Cardiovascular Science

Open Access

Case Report

01 April 2025

Behcet’s Disease: An Uncommon Cause of Severe Tricuspid Stenosis

Behçet’s disease is a vasculitic condition of unknown etiology that is characterized by oral and genital ulcers as well as various skin and ocular lesions. Cardiovascular manifestations of Behçet’s disease are rare, with very few cases having been reported previously in literature. We report a case of severe tricuspid stenosis and pulmonary artery aneurysm in a 29-year-old man with Behçet’s disease, who demonstrated characteristic vascular findings on computed tomography angiography and diagnostic valvular findings on transthoracic echocardiogram and cardiac magnetic resonance imaging. The patient’s Behçet’s disease was treated initially with cyclophosphamide, azathioprine, and prednisone, which subsequently led to complete resolution of the pulmonary artery aneurysm. As for the tricuspid stenosis, though symptoms were managed with diuretic therapy, the severity of valvular dysfunction required consideration and an attempt at tricuspid valve replacement surgery, which unfortunately was met with complications and led to an unfavorable outcome of refractory cardiogenic shock and death. Given the rarity of cardiovascular involvement in patients with Behçet’s disease, along with the lack of clear treatment guidelines, management of findings of tricuspid stenosis and pulmonary artery aneurysm in these patients can be challenging.

Neiberg de Alcantara Lima*

Francisco Flavio Costa Filho

Euton Freitas de Castro Júnior

Shivani Reddy

Henrique Carvalho Lima Farias

Gunter Gerson

Ane Karoline Medina Neri

Cardiovasc. Sci.

2025,

2

(2), 10002;

DOI: 10.70322/cvs.2025.10002

Open Access

Review

17 April 2025

Advances in Cardiac Resynchronisation Therapy

Cardiac resynchronisation therapy (CRT) has emerged as a transformative treatment in heart failure management, particularly for patients with significant left ventricular systolic dysfunction in the context of electrical dyssynchrony. Over time, CRT has evolved to address broader patient populations and more complex clinical scenarios. Despite its well-documented benefits in improving survival, reducing hospitalisation and enhancing quality of life, approximately 30% of patients fail to respond, making ongoing research critical for optimising outcomes. This review provides a comprehensive update on the evolving landscape of CRT therapy. Focus is placed on expanding indications, novel assessment techniques for dyssynchrony, application in special populations and innovations in device programming.

Nimai Desai*

Nihit Shah

Zafraan Zathar

Sophie Thompson

Jamie Walton

Harkaran Kalkat

Taaru Narayanan

Peysh A. Patel

Cardiovasc. Sci.

2025,

2

(2), 10003;

DOI: 10.70322/cvs.2025.10003

Open Access

Article

27 April 2025

The Impact of Pacemaker Programming on Morbidity in Heart Transplant Recipients

Pacemaker programming recommendations in patients post-heart transplant include a higher lower rate limit, activating rate response mode, maximising battery longevity and minimising ventricular pacing in patients without atrioventricular block. This study sought to investigate how variability in pacemaker programming following orthotopic heart transplant affects morbidity. We conducted a retrospective analysis of heart transplant recipients at a single transplant centre between 1991 and 2023. Patients requiring pacemaker implantation following transplantation were matched with non-pacemaker recipients by age, sex and height. Patient and device characteristics were reviewed. Clinical outcomes, programming and physiological parameters were compared within the pacemaker group and between subject and comparator groups. Forty-five heart transplant recipients were included: 15 with pacemakers and 30 without. Within the pacemaker group, 20% were programmed with LRL > 60 bpm, rate-response mode in 47% and algorithms minimising ventricular pacing in 27%. Fifty-three percent were NYHA class I, and 46% NYHA class II; resting heart rate was similar between the groups (85 (SD14.9) and 79 (SD8) bpm: p = 0.33). NYHA class I group achieved a higher workload (METS 9 (SD2.7) vs. 6.9 (SD1) mL/kg/min: p = 0.21), and peak heart rate (135 (18.8) vs. 123 (14.8) bpm: p = 0.29) during exercise stress echocardiogram (ESE). The pacemaker group was more symptomatic than the comparator group (NYHA class II 46% vs. 10%: p = 0.016) and exhibited higher rates of cardiac allograft vasculopathy (53% vs. 10%: p = 0.005). There is substantial variability in pacemaker programming in heart transplant recipients. Patients who require a pacemaker have a greater symptom and comorbidity burden than those without. No identifiable physiological or programming differences stratified the greater morbidity within the pacemaker cohort.

Matanyahu Rubinstein

Emilia Nan Tie

Jeremy William

Caitlin Cheshire

James L. Hare

Justin A. Mariani

David M. Kaye

Sarah J. Gutman

Hitesh C. Patel*

Cardiovasc. Sci.

2025,

2

(2), 10004;

DOI: 10.70322/cvs.2025.10004

Open Access

Article

16 May 2025

Application of Principal Component Analysis to Heterogenous Fontan Registry Data Identifies Independent Contributing Factors to Decline

Single ventricle disease is a serious and deadly illness, and advances in clinical management of individuals with Fontan circulation over the past two decades have yet to yield acceptable survival. Patients remain at risk of developing a diverse assortment of Fontan-associated comorbidities that ultimately require a heart transplant. Our goal in this observational cohort study was to determine if application of principal component analysis (PCA) to heterogeneous data collected from a sizable Fontan cohort (n = 140) would predict functional decline. The data, broadly comprised of blood biomarkers, lymphatic biomarkers, measures of cardiac and vascular function, and exercise (VO₂max), were collected at a single site over 11 years; 16 events occurred over that time that we consider here as a single composite outcome measure. The standardized data was transformed via PCA, and principal components (PCs) characterizing >5% of total variance were thematically labeled based on their constituents and tested for association with the composite outcome. We found that the 6th PC (PC6), which represents 7.1% of the total variance, is superior to ejection fraction (EF) as a measure of proportional hazard, is greatly influenced by blood serum biomarkers and superior vena cava flow, and displays the greatest accuracy (according to area under the curve analysis) for classifying Fontan patients. In bivariate hazard analysis, we determined that models combining lymphatic dysfunction (PC6) and systolic function (EF or PC5) were most accurate, with the former having the highest c-statistic, and the latter having the greatest AIC. Our findings support our hypothesis that improved prognostication in a Fontan population should utilize a multifactorial model.

Margaret R. Ferrari

Michal Schäfer

Michael V. Di Maria

Kendall S. Hunter*

Cardiovasc. Sci.

2025,

2

(2), 10005;

DOI: 10.70322/cvs.2025.10005

Open Access

Case Report

09 June 2025

Acute Myocardial Infarction with Multiple Giant Coronary Artery Aneurysms in a Juvenile with Kawasaki Disease

Coronary artery aneurysm (CAA), the most risky late complication of Kawasaki disease (KD), is associated with severe adverse cardiac events, such as acute myocardial infarction (AMI), in young patients. Herein, we describe a 16-year-old boy who suffered from occasional angina attack after a recent myocardial infarction due to multiple giant CAAs during the asymptomatic period of KD. Coronary angiography (CAG) revealed multiple large CAAs of about 9 mm in diameter at the left anterior descending artery (LAD) and more than 12 mm at the left circumflex coronary artery (LCX). To optimize the management and reduce the morbidity and mortality of giant CAAs, it is imperative to consider antecedent KD at the earliest possible stage, particularly in young patients with angina pectoris or AMI but lacking traditional risk factors for atherosclerosis. Long-term follow-up with an electrocardiogram (ECG), echocardiogram, or coronary computed tomography angiography (CCTA) is essential and should not be overlooked. In addition, this case highlights the great significance of working out a more comprehensive and effective management strategy for such KD juveniles, including drugs, percutaneous coronary intervention (PCI) or even surgery.

Congyi Cheng

Lei Qiao*

Wenqiang Chen*

Chungang Zhai*

Cardiovasc. Sci.

2025,

2

(2), 10006;

DOI: 10.70322/cvs.2025.10006