to survival and complications, implantable cardioverter defibrillator lead removal using extracorporeal circulation may be well justified as a last therapeutic option, eg, in case of large bacterial vegetations.

Cor triatriatum: short review of the literature upon ten new cases.

Cor triatriatum is defined as a membrane within the left atrium, which might lead to restricted pulmonary venous return. Diagnosis is usually achieved by echocardiography, therapy of choice is excision of the membrane. Upon ten new cases, the association with other congenital heart diseases (CHDs), clinical symptoms and the surgical approach are discussed. Eight of ten patients were children, six of them aged<1 year. Additional CHDs included atrial and ventricular septal defects, partial anomalous pulmonary venous return and complex CHD. Surgery was performed in all cases. Prognosis is related to associated CHD.

Prevalence and prognostic impact of comorbidities in patients with severe aortic valve stenosis.

In patients with severe aortic valve stenosis (valve area 41.8 ml/min/1.73 m(2) (event-free survival rate of 38% vs 93%, p = 0.004). Thus, in patients with severe AS, comorbidities are frequent, and particularly kidney disease significantly impacts longterm outcome.

[Surgical therapy of end-stage heart failure. State of the art 2006]. / Chirurgische Therapie der Herzinsuffizienz : State of the Art 2006.

Cardiac transplantation still remains the gold standard despite recent success in organ-preserving therapy. However, organ shortage forces to process alternative therapies. Cardiac resynchronization therapy and cardiac contractility modulation are new and promising therapies, which are able either to delay or even prevent the need for cardiac transplantation. High-risk cardiac revascularization and valve replacement is another important therapy in especially evaluated patients. With newer organ-protective procedures and novel treatment options like the off-pump bypass surgery, end-stage heart failure could be treated successfully. The volume reduction surgery, the so-called Batista procedure, has its indication only in special selected patients with dilated cardiomyopathy. A modified procedure, the surgical ventricular restoration (SVR) therapy, however, has a definitive potential and is evaluated in a multicenter trial (RESTORE). Mechanical cardiac assist devices have still a high impact in the therapy of acute or chronic end-stage heart failure. By means of smaller devices with axial rotary blades, the high rates of thromboembolic events, infections and mechanical device complications were significantly reduced. Survival to transplant with mechanical assist device support is nowadays around 80%. New centrifugal pumps are tested in clinical trials right now. Passive cardiac support devices and the total artificial heart are limited to special indications. The organ shortage could be overcome with the xenotransplantation. However, the problem of hyperacute rejection and the risk of transfection of animal diseases limit the clinical use considerably. The cell therapy has probably the highest potential for the future. Still unsolved are problems with the potential trigger of arrhythmias and ethical preconceptions regarding embryonic stem cells. In summary, cardiac transplantation still remains the gold standard in the therapy of end-stage heart failure with 10-year survival rates of 50%. With increasing donor shortage a potential combination therapy of organ-preserving cardiac surgery and cell transplantation might be the future for the 21st century.

Fatal leaflet escape in an Edwards TEKNA aortic prosthesis.

The case is reported of a 26-year-old male patient who died eight years after the replacement of an aortic valve with a bileaflet mechanical valve (TEKNA; Edwards, USA). Following prosthesis implantation, the patient had been in a good state of health, and his death occurred unexpectedly. Forensic autopsy revealed a leaflet escape, with two fragments of the leaflet being found bilaterally in the common iliac arteries. Death occurred due to an acute cardiac insufficiency. Immunohistochemical investigations revealed fresh myocardial fiber necroses. Stereomicroscopic and scanning electron microscopic investigations demonstrated surface erosions of the leaflet. Although the valve was withdrawn from the market in June 2000, it had previously been implanted in over 18,000 patients. Thus, from a clinical viewpoint, the question of using a prophylactic replacement in affected patients must be discussed.

Temporary right heart support with percutaneous jugular access.

PURPOSE: Temporary right heart bypass has shown to improve hemodynamic stability and safety in beating heart revascularization. We sought to evaluate feasibility and safety of a right ventricular assist device percutaneously implanted in the right jugular vein. DESCRIPTION: The A-Med jugular coaxial cannula (A-Med Systems Inc, West Sacramento, CA) is designed for percutaneous implantation. Blood from the right heart is drained through the outer tube of this two-cannula device to a microcentrifugal pump and returned into the pulmonary artery through the inner tube. EVALUATION: In 10 patients scheduled for elective coronary bypass grafting without cardiopulmonary bypass, a total of 27 coronary anastomoses were performed with right heart support. Arterial pressure was significantly higher with right heart support when the heart was dislocated to access posterior and posterolateral anastomosis sites. Implantation through the right internal jugular vein was feasible without complications in all patients and facilitated the procedure. CONCLUSIONS: This initial study suggests safety and feasibility of temporary right heart support using percutaneous jugular access for posterior and posterolateral coronary bypass grafting.

Comparison of 3D segmented gradient-echo and steady-state free precession coronary MRI sequences in patients with coronary artery disease.

OBJECTIVE: Our objective was to compare two state-of-the-art coronary MRI (CMRI) sequences with regard to image quality and diagnostic accuracy for the detection of coronary artery disease (CAD). SUBJECTS AND METHODS: Twenty patients with known CAD were examined with a navigator-gated and corrected free-breathing 3D segmented gradient-echo (turbo field-echo) CMRI sequence and a steady-state free precession sequence (balanced turbo field-echo). CMRI was performed in a transverse plane for the left coronary artery and a double-oblique plane for the right coronary artery system. Subjective image quality (1- to 4-point scale, with 1 indicating excellent quality) and objective image quality parameters were independently determined for both sequences. Sensitivity, specificity, and accuracy for the detection of significant (> or = 50% diameter) coronary artery stenoses were determined as defined in invasive catheter X-ray coronary angiography. RESULTS: Subjective image quality was superior for the balanced turbo field-echo approach (1.8 +/- 0.9 vs 2.3 +/- 1.0 for turbo field-echo; p < 0.001). Vessel sharpness, signal-to-noise ratio, and contrast-to-noise ratio were all superior for the balanced turbo field-echo approach (p < 0.01 for signal-to-noise ratio and contrast-to-noise ratio). Of the 103 segments, 18% of turbo field-echo segments and 9% of balanced turbo field-echo segments had to be excluded from disease evaluation because of insufficient image quality. Sensitivity, specificity, and accuracy for the detection of significant coronary artery stenoses in the evaluated segments were 92%, 67%, 85%, respectively, for turbo field-echo and 82%, 82%, 81%, respectively, for balanced turbo field-echo. CONCLUSION: Balanced turbo field-echo offers improved image quality with significantly fewer nondiagnostic segments when compared with turbo field-echo. For the detection of CAD, both sequences showed comparable accuracy for the visualized segments.