External Validation of a Risk Score Model for Predicting Major Clinical Events in Adults After Atrial Switch.

BACKGROUND: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model. METHODS AND RESULTS: A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty-five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell C index of 0.73 [95% CI, 0.65-0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03-0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function. CONCLUSIONS: We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.

Aortic valve replacement in non-elderly: the gap between reality, guidelines and evidence.

OBJECTIVES: There are several treatment options for non-elderly adults (18-60 years) in need of aortic valve replacement (AVR): a mechanical or biological substitute, a homograft, the Ross procedure or nowadays even transcatheter aortic valve replacement. This study evaluated the current opinions and practices of cardiac surgeons and cardiologists concerning AVR strategies in this patient group. METHODS: A 43-item online survey was sent out to several scientific organizations, individual cardiac surgeons, cardiologists and residents/fellows worldwide. RESULTS: Two hundred twenty-two physicians from 33 different countries answered the survey. The UK (24%), Belgium (23%) and North America (22%) are best represented. A mechanical valve is the most frequently used substitute in non-elderly. With increasing patient age, the popularity of a biological valve increases. Transcatheter aortic valve replacement and the Ross procedure are each used in <10% of this cohort. Only 12% of the cardiac surgeons has experience with the Ross surgery. In 23%, the patient seems not to be involved in the decision on the type of valve substitute. CONCLUSIONS: The preferred surgical treatment for non-elderly patients in need of AVR differs widely. To enable a shared decision-making process including patient preferences, there is more need for collaboration between cardiac departments, as well as education and scientific research about patient-centred outcomes of the different AVR techniques.

Preterm Birth Is Associated With Adverse Cardiac Remodeling and Worse Outcomes in Patients With a Functional Single Right Ventricle.

OBJECTIVE: To assess the effects of preterm birth on cardiac structure and function and transplant-free survival in patients with hypoplastic left heart syndrome and associated anomalies throughout the staged palliation process. STUDY DESIGN: Data from the Single Ventricle Reconstruction trial were used to assess the impact of prematurity on echocardiographic measures at birth, Norwood, Stage II, and 14 months in 549 patients with a single functional right ventricle. Medical history was recorded once a year using medical records or telephone interviews. Cox regression models were applied to analyze transplant-free survival to age 6 years. Causal mediation analysis was performed to estimate the mediating effect of birth weight within this relationship. RESULTS: Of the 549 participants, 64 (11.7%) were born preterm. Preterm-born participants had lower indexed right ventricle end-diastolic volumes at birth but higher volumes than term-born participants by age 14 months. Preterm-born participants had an increased risk of death or heart transplantation from birth to age 6 years, with an almost linear increase in the observed risk as gestational age decreased below 37 weeks. Of the total effect of preterm birth on transplant-free survival, 27.3% (95% CI 2.5-59.0%) was mediated through birth weight. CONCLUSIONS: Preterm birth is associated with adverse right ventricle remodeling and worse transplant-free survival throughout the palliation process, in part independently of low birth weight. Further investigation into this vulnerable group may allow development of strategies that mitigate the impact of prematurity on outcomes in patients with hypoplastic left heart syndrome.

MeVisLab-OpenVR prototyping platform for virtual reality medical applications.

PURPOSE: Virtual reality (VR) can provide an added value for diagnosis and/or intervention planning. Several VR software implementations have been proposed but they are often application dependent. Previous attempts for a more generic solution incorporating VR in medical prototyping software (MeVisLab) were still lacking functionality precluding easy and flexible development. METHODS: We propose an alternative solution that uses rendering to a graphical processing unit (GPU) texture to enable rendering arbitrary Open Inventor scenes in a VR context. It facilitates flexible development of user interaction and rendering of more complex scenes involving multiple objects. We tested the platform in planning a transcatheter cardiac stent placement procedure. RESULTS: This approach proved to enable development of a particular implementation that facilitates planning of percutaneous treatment of a sinus venosus atrial septal defect. The implementation showed it is intuitive to plan and verify the procedure using VR. CONCLUSION: An alternative implementation for linking OpenVR with MeVisLab is provided that offers more flexible development of VR prototypes which can facilitate further clinical validation of this technology in various medical disciplines.

Case report: Two sisters with light-chain cardiac amyloidosis, a mere coincidence?

Background: Light-chain amyloidosis has always been described as a sporadic disease caused by plasma cell dyscrasia. Cardiac amyloidosis refers to cardiac involvement with infiltration of amyloid fibrils in the myocardium. The degree of cardiac involvement is the greatest predictor of prognosis. To our knowledge, AL cardiac amyloidosis has only been reported once before in first-degree relatives. Case summary: In this report, we describe the unusual cases of two sisters with light-chain cardiac amyloidosis. The first patient underwent autologous stem cell transplantation and remained in remission for 10 years until the disease relapsed and she died of end-stage heart failure. The second patient was promptly started on a chemotherapy regimen but died shortly after her initial diagnosis due to rapid progression of cardiac dysfunction. Conclusion: Cardiac amyloidosis is a severe life-threatening condition which requires a multidisciplinary diagnostic and therapeutic approach. Based on this case report, a genetic cause for AL amyloidosis might be suspected or is this a purely coincidental finding? Counselling, screening, and follow-up of other family members are very challenging. As is often the case with rare diseases, many unsolved questions remain, representing important challenges for clinicians.

Starting an aortic valve repair program: is it worthwhile? Aortic valve repair compared to replacement.

OBJECTIVES: Increasing evidence suggests that aortic valve (AV) repair may have better long-term outcomes than AV replacement for aortic insufficiency. However, most literature is limited to centres of excellence and has not sufficiently been replicated in nascent programs. This single-centre experience of a newly established AV repair program aims to compare short-term outcomes of AV sparing versus replacement surgery. METHODS: A retrospective cohort study of patients who underwent elective surgery for aortic regurgitation or aortic root dilatation at the Leuven University Hospital between 2013 and 2018 was performed. Patients with a critically ill presentation, endocarditis, aortic stenosis or requiring redo surgery were excluded. Patients were assigned to repair versus replacement based on preoperative intention to preserve the AV. Nearest neighbour propensity score matching was performed to compare both groups. Safety (mortality, morbidity), efficiency (cross-clamp and bypass times) and efficacy end points (repair rate, postoperative echocardiography) were compared. RESULTS: One hundred and seven patients underwent AV surgery (48 repair, 59 replacement), from which 2 groups of 23 matched patients were created. There were 1 death and 2 reoperations after repair and no death and 1 reoperation after replacement. Extracorporeal circulation and aortic cross-clamp time were significantly longer while ventilation and total hospital stay were significantly shorter after AV repair. Echocardiographic follow-up showed comparable aortic regurgitation but lower transvalvular gradients after repair. Freedom from major complications was comparable in both cohorts. CONCLUSIONS: Early results suggest the feasibility of replicating experienced centres' perioperative and short-term outcomes in nascent programs.

Iron Deficiency Is Associated With Impaired Biventricular Reserve and Reduced Exercise Capacity in Patients With Unexplained Dyspnea.

BACKGROUND: Iron deficiency (ID) is frequent and associated with diminished exercise capacity in heart failure (HF), but its contribution to unexplained dyspnea without a HF diagnosis at rest remains unclear. METHODS AND RESULTS: Consecutive patients with unexplained dyspnea and normal echocardiography and pulmonary function tests at rest underwent prospective standardized cardiopulmonary exercise testing with echocardiography in a tertiary care dyspnea clinic. ID was defined as ferritin of <300 µg/L and a transferrin saturation of <20% and its impact on peak oxygen uptake (peakVO2), biventricular response to exercise, and peripheral oxygen extraction was assessed. Of 272 patients who underwent cardiopulmonary exercise testing with echocardiography, 63 (23%) had ID. For a similar respiratory exchange ratio, patients with ID had lower peakVO2 (14.6 ± 7.6 mL/kg/minvs 17.8 ± 8.8 mL/kg/min; P = .009) and maximal workload (89 ± 50 watt vs 108 ± 56 watt P = .047), even after adjustment for the presence of anemia. At rest, patients with ID had a similar left ventricular and right ventricular (RV) contractile function. During exercise, patients with ID had lower cardiac output reserve (P < .05) and depressed RV function by tricuspid s' (P = .004), tricuspid annular plane systolic excursion (P = .034), and RV end-systolic pressure-area ratio (P = .038), with more RV-pulmonary artery uncoupling measured by tricuspid annular plane systolic excursion/systolic pulmonary arterial pressure ratio (P = .023). RV end-systolic pressure-area ratio change from rest to peak exercise, as a load-insensitive metric of RV contractility, was lower in patients with ID (2.09 ± 0.72 mm Hg/cm2 vs 2.58 ± 1.14 mm Hg/cm2; P < .001). ID was associated with impaired peripheral oxygen extraction (peakVO2/peak cardiac output; P = .036). Cardiopulmonary exercise testing with echocardiography resulted in a diagnosis of HF with preserved ejection fraction in 71 patients (26%) based on an exercise E/e' ratio of >14, with equal distribution in patients with (28.6%) or without ID (25.4%, P = .611). None of these findings were influenced in a sensitivity analysis adjusted for a final diagnosis of HFpEF as etiology for the unexplained dyspnea. CONCLUSIONS: In patients with unexplained dyspnea without clear HF at rest, ID is common and associated with decreased exercise capacity, diminished biventricular contractile reserve, and decreased peripheral oxygen extraction.

Assessment of long-term cardiac adaptation in adult patients with type II atrial septal defect : A cardiovascular magnetic resonance (CMR) study.

OBJECTIVES: In type II atrial septal defect (ASD) patients, the left-to-right (LR) shunt causes adaptation of the heart and circulation. The study objective was to evaluate with cardiovascular magnetic resonance imaging (CMR) the impact of LR shunt on left (LV) and right ventricular (RV) volumes, function, and myocardial strain. METHODS: Thirty-five patients (42 ± 17 years, 17 male) were compared to a control group (n = 40). Cine imaging was used to calculate ventricular volumes and ejection fraction (EF), global longitudinal (GLS) and circumferential strain (GCS), and longitudinal free wall (FWS) and interventricular septal (IVS) strain. Phase-contrast imaging was used to calculate pulmonary flow to systemic flow ratio (Qp/Qs). RESULTS: The LR shunt (Qp/Qs 2.2 ± 0.6) resulted in larger RV end-diastolic volume (EDVi) (152 ± 42 vs 82 ± 11 ml/m2), lower LV EDVi (72 ± 16 vs 83 ± 9 ml/m2), and higher RV/LV EDVi ratio (2.2 ± 0.5 vs 1.0 ± 0.1) than controls (all p < 0.001). Functionally, stroke volumes were larger in RV and lower in LV (both p < 0.001) with a strong trend toward lower RV EF in patients (p = 0.08). The LR shunt negatively impacted RV GLS (p = 0.03) but not RV GCS. Longitudinal IVS but not RV FWS were significantly lower in patients, i.e., p < 0.001, of longitudinal IVS. Shunt severity correlated with RV size and stroke volume, right atrial size, and pulmonary trunk diameter (all p < 0.001), but not with functional nor strain parameters. CONCLUSION: Long-term cardiac adaptation in ASD patients, with RV overfilling and LV underfilling, has a negative impact on systolic RV performance, a phenomenon which likely can be attributed to longitudinal dysfunction of the interventricular septum. KEY POINTS: • An LR shunt in type II ASD patients causes cardiac remodeling characterized by RV overfilling and conversely underfilling of the left ventricle. • At the long term, there is evidence of systolic dysfunction of the right ventricle in this group of patients. • Septal dysfunction underlies the observed impairment in RV function.

Outcome of arterial switch operation for transposition of the great arteries. A 35-year follow-up study.

BACKGROUND: Arterial switch operation (ASO) is today the first-choice surgical treatment for patients with transposition of the great arteries. Long-term outcome data still remain scarce. Moreover, the course of these patients is not uneventful. Therefore, we wanted to evaluate long-term outcome and determine on which variables to focus during follow-up. METHODS: Clinical records of 318 patients who underwent ASO between October 1981 and July 2018 were reviewed. Perioperative, post-operative, and interventional data were collected to determine mortality and the need for re-intervention. Descriptive statistics and Kaplan-Meier survival analysis were performed. RESULTS: Mean follow-up time was 11.1 SD 8.5 years (range 0-35) with a mean age of 12.5 SD 9.0 years (range 0-37) at latest follow-up. In-hospital mortality was 7.5% and overall survival 90.9% for a maximum follow-up time of 35 years. Causes of early mortality were cardiogenic shock, severe pulmonary hypertension, septic shock and multiple organ failure. Causes of late mortality were cardiogenic shock, severe pulmonary hypertension, pacemaker lead fracture and fire death. Re-intervention free survival at 5-year was 91.6%, at 10-year 90.7%, at 20-year 79.2%. For all survivors, the most frequent sequelae after ASO were pulmonary artery stenosis (80.9%), of which 13.5% needed an intervention. The threshold for intervening on lesions at the level of the pulmonary artery bifurcation was higher and the percutaneous re-intervention rate was higher for non-bifurcation lesions. CONCLUSIONS: Despite a relatively high peri-operative mortality, TGA patients have an excellent overall long-term survival. However, a large proportion of patients requires re-interventions, mainly for pulmonary artery stenosis.

Outcome of the Glenn procedure as definitive palliation in single ventricle patients.

OBJECTIVES: In selected single ventricle patients, a Glenn procedure (SV-Glenn) may be considered as definitive palliation. Either the patient is unsuited to progress to a Fontan circulation or a SV-Glenn circulation is preferred. This study aimed at describing the clinical course, and long-term mortality/morbidity of SV-Glenn patients. METHODS: All SV-Glenn patients followed at the University Hospitals Leuven before May 2018 were included. Patients who underwent, or were awaiting, TCPC completion and those who underwent a Glenn in the setting of a biventricular circulation one-and-a-half repair (OAHR), were excluded. RESULTS: Of 65 Glenn-only patients identified, 21 (32%) had OAHR, whereas 44 (68%) were SV-Glenn patients. Of SV-Glenn patients, 19 died within 6 months after the Glenn procedure. Of 25 SV-Glenn survivors, median age at Glenn was 6.3 (IQR 1.2-29.7) years. Eight were unsuited for TCPC completion; in 17 SV-Glenn was preferred over TCPC completion. Over a median follow-up time of 11 (IQR 3-18) years after the Glenn procedure, 5 (20%) patients died. At latest follow-up 10 (40%) had heart failure, 5 (20%) had atrial and 4 (16%) ventricular arrhythmias, 2 (8%) a thromboembolic event, 7 (28%) required pacemaker implantation, and 2 (8%) had infective endocarditis but none developed cirrhosis or protein-losing enteropathy. Mean saturation at latest follow-up was 87 ± 7%. CONCLUSION: SV-Glenn patients represent a unique and heterogeneous patient population. Outcome was reasonable, although comorbidities, such as heart failure and arrhythmias were not uncommon. In SV-Glenn patients, 'classic' complications related to Fontan physiology, such as cirrhosis and protein-losing enteropathy, were absent.

Heart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care.

There is mounting recognition that some of the most urgent problems of adult congenital heart disease (ACHD) are the prevention, diagnosis, and management of heart failure (HF). Recent expert consensus and position statements not only emphasize a specific and pressing need to tackle HF in ACHD (ACHD-HF) but also highlight the difficulty of doing so given a current sparsity of data. Some of the challenges will be addressed by this review. The authors are from 3 different centres; each centre has an established subspeciality ACHD-HF clinic and is able to provide heart transplant, multiorgan transplant, and mechanical support for patients with ACHD. Appropriate care of this complex population requires multidisciplinary ACHD-HF teams evaluate all possible treatment options. The risks and benefits of nontransplant ACHD surgery, percutaneous structural and electrophysiological intervention, and ongoing conservative management must be considered alongside those of transplant strategies. In our approach, advanced care planning and palliative care coexist with the consideration of advanced therapies. An ethos of shared decision making, guided by the patient's values and preferences, strengthens clinical care, but requires investment of time as well as skilled communication. In this review, we aim to offer practical real-world advice for managing these patients, supported by scientific data where it exists.

Advanced care planning in adult congenital heart disease: Transitioning from repair to palliation and end-of-life care.

As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2]. The scientific adult CHD (ACHD) community has been working hard to identify variables related to worse outcomes, modifying those where possible in order to improve survival. Indeed, survival in adults with CHD has increased, but consequently, on top of CHD-related complications, patients are increasingly exposed to the standard cardiovascular risk factors. Therefore, a program for lifelong coaching on health behavior and life style management becomes indispensable. More emerging is that a substantial number of patients, in particular those with complex heart defects, will eventually end up in a stage with hardly any medical or interventional options left. Our healthcare provision has to be prepared to organize care for this specific group of patients who will die prematurely and require the timely development and establishment of advanced care planning. Advanced care planning should preferentially be set-up in expert CHD centers. The long-lasting relationship in ACHD care with healthcare providers offers an excellent basis with regards to prognosis, advanced care planning and end-of-life issues.

Postoperative left ventricular function in different types of pulmonary hypertension: a comparative study.

OBJECTIVES: Left ventricular dysfunction after pulmonary endarterectomy is well described. Left ventricular failure has only been described after lung transplantation for pulmonary arterial hypertension (PAH). We sought to identify factors that contribute to this failure and hypothesized that atrial septostomy before transplantation could prevent this complication. METHODS: From our database, all bilateral lung transplants for PAH (n = 24) and all pulmonary endarterectomies, with a minimal reduction of 800 dyn⋅s⋅cm-5 (n = 27), were selected. Perioperative demographic and echocardiographic data were analysed. RESULTS: In patients with PAH, pulmonary hypertension was diagnosed at a significantly younger age, and time between diagnosis and surgery was significantly longer. Before surgery, right ventricular dimensions were significantly larger and left ventricular wall thicknesses were significantly smaller, but left ventricular diastolic dysfunction was similar. Surgery caused a significant decrease in right ventricular dimensions (less extensive after pulmonary endarterectomy) and caused a significant increase in left ventricular dimensions. Pre-transplant atrial septostomy caused increased left ventricular dimensions, stroke volume and cardiac index. Two patients developed post-transplant left ventricular failure. Compared with other PAH patients, they were younger (<12 years) at diagnosis, time between diagnosis and surgery lasted 2.5 times longer, left ventricular mass was smaller and pre-transplant pulmonary vascular resistance was higher. CONCLUSIONS: In PAH, age at diagnosis is younger and left ventricular preload deprivation lasts longer than that in chronic thromboembolic pulmonary hypertension. Together with lower residual pulmonary vascular resistance and higher increases in preload, this might explain left ventricular failure after lung transplantation. Pre-transplant atrial septostomy might prevent post-transplant left ventricular failure.

Pulmonary Valve Procedures Late After Repair of Tetralogy of Fallot: Current Perspectives and Contemporary Approaches to Management.

Few topics in adult congenital heart disease have approached the level of scrutiny bestowed on pulmonary valve replacement (PVR) strategies late after tetralogy of Fallot (TOF) repair. Despite the successes of primary surgery for TOF, there is a growing group of adults with residual right ventricular outflow tract and pulmonary valve dysfunction. Patients with residual chronic pulmonic regurgitation as a consequence of earlier surgery can later develop symptoms of exercise intolerance and complications including heart failure, tachyarrhythmias, and sudden cardiac death. Optimal timing of PVR has sparked debate, which has catalyzed increasing research efforts over the past decade. Although performance of PVR in the absence of symptoms is currently on the basis of the rationale that achievement of complete reverse remodelling is highly desirable, whether this approach results in improvement in patient outcomes in the long-term has yet to be shown. Surgical PVR and percutaneous pulmonary valve intervention are different techniques with specific advantages and disadvantages that require careful consideration for each individual patient, alongside the need for requisite reinterventions over the course of a patient's lifetime. Criteria pertaining to referral strategies are ever being refined as newer technologies for percutaneous therapies continue to evolve. In this article we review the literature surrounding the indications for, the optimal timing of, and the approaches to pulmonary valve procedures in adults with previously repaired TOF.

Early versus late pulmonary valve replacement in patients with transannular patch-repaired tetralogy of Fallot.

OBJECTIVES: Although the effects of pulmonary regurgitation after tetralogy of Fallot repair are detrimental, timing of pulmonary valve replacement (PVR) is unclear. Our goal was to evaluate the midterm efficacy and safety of early PVR. METHODS: Patients with tetralogy of Fallot who underwent repair from 1962 to 2015 were included from the local database. Statistical analyses compared patients who underwent early PVR (age ≤16 years), late PVR and no PVR. The timing of the intervention was compared for efficacy-all-cause mortality and the combined end-point of all-cause mortality, ventricular tachycardia and defibrillator implantation-and for safety-the combined end-point of 1-year postoperative mortality after PVR, endocarditis and reintervention. Echocardiographic and electrocardiographic data at the last follow-up examination were compared across the 3 groups. RESULTS: Two hundred seventy-three patients (age 21 ± 5 years; 52% female) were included. The mean follow-up was 24 (95% confidence interval 22.7-26.2) years; the observed median was 21 years (interquartile range 11-31). No significant difference in survival was found between the early PVR (n = 106; 39%), the late PVR (n = 47; 17%) and the no PVR groups (n = 120; 44%) (P = 0.990). No significant difference in the combined efficacy end-point was noted between patients who underwent early PVR compared with patients who underwent late PVR (P = 0.247). Worse event-free survival for the 3-point safety end-point was observed after early PVR (P < 0.001). Right ventricular morphology (P < 0.001) and function (P < 0.001) were better preserved in the patient group that underwent PVR before the age of 16 years. CONCLUSIONS: As expected, PVR-related morbidity was higher in patients who underwent early PVR but the midterm outcome was similar. Nevertheless, better preservation of right ventricular morphology and function in the early PVR group might result in better long-term survival.

Additional tricuspid annuloplasty in mitral valve surgery results in better clinical outcome.

OBJECTIVE: The clinical benefit of tricuspid annuloplasty (TA) in patients undergoing mitral valve surgery (MVS) is still debated. We evaluated the immediate surgical success, postoperative outcome and the medium-term effect of TA in MVS. METHODS: Patients were included between September 2003 and December 2009 and followed until September 2013 to achieve a median follow-up time of 5 years (IQR 3.7-6.9). The end point of mortality due to cardiac causes and combined end point of cardiac mortality or hospitalisation for heart failure were evaluated. Propensity score adjusted Cox regression was used to evaluate the clinical benefit of TA at the time of MVS. RESULTS: Of 150 patients (84 female; 67±12 years), 82 presented with tricuspid regurgitation (TR) <2/4 and underwent isolated MVS. Of 68 patients presenting with TR≥2/4, 31 underwent isolated MVS whereas 37 underwent additional TA. In patients with preoperative TR≥2/4, TR was significantly reduced until 5 years postoperatively (mean reduction 0.81±1.31; p=0.04) when additional TA was done. The combined end point occurred in 29% vs 6% at 1 year and in 57% vs 39% at 5 years follow-up for patients with isolated MVS and patients undergoing concomitant TA, respectively. Patients with preoperative TR≥2/4 had worse unadjusted survival than those with TR<2/4 (logrank p=0.009). In the patients with TR≥2/4, propensity score-adjusted risk for the combined end point was higher in those with isolated MVS versus MVS with additional TA (Cox HR 2.855 (1.082-7.532), p=0.035). CONCLUSIONS: Additional TA is an effective surgical measure to reduce functional TR severity. This approach results in a decreased risk of cardiac mortality and hospitalisation in patients with preoperative TR≥2/4.

Prediction of hemodynamic improvement after pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension using non-invasive imaging.

Pulmonary endarterectomy (PEA) is the recommended treatment in chronic thromboembolic pulmonary hypertension (CTEPH). Prediction of outcome after PEA remains challenging. In search for pre-operative predictors we evaluated non-invasive parameters measured by chest CT-scan and echocardiography. Between May 2004 and January 2009, 52 consecutive patients with CTEPH who underwent PEA (59.6 % female, mean age 58.9 ± 13.4 years) were included. Prior to surgery, pulmonary artery (PA) diameter indices were calculated by chest CT scan and different echocardiographic measurements to evaluate pulmonary hypertension were obtained. Hemodynamic improvement after PEA was defined as a pulmonary vascular resistance (PVR) <500 dyn s cm(-5) and a mean pulmonary artery pressure <35 mmHg 3 days after PEA. Mortality was evaluated at day 30. Mean pulmonary artery pressure (PAP) at baseline was 40.1 ± 8.5 mmHg, with a PVR of 971 ± 420 dyn s cm(-5). Persistent pulmonary hypertension was observed in 15 patients (28.8 %). Gender, pre-operative mean PAP, PA diameter indices, and tricuspid annular plane systolic excursion were all predictors for hemodynamic improvement after PEA. The indexed PA diameter on CT was the only independent predictor for hemodynamic improvement: 19.4 ± 2.4 versus 22.9 ± 4.9 mm/m(2) in those without improvement (OR 0.76: 95 % CI 0.58-0.99, p = 0.04). All patients who died within 30 days (9.6 %) had persistent pulmonary hypertension, with a post-operative mean PAP of 51.6 ± 14.1 mmHg and PVR of 692 ± 216 dyn s cm(-5). The pre-operative PA diameter indexed for body surface area is the only independent predictor for hemodynamic improvement after PEA in CTEPH patients. In all patients who died within 30 days after PEA, persistent pulmonary hypertension was present.

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.

Outcome and determinants of prognosis in patients undergoing isolated tricuspid valve surgery: retrospective single center analysis.

AIMS: Although tricuspid valve (TV) surgery has become more popular, isolated TV surgery is infrequently performed. The aims of this study were (1) to evaluate the postoperative and long-term mortality of patients undergoing isolated TV surgery, (2) to compare the outcomes of patients undergoing their first TV surgery or TV reoperation, and (3) to assess the additive value of echocardiographic and invasive hemodynamic evaluations for predicting postoperative outcome. METHODS: We followed a contemporary cohort of patients undergoing isolated TV surgery from January 1, 1995, through December 31, 2011. Preoperative demographic, echocardiographic, hemodynamic, and operative data were included. Outcome was all-cause mortality. RESULTS: Ninety-two patients (38% male; mean age: 56±14 years) were included. Kaplan-Meier survival analyses showed that 30-day, 3-month, 5-year, and 10-year mortality were 7.9%, 15.2%, 25.7%, and 53.7%, respectively. No difference in outcome was found between patients undergoing first TV surgery (n=61) and TV reoperation (n=31) (p=0.669). Univariable Cox analysis identified age (p<0.0001), extracardiac vascular disease (p=0.001), glomerular filtration rate (p=0.022), NYHA classification (p=0.010), and mean pulmonary artery pressure (p=0.005) as predictors of mortality. Multivariable analysis identified significant associations with outcome, only for age (p=0.010) and NYHA functional class (p=0.044). In younger patients (<59 years), mean pulmonary artery pressure was associated with the worse outcome (p=0.024). CONCLUSIONS: Isolated TV surgery is still associated with important postoperative and long-term mortality, both for first TV surgery and TV reoperation. Pre-operative NYHA functional class and, in younger patients, pulmonary hypertension appear to determine prognosis.