Definition, prevalence, and clinical significance of mitral annular disjunction in different patient cohorts: A systematic review.

BACKGROUND: Mitral annular disjunction (MAD) is a structural abnormality involving a distinct separation of the left atrium/mitral valve annulus and myocardium continuum. The literature around MAD has increased over recent years, thus we sought to review the current data on the definition, prevalence, and clinical outcomes of MAD. METHODS: A search of MEDLINE and EMBASE was conducted to identify studies which evaluated MAD in any patient cohort. The study results were synthesized narratively. RESULTS: A total of 12 studies were included with 3925 patients (average age 62 years, 63% male). The pooled prevalence of MAD in patients with mitral valve prolapse and/or Barlow's disease was 30.1%. In a general population, MAD prevalence was 8.7%. The definition of MAD was not consistent across all studies. In terms of clinical outcomes, only one study reported MAD to be associated with ventricular arrhythmias. CONCLUSIONS: MAD is an increasingly recognized finding amongst patients undergoing cardiac imaging. This review highlights the need for agreed definitions for clinically significant MAD and how identified MAD should be managed. At present, there is insufficient evidence that MAD is associated adverse clinical outcomes.

Complementary role of cardiac CT in the diagnosis and management of mechanical aortic valve thrombosis with a novel low-dose, ultraslow thrombolysis treatment strategy.

A 31-year-old man presented to our emergency department with a 3-day history of progressive breathlessness, fatigue and exertional angina. His history included a mechanical aortic valve replacement (mAVR) for rheumatic heart disease at age 19 years. He could no longer afford medication prescription costs and consequently had not taken oral anticoagulation for 2 months. Transthoracic echocardiography (TTE) demonstrated mechanical prosthetic valve obstruction (PVO) and severe left ventricular (LV) systolic dysfunction; however, valve visualisation was limited by mAVR-related artefact. The patient declined transoesophageal echocardiography. Valve haemodynamics failed to improve despite a prolonged course of parenteral anticoagulation. Multidetector cardiac CT scan was performed which confirmed prosthetic valve thrombosis. A novel low-dose, ultraslow thrombolysis regimen was administered to mitigate the associated bleeding and embolic stroke risk. The patient made an excellent recovery and was discharged on day 30, with repeat cardiac CT scan showing complete resolution of mechanical PVO and normalisation of valve and LV function on TTE.

MINOCA-induced apical ballooning case report: a diagnostic conundrum.

BACKGROUND: Myocardial infarction with non-obstructive coronary arteries (MINOCA) is a recently described phenomenon where no flow-limiting lesions are noted on coronary angiography in a patient with electrocardiogram changes, elevated cardiac biomarkers, and symptoms suggesting acute myocardial infarction. Patients with MINOCA can also potentially develop structural cardiac defects through ischaemic injury. Therefore, the absence of a flow-limiting lesion on angiography coupled with structural defects (e.g. apical ballooning) can very easily result in a diagnosis of Takotsubo cardiomyopathy (TTC). This can lead to potentially serious consequences since treatment options between TTC and MINOCA are different. CASE SUMMARY: We report a case of a patient presenting with features suggestive of TTC but where the final diagnosis was of a MINOCA that induced an apical ventricular septal defect (VSD). Reaching the correct diagnosis proved challenging given that there is no gold standard diagnostic modality for diagnosing MINOCA. CONCLUSION: Imaging adjuncts played a vital role in both diagnosing the underlying MINOCA as well as revealing and planning closure of the resultant VSD. Cardiovascular magnetic resonance imaging played an instrumental role in establishing the patient's primary pathology and in planning a remediation of the structural defect. Structural myocardial defects in a patient with a diagnosis of TTC should prompt clinicians to further investigate whether there is an underlying infarct aetiology (MINOCA).

Cardiomyopathy in the peripartum period due to left ventricular non-compaction and association with Ebstein's anomaly: a case report.

BACKGROUND: Left ventricular non-compaction (LVNC) cardiomyopathy is a persistence of abnormal foetal myocardium and is a rare cause of cardiomyopathy in the peripartum period. Unlike other causes of peripartum cardiomyopathy which typically improve, LVNC has significant long-term personal and family implications and needs lifelong follow-up. CASE SUMMARY: We describe a unique case of a 30-year-old woman who developed cardiomyopathy in the peripartum period which was revealed on cardiovascular magnetic resonance imaging to be due to occult LVNC. Our patient also had Ebstein's anomaly, which is a known LVNC association. DISCUSSION: Cardiomyopathy in the peripartum period can be a decompensation of previously asymptomatic subclinical cardiomyopathy. It is important to assess for LVNC in patients presenting with this. Cardiovascular magnetic resonance imaging is the gold-standard imaging modality and allows accurate diagnosis of LVNC, associated structural complications and rare associations such as Ebstein's anomaly. Left ventricular non-compaction is irreversible and has implications for patients and their family members.

Accuracy and Prognostic Value of Physiologist-Led Stress Echocardiography for Coronary Disease.

BACKGROUND: We demonstrated that physiologist-led stress echocardiography (PLSE) is feasible for coronary artery disease (CAD) assessment. We sought to extend our work by assessing its accuracy and prognostic value. METHODS: Retrospective study of 898 subjects undergoing PLSE (n=393) or cardiologist-led stress echocardiography (CLSE) (n=505) for CAD assessment using exercise or dobutamine. For accuracy assessment, the primary outcome was the ability of stress echocardiography to identify significant CAD on invasive coronary angiography (ICA). Incidence of 24-month non-fatal MI, total and cardiac mortality, revascularisation and combined major adverse cardiac events (MACE) were assessed. RESULTS: Demographics, comorbidities, CAD predictors, CAD pre-test probability and cardiac medications were matched between the PLSE and CLSE groups. PLSE had high sensitivity, specificity, positive and negative predictive value and accuracy (85%, 74%, 69%, 88%, 78% respectively). PLSE accuracy measures were similar and non-inferior to CLSE. There was a similar incidence of individual and combined outcomes in PLSE and CLSE subjects. Negative stress echocardiography conferred a comparably low incidence of non-fatal MI (PLSE 1.4% vs. CLSE 0.9%, p=0.464), cardiac mortality (0.6% vs. 0.0%, p=0.277) and MACE (6.8% vs. 3.1%, p=0.404). CONCLUSION: This is the first study of the accuracy compared with gold standard of ICA, and prognostic value of PLSE CAD assessment. PLSE demonstrates high and non-inferior accuracy compared with CLSE for CAD assessment. Negative PLSE and CLSE confer a similarly very low incidence of cardiac outcomes, confirming for the first time the important prognostic value of PLSE.

Radiation-induced dystrophic calcification and severe valvular stenosis: the central role of multimodality 3D cardiac imaging in disease assessment and planning of combined transcatheter aortic and mitral valve replacement.

Cardiac disease after mediastinal radiotherapy can result in progressive valvular thickening and dystrophic calcification with ensuing leaflet restriction and dysfunction. This can ultimately manifest as valvular stenosis and/or regurgitation. We report a case of a 61-year-old woman with symptomatic severe aortic stenosis and severe mitral stenosis due to severe dystrophic calcification postmediastinal radiotherapy for lymphoma. She was deemed surgically inoperable due to dense, continuous calcification throughout the leaflets and annuli of both valves, aortomitral continuity, proximal coronary arteries and proximal porcelain aorta. She underwent simultaneous transcatheter aortic valve replacement and transcatheter mitral valve replacement with an excellent technical and clinical result at 7-month follow-up. We also describe the central role of multimodality three-dimensional transoesophageal echocardiography and multidetector cardiac CT imaging in assessing the severity of valve disease, characterising the nature of cardiac calcification and guiding decisions on surgical operability and transcatheter intervention.

The prognostic value of Tei index in acute myocardial infarction: a systematic review.

BACKGROUND: Echocardiographic evaluation of left ventricular ejection fraction (LVEF) is used in the risk stratification of patients with an acute myocardial infarction (AMI). However, the prognostic value of the Tei index, an alternative measure of global cardiac function, in AMI patients is not well established. METHODS: We conducted a systematic review, using MEDLINE and EMBASE, to evaluate the prognostic value of the Tei index in predicting adverse outcomes in patients presenting with AMI. The data was collected and narratively synthesised. RESULTS: A total of 16 studies were including in this review with 2886 participants (mean age was 60 years from 14 studies, the proportion of male patients 69.8% from 14 studies). Patient follow-up duration ranged from during the AMI hospitalisation stay to 57.8 months. Tei index showed a significant association with heart failure episodes, reinfarction, death and left ventricular thrombus formation in 14 out of the 16 studies. However, in one of these studies, Tei index was only significantly predictive of cardiac events in patients where LVEF was <40%. In two further studies, Tei index was not associated with predicting adverse outcomes once LVEF, left ventricular end-systolic volume index and left ventricular early filling time was taken into consideration. In the two remaining studies, there was no prognostic value of Tei index in relation to patient outcomes. CONCLUSIONS: Tei index may be an important prognostic marker in AMI patients, however, more studies are needed to better understand when it should be used routinely within clinical practice.

Percutaneous device closure of a large complex aortic root pseudoaneurysm.

Pseudoaneurysm of the aorta is a rare and high-risk complication of native aortic valve endocarditis and can develop following cardiac surgery for aortic valve and root disease. If left untreated, there is a significant risk of aortic wall rupture and fatality. Given the high risk of mortality associated with traditional open surgical repair of aortic pseudoaneurysms, endovascular approach is a potentially favourable alternative. We describe a unique case of a young woman with a large and complex paraprosthetic pseudoaneurysm with multiple communicating fistulae which was successfully percutaneously treated with two occluder devices. In this technically challenging case, a minor but unique procedural complication is also illustrated.

Unique characterization of complex endocarditic vegetations using 3D TOE.

A 31-year-old male was admitted with suspected infective endocarditis, given a history of i.v. drug use, lung and cerebral abscesses and Staphylococcus aureus bacteraemia. TTE imaging was limited given supine positioning and mechanical ventilation but suggested a posterior mitral valve leaflet (PMVL) mass. Three-dimensional TOE provided uniquely detailed assessment of two complex infective masses. The attachment of the presumed P2 mass on TTE was indeterminant even on 2D-TOE, appearing attached to the PMVL or AMVL depending on rotational view (Fig. 1). 3D-TOE imaging and subsequent multiplanar and volume-rendered reconstruction revealed this to be a complex, large vegetation attached to the anterior aspect of the anterolateral commissure with mobile heads prolapsing into the left atrium and causing mild mitral regurgitation through a small basal perforation (Figs 2, 3 and Video 1). The second mass was a filamentous vegetation attached to the LVOT, prolapsing towards but not contacting the aortic valve (Fig. 4 and Video 2). Comprehensive assessment of complex vegetations is crucial for optimal surgical planning. 3D-TOE allows rapid, accurate, unique assessment of such masses through unlimited multiplanar reconstructions, volume-rendered real-time imaging and colour full-volume regurgitation assessment which may not always possible on 2D-TTE or 2D-TOE. 3D imaging should be routinely used in TOE and in particular in suspected endocarditis.

When multimodality cardiac imaging saves the day: rare cause of embolic strokes.

Hypereosinophilic syndrome (HES) is a rare disorder characterised by eosinophilic infiltration of tissues. Myocardial infiltration occurs in 50%-60% of HES and leads to a condition called Loeffler's endocarditis. This can lead to endomyocardial injury with resultant superimposed thrombus formation and embolic stroke. We describe the case of a 57-year-old female patient presenting with neurological symptoms who was found to have multiple embolic strokes on a background of long-standing eosinophilia. Following a series of investigations, including transthoracic and transoesophageal echocardiographies and cardiovascular MRI, she was confirmed to have Loeffler's endocarditis with left ventricular thrombus. She was treated successfully with steroids and anticoagulation. We describe the pathophysiology of HES and Loeffler's endocarditis and stress the crucial role of multimodality cardiac imaging in establishing its diagnosis and treatment monitoring.

3D echocardiography allows rapid and accurate surgical planning in complex aortic root abscess cases.

Despite 3D echocardiography (3DE) acquiring significantly greater data than standard 2D echocardiography (2DE), it is underutilized in assessing cardiac anatomy and physiology. A key advantage is the ability of a single 3DE acquisition to be post-processed to generate volume rendered 3D models and an unlimited number of multiplanar reconstruction (MPR) images. We describe the case of a highly anxious patient with life-threatening complex aortic valve endocarditis and aortic root abscess, refusing transesophageal echocardiography (TOE) under general anaesthesia with tachycardia, breathlessness and acute kidney injury precluding accurate or safe gated (computed tomography) CT, who was comprehensively assessed with a rapid 3DE-TOE under sedation. This led to timely surgery and an excellent outcome for the patient.

Mitral annular disjunction: A systematic review of the literature.

BACKGROUND: Mitral annular disjunction (MAD) is a structural abnormality where there is a separation between the mitral valve annulus and the left atrial wall which is not well understood. METHODS: We conducted a systematic review to evaluate the prevalence of MAD, factors associated with MAD and clinical outcomes among patients with MAD. RESULTS: A total of 19 studies were included in this review, and the number of noncase report studies had between 23 and 1439 patients. The pooled rate of MAD in studies of myxomatous mitral valve patients was 66/130 (50.8%, 3 studies), and among patients with mitral valve prolapse was 95/291 (32.6%, 3 studies). One study suggests that 78% of patients with MAD had mitral valve prolapse, and another suggested it was strongly associated with myxomatous mitral valve disease (HR 5.04 95% CI 1.66-15.31). In terms of clinical significance, it has been reported that MAD with disjunction > 8.5 mm was associated with nonsustained ventricular tachycardia (OR 10 95% CI 1.28-78.1). There is also evidence that gadolinium enhancement in papillary muscle (OR 4.09 95% CI 1.28-13.05) and longitudinal MAD distance in posterolateral wall (OR 1.16 95% CI 1.02-1.33) was predictive of ventricular arrhythmia and late gadolinium enhancement in anterolateral papillary muscle was strongly associated with serious arrhythmic event (OR 7.35 95% CI 1.15-47.02). CONCLUSIONS: Mitral annular disjunction appears to be common in myxomatous mitral valve disease and mitral valve prolapse which can be detected on cardiac imaging and may be important because of its association with ventricular arrhythmias and sudden cardiac death.

The central role of CT coronary angiography in postcardiac arrest care in the young adult.

A 32-year-old man with no medical history went into ventricular fibrillation while running at the gym. He was transferred to our tertiary centre post successful resuscitation where admission electrocardiography and echocardiography were unremarkable. The initial cause of cardiac arrest was suspected arrhythmogenic and he was admitted for further investigations including exercise testing, ajmaline challenge, CT coronary angiography (CTCA) and cardiovascular MRI, with the likely outcome of cardioverter-defibrillator implantation. CTCA, however, revealed significant stenosis in the proximal left anterior descending artery as the likely cause for his arrest. Invasive coronary angiography confirmed this and facilitated successful stent implantation, avoiding the need for implantable cardioverter-defibrillator implantation. This case highlights the importance of CTCA, a non-invasive and readily-available test in the investigation of young patients postcardiac arrest, who require active exclusion of coronary artery disease and anomalous coronary anatomy, though they represent a low-risk population group.