Fontan Circulation and Aortic Stiffness: Insights into Vascular Dynamics and Haemodynamic Interplay.

Increased aortic stiffness predisposes cardiac afterload and influences cardiac function. Congenital heart diseases involving aortic arch malformation and extended cardiovascular surgery, i.e. univentricular heart diseases, can lead to increased aortic stiffness. This study aimed to investigate whether Fontan patients (FO) have increased aortic stiffness within distinct aortic segments, and whether these parameters relate to Fontan-specific haemodynamics. In a prospective case-control study, 20 FO and 49 heart-transplanted control subjects with biventricular circulation underwent invasive cardiac catheterisation. We invasively measured pulse wave velocity (PWV) in the ascending aorta and along the entire aorta. Haemodynamic parameters, including end-diastolic pressure, pulmonary artery pressure, the cardiac index and systemic vascular resistance index were also assessed. FO exhibited significantly higher ascending aorta PWV (aPWV) than controls (FO: 7.2 ± 2.4 m/s|Controls: 4.9 ± 0.7 m/s, p < 0.001) and compared to the inner group central aorta PWV (cPWV; FO: 5.5 ± 1.2 m/s|Controls: 5.3 ± 1.0 m/s). Multivariate analysis confirmed this aPWV elevation in FO even after adjusting for age and BMI. aPWV and cPWV were almost identical within the control group. Correlation analyses revealed associations between cPWV and blood pressure in controls, while correlations were less apparent in FO. We detected no significant association between the aPWV and other haemodynamic parameters in any of our groups. FO exhibit increased aPWV, indicating specific vascular stiffness in the ascending aorta, while their overall aortic stiffness remains comparable to controls. Further research is needed to understand the implications of these findings on Fontan circulation and long-term cardiovascular health. CENTRAL MESSAGE: Fontan patients show increased aortic arch pulse wave velocity, suggesting specific vascular stiffness. PERSPECTIVE STATEMENT: Our study offers rare insights into pulse wave velocity in Fontan patients, highlighting increased arterial stiffness in the aortic arch. Vascular stiffness was particularly increased in the area of surgical reconstruction. This indicates the need for further research on vascular stiffness in Fontan circulation to understand its impact on cardiovascular health. CLINICAL TRIAL REGISTRATION: German clinical trial registration, DRKS00015066.

Routine follow-up transjugular liver biopsy in Fontan patients: technical considerations and safety of an initial case series and literature review.

Introduction: Patients with Fontan palliation are susceptible to congestive hepatopathy and Fontan-associated liver disease (FALD) because of hemodynamic changes. The staging of liver fibrosis involves various methods, including invasive biopsy. Transjugular liver biopsy (TJLB) offers a less invasive alternative, enhancing liver disease surveillance in routine cardiac catheterization. We detail the technical aspects, share initial outcomes, and discuss existing literature. Methods/results: During routine follow-up cardiac catheterization indicated by hemodynamic or clinical alterations, four patients aged between 16 and 26 years with univentricular Fontan circulation and three patients with biventricular circulation underwent TJLB during routine surveillance catheterization. The examinations were performed under conscious sedation and local anesthesia without general anesthesia. Jugular access was obtained at the site of liver localization, and a 5 F multipurpose catheter was inserted into the liver veins. After hand angiography to delineate the local hepatic venous anatomy, an exchange wire was used to place the bioptome, and three consecutive biopsies were performed. There were no complications, especially perforation or bleeding. The technical success rate was 100%, with all obtained samples appropriate for histopathological diagnostics. The total additional procedure time was less than 20 min. Conclusion: TJLB is an attractive alternative method for obtaining liver specimens in the scope of FALD care. We believe that it should be performed during routine hemodynamic evaluations in Fontan patients and can be performed safely with very low additional time expenditure. As the biopsy site is intravascular, the risk of external bleeding or hematoma is significantly reduced despite the high intrahepatic pressures and the usually impaired coagulation profile in these patients. Based on our initial experience and the lower complication rates compared with other techniques, TJLB should be considered a standard approach in these patients and used more often during the long-term follow-up of Fontan patients. It can be performed in the same setting whenever a hemodynamic assessment of patients with congenital heart defects is required.

MRI pulmonary artery flow detects lung vascular pathology in preterms with lung disease.

BACKGROUND: Pulmonary vascular disease (PVD) affects the majority of preterm neonates with bronchopulmonary dysplasia (BPD) and significantly determines long-term mortality through undetected progression into pulmonary hypertension. Our objectives were to associate characteristics of pulmonary artery (PA) flow and cardiac function with BPD-associated PVD near term using advanced magnetic resonance imaging (MRI) for improved risk stratification. METHODS: Preterms <32 weeks postmenstrual age (PMA) with/without BPD were clinically monitored including standard echocardiography and prospectively enrolled for 3 T MRI in spontaneous sleep near term (AIRR (Attention to Infants at Respiratory Risks) study). Semi-manual PA flow quantification (phase-contrast MRI; no BPD n=28, mild BPD n=35 and moderate/severe BPD n=25) was complemented by cardiac function assessment (cine MRI). RESULTS: We identified abnormalities in PA flow and cardiac function, i.e. increased net forward volume right/left ratio, decreased mean relative area change and pathological right end-diastolic volume, to sensitively detect BPD-associated PVD while correcting for PMA (leave-one-out area under the curve 0.88, sensitivity 0.80 and specificity 0.81). We linked these changes to increased right ventricular (RV) afterload (RV-arterial coupling (p=0.02), PA mid-systolic notching (t2; p=0.015) and cardiac index (p=1.67×10-8)) and correlated echocardiographic findings. Identified in moderate/severe BPD, we successfully applied the PA flow model in heterogeneous mild BPD cases, demonstrating strong correlation of PVD probability with indicators of BPD severity, i.e. duration of mechanical ventilation (rs=0.63, p=2.20×10-4) and oxygen supplementation (rs=0.60, p=6.00×10-4). CONCLUSIONS: Abnormalities in MRI PA flow and cardiac function exhibit significant, synergistic potential to detect BPD-associated PVD, advancing the possibilities of risk-adapted monitoring.

Case report: Paracorporeal lung assist device for 215 days as a bridge-to-lung transplantation in a patient with bronchopulmonary dysplasia and severe pulmonary hypertension.

Pulmonary hypertension (PH) is a known and life limiting complication of preterm born young adults with bronchopulmonary dysplasia (BPD), ultimately leading to progressive right ventricular (RV) failure. Prognosis remains poor, especially in patients unresponsive to modern vasoactive pharmacotherapy. Therefore, lung transplantation presents the treatment of choice to avert cardiac failure. With limited donor organ availability and long waiting times, the implantation of a paracorporeal lung assist device (PLAD) is a way to bridge the patient as an alternative to veno-arterial ECMO. Herein, we present the case of a prematurely born 23-year-old female, who developed severe PH due to BPD and consequently experienced therapy refractory RV failure. Urgent PLAD implantation was performed and the patient successfully underwent double-lung transplantation after 215 days of PLAD support. No major PLAD-associated complications occurred and full recovery of RV function could be observed after double-lung transplantation.

Energy Drinks and Their Acute Effects on Heart Rhythm and Electrocardiographic Time Intervals in Healthy Children and Teenagers: A Randomized Trial.

Beyond their effect on blood pressure, the effect of energy drinks on heart rate in children and teenagers has not been evaluated until now. Thus, this study aimed to investigate the acute cardiovascular effects of energy drinks in healthy children and teenagers. Twenty-six children and adolescents (mean age 14.49 years) received a commercially available energy drink (ED) and placebo on two consecutive days based on the maximum caffeine dosage as proposed by the European Food Safety Authority. Heart rhythm and electrocardiographic time intervals were assessed in a prospective, randomized, double-blind, placebo-controlled, crossover clinical study design. ED consumption resulted in a significantly increased number of supraventricular extrasystoles (SVES) compared to the placebo, whereas supraventricular tachycardia or malignant ventricular arrhythmias were not observed. The mean heart rate (HR) was significantly lower following consumption of EDs. In contrast, QTc intervals were not affected by EDs. Being the first of its kind, this trial demonstrates the cardiovascular and rhythmological effects of EDs in minors. Interestingly, EDs were associated with adverse effects on heart rhythm. Whether higher dosages or consumption in children with preexisting conditions may cause potentially harmful disorders was beyond the scope of this pilot study and remains to be determined in future trials. Trial Registration Number (DRKS-ID): DRKS00027580.

Real-time 3D-echocardiography of the right ventricle-paediatric reference values for right ventricular volumes using knowledge-based reconstruction: a multicentre study.

BACKGROUND: Real-time 3D echocardiography is a promising method for non-invasive assessment of right ventricular performance in children with congenital heart disease. Volume quantification using knowledge-based reconstruction (KBR) enables the calculation of right ventricular dimensions by matching endocardial landmarks with a reference library of right ventricular shapes. However, paediatric reference values for volumes based on KBR are missing. Aim of this study was to establish reference values for right ventricular volumes in a large paediatric population using 3D echocardiography and KBR. METHODS: In a multicentre prospective-design study, 545 healthy children and adolescents (age range, 1 day to 216 months) underwent 3D echocardiography of the right ventricle using two different vendors (iE33, Philips or Vivid 7, GE). Volume analysis was performed by a semiautomatic quantification software (VMS, Ventripoint Diagnostics Ltd., Washington, US). Reference centiles were computed using Cole's LMS method and the gamlss package in R. For vendor comparison, 3D datasets were recorded subsequently in 20 subjects using both ultrasound devices. RESULTS: 3D datasets of 406/545 (74.5%) subjects provided an adequate image quality. Right ventricular volumes had a significant association with age, body size and sex. We created sex-specific percentiles indexed to body surface area (BSA). Intra- and interobserver-variation for all volume calculations were excellent with intraclass correlation coefficients (ICCs) between 0.973-0.998. Agreement of both vendors showed slightly higher end-diastolic and stroke volumes (bias ± standard deviation 2.2%±6.8% respectively 4.5%±8.1%) and smaller end-systolic volumes (-0.9±10.3%) using Philips datasets. CONCLUSIONS: Calculation of ventricular volumes by KBR allows reliable non-invasive assessment of right ventricular volumes with excellent intra- and interobserver variations. The calculated percentiles based on a large paediatric population serve as a reference and may facilitate the use of real-time 3D echocardiography for the analysis of right ventricular size and function.

Long-term outcome of perimembranous VSD closure using the Nit-Occlud® Lê VSD coil system.

OBJECTIVE: This study presents data from the admission trial to show the feasibility, safety and effectiveness of the Nit-Occlud® Lê VSD in the treatment of perimembranous ventricular septal defects with an aneurysmal configuration and a diameter up to 8 mm. BACKGROUND: The majority of ventricular septal defects (VSD) are still closed surgically, while a less invasive transcatheter treatment by closure devices is available. Device-based closure is reported to be associated with the risk of complete atrio-ventricular block, especially with double-disc devices in perimembranous defects. METHODS: In six tertiary centers in Germany and Israel, an interventional closure of a periembranous VSD was attempted in 88 patients using the Nit-Occlud® Lê VSD. RESULTS: The interventional VSD closure was performed in 85 patients. Patients had a median age of 8.0 (2-65) years and a median body weight of 26.7 (10-109) kg. A complete closure of the defects was achieved in 85.4% 2 weeks after device implantation, in 88.9% after three months and in 98.6% at the 5-year follow-up. There was no incidence of death during the study nor did any patient suffer of permanent atrio-ventricular block of higher degree. Serious adverse events, by definition, are potentially life-threatening or require surgery to correct, while major serious events require medical or transcatheter intervention to correct. The study results exhibit a serious adverse event rate of 3.5% (3/85 patients) and a major adverse event rate of 5.9% (5/85 patients). CONCLUSION: The Nit-Occlud® Lê VSD coil offers the possibility of an effective and safe approach in patients with aneurysmal perimembranous ventricular septal defects.

Anakinra And Etanercept Combination Treatment in a Child With Severe, Nonresponsive Kawasaki Disease.

Kawasaki disease (KD) patients' resistance to treatment with intravenous immunoglobulins (IVIG) places them at high risk for an unfavorable progression of the disease. In these patients, there has been little evidence that alternative treatments are effective. Nevertheless, biologicals such as an interleukin-1-receptor blocker and tumor-necrosis-factor-α inhibitors increasingly have been used. If the patient does not respond to one of these therapeutics, a combination of 2 biologicals might be an alternative, but this is not yet generally accepted due to the potentially increased risk of infection. Here we report on a 3-month-old boy suffering from severe refractory KD. KD diagnosis was delayed due to the misinterpretation of a urinary tract infection and to the short and nonsimultaneous presence of classical KD symptoms. After complete KD later was able to be diagnosed, treatment with intravenous immunoglobulins was administered. However, the disease proved resistant to 2 courses of IVIG, as well as to corticosteroids. The patient developed giant coronary artery aneurysms early during the course of disease. Anakinra was initiated, but even with stepwise higher anakinra dosages, he remained febrile and coronary artery dimensions increased. Therefore, etanercept was added as a second biological. Only under combination treatment with anakinra and etanercept were his inflammation and fever able to be completely resolved. Coronary artery dimensions improved over time.

Takotsubo Cardiomyopathy Complicating Percutaneous Pulmonary Valve Implantation in a Child.

Takotsubo cardiomyopathy describes a sudden onset cardiomyopathy with acute impairment of left ventricular function and spontaneous resolution over time. Only a few cases of Takotsubo cardiomyopathy in childhood have been described. We report the case of a 12-year-old girl with repaired tetralogy of Fallot who experienced acute onset of left ventricular dysfunction without coronary arterial involvement, suggesting Takotsubo cardiomyopathy, during an interventional catheterization procedure. Cardiogenic shock necessitated mechanical circulatory support with extracorporeal membrane oxygenator for ten days and mechanical ventilation for 12 days. The girl recovered without sequelae. Percutaneous pulmonary valve implantation was performed four months later without complications. Unusual aspects of this case include the use of mechanical circulatory support during the recovery phase of Takotsubo cardiomyopathy in a patient with congenital heart disease.

Left Atrial Volumes and Phasic Function in Healthy Children: Reference Values Using Real-Time Three-Dimensional Echocardiography.

BACKGROUND: Evaluation of left atrial (LA) size and function is important in congenital and acquired pediatric cardiac disease. Real-time three-dimensional echocardiography (3DE) offers noninvasive assessment of cardiac volumes and phasic function independent of geometric assumptions. The aim of this prospective multicenter study was to establish pediatric reference values for LA 3DE volumes and phasic function based on a large cohort of healthy children. METHODS: LA data sets of 432 subjects (0 days-222 months) were analyzed prospectively using a vendor-independent software. LA volumes (maximal [Vmax], minimal [Vmin], and before atrial contraction) as well as phasic function (active and passive emptying fraction [EF]) were assessed. For volumes, sex-specific reference values, percentiles, and z-scores were calculated by the LMS method of Cole and Green. RESULTS: Absolute volumes increased with age and body surface area. Active EF and relative duration of atrial emptying tended to increase with increasing R-R intervals, while passive EF decreased. Reproducibility of volumes was very good (intra- and interobserver variability for Vmax and Vmin (mean bias ± SD, 0.1 ± 0.9 mL and 0.7 ± 2.8 mL). Volumes were well correlated with cardiac magnetic resonance measurements showing known underestimation of volumes by 3DE (mean bias ± SD, Vmax -14.2 ± 14 mL; Vmin -11.5 ± 10 mL). CONCLUSIONS: Pediatric LA volumes and phasic function indices were reproducibly measured by 3DE. The provided pediatric reference values can be the basis for evaluation of the LA by 3DE and contribute to detection of LA dysfunction and follow-up of patients with congenital heart diseases.

Risk Factors and Outcome of Post Fallot Repair Junctional Ectopic Tachycardia (JET).

BACKGROUND: Junctional ectopic tachycardia (JET) is a common arrhythmia causing hemodynamic impairment following corrective cardiac surgery such as tetralogy of Fallot (TOF) repair. METHODS: We report our experience with postoperative JET following surgical repair of TOF. The retrospective study was done from 2003 to 2012 with a total of 105 patients who underwent TOF repair. These patients' clinical and electrocardiographic data (pre-, intra-, and postoperative) were monitored to identify risk factors for the occurrence of JET and to evaluate the outcome of the affected patients. RESULTS: Incidence-Fourteen patients developed JET, with only four patients going directly from sinus rhythm to JET. In all others, either a transient atrioventricular (AV) block or a junctional rhythm preceded JET, mostly intraoperatively, showing a significant relation ( P = .010). Age-Patients with JET were of younger age ( P = .025) and had longer cardiopulmonary bypass ( P = .044) and aortic cross-clamping times ( P = .038). Increased cost and care-The occurrence of JET was associated with a longer stay in the intensive care unit (ICU) and a prolonged need for inotropic support and mechanical ventilation. Time to rate control correlated with length of ICU and hospital stay. MORTALITY: All JET patients converted into sinus rhythm, one of them died shortly after cessation of JET and two patients subsequently developed a first-degree AV block. CONCLUSION: The occurrence of JET remains an important complication during the initial postoperative period by increasing mechanical ventilation time, the need for inotropic support, and prolonging the length of ICU and hospital stay. Risk factors are younger age, longer aortic cross-clamping/bypass times, and intraoperative arrhythmias.

Early outcomes of percutaneous pulmonary valve implantation using the Edwards SAPIEN 3 transcatheter heart valve system.

AIMS: Multiple surgical revisions are often necessary in individuals with congenital heart defects affecting the RVOT or pulmonary valve. There are no multicentre data on the feasibility and safety of percutaneous pulmonary valve implantation (PPVI) using the SAPIEN 3 (S3) transcatheter heart valve. The aim of this study was to explore the short-term safety, feasibility, and haemodynamic outcomes of PPVI using the S3 transcatheter heart valve. METHODS AND RESULTS: Pulmonic S3 is an observational registry of patients undergoing PPVI with the S3 valve across centres in Europe and Canada. Data for 82 patients (mean age 27.3 years) were obtained. The most common underlying diagnosis was tetralogy of Fallot (ToF) (58.5%), with 16.0% of patients having native RVOT anatomy; 90.2% received pre-stenting. Prosthesis dislodgement occurred in one patient and conduit perforation in another. Both were successfully resolved without the need for open surgery. Peak systolic gradient over the RVOT fell from 46.3 mmHg to 17.2 mmHg, moderate/severe pulmonary regurgitation from 86.3% to 0.0%, and NYHA ≥II from 86.0% to 15.2%. During follow-up, valve thrombosis was observed in two patients which resolved with adequate anticoagulation. No other procedural complications, endocarditis, stent fracture or death were reported within two years. CONCLUSIONS: PPVI with the S3 valve appears feasible and safe in a wide range of patients with congenital heart defects, with good short-term haemodynamic and functional outcomes.

Cardiovascular pre-participation screening in young athletes: Recommendations of the Association of European Paediatric Cardiology.

Sudden death in young competitive athletes can be avoided by implementation of pre-participation screening programmes. A screening programme should be performed only by trained physicians and should include the athlete's personal and family history, physical examination results, and the readings from a 12-lead-electrocardiogram. The athlete should undergo this screening programme every second year to detect progressive diseases. In addition, the programme should include detailed instructions to the athletes to pause training during infections in order to prevent sudden death due to myocarditis.

Lateral Atrial Tunnel Fontan Operation Predisposes to the Junctional Rhythm.

The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis. Medical records were retrospectively reviewed. The hospital survival was 100%. In EC group, cardiopulmonary bypass time (CPB) was shorter (p = 0.004) and less patients needed aortic cross-clamping (p < 0.001). Children after EC stayed longer in the hospital (p = 0.016) and manifested more often prolonged effusions (p = 0.038). The incidence of all forms of junctional rhythm was higher in the LT group, early postoperatively (p < 0.001), during hospitalization (p = 0.004) and at discharge (p < 0.001). Children after LT required more often temporary pacemaker stimulation (p < 0.001). Patients without postoperative normofrequent sinus rhythm had longer CPB time (p = 0.008) and were more often operated on with aortic cross-clamping (p = 0.028). Lateral atrial tunnel Fontan operation with fenestration facilitates early adaptation to the total passive pulmonary flow, but predisposes the patients to the loss of sinus rhythm. The crucial role in the preservation of sinus rhythm plays the last step of the multistage surgery of the single ventricle malformations, probably not only the surgical technique but also factors associated with the cardiopulmonary bypass.

Perturbation of the microvascular glycocalyx and perfusion in infants after cardiopulmonary bypass.

OBJECTIVE: Perturbation of the endothelial glycocalyx is discussed in the pathogenesis of complications related to cardiopulmonary bypass. We evaluated the effects of cardiopulmonary bypass on the microcirculation and the microvascular endothelial glycocalyx of infants undergoing surgery for congenital cardiac defects. METHODS: The microcirculation was visualized at the ear conch using Sidestream dark field imaging before surgery (T0), after admission to the intensive care unit (T1), 24 hours postsurgery (T2), and 7 days postsurgery (T3). Glycocalyx thickness was assessed by measurement of the perfused boundary region. Microcirculatory parameters included total and perfused vessel density, vessel diameters, and microcirculatory flow index. RESULTS: A total of 40 infants undergoing cardiac surgery (36 with cardiopulmonary bypass, 4 without cardiopulmonary bypass) were examined. As controls, measurements before and after cardiac catheterization (n = 6) and before and after surgery for cleft palate (n = 9) were performed. After surgery with cardiopulmonary bypass, the perfused boundary region was significantly increased, indicating reduced glycocalyx thickness at T1 compared with preoperative values with a stepwise return to baseline by T3. In the control groups, no significant perfused boundary region changes were noted. Furthermore, after cardiopulmonary bypass, a transient, significant reduction of the microcirculatory flow index and the perfused vessel density was seen at T1. Similar changes were observed after cardiac surgery without cardiopulmonary bypass, but not in the other controls. CONCLUSIONS: Our study reveals for the first time local perturbations of the endothelial glycocalyx and microvascular perfusion in infants after surgery with cardiopulmonary bypass. Microcirculatory monitoring might be a useful tool to evaluate interventions aiming at reduction of bypass-related complications.

Influence of pulmonary artery size on early outcome after the Fontan operation.

BACKGROUND: Small pulmonary arteries (PAs) are often considered as a contraindication for the Fontan operation (FO). The aim of this study was to evaluate if the PA size is still one of the major impact factors on the postoperative early outcome. METHODS: Data of 146 patients, with a median age of 2.0 years (range, 1.5 to 18 years) and a median weight of 12.45 kg (range, 7.7 to 64.7 kg) who underwent a modified FO in the same center between 2007 and 2012 were retrospectively analyzed with respect to the traditional McGoon ratio, Nakata index, and modified indices (measuring the narrowest diameters). RESULTS: Patients with a McGoon ratio of 1.6 or less (modified≤1.2) or a Nakata index of 150 mm2/m2 or less (modified≤100 mm2/m2) were not at a higher risk of longer mechanical ventilation (p=0.87 [0.1] and p=0.68 [0.52], respectively), longer stay (p=0.52 [0.18] and p=0.54 [0.38], respectively) in the intensive care unit, prolonged hospital stay (p=0.08 [0.26] and p=0.22 [0.29], respectively) or effusions (p=0.25 [0.37] and p=0.13 [0.06]), respectively). Younger and smaller children tended to have smaller PAs, but younger age (<24 months) and lower weight (<12 kg) were not predictive for poor early postoperative outcome. CONCLUSIONS: Small PAs do not significantly affect the early postoperative period after FO. In our opinion, there is no need to postpone the FO due to "smaller" PAs. The palliative procedures performed before FO to increase the size of the PA at the expense of volume overload of the single ventricle and the possible complications of prolonged cyanosis must be carefully weighed.

Effect of antiandrogen treatment on bone density and bone geometry in adolescents with polycystic ovary syndrome.

STUDY OBJECTIVES: To determine the impact of antiandrogen treatment on bone density and geometry. DESIGN: Prospective cohort investigation. SETTING: Academic research institute. PARTICIPANTS: 38 (age 14.96 ± 1.42 yr) subjects with PCOS. INTERVENTIONS: Treated with metformin (n = 17) or metformin and antiandrogen (n = 21). MAIN OUTCOME MEASURE: Bone density and geometry parameters at baseline and after a mean duration of 1.92 ± 0.88 years using peripheral quantitative computed tomography of the forearm. RESULTS: At baseline, z-scores for trabecular (0.53 ± 1.02) and cortical BMD (0.79 ± 1.55) as well as total (0.62 ± 1.07) and medullary cross sectional area (CSA) (0.79 ± 1.29) were elevated. Cortical CSA (-0.01 ± 1.10) and bone strength strain index (SSI) z-scores (-0.01 ± 1.10) were normal. Muscle CSA z-score (0.12 ± 1.70) was normal, but grip strength (-1.60 ± 1.15) was significantly reduced. There were no significant changes within and between the two treatment options in respect to bone density and bone geometry parameters. With antiandrogen treatment, free androgen index (FAI) was significantly lower and grip strength further decreased (P < .001). CONCLUSIONS: No significant changes in bone mineral density and geometry parameters took place in PCOS women irrespective of treatment followed over a time of almost two years. General muscle weakness expressed as low grip strength may influence further bone development in PCOS.

Right ventricle-to-pulmonary artery shunt related complications after Norwood procedure.

OBJECTIVE: The right ventricle-to-pulmonary artery (RV-PA) shunt in the Norwood procedure (NP) for children with hypoplastic left-heart syndrome (HLHS) provides stable early hemodynamics and improves survival in many centers. However, lower pulmonary-to-systemic flow ratio causes early cyanosis and may require earlier second-stage procedure. The aim of the study was to present shunt-related results after NP with RV-PA shunt and our technique of RV-PA shunt construction. METHODS: Between June 2001 and August 2010, 236 children with HLHS and variants underwent NP with RV-PA shunt, and were operated on by the same surgeon. The medical records were retrospectively reviewed. RESULTS: To date, 180 children at a mean age of 7.0 ± 1.6 months with a mean weight of 6.4 ± 0.9 kg underwent second-stage procedure. The mean systemic oxygen saturation before stage 2 operation was 74.8 ± 6.6% and mean arterial partial oxygen pressure was 32.8 ± 6.7 mm Hg. These two parameters were significantly lower than after NP (p = 0.029, p < 0.001, respectively). Between stage 1 and 2 operation, three children (1.3%) died due to the shunt obstruction. Four children (1.7%) underwent re-operations due to shunt problems (one of them died), and the other four (1.7%), stent implantation in RV-PA shunt. Two infants (1.1%) developed aneurysm of the right ventricle infundibulum, which was resected during stage 2 without complications. One child required early (before fifth month of age) second-stage procedure due to the shunt obstruction. The patients with right-sided to the neo-aorta course of the RV-PA shunt had significantly more frequent delayed sternal closure after NP than children with left-sided shunt (35.5% vs 14.1%; p = 0.008). CONCLUSIONS: The RV-PA shunt can be a safe and efficient technique in providing optimal pulmonary blood flow in the children with HLHS after Norwood procedure, performed with minimal rate of complications. In our experience, the use of RV-PA shunt in NP does not require earlier second-stage procedure.

Impaired bone geometry after heart and heart-lung transplantation in childhood.

BACKGROUND: Impaired bone health has been advocated after solid organ transplantation in adult and pediatric patients. Osteoporosis accompanied by fractures have been found also in heart transplantation recipients. METHODS: Parameters of bone mineral density (BMD) and bone geometry were measured in 34 patients (15 females, mean age: 14.6 ± 5.5 years) 5.28 ± 5.16 years after heart transplantation (n=30) and heart-lung transplantation (n=4) using peripheral quantitative computed tomography. Transplantation had been performed because of dilated cardiomyopathy in 25, congenital heart disease in five, idiopathic pulmonary hypertension in three patients, and arrhythmogenic right ventricular dysplasia in one patient at a mean age of 9.4 ± 6.1 years. RESULTS: Trabecular BMD (z scores -0.82 ± 0.50, P<0.01) was reduced and cortical BMD (0.62 ± 1.17, P<0.05) increased in patients (n=11) on steroids, whereas patients on immunosuppression with tacrolimus showed normal trabecular BMD (-0.19 ± 1.03). All patients had normal total cross-sectional area (CSA; -0.22 ± 1.50) and relatively enlarged medullary CSA (0.44 ± 1.37) resulting in smaller cortical CSA (-0.91 ± 1.20, P<0.01). Mean muscle CSA was significantly reduced (-2.02 ± 0.99, P<0.001) irrespective of glucocorticoid treatment and correlated significantly with cortical CSA (r=0.43, P=0.001). CONCLUSION: After heart transplantation and heart-lung transplantation in childhood, all our patients showed altered bone geometry and low muscle CSA. Patients on glucocorticoid treatment had additionally lower trabecular BMD. The clinical impact of these findings on increased risk for fractures has to be determined.

Unique technique of surgery in an unusual variety of Scimitar syndrome: a case report.

Scimitar syndrome is a rare congenital anomaly characterized by total or partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We present a seven year old girl with a systolic murmur who was diagnosed as having a Scimitar syndrome with unusual drainage of the right pulmonary veins. The unique technique of surgery in this patient was appropriate to the unusual, previously not described anatomy.