Systemic Ventricular Function Research Articles

TAM ATRİYOVENTRİKÜLER BLOK VE İZOLE DOĞUŞTAN DÜZELTİLMİŞ BÜYÜK ARTERLERİN TRANSPOZİSYONU OLAN ASEMPTOMATİK BİR OLGU

Corrected transposition of the great arteries (cTGA) is a rare congenital heart disease. c-TGA occurs in 0.5% of patients born with congenital heart disease. Other congenital heart defects commonly accompany this anomaly. Prognosis depends on systemic ventricular function, anomalies accompanying anomalies. The patient was conduction

Mid-regional pro-adrenomedullin: an indicator of the failing Fontan circuit in patients with univentricular hearts?

In patients after the Fontan procedure, assessment of a failing Fontan circuit is difficult. Natriuretic peptides failed to be reliable markers of functional status or systemic ventricular function in this patient cohort. The aim of the study was to assess the clinical utility of mid-regional pro-adrenomedullin (MR-proADM) in patients after the Fontan procedure. Plasma MR-proADM levels were measured in 53 patients after the Fontan procedure and compared with clinical status, echocardiographic, and laboratory parameters including NT-proBNP. Median MR-proADM levels were 0.668 nmol/L in patients with a failing Fontan circuit as compared with 0.357 nmol/L in those without Fontan failure (P = 0.001). Levels of MR-proADM were significantly related to the presence of Fontan failure (r = 0.444, P = 0.001), NYHA class (r = 0.434, P < 0.001), and γ-glutamyltransferase levels (r = 0.554, P < 0.001). According to receiver operating characteristic (ROC) curve analysis, Fontan failure was best predicted by MR-proADM [area under the curve (AUC) 0.985, P = 0.001], NT-proBNP (AUC 0.947, P = 0.003), NYHA class (AUC 0.962, P = 0.002), and the inspiratory/expiratory ratio of the inferior vena cava diameter (AUC 0.973, P = 0.007). The optimal cut-off of MR-proADM for the prediction of Fontan failure was 0.520 nmol/L with a sensitivity of 100%, specificity of 93.9%, positive predictive value of 57.1%, negative predictive value of 100%, and overall accuracy of 94.3%. However, the data should also be validated in a larger cohort of patients. Serial measurements of MR-proADM levels may help identify patients at risk for a failing Fontan circulation especially when exceeding 0.520 nmol/L. In these patients, intensified medical care should be considered to prevent further clinical deterioration.

Risk factors for major adverse events late after Fontan palliation.

Risk factors for major adverse events late after Fontan palliation are unknown. Prior studies have suggested ventricular function and morphology as important risk factors. The aim of this study is to (1) characterize the late major adverse event profile in adult Fontan patients and (2) identify additional risk factors that may contribute to adverse outcomes. A retrospective review of all adult patients >15 years post-Fontan seen at a tertiary academic center was conducted. Clinical, laboratory, cardiac data, and abdominal imaging were collected via chart review. Major adverse events (death, cardiac transplantation, or listing) were identified, and timing of events was plotted using Kaplan-Meier methods. Univariate and multivariate logistic regression was used to determine independent predictors of late-term events. A total of 123 adult Fontan patients were identified (mean time post-Fontan 22.4 years [±4.4]). Major adverse events occurred in 19/123 patients (15%). In this 15-year survivor cohort, transplant-free survival rates were 94.6%, 82.9%, and 59.8% at 20, 25, and 30 years postoperation, respectively. Modes of death were Fontan failure with preserved function (4), congestive heart failure with decreased function (2), sudden death (2), thromboembolic event (1), post-Fontan conversion (2), and posttransplant (2). No differences in adverse outcomes were found based on morphology of the systemic ventricle, Fontan type, or systolic ventricular function. On the other hand, features of portal hypertension (OR 19.0, CI 4.7-77.3, P < .0001), presence of a pacemaker (OR 13.4, CI 2.6-69.8, P = .002), and systemic oxygen desaturation (OR 0.86, CI 0.75-0.98, P = .02) were risk factors for major adverse events in the multivariate analysis. In adult Fontan patients surviving >15 years post-Fontan, portal hypertension, oxygen desaturation, and need for pacemaker were predictive of adverse events. Traditional measures may not predict late-term outcomes in adult survivors; further study of the liver's role in late outcomes is warranted.

Is systemic right ventricular function by cardiac MRI related to the degree of tricuspid regurgitation in congenitally corrected transposition of the great arteries?

Background and MethodsSystemic right ventricular dysfunction and tricuspid regurgitation (TR) are frequently encountered in patients with congenitally corrected transposition of the great arteries (CCTGA). Studies using echocardiography have suggested a relationship between the degree of TR and systemic right ventricular dysfunction; however, assessment of systemic right ventricular function by echocardiography is limited. Cardiac MRI (CMR) is the gold standard for volumetric assessment of the systemic right ventricle. We performed a retrospective cohort study at our center evaluating all adult patients with CCTGA who underwent a CMR between 1/1999 and 1/2013 to determine the relationship between the degree of TR and systemic right ventricular function. ResultsOf the 33 patients identified, 12 had ≤mild TR (37%), 13 had moderate TR (40%), and 8 had severe TR (24%). Mean age at CMR was 38years (23–64). Mean right ventricular ejection fraction (45% vs. 41% vs. 42%, p=0.68) and mean indexed right ventricular end diastolic volume (122ml/m2 vs. 136ml/m2 vs. 138ml/m2p=0.36) were not significantly different for patients with ≤mild TR, moderate TR or severe TR. The degree of TR was not associated with additional congenital lesions, prior procedures, presence of an intraventricular conduction delay, or decreased left ventricular function. ConclusionNo association between the degree of TR and right ventricular volume or ejection fraction by CMR was identified. Failure to show worsening function or increased volume with greater degrees of TR suggests that the degree of regurgitation alone may not fully explain the heterogeneity in right ventricular size and function.

Intermediate-Term Results of Extracorporeal Membrane Oxygenation Support Following Congenital Heart Surgery

Although there are considerable data regarding in-hospital results of congenital heart surgery (CHS) patients requiring postoperative extracorporeal membrane oxygenation (ECMO) support, there is limited information on intermediate-term outcomes. A single-institution retrospective review of 25 consecutive postoperative CHS patients who required ECMO and survived to hospital discharge between January 2003 and June 2008. Survival was estimated by the Kaplan-Meier method. At a median follow-up of 3.3 years (interquartile range: 1.2-5.9 years), there was one death which occurred at six months postsurgery. Kaplan-Meier-estimated survival at three years was 95% (95% confidence interval: 90%-100%). Indications for ECMO included extracorporeal cardiopulmonary resuscitation (48%), systemic hypoxia (4%), postoperative low-cardiac output syndrome (28%), and intraoperative failure to wean off cardiopulmonary bypass (20%). Following ECMO support, 65% of patients had unplanned cardiac reinterventions (three requiring operative interventions, six requiring percutaneous interventions, and four requiring both), and 47% of patients required unplanned hospitalizations. In all, 29% of patients developed neurological deficits and 12% of patients developed chronic respiratory failure. No patients developed renal failure. Overall, systemic ventricular (SV) function normalized in 83% of patients, whereas 17% of patients had persistent mild-to-moderate SV dysfunction. Intermediate-term patient survival of ECMO following CHS is encouraging. However, neurological impairment and unplanned cardiac reinterventions remain significant concerns. Further delineation of risk factors to improve patient outcomes is warranted.

Isovolumic contraction acceleration before and after percutaneous closure of atrial septal defects.

AimTo compare systemic right ventricular function by isovolumic myocardial acceleration before and 6 months after the percutaneous closure of atrial septal defects (ASD).Material and methodsPatients admitted to our tertiary center for the percutaneous closure of atrial septal defects between January 2010 and August 2012 constituted the study group. Right ventricular function of patients was assessed by tissue Doppler echocardiography before and after surgery. Echocardiographic data in patients were compared to age-matched controls without any cardiac pathology and studied in identical fashion mentioned below.ResultsA total of 44 patients (24 males, 20 females) and 44 age-matched controls (25 males, 19 females) met the eligibility criteria for the study. Right ventricular end-diastolic and end-systolic volume, right ventricular end-diastolic diameter measurements on echocardiogram, and pulmonary artery pressures in both pre- and post-ASD groups were significantly higher than in controls. Tricuspid annular plane systolic excursion and isovolumic myocardial acceleration measurements significantly increased after the percutaneous closure of the defect; however, post-ASD measurements were still significantly lower than the controls.ConclusionsAtrial septal defect device closure resulted in a significant increase of isovolumic myocardial acceleration measurements. Tissue Doppler analysis of regional myocardial function offers new insight into myocardial compensatory mechanisms for acute and chronic volume overload of both ventricles.

Clinical experience of more than 2 months usage of extracorporeal membrane oxygenation (Endumo®4000) without circuit exchange

A 5-year-old girl with right atrial isomerism, complete atrioventricular septal defect, hypoplastic left ventricle, double outlet right ventricle, and mixed-type total anomalous pulmonary venous connection with totally occluded left pulmonary veins presented at our center for fenestrated total cavo-pulmonary connection with an extra cardiac conduit at the age of 3 years. Eleven months after the Fontan completion, she developed protein-losing enteropathy (PLE). Spontaneously closed fenestration was thought to be the cause of the PLE, and she underwent revision of fenestration at the age of 5 years. After the operation, PLE did not improve, and newly developed hypoxemia impaired her systemic ventricular function, leading to the initiation of veno-arterial extracorporeal membrane oxygenation (ECMO) with the Endumo(®) system 18 days after the operation to treat her hemodynamic instability. Although the ECMO circuit was changed three times during the first 8 days, the fourth circuit could be used for 74 days without hemolysis and serum leakage, until the patient unfortunately died 82 days after the operation due to multi-organ failure.

Fontan hemodynamics from 100 patient-specific cardiac magnetic resonance studies: A computational fluid dynamics analysis

This study sought to quantify average hemodynamic metrics of the Fontan connection as reference for future investigations, compare connection types (intra-atrial vs extracardiac), and identify functional correlates using computational fluid dynamics in a large patient-specific cohort. Fontan hemodynamics, particularly power losses, are hypothesized to vary considerably among patients with a single ventricle and adversely affect systemic hemodynamics and ventricular function if suboptimal. Fontan connection models were created from cardiac magnetic resonance scans for 100 patients. Phase velocity cardiac magnetic resonance in the aorta, vena cavae, and pulmonary arteries was used to prescribe patient-specific time-averaged flow boundary conditions for computational fluid dynamics with a customized, validated solver. Comparison with 4-dimensional cardiac magnetic resonance velocity data from selected patients was used to provide additional verification of simulations. Indexed Fontan power loss, connection resistance, and hepatic flow distribution were quantified and correlated with systemic patient characteristics. Indexed power loss varied by 2 orders of magnitude, whereas, on average, Fontan resistance was 15% to 20% of published values of pulmonary vascular resistance in single ventricles. A significant inverse relationship was observed between indexed power loss and both systemic venous flow and cardiac index. Comparison by connection type showed no differences between intra-atrial and extracardiac connections. Instead, the least efficient connections revealed adverse consequences from localized Fontan pathway stenosis. Fontan power loss varies from patient to patient, and elevated levels are correlated with lower systemic flow and cardiac index. Fontan connection type does not influence hemodynamic efficiency, but an undersized or stenosed Fontan pathway or pulmonary arteries can be highly dissipative.

Adverse impact of chronic subpulmonary left ventricular pacing on systemic right ventricular function in patients with congenitally corrected transposition of the great arteries

BackgroundPatients with congenitally corrected transposition of the great arteries (ccTGA) are at high risk of heart block requiring subpulmonary left ventricular (LV) pacing. Long-term right ventricular (RV) pacing in congenitally normal hearts is associated with LV dysfunction. We examined the effects of univentricular subpulmonary LV pacing on the systemic RV in a ccTGA cohort. MethodsccTGA patients with two echocardiographic studies at least 6months apart were included. Records of 52 patients, 22 with pacing, were retrospectively reviewed. Seven patients with biventricular pacing were included for comparison. ResultsThe LV-Paced Group experienced deterioration in the RV fractional area change (RVFAC) (28.7±10.0 vs. 21.9±9.1%; P=0.003), systemic atrioventricular valve regurgitation (P=0.019) and RV dilatation (end-diastolic area 32.7±8.7 vs. 37.2±9.0cm2; P=0.004). There was a corresponding deterioration in NYHA class (P=0.013). Multivariate Cox regression analysis showed that pacing was an independent predictor of deteriorating RV function and RV dilation (hazard ratio 2.7(10–7.0) and 4.7(1.1–20.6) respectively). None of these parameters changed significantly in the Un-paced Group. The CRT Group showed improvement in RVFAC (22.0% to 30.7% (P=0.030) and NYHA class (P=0.030), despite having lower baseline RVFAC (22.0±5.7 vs. 31±9.7%; P=0.025) and greater dyssynchrony (RV total isovolumic time 13.4±2.1 vs. 9.3±4.2s/min; P=0.016) when compared to the Un-Paced Group. ConclusionsUniventricular subpulmonary LV pacing in patients with ccTGA predicted deterioration in RV function and RV dilatation over time associated with deteriorating NYHA class. Alternative primary pacing strategies such as biventricular pacing may need consideration in this vulnerable group already highly prone to mortality from systemic RV failure.

Preprocedural Imaging in Patients with Transposition of the Great Arteries Facilitates Placement of Cardiac Resynchronization Therapy Leads

The purpose of this study is to review a series of patients with complex congenital heart disease in whom preprocedural imaging was used to assist placement of cardiac resynchronization therapy (CRT) leads. CRT may be beneficial in patients with a failing systemic ventricle and transposition of the great arteries (TGA). However, complex coronary venous anatomy can be challenging for placement of CRT leads. Between October 2006 and June 2012, seven patients with either dextro-TGA (d-TGA) or levo-TGA (l-TGA) underwent preprocedural imaging prior to placement of CRT leads (three, d-TGA and four, l-TGA). Three patients underwent cardiac computed tomography (CT) and four underwent coronary angiography, which included levophase imaging of the coronary sinus (CS) or direct contrast injection of the CS. Where CS anatomy was appropriate with drainage into the systemic venous circulation, a transvenous approach was planned. In all other cases, the patient was referred for surgical placement of epicardial leads. Seven patients were identified with either d-TGA or l-TGA who had undergone preprocedural imaging prior to placement of CRT leads (three, d-TGA and four, l-TGA). Three patients underwent cardiac CT and four underwent coronary angiography, which included levophase imaging of the CS or direct contrast injection of the CS. All seven patients had successful CRT lead placement guided by preprocedure imaging. Three patients required surgical placement whereas four were able to undergo transvenous placement. There were no complications. The majority of patients (four of seven) had improvement in New York Heart Association class as well as subjective improvement in exercise tolerance and energy. The majority of patients also had subjective improvement in systemic right ventricular function by echocardiogram and objective improvement in fractional area change of the right ventricle. The follow-up period ranged from 13 months to 55 months with a mean follow-up of 39 months. Placement of biventricular leads for CRT in patients with l-TGA or d-TGA is feasible. Preprocedural imaging of the CS allows for better assessment of its anatomy and helps determine procedural approach for CRT placement, thereby limiting unnecessary procedures. In the majority of patients, there was subjective improvement in functional status and right ventricular function; in addition, there was objective improvement in echocardiographic parameters of right ventricular function after CRT placement.

Aerobic training in adults after atrial switch procedure for transposition of the great arteries improves exercise capacity without impairing systemic right ventricular function

BackgroundExercise training safely and efficiently improves symptoms in patients with heart failure due to left ventricular dysfunction. However, studies in congenital heart disease with systemic right ventricle are scarce and results are controversial. In a randomised controlled study we investigated the effect of aerobic exercise training on exercise capacity and systemic right ventricular function in adults with d-transposition of the great arteries after atrial redirection surgery (28.2±3.0years after Mustard procedure). Methods48 patients (31 male, age 29.3±3.4years) were randomly allocated to 24weeks of structured exercise training or usual care. Primary endpoint was the change in maximum oxygen uptake (peak VO2). Secondary endpoints were systemic right ventricular diameters determined by cardiac magnetic resonance imaging (CMR). Data were analysed per intention to treat analysis. ResultsAt baseline peak VO2 was 25.5±4.7ml/kg/min in control and 24.0±5ml/kg/min in the training group (p=0.3). Training significantly improved exercise capacity (treatment effect for peak VO2 3.8ml/kg/min, 95% CI: 1.8 to 5.7; p=0.001), work load (p=0.002), maximum exercise time (p=0.002), and NYHA class (p=0.046). Systemic ventricular function and volumes determined by CMR remained unchanged. None of the patients developed signs of cardiac decompensation or arrhythmias while on exercise training. ConclusionsAerobic exercise training did not detrimentally affect systemic right ventricular function, but significantly improved exercise capacity and heart failure symptoms. Aerobic exercise training can be recommended for patients following atrial redirection surgery to improve exercise capacity and to lessen or prevent heart failure symptoms. (Clinical Trial Registration: ClinicalTrials.gov #NCT00837603)

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Circulating microRNA expression profile and systemic right ventricular function in adults after atrial switch operation for complete transposition of the great arteries

BackgroundData on the use of circulating microRNAs (miRNAs) as biomarkers of cardiovascular diseases are emerging. Little, however, is known on the expression profile of circulating of microRNAs in congenital heart malformations with a systemic right ventricle that is prone to functional impairment. We aimed to test the hypothesis that circulating miRNA profile is altered in patients late after atrial switch operation for complete transposition of the great arteries (TGA) and further explored possible relationships between alteration of circulating miRNAs and systemic ventricular contractility.MethodsCirculating miRNA expression profiling of serum samples from 5 patients and 5 healthy controls was performed. The results were validated in 26 patients and 20 controls using real-time quantitative reverse-transcription polymerase chain reaction for candidate miRNAs with fold changes >3 by expression profiling. Systemic ventricular myocardial acceleration during isovolumic contraction (IVA) was determined by colour tissue Doppler echocardiography.ResultsCompared with controls, patients had significantly lower systemic ventricular IVA (p = 0.002). Of the 23 upregulated miRNAs identified by profiling, 11 were validated to be increased in patients compared with controls: miR-16, miR-106a, miR-144*, miR-18a, miR-25, miR-451, miR-486-3p, miR-486-5p, miR-505*, let-7e and miR-93. Among the validated 11 miRNAs, miR-18a (r = −0.45, p = 0.002) and miR-486-5p (r = −0.35, p = 0.018) correlated negatively with systemic ventricular IVA for the whole cohort.ConclusionsA distinct serum miRNA expression signature exists in adults with complete TGA after atrial switch operation, with serum miR-18a and miR-486-5p being associated with systemic ventricular contractility.

Declining Cardiopulmonary Exercise Capacity Is Not Associated with Worsening Systolic Systemic Ventricular Dysfunction in Adults with Transposition of Great Arteries after Atrial Switch Operation

Cardiopulmonary exercise capacity is often reduced in patients with transposition of the great arteries after atrial switch operation. Reduced exercise capacity may be caused by deterioration of systemic right ventricular function over time. This study analyzed serial changes in systemic right ventricular function and cardiopulmonary exercise capacity in young adults with transposition of the great arteries after atrial redirection surgery. Twenty-one patients (37% female, mean age 23.2 ± 3.3, mean age at surgery 12.8 ± 14 years) with transposition of the great arteries after atrial switch operation were included in this study. Patients were followed up for a mean period of 39.6 ± 13.1 months. Exercise capacity expressed as peak VO2 max, systemic right ventricular function and subpulmonary left ventricular function assessed by cardiac magnetic resonance imaging and NT-proBNP levels were obtained at baseline and follow-up. Changes in peak VO2 max were correlated to changes in cardiac magnetic resonance imaging and NT-proBNP levels. Baseline peak VO2 max decreased significantly (28.31 ± 5.80 mL/kg/min vs. 25.17 ± 5.71 mL/kg/min, P = .005) on follow-up. Cardiac magnetic resonance imaging parameters of systemic right ventricular ejection fraction as well as subpulmonary left ventricular ejection fraction remained unchanged (44.68 ± 6.59% vs. 45.65 ± 9.60%, P = .54, 60.18 ± 6.29% vs. 61.52 ± 5.30%, P = .35). NT-proBNP levels did not increase (211.7 ± 85.7 ng/mL vs. 261.2 ± 182.2 ng/mL, P = .16). After atrial switch operation for transposition of the great arteries we observed a declining functional exercise capacity. This was not associated with worsening systemic right ventricular function, suggesting that other factors are contributing to the decline in physical exercise capacity.

Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality

The population of adults with congenital heart disease (ACHD) is growing and ageing. Limited information about the diagnostic spectrum of this emerging population, its resource utilization at tertiary ACHD centres, and especially about prognostic parameters is available. Retrospective cohort study on all ACHD patients ≥60 years of age under active follow-up. All-cause mortality was the primary outcome measure. Out of a total population of 7315 ACHD patients, 375 [190 females (50.7%), mean age 64.8 ± 5.9 years] fulfilled the inclusion criteria. During a median follow-up of 5.5 (IQR 3.1-8.6) years, 55 of the 375 patients died. The number of interventions (P = 0.0006), the number and length of hospitalization (P < 0.0001), and the number of outpatient clinic visits (P < 0.0001) were significantly higher in patients ≥60 compared with patients aged between 20 and 60 years. Patients ≥60 years of age with moderate or severe congenital heart defects had worse survival prospects than their age- and gender-matched comparison population. On multivariate Cox analysis, coronary artery disease [hazard ratio (HR): 5.04; 95%CI: 1.88-13.51, P = 0.0014], symptoms of heart failure (HR: 2.36; 95%CI: 1.05-5.29, P < 0.05), NYHA class (HR: 1.96; 95%CI: 1.18-3.26, P < 0.01), and moderate to severe reduction in systemic ventricular systolic function (HR: 1.90; 95%CI: 1.20-2.99, P < 0.001) were the strongest prognostic factors. There is a growing number of elderly ACHD patients with high mortality rates and a higher utilization of healthcare resources compared with younger patients. Acquired morbidities, such as coronary artery disease, seem to be key determinants of outcome in this older population in conjunction with the underlying congenital heart disease.

Angiopoietin-2 in Adults with Congenital Heart Disease and Heart Failure

BackgroundChronic heart failure is an important cause for morbidity and mortality in adults with congenital heart disease (ACHD). While NT-proBNP is an established biomarker for heart failure of non-congenital origin, its application in ACHD has limitations. The angiogenic factors Angiopoietin-1 and -2 (Ang-1, Ang-2), vascular endothelial growth factor (VEGF), and soluble receptor tyrosine kinase of the Tie family (sTie2) correlate with disease severity in heart failure of non-congenital origin. Their role in ACHD has not been studied.MethodsIn 91 patients Ang-2 and NT-proBNP were measured and related to New York Heart Association class, systemic ventricular function and parameters of cardiopulmonary exercise testing. Ang-1, VEGF, and sTie2 were also measured.ResultsAng-2 correlates with NYHA class and ventricular dysfunction comparable to NT-proBNP. Further, Ang-2 showed a good correlation with parameters of cardiopulmonary exercise testing. Both, Ang-2 and NT-proBNP identified patients with severely limited cardiopulmonary exercise capacity. Additionally, Ang-2 is elevated in patients with a single ventricle physiology in contrast to NT-proBNP. VEGF, Ang-1, and sTie2 were not correlated with any clinical parameter.ConclusionThe performance of Ang-2 as a biomarker for heart failure in ACHD is comparable to NT-proBNP. Its significant elevation in patients with single ventricle physiology indicates potential in this patient group and warrants further studies.

IS SYSTEMIC RIGHT VENTRICULAR FUNCTION BY CARDIAC MRI RELATED TO THE DEGREE OF TRICUSPID REGURGITATION IN CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES?

Abstract Background and Methods Systemic right ventricular dysfunction and tricuspid regurgitation (TR) are frequently encountered in patients with congenitally corrected transposition of the great arteries (CCTGA). Studies using echocardiography have suggested a relationship between the degree of TR and systemic right ventricular dysfunction; however, assessment of systemic right ventricular function by echocardiography is limited. Cardiac MRI (CMR) is the gold standard for volumetric assessment of the systemic right ventricle. We performed a retrospective cohort study at our center evaluating all adult patients with CCTGA who underwent a CMR between 1/1999 and 1/2013 to determine the relationship between the degree of TR and systemic right ventricular function. Results Of the 33 patients identified, 12 had ≤mild TR (37%), 13 had moderate TR (40%), and 8 had severe TR (24%). Mean age at CMR was 38years (23–64). Mean right ventricular ejection fraction (45% vs. 41% vs. 42%, p =0.68) and mean indexed right ventricular end diastolic volume (122ml/m 2 vs. 136ml/m 2 vs. 138ml/m 2 p =0.36) were not significantly different for patients with ≤mild TR, moderate TR or severe TR. The degree of TR was not associated with additional congenital lesions, prior procedures, presence of an intraventricular conduction delay, or decreased left ventricular function. Conclusion No association between the degree of TR and right ventricular volume or ejection fraction by CMR was identified. Failure to show worsening function or increased volume with greater degrees of TR suggests that the degree of regurgitation alone may not fully explain the heterogeneity in right ventricular size and function.

Correlations between B-type natriuretic peptide levels and nongeometric echocardiographic parameters in pediatric cardiomyopathies

B-type natriuretic peptide is a conventional cardiac biomarker in adult cardiology, however, it is not commonly used in pediatric cases. After introducing B-type natriuretic peptide measurements in pediatric patients for the evaluation of systemic right ventricular function, the authors wanted to evaluate the value of plasma B-type natriuretic peptide and compare the results with systolic and diastolic myocardial parameters obtained with conventional echocardiography and tissue Doppler imaging in children with cardiomyopathy. Between 2007 and 2010, 58 plasma B-type natriuretic peptide measurements were performed in 32 children (dilated cardiomyopathy in 20, hypertrophic cardiomyopathy in 10 and non-compacted cardiomyopathy in 2 cases). The age of the patients was 7.9±6.6 years (mean±SD). Plasma B-type natriuretic peptide was measured using an electrochemiluminescent assay within one day from echocardiographic evaluation. As compared to normal values, children with the 3 types of cardiomyopathies showed significant differences in plasma B-type natriutretic peptide levels (dilated cardiomyopathy vs normal, p<0.001; hypertrophic cardiomyopathy vs. normal, p<0.01; non-compacted cardiomyopathy vs. normal, p<0.001). There was a significant negative correlation (r = -0,63; p<0,01) between B-type natriuretic peptide levels (range, 12-7002 ng/L; mean±SD, 1531±1750 ng/L) and linEF values (range, 4-50%; mean±SD, 22.5±13%). For B-type natriuteric peptide, a cut-off point of 1000 ng/L proved to differentiate significantly decreased linEF values (<17%). B-type natriuretic peptide levels significantly correlated with left ventricular end-diastolic diameter (r = 0.899; p<0.001), with left ventricular anular S wave parameters (r = 0.689; p<0.001) and with E/e ratio (r = 0.43; p<0.05). B-type natriuretic peptide measurements are recommended in all types of cardiomyopathies.

Long-term Outcome and Quality of Life after Arterial Switch Operation: A Prospective Study with a Historical Comparison

AIM.: The study aims to describe the long-term cardiological and psychological results of our first surgical cohort of arterial switch operation (ASO) patients and compare the results with our earlier series of Mustard patients. METHODS.: Twenty-four survivors of ASO operated in our center (1985-1990) were evaluated by electrocardiography, echocardiography, magnetic resonance imaging, exercise testing, 24-hour Holter-monitoring, and health-related quality of life questionnaire. The results were compared with 58 adult Mustard patients who were evaluated in 2001 using the same study protocol. RESULTS.: Arterial switch operation was performed at a median age of 13 days and Mustard operation at 2 years. Median follow-up was 22 years (range 20-25) and 25 years (22-29), respectively. After ASO, survival was better (P =.04). The event-free survival after 22 years was 77% after ASO vs. 44% after Mustard (P =.03). Good systemic ventricular function was present in 93% after ASO vs. 6% after Mustard (P <.01). Exercise capacity in ASO was 85% of predicted, compared with 72% in Mustard patients (P =.01). Aortic regurgitation was found in 21% of ASO patients vs. 16% in Mustard patients. Arterial switch patients vs. Mustard patients reported significantly better quality of life and less somatic complaints. CONCLUSION.: The progression made in surgical treatment for transposition of the great arteries from Mustard to ASO has had a positive impact on survival, cardiac function, exercise capacity, and also self-reported quality of life and somatic complaints. Longer follow-up is warranted to monitor aortic regurgitation.

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