Degree Of Regurgitation Research Articles

NT-proBNP as Marker of Ventricular Dilatation and Pulmonary Regurgitation After Surgical Correction of Tetralogy of Fallot: A MRI Validation Study.

The goal of this study is to evaluate whether NT-proBNP plasma levels may help as a screening biomarker for monitoring right ventricular dilatation, pulmonary regurgitation and the onset of heart failure in patients with repaired Tetralogy of Fallot. Our single-centre observational prospective study involved 43 patients (15.1years, SD=8) with corrected Tetralogy of Fallot. Data collection included: clinical parameters (electrocardiogram, chest X-ray, NYHA scale, time since last surgery), biochemistry (NT-proBNP levels) and MRI values (ventricular volumetry, pulmonary flow assessment). Mean time since last surgery was 13.5years (SD=7.8). There was a statistically significant correlation between the NT-proBNP levels (187.4pg/ml, SD=154.9) and right ventricular dilatation for both the right ventricular end-diastolic volume (124.9ml/m2, SD=31.2) (Pearson=0.19, p<0.01) and end-systolic volume (56.1ml/m2, SD=18.8) (Pearson=0.21, p<0.01) and also with the pulmonary regurgitation fraction (36.5%, SD=16, Pearson=0.12, p<0.01). No significant correlation was found between NT-proBNP and right ventricular ejection fraction (54.6%, SD=10.6, Pearson=-0.07), left ventricular ejection fraction (59.9%, SD=7.1, Pearson=-0.18) or any clinical parameters. The receiver operating curve analysis evidenced that a NT-proBNP cut-off value above 133.2pg/ml predicted the presence of dilated right ventricular end-diastolic and end-systolic volumes over centile 95 (sensitivity 82 and 83% and specificity 93 and 79%, respectively). In conclusion, in patients with surgically corrected Tetralogy of Fallot, NT-proBNP levels correlate with right ventricular dilatation and the degree of pulmonary regurgitation. Ambulatory determination of NT-proBNP might be an easy, readily available and cost-effective alternative for MRI follow-up evaluation of these patients.

Aortic valve repair in the paediatric population: insights from a 38-year single-centre experience.

To analyse our institutional results in the setting of paediatric aortic valve (AV) repair. Primary end-points were overall survival, freedom from AV reoperation and freedom from AV replacement. A retrospective analysis of all patients under 18 years of age operated on from 1977 to 2015 in a single tertiary care level institution. Patients were included if they benefited from any type of AV repair procedure, including commissurotomy, leaflet shaving or plication, or leaflet augmentation. All data were gathered from patients' medical records, operative reports and referring paediatric cardiologists. The median follow-up was 50 months (IQR [13-140]). Sixty-six patients were included. Indications for surgery were aortic stenosis, aortic regurgitation and mixed disease in 13 (19%), 36 (55%) and 17 (26%) patients, respectively. According to El Khoury's functional classification, among the 55 patients with some degree of regurgitation there were 5 type Ib regurgitation, 23 type II and 27 type III. During AV repair, additional procedures were performed in 36 patients, VSD closure, subaortic membrane resection and mitral valve repair being the most frequent (18, 8 and 7 patients). RACHS score was predominantly 2 (98.5% of patients). The in-hospital mortality rate was 1.5% (1/66). Major morbidity included 10 pericardial effusions (1 pericardocentesis), 1 low cardiac output syndrome and 1 stroke. There were three late deaths (at 104, 140 and 179 months after repair). All were cardiac related. Overall 5- and 10-year survival rates were 100 and 95.7%. The rates of freedom from AV reoperation and AV replacement at 5 and 10 years were 90.6, 72.1 and 92.5, 82.7%, respectively. During follow-up, there was no occurrence of valve-related complication (endocarditis, thromboembolism and bleeding). In our experience, AV repair in the paediatric population provides excellent results in terms of both overall survival and valve-related reoperation. It obviates the need for chronic anticoagulation and in most cases delays the time at which more complex surgery such as the Ross procedure should be undertaken.

Timing of Surgical Intervention for Aortic Regurgitation.

Aortic regurgitation is a frequently encountered condition, in which traditional measurements of severity have proven to be of limited value in identifying those who would be best served by aortic valve replacement. Novel methods of assessing severity are vital, particularly as an entirely new paradigm of aortic regurgitation has surfaced, with the advent of transcatheter aortic valve replacement (TAVR), and the adverse events that are being observed with varying degrees of aortic regurgitation. With that in mind, a comprehensive assessment of aortic regurgitation should now include indexed left ventricular systolic volumes and a comprehensive assessment of right ventricular function, in addition to the quantitative measures that are currently recommended. Cardiac MRI also provides valuable information and should be strongly considered, particularly in challenging cases. The incremental value of additional echocardiographic parameters such as strain imaging, speckle tracking imaging, and tissue Doppler imaging remains unclear, and evidence for their utility is not, as yet, compelling. However, the field of aortic regurgitation assessment has been reinvigorated by the prevalence of paravalvular regurgitation post-TAVR, and many of the abovementioned parameters may need to be re-visited so that we can more accurately determine prognosis and risk stratify patients in a more reliable and evidence-based manner.

Sinus Plication to Improve Valve Configuration in Bicuspid Aortic Valve Repair—Early Results

Previous experience with repair of the regurgitant bicuspid aortic valve (BAV) has shown a strong influence of commissural orientation on repair durability. We have modified commissural orientation by asymmetric plication of the fused sinuses. We analyzed the results of the modified technique. Between 2009 and 2014, 35 patients with BAV and commissural orientation of no more than 160° underwent aortic valve repair, including plication of the fused sinuses and circular annuloplasty. The control group consisted of 21 historic controls undergoing operations between 2000 and 2008 but without sinus plication; subcommissural plication was used as annuloplasty. The groups were similar in most respects; differences existed in preoperative annular diameter, prevalence of complete fusion, use of a pericardial patch, and plication of the fused cusp. At discharge the degree of aortic regurgitation (AR) was significantly lower in the study group (p= 0.004). Survival after 5 years was 100% in the study group and 95.2% in the control group after 5 and 10 years. Five-year freedom from reoperation in the study group was 93.0% and 57.1% in the control group (p= 0.0013); freedom from valve replacement was 95.8% and 79.3% (p= 0.036). Freedom from recurrent AR grade II or higher was 67.1% in the study group and 33.3% in the control group (p= 0.0024). Mean postoperative peak gradient was significantly lower in the study group (14.3 ± 6.5 mm Hg versus 28.9 ± 18.5 mm Hg, p= 0.003). Plicating the fused sinuses and thus reducing root circumference in the fused part changes commissural orientation of the BAV which leads to better short- and midterm stability in BAV repair.

Erratum to: Surgical indication for functional tricuspid regurgitation at initial operation: judging from long term outcomes.

The assessment and management of tricuspid valve disease have evolved substantially during the past several years. Whereas tricuspid stenosis is uncommon, tricuspid regurgitation is frequently encountered and it is most often secondary due to annular dilatation and leaflet tethering from right ventricular remodelling. The indications for tricuspid valve surgery to treat tricuspid regurgitation are several and mainly related to the underlying disease, to the severity of insufficiency and to the right ventricular function. Surgical tricuspid repair has been avoided for years, because of the misleading concept that tricuspid regurgitation should disappear once the primary left-sided problem has been eliminated. Instead, during the last decade, many investigators have reported evidence in favor of a more aggressive surgical approach to functional tricuspid regurgitation, recognising the risk of progressive tricuspid insufficiency in patients with moderate or lesser degrees of tricuspid regurgitation and tricuspid annular dilatation. This concept, along with the long-term outcomes of principal surgical repair techniques are reported and discussed. Last, novel transcatheter therapies have begun to emerge for the treatment of severe tricuspid regurgitation in high-risk patients. Hence, very preliminary pre-clinical and clinical experiences are illustrated. The scope of this review is to explore the anatomic basis, the pathophysiology, the outcomes and the new insights in the management of functional tricuspid regurgitation.

Early results of valve-sparing ascending aortic replacement in type A aortic dissection and aortic insufficiency

&lt;p&gt;&lt;strong&gt;Aim:&lt;/strong&gt; The study was designed to investigate predictors of effective valve-sparing ascending aortic replacement in patients with Stanford type A aortic dissection combined with aortic insufficiency and to analyze efficacy and safety of this kind of surgery.&lt;br /&gt;&lt;strong&gt;Methods:&lt;/strong&gt; From January 2010 to December 2015, 49 patients with Stanford type A aortic dissection combined with aortic insufficiency underwent ascending aortic replacement. All patients were divided into 3 groups: valve-sparing procedures (group 1, n = 11), combined aortic valve and supracoronary ascending aortic replacement (group 2, n = 12), and Bentall procedure (group 3, n = 26). We assessed the initial status of patients, incidence of complications and efficacy of valve-sparing ascending aortic replacement.&lt;br /&gt;&lt;strong&gt;Results:&lt;/strong&gt; The hospital mortality rate was 8.2% (4/49 patients). The amount of surgical correction correlated with the initial diameter of the aorta at the level of the sinuses of Valsalva. During the hospital period, none of patients from group 1 developed aortic insufficiency exceeding Grade 2 and the vast majority of patients had trivial aortic regurgitation. The parameters of cardiopulmonary bypass, cross-clamp time and circulatory arrest time did not correlate with the initial size of the ascending aorta and aortic valve blood flow impairment, neither did they influence significantly the incidence and severity of neurological complications. The baseline size of the ascending aorta and degree of aortic regurgitation did not impact the course of the early hospital period.&lt;br /&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Supracoronary ascending aortic replacement combined with aortic valve repair in ascending aortic dissection and aortic regurgitation is effective and safe. The initial size of the ascending aorta and aortic arch do not influence immediate results. The diameter of the aorta at the level of the sinuses of Valsalva and the condition of aortic valve leaflets could be considered as the limiting factors. Further long-term follow-up is needed.&lt;/p&gt;&lt;div class="well well-small"&gt;&lt;strong&gt;Funding&lt;/strong&gt;&lt;/div&gt;&lt;p&gt;&lt;strong&gt;&lt;/strong&gt; The study has been performed within the framework of the 2015-2017 government task, “Cardiovascular diseases” platform, Theme No. 4 Research on genome/cellular mechanisms responsible for aorta/aortic valve pathology development and elaboration of new methods of its multimodality treatment including hybrid technologies.&lt;br /&gt;&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conflict of interest&lt;/strong&gt;&lt;/p&gt;&lt;p&gt;&lt;strong&gt;&lt;/strong&gt;The authors declare no conflict of interest.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Acknowledgement&lt;/strong&gt;&lt;/p&gt;&lt;p&gt;The authors express their deep gratitude for assistance in diagnostics and management of patients with aortic pathologies, as well as in preparation of this article to A.Yu. Bakanov, PhD, Head of Research Laboratory of Perfusiology and Cardiac Protection; V.V. Volkov, Fellow of Research Laboratory of Perfusiology and Cardiac Protection; A.V. Naymushin, PhD, Head of Anesthesiology &amp;amp; Resuscitation/ICU-2 Department; I.V. Basek, Phd, Head of X-Ray Computer Tomography Department and the specialists of X-Ray Computer Tomography Department, as well as to the employees of Research Center for Non-Coronary Heart Diseases and to specialists of cardiovascular surgery departments.&lt;/p&gt;

Individualized Surgical Treatments for Children with Ebstein Anomaly.

Objective Ebstein anomaly is a rare type of tricuspid malformation. The present surgical methods to resolve this anomaly include tricuspid valvuloplasty, palliative surgery, and tricuspid valve replacement. The purpose of this study was to evaluate the short- and midterm outcomes of different surgical treatments among children with Ebstein anomaly. Methods This was a retrospective study of 136 Ebstein anomaly patients undergoing surgery at our institution from January, 2006 to August, 2015. This cohort included 118 patients receiving tricuspid valvuloplasty, 14 patients receiving palliative surgery and 4 patients receiving tricuspid valve replacement. Results There were two in-hospital deaths and one delayed death 6 months after bidirectional cavopulmonary shunt during a second-stage operation; thus, the mortality rate was 2.2% (3/136). The follow-up variables included echocardiography, chest radiography, oxygen saturation, and cardiac function. After a mean follow-up duration of 35.8 ± 16.5 months (range: 6-98 months), all survivors had no indications for reoperation; the oxygen saturation following radical surgery and palliative surgery was 95 to 100% and 85 to 95%, respectively. Most of the patients exhibited an improved New York Heart Association (NYHA) functional class from III or IV preoperatively to I or II at follow-up; only two patients who underwent the Fontan procedure continued to exhibit NYHA functional class III. In most patients, tricuspid regurgitation (TR) degree decreased from moderate or severe preoperatively to mild or moderate at follow-up and only six patients continued to exhibit severe TR at follow-up. Conclusions Individualization of surgical methods based on different indications, including age, associated malformations, tricuspid anatomy, cardiac function, and intraoperative status, could effectively improve the outcomes of Ebstein anomaly patients.

Surgical indication for functional tricuspid regurgitation at initial operation: judging from long term outcomes.

The assessment and management of tricuspid valve disease have evolved substantially during the past several years. Whereas tricuspid stenosis is uncommon, tricuspid regurgitation is frequently encountered and it is most often secondary due to annular dilatation and leaflet tethering from right ventricular remodelling. The indications for tricuspid valve surgery to treat tricuspid regurgitation are several and mainly related to the underlying disease, to the severity of insufficiency and to the right ventricular function. Surgical tricuspid repair has been avoided for years, because of the misleading concept that tricuspid regurgitation should disappear once the primary left-sided problem has been eliminated. Instead, during the last decade, many investigators have reported evidence in favor of a more aggressive surgical approach to functional tricuspid regurgitation, recognising the risk of progressive tricuspid insufficiency in patients with moderate or lesser degrees of tricuspid regurgitation and tricuspid annular dilatation. This concept, along with the long-term outcomes of principal surgical repair techniques are reported and discussed. Last, novel transcatheter therapies have begun to emerge for the treatment of severe tricuspid regurgitation in high-risk patients. Hence, very preliminary pre-clinical and clinical experiences are illustrated. The scope of this review is to explore the anatomic basis, the pathophysiology, the outcomes and the new insights in the management of functional tricuspid regurgitation.

Tricuspid valve repair for severe tricuspid regurgitation due to pacemaker leads.

Tricuspid valve regurgitation due to pacemaker leads is a well-known complication. Although some reports have suggested that pacemaker leads should be surgically explanted, strongly adhered leads cannot always be removed. The aim of this study was to describe our tricuspid valve repair techniques with pacemaker leads left in situ. Our retrospective study investigated 6 consecutive patients who required tricuspid valve surgery for severe regurgitation induced by pacemaker leads. From the operative findings, we identified 3 patterns of tricuspid valve and pacemaker lead involvement. In 3 patients, the leads were caught in the chordae, in 2 patients, tricuspid regurgitation was caused by lead impingement on the septal leaflet, and in 3 patients, tricuspid valve leaflets had been perforated by the pacemaker leads. During surgery, all leads were left in situ after being separated from the leaflet or valvular apparatus. In addition, suture annuloplasty was performed for annular dilatation in all cases. In one patient, the lead was reaffixed to the annulus after the posterior leaflet was cut back towards the annulus, and the leaflet was then closed. There was one hospital death due to sepsis. The degree of tricuspid regurgitation was trivial in all surviving patients at discharge. During a mean follow-up of 21 months, one patient died from pneumonia 20 months after tricuspid valve repair. In patients undergoing tricuspid valve surgery due to severe tricuspid regurgitation caused by pacemaker leads, the leads can be left in situ after proper repair with annuloplasty.

Prolonged Tp-e Interval and Tp-e/QT Ratio in Children with Mitral Valve Prolapse.

Although it is considered to be a benign condition, previous studies have shown that a subset of patients with mitral valve prolapse (MVP) may be at risk of ventricular arrhythmia and sudden cardiac death (SCD). Previous studies have suggested that the interval between the peak and the end of the T wave (Tp-e) can be used as a marker for the transmural dispersion of repolarization. Increased Tp-e interval and Tp-e/QT ratio are associated with ventricular arrhythmias and SCD. The aim of this study was to assess alterations in ventricular repolarization by using the Tp-e interval and Tp-e/QT ratio in children with MVP and to investigate their relationships with the degree of valvular regurgitation. This study prospectively investigated 110 children with MVP and 107 age- and sex-matched healthy control subjects. Tp-e interval, Tp-e/QT ratio, and QT and QTc dispersions were measured from a 12-lead electrocardiogram and compared between groups. QT and QTc dispersions, Tp-e interval, and Tp-e/QTc ratio were found to be significantly higher in patients with MVP. A positive correlation was found between Tp-e/QTc ratio and increase in the degree of mitral regurgitation (MR) (p<0.05; r=0.2). However, the degree of MR was not associated with QT, QTc, or Tp-e intervals; QT, QTc, or Tp-e dispersions; or Tp-e/QT ratio (all p values >0.05). Individuals with MVP may be more prone to ventricular arrhythmias due to prolonged QTd, QTcd, and Tp-e interval and increased Tp-e/QT and Tp-e/QTc ratios. Therefore, due to their longer life expectancy, children with MVP should be followed up on regarding life-threatening arrhythmias.

Balloon‐Expandable Transcatheter Aortic Valves Can Be Successfully and Safely Implanted Transfemorally Without Balloon Valvuloplasty

To assess the necessity for balloon aortic valvuloplasty (BAV) during transfemoral transcatheter aortic valve implantation (TAVI) when using balloon-expandable valves. BAV is a usual part of TAVI procedures, prior to valve implantation. However, the benefits and necessity of this are unknown and recent evidence in self-expanding valves suggests it may not be necessary. Retrospective single-center study of 154 patients undergoing first-time, transfemoral TAVI for native aortic valve stenosis, with (N = 76), and without (N = 78), BAV as part of the procedure. Data collected included demographic, procedural, and outcome data. BAV did not alter VARC-2 defined procedural success or early safety compared to not performing a BAV, including mortality, degree of aortic regurgitation, or need for post-TAVI balloon dilatation, although there was a strong trend to reduced stroke when not performing a BAV. There was a significantly reduced procedural time (P = 0.01) and fluoroscopic time (P < 0.001) without performing a BAV. There were no differences in cerebral embolization (solid, gaseous, or total emboli) noted between the 2 groups, as measured on transcranial doppler (TCD). TAVI can be effectively and safely performed without a BAV and this results in reduced procedural and fluoroscopic times, although embolization to the brain is not reduced. There is a trend toward reduced stroke risk. (J Interven Cardiol 2016;29:319-324).

Dilatation of the ascending aorta is associated with presence of aortic regurgitation in patients after repair of tetralogy of Fallot

To evaluate the association between aortic morphology and elasticity with aortic regurgitation in surgically corrected of tetralogy of Fallot (TOF) patients. We retrospectively identified 72 consecutive patients with surgically corrected TOF and 27 healthy controls who underwent cardiac MRI evaluation. Velocity-encoded cine MRI was used to quantify degree of aortic regurgitation (AR) in TOF patients. Ascending aorta diameters were measured at standard levels on MRA images. Aortic pulse-wave velocity (PWV) was quantified with MRI. Morphological and functional MRI variables were compared between groups of TOF patients with and without clinically relevant AR and controls. The association between aortic morphology and elasticity with the presence of AR was evaluated using univariate and multivariate logistic regression. The majority of TOF patients had only trace AR. Nine TOF patients (12 %) had an AR fraction higher than 15 %. Indexed aorta diameter at the sinotubular junction (p = 0.007), at the RPA level (p = 0.006), and low left ventricular ejection fraction (LVEF) (p = 0.015) showed the strongest associations with the presence of at least mild AR, which persisted after controlling for age and gender. Increased ascending aorta dimension is associated with AR in patients after repair of TOF. LVEF was also low in the group of patients with relevant AR compared to those without, suggesting even mild to moderate AR may contribute to LV dysfunction in these patients. Enlarged ascending aorta may be an indication for precise quantification of regurgitant fraction with MRI, since symptomatic patients may need aortic valve repair when moderate regurgitation is present.

Prognostic significance of mild aortic regurgitation in predicting mortality after transcatheter aortic valve replacement

ObjectiveModerate to severe aortic regurgitation after transcatheter aortic valve replacement is associated with worse outcomes. The impact of mild aortic regurgitation has been less clear, possibly because of the broad categories that have been used in clinical trials, but holds increasing importance in the study of next-generation devices in low- and intermediate-risk cohorts. A more granular scheme, which is common in clinical practice and proposed for future trials, may add prognostic value. MethodsWe evaluated all patients undergoing transfemoral transcatheter aortic valve replacement at the Cleveland Clinic from 2006 to 2012. The degree of aortic regurgitation after transcatheter aortic valve replacement was reported from the echocardiography database based on a clinical, transthoracic echocardiogram performed within 30 days of the procedure. Aortic regurgitation was finely discriminated on the basis of a multiwindow, multiparametric, integrative approach using our usual clinical scale: none, trivial to 1+, 1+, 1 to 2+, 2+, 2 to 3+, 3+, 3 to 4+, or 4+. ResultsThere were 237 patients included in the analysis. By controlling for age, gender, Society of Thoracic Surgeons score, baseline ejection fraction, and aortic regurgitation before transcatheter aortic valve replacement, there was a significant increase in mortality for each half grade of aortic regurgitation compared with the complete absence of aortic regurgitation after transcatheter aortic valve replacement. The unit hazard ratio for each 1+ increase in aortic regurgitation after transcatheter aortic valve replacement was 2.26 (95% confidence interval, 1.48-3.43; P < .001) considering aortic regurgitation as a continuous variable. Other clinical variables did not significantly affect mortality. ConclusionsEven mild aortic regurgitation after transcatheter aortic valve replacement is associated with worse long-term mortality. There may be prognostic value in reporting milder categories of aortic regurgitation with more granular gradations.

21 Feature tracking cardiac magnetic resonance to assess LV mechanics in different cardiac overload caused by aortic valve disease

Background In aortic valve disease, left ventricular (LV) dimensions and ejection fraction are important parameters for decision making. However, the effects of pressure overload caused by aortic stenosis or/and volume overload, due to aortic regurgitation, lead to different LV remodelling, concentric and eccentric hypertrophy, respectively, which may differently alter LV mechanics. We aimed to characterise LV mechanics, in terms of longitudinal strain/deformation using feature tracking cardiac magnetic resonance (FT-CMR) in patients with various degree of aortic stenosis and aortic regurgitation and preserved LV ejection fraction (LVEF). Methods Seventy-one patients (14 with normal valve function, 29 with aortic stenosis and 28 with aortic regurgitation), mean age 45 ± 19 years, 70% men, who underwent clinically indicated CMR and showed preserved LVEF (>50%) were included. LV volumes, LVEF and mass were measured on steady-state free precession (SSFP) cine images. FT-CMR analysis was performed offline using tissue-tracking software (CVI42, Circle Cardiovascular Imaging Inc.) to estimate LV global longitudinal strain (GLS) from two long-axis SSFP cine images (Figure 1). To correct for the LV remodelling process, LV GLS was corrected for LV end-diastolic volume. Results There were significant differences in LV volumes, mass and ejection fraction across the 3 groups of patients (Table 1): patients with aortic regurgitation showed significantly larger LV volumes, and lower LVEF compared to patients with normal aortic valve function and patients with aortic stenosis. There were no differences in LV GLS across the groups. However, after correcting for LV end-diastolic volume, patients with aortic regurgitation showed more impaired LV GLS as compared to the other groups. Conclusions LV mechanics significantly differ across normal functioning and different type of aortic valve dysfunction (stenosis and regurgitation), with aortic regurgitation showing the most impaired LV GLS corrected for LV end-diastolic volume, despite preserved LVEF.

Long-term galsulfase enzyme replacement therapy in Taiwanese mucopolysaccharidosis VI patients: A case series

BackgroundInformation regarding the long-term outcome of enzyme replacement therapy (ERT) with recombinant human N-acetylgalactosamine 4-sulfatase (rhASB, galsulfase, Naglazyme®, BioMarin Pharmaceutical Inc.) for Taiwanese patients with mucopolysaccharidosis (MPS) VI is limited. MethodsNine Taiwanese patients with MPS VI (4 males and 5 females; age range, 1.4 to 21.1years) treated with weekly intravenous infusions of galsulfase (1.0mg/kg) in 5 medical centers in Taiwan were reviewed. A set of biochemical and clinical assessments were evaluated annually. ResultsAfter 6.2 to 11.2years of galsulfase treatment, 6 patients experienced improvement over baseline in the 6-minute walk test by a mean of 150m (59% change over time), and 3 patients also increased the 3-minute stair climb test by a mean of 60 steps (46%). In a manual dexterity test, 3 patients decreased the time required to pick up 10 coins and put the coins into a cup by 15s (33%). Shoulder range of motion in all 9 patients improved, and Joint Pain and Stiffness Questionnaire scores improved by 0.42 points (21%). Four patients showed improved pulmonary function. Five patients had positive effects on cardiac-wall diameters. Four patients had improved cardiac diastolic function. Liver and spleen sizes as measured by abdominal ultrasonography remained the same or decreased in all 9 patients. However, the severity degree of valvular stenosis or regurgitation did not show improvement despite ERT. A mean overall 69% decrease in urinary glycosaminoglycan (GAG) excretion indicated a satisfactory biomarker response. ConclusionsLong-term ERT was beneficial and safe for Taiwanese patients with MPS VI. This treatment reduced urinary GAG and had positive effects on a wide range of clinical functional assessments including endurance, mobility, joint function, pulmonary function, liver and spleen size, cardiac hypertrophy and diastolic dysfunction.

Abstract 208: Impact of Symptoms and Degree of Valvular Regurgitation on Patient-perceived Health Status in Patients With Aortic Regurgitation

Background: Patients (pts) with severe aortic regurgitation (AI) often present with symptoms of shortness of breath (SOB) or heart failure (HF); however, the effect of these symptoms and severity of aortic regurgitation on patient-perceived health status is unknown. Methods: Kansas City Cardiomyopathy Questionnaire (KCCQ) data were collected using tablet computers from pts presenting for routine outpatient cardiovascular assessment between 6/1/2011 and 8/31/2012 who also underwent echocardiography within 90 days. Pts with other valvular lesions (including aortic stenosis) moderate or greater in severity, congenital heart disease, hypertrophic cardiomyopathy, pericarditis or constriction, cardiac amyloid, sarcoid, restrictive cardiomyopathy, or pulmonary hypertension were excluded. Demographic, clinical, historical, and echocardiographic data was collected as per routine. Multiple linear regression was used to assess the association of this information and valve lesion severity with KCCQ summary score (SS). Results: 590 pts [age 61.9±13.8 years, 363 (61.5%) men, 132 (22%) with prior aortic valve surgery] were identified; 452 with mild, 86 with moderate and 52 with severe AI. Mean KCCQ SS was 74.5±23.7; 74.3±23.0 with mild AI, 73.8±26.2 with moderate AI, and 76.7±19.4 with severe AI (p=NS). SOB was present in 290 pts (49%, KCCQ SS 65.3±23.7), HF in 43 (7.3%, KCCQ SS 50.1±22.3) and both in 36 pts (KCCQ SS 47.0±21.7). After adjusting for multiple factors, linear regression (r2=0.28, p&lt;0.001) revealed that the presence of SOB (β: -13.841, p&lt;0.001) and heart failure (β:-19.484, p&lt;0.001) were associated with lower SS; however, the degree of AI and history of aortic valve surgery were not associated with KCCQ SS. Male sex (β: 7.279, p=0.001), heart rate (β: -0.223, p=0.005), prior CAD (β: -4.364, p=0.038) and BMI (β:-0.357, p=0.035) were also significant in this model. Pt age, presence of atrial fibrillation, angina, hypertension, diabetes, prior CAD, renal disease, cancer, cerebrovascular disease, peripheral vascular disease, left ventricular diastolic dimension and ejection fraction were not significant predictors. Conclusions: In stable outpatients with AI, KCCQ scores correlate with the presence of shortness of breath and heart failure symptoms, but are not affected by the degree of AI or history of aortic valve surgery. Symptoms rather than diagnosis drives patient perceived health status.

The specifics of adhesion function of endothelium in various clinical variants of primary mitral valve prolapse

Aim . To assess the condition of adhesion function of endothelium in various clinical variants of primary mitral valve prolapse (MVP). Material and methods. Totally, 91 patient studied with primary MVP at the age 21 (19-24) y. First grade mitral regurgitation was found in 45,1% and II — in 54,9% patients. MVP was solitary variant (6,6%) and comorbid with 1-3 minor anomalies of the heart (93,4%). Doppler-echocardiography was done on Vivid07 equipment (Israel). The grade of systemic involvement of connective tissue was 2 (1,5-4,0) points. Controls were 10 healthy persons, matched by age, sex, smoking, body mass index. By the immune enzyme method we checked plasmatic concentrations of L-, E-, Р-selectins, ICAM-1, VCAM-1, PECAM-1 (Bender MedSystems GmbH, Austria). Findings are presented as mediana (25-75 percentiles). Results . In MVP patients the levels of Е-selectin — 43,0 (33,7-54,8) ng/ mL, ICAM-1 — 669,9 (546,4-883,3) ng/mL and VCAM-1 — 925,0 (707,5- 1215,0) ng/mL, were significantly higher, and the level of РЕСАМ-1 — 49,8 (40,4-63,2) ng/mL, in opposite, lower than in control group. L- and P-selectins levels in MVP group were measured as relevant to controls values (p>0,05). In regurgitation cases of II degree, the level of E-selectin and ICAM-1 were maximal (p<0,05). Values of VCAM-1 in MVP patients independently from degree of regurgitation were significantly higher than controls values. There was moderate direst relation of the quantity of minor heart anomalies and level of ICAM-1 (rs=+0,30, р<0,05). In MVP group with increased E-selectin significantly higher was aorta diameter at the level of Valsalva sinuses — 2,5 (2,3-2,7) cm, than in PMV without increased E-selectin level — 2,3 (2,2-2,5) cm (р<0,05). Conclusion . In patients with primary MVP there is an increase of circulating concentrations of E-selectin, ICAM-1, VCAM-1, that represents the level of adhesion, and witnesses about subclinical endothelial dysfunction. For screening evaluation of severity of adhesion disorders of endothelium in MVP it is important to take into account its hemodynamic significance, multiple minor heart anomalies and data on aorta diameter on the level of Valsalva sinuses.

Hemodynamics and Exercise Tolerance after Senning Operation for Transposition of Great Arteries and Its Limiting Factors: A Longitudinal Study

Objectives: The purpose of this longitudinal study was to assess hemodynamics and exercise capacity in patients after Senning operation and to determine its limiting factors. Methods: 87 long-term survivors of the Senning operation for transposition of great arteries were recruited for a longitudinal follow-up study consisting of two cross-sectional evaluations at a mean of 12.6 and 22.6 years after surgery, respectively. The protocol included a questionnaire, echocardiography, radionuclide angiocardiography, magnetic resonance imaging and cardiopulmonary exercise test. Decreased exercise capacity was defined as maximum oxygen uptake <-2 Z-scores. Results: Hemodynamic variables (right ventricular function and degree of tricuspid regurgitation) and chronotropic competence did not change significantly with time. Decreased exercise tolerance was present in 55.6 and 49.3% of patients at the first and second evaluation, respectively (NS) and had multi-variable correlation with lower right ventricular end-diastolic volume (P=0.039, OR 0.963) and increased ventilation/carbon dioxide production slope (P=0.040, OR 1.293). None of other hemodynamic variables correlated with decreased functional capacity. Conclusions: Long-term survivors of the Senning operation have stable hemodynamics and exercise capacity on longitudinal follow-up. Decreased exercise tolerance is found in approximately half of the patients and is significantly associated with lower ventilation efficiency. This data may point to the usefulness of targeted respiratory rehabilitation in this patient group.

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot

BackgroundUnfavorable left ventricular (LV) remodelling may be associated with adverse outcomes after Tetralogy of Fallot (TOF) repair. We sought to assess T1 cardiovascular magnetic resonance (CMR) markers of diffuse LV myocardial fibrosis in children after TOF repair, and associated factors.MethodsIn this prospective, cross-sectional study, native (=non-contrast) T1 times and extracellular volume fraction (ECV) were quantified in the LV myocardium using CMR. Results were related to ventricular volumes and function, degree of pulmonary regurgitation, as well as surgical characteristics, and exercise capacity.ResultsThere was no difference in native T1 times or ECV between 31 TOF patients (age at CMR 13.9 ± 2.4 years, 19 male) and 15 controls (age at CMR 13.4 ± 2.6 years, 7 male). Female TOF patients had higher ECVs than males (25.2 ± 2.9 % versus 22.7 ± 3.3 %, p < 0.05). In the patient group, higher native T1 and ECV correlated with higher Z-Scores of right and left ventricular end-diastolic volumes, but not with reduced left and right ventricular ejection fraction or higher pulmonary regurgitation fraction. Longer cardiopulmonary bypass and aortic cross clamp times at surgery correlated with increased native T1 times and ECVs (r = 0.48, p < 0.05 and r = 0.65, p < 0.01, respectively). Maximum workload (percent of predicted for normal) correlated inversely with ECV (r = -0.62, p < 0.05). Higher native T1 times correlated with worse LV longitudinal (r = 0.50, p < 0.05) and mid short axis circumferential strain (r = 0.38, p < 0.05).ConclusionsAs compared to controls, TOF patients did not express higher markers of diffuse fibrosis. Longer cardiopulmonary bypass and aortic cross clamp times at surgery as well as biventricular enlargement and reduced exercise tolerance are associated with markers of diffuse myocardial fibrosis after TOF repair. Female patients have higher markers of diffuse myocardial fibrosis than males.

Long-term results of surgical treatment of patients with aortic valve disease and functional mitral insufficiency

To analyze long-term surgical results in patients with aortic valve disease and concomitant mitral regurgitation (MR) depending on volume of valve surgery. It was studied 5-year results in 71 patients with aortic valve disease and different degree of mitral regurgitation. Patients were divided into 3 groups. Control group included 40 patients after aortic valve replacement (AVR) and no mitral insufficiency. The 2nd group consisted of 16 patients after AVR and concomitant mitral regurgitation degree 2-3. The 3rd group - 15 patients after aortic and mitral valve replacement. Mortality in long-term postoperative period, quality of life, incidence of complications and echocardiography data were analyzed. There was no significant improvement of mitral regurgitation after AVR. It was showed that persistent MR decreases remote survival and quality of life as well as deteriorates echocardiography data. Herewith these data in the 3rd group did not differ from the control group. Double-valve replacement may be advisable in patients with aortic valve disease and concomitant moderate MR because persistent MR deteriorates long-term However prolonged time of cardiopulmonary bypass, aortic cross-clamping, greater blood loss require comprehensive approach to advanced cardiac surgery.