Severe Tricuspid Valve Regurgitation Research Articles

Clinical and echocardiographic characterization of patients hospitalized for severe tricuspid valve regurgitation: a single tertiary-care center experience with two-year follow-up.

Tricuspid regurgitation (TR) is a common acquired valvular heart disease. Recently new transcatheter treatment options for severe TR have emerged that could change the management of this condition, which warrants better characterization of this specific patient group. The aim of the study was to describe the clinical and echocardiographic characteristics of patients with severe TR and to evaluate their short- and mid-term prognosis. This retrospective, observational single-center study enrolled consecutive patients with severe TR hospitalized between January 2016 and September 2021 in the 1st Chair and Department of Cardiology, Medical University of Warsaw, Poland. The severity of heart failure (HF) was evaluated using New York Heart Association (NYHA) classification. Echocardiographic assessment was performed by experienced sonographer. EuroSCORE II and TRI-SCORE were computed for each patient. 12- and 24-month clinical outcomes were reported. There were 172 patients [93 female (54.1%)] included. The mean age was 76.4 (± 10.5) years. The most common comorbidities included: atrial fibrillation (84.9%), hypertension (68.0%), chronic kidney disease (54.1%), coronary artery disease (45.3%), diabetes mellitus (30.9%). The median EuroSCORE II and TRI-SCORE values were 4.68% and 14.0%, respectively. The median follow-up was 24 months. The overall mortality was 29.7% at 1 year and 47.3% at 2 years. TR grade, TAPSE <17 mm, TAPSE/SPAP <0.26 and right atrial area were significant factors associated with mortality. Patients presenting with severe TR are characterized by a large comorbidity burden and poor prognosis, despite intensive heart failure management.

Carcinoid syndrome with right-sided valve involvement - a case report and review of the literature.

The survival of patients with neuroendocrine tumors has substantially improved with modern treatment options. Although the associated carcinoid syndrome can be diagnosed early and controlled effectively, cardiologists still encounter patients with cardiac manifestations, particularly among individuals with persistently high levels of vasoactive mediators. Treatment options have been limited to surgical valve replacement in fully manifested disease. Since surgery is not always feasible, transcatheter valve implantation is becoming an interesting alternative. A case of a 50-year-old woman with carcinoid syndrome and right-sided valvular heart disease is presented. Moderate pulmonary valve stenosis and severe tricuspid valve regurgitation were diagnosed during the evaluation and treatment of neuroendocrine tumor. The possibility of rare valve involvement and the need for interdisciplinary cooperation in the diagnosis, monitoring and treatment of patients with neuroendocrine tumors producing vasoactive substances must be emphasized. The patient had a typically presenting carcinoid syndrome with a rare cardiac manifestation. Although monitoring and treatment were carried out in accordance with recommendations and appropriate to the clinical condition, rapid progression of the metastatic disease ultimately precluded invasive cardiac intervention.

Unusual origins of cardiac insufficiency: a case of iliac arteriovenous fistula post-spinal disc surgery

In this case report, we present the unique and intriguing case of a 57-year-old man who experienced exertional palpitations and shortness of breath for 5 years. He was diagnosed with idiopathic heart failure three years ago, leading to diuretic treatment. Physical examination revealed notable left lower extremity swelling, severe varicose veins, and cardiac murmurs. Echocardiography showed significant cardiac enlargement and severe functional mitral and tricuspid valve regurgitation. Computed tomography (CT) imaging uncovered a 10 mm left common iliac arteriovenous fistula, causing abnormal early filling of the inferior vena cava (IVC) and marked IVC dilation. Open surgical repair of the arteriovenous fistula resulted in symptom relief and improved cardiac function. This case underscores the importance of considering unusual causes in heart failure patients and highlights the value of early diagnosis and intervention in complex cardiac-vascular interactions.

Incidence and predictors of the novo significant mitral and tricuspid regurgitation in patients with permanent atrial fibrillation

Abstract Introduction Atrial fibrillation (AF) is related to atrial remodeling and annular enlargement, which leads to functional mitral and tricuspid regurgitation. These two entities can derive in heart failure with preserved left ventricular ejection fraction (LVEF), the reason why it is becoming a rising concern in recent years. However, its incidence and predictors have not been well established. Purpose This study aimed to assess the incidence and predictors of significant atrial functional mitral and tricuspid regurgitation in patients with permanent AF. Methods The study population comprised 547 patients from a Spanish health area diagnosed with permanent AF between 2014 and 2019 (CardioCHUVI-AF registry; ClinicalTrials.gov identifier: NCT04364516). Individuals who presented with atrial enlargement at the diagnosis, primary moderate to severe valve disease, previous cardiac surgery (including valve replacement or repair), decreased LVEF, pulmonary hypertension, or right ventricular pacing that could interfere with tricuspid valve closure were excluded. The median age was 78.0 (IQ 70.4-85.6), and men represented 43% of the population (235 patients) compared to women, which represented 57% (312 patients). During a median follow-up of 3.5 years (IQ 2.03-4.95) we assessed the incidence of moderate to severe de novo mitral and tricuspid valve regurgitation and its predictors. Results The development of moderate to severe mitral and tricuspid valve regurgitation was higher after three years of diagnosis. The incidence of mitral regurgitation was 3.8 (3.0-4.9) per 100 persons/year. The incidence of tricuspid regurgitation was 5.5 (4.5-6.8) per 100 persons/year. In addition, multivariate analysis showed an association between the development of mitral regurgitation and older age (HR 1.05 [CI 95% 1.01-1.09]), female sex (HR 1.82 [CI 95% 1.1-3]) and prior admission for HF (HR 2.35 [CI 95% 1.18-4.7]). On the other hand, the development of tricuspid regurgitation did not show an association with age (HR 1.02 [CI 95% 0.97-1.05]), but it did with prior admission for HF (HR 2.11 [CI 95% 1.17-3.79]) and female sex (HR 2.17 [CI 95% 1.43-3.3]). Conclusion Permanent atrial fibrillation was associated with an increased risk of developing atrial functional mitral and tricuspid regurgitation, especially after three years of the diagnosis and in the female sex. Older age was associated with a higher incidence of mitral regurgitation. Prior admission for HF was associated with a higher incidence of tricuspid regurgitation. Based on the preceding, monitoring a specific profile of patients with atrial fibrillation may be especially important in preventing and early treating valve disease that can compromise their prognosis.Incidence of mitral regurgitationIncidence of tricuspid regurgitation

Abstract 15479: High Output Cardiac Failure Associated With Arteriovenous Fistula

Introduction: High output heart failure (HOHF) secondary to arteriovenous fistula (AVF) is known. However, having a high index of suspicion for the latter when encountering diuretic resistance in patients with a remote history of hemodialysis is key. Case A 68-year-old female with a history of kidney transplant in 2012 with no recent dialysis, hypertension, hyperlipidemia, pulmonary hypertension, heart failure with reduced ejection fraction, and atrial fibrillation presented with decompensated heart failure and acute kidney injury (AKI). Physical exam was remarkable for anasarca, and no apparent fistulas were noted. She was started on milrinone and furosemide drips. Clinical decision making Initial transthoracic echocardiogram (TTE) revealed an ejection fraction (EF) of 20%, which declined from 35%, along with new mitral and tricuspid regurgitation. Her hospitalization was complicated by altered mental status, worsening AKI requiring hemodialysis, atrial fibrillation with rapid ventricular response, and resistance to multiple diuretics and after-load reduction combinations. Diuretics and inotropic support were discontinued due to worsening clinical status. Given concerns for valvular disease, a transesophageal echocardiogram was obtained, confirming moderate to severe mitral and tricuspid valve regurgitation, bi-atrial enlargement, and reduced biventricular function. However, the patient was not clinically optimized for Mitra-clip. Further hemodynamics and volume status assessment with RHC revealed a mean PAP 44, PCWP 26, and CO 11 L/min. RHC results raised suspicion for HOHF. Interestingly, the patient was noted to have a right inguinal surgical scar with a palpable thrill, and an arteriovenous graft duplex revealed high volume flow. The patient underwent ligation of the right inguinal fistula. Repeat TTE two weeks post AVF ligation showed EF of 40% and improved functional mitral and tricuspid valve regurgitation. The patient ultimately was taken off hemodialysis and had clinically improved heart failure. GDMT was optimized, and outpatient evaluation for mitral valve intervention was scheduled. Conclusion Longstanding AVF can cause HOHF. Early index of suspicion is warranted for AVF ligation as it results in rapid EF recovery.

T-TEER: description of adevelopment process

Tricuspid valve regurgitation (TR) is becoming increasingly more clinically important. While considered as an accompanying symptom of left heart pathologies in the past, TR is now seen as an independent and clinically significant condition. TR can lead to volume overload of the right ventricle, resulting in dilatation of the tricuspid valve annulus and worsening of the regurgitation. Undetected or untreated severe TR can lead to recurrent cardiac decompensation with hospitalization, reduced quality of life and death. Previous treatment options were limited to cardiac surgery and associated with high complication and mortality rates, especially in isolated TR. Therefore, many patients are considered inoperable so that the new interventional treatment measures nowadays often represent the only treatment option. Interventional treatment options such as the edge-to-edge procedure (T-TEER) with TriClip™ or the PASCAL™ system are very safe interventions that have already shown promising results, including reduction of TR, improvement in heart failure symptoms and the quality of life. The influence on the mortality and the necessity for hospitalization due to heart failure are currently being investigated in several randomized studies. Patient selection and timing of the intervention are crucial. Cardiovascular imaging plays adecisive role in selecting the appropriate method and timing of the intervention. The prognosis depends on factors, such as the severity of TR, right ventricular dysfunction, and pulmonary arterial hypertension. Overall, interventional TR treatment is apromising advancement in treatment from which many patients can benefit in the future.

The Impact of Ejection Fraction on Major Adverse Limb Events after Lower Extremity Revascularization

Peripheral arterial disease (PAD) is commonly associated with coronary artery disease, and echocardiography is frequently performed before lower extremity revascularization (LER). However, the incidence of various echocardiographic findings in patients with PAD and their impact on the outcomes of LER has not been well studied. Reduced ejection fraction (EF)≤40% is associated with increased major adverse limb events (MALE) after LER. The electronic medical records of patients undergoing LER in a single center were reviewed. Patients were divided based on the presence or absence of reduced EF. Patient, transthoracic echocardiogram, procedural characteristics, and outcomes were compared between the 2 groups. A total of 1,114 patients (N=131, 11.8% with reduced EF) underwent LER between 2013 and 2019. Patients with reduced EF were more likely to be male and have a history of coronary artery disease and heart failure. Furthermore, they were more likely to have diastolic dysfunction with moderate to severe mitral and tricuspid valve regurgitation. Patients with reduced EF were more likely to undergo LER for chronic limb-threatening ischemia, and to be treated with endovascular procedures. Perioperatively, patients with reduced EF were more likely to develop myocardial infarction. Lastly, the 2 groups had no difference in overall MALE or major amputation. However, on Kaplan-Meier curves, MALE-free survival was significantly lower for patients with reduced EF. Regression analysis demonstrated that indication and not EF was associated with MALE and MALE-free survival. Reduced EF is associated with decreased MALE-free survival for patients with PAD undergoing LER.

Management of severe tricuspid valve regurgitation due to ruptured papillary muscle in a patient with mediastinitis early after heart transplant.

Even though severe tricuspid regurgitation is not uncommon after cardiac transplantation, primary severe tricuspid regurgitation is rare. We present such a case with additional complexities. The patient was 44-year-old man with a HeartWare durable left ventricular assist device (Heartware Inc) who received a temporary right ventricular assist device (RVAD) with a ProtekDuo cannula (LivaNova Inc USA) for refractory ventricular fibrillation and underwent a heart transplant as United Network for Organ Sharing Status 1, in the presence of partially compensated cardiogenic shock, renal failure. Given complex re-operative surgery in a volume-overloaded patient with unknown pulmonary vascular resistance, an RVAD cannula was preserved and re- inserted during cardiac transplant. Postoperatively he required hemodialysis, had severe primary tricuspid regurgitation discovered after RVAD removal and developed Enterobacter mediastinitis. He underwent complex tricuspid valve repair for flail tricuspid leaflet due to ruptured papillary muscle likely due to RVAD cannula injury, after multiple mediastinal washouts and was followed by delayed chest reconstruction. The patient is doing well, 6 months after discharge to home, asymptomatic, without re-admissions, on renal recovery path, with no tricuspid regurgitation and good biventricular function. Replacing the tricuspid valve in presence of hemodialysis catheter, immunosuppression and mediastinitis could be high risk for endocarditis. Even though we have short-term follow-up, tricuspid valve repair can be an effective way of managing primary severe regurgitation especially when there is a desire or need to avoid valve replacement.

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction

BackgroundHybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) is an initial management strategy for infants with critical left heart obstruction and serves as palliation until subsequent operations are pursued. ObjectivesThis study sought to determine patient characteristics and factors associated with subsequent outcomes for infants who underwent hybrid palliation. MethodsFrom 2005 to 2019, 214 of 1,236 prospectively enrolled infants within the Congenital Heart Surgeons’ Society’s critical left heart obstruction cohort underwent hybrid palliation across 24 institutions. Multivariable hazard modeling with competing risk methodology was performed to determine risk and factors associated with outcomes of biventricular repair, Fontan procedure, transplantation, or death. ResultsPreoperative comorbidities (eg, prematurity, low birth weight, genetic syndrome) were identified in 70% of infants (150 of 214). Median follow-up was 7 years, ranging up to 17 years. Overall 12-year survival was 55%. At 5 years after hybrid palliation, 9% had biventricular repair, 36% had Fontan procedure, 12% had transplantation, 35% died without surgical endpoints, and 8% were alive without an endpoint. Factors associated with transplantation were absence of ductal stent, older age, absent interatrial communication, smaller aortic root size, larger tricuspid valve area z-score, and larger left ventricular volume. Factors associated with death were low birth weight, concomitant genetic syndrome, cardiopulmonary bypass use during hybrid palliation, moderate to severe tricuspid valve regurgitation, and smaller ascending aortic size. ConclusionsMortality remains high after hybrid palliation for infants with critical left heart obstruction. Nonetheless, hybrid palliation may facilitate biventricular repair for some infants and for others may serve as stabilization for intended functional univentricular palliation or primary transplantation.

The efficacy and safety of adaptive servo-ventilation therapy for heart failure with preserved ejection fraction.

It is unclear whether adaptive servo-ventilation (ASV) therapy for heart failure with preserved ejection fraction (HFpEF) is effective. The aim of this study was to investigate the details of ASV use, and to evaluate the effectiveness and safety of ASV in real-world HFpEF patients. We retrospectively enrolled 36 HFpEF patients at nine cardiovascular centers who initiated ASV therapy during hospitalization or on outpatient basis and were able to continue using it at home from 2012 to 2017 and survived for at least one year thereafter. The number of hospitalizations for heart failure (HF) during the 12months before and 12months after introduction of ASV at home was compared. The median number of HF hospitalizations for each patient was significantly reduced from 1 [interquartile range: 1-2] in the 12months before introduction of ASV to 0 [0-0] in the 12months after introduction of ASV (p < 0.001). In subgroup analysis, reduction in heart failure hospitalization was significantly greater in female patients, patients with a body mass index < 25, and those with moderate or severe tricuspid valve regurgitation. In patients with HFpEF, the number of HF hospitalizations was significantly decreased after the introduction of ASV. HFpEF patients with female sex, BMI < 25, or moderate to severe tricuspid valve regurgitation are potential candidates who might benefit from ASV therapy.

Transcatheter closure of atrial septal defect in the elderly: a systematic review and meta-analysis

ObjectiveDespite the establishment of transcatheter closure as the treatment of choice in adults with secundum atrial septal defects (ASDs), the effectiveness of this approach in the elderly is disputed. This...

Prognostic Factors for Prolonged In-Hospital Stay in Patients with Heart Failure.

Background and Objectives: Heart failure (HF) is a threatening health condition that is associated with an increasing prevalence and high expenses because of frequent patient hospitalizations. The purpose of this study was to evaluate the factors that influence the length of in-hospital stay in HF patients. Materials and Methods: A total of 220 patients (43.2% men), admitted to the Department of Cardiology, Kaunas Hospital of Lithuanian University of Health Sciences from the 1st of January 2021 to the 31st of May 2021, were included in this study. According to the length of in-hospital stay, patients were stratified into two groups: the first group's length of stay (LOS) was from 1 to 8 days, and the second group's LOS was 9 days or more. Results: The median LOS was 8 (6-10) days. Multivariate logistic regression analysis revealed five predictors as independent factors associated with prolonged hospitalization. These predictors included treatment interruption (OR 3.694; 95% CI 1.080-12.630, p = 0.037), higher value of NT-proBNP (OR 3.352; 95% CI 1.468-7.659, p = 0.004), estimated glomerular filtration rate (eGFR) ≤ 50 mL/min/1.73 m2 (OR 2.423; 95% CI 1.090-5.383, p = 0.030), systolic blood pressure (BP) ≤ 135 mmHg (OR 3.100; 95% CI 1.421-6.761, p = 0.004) and severe tricuspid valve regurgitation (OR 2.473; 95% CI 1.086-5.632, p = 0.031). Conclusions: Several variables were identified as significant clinical predictors for prolonged length of in-hospital stay in HF patients where treatment interruption, higher NT-proBNP value and lower systolic BP at admission were the most important.

Advances in Imaging for Tricuspid Transcatheter Edge-to-Edge Repair: Lessons Learned and Future Perspectives.

Severe tricuspid valve (TV) regurgitation (TR) has been associated with adverse long-term outcomes in several natural history studies, but isolated TV surgery presents high mortality and morbidity rates. Transcatheter tricuspid valve interventions (TTVI) therefore represent a promising field and may currently be considered in patients with severe secondary TR that have a prohibitive surgical risk. Tricuspid transcatheter edge-to-edge repair (T-TEER) represents one of the most frequently used TTVI options. Accurate imaging of the tricuspid valve (TV) apparatus is crucial for T-TEER preprocedural planning, in order to select the right candidates, and is also fundamental for intraprocedural guidance and post-procedural follow-up. Although transesophageal echocardiography represents the main imaging modality, we describe the utility and additional value of other imaging modalities such as cardiac CT and MRI, intracardiac echocardiography, fluoroscopy, and fusion imaging to assist T-TEER. Developments in the field of 3D printing, computational models, and artificial intelligence hold great promise in improving the assessment and management of patients with valvular heart disease.

Comparative study of tricuspid valve repair using ring vs. synthetic band in severe functional tricuspid valve regurgitation

Background: Functional tricuspid valve regurgitation secondary to left-sided valve disease remains a common problem. There are different surgical techniques for tricuspid valve repair; however, the superiority of one approach over the other has not been proven. Our objective was to compare the short-term results of ring versus synthetic band annuloplasty to repair functional severe tricuspid regurgitation in patients with left-sided valve lesions.&#x0D; Methods: This retrospective study includes 60 patients who underwent left-sided valve replacement with concomitant tricuspid valve repair for severe tricuspid regurgitation. Patients were divided into group A (n= 30), patients with rigid rings, and group B (n= 30), patients with synthetic bands.&#x0D; Results: The preoperative demographic and clinical data were non-significant between both groups. In the preoperative data, the tricuspid annular plane systolic excursion (TAPSE) was significantly higher in the ring group (2.84 ± 0.53 vs. 2.3 ± 0.4, P&lt; 0.001). Hospital stay was more prolonged in group B (10.05 ± 1.57 vs. 11.7 ± 2.76 days, P=0.006). There were no differences in other operative and postoperative data between groups. After a six-month follow-up, both groups had no significant difference regarding the clinical data or the degree of tricuspid valve regurgitation.&#x0D; Conclusion: Tricuspid valve annuloplasty with a rigid ring or synthetic band for tricuspid regurgitation could have a good short-term outcome.

Echocardiography Parameters in Behcet's Disease, A Comparative Study.

Behçet's disease (BD) is a chronic inflammatory disease with multiple organ involvements. Although cardiac involvement is not common, it can increase patient morbidity and mortality and decrease life quality. In the present study, echocardiographic abnormalities in BD with no cardiac symptoms were investigated. This cross-sectional descriptive-analytic study was performed on patients referred to Imam Reza Hospital in Mashhad from 2015 to 2018. The participants were divided into control and BD groups. Patients with BD were diagnosed based on ISG criteria. All participants underwent transthoracic echocardiography. Echocardiographic parameters were compared between BD and control groups. In this study, the severity of aortic (AR), mitral (MR), and tricuspid valve regurgitation (TR) was higher in BD than in the control group, while only TR severity showed a statistically significant difference (p-value < 0.001). Systolic Pulmonary Artery Pressure (sPAP) was significantly higher in BD compared to the control group (24.6 mmHg versus 22.81 mmHg, respectively) (pvalue = 0.019). It seems echocardiography is valuable in evaluating a cardiac function, even in asymptomatic patients. Cases with moderate mitral and aortic regurgitation were only observed in BD. Long-term follow-up is suggested, and further studies are required.

SUCCESSFUL MYOCARDIAL REVASCULARIZATION IN A 92-YEAR-OLD FEMALE PATIENT: A CASE REPORT

Introduction. Patients with acute myocardial infarction should be treated with early revascularization. Patients over 73 years have a higher risk of infarct-related cardiogenic shock, which is a leading cause of lethality. Unfortunately, little is known about myocardial revascularization care for elderly people in Ukraine. We presented a 92-year-old lady with an acute chest pain case report, which was not revealed by analgesics.&#x0D; Important clinical findings. Coronary angiography revealed thrombotic occlusion of the distal segment of the left anterior descending artery (LAD) and floated thrombus in LAD mid-segment; stenosis of the left circumflex artery (LCx) close to the first obtuse marginal artery (OM1).&#x0D; The main diagnosis. Acute myocardial infarction with ST-segment elevation of the left ventricle anterior wall (anterior-STEMI). Calcific aortic valve disease, severe aortic valve stenosis, significant mitral and tricuspid valve regurgitation, pulmonary hypertension. Heart failure with reduced LVEF (&lt;40%), NYHA class III symptoms.&#x0D; Intervention. The decision was to perform urgent stenting in LAD and balloon angioplasty in LCx. Successful hospital discharge was after 14 days.&#x0D; Conclusions. Take-away lessons: Elderly patients with acute myocardial infarction can be successfully treated in Ukraine. Age and comorbidities are not a contraindication for early myocardial revascularization.

Transcatheter heterotopic valve implantation with the TricValve system: focus on preoperative assessment and patient selection.

Nontreated severe symptomatic tricuspid valve regurgitation is associated with significant morbidity and mortality. When a surgical correction is not feasible due to high operative risk, transcatheter treatment of the tricuspid valve has been suggested as an interesting valuable option. Caval valve implantation has been proposed to overcome the tricuspid valve regurgitation mechanism reducing the effect of insufficiency on the splanchnic systems. Given the technical simplicity of the caval valve implantation technique when compared with other transcatheter devices, it is a suitable option for a wide variety of patients. Eligibility criteria and patient selection are still matter of debate. We hereby describe the case of a patient successfully treated with bicaval implants with regard to preoperative assessment.

A Rare Case of Cor Triatriatum Dexter With Severe Rheumatic Mitral Stenosis With Severe Organic Tricuspid Valve Regurgitation Masquerading as Acute Pulmonary Embolism in a Young

A Rare Case of Cor Triatriatum Dexter With Severe Rheumatic Mitral Stenosis With Severe Organic Tricuspid Valve Regurgitation Masquerading as Acute Pulmonary Embolism in a Young

322 TRICUSPID VALVE REGURGITATION AFTER TRANSVENOUS LEAD EXTRACTION: WE NEED TO KNOW MORE

Abstract Since 2003 a 73-year-old man was submitted to a pacemaker implantation because of atrio-ventricular block. After fourteen years, upgrading to CRT-D was made due to recovery for acute heart failure in a dilated cardiomyopathy with severe left ventricular systolic dysfunction. In 2019 and 2020 he presented pocket erosion therefore underwent to a submuscular reimplantation. After one year, he came to Arrhythmology outpatient appointment with inflammatory signs of the CRT-D pocket. The patient was started on Meropenem and Vancomicin. Transthoracic (TTE) and transesophageal echocardiogram didn't reveal any vegetation on valvular apparatus or on leads. The patient was submitted to transvenous lead extraction (TLE) with bidirectional rotational mechanical sheats and a epicardial lead on postero-lateral left ventricular wall was implanted. The post-procedural TTE showed a moderate to severe organic tricuspid valve regurgitation with annular dilatation despite the patient was asymptomatic. There weren't flail leaflets or tricuspid valve damage. The reimplantation of right atrial and ventricular leads was hindered by complex venous anatomy documented by intraprocedural phlebography. After Heart Team discussion, we agreed to tricuspid valve repair with annuloplasty ring and other two epicardial leads - right atrial and ventricular - was implanted. We achieve a successful result without complications. The patients was discharged without symptoms; the device interrogation showed good assessment of lead impedances, sensing thresholds, and capture thresholds. Conclusion The mechanisms of tricuspid valve regurgitation following TLE are complex. There isn't a standardized best practice to approach this phenomenon. It's important after TLE to assess tricuspid valve structure and function with TTE in order to understand the incidence of underlying potential mechanism and how to prevent it. Complex venous anatomy could direct the choice toward epicardial leads implantation.

169: SITOSTEROLEMIA PRESENTING WITH SEVERE HYPERTENSION AND CONGESTIVE HEART FAILURE IN A CHILD

Introduction: Sitosterolemia is an autosomal recessive condition caused by pathogenic variants in the ABCG5/ABCG8 genes that prohibit the body from excreting plant sterols, causing build-up in the soft tissues and systemic vasculature. Pediatric vasculopathy secondary to sitosterolemia is poorly described, and there is no clear guidance on the management of complications, including cardiac dysfunction. This case report describes the diagnosis and acute management of a child with severe systemic hypertension, multiple levels of arterial stenosis, and cardiomyopathy presenting in congestive heart failure and ultimately diagnosed with sitosterolemia. Description: An 8-year-old Amish male with no past medical history presented to the emergency department with emesis and associated fatigue and abdominal pain. Vital signs were significant for severe systemic hypertension (BP 220/110), a gallop rhythm, hepatomegaly, cardiomegaly, and mid-aortic narrowing on CT angiogram. Exam was also notable for bilateral knee xanthomas. Laboratory data after admission to the pediatric cardiac ICU showed elevated BNP (peak 4418 pg/mL) and total cholesterol (317 mg/dL) levels. Echocardiogram showed severe mitral and tricuspid valve regurgitation, severely dilated and concentrically hypertrophied and dysfunctional left ventricle, ejection fraction of 30%, and pulmonary hypertension. Consultations included genetics, cardiology, nephrology, and vascular surgery. Initial management included a continuous Milrinone infusion and loop diuretics with improved BP and congestive heart failure symptoms. Cardiac catheterization including coronary and renal angiograms demonstrated severe proximal right coronary artery stenosis and bilateral renal artery stenosis. Right coronary artery and left renal artery angioplasties were performed with good results, and showed significant improvement in parameters of ventricular function and pulmonary arterial hypertension. Genetic testing and positive serum plant sterols confirmed the diagnosis of sitosterolemia. Discussion: Sitosterolemia is a rare genetic disorder and a rare cause of pediatric arteriopathy. Early recognition can prevent the development of life-threatening systemic hypertension, arterial stenosis including coronary artery disease, and cardiomyopathy.