Search

ObjectivePatients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are prone to sRV dysfunction. Pharmacological options for sRV failure remain poorly defined. This study aims to investigate the tolerability and effects of sacubitril/valsartan on sRV failure in adult patients with sRV.MethodsIn this two-centre, prospective cohort study, all consecutive adult patients with symptomatic heart failure and at least moderately reduced sRV systolic function were initiated on sacubitril/valsartan and underwent structured follow-up.ResultsData of 40 patients were included (40% female, 30% ccTGA, median age 48 (44–53) years). Five patients discontinued therapy during titration. Median follow-up was 24 (12–36) months. The maximal dose was tolerated by 49% of patients. No episodes of hyperkalaemia or renal function decline occurred. Six-minute walking distance increased significantly after 6 months of treatment (569±16 to 597±16 m, p=0.016). Serum N-terminal-prohormone brain natriuretic peptide (NT-proBNP) levels decreased significantly after 3 months (567 (374–1134) to 404 (226–633) ng/L, p<0.001). Small, yet consistent echocardiographic improvements in sRV function were observed after 6 months (sRV global longitudinal strain: −11.1±0.5% to −12.6±0.7%, p<0.001, and fractional area change: 20% (16%−24%) to 26% (19%−30%), p<0.001). The linear mixed-effects model illustrated that after first follow-up moment, no time effect was present for the parameters.ConclusionsTreatment with sacubitril/valsartan was associated with a low rate of adverse effects in this adult sRV cohort. Persisting improvement in 6-minute walking test distance, NT-proBNP levels and echocardiographic parameters of sRV function was observed in an on-treatment analysis and showed no differential response based on sex or anatomy.

ObjectivePharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients.MethodsData on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported. Patients with a systemic RV ejection fraction of ≤35% who were symptomatic despite treatment with β-blocker and ACE-inhibitor/angiotensin II receptor-blockers were started on sacubitril/valsartan. This cohort underwent structural follow-up including echocardiography, exercise testing, laboratory investigations and quality of life (QOL) assessment.ResultsSix-month follow-up data were available in 18 out of 20 patients, including 12 (67%) patients with TGA after atrial switch and 6 (33%) patients with ccTGA. N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) decreased significantly (950–358 ng/L, p<0.001). Echocardiographic systemic RV fractional area change and global longitudinal strain showed small improvements (19%–22%, p<0.001 and −11% to −13%, p=0.014, respectively). The 6 min walking distance improved significantly from an average of 564 to 600 m (p=0.011). The QOL domains of cognitive function, sleep and vitality improved (p=0.015, p=0.007 and p=0.037, respectively).ConclusionsWe describe the first patient cohort with systemic RV failure treated with sacubitril/valsartan. Treatment appears feasible with improvements in NT-pro-BNP and echocardiographic function. Our positive results show the potential of sacubitril/valsartan for this patient population.

BackgroundThe current diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is unacceptably long, causing loss of quality-adjusted life years and excess mortality. Validated screening strategies for early CTEPH diagnosis are lacking. Echocardiographic screening among all PE survivors is associated with overdiagnosis and cost-ineffectiveness. We aimed to validate a simple screening strategy for excluding CTEPH early after acute PE, limiting the number of performed echocardiograms.MethodsIn this prospective, international, multicentre management study, consecutive patients were managed according to a screening algorithm starting 3 months after acute PE to determine whether echocardiographic evaluation of pulmonary hypertension (PH) was indicated. If the ‘CTEPH prediction score’ indicated high pretest probability or matching symptoms were present, the ‘CTEPH rule-out criteria’ were applied, consisting of ECG reading and N-terminalpro-brain natriuretic peptide. Only if these results could not rule out possible PH, the patients were referred for echocardiography.Results424 patients were included. Based on the algorithm, CTEPH was considered absent in 343 (81%) patients, leaving 81 patients (19%) referred for echocardiography. During 2-year follow-up, one patient in whom echocardiography was deemed unnecessary by the algorithm was diagnosed with CTEPH, reflecting an algorithm failure rate of 0.29% (95% CI 0% to 1.6%). Overall CTEPH incidence was 3.1% (13/424), of whom 10 patients were diagnosed within 4 months after the PE presentation.ConclusionsThe InShape II algorithm accurately excluded CTEPH, without the need for echocardiography in the overall majority of patients. CTEPH was identified early after acute PE, resulting in a substantially shorter diagnostic delay than in current practice.

Predicting heart failure events in patients with a systemic right ventricle (sRV) due to transposition of the great arteries (TGA) is important for timely intensification of follow-up. This study assessed the value of strain compared with currently used parameters as predictor for heart failure–free survival in patients with sRV.

This study describes the different types of congenital vascular rings according to their anatomy, symptoms, and age at clinical onset and reports the surgical outcomes.

ObjectiveThis study assessed adult survival and morbidity patterns in patients who underwent atrial correction according to Mustard or Senning for transposition of the great arteries (TGA).MethodsIn 76 adult patients with TGA (59% male) after atrial correction, long-term survival and morbidity were investigated in three periods: early (<15 years postoperatively), midterm (15–30 years postoperatively) and late (>30 years postoperatively).ResultsThe Mustard technique was performed in 41 (54%) patients, and the Senning technique was performed in 35 (46%) patients aged 3.1 (IQR: 2.1–3.8) and 1.0 (IQR: 0.6–3.1; p<0.01) years, respectively. Adult survival was 82% at 39.7 (IQR: 35.9–42.4) years postoperatively and exceeded 50 years in four patients. Supraventricular tachycardia (SVT) occurred in 51% of patients. The incidences of ventricular arrhythmia (0%, 8% and 13%; p<0.01), heart failure (0%, 5% and 19%; p<0.01) and surgical reinterventions (0%, 5% and 11%; p=0.01) increased from early to late follow-up. At last follow-up, RV function was depressed in 31 (46%) patients, and New York Heart Association functional class was ≥2 in 34 (48%) patients. Bradyarrhythmia, SVT and ventricular arrhythmia were associated with depressed RV function (OR: 4.47, 95% CI 1.50 to 13.28, p<0.01; OR: 3.74, 95% CI 1.26 to 11.14, p=0.02; OR: 14.40, 95% CI 2.80 to 74.07, p<0.01, respectively) and worse functional capacity (OR: 2.10, 95% CI 0.75 to 5.82, p=0.16; OR: 2.87, 95% CI 1.06 to 7.81, p=0.04; OR: 8.47, 95% CI 1.70 to 42.10, p<0.01, respectively).ConclusionsIn adult patients with TGA, survival was 82% at 39.7 (IQR: 35.9–42.4) years after atrial correction. Morbidity was high and included SVT as most frequent adverse event. Ventricular arrhythmias, heart failure and surgical reinterventions were common during late follow-up. Adverse events were associated with depressed right ventricle function and reduced functional class.

Supplemental Digital Content is available in the text.

Background Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of 18 years could predict the chance of congenital heart surgery in adulthood.Design and methods Of 10,300 patients from the CONCOR national registry, we used general patient characteristics at age 18 years, underlying congenital heart defect, history of complications, and interventions in childhood as potential predictors of congenital heart surgery occurring from age 18 years up to age 40 and 60 years. Cox regression was used to calculate hazard ratios with 95% confidence intervals. Analyses were performed separately for all congenital heart surgery and for valvular surgery alone.Results Altogether 2427 patients underwent congenital heart surgery after age 18 years, 1389 of whom underwent valvular surgery. Underlying heart defect, male sex, multiple defects, childhood endocarditis, supraventricular arrhythmia, aortic complications and paediatric cardiovascular surgery, independently predicted adult congenital heart surgery. The mean chance of congenital heart surgery was 22% up to age 40 and 43% up to age 60 years; individual chances spanned from 9–68% up to age 40 and from 19–93% up to age 60 years.Conclusion At the time of transition from paediatric to adult cardiology, an easily obtainable set of characteristics of patients with congenital heart disease can meaningfully inform cardiologists about the patient's individual chance of surgery in adulthood. Our findings warrant validation in other cohorts.

IMPORTANCE: Adults late after total correction of tetralogy of Fallot (TOF) are at risk for major complications. Cardiovascular magnetic resonance (CMR) imaging is recommended to quantify right ventricular (RV) and left ventricular (LV) function. However, a commonly used risk model by Khairy et al requires invasive investigations and lacks CMR imaging to identify high-risk patients. OBJECTIVE: To implement CMR imaging in noninvasive risk stratification to predict major adverse clinical outcomes. DESIGN, SETTING, AND PARTICIPANTS: This multicenter study included 575 adult patients with TOF (4.083 patient-years at risk) from a prospective nationwide registry in whom CMR was performed. This study involved 5 tertiary referral centers with a specialized adult congenital heart disease unit. Multivariable Cox hazards regression analysis was performed to determine factors associated with the primary end point. The CMR variables were combined with the noninvasive components of the Khairy et al risk model, and the C statistic of the final noninvasive risk model was determined using bootstrap sampling. The data analysis was conducted from January to December 2016. MAIN OUTCOMES AND MEASURES: The composite primary outcome was defined as all-cause mortality or ventricular arrhythmia, defined as aborted cardiac arrest or documented ventricular fibrillation and ventricular tachycardia (lasting ≥30 seconds or recurrent symptomatic). RESULTS: Of the 575 patients with TOF, 57% were male, and the mean (SD) age was 31 (11) years. During a mean (SD) follow-up of 7.1 (3.5) years, the primary composite end point occurred in 35 patients, including all-cause mortality in 13 patients. Mean (SD) RV ejection fraction (EF) was 44% (10%), and mean (SD) LV EF was 53% (8%). There was a correlation between RV EF and LV EF (R, 0.36; 95% CI, 0.29-0.44; P &lt; .001). Optimal thresholds for ventricular function (RV EF &lt;30%: hazard ratio, 3.90; 95% CI, 1.84-8.26; P &lt; .001 and LV EF &lt;45%: hazard ratio, 3.23; 95% CI, 1.57-6.65; P = .001) were independently predictive in multivariable analysis. Both thresholds were included in a point-based noninvasive risk model (C statistic, 0.75; 95% CI, 0.63-0.85) and combined with the noninvasive components of the Khairy et al risk model. CONCLUSIONS AND RELEVANCE: In patients with repaired TOF, biventricular dysfunction on CMR imaging was associated with major adverse clinical outcomes. The quantified thresholds (RV EF &lt;30% and LV EF &lt;45%) may be implemented in noninvasive risk stratification.

BackgroundAlthough QRS duration >180 ms has prognostic value in adults with tetralogy of Fallot (TOF), its sensitivity to predict mortality is low. Fragmented QRS complexes, a simple measurement on ECG, are related to myocardial fibrosis and dysfunction in patients with TOF. Our objective was to determine whether QRS fragmentation predicts major outcomes in TOF.MethodsThis multicentre study included adult patients with TOF from a prospective registry. Notches in the QRS complex in ≥2 contiguous leads on a 12-lead ECG, not related to bundle branch block, were defined as QRS fragmentation, which was classified as none, moderate (≤4 leads) or severe (≥5 leads). The primary and secondary outcomes were all-cause mortality and clinical ventricular arrhythmia, respectively.ResultsA total of 794 adult patients with TOF (median age 27 years, 55% male; 52% no QRS fragmentation, 32% moderate, 16% severe) were included. During long-term (median 10.4 years) follow-up, 46 (6%) patients died and 35 (4%) patients had ventricular arrhythmias. Overall, 10-year survival was 98% in patients without fragmented QRS complexes, 93% in patients with moderate QRS fragmentation and 81% in patients with severe QRS fragmentation. In multivariable Cox hazards regression analysis, extent of QRS fragmentation (HR: 2.24/class, 95% CI 1.48 to 3.40, p<0.001) remained independently predictive for mortality, whereas QRS duration was not predictive (p=0.85). The extent of QRS fragmentation was also independently predictive for ventricular arrhythmia (HR: 2.00/class, 95% CI 1.26 to 3.16, p=0.003).ConclusionsThe extent of QRS fragmentation is superior to QRS duration in predicting mortality in adult patients with TOF and may be used in risk stratification.

Anomalous aortic origin of a right coronary artery (AAORCA) with an interarterial course merits further evaluation; however, robust risk assessment strategies for myocardial ischemia and sudden cardiac death are currently lacking. The aim of this study is to explore the potential role of fractional flow reserve (FFR), instantaneous wave-free ratio (iFR), and intravascular ultrasound (IVUS) in patients with AAORCA.

.

Right ventricular (RV) function is an important prognostic marker in patients with pulmonary hypertension. The present evaluation assessed the prognostic value of RV longitudinal peak systolic strain (LPSS) in patients with pulmonary hypertension.

The assessment of right ventricular (RV) function with two-dimensional echocardiography can be challenging in patients with pulmonary hypertension, especially in those with chronic pulmonary disease. The aim of the present study was to evaluate the feasibility of measuring RV longitudinal peak systolic strain (LPSS) in the echocardiographic subcostal view in patients with suspected pulmonary hypertension.

Objective. A first step in the delivery of tailored care is answering the following question: does health care meet the needs of patients? Therefore patients' perspective on health care use and their needs was examined. The design used was cross‐sectional questionnaire study.

To our knowledge, studies evaluating the quality of life (QoL) in patients with a history of acute pulmonary embolism (PE) are not available, even though QoL is a key outcome component of medical care and a predictor of disease-specific prognosis.

To investigate the age at menarche, the prevalence of menstrual cycle (interval) disorders, and determinants in women with congenital heart disease (CHD).

The aim of the study was to evaluate segmental and global right ventricular function at rest and during stress in patients having a systemic morphologically right ventricle. We studied 17 patients after atrial correction for transposition, 13 with congenitally corrected transposition, and 11 age-matched controls using cardiovascular magnetic resonance at rest and during stress with dobutamine given at 15 micrograms per kilogram per minute. Blood was drawn to obtain levels of brain natriuretic peptide. Right ventricular ejection fraction was calculated, and wall-motion and wall-thickening were assessed, in 4 segments of a midventricular slice. The findings for the systemic right ventricle were compared to the left ventricle in controls. Patients with transposition showed a decreased ejection fraction at rest (57 percent versus 69 percent, p equal to 0.005), decreased wall motion of the anterior, lateral and septal wall (p less than 0.01, p less than 0.01, and p less than 0.01) and decreased thickening of the lateral wall (p less than 0.01). Patients with congenitally corrected transposition showed normal ejection fraction, wall thickening, and wall motion at rest. During dobutamine stress, we found no significant differences in wall motion and thickening between the two groups. Ejection fraction, also increased to comparable values during stress, at 67 percent versus 66 percent, p not being significant. In both groups, we observed similarly increased levels of brain natriuretic peptide (p equal to 0.02 and 0.03, respectively). We conclude that only patients with transposition showed segmental wall motion and wall thickening abnormalities at rest. After dobutamine stress, however, segmental and global right ventricular dysfunction was similar in both groups.

To investigate whether exercise-induced hypertension in successfully repaired adult post-coarctectomy patients is associated with hypertension on 24-h blood pressure measurement and increased left ventricular mass.

Selexipag has been recognised as effective treatment for pulmonary arterial hypertension (PAH). However, evidence for its use in PAH associated with congenital heart disease (CHD) is limited to those with PAH after simple defect correction. We evaluated the response to selexipag in a wider range of PAH-CHD patients.

Magnetic resonance (MR) angiography and flow mapping have the potential to become a major noninvasive diagnostic tool for the assessment of coronary artery bypass graft morphology and function. Several MR sequences, such as conventional non‐respiratory compensated methods, and phase contrast cine flow sequences have been reported for the evaluation of bypass graft patency. However the visualization of different graft segments and the detection of graft stenosis remains difficult. Recent advances in MR coronary angiography and flow mapping are volume coronary angiongraphy with targeted scans, navigator gated angiography, contrast‐enhanced angiography, and breath‐hold or navigator gated flow sequences. Future approaches, such as navigator gated fast MR techniques resulting in high‐resolution angiography in combination with breath‐hold MR flow mapping with high temporal resolution, might allow a comprehensive evaluation of bypass graft stenosis and function. This review article will address the major issues concerning the MR evaluation of bypass grafts. J. Magn. Reson. Imaging 1999;10:434–441. © 1999 Wiley‐Liss, Inc.

Magnetic resonance (MR) angiography and flow mapping have the potential to become a major noninvasive diagnostic tool for the assessment of coronary artery bypass graft morphology and function. Several MR sequences, such as conventional non-respiratory compensated methods, and phase contrast cine flow sequences have been reported for the evaluation of bypass graft patency. However the visualization of different graft segments and the detection of graft stenosis remains difficult. Recent advances in MR coronary angiography and flow mapping are volume coronary angiongraphy with targeted scans, navigator gated angiography, contrast-enhanced angiography, and breath-hold or navigator gated flow sequences. Future approaches, such as navigator gated fast MR techniques resulting in high-resolution angiography in combination with breath-hold MR flow mapping with high temporal resolution, might allow a comprehensive evaluation of bypass graft stenosis and function. This review article will address the major issues concerning the MR evaluation of bypass grafts. J. Magn. Reson. Imaging 1999;10:434–441. © 1999 Wiley-Liss, Inc.

In a clinical study, the value of thallium-201 reinjection was studied in 139 patients with suspected or known coronary artery disease who showed one or more persistent defects after conventional stress-redistribution imaging. Fifty-nine (42%) patients had sustained a Q-wave myocardial infarction. Sixty-eight (49%) patients showed a reversible defect in at least one myocardial segment at redistribution, while 71 (51%) had persistent defects only. Following reinjection additional segmental filling-in was seen in 95 (68%) patients, including 50 of the 68 (74%) patients with reversible defects and 45 of the 71(63%) with persistent defects only. On the immediately post-exercise images, 458 (47%) of 973 segments showed perfusion defects. At redistribution 105 (23%) of the 458 defects showed filling-in, whereas of the remaining 353 persistent defects 164 (46%) resolved additionally after reinjection. Thirteen (10%) of 133 Q-wave related defects showed filling-in at redistribution compared with 22 (27%) of 82 remote defects (P = 0.001). After reinjection additional filling-in of defects was seen in 47 (39%) of 120 Q-wave related defects compared with 35 (58%) or 60 remote defects (P = 0.015). Overall, 60 (45%) of 133 Q-wave related defects resolved compared with 57 (70%) of 82 remote defects (P = 0.005). Thus reinjection of thallium-201 (1) revealed reversible defects in 63% of patients with only persistent defects at redistribution, (2) demonstrated additional filling-in in 74% of patients who already showed reversible defects at redistribution, (3) showed viability in 46% of segments initially classified as persistent, and (4) demonstrated filling-in in 39% of Q-wave related segments, although this was less conspicuous than in remote segments.

To assess the efficacy of the newly developed contrast agent gadolinium (Gd) benzyloxy propionic tetraacetic-acid BOPTA to detect acute myocardial infarction in patients using magnetic resonance imaging (MRI), 24 patients (age 53.3±8.3) were examined 9.3±3.7 days after a first myocardial infarction. Short axis Tl weighted images were obtained at 3 slice levels, before, immediately after injection of Gd-BOPTA, and after 15min, 30min, 45min. Patients were divided into 2 groups according to the dose of Gd-BOPTA (0.05mmol/kg and 0.1mmol/kg). Contrast to noise ratio, signal intensity enhancement of normal and infarcted myocardium and signal intensity of infarcted to signal intensity of normal myocardium (SI inf/norm) was quantified. Contrast to noise ratio was not affected by the type of dosage (0.05 mmol/kg, 5.75±0.85 vs. 0.1mmol/kg, 5.32±0.69). Enhancement of normal and infarcted myocardium increased immediately after administration of 0.05mmol/kg Gd-BOPTA and gradually decreased thereafter (p&lt;0.002 for normal myocardium) (see Figure). After 0.1mmol/kg Gd-BOPTA administration, myocardial enhancement increased rapidly but showed no decrease within 45 minutes after administration (see Figure). Mean SI inf/norm was significantly improved after Gd-BOPTA administration (p&lt;0.0005 for either dosage), and 0.05mmol/kg produced significantly higher SI inf/norm than 0.1mmol/kg (1.42±0.069 vs. 1.34±0.055 respectively, p=0.015).

Aims To evaluate the additional value of dobutamine stress testing in patients with repaired tetralogy of Fallot (TOF) by relating stress imaging parameters at baseline to relevant parameters of clinical condition and right ventricular (RV) size during a serial follow-up. Methods and results We prospectively included 27 patients (14 ± 4 years at baseline), who were studied twice with a 5-year interval. Patients underwent cardiovascular magnetic resonance imaging to assess RV systolic and diastolic function at rest and during dobutamine stress. Normal response to dobutamine was defined as a decrease in RV end-systolic volume, and a increase in RV ejection fraction (EF) during stress. Exercise testing and electrocardiography were performed to determine peak oxygen uptake (peak VO2), QRS duration, and QT interval corrected for heart rate (QTc) interval. RV volumes, QRS duration, and QTc interval increased significantly from baseline to follow-up; peak VO2 tended to decrease (95 ± 20–89 ± 14%, P = 0.086). Response to dobutamine was normal in 26 of 27 patients and remained stable during the follow-up [relative increase in RVEF during stress: +25 ± 9% (baseline) vs. +27 ± 10% (follow-up)]. A smaller relative increase in RVEF during stress at baseline related to a larger relative decrease in peak VO2 during the follow-up (r = 0.59, P = 0.004). No significant associations were found with the relative increase in QRS duration, QTc interval, or RV end-diastolic volume during a 5-year follow-up. Conclusion In a young TOF population, response to dobutamine stress was normal and remained stable during the 5-year follow-up. A smaller increase in RVEF during stress at baseline was predictive for a larger decrease in peak VO2 during the 5-year follow-up.

The presence and progressive nature of primary myocardial involvement in Marfan syndrome are debated. The aim of this study was to evaluate the clinical relevance of left ventricular (LV) and right ventricular (RV) strain in adult patients with Marfan syndrome without significant valvular disease.

Myocardial DNA concentration and ventricular weight were measured in the hearts of normal and spontaneously hypertensive rats varying in age from 1 day to 1 year and the ventricular DNA content calculated. A linear relation between ventricular DNA content and ventricular weight was observed in both rat strains. This linear relation was deduced theoretically, assuming that the number of ventricular non-myocytes per heart was proportional to ventricular weight and that the number of ventricular myocytes per heart was constant after the first postnatal week. The fit with experimental data produced values of the number of ventricular myocytes (42.5 106) and non-myocytes (199 106 per gram of ventricular tissue) in normal rat heart. Using published data on ventricular volume occupied by myocytes (80%) and myocardial specific density (1.06 g·ml–1), the mean(SD) volume of individual myocytes could be calculated (17758(2632) multiplied by ventricular weight, expressed in µm3·g). The values obtained compared well with experimental observations reported in the present study (18504(131) µm3·g) and in other studies. Hearts from spontaneously hypertensive rats had lower numbers of myocytes (37.4 106) and non-myocytes (180 106 per gram) per heart than normal hearts with identical ventricular weights. Hearts of normal 2 month old rats that were made hypertrophic by various means were identical to non-hypertrophic hearts of normal older rats with identical ventricular weights in relation to the number of ventricular myocytes and non-myocytes per heart. It is concluded that ventricular hypertrophy induced in young adult rats might be regarded as normal growth occurring at an accelerated rate.