Transcatheter closure of a non-restrictive patent ductus arteriosus with an Amplatzer muscular ventricular septal defect occluder.
We report on successful occlusion of a large unrestrictive PDA in an adult patient with pulmonary hypertension.
Biomedical subjects
Publications and source records attributed to Andreas Eicken.
We report on successful occlusion of a large unrestrictive PDA in an adult patient with pulmonary hypertension.
We report on successful treatment of persistent late postoperative chylothorax with coil occlusion of significant aortopulmonary collaterals in a young infant with functionally univentricular heart after bidirectional cavopulmonary connection.
OBJECTIVE: Pulmonary regurgitation is the predominant problem in the long-term follow-up of tetralogy of Fallot (TOF) patients after primary repair. Apart from standard homograft implantation, a percutaneous valve delivery approach has been described recently. A right ventricular outflow tract (RVOT) diameter of greater than 22mm, however, precludes percutaneous valve delivery. We describe a novel technique with a transventricular implantation of a stented bio-prosthesis without cardiopulmonary bypass that allows for implantation of prosthesis with diameters greater than 22mm. METHODS: All patients (9-27 years of age) had undergone total correction of TOF at a mean age of 4.2+/-4.0 years. The RVOT was enlarged at that time with a transannular patch in all but one patient. All patients presented with severe pulmonary regurgitation without any significant RVOT obstruction. Mean MRI pulmonary regurgitation was 53+/-8%. The mean magnetic resonance imaging (MRI) right ventricular end diastolic volume index (RVEDVI) was 143+/-23ml/m(2), with a mean MRI right ventricular ejection fraction (RVEF) of 46+/-9%. In another two patients indication for treatment was based on reduced exercise capacity with patients being in NYHA Class III. After repeat sternotomy, a porcine valve mounted inside a self-expandable stent, covered with No-React treated porcine pericardium (Shelhigh, Model NR-4000MIS), was introduced just beneath the RVOT without use of cardiopulmonary bypass. External sutures were placed at the proximal and distal site of the valve to ensure fixation. RESULTS: The implantations were uneventful, with the patients hemodynamically stable throughout the procedure. One patient with severely dilated RVOT (up to 31mm) exhibited paravalvular leakage and the valve was replaced by a homograft after 2 days. At 6-12 month follow-up the remaining five patients exhibited no more than mild pulmonary regurgitation. The mean MRI RVEDVI was 94+/-18ml/m(2), with a mean MRI RVEF of 58+/-27%. CONCLUSIONS: Cardiopulmonary bypass for repeat RVOT interventions can be avoided in selected patients with this newly available device. In combination with a wide range of prosthesis sizes it offers yet another important treatment option.
Erythrocytosis is an adaptive response to improve oxygen transport in cyanotic congenital heart disease (CCHD). However, at highly increased hematocrit levels patients may experience hyperviscosity symptoms. Iron deficiency in CCHD patients is often overlooked due to elevated hemoglobin concentrations. A 29-year-old male with CCHD was readmitted to our outpatient clinic. Red blood cells (11.65*10(12)/L), hemoglobin (25.7 g/dL), and hematocrit (80%) were extremely elevated. Measurements of iron supply showed a constellation typical for iron deficiency with low ferritin (13.2 microg/L), and high sTfR (20 mg/L). We present a case of extremely high red blood cell counts with concomitant iron deficiency. For appropriate management and to avoid misinterpretation of the iron status, ferritin and sTfR should always accomplish laboratory examination of CCHD patients.
BACKGROUND: Doppler myocardial echocardiography (DME) may be an excellent additional means of assessing determinants of systolic and diastolic ventricular function in patients with tricuspid atresia (TA) after total cavopulmonary connection (TCPC). METHODS AND RESULTS: Thirty-three patients with TA and normal systolic shortening/ejection fraction (M-mode) after TCPC were studied by DME at a median age of 7.6 years (range 1.5-17 years). These results were compared to 16 age matched normal controls. Median time under a cavopulmonary shunt was 5.2 years (range 0.6-13.3 years). Isovolumic acceleration and isovolumic velocity did not differ significantly. All other systolic (S-wave acceleration-velocity and S-wave duration) and diastolic DME indices (E-deceleration-velocity, A-velocity and E/A ratio) were significantly lower in TCPC patients in comparison to normals (p<0.0001). Furthermore, isovolumic relaxation time (IRT) was significantly prolonged in the patient group (p<0.0001). Even though there was a significant correlation between the time of volume unloading and left ventricular end-diastolic diameter, exclusion of all patients with short period of volume unloading and relatively large systemic ventricles did not alter the results. CONCLUSIONS: Patients with TA after TCPC and normal shortening and ejection fraction have normal isovolumic acceleration. Load dependent DME indices of systolic and diastolic function, however, were significantly reduced. These findings suggest that the described DME indices mirror the "normal" hemodynamics after TCPC in patients with TA. It needs to be assessed, whether this is an index of adverse prognosis in long-term surveillance of these patients.
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BACKGROUND: Implantable cardioverter defibrillators (ICD) proved to be effective in the prevention of sudden cardiac death in adults. In children, the experience of ICD therapy is limited. This retrospective study was undertaken to review our experience with ICD implantation in children with special consideration of psychosocial impact of this therapy. METHODS AND RESULTS: Sixteen children (f:5, m:11, median age 12.2 years, range 4-15.9 years) received an ICD. Eleven patients had survived sudden cardiac death with documented ventricular fibrillation (VF) and five patients had sustained ventricular tachycardia (VT) with hemodynamic significance. The underlying heart disease was congenital in 5, hypertrophic cardiomyopathy in 2, myocarditis in 2 and primary electrical in 7 patients. All leads were implanted transvenously. Mean follow up was 43.1 months (range 1-105 months). All patients are alive. In 7 patients, a total of 387 sustained VT episodes were detected by the ICD. At follow-up, 10 inappropriate shocks were delivered in four patients. One early and six late lead revisions were done in seven patients. 12/16 (75%) patients had concomitant antiarrhythmic drug therapy. About half of the adolescents showed signs of depression and/or anxiety. CONCLUSION: ICD therapy via transvenous access for prevention of sudden cardiac death is feasible and effective even in small children. However, the occurrence of lead complications is significant. Since about half of the adolescents showed signs of depression and/or anxiety, professional psychological surveillance should be considered in these patients.
AIMS: The current study was designed to assess midterm results of stent implantation into the aorta for native and recurrent coarctation (CoA) in children and young adults. METHODS AND RESULTS: Forty-three patients (native CoA, 8; female, 12) were treated with stent implantation at a median age of 16.8 years (range 7.9-44.8 years). Only stents dilatable to an adult size aorta were implanted. All but two patients with functionally univentricular hearts had arterial hypertension. Exercise tests, 24-h blood pressure, clinical examination, echocardiography, and elective catheterization were used to assess follow-up. The narrowed segment was widened significantly from a median of 8 to 12.4 mm (P < 0.0005). The peak-to-peak gradient between the ascending and the descending aorta was lowered significantly from a median of 22 mmHg to 1 mmHg (P < 0.0005). No major complications occurred. The systolic blood pressure at the right arm was lowered significantly (P < 0.0005) from 144 mmHg before stent implantation to 128 mmHg at the last visit. At a median follow-up of 30 months (3-72 months), 68% of all patients were classified to be normotensive. CONCLUSION: Stent implantation for selected patients with recurrent and native CoA is safe and may effectively reduce the blood pressure gradient across the CoA site. We suggest using only stents dilatable to an adult size aorta. However, arterial hypertension persists in a significant number of the patients. Impaired elastic properties of the aorta may be the cause for this finding.
Early shunt stenosis may occur after a Norwood-Sano operation. We report our experience with three patients who were treated successfully by stent implantation into the Sano shunt. Hence, a surgical shunt revision could be avoided.
BACKGROUND: Despite primary success, 9% of patients develop aortic (pseudo)aneurysms after surgical repair of aortic coarctation at or near the site of repair late after operation. CASE STUDY: A chest X-ray in a 32-year-old asymptomatic man, 31 years after coarctation repair, depicted a ballooning of the distal aortic arch. A multislice CT confirmed an aneurysm. The aneurysm was resected using selective head perfusion. CONCLUSION: At follow-up of patients after coarctation repair, one should anticipate aneurysm formation, even decades after successful surgery and even if clinically silent. Therefore, the chest X-ray should be part of the follow-up examination. Deep hypothermic circulatory arrest can be avoided in selected patients.
Patients with right ventricular outflow tract reconstruction often require redo operations with time. Unique surgical problems exist in this group of patients. We report an innovative method of implantation of a pulmonary valve without the use of cardiopulmonary bypass.
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Implantable cardioverter defibrillator therapy is feasible and effective even in children. Unique surgical problems exist in very young patients. We report the subcutaneous placement of a defibrillation lead together with an abdominal active-can device.
BACKGROUND: The quest for an alternative to homografts for reconstruction of the right ventricular outflow tract is ongoing. The Shelhigh No-React (NR-4000PA series) treated porcine pulmonic valve conduit (SPVC) was developed as a potential alternative. METHODS: During a 12-month period from May 2004 to May 2005, the SPVC was implanted in 34 patients, of whom 62% were younger than 1 year. Median age at operation was 7 months (range, 5 days to 12 years). Thirteen SPCV conduits size 10, 11 size 12, 8 size 14, and 2 size 16 were initially implanted. Since May 2005, however, we have temporarily abandoned its implantation as we were concerned about a number of early failures. RESULTS: Until November 2005, 1 early and 1 late death have occurred. Both were not conduit related. Fifteen conduits were replaced in 13 patients. Of these, 10 were size 10, 3 size 12, 2 size 14, and none size 16. Mean time to replacement of the SPVC was 313 +/- 116 days. A pseudointimal peel formation and chronic inflammation with foreign-body reaction was found in all explanted conduits at all levels. The maximum of the inflammatory reaction occurred at the valvular level around the porcine tissues, with shrinkage of the valve and hemodynamic compromise. At valvular level, small punctuate calcifications were observed in 2 cases. In 6 patients an acute inflammatory component was observed. At late follow-up (mean follow-up 366 +/- 102 days, 34 patient-years), echocardiography showed a mean graft gradient of 39.8 +/- 29.7 mm Hg, with mild to moderate insufficiency in 4 patients. CONCLUSIONS: Although the No-React treated valve largely resists calcification, pseudointimal peel formation was found in all explanted conduits and led to multilevel conduit stenoses. The small-sized SPVC can not be regarded as an ideal conduit for right ventricular outflow tract reconstruction.
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OBJECTIVES: Sudden cardiac death is a leading cause of mortality in patients with congenital cardiac disease after surgical correction and is potentially preventable. The identification of patients at risk is therefore of major interest. We sought to assess the prognostic value of impaired cardiac autonomic nervous activity in patients with congenital cardiac disease. METHODS: Forty-three consecutive patients with congenital cardiac disease were included in this prospective study. Parameters of heart rate turbulence and heart rate variability were calculated from Holter electrocardiograms. In addition, serum brain natriuretic peptide levels were measured. A combined end point of sudden cardiac death or nearly missed sudden cardiac death was used. RESULTS: During a mean follow up of 27 +/- 12.7 months, 5 patients died, and another 2 were successfully resuscitated. On univariate analysis, both brain natriuretic peptide levels and parameters of heart rate variability and heart rate turbulence were associated with impaired prognosis. On multivariate analysis, pathologic heart rate turbulence was found to be the strongest independent risk stratifier (hazard ratio, 61.5; P < .001). CONCLUSIONS: Impaired cardiac autonomic nervous activity is associated with an increased risk of sudden cardiac death in congenital cardiac disease. Our results suggest that heart rate turbulence might be superior to established markers of cardiac autonomic dysfunction, such as heart rate variability. The combined use of heart rate turbulence, heart rate variability, and markers of neurohormonal activation, such as brain natriuretic peptide, might further improve the prognostic value.
Fenestration is still widely used in right heart bypass operations. Our study was conducted to assess its need in the most recent modification, the completion of a total cavopulmonary connection with an extracardiac tube. The extracardiac approach was introduced at our institution in January, 1999. Since June of 2000, no patient had a fenestration. If more than 1 risk factor amongst ventricular function being more than moderately impaired, atrioventricular valvar regurgitation more than moderate, mean pulmonary arterial pressure more than 15 millimetres of mercury, mean atrial pressure higher than 12 millimetres of mercury, pulmonary arterial distortion, or other than sinus rhythm was present preoperatively, the patient was considered a "high risk" candidate. Postoperatively elevated pulmonary arterial pressure higher than 16 millimetres of mercury, prolonged effusions and requirement for drainage longer than 7 days, and death were considered endpoints in the statistical analysis. Our study group included 84 patients who underwent surgery up to August, 2004. A previous bidirectional cavopulmonary anastomosis had been accomplished in 73 patients at a mean age of 27.01 plus or minus 32.60 months, with a median of 11.5 months, without creating an additional source of flow of blood to the lungs. At the time of the total cavopulmonary connection, the mean age was 66.4 plus or minus 60.1 months, with a median of 37.1 months, and a range from 17.3 to 251.2 months, with 50 patients being younger than 48 months. We deemed 16 patients to be at "high risk". These patients were older at the time of bidirectional cavopulmonary anstomosis (p smaller than 0.016), at the time of completion (p smaller than 0.019), and also differed in size at time of completion (p smaller than 0.020). They required a longer time on cardiopulmonary bypass (p smaller than 0.015), and reached higher early postoperative pulmonary arterial pressures after completion (p smaller than 0.025). There were no differences between groups of patients having up to 1 or more risk factors in regard to need for intubation (p smaller than 0.511), pulmonary arterial pressures after extubation (p smaller than 0.817), and duration of chest drainage (p smaller than 0.650). Three patients died, one in the group deemed at high risk. There was no death in the last 38 patients. We conclude that a total cavopulmonary connection with an extracardiac tube can be performed without fenestration, even if the patients are deemed to be at increased risk. Early staging of patients with functionally univentricular physiology might be one of the keys for these findings.
Mechanical ventilation support and diaphragm pacing has improved the prognosis of patients with idiopathic congenital central hypoventilation syndrome (CCHS; Ondine's curse). However, severe bradyarrhythmias may occur. This report is about a patient who was supplied with a bilateral diaphragm pacing system at early childhood. At the age of 17 years, he experienced multiple syncopes due to sinus nodal arrest, which was successfully treated by the implantation of a dual chamber pacemaker.