Association of European Paediatric and Congenital Cardiology practical recommendations for surveillance and prevention of cardiac disease in childhood cancer survivors: the importance of physical activity and lifestyle changes From the Association of European Paediatric and Congenital Cardiology Working Group Sports Cardiology, Physical Activity and Prevention, Working Group Adult Congenital Heart Disease, Working Group Imaging and Working Group Heart Failure.

BACKGROUND: Childhood cancer survivors are at increased risk of developing cardiovascular diseases, presenting as the main causes of morbidity and mortality within this group. Besides the usual primary and secondary prevention in combination with screening during follow-up, the modifiable lifestyle factors of physical activity, nutrition, and body weight have not yet gained enough attention regarding potential cardiovascular risk reduction. OBJECTIVE: These practical recommendations aim to provide summarised information and practical implications to paediatricians and health professionals treating childhood cancer survivors to reduce the risk of cardiovascular late effects. METHODS: The content derives from either published guidelines or expert opinions from Association of European Paediatric and Congenital Cardiology working groups and is in accordance with current state-of-the-art. RESULTS: All usual methods of prevention and screening regarding the risk, monitoring, and treatment of occurring cardiovascular diseases are summarised. Additionally, modifiable lifestyle factors are explained, and clear practical implications are named. CONCLUSION: Modifiable lifestyle factors should definitely be considered as a cost-effective and complementary approach to already implemented follow-up care programs in cardio-oncology, which can be actively addressed by the survivors themselves. However, treating physicians are strongly encouraged to support survivors to develop and maintain a healthy lifestyle, including physical activity as one of the major influencing factors. This article summarises relevant background information and provides specific practical recommendations on how to advise survivors to increase their level of physical activity.

Cardiovascular Function and Exercise Capacity in Childhood Cancer Survivors.

INTRODUCTION: Childhood cancer survivors (CCS) might be at high risk of additional chronic diseases due to cardiotoxic side effects. The aim of this study was to analyze long-term side effects of cancer therapy on vascular structure/function, cardiac biomarkers and on physical activity. METHODS: In total, 68 asymptomatic patients aged 16-30 years with childhood cancer (diagnosed 10.6 ± 3.9 years ago) were examined from 2015-2020. (Central) blood pressure and pulse wave velocity were registered via the oscillometric method, while carotid intima-media thickness (cIMT) was measured non-invasively by ultrasound. cIMT values of patients were compared to healthy controls (n = 68; aged 22.3 ± 3.5 years). Patients' exercise capacity was recorded. The plasma N-terminal pro-brain natriuretic protein (NTproBNP) and troponin levels were measured as cardiac biomarkers. CCS were categorized in groups with low, moderate and high anthracyclines. RESULTS: No differences were found in cIMT between patients and controls as well as between patients with various anthracycline dosage. Patients with high dose anthracyclines showed a significant lower performance versus patients with moderate dose anthracyclines (84.4% of predicted VO2peak; p = 0.017). A total of 11.6% of CCS had abnormal NTproBNP values which correlated with received anthracycline dosage (p = 0.024; r = 0.343). CONCLUSION: NTproBNP levels and exercise capacity might be early markers for cardiovascular dysfunction in CCS and should be included in a follow-up protocol, while cIMT and troponin seem not to be adequate parameters.

Subclinical Cardiac Dysfunction in Childhood Cancer Survivors on 10-Years Follow-Up Correlates With Cumulative Anthracycline Dose and Is Best Detected by Cardiopulmonary Exercise Testing, Circulating Serum Biomarker, Speckle Tracking Echocardiography, and Tissue Doppler Imaging.

Background: Survivors of childhood cancer are at risk for anthracycline- and/or radiotherapy-induced cardiotoxicity. Aims: The aim of this study was to assess clinical, laboratory, and imaging parameters of subclinical cardiovascular disease in childhood cancer survivors. Methods: Patients underwent cardiopulmonary exercise test (CPET), laboratory testing, transthoracic echocardiography (TTE) with tissue doppler imaging (TDI) and speckle tracking. A subset of patients also underwent cardiovascular magnetic resonance imaging (CMR). Findings were correlated to cumulative anthracycline and exposure to mediastinal irradiation during cancer treatment. In a subgroup analysis, TTE and CMR findings were compared to data from 40 gender- and age-matched patients with childhood onset hypertrophic cardiomyopathy (HCM). Results: Cardiac evaluation was performed in 79 patients (43 males) at 11.2 ± 4.5 years after cancer treatment. Oncologic diagnosis at a median age of 12.0 years was Hodgkin lymphoma in 20, sarcoma in 17, acute leukemia in 24, relapse leukemia in 10, and others in 8 patients. Cumulative anthracycline dose exceeded 300 mg/m2 in 28 patients. Twenty six patients also received mediastinal irradiation. Decreased peak respiratory oxygen uptake in % predicted on CPET, increased levels of N-terminal pro-brain natriuretic peptide (NTproBNP), increased global longitudinal strain on TTE speckle tracking, and diastolic dysfunction on TDI were the most prominent findings on detailed cardiology follow-up. In contrast to HCM patients, childhood cancer survivors did not show left ventricular hypertrophy (LVPWd z-score median 0.9 vs. 2.8, p < 0.001), hyperdynamic systolic function on TTE (Ejection fraction 62 ± 7 vs. 72 ± 12%, p = 0.001), or fibrotic myocardial changes on CMR (Late gadolinium positive 0/13 vs. 13/36, p = 0.001; extracellular volume fraction 22 ± 2 vs. 28 ± 3, p < 0.001) at time of follow-up. There was no correlation between chest radiation exposure and abnormal cardiac findings. Cumulative anthracycline dose was the only significant independent predictor on multivariate analysis for any cardiovascular abnormality on follow-up (p = 0.036). Conclusion: Increasing cumulative anthracycline dose during cancer treatment correlates with subclinical cardiac dysfunction in childhood cancer survivors best detected by elevated cardiac serum biomarkers, decreased exercise capacity on CPET, and abnormalities on echocardiographic speckle tracking and TDI.

Cardiovascular Biomarkers in Amniotic Fluid, Umbilical Arterial Blood, Umbilical Venous Blood, and Maternal Blood at Delivery, and Their Reference Values for Full-Term, Singleton, Cesarean Deliveries.

Background: Several cardiovascular biomarkers have regulatory functions in perinatal physiology. Aim: This study aimed to analyze the feto-maternal distribution pattern of biomarkers in samples of amniotic fluid, umbilical arterial blood, umbilical venous blood, and maternal blood samples, and to establish reference values. Each linked sample set consisted of the combined samples obtained in an individual pregnancy. Study design: We performed a prospective, observational, cross-sectional, single-center study. Subjects: The sample cohort included 189 neonates who were born to 170 mothers. A total of 162/189 neonates were full term and 129/189 were delivered by elective cesarean section. Outcome measures: Midregional pro-adrenomedullin (MRproADM [nmol/L]), midregional pro-atrial natriuretic peptide (MRproANP [pmol/L]), brain natriuretic peptide (BNP [pg/mL]), N-terminal pro-brain natriuretic peptide (NTproBNP [pg/mL]), copeptin [pmol/L], and high-sensitive troponin I (hsTnI [pg/mL]) levels were measured. Results: In singleton, full-term, primary cesarean deliveries (n = 91), biomarker levels (median, [IQR]) at delivery were as follows. MRproADM levels in umbilical arterial blood/umbilical venous blood/amniotic fluid/maternal blood were 0.88 (0.20)/0.95 (0.18)/2.80 (1.18)/1.10 (0.54), respectively. MRproANP levels were 214.23 (91.38)/216.03 (86.15)/0.00 (3.82)/50.67 (26.81), respectively. BNP levels were 14.60 (25.18)/22.08 (18.91)/7.15 (6.01)/6.20 (18.23), respectively. NTproBNP levels were 765.48 (555.24)/816.45 (675.71)/72.03 (55.58)/44.40 (43.94), respectively. Copeptin levels were 46.17 (290.42)/5.54 (9.08)/9.97 (7.44)/4.61 (4.59), respectively. Levels of hsTnI were 6.20 (4.25)/5.60 (5.01)/0.45 (1.73)/2.50 (2.40), respectively. Conclusion: We determined reference values for biomarkers in term neonates delivered by primary cesarean section in amniotic fluid, umbilical arterial and venous blood, and maternal blood. Biomarkers in the fetal circulation appear to be of primary fetal origin, except for MRproADM.

Feasibility, safety and diagnostic impact of endomyocardial biopsies for the diagnosis of myocardial disease in children and adolescents.

AIMS: Endomyocardial biopsies (EMBs) are performed infrequently in children owing to significant past complication rates and controversial discussions about the therapeutic value of results. The objective of this study was to investigate the safety and feasibility of EMBs for suspected myocardial disease in relation to their clinical value. METHODS AND RESULTS: We performed a retrospective multicentre review of the Working Group for Interventional Cardiology of the German Society for Paediatric Cardiology. During three consecutive years, 206 EMBs (84 female/mean age 8.95±6.62 years) were performed and analysed at 15 heart centres. In the majority of cases, biopsies were taken from the right ventricle (RV/89.8%; p<0.001). The overall complication rate was 9.7%, whereas major complications occurred in only 0.97% of cases. Risk factors associated with a higher complication rate were biopsy during the first year of life (20.5%) and from the left ventricle (31.1%) (p<0.05). There was no procedure-related mortality. Treatment was changed in 18.0% of cases based on biopsy results. CONCLUSIONS: Today, endomyocardial biopsies in older children with suspected myocardial disease can be performed safely with a low risk of major complications and mortality, whereas the risk of complications if the biopsy is carried out in the first year of life or taken from the left ventricle remains high.

Cardiovascular biomarkers in paired maternal and umbilical cord blood samples at term and near term delivery.

BACKGROUND: Cardiovascular biomarkers might help to identify fetuses or pregnancies at risk. AIM: To examine the umbilical cord neonatal and maternal levels of cardiovascular biomarkers at the time of delivery, and to correlate maternal and fetal biomarker levels to each other, to gestational age and to delivery mode. STUDY DESIGN: In a prospective, observational, cross-sectional, single-center study biomarkers were measured in paired maternal and umbilical venous cord blood samples. SUBJECTS: The sample cohort included 66 sets of fetal and maternal blood samples (11 after multiple gestation, 53 after cesarean section, 17 after exposure to labor). OUTCOME MEASURES: Midregional pro-adrenomedullin (MRproADM), midregional-pro atrial natriuretic peptide (MRproANP), brain natriuretic peptide (BNP), n-terminal-pro brain natriuretic peptide (NTproBNP), copeptin, and high sensitive troponin I (hsTnI) levels were measured. RESULTS: Mean ± SEM for biomarker levels in umbilical venous/maternal blood were: MRproADM [nmol/L] 1.02 ± 0.04/1.24 ± 0.08, MRproANP [pmol/L] 215.53 ± 12.96/54.65 ± 3.41, BNP [pg/mL] 32.02 ± 3.37/19.76 ± 3.29, NTproBNP [pg/mL] 1228.94 ± 91.73/71.48 ± 8.65, copeptin [pmol/L] 103.42 ± 22.89/10.41 ± 1.71, and hsTnI [pg/mL] 13.54 ± 5.17/4.91 ± 2.37. Fetal MRproANP, NTproBNP, and BNP were inversely correlated with gestational age. Maternal and fetal MRproANP (r=0.472, p=0.002) and copeptin (r=0.572, p<0.001) levels were correlated, whereas there was no feto-maternal correlation for the other biomarkers. Fetal copeptin was elevated after exposure to labor. CONCLUSIONS: Biomarker levels appear to be regulated independently in mother and fetus. Fetal biomarkers are influenced by gestational age and delivery mode. In this study on term and near term pregnancies without specific fetal pathology, correlation between paired maternal and fetal biomarker levels was weak or not demonstrable.

Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle.

Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart.

Impedance cardiography (electrical velocimetry) and transthoracic echocardiography for non-invasive cardiac output monitoring in pediatric intensive care patients: a prospective single-center observational study.

INTRODUCTION: Electrical velocimetry (EV) is a type of impedance cardiography, and is a non-invasive and continuously applicable method of cardiac output monitoring. Transthoracic echocardiography (TTE) is non-invasive but discontinuous. METHODS: We compared EV with TTE in pediatric intensive care patients in a prospective single-center observational study. Simultaneous, coupled, left ventricular stroke volume measurements were performed by EV using an Aesculon® monitor and TTE (either via trans-aortic valve flow velocity time integral [EVVTI], or via M-mode [EVMM]). H0: bias was less than 10% and the mean percentage error (MPE) was less than 30% in Bland-Altman analysis between EV and TTE. If appropriate, data were logarithmically transformed prior to Bland-Altman analysis. RESULTS: A total of 72 patients (age: 2 days to 17 years; weight: 0.8 to 86 kg) were analyzed. Patients were divided into subgroups: organ transplantation (OTX, n = 28), sepsis or organ failure (SEPSIS, n = 16), neurological patients (NEURO, n = 9), and preterm infants (PREM, n = 26); Bias/MPE for EVVTI was 7.81%/26.16%. In the EVVTI subgroup analysis for OTX, NEURO, and SEPSIS, bias and MPE were within the limits of H0, whereas the PREM subgroup had a bias/MPE of 39.00%/46.27%. Bias/MPE for EVMM was 8.07%/37.26% where the OTX and NEURO subgroups were within the range of H0, but the PREM and SEPSIS subgroups were outside the range. Mechanical ventilation, non-invasive continuous positive airway pressure ventilation, body weight, and secondary abdominal closure were factors that significantly affected comparison of the methods. CONCLUSIONS: This study shows that EV is comparable with aortic flow-based TTE for pediatric patients.

Retrospective analysis of the effect of angiotensin II receptor blocker versus ß-blocker on aortic root growth in paediatric patients with Marfan syndrome.

OBJECTIVES: Cardiovascular pathology, including aortic root dilation at the level of sinus of Valsalva (SV), is one of the major causes of morbidity in paediatric patients with Marfan syndrome (MFS). ß-Blocker (BB) is well established to slow aortic dilation in MFS. Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS. METHODS: 215 patients with MFS (9.01 ± 5.7 years) were subject to a standardised diagnostic programme. Aortic root dilation was evaluated and followed up by echocardiography. In 48 cases, BB and ARB effects on aortic root dilation were evaluated. Effect of treatment was measured by comparison of z scores of SV before and after treatment initiation. RESULTS: Treatment by ARB and BB leads to significant reduction of SV dilation (p<0.05). The deviation of SV enlargement from normal as expressed by the rate of change in z scores was significantly reduced by a mean difference of -0.56 ± 0.71 z scores (p<0.001) under ARB therapy and by a mean difference of -0.35 ± 0.68 z scores (p<0.05) under BB therapy. The prophylactic effect of ARB and BB on aortic root dilation is similar in both groups (p>0.05). CONCLUSIONS: Both concepts lead to a significant reduction of SV dilation. The effect of ARB and BB is similar. This is the first study concerning the comparison of ARB and BB in previously untreated paediatric patients with MFS. The results of the study show that both treatment strategies are beneficial in paediatric and adolescent patients.

The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome.

AIM: Due to age-dependent manifestations, diagnosis of Marfan syndrome (MFS) in children and adolescents is sophisticated. Although revised Ghent criteria is a major step forward, its utility in children is still restricted due to expensive and technically advanced diagnostics. As early diagnosis submits long-term benefits concerning prognosis, the need of an appropriate diagnostic tool for risk stratification of suspected paediatric patients with Marfan is justified. METHODS AND RESULTS: Sixty paediatric patients with Marfan were subject to a standardized diagnostic programme. All clinical symptoms of the revised Ghent nosology were analysed concerning age at first clinical manifestation, prevalence and likelihood ratio for MFS. Symptoms with early onset, high prevalence and high positive likelihood ratio were identified and combined for a risk score called Kid-Short Marfan Score (Kid-SMS). Three risk categories for suspicion of Marfan syndrome were developed. Finally, the Kid-SMS was operated in 130 paediatric patients with suspected MFS. Kid-SMS identified significantly more suspected patients with Marfan compared with Ghent nosology, revised Ghent and genetics alone without oversensitivity. CONCLUSION: Whereas diagnosis of MFS in childhood is sophisticated, Kid-SMS is a useful tool for risk stratification of suspected paediatric patients with Marfan by easy executable diagnostics, especially for paediatricians and paediatric cardiologists.

Impact of age and gender on cardiac pathology in children and adolescents with Marfan syndrome.

Cardiac pathologies are the major aspect in the treatment strategies for Marfan syndrome (MFS). In this progressive disease, less is known about manifestations and progression of cardiovascular symptoms in children. To define a certain decision regarding therapeutic options, knowledge concerning the onset of cardiovascular findings is essential. From 1998 to 2011, suspected pediatric Marfan patients were subjected to a standardized diagnostic program. Cardiovascular findings were analyzed in terms of age at first clinical manifestation, prevalence and gender differences, morbidity, mortality, and treatment. Marfan syndrome was diagnosed in 82 patients (46 boys; mean age at diagnosis, 9.0 ± 5.7 years). At first presentation, aortic root dilation was found in 56 % of patients, mitral valve prolapse in 31 %, whereas pulmonary artery dilation was detected in 22 % and tricuspid valve prolapse in only 17 % of patients. Aortic (2.5 %) and mitral valve regurgitations (22 %) are significantly correlated with aortic root dilation (p < 0.01) and mitral valve prolapse (p < 0.05) but without relevant progression during childhood. Prophylactic medication was initiated for 42 % of the patients (mean age, 8.0 ± 4.5 years) because of progressive aortic root dilation. Aortic dissection did not appear. Aortic root surgery was needed for 4 % of the patients. Gender-specific differences in cardiovascular findings, progression of disease, or treatment did not appear. Comparable with adults, aortic root dilation is the most frequent cardiovascular finding in children and associated with relevant morbidity, whereas aortic and mitral valve regurgitation are of minor clinical relevance. Manifestation at an early age and slow progression of cardiovascular findings underscore the necessity of repeated echocardiographic examinations for early diagnosis and start of prophylactic treatment.

Prevalence of Anemia in Children with Congestive Heart Failure due to Dilated Cardiomyopathy.

Introduction. Anemia is prevalent in adult heart failure patients and appears to be an independent risk factor for morbidity and mortality. The purpose of this work is to determine the prevalence of anemia in children with heart failure from dilated cardiomyopathy (DCM) and to evaluate its influence on morbidity and mortality. Methods. A homogenous group of 58 children with congestive heart failure from DCM was evaluated for heart failure symptoms, appearance of anemia, hospitalization, age of first clinical appearance, necessity of transfusion, and death during medical attendance. Anemic and nonanemic patients were analyzed for differences in age distribution, morbidity, and mortality. Results. Anemia was present in 64% of DCM patients. Hospitalization secondary to heart failure was significantly elevated in heart failure patients with anemia (mean 35.1 ± 40.5 versus 9.97 ± 9.65 days per year, P < 0.05). However, mortality was not elevated. Significant relations of age and prevalence of anemia or age and severity of anemia did not appear. Conclusion. Anemia is prevalent in pediatric patients with congestive heart failure from DCM and appears in all age classes. Hospitalization as a surrogate of morbidity is elevated in heart failure patients developing anemia, but mortality risk did not increase.

Cardiovascular computed tomography angiography in newborns and infants with suspected congenital heart disease: retrospective evaluation of low-dose scan protocols.

Twelve infants (body weight, 3.59±1.36 kg) with congenital heart disease underwent a clinically indicated cardiovascular computed tomography angiography (CTA) study. Retrospectively, we investigated the feasibility and diagnostic capability of different non-electrocardiogram-triggered CTA protocols and assessed radiation doses. Scans were performed on a 256-multislice CT (MSCT) scanner with the vendor-preset helical protocol at 120 kV for babies, with axial single-shoot scans at 120 kV and 80 kV. The 80-kV protocol led to significantly reduced mean effective doses of 0.29±0.08 mSv (P=.017) and renders diagnostic image quality. All major cardiovascular defects were detected on MSCT, and all images were of diagnostic quality.

Left coronary artery occlusion after percutaneous pulmonary valve implantation.

Percutaneous pulmonary valve implantation (PPVI) is an attractive option for patients with pulmonary valve insufficiency or stenotic right ventricular outflow tracts. We present the case of a 26-year-old patient in which PPVI was used to treat d-transposition of the great arteries, uncommon coronary artery anatomy, and conduit stenosis that resulted from multiple operations on the right outflow. Days after discharge to home, she experienced acute chest discomfort correlating with ischemia noted on an electrocardiogram and elevated troponin levels. Coronary angiography confirmed mechanical compression of the left anterior descending coronary artery. The valve was removed in an emergency operation and replaced with a biological conduit.

Early stage 2 palliation is crucial in patients with a right-ventricle-to-pulmonary-artery conduit.

BACKGROUND: Improved survival after Norwood stage 1 palliation is giving more patients the opportunity to reach stage 2 palliation; thus, more patients are exposed to the risk of interstage death. METHODS: A single-center review of patients who underwent stage 1 palliation from January 1998 to December 2007 (n = 58) was performed. Pulmonary blood flow was established either by a modified Blalock-Taussig-shunt (mBTS, n = 33) or a right ventricle-to-pulmonary artery conduit (RVPAC, n = 25). RESULTS: Hospital, interstage, and 1-year survival was not significantly different between groups. However, Kaplan-Meier survival analysis reflected a significantly higher survival probability for RVPAC patients until the age of 120 days (RVAPC, 92% ± 5% [standard error of the mean]; 95% confidence interval, 82 to 100; mBTS, 63% ± 9%; 95% confidence interval, 48 to 82; p = 0.01). During a 1-year follow-up, all 11 nonsurvivors with mBTS died at an age younger than 120 days, including 2 patients with early stage 2 palliation. In contrast, besides 2 early deaths, all RVPAC patients (n = 5) showed later attrition at an age older than 120 days while awaiting stage 2 palliation. Interstage death occurred significantly later among RVPAC patients (RVPAC, 146 ± 60 days versus mBTS, 81 ± 23 days; p = 0.01). After stage 2 palliation, all patients with RVPAC survived, including 7 patients with surgery at an age younger than 120 days. All interstage and late deaths were related to compromising cardiac lesions with no statistical difference between groups. CONCLUSIONS: After Norwood stage 1 palliation, survival was improved with RVPAC for the first 4 months. However, a loss of the favorable primary outcome was present by delaying stage 2 palliation beyond the age of 120 days. Progressive volume load as a result of conduit regurgitation may play a crucial role for later attrition. Residual lesions should be addressed early to preserve cardiac function.

First experience with a new drug-eluting balloon for the treatment of congenital pulmonary vein stenosis in a neonate.

Paclitaxel-eluting balloons are a new and innovative method in the treatment of in-stent stenosis and small vessel disease in adult cardiac pathology. The treatment of congenital pulmonary vein stenosis is difficult to manage, and results in a high mortality rate due to residual or recurrent stenosis. We report the first case of treatment for neonatal pulmonary vein restenosis with a paclitaxel-eluting balloon.

Mechanisms underlying air aspiration in patients undergoing left atrial catheterization.

BACKGROUND: Air embolism in patients undergoing percutaneous interventions requiring access to the left atrium (LA) represents a potentially fatal complication. Here we tested if a decline in LA pressures following sedation represents an important mechanistic link underlying air intrusion into the LA. METHODS AND RESULTS: Left atrial pressures were measured in 26 consecutive patients (49 +/- 14 years; 27% male), who underwent percutaneous atrial septal occlusion for persistent foramen ovale or secundum atrial septal defects. Patients either received sedation by propofol allowing for guidance by transesophageal echocardiography (n = 13) or underwent occluder implantation without sedation and under fluoroscopic control only (n = 13). Whereas mean expiratory LA pressures remained unchanged in either group, sedation provoked a marked decline in the mean inspiratory LA pressure as compared to non-sedated patients (Delta p 6.9 +/- 8.6 mm Hg vs. 0.1 +/- 1.2 mm Hg in nonsedated patients, P < 0.001). Ex vivo experiments evaluating the air-tightness of different sheaths in response to negative pressures revealed air aspiration at -13.4 +/- 1.2 mm Hg of suction in all cases, once a guide wire was inserted. CONCLUSIONS: Negative LA pressures in conjunction with air-leaking sheaths are identified as potentially important factors for air intrusion into the LA with the patient's sedation being a primary risk factor to lower LA pressure levels. The results advocate close monitoring of LA pressures during intervention, prevention of airway collapse and protection of LA sheaths from communication with the atmosphere, during procedures under sedation.

Real-time 3-dimensional echocardiography for follow-up of Waterston shunt.

A 37-year-old man presented with Eisenmenger's reaction. He had progressive cyanosis and signs of right-sided heart failure. In early childhood he was palliated with a Waterston shunt for cyanotic congenital heart disease. Two-dimensional echocardiography provided only a rudimentary view of his heart's anatomy and hemodynamics. Real-time three-dimensional echocardiography (RT3DE) was superior in visualizing both the size and the shape of the large shunt. RT3DE easily identifies and quantifies a key pathogenic factor for pulmonary hypertension with irreversible vascular damage in such patients. If applied early, RT3DE may prove to be a valuable follow-up tool.

Noninvasive follow-up of complications after the Takeuchi operation.

Anomalous origin of the left coronary artery from the pulmonary artery is a rare lesion. Untreated, up to 90% of patients die before 1 year of age. The Takeuchi operation and direct coronary artery reimplantation have resulted in low mortality rates and excellent functional outcome. We present the case of a 6-year-old girl with impaired exercise tolerance in whom anomalous origin of the left coronary artery from the pulmonary artery was detected. A Takeuchi operation was performed. Routine follow-up was successfully done by echocardiography. The postoperative anatomy was visualized in detail, including a baffle-to-pulmonary artery fistula and a coronary artery stenosis. Potentially, given current echocardiographic technology in the hands of experienced operators, routine follow-up may be done noninvasively, leaving angiography for nonroutine indications.