Insufficiency of a Damus-Kaye-Stansel anastomosis in a Fontan patient: Transfemoral implantation of an Edwards Sapien 3 valve.

We present a 22-year-old patient with a univentricular heart who had already undergone five open heart surgeries including a Damus-Kaye-Stansel procedure, Fontan completion and tricuspid valve replacement. In addition, epimyocardial pacemaker implantation and repeated revisions had been necessary. He developed symptomatic free regurgitation of the pulmonary portion of his DKS anastomosis. To avoid additional high-risk open-heart surgery, we successfully implanted an Edwards Sapien 3 valve transfemorally in the pulmonary portion of the DKS anastomosis relieving insufficiency.

Clinical Care for Cardiovascular Disease in Patients With Williams-Beuren Syndrome.

Williams-Beuren syndrome (WBS) is a congenital multisystem disorder affecting the cardiovascular, central nervous, and musculoskeletal systems. Cardiovascular abnormalities, which consist principally of vascular stenoses, occur in approximately 80% of people with WBS and are the predominant cause of early morbidity and mortality. Supravalvar aortic stenosis and peripheral pulmonary artery stenosis are the most common stenotic lesions in WBS, though other stenoses often occur, including stenoses of the coronary arteries. Approximately one-third of people with WBS undergo cardiovascular interventions. The risk of sudden cardiac death is markedly higher than the general population, with most events occurring in the periprocedural period. Because of the rarity of WBS and the often-complex nature of the cardiovascular abnormalities, most physicians, including cardiologists, have limited experience in caring for patients with WBS. Further, heretofore, clinical cardiovascular management guidelines based on international expert consensus have not been available. This state-of-the-art review provides a comprehensive synopsis of the cardiovascular abnormalities in WBS and presents clinical management guidelines based on the authors' expert consensus.

[Fetal ECG and arrhythmias]. / Fetales EKG und Arrhythmien.

The normal fetal heart rate ranges between 110 und 180 beats per minute (bpm). Intrauterine arrhythmias are not an uncommon finding. Fetal echocardiography (ECG) allows for correct diagnosis of the arrhythmia, which is prerequisite for decision making and treatment. Most fetal rhythm disturbances are the result of premature atrial contractions and are of little clinical significance. Intrauterine bradycardias (heart rate < 110 bpm) result from sinus node dysfunction, complete AV block and nonconducted atrial bigeminy. Isolated complete heart block related to maternal anti-SSA/Ro or SSB/La auto-antibodies is irreversible in almost all fetuses. Anti-inflammatory therapy and chronotropic medication may improve outcome. Newborn babies often require pacemaker implantation to augment cardiac output. Intrauterine tachycardias (heart rate > 180 bpm) are most commonly related to supraventricular tachycardia and atrial flutter. Specific antiarrhythmic medication is available to stop the arrhythmia and to prevent hemodynamic deterioration.

Management of emergencies in adults with congenital cardiac disease.

The aim of the study was to assess the quantity and nature of emergencies affecting adults with congenital cardiac disease (CCD) and evaluate infrastructural requirements for adequate management. There is an increasing number of adults with CCD requiring specialized complex care. This multicenter study evaluated all emergency admissions to 1 of 5 centers for adults with CCD within 1 year. Within 1 year, there were 1,033 admissions of adults with CCD, and 201 (160 patients; age 16 to 71 years) were emergencies. Underlying cardiac anomalies were univentricular heart (22%), complete transposition (14%), tetralogy of Fallot (21%), and others (43%). Seventy percent of patients had undergone previous cardiac surgery. The main reason for acute admission was cardiovascular (arrhythmia, heart failure, syncope, aortic dissection, and endocarditis). Diagnostic procedures most often assigned were echocardiography (n = 223), chest x-ray (n = 95), Holter electrocardiography (n = 85), cardiac catheterization/electrophysiologic study (n = 39), and others (n = 143). Forty-six patients underwent surgery (cardiovascular n = 41, general n = 5) or electrophysiologic treatment (n = 41). One hundred twenty-six of 201 emergencies (63%) required cooperation with another specialized department: surgery (n = 46), internal medicine (n = 42), neurology (n = 12), ophthalmology (n = 6), otorhinolaryngology (n = 5), gynecology (n = 5), psychiatry (n = 4), radiology (n = 3), dermatology (n = 2), and orthopedics (n = 2). In conclusion, physicians and consultants attending adult patients with CCD need a high degree of specialized experience concerning the cardiac anomaly to manage emergencies properly. Furthermore, a wide range of noncardiac diagnostic and therapeutic procedures must be available. Data support the demand for a multidisciplinary approach in specialized centers for adequate care of adults with CCD.

Quantification of pulmonary regurgitation and prediction of pulmonary valve replacement by echocardiography in patients with congenital heart defects in comparison to cardiac magnetic resonance imaging.

Pulmonary regurgitation (PR) is common in patients with congenital heart defects (CHD) and contributes to morbidity and mortality in the long-term. We investigated in this retrospective analysis whether readily accessible echocardiographic parameters are useful for quantification of PR and for predicting pulmonary valve replacement (PVR) in comparison to the gold-standard phase contrast (PC) flow measurements from cardiovascular magnetic resonance (CMR). Continuous wave (CW) Doppler and colour flow images in echocardiograms from 53 patients with CHD were analysed. Slope and jet-to-RVOT ratio correlated significantly with CMR-assessed regurgitation fraction (RF), whereas pressure half time (PHT) showed an inverse correlation. Patients with mild PR in CMR had significantly higher PHT, lower slope and jet-to-RVOT ratio than patients with moderate or severe regurgitation. The AUC regarding PR severity was 0.778 for PHT (95% CI, 0.649-0.907; P = 0.007 for CMR-RF ≤ 35%), 0.744 for slope (95% CI, 0.603-0.885; P = 0.017 for CMR-RF > 35%) and 0.652 for jet-to-RVOT ratio (95% CI, 0.473-0.860; P = 0.168 for CMR-RF > 35%). The optimal cut-off values calculated from ROC analysis were 95 ms for PHT and 4.9 m/s2 for slope. In logistic regression analysis, slope emerged as the most valuable parameter for predicting the indication for PVR (OR 12.9, 95% CI, 1.8-90.9, P = 0.010). In conclusion, echocardiographic assessment of PR was feasible. Both parameters, PHT and in particular slope, were predictors for PVR. Thus, echocardiography appears appropriate in the management of patients with PR.

Prognostic value of lymphatic and blood vessel invasion in neuroendocrine tumors of the lung.

Few data on the influence of vessel invasion on the progression of neuroendocrine lung tumors are available. Because of the lack of specific markers, previous studies could not reliably discriminate lymphatic and blood vessels. By immunostaining for podoplanin, specific for lymphatic endothelium, and CD34 antigen, we assessed lymphatic and blood vessel invasion in 120 tissue specimens of patients with neuroendocrine lung tumors. Lymphovascular invasion was correlated with clinicopathologic parameters, and its prognostic relevance was evaluated. Lymphatic vessels were identified exclusively at the tumor invasion front, whereas blood capillaries were also seen within tumors. Lymphatic vessel as well as lymphatic and blood vessel invasion was prevalent in patients with high-grade neuroendocrine tumors and advanced tumor stages, closely associated with lymph node metastases (P < 0.0001). In univariate analysis, these two invasion types correlated with decreased disease-free survival (both P < 0.0001), whereas blood vessel invasion alone did not. In multivariate analysis, only tumor grade and lymph node status remained statistically significant factors for prognosis (P = 0.016 and P < 0.0001). Our results suggest that evaluation of lymphatic vessel invasion is important in neuroendocrine lung tumors serving as a prognostic parameter for disease-free survival.

Normality of cardiopulmonary capacity in children operated on to correct congenital heart defects.

BACKGROUND: One of the main goals of corrective surgery of congenital heart defects in children is the improvement of quality of life, which in young children is predominantly determined by exercise capacity. It is not known whether this goal can be achieved in school-aged children who have undergone cardiac surgery in infancy. OBJECTIVES: To determine if primary school-aged children who underwent surgery to correct congenital heart defects in infancy are physically as fit as their peers. METHODS: We examined 84 children with congenital heart defects, aged 4 to 11 years, after surgical repair. Fifty-two children had simple defects (ie, atrial or ventricular septal defect, coarctation of the aorta). Thirty-two children had complex defects (ie, tetralogy of Fallot, pulmonary atresia with ventricular septal defect). All patients underwent exercise testing performed on a specially modified bicycle ergometer. Ninety-eight sex- and age-matched healthy children served as the control group. RESULTS: There was no gender difference, either in healthy children or in the group with congenital heart defects, regarding exercise testing and that the healthy children reached a mean +/- SD normalized maximal performance of 2.8 +/- 0.3 W/kg. The same range was found for the children who had undergone surgery to correct simple heart defects. The children operated on to correct complex heart defects showed significantly impaired mean normalized maximal performance, although this tended to be lower in the group that had pulmonary atresia with a ventricular septal defect than in the group with tetralogy of Fallot (mean normalized maximal performance, 1.9 W/kg vs 2.3 W/kg). CONCLUSIONS: The goal of normal exercise capacity in childhood after heart surgery is achieved in those with simple heart defects only. In children with complex heart defects impaired exercise performance persists, depending on the severity of the heart defect and probably on chronotropic incompetence.

Midterm results after arterial switch operation for transposition of the great arteries: a single centre experience.

BACKGROUND: The arterial switch operation (ASO) has become the surgical approach of choice for d-transposition of the great arteries (d-TGA). There is, however an increased incidence of midterm and longterm adverse sequelae in some survivors. In order to evaluate operative risk and midterm outcome in this population, we reviewed patients who underwent ASO for TGA at our centre. METHODS: In this retrospective study 52 consecutive patients with TGA who underwent ASO between 04/1991 and 12/1999 were included. To analyze the predictors for mortality and adverse events (coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation), a multivariate analysis was performed. The follow-up time was ranged from 1-10 years (mean 5 years, cumulative 260 patient-years). RESULTS: All over mortality rate was 15.4% and was only observed in the early postoperative period till 1994. The predictors for poor operative survival were low APGAR-score, older age at surgery, and necessity of associated surgical procedures. Late re-operations were necessary in 6 patients (13.6%) and included a pulmonary artery patch enlargement due to supravalvular stenosis (n = 3), coronary revascularisation due to coronary stenosis in a coronary anatomy type E, aortic valve replacement due to neoaortic valve regurgitation (n = 2), and patch-plasty of a pulmonary vein due to obstruction (n = 1). The dilatation of neoaortic root was not observed in the follow up. CONCLUSIONS: ASO remains the procedure of choice for TGA with acceptable early and late outcome in terms of overall survival and freedom of reoperation. Although ASO is often complex and may be associated with morbidity, most patients survived without major complications even in a small centre.

Catheter ablation of idiopathic left and right ventricular tachycardias in the pediatric population using noncontact mapping.

BACKGROUND: Idiopathic ventricular tachycardia (VT) in children with a structurally normal heart can cause significant morbidity, and although rare, mortality. Conventional activation and pace mapping may be limited by nonsustained tachycardia or unstable hemodynamics. OBJECTIVE: The aim of this study was to assess feasibility of catheter ablation of idiopathic VT in the pediatric population guided by noncontact mapping. METHODS: Twenty consecutive pediatric patients with idiopathic VT underwent electrophysiologic study with the intention to use the noncontact mapping system EnSite 3000 (EnSite Array, St. Jude Medical Inc., Minneapolis, Minnesota). The multielectrode balloon array was introduced into the left or right ventricle, respectively, and tachycardia was analyzed using color-coded isopotential maps as well as reconstructed unipolar electrograms on the virtual geometry. The region of origin was identified in all of them, and the site of earliest activation with a QS pattern of the unipolar electrograms was guided for sites of ablation. RESULTS: Idiopathic VT originated from the right ventricular outflow tract in 6 patients, from the left ventricle in 8, and from the aortic sinus cusp in 6 in this cohort with a median age of 14.4 (range: 4.8 to 20.9) years. Ablation was attempted in 18 of 20 children, and was acutely successful in 17 of these 18 (94%). During a mean follow-up of 2.3 +/- 1.7 years, VT recurred in 3, 2 of them have been treated with a second procedure, resulting in an overall intermediate-term success in 16 of 18 (89%) children with idiopathic VT. CONCLUSION: Noncontact mapping can safely and effectively be used to map and guide catheter ablation of the tachycardia substrate of idiopathic VT in pediatric patients.

Incidence of coronary artery injury immediately after catheter ablation for supraventricular tachycardias in infants and children.

BACKGROUND: Several case reports have documented coronary artery stenosis or even occlusion after radiofrequency energy ablation (RFA) in children and adults. Coronary involvement early and late after RFA has also been described in animal models. OBJECTIVE: The purpose of this study was to assess prospectively the incidence of coronary artery injury early after catheter ablation for supraventricular tachycardias (SVT) in children. METHODS: From October 2002 to January 2008, 212 consecutive patients younger than 21 years with SVT underwent selective coronary angiography before and 30 minutes after RFA or cryoablation. RESULTS: Median patient age was 12 years (range 0.3-20.4 years), and median body weight was 47 kg (range 5.5-130 kg). An accessory pathway was diagnosed in 112 patients, AV nodal reentrant tachycardia (AVNRT) in 84 patients, and both an accessory pathway and AVNRT in 16 patients. Congenital heart disease was present in 31 patients. In 2 of 117 patients who had RFA for an accessory pathway, an acute reduction in luminal diameter of the coronary artery adjacent to the ablation site was observed. These two patients with a structurally normal heart showed ST-segment changes with normalization of the ECG within 1 week. Both were clinically asymptomatic, and two-dimensional echocardiography documented normal ventricular function. Noninvasive testing did not reveal any evidence of persistent myocardial ischemia. No coronary artery damage was noted after ablation for AVNRT or after ablation with cryoenergy. CONCLUSION: Coronary artery narrowing adjacent to the RFA site was noted in 2 of 117 patients with an accessory pathway and occurred only in patients with a posteroseptal pathway. Coronary angiography could be helpful in avoiding coronary lesions in these settings.

Risk of sudden death in the Williams-Beuren syndrome.

Williams-Beuren syndrome (WBS) is a genetic disorder characterized by a distinctive facial gestalt, mental retardation, mild growth deficiency, and cardiovascular disease. The occurrence of sudden death in the WBS is known from several case reports, but information about the risk of sudden death as derived from the data of a large cohort of patients is lacking. We analyzed the data of 293 WBS patients who had been treated for 43 years at the same two institutions. We thus collected 5,190 patient years without loss to follow-up. During this period ten patients died. Five of them died from: reticulosarcoma (1), after accident (1), heart failure (1), following heart surgery (2). Of the remaining five patients, four died suddenly and one died of unknown cause suggestive of sudden cardiac death. Thus, the incidence of sudden death in our WBS cohort amounts to 1/1,000 patient years. This risk of sudden death is comparable to that following surgery for congenital heart disease, and is 25-100-fold higher compared to the age-matched normal population.