Follow-up of hypertension after aortic coarctation stent implantation based on safe and effective re-intervention: a retrospective cohort study.

Background: Stent implantation for aortic coarctation (CoA) is associated with anatomical and hemodynamic advantages. Although the anatomy is corrected after the intervention, patients often still suffer from hypertension during the follow-up. Its risk factors remain unclear. There are only a few studies on the long-term follow-up of hypertension after stenting. This study aimed to investigate the factors influencing systemic hypertension after CoA by transcatheter stent placement. Methods: A total of 82 patients (native CoA: 37; men: 53) who underwent stent implantation for CoA at a median age of 123 months (range, 40 days-44 years) between January 2004 and September 2012 were included in this study. Patients with native or recurrent CoA after surgery, simple CoA or complex CoA associated with other cardiac anomalies were recruited in this study. Only stents that were expandable to adult size were implanted. After the initial stent implantation, the patients were followed up for 76.7 months (range, 6-151 months). Those who met the indications for a re-intervention underwent a balloon dilatation or stent implantation to relieve restenosis and alleviate the hypertension caused by restenosis. Patients with hypertension were treated with anti-hypertensive agents. Results: In the initial intervention, 87 stents were implanted in 82 patients. Seventy-two patients (87.8%) were diagnosed with hypertension before treatment, but only 23 (28.0%) patients had hypertension at the last follow-up, which is a number significantly lower than that before the intervention (P<0.001). Cox regression analysis showed that age at stent implantation was significantly correlated with the incidence of hypertension, which indicates that the younger the patient receiving the intervention, the lower the incidence of hypertension. Moreover, the incidence of hypertension in follow-up is related to the diagnosis. It was higher in the patients with simple CoA than those with complex CoA. There was no significant correlation between native CoA and recurrent CoA. Conclusions: The incidence of hypertension after CoA stent implantation was significantly lower than that before the intervention. Age at stent implantation is an essential influencing factor for paradoxical hypertension after CoA stent implantation. Complex CoA in patients was related to a significantly lower prevalence of follow-up hypertension.

Stenting of critical aortic coarctation in neonates between 600 and 1,350 g. Using a transfemoral artery approach. A single center experience.

Background: Stenting of aortic coarctation (CoA) in newborns with a very low bodyweight remains rare and challenging. In this study we aim to highlight on two points: first the feasibility of CoA stenting in such babies and second the importance of using echocardiogram for guiding the intervention without the need for contrast agent. Methods: Between 2020 and 2022 three preterm babies with very low (VLWB) and extremely low weight (ELWB) underwent CoA-stenting in our center. The weight of the patients at time of intervention was 1,350, 1,200, and 600 g, respectively. The femoral artery was chosen in all patients as vascular access. Transthoracic echocardiography, sonography of the femoral arteries and head ultrasound were applied for follow up. Results: All three interventions were successfully done, with no complications. Coronary stents were implanted. In one Patient (1,350 g) the stent was inserted without sheath. In two patients with renal failure, the stenting was performed under echocardiography-guidance without contrast agent. The follow up showed a preserved function of the left ventricle in all patients. No relevant gradient was reported and no stent re-intervention was required. Sonographic follow up showed a patent femoral artery in all patients. Two patients were operated 73 and 110 days after stenting, and the stents were successfully removed. In the third patient the intervention was performed 130 days ago and he is waiting for the operation. Conclusion: CoA-stenting in VLWB and ELWB is feasible and can bridge them to the next surgery without complications. Echocardiography-guided CoA-stenting in VLWB is a considerate option especially in patients with renal failure. Accessing the femoral artery by experienced doctors, using local anesthesia before the puncture and before removing the sheath might help to protect the vessel from stenosis or occlusion.

Duct Stenting vs. Modified Blalock-Taussig Shunt: New Insights Learned From High-Risk Patients With Duct-Dependent Pulmonary Circulation.

Background: As no data were available on the comparison of outcomes between modified Blalock-Taussig shunts (MBTs) vs. duct-stenting (DS) in patients with pulmonary atresia (PA) and an increased ductal tortuosity and in patients with pulmonary atresia and intact septum (PA-IVS) with right ventricle-dependent coronary circulation (RVDCC), we aimed to perform a single-center retrospective evaluation. Methods: Between 2010 and 2019, 127 patients with duct-dependent pulmonary circulation (DDPC) underwent either MBTs (without additional repairs) (n = 56) or DS (n = 71). The primary endpoint was defined as arriving at the next planned surgery (Glenn or biventricular repair) avoiding one of the following: (1) unplanned surgery or unplanned perforation of the pulmonary valve (PVP) with a stent, (2) procedure-related permanent complications, and (3) death. Two subgroups were considered: (1) patients who had a ductal curvature index (DCI) >0.45 (n = 32) and (2) patients with PA-IVS and RVDCC (n = 13). Ductal curvature index (DCI) was measured in all the patients to assess the tortuosity of the ducts. Patients with DCI >0.45 were considered as being in a high-risk group for the duct-stenting; a previous study showed that the patients with a DCI < 0.45 had a better outcome when compared with those with a DCI> 0.45. Results: The primary outcome was achieved equally in the two groups (77.5% in DS, 75% in MBTs). Hospital deaths, need for ECMO, and the occurrence of major complications was more frequent in the group with MBTs with an Odds Ratio (OR) of 5, 0.8, and 4, respectively, and a 95% Confidence Interval (CI) 1.1-22.6, 0.7-0.9, and 1.6-10.3, respectively, and a P-value < 0.05. For the two subgroups, the primary outcome was achieved in 64% of patients with a DCI >0.45 who received MBTs compared to 20% in those with DS (OR 3.5, 95% CI 1.2-10, P 0.005). While 74.1% of the patients with PA-IVS and RVDCC after DS had achieved the primary outcome, all patients with MBTs had an impaired outcome (OR 3.5, 95%CI 1-11.2, P 0.004). Conclusion: MBTs showed a better outcome in patients with tortuous ducts compared to DS. DS seems to be superior in patients with DDPC with DCI <0.45 and patients with PA-IVS with RVDCC.

Prospective multicenter study of the breakable babystent for treatment of aortic coarctation in newborns and infants.

To assess the efficacy and safety of a breakable BabyStent to treat complex aortic coarctation (CoA) in early childhood. Although recommended in several guidelines, there is no approved aortic stent for young infants, because of the dilemma between two mandatory requirements: expandable up to adult size on the one hand, and small enough to fit through a baby's femoral artery on the other. Prospective interventional, multi-center clinical trial with the breakable Osypka BabyStent® (OBS). The OBS is a low-profile, 15-mm long cobalt-chromium stent, pre-mounted on a 6 mm balloon and inserted via a 4 Fr sheath. After implantation, its diameter is adjustable from 6 to 12 mm by balloon dilation. Further dilation opens predefined joints enabling unrestricted growth. Nineteen patients (9 male), median age 112 days (range: 7-539), median body weight 5.6 kg (range: 2.4-8.4) were deemed high risk and underwent stent implantation. Of those, 74% suffered from re-CoA following surgery, 53% had additional cardiac and 21% noncardiac malformations. Our primary combined endpoint was fulfilled: All stents were implanted in the desired region, and a >50% intrastenotic diameter-extension was achieved in 15 patients (78.9%, 80% confidence interval [62.2; 90.5], 95% confidence interval [54.4; 93.9]). Secondary endpoint confirmed that the OBS fits the baby's femoral vessel diameter. All children survived the procedure and 12-month follow-up. This stent enables percutaneous stenting of complex aortic coarctation to treat high-risk newborns and infants.

Use of the ductal curvature index to assess the risk of ductal stenting in patients with duct-dependent pulmonary circulation.

BACKGROUND: To evaluate whether a quantitative curvature threshold can contribute to risk stratification of ductal stenting in patients with a duct-dependent pulmonary circulation (DDPC). METHODS: A single-center retrospective analysis was performed. The ductal curvature index (DCI) was calculated in 71 patients with DDPC. The ducts were divided into four classes based on quartile thresholds of DCI: class I (≤0.12), class II (0.13-0.33), class III (0.34-0.44), and class IV (≥0.45). The primary outcome of this study was defined as free from all of the following: (I) intervention related death, (II) need of unplanned surgery (III) need of unplanned pulmonary valve (PV) perforation with stent in right ventricular outflow tract (RVOT), and (IV) intervention related permanent complications. RESULTS: Eighty percent of patients in class IV (DCI ≥0.45) failed to achieve the primary outcome; odds ratio (OR) 9 and 95% confidence interval (CI): 3.7-21.4 compared to 26.6% in all classes with DCI <0.45. 66.7% of these patients needed unplanned surgery or PVP with RVOT stent; OR 12.4 (95% CI: 4-39). 80% of major complications were observed in class IV (P<0.01). Need of pulmonary arterioplasty was in class IV 53.3%; OR 3.3 (95% CI: 1.5-7.1). CONCLUSIONS: DCI can be useful to guide the clinical decision-making in patients with torqued ducts. Patients with a DCI ≥0.45 belong to a high-risk group, in which ductal stenting is associated with an elevated risk for early surgery or unplanned re-intervention.

2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.

Severe midaortic syndrome: a stepwise approach to treatment with drug-eluting balloons: a case report.

BACKGROUND: Severe forms of the midaortic syndrome cause persistent arterial hypertension and can lead to angina abdominalis. Untreated, morbidity, and mortality are significant. In addition to palliation through bypass surgery, few other therapeutic approaches lead to a long-term relief. Drug-eluting balloons (DEB) covered with paclitaxel, a cytostatic drug, have proven to be effective in bifurcational lesions and for in-stent stenoses in coronary arteries. CASE SUMMARY: In a 15-year-old girl with severe midaortic syndrome and multiple collateral arteries, four interventional balloon dilatations with DEB of increasing diameters resolved the stenosis within 8 months. After a procedure free interval of now 2.7 years, the anatomical and the physical condition of the patient remained unchanged. DISCUSSION: This stepwise approach, with a low procedural risk and a lasting result may justify further investigations into this combined treatment.

First follow-up of a breakable stent for implantation in infants dedicated for a life-long stay.

OBJECTIVE AND METHODS: The use of conventional metal stents in infants is severely limited by subsequent somatic growth. The use of a breakable balloon expandable stent (BS) designed for initial implant at small diameters but with properties that allow unlimited dilation in line with growth has potential advantages in this patient group. This study reports our experience with this stent between 2010 and 2014. A total of 17 BS were implanted in 14 infants (mean age 4.8 months). All but one stent was placed into the aorta to treat coarctation. RESULTS: All implantations were successful and initial gradients dropped from a mean of 25-6 mm Hg (range from 1-50 down to 0-24 mm Hg). Mean follow-up was 3.3 years (range 5 days to 7 years) with a total cumulative follow-up of 46.7 patient years. Stent redilation was performed a median of 2.5 times (range 0-5). Sixteen stents in 13 patients remain in place. Following redilation beyond 10 mm, circumferential integrity of the BS was lost in 10 patients. No further stent implantation or related surgery was necessary. A 3 mm dissection occurred in one patient after redilation. CONCLUSIONS: The BS performed well in terms of relief of stenosis and could be successfully dilated during the phase of the infants' most rapid growth. Mild intimal proliferation occurred in some patients early after implantation. In the course of the stepwise redilations and growth adjustments, both, planned longitudinal and transverse fractures occurred without allowing a collapse of the stented area.

Promising Outcome of Anatomic Correction of Corrected Transposition of the Great Arteries.

BACKGROUND: Anatomic correction of corrected transposition of the great arteries with associated lesions, utilizing the morphologic left ventricle as a systemic pumping chamber, is the preferred method in many centers. The purpose of this study was to analyze functional outcome after anatomic correction. METHODS: Between Jan 1997 and May 2016, 63 patients with corrected transposition of the great arteries and associated lesions underwent anatomic correction. Forty-two patients (67%) underwent palliation before correction, including 14 patients (22%) who required training of systemic ventricle. The double switch procedure was performed in 37 patients; 25 patients underwent the Senning-Rastelli operation, and 1 patient underwent the Senning-Nikaidoh procedure. The median age at correction was 1.6 ± 3.7(SD) years (range, 0.2 to 17.8 years). RESULTS: The survival and freedom from any event was 95% and 71%, respectively, at 15-year follow-up. The combined freedom from death, failure of systemic ventricle, or heart transplant was 93% at 15-year follow-up regardless of procedure type. Sinus rhythm was present in 49 patients, with 14 patients requiring pacemaker (22%)-8 preoperatively, 4 early postoperatively, and 2 late postoperatively. Neurological development is normal in all patients. Fifty-four percent of the patients are not on medication. CONCLUSIONS: Anatomic correction of corrected transposition of the great arteries is a safe procedure that provides encouraging survival and functional benefits. Ninety-three percent preservation of morphological left ventricle function in 15 years of follow-up supports the concept of anatomic correction. Longer follow-up is needed to confirm superiority of this approach over other management strategies.

[Antibradycardia therapy : Indication and implementation]. / Antibradykarde Therapie : Indikation und Umsetzung.

Pacemaker therapy (PMT) in grown-up congenital heart disease (GUCH) must meet the demands of a young, dynamic and heterogeneous group of patients. The duration of the therapy has to be planned for several decades and should be accompanied by an invasive procedure at the very least. Most of the patients enter adulthood with their pacemaker (PM) already implanted; for others the indications can be derived from the published guidelines for GUCH and PMT, but need to be adjusted to the individual situation of the patient. Depending on the underlying heart disease the decision on the use of either an epimyocardial or a transvenous PM system has to be made. Both electrodes and PM should correspond to the latest technical developments to optimally adapt to the patients' multiple requirements. In the case of PM system revisions abandoned leads should be removed and vascular stenosis or occlusions cleared to be prepared for later revisions. During any cardiac surgery epimyocardial PM systems should be checked against the patient's needs and expanded or revised accordingly. Epimyocardial resynchronization systems in particular offer more opportunities for compensating for cardiac dysfunction with greater reliability using a second ventricular lead. The PMT is an essential part of the medical treatment for many patients with GUCH and contributes significantly to the well-being and quality of life. Against this background, a competent and consequent follow-up regime requires experienced physicians. An integrated telemetric monitoring system for the PM has proven valuable and supports the early recognition of cardiac arrhythmia.

Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

UNLABELLED: : The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction. METHODS: The writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2015) and held five face-to-face meetings with votings. Clinical trials, guidelines, and reviews limited to paediatric data were searched using the terms 'pulmonary hypertension' and 5-10 other keywords, as outlined in the other nine articles of this special issue. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on paediatric data only, or on adult studies that included >10% children. RESULTS: A total of 9 original consensus articles with graded recommendations (COR/LOE) were developed, and are summarised here. The topics included diagnosis/monitoring, genetics/biomarker, cardiac catheterisation, echocardiography, cardiac magnetic resonance/chest CT, associated forms of PH, intensive care unit/ventricular assist device/lung transplantation, and treatment of paediatric PAH. CONCLUSIONS: The multipaper expert consensus statement of the European Paediatric PVD Network provides a specific, comprehensive, detailed but practical framework for the optimal clinical care of children with PH.

Diagnostics, monitoring and outpatient care in children with suspected pulmonary hypertension/paediatric pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

Pulmonary hypertension (PH) is a condition of multiple aetiologies with underestimated prevalence and incidence. Indeed, despite access to modern therapies, pulmonary hypertensive vascular disease (PHVD) remains a progressive, usually life-limiting condition, severely impacting on the patients' well-being. We herein provide practical, expert consensus recommendations on the initial diagnostic work-up, clinical management and follow-up of children and adolescents with PH/PHVD, including a diagnostic algorithm. The major topics and methods that need to be tailored and put into context of the individual patient include PH classification, clinical signs and symptoms, basic diagnostic and advanced imaging measures (ECG, chest X-ray, transthoracic echocardiography, cardiac magnetic resonance, chest CT angiography, cardiac catheterisation, ventilation-perfusion lung scan, abdominal ultrasound), lung function tests, 6 min walk and cardiopulmonary exercise testing, sleep study (polysomnography), laboratory/immunological tests, considerations for elective surgery/ general anaesthesia, physical education and exercise, flying on commercial airplanes, vaccinations, care of central intravenous lines and palliative care. Due to the complexity of PH/PHVD, the clinical care has to be multidisciplinary and coordinated by a dedicated specialist paediatric PH centre, not only to decrease mortality but to allow children with PH/PHVD to reach a reasonable quality of life.

Enhanced left ventricular training in corrected transposition of the great arteries by increasing the preload.

OBJECTIVES: Patients with congenitally corrected transposition of the great arteries (ccTGA) without ventricular septal defect or left ventricular outflow tract obstruction need training of the morphological left ventricle (mLV) to undergo a double switch operation (DS) (atrial plus arterial switch). Establishing a sufficient gradient by pulmonary artery banding (PAB) is difficult and multiple re-pulmonary artery bandings (re-PABs) may be necessary to achieve the required physiology. We evaluate a new approach, consisting of a loose PAB in combination with an artificial atrial septal defect (aASD) to not only increase afterload but to dynamically enhance preload as well, in terms of the effectiveness of training of the mLV and the subsequent functional outcome after a DS. METHODS: Six consecutive patients under 6 years of age with ccTGA, who were eligible for anatomical correction, underwent enhanced left ventricular training (eLVT). Four of these patients had failed to reach sufficient mLV pressure by PAB only. RESULTS: Required systemic pressures in the mLV were achieved after a mean of 1.2 years of eLVT with no further re-PAB necessary in any patient. All patients underwent a successful DS with an uneventful postoperative period. At a mean follow-up period of 1.9 (range 0.4-3.8) years, stable LV cardiac function was observed in all patients. CONCLUSIONS: The eLVT provides additional training of the mLV to bring patients to a DS. Even in situations when isolated conventional PAB is not effective enough, the novel combination of pressure and volume loading may help with LV retraining and reduce the need for sequential pulmonary band tightening.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia.

BACKGROUND: Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. PATIENTS AND METHODS: A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. RESULTS: Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). CONCLUSION: Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

Transvascular closure of single and multiple muscular ventricular septal defects in neonates and infants < 20 kg.

PURPOSE: While surgical closure of ventricular septal defects (VSD) is still the gold standard, we review our experience with the interventional closure of single and multiple muscular VSD in newborns and infants under 20 kg. METHOD: Between 2004 and 2012 transcatheter closure of ten Swiss cheese VSD, six single muscular and one apical VSD was attempted in 17 patients between 10 days and 7.3 years of age and with a bodyweight ranging from 2.2 to 19 kg. Seven patients had had a significant shunt after cardiac surgery, five patients a shunt induced congestive heart failure and in five patients postponement of surgery was intended. RESULTS: A total of 20 devices was successfully implanted in 15 of 17 (88%) patients, reducing the interventricular shunt and improving the haemodynamic situation in 14 patients. An acute AV-block led to immediate removal of the device in a patient of 2.2 kg. One Amplatzer muscular VSD occluder could not be delivered due to the sharp bending of the delivery sheath in 2004. Three patients died during follow-up not related to the intervention. Re-intervention was necessary in one patient with Swiss cheese VSD. CONCLUSION: Interventional closure of muscular VSD is possible in newborns and infants and presents an eligible treatment option. New occlusion systems with miniaturized introducer sheaths of 4-6 French have extended the spectrum of treatable lesions. An individual and interdisciplinary risk-benefit stratification is required to choose from surgical, interventional, or combined strategies.

Does the shunt type determine mid-term outcome after Norwood operation?

OBJECTIVES: With improved short-term outcomes the right ventricular to pulmonary artery shunt (Sano) has become the preferred pulmonary blood source in the Norwood procedure in many centres. However, most studies analysed consecutive cohorts, with a first modified Blalock-Taussig shunt (BT) followed by the Sano cohort. Besides, neither comprehensive preoperative risk analysis nor outcome beyond 1 year of age was investigated. METHODS: This study reviews 109 neonates undergoing the Norwood procedure in the same interval between October 2002 and December 2009. The Sano (38) or BT shunt (71) was assigned according to the surgeon's preference. Two neonates subsequently underwent successful biventricular repair and were excluded. The Aristotle comprehensive score (ACS) was used to evaluate preoperative risk, with high-risk patients (n = 39) classified as having an ACS ≥ 20, and low-risk patients (n = 68) given an ACS <20. RESULTS: Mean Aristotle score at the Norwood operation was 18.8 ± 0.4 and 18.9 ± 0.3 (P = 0.9) in Sano and BT, respectively. Mean follow-up interval was 4.1 ± 2.1 years (range: 1.7-8.9 years). Actuarial survival was similar, stabilizing from the 8th postoperative month onwards at 78.6 ± 4.9% (95% CI: 67.0-86.5%) for Sano and 78.4 ± 6.8% (95% CI: 61.4-88.6%) for BT; P = 0.95. Midterm actuarial survival was higher in low-risk patients, 88.2 ± 3.9% (95% CI: 77.8-93.9%) than in high-risk patients: 61.5 ± 7.8% (95% CI: 44.5-74.7%, P = 0.0003). No survival benefit was detected in low- or high-risk cases for either shunt type. Risk factors for midterm mortality were cardiorespiratory failure requiring ventilation (13/34, P = 0.004), and ACS ≥ 20 (15/39, P = 0.001), but not shunt type (8/37, P = 0.95). Increased number of shunt-related interventions before the Glenn procedure were noted with Sano (32.4 versus 6.5%, P = 0.002). CONCLUSIONS: Preoperative risk factors, regardless of shunt type, influence midterm survival after the Norwood procedure with an excellent outcome in low-risk patients, while high-risk cases still incur a significant mortality. Sano shunt interventions occurred with increased numbers. Although, Sano shunt may be the only feasible option in some instances, given the possible negative effects of ventriculotomy on right ventricle function, the widespread use of Sano shunt should be reconsidered.

Functional outcome of anatomic correction of corrected transposition of the great arteries.

OBJECTIVE: Anatomic correction of corrected transposition of the great arteries, utilizing the morphologic left ventricle as a systemic pumping chamber, is considered the preferred method. The purpose of the study was to analyze the intermediate functional outcome following anatomical correction. METHODS: Between 1997 and 6/2010, 23 patients with corrected transposition of the great arteries and associated lesions underwent anatomical correction. Seventeen (74%) and six patients (26%) had situs solitus {S,L,L} and situs invs {I,D,D}, respectively. Fifteen patients (65%) had undergone 18 palliations before the corrective operation. The median age at palliation was 0.23 years, with a range of 0.016-8.4 years. A corrective, modified Senning-arterial switch procedure was performed in nine patients, 13 patients underwent a modified Senning-Rastelli procedure, and in one patient a combination of modified Senning and aortic translocation (Bex/Nikaidoh) was used. The median age at the corrective operation was 2 years (from 0.3 to 15.7 years). RESULTS: There was no mortality or heart transplant within the mean follow-up of 3.4 years. Freedom from reintervention was 77% at 5 years. There were no signs of obstruction of the systemic and pulmonary venous tunnels. The function of both ventricles was normal in all patients, even in the four patients who required retraining of the left ventricle. Mild aortic regurgitation was noticed in three patients. Preoperatively detected significant tricuspid regurgitation either disappeared or became trivial after the operation in all the six patients. All patients except two are in sinus rhythm; one patient is pacemaker-dependent preoperatively and one is pacemaker-dependent postoperatively. There were no clinically apparent neurological problems. All patients, but one, are in the New York Heart Association (NYHA) class I. CONCLUSIONS: Anatomic correction of corrected transposition of the great arteries can be performed in selected patients without mortality and with acceptable morbidity. The mid-term functional outcome is excellent, resulting in normal ventricular function, even in retrained left ventricles, and minimal incidence of complete heart block. The long-term function of the aortic valve, intraventricular tunnels, conduits, and ventricles requires close surveillance.

First biodegradable metal stent in a child with congenital heart disease: evaluation of macro and histopathology.

New developments in stent technology led to the first biodegradable magnesium stents. To overcome the fundamental restrictions of conventional stent implantation, these new stents may improve interventional therapy, also in small children. What remains after complete degradation of a magnesium stent is of particular interest and concern. At the autopsy, 2 months after the projected complete degradation time of the 3.0 x 10 mm(2) stent, no solid compounds were detected, and the vessel diameter had increased slightly to 3.7 mm. Histological preparation revealed an amorphous to jelly-like substitute of the magnesium struts mainly consisting of calcium phosphate covered by fibrotic tissue. Immunological staining revealed no relevant inflammatory reaction to the stent material. Neointima proliferation was detected around the struts with some cellular infiltration of the calcium-phosphate material. These pathological and histological findings show minimal alteration of the vessel wall and an increase of the arterial diameter after stent degradation. This is an important precondition for further use of biodegradable stents in small infants. Further observations have to prove whether these findings do reproduce in other settings also.