Role of three-dimensional transesophageal echocardiography in cardiac myxomas: an imaging challenge.

Nowadays, the diagnosis of cardiac myxomas (CM), particularly the histological types, remains a challenge. Two-dimensional (2D) transthoracic (TT) and transesophageal (TEE)  echocardiography (ECHO) represent the first steps in the imaging pathway. 3D ECHO,  implemented in imaging practice, appears to be an emerging diagnostic technique that overcomes some of the limitations of 2D ECHO while integrating the information provided by magnetic resonance (MRI). However, its role in the imaging arena is still debatable. Analyzing 17 myxomas in 13 patients, the study uncovers a diverse anatomical spectrum. Classical CM morphology is a minority, with most myxomas being sessile and originating from unexpected locations (right ventricular outflow tract and left atrial appendage). Texture and size variations are also noted. Comparing imaging, 2D TEE outperforms 2D TT in visualizing anatomical features, especially attachment types. 3D TEE confirms 2D TT findings and offers more detailed assessments, identifying peduncles missed in four cases by 2D TEE. Two small recurrent myxomas were exclusively detected by 3D TEE, not by 2D TEE or MRI. Two patients have papillary myxomas, one has an embolism. Another patient with a solid myxoma also suffers an embolism, with a clot found at the apex during surgery. Our study showed that CM has a wide anatomical spectrum beyond the typical features, making the diagnosis challenging. Therefore, a multimodality imaging approach is essential for distinguishing CM from other cardiac masses and differentiating myxoma histological types. These findings stress the importance of incorporating 3D ECHO alongside other imaging techniques for a comprehensive evaluation.

Imaging in transcatheter native mitral valve replacement with Tendyne mitral valve system: echocardiographic pathway for the interventional imager.

The interaction between the implanter team and the imager team is critical to the success of transcatheter native mitral valve replacement (TMVR), a novel interventional procedure in the therapeutic arsenal for mitral regurgitation. This imaging scenario necessitates the addition of a new dedicated professional figure, dubbed "the interventional imager," with specific expertise in structural heart disease procedures. As its clinical application grows, knowledge of the various imaging modalities used in the TMVR procedure is required for the interventional imager and beneficial for the interventional implanter team. The purpose of this review is to describe the key steps of the procedural imaging pathway in TMVR using the Tendyne mitral valve system, with an emphasis on echocardiography. Pre-procedure cardiac multi-modality imaging screening and planning for TMVR can determine patient eligibility based on anatomic features and measurements, provide measurements for appropriate valve sizing, plan/simulate the access site, catheter/sheath trajectory, and pros- thesis positioning/orientation for correct deployment and predict the risks of potential procedural complications and their likelihood of success. Step-by-step echocardiographic TMVR intraoperative guidance includes: apical access assessment; support for catheter/sheath localization, trajectory and positioning, valve positioning and clocking; post deployment: correct clocking; hemodynamic assessment; detection of perivalvular leakage; obstruction of the left ventricular outlet tract; complications. Knowledge of the multimodality imaging pathway is essential for interventional imagers and critical to the procedure's success.

A complex unit for a complex disease: the HCM-Family Unit.

Hypertrophic cardiomyopathy (HCM) is a group of heterogeneous disorders that are most commonly passed on in a heritable manner. It is a relatively rare disease around the globe, but due to increased rates of consanguinity within the Kingdom of Saudi Arabia, we speculate a high incidence of undiagnosed cases. The aim of this paper is to elucidate a systematic approach in dealing with HCM patients and since HCM has variable presentation, we have summarized differentials for diagnosis and how different subtypes and genes can have an impact on the clinical picture, management and prognosis. Moreover, we propose a referral multi-disciplinary team HCM-Family Unit in Saudi Arabia and an integrated role in a network between King Faisal Hospital and Inherited and Rare Cardiovascular Disease Unit-Monaldi Hospital, Italy (among the 24 excellence centers of the European Reference Network (ERN) GUARD-Heart).   Graphical Abstract.

Phenotypic delineation of the retinal arterial macroaneurysms with supravalvular pulmonic stenosis syndrome.

Retinal arterial macroaneurysms with supravalvular pulmonic stenosis (RAMSVPS), also known as Familial Retinal Arterial Macroaneurysms (FRAM) syndrome, is a very rare multisystem disorder. Here, we present a case series comprising ophthalmologic and systemic evaluation of patients homozygous for RAMSVPS syndrome causative IGFBP7 variant. New clinical details on 22 previously published and 8 previously unpublished patients are described. Age at first presentation ranged from 1 to 34 years. The classical feature of macroaneurysms and vascular beading involving the retinal arteries was universal. Follow up extending up to 14 years after initial diagnosis revealed recurrent episodes of bleeding and leakage from macroaneurysms in 55% and 59% of patients, respectively. The majority of patients who underwent echocardiography (18/23) showed evidence of heart involvement, most characteristically pulmonary (valvular or supravalvular) stenosis, often requiring surgical correction (12/18). Four patients died in the course of the study from complications of pulmonary stenosis, cerebral hemorrhage, and cardiac complications. Liver involvement (usually cirrhosis) was observed in eight patients. Cerebral vascular involvement was observed in one patient, and stroke was observed in two. We conclude that RAMSVPS is a recognizable syndrome characterized by a high burden of ocular and systemic morbidity, and risk of premature death. Recommendations are proposed for early detection and management of these complications.

Cavitation phenomenon in mechanical prosthetic valves: Not only microbubbles.

INTRODUCTION: Microbubbles (MBs) or cavitation is high-velocity, echo-bright findings present during the closing or opening of a mechanical valve (MVP). Cavitation bubble growth or gas emboli are less frequently described. We evaluated the hemodynamic parameters involved in the formation of gas emboli and the impact of gas emboli on requests for additional investigations. METHODS AND RESULTS: Transthoracic echocardiographic studies (TTE) of 57 patients (31 males, mean age 46.8 ± 13.8 years) with gas emboli were evaluated after heart valve replacement surgery. The majority (72%, n = 42) had a mitral or combined mitral/aortic MVP, with 28% (n = 16) an aortic MVP. The last TTE with and without gas emboli were considered for the same patient and the no emboli group was the control group (42 patients). The patient's blood pressure (BP) and heart rate (HR) were available for each TTE. Comparing the two TTEs, the systolic and diastolic BP, transmitral and aortic gradients, and left ventricular ejection fraction were similar but the HR (80.9 ± 18.7 vs 72.5 ± 13.9 bpm, P = .02) was significantly higher in the group with gas emboli. A TEE was performed 52 times in 27 patients, due to gas emboli, with one case positive for thrombus/vegetation. For 19 patients, a brain CT was requested. In two patients, the indication for the brain CT was gas emboli but the result was negative. CONCLUSION: Gas emboli are frequently present and associated to an increased HR. They can cause the misdiagnosis of endocarditis or thrombus formation with significant additional requests for diagnostic examinations.

Ventricular tachycardia: beginning and ending fate of a benign invasive cardiac lipoma.

Cardiac lipomas are rare benign heart tumors. Their clinical manifestations primarily depend on their dimension and location. We describe a case of a 14-year-old boy complaining of palpitations. ECG showed non-sustained ventricular tachycardia (NSVT). Echocardiography and cardiac MRI showed a large apical mass suggestive of a cardiac lipoma. The patient underwent open-heart surgery which detected a pericardial mass, partially attached to the myocardium, and crossed by the distal segment of LAD. This critical anatomical relationship prevents its complete resection. The biopsy confirmed mature fat tissue. Postoperatively, Propranolol was started for NSVT episodes. After 8 months, he presented with VT recurrence. A subcutaneous ICD (S-ICD) was implanted. Two months later, he was admitted for an electrical storm with six appropriate shocks. Since then, amiodarone was added. Lipomas are not always benign and their surgery might be very difficult and unhelpful. A multidisciplinary team is crucial for their diagnosis and management.

Imaging Cardiovascular Emergencies: Real World Clinical Cases.

Cardiovascular emergencies represent life-threatening conditions requiring a high index of clinical suspicion. In an emergency scenario, a simple stepwise biomarker/imaging diagnostic algorithm may help prompt diagnosis and timely treatment along with related improved outcomes. This article describes several clinical cases of cardiovascular emergencies, such as coronary stent thrombosis-restenosis, takotsubo syndrome, acute myocarditis, massive pulmonary embolism, type A acute aortic dissection, cardiac tamponade, and endocarditis.

Evaluation of the usefulness of a D dimer test in combination with clinical pretest probability score in the prediction and exclusion of Venous Thromboembolism by medical residents.

INTRODUCTION: Venous thromboembolism (VTE) requires urgent diagnosis and treatment to avoid related complications. Clinical presentations of VTE are nonspecific and require definitive confirmation by imaging techniques. A clinical pretest probability (PTP) score system helps predict VTE and reduces the need for costly imaging studies. d-dimer (DD) assay has been used to screen patients for VTE and has shown to be specific for VTE. The combined use of PTP and DD assay may improve exclusion of VTE and safely avoid imaging studies. MATERIALS AND METHODS: We prospectively used the Wells PTP score and a DD test to evaluate 230 consecutive patients who presented with VTE symptoms. The receiver operating characteristic curve was used to identify a new DD cutoff value, which was applied to VTE diagnosis and compared with the upper limit of locally established reference range for prediction of thrombosis alone and in combination with the clinical PTP score. RESULTS: We evaluated 118 patients with VTE symptoms fulfilling the inclusion criteria, 64 (54.2%) with clinically suspected deep vein thrombosis (DVT) and 54 (45.8%) with symptoms of pulmonary embolism (PE). The PTP was low in 28 (43.8%) and moderate/high in 36 (56.25%) of the suspected DVT patients, and low in 29 (53.7%) and moderate/high in 25 (46.3%) of the suspected PE patients. Eighteen cases were confirmed by imaging studies: 9 DVT and 9 PE. The agreement between confirmed cases and PTP was significant with PE but not DVT. The negative predictive value for both DVT and PE with current DD cutoff value of <250 µg/L DDU was 100%, whereas with the calculated cutoff the NPV was 88%. CONCLUSIONS: We confirm that PTP score is valuable tool for medical residents to improve the detection accuracy of VTE, especially for PE. The DD cutoff value of 250 µg/L FEU is ideal for excluding most cases of low PTP; however, the calculated cutoff was less specific for the exclusion of VTE.

Original Article--Mid-term Clinical and Hemodynamic Outcomes in Middle Age Patients Post Trifecta™ (Abbott) Aortic Valve Replacement: A Single-center Study.

Background: Trifecta™ is an externally wrapped bovine pericardial aortic valve with a favorable hemodynamic performance and acceptable rate of freedom from structural valve degeneration. However, recent reports of early Trifecta valve failure question its durability. Rheumatic valve disease in young population is predominant, there is no data on the Trifecta performance, durability or safety available worldwide over the middle age population, as well as there are no previous similar reports from Middle East region. We report on mid-term clinical and hemodynamic performance of Trifecta valve in middle age population with small aortic annuls, a single center report. Methods: A retrospective study of all patients who underwent Trifecta™ SAVR between June 2014 and December 2019 at a cardiac center in Riyadh, Saudi Arabia. Hemodynamic performance was analyzed by longitudinal Doppler echocardiography. Results: During a 5.5-year period, a total of 24 patients underwent SAVR with Trifecta™ valves at our heart center. Patients had a median age of 47.1 years (IQR = 37) and male:female ratio of 1:1. Patients had a median EuroScoreII of 2 (IQR = 1). The 19 mm valve was the most frequently used (29.2%) followed by the 21 mm and 23 mm (both 25.0%). Concomitant procedure was (54.2%). All patients survived their operations and most (87.0%) had their symptoms relieved. A 30-day mortality rate of 4.2%. Patients were followed up for a median of 58.5 months (IQR = 38), during which 8.3% required re interventions, giving an overall freedom from re-intervention of 91.7%. The re-intervention after valve implantation was recorded in two (8.3%). These re-interventions were performed 3 years post initial surgery and were due to structural valve deterioration (SVD). The mean gradient reduced significantly from 43.6 ± 23.9 mmHg (median = 41, IQR = 21) at baseline to 13.0 ± 7.4 mmHg (median = 11, IQR = 5) one-week post intervention (p < 0.001). The average mean gradients were stable from post-operation to follow-up (13.0 ± 7.4 vs 14.1 ± 6.4 mmHg). Conclusion: Our study confirms excellent mid-term durability, clinical and hemodynamic performance of the Trifecta™ valve in middle age population, despite the fact of non-statically significant trend-up of transvalvular gradient over the follow up period. Further long-term studies with larger sample-size are warranted to confirm these results.

First report of the clinical characteristics and outcomes of cardiac amyloidosis in Saudi Arabia.

AIMS: Cardiac amyloidosis (CA) is a potentially fatal multisystemic disease that remains significantly underdiagnosed, particularly in the Middle East. This study aims to evaluate the prevalence and clinical characteristics of CA in a high-risk population at a tertiary centre in Saudi Arabia. METHODS: This cross-sectional, retrospective, single-centre study was conducted at a tertiary hospital in Riyadh, Saudi Arabia. We reviewed the medical records of heart failure patients seen between August 2018 and July 2022 who exhibited red flags for CA and subsequently underwent CA screening. Red flags that prompted the workup included at least two of the following factors: the presence of unilateral or bilateral carpal tunnel syndrome, a family history of transthyretin amyloid (ATTR) amyloidosis and specific electrocardiographic features (relative/absolute low QRS voltage, pseudoinfarct pattern and atrioventricular/interventricular conduction abnormalities). Echocardiographic red flags included mainly increased wall thickness (≥12 mm), significant diastolic dysfunction, reduced left ventricular (LV) longitudinal function, right ventricular (RV) dysfunction and elevated right atrial (RA)/pulmonary artery (PA) pressure. Cardiac magnetic resonance (CMR) red flags included aspects similar to those in an echocardiogram as well as a subendocardial or transmural late gadolinium enhancement (LGE) pattern. These patients were assessed for CA through technetium-99m pyrophosphate ([99mTc]Tc-PYP) bone scintigraphy, serum and urine protein electrophoresis with immunofixation and a serum-free light chain assay. RESULTS: A total of 177 patients were screened, of which 21.0 (11.9%) patients were diagnosed with transthyretin amyloid CA (ATTR-CA) and 13 (7.3%) patients were diagnosed with light chain CA (AL-CA). Compared with patients with negative/equivocal [99mTc]Tc-PYP scans (grades 0-1), patients with positive [99mTc]Tc-PYP scans (grades 2-3) were older (78.0 vs. 68.0 years, P < 0.001), had higher levels of troponin (P = 0.003) and N-terminal pro-brain natriuretic peptide (NT-proBNP) (P < 0.001), had a higher LV mass index (P < 0.001), displayed a more depressed global longitudinal strain (GLS) (P < 0.001) with a greater prevalence of a relative apical sparing pattern (P < 0.001) and demonstrated a higher incidence of first-degree atrioventricular block (P = 0.008) and low voltage patterns on electrocardiography (P < 0.001). Patients with ATTR-CA and AL-CA were more likely to have a subendocardial or transmural LGE pattern on CMR (P < 0.001) and had a significantly lower overall survival (P < 0.001) when compared with other heart failure aetiologies. CONCLUSIONS: This is the first study to describe the clinical characteristics and outcomes of CA in the Middle East and Saudi Arabia. The prevalence of CA among screened heart failure patients here aligns with major international studies, suggesting significant underdiagnosis in the region. Therefore, larger multicentric studies and regional screening programmes are urgently needed to accurately characterize the epidemiology and outcomes of CA in the Middle East.

Transforming Heart Failure Management: The Power of Strain Imaging, 3D Imaging, and Vortex Analysis in Echocardiography.

Heart failure (HF) remains a critical global health challenge, necessitating advancements in diagnostic and therapeutic strategies. This review explores the evolution of imaging technologies and their impact on HF management, focusing on three-dimensional echocardiography (3DE), myocardial strain imaging, and vortex dynamics imaging. Three-dimensional echocardiography enhances traditional echocardiography by providing more accurate assessments of cardiac structures, while myocardial strain imaging offers the early detection of subclinical myocardial dysfunction, crucial in conditions such as chemotherapy-induced cardiotoxicity and ischemic heart disease. Vortex dynamics imaging, a novel technique, provides insights into intracardiac flow patterns, aiding in the evaluation of left ventricular function, valve diseases, and congenital heart anomalies. The integration of these advanced imaging modalities into clinical practice facilitates personalized treatment strategies, enabling the earlier diagnosis and more precise monitoring of disease progression. The ongoing refinement of these imaging techniques holds promise for improving patient outcomes and advancing the field of precision medicine in HF care.

Outcome of patient undergoing redo mitral valve surgery with incidence rate of mitral valve infective endocarditis.

BACKGROUND: The incidence of infective endocarditis (IE) in patients undergoing redo mitral valve (MV) surgery was evaluated. The outcomes of all the patients and the patients' specific characteristics were recorded. The patients were analyzed to further the research of IE in this population. METHOD: This was a retrospective review of patients admitted for redo MV surgery with a prospective follow-up of electronic medical records at King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia, from 2009 to 2019. Pre/intra/post-operative factors contributing to mortality, morbidity, and freedom of adverse events were analyzed. RESULT: A total of 211 patients underwent redo MV surgery, and 41 patients (19.4%) had IE; and 51% of this subset of patients, 21 individuals, developed IE after the initial MV surgery. MV stenosis was moderate/severe in 50 patients. Furthermore, MV regurgitation was present in 89 patients. Multivariate analysis of the data revealed multiple factors influencing mortality: age, peripheral vascular disease, concomitant procedures, peripheral vascular disease, red blood cell transfusions, preoperative mechanical valves, and active IE. In-hospital Mortality was 10.9%. The one-, five-, and ten-year survival was 88%, 79%, and 69% across all patients. CONCLUSION: Although redo MV surgery has acceptable outcomes; the presence of IE or concomitant procedures is a significant health detriment in these patients. Our study highlights the need for careful patient management and more in-depth research in this area to improve patient outcomes.

A relapsed Pseudomonas stutzeri prosthetic valve endocarditis: a case report and review of the literature.

BACKGROUND: Pseudomonas stutzeri is a nonfluorescent denitrifying bacterium widely distributed in the environment, and it has also been isolated as an opportunistic pathogen from humans. It is a Gram-negative bacterium and a common inhabitant of soil and water. CASE PRESENTATION: We report the case of a 51-year-old arab gentleman who has systemic lupus erythematous complicated by lupus nephritis and underwent renal transplantation twice. He underwent mitral valve replacement and 4 years later was diagnosed with prosthetic valve endocarditis caused by Pseudomonas stutzeri. CONCLUSIONS: Literature review was conducted and revealed that this pathogen may be of a particular medical relevance in immunocompromised patients. Our case proves that early infection and relapse despite optimal antibiotics course are possible outcomes of Pseudomonas stutzeri endocarditis. To the best of our knowledge, this is the second case of fulminant early prosthetic valve endocarditis occurring only 1 month post-cardiac surgery with relapse despite a complete antibiotics course.

Ultrasound Imaging of the Abdominal Aorta: A Comprehensive Review.

Ultrasound is the imaging modality of choice for the initial evaluation of disorders that involve the abdominal aorta (AA). The diagnostic value of ultrasound resides in its ability to allow assessment of the anatomy and structure of the AA using two- dimensional, three-dimensional, and contrast-enhanced imaging. Moreover, ultrasound permits evaluation of the physiologic and hemodynamic consequences of abnormalities through Doppler interrogation of blood flow, thus enabling the identification and quantification of disorders within the AA and beyond its boundaries. The approach to ultrasound imaging of the AA varies, depending on the purpose of the study and whether it is performed in a radiology or vascular laboratory or in an echocardiography laboratory. The aim of this review is to demonstrate the usefulness of ultrasound imaging for the detection and evaluation of disorders that involve the AA, detail the abnormalities that are detected or further assessed, and outline its value for echocardiographers, sonographers, and radiologists.

Spectral Doppler Interrogation of the Pulmonary Veins for the Diagnosis of Cardiac Disorders: A Comprehensive Review.

Data obtained from echocardiographic studies are used on a daily basis to guide clinical decision-making regarding patient management and the need for additional diagnostic investigations. Interrogation of blood flow in the pulmonary veins by spectral, most often pulsed-wave, Doppler is an important component of any comprehensive echocardiographic study. Whereas it is most often used to help assess left-sided filling pressure and quantify the severity of mitral regurgitation, the pulmonary vein Doppler profile provides added diagnostic insights into several disorders that affect heart function and allows assessment of their hemodynamic consequences on the heart. The aim of this review is to summarize current knowledge in the field of PV Doppler interrogation, highlight the physiological and pathological parameters that influence it, and delineate the manifestations of various cardiovascular disorders on the flow profile.