Quadricuspid aortic valve with aortic insufficiency: a rare echocardiographic finding

Introduction: Quadriscupidaortic valve (QAV) is a rare congenital heart defect typically found incidentally without any associated cardiac defects. The functional status of QAV is pure aortic insufficiency (AI), however, clinical manifestations are dependent on the functional status of the valve, presenting in the fifth or sixth decade of life due to progressive degeneration of the leaflets. In our case, we present a 37-year-old female who developed post-partum dyspnea with elevated brain natriuretic peptide (BNP) levels concerning for heart failure. Transesophageal echocardiography (TEE) revealed a preserved ejection fraction with aortic regurgitation consistent with valvular heart failure, however, incidentally showed a QAV. Case Presentation: A 37-year-old female 5 days post cesarean section presented with dyspnea on exertion. Her physical examination was significant for a decrescendo diastolic murmur at the aortic area and bibasilar rales. Pertinent labs revealed a BNP level elevation of 345 pg/mL with normal troponin levels. Given her symptoms and elevated biomarkers, a transthoracic echocardiogram was obtained and was suggestive of AI. She was referred for transesophageal echocardiogram for better visualization of the aortic valve. TEE revealed a QAV with all four leaflets equal in size with normal thickness and mobility. Moderate malcoaptation of all valves was present and severe AI was visualized. Planimetry of aortic regurgitant orifice was measured at 0.29 cm2, the AI jet was greater than 65% of left ventricular outflow tract, pressure half time calculated at 252 ms, and venacontracta measured 0.6 cm. Her systolic (ejection fraction 60%) and diastolic function were both preserved. After diuresis, she was discharged home and followed up with structural heart and cardiac surgery. She was to have a CT coronary angiography performed part of her pre-operative evaluation, but was lost to follow up. Discussion: QAV is a rare congenital cardiac anomaly that is typically found incidentally. The most prevalent complication of QAV is AI, however, these patients are also at increased risk for infective endocarditis. This is due to the progressive degeneration of the leaflets from the asymmetric mechanical stress around the four cusps. Echocardiography allowed for visualization of the aortic valve and for quantification of the degree of AI. Given her clinical presentation and cardiac risk factors, it was unclear what was causing her symptoms on admission. Through echocardiography, a diagnosis was made and the patient was able to receive appropriate care. The advancement in imaging techniques has increased the capability to diagnose QAV and its complications. The definitive treatment of QAV with AI is valve replacement, which was recommended to our patient. Conclusion: QAV is a rare congenital disease that most commonly manifests with AI. QAV is typically found incidentally in the fifth and sixth decade of life and best visualized by TEE. Definitive management of QAV is valve replacement.https://scholarlycommons.henryford.com/merf2020caserpt/1034/thumbnail.jp

DOUBLE-TROUBLE: TAKOTSUBO AND ACUTE CORONARY SYNDROME IN A YOUNG WOMAN

Background: The original case series of patients with Takotsubo Syndrome (TTS) reported no significant epicardial coronary artery disease during angiography. However, recent evidence suggests an increasing overlap between the two diseases. We report a case of a 48-year old woman who had untreated generalized anxiety disorder and presented with angina. Case: A 48-year old woman with untreated general anxiety disorder presented with a 5 hour history of angina. An electrocardiogram demonstrated a prolonged QTc, no ST segment changes and new T-wave inversions in the anterolateral leads. High-sensitivity troponin was 4,336 ng/L and her InterTAK score was 91 with a 99.6% probability of TTS. Decision-making: Due to her persistent chest pain and EKG changes the patient underwent emergent left heart catheterization which showed critical occlusion of the 1st diagonal and 71% stenosis of the distal left circumflex. She underwent a primary PCI of both lesions. Her chest pain resolved after 6 hours of a nitrolgycerin infusion postoperatively and a transthoracic echocardiogram showed hypokinesis of the mid-distal apical, periapical, septal, lateral, inferior and anterior wall with an ejection fraction of 30-35%. The distribution of hypokinesia was out of proportion to the territory supplied by the culprit artery, suggesting a possibility of the apical type of Takotsubo syndrome. She was started on guideline-directed medical therapy for heart failure with reduced ejection fraction and dual antiplatelet therapy Conclusion: Patients with TTS may have coexistent significant epicardial CAD. Prolonged QTc and lack of ST-segment elevation in patients with CAD may help identify an additional diagnosis of TTS

TCT-378 Not Every TEE Is a “Standard of Care” TEE

Background: Intraprocedural structural heart imaging is more challenging and has unique differences from standard of care (SOC) imaging. However, the variations in time and complexity of different types of SOC transesophageal echocardiographs (TEEs) versus interventional TEEs is not well studied. In this study, we aim to compare the complexity of SOC nonvalvular indication TEE with SOC valvular TEE studies and interventional TEEs performed in the guidance of transcatheter edge-to-edge repair (TEER) MitraClip (Abbott Vascular) procedures. Methods: A retrospective case-control analysis was performed on 200 patients who underwent TEE in the Henry Ford Health System. One hundred cases of interventional TEE-guided TEER were compared with 73 nonvalvular (endocarditis and stroke evaluation) SOC TEEs and 27 valvular (preprocedural mitral, aortic, and tricuspid valve evaluations) SOC TEEs. Complexity was quantified by the total procedure duration, the total number of images, and the number of 3-dimensional (3D) clips captured. The mean, median, and SD were compared between these groups. The Kruskal-Wallis test was used to evaluate statistical significance. Results: The mean duration of TEE procedures, the number of images, and the number of 3D clips were all significantly higher in the interventional imaging TEER group compared with the noninterventional groups (P \u3c 0.0001) (Table 1). The duration and number of images were also significantly higher among valvular compared with nonvalvular SOC TEE groups (P \u3c 0.0002) as well as number of 3D clips (P \u3c 0.0012). Conclusion: Interventional TEE was more complicated and time-consuming compared with SOC TEE performed for both nonvalvular and valvular indications. The latter was also more complex than SOC nonvalvular TEE. This is the first study of its kind demonstrating objective differences between interventional and 2 SOC TEE groups. These results emphasize the need of dedicated training for intraprocedural imaging as well as restructuring of reimbursement codes. Categories: STRUCTURAL: Valvular Disease: Mitra

TCT-374 Structural Heart Intraprocedural Versus Nonprocedural Transesophageal Echocardiography: A Quantitative Analysis of Complexity

Background: Transesophageal echocardiography (TEE) is an essential tool in many structural heart procedures, such as transcatheter mitral valve edge-to-edge repair (TEER). Interventional procedural TEE requires a unique skill set. This study aims to evaluate the complexity of interventional structural heart TEE used to guide TEER compared with standard of care (SOC) TEE studies performed at a single center. Methods: A retrospective case-control analysis was performed of 200 patients who underwent TEE in the Henry Ford Health System. One hundred cases of interventional TEE-guided TEER were compared with 100 controls of SOC TEE. Complexity was quantified by the total duration of the procedure, the total number of images, and the number of 3-dimensional clips captured. The mean, median, and SD were compared between these 2 groups. Wilcoxon rank sum tests were used to evaluate statistical significance. Results: One hundred intraprocedural TEE studies to guide TEER and 100 SOC TEE studies were analyzed. The mean duration of TEE procedures, the number of images, and the number of 3-dimensional clips were all significantly higher in the TEER group (P \u3c 0.0001) (Table 1). Conclusion: Interventional TEE guidance for TEER is significantly more complex and more time-consuming than SOC TEE. This is the first large-scale study demonstrating objective differences between interventional and SOC TEE. This conclusion implicates the necessity of dedicated training programs for interventional imaging, in addition to the necessity of reviewing the current reimbursement codes to account for such a difference. Categories: STRUCTURAL: Valvular Disease: Mitra

Predicting global habitat suitability for stony corals on seamounts

Aim Globally, species distribution patterns in the deep sea are poorly resolved, with spatial coverage being sparse for most taxa and true absence data missing. Increasing human impacts on deep-sea ecosystems mean that reaching a better understanding of such patterns is becoming more urgent. Cold-water stony corals (Order Scleractinia) form structurally complex habitats (dense thickets or reefs) that can support a diversity of other associated fauna. Despite their widely accepted ecological importance, records of scleractinian corals on seamounts are patchy and simply not available for most of the global ocean. The objective of this paper is to model the global distribution of suitable habitat for stony corals on seamounts. Location Seamounts worldwide. Methods We compiled a database containing all accessible records of scleractinian corals on seamounts. Two modelling approaches developed for presence-only data were used to predict global habitat suitability for seamount scleractinians: maximum entropy modelling (Maxent) and environmental niche factor analysis (ENFA). We generated habitat-suitability maps and used a cross-validation process with a threshold-independent metric to evaluate the performance of the models. Results Both models performed well in cross-validation, although the Maxent method consistently outperformed ENFA. Highly suitable habitat for seamount stony corals was predicted to occur at most modelled depths in the North Atlantic, and in a circumglobal strip in the Southern Hemisphere between 20° and 50° S and shallower than around 1500 m. Seamount summits in most other regions appeared much less likely to provide suitable habitat, except for small near-surface patches. The patterns of habitat suitability largely reflect current biogeographical knowledge. Environmental variables positively associated with high predicted habitat suitability included the aragonite saturation state, and oxygen saturation and concentration. By contrast, low levels of dissolved inorganic carbon, nitrate, phosphate and silicate were associated with high predicted suitability. High correlation among variables made assessing individual drivers difficult. Main conclusions Our models predict environmental conditions likely to play a role in determining large-scale scleractinian coral distributions on seamounts, and provide a baseline scenario on a global scale. These results present a first-order hypothesis that can be tested by further sampling. Given the high vulnerability of cold-water corals to human impacts, such predictions are crucial tools in developing worldwide conservation and management strategies for seamount ecosystems. © 2009 Blackwell Publishing Ltd

Construct validity of a continuous metabolic syndrome score in children

<p>Abstract</p> <p>Objective</p> <p>The primary purpose of this study was to examine the construct validity of a continuous metabolic syndrome score (cMetS) in children. The secondary purpose was to identify a cutpoint value(s) for an adverse cMetS based on receiver operating characteristic (ROC) curve analysis.</p> <p>Methods</p> <p>378 children aged 7 to 9 years were assessed for the metabolic syndrome which was determined by age-modified cutpoints. High-density-lipoprotein cholesterol, triglycerides, the homeostasis assessment model of insulin resistance, mean arterial pressure, and waist circumference were used to create a cMetS for each subject.</p> <p>Results</p> <p>About half of the subjects did not possess any risk factors while about 5% possessed the metabolic syndrome. There was a graded relationship between the cMetS and the number of adverse risk factors. The cMetS was lowest in the group with no adverse risk factors (-1.59 ± 1.76) and highest in those possessing the metabolic syndrome (≥3 risk factors) (7.05 ± 2.73). The cutoff level yielding the maximal sensitivity and specificity for predicting the presence of the metabolic syndrome was a cMetS of 3.72 (sensitivity = 100%, specificity = 93.9%, and the area of the curve = 0.978 (0.957-0.990, 95% confidence intervals).</p> <p>Conclusion</p> <p>The results demonstrate the construct validity for the cMetS in children. Since there are several drawbacks to identifying a single cut-point value for the cMetS based on this sample, we urge researchers to use the approach herein to validate and create a cMetS that is specific to their study population.</p

Echinococcus granulosus : epidemiology and state-of-the-art of diagnostics in animals

Diagnosis and detection of Echinococcus granulosus (sensu lato) infection in animals is a prerequisite for epidemiological studies and surveillance of echinococcosis in endemic, re-emergent or emergent transmission zones. Advances in diagnostic approaches for definitive hosts and livestock, however, have not progressed equally over the last 20 years. Development of laboratory based diagnostics for canids using coproantigen ELISA and also coproPCR, have had a huge impact on epidemiological studies and more recently on surveillance during hydatid control programmes. In contrast, diagnosis of cystic echinococcosis (CE) in livestock still relies largely on conventional post-mortem inspection, despite a relatively low diagnostic sensitivity especially in early infections, as current serodiagnostics do not provide a sufficiently specific and sensitive practical pre-mortem alternative. As a result, testing of dog faecal samples by coproantigen ELISA, often combined with mass ultrasound screening programmes for human CE, has been the preferred approach for monitoring and surveillance in resource-poor endemic areas and during control schemes. In this article we review the current options and approaches for diagnosis of E. granulosus infection in definitive and animal intermediate hosts (including applications in non-domesticated species) and make conclusions and recommendations for further improvements in diagnosis for use in epidemiological studies and surveillance schemes

Cardiac Dysrhythmias and Neurological Dysregulation: Manifestations of Profound Hypomagnesemia

Magnesium is the second most common intracellular cation and serves as an important metabolic cofactor to over 300 enzymatic reactions throughout the human body. Among its various roles, magnesium modulates calcium entry and release from sarcoplasmic reticulum and regulates ATP pumps in myocytes and neurons, thereby regulating cardiac and neuronal excitability. Therefore, deficiency of this essential mineral may result in serious cardiovascular and neurologic derangements. In this case, we present the clinical course of a 76-year-old woman who presented with marked cardiac and neurological signs and symptoms which developed as a result of severe hypomagnesemia. The patient promptly responded to magnesium replacement once the diagnosis was established. We herein discuss the clinical presentation, pathophysiology, diagnosis, and management of severe hypomagnesemia and emphasize the implications of magnesium deficiency in the cardiovascular and central nervous systems. Furthermore, this case highlights the importance of having high vigilance for hypomagnesemia in the appropriate clinical setting

Cardiac Dysrhythmias and Neurological Dysregulation: Manifestations of Profound Hypomagnesemia.

Magnesium is the second most common intracellular cation and serves as an important metabolic cofactor to over 300 enzymatic reactions throughout the human body. Among its various roles, magnesium modulates calcium entry and release from sarcoplasmic reticulum and regulates ATP pumps in myocytes and neurons, thereby regulating cardiac and neuronal excitability. Therefore, deficiency of this essential mineral may result in serious cardiovascular and neurologic derangements. In this case, we present the clinical course of a 76-year-old woman who presented with marked cardiac and neurological signs and symptoms which developed as a result of severe hypomagnesemia. The patient promptly responded to magnesium replacement once the diagnosis was established. We herein discuss the clinical presentation, pathophysiology, diagnosis, and management of severe hypomagnesemia and emphasize the implications of magnesium deficiency in the cardiovascular and central nervous systems. Furthermore, this case highlights the importance of having high vigilance for hypomagnesemia in the appropriate clinical setting

Understanding arrhythmogenic right ventricular cardiomyopathy: Progression from diagnosis to demise

Introduction: Arrhythmogenic right ventricularcardiomyopathy (ARVC) is an under-recognized lethal primary disease of heart muscle which results in fibrofatty replacement of the right ventricle (RV). Familial in origin in 30% of cases, the prevalence of ARVC is estimated at 1 of 2000 to 5000 patients. It may be silent for decades prior topresentation and may lead to sudden cardiac death. Ventricular arrhythmias are the usual initial presentation in the form of palpitations or syncope. ARVC is a progressive disease involving RV, and rarely present with left ventricle (LV) manifestations. Presence of LV dysfunction predicts higher adverse outcomes. Transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) are the initial diagnostic modalities. Implant-able cardioverter defibrillator (ICD) lowers the rate of cardiac and non-cardiac mortality Herein, we present a case of ARVC that demonstrates the natural history of the disease. Case Presentation: Ms. C.F. was a 52-year-old woman with history of hypertension and atrial flutter who presented initially with palpitations. She was found to be in ventriculartachycardia (VT) requiring chemical cardioversion. Dobutmaine stress testing revealed dilated RV and induced her VT. Cardiac catheterization showed normal epi-cardial coronaries. CMR showed fat in the RV free wall near the apex and prominent RV trabeculation. Electrophysiology study revealed inducible VT. ICD implantation was recommended for primary prevention given her ARVC; however, the patient declined any invasive procedures and elected medical management only. She also refused genetic testing. Subsequent outpatient cardiology follow up documented recurrent palpitations with episodes of atrial flutter and frequent PVCs. Over the subsequent decade, she continued to decline ICD despite numerous recommendations and counseling, and was unfortunately nonadherent to her medications. She had multiple readmissions with syncope and right heart failure exacerbations with progressive worsening of RV dila-tation and function with preserved LV function on TTE. After 13 years from initial presentation, the patient presented to the emergency in cardiogenic shock requiring ino-tropic support and intubation. At that time, her LV function was significantly diminished with ejection fraction of 20%. She refused invasive testing or any form of hemody-namic monitoring. She furthermore refused consideration of advanced heart failure therapies. Given rapid deterioration in her clinical course, she agreed to hospice enrollment and expired soon afterwards. Conclusion: As our case demonstrates, the natural course involves progressive RV failure culminating in cardiogenic shock and recurrent arrhythmias with risk for sudden cardiac death. LV dysfunction confers higher mortality rates. Early ICD implantation is of paramount importance. Furthermore, education/counseling, genetic testing, and prompt referral to advanced heart failure for advanced therapies and/or cardiac transplantation consideration is essential