natriuretic-peptide--brain and Transposition-of-Great-Vessels

The aim of the present study is to determine the long-term effects of a ten-week exercise training program in adult patients with a systemic right ventricle.. All patients who participated in a 2009 randomized controlled trial were approached. At approximately three years of follow-up from initial baseline, patients underwent cardiopulmonary exercise testing, filled out two quality of life questionnaires, and NT proBNP levels were measured. All examinations were performed according to the protocols of the 2009 trial. In addition, patients were asked about their current sports habits.. Of the 54 patients who were randomized in the 2009-trial 40 participated in the current re-evaluation (male 50%, ccTGA 35%, age 36 ± 10 years, intervention group n=22, control group n=18). After three years, no persistent effect of exercise training on V'O₂peak training remained (-2% of predicted, 95% CI -3% to 5%; p=.56). However, patients who already participated in regular sports or exercise at baseline (n=23/40 (58%)) showed higher V'O₂peak of 13% of predicted (95% CI 4% to 23%; p>.01) and a decrease of 62% in plasma NT-proBNP (95% CI -115% to -10%; p>.03) during follow-up, when compared to patients who did not. Moreover, sports were associated with a lower incidence of clinical events (p=.032).. Short-term beneficial effects of exercise training did not persist over a three-year follow-up period. However, sports participation at baseline was associated with better exercise capacity, lower neurohormone levels, and increased event-free survival.

Topics: Adult; Biomarkers; Cross-Sectional Studies; Exercise Test; Exercise Therapy; Female; Follow-Up Studies; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Quality of Life; Sports; Surveys and Questionnaires; Survival Analysis; Transposition of Great Vessels; Treatment Outcome; Ventricular Dysfunction, Right

B-type natriuretic peptide (BNP) is an established marker for heart failure assessment, but the prognostic quality of BNP after atrial switch operation (ASO) has not yet been elucidated.. In 89 patients (median age, 24 years; range, 15-35 years) after ASO, BNP was measured. During a 48-months follow-up we focused on critical cardiac events, defined as decompensation, sudden cardiac death or need for heart transplantation. BNP was considerably lower in 81 patients in functional class (FC) I/II (median, 35 pg/ml; range, 3-586 pg/ml) than in 6 patients in FC III/IV (median, 246 pg/ml; range, 14-1,150 pg/ml, P≤0.073). BNP was significantly higher after Mustard than after Senning procedure (P≤0.030). There was no significant difference in BNP between simple or complex transposition of the great arteries (TGA) (P≤0.44). Eleven subjects (13%, 95% CI: 7-22%) had a critical cardiac event within 48 months. On ROC analysis BNP had a high predictive value regarding discrimination of patients with and without critical events (area under the ROC curve, 0.90; 95% CI: 0.76 to >0.99, P<0.001). The cut-off was 85 pg/ml (sensitivity, 88%; specificity, 85%). Additionally, estimated event-free-survival was longer after Senning than after Mustard procedure (P≤0.017). There was no significant difference in outcome between patients with simple or complex TGA with regard to occurrence of critical events.. BNP is a sensitive and specific prognostic marker for critical cardiac events after ASO.

Topics: Adolescent; Adult; Cardiac Surgical Procedures; Disease-Free Survival; Female; Follow-Up Studies; Heart Ventricles; Humans; Male; Natriuretic Peptide, Brain; Survival Rate; Transposition of Great Vessels

To assess whether exercise training in adult patients with a systemic right ventricle (RV) improves exercise capacity and quality of life and lowers serum N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels.. Multi-centre parallel randomized controlled trial.. Patients with a systemic RV due to congenitally or surgically corrected transposition of the great arteries.. Fifty-four adult patients with a systemic RV, were randomized using unmarked opaque envelopes to an intervention group (n = 28) with three training sessions per week for 10 consecutive weeks, and a control group (n = 26). Randomization was stratified by participating centre. At baseline, and follow-up, we determined maximal exercise capacity (V'O(2peak)), serum NT-proBNP levels, and quality of life by means of the SF-36, and the TAAQOL Congenital Heart Disease questionnaires. The final analysis was performed by linear regression, taking into account the stratified randomization.. Forty-six patients were analysed (male 50%, age 32 ± 11 years, intervention group n = 24, control group n = 22). Analysis at 10 weeks showed a significant difference in V'O(2peak) (3.4 mL/kg/min, 95% CI: 0.2 to 6.7; P = 0.04) and resting systolic blood pressure (-7.6 mmHg, 95% CI: -14.0 to -1.3; P = 0.03) in favour of the exercise group. No significant changes were found in serum NT-proBNP levels or quality of life in the intervention group or in the control group nor between groups. None of the patients in the intervention group had to discontinue the training programme due to adverse events.. In adult patients with a systemic RV exercise training improve exercise capacity. We recommend to revise restrictive guidelines, and to encourage patients to become physically active. (. The study was registered at http://trialregister.nl. Identifier: NTR1909.).

Topics: Adult; Congenitally Corrected Transposition of the Great Arteries; Exercise Therapy; Exercise Tolerance; Female; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Prospective Studies; Quality of Life; Transposition of Great Vessels; Treatment Outcome; Ventricular Dysfunction, Right; Young Adult

Pharmacological blockade of the renin-angiotensin system improves exercise tolerance in patients with left ventricular dysfunction, yet its impact on patients with systemic right ventricles (RVs) remains unknown.. A multicenter, randomized, double-blind, placebo-controlled, crossover clinical trial was performed to assess the effects of losartan on exercise capacity and neurohormonal levels in patients with systemic RVs. Of 29 patients studied (age, 30.3+/-10.9 years), 21 had transposition of the great arteries with a Mustard baffle, and 8 had congenitally corrected transposition of the great arteries. Baseline values were as follows: VO2max, 29.8+/-5.6 mL.kg(-1).min(-1) (73.5+/-12.9% predicted value); RV ejection fraction, 41.6+/-9.3%; N-terminal pro brain natriuretic peptide (NT-proBNP), 257.7+/-243.4 pg/mL (normal <125 pg/mL); and angiotensin II, 5.7+/-4.9 pg/mL (normal <5.0 pg/mL). Comparing losartan to placebo showed no differences in VO2max (29.9+/-5.4 versus 29.4+/-6.2 mL.kg(-1).min(-1); P=0.43), exercise duration (632.3+/-123.0 versus 629.9+/-140.7 seconds; P=0.76), and NT-proBNP levels (201.2+/-267.8 versus 229.7+/-291.5 pg/mL; P=0.10), despite a trend toward increased angiotensin II levels (15.2+/-13.8 versus 8.8+/-12.5 pg/mL; P=0.08).. In adults with systemic RVs, losartan did not improve exercise capacity or reduce NT-proBNP levels. Minimal baseline activation of the renin-angiotensin system may explain this lack of benefit and imply an alternative pathophysiological mechanism for the progressive ventricular dysfunction and impaired exercise capacity observed in such patients.

Topics: Adult; Angiotensin II; Angiotensin II Type 1 Receptor Blockers; Cross-Over Studies; Double-Blind Method; Echocardiography, Transesophageal; Epinephrine; Exercise; Humans; Losartan; Natriuretic Peptide, Brain; Norepinephrine; Oxygen Consumption; Peptide Fragments; Transposition of Great Vessels; Treatment Outcome; Ventricular Dysfunction, Right

Background Adults with a systemic right ventricle (sRV) have a high risk of cardiac complications. This study aimed to identify prognostic markers in adults with sRV based on clinical evaluation, echocardiography, and blood biomarkers. Methods and Results In this prospective cohort study, consecutive clinically stable adults with sRV caused by Mustard- or congenitally corrected transposition of the great arteries were included (2011-2013). Eighty-six patients were included (age 37±9 years, 65% male, 83% New York Heart Association functional class I, 76% Mustard transposition of the great arteries, 24% congenitally corrected transposition of the great arteries). Venous blood sampling was performed including N-terminal pro B-type natriuretic peptide, high-sensitive-troponin-T, high-sensitivity C-reactive protein, growth differentiation factor-15, galectin-3, red cell distribution width, estimated glomerular filtration rate, and hemoglobin. Besides conventional echocardiographic measurements, longitudinal, circumferential, and radial strain were assessed using strain analysis. During a median follow-up of 5.9 (interquartile range 5.3-6.3) years, 19 (22%) patients died or had heart failure (primary end point) and 29 (34%) patients died or had arrhythmia (secondary end point). Univariable Cox regression analysis was performed using dichotomous or standardized continuous variables. New York Heart Association functional class >I, systolic blood pressure, and most blood biomarkers were associated with the primary and secondary end point (galectin-3 not for primary, N-terminal pro B-type natriuretic peptide and high-sensitivity C-reactive protein not for secondary end point). Growth differentiation factor-15 showed the strongest association with both end points (hazard ratios; 2.44 [95% CI 1.67-3.57, P<0.001], 2.00 [95% CI 1.46-2.73, P<0.001], respectively). End-diastolic basal dimension of the subpulmonary ventricle was associated with both end points (hazard ratio: 1.95 [95% CI 1.34-2.85], P<0.001, 1.70 [95% CI 1.21-2.38, P=0.002], respectively). Concerning strain analysis, only sRV septal strain was associated with the secondary end point (hazard ratio 0.58 [95% CI 0.39-0.86], P=0.006). Conclusions Clinical, conventional echocardiographic, and blood measurements are important markers for risk stratification in adults with a sRV. The value of novel echocardiographic strain analysis seems limited.

Topics: Adult; Arrhythmias, Cardiac; Arterial Switch Operation; Biomarkers; Blood Proteins; C-Reactive Protein; Congenitally Corrected Transposition of the Great Arteries; Echocardiography; Female; Galectin 3; Galectins; Growth Differentiation Factor 15; Heart Failure; Humans; Longitudinal Studies; Male; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Predictive Value of Tests; Progression-Free Survival; Prospective Studies; Risk Assessment; Risk Factors; Time Factors; Transposition of Great Vessels; Troponin T; Ventricular Function, Right

Dextro-transposition of the great arteries (d-TGA) patients is at high risk of developing right ventricular dysfunction and tricuspid regurgitation in adulthood. Determining the relation between echocardiographic parameters, N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels and the New York Heart Association (NYHA) functional class may help determining the best time to operate them.. Patients with simple d-TGA operated in infancy with an atrial switch procedure (Mustard or Senning operation) were followed up in our Adult Congenital Heart Disease Unit. Analytical, echocardiographic, and clinical parameters were determined to evaluate the correlation between right echocardiographic ventricular function, NT-pro-BNP levels, and NYHA functional class.. Twenty-four patients with d-TGA were operated in infancy of whom 17 alive patients had simple d-TGA. Nine patients had NT-pro-BNP levels lower than 200 pg/mL and eight patients were above 200 pg/mL. Patients with lower hemoglobin concentration, higher right ventricular diameter or under diuretic treatment showed significant higher NT-pro-BNP levels (above 200 pg/dL). The Spearman test showed a positive correlation between basal right ventricular diameter and tricuspid regurgitation with pro NT BNP levels (correlation coefficient of .624; P=.017 and .490; P=.046, respectively) and a negative correlation with the right ventricle fractional area change (-.508, P=.045). No correlation was seen between NT-pro-BNP levels and the rest of echocardiographic parameters or the NYHA functional class.. NT-pro-BNP levels showed a positive correlation with basal right ventricular diameter and tricuspid regurgitation but not with NYHA association functional class in d-TGA patients.

Topics: Adult; Echocardiography; Female; Heart Ventricles; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Transposition of Great Vessels; Ventricular Dysfunction, Right

The patients after Mustard and Senning corrections of transposition of the great arteries (TGA) are at an increased risk of unexpected death. The aim of this study was to identify markers allowing risk stratification of patients after atrial switch correction of TGA to provide them with optimum care.. In this study, 87 patients were retrospectively evaluated after atrial switch correction of TGA followed-up between 2005 and 2015. The mortality during the follow-up was 9% (8 cardiac deaths). Markers significantly predictive of death using univariable Cox proportional hazard ratio survival analysis were: N-terminal pro-B-type natriuretic peptide (NT-proBNP), ejection fraction and end-diastolic dimension of the systemic right ventricle, mitral E, e', and s'. Surprisingly, the Doppler parameters of mitral valve in subpulmonary ventricle were more important for prognosis than those of systemic tricuspid valve. In multivariable analysis, the only independent predictors of mortality were NT-proBNP (P = .00048; AUC 0.97) and the velocity of early diastolic filling (mitral E) in subpulmonary ventricle (P = .01815; AUC 0.81). According to Kaplan-Meier survival analysis, patients with NT-proBNP > 1000 pg/ml are at high risk of death. Patients with mitral E < 68 cm/s are also at an increased risk of death.. NT-proBNP is the most reliable prognostic mortality factor and should be measured regularly in TGA patients after Mustard or Senning correction. Diastolic filling velocity of the subpulmonary left ventricle (mitral E) may be more important for prognosis than systolic function of the systemic right ventricle.

Topics: Adult; Biomarkers; Cardiac Surgical Procedures; Czech Republic; Echocardiography, Doppler; Female; Follow-Up Studies; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Postoperative Period; Prognosis; Retrospective Studies; Risk Assessment; Risk Factors; Survival Rate; Time Factors; Transposition of Great Vessels; Young Adult

The aims of our study were to describe plasma brain natriuretic peptide (BNP), Troponin I (TnI), and Cystatin C (Cys-C) concentration kinetics in the postoperative period after arterial switch operation in neonate, and to test the correlation between the plasma biomarkers and early clinical outcomes. We prospectively enrolled 29 neonates who underwent ASO. All patients received Custodiol cardioplegia. Blood samples were collected preoperatively (one day before) and in the ICU immediately after admission, and then 6, 12, 24, and 48 h after surgery. TnI peak (mean 17.23 ± 7.0 ng/mL) occurred between the arrival in the ICU and the 6th hour, then we had a constant decrease. TnI had a good correlation with the inotropic support time (r = 0.560, p = 0.0015) and ICU time (r = 0.407, p = 0.028), less than with ventilation and Hospital stay (r = 0.37, p = 0.0451 and r = 0.385, p = 0.0404). BNP peak (mean 4773.79 ± 2724.52 ng/L) was in the preoperative time with a constant decrease after the operation and it had no significant correlations with clinical outcomes. The CyS-C had the highest preoperative values, which decreased during the operating phase, and then constantly increased upon arrival to the ICU with a peak at 48 h (mean 1.76 ± 0.35 mg/L). CyS-C peak had a good correlation with a plasmatic creatinine peak (r = 0.579, p = 0.0009) but not with other clinical outcomes. Our study demonstrated significant correlations between the Tnl peak and early clinical outcomes in neonates undergoing arterial switch operation. Other plasma biomarkers such as the BNP and CyS-C had no direct correlation.

Topics: Arterial Switch Operation; Biomarkers; Cystatin C; Female; Humans; Infant, Newborn; Male; Natriuretic Peptide, Brain; Postoperative Period; Predictive Value of Tests; Prospective Studies; Transposition of Great Vessels; Treatment Outcome; Troponin I

Myocardial fibrosis is a potential pathophysiological mechanism leading to systemic right ventricular (SRV) deterioration. We hypothesize that circulating levels of collagen deposition markers are elevated in patients with SRV remodeling and this elevation may have a predictive value.. We prospectively evaluated 56 patients with D-TGA after the atrial switch procedure (mean age 25.6 ± 4.8, range 18-37 years; 67% males). Serum levels of procollagen type III amino-terminal propeptide (PIIINP), collagen type I carboxy-terminal telopeptide (CITP), procollagen type I N-terminal propeptide (PINP), matrix metalloproteinase (MMP 1, MMP 9) and a tissue inhibitor of matrix metalloproteinase (TIMP 1) and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) were measured and compared with healthy controls. The relationship between these serum markers, echocardiographic and cardiac magnetic resonance parameters and the outcome at a follow-up of 61 months (range, 24-85 months) was determined.. Compared with the healthy control group, the study group had significantly higher levels of TIMP1, PIIINP, CITP, PINP and NT-pro-BNP (p<0.05, each). The levels of PIIINP and CITP were significantly higher among patients with an SRV mass index above the mean value. The level of PIIINP was significantly higher among patients with an SRV EDV index above the mean value. CITP was significantly elevated in SRV late gadolinium enhanced (LGE) positive patients, compared to patients without SRV LGE. MMP9 and TIMP1 predicted an adverse clinical outcome on univariate Cox proportional hazard survival analysis in addition to well proven predictors of outcome (SRV EF and NYHA).. We demonstrated a pattern of altered collagen turnover adversely related with the indices of SRV remodeling and an adverse clinical outcome in patients with SRV.

Topics: Adolescent; Adult; Arterial Switch Operation; Biomarkers; Case-Control Studies; Cross-Sectional Studies; Female; Follow-Up Studies; Humans; Male; Matrix Metalloproteinase 9; Natriuretic Peptide, Brain; Peptide Fragments; Procollagen; Proportional Hazards Models; Prospective Studies; Tissue Inhibitor of Metalloproteinase-1; Transposition of Great Vessels; Ventricular Remodeling; Young Adult

In congenitally corrected transposition of the great arteries the right ventricle (RV) supports systemic circulation, and patients are prone to develop heart failure over time. Chronic volume overload secondary to obesity may contribute to premature dysfunction of the systemic RV.. The aim of our study was to assess the systemic RV function in overweight/obese adult patients with congenitally corrected transposition of the great arteries.. Transthoracic echocardiographic studies and laboratory testing (N-terminal pro-B-type natriuretic peptide [NT-proBNP] assessment) were performed in patients with congenitally corrected transposition, who were scheduled for a routine examination, and the body mass index was calculated for each patient.. We studied 56 adults (31 men; mean age 33.9 years); 22 of whom were overweight (body mass index [BMI] of 25-29.9 kg/m²) or obese (BMI of 30 kg/m² or more), and 34 of whom were normal weight (BMI below 25 kg/m²). Age, gender, heart rate, and blood pressure were similar in both groups. The mean NT-proBNP levels were not significantly different. On echocardiography, the overweight/obese patients had a decreased systemic RV fractional area change (0.38) compared to normal weight patients (0.43); p = 0.02. Moreover, a significant reduction in the global longitudinal strain in the overweight/obese group was observed (-15.3% vs. -18.3%; p = 0.01).. Overweight/obesity in adult patients with congenitally corrected transposition of the great arteries is associated with impaired systemic RV function.

Topics: Adult; Cross-Sectional Studies; Echocardiography; Female; Humans; Male; Middle Aged; Natriuretic Peptide, Brain; Overweight; Peptide Fragments; Transposition of Great Vessels; Ventricular Dysfunction, Right; Young Adult

Late systolic dysfunction of the systemic right ventricle (RV) in patients with transposition of the great arteries (TGA) is of major concern. Right ventricular global longitudinal strain (GLS) might be able to identify early dysfunction.. Adults with TGA after Mustard operation (TGA-Mustard) or congenitally corrected-TGA (ccTGA) underwent echocardiography, electrocardiography, and NT-proBNP measurement. Using speckle-tracking echocardiography, we analysed longitudinal strain and strain rate, and compared findings in both patients groups, to healthy controls and with clinical parameters. We included 42 patients (mean age 37 ± 7 years, 69% male) with a systemic RV [32 TGA-Mustard (34 ± 4 years after corrective surgery) and 10 ccTGA], and 32 healthy controls (mean age 36 ± 11 years). Global longitudinal strain of the systemic RV was lower in patients than GLS of the systemic LV in controls (-14.2 ± 3.5 vs. -20.0 ± 3.0%, P < 0.001). Average LS of the RV lateral wall was lower in patients with TGA-Mustard (-15.5 ± 3.4%) than ccTGA (-18.3 ± 3.6%, P = 0.047). Right ventricular GLS tended to be lower in patients in NYHA class II than I, and correlated with NT-proBNP (r = 0.49, P = 0.001), RV fractional area change (r = -0.39, P = 0.019), RV apex-base-diameter (r = 0.37, P = 0.021), and QRS duration (r = 0.41, P = 0.014).. Global longitudinal strain of the systemic RV in patients is lower than GLS of the systemic LV in healthy controls, especially in the apical segment, and tended to be lower in TGA-Mustard than ccTGA patients. Since RV GLS correlates with RV function, myocardial deformation is useful as a more quantitative tool to measure systemic RV function. Decreased GLS was associated with elevated NT-proBNP and tended to correlate with worsening NYHA class, which strengthens the potential prognostic value of GLS in patients with a systemic RV.

Topics: Adult; Biomarkers; Congenitally Corrected Transposition of the Great Arteries; Echocardiography; Female; Humans; Male; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Predictive Value of Tests; Prognosis; Prospective Studies; Risk Assessment; Risk Factors; Sensitivity and Specificity; Systole; Transposition of Great Vessels; Ventricular Dysfunction, Right

Transposition of the great arteries (TGA) requires early surgical repair during the neonatal period. Several preoperative factors have been identified for the postoperative poor outcome after arterial switch operation (ASO). However, the data remain uncertain an association. Therefore, we investigated the preoperative factors which affect the early postoperative outcomes. Between March 2005 and May 2012, a retrospective study was performed which included 126 infants with an ASO for TGA. Preoperative data included the vasoactive inotropic score (VIS) and baseline hemodynamics. Early postoperative outcomes included the duration of mechanical ventilation, the length of stay in the intensive care unit and hospital, and early mortality. Multivariate linear regression and receiver operating characteristics analysis were performed. The duration of mechanical ventilation was significantly correlated with the preoperative mechanical ventilator support and VIS, and CPB time. On multivariate linear regression analysis, a higher preoperative VIS, preoperative B-type natriuretic peptide (BNP) level, and the CPB time were identified as independent risk factors for delayed mechanical ventilation. Preoperative VIS (OR 1.154, 95 % CI 1.024-1.300) and the CPB time (OR 1.034, 95 % CI 1.009-1.060) were independent parameters predicting early mortality. A preoperative VIS of 12.5 had the best combined sensitivity (83.3 %) and specificity (85.3 %) and an AUC of 0.852 (95 % CI 0.642-1.061) predicted early mortality. Our results suggest that preoperative VIS and BNP can predict the need for prolonged postoperative mechanical ventilation. Moreover, preoperative VIS may be used as a simple and feasible indicator for predicting early mortality.

Topics: Cardiotonic Agents; Female; Humans; Infant; Infant, Newborn; Intensive Care Units; Length of Stay; Male; Myocardial Contraction; Natriuretic Peptide, Brain; Postoperative Complications; Postoperative Period; Predictive Value of Tests; Preoperative Period; Prognosis; Retrospective Studies; Risk Factors; Severity of Illness Index; Transposition of Great Vessels; Treatment Outcome

The aim of the study was to investigate serum NT-proBNP levels in adult patients with transposition of the great arteries (d-TGA) corrected by atrial switch procedures (Mustard or Senning) operation and to assess the relationship with ventricular impairment and NYHA class.. Serum NT-proBNP levels were measured in a group of 81 consecutive adult patients (59 males, mean age 27 years and 22 females, mean age 28 years) with transposition of the great arteries (TGA) after surgical correction in childhood, and in a control group of 25 healthy individuals (16 males, mean age 32 years, and 9 females, mean age 29 years). Age-matched correlation of NT-proBNP concentrations in TGA patients after Mustard or Senning correction was performed, but this correlation was considered not significant (p=0.08).. Concentrations of NT-proBNP in patients with TGA were significantly elevated compared to the control group of healthy individuals (203 ng/L vs. 41 ng/L, p<0.0001). Patients after the Mustard repair had significantly higher NT-proBNP values than patients after the Senning operation (234 ng/L vs. 148 ng/L, p=0.0023). NT-proBNP correlated negatively with the systemic right ventricular ejection fraction with the greatest significance in patients after Mustard correction (r=-0.32, p<0.0001). The concentration of NT-proBNP was also associated with NYHA functional class (p=0.0035) with the greatest significance in patients with Mustard correction (p=0.028).. Elevated levels of NT-proBNP appear to be a useful tool in assessing heart failure in patients with transposition of the great arteries after atrial switch correction.

Topics: Adult; Arterial Switch Operation; Female; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Transposition of Great Vessels; Ventricular Dysfunction

To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair.. This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age <15 years, and were evaluated in-hospital every 10 years. Survival status was obtained of 86 patients. Median follow-up was 35 (IQR 34-38) years. Cumulative survival was 84% after 10 years, 80% after 20 years, 77% after 30 years, and 68% after 39 years. Cumulative survival free of events (i.e. heart transplantation, arrhythmias, reintervention, and heart failure) was 19% after 39 years. Reinterventions were mainly required for baffle-related problems. Supraventricular and ventricular arrhythmias occurred in 28 and 6% of the patients, respectively. Pacemaker and/or ICD implantation was performed in 39%. Fifty survivors participated in the current in-hospital investigation including electrocardiography, 2D-echocardiography, cardiopulmonary-exercise testing, NT-proBNP measurement, Holter monitoring, and cardiac magnetic resonance. Right ventricular systolic function was impaired in all but one patient at last follow-up, and 14% developed heart failure in the last decade. NT-proBNP levels [median 31.6 (IQR 22.3-53.2) pmol/L] were elevated in 92% of the patients. Early postoperative arrhythmias were a predictor for late arrhythmias [HR 3.8 (95% CI 1.5-9.5)], and development of heart failure [HR 8.1 (95% CI 2.2-30.7)]. Also older age at operation was a predictor for heart failure [HR 1.26 (95% CI 1.0-1.6)].. Long-term survival after Mustard repair is clearly diminished and morbidity is substantial. Early postoperative arrhythmias are a predictor for heart failure and late arrhythmias.

Topics: Adult; Arrhythmias, Cardiac; Biomarkers; Cardiac Surgical Procedures; Child, Preschool; Echocardiography; Electrocardiography; Electrocardiography, Ambulatory; Epidemiologic Methods; Exercise Test; Female; Heart Failure; Humans; Infant; Magnetic Resonance Angiography; Male; Natriuretic Peptide, Brain; Peptide Fragments; Postoperative Complications; Reoperation; Transposition of Great Vessels; Ventricular Dysfunction, Right

Topics: Adolescent; Adult; Biomarkers; Cardiac Surgical Procedures; Child; Child, Preschool; Congenitally Corrected Transposition of the Great Arteries; Hemodynamics; Humans; Infant; Natriuretic Peptide, Brain; Pilot Projects; Prospective Studies; Pulmonary Artery; Risk Factors; Time Factors; Transposition of Great Vessels; Treatment Outcome; Ultrasonography; Up-Regulation; Ventricular Function, Left; Young Adult

Two decades after surgery for transposition of the great arteries, the clinical status, cardiac function, cardiorespiratory performance, and neurohormonal activity of patients who underwent either atrial switch (Mustard) operations or arterial switch operations (ASOs) were compared. Sixty-two patients with simple transposition of the great arteries who underwent either Mustard (n = 34) or ASO (n = 28) procedures were included in this cross-sectional study. Following the same study protocol, clinical workup including echocardiography, stress testing, and blood work was completed for all patients. Mean ages in the 2 groups were comparable, at 20.6 ± 2.1 and 20.6 ± 3.4 years in the ASO and Mustard groups, respectively. All ASO patients were in New York Heart Association class I, whereas 59% of Mustard patients were in class II or III. Peak oxygen uptake was higher in ASO patients (percentage of predicted 80% vs 69%, p <0.01). Compared with healthy subjects, the mean Tei index for systemic ventricle was high in the 2 groups, but this parameter was significantly higher in Mustard than ASO patients (0.60 ± 0.16 vs 0.47 ± 0.14, p <0.01). The median plasma N-terminal pro-brain natriuretic peptide level in ASO patients was within the normal range, but the Mustard group had significantly higher levels (42 ng/ml [range 18 to 323] vs 172 ng/ml [range 26 to 1,018], p <0.0001). In conclusion, this cross-sectional assessment 2 decades after surgery reveals better clinical status in patients who underwent ASO compared with Mustard patients. This holds in terms of cardiac function, cardiorespiratory performance, and neurohormonal activity.

Topics: Cardiac Surgical Procedures; Cross-Sectional Studies; Female; Follow-Up Studies; Germany; Humans; Male; Natriuretic Peptide, Brain; Prognosis; Retrospective Studies; Survival Rate; Time Factors; Transposition of Great Vessels; Young Adult

Topics: Adult; Arginine; Female; Heart Atria; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Postoperative Complications; Transposition of Great Vessels; Ventricular Dysfunction

B-type natriuretic peptide is used in the diagnosis, risk stratification, and management of adult patients with cardiac disease. However, its use in infants with congenital heart disease has been limited, particularly in the perioperative period. Our objective was to determine the alterations in perioperative B-type natriuretic peptide levels and their predictive value on postoperative outcomes, in infants undergoing congenital heart surgery.. We prospectively enrolled 115 patients: 24 with univentricular heart disease undergoing a modified Norwood procedure, 11 with d-transposition of the great arteries, 55 with hemodynamically important left-to-right shunt, and 25 with tetralogy of Fallot undergoing primary repair. Clinical data and B-type natriuretic peptide samples were collected before and 2, 12, and 24 hours after cardiopulmonary bypass. Univariate analysis and multivariate linear regression analysis were performed.. The perioperative B-type natriuretic peptide levels were lesion specific. Patients with d-transposition of the great arteries and univentricular heart disease had high preoperative B-type natriuretic peptide levels that decreased postoperatively, and those with hemodynamically important left-to-right shunts and tetralogy of Fallot had lower preoperative levels that increased during the first 12 hours postoperatively. The patients with univentricular heart disease with an adverse outcome had a significantly greater 24-hour B-type natriuretic peptide level than those without (P < .05). Those with hemodynamically important left to right shunts and an adverse outcome had a greater 12-hour B-type natriuretic peptide level than those without (P < .05). A 12-hour postoperative/preoperative ratio greater than 45 was 100% sensitive and 82% specific for an adverse outcome in the patients with tetralogy of Fallot.. The perioperative changes in B-type natriuretic peptide levels and their ability to predict outcomes are lesion-specific. Characterization of these changes might be useful in caring for infants after congenital heart surgery.

Topics: Biomarkers; Cardiac Surgical Procedures; Female; Heart Defects, Congenital; Heart Ventricles; Hemodynamics; Humans; Infant; Infant, Newborn; Lactic Acid; Male; Multivariate Analysis; Natriuretic Peptide, Brain; Norwood Procedures; Perioperative Period; Predictive Value of Tests; Prospective Studies; Risk Factors; Sensitivity and Specificity; Tetralogy of Fallot; Time Factors; Transposition of Great Vessels; Treatment Outcome

After atrial redirection surgery (Mustard-Senning operations) for transposition of the great arteries (TGA), the systemic right ventricle (RV) suffers from late systolic failure with high morbidity and mortality. Mechanisms of late RV failure are poorly characterized. We hypothesized that diffuse interstitial expansion representing diffuse fibrosis is greater in systemic RVs of patients following Mustard-Senning surgery and that it would be associated with other markers of heart failure and disease severity.. We used equilibrium contrast cardiovascular magnetic resonance (CMR) imaging to quantify extracellular volume (ECV) in the septum and RV free wall of 14 adults presenting to a specialist clinic late after surgery for TGA (8 Mustard, 6 female, median age 33). These were compared with 14 age-and sex-matched healthy volunteers. Patients were assessed with a standardized CMR protocol, NT-brain natriuretic peptide (NT-proBNP), and cardiopulmonary exercise (CPEX) testing. The mean septal ECV was significantly higher in patients than controls (0.254 ± 0.036 vs. 0.230 ± 0.032; P = 0.03). NT-proBNP positively related to septal ECV (P = 0.04; r = 0.55). The chronotropic index (CI) during CPEX testing negatively related to the ECV (P = 0.04; r = -0.58). No relationship was seen with other CMR or CPEX parameters. R.V free wall ECV was difficult to measure (heavy trabeculation, sternal wires, blood pool in regions of interest) with high and poor inter-observer reproducibility: this analysis was abandoned.. Septal interstitial expansion is seen in adults late after atrial redirection surgery for TGA. It correlates well with NT-proBNP and CI and may have a role in the development of RV systolic impairment. Measuring interstitial expansion in the RV free wall is difficult using this methodology.

Topics: Adult; Analysis of Variance; Cardiac Surgical Procedures; Cardiomyopathies; Case-Control Studies; Cohort Studies; Contrast Media; Exercise Test; Female; Fibrosis; Follow-Up Studies; Gadolinium; Heart Ventricles; Humans; Image Enhancement; Magnetic Resonance Imaging, Cine; Male; Natriuretic Peptide, Brain; Peptide Fragments; Reference Values; Severity of Illness Index; Statistics, Nonparametric; Transposition of Great Vessels; Ultrasonography; Ventricular Dysfunction, Right

The usefulness of natriuretic peptides to assess myocardial function in patients with a systemic morphological right ventricle is still unclear. In this report we describe the clinical course of a young woman with congenitally corrected transposition of the great arteries (ccTGA) who suffered from a progressive deterioration of myocardial function after child birth despite intensive medical treatment and additional cardiac resynchronization therapy. In this woman, serial measurement of NT-proBNP levels was related to the velocity time integral over the aortic valve and indicated worsening of the patient's haemodynamic status and finally death.

Topics: Adult; Biomarkers; Defibrillators, Implantable; Disease Progression; Fatal Outcome; Female; Humans; Natriuretic Peptide, Brain; Peptide Fragments; Pregnancy; Pregnancy Complications; Prognosis; Transposition of Great Vessels; Treatment Outcome; Ultrasonography

Heart failure is common late after Senning or Mustard palliation of transposition of the great arteries (TGA). Although cardiac magnetic resonance (CMR) is the gold standard for evaluating systemic right ventricular performance, additional information regarding heart failure status might be gleaned from the surface ECG and circulating N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. The interrelationships between these heart failure markers were examined in adults late after Mustard and Senning surgery.. Thirty-five consecutive adults with Senning or Mustard repair of TGA attending a dedicated congenital heart failure clinic were studied. Assessment included symptom assessment, venous blood sampling for measurement of circulating NT-proBNP levels, surface 12-lead ECG and CMR for the assessment of right ventricular systolic function and determination of indexed right ventricular volumes.. Mean age was 29 ± 6.5 years, 54% had undergone Mustard surgery. Compared with those with uncomplicated surgery, patients with complex surgical history had higher NT-proBNP levels (55 ± 26 vs 20 ± 35 pmol/l; p=0.002) and longer QRS duration (116 ± 28 ms vs 89 ± 11 ms; p=0.0004) while showing no difference in New York Heart Association class and right ventricular function. There was a significant relationship between diastolic and systolic right ventricular volumes and both NT-proBNP levels (r=0.43, p=0.01; r=0.53, p=0.001, respectively) and QRS duration (r=0.47, p=0.004; r=0.53, p=0.001, respectively).. Circulating NT-proBNP levels and several surface ECG parameters constitute safe, cost-effective and widely available surrogate markers of systemic right ventricular function and provide additional information on heart failure status. Both measures hold promise as prognostic markers and their association with long-term outcome should be determined.

Topics: Adult; Biomarkers; Electrocardiography; Female; Humans; Long QT Syndrome; Magnetic Resonance Angiography; Male; Natriuretic Peptide, Brain; Palliative Care; Peptide Fragments; Postoperative Complications; Prognosis; Stroke Volume; Transposition of Great Vessels; Ventricular Dysfunction, Right; Young Adult

To evaluate the relation of echocardiographic parameters of diastolic function, exercise capacity (expressed as peakVO(2)) and NT-proBNP in patients with transposition of the great arteries (TGA) and Mustard procedure.. Diastolic function was determined by measuring tricuspid flow velocities (Ea/Aa ratio), isovolumic relaxation time (IVRT), and deceleration time (DT). E/Ea ratios were calculated. For assessment of systolic function, CMR was applied.. E/A (r = 0.07, p = 0.66), E/Ea medial (r = 0.03, p = 0.84), E/Ea lateral (r = -0.01, p = 0.92), IVRT (r = -0.13, p = 0.44), and DT (r = -0.05, p = 0.76) were not correlated with peakVO(2). NT-proBNP showed a significant correlation with IVRT (r = 0.44, p = 0.004) and Ea/Aa medial (r = -0.34, p = 0.025). No correlation was found between RV systolic function and peakVO(2) (r = 0.07, p = 0.63).. Exercise capacity in patients with TGA and Mustard procedure is not related to echocardiographic parameters of diastolic function. NT-proBNP is associated with selected echocardiographic parameters of diastolic function.

Topics: Adult; Biomarkers; Cardiac Surgical Procedures; Diastole; Echocardiography; Exercise Test; Exercise Tolerance; Female; Humans; Male; Natriuretic Peptide, Brain; Oxygen Consumption; Peptide Fragments; Systole; Transposition of Great Vessels; Ventricular Function, Right

To compare B-type natriuretic peptide (BNP) levels with response of systemic right ventricular function to Dobutamine stress.. Sixteen patients aged 25.6 +/- 3.7 years (eight each after Senning or Mustard repair of complete transposition) were studied. Transoesophageal imaging was performed in the catheterization laboratory under general anaesthesia before and at the end of a 10 min infusion of 5 microg/kg/min of Dobutamine. The BNP levels were measured at rest. Myocardial Doppler data were acquired before and at peak stress in a four-chamber view. The BNP (pg/mL) values of 67.3 +/- 47.5 (14-189) were elevated. There was no correlation between BNP and IVA, strain, or systolic and diastolic velocities at rest. Dobutamine stress led to a significant increase in IVA, s-velocity, and strain but no significant change in e-velocity. A correlation was found between increase in IVA under Dobutamine and BNP levels (r = 0.57, P < 0.02).. Elevated BNP levels correlate with response of systolic right ventricular function assessed by IVA to Dobutamine stress.

Topics: Adolescent; Cardiotonic Agents; Dobutamine; Echocardiography, Stress; Echocardiography, Transesophageal; Female; Humans; Linear Models; Male; Myocardial Contraction; Natriuretic Peptide, Brain; Transposition of Great Vessels; Ventricular Dysfunction, Right; Young Adult

To assess neurohormonal activation of cardiac failure in adults with congenitally corrected transposition, and to determine the most sensitive marker for recognition of the cardiac failure.. The onset of morphologically right ventricular dysfunction is unpredictable in patients with congenitally corrected transposition, the combination of discordant atrioventricular and ventriculo-arterial connections, and its markers are unknown.. We measured amino terminal pro brain natriuretic peptide in 19 patients, aged 35 plus or minus 13.1 years, and in 19 control subjects. Morhologically right ventricular function was assessed by echocardiography, including tissue Doppler echocardiography and magnetic resonance imaging or multislice computed tomography.. The patients showed a highly significant increase in the levels of amino terminal pro brain natriuretic peptide, the levels being significantly elevated even in asymptomatic patients. Left atrial dimensions were larger in patients, and significantly lower tissue Doppler echocardiographic velocities were measured at the lateral site of the tricuspid annulus and at the basal segment of the interventricular septum. The ejection fraction of the morphologically right ventricle correlated significantly with the levels of brain natriuretic peptide, and with left atrial dimensions.. Neurohormonal activation is present in patients with congenitally corrected transposition even when they are asymptomatic. It is correlated with left atrial dimensions and tissue Doppler echocardiographic parameters. Levels of brain natriuretic peptide, and peak tricuspid early diastolic annular velocity, are the earliest and most sensitive markers of morphologically right ventricular dysfunction.

Topics: Adolescent; Adult; Aged; Biomarkers; Case-Control Studies; Echocardiography; Female; Heart Failure; Humans; Immunoassay; Male; Middle Aged; Natriuretic Peptide, Brain; Neurotransmitter Agents; Radiography; Slovenia; Transposition of Great Vessels; Ventricular Function, Right; Young Adult

Exercise is recommended in patients with left ventricular failure. Although right ventricular failure is common in patients with a systemic right ventricle (RV), no data are available on the effect of physical activity in these patients. The aim of this study was to evaluate the relation of physical activity and cardiac function, exercise capacity, and quality of life in patients with a systemic RV. Forty-seven patients (64% men, mean age 35 years, range 21 to 69) with a systemic RV (31 with an atrially switched transposition of the great arteries and 16 with a congenitally corrected transposition of the great arteries) were included. Cardiac function was assessed by cardiovascular magnetic resonance or computed tomography, exercise tests, and serum N-terminal prohormone brain natriuretic peptide (NT-pro-BNP) levels. Habitual physical activity was assessed using the Short Questionnaire to Assess Health-Enhancing Physical Activity (SQUASH) and quality of life using the Medical Outcomes Study Short Form 36-Item Health Survey. Mean systemic right ventricular ejection fraction was impaired (36.8 +/- 7.8%), as was maximal exercise capacity (78.5 +/- 23.9% of predicted). NT-pro-BNP level was increased (median 269 ng/L, range 34 to 4,476). Mean SQUASH score was 6,808 +/- 3,241, indicating a decreased level of habitual physical activity. Although patients' scores on mental quality-of-life domains were comparable to the general population, scores on most physical quality-of-life domains were significantly lower. SQUASH scores were found to be a significant predictor for exercise capacity (p <0.01) and physical quality of life (p <0.001). However, we found no relation between SQUASH scores and right ventricular ejection fraction or NT-pro-BNP. In conclusion, physical activity in patients with a systemic RV is positively associated with exercise capacity and quality of life, irrespective of cardiac performance.

Topics: Adult; Aged; Exercise Test; Exercise Tolerance; Female; Heart Failure; Heart Ventricles; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Motor Activity; Natriuretic Peptide, Brain; Peptide Fragments; Quality of Life; Stroke Volume; Tomography, X-Ray Computed; Transposition of Great Vessels; Ventricular Dysfunction, Right

Assessment of systemic right ventricular (RV) function is inherently difficult. In adults, plasma brain natriuretic peptide (BNP) level has been shown to reflect systemic ventricular dysfunction. We sought to test the hypothesis that plasma BNP is a biomarker of systemic RV function in patients after atrial switch operation.. We determined the RV function in 44 patients, 35 after Senning and 9 after Mustard operation, aged 19.7+/-4.0 years, by tissue Doppler echocardiography and determination of myocardial performance index (MPI). The results were compared to the left ventricular function of 14 age-matched controls. Their plasma BNP levels were correlated with indices of systemic ventricular function.. Compared with controls, the patients had greater MPI (p<0.001), lower systemic ventricular free wall-annular early diastolic (p<0.001), late diastolic (p<0.001), and systolic velocities (p=0.001), lower septal-annular early diastolic (p<0.001), late diastolic (p<0.001), and systolic velocities (p<0.001), and higher BNP levels (p=0.03). Plasma BNP levels correlated positively with MPI (r=0.43, p=0.001) and negatively with the free wall- and septal-annular myocardial velocities (r=-0.32 to -0.47, p<0.05). The area under the receiver operating characteristic curve for BNP to detect ventricular dysfunction (MPI >0.45) in patients was 0.67 (p=0.04). A BNP level of 36 pg/ml had a sensitivity of 55%, specificity of 86%, positive predictive value 80%, negative predictive value of 64%, and an accuracy of 70% for detecting systemic ventricular dysfunction.. Plasma BNP has modest accuracy in the detection of systemic RV dysfunction in patients after atrial switch operation.

Topics: Adult; Biomarkers; Cardiac Surgical Procedures; Case-Control Studies; Diastole; Echocardiography, Doppler; Female; Humans; Male; Natriuretic Peptide, Brain; ROC Curve; Sensitivity and Specificity; Statistics, Nonparametric; Systole; Transposition of Great Vessels; Ventricular Dysfunction, Right

In acquired heart disease, brain natriuretic peptide (BNP) and N-Terminal pro-brain natriuretic peptide (NT-proBNP) are increasingly used as diagnostic and prognostic markers. In adult congenital heart disease, the abnormal anatomy and physiology complicate assessment of cardiac function. We studied the clinical correlates of measurement of natriuretic peptides (NP) in adults with a right ventricle in the systemic position or with Fontan-type physiology.. A prospective longitudinal study (follow up time 23+/-13 months, mean+/-S.D.) was conducted in a specialised centre on 61 patients (age 26+/-8 years; NYHA class 1.5+/-0.6) including Senning/Mustard corrected transposition, congenitally corrected transposition and Fontan/total cavopulmonary connection. Plasma NP concentration was compared with NYHA class, exercise capacity and echocardiographically determined systemic systolic ventricular function.. Neurohormone concentrations were generally elevated (mean=290% of upper reference limit) and related to NYHA class (P<0.001, NYHA I vs. II-IV). No clinically significant relationship to ventricular function or exercise capacity was found however. An NP measurement could not predict the future course of the disease in terms of functional status or ventricular function.. In contrast to patients with acquired heart disease, measurement of NP seems to have low clinical value in adults with a right ventricle in the systemic position or with Fontan-type physiology.

Topics: Adolescent; Adult; Echocardiography; Female; Fontan Procedure; Heart Defects, Congenital; Humans; Longitudinal Studies; Luminescence; Male; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Prognosis; Prospective Studies; Radioimmunoassay; Transposition of Great Vessels

Natriuretic peptide levels are associated with cardiac output and ventricular function. We hypothesized that concomitant measurement of the peptide fragments and the hemodynamic parameters could elucidate the associations of these parameters after pediatric cardiac surgery.. After approval of the institutional review board and parents' informed consent, we investigated the clinical data of eight neonates undergoing correction of transposition of the great arteries. We measured the level of N-terminal fragments of prohormones of atrial and brain natriuretic peptides (NT-proANP, NT-proBNP) preoperatively, postoperatively and 12, 24, 48, and 72 h after arrival in the intensive care unit. The hemodynamic status was assessed by transpulmonary thermodilution at the same time points. Creatinine and other laboratory values were analyzed in the first 48 h postoperatively.. NT-proBNP levels were inversely correlated with cardiac index (CI, r = -0.47, P = 0.030), stroke volume index (r = -0.65, P = 0.005), and global end-diastolic volume index (GEDI; r = -0.63, P = 0.011). There was strong inverse correlation between the change of NT-proBNP levels and the change of CI between two consecutive measurements during the postoperative period (r = -0.79, P = 0.001). The NT-proBNP level 12 h after surgery was strongly correlated with the creatinine level of the postoperative 24th hour (r = 0.81, P = 0.014).. NT-proBNP correlated with the hemodynamic parameters and with the severity of renal dysfunction. Therefore, NT-proBNP is a reliable indicator of the circulatory state and the severity of a low output syndrome after arterial switch operation in neonates.

Topics: Cardiac Output; Humans; Infant, Newborn; Linear Models; Natriuretic Peptide, Brain; Peptide Fragments; Postoperative Period; Thermodilution; Transposition of Great Vessels

After Mustard repair for transposition of the great arteries, the right ventricle serves as a chronically overloaded systemic ventricle (SV). Thus, during long-term follow-up, dysfunction of the right ventricle with consecutive heart failure (HF) is not unusual. Early signs and symptoms of HF are depressed parameters of right ventricular (RV) function at rest and exercise intolerance. It was hypothesized that the measurement of RV function parameters, N-terminal pro-brain natriuretic peptide (NT-pro-BNP), and peak oxygen uptake (VO2max) during exercise testing were suitable for the early detection of subclinical HF. In 33 asymptomatic adolescents and adults who had undergone Mustard repair, RV function was analyzed by the myocardial performance index (Tei index). NT-pro-BNP and VO2max were also determined. The corresponding data from 52 patients operated on for left-to-right shunt defects without residual lesions served as reference data. In patients who underwent the Mustard procedure, the Tei index and NT-pro-BNP were elevated (mean Tei index of the SV 0.63 +/- 0.17 vs 0.34 +/- 0.05, p <0.002; mean NT-pro-BNP 240 +/- 230 vs 57 +/- 39 pg/ml, p <0.0001), and VO2max was reduced (27 +/- 6 vs 32 +/- 6 ml x kg(-1) x min(-1), p <0.002). A good correlation was found between the Tei index and VO2max (r = -0.83, p <0.0001). In conclusion, RV function is depressed in most patients with Mustard repair. Ventricular dysfunction in such asymptomatic or minimally symptomatic patients can be detected by measurement of the Tei index, NT-pro-BNP, and VO2max. These parameters are simple and reliable screening methods to stratify patients with impaired cardiac dysfunction before they become symptomatic.

Topics: Adolescent; Adult; Cardiac Surgical Procedures; Disease Progression; Echocardiography, Doppler; Exercise Test; Exercise Tolerance; Female; Follow-Up Studies; Heart Failure; Humans; Male; Middle Aged; Natriuretic Peptide, Brain; Observer Variation; Oxygen Consumption; Peptide Fragments; Postoperative Period; Prognosis; Prospective Studies; Protein Precursors; Transposition of Great Vessels; Ventricular Function, Right

To examine retrospectively the changes in ECG parameters over time and their correlation with other quantitative right ventricular (RV) function parameters in patients with chronic RV pressure overload caused by congenital heart disease.. 48 patients with chronic RV pressure overload caused by the following congenital heart diseases were studied: nine with congenitally corrected transposition of the great arteries (TGA), 12 with surgically corrected TGA, and 27 with a subpulmonary pressure overloaded RV. QRS duration and dispersion were measured manually from standard ECG recorded twice within five years. RV end diastolic volume (EDV) and RV mass were determined by magnetic resonance imaging. Brain natriuretic peptide (BNP) plasma concentrations were measured.. QRS duration and QRS dispersion increased in all patient groups during the follow up period. QRS duration increased significantly in the congenitally corrected TGA (p = 0.04) and the subpulmonary pressure overloaded RV groups (p = 0.01). QRS dispersion increased significantly in patients with surgically corrected TGA (p = 0.03) and in the subpulmonary pressure overloaded RV group (p = 0.02). A significant correlation was found between QRS duration and RVEDV (r = 0.71, p < 0.0001). RV mass was significantly correlated with QRS duration in patients with tetralogy of Fallot (r = 0.67, p = 0.01). Mean (SD) plasma brain natriuretic peptide concentrations (6.6 (5.4) pmol/l) were increased compared with normal reference values but no correlation was found with ECG parameters or RV systolic pressure. No malignant arrhythmia or sudden death occurred.. ECG parameters worsened gradually in asymptomatic or minimally symptomatic patients with chronic RV pressure overload, regardless of the nature of their congenital heart disease. In all patients, a significant positive correlation was found between QRS duration and RVEDV. In patients with tetralogy of Fallot there was also a correlation between QRS duration and RV mass.

Topics: Adult; Blood Pressure; Chronic Disease; Electrocardiography; Female; Heart Defects, Congenital; Humans; Magnetic Resonance Angiography; Male; Natriuretic Peptide, Brain; Retrospective Studies; Tachycardia, Supraventricular; Tachycardia, Ventricular; Transposition of Great Vessels; Ventricular Dysfunction, Right