Mathieu N. Suleiman

Background: Primary cardiac tumors (PCTs) are exceptionally rare, accounting for less than 0.7% of all cardiac masses. Among these, cardiac hemangiomas are uncommon benign neoplasms, accounting for less than 2% of all PCTs, with the capillary subtype representing only a small fraction. Clinical presentation is often nonspecific and depends on the size, location, and mobility of the tumor. Syncope as the initial manifestation is exceedingly rare. Case Summary: We report the case of a 53-year-old woman who presented with recurrent episodes of syncope and no preceding cardiac symptoms. Initial neurological investigations were unremarkable. Transthoracic echocardiography revealed a mobile, pedunculated intraventricular mass attached to the mid-ventricular septum near the posteromedial papillary muscle. Cardiac MRI confirmed a well-defined, hypervascular lesion consistent with a benign cardiac tumor, initially suspected to be cardiac myxoma. The patient underwent successful surgical excision via superior partial sternotomy. Histopathological and immunohistochemical analyses confirmed the diagnosis of a capillary hemangioma. The postoperative course was uneventful, and postoperative imaging showed no recurrence. In addition, we provide a systematic review of the literature, identifying 46 studies comprising 49 patients with histopathologically confirmed cardiac capillary hemangiomas or mixed hemangiomas containing a capillary component. The review demonstrated considerable heterogeneity in patient age, clinical presentation, and tumor localization, with the right atrium and left ventricle being the most frequently affected sites. Surgical resection was the predominant treatment approach, while long-term follow-up data remained limited. Discussion: This case highlights the diagnostic challenge of distinguishing cardiac hemangiomas from other benign cardiac masses based solely on imaging. It underscores the importance of considering cardiac causes in patients presenting with unexplained syncope and the role of early echocardiographic evaluation. Definitive diagnosis requires histopathological confirmation, and surgical resection may be beneficial to prevent hemodynamic or embolic complications and to establish tissue diagnosis. Our systematic review also shows that diagnosing these rare tumors remains challenging, as their clinical presentations vary widely and their imaging features often resemble those of other cardiac masses.

Background: Cardiac surgery using cardiopulmonary bypass uses controlled hypoperfusion which leads to relative organ damage. Acute kidney injury is the most frequent and most important organ failure, in particular in patients with chronic kidney disease. To date, there are no approved drug treatments that could effectively prevent acute kidney injury. SP16, an agonist of the low-density lipoprotein receptor-related protein 1, has been shown to exert both kidney- and cardioprotective effects in preclinical trials. Early clinical use of SP16 in phase I trials was safe. Administration of SP16 had beneficial trends on inflammatory response and infarct size in patients with ST-segment elevation myocardial infarction. The primary objective of this phase IIa trial is to demonstrate that injection of SP16 is safe and superior to placebo in preventing cardiac surgery-associated acute kidney injury within 7 days after surgery. Methods: This randomised, double-blinded, placebo-controlled, single-centre study evaluates the efficacy and safety of SP16 in 120 high-risk chronic kidney disease patients with disease stage G2-G3b undergoing cardiac surgery who are randomised into one of two treatment groups in a 1:1 ratio: SP16 (12 mg) or placebo. The study medication is administered via two subcutaneous injections, with the first dose given before surgery, followed by an additional dose after 9 h. Primary endpoints are the incidence of acute kidney injury during 7 days post-surgery and the frequency of adverse events within 72 h after index surgery. Important secondary endpoints include the incidence of major adverse kidney events at day 90 and impact on cardiac function. Safety assessments encompass adverse events, vital signs, electrocardiograms and routine safety laboratory tests. Additional evaluations include pharmacokinetics and immunological biomarkers. Discussion: This single-centre phase IIa trial will assess the incidence of cardiac surgery-associated acute kidney injury, describing the kidney-protective potential of SP16 and its safety profile in patients undergoing cardiac surgery. Trial registration: EUCT, EUCT number 2025-522491-89-00. Registered on February 2, 2026, https://euclinicaltrials.eu/ctis-public/view/2025-522491-89-00 . Patient recruitment is planned to start on August 1, 2026.

Background/Objectives: Progress in diagnostic and therapeutic strategies has resulted in an increasing prevalence of adults with congenital heart disease (ACHD), including those involving genetically determined syndromes. This study aimed to characterize prevalence, congenital phenotypes, heart failure (HF) stages, comorbidity burden, and current medical management of ACHD and concomitant genetically determined syndromes enrolled in a prospective HF-focused registry. Methods: The PATHFINDER-CHD Registry is a German-based (est. 2022) multicenter observational registry. This web-based platform consecutively tracks ACHD patients across the heart failure spectrum, including those with current or prior HF, as well as those at high structural or functional risk. HF stage was classified using a modified ACC/AHA scheme adapted for CHD; functional capacity was graded according to the Perloff classification. Baseline demographics, CHD anatomy, prior surgical/interventional treatment, cardiac and extracardiac comorbidities, and medication were collected from medical records. Results: Among 1987 enrolled ACHD, 107 (5.4%) had a genetic syndrome (n = 65, 60.7% women; mean age 33.5 ± 9.4 years; range 18–68). Most common syndromes were trisomy 21 (n = 49; 45.8%) and 22q11.2 deletion (n = 27; 25.2%); 31 patients (30.0) had rarer syndromes. Predominant CHD diagnoses were atrioventricular septal defect (n = 42, 39.3%), tetralogy of Fallot (n = 19, 17.8%), and pulmonary atresia with ventricular septal defect (n = 7, 6.5%). A systemic left ventricle was present in 102 (95.3%); 40 (37.4%) had primarily cyanotic CHD, and 7 (6.5%) an Eisenmenger physiology. Most patients (n = 71; 66.4%) had undergone definite surgical repair; 25 patients (23.3%) had at least one catheter intervention, including transcatheter valve implantation in 17 cases (15.9%). HF stage was mainly B (n = 30, 28.0%) or C (n = 75, 70.1%). Perloff functional class I/II was present in 97 (90.7%). Leading cardiac comorbidities included intrinsic aortopathy (n = 49, 45.8%), pulmonary arterial hypertension (n = 12, 11.2%), and arrhythmias (n = 10, 9.3%). Frequent extracardiac comorbidities were thyroid dysfunction (n = 34, 31.8%), kidney disease (n = 16, 15.0%), hyperuricemia (n = 13, 12.1%), and depression (n = 15, 14.0%). Pharmacotherapy was used in 66 patients (61.7%). Beta-blockers (n = 25, 23.4%) were common, while ACEi/ARB (n = 9, 8.4%), diuretics (n = 10, 9.3%), MRAs (n = 8, 7.5%), and SGLT2 inhibitors (n = 3; 2.8%) were infrequently prescribed; no patient received ARNI or digitalis. For targeted treatment of pulmonary arterial hypertension, phosphodiesterase-5 inhibitors (n = 7, 6.5%), endothelin receptor antagonists (n = 6, 5.6%), or prostacyclin analogues (n = 1, 0.9%) were used. As oral anticoagulants, vitamin K antagonists or direct oral anticoagulants (DOACs) were prescribed in 17 cases (15.9%). Forty-one patients (38.3%) received thyroid hormone replacement. Conclusions: Syndromic ACHD constitute a small but clinically high-risk subgroup within an HF-oriented registry, marked by complex CHD, substantial cardio–extracardiac multimorbidity (notably aortopathy, PAH, thyroid disease, renal dysfunction, depression), and low utilization of contemporary HF therapies. These data support specialized, interdisciplinary, longitudinal care pathways and prospective studies addressing outcomes and evidence-based HF management in syndromic ACHD.

Background: Advances in diagnosis and treatment have led to a growing population of adults with congenital heart disease (ACHD). Despite increasing life expectancy, their clinical needs—especially in older age—remain poorly defined. Cardiac and non-cardiac comorbidities are prevalent, and emerging evidence suggests accelerated biological aging compared to the general population. However, data on older patients and geriatric patients with CHD are limited. Objectives: This study aimed to characterize patients with CHD aged ≥50 years, focusing on functional status, comorbidities, sex-specific differences, and therapeutic patterns. Methods: The PATHFINDER-CHD Registry is a prospective, observational, multicenter registry enrolling patients with CHD with manifest heart failure (HF), HF history, or high HF risk. Data include anatomy, prior treatments, comorbidities, and medication use. Results: Among 1935 patients, 297 were ≥50 years old. Most had acyanotic CHD (62%); Tetralogy of Fallot (21%) was the most frequent diagnosis. A morphologic right systemic ventricle was present in 12%, and 5% had univentricular hearts. HF was manifest in 21%; 44% were classified as ACC/AHA stage B, 51% as stage C, yet 77% were in Perloff class I/II. Common cardiovascular comorbidities included aortopathy (55%), hypertension (37%), and arrhythmia (33%). Non-cardiac comorbidities included thyroid dysfunction (25%), renal impairment (18%), and neurological disease (13%). Sex-specific differences were observed. Despite HF burden, SGLT2 inhibitors and ARNIs were used in only 17% and 8.4%, respectively. Conclusions: Older patients with CHD represent a clinically complex cohort with high comorbidity burden. The findings support the concept of accelerated aging and emphasize the need for tailored interdisciplinary care strategies.

Background: Constrictive pericarditis (CP) is a rare but significant pericardial disease resulting in impaired ventricular filling and heart failure symptoms, often following cardiac surgery. Its clinical presentation complicates diagnosis, mimicking other causes of heart failure. Recent technological advances, including artificial intelligence (AI)-based non-invasive pulse wave analysis (AI-PWA), have the potential for improved haemodynamic assessment and clinical decision-making. Objectives: This study evaluates the clinical utility of AI-PWA in assessing central aortic blood pressure (CABP), arterial stiffness and cardiac function in CP. Methods: This prospective case-control study enrolled 12 adult CP patients and 12 age- and sex-matched healthy controls. CABP and peripheral blood pressure (PBP) were measured using the VascAssist2. Haemodynamic parameters, including pulse wave velocity (PWV), augmentation index@75 (AIx@75), cardiac index, stroke volume and dP/dtmax, were assessed and compared between groups. Results: CP patients showed significantly lower mean CABP than systolic PBP (101.8±23.4 mm Hg vs 112.3±22.9 mm Hg). PWV showed elevated values (>9 m/s) in nnn (42%) of cases, indicating increased arterial stiffness (8.88±1.94 m/s). AIx@75 was higher in CP patients (22.55±8.36%) compared with controls (16.38±6.53%), reflecting increased wave reflection, increased systemic vascular resistance or enhanced aortic compliance. Cardiac performance was notably impaired in the CP group, with reduced stroke volume (64.8±18.8 mL vs 94.9±25.0 mL, p=0.003) and dP/dtmax (724.9±228.2 mm Hg/s vs 1055.3±203.2 mmHg/s, p=0.0011), indicating impaired ventricular function. The heart failure index was significantly higher in CP patients (31.8±18.3% vs . 6.4±6.5%, p<0.001), indicating substantial functional compromise. Conclusion: AI-PWA provides clinically relevant insights into central haemodynamics and arterial stiffness in CP patients. This non-invasive approach may enhance diagnosis and management of CP and should be considered for integration into routine cardiologic evaluation protocols.

Background: The PATHFINDER-CHD Registry is a prospective, multicenter, non-interventional registry across tertiary care centers in Germany. The aim is to analyze real-world data on adults with congenital heart defects (ACHD) or hereditary connective tissue disorders who have manifest heart failure (HF), a history of HF, or are at significant risk of developing HF. This analysis investigates the prevalence and clinical impact of overweight and obesity in this unique population. Methods: As of 1st February, 2025, a total of 1490 ACHD had been enrolled. The mean age was 39.4 ± 12.4 years, and 47.9% were female. Patients were categorized according to Perloff’s functional class and the Munich Heart Failure Classification for Congenital Heart Disease (MUC-HF-Class). Results: The most common congenital heart disease (CHD) in this cohort was Tetralogy of Fallot, transposition of the great arteries, and congenital aortic valve disease. Marfan syndrome was the most common hereditary connective tissue disease. Of the patients, 46.1% were classified as overweight (32.8%) or obese (13.3%), while 4.8% were underweight. The highest prevalence of overweight (47.1%) was observed among patients who had undergone palliative surgery, whereas untreated patients showed the highest proportion of normal weight (57.2%). Cyanotic patients were predominantly of normal weight. Patients with univentricular circulation exhibited significantly lower rates of overweight and obesity (35%; p = 0.001). Overweight and obesity were statistically significantly associated with arterial hypertension, diabetes mellitus, and sleep apnea (all p < 0.001). High BMI was linked to increased use of HF-specific medications, including SGLT2 inhibitors (p = 0.040), diuretics (p = 0.014), and angiotensin receptor blockers (p = 0.005). Conclusions: The data highlight the clinical relevance of overweight and obesity in ACHD with HF, emphasizing the need for individualized prevention and treatment strategies. The registry serves as a critical foundation for the optimization of long-term care in this population.

Background/Objectives: Heart failure (HF) poses a major challenge in managing adults with congenital heart defects (ACHD). Emerging evidence suggests that HF in ACHD increases the risk of underweight due to heightened metabolic demands, gastrointestinal complications, and psychological factors such as anxiety and depression. Despite its critical implications, few studies have examined this association. This study evaluates the relationship between HF and underweight—defined as a body mass index (BMI) < 18.5—in ACHD. Methods: The Pathfinder-CHD Registry is a prospective, observational, web-based HF registry including ACHD with manifest HF, history of HF, or significant risk for HF. It documents congenital diagnoses, HF type, comorbidities, and treatments. Patients were categorized by BMI into mild (17.00–18.49), moderate (16.00–16.99), and severe (p = 0.002). Underweight correlated with younger age (p < 0.001) and CHD type (p = 0.02). Notably, 42.9% of underweight patients had cyanotic CHD. Conclusions: Underweight is an underrecognized problem in ACHD with HF. Adults with complex CHD or connective tissue disorders are disproportionately affected. Underweight should be seen as an alarm sign requiring personalized, multidisciplinary management, including nutritional support, tailored therapy, and close monitoring to improve outcomes.

Background: Aortic coarctation (CoA) is a congenital anomaly leading to upper-body hypertension and lower-body hypotension. Despite surgical or interventional treatment, arterial hypertension may develop and contribute to morbidity and mortality. Conventional blood pressure (BP) measurement methods lack precision for individual diagnoses and therapeutic decisions. This study evaluated the use of artificial intelligence-based pulse wave analysis (AI-PWA) to assess central aortic blood pressure (CABP) and related parameters in post-treatment CoA patients. Methods: This exploratory, cross-sectional study enrolled 47 adults with CoA, between June 2023 and May 2024. Peripheral BP (PBP) was conventionally measured, and CABP was assessed using the VascAssist2 (inmediQ, Butzbach, Germany). Hypertension was defined by systolic BP≥140 mm Hg and/or diastolic BP≥90 mm Hg for PBP. Using AI-PWA, patients with systolic CABP≥130 mm Hg and/or diastolic BP≥90 mm Hg were classified as hypertensive. Results: The study cohort’s age was 41.5±13.7 years, with all patients having undergone previous aortic surgery or intervention. PBP measurements showed a systolic BP of 135.4±14.4 mm Hg at the upper and 147.8±20.3 mm Hg at the lower extremities. CABP measurements were significantly lower, with a systolic BP of 114.3±15.8 mm Hg (p<0.001). Overall, 32 patients (68.1%) were diagnosed as hypertensive, either by PBP measurement (n=13/27.7%), because of antihypertensive treatment (n=9; 40.4%), or a combination of both. The measurement of PBP was more likely to indicate arterial hypertension than the measurement of CABP (n=12; 25.5% vs n=4; 8.5%). Pulse wave velocity, indicative of aortic stiffness, averaged 9.1 m/s, with higher values in 13 patients (27.7%), including 4 after end–end anastomosis, 2 after graft interposition and 7 after stent placement/angioplasty as the most recent procedure. An increased augmentation index as an indicator of arterial stiffness was observed in nine patients (19.1%). Comparing PBP and CABP in the entire collective, significant differences were found for CABP in relation to the procedure performed, with higher values in patients after prosthesis interposition as their last treatment (p<0.05). Conclusion: AI-PWA provides valuable insights into cardiovascular stress in CoA patients, beyond PBP measurements. The study highlights the need to incorporate CABP measurements into clinical practice to avoid overdiagnosis of hypertension. Further research with larger cohorts is needed to validate these findings and refine management strategies for CoA patients.

Background: Calcified amorphous tumor (CAT) is a rare, mostly incidental tumor-like cardiac lesion of unknown histogenesis. Current imaging modalities do not differentiate between CAT and other masses. As it can be a source for embolization, surgical excision of CAT is mandatory. CAT in patients with Granulomatosis with polyangiitis (GPA) is exceedingly rare. Methods: This systematic literature review was prompted by the case of a CAT in a patient with GPA. The search of all types of studies in two databases (PubMed and Scopus) was conducted through November 2024 to identify the relevant studies. Results: Nine studies were included describing cases of patients being diagnosed with GPA and a cardiac mass. All included patients had a histopathological examination of the either biopsied or surgically resected mass. Only one case reported a CAT. In our case, the patient was diagnosed with GPA through a kidney biopsy, whereas a cardiac mass in the right atrium was diagnosed by echocardiography during evaluation for possible kidney transplantation. One year later a progression was observed, and the mass was resected. The histopathological examination revealed a CAT. The patient could be successfully discharged in a good clinical condition. Conclusions: This systematic literature search and case report highlight the importance of regular echocardiographic examination in patients with GPA. Moreover, surgical excision is crucial for the diagnosis and for further therapy planning, regardless of whether the mass is neoplastic or not.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19–58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning–Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning–Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.