Systolic Gradient Research Articles

Outcomes after implantation of right-sided mechanical valve prostheses in congenital heart disease

BackgroundBioprosthetic valves are often used for pulmonary valve replacement (PVR) and tricuspid valve replacement (TVR) because of concerns about mechanical valve thrombosis in the right heart. The purpose of this...

High prevalence of pre-eclampsia in women with coarctation of the aorta.

The aim was to study pregnancy outcomes in women with coarctation of the aorta (CoA) and associations to hypertensive disorders of pregnancy. Maternal morbidity and mortality are higher in women with heart disease and pre-eclampsia. Chronic hypertension, frequently encountered in CoA, is a risk factor for pre-eclampsia. Clinical data from the National Unit for Pregnancy and Heart Disease database was reviewed for pregnant women with CoA from 2008 to 2021. The primary outcome was hypertensive pregnancy disorders. The secondary outcomes were other cardiovascular, obstetric, and foetal complications. Seventy-six patients were included, with a total of 87 pregnancies. Seventeen (20%) patients were treated for chronic hypertension before pregnancy. Fifteen (20%) patients developed pre-eclampsia, and 5 (7%) had pregnancy-induced hypertension. Major adverse cardiac events developed in four (5%) patients, with no maternal or foetal mortality. Maternal age at first pregnancy [odds ratio (OR) 1.37], body mass index before first pregnancy (OR 1.77), and using acetylsalicylic acid from the first trimester (OR 0.22) were statistically significantly associated with pre-eclampsia. At follow-up (median) 8 years after pregnancy, 29 (38%) patients had anti-hypertensive treatment, an increase of 16% compared to pre-pregnancy. Five (7%) patients had progression of aorta ascendens dilatation to >40 mm, seven (9%) had an upper to lower systolic blood pressure gradient >20 mmHg, and six (8%) had received CoA re-intervention. Pre-eclampsia occurred in 20% of women with CoA in their first pregnancy. All pre-eclamptic patients received adequate anti-hypertensive treatment. All CoA patients were provided multi-disciplinary management, including cardiologic follow-up, to optimize maternal-foetal outcomes.

Effects of oral propranolol on the resistive and pulsatility indices of major abdominal vasculatures in domestic short-haired cats.

No study has been performed regarding the effects of oral administration of propranolol on pulse-wave spectral Doppler indices of major abdominal vessels in healthy adult cats. The objective of this study was to assess the pulse-wave spectral Doppler indices of abdominal aorta, caudal vena cava, and portal vein in clinically normal adult domestic short-haired (DSH) cats, before and after propranolol ingestion. Twenty intact adult client-owned DSH cats were evaluated (10 males and 10 females). A duplex Doppler ultrasonography machine with a 10-MHz frequency linear transducer was used. Peak systolic velocity, end-diastolic velocity (EDV), resistive index (RI), pulsatility index (PI), and pressure gradient were measured. All the cats received 1mg/kg of propranolol tablet, and after 2 h, ultrasonography measurements were repeated. The mean RI of the aorta and caudal vena cava significantly decreased in male cats following oral administration of propranolol after 2 h (p = 0.03, p = 0.02). In the caudal vena cava, the PI decreased from 2.98±0.62 to 1.15±0.19 post-propranolol ingestion (p = 0.01). The mean EDV in the caudal vena cava of males and portal veins of females significantly decreased after propranolol ingestion (p = 0.04, p = 0.02). This study showed that propranolol decreased the PI of the aorta and PI and RI of the caudal vena cava in healthy normal cats 2 h post-propranolol ingestion at the dosage of 1mg/kg.

Aortic Coarctation Stenting in Adolescents and Adults: A Single-Center Experience.

The aim of this study was to evaluate the short and midterm results of aortic coarctation (AoCo) stenting in an adolescent and adult population. All patients with a AoCo older than 14years treated by stent placement between December 2000 and November 2016 were included in this study. Twenty-eight patients with an invasive peak systolic pressure gradient >20mmHg were identified. Number of redilations, non-invasive systolic blood pressure, peak systolic pressure gradient, antihypertensive medication intake, claudication status and complications were evaluated. Twenty-two covered and 6 uncovered stents were successfully placed. Peak systolic pressure gradient decreased immediately after stenting from a mean of 32 mmHg to 0mmHg (± 7mmHg). Mean AoCo diameter increased from 8 tot 16mm (± 4mm). Peripheral arterial injury was seen in 2 patients (7.1%). The mean follow-up time was 60 ± 49months. Redilation of the stent was required in 4 patients, 2 to accommodate for growth and 2 for restenosis. Six (35%) patients could stop all antihypertensive medication. All claudicants (6/28) became and remained asymptomatic after surgery and during their follow-up. No aneurysms, stent fractures or dissections were noticed. There were 2 stent migrations during the first procedure with only 1 needing additional stent placement. Aortic coarctation stenting is a safe and effective treatment that significantly reduces the peak systolic pressure gradient. Antihypertensive medication can be reduced, and increase of walking distance in claudicants can be obtained. Younger patients may need more frequent reinterventions to accommodate for growth.

Risk factors for recurrence after surgical repair of coarctation of the aorta in children: a single-center experience based on 51 children.

Coarctation of the aorta (CoA), is a congenital malformation, often combined with several cardiac abnormalities. At present, the operation effect is satisfactory, but postoperative restenosis is still a matter. Identification of risk factors for restenosis and prompt therapy adjustments may improve patient outcomes. A retrospective clinical study of patients under 12 who had CoA repair in 2012-2021, with a randomized cohort population of 475 patients. A total of 51 patients (M/F: 30/21) with a mean age of 5.33 (2.00-15.00) months and a median weight of 5.60 (4.20-10.00) kg. The mean follow-up was 8.93 (3.77-19.37) months. Patients were divided into 2 groups: no-restenosis (n-reCoA) (G1, 38 patients) and restenosis (reCoA) (G2, 13 patients). ReCoA was defined as a restenosis requiring interventional or surgery or a pressure gradient >20 mmHg at the repair site as reported by B-ultrasound with the presence of an upper and lower limb blood pressure gradient or growing dysplasia. The overall reCoA incidence was 25% (13/51). In multivariate COX regression, smaller preoperative z-score of the ascending aorta (P = 0.009, HR = 0.68) and transverse aortic arch (P = 0.015, HR = 0.66), arm-leg systolic pressure gradient ≥12.5 mmHg at discharge (P = 0.003, HR = 1.09) were independent risk factors for reCoA. The overall outcome of CoA surgery is successful. Smaller preoperative z-score of the ascending aorta and transverse aortic arch, and an arm-leg systolic pressure gradient ≥12.5 mmHg at discharge increase reCoA risk, and closer follow-up for such patients are required especially within 1 postoperative year.

Coarctation Angioplasty in Young Children: Does IMPACT Registry Data Accurately Reflect Clinical Outcomes?

Balloon angioplasty for recoarctation of the aorta is routinely performed in young children and is considered successful when the systolic gradient is reduced to < 10mmHg. IMPACT defines acute procedural success solely based on a final gradient of < 10mmHg and stratifies participating institutions based on these acute outcomes. Between February 2012 and December 2020, IMPACT data was analyzed for 110 coarctation interventions. Electronic medical records were reviewed, and primary endpoints were one of the following: (1) final analysis end date (June 2021), (2) patient death, or (3) most recent transcatheter or surgical reintervention. 64 (58.2%) interventions had a post-procedure CA gradient < 10mmHg. Comparison of clinical patient outcome for acute success demonstrated no significant relationship using IMPACT (p = 0.70) criteria. There was no statistically significant difference between clinical success and failure for: pre- and post-treatment systolic gradients; absolute or percent change in systolic gradient; and pre-treatment aorta diameter. Clinical outcome and patient age did show a significant difference (p = 0.0093) with better clinical outcomes in older patients. Our analysis did not reveal a statistically significant difference between IMPACT criteria for successful treatment of CA and clinical outcome. These findings underscore a need to identify other clinical metrics that better predict outcome following CA balloon angioplasty treatment.

P122 CONTRAST–ENHANCED ECHOCARDIOGRAPHY IN BETTER STUDYING (OR EXCLUDING) HYPERTROPHIC CARDIOMYOPATHY. TWO CASES

Abstract Aim of this case–series report is to elucidate the potential role of contrast echocardiography (C–Echo) to optimize and improve the study of patients with Hypertrophic Cardiomyopathy (HCM). However, this report is also aimed to demonstrate the role of C–Echo in excluding a HCM diagnosis. Case 1. A young male patient, 27 y–o, presented to our Emergency Department (ED) because of chest pain and dyspnea. He referred a diagnosis of HCM without LVOT obstruction. Trans–Thoracic Echocardiogram (TTE) demonstrate a severe hypertrophy of interventricular septum with a maximum diastolic thickness of 25 mm. There was no a significant LVOT gradient. Contrast enhanced echocardiography, by left ventricle opacification (LVO) (Sonovue, Bracco Imaging, Milan, Italy), demonstrated: an interventricular septum thickness of 32 mm (7 mm more than TTE measurement); a clear touching of mid–ventricular walls with a max systolic gradient of 40 mmHg (figure 1). This aspect was no clearly detectable by TTE alone. Case 2. A young male patient, 31 y–o, presented to our ED because of palpitations. At 12–leads ECG he presented a sinus rhythm with frequent monomorphic premature ventricular complexes (PVC). A TTE was performed. It put a suspicion of left ventricle (LV) APICAL HCM, because of an apical diastolic thickness of 26 mm. A contrast enhanced echocardiography by LVO (Sonovue, Bracco Imaging, Milan, Italy) was planned. It allowed to excluding a HCM by demonstrating a LV apical false tendon mimicking endocardial border (figure 2). LV false tendons are fibromuscular bands that traverse LV cavity and are generally considered to be benign. Thay may also contain conduction tissue (Purkinje fibers) and have been identified as a source of ventricular arrhythmias. They are most commonly located near the LV apex. Conclusion It is possible to conclude that, by LV cavity opacification optimization of echocardiographic planes and views, C–Echo represents a suitable tool for: 1) a more accurate LV wall thickness (WT) measurement; 2) acquiring additional informations about intraventricular gradients in patients with HCM. In addition, C–Echo allows to exclude a HCM diagnosis in presence of LV apical false tendons.

Coarctation of the aorta in an asymptomatic infant: 'a touchy diagnosis'.

An 8-month-old male reported for cardiac evaluation for a heart murmur. On physical examination, he was non-dysmorphic, pulse oximetry was 98%, and cardiac examination revealed strong upper extremity pulses, impalpable lower extremity pulses, normal S1 and S2, no clicks, a grade 2/6 low pitch, and a vibratory ejection systolic murmur best heard at the lower left sternal border (deemed Still’s murmur). His electrocardiogram demonstrated biventricular hypertrophy. His transthoracic echocardiogram (TTE) demonstrated discrete coarctation of the aorta (COA). Suprasternal imaging showed discrete COA (vertical arrow) formed by the subclavian shelf and dilatated descending thoracic aorta (DA), and a horizontal arrow denotes the left subclavian artery (Panel A). Colour flow mapping demonstrated turbulence across the COA (horizontal arrow) (Panel B, Supplementary data online, Video). Continuous Doppler interrogation demonstrated a peak systolic gradient of 67 mmHg (Panel C) with a typical sawtooth spectral pattern indicating severe COA (vertical arrow shows flow extending into diastole). He underwent elective surgery with resection of the coarctated segment and end-to-end anastomosis of the DA. The excised segment demonstrated a pinhole lumen (Panel D). He continues to do well at follow-up.

Reclassification of moderate aortic stenosis based on data-driven phenotyping of hemodynamic progression

The management and follow-up of moderate aortic stenosis (AS) lacks consensus as the progression patterns are not well understood. This study aimed to identify the hemodynamic progression of AS, and associated risk factors and outcomes. We included patients with moderate AS with at least three transthoracic echocardiography (TTE) studies performed between 2010 and 2021. Latent class trajectory modeling was used to classify AS groups with distinctive hemodynamic trajectories, which were determined by serial systolic mean pressure gradient (MPG) measurements. Outcomes were defined as all-cause mortality and aortic valve replacement (AVR). A total of 686 patients with 3093 TTE studies were included in the analysis. Latent class model identified two distinct AS trajectory groups based on their MPG: a slow progression group (44.6%) and a rapid progression group (55.4%). Initial MPG was significantly higher in the rapid progression group (28.2 ± 5.6 mmHg vs. 22.9 ± 2.8 mmHg, P < 0.001). The prevalence of atrial fibrillation was higher in the slow progression group; there was no significant between-group difference in the prevalence of other comorbidities. The rapid progression group had a significantly higher AVR rate (HR 3.4 [2.4–4.8], P < 0.001); there was no between-group difference in mortality (HR 0.7 [0.5–1.0]; P = 0.079). Leveraging longitudinal echocardiographic data, we identified two distinct groups of patients with moderate AS: slow and rapid progression. A higher initial MPG (≥ 24 mmHg) was associated with more rapid progression of AS and higher rates of AVR, thus indicating the predictive value of MPG in management of the disease.

ENDOVASCULAR TREATMENT OF AORTIC COARCTATION

Objectives: The aim of this study is to present our center’s experience with the endovascular treatment inaortic coarctation with a follow-up of 10 years. Background: Stent implantation has been evolved as an important therapeutic strategy for coarctation of the aorta. However, available data is frequently flawed by short follow up, retrospective data and disparity in the approach. Methods: Clinical data, imaging studies and angiographic outcomes of 24 patients treated for CoA were reviewed between january 2007 to december2018. We included native diagnosed aortic coarctation (NCO) and recurrent coarctation. Results: There were 57% male patients and 43% female with a mean age of 26+/- 11 years. 90% of patients had NCO and 10% had re-coarctation after surgical repair in childhood (21+/- 4years after initial repair). Endovascular treatment was performed using BMS (PalmazGenesis peripheral stent, Cordis) in 71% of cases. Covered stent (mounted covered CP stent; Numed) was placed in 2 patients. One patient was treated by balloon angioplasty. Acute procedural success had reached 92% in our series with a technical failure in 2 cases. Mean lesion length and diameter were respectively 15,3+/-11,9 mm and 7,2+/- 2,7mm. Mean systolic pressure gradient decreased from 51,4 +/- 20,4 mm Hg to 7,7±10 mmHg (P &lt; 0,05). After a mean follow-up of 60 +/- 38 months, the blood pressure profile was evaluated. the CT scan showed a local aortic wallanevrysms in 1 patient (4.1%). Surgical intervention was needed in 2 patients forrecoarctationand aortic valve replacement. Conclusion: Percutaneous aortic coarctation treatment is effective with an acceptable safety and a good long-termoutcome. Therefore, patients require regular clinical and radiological monitoring tobalance blood pressure, look for local aortic wall complications and assess associated lesions such as aortic bicuspid valve and ascending aorta dimension.

Stent-Graft Placement for Radiation-Induced Abdominal Aortic Stenosis after Renal Autotransplantation.

Renovascular hypertension (RVH) is a common cause of secondary hypertension. However, there have been no reports on RVH due to radiation-induced abdominal aorta stenosis after renal autotransplantation. A 27-year-old woman with a history of neuroblastoma treated by radiation therapy and RVH treated with renal autotransplantation presented with hypertension and dyspnea. At age 19, she had experienced hypertensive heart failure due to RVH from radiation-induced left renal artery stenosis and had undergone renal autotransplantation involving the extraction of her left kidney. Her systolic blood pressure (BP) was well-controlled but had increased progressively. She was diagnosed with hypertensive heart failure and admitted to hospital. Although her dyspnea soon subsided after treatment, her BP remained high. Renal artery ultrasound revealed no obvious stenosis. The ankle brachial pressure index (ABI) showed a significant bilateral decrease to 0.71/0.71 (right/left) from 0.94/0.95 eight years before. Magnetic resonance angiography and aortic angiography revealed severe stenosis in the abdominal aorta, and the systolic pressure gradient of intra-aortic blood flow, distal and proximal to a stenotic lesion, was 58 mmHg. These arterial stenoses in the irradiated area were highly suggestive of radiation-induced vasculopathy. She finally underwent an endovascular VIABAHN VBX balloon-expandable stent-graft placement for this radiation-induced abdominal aorta stenosis, which resolved the pressure gradient. After the procedure, her ABI improved to 0.91/0.88 and her BP was well-controlled. This is the first case of successful stent-graft placement for RVH after renal autotransplantation due to radiation-induced abdominal aorta stenosis as a consequence of neuroblastoma.

Apical Aneurysms and Mid–Left Ventricular Obstruction in Hypertrophic Cardiomyopathy

BackgroundApical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%. ObjectivesThe authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms. MethodsThe authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities). ResultsThere were 108 patients with a mean age of 57.4 ± 13.5 years; 40 (37%) were women. A total of 103 aneurysm patients (95%) had mid-LV obstruction with mid-LV complete systolic emptying. Of the patients with obstruction, 84% had a midsystolic Doppler signal void, a marker of complete flow cessation, but only 19% had Doppler systolic gradients ≥30 mm Hg. Five patients (5%) had relative hypokinesia in mid-LV without obstruction. Aneurysm size is not bimodal but appears distributed by power law, with large aneurysms decidedly less common. Comparing mid-LV obstruction aneurysm patients with all control groups, the short-axis (SAX) systolic areas were smaller (P < 0.007), the percent SAX area change was greater (P < 0.005), the papillary muscle (PM) areas were larger (P < 0.003), and the diastolic PM areas/SAX diastolic areas were greater (P < 0.005). Patients with aneurysms had 22% greater SAX PM areas compared with those with elevated LV velocities but no aneurysms (median: 3.00 cm2 [IQR: 2.38-3.70 cm2] vs 2.45 [IQR: 1.81-2.95 cm2]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying. ConclusionsThe great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.

Balloon-expandable stents for native coarctation of the aorta in children and adolescents.

Although balloon-expandable stent implantation for native aortic coarctation is a preferred method in the adolescent age group, there are insufficient data about indications for and the efficacy of the procedure in a younger age group. The aim of this study was to compare and evaluate the data of young pediatric and adolescent patients who underwent balloon-expandable stent implantation because of native aortic coarctation. The retrospective analysis included the demographic characteristics and data related to the procedure and follow-up of patients who underwent stent implantation for native aortic coarctation between August 2010 and November 2017. Patients with re-coarctation were excluded from the study. The patients were separated into 2 groups as the adolescent group (Group I: 10-18 years) and the pediatric group (Group II: ≤ 9.9 years). Group-I comprised of 18 patients and Group-II, 32 patients. Covered stent was implanted to 32 (73%) patients and uncovered stent to 12 (27%) patients. The procedural success rate was 100%. Following stent implantation, peak systolic gradient decreased significantly in both groups (P < .0001) (Group-I: from 35.9 ± 16.6 mm Hg-2.2 ± 3.4 mm Hg, Group II: from 34 ± 13.3 mm Hg-3 ± 4.09 mm Hg). Complications developed in 3 patients, and all in Group I. Femoral hematoma developed in 1 patient, balloon rupture occurred during the procedure in 1 patient, and there was temporary loss of pulse in 1 patient. All the complications were treated successfully. All the patients were taking anti-hypertensive drugs before intervention and during the mean 23-month follow-up period (range, 2-84 months), hypertension recovered in 35 (80%) patients and drugs were terminated. Stent implantation for aortic coarctation in the pediatric age group may provide pleasing results, reducing the coarctation gradient, providing effective dilatation in the lesion area and eliminating hypertension.

Engineering a New Polymeric Heart Valve Using 3D Printing-TRISKELION.

Background and Objectives: Developing a prosthetic heart valve that combines the advantageous hemodynamic properties of its biological counterpart with the longevity of mechanical prostheses has been a major challenge for heart valve development. Anatomically inspired artificial polymeric heart valves have the potential to combine these beneficial properties, and innovations in 3D printing have given us the opportunity to rapidly test silicone prototypes of new designs to further the understanding of biophysical properties of artificial heart valves. TRISKELION is a promising prototype that we have developed, tested, and further improved in our institution. Materials and Methods: STL files of our prototypes were designed with FreeCad 0.19.2 and 3D printed with an Agilista 3200W (Keyence, Osaka, Japan) using silicones of Shore hardness 35 or 65. Depending on the valve type, the support structures were printed in AR-M2 plastics. The prototypes were then tested using a hemodynamic pulse duplicator (HKP 2.0) simulating an aortic valve cycle at 70 bpm with 70 mL stroke volume (cardiac output 4.9 L/min). Valve opening cycles were visualized with a high-speed camera (Phantom Miro C320). The resulting values led to further improvements of the prototype (TRISKELION) and were compared to a standard bioprosthesis (Edwards Perimount 23 mm) and a mechanical valve (Bileaflet valve, St. Jude Medical). Results: We improved the silicone prototype with currently used biological and mechanical valves measured in our setup as benchmarks. The regurgitation fractions were 22.26% ± 4.34% (TRISKELION) compared to 8.55% ± 0.22% (biological) and 13.23% ± 0.79% (mechanical). The mean systolic pressure gradient was 9.93 ± 3.22 mmHg (TRISKELION), 8.18 ± 0.65 mmHg (biological), and 10.15 ± 0.16 mmHg (mechanical). The cardiac output per minute was at 3.80 ± 0.21 L/min (TRISKELION), 4.46 ± 0.01 L/min (biological), and 4.21 ± 0.05 L/min (mechanical). Conclusions: The development of a heart valve with a central structure proves to be a promising concept. It offers another principle to address the problem of longevity in currently used heart valves. Using 3D printing to develop new prototypes provides a fast, effective, and accurate way to deepen understanding of its physical properties and requirements. This opens the door for translating and combining results into modern prototypes using highly biocompatible polymers, internal structures, and advanced valve layouts.

Abstract 14458: It Takes Three to Tango: A Case of Occult Streptococcus Gordonii Infectious Endocarditis After Dental Cleaning Identified by History, Multi-Modal Imaging, and a Multi-Disciplinary Effort

Introduction: We present a case of a 65 year-old man with severe aortic regurgitation requiring a stented 23mm Edwards bioprosthetic aortic valve (BioAVR), who presented with 3-months of fevers and fatigue found to have Streptococcus gordonii ( S. gordonii ) infectious endocarditis (IE). He underwent dental cleaning 3-months prior sans prophylactic antibiotics. An echocardiogram on admission revealed increased peak/mean systolic gradients across the BioAVR to 46/26mmHg compared to gradients one year prior (32/19mmHg). Initially, a vegetation was not visualized by transthoracic echocardiogram (TTE) despite radiographic evidence of multi-organ emboli. Serial TTEs and transesophageal echocardiograms (TEEs) were performed due to persistent symptoms. Eventually, a new vegetation was visualized by TEE. A team effort involving cardiology, infectious disease, neurology, and cardiac surgery was required for management. Revision of the BioAVR was not pursued due to resolution of the vegetation. The patient was discharged after a prolonged hospitalization with recommendations to take amoxicillin 2g prior to future dental work. Results: Figure 1. A new 0.5cm vegetation visualized by TEE (A) with enlargement to 0.8cm (B), followed by a healed vegetation (C), and resolution 7-months later. Conclusions: S. gordonii has historically been a rare cause of IE therefore requiring a high clinical suspicion for diagnosis. Approximately 8% of IE cases are caused by streptococci. However, a recent study in Denmark on streptococci IE implicated S. gordonii in 44.2% of IE cases . S. gordonii is a gram-positive cocci found in oral flora known to cause subacute bacterial endocarditis. Our case highlights the importance of detailed history-taking to identify sources of infection, serial multi-modal imaging evaluation despite initial negative findings particularly with persistent clinical symptoms, and a multidisciplinary approach to IE cases due to multi-organ involvement.

Abstract 14923: A Special Type Aortic Left Ventricular Tunnel With Bicuspid Aortic Valve: A Case Report

Introduction: Aortic-left ventricular tunnel (ALVT) is an extremely rare congenital cardiac lesion that is an abnormal passage connecting the ascending aorta to the left ventricle. ALVT can be diagnosed by the use of real-time two-dimensional and continuous-wave Doppler echocardiography. Case presentation: A two-year and seven-month-old boy with recently diagnosed influenza presented to the hospital because of severe tachypnea and fatigue after running. Physical examination revealed a grade 3/6 systolic and diastolic murmur at the second and third left intercostal space. Transthoracic echocardiography (GE E95 with a 5-MHz scanning head) demonstrated that the aortic valve was a bicuspid aortic valve (BAV) with mild-moderate insufficiency during diastole, the peak systolic velocity (PSV) and gradient of aortic valve were 1.9m/s and 14mmHg, respectively; The aortic sinotubular junction (ASJ) was stenotic, the PSV and gradient of ASJ were 2.7m/s and 29mmHg, respectively; A tubular-like structure originated in the outlet septum beneath the right coronary cusp and terminated in the ascending aorta superior to the right coronary aortic sinus (Fig. A, B), CDFI and continuous-wave Doppler showed unobstructed to-and-fro (systolic and diastolic) flow in the tunnel with a high-velocity color signal that aliased during systole (Fig. C-E). Clinical considerations for congenital heart disease: ALVT combines BAV with aortic insufficiency and supravalvar aortic stenosis. The patient was referred for possible surgical correction. Discussion: In this patient, the type of ALVT does not belong to any one of Hovaguimian’s classifications. Two-dimensional echocardiography can display the tunnel-like structure of ALVT in real-time, and CDFI can identify that two-stage bidirectional shunted blood flow, systolic ventricular ejection occurs through both the tunnel and the semilunar valve, and the diastolic flow reverses from the aorta to ventricle via ALVT.

Abstract 12290: Effects of Hemodynamic Triggers and Genetic Predisposition on Endocardial Fibroelastosis Recurrence

Introduction: Endocardial fibroelastosis (EFE) can be the cause of diastolic dysfunction in congenital heart defects. It is derived through endothelial-to-mesenchymal transformation (EndMT), and flow disturbances have been associated with its development. Surgical resection is the only available treatment, but recurrence is often observed. We sought to determine hemodynamic predictors and genetic predisposition of EFE recurrence for preoperative risk stratification. Methods: In 93 patients undergoing LV-EFE resection from 01/2010-03/2021, univariate and multivariable competing risks analysis was applied to determine the effects of hemodynamic parameters on EFE recurrence. In 48 patients with EFE, we classified patient’s rare de novo , damaging missense, and loss of function variants from whole exome sequencing through gene ontology and pathway network enrichments analysis associated with EndMT and/or reduced myocardial contraction or conduction. Results: 51 patients (54.8%) had EFE recurrence with a median time to recurrence of 2.2 years (95% CI 1.3-3.0). We identified mitral valve area (&lt;1 cm 2 ), LV filling pressures (≥9 mmHg), aortic valve (AoV) mean systolic gradient (≥25 mmHg), and AoV diameter (Z-score ≤ -1.6) as predictors of faster time to recurrence (Fig. 1A). In a scoring system, these predictors together achieved an area under the curve of 0.79. Rare variants in the cohort were found in genes enriched for functions related to EndMT and myocardial conduction/contraction, with 75% of patients possessing a variant in such a gene. The combination of both (n=16) was associated with a significantly higher yearly recurrence incidence (p&lt;0.05, Fig. 1B). Conclusions: The degree of hemodynamic disturbances and genetic predisposition predict the time to recurrence in patients after EFE resection. Understanding mechanisms behind EFE progression might provide an opportunity to prevent recurrence and develop new treatment options.

Impact of atrial fibrillation in clinical outcomes of low gradient aortic stenosis

Abstract Introduction Aortic stenosis (AS) is the most common significant valvular heart disease in developed countries. A significant portion of the AS populations have low-gradient AS (LGAS), defined as aortic valve area ≤1.0 cm2 and a trans-aortic mean systolic gradient and peak velocity &amp;lt;40 mmHg and &amp;lt;4 m/s, respectively. LGAS has been shown previously to have worse mortality compared to high-gradient AS (HGAS). Atrial fibrillation (AF) is associated with LGAS and AF has been associated with worse outcomes compared to sinus rhythm (SR) in HGAS. The prognostic impact of AF in LGAS is not well described in previous literature. Hypothesis AF will be associated with worse clinical outcomes compared to SR in patients with LGAS. Methods 3400 patients diagnosed with LGAS from 2010–2020 were retrospectively identified and analyzed. Their electrical rhythm was analyzed at the time of their echocardiographic diagnosis of LGAS and patients were split into 3 separate groups: SR (n=2036), SR with history of AF (n=519), and AF (n=845). After adjustment for age, sex, and Charlson Comorbidity Index (CCI), primary endpoints of overall mortality and cardiac mortality were assessed for patients. Results Compared to those with SR, patients with AF and history of AF had significantly higher overall mortality (HR 1.52, p&amp;lt;0.0001 and HR 1.22, p=0.004, respectively) and cardiac mortality (HR 2.05, p&amp;lt;0.0001 and HR 1.37, p=0.03, respectfully) (Figure 1). On further sub group analysis, AF seemed to be most importantly associated with mortality and cardiac mortality in patients with preserved ejection fraction (EF &amp;gt;50%, normal flow LGAS) compared to those patients with reduced EF (classical low-flow LGAS), where there was no statistically significant difference in outcomes between AF and SR (Figure 2). Conclusions Atrial fibrillation, compared to sinus rhythm, is associated with worse overall mortality and cardiac mortality in patients with LGAS and preserved EF. Specifically, this association was strongest in patients with preserved EF &amp;gt;50%. Given these findings, the presence of AF should be factored into clinical decision making regarding LGAS management given the higher risk of age, sex, and CCI adjusted overall and cardiac mortality. Further research needs to be done to see if earlier aortic valve intervention in these patients would improve mortality compared to their SR counterparts. Funding Acknowledgement Type of funding sources: None.

The impact of transcatheter edge-to-edge repair on right ventricle-pulmonary artery coupling in patients with functional mitral regurgitation.

The impact of transcatheter edge-to-edge repair on right ventricle-pulmonary artery coupling in patients with functional mitral regurgitation.

Clinical, Experimental, and Computational Validation of a New Doppler-Based Index for Coarctation Severity Assessment

Long-term morbidity including hypertension often persists in coarctation patients despite current guidelines. Coarctation severity can be invasively assessed via peak-to-peak catheter pressure gradient (PPCG), which is estimated noninvasively via simplified Bernoulli equation and conventionally reported as peak instantaneous Doppler gradient (PIDG). However, underlying simplifications of the equation limit diagnostic accuracy. We studied the diagnostic performance of a new Doppler-based diastolic index called the continuous flow pressure gradient (CFPG) versus conventional indices in assessing coarctation severity. In a rabbit model mimicking human aortic coarctation, temporal blood pressure waveforms revealed the diastolic instantaneous pressure gradients and spectral Doppler features impacted by coarctation severity. We therefore hypothesized that CFPG provides superior correlation with coarctation gradients measured invasively. PIDG and CFPG were quantified using color flow echocardiography in humans and rabbits with discrete coarctations. Results were compared with PPCG in rabbits (n=34) and arm-leg systolic gradients (n=25) in humans via 1-way analysis of variance, Pearson's correlation, linear regression, and Bland-Altman analysis. A threshold of CFPG ≥ 4.6mm Hg was identified via the Youden index as representative of PPCG ≥ 20mm Hg (the current guideline value for coarctation intervention) in rabbits, while a CFPG ≥1.0mm Hg represented an arm-leg systolic gradient ≥20mm Hg in humans. Accuracy measures revealed superior correlation of CFPG (R2>0.80) and mild receiver operating characteristic improvement (area under the receiver operating characteristic curve, 0.94-0.95) compared with PIDG (R2<0.63; area under the receiver operating characteristic curve, 0.89-0.95). Inter-/intraobserver variability tested by intraclass correlation coefficient revealed measurement reliability with differences ≤8.2% and 10.7%, respectively. Computational simulations of anesthetized versus conscious hemodynamics showed parameters were minimally impacted by isoflurane inherent in the dataused to derive CFPG. These results confirm the potential diagnostic accuracy of CFPG in echocardiography-based coarctation severity assessment. We are optimistic that CFPG will be useful for translation of results from preclinical studies that revisit current guidelines to limit morbidity in humans with aortic coarctation.