Effectiveness of the risk stratification proposed by the 2022 European Heart Rhythm Association Expert Consensus statement on arrhythmic mitral valve prolapse.

BACKGROUND: Recently, an expert consensus statement proposed indications where implantation of a primary prevention implantable cardioverter-defibrillator (ICD) may be reasonable in patients with mitral valve prolapse (MVP). The objective was to evaluate the proposed risk stratification by the expert consensus statement. METHODS: Consecutive patients with MVP without alternative arrhythmic substrates with cardiac magnetic resonance imaging (CMR) were included in a single-center retrospective registry. Arrhythmic MVP (AMVP) was defined as a total premature ventricular complex burden ≥5%, non-sustained ventricular tachycardia (VT), VT, or ventricular fibrillation. The end point was a composite of SCD, VT, inducible VT, and appropriate ICD shocks. RESULTS: In total, 169 patients (52.1% male, median age 51.4 years) were included and 99 (58.6%) were classified as AMVP. Multivariate logistic regression identified the presence of late gadolinium enhancement (OR 2.82, 95%CI 1.45-5.50) and mitral annular disjunction (OR 1.98, 95%CI 1.02-3.86) as only predictors of AMVP. According to the EHRA risk stratification, 5 patients with AMVP (5.1%) had a secondary prevention ICD indication, while in 69 patients (69.7%) the implantation of an ICD may be reasonable. During a median follow-up of 8.0 years (IQR 5.0-15.6), the incidence rate for the composite arrhythmic end point was 0.3%/year (95%CI 0.1-0.8). CONCLUSION: More than half of MVP patients referred for CMR met the AMVP diagnostic criteria. Despite low long-term event rates, in 70% of patients with AMVP the implantation of an ICD may be reasonable. Risk stratification of SCD in MVP remains an important knowledge gap and requires urgent investigation.

Stent expansion of restrictive Fontan conduits to nominal diameter and beyond.

BACKGROUND: Mechanical factors may cause bottlenecks in a Fontan circuit. Extracardiac conduits (ECC) are placed at a young age, but the materials do not allow growth. Restriction in ECC dimensions may deteriorate the function of the circuit. AIMS: This study aimed to evaluate the feasibility and safety of stent expansion of an ECC to the nominal dimension at the time of implant and, if possible, beyond nominal. METHODS: Retrospective, single-center observational review of all ECC Fontan patients who received a stent to expand a previously placed surgical conduit. RESULTS: A total of 44 restrictive conduits were stented over a 14-year study period with a median of 11.8 (interquartile ranges [IQR]: 9.1-13.8) years after ECC placement. Cross-sectional areas were a median of 30% (IQR: 21-42) smaller than the originally placed ECC; there was no gradient in 23/44 patients and in 21/44, a minimal gradient of 1.3 ± 0.5 (range 1-3 mmHg). All conduits could be enlarged with a significant (p < 0.0001) increase in diameter from 13.6 ± 1.8 to 19.2 ± 1.2 mm, corresponding to a median cross-sectional area increase of 171% (IQR: 153-220). In three patients where the conduits were not contracted, expansion of between 127% and 165% was obtained. There were no conduit ruptures and only one minor complication. CONCLUSIONS: ECC in some Fontan patients become smaller than nominal over time, usually without overt symptoms. The dimensions of ECC's can be safely and significantly increased to nominal or even beyond employing stenting. It allows adjustment of ECC dimensions to compensate for somatic growth.

Management of acute heart failure in adult patients with congenital heart disease.

Heart failure is an increasing reason for hospitalization and the leading cause of death in patients with adult congenital heart disease (ACHD). Recently, the European Society of Cardiology and the American Heart Association published consensus documents on the management of chronic heart failure in ACHD patients. However, little data and/or guidelines are available for the management of (sub)acute heart failure. The ACHD population is heterogeneous by definition and often has complex underlying anatomy, which could pose a challenge to the physician confronted with the ACHD patient in (sub)acute heart failure. Recognizing the underlying anatomy and awareness of the possible complications related would result in better treatment, avoid unnecessary delays, and improve outcomes of the ACHD patient with (sub)acute heart failure. This review focuses on the management of (sub)acute heart failure in ACHD with specific attention to lesion-specific issues.

Transvenous valve-in-valve replacement preserving the function of a transvalvular defibrillator lead.

Although feasibility and efficacy of percutaneous tricuspid valve-in-valve implantation have been established, a transtricuspid pacing or defibrillator lead might preclude this technique: lead damage can cause lead dysfunction resulting in inappropriate or inefficient pacing or shocks. In these cases, lead removal is thought to be the only option. We describe a patient who presented with rapid clinical deterioration due to tricuspid valve stenosis early after implantation of an internal defibrillator with a transvalvular shock-lead. A transvenous valve-in-valve implantation of the tricuspid valve was performed after protecting the defibrillator-lead with a custom-made covered stent. We describe the technical issues, the clinical outcome, and the evolution of lead function after implantation.

Right heart morphology in elevated pulmonary artery pressure: relationship between echocardiographic and right heart catheterization data.

BACKGROUND: When elevated pulmonary artery pressure (PAP) is assessed by echocardiography, right heart morphology is always considered. The goal of this study was to evaluate how right heart geometry changes with increasing right ventricular pressure load. SUBJECTS AND METHODS: Data from patients undergoing transthoracic echocardiography with subsequent right heart catheterization within a time period of 6 months were retrospectively analysed. First, Spearman-rho coefficients between mean PAP and right heart parameters were calculated. Second, the population was divided into tertiles according to mean PAP and Kruskal-Wallis variance analysis between variables was performed. RESULTS: Fifty-four patients (23 female, median age 77 years, IQ range 63-83) were selected. Mean PAP (median 27 mmHg, IQ range 24-36), right atrial (RA) dilatation (median 1, IQ range 0-2), tricuspid insufficiency (TI) severity (median 1.5, IQ range 0-2) and right ventricular (RV) dilatation (median 0, IQ range 0) were included. Significant correlations with mean PAP were found for RA dilatation (rho = 0.380; P = 0.005) and TI severity (rho = 0.294; P = 0.032). No correlation with RV dilatation could be shown (rho = 0.241; P = 0.081). Across the tertiles [(1) mean PAP 25 mmHg; (2) mean PAP 26-30 mmHg; (3) mean PAP > or = 31 mmHg)], variance analysis showed a significant increase in RA dilatation (P = 0.009) and TI severity (P = 0.040). No change in RV dilatation across groups was observed (P = 0.216). CONCLUSIONS: RA dilatation and TI severity significantly increase with increasing RV pressure load. No increase in RV dilatation was observed in the studied population. Hence, absence of RV dilatation does not exclude the presence of elevated PAP.

Biomarkers in transposition of the great arteries after arterial switch operation: A pilot trial with deep phenotyping.

INTRODUCTION: Transposition of the great arteries (TGA) is a cyanotic congenital heart defect for which the arterial switch operation (ASO) is the preferred surgical repair. This study wanted to investigate whether a panel of biomarkers could identify morphologic as well as hemodynamic changes obtained by cardiac magnetic resonance (CMR). METHODS: Forty-four adult patients were included. Blood samples were collected to measure a broad range of biomarkers (galectin-3, ST2, GDF-15, PINP, ICTP, PIIINP, IGF-1, NT-proBNP, and hs-Tn). CMR was performed at rest and during exercise to assess cardiac function and morphology. Explorative statistics were performed between biomarker levels and CMR findings. RESULTS: All patients were asymptomatic. While galectin-3, GDF-15, and NT-proBNP levels were within normal ranges, increased ST2, PINP, PIIINP, and ICTP levels were found in 20.5%, 34.1%, 45.5%, and 27.3% of patients, respectively. Moreover, 3 and 2 patients, respectively, showed elevated IGF-1 and hs-Tn levels. Although the ejection fraction of both ventricles was within normal limits, impaired cardiac reserve was found in 20 and 25% of patients for left and right ventricle, respectively. CMR revealed no evidence of diffuse interstitial fibrosis, while 4 patients showed focal ischemic scarring. However, no significant associations between serum biomarkers and CMR data could be detected. CONCLUSION: The results suggest that in asymptomatic ASO-repaired TGA patients serum level biomarkers are elevated and that this increase is not associated with morphological changes nor with a decreased cardiac reserve. Further study with larger sample sizes is required to draw conclusions with greater confidence.

Serial Cardiac Magnetic Resonance Imaging in Patients with Mitral Valve Prolapse-A Single-Center Retrospective Registry.

Background: Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) are common valvular abnormalities that have been associated with ventricular arrhythmias (VA). Cardiac magnetic resonance imaging (CMR) has a key role in risk stratification of VA, including assessment of late gadolinium enhancement (LGE). Methods: Single-center retrospective analysis of patients with MVP or MAD who had >1 CMR and >1 24 h Holter registration available. Data are presented in detail, including evolution of VA and presence of LGE over time. Results: A total of twelve patients had repeated CMR and Holter registrations available, of which in four (33%) patients, it was conducted before and after minimal invasive mitral valve repair (MVR). After a median of 4.7 years, four out of eight (50%) patients without surgical intervention had new areas of LGE. New LGE was observed in the papillary muscles and the mid to basal inferolateral wall. In four patients, presenting with syncope or high-risk non-sustained ventricular tachycardia (VT), programmed ventricular stimulation was performed and in two (50%), sustained monomorphic VT was easily inducible. In two patients who underwent MVR, new LGE was observed in the basal inferolateral wall of which one presented with an increased burden of VA. Conclusions: In patients with MVP and MAD, repeat CMR may show new LGE in a small subset of patients, even shortly after MVR. A subgroup of patients who presented with an increase in VA burden showed new LGE upon repeat CMR. VA in patients with MVP and MAD are part of a heterogeneous spectrum that requires further investigation to establish risk stratification strategies.

Aorta pathology and pregnancy-related risks in adult congenital cardiac disease: does the aorta dilate during pregnancy?

Background: Aortic dilatation and pregnancy are major concerns in women with aortopathy (AOP). This single-centre retrospective analysis focuses on the evolution of aortic diameters during and after pregnancy in women with Marfan syndrome (MS), Turner syndrome (TS) and bicuspid aortic valve (BAV) aortopathy. Methods and results: Thirty-eight women who had one or more single pregnancies were included. The ascending aorta was measured during pregnancy and postpartum. During pregnancy, a significant increase of diameters of the sinus aortae (median 1.4 mm; [-1.3; 3.8]) and ascending aorta (median 2.1 mm; [0.0; 4.0]) was noted. Systemic hypertension gives dilation of the aorta, but it did not influence the overall trajectory during pregnancy. Conclusion: Significant aortic dilatation is noted during pregnancy in women with underlying AOP, even persisting in the long term. Pre-existing systemic hypertension is associated with larger aortic diameters prior to pregnancy. More research on a larger study population however is needed.

Transcatheter device closure of atrial septal defects in patients above age 60.

BACKGROUND: Closure of an atrial septal defect (ASD) mostly causes reverse remodelling of the right heart, a decrease in pulmonary artery pressures (PASP), and improves functional capacity. However, abolition of the left-to-right shunt may be unfavourable in older patients. This study aimed at evaluating patients older than 60 years who underwent ASD closure. METHODS AND RESULTS: Forty-seven patients (mean age 69 5 years, 19.1% male) with ASD type secundum who underwent transcatheter device closure were included. Echocardiographic data before and after closure were obtained. Follow-up data until the last follow-up visit were retrieved. Age-, gender- and BMI-matched controls were selected. Before closure, ASD patients had a higher right ventricular end-diastolic diameter (RVEDD), right (RA) and left atrial (LA) dimensions, and PASP when compared to controls. After closure, RVEDD and PASP decreased whereas LVEDD and E/A-ratio increased. RVEDD, PASP, LA dimensions, left ventricular ejection fraction (LVEF) and E/A-ratio were higher than controls. NYHA class improved significantly after ASD-closure. During a median follow-up time of 3.3 years, 6 patients died and 16 were hospitalized because of cardiac events. CONCLUSION: Device closure in the elderly improved functional capacity, with a decrease, but not a normalization of RV dimensions and PASP, and an increase in LV dimensions, EF and mitral E/A-ratio. Atrial arrhythmias and coronary ischaemic events are common in this population.

Increased pulmonary artery pressures during exercise are related to persistent tricuspid regurgitation after atrial septal defect closure.

OBJECTIVE: Although closure of an atrial septal defect type secundum often normalizes right heart dimensions and pressures, mild tricuspid insufficiency might persist. This study aimed at (1) identification of determinants explaining the persistence of tricuspid insufficiency after atrial septal defect closure, and (2) evaluation of functional capacity of patients with persistent mild tricuspid insufficiency. METHODS AND RESULTS: Twenty-five consecutive patients (age 42+17 y) were included from the outpatient clinic of congenital heart disease at the University Hospitals of Leuven. All underwent transthoracic echocardiography, semi-supine bicycle stress echocardiography and cardio-pulmonary exercise testing. Six patients (24%) had mild tricuspid insufficiency (2/4) compared to 19 patients (76%) with no or minimal tricuspid insufficiency ( 1/4) as assessed by semi-quantitative colour Doppler echocardiography. Mann-Whitney U and Fisher's exact tests were performed where applicable. Patients with persistent mild tricuspid insufficiency were significantly older than those with no or minimal tricuspid insufficiency (P = 0.042). At rest, no differences in right heart configuration, mean pulmonary artery pressure or right ventricular function were found. At peak exercise, mean pulmonary artery pressure was significantly higher in patients with mild persistent tricuspid insufficiency (P = 0.026). Peak oxygen uptake was significantly lower in patients with mild persistent tricuspid insufficiency (P = 0.019). CONCLUSIONS: Mild tricuspid insufficiency after atrial septal defect repair occurs more frequently in older patients and in patients with higher mean pulmonary artery pressure at peak exercise. In patients with mild tricuspid insufficiency, functional capacity was more reduced. Mild tricuspid insufficiency could be a marker of subclinical persistent pressure load on the right ventricle.

Secondary tricuspid valve regurgitation after isolated aortic valve replacement: outcomes and predictors.

OBJECTIVES: We sought to assess the evolution of secondary tricuspid regurgitation (TR) after isolated aortic valve replacement (AVR) and its impact on mortality and to identify possible clinical and echocardiographic predictors of persisting and new-onset TR (TR de novo) after isolated AVR. METHODS: Clinical and echocardiographic data of 441 patients, consecutively operated for isolated AVR between January 2017 and January 2020, were retrospectively collected. Four time points were included: preoperative, discharge, 3-6 months and last available follow-up. We followed patients with at least moderate TR (TR ≥2) over time and monitored the impact on survival. Logistic regression analysis was performed to identify possible predictors of persistent TR and TR de novo. RESULTS: Median follow-up was 33 months. Incidence of TR ≥2 changed over the time points. Twenty-three percent of patients with preoperative TR ≥2 had persistent TR at 3-6 months follow-up, and this phenomenon was predicted by age at regression analysis. Preoperative TR ≥2 was associated with a 3-fold higher risk to die. At 3- to 6-month follow-up, 12% of patients developed TR de novo. At least moderate preoperative mitral regurgitation (≥2) was predictive of TR de novo. CONCLUSIONS: Patients with TR ≥2 undergoing isolated AVR had worse long-term survival, and this was particularly evident in the elderly. Older patients were also more prone to have persistent TR after AVR. Some patients developed TR de novo after isolated AVR, but this did not affect survival.

Short-term results of serial cardiopulmonary exercise testing in adults with repaired coarctation of the aorta.

BACKGROUND: Data on the evolution of exercise capacity in adults with repaired coarctation of the aorta (CoA) are scarce. We aimed to investigate the evolution and change of measures of exercise capacity obtained by cardiopulmonary exercise testing (CPET) in adults with repaired CoA. METHODS: Patients 16 years of age and older with CoA, who performed at least two maximal CPETs in our institution, were included in the study. The first and last available tests were used for comparative statistical analysis of common exercise variables. RESULTS: Sixty patients (43 men) performed serial maximal CPET. Mean age at first assessment was 30 ± 10 years (range 17-68). Mean time between first and last assessment was 3.5 years (range 1-7). Mean peak VO2 was 85.6 ± 20.4% of the predicted value at the initial test, and 87.0 ± 20.5% at the final test (p = 0.294). There were no significant differences in the mean values of oxygen pulse, VO2 at anaerobic threshold, systolic and diastolic blood pressures and peak heart rate between the two assessments. There was a slightly higher VE/VCO2 slope at the final test (p = 0.047). Higher age and Borg scale were found to be related with a decline in percent-predicted peak VO2 from initial to final assessment. CONCLUSION: In adults with repaired CoA, we found no significant change in peak VO2 during a mean follow-up of 3.5 years, yet a small increase in VE/VCO2 slope was observed. Higher age was predictive for a decline in percent-predicted peak VO2, starting in the third decade of life.

Decreased cardiac reserve in asymptomatic patients after arterial switch operation for transposition of the great arteries.

BACKGROUND: Exercise capacity is impaired in patients after arterial switch operation (ASO) for complete transposition of the great arteries. Maximal oxygen consumption is related with outcome. OBJECTIVES: This study assessed ventricular function by advanced echocardiography and cardiac magnetic resonance (CMR) imaging at rest and during exercise, to determine exercise capacity in ASO patients, and to correlate exercise capacity with ventricular function as potential early marker of subclinical impairment. METHODS: Forty-four patients (71% male, mean age 25 ± 4 years - range 18-40 years) were included during routine clinical follow-up. Assessment involved physical examination, 12­lead ECG, echocardiography, and cardiopulmonary exercise test (CPET) (day 1). On day 2 CMR imaging at rest and during exercise was performed. Blood was sampled for biomarkers. RESULTS: All patients reported New York Heart Association class I, the overall cohort had an impaired exercise capacity (80 ± 14% of predicted peak oxygen consumption). Fragmented QRS was present in 27%. Exercise CMR showed that 20% of patients had abnormal contractile reserve (CR) of the left ventricle (LV) and 25% had reduced CR of the right ventricle (RV). CR LV and CR RV were significantly associated with impaired exercise capacity. Pathological patterns on myocardial delayed enhancement and hinge point fibrosis were detected. Biomarkers were normal. CONCLUSION: This study found that in some asymptomatic ASO patients electrical, LV and RV changes at rest, and signs of fibrosis are present. Maximal exercise capacity is impaired and seems to be linearly related to the CR of the LV and the RV. Therefore, exercise CMR might play a role in detecting subclinical deterioration of ASO patients.

In vivo performance of a tri-leaflet mechanical heart valve prosthesis in an ovine model.

OBJECTIVES: We present the long-term results of a trileaflet (Triflo) versus bileaflet (On-X) mechanical valve in both aortic and pulmonary positions in a sheep model. METHODS: The Triflo valve was implanted in 21 female sheep in aortic (n = 8) and pulmonary position (n = 13). The On-X valve was implanted in 7 female sheep in aortic (n = 1) and pulmonary (n = 6) positions. No antithrombotic medication of any kind was given postoperatively. In the aortic group, survival cohorts were 3 and 5 months. In the pulmonary group, survival cohorts were 10 and 20 weeks. Valve performance was assessed using haematology, echocardiography and acoustic measurements combined with post-mortem pathology analysis of the downstream organs. RESULTS: The mean gradients were lower for the Triflo valve in both pulmonary [4.30 mmHg (3.70-5.73) vs 6.80 mmHg (4.63-7.96), P = 0.012] and aortic [5.1 mmHg (4.2-7.7) vs 10.7 mmHg (8.7-12.9), P = 0.007] positions. Peak gradients were lower for the Triflo valve in both pulmonary [8.05 mmHg (6.75-10.23) vs 13.15 mmHg (9.20-14.76), P = 0.005] and aortic [8.7 mmHg (7.5-12.5) vs 16.5 mmHg (14.2-19.6), P = 0.009] positions. In both positions, leaflets and housing surface were free from any deposits macro- and microscopically and comparable to nonimplanted control valves. Peripheral organs showed no signs of thrombo-embolic damage. Biochemical and haematological were comparable to preoperative. The closing click sound pressure level of the Triflo was significantly lower in both aortic [108.4 sound pressure level (102.0-115.7) vs 111.7 sound pressure level (105.5-117.0), P < 0.001] and pulmonary [103.6 sound pressure level (99.1-108.9) vs 118.5 sound pressure level (116.7-120.2), P < 0.001] position. CONCLUSIONS: Preliminary in vivo results of the Triflo valve are promising in both aortic and pulmonary positions in an ovine model. Excellent haemodynamics, stable long-term function, low valve noise and no thrombo-embolic events in the absence of antithrombotic medication lay the foundation to a future clinical first-in-man trial.

Short-term Prognostic Value of Heart Failure Diagnosis in a Contemporary Cohort of Patients With Adult Congenital Heart Disease.

BACKGROUND: Heart failure (HF) is the primary cause of premature death in adult congenital heart disease (ACHD). This study aimed to describe the impact of a HF diagnosis on short-term prognosis and to investigate the added prognostic value of an HF diagnosis to the ACHD Anatomic and Physiologic classification (ACHD-AP). METHODS: This study included 3995 patients followed in a tertiary care centre (last follow-up after January 1, 2010). Survival curves were plotted, and predictors of the primary end point (death, heart transplantation, or ventricular assist device [VAD]) were identified with the use of Cox proportional hazard models and compared with the use of Harrell's C-statistic. RESULTS: Mean age at baseline was 35.7 ± 13.3 years. The prevalence of ACHD-HF was 6.4%. During a median follow-up of 3.1 years (IQR 2.1-3.6 years), 27.3% of ACHD-HF patients reached the primary end point, compared with 1.4% of ACHD patients without HF. Event-free survivals were 78.3%, 61.9%, and 57.5% at 1, 3, and 5 years in ACHD-HF patients, compared with 99.3%, 98.3%, and 98.0% in ACHD patients without HF (P < 0.001). An HF diagnosis (HR 6.9, 95% CI 4.3-11.2) and the physiologic classification (HR 2.6, 95% CI 1.9-3.7) were independently associated with the primary end point. The addition of HF to the ACHD-AP classification yielded a Harrell's C-index of 0.8631, providing a significant improvement over the ACHD-AP classification alone (P = 0.0003). CONCLUSIONS: The risk of mortality, transplantation, or VAD is increased in ACHD-HF patients. An HF diagnosis appears to be a valuable prognostic marker in addition to the ACHD-AP classification.

Left atrial strain in patients after arterial switch operation for transposition of the great arteries.

BACKGROUND: Left atrial (LA) strain, comprising LA reservoir, conduit and contractile function could add mechanistic information of patients after arterial switch operation (ASO) for transposition of the great arteries (TGA). ASO patients might have abnormal ventriculoarterial coupling, which makes them vulnerable to left ventricle (LV) dysfunction and results in reduced exercise capacity. This explorative study aimed to evaluate the relation between LA strain, atrial size, ventricular function, and exercise data obtained by cardiopulmonary exercise testing (CPET). METHODS: In a cohort of 44 patients (71% male, mean age 25 ± 4 years) LA strain was measured using transthoracic speckle-tracking echocardiography. Further assessment involved standard echocardiography, CPET evaluation, and blood sampling. LA strain values were compared to normal values. Correlations were calculated. Regression analysis with all strain variables to the CPET data was performed. RESULTS: LA reservoir, conduit and contractile strain were normal in 30%, 89% and 50% of the patients, respectively. LA reservoir/contractile strain correlated to LV ejection fraction (ρ 0.310/-0.159, respectively) and LA reservoir/conduit strain correlated to the LA volume index (ρ 0.336/-0.357, respectively). None of the individual LA strain parameters were associated with the CPET variables. In multivariate regression analysis, LA contractile strain was significantly associated with the percentage of predicted maximal heart rate (ß - 2.555). CONCLUSIONS: These data suggest that in TGA patients after ASO repair LA strain is impaired and correlates with LA size and LV function. However, impaired LA strain wasn't associated with the standard CPET parameters. As such, clinical significance needs to be further unravelled.

Pulmonary arterial pressure and right ventricular dilatation independently determine tricuspid valve insufficiency severity in pre-capillary pulmonary hypertension.

BACKGROUND AND AIM OF THE STUDY: Elevated pulmonary artery systolic pressure (PASP) causes functional tricuspid valve insufficiency (TI). However, the differential contribution of pressure load and right ventricular (RV) dilatation is not well established. The study aim was to evaluate both variables in relation to TI. METHODS: A cross-sectional study was performed of consecutive transthoracic echocardiographic studies of patients with pre-capillary pulmonary hypertension (PH). Both, demographic data and echocardiographic RV parameters were reviewed. TI was graded semi-quantitatively with color Doppler flow imaging. Trend analyses for TI severity (TI grade 0/4, 1/4, 2/4, 3/4, or 4/4) were performed. A proportional odds logistic regression analysis was carried out to identify independent predictors of TI severity. RESULTS: Eighty-one patients (56 females, 25 males; mean age 60 +/- 15 years) with pre-capillary PH were evaluated. Patients with more severe TI had a significantly lower body mass index, a lower mean systemic blood pressure, a shorter pulmonary acceleration time, a higher tricuspid regurgitant gradient, and a more dilated right ventricle. From the echocardiographic parameters, RV dilatation (p = 0.0143) and the tricuspid regurgitant gradient (p = 0.0026) were independently related to the degree of TI. CONCLUSION: In patients with pre-capillary PH, PASP and RV dilatation were both related to the increasing severity of TI. When focusing on TI to improve the prognosis of patients with pre-capillary PH, both PASP and RV dimensions should be taken into consideration.

Tricuspid valve regurgitation: prevalence and relationship with different types of heart disease.

OBJECTIVES: The clinical impact of tricuspid valve regurgitation (TR) is in most cases undetermined.This study aimed at evaluating: (1) the prevalence of TR, (2) the degree of its relationship with several types of heart disease and (3) the association of the routine echocardiographic variables with TR in each cardiac pathology. METHODS AND RESULTS: The database of the University Hospitals of Leuven was searched for all transthoracic echocardiographic studies, performed between November 2010 and January 2011, to reach a target of at least 2000 patients. The study population was divided into predefined categories. Significant TR was defined as graded > 2/4 on colour Doppler. In total 2054 consecutive echocardiographic studies were retrieved. The prevalence of significant TR was 10.2% in the total population, ranging from 1.1% to 34.4%. TR was strongly associated with pulmonary arterial hypertension (PAH) [OR 35.8 (11.7-110.3; P < 0.001)], valvular heart disease [OR 6.7 (2.3-19.2; P < 0.001)], heart transplantation [OR 10.4 (3.4-31.8; P < 0.001)], non-ischaemic cardiomyopathy [OR 6.2 (1.8-21.3; P = 0.004)], and ischaemic cardiomyopathy [OR 5.6 (1.5-21.8; P = 0.012)]. TR was significantly related to the degree of mitral valve regurgitation (MR) in all categories (P < 0.001). CONCLUSION: TR occurs frequently and is in a different degree related to the underlying heart disease. TR was also associated to MR. This might suggest valvular interdependence between the tricuspid and mitral valves.

Predictors of mid-term event-free survival in adults with corrected tetralogy of Fallot.

OBJECTIVES: Patients who underwent corrective surgery for tetralogy of Fallot (TOF) have increased long-term risk of cardiovascular morbidity and mortality. Yet, limited information is available on how to evaluate the risk in this population. Therefore, the aim of this study was to investigate the prognostic value of aerobic exercise capacity, along with other related parameters, at medium-term follow-up in adult patients with tetralogy of Fallot. METHODS AND RESULTS: Between 2000 and 2003, 92 adults (age 26.2 +/- 7.8 years; 63 male) with corrected TOF or TOF-type morphology underwent a cardiopulmonary exercise test (CPET) until exhaustion and echocardiography. During a mean follow-up of 7.3 +/- 1.2 years (range 0.9 to 9.3 years), 2 patients died and 26 patients required at least 1 cardiac-related intervention at a mean age of 28.9 +/- 7.9 years. Event-free survival tended to be higher in patients with the classical type of TOF (P = 0.061). At multivariate Cox analysis, age at CPET [hazard ratio (HR): 1.13, P = 0.006], age at correction (HR: 0.82, P = 0.037), right ventricular (RV) function (HR: 4.94, P = 0.001), QRS duration (HR: 1.02, P = 0.007), percentage of predicted peak oxygen uptake (peak VO2%) (HR: 0.96, P=0.029) and ventilatory efficiency slope (VE/VCO2 slope) (HR: 1.13, P = 0.021) were significantly related to the incidence of death/cardiac-related intervention during medium follow-up. CONCLUSIONS: Early corrective surgery and a well-preserved RV are associated with a better outcome in adults with corrected TOF. Furthermore, CPET provides important prognostic information; peak VO2% and VE/VCO2 slope are independent predictors for event-free survival in patients with corrected TOF.

Adverse functional remodelling of the subpulmonary left ventricle in patients with a systemic right ventricle is associated with clinical outcome.

AIMS: Early recognition of adverse remodelling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to short-term clinical evolution in this population. METHODS AND RESULTS: Thirty-three patients (76% male) with sRV (atrial switch repair for D-transposition of the great arteries and congenitally corrected transposition of the great arteries) underwent detailed phenotyping including exercise cardiac magnetic resonance and were followed over mean follow-up time of 3 years. Mean age was 40 ± 8 (range 26-57) years at latest follow-up. Adverse outcome was a composite of heart failure (HF) and tachyarrhythmia. Descriptive statistics and univariate cox regression analyses were performed. When compared with baseline: (i) most patients remained in New York Heart Association functional class I (76%), (ii) the degree of severity of the systemic atrioventricular valve regurgitation rose, and (iii) more electrical instability was documented at latest follow-up. Six (18%) of a total of 9 events were counted as first cardiovascular events (9% HF and 9% arrhythmia). NT-proBNP, oxygen pulse, left ventricle end-diastolic volume index (LVEDVi), and stroke volume index (SVi) of the subpulmonary left ventricle (LV) both in rest and at peak exercise were significantly associated with the first cardiovascular event. CONCLUSION: NT-proBNP was by far the best prognostic marker for clinical outcome. Adverse remodelling with increase of LVEDVi and SVi of the subpulmonary LV at rest and during exercise were associated with worse clinical outcome. We theorize that remodelling of the subpulmonary ventricle might be an early sign of a failing sRV circulation.