Cardiology Commons^™

Pediatric Pulmonary Artery Aneurysms Causing Pulmonary Hemorrhage: A Case Of Transcatheter Intervention While On Va Ecmo Support, Igor Areinamo

Research Days

Background: The incidence of pulmonary artery aneurysm (PAA) in children remains unknown. There are few reports of a transcutaneous interventional approach to address this type of pathology. Management of PAAs while on VA ECMO has not been described in pediatrics.

Objectives/Goal:

Methods/Design:

Case: 9-year-old previously healthy male presented with MRSA associated ARDS and sepsis requiring urgent VA ECMO cannulation. Chest CT done on ECMO day 22 showed development of right sided PAAs. Patient had an episode of significant pulmonary hemorrhage. Angiogram demonstrated two fusiform right PAAs, one of which had ruptured and was bleeding into the lung parenchyma. Patient had …

Online Echocardiography Modules For Pediatric Cardiology Fellows, Sarah Studyvin, Doaa Aly, Tyler Johnson, Laura Kuzava, Alison Samrany, Nitin Madan, Sanket Shah

Research Days

Background: Adequate echocardiographic (echo) education is integral to successful cardiology training. Online education is proven to be effective in other specialties, but pediatric echo training data are lacking.

Objectives/Goal: Our aim was to design and assess the role of a novel online module-based curriculum to enhance pediatric cardiology fellows’ echo education.

Methods/Design: Four interactive modules, focused on principles and interpretation of a normal echo, were delivered to fellows. Content included high quality 2D and 3D echo clips as well as interactive quizzes and could be accessed from a phone, tablet, or computer. All fellows completed pre- and post-tests, validated by …

Constrictive Pericarditis After Repair Of A Ruptured Sinus Of Valsalva, Sarah Studyvin, Laura Kuzava

Research Days

Background: Constrictive pericarditis is an uncommon complication of cardiac surgery. We report a patient who developed constrictive pericarditis after ruptured sinus of Valsalva (RSOV) repair.

Methods: A 23-year-old male presented with exertional dyspnea one year after RSOV repair. TTE showed a small, circumferential effusion with thickened pericardium, ventricular septal bounce, left atrial enlargement, diastolic hepatic flow reversal, and trivial mitral regurgitation without stenosis. He underwent cardiac catheterization, which revealed elevated filling pressures (RVEDP 16 mmHg, LVEDP 18 mmHg), RVEDP/RVSP ratio < 0.5, and a low cardiac index (1.65 L/min/m2). Cardiac MRI confirmed pericardial thickening with paradoxic septal motion, dilated pulmonary veins and retrograde flow in the SVC.

Results: Pericardiectomy of thickened and adherent pericardium was performed. The central venous pressure decreased from 23 to 7 mmHg and …

Prenatal Diagnosis Of An Uncommon Form Of A Hypoplastic Left Heart Syndrome Variant., Anmol Goyal

Research Days

Background: Mitral valve dysplasia syndrome (MVDS) is a rare form of congenital heart disease, similar to hypoplastic left heart syndrome (HLHS). Prenatal identification is important for counseling, delivery planning and postnatal management.

Case: A 39 year-old woman underwent fetal echocardiography at ~34 weeks gestation for evaluation of fetal cardiomegaly and hydrops revealing: biatrial enlargement, mild-to-moderate mitral and tricuspid valve insufficiency, echobright mitral valve apparatus, biventricular dilation, severe LV fibroelastosis (EFE) and systolic dysfunction, mild hypoplasia of aortic valve annulus and aortic arch, and a thick restrictive atrial septum (RAS) with left to right flow (Fig 1a-c).

Decision‐making: Although critical aortic …

Two-Dimensional Speckle -Tracking Echocardiography Derived Longitudinal Strain In Patients With Unstable Angina And Normal Left Ventricular Ejection Fraction, Srinivasan Giridharan, Arumugam Aashish, Palamalai Arun Prasath, Selvaraj Karthikeyan, Sasinthar Rangasamy, Balasubramaniyan Amirthaganesh, Pazhanivel Vijayalakshmi

Journal of the Saudi Heart Association

Longitudinal strain (LS) measurements by Speckle Tracking Echocardiography (STE) is a point of care imaging modality that had been documented to have a role in NSTEMI/STEMI and stable Coronary artery disease (CAD). This study aims to determine the accuracy of LS parameters in diagnosis and predicting severity of CAD in unstable angina patients with no regional wall motion abnormalities. It was a prospective analytical study in which 143 low-risk Unstable angina patients underwent STE. Based on Coronary angiogram results, the study participants were divided into obstructive CAD and control group. Patients with obstructive CAD (n ¼ 79) had significantly higher …

Partial Vena Cava Occlusion (Vco) To Counteract Refractory Heart Failure: A New Era In Interventional Heart Failure Strategy., Yasar Sattar, Monil Majmundar, Talal Almas, David Song, Waqas Ullah, Homam Moussa Pacha, Mohamed Zghouzi, Islam Y Elgendy, Fatir Murtaza, M Chadi Alraies

Abington Jefferson Health Papers

Background: Patients with acute decompensated heart failure are prone to recurrent exacerbation leading to poor quality of life when they do not respond to an optimal medical regimen. Due to the lack of linear positive inotropy response to increasing preload in heart failure patients, increasing preload is associated with poor outcomes. Partial occlusion of either IVC or SVC is a proposed novel treatment that can improve cardiac function or quality of life by altering preload/pressure in heart failure (HF) patients unresponsive to diuretics.

Methods: PubMed, Ovid (MEDLINE), and Cochrane database we searched using the MeSH terms including "Superior vena cava …

Standardized Point-Of-Care Ultrasound (Pocus) Set-Up For Cardiac Evaluation During Covid-19 Pandemic In A Pediatric Population., Anmol Goyal

Research Days

Background: The novel coronavirus SARS-CoV2 is known to cause significant cardiac dysfunction, effusion, thromboembolism and coronary dilation. Currently no pediatric specific guidelines exist from ACC or ASE for use of POCUS. Our objective was to identify ideal pediatric appropriate POCUS equipment and standardize its use.

Methods: A Phillips Lumify probe paired with Android-Samsung tablet and a Butterfly iQ probe paired with an iPad were considered. A workflow algorithm and cleaning instructions were created. If the POCUS scan answered the clinical question, then a detailed echo was deferred. If the POCUS scan revealed findings requiring comprehensive evaluation, a follow-up echo was …

Tricuspid Atresia With Totally Anomalous Pulmonary Venous Connection: An Uncommon Finding, Anmol Goyal

Research Days

Background: Tricuspid atresia comprises 3-4% of all congenital heart disease (CHD). Anatomic findings are no true connection between the right atrium (RA) and right ventricle (RV) with concomitant findings of a ventricular septal defect (VSD), right ventricular hypoplasia, abnormalities of right ventricular outflow tract, pulmonary valve and pulmonary arteries. Common associated lesions have been described including transposition of great vessels, hypoplasia of aortic arch, double outlet ventricle or a common arterial trunk. Total anomalous pulmonary venous connection (TAPVC) is relatively rare and we present a case in addition to the six previously described cases in literature.

Case: A 33-year-old woman …

Upregulation Of Inflammatory Cytokines In Pulmonary Embolism Using Biochip-Array Profiling., Emily Bontekoe, Yevgeniy Brailovsky, Debra Hoppensteadt, Jack Bontekoe, Fakiha Siddiqui, Joshua Newman, Omer Iqbal, Trent Reed, Jawed Fareed, Amir Darki

Department of Medicine Faculty Papers

The complex pathophysiology of pulmonary embolism (PE) involves hemostatic activation, inflammatory processes, cellular dysfunction, and hemodynamic derangements. Due to the heterogeneity of this disease, risk stratification and diagnosis remains challenging. Biochip-array technology provides an integrated high throughput method for analyzing blood plasma samples for the simultaneous measurement of multiple biomarkers for potential risk stratification. Using biochip-array method, this study aimed to quantify the inflammatory biomarkers such as interleukin (IL)-1α, IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, vascular endothelial growth factor (VEGF), interferon gamma (IFN-γ), tumor necrosis factor alpha (TNF-α), monocyte chemoattractant protein-1 (MCP-1), and epidermal growth factor (EGF) in 109 clinically …

Use Of Non-Vitamin K Antagonist Oral Anticoagulants Among Patients With Nonvalvular Atrial Fibrillation And Multimorbidity, Steve Deitelzweig, Allison Keshishian, Amiee Kang, Amol Dhamane, Xuemei Luo, Christian Klem, Lisa Rosenblatt, Jack Mardekian, Jenny Jiang, Huseyin Yuce, Gregory Y. H. Lip

Publications and Research

Introduction

Non-valvular atrial fibrillation (NVAF) is often accompanied by multiple comorbid conditions, which increase the associated risks and complexity of patient management. This study evaluated the risk of stroke/systemic embolism (SE) and major bleeding (MB) among multimorbid patients with NVAF who were prescribed non-vitamin K antagonist oral anticoagulants (NOACs) or warfarin.

Methods

A retrospective study of patients with NVAF and high multimorbidity who initiated apixaban, dabigatran, rivaroxaban, or warfarin from 1 January 2013 to 30 September 2015 was conducted using five insurance claims databases. Multimorbidity was defined as six or more comorbid conditions, and 1:1 propensity score matching (PSM) was …

The Incidence Of Acute Pulmonary Embolism With Covid-19 Pneumonia In Saudi Arabia: A Retrospective Single-Center Study, Samah I. Abohamr, Mubarak A. Aldossari, Hala A. Amer, Hiba M. Saadeddin, Sara W. Abdelhamid, Fayaz A. Bhat, Eman Abdul Aziz Elsheikh

Journal of the Saudi Heart Association

Background: Computed tomography (CT) plays a significant role in the treatment of coronavirus disease 2019 (COVID-19) and has helped to identify patients with serious complications. We investigated the clinical characteristics and chest CT findings of COVID-19 positive patients complicated with acute pulmonary embolism (APE). Methods: A retrospective, record-based, case-series study was performed examining 483 patients admitted to King Saud Medical City during the pandemic, from April 2020 to June 2020. Of these, 92 patients who underwent chest CT scans were included in the final analysis. The incidence of APE, clinical presentations, radiological patterns, and patient outcomes were assessed …

Syncope Or Seizure?, Joseph Heron, Kevin Dwyer

Rowan-Virtua Research Day

Syncope is a common complaint in the emergency departments, accounting for 1-2% of visits, and can approach admission rates of a staggering 85%. The causes and conditions can be numerous, ranging from benign to life threatening. A good background history can go a long way in determining the etiology of the patient’s syncope. We describe a case of an elderly male who presented with a reported chief complaint of seizures, another syncope-mimic seen in the emergency department. He had a history of CAD, HTN, AAA, and osteoarthritis but no prior history of cardiac arrhythmia, MI, or structural heart disease. The …

The Effect Of Carfilzomib And Bortezomib Based Regimes On Cardiotoxicity In Multiple Myeloma Patients At Cooper University Hospital, Ami Patel, Tulin Budak-Alpdogan, Stalam Tapati

Rowan-Virtua Research Day

Introduction

• Multiple myeloma (MM) is a cancer of plasma cells, which is a white blood cell that normally produces antibodies
• Treatment in patients younger than 65 years old is typically high dose chemotherapy, usually with bortezomib based regimens or lenalidomide dexamethasone, followed by a stem cell transplant
• For patients with relapsed myeloma, carfilzomib is usually the treatment of choice
• Carfilzomib is a highly selective, irreversible proteasome inhibitor that binds to the 20 S proteasome. Several studies have illustrated that carfilzomib has been associated with cardiovascular adverse events (CVAE).
• Current literature on the role and effect of bortezomib on cardiotoxicity is …

Dynamic Left Ventricular Obstruction Post Transcatheter Aortic Valve Replacement (Tavr): The “Suicide Lv” Phenomenon, Brian Chen

Rowan-Virtua Research Day

• The transcatheter aortic valve replacement (TAVR) has become a relatively common treatment option for aortic stenosis, especially given the growing number of older patients with severe degenerative aortic stenosis

• While the procedure is overall very safe, there are still a number of potentially serious complications including bleeding, stroke, and cardiac tamponade. One rare complication is a dynamic left ventricular obstruction post valve deployment, which has been called the “Suicide LV” phenomenon.

• Rapid hemodynamic compromise resulting from this can result in significant morbidity and mortality if not diagnosed in time.

• Given the increasing frequency of patients undergoing …

The Coumadin Ridge: Incidental Finding Mimicking Thrombus On Echocardiogram, Keith Brown Jr., Matthew Everwine, Keith R. Anacker, Jose Nieves

Rowan-Virtua Research Day

The left atrial ridge or “coumadin ridge” is a cardiac anatomic structure located between the left pulmonary vein and the orfice of the left atrial appendage. Historically, patients were misdiagnosed with intra-atrial thrombus and were incorrectly placed on anticoagulation with warfarin, ultimately acquiring the name of the “coumadin ridge”. Fortunately this anatomical variant is now more commonly recognized, however when improperly identified can lead to unnecessary testing, cost, diagnosis anxiety, and inappropriate treatment for the patient. This purpose of this case is to bring awareness to this common clinical dilemma in an attempt to reduce ambiguity and unnecessary workups surrounding …

Retrograde Thromboembolism From The Proximal Descending Thoracic Aorta Leading To Recurrent Acute Cerebrovascular Events, Mark Staroelsky, Akash Patel, Gregory Holland, Nishant Parikh, Peter Bulik

Rowan-Virtua Research Day

In the United States, approximately 800,000 individuals experience a stroke every year

Nearly 25% of strokes are recurrent

Cryptogenic strokes, or those with unknown causes after testing, make up a significant portion of ischemic strokes, as many as 32%

Retrograde thromboembolic events originating from the proximal descending thoracic aorta should be considered as a potential etiology in cryptogenic strokes

The appropriate management of embolic events from aortic atheroma needs further research

Here, we present a case of a 55-year-old male who had recurrent cryptogenic strokes whose origin was discovered to stem from retrograde embolic phenomena from atheroma located within the …

An Unusual Ed Case: Scrotal Edema From A Thoracic Aortic Aneurysm In A 58-Year-Old Male, Yevgeniy Polunin, Emily Nguyen, Bhumi Shah

Rowan-Virtua Research Day

Scrotal edema is not an uncommon presentation in emergency medicine. We describe the case of a 58-year-old male with no prior medical history who presented to the emergency department (ED) due to progressive scrotal edema. The patient noted increased painless testicular enlargement, dry cough, and dyspnea on exertion over the course of several weeks. Scrotal edema was evaluated by an Ultrasound (US), showing bilateral testicular edema with concern for congestive heart failure (CHF). Computed tomography pulmonary angiography (CTPE) scan showed a large 6.51 cm thoracic aortic aneurysm (TAA), cardiomegaly with dilated left sided chambers, and a small pericardial effusion. Subsequently, …

An Unusual Ed Case: Cardiac Tamponade Presenting As Hiccups, Devleen Baksi, Nicholas Tomasello, James Espinosa, Alan Lucerna

Rowan-Virtua Research Day

Cardiac tamponade is an emergent life threating condition that depending on cause can quickly progress to death. The rate of accumulation of a pericardial effusion often can determine the clinical severity. Typically, shortness of breath and hypotension manifest; however, in this unusual case, the initial presenting complaint was persistent hiccups.

An Unusual Ed Case: Aortic Aneurysm Presenting As Chf Exacerbation, Darren Finn, James Espinosa, Russell Mordecai, Kishan Patel

Rowan-Virtua Research Day

There has been a significant increase in the incidence of thoracic aortic aneurysms, currently making aneurysmal disease the 18th most common cause of death within the United States. This disease is typically an insidious one, with the aorta growing an average of only 0.1 cm per year. Consequently, most patients remain asymptomatic until late stages of the disease when dissection or worse, rupture has occurred. Under extremely rare circumstances, thoracic aortic aneurysms can present clinically due to mass effect. Airway, esophageal and vascular compression, secondary to aortic arch dilation has been previously documented. The condition can be acquired or congenital, …

Bilateral Lower Extremity Ischemia Following Cardiac Catheterization, Moses Avedikian, Alan Lucerna, Trent Malcolm

Rowan-Virtua Research Day

Acute limb ischemia is a rare yet limb-threatening presentation where rapid diagnosis and intervention is warranted. Bilateral lower extremity ischemia can be a rare complication following procedures, such as a cardiac catheterization with access obtained in the groin. We describe a case of a 79-year-old female who presented with bilateral lower extremity ischemia following a cardiac catheterization 2 weeks prior. The patient underwent CT angiography, which demonstrated occlusion with no contrast present in the bilateral femoral arteries.

Individual Versus Sequential Saphenous Vein Grafts For On-Pump Coronary Artery Bypass Grafting – Does Smaller Coronaries In Indians Affect Graft Choice? - A Mid-Term Patency Comparison, Manoj M. Joshi, Saptarshi Paul, Krishnarao N. Bhosle, Suraj W. Nagre, Hrishikesh Parashi, Manish Jadhao, Kunal Rawekar, Vignesh Ravikumar, Vishal Sawkar, Joshua A. Selwyn

Journal of the Saudi Heart Association

Purpose: Although multiarterial grafting or bilateral mammary artery use is being increasingly emphasized for contemporary coronary artery bypass grafting (CABG) practice, saphenous vein graft (SVG) still accounts for 80% of all CABG conduits (Park et al., 2020) [1]. In India, both the individual and sequential saphenous grafting techniques are used arbitrarily, and there has not been a study that compares the mid-term patency of these two. This is specially relevant in view of smaller coronaries in Indians than the Caucasian counterparts. This study aims to compare the patency for on pump CABG's. Methods: In the present study, 323 patients underwent …

A Quality Improvement Approach To Reduce 30-Day Readmissions And Mortality In Patients With Acute Decompensated Heart Failure, Yee Yin Hoo, Wardati Mazlan-Kepli, Wan Nurul Huda Wan Hasan, Fan Jie Chen, Prashanthini Devadas, Yan Yee Chow, Qian Yi Sow, Azrol Amar Azizan, Abdul Muizz Abd Malek, Glendon Seng Kiong Lau, Ping Lik Chua

Journal of the Saudi Heart Association

Objectives: Heart failure [HF] hospital readmissions are a continued challenge in the care of HF patients, which contribute substantially to the high costs of the disease and high mortality rate in lower to middle income country. We implemented a quality improvement project to improve patient outcomes and resource utilization. Methods: This study was a prospective cohort design with a historical comparison group. It was conducted to assess the difference in 30-day readmissions and mortality and to assess compliance rate with HF guideline between the historical pre-intervention audit 1 cohort and prospective post-intervention audit 2 cohorts. Audit 1 cohort were recruited …

Challenging Diagnosis Of Patent Foramen Ovale In Presence Of A Large Eustachian Valve., Marouane Boukhris, Jean-Bernard Masson, Jeannot Potvin

Journal of the Saudi Heart Association

We report the case of a 39-year-old woman who presented with cryptogenic stroke. Conventional and contrast echocardiography modalities noticed a large Eustachian valve (EV) but failed to identify any right-to-left shunt. Conversely, contrast-transcranial Doppler revealed a grade 3 right-to-left shunt. Contrast echocardiography was repeated with bubble injections through both an upper extremity and a femoral vein, 10 min apart from each other. While no shunt was observed following upper extremity injection, > 20 bubbles crossed the inter-atrial septum when contrast was injected via femoral vein confirming the diagnosis of patent foramen ovale.

Anomalous And Diseased Left Main Coronary Artery Arising From The Right Coronary Sinus In An Elderly Lady, Salah E. Altarabsheh, Salil V. Deo, Abeer M. Rababa'h, Fadi H. Alhusban, Rami A. Alsharbini

Journal of the Saudi Heart Association

We report a case of 66-year-old female patient who presented with unstable angina and New York Heart Association Class III symptoms. Echocardiogram demonstrated wall motion abnormalities in the anterior and inferior walls. Coronary angiography demonstrated a severely diseased right coronary artery (RCA) and anomalous left main (LM) coronary artery arising from the right coronary sinus and courses posterior to the aorta and runs between the aorta and the main pulmonary artery with severe multiple atherosclerotic disease. Patient underwent successful coronary artery bypass grafting and was dismissed in good general status.

Cardiovascular Complications Of Systemic Lupus Erythematosus: Impact Of Risk Factors And Therapeutic Efficacy--A Tertiary Centre Experience In An Appalachian State, Elise Danielle Mcveigh, Amna Batool, Arnold J. Stromberg, Ahmed K. Abdel-Latif, Nayef Mohammed Kazzaz

Statistics Faculty Publications

OBJECTIVES: Cardiovascular complications became a notable cause of morbidity and mortality in patients with lupus as therapeutic advancements became more efficient at managing other complications. The Appalachian community in Kentucky has a higher prevalence of traditional cardiovascular risk factors, predisposing them to cardiovascular events. Namely, the mean body mass index of the members of the Kentucky Appalachian community was reported at 33 kg/m² and 94.3% of male members of this community use tobacco. We sought to identify risk factors that predispose patients with lupus to cardiovascular morbidities and examine the effect of immunomodulatory drugs.

METHODS: We identified 20 UKHS …

Predictive Value Of Hatch Scoring And Waist-To-Height Ratio In Atrial Fibrillation Following Coronary Artery Bypass Operations Performed With Cardiopulmonary Bypass, Muhammed Savran, Mesut Engin, Orhan Guvenc, Hasan F. Yüksek, Sadık Ahmet Sünbül, Tamer Turk, Yusuf Ata, Ufuk Aydın, Ahmet F. Ozyazicioglu

Journal of the Saudi Heart Association

Objectives: Postoperative atrial fibrillation (PoAF), an important clinical condition that can occur after coronary artery bypass graft (CABG) operations, may bring about cerebrovascular risks, prolong hospital stay and increase treatment costs. In this prospective study, we aimed to investigate the predictive value of HATCH score and waist/height ratio (WHR) values in revealing the development of PoAF after CABG operations. Methods: Patients who underwent isolated CABG surgery with cardiopulmonary bypass between May 2019 and November 2019 were prospectively included in the study. Preoperative demographic characteristics, laboratory parameters, and operative parameters of the patients were recorded prospectively. Results: A total of 255 …

Incidence And Predictors Of Left Ventricular (Lv) Thrombus After St-Elevation Myocardial Infarction (Stemi) In The Holy Capital Of Saudi Arabia, Azmat Khadija Niazi, Hoda Kassem, Ghada Shalaby, Sheeren Khaled, Muhannad Saleh Alzahrani, Hassan Mohammad Ali, Fatima Aboulenein

Journal of the Saudi Heart Association

Background: Patients with acute myocardial infarction (AMI) especially those with large MI (myocardial infarction) as identified by ST elevation in multiple contiguous ECG leads or anterior MI, may suffer significant myocardial damage leading to impaired wall motion and contractility which may lead to the formation of left ventricular thrombus (LVT) in the patient. This study was aimed to establish the incidence of LV thrombus and determine the predictors associated with the formation of LV thrombus in patients with AMI. Methods: This retrospective study was held at the only cardiothoracic centre of Makkah, which provides tertiary level cardiac services. A total …

Feasibility Of The Use Of A Stereoscopic Camera In The Final Inclusion (Sci-Fi) Of Donor Organ Assessment Using Virtual Reality Technology, Ismael A. Salas De Armas, Juan E. Marcano, Jayeshkumar A. Patel, Mehmet H. Akay, Manish K. Patel, Bindu Akkanti, Marwan F. Jumean, Sachin Kumar, Rajko Radovancevic, Sriram Nathan, Biswajit Kar, Igor D. Gregoric

The VAD Journal

Objective: Donor organ assessment is challenging, particularly when physicians and surgeons have to make decisions remotely. To improve communication and preparation for the transplant process, we proposed that a versatile, secure, and transportable video presence would enable a detailed assessment of donor organs.

Methods: A virtual reality camera with a 360-degree visualization range was used with a smartphone receiver to enable integral assessment of the donor's heart in the operating room, with visualization of the organs in real time. Prospective, observational data was collected in two cases in this proof-of-concept study. An assessment physician evaluated the donor heart remotely, and …

Removal Of Impella In The Setting Of Left Ventricular Thrombus: A Potential Indication For Cerebral Embolic Protection Devices, Miro Asadourian, Avinash Sharma, Richard Kiel, Felice Lin, Manminder Bhullar

The VAD Journal

Successful percutaneous mechanical circulatory support (MCS) has been used for acute stabilization of cardiogenic shock (CS). Improved survival outcomes have been observed in patients with CS from an acute myocardial infarction (AMI) who undergo implantation of left ventricular (LV) to ascending aorta rotodynamic pumps, such as the Impella® device (Abiomed). However, thrombotic events are a known complication of such devices in poor flow states such as CS. There is limited evidence regarding the management of patients who develop an LV thrombus after Impella insertion. Currently, the Sentinel cerebral protection system (SCPS, Boston Scientific) is the only FDA-approved device for cerebral …

Early Systolic Dysfunction And Impact Of Gene Mutation Severity In Marfan Syndrome, Aaron Clem, Lois J. Starr, David Danford, Mary Craft, Ling Li, Jason Christensen, Anji Yetman

Child Health Research Institute Pediatric Research Forum

Background

Marfan syndrome is caused by a mutation in the fibrillin-1 gene that manifests with a variety of features including aortic root dilation. Recent research has identified a primary cardiomyopathy in patients with Marfan syndrome, hypothesized to be due to the presence of abnormal fibrillin-1 in the myocardium. Controversy over the nature and significance of this cardiomyopathy remains. Echocardiographic measurement of the first-phase of ejection (defined as the beginning of systole to peak aortic valve flow) may be more sensitive to systolic dysfunction and provide useful clinical information. The purpose of this study is to:

1. Assess systolic dysfunction in patients …