Cardiac amyloidosis: A survey of current awareness, diagnostic modalities, treatment practices, and clinical challenges among cardiologists in selected Middle Eastern countries.

BACKGROUND: Cardiac amyloidosis (CA) is a chronic progressive disease caused by the deposition of amyloid fibrils in cardiac tissues. Diagnosis and management of CA are complicated and have developed over the years. HYPOTHESIS: Middle Eastern countries have significant knowledge disparities in diagnosing, managing, and treating different subtypes of CA. METHODS: An online survey was sent to cardiologists in four countries (Saudi Arabia, Lebanon, Egypt, and Iraq) interested in heart failure and practicing for more than a year. The survey questioned the characteristics of the participants and their institutions. It addressed their knowledge and practices in CA specifically diagnostic modalities, treatment options, and interest in education and knowledge exchange. RESULTS: A total of 85 physicians participated in the survey. There was a variation in the participating cardiologists' knowledge, experience level, and readiness of their institutes to manage patients with ATTR-CM. Most participants believed that a high rate of ATTR-CM misdiagnosis existed. Participants' knowledge of the diagnostic modalities and "red flags" raising suspicion about ATTR-CM varied. Another challenge was the availability of essential diagnostic modalities among various cardiology centers. A knowledge gap was also observed regarding updates in ATTR-CM management. However, there was a high endorsement of the need for more education, physician networking, and knowledge exchange. CONCLUSIONS: This survey highlighted the need for increasing awareness levels among cardiologists in the four selected Middle Eastern countries. Cardiologists are most likely to benefit from additional training and knowledge exchange on the latest management advances of this disease. Thus, measures must be taken to focus on the physician's awareness of ATTR-CM patient journey to achieve a better quality of care and outcome.

Ultrasound Imaging of the Superior Vena Cava: A State-of-the-Art Review.

Greater interest in imaging the superior vena cava (SVC) in recent years has arisen because of increased focus on disorders of the right heart; the growing use of transvenous access lines, dialysis catheters, and device leads; and the emergence of right ventricular mechanical circulatory support systems via the transcatheter approach. As a low-pressure venous conduit in the right upper mediastinum, the SVC is prone to compression by various pathologic processes, to invasion by malignancies originating in nearby structures, and to complications arising from intraluminal device leads and indwelling catheters. Computed tomography and magnetic resonance venography are the modalities of choice for structural imaging of the SVC. Ultrasound allows a reasonable, yet less detailed anatomic assessment of this venous conduit. Spectral and color Doppler imaging by ultrasound are the most valuable noninvasive tools for the interrogation of SVC blood flow, a marker of the filling pattern of the right heart. Analysis of the velocity, duration, and direction of the Doppler waveforms and their phasic response to respiration makes it possible to distinguish normal from abnormal flow patterns and offers diagnostic insights into disorders that affect right heart function. The aims of this review are to demonstrate the added value SVC imaging provides during transthoracic and transesophageal echocardiographic studies, to outline its usefulness for the detection and evaluation of structural abnormalities, and to detail the role of spectral Doppler imaging in aiding the diagnosis of various disorders that affect the right heart.

Does Secondary Antibiotic Prophylaxis Improve Clinical Outcomes in Adult Rheumatic Heart Disease Patients Post-Valve Replacement?

BACKGROUND: Secondary prevention of recurrent rheumatic fever in individuals with rheumatic heart disease (RHD) requires continuous antibiotic prophylaxis. However, the impact of antibiotic prophylaxis on the outcome of patients with severe RHD who underwent heart valve replacement is unknown. The objective of the study was to assess the relationship between the use of antibiotics as secondary prophylaxis in RHD patients who underwent valve replacement and clinical outcomes including mortality, reoperation, and valve-related hospitalization. METHODS: We retrospectively compared outcomes of adult patients who underwent heart valve replacement for RHD at our institution from 1990 through 2014 and who received secondary antibiotic prophylaxis (prophylaxis group) with those who did not receive prophylaxis (nonprophylaxis group) using propensity score matching analysis. RESULTS: A total of 1094 patients (56% females, median age 40 years, range 31-53 years) were included with a median follow-up of 9.6 years (range 2.9-12.6 years). Antibiotic prophylaxis was prescribed in 201 patients (18%). Propensity score matching analysis demonstrated no significant difference in overall survival (95% [92%-98%] vs 97% [95%-99%], respectively; P = .7), valve-related hospitalization-independent survival (72% [range 65%-78%] vs 81% [range 76%-88%]; P = .25), and redo valve surgery-independent survival [76% [range 70%-83%] vs 75% [range 72%-79%]; P = .41) at 10-year follow-up in the nonantibiotic prophylaxis versus the antibiotic prophylaxis group. CONCLUSION: Secondary antibiotic prophylaxis among adult RHD patients following valve replacement is not associated with improved clinical outcomes.

Three-dimensional printing in integrated multi-modality imaging approach for management of prosthetic valves infective endocarditis.

After heart failure, infectious endocarditis is the second leading cause of death in patients with prosthetic valves. Aortic pseudoaneurysms are a serious complication of infective endocarditis in mechanical or bioprosthetic aortic prostheses. Diagnostic and management challenges are posed by aortic pseudoaneurysms. In these cases, a multi-modality imaging approach with a heart team is recommended. We described two cases of aortic pseudoaneurysms that developed as a result of infective endocarditis. The first case involved a TAVI patient who developed infective endocarditis as a result of diabetic foot complications. Because traditional echocardiography and computed tomography failed to show the anatomy of the lesion, we used 3D printing to show the anatomy, extension of the pseudoaneurysm, and proximity to the right coronary artery. The second case involved a patient who underwent Bentall's surgery with an aortic root and mechanical aortic valve and later developed infective endocarditis complicated by pseudoaneurysms. In this case, 3D printing was used for preoperative surgical planning.

Multiparametric Approach for the Assessment of Mechanical Prosthetic Tricuspid Leaflet Function.

BACKGROUND: There is a lack of comprehensive echocardiographic data to allow discrimination of normal versus abnormal mechanical prosthetic tricuspid valve (MPTV) leaflet function. The identification of such parameters is essential to optimize diagnostic and therapeutic measures. METHODS: The authors investigated bileaflet MPTV function by comparing transthoracic echocardiographic data from 21 episodes of leaflet dysfunction due to valve thrombosis in 12 patients with data from 56 individuals with normal MPTV function. All episodes of dysfunction were confirmed by transesophageal echocardiography and/or cine fluoroscopy. Transthoracic echocardiography-derived two-dimensional, color, and spectral Doppler variables, including MPTV peak early diastolic velocity (E velocity), mean gradient, pressure half-time, time-velocity integral (TVI) of the MPTV, ratio of TVIMPTV to TVI of the left ventricular outflow tract (LVOT) and TVI of the right ventricular outflow tract (RVOT), and continuity-derived effective orifice area, were measured in both groups. RESULTS: Most episodes of MPTV dysfunction resulted from simultaneous involvement of both leaflets (57%), with leaflet(s) often immobilized in the open or semiopen position (71%). Transthoracic and transesophageal echocardiography performed similarly in detecting abnormal leaflet motion (90% vs 88%, P = .68), whereas transesophageal echocardiography was better in identifying MPTV thrombosis (31% vs 14%, respectively, P = .01). Color Doppler demonstrated flow propagation abnormalities in 67% of episodes of leaflet dysfunction but not in the control group (P < .0001). Doppler variables associated with MPTV leaflet dysfunction included E velocity > 1.6 m/sec, mean gradient > 5 mm Hg, PHT > 157 msec, TVIMPTV > 42 cm, TVIMPTV/TVILVOT > 2.3, TVIMPTV/TVIRVOT > 3.0, and continuity-derived effective orifice area ≤ 1.1 cm2, with most variables showing high and similar accuracy (area under the curve ≥ 95%). CONCLUSIONS: This study represents the first comprehensive echocardiographic assessment of MPTV leaflet dysfunction that provides parameters and criteria to distinguish normal versus abnormal prosthetic valve function.

Constrictive pericarditis following heart transplantation: Reality or fiction?

Constrictive pericarditis (CP) is a curable cause of diastolic heart failure with prior cardiac surgery being a recognizable etiology. We report a patient who developed CP one year following heart transplantation. Several clinical and imaging related factors may lead to diagnostic delays in similar patients, including the mistaken belief that transplanted hearts are devoid of pericardium and thus do not develop constriction. Post-transplantation pericardial effusion, mediastinitis, and cardiac rejection predispose to future CP. Caretakers should consider this entity in allograft recipients who develop heart failure symptoms of unclear etiology.

Established and candidate transthyretin amyloidosis variants identified in the Saudi population by data mining.

BACKGROUND: Familial transthyretin (TTR) amyloidosis (ATTR) is an autosomal dominant disease with significant phenotypic heterogeneity. Its prevalence in Saudi Arabia has not previously been investigated. An existing exome variant database of Saudi individuals, sequenced to globally investigate rare diseases in the population, was mined for TTR variants and filtered for missense mutations resulting in single amino acid changes. A total of 13,906 Saudi exomes from unrelated individuals were analyzed blindly. RESULTS: Three TTR variants known to be associated with ATTR amyloidosis were identified. Additionally, three novel TTR mutations were identified. Structural analysis of the three novel variants suggests that at least two could be amyloidogenic. The most common variant associated with amyloidosis was p.Val142Ile (allele frequency 0.001). Further investigation of these variants and their translation to clinical practice may help to diagnose, monitor, and manage patients with ATTR amyloidosis. CONCLUSION: Multiple TTR variants potentially associated with systemic ATTR amyloidosis were identified in the Saudi population. Early diagnosis and intervention, facilitated by familial genetic testing of patients with ATTR amyloidosis, may benefit in the management of this disease. Early diagnosis could be enhanced through inclusion of ATTR variants in existing population-based screening programs.

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.

Diagnostic Accuracy of Dobutamine Stress Echocardiography for Detection of Cardiac Allograft Vasculopathy in Orthotopic Heart Transplant Patients.

OBJECTIVE: Cardiac allograft vasculopathy is one of the leading causes of late graft failure and subsequent death in orthotopic heart transplant. Although invasive coronary angiography is the gold standard modality for detection of cardiac allograft vasculopathy, dobutamine stress echocardiography has been recently frequently used as an alternative. Our aim was to evaluate the diagnostic performance of dobutamine stress echocardiography for detection of cardiac allograft vasculopathy in transplant patients. METHODS: A retrospective analysis was conducted using a total of 150 dobutamine stress echocardiographic exams that were performed on 99 patients in our institution, with paired coronary angiogram and no acute rejection, within a median of 538 [interquartile range 371-816] days. Sensitivity and specificity of dobutamine echocardiography to detect allograft vasculopathy was evaluated. Allograft vasculopathy was defined as Grade 1 or higher based on ISHLT criteria. A positive dobutamine stress echo result was defined by new or worsening wall motion abnormality. RESULTS: Median age of the population at transplant was 34 [interquartile range 22-46] years; 76 (77%) patients were male. Allograft vasculopathy was present in 31 (20.6%) out of 150 coronary angiograms. Only 7 (4.6%) of that number were positive on dobutamine stress echocardiography. Sensitivity and specificity for allograft vasculopathy detection was 3% and 94%, respectively. Out of 7 false positive dobutamine stress echocardiograms, two were in patients with myocardial bridging. Two patients with mild acute rejection had both negative dobutamine stress echo. CONCLUSIONS: Overall, positivity of dobutamine stress echocardiography in patients after heart transplant is low. It has high specificity, but very low sensitivity for detection of cardiac allograft vasculopathy. Dobutamine stress echocardiography should only be cautiously used as an alternative to coronary angiography.

The long term results of the Ross procedure: The importance of candidate selection.

The Ross procedure has been considered in children as an optimal surgical procedure due to potential growth of the aortic annulus, lack of anticoagulation requirement, very low morbidity rate and excellent survival. Five-hundred-thirty-six (366 male, mean age 29.4 ± 11.1 years) underwent Ross procedure between 1990 and 2016 and had complete clinical and echocardiographic follow-up. Mean follow-up was 16.3 ± 4.9 years. Patients were divided in 2 groups according to age at surgery. Group 1 consisted of 320 (60%) patients less than 18 years old (223 male, mean age at surgery of 9.5 ± 5.6 years). Group 2 consisted of 216 (40%) patients older than 18 years of age (143 male, mean age at surgery of 26.3 ± 8.2 years). One-hundred-thirty (24%) patients had a redo procedure or surgery. Freedom from all re-operation and or percutaneous reintervention on either the aortic and pulmonary valves was 99% after 1 year, 94% after 5 years, 89% after 10 years, 83% after 15 years and 78% after 20 years. Freedom from redo surgery for AV 99% after 1 year, 94% after 5 years, 90% after 10 years, 81% after 15 years and 80% after 20 years. Freedom from redo surgery for PV was 100% after 1 year, 95% after 5 years, 89% after 10 years, 78% after 15 years and 76% after 20 years. The ideal candidate for Ross operation is a patient with congenital aetiology and an aortic root diameter ≤ 15 mm/m2. A pulmonary fresh preserved homograft seems to perform better on the long term.

Three dimensional transesophageal echocardiography: a missing link in infective endocarditis imaging?

The role of two dimensional (2D) echocardiography (ECHO) for the diagnosis and clinical decision making in infective endocarditis (IE) has been extensively studied and described in the medical literature. Some reports have demonstrated the incremental value of three dimensional (3D) transesophageal (TE) ECHO in the setting of IE. However, a systematic review focusing on the role of 3D imaging is lacking. In this manuscript, we examine the role of 3D TE ECHO in the diagnosis of IE. IE is a challenging disease in which 2D transthoracic (TT) and TE ECHO have complementary roles and are unequivocally the mainstay of diagnostic imaging. Still, 2D imaging has important limitations. Technological advances in 3D imaging allow for the reconstruction of real-time anatomical images of cardiac structure and function. 3D imaging has emerged as a diagnostic technique that overcame some of the limitations of 2D ECHO. Currently, both transthoracic and transesophageal echocardiography transducers are able to generate 3D images. However, 3D TE ECHO provides images of a higher quality in comparison to 3D TT ECHO, and is the best echocardiographic modality able to allow for a detailed anatomical imaging. 3D TE ECHO may represent the key adjunctive echocardiographic technique being able to positively impact on IE-related surgical planning and intervention and to facilitate the interaction between the surgeon and the imaging specialist. Importantly, 3D TE ECHO is not the recommended initial modality of choice for the diagnosis of IE; however, in highly specialized centers, it has become an important complementary technique when advanced surgical planning is required. Furthermore, anatomical imaging has become the link between the different techniques that play a role in IE imaging. In fact, both computed tomography and magnetic resonance allow three dimensional reconstruction. An important future goal should allow for the fusion among various imaging modalities. Our review highlights the role of 3D TE ECHO in IE imaging and emphasize where it offers incremental value.

Sudden death as a first manifestation of left atrium thrombus in rheumatic severe mitral stenosis.

Left atrium and left atrial appendage thrombus is common in patients with mitral stenosis, causing significant morbidity and mortality. We described a case of rheumatic mitral stenosis, a 48-year-old female patient, who had undergone percutaneous transvenous mitral commissurotomy 26 years back. She presented with an episode of palpitations and breathlessness on mild exertion. She found to have rapid atrial fibrillation and heart failure. Her echocardiography showed severe mitral stenosis and large left atrial thrombus intermittently obstructing the mitral valve. While she was prepared for urgent valve replacement she had cardiac arrest and died. The echocardiogram showed the thrombus was stuck and closed the mitral valve orifice. From this events we did a review in our institution about the mitral stenosis patients who found to have left atrium thrombus by routine transthoracic echocardiography in five years period. Looking for factors contributing to thrombus formation and outcome predictor, as to outline management plan. .

Age related structural and functional changes in left ventricular performance in healthy subjects: a 2D echocardiographic study.

Left ventricular (LV) adaptation to aging is currently poorly understood. We aimed to characterize age related changes in LV structure and function by studying a large group of healthy subjects across a wide age range. Prospectively enrolled healthy volunteers (n = 778, 327 females; age 18 to 100 years, mean age 49.8 ± 18.1 years), were divided into 4 age groups: 18 to 34 years (n = 165); 35 to 49 years (n = 242), 50 to 79 years (n = 334) and ≥ 80 years (n = 40). All subjects underwent clinical examination, as well as comprehensive transthoracic echocardiogram [TTE]. Body mass index, systolic blood pressure (BP), and left atrial volume (p < 0.0001) increased with age while diastolic BP (p < 0.0001) decreased over time. LV mass/body surface area (BSA) and relative wall thickness increased with age (p < 0.0001) coincident with worsening parameters of diastolic function (E/A and E/Em, p < 0.0001). The ejection fraction and Sm did not change significantly. Stroke volume, ejection time index, flow rate and stroke work significantly increased with age (p < 0.01). The arterial elastance (Ea), a measure of ventricular afterload, and ventricular elastance (Ees), an index of LV systolic stiffness did not change with age nor did their ratio (Ees/Ea) the latter being an expression of ventricular-arterial coupling. Age, gender and LVM were the main independent variables associated with LV systolic function. In conclusion, LV adaptation to aging in a healthy cohort is characterized by concentric LV remodeling, increased contractility and preserved ventricular-arterial coupling.

Echocardiography in Pulmonary Arterial Hypertension.

PURPOSE OF REVIEW: Pulmonary arterial hypertension (PAH) is a devastating disease which can result in right heart (RH) failure and death. Herein, we discuss the current clinical applications of echocardiography in PAH. RECENT FINDINGS: Advanced echocardiographic techniques (strain, strain rate, 2D-speckle tracking strain, and three-dimensional echocardiography) may reveal in the near future additional important insights into RH structure and function. Although right-heart catheterization is mandatory for a definitive diagnosis, echocardiography (resting and exercise) represents a key noninvasive imaging test on the diagnostic-prognostic-therapeutic PAH algorithm.

Left ventricular assessment in patients with systemic light chain amyloidosis: a 3-dimensional speckle tracking transthoracic echocardiographic study.

Cardiac involvement in systemic light chain (AL) amyloidosis carries a poor prognosis mainly through involvement of the left ventricular (LV) myocardium. Despite its limitations, two-dimensional transthoracic echocardiography (2D-TTE) remains the main tool used for the assessment of LV systolic function in AL patients. We hypothesize that 3D-TTE coupled with speckle tracking imaging allows earlier detection of LV systolic dysfunction than 2D-TTE in AL amyloidosis. We prospectively studied 71 subjects including 58 patients with confirmed AL amyloidosis (mean age 66 ± 10 years, 60% male) and 21 healthy control (mean age 64 ± 7 years, 48% male) from 2011 to 2014 at the University Hospital of Limoges. The AL patients were divided into three groups according to Mayo Clinic (MC) staging and all subjects underwent 2D-TTE and 3D-TTE at the same setting. Using 2D-TTE, there was no significant difference in LV ejection fraction (EF) between the groups [LVEF = 63 ± 7% (control), 59 ± 6% (MC stage I), 60 ± 8% (MC stage II) and 57 ± 14% (MC stage III) (p = 0.24)]. In contrast, 3D-TTE demonstrated significantly worse LV systolic function in stage II and III patients using 3D-LVEF [MC II and III 45 ± 8% and 39 ± 12% vs. control 53 ± 8% (p < 0.0001)], global longitudinal strain (GLS) [MC II and III - 11 ± 4% and - 8 ± 3% vs. control - 15 ± 3% (p < 0.0001)] and global radial strain (GRS) [MC II and III 14 ± 9% and 10 ± 8% vs. control 25 ± 10% (p < 0.0001)]. Furthermore, MC III patients had significantly worse global circumferential strain and area tracking [- 17 ± 6% and - 25 ± 8% vs. - 24 ± 7% and - 36 ± 7% for control (p < 0.0001)]. Additionally, MC I had significantly better 3D GLS, GRS and global strain (- 15 ± 3%, 25 ± 10% and 28 ± 12%) than MC II (- 11 ± 4%, 14 ± 9% and 16 ± 10%) and MC III patients (- 8 ± 3%, 10 ± 8% and 12 ± 8%), respectively. Despite an apparently preserved LVEF by 2D-TTE, AL patients in MC stage II and III demonstrate evidence of LV systolic dysfunction by 3D imaging using LVEF and strain analysis. Worse LV involvement by AL amyloidosis was associated with more impaired 3D-TTE LV systolic parameters.

Comparison of echocardiographic parameters in Fabry cardiomyopathy and light-chain cardiac amyloidosis.

BACKGROUND: Fabry cardiomyopathy (FC) and light-chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness. AIMS: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS). METHODS: A two-center echocardiographic analysis was performed, comprising 118 patients with IVS ≥12 mm (FC and AL 59 patients each) matched by IVS. RESULTS: Fabry cardiomyopathy patients had larger LV end-diastolic diameter (47.7 [44.0-50.9] vs 45.0 [41.5-49.0] mm, P = 0.002), better LV ejection fraction (EF 68.7 [63.4-74.0] vs 63.0 [54.0-70.0]%, P = 0.001) and midwall fractional shortening (midFS 14.8 [13.0-16.1] vs 12.1 [8.9-15.0]%, P = 0.006). LV EF <40% was rare in both (2% vs 7%, P = 0.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, P = 0.004), with higher E/e' ratio (13.6 [10.2-18.8] vs 9.8 [7.5-12.3], P < 0.0001). Average E/e' ratio and midFS were significantly associated with NYHA severity in both groups (P < 0.05 for all). CONCLUSIONS: Matched AL patients had worse LV diastolic function than FC, driven by E/e'. Significant LV systolic dysfunction was rare overall. MidFS and E/e' were associated with heart failure severity in both groups.