Biventricular Function Research Articles

Abstract 4143264: A Case of Spike-on-T Phenomenon and Polymorphic Ventricular Tachycardia

Background: R-on-T phenomenon occurs when an electrical stimulus is delivered at a critical point during ventricular repolarization. This can initiate ventricular arrhythmias like polymorphic ventricular tachycardia (PMVT). We describe a case of ventricular pacemaker spikes delivered on the T wave causing PMVT. Case: A 53-year-old female with CAD s/p stent, postpartum cardiomyopathy s/p Bi-V CRT-D (Boston Scientific G124), and paroxysmal atrial fibrillation presented for elective endoscopy and colonoscopy to evaluate her dysphagia and abdominal pain. Her CRT-D was reprogrammed from DDD pacing with lower rate limit (LRL) 50 bpm to an asynchronous Bi-V DOO mode at 50 bpm (‘electrocautery mode’) for the procedure (tachy therapy disabled). Her LV-RV offset was 40 msec. Prior to receiving sedation or medications, she was found unresponsive. Telemetry showed Spike-on-T phenomenon which initiated PMVT. She was externally defibrillated with 200J and received magnesium and an IV amiodarone bolus. She returned to sinus rhythm, but one minute later had another Spike-on-T event initiating PMVT. She was successfully defibrillated with 360J. Post-shock EKG showed an asynchronous Bi-V paced rhythm at 50 bpm. Decision Making: The patient was admitted to the CCU for post-resuscitation care. Her electrolytes and cardiac enzymes were unremarkable. Her CRT-D was reprogrammed to DDD 80-140 bpm. Transthoracic echocardiogram showed normal biventricular systolic function. Cardiac catheterization did not show obstructive CAD. After reprogramming of her device, she had no further events. After initial treatment with IV amiodarone load, she was discharged home on oral magnesium gluconate. Discussion: The only intervention prior to her procedure was device reprogramming (DDD 50 bpm to DOO 50 bpm). Telemetry showed pacer spikes initiating PMVT. Given the LV-RV offset of 40 msec, she would have received these two tightly coupled pacemaker spikes in an asynchronous mode, in this unfortunate instance during her T wave. While her bowel preparation may have led to electrolytes abnormalities, post-resuscitation electrolytes were normal. Fortunately, she received prompt therapy and was reprogrammed with increased LRL. Conclusion: We described a case of Spike-on-T PMVT prior to colonoscopy without obvious provocation other than asynchronous pacemaker spikes. Reprogramming devices in DOO mode with increased LRL may prevent PVCs and asynchronous pacemaker spikes from triggering PMVT.

Abstract 4145861: Systemic Sclerosis is Associated with Sub-Clinical Abnormalities in Myocardial Energetics, Perfusion and Increased Fibrosis

Background: Primary cardiac involvement in systemic sclerosis (SSc) is heterogenous, poorly defined and associated with significant cardiac morbidity and mortality. The underlying mechanism is not well understood but may include the impact of coronary microvascular dysfunction and myocardial inflammation. Myocardial cellular metabolic alterations in SSc are yet to be established. Aims: Using 31 Phosphorous magnetic resonance spectroscopy ( 31 P MRS) and cardiovascular magnetic resonance (CMR), we aimed to establish if myocardial energetics, measured as phosphocreatine (PCr) /ATP ratio, and quantitative myocardial perfusion were reduced in individuals with SSc compared to healthy volunteers (HV). Methods: A total of 35 age and sex matched participants were recruited (25 SSc, 10 HV). All subjects underwent 31 P MRS and comprehensive CMR protocol including volumetric analysis, quantitative myocardial perfusion and T1 mapping. Results: Table-1 shows clinical and CMR / 31 P MRS data. Bi-ventricular volumes, systolic function and left atrial function were similar in both groups. NT-Pro BNP and myocardial extra cellular volume (ECV) were significantly increased in SSc. Both PCr/ATP and myocardial perfusion reserve (MPR) were significantly reduced in SSc compared with controls. The disease activity as measured by the European Scleroderma Trials and Research group (EUSTAR) activity index did not correlate with either NTproBNP (P=0.79), PCr/ATP (P=0.35) or MPR (P=0.6). Conclusions: In addition to validating our previous observations of reduced perfusion and increased myocardial fibrosis in SSc, we have demonstrated for the first time significant reductions in myocardial energetics in patients with SSc. These findings provide further valuable insight into the pathogenesis of primary myocardial disease in SSc.

Abstract 4112639: A Rare Case of Atypical Sarcoidosis Presenting as an Intra-Atrial Septal Mass

Introduction: Sarcoidosis is a granulomatous disease that can affect multiple organ systems, including the heart. Cardiac Sarcoidosis (CS) is one of the most severe manifestations. It is characterized by a dysregulated T-cell driven immune response which generates non-necrotic inflammatory granulomas with myocardial infiltration. CS has a range of clinical syndromes: impaired conduction, arrhythmias, heart failure, and sudden cardiac death. We discuss a rare presentation of CS as an intra-atrial septal mass. Case Description: A 27-year-old Caucasian female with history of depression on citalopram presented with a one-month history of palpitations, fatigue, and dyspnea with exertion. An EKG demonstrated sinus rhythm with first-degree AV block. A cardiac monitor revealed intermittent 2-1 AV block and high degree AV block occurring mostly during sleep. An echocardiogram revealed normal biventricular function and morphology. Due to the concern for infiltrative cardiomyopathy, she underwent a cMRI which revealed a focal mass centered along the intra-atrial septum extending from the fossa ovalis to the septal AV junction, measuring 4.5 cm in size. She underwent pacemaker implantation with mild improvement of symptoms. PET scan demonstrated hypermetabolic activity of the intra-atrial region. She underwent surgical resection of the cardiac mass. Pathology was significant for fibrotic tissue with non-caseating granuloma and multinucleated giant cells consistent with an extremely rare presentation of CS. She was started on immunosuppressive medications. Subsequent cMRI and PET demonstrated resolution of the mass and hypermetabolic activity. Discussion: CS is a severe condition that most commonly presents with symptoms of heart failure, conduction abnormalities, and cardiac morphological changes of hypertrophy or thinning. However, it is often missed as a diagnosis, even in patients with extracardiac sarcoidosis. It is rare for CS to present as an isolated cardiac mass, especially in a younger patient as in this case. Advances in imaging techniques such as echocardiography, MRI, and PET have improved the ability to diagnosis CS and provide timely specialized treatment. This case highlights the need to include CS as an important diagnosis on the differential for a patient presenting with conduction abnormalities, heart block, and dyspnea. It also accentuates the critical role of multimodality imaging in working up this disease.

Abstract 4138424: Ring of Power: Unraveling a Rare Diagnosis in a Young Male with Palpitations

Case Description: A 36-year-old male presented for an outpatient treadmill ECG stress test for further evaluation of palpitations exacerbated by exercise. He had no ischemic ECG changes, but developed a sustained episode of polymorphic VT at 13 minutes (Figure 1a). The test was stopped and the VT self-terminated after 43 seconds. The patient was admitted for further workup. Lab workup was unremarkable; baseline ECG demonstrated no ST-T wave changes and displayed normal QT and QRS duration. TTE showed mild biventricular dysfunction. Coronary CT angiogram showed type 3 subtype of Vieussens’ arterial ring (VAR), with an absent left main and super-dominant RCA supplying both LAD and LCx. FFR-CT showed non-hemodynamically significant stenosis in the mid-distal LAD (Figure 1b). Left heart catheterization confirmed presence of VAR and mild diffuse disease in a small-caliber mid-distal LAD (Figures 1c&d). No malignant course or dynamic coronary compression was present. Cardiac MRI revealed normal biventricular function and the absence of late gadolinium enhancement. An exercise stress test, now with myocardial perfusion, was performed and was entirely normal without evidence of myocardial ischemia. Genetic testing for catecholaminergic polymorphic VT was negative. After a multidisciplinary discussion, it was decided not to pursue CABG in the absence of demonstrable ischemia at peak exercise. He underwent an ICD placement for secondary prevention and was discharged on beta blockers. Discussion: Three distinct subtypes of VAR have been described, with type 3 being the rarest. The coronary anomaly was first described by Raymond de Vieussens in 1706, and represents an embryologic remnant of the conotruncal circle. It may be diagnosed incidentally in asymptomatic patients or present with angina especially if underlying CAD, aneurysms or fistulae are present. We report the first case of exercise-induced polymorphic VT in a type 3 VAR patient, theorizing that intermittent myocardial ischemia at peak exercise may induce VT in these patients. Surgical revascularization may be considered if myocardial ischemia is demonstrated on stress testing, although its benefit in patients without such evidence of ischemia is unknown.

Abstract 4119267: Diagnosing An Uncommon Presentation of Cardiac Sarcoidosis with Isolated Bi-Atrial Involvement: A Case Report

Background: Sarcoidosis, a systemic granulomatous inflammatory disorder can involve various organs, including the heart but isolated bi-atrial cardiac involvement is rare. Advanced cardiac imaging especially in atypical presentations, can aid in early diagnosis. Case: A 59 year-old man with history of biopsy-proven pulmonary sarcoidosis presented with non exertional chest pain for 2 months. EKG, cardiac enzymes, and Initial echocardiogram(TTE) was unremarkable. Stress echocardiogram ruled out myocardial ischemia. CT scan noted mediastinal lymphadenopathy consistent with known sarcidosis. In absence of other explainable etiolgies for chest pain, Cardiac MRI was done and showed preserved biventricular function, subepicardial enhancement in the basal inferior, inferolateral, and anterolateral walls. The enhancement raised suspicion for CS. However, symptoms resolved spontaneously, cardiac workup paused and he was monitored conservatively with serial Echocardiogram(TTE) until onset of dyspnea 3 years later. Repeat TTE and EKG then noted newly enlarged left atrium and atrial tachycardia. Further testing with Cardiac positron emission tomographic imaging with F-18 fluorodeoxyglucose (FDG-PET CT) showed abnormal myocardial uptake in both atrial posterior walls but no ventricular involvement, indicative of isolated bi-atrial inflammation in CS. He was treated with prednisone and methotrexate. Successful symptom and inflammation resolution on FDG-PET CT occurred in 3 months Discussion: CS is rare and seen clinically in about 5% and diagnosed postmortem in 25% of sarcoidosis cases. Symptoms vary widely, with potential for severe heart complications. Left ventricle and interventricular septum are commonly involved, but isolated bi-atrial involvement is rare. Early diagnosis aided by Cardiac MRI and FDG-PET CT is crucial. Prednisone is mainstay of treatment, often combined with methotrexate. Cases of concurrent atrial CS involvement and bi-atrial fibrosis with Left ventricular hyperenhancement are documented, but this is the first known report of isolated bi-atrial hyperenhancement on FDG-PET CT for CS. This case highlights the need for symptom correlation with clinical and imaging follow-up, especially in atypical presentations.

Biventricular Diastolic Functions before and After Interventional Occlusion of Interatrial Communication

Biventricular Diastolic Functions before and After Interventional Occlusion of Interatrial Communication

The dynamic increment of epicardial adipose tissue is associated with reverse remodeling after guideline-directed medical treatment in dilated cardiomyopathy patients

Abstract Background/Introduction Epicardial adipose tissue (EAT) accumulation is associated with adverse prognosis in patients with HFpEF and a better prognosis in HFrEF patients. Whether EAT is playing a protective role in dilated cardiomyopathy (DCM) patients have been described sparingly. Purpose Aim to explore whether the epicardial fat would change along with the dynamic changes in cardiac function and volumes after receiving guideline-directed medical therapy (GDMT) in patients with DCM. Methods We prospectively enrolled 232 DCM patients who underwent complete baseline and follow-up Cardiac magnetic resonance (CMR) examinations. The measurement of the epicardial fat composition includes epicardial fat thickness, paracardial fat thickness, and paracardial fat volume, while the measurement of body fat composition includes subcutaneous and visceral abdominal fat, while We used univariable and multivariable logistic regression to evaluate the association between different body and cardiac fat depots with the changes in bi-ventricular structure (delta indexed EDV) and function (delta EF). Results The study included 232 patients (mean age 46.1±14.1 years, 157 (67.7%) males). After GDMT, we found that the visceral fat thickness and EAT (thickness and volume) tend to significantly increase. The increase in the (left ventricular reverse remodeling) LVRR group was significantly higher compared with the non-LVRR group. Independently with the changes of BMI and body fat depots, the increment of EAT was associated with the improvement in bi-ventricular function and volumes (significantly positively correlated with the improvement of LVEF (r = 0.499, p < 0.001), and RVEF (r = 0.482, p < 0.001), negatively correlated with delta LVEDVi (r = -0.407, p < 0.001), delta RVEDVi (r = -0.305, p < 0.001)). Conclusions After GDMT, the increment of EAT was positively correlated with the improvement of bi-ventricular structure and function, independent of the changes in BMI and body fat depots.Graphic Abstract

Mortality and heart failure in patients with severe tricuspid regurgitation. Impact of RV volumes and function by CMR.

Abstract Background Beyond symptoms, right ventricle (RV) dilatation and dysfunction are established criteria for intervention in patients with significant tricuspid regurgitation (TR); however defined thresholds to support intervention are lacking. As a result, the optimal timing for intervention remains controversial and it is commonly undertaken at a late stage. Purpose To describe prognostic cut-off values of RV size and function by Cardiac Magnetic Resonance in a multicentre cohort of patients with at least severe TR. Methods The study enrolled patients with at least severe TR assessed by 2D echocardiography who underwent a contemporary cardiac magnetic resonance (CMR) study. Conventional parameters of biventricular volume and function were assessed in all patients. A combined endpoint of hospital admission due to heart failure (HF) and all-cause mortality was defined. Results 318 patients with severe TR recruited from 5 tertiary care hospitals were included in this study (70 ± 11 years, 67% female, 73% NYHA I or II). Regarding the etiology, 10% were primary TR, 1% CIED-related TR, 46 % atrial secondary TR and 43% ventricular secondary TR. After a median follow-up of 28 months (IQR: 10-54 months), 42% of the patients (n=134) experienced the combined endpoint (n=113 were admitted due to HF, n=61 died). After adjusting for age, NYHA class, comorbidities, type of TR and LVEF in a multivariate Cox proportional model (LR Chi-Square: 88.01, p<0.001), RV-EDV and RVEF were independently associated with all-cause mortality and heart failure (adjusted HR for RV-EDV per ml/m2 =1.008 [1.005-1.012], p<0.001 and RVEF HR per 1%=0.961 [0.942-0.981], p<0.001). Thresholds of RV-EDV ≥ 93 ml/m2, RV-ESV ≥ 39 ml/m2 and RVEF ≤ 58% held the best accuracy to predict outcomes (figure). In multivariable analysis, prognostic cut-off values of RV-EDV, RV-ESV and RVEF was associated with 1.91, 2.03, and 1.76-fold increased risk of HF or all-cause mortality respectively (RV-EDV HR: 1.91 [1.34-2.71], p<0.001, RV-ESV HR 2.03 [1.42-2.89], p<0.001 and RVEF HR1.76 [1.25-2.49], p=0.001). Conclusion RV size and function are crucial for determining optimal timing for TR intervention. CMR remains the gold standard to assess RV remodeling. Cut-off values of RV volume and function by CMR are defined in a multicentre cohort of patients based on outcome data; proposed thresholds differ from those previously established by echocardiography.Kaplan Meier curves

Effective RV ejection fraction by CMR: a new index of RV function in patients with severe TR

Abstract Background Right ventricle (RV) dysfunction represent an formal indication for intervention in patients with significant tricuspid regurgitation (TR). RV ejection fraction (RVEF) by Cardiac Magnetic Resonance (CMR) is considered the gold standard of RV function; however it is influenced by changes of preload conditions and may remain unaffected until late stages in severe TR. Effective RV ejection fraction (eRVEF) is corrected by the amount of TR and may reflect an earlier parameter of RV dysfunction. Purpose To compare the prognostic impact of both indices of RV function: RVEF and eRVEF in a multicentre cohort of patients with severe TR. Methods Patients with at least severe TR assessed by 2D echocardiography with a contemporary cardiac magnetic resonance (CMR) study were included in this study. In addition to conventional parameters of biventricular volume and function, eRVEF was assessed in all patients. eRVEF was calculated according to the formula: eRVEF=net pulmonary forwardflow/RV− EDV (figure). A combined endpoint of hospital admission due to right heart failure and cardiovascular mortality was defined. Results 271 patients with severe TR recruited from 5 tertiary care hospitals were included in this study (age 71 ± 11 years, 67% female, 83% NYHA I or II). Regarding the etiology, 10% were primary TR, 1% CIED-related TR, 46 % atrial secondary TR and 43% ventricular secondary TR. After a median follow-up of 27 months (IQR: 10-53 months), 41% of the patients (n=112) experienced the combined endpoint (n=97 were admitted due to HF, n=49 died). After adjusting for age, NYHA class, comorbidities, type of TR and LVEF in multivariate Cox proportional models, both RVEF and eRVEF were independently associated with all-cause mortality and heart failure (adj HR for RVEF HR per 1%=0.96 [0.94-0.98], p<0.001 and for eRVEF per 1%=0.98 [0.96-0.99], p<0.001). A value of eRVEF of ≤37% were associated with impaired prognosis (figure). eRVEF identified higher rates of RV dysfunction compared to RVEF (49% vs 38%, p<0.001). eRVEF was superior to RVEF to predict outcomes (higher AUC 0.70 [0.63-0.76] vs 0.66 [0.59-0.73, p<0.001; Δ C-statistic 0.139 [0.040-0.237], p=0.05). Conclusion RV function is crucial for determining optimal timing for TR intervention. eRVEF is corrected by the amount of TR and identify higher rates of RV dysfunction in severe TR. eRVEF held stronger association with outcome compared to RVEF.eRVEF calculationKaplan Meier Curves

Clustering analysis based on clinically available data for improving risk stratification and outcome prediction of hypertension

Abstract Background Hypertension (HTN) is a heterogeneous condition with several factors contributing to adverse outcomes. Machine learning can harness complex data to unveil outcome predictors and guide prevention. Purpose To use clustering analysis on clinical data to identify HTN risk profiles and their associations with cardiovascular abnormalities and clinical outcomes. Methods A cohort of 14,840 UK Biobank hypertensive participants without pre-existing heart failure (HF) who underwent cardiovascular magnetic resonance (CMR) assessment was examined using K-means clustering on 79 clinical attributes. The clusters’ association with incident HF, mortality, atrial fibrillation (AF), and major adverse cardiovascular events (MACE) were evaluated, alongside imaging characteristics, with mediation analysis probing the role of imaging in risk differentiation. All post-hoc analyses were adjusted for vascular risk factors (VRFs), including diabetes, hypercholesterolaemia, previous myocardial infarction. Results Three distinct clusters differing considerably in lifestyle habits, cardiovascular risk factors, electrocardiographic parameters, and cardiometabolic profiles were identified. Cluster 1 (lowest risk), predominantly females, exhibited the most favourable metabolic profile and outcomes. Cluster 2 (highest risk), predominantly males, had the most adverse metabolic and cardiovascular profiles, and the highest risk of all adverse outcomes, especially AF and HF (hazard ratio (HR) 1.80 [1.45–2.25] and 1.85 [1.39–2.46]; p <0.005). This group was associated with more eccentric LV hypertrophy (LVH), overall poorer left atrial emptying function (LAEF), bi-ventricular systolic function and LV mechanics, and higher systemic vascular resistance (Figure 1). Cluster 3 (intermediate risk) was a more heterogeneous group with the highest burden of diabetes and adiposity and a moderate rise in AF and MACE risk (HR 1.61 [1.12-2.32] and 1.30 [1.07-1.58]; p <0.05). This cluster was associated with concentric LV remodelling and better indices of cardiac mechanics, but greater impairment in LA booster pump function (active LAEF) than Cluster 2 (Figure 1). Cluster 2's effect on MACE was mostly mediated through LV mass increase (proportion mediated, 67%), followed by changes (reducing) in LV global longitudinal strain (33%), and ejection fraction (25.4%). The indirect effect of CMR changes in the association between cluster 3 and MACE was much smaller with the highest proportion of the effects mediated through increased myocardial wall thickness (26%) but not LV mass, and changes in all the components of LA function. Conclusions Clinical data clustering provides insight into distinct HTN risk profiles, each with specific imaging characteristics and clinical trajectories, hinting at different pathophysiological mechanisms. Enhanced phenotyping can facilitate tailored interventions based on the disease subtype and improve clinical outcomes.Figure 1.

Cardiac electrical and functional activity following an outdoor cold-water swimming event

AimsParticipation in outdoor cold-water swimming (OCWS) events combines endurance exercise and cold exposure. Concerns have emerged about the potential risk of acute adverse cardiac events during OCWS, particularly during endurance events. We analysed the effect of prolonged OCWS on cardiac function in trained athletes. MethodsThe swimming event consisted of laps over a 1000-m course, for up to 6 h, in water at 15 °C. Twenty participants (11 males, 47.3 ± 8.6 years old) were included. Core temperature (Tcore) was monitored using an ingestible temperature sensor during and up to 1 h after the swim. Body composition, blood pressure, electrocardiogram (ECG), and transthoracic echocardiography were assessed 1 day before the event and within the first hour upon completion of the swim. ResultsMean body mass index was 27.1 ± 5.1 kg/m2 and fat mass was 25.2 ± 9.1 %. Mean duration of swimming was 214 ± 115 min. Minimum Tcore was 35.6 ± 1.3 °C. A significant lengthening of the QT interval corrected (QTc) for heart rate was observed post-exercise (437.7 ± 27.7 vs. 457.2 ± 35.9 ms, p = 0.012), with 5 participants exhibiting post-exercise QTc >500ms. OCWS did not alter the biventricular systolic function and left ventricular relaxation. No correlation was observed between ΔQTc and ΔTcore. ConclusionOCWS seemed to acutely delay post-exercise cardiac repolarization without alteration of cardiac function in a healthy trained population. Additional investigations would be warranted to explore the clinical implications of QT lengthening and its relationship with autonomic nervous system regulation during OCWS.

Expect the unexpected: fulminant myocardial cytotoxic Injury from Trabectedin

BackgroundTrabectedin (Tbt) is an alkylating agent prescribed for soft tissue sarcomas after treatment failure of first line agents. While cardiomyopathy can occur with Tbt treatment after anthracycline exposure, Tbt-induced fulminant myocardial cytotoxic injury in the setting of other systemic cytotoxicity associated with Tbt has not been reported.Case presentation51-year-old female with hypertension, hyperlipidemia, metastatic leiomyosarcoma with progression of disease despite several lines of chemotherapy including doxorubicin-based therapy was started on Trabectedin (Tbt) 5 days prior to presentation with symptoms of fever, myalgias, arthralgias, and palpitations. She was admitted for management of rhabdomyolysis, acute kidney and liver injuries which were reportedly known to be associated with Tbt treatment. A baseline electrocardiogram (ECG) revealed sinus tachycardia with non-specific T-wave changes, and a transthoracic echocardiogram (TTE) was unremarkable. However, on day 3 of hospitalization, an episode of asymptomatic sustained monomorphic ventricular tachycardia with a heart rate of 150 beats per minute was captured on telemetry. A 12-lead ECG revealed new septal T-wave inversions. Labs revealed rising hs-TnI levels (peak at 37,933ng/L) and serum markers suggested multi-organ failure. Steroids were initiated given its role in treating multi-organ Tbt-induced toxicity. A cardiac MRI to rule out myocarditis and left heart catheterization to rule out obstructive coronary artery disease were forgone due to acute renal failure. A right heart catheterization with an endomyocardial biopsy was performed revealing normal cardiac filling pressures and indices. Pathology showed cytoplasmic vacuoles indicating drug-induced myocardial cytotoxicity. Serial echocardiograms revealed preserved biventricular function. The patient’s clinical condition deteriorated with multi-organ failure despite maximal supportive care in the intensive care unit. She ultimately passed away, and an autopsy was declined.ConclusionThis is the first reported case of fulminant myocardial injury after initiation of Tbt with histologic evidence of drug-induced myocardial cytotoxicity. While it is unclear if anthracyclines potentiate Tbt cytotoxic injury as in this case, it is plausible; and that Tbt-induced cardiotoxicity ranges from subclinical to fulminant. Given increasing use of Tbt in refractory high-grade sarcomas, raising awareness of its toxicity profile will improve early detection and outcomes.

Surgical Closure of Multiple Muscular Ventricular Septal Defects in Children Using 3D-Printed Models.

Multiple muscular ventricular septal defects (VSDs) are often difficult to visualize and access surgically. The main challenge is identifying all defects intraoperatively, without which residual defects are inevitable. Patient-specific three-dimensional (3D) printed models can help accurately demonstrate intracardiac anatomy. We present our experience using this technology to surgically close multiple muscular VSDs . Data of all patients with multiple VSDs in whom a 3D-printed model was used to aid surgical planning between September 2021 and July 2023 was collected retrospectively. Our approach involved generating a 3D model from a preoperative computerized tomography scan for each patient, which was then used to precisely identify the location of the multiple VSDs and plan surgical intervention. Six patients underwent closure of multiple VSDs using a 3D model. The mean age at surgery was 3.5 years (SD ± 2.8 years). Five (83.3%) patients had previously undergone pulmonary artery banding. The VSDs were approached through the right atrium in three (50%) and the right ventricle in three (50%) patients. Mean cardiopulmonary bypass and myocardial ischemia times were 185.2 min (SD ± 94.8 min) and 147.5 min (SD ± 86 min), respectively. There was no postoperative heart block or a hemodynamically significant residual VSD. All six patients had normal biventricular function at a median follow-up duration of 1.7 months (interquartile range: 1.2-7.4 months). 3D printing to aid closure of multiple VSDs is safe, reliable, and reproducible. We recommend adding 3D printing to surgeons' armamentarium when faced with the challenge of closing multiple muscular VSDs in children.

8238 Thyroidectomy for Thyroid Storm Complicated by Drug-Induced Hepatotoxicity and Thyrotoxic Cardiopulmonary Disease

Abstract Disclosure: T.A. John: None. A.C. Suarez: None. J. Genkil: None. S. Patil: None. C. Anastasopoulou: None. Case Presentation: A 59-year-old female with hypertension and type 2 diabetes presented with palpitations, and several months of hyperthyroid symptoms, lower extremity swelling, and dyspnea on exertion. On presentation, patient was diaphoretic, tachycardic, volume overloaded and had symmetric swelling of the thyroid gland. Lab results revealed FT4 3.28 ng/dL, total T3 384.59 ng/dL, TSH < 0.01 µIU/mland elevated TSI. Electrocardiogram showed sinus tachycardia. Chest radiography showed pulmonary edema. Echocardiography showed a reduced ejection fraction of 35%, dilation of right ventricle, severe mitral and tricuspid regurgitation, and severely elevated pulmonary artery systolic pressure (55-60 mm Hg). Thyroid ultrasound revealed enlarged, heterogeneous thyroid gland with normal vascularity. The diagnosis of thyroid storm due to Graves' disease was made with a score of 50 on the Burch-Wartofsky Point Scale. Patient was started on propylthiouracil (PTU), metoprolol tartrate and furosemide. Corticosteroid therapy was not utilized due to the risk of worsening heart failure from sodium retention. After two days of PTU, patient developed drug induced liver injury (DILI). Patient was then transitioned to methimazole which was also discontinued thereafter due to progression of DILI. She was started on potassium iodide to suppress thyroid hormone production, and N-Acetyl cysteine (NAC) for DILI. Due to persistent thyrotoxicosis, the patient underwent a total thyroidectomy on hospital day 13 and was started on levothyroxine for post-surgical hypothyroidism. Liver function returned to normal on day 16. A surveillance echocardiogram obtained 3 months later showed recovered biventricular function (ejection fraction 55-60%), mild mitral and tricuspid regurgitation and normal pulmonary pressures. Educational value: Thyroid storm is an uncommon, life-threatening emergency. Thioamides remain the cornerstone of treatment, but rarely, they can result in adverse events such as hepatotoxicity. When thioamides are contraindicated, other agents (e.g., iodine, lithium, potassium perchlorate, cholestyramine, glucocorticoids) can be utilized; however, refractory cases might require total thyroidectomy as definitive treatments. Our patient had marked recovery of cardiac function and resolution of pulmonary hypertension and DILI after timely surgical intervention. Multidisciplinary care is pivotal for the successful management of thyroid storm. Presentation: 6/3/2024

Early Detection of Myocardial Involvement in Thalassemia Intermedia Patients: Multiparametric Mapping by Magnetic Resonance Imaging.

No study has assessed myocardial T1 and T2 values in patients with beta-thalassemia intermedia (β-TI). To assess the prevalence of myocardial involvement in β-TI patients by T2* relaxometry and native T1 and T2 mapping and to determine the correlation of myocardial relaxation times with demographic and clinical parameters. Prospective matched-cohort study. 42 β-TI patients (27 females, 39.65 ± 12.32 years), enrolled in the Extension-Myocardial Iron Overload in Thalassaemia Network, and 42 age- and sex-matched healthy volunteers (27 females, 40.01 ± 11.36 years) and thalassemia major (TM) patients (27 females, 39.27 ± 11.57 years). 1.5 T/multi-echo gradient echo, modified Look-Locker inversion recovery, multi-echo fast-spin-echo, cine balanced steady-state-free precession, and late gadolinium enhancement (LGE) sequences. Hepatic, pancreatic, and left ventricular (LV) T2* values, LV native T1 and T2 values, biventricular ejection fractions and volumes, and presence and extent of replacement myocardial fibrosis. Comparisons between two groups were performed with two-sample t tests, Wilcoxon's signed rank tests, or χ2 testing. Correlation analysis was performed using Pearson's or Spearman's test. P < 0.05 was considered statistically significant. β-TI patients had significantly higher LV T2 values than healthy subjects (56.84 ± 4.03 vs. 52.46 ± 2.50 msec, P < 0.0001) and significantly higher LV T1 values than TM patients (1018.32 ± 48.94 vs. 966.66 ± 66.47 msec, P < 0.0001). In β-TI, female gender was associated with significantly increased LV T1 (P = 0.041) and T2 values (P < 0.0001), while splenectomy and presence of regular transfusions were associated with significantly lower LV T1 values (P = 0.014 and P = 0.001, respectively). In β-TI patients, all LV relaxation times were significantly correlated with each other (T2*-T1: P = 0.003; T2*-T2: P = 0.003; T1-T2: P < 0.0001). Two patients with a reduced LV T2* also had a reduced LV T1, while only one had a reduced LV T2. Three patients had a reduced LV T1 but a normal LV T2*; 66.7% of the patients had an increased LV T2. All LV relaxation times were significantly correlated with pancreas T2* values (T2*: P = 0.033; T1: P < 0.0001; T2: P = 0.014). No LV relaxation time was associated (P > 0.05) with hepatic iron concentration, biventricular function parameters, or LGE presence. The combined use of all three myocardial relaxation times has potential to improve sensitivity in the detection of early/subclinical myocardial involvement in β-Tl patients. 2 TECHNICAL EFFICACY: Stage 2.

Characteristics of altered biventricular hemodynamics after arterial switch operation for patients with d-transposition of the great arteries with preserved ejection fraction: a four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) study.

The long-term monitoring of biventricular function is essential to identify potential functional decline in patients following the arterial switch operation (ASO). The underlying pathophysiological mechanisms responsible for altered biventricular hemodynamics in ASO patients are not yet well understood. This study sought to: (I) compare the biventricular kinetic energy (KE) and vorticity of ASO patients and age- and sex-matched controls; (II) investigate the associations of four-dimensional (4D) flow biventricular hemodynamics parameters and neo-aortic root dilation, supravalvular pulmonary stenosis, and pulmonary artery transvalvular pressure difference. A total of 34 patients with dextro-transposition of the great arteries (D-TGA) who underwent ASO, and 17 age- and gender-matched healthy controls were prospectively recruited for this study. All the subjects underwent cine and 4D flow and late gadolinium enhancement scans, and all the patients underwent echocardiography within two weeks of cardiovascular magnetic resonance (CMR) imaging. The following four flow components were analyzed: direct flow, retained inflow, delayed ejection flow, and residual volume. In addition, the following six phasic blood flow KE parameters, normalized to the end-diastolic volume (EDV) and vorticity, were analyzed for both the left ventricle (LV) and right ventricle (RV): peak systolic phase, average systolic phase, peak diastolic phase, average diastolic phase, peak E-wave phase, and peak A-wave phase. The independent sample Student's t-test, Mann-Whitney U-test, univariable and multivariable stepwise regression analyses, intra and inter-observer variability analyses were used to compare patients and controls. In relation to the LV, the D-TGA patients had significantly decreased average vorticity, peak systolic vorticity, systolic vorticity, diastolic vorticity, and peak A-wave vorticity compared to the controls (all P<0.01). In relation to the RV, the pulmonary stenosis group had significantly increased peak E- and A-wave kinetic energy normalized to the end-diastolic volume (KEiEDV), and peak and average vorticity compared to the non-pulmonary stenosis group (all P<0.05). in the multivariable logistic regression model analysis, diastolic KEiEDV, peak E-wave KEiEDV peak A-wave KEiEDV, and average vorticity were associated a with transvalvular pressure difference (β=13.54, P<0.001 for diastolic KEiEDV; β=105.26, P<0.001 for peak E-wave KEiEDV; β=-49.36, P=0.027 for peak A-wave KEiEDV; and β=-56.37, P<0.001 for average vorticity). We found that 4D flow biventricular hemodynamics were more sensitive markers than the ejection fraction in the postoperative D-TGA patients. The RV diastolic KEiEDV parameters and average vorticity were risk factors for pulmonary artery obstruction in the multivariable model.

Evaluation of biventricular function by cadmium-zinc-telluride SPECT gated tomographic radionuclide angiography: Comparison to conventional SPECT.

We compared and analyzed the consistency and repeatability of left and right ventricular ((LV/RV) functions obtained by gated-equilibrium radionuclide ventriculography (ERNV) with cadmium-zinc-telluride single-photon emission computed tomography (CZT-SPECT) and conventional SPECT (C-SPECT) with sodium iodide crystal detectors. Seventy-seven patients were included in the retrospective study. Both C-SPECT and CZT-SPECT imaging were performed on the same day. Correlations and differences in LV/RV ejection fraction (LVEF and RVEF), peak ejection rate (PER), and peak filling rate (PFR) were compared between the 2 models. Cardiac magnetic resonance (CMR) was partially used as the gold standard, and ultrasound results were included for comparative analysis. Interobserver reproducibility of each parameter obtained by the 2 cameras was compared. Between the 2 cameras, there were no significant difference in LVEF, LVPER, LVPFR, and RVPER (P > .05) and there were in RVEF and RVPFR (P < .05 or .001). The correlations (R value) were 0.831 (LVEF, excellent), 0.619 (RVEF, good), 0.672 (LVPER, good), 0.700 (LVPFR, good), 0.463 (RVPER, normal), and 0.253 (RVPFR, poor). There were no significant difference between CMR and CZT-SPECT in LVEF (P > .05) while there were between CMR and both C-SPECT and ultrasound (P < .05). The correlations were all good (R = 0.660, 0.658, and 0.695). There were no significant difference between CMR and both C-SPECT and CZT-SPET in RVEF (P > .05) and the correlations were good (R = 0.771 and 0.745). For repeatability, the intraclass correlation coefficient of RVPFR by C-SPECT was good (intraclass correlation coefficient = 0.698) and excellent for the rest of the groups (0.823-0.989). The repeatability of LVEF and RVEF was better for CZT-SPECT than for C-SPECT. The repeatability of PER was better for both cameras than PFR. CZT-SPECT tomographic ERNV correlated well with C-SPECT planar ERNV in evaluation of biventricular systolic function and LV diastolic function. Compared with the "gold standard" CMR, both models had good correlation in measuring LV/RVEF. CZT-SPECT had better inter-group reproducibility than C-SPECT. The accuracy of RV diastolic function need further study. CZT-SPECT tomographic ERNV will play an important and unique role in the clinical application of accurate evaluation of biventricular function in the future.

Alteration of cardiac structure and function and its prognostic value in patients with Takayasu arteritis: a cardiac magnetic resonance study.

To investigate the prevalence and characteristics of late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) and its prognostic value in patients with Takayasu arteritis (TA). Sixty TA patients with a CMR examination were retrospectively included. All TA patients were divided into with LGE-positive and LGE-negative groups. Bi-ventricular function and location, distribution, and pattern of left ventricular (LV) LGE were evaluated in both LGE-positive and LGE-negative groups. Primary outcome was defined as a composite of cardiovascular death, hospitalization for heart failure, coronary artery revascularization, and stroke. Univariate and multivariate Cox proportional hazard regression analyses were used to evaluate the association between variables and primary outcomes. Sixty consecutive TA patients were enrolled in this study. The mean age was 38.2 ± 13.8 years and 54 patients (54/60, 90.0%) were female. LGE-positive was observed in twenty-one (21/60, 35%) patients in the total patients with TA. LGE was predominantly distributed in the middle wall and subendocardial. The patchy and infarcted LGE patterns were the most common. Compared with the LGE-negative group, the LGE-positive group had reduced LV ejection fraction (P = 0.033), elevated LV end-diastolic volume index (P = 0.008), LV end-systolic volume index (P = 0.012), and LV mass (P = 0.008). During a median follow-up period of 1,892 days (interquartile range: 1,764-1,988 days), the primary outcomes occurred in thirteen patients. In the univariate analysis, LGE-positive (hazard ratio [HR] = 4.478, 95% confidence interval [CI]: 1.376-14.570; P = 0.013) were independently associated with the primary outcomes. However, LGE-positive did not retain its value as an independent predictor of primary outcomes in the multivariate analysis. Instead, LVMI (HR = 1.030, 95%CI: 1.013-1.048; P = 0.001) was the strongest independent predictor of primary outcomes in patients with TA. The Kaplan-Meier plot revealed that patients with LVMI ≥ 57.5 g/m2 have a worse prognosis. LGE-positive detected by CMR was observed in 35% of total TA patients with different distributions and patterns. LGE is associated with adverse LV remodeling and worsen cardiac function. However, LVMI rather than LGE can provide independent prognostic information in patients with TA.

Survival of Children With Critical Congenital Heart Defects in the National Birth Defects Prevention Study.

Critical congenital heart defects (CCHDs) are associated with considerable morbidity and mortality. This study estimated survival of children with nonsyndromic CCHDs and evaluated relationships between exposures of interest and survival by CCHD severity (univentricular or biventricular function). This analysis included 4380 infants with CCHDs (cases) born during 1999-2011 and enrolled in the National Birth Defects Prevention Study, a multisite, population-based case-control study of major birth defects. Cases were linked to state death files. Nonparametric Kaplan-Meier survival functions were used to estimate 1- and 5-year survival probabilities overall and by severity group (univentricular/biventricular) stratified by demographic and clinical exposure variables of interest. The log-rank test was used to determine whether stratified survival curves were equivalent. Survival and 95% confidence intervals (CIs) were also estimated using Cox proportional hazards modeling adjusted for maternal age, education, race/ethnicity, study site, and birth year. One- and five-year survival rates were 85.8% (CI 84.7-86.8) and 83.7% (CI 82.5-84.9), respectively. Univentricular 5-year survival was lower than biventricular case survival [65.3% (CI 61.7-68.5) vs. 89.0% (CI 87.8-90.1; p < 0.001)]. Clinical factors (e.g. preterm birth, low birthweight, and complex/multiple defects) were associated with lower survival in each severity group. Sociodemographic factors (non-Hispanic Black race/ethnicity, <high school education, smoking, and lower household income) were only associated with survival among biventricular cases. Mortality among children with CCHDs occurred primarily in the first year of life. Survival was lower for those with univentricular defects, and social determinants of health were most important in predicting survival for those with biventricular defects.

Left ventricular reverse remodeling after combined ARNI and SGLT2 therapy in heart failure patients with reduced or mildly reduced ejection fraction

BackgroundCardiac remodeling is an adverse phenomenon linked to heart failure (HF) progression. Cardiac remodeling could represent the real therapeutic goal in the treatment of patients with HF and reduced ejection fraction (HFrEF), being potentially reversed through different pharmacotherapies. Currently, there are well-established drugs such as ACEi/ARBs and β-blockers with anti-remodeling effects. More recently, ARNI effects on cardiac remodeling were also demonstrated; additional potential benefits of gliflozins remain non clearly demonstrated. Aim of studyTo evaluate possible changes in cardiac remodeling in patients with HFrEF/HFmrEF in treatment with ARNI or ARNI plus SGLT2i and the potential benefit on cardiac remodeling of adding SGLT2i to ARNI. MethodsBetween June 2021 and August 2023, 100 consecutive patients with HFrEF/HFmrEF underwent conventional and advanced echocardiography (TDI, 2DSTE): patients were therefore divided into three groups according to therapy with neither ARNI nor SGLT2i, just ARNI or both. After 3 months, all patients underwent echocardiographic follow-up. ResultsAfter a 3 months of therapy, significant improvements were observed for LVEF, LVEDD, LVEDV, LVESV, LV mass, E/e’, LV GLS, TAPSE (ANOVA p< 0.01 in all cases), RV S’ velocity (ANOVA p< 0.001).The trend in favor of additional treatment with SGTL2i over ARNI remained statistically significant even after multivariable analysis (p< 0.001 for LVEF, LVEDD; p< 0.01 for LV GLS, TAPSE, TRVS; p< 0.05 for LV mass). ConclusionsSGLT2i therapy when added to the standard treatment for HFrEF and HFmrEF is associated with an improved biventricular function and ventricular dimensions at follow-up.