Evolving consequences of right coronary artery to right atrium: coronary cameral fistula-a case report.

Background: Coronary cameral fistula is a rare diagnosis, which may be picked up incidentally on cardiac imaging. While majority of cases is asymptomatic, they can be complicated by myocardial ischaemia, arrhythmias, heart failure, infective endocarditis, and rarely rupture or thrombosis of the fistula leading to sudden death. Case summary: A 73-year-old female presents with fever, lethargy, and examination finding of a continuous cardiac murmur. CT coronary angiogram confirmed the presence of a coronary cameral fistula, with an aneurysmal RCA seen arising from the right coronary sinus, following an extensive tortuous course wrapping around the heart, and terminating at the right atrium. While there was initial streptococcus bacteraemia identified on blood culture sampling, no obvious masses were detected on the valves, chambers, or along the course of the fistula. Over time, she develops anginal chest pain and heart failure symptoms, with progressive dilatation of the right ventricle and functional tricuspid regurgitation secondary to shunting of the fistula into the right chambers. Surgical intervention was then pursued and successfully addressed these complications. Discussion: This case report highlights the importance of advanced imaging modalities for accurate diagnosis of coronary cameral fistulae, addressing late manifestations of the disease and the necessity for a collaborative, multidisciplinary approach in managing complex cardiac anomalies.

Postnatal cardiovascular morbidity following preterm pre-eclampsia: An observational study.

OBJECTIVE: To explore the nature of postnatal cardiovascular morbidity following pregnancies complicated by preterm pre-eclampsia and investigate associations between pregnancy characteristics and maternal postnatal cardiovascular function. STUDY DESIGN: This was an observational sub-study of a single-centre feasibility randomised double-blind placebo-controlled trial (https://www. CLINICALTRIALS: gov; NCT03466333), involving women with preterm pre-eclampsia, delivering before 37 weeks. Eligible women underwent echocardiography, arteriography and blood pressure monitoring within three days of birth, six weeks and six months postpartum. Correlations between pregnancy and cardiovascular characteristics were assessed using Spearman's correlation. MAIN OUTCOME MEASURES: The prevalence of cardiovascular dysfunction and remodelling six months following preterm pre-eclampsia. RESULTS: Forty-four women completed the study. At six months, 27 (61 %) had diastolic dysfunction, 33 (75 %) had raised total vascular resistance (TVR) and 18 (41 %) had left ventricular remodelling. Sixteen (46 %) women had de novo hypertension by six months and only two (5 %) women had a completely normal echocardiogram. Echocardiography did not change significantly from six weeks to six months. Earlier gestation at delivery and lower birthweight centile were associated with worse six-month diastolic dysfunction (E/E': rho = -0.39, p = 0.001 & rho = -0.42, p = 0.005) and TVR (rho = -0.34, p = 0.02 & rho = -0.37, p = 0.01). CONCLUSIONS: Preterm pre-eclampsia is associated with persistent cardiovascular morbidity-six months postpartum in the majority of women. These cardiovascular changes have significant implications for long-term cardiovascular health. The graded severity of diastolic dysfunction and TVR with worsening pre-eclampsia phenotype suggests a dose-effect. However, the mechanistic link remains uncertain.

Resolution of echocardiographic features of left ventricular non-compaction and systolic dysfunction following treatment for heart failure.

Isolated left ventricular non-compaction (IVNC) is a myocardial disease, thought to be related to abnormal embryological development of the myocardium. The condition may be associated with left ventricular dysfunction and heart failure, as well as arrhythmias and thrombo-embolism. The left ventricular dysfunction is generally regarded to be progressive in nature, leading to worsening heart failure and death. We report a case in which initial investigations fulfilled the diagnostic criteria for IVNC. However, follow-up investigation demonstrated recovery of left ventricular systolic function as well as resolution of the features of non-compaction, making a retrospective diagnosis of myocarditis more likely.

Echocardiographic detection of atrial septal defects: the forgotten view.

The right parasternal view is conventionally used to assess the aortic valve and the ascending aorta. We describe two cases in which the right parasternal view was helpful in assessment of the interatrial septum and the detection of atrial septal defects (ASD), including one case in which this view confirmed the presence of a defect not seen in any other echocardiographic view. Use of the right parasternal view should be considered for assessment of the interatrial septum, particularly when there is a high index of suspicion for an atrial septal defect but no defect is seen on conventional views. It should also be used to exclude the possibility of multiple defects even when an ASD is visualized using conventional imaging planes.

Tricuspid regurgitation: contemporary management of a neglected valvular lesion.

Right-sided cardiac valvular disease has traditionally been considered less clinically important than mitral or aortic valve pathology. However, detectable tricuspid regurgitation (TR) is common and recent data suggest that significant TR can lead to functional impairment and reduced survival, particularly in patients with concomitant left-sided valvular disease. The tricuspid valve is a complex anatomical structure and advances in three dimensional echocardiography and cardiac MRI have contributed to a greater understanding of tricuspid valve pathology. These imaging techniques are invaluable in determining the aetiology and severity of TR, and provide an assessment of right ventricular function and pulmonary artery pressure. TR is more prevalent in women and those with a history of myocardial infarction and heart failure. It also occurs in about 10% of patients with rheumatic heart disease. Chronic severe TR may have a prolonged clinical course culminating in the development of fatigue and poor exercise tolerance due to a reduced cardiac output. Approximately 90% of cases of TR are secondary to either pulmonary hypertension or intrinsic right ventricular pathology and about 10% are due to primary tricuspid valve disease. Primary causes such as Ebstein's anomaly, rheumatic disease, myxomatous changes, carcinoid syndrome, endomyocardial fibrosis, and degenerative disease have characteristic morphological features readily identifiable by echocardiography. Ascertaining an accurate right ventricular systolic pressure is important in separating primary from secondary causes as significant TR with a pressure <40 mm Hg implies intrinsic valve disease. Cardiac MRI may be indicated in those with inadequate echocardiographic images and is also the gold standard for the evaluation of right ventricular function and morphology. The assessment of leaflet morphology, annular dimensions, and pulmonary artery pressure are particularly important for determining subsequent management. Along with appropriate treatment of the underlying cause of TR and pulmonary hypertension, management guidelines indicate a move towards more aggressive treatment of TR. In those undergoing left-sided valve surgery, tricuspid valve repair is universally recommended in the presence of severe coexistent TR; in those with isolated severe TR, surgery is recommended in the presence of symptoms or progressive right ventricular dilatation or dysfunction.

Postnatal Enalapril to Improve Cardiovascular Function Following Preterm Preeclampsia (PICk-UP):: A Randomized Double-Blind Placebo-Controlled Feasibility Trial.

Hypertensive disease in pregnancy is associated with future cardiovascular disease and, therefore, provides an opportunity to identify women who could benefit from targeted interventions aimed at reducing cardiovascular morbidity. This study focused on the highest-risk group, women with preterm preeclampsia, who have an 8-fold risk of death from future cardiovascular disease. We performed a single-center feasibility randomized controlled trial of 6 months' treatment with enalapril to improve postnatal cardiovascular function. Echocardiography and hemodynamic measurements were performed at baseline (<3 days), 6 weeks, and 6 months postdelivery on 60 women. At randomization, 88% of women had diastolic dysfunction, and 68% had concentric remodeling/hypertrophy. No difference was seen in total vascular resistance (P=0.59) or systolic function (global longitudinal strain: P=0.14) between groups at 6 months. However, women treated with enalapril had echocardiographic measurements consistent with improved diastolic function (E/E'[the ratio of early mitral inflow velocity and early mitral annular diastolic velocity]: P=0.04) and left ventricular remodeling (relative wall thickness: P=0.01; left ventricular mass index: P=0.03) at 6 months, compared with placebo. Urinary enalapril was detectable in 85% and 63% of women in the enalapril arm at 6 weeks and 6 months, respectively. All women responded positively to taking enalapril in the future. Our study confirmed acceptability and feasibility of the study protocol with a recruitment to completion rate of 2.2 women per month. Importantly, postnatal enalapril treatment was associated with improved echocardiographic measurements; these early improvements have the potential to reduce long-term cardiovascular disease risk. A definitive, multicenter randomized controlled trial is now required to confirm these findings. Registration- URL: https://www.clinicaltrials.gov; Unique identifier: NCT03466333.

Left ventricular non-compaction in identical twins with thalassaemia and cardiac iron overload.

Cardiac disease in patients with transfusion-dependent beta-thalassaemia major is well described. Cardiac manifestations may include left ventricular wall thickening and both systolic and diastolic dysfunctions. We describe a group of family members, including a pair of identical twins, each of whom suffered from thalassaemia major requiring multiple transfusions. Cardiac magnetic resonance demonstrated myocardial iron overload, and impairment of systolic function. Echocardiography confirmed both significant left ventricular systolic and diastolic impairment, along with features consistent with left ventricular non-compaction. This finding has not been noted in association with thalassaemia-related cardiac disease before. We then review the cardiac manifestations which occur in association with thalassaemia major.

Severe mitral and aortic regurgitation in association with ankylosing spondylitis.

Aortic disease and aortic valve regurgitation are well documented in association with ankylosing spondylitis, although involvement of the mitral valve occurs more rarely. We report a case of severe mitral and aortic regurgitation in association with ankylosing spondylitis. We then discuss the characteristic cardiac manifestations that may occur in association with ankylosing spondylitis and the associated echocardiographic features.

Acute Systemic Lupus Erythematous and Antiphospholipid Syndrome With Cardiac Complications.

This case illustrates the potential for multiple cardiovascular complications in acute systemic lupus erythematous with antiphospholipid syndrome. (Level of Difficulty: Beginner.).

Abnormal Remodeling of Subcutaneous Small Arteries Is Associated With Early Diastolic Impairment in Metabolic Syndrome.

BACKGROUND: Small artery pathophysiology is frequently invoked as a cause of obesity-related diastolic heart failure. However, evidence to support this hypothesis is scant, particularly in humans. METHODS AND RESULTS: To address this, we studied human small artery structure and function in obesity and looked for correlations between vascular parameters and diastolic function. Seventeen obese patients with metabolic syndrome and 5 control participants underwent echocardiography and subcutaneous gluteal fat biopsy. Small arteries were isolated from the biopsy and pressure myography was used to study endothelial function and wall structure. In comparison with the control group, small arteries from obese participants exhibited significant endothelial dysfunction, assessed as the vasodilatory response to acetylcholine and also pathological growth of the wall. For the obese participants, multiple regression analysis revealed an association between left atrial volume and both the small artery wall thickness (ß=0.718, P=0.02) and wall-to-lumen ratio (ß=0.605, P=0.02). Furthermore, the E:E' ratio was associated with wall-to-lumen ratio (ß=0.596, P=0.02) and inversely associated with interleukin-6 (ß=-0.868, P=0.03). By contrast, endothelial function did not correlate with any of the echocardiographic parameters studied. CONCLUSIONS: Although the small arteries studied were not cardiac in origin, our results support a role for small artery remodeling in the development of diastolic dysfunction in humans. Further direct examination of the structure and function of the myocardial resistance vasculature is now warranted, to elucidate the temporal association between metabolic risk factors, small artery injury, and diastolic impairment.

One-Month Global Longitudinal Strain Identifies Patients Who Will Develop Pacing-Induced Left Ventricular Dysfunction over Time: The Pacing and Ventricular Dysfunction (PAVD) Study.

BACKGROUND: Predicting which individuals will have a decline in left ventricular (LV) function after pacemaker implantation remains an important challenge. We investigated whether LV global longitudinal strain (GLS), measured by 2D speckle tracking strain echocardiography, can identify patients at risk of pacing-induced left ventricular dysfunction (PIVD) or pacing-induced cardiomyopathy (PICMP). METHODS: Fifty-five patients with atrioventricular block and preserved LV function underwent dual-chamber pacemaker implantation and were followed with serial transthoracic echocardiography for 12 months for the development of PIVD (defined as a reduction in LV ejection fraction (LVEF) ≥5 percentage points at 12 months) or PICMP (reduction in LVEF to <45%). RESULTS: At 12 months, 15 (27%) patients developed PIVD; of these, 4 patients developed PICMP. At one month, GLS was significantly lower in the 15 patients who subsequently developed PIVD, compared to those who did not (n = 40) (GLS -12.6 vs. -16.4 respectively; p = 0.022). When patients with PICMP were excluded, one month GLS was significantly reduced compared to baseline whereas LVEF was not. One-month GLS had high predictive accuracy for determining subsequent development of PIVD or PICMP (AUC = 0.80, optimal GLS threshold: <-14.5, sensitivity 82%, specificity 75%); and particularly PICMP (AUC = 0.86, optimal GLS threshold: <-13.5, sensitivity 100%, specificity 71%). CONCLUSIONS: GLS is a novel predictor of decline in LV systolic function following pacemaker implantation, with the potential to identify patients at risk of PIVD before measurable changes in LVEF are apparent. GLS measured one month after implantation has high predictive accuracy for identifying patients who later develop PIVD or PICMP.

An unusual case of combined classic and reverse twiddler's syndrome.

In recent years the phenomenon of reverse twiddler's syndrome has been described, characterized by pulse generator manipulation resulting in lead advancement rather than retraction. We describe what we believe to be the first reported case of both classic and reverse twiddler's syndrome occurring simultaneously in a patient with a biventricular implantable cardioverter-defibrillator.

Percutaneous device closure of atrial septal defect results in very early and sustained changes of right and left heart function.

OBJECTIVES: To investigate the echocardiographic effects of percutaneous closure of secundum atrial septal defect (ASD) in adults and assess which pre-closure parameters predict good response to closure. BACKGROUND: ASD is a common congenital heart disease often undiscovered until adulthood. ASD closure has been revolutionized by the use of percutaneous devices. The effects of these procedures on echocardiographic parameters are not well characterized. METHODS: Patients undergoing percutaneous device closure of ASD between June 2007 and June 2009 had 3 sequential echocardiograms reviewed: pre-procedure, immediate post-procedure (24 hours) and 6-8 weeks post-procedure. Significant changes from baseline were investigated using paired t-test/1-way ANOVA. Pearson correlation (2-tailed) tests were used to categorize patients as 'good responders' to closure in terms of selected parameters. RESULTS: 129 echocardiograms in 43 consecutive patients were included. Remodeling of both ventricles occurred immediately following ASD closure and was sustained. Right ventricular (RV) diameter in diastole decreased by 13.5% and 19.3% compared to baseline at 24 hours and 6-8 weeks post-closure, respectively (p<0.05); Left ventricular (LV) diameter in diastole increased by 8.5% and 15.6%, respectively (p<0.05). Functional parameters of the RV also demonstrated early and sustained decreases (TAPSE decreased by 8.3% and 17% compared to baseline at 24 hours and 6-8 weeks post-closure, respectively (p<0.05)). Smaller RV baseline diameter appeared to predict good response to closure. CONCLUSIONS: Percutaneous ASD closure has immediate, sustained benefits on multiple echocardiographic parameters. Good responders have smaller RV at baseline, suggesting early closure is preferable.

Vasodepressor syncope and recurrent pharyngeal carcinoma: a form of carotid sinus syndrome?

Syncope occurring in the setting of head and neck malignancy may have several possible causes. Local effects of a tumour in the parapharyngeal area may produce a syncopal syndrome similar to carotid sinus hypersensitivity, but with distinctive differentiating features. This article presents a case of profound vasodepressor syncope in a patient with recurrent pharyngeal malignancy, and discusses the mechanisms by which this occurs and the possible therapeutic options.

Paradoxical systolic and diastolic flow abnormalities in hypertrophic cardiomyopathy with mid-cavity systolic obstruction.

Systolic obstruction of the left ventricular outflow tract, either at rest or during provocation, is a frequent finding in patients with hypertrophic cardiomyopathy. Mid-cavity obstruction is uncommon, and intraventricular diastolic pressure gradients in association with either mid-cavity of apical hypertrophy are reported only rarely. We describe a patient with hypertrophic cardiomyopathy with evidence of systolic mid-cavity obstruction, and with complex diastolic paradoxical flow abnormalities within the left ventricular cavity detected by colour and pulsed-wave Doppler. Contrast echocardiography confirmed the presence of diastolic and systolic obstruction at the mid-ventricular level, with evidence of an apical cavity sequestered from the main body of the left ventricle during systolic mid-cavity obliteration.

Mycobacterium tuberculosis causing infection of an implantable biventricular defibrillator.

Mycobacterial infection of implantable cardiac devices is rare, and infection of pacing system components with M. tuberculosis has been reported on only three previous occasions, involving epicardial pacing systems in two cases. We report here a pocket infection with M. tuberculosis in a transvenous biventricular defibrillator.