Long-Term Outcomes After Septal Reduction Therapies in Obstructive Hypertrophic Cardiomyopathy: Insights From the SHARE Registry.

BACKGROUND: Septal reduction therapy (SRT) provides substantial symptomatic improvement in patients with obstructive hypertrophic cardiomyopathy (HCM). However, long-term disease course after SRT and predictors of adverse outcomes have not been systematically examined. METHODS: Data from 13 high clinical volume HCM centers from the international SHARE (Sarcomeric Human Cardiomyopathy Registry) were analyzed. Patients were followed from the time of SRT until last follow-up or occurrence of heart failure (HF) composite outcome (cardiac transplantation, implantation of a left ventricular assist device, left ventricular ejection fraction <35%, development of New York Heart Association class III or IV symptoms), ventricular arrhythmias composite outcome (sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter defibrillator therapy), or HCM-related death. Cox proportional hazards models were used to identify predictors of outcome. RESULTS: Of the 10 225 patients in SHARE, 1832 (18%; 968 [53%] male) underwent SRT, including 455 (25%) with alcohol septal ablation and 1377 (75%) with septal myectomy. The periprocedural 30-day mortality rate was 0.4% (8 of 1832) and 1499 of 1565 (92%) had a maximal left ventricular outflow tract gradient <50 mm Hg at 1 year. After 6.8 years (range, 3.4-9.8 years; 12 565 person-years) from SRT, 77 (4%) experienced HCM-related death (0.6% per year), 236 (13%) a composite HF outcome (1.9% per year), and 87 (5%) a composite ventricular arrhythmia outcome (0.7% per year). Among adults, older age at SRT was associated with a higher incidence of HCM death (hazard ratio, 1.22 [95 CI, 1.1-1.3]; P<0.01) and the HF composite (hazard ratio, 1.14 [95 CI, 1.1-1.2] per 5-year increase; P<0.01) in a multivariable model. Female patients also had a higher risk of the HF composite after SRT (hazard ratio, 1.4 [95 CI, 1.1-1.8]; P<0.01). De novo atrial fibrillation occurred after SRT in 387 patients (21%). Among pediatric patients followed for a median of 13 years after SRT, 26 of 343 (16%) developed the HF composite outcome, despite 96% being free of recurrent left ventricular outflow tract obstruction. CONCLUSIONS: Successful short- and long-term relief of outflow tract obstruction was observed in experienced multidisciplinary HCM centers. A subset of patients progressed to develop HF, but event-free survival at 10 years was 83% and ventricular arrhythmias were rare. Older age, female sex, and SRT during childhood were associated with a greater risk of developing HF.

Wideband black-blood late gadolinium enhancement imaging for improved myocardial scar assessment in patients with cardiac implantable electronic devices.

PURPOSE: Wideband phase-sensitive inversion recovery (PSIR) late gadolinium enhancement (LGE) enables myocardial scar imaging in implantable cardioverter defibrillators (ICD) patients, mitigating hyperintensity artifacts. To address subendocardial scar visibility challenges, a 2D breath-hold single-shot electrocardiography-triggered black-blood (BB) LGE sequence was integrated with wideband imaging, enhancing scar-blood contrast. METHODS: Wideband BB, with increased bandwidth in the inversion pulse (0.8-3.8 kHz) and T2 preparation refocusing pulses (1.6-5.0 kHz), was compared with conventional and wideband PSIR, and conventional BB, in a phantom and sheep with and without ICD, and in six patients with cardiac devices and known myocardial injury. ICD artifact extent was quantified in the phantom and specific absorption rate (SAR) was reported for each sequence. Image contrast ratios were analyzed in both phantom and animal experiments. Expert radiologists assessed image quality, artifact severity, and scar segments in patients and sheep. Additionally, histology was performed on the sheep's heart. RESULTS: In the phantom, wideband BB reduced ICD artifacts by 62% compared to conventional BB while substantially improving scar-blood contrast, but with a SAR more than 24 times that of wideband PSIR. Similarly, the animal study demonstrated a considerable increase in scar-blood contrast with wideband BB, with superior scar detection compared with wideband PSIR, the latter confirmed by histology. In alignment with the animal study, wideband BB successfully eliminated severe ICD hyperintensity artifacts in all patients, surpassing wideband PSIR in image quality and scar detection. CONCLUSION: Wideband BB may play a crucial role in imaging ICD patients, offering images with reduced ICD artifacts and enhanced scar detection.

Prevention of esophageal lesions during atrial fibrillation catheter ablation using esophageal temperature monitoring: A systematic review and meta-analysis.

INTRODUCTION: The use of esophageal temperature monitoring (ETM) for the prevention of esophageal injury during atrial fibrillation (AF) ablation is often advocated. However, evidence supporting its use is scarce and controversial. We therefore aimed to review the evidence assessing the efficacy of ETM for the prevention of esophageal injury. METHODS: We performed a meta-analysis and systematic review of the available literature from inception to December 31, 2022. All studies comparing the use of ETM, versus no ETM, during radiofrequency (RF) AF ablation and which reported the incidence of endoscopically detected esophageal lesions (EDELs) were included. RESULTS: Eleven studies with a total of 1112 patients undergoing RF AF ablation were identified. Of those patients, 627 were assigned to ETM (56%). The overall incidence of EDELs was 9.8%. The use of ETM during AF ablation was associated with a non significant increase in the incidence of EDELs (12.3% with ETM, vs. 6.6 % without ETM, odds ratio, 1.44, 95%CI, 0.49, 4.22, p = .51, I2 = 72%). The use of ETM was associated with a significant increase in the energy delivered specifically on the posterior wall compared to patients without ETM (mean power difference: 5.13 Watts, 95% CI, 1.52, 8.74, p = .005). CONCLUSIONS: The use of ETM does not reduce the incidence of EDELs during RF AF ablation. The higher energy delivered on the posterior wall is likely attributable to a false sense of safety that may explain the lack of benefit of ETM. Further randomized controlled trials are needed to provide conclusive results.

Cardiac magnetic resonance biomarkers as surrogate endpoints in cardiovascular trials for myocardial diseases.

Cardiac magnetic resonance offers multiple facets in the diagnosis, risk stratification, and management of patients with myocardial diseases. Particularly, its feature to precisely monitor disease activity lends itself to quantify response to novel therapeutics. This review critically appraises the value of cardiac magnetic resonance imaging biomarkers as surrogate endpoints for prospective clinical trials. The primary focus is to comprehensively outline the value of established cardiac magnetic resonance parameters in myocardial diseases. These include heart failure, cardiac amyloidosis, iron overload cardiomyopathy, hypertrophic cardiomyopathy, cardio-oncology, and inflammatory cardiomyopathies like myocarditis and sarcoidosis.

[Cardiac CT in 2024]. / CT cardiaque en 2024.

Coronary Computed Tomography Angiography (CCTA) has now become an established tool in the diagnostic process for patients suspected of coronary artery disease. In light of rapid technological development, CCTA has evolved into an imaging modality providing both anatomical and functional information to guide patient management. In this article, we describe the role of cardiac CT in assessing atherosclerotic plaque, chest pain evaluation, cardiovascular risk stratification, planning and guiding coronary intervention, as well as structural heart diseases.

Le scanner coronarien est actuellement un outil reconnu dans le processus diagnostique des patients chez qui on suspecte une maladie coronarienne. Bénéficiant d'un développement technologique rapide et procurant des informations tant morphologiques que fonctionnelles, le CT cardiaque devient une modalité d'imagerie incontournable pour orienter la prise en charge des patients. Dans cet article, nous décrivons le rôle du CT cardiaque dans l'évaluation de la plaque d'athérosclérose, des douleurs thoraciques, de la stratification du risque cardiovasculaire, de la planification et du guidage de l'intervention coronarienne, ainsi que des maladies cardiaques structurelles.

[Cardiology: what's new in 2023]. / Cardiologie: ce qui a changé en 2023.

The year 2023 has been extremely rich in new publications in the various subfields of cardiology. Furthermore, the European Society of Cardiology (ESC) has issued revised guidelines focused on the management of acute coronary syndrome (ACS) and endocarditis, as well as an update on the recommendations for the management of heart failure and cardiovascular prevention. The most significant updates according to the Cardiology Department of CHUV are summarized in this review article.

L'année 2023 a été extrêmement riche en nouvelles publications dans les différents sous-domaines de la cardiologie. De plus, la Société européenne de cardiologie (ESC) a formulé des directives révisées axées sur le management du syndrome coronarien aigu (SCA) et de l'endocardite ainsi qu'une mise à jour des recommandations sur la prise en charge de l'insuffisance cardiaque et la prévention cardiovasculaire. Les nouveautés les plus importantes selon l'équipe du Service de cardiologie du CHUV sont résumées dans cet article de synthèse.

[Cardiovascular magnetic resonance in clinical practice: who, when and why?] / Résonance magnétique cardiovasculaire en pratique clinique : pour qui, quand et pourquoi ?

Cardiovascular magnetic resonance (CMR) is an imaging modality with growing indications in cardiology. The purpose of this article is to illustrate the current clinical applications of CMR across the spectrum of ischemic heart disease, non-ischemic cardiomyopathies, cardiac arrhythmias and valvular or vascular heart disease. The strengths of CMR lie in its ability to comprehensively image, without the need for ionizing radiation, cardiac and vascular anatomy, function, perfusion, viability and physiology, providing a powerful non-invasive tool for patient diagnosis and prognostication.

La résonance magnétique cardiovasculaire (RMC) est une modalité d'imagerie avec des applications croissantes en cardiologie. Le but de cet article est d'illustrer les applications cliniques actuelles de la RMC dans la cardiopathie ischémique, dans le domaine des cardiomyopathies, les troubles du rythme cardiaque et les maladies valvulaires ou vasculaires. Ses points forts résident dans sa capacité à imager de manière complète, sans besoin de rayonnement ionisant, l'anatomie, la fonction, la perfusion, la viabilité et la physiologie cardiaques et vasculaires, offrant un puissant outil non invasif pour le diagnostic et le pronostic des patients.

[Cardiology: what's new in 2022]. / Cardiologie : ce qui a changé en 2022.

The year of 2022 was marked by many novelties in the fields of interventional cardiology, heart failure, electrophysiology, cardiac imaging, and congenital heart disease. These advances will certainly change our daily practice, on top of improving the diagnosis and treatment of many heart conditions. In addition, the European Society of Cardiology has updated its guidelines on pulmonary hypertension, ventricular arrhythmias and sudden death, cardiovascular assessment of patients undergoing non-cardiac surgery. The members of the Cardiology division of Lausanne University Hospital (CHUV) here present the publications which they considered to be the most important of the past year.

L'année 2022 a été marquée par de nombreuses nouveautés dans les domaines de la cardiologie interventionnelle, de l'insuffisance cardiaque, de l'électrophysiologie, de l'imagerie cardiaque et des cardiopathies congénitales. Ces progrès vont certainement faire évoluer notre pratique quotidienne, en plus d'améliorer le diagnostic et le traitement de nombreuses cardiopathies. Par ailleurs, la Société européenne de cardiologie a mis à jour ses recommandations portant sur l'hypertension pulmonaire, les arythmies ventriculaires et la mort subite ainsi que le bilan cardiologique avant une chirurgie non cardiaque. Les membres du Service de cardiologie du CHUV vous présentent ici les travaux qui leur ont semblé être les plus importants de l'année écoulée.

[An update on some atypical electrocardiographic forms of acute coronary syndrome The "STEMI equivalents"]. / Mise au point sur certaines formes électrocardiographiques atypiques du syndrome coronarien aigu - « Équivalents STEMI ¼.

The diagnosis of acute coronary syndrome with ST segment elevation (STEMI) is based on clinical symptoms suggestive of acute myocardial ischemia and precise ECG criteria. STEMI is due to an acute occlusion or subocclusion of a coronary artery, generating a transmural ischaemia, requiring coronary angiography with urgent coronary revascularization. However, some authors consider the current STEMI ECG criteria to be too restrictive: up to 30 % of patients who do not meet these criteria present with critical coronary artery stenosis or acute coronary occlusion. Atypical electrocardiographic patterns, known as "STEMI equivalents", can be found in some cases and justify a rapid cardiological evaluation. We present a pragmatic review of five STEMI equivalents.

Le diagnostic de syndrome coronarien aigu avec sus-décalage du segment ST (ST-Elevation Myocardial Infarction (STEMI)) est basé sur une clinique évocatrice d'une ischémie myocardique et des critères ECG précis. Le STEMI est le reflet d'une (sub)occlusion aiguë d'une artère coronaire engendrant une ischémie transmurale, nécessitant une coronarographie avec un geste de revascularisation en urgence. Certains auteurs considèrent les critères ECG actuels du STEMI comme étant trop restrictifs : jusqu'à 30 % des patients ne remplissant pas ces critères présenteraient une sténose coronarienne critique ou une occlusion coronarienne aiguë. Des aspects électrocardiographiques atypiques, dits « équivalents STEMI ¼, peuvent être mis en évidence dans certains cas et justifient une prise en charge cardiologique rapide. Nous présentons une revue pragmatique de cinq équivalents STEMI.

[Cardiology]. / Cardiologie.

Significant advances have been made in 2021 in the areas of interventional cardiology, heart failure, cardiac imaging, electrophysiology and congenital heart disease. In addition to improving the screening, diagnosis and management of many heart diseases, these advances will change our daily practice. Moreover, the European Society of Cardiology has updated its guidelines on heart failure, valve disease, cardiac pacing and cardiovascular disease prevention. As in previous years, members of the Cardiology division of Lausanne University Hospital (CHUV) came together to select and present to you the papers that they considered to be the most important of the past year.

De nombreux progrès ont été réalisés en 2021 dans les domaines de la cardiologie interventionnelle, de l'insuffisance cardiaque, de l'imagerie cardiaque, de l'électrophysiologie et des cardiopathies congénitales. En plus d'améliorer le dépistage, le diagnostic et la prise en charge de nombreuses cardiopathies, ces avancées vont faire évoluer notre pratique quotidienne. Par ailleurs, la Société européenne de cardiologie a mis à jour ses recommandations portant sur l'insuffisance cardiaque, les valvulopathies, la stimulation cardiaque et la prévention des maladies cardiovasculaires. Comme les années précédentes, les membres du Service de cardiologie du CHUV se sont réunis pour sélectionner et vous présenter les travaux qui leur ont semblé être les plus importants de l'année écoulée.

Impact of manual thrombectomy on microvascular obstruction in STEMI patients.

OBJECTIVE: To assess the effect of manual thrombectomy (MT) on microvascular obstruction (MVO) using cardiac magnetic resonance (CMR) in ST-segment elevation myocardial infarction (STEMI) patients undergoing primary percutaneous coronary intervention (PCI). METHODS AND RESULTS: Three hundred and eighty-three patients admitted for STEMI and undergoing CMR fulfilled the inclusion criteria and were categorized into two groups (did or did not undergo MT). The two primary endpoints were the occurrence and extent of MVO, analyzed as a categorical variable and as a semicontinuous variable. Among the 383 patients, 49.1% exhibited MVO. Both the incidence of MVO and the median number of segments presenting with MVO were significantly higher in the MT group than in the no-MT group, (59.5 vs. 38.9%, p < .001) and (1.5 [0;4] vs. 0 [0;2], p < .001). Analysis stratified on coronary thrombus grade showed similar results, only in patients with a high thrombus burden (60.7 vs. 43.5%, p = .004, and 2 [0;4] vs. 0 [0;3], p = .001. When adjusting for baseline differences, MT remained a determinant of MVO occurrence and extent (odds ratio, OR 1.802 [95% confidence interval, CI 1.080-3.009], p = .024) and ß = .137, p = .024) in patients with a high thrombus grade. CONCLUSION: In STEMI patients, MT was associated with the occurrence and extent of MVO, on CMR, especially in patients with a high thrombus burden.

Interatrial septum dissection and atrial wall hematoma following transseptal puncture: A systematic review of the literature.

BACKGROUND: Interatrial septum (IAS) dissection due to transseptal puncture (TSP) is a rare, underreported complication of the procedure. Data on the mechanism, diagnosis, and management of this complication are lacking. METHODS: We conducted a systematic review of all reported cases of IAS dissection with or without associated LA hematoma due to TSP, by thoroughly searching MEDLINE and EMBASE through May 2019. RESULTS: After screening of n = 882 studies, eight studies with a total of 19 patients addressed the complication of IAS dissection and/or LA hematoma secondary to TSP. Median age was 63 years with a 1:1 male to female ratio. Ablation of atrial fibrillation was the most frequently reported procedure (84%). Diagnosis was established using fluoroscopy with contrast injection (58%), TEE (32%) or intracardiac echocardiography (5%). The mechanism identified involved puncture of the septum secundum portion of the IAS, leading to transient needle passage into the extracardiac space. In the majority of patients, the hematoma remained localized in the IAS and management was conservative with progressive resolution of the hematoma during follow-up (95%). Two patients (11%) required further intervention by either pericardiocentesis or surgical drainage due to hemodynamic instability. CONCLUSIONS: IAS dissection with or without hematoma after TSP remains an underdiagnosed entity. The main mechanism involves lesion to the septum secundum portion of the IAS, resulting in needle passage into the extracardiac space and local bleeding. Although conservative management may be sufficient in the majority of cases, interventional cardiologists should be familiar with this complication and its diagnosis.

[The year in cardiology : 2018]. / Cardiologie.

This review article provides a broad overview of the novelties in cardiology in 2018. Advances in interventional cardiology and cardiovascular prevention, heart failure, electrophysiology and non-invasive cardiovascular imaging have provided important new insights in the pathophysiology, diagnosis and treatment of ischemic and valvular heart disease, heart failure, rhythm disorders and cardiomyopathies. This article provides an overview of the most relevant articles published in 2018.

L'année 2018 a été riche en nouveautés dans les domaines de la cardiologie interventionnelle, de l'insuffisance cardiaque, de l'électrophysiologie et de l'imagerie cardiaque. Les progrès dans ces domaines respectifs ont fourni de nouveaux outils pour le diagnostic et le traitement des cardiopathies ischémiques et valvulaires, de l'insuffisance cardiaque à fraction d'éjection réduite ou préservée, des troubles du rythme et des cardiomyopathies. Cet article fournit un aperçu des articles les plus pertinents publiés en 2018.

Impact of CD14 Polymorphisms on Anti-Apolipoprotein A-1 IgG-Related Coronary Artery Disease Prediction in the General Population.

OBJECTIVE: We aimed to determine whether autoantibodies against apoA-1 (apolipoprotein A-1; anti-apoA-1 IgG) predict incident coronary artery disease (CAD), defined as adjudicated incident myocardial infarction, angina, percutaneous coronary revascularization, or bypass grafting, in the general population. We further investigated whether this association is modulated by a functional CD14 receptor single nucleotide polymorphism. APPROACH AND RESULTS: In a prospectively studied, population-based cohort of 5220 subjects (mean age 52.6±10.7 years, 47.4% males), followed over a median period of 5.6 years, subjects positive versus negative for anti-apoA-1 IgG presented a total CAD rate of 3.9% versus 2.8% (P=0.077) and a nonfatal CAD rate of 3.6% versus 2.3% (P=0.018), respectively. After multivariate adjustment for established cardiovascular risk factors, the hazard ratios of anti-apoA-1 IgG for total and nonfatal CAD were: hazard ratio=1.36 (95% confidence interval, 0.94-1.97; P=0.105) and hazard ratio=1.53 (95% confidence interval, 1.03-2.26; P=0.034), respectively. In subjects with available genetic data for the C260T rs2569190 single nucleotide polymorphism in the CD14 receptor gene (n=4247), we observed a significant interaction between anti-apoA-1 IgG and rs2569190 allele status with regards to CAD risk, with anti-apoA-1 IgG conferring the highest risk for total and nonfatal CAD in non-TT carriers, whereas being associated with the lowest risk for total and nonfatal CAD in TT homozygotes (P for interaction =0.011 and P for interaction =0.033, respectively). CONCLUSIONS: Anti-apoA-1 IgG are independent predictors of nonfatal incident CAD in the general population. The strength of this association is dependent on a functional polymorphism of the CD14 receptor gene, a finding suggesting a gene-autoantibody interaction for the development of CAD.

[TAVI in 2018 : new indications and open questions]. / TAVI en 2018 : nouvelles indications et questions ouvertes.

The last years, various large randomized studies have demonstrated the benefit of TAVI (transcatheter aortic valve implantation) over medical therapy or over conventional surgical treatment in inoperable or very high risk patients with symptomatic severe aortic stenosis. More recently, new data has also shown that among patients at intermediate risk, TAVI is at least as beneficial as surgical valve replacement. In this article, we will focus on the new recommendations of the European society of cardiology but also on issues that remain open.

Ces dernières années, différentes grandes études randomisées ont démontré le bénéfice du TAVI (transcatheter aortic valve implantation) par rapport au traitement médicamenteux ou au traitement chirurgical conventionnel chez les patients avec sténose aortique sévère symptomatique inopérables ou à très haut risque. Plus récemment, de nouvelles données ont également démontré que parmi les patients à risque intermédiaire, le TAVI est au moins aussi bénéfique que le remplacement valvulaire chirurgical. Dans le présent article, nous allons nous intéresser aux nouvelles recommandations de la Société européenne de cardiologie mais également aux questions qui demeurent ouvertes.

[Approach to chronic mitral regurgitation in 2016]. / L' insuffisance mitrale: mise au point en 2016.

Mitral regurgitation (MR) is the most frequent valvular disease in industrialised countries. MR is classified as primary (mostly degenerative with valve prolapse) or secondary (mainly due to underlying ischemic heart disease resulting in deformation of the valve structure). Surgical repair represents the optimal treatment for severe primary MR, whereas the benefits of surgical correction of secondary MR are controversial. Over the past few years, transcatheter techniques have been developed to treat MR, such as the percutaneous edge-to-edge procedure (MitraClip). These approaches represent a novel therapeutic choice for patients judged inoperable by the "heart team". This review article aims to summarize the principles of MR assessment and discuss current therapeutic options for severe MR, taking into account the latest advances in the field.

Accelerated hypertension following mavacamten introduction in severe obstructive hypertrophic cardiomyopathy with hypertension: a case report.

Background: Mavacamten in Phase 2 and 3 clinical trials was well tolerated, reduced left ventricular outflow tract obstruction (LVOTO), and improved exercise capacity and symptoms. However, due to its recent introduction in the market, there is limited evidence from real-world patients with severe/multiple comorbidities and/or who are exposed to potential treatment interactions. Hypertension is common in patients with hypertrophic cardiomyopathy (HCM), but its impact on the treatment of LVOTO is undefined. Case summary: A 55-year-old man with severely obstructive symptomatic HCM and Grade I arterial hypertension underwent treatment with mavacamten 5 mg. He presented an accelerated hypertension from Day 10 of treatment. On admission, he reported improvement of his dyspnoea [New York Heart Association (NYHA) Class II] and NT-pro BNP decreased to 1646 ng/L. Echocardiography showed a left ventricular ejection fraction of 60% with reduced systolic anterior motion and LVOTO (max 21 mmHg). Causes of secondary hypertension were excluded, and blood pressure (BP) was controlled by eplerenone and amlodipine introduction. Accelerated hypertension was concluded as a final diagnosis, and a potential causal link with the introduction of mavacamten was made. Evolution up to Day 135 proved a stabilization of the BP profile and of the LVOT gradient (max 36 mmHg) as well as improvement in functional capacity (NYHA Class I). Discussion: We hypothesize that rapid relief of excess afterload may induce alterations potentially leading to high BP in patients with impaired peripheral vascular resistances. Patients with severe obstructive HCM and hypertension should be given special attention during mavacamten titration and should self-monitor the BP during this phase.

Cardiac Magnetic Resonance Evaluation of LV Remodeling Post-Myocardial Infarction: Prognosis, Monitoring and Trial Endpoints.

Adverse left ventricular remodeling (ALVR) and subsequent heart failure after myocardial infarction (MI) remain a major cause of patient morbidity and mortality worldwide. Overt inflammation has been identified as the common pathway underlying myocardial fibrosis and development of ALVR post-MI. With its ability to simultaneously provide information about cardiac structure, function, perfusion, and tissue characteristics, cardiac magnetic resonance (CMR) is well poised to inform prognosis and guide early surveillance and therapeutics in high-risk cohorts. Further, established and evolving CMR-derived biomarkers may serve as clinical endpoints in prospective trials evaluating the efficacy of novel anti-inflammatory and antifibrotic therapies. This review provides an overview of post-MI ALVR and illustrates how CMR may help clinical adoption of novel therapies via mechanistic or prognostic imaging markers.

Recurrence of an undifferentiated pleomorphic pulmonary artery sarcoma 8 years after initial presentation: a case report.

Background: Primary cardiac tumors remain exceptionally rare, characterized by a poor prognosis. Among them, sarcomas originating in the pulmonary arteries constitute the most infrequent subgroup within primary cardiac sarcomas. Case summary: This report presents the case of a 76-year-old female experiencing a recurrence of an undifferentiated pleomorphic intracardiac pulmonary artery sarcoma located in the right ventricular outflow tract, manifesting 8 years after initial remission. Successful outcomes were attained through a combination of surgical resection, state-of-the-art radiotherapy, and chemotherapy. This comprehensive approach proved essential for optimizing both survival and quality of life. Discussion: The unexpectedly prolonged recurrence-free survival observed in this case underscores the effectiveness of the comprehensive multimodal treatment approach outlined in the existing literature. This highlights the pivotal role of a multidisciplinary strategy in addressing primary cardiac sarcomas, particularly those arising in the pulmonary arteries.