Introduction Severe symptomatic aortic stenosis is associated with high mortality without intervention. However, true waiting times for aortic valvular intervention (AVI) and the risks associated with it are not known. Objectives To measure the waiting time between referral and AVI. To determine the impact of the waiting time for AVI by assessing the occurrence of adverse events during this period. To assess predictors of adverse outcomes during this period in view to identify patients (pts) who may require earlier AVI. Methods Retrospective single-center study of consecutive outpatients referred for AVI (either surgically or transcatheter (TAVI)) since 2014 to 2018. The primary endpoint was hospitalization due to heart failure or death from any cause, occurring in the waiting time for AVI. Cox regression analysis was performed. Results Were included 120 pts (54% male, mean age 75±9 years). 113 (94%) pts had high-gradient aortic stenosis. They were mainly in NHYA class II (56%). Fatigue was the main symptom (83%). The median NT-proBNP value was 819 (IQR 319–1780) ng/L. The mean peak velocity was 4.5±0.5 m/s, median gradient of 45 (IQR 42–54) mmHg, mean VTIs ratio of 0,21±0.04, with a mean estimated valvular area of 0.7±0.2 cm2 (0.4±0.1 cm2/m2). During a mean follow-up of 24±14 months since referral, 108 (90%) pts were submitted to AVI (75 pts underwent surgery; 33 pts underwent TAVI). The median waiting time for AVI was 4 (IQR 2–6) months (0–35 months). The median waiting time for surgery was 3 (IQR 2–6) months and for TAVI was 4 (IQR 3–8) months (p=0.25). The primary endpoint occurred in 19 (16%) pts: 13 (11%) pts were hospitalized due to heart failure and 7 (6%) pts died. The median time between referral and the occurrence of the primary endpoint was 3 (IQR 1–9) months. In univariate analysis, age >80 years, NHYA class ≥3, prior stroke and NT-proBNP were positively associated with the occurrence of the primary endpoint (p<0.05). After multivariate analysis, prior stroke (HR 5; 95% CI 1.2–24; p=0.03) and NT-proBNP (HR 1/unit; 95% CI 1–1.001; p=0.01) were independently associated with events occurrence. NT-proBNP was an independent predictor of events with a good discriminative value (area under the ROC curve 0.73; 95% CI 0.61–0.83; p=0.004). NT-proBNP cut-off value of >1207ng/L identified pts with an event while waiting AVI with a sensitivity and specificity value of 69 and 73%, respectively. Left ventricle ejection fraction, severity parameters of aortic stenosis, systolic pulmonary artery pressure, concomitant coronary artery disease and the time between diagnosis and referral were not associated with the primary endpoint. Conclusion Mortality and worsening of heart failure while waiting for aortic valvular intervention occurred frequently. Factors such NT-pro-BNP and personal history of stroke can help to identify pts who may benefit from earlier intervention. Funding Acknowledgement Type of funding source: None
Introduction Atrial septal defects (ASD) may be a cause of pulmonary hypertension (PH) specially when they are only detected in adulthood. Sinus venosus type ASD are rare, with an estimated prevalence of 4-11%, and frequently they are associated with anomalous venous return of the right superior pulmonary vein (RSPV). Surgical closure is safe and effective, and it is associated with normal life expectancy when performed before age 25; the risk of PH is higher in untreated defects or late closure. Clinical case The authors present the case of a 74-years old female patient with previous diagnosis of a sinus venosus type ASD. Closure of the shunt and correction of venous return was performed when the patient was 36. Follow up in the following years was normal, and the patient was discharged from the congenital surgical center. The patient was referred to our PH unit due to symptomatic PH for etiological investigation (PSAP of 70 mmHg in transthoracic echocardiogram). After excluding PH related to left heart disease and lung disease, the most likely cause was pulmonary arterial hypertension due to late closure of left to right shunt, but complete investigation was performed. A transesophageal echocardiogram showed dilatation of right heart chambers and a communication of 36 mm at the high atrial septum between the RSPV and superior vena cava entrance with spontaneous left to right shunt. A severe dilation of coronary sinus (maximal dimension 33.4mm) suggestive of persistent left superior vena cava (PLSVC) was also found. A cardiac magnetic resonance was performed showing dilated right chambers, abnormal drainage of right superior pulmonary vein to right atrium, a dilated coronary sinus with a PLSVC and Qp/Qs 1.7. Right heart catheterization showed a mean pulmonary artery pressure of 25 mmHg with normal pulmonary vascular resistance (2.4 UWood) suggesting that the intracardiac shunt is the responsible for the PH with reversible pulmonary vascular disease. The patient was proposed to surgical repair. Conclusion The authors present a rare clinical case of an undiagnosed persisting sinus venous ASD after surgical repair. Failure of shunt closure led to the development of PH, emphasizing the need to maintain lifelong follow up of these patients in specialized centers. Abstract P694 Figure.
Background Exercise stress echocardiography (ESE) is routinely used in adults but its role in children (C) is less established Purpose To assess the feasibility and clinical value of ESE in outpatient children Methods We enrolled 309 consecutive C (mean age = 14,1 ± 2,6 years, range 6-17 yrs) who underwent treadmill ESE between 2002 and 2019: One group (Group I) of 258 C including: 237 with exercise related symptoms (chest pain and/or dyspnea and/ or lypotimia-syncope), 15 with resting ECG alterations, 6 with positive ECG stress test and other group of C (Group II) including: 10 asymptomatic for screening requested by parents, 11 with symptoms unrelated to exercise, 12 with antecedents of sudden death in the family, and 17 with known pathology - 10 with hypertrophic cardiomyopathy, 2 with aortic coarctation, 1 each with Cortriatriatum sinister, pulmonary stenosis, subaortic stenosis, bicuspid aortic valve, left ventricular hypertrophy related to arterial hypertension, aortic switch operation. Regional wall motion abnormalities (RWMA) by 2-D and continuous wave Doppler (transvalvular or transaortic or intraventricular (IVG) gradients were assessed in all. Results The success rate was 309/309 (100%). Only one complication (allowing asthma diagnosis by serendipity) occurred: a severe asthmatic crisis in one girl studied because of chest pain with exercise (with ESE negativity), Stress-induced RWMA occurred in 2 pts (one with HCM, the other with normal coronary arteries). A significant orthostatic exercise induced IVG (> 30 mmHg) was present in 101 of the 258 C (39%) studied due to symptoms, ECG alterations or positive stress ECG. In group II the C with induced IVG attained greater heart rate (HR) 184 ± 12 vs 174 ± 16 (p < 0,001); greater blood pressure (BP) 150 ± 19 mmHg vs 136 ± 23 mmHg (p < 0,001). The OR to the reproduced symptoms that motivated the exam during the SE comparing the 101 C with IVG with the 158 without IVG was 8,22 (4,83-13,99) p < 0,001 (95% CI). Conclusions Treadmill ESE is feasible and safe in young people. RWMA are of limited usefulness in our outpatient C group. Doppler often documents significant exercise induced IVG, occult at rest that associate with symptoms. Abstract P794 Figure. ESE Induced IVG in a C with chest pain
Background In our experience, treadmill exercise echocardiography (SE) is feasible and safe in children (C). Regional wall motion abnormalities (RWMA) are of limited usefulness, but Doppler often - (in 39% from 258 previously studied C) - documents significant intraventricular gradients (IVG), occult at rest, and allowing to document a possible explanation for exercise related symptoms, or abnormal resting or stress-ECG findings. Purpose - To assess the effect of ß blockers on the occurrence of IVG, in C, with symptoms or abnormal resting or stress-ECG findings. Methods – We repeated SE in 66 of the 101 C – (with normal echocardiogram at rest) - that developed IVG on exertion, under treatment with ß blockers. These 66 C who repeated the SE under treatment with ß blockers are the study group. 15 (23%) of them were female and the mean age of the group was 14,6 ± 1,7 years old (11 to 17). They all underwent SE with 2D and Doppler echocardiographic evaluation of, and during treatment with ß blockers. Results Mean IVG in those 66 C submitted to SE was 105 ± 38 mmHg in the first SE evaluation. In SE evaluation performed under ß blockers, 37 of them didn’t develop IVG and in 29 of them IVG was significantly reduced to a mean IVG of 58 ± 32 mmHg (p< 0,0001). The mean heart rate attained at peak exercise was 178 ± 15 bpm in the first SE evaluation and 157 ± 9 bpm in the evaluation performed under treatment with ß blockers (p < 0, 0001). 47 of these C reproduced clinical symptoms (that were indication to SE) of beta-blockers, and only 7 reproduced the symptoms under treatment with beta blockers (p< 0,0001). Conclusions In C with symptoms, abnormal rest or exercise ECG on medical evaluation and IVG on exertion, treatment with oral ß blockers prevented the occurrence of IVG or significantly reduced its magnitude. These changes were associated to significant clinical improvement in 85 % of the symptomatic population. Abstract P945 Figure. SE without and with beta-blockers
Introduction The evaluation of real severity of "low-flow low-gradient" aortic stenosis (LFLG AS) is particularly challenging. TOPAS study demonstrated that projected aortic valve area at a normal transvalvular flow rate (AVAproj) derived from dobutamine stress echocardiography (DSE) is superior to the traditional Doppler indices to discriminate true severe-AS and pseudosevere-AS. Purpose To compare two echocardiographic methods to estimate severity of LFLG AS with DSE (aortic valve area (AVA) estimated by continuity equation (AVA-CE) and simplified method of AVAproj) in patients (pts) with low transvalvular flow rate (<250mL/seg). Methods Unicentric, retrospective study, that included pts with LFLG AS undergoing DSE with low dose dobutamine protocol, during Nov 2013-Dec 2018 period. Evaluation at rest and peak DSE of vital signs, mean transaortic gradient, aortic VTI, LVOT VTI and VTI ratio, valvulo-arterial impedance (ZVA), AVA-CE, simplified method of AVAproj and global longitudinal strain (GLS). Results A total of 27 DSE were performed in 23 different pts, mean age of 76 ± 8 years, 82% male. At rest 55% in sinus rhythm, mean heart rate (HR) was 76 ± 12 bpm, mean systolic arterial pressure (SAP) was 122 ± 22 mmHg, mean ZVA 4.3 ± 2 mmHg/ml/m2; mean diameter of LVOT was 21,7 ± 2,6cm, mean of mean aortic gradients 21 ± 7 mmHg, 67% of pts had a VTI ratio at rest compatible with severe AS and remaining compatible with moderate AS. Estimated mean AVA-CE was 0.86 ± 0.29 cm2 with 67% of pts classified as severe AS. Mean left ventricular ejection fraction at rest was 31 ± 9%, systolic volume index 28,7 ± 8 mL/m2 and GLS -5,9%. During low dose perfusion protocol of dobutamine 100% patients remained asymptomatic, mean HR was 110 ± 25 bpm, mean SAP was 123 ± 26 mmHg, mean ZVA 3.6 ± 1.7 mmHg/mL/m2, mean of mean aortic gradients 28 ± 9mmHg, 37% of pts presented VTI ratio compatible with severe AS and remaining compatible with moderate AS. Mean flow reserve was 16 ± 16% and mean GLS-7.2%. AVA-CE was 1,06 ± 0,35 cm2 with 56% of pts classified as severe AS and mean projected AVA was 1.01 ± 0.22cm2, without significant difference in AVA estimated by the two methods (p = 0.344). Projected AVA allowed re-classification of AS in 22% of pts (5 patients), with 31% of severe AS reclassified as moderate AS while AVA-CE allowed re-classification in 13% (3 patients), with 19% of severe AS reclassified as moderate AS. Considering medium follow up of 24 months, 6 patients were submitted aortic valve replacement surgery and another 6 patients to transcatheter aortic valve replacement. The simplified projected valve area calculation show no significant therapeutic impact in the selection of this patients. Conclusion The simplified projected valve area calculation is technically feasible and accessible. This study shows a good correlation in pts with low cardiac flow. If AVAproj method had been used 2 extra patients would have been reclassified during DSE.
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