Transcatheter Closure of Atrial Septal Defects using the GORE(®) Septal Occluder in Children Less Than 10 kg of Body Weight.

The GORE(®) Septal Occluder (GSO) is a well-evaluated device for interventional ASD closure with closure rates comparable to the Amplatzer(®) Septal Occluder (ASO), but there are no published reports of its use in small children weighing less than 10 kg. This may be due to the necessity of a large-sized introducing sheath of at least 10 Fr and therefore the assumed risk of complications in vascular access. The GSO is an alternative option for interventional ASD closure in children weighing less than 10 kg. Fourteen infants and children with a median body weight 8900 g (range 6350-9650 g) underwent successful ASD closure using the GSO. The closure was performed under fluoroscopic and transthoracic echocardiographic guidance. Postprocedure, the vessels passed by the occluder and delivery catheter were examined by duplex sonography. The median ASD diameter was 11 mm (5-17 mm), and the median GSO size was 22.5 mm (15-30 mm), whereas the median ASO left disc size that would have been recommended was 25 mm (17-31 mm). All ASDs were successfully closed. During a median follow-up of 1.57 years (range 0.5-4.2), no complications like erosion, embolization, arrhythmias, or vascular injuries occurred. Although using a 10-Fr introducer sheath, no vascular complications were detected. Our data suggest that the small usable size as well as the soft and flexible design of the device allows successful use of the GSO in young children.

Intra- or extracardiac Fontan operation? A simple strategy when to do what.

INTRODUCTION: The complete Fontan circulation is the definite palliation for many complex congenital cardiac lesions. After bi-directional Glenn anastomosis (BDG), two well-established techniques - intracardiac tunneling and extracardiac prosthesis - are available for completion, although the choice of technique is still a matter of debate. MATERIAL AND METHODS: We retrospectively reviewed the surgical and clinical records of patients with single ventricle physiology, who underwent intracardiac (group I) or extracardiac (group II) Fontan palliation after BDG. RESULTS: Complete data were available in 72 patients. Thirty-eight patients received intracardiac (median weight: 12.6 kg) and 34 patients extracardiac repair (median weight: 15.6 kg). Patients with intracardiac tunneling had longer cardiopulmonary bypass (CBP) time (170 min vs. 104 min; p < 0.001), longer ventilatory (39 h vs. 21 h; p = 0.009) and longer inotropic support (48 h vs. 10 h; p < 0.001). Ventilatory and inotropic support were dependent on CPB (r = 0.69 and r = 0.637) and on aortic cross-clamping (r = 0.785 and r = 0.705 only group I), but not dependent on age, weight or pulmonary artery pressure (PAP). CONCLUSIONS: Both techniques are feasible without perioperative mortality. Normally developed children with good hemodynamics after BDG received an elective extracardiac procedure without fenestration later. Patients with developmental retardation, severe progressive cyanosis, myocardial dysfunction, or moderate to severe atrio-ventricular valve insufficiency are scheduled for an earlier intracardiac baffle repair with routine fenestration, as they are at higher risk. Prolonged CPB and aortic cross-clamping times adversely impact the early postoperative course. Further strategies must be developed to avoid these effects, particularly in the patient group at higher imminent risk.

Continuous regional myocardial blood flow measurement: validation of a near-infrared laser Doppler device in a porcine model.

OBJECTIVE: RMBF measurement is a major concern in various clinical and experimental settings, but no validated device for RMBF is currently available. METHODS: An LVP-triggered laser Doppler to measure RMBF was validated by simultaneous fluorescent MS RMBF in a porcine LAD flow reduction model (n = 10 pigs). The laser probe was positioned on the left ventricle's anterior wall. LAD blood flow reduction was achieved by a shaft-driven occluder positioned proximal to the transit-time flow meter measuring coronary blood flow. RMBF was measured at baseline; after the reduction of LAD blood flow to 70% and 30% of baseline; at 20 and 120 minutes of reperfusion; and, finally, 15 minutes after LAD occlusion. RESULTS: Laser Doppler RMBF (LDU) correlated strongly with MS RMBF under all tested conditions: baseline (epicardial 194.7 ± 41.9, endocardial 130.2 ± 29.2); 70% baseline-flow (epicardial 160.4 ± 27.7, endocardial 112.1 ± 15.1); 30% baseline-flow (epicardial 44.3 ± 5.5, endocardial 32.9 ± 9); 20 minutes reperfusion (epicardial 175.8 ± 33.6, endocardial 126.5 ± 30); 120 minutes reperfusion (epicardial 146.3 ± 31.1, endocardial 107.1 ± 29.7); and complete LAD occlusion (epicardial 10.5 ± 5.8 endocardial 1.4 ± 0.3) (r = 0.986-0.962, p < 0.001). CONCLUSIONS: This new blood pressure waveform-triggered laser Doppler probe is able to measure RMBF at different depths online in the beating heart.

Mid-term results of a modified arterial switch operation using the direct reconstruction technique of the pulmonary artery.

BACKGROUND: There is ongoing discussion as to whether it is beneficial to avoid pulmonary sinus augmentation in the arterial switch operation. We report a single-surgeon series of mid-term results for direct pulmonary artery anastomosis during switch operation for transposition of the great arteries (TGA). METHODS: This retrospective study includes 17 patients with TGA, combined with an atrial septal defect, patent foramen ovale or ventricular septal defect. Patient data was analyzed from hospital charts, including operative reports, post-operative course, and regular follow-up investigations. The protocol included cardiological examination by a single pediatric cardiologist. Echocardiographic examinations were performed immediately after arrival on the intensive unit, before discharge, and then after three, six, and 12 months, followed by yearly intervals. Pulmonary artery stenosis (PAS) was categorized into three groups according to the Doppler-measured pulmonary gradient: grade I (trivial stenosis) = increased pulmonary flow with a gradient below 25 mm Hg; grade II (moderate stenosis) = a gradient ranging from 25 to 49 mm Hg; and grade III (severe stenosis) = a gradient above 50 mm Hg. Follow-up data was available for all patients. The length of follow-up ranged from 1.2 to 9.7 years, median: 7.5 years (mean 6.1 years ± 14 months). RESULTS: During follow-up, 12 patients (70.6%) had no (or only trivial) PAS, five patients (29.4%) had moderate stenosis without progress, and no patient had severe PAS. Cardiac catheterization after arterial switch operation was performed in 11 patients (64.7%) and showed a good correlation with echocardiographic findings. During follow-up there was no reintervention for PAS. CONCLUSIONS: Direct reconstruction of the neo-pulmonary artery is a good option in TGA with antero-posterior position of the great vessels, with very satisfactory mid-term results.

Significance of patient categorization for perioperative management of children with tetralogy of Fallot, with special regard to co-existing malformations.

BACKGROUND: The aim of our study was to facilitate perioperative calculation of potential risk factors on the outcome of corrective surgery for children with tetralogy of Fallot. METHODS: The medical records of 81 (44 female and 37 male) out of a total of 87 patients undergoing complete surgical repair of tetralogy of Fallot between 1988 and 2004 at the Children's Hospital of the Johannes Gutenberg University of Mainz were reviewed. PATIENTS were divided into four categories, depending on the severity of pulmonary stenosis and cyanosis, as well as on the type of pulmonary circulation. RESULTS: Additional malformations did not affect mortality rates, but did directly affect the number of pleural effusions, time of epinephrine administration, duration of surgery, bypass, and ischemia, as well as length of hospitalization and intensive care unit treatment. In contrast to longer periods of extracorporeal circulation and ischemia during surgery, which are directly related not only to more complex anatomical situations but also to higher mortality and complication rates, the much-debated question of age at surgery had no influence either on the surgical approach itself or on the post-operative outcome. CONCLUSIONS: Our patient categorization, and evaluation of potential pre-operative risk factors and intraoperative parameters, should prove useful for the future planning and execution of therapeutic procedures in institutions around the world.

Cardiac myxomas: short- and long-term follow-up.

BACKGROUND: Cardiac myxomas are the most frequently encountered benign intracardiac tumors, that, if left untreated, are inexorably progressive and potentially fatal. Patients with cardiac myxoma can be treated only by surgical removal. This study summarizes our experience over 22 years with these tumors. METHODS: Fifty seven patients (M/F: 14/43, age: 57.9 +/- 14.6 years) with cardiac myxomas underwent surgical resection at our institution. There were 82.4% left atrial myxomas, 14.0% right atrial myxomas, 3.6% biatrial myxomas. The duration of symptoms prior to surgery ranged from 6 to 1,373 days (median 96 days). The surgical approach comprised complete wide excision. The diagnostic methods, incidence of thromboembolic complications, valve degeneration, surgical repair techniques, recurrence and re-operation were reviewed and the Kaplan-Meier survival curve was calculated. RESULTS: There were no in-hospital deaths. Hospital stay amounted to a mean of 13.7 +/- 6.9 days. Late follow-up was available for 54 (94.7%) patients for a median 7.5 years after surgery (23 days to 21.4 years). Fifty two patients are alive, while five patients had died after a mean interval of 6.3 years. Cause of death was cardiac in 40% of the patients (n = 2) and non-cardiac in the other 60% (n = 3). CONCLUSIONS: Surgical excision of cardiac myxoma carries a low operative risk and gives excellent short-term and long-term results. Surgical excision of the tumor appears to be curative, with few recurrences at long-term follow-up. After diagnosis, surgery should be performed urgently, in order to prevent complications such as embolic events or obstruction of the mitral orifice. Follow-up examination, including echocardiography, should be performed regularly.

Surgical treatment of aortic coarctation in adults: Beneficial effect on arterial hypertension.

BACKGROUND: The aim of this study was to determine the outcome after surgical repair of aortic coarctation in adults, analysing its effect on arterial blood pressure. METHODS: Twenty-five adults (9 women, 16 men), mean age 43.4 years (19 to 70 years), underwent aortic coarctation surgical repair. All patients suffered from preoperative hypertension. Mean blood pressure was 182/97 mm Hg. Sixteen (64%) patients demonstrated reduced load capacity. Operative technique was resection and end-to-end anastomosis for 5 patients (20%), interposition of a Dacron-tube graft for 3 patients (12%), Dacron-patch dilatation was performed in 7 (28%) patients, and in 10 (40%) patients we performed an extra-anatomical bypass graft. RESULTS: Early mortality occurred in 1 patient (4%). The mean blood pressure was reduced [systolic 182 mm Hg vs. 139 mm Hg (p < 0.001), diastolic 97 mm Hg vs. 83 mm Hg (p < 0.001)] in all patients. In 12 patients, blood pressure normalized immediately after surgery, in 7 patients it remained slightly elevated (systolic blood pressure between 140-160 mm Hg), and 1 patient suffered from prolonged arterial hypertension. Preoperatively, all patients were treated with antihypertensive drugs. Eleven of 20 patients received long-term medication during follow- up. In the remaining 4 patients, medication lists were unobtainable in retrospect. The mean follow-up was 7.1 years (min. 1.0 years; max. 16.6 years). One patient (5%) died from cardiac failure 12.4 years after the operation. On average, the New York Heart Association (NYHA) class was improved by 0.92. CONCLUSIONS: The surgical repair of aortic coarctation in adults can be performed with low surgical risk. Surgery reduces hypertension and permits more effective medical treatment.

Anomalous origin of the left coronary artery from the pulmonary artery: mid-term results after surgical correction.

OBJECTIVES: Children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) are at risk for myocardial infarction and death. This retrospective study shows the mid-term follow-up after the use of aortic implantation and alternative methods to achieve coronary transfer. METHODS: Since 1990 seven consecutive children underwent primary repair of ALCAPA. Age at operation ranged from 2 to 71 months (median 11 months). Operative techniques included ligation (n = 1), intrapulmonary tunnel (n = 1), and aortic implantation (n = 5). One patient with severe mitral valve incompetence underwent additional mitral valve replacement. A 4-month-old patient was successfully treated after the operation with a left heart assist device. RESULTS: One death in the series occurred at 2 weeks after intrapulmonary tunneling. The mid-term results were evaluated in the six survivors with a follow-up mean of 98 months (ranged 58-168). In all surviving patients with two-vessel coronary blood supply, left ventricular end-diastolic volume and left ventricular ejection fraction returned to near normal values 2-12 months postoperatively. The mitral valve incompetence decreased in all patients with a native mitral valve. One patient with coronary ligation showed severe mitral valve regurgitation and received additional mitral valve replacement concomitantly. Six years after primary valve replacement of a 21 mm SJM (Saint Jude Medical) a change of the mechanical valve to a 27 mm valve was necessary because of development of severe stenosis due to growth. CONCLUSIONS: It is always preferable to establish an antegrade flow of oxygenated blood through the coronary arteries and to create a two-coronary artery system. Mitral valve annuloplasty or replacement may be necessary for patients with severe mitral valve incompetence.

[Development of the Fontan procedure as therapeutic concept for children with complex congenital heart disease]. / Entwicklung der Fontan-Prozedur als Behandlungskonzept für Kinder mit angeborenen komplexen Herzfehlern.

The spectrum of congenital heart defects reaches from very simple to extremely complex anomalies. 3 decades earlier, the prognosis of children with univentricular heart defects was poor. The Fontan procedure has been offered to patients with a variety of complex cyanotic heart diseases and has resulted in decreased mortality. This review summarizes the development and modifications of the Fontan procedure. The advances in preoperative, intraoperative und postoperative patient management have resulted in greatly improved survivals for even the most complex congenital defects. Over the past 3 decades, the survival of infants with critical congenital heart defects has increased to 90%.

Late results after PTCA for coronary stenosis after the arterial switch procedure for transposition of the great arteries.

BACKGROUND: The arterial switch operation has become the surgical approach of choice for d-transposition of the great arteries, but there is an increased awareness of adverse sequelae in some survivors. Long-term patency and normal function of the translocated coronary arteries must be achieved. It is reported that dependent of the prior coronary status, 3% to 11% of all survivors have proximal coronary stenosis or complete occlusion develop after arterial switch operations. However, treatment of these stenoses is still a matter of debate. Late results after percutaneous transluminal coronary angioplasty (PTCA) for coronary stenosis after the arterial switch operation for d-transposition of the great arteries are reported. METHODS: Seven children after arterial switch operation for d-transposition of the great arteries who had subsequently undergone PTCA for coronary stenosis were angiographically re-evaluated 3 to 15 months after the initial PTCA and again after 3 to 5 years. RESULTS: All children survived the initial PTCA procedure. There were no late deaths. The degree of stenosis before PTCA ranged from 74% to 97%; immediately after PTCA from 5% to 10%; at 3 to 15 months after PTCA from zero to 6%; and at 3 to 5 years after PTCA from zero to 3%. Three to 5 years after PTCA all children showed normal development of the treated coronary artery. CONCLUSIONS: Primary PTCA of stenotic proximal coronary arteries after the arterial switch procedure for d-transposition of the great arteries seems to be an effective treatment with excellent long-term results.

Aortic valve papillary fibroelastoma.

Cardiac papillary fibroelastoma (CPF) is a rare primary benign cardiac tumor. Before the use of echocardiography, the lesion was identified at autopsy or incidentally during cardiac surgery. CPF is the third most common primary cardiac tumor after atrial myxoma and lipoma, and is the most common tumor of the valvular endothelium. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) permit diagnosis of the tumor in living patients. CPF may be the cause of cerebrovascular or cardiac ischemia due to embolization or occlusion of the vascular ostia. Embolic material may arise from fragments of the tumor itself, or from surrounding thrombus. The case is reported of a patient with CPF of the aortic valve in whom TEE diagnosis was conducted and the tumor removed surgically.

Assessment of chronic aortic dissection: contribution of different ECG-gated breath-hold MRI techniques.

OBJECTIVE: Our objective was to evaluate the impact of different rapid MRI techniques for the assessment and follow-up of chronic aortic dissections. MATERIALS AND METHODS: Fifty-three patients (41 postoperative Stanford type A, 12 type B dissections) were scanned at 1.5 T during a 3-year period. The study reviewed ECG-gated breath-hold black blood sequences and 3D contrast-enhanced MR angiography of the thoracic aorta supplemented by segmented cine and phase-contrast imaging as well as abdominal contrast-enhanced MR angiography. A retrospective separate analysis of black blood acquisitions and contrast-enhanced MR angiograms from a total of 72 examinations was performed by two radiologists to evaluate detection of intimal flaps and assess image quality. RESULTS: Sensitivity and specificity of black blood sequences compared with those of contrast-enhanced MR angiography in detecting intimal flaps were 87% and 94% for the thoracic aorta, and 54% and 97% for the supraaortic branches, respectively. Contrast-enhanced MR angiography was subjectively rated as superior to black blood techniques for visualizing intimal flaps and yielded better overall image quality (p < 0.001). Aortic valve competence can be assessed on segmented cine techniques. Phase-contrast sequences enabled the quantification of regurgitant flow across the aortic valve and the analysis of flow patterns in the true and false channels. CONCLUSION: Contrast-enhanced MR angiography is superior to black blood MRI in detecting the presence or absence of intimal flaps and is particularly useful in assessing supraaortic branch vessel involvement. Cine and phase-contrast techniques should be included in the imaging follow-up to diagnose possible complications of chronic aortic dissections.

Gene expression profiling of human stenotic aorto-coronary bypass grafts by cDNA array analysis.

OBJECTIVE: Aorto-coronary bypass graft disease with its increasing clinical signification represents an unsolved problem in cardiological and heart surgery practice. Late occlusion of autologous saphenous vein grafts is due to medial and neointimal thickening secondary to migration and proliferation of smooth muscle cells (SMCs) and the subsequent formation of atherosclerotic plaques. This study is aimed at identifying differentially expressed genes in human stenotic bypass grafts to detect unknown pathomechanism and to identify novel targets for prophylactic treatment options. METHODS: Stenotic saphenous aorto-coronary bypass grafts (n=5) were retrieved during re-do aorto-coronary bypass surgery. Ungrafted saphenous vein segments (n=5) were taken from the same group of patients and served as internal controls. cDNA samples were prepared and hybridized to cDNA arrays. RESULTS: Some of the differentially expressed genes complied with expected gene expression including upregulation of c-jun and CDK10. In addition, previously unidentified gene expression patterns were detected such as upregulation of HSP70, fibronectin1, erbB3 proto-oncogene and c-myc. To confirm the latter finding, upregulation of c-myc in neointimal and medial SMCs of stenotic graft segments was confirmed by in situ hybridization studies and by immunohistochemistry. CONCLUSION: Gene expression patterns of human stenotic bypass grafts retrieved by re-do operations can be reliably analyzed by cDNA array technology. With this technique, new therapeutic targets in patients could be identified as shown by the findings regarding c-myc. c-myc is a proto-oncogene acting as a transcription factor and blocking c-myc has shown a reduction of neointima formation in animal models. Our study yields a rational for the use of antisense c-myc oligonucleotides to reduce neointima formation and to avoid stenosis in patients.

Changes in lymphocyte subsets after cardiac surgery in children.

UNLABELLED: Children undergoing cardiopulmonary bypass (CPB) operations have an increased risk of developing severe infections. Impairment of the immune system may contribute to the development of sequelae such as capillary leaks, pulmonary dysfunction and auto-immune reactions. The objective of this study was to investigate the impact of cardiac surgery with CPB on the immune system of infants and young children. We conducted a prospective study to investigate the changes in circulating lymphocyte subpopulations in a sample of 21 consecutive infants and young children undergoing cardiac surgery for congenital heart disease. The following statistically significant ( P<0.05) results were obtained: leucocyte counts rose 6 h after surgery due to the increase in neutrophils. Absolute T-cell number and absolute T-helper cell number decreased within 24 h after CPB. The proportion of T-cells expressing the T-cell receptor gammadelta as well as natural killer cells increased during CPB. In contrast, the proportion of T-cells expressing activation markers (CD25, CD45R0) decreased within 24 h after CPB, as did the number of cells expressing adhesion molecules (CD11b and ICAM). CONCLUSION: during cardiac surgery with cardiopulmonary bypass, absolute natural killer cell counts increase while T-cells decrease, presumably due to an extravasation or adhesion of activated T-cells. The relevance of this finding regarding the risk of infection is discussed.