LEFT ATRIAL COMPRESSION FROM ACHALASIA - THE DIAGNOSTIC POWER OF ECHOCARDIOGRAM

Background: Left atrial compression by an extracardiac mass can arise from multiple structures surrounding the heart. Differentials include hiatal hernias, mediastinal masses, dissecting aortic aneurysms, esophageal malignancies, and rarely esophageal abnormalities such as achalasia. We present a case of a patient who during a routine transthoracic echocardiogram (TTE) was found to have left atrial compression secondary to achalasia, determined using appropriate maneuvers during image acquisition. Case: A 69-year-old female with diabetes underwent a TTE as part of workup for labile, uncontrolled blood pressure. Simultaneously, she was also being worked up for symptoms of dysphagia, weight loss and chronic cough. TTE revealed a 3.3 x 5 cm heterogeneous extracardiac mass compressing the left atrium. Decision-making: To determine the source of the mass, she was given a carbonated beverage to drink during the imaging acquisition, which was seen within the mass with a change in echo density, confirming GI origin. Imaging with CT scan revealed a dilated esophagus, and furthermore, EGD and esophageal manometry confirmed a diagnosis of achalasia. Conclusion: Echocardiographic imaging, with appropriate maneuvers, can be effective in identifying masses of gastrointestinal origin, differentiating them from vascular or mediastinal origin. Echocardiography has shown to be a strong, non-invasive tool to not only diagnose cardiac diseases, but also extracardiac manifestations of various organ systems

Hemodynamic and Echocardiographic Assessment of Left Ventricle Recovery with Left Ventricular Assist Devices: Do We Explant?

Introduction: Explantation of left ventricular assist devices (LVAD) after left ventricular (LV) recovery is estimated to occur in 1-2% of cases. Herein, we present a case of hemodynamic and echocardiographic assessment of LV recovery during outflow graft balloon occlusion leading to LVAD explantation. Case Report: A 56-year-old female with medical history of systolic heart failure due to non-ischemic cardiomyopathy with LVEF 25%. She underwent an urgent HeartMate 3 LVAD implant after an admission for cardiogenic shock. Post LVAD course was complicated by driveline infection. History was notable for admissions due to low-flow alarms in the setting of dehydration. On echocardiogram, progressive LVEF improvement was noted although with suboptimal images. CT angiography did not demonstrate any occlusion of the cannulas. Right heart catheterization showed stable cardiac index despite minimal flow on LVAD. Cardiopulmonary testing was favorable. After multi-disciplinary discussion, patient underwent LVAD wean study in the cath lab under hemodynamic and transesophageal echo (TEE) guidance with therapeutic anticoagulation. LVAD was turned off for 10 minutes with outflow graft occluded by Armada 14 mm x 20 cm peripheral balloon. Wiring of the outflow graft from aorta and balloon occlusion were visualized by TEE (Figure). The left and right ventricular function were similar to baseline with no change in mitral regurgitation. Cardiac index was normal (Figure). Patient subsequently underwent successful LVAD explant. She is doing well with NYHA class I symptoms and LVEF 45-50% noted upon 3-months follow-up LVAD explantation is a feasible option in LV recovery after appropriate hemodynamic and echocardiographic assessment. TEE is an essential tool, especially in patients with suboptimal windows. Outflow graft balloon occlusion can be used if there is concern about falsely poor results related to backflow or ongoing LVAD support at low speed leading to falsely improved results

National Landscape of Hospitalizations in Patients with Left Ventricular Assist Device. Insights from the National Readmission Database 2010-2015

The number of patients with left ventricular assist devices (LVAD) has increased over the years and it is important to identify the etiologies for hospital admission, as well as the costs, length of stay and in-hospital complications in this patient group. Using the National Readmission Database from 2010 to 2015, we identified patients with a history of LVAD placement using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code V43.21. We aimed to identify the etiologies for hospital admission, patient characteristics, and in-hospital outcomes. We identified a total of 15,996 patients with an LVAD, the mean age was 58 years and 76% were males. The most common cause of hospital readmission after LVAD was heart failure (HF, 13%), followed by gastrointestinal (GI) bleed (11.8%), device complication (11.5%), and ventricular tachycardia/fibrillation (4.2%). The median length of stay was 6 days (3-11 days) and the median hospital costs was $12,723 USD. The in-hospital mortality was 3.9%, blood transfusion was required in 26.8% of patients, 20.5% had acute kidney injury, 2.8% required hemodialysis, and 6.2% of patients underwent heart transplantation. Interestingly, the most common cause of readmission was the same as the diagnosis for the preceding admission. One in every four LVAD patients experiences a readmission within 30 days of a prior admission, most commonly due to HF and GI bleeding. Interventions to reduce HF readmissions, such as speed optimization, may be one means of improving LVAD outcomes and resource utilization

Safety, feasibility, and outcomes of transcaval access for the delivery of Impella microaxial-flow pump 5.0 in patients with acute heart failure

Background: Transcaval access (TCA) may enable fully percutaneous mechanical circulatory support (MCS) without the hazards of vascular complication in patients with heart failure that require left ventricular unloading. Purpose: To review the safety, feasibility, and outcomes of using TCA to deliver Impella 5.0 MCS in patients with ischemic and non-ischemic systolic acute heart failure. Methods: This single center retrospective study included all patients that underwent TCA placement of a 5.0 Impella from June 2015 to January 2021. Demographic, clinical and procedural variables, and in-hospital outcomes were collected. The procedure was performed by electrifying a caval guidewire and advancing it into a pre-positioned aortic snare. After exchanging for a rigid guidewire, a 22 or 24Fr sheath was delivered into the aorta and then the Impella 5.0 was placed in the left ventricle through TCA sheaths. Results: A total of 43 patients were included in the analysis. The average age was 56.9 years (interquartile range [IQR], 52-65.5), of which, 70%(n=30) were males. Fifteen patients had non-ischemic cardiomyopathy and 28 had ischemic cardiomyopathy. Baseline average left ventricular ejection fraction prior to implantation was 23.6% (IQR, 13.75-29.75). 86% of the patients were in category C-D of the SCAI classification schema for cardiogenic shock (CS), 39.5% required inotropes and 48.8% required pressors prior to the procedure; 54% had a prior MCS in place. Only 18.6% of the cases had prior CT imaging reviewed for planning. TCA was successful in all attempted patients and the MCS delivery was achieved in 100% of the cohort. The available hemodynamic parameters prior and after Impella 5.0 implantation via Table 1 TCA are summarized in table 1. From the total cohort, only 29 patients survived to explant device and TCA sheath. The explant was successful in all patients using nitinol occluders; two patients required a covered stent at the arteriotomy site due to right sided heart failure from residual fistula; no surgical repair was necessary. All residual fistulous tracks were graded as1 from Impella insertion/removal site was observed in 9.3%,which didn\u27t require further intervention. No vascular complication of the access site was observed with TCA. During hospitalization, 20.9% had VT/VF and 4.7% a PEA after implantation (all CS patients). 13.9% of the patients had AKI requiring hemodialysis and no stroke was observed in the entire group. The average length of stay for entire cohort was 16.3 days (IQR, 3.25-18.75). Conclusions: Transcaval access of 5.0 Impella is safe and feasible under expert hands for patients where more conventional MCS devices do not provide enough support or have inadequate peripheral arterial access

Smoldering recurrent pericarditis presenting as a loculated pericardial effusion mimicking pericardial cyst

Background Pericardial effusion could manifest as a loculated cyst on cardiac imaging. Anakinra (Interleukin-1 receptor antagonist) is showing promising results for treating recurrent resistant pericarditis. Case A 24-year-old male with no medical history presented with pleuritic chest pain after flu-like symptoms. He notes 3-4 similar episodes in past few years. Workup revealed diffuse ST elevations on EKG and a new right middle lobe opacity on chest X-ray. Chest CTA showed a pericardial cyst corresponding to the opacity seen on radiograph. No pericardial cyst or effusion was identified on echocardiogram. Further testing resulted in a positive rhinovirus/enterovirus PCR with unremarkable autoimmune workup. Decision-making Outpatient Cardiac MR to follow on the pericardial cyst was completed 6 weeks after the initial presentation. Interestingly, an interval resolution of the pericardial cyst seen on the prior CT chest noted. Patient endorsed recurrent symptoms with doubling of colchicine dose and resuming non-steroidal agents. He continued to have pericarditis flares, and a short course of prednisone was added. Eventually, patient was started on Anakinra, in addition to colchicine and ibuprofen, with improvement in symptoms. Conclusion Loculated pericardial effusion could mimic a pericardial cyst. Clinical correlation is imperative. Interleukin-1 receptor antagonists could result in symptomatic relieve in patients suffering from recurrent persistent pericarditis

A RARE CASE OF EOSINOPHILIC MYOCARDITIS PRESENTING WITH REFRACTORY VENTRICULAR TACHYCARDIA AND CARDIOGENIC SHOCK

Background: Eosinophilic myocarditis (EM) is a rare etiology of cardiogenic shock. There are few reports of eosinophilic myositis associated with orbital myositis. Herein, we present a rare case of EM presented with refractory ventricular tachycardia (VT). Case: 55-Year-old female with medical history of orbital myositis on chronic oral prednisone, bicuspid aortic valve, abdominal aortic aneurysm. She presented to an outside hospital with palpitations and found to have a wide complex tachycardia with atrioventricular dissociation requiring subsequent cardioversions. Echocardiogram showed left ventricle ejection fraction at 51% and normal right ventricle. Coronary angiogram showed non-obstructive coronary artery disease. Course was complicated by refractory VT, intubation, and cardiogenic shock that warranted escalation of care. On arrival, patient was in VT storm and required multiple shocks despite anti-arrhythmics. Patient was placed on venoarterial extracorporeal membrane oxygenation(VA-ECMO). Decision-making: Due to rapid progression of her cardiogenic shock, giant cell myocarditis was suspected. Other differential included sarcoidosis, viral or other acute myocarditis. Due to instability, cardiac magnetic resonance was not performed. Endomyocardial biopsy revealed a small focus of eosinophils infiltrating a focus with myocardial cell damage and edema consistent with EM. There was no peripheral eosinophilia. Extensive rheumatological and autoimmune workup in addition to viral panels were negative. Patient was started on high dose steroids that was gradually tapered down to oral prednisone. Due persistent VT, VT ablation attempt was unsuccessful. Patient was deemed not a candidate for heart transplant. Eventually, with mexilitine, amiodarone and steroids, VA-ECMO was weaned off. Unfortunately, her course was also complicated by acute renal injury requiring hemodialysis, sepsis, and shock liver. Patient enrolled in hospice and expired. Conclusion: EM can be presented without peripheral eosinophilia. EM should be suspected in patients presenting with arrhythmia or cardiogenic shock and have history of orbital myositis for early immunosuppressive therapy

Hybrid continuity equation using multidetector CT and echocardiography aids reclassifying severity in patients with paradoxical low flow low gradient severe aortic stenosis

Background: Paradoxical low fow low gradient aorticstenosis (PLFLGAS) is a complex clinical entity withchallenges in accurate diagnosis. Doppler echocardiography (DE) may understate the left ventricular outfow tract (LVOT) area and may introduce inaccuracies incalculation of the valve area. Multidetector computed tomography (MDCT) has been shown to more accurately characterize the LVOT and annulus. We thus hypothesized that utilizing a hybrid modifed continuity equation by substituting LVOT diameter and derived cross sectional area from MDCT, patients with severe PLFLGAS would be reclassifed to a lesser severity. Methods: Patients being evaluated for transcatheter aortic valve replacement (TAVR) between January 2015 to July 2017 who had both echo and MDCT data available were retrospectively reviewed. Inclusion criteria consisted of aortic valve area (AVA) \u3c 1 cm2by DE, left ventricular ejection fraction ≥ 50%, stroke volume index (SVI) \u3c 35 mL/m2, and mean aortic pressure gradient ≤ 40 MMHG by DE. AVA was then recalculated substituting MDCT LVOT data into the equation. The cut off for severe AS with the modifed continuity equation was set as \u3c 1.2 cm2as previously reported. Results: 67 patients were ultimately included in the analysis (mean age 79.9 ± 8.6 years, 49% female). Mean LVOT area was signifcantly larger by MDCT compared with DE (4.8 ± 1.0 vs. 3.3 ± 0.6 cm2; p \u3c 0.001). Modifed continuity equation yielded a signifcantly larger AVA (1.04 ± 0.24 vs. 0.72 ± 0.17 cm2; p \u3c 0.001). Twenty four percent (n=16) of patients were ultimately reclassifed from severe to moderate AS by merging MDCT and Doppler data. The 16 patients reclassifed into the moderate category had larger DE AVA (0.86 ± 0.08 vs. 0.67 ± 0.16 cm2; p\u3c0.001) and higher SVI (30.9 ± 2.8 vs. 26.9 ± 5.4; p\u3c0.001) compared to those who remained in the severe range. Conclusion: Merging MDCT with DE reclassifes nearly a quarter of patients with severe PLFLGAS into the moderate category. Considering patients are currently referred for aortic valve interventions (surgical or TAVR) based on DE data, future studies will need to assess if proceeding or deferring interventions can be based on combining MDCT and DE

A Case of Muscular Dystrophy with Dilated Cardiomyopathy: Do Not Forget Your Basics

Introduction: Becker muscular dystrophy (BMD) is an X-linked recessive disorder with dystrophin mutation. Dilated Cardiomyopathy (DCM) is a leading cause of death in BMD patients. Herein, we are presenting a patient with BMD that initially sought medical attention for acute onset of systolic heart failure that highlights the importance of careful clinical assessment and appropriate work up. Case Report: A 29-year-old male with medical history of asthma presented to the hospital with progressive dyspnea and leg swelling. He was diagnosed with DCM with an LVIDD of 6.5 cm and LV ejection fraction of 20-25% by echocardiogram. Coronary angiogram revealed no coronary artery disease. Initial blood work and electrocardiogram are below (Figure). Cardiac MRI showed severely reduced biventricular systolic function with near circumferential, sub-epicardial to mid-myocardial delayed gadolinium enhancement (Figure). Initial differential diagnosis included prior myocarditis vs. burnt out sarcoidosis. It was subsequently noted that patient began recurrently falling with muscle weakness from age 20 years with chronically elevated AST and CK. His exam was notable for atrophy of the bilateral quadriceps muscles, decreased muscular strength and bilateral calves hypertrophy. Electromyography showed evidence of chronic proximal and distal myopathy, predominantly affecting the lower extremity. Skeletal muscle biopsy showed fascicular atrophy and hypertrophy, focal endomyosial fibrosis and an increase of central nuclei without evidence of inflammation or granuloma which was most suggestive of a muscular dystrophy. Genetic testing was then completed and showed hemizygous dystrophin mutation confirming diagnosis of BMD. BMD has a diffuse phenotype and should be considered in young patients with cardiomyopathy and chronically elevated CK and AST. A thorough clinical history, exam, and CMR can assist in directing need for skeletal muscle biopsy and subsequent genetic testing

To close or not to close a patent foramen ovale: That is the question.

LEARNING OBJECTIVE #1: Recognizing that cardiac shunting often occur in setting of severe pulmonary hypertension (PH) and serves to relieve the severe increase in the right sided pressures. LEARNING OBJECTIVE #2: Recognizing one of the contraindications to patent foramen ovale (PFO) closure. CASE: A 65-year old female presented to Heart Failure clinic after a follow up transthoracic echocardiography (TTE). She complained of dyspnea, bilateral lower extremity edema, and right 4th digit pain. On physical exam, patient was noted to have darkening of her right 4th digits. She had hypoxia with higher oxygen requirement than baseline. Past medical history is significant for Interstitial Lung Disease (ILD), Pulmonary Hypertension (PH) groups (2 and 3), coronary artery disease with three prior stents, and chronic diastolic dysfunction. Her TTE showed EF of 50%, pulmonary artery systolic pressure of 60 mmHg, and large (greater than 20 bubbles) PFO, that is new, with predominantly right to left shunting across the atrial septum. Patient was hospitalized due to concern for paradoxical emboli and for her hypoxia. Right heart catheterization (RHC) was done that showedmean Pulmonary arterial pressure of 45 mmHg, wedge pressure of 8 mmHg, cardiac index of 2.13 L/min/m2, pulmonary vascular resistance of 840 dynes.sec.cm-5 (10.5 Wood units), and systemic vascular resistance (SVR) of 797.84 dynes.sec.cm-5 at rest. CT angiography was done that showed acute pulmonary embolus (PE) to segmental branches of right lower lobe and demonstrated stable ILD. An upper extremity arterial study demonstrated reduced perfusion to the right 4th digit attributable to a possible embolic event. High intensity heparin was initiated. Risks and Benefits of PFO closure were discussed at a multi-disciplinary meeting. Given that patient\u27s PVR \u3e 2/3 of SVR in the RHC, PFO closure was deferred. The patient was ultimately discharged on warfarin. IMPACT: Careful decision making in PFO closure should be pursued for PH patients, even in presence of a known indication for closure. One of the contraindications to PFO closure is the irreversible pulmonary hypertension (PVR \u3e2/3 SVR or pulmonary artery pressure \u3e 2/3 systemic arterial pressure). It is important to recognize that closure of PFO in those situations could precipitate decompensation of right ventricular (RV) function and sudden drop in cardiac output which could be fatal. DISCUSSION: While PFO closure may be pursued in cases of paradoxical emboli, a risk/benefit analysis, especially looking at PH, is necessary. In this case, acute PE is the likely cause of acute rise in the right sided cardiac pressures. Eventually, the acute PFO provided a necessary outflow tract for right sided pressure overload

Paradoxical low gradient aortic stenosis reclassified using hybrid continuity equation by multidectector CT: Insights into diastolic function and post TAVR outcomes

Background: Paradoxical low flow low gradient aortic stenosis (PLF-AS) is a complex clinical entity posing challenges in diagnosis and management. Left ventricular outflow tract (LVOT) diameter underestimation is a key reason for inaccuracies in quantitation. We investigated the utility of a hybrid modified continuity equation using multidetector computed tomography (MDCT) derived LVOT diameter/cross sectional area and Doppler echocardiography (DE) to study the impact of reclassifying patients with severe PLF-AS. We further analyzed differences in diastolic parameters and outcomes in reclassified patients post-transcatheter aortic valve replacement (TAVR). Methods: Patients being evaluated for TAVR between January 2015 to July 2017 with both DE and MDCT data available were retrospectively reviewed. PLF-AS defined as valve area (AVA) ≤ 1 cm2 by DE, left ventricular EF ≥ 50%, stroke volume index (SVI) ≤ 35 mL/m2, and mean aortic pressure gradient ≤ 40 mmHg by DE. AVA was recalculated substituting MDCT LVOT data into the equation. The cut offfor severe AS with the hybrid equation was set as ≤ 1.2 cm2 as previously reported. Diastolic Parameters and TAVR outcomes (NYHA class, mortality and hospital admissions for CHF) were analyzed. Results: 67 patients were included in the analysis (mean age 79.9 ± 8.6 years, 49% female). Twenty four percent (n=16) of patients were ultimately reclassified from severe to moderate AS by merging MDCT and DE data. There was a significantly higher number of patients with diastolic dysfunction (grade I or higher) (95% vs 75%; p=.03) and restrictive physiology (grade 3 diastolic dysfunction) (33% vs. 8%; p=.032) in those who remained in the severe AS group using the hybrid equation. NYHA class prior to valve replacement was worse in the patients in the severe category (3.05 vs. 2.6; p=0.013). At a mean f/u of 19 months 67% (n=45) underwent TAVR (11 reclassified as moderate AS and 34 as severe AS). Those who remained in the severe AS range had a greater improvement in their SVI (35.5 vs. 26.9 p≤.001) aft er TAVR, and average improvement in NYHA class was significantly better in the severe AS group (1.63 vs 1.11 p=0.042 ). There was no significant difference between the two groups with regards to CHF hospitalization or all-cause mortality. Conclusion: Merging MDCT with DE reclassifies nearly a quarter of patients with severe PLF-AS into the moderate category. Patients with severe AS per hybrid equation have more advanced DD and had a more significant improvement in NYHA class and SVI. Future studies will need to assess if proceeding or deferring interventions can be based on combining MDCT and DE