Ventricular Obstruction Research Articles

Unusual cause of tricuspid valve obstruction after surgical aortic valve replacement.

Unruptured sinus of Valsalva aneurysm can present with manifestations due to local compression or protrusion into the chambers. Right ventricular inflow obstruction is a rare manifestation. This image highlights the tricuspid valve obstruction due to a Valsalva aneurysm in a patient after surgical aortic valve replacement.

Cardiovascular complications of anorexia nervosa – a clinical case with transient left ventricular hypertrophy and obstruction in the ventricular outflow tract

Anorexia nervosa is an eating disorder with a multisystemic nature, affecting female adolescents predominantly. The disease is potentially fatal with cardiac complications being the leading cause. We present a clinical case of a 16-year-old girl with anorexia nervosa after a mental trauma, hospitalized in our Clinic in an extremely severe, pre comatose state with weight 26 kg, BSA – 1.12 m2 and BMI – 10.4. On the background of severe abnormalities in homeostasis, bradycardia with left ventricular extrasystoles in bigeminy and episodes of tachycardia with a frequency of 238/min. Echocardiography revealed reduce in the heart size and the left ventricular muscle mass, MI – I + degree and a small, apical pericardial effusion. During the echocardiographic follow-up, there was signifi cant hypertrophy of the left ventricle with obstruction in the  outfl ow tract, with a reduction in ventricular size, without signifi cant change in left ventricular muscle mass. As a result of long-term, comprehensive treatment, with the participation of a team of specialists, the patient achieved true behavioural criticality, improved food tolerance, weight gain up to 46 kg with BMI - 18.4 and normalization of cardiac anatomy. Cardiac complications of anorexia nervosa are common, varied, and potentially lethal and require targeted cardiac monitoring and treatment. Left ventricular hypertrophy with obstruction in the ventricular outfl ow tract, fi rst described in 2006, is a rare but potentially fatal cardiac complication in patients with the disease.

Left Ventricular Assist Device Outflow Graft Obstruction Caused by Extrinsic Compression

<h3>Introduction</h3> Left ventricular assist device (LVAD) outflow graft obstruction is a rare, but serious complication. A polytetrafluoroethylene (PTFE) graft covering has been used by some surgeons to reduce the frequency of adhesions and risk upon re-entry to the chest. Extra-luminal accumulation of material into the space between the graft and PTFE covering is one extremely rare cause of external compression and outflow graft obstruction. <h3>Case Report</h3> A 49-year-old male with a history of heart failure status post HeartWare LVAD with pump exchange one year previous due to thrombosis presented with a low flow alarm. His normal LVAD parameters were Flow 5.8 LPM, speed 3000 RPM, and power 6 Watts (W). On interrogation, he had a drop in flow to 2 LPM and power to 4 W beginning two days prior to presentation. Lab work was inconsistent with acute anemia, thrombosis, or hemolysis. Computed tomography angiography of the chest demonstrated outflow graft narrowing. He was taken to the OR for exploration which revealed accumulation of thick, proteinaceous material had accumulated between the outflow graft and the PTFE covering causing external compression and flow limiting stenosis. After removal of the external covering and relief of the obstruction, his flows increased from 2 to 6 LPM and power from to 4 to 6 W. <h3>Summary</h3> Evaluation of LVAD alarms can be difficult. Upon review of labs and imaging, an extraluminal compression of the outflow graft was suspected. LVAD outflow obstruction is a rare, but serious complication. One cause is accumulation of proteinaceous material between the semipermeable graft and the impermeable PTFE covering thought to be derived plasma contents. There is no consensus on treatment. Case series suggest percutaneous stenting or pump exchange. Surgical intervention can provide immediate relief of this problem.

Effect of Ventriculoperitoneal Shunt on the Recovery of Brain Function in Children with Hydrocephalus

Objective: To analyze the effect of ventriculoperitoneal shunt on the recovery of brain function in children with hydrocephalus. Methods: The clinical data of 40 children with hydrocephalus were retrospectively analyzed. Ventriculoperitoneal shunt was performed with 9003 shunt tube and P.S. Shunt tube, B.C.E. shunt tube. Electroencephalogram (EEG), and brain CT/MRI were performed before and after surgery, and postoperative follow-up was carried out to observe the therapeutic effect. Results: In this study, there were seven cases of intracranial injury, seven cases of congenital hydrocephalus, 11 cases of ventricular end obstruction, three cases of abdominal end obstruction, nine cases complicated with bacterial infection, and 3 cases of shunt entering the scrotum. The prognosis of all the children was good, and there were no significant changes in eight cases. Conclusion: Ventriculoperitoneal shunt is effective in the treatment of children with hydrocephalus.

Pathophysiology and Clinical Management of Intraventricular Hemorrhage and Post-Hemorrhagic Hydrocephalus

Intraventricular hemorrhage(IVH)in patients with intracerebral hemorrhage is an independent risk factor. IVH can cause acute hydrocephalus by impairing cerebrospinal fluid dynamics. However, the pathological mechanism remains clear. In addition to the conventional concept of ventricular system obstruction by hematoma clots, secondary effects of heme and iron originating from hemoglobin might contribute to ventricular enlargement. The toxicity of accumulated hematoma in the ventricles might also influence the hydrocephalus and cause poor outcomes. An external ventricular drainage should be inserted promptly to control the intracranial pressure. Moreover, the hematoma should be removed as soon as possible to minimize the toxicity of the hematoma. The use of thrombolytic agents significantly reduces both the duration of ventricular drainage and mortality compared to external ventricular drainage alone. However, the functional outcome is not improved in patients with IVH. As another surgical option, endoscopy may be useful to evacuate the hematoma immediately. Finally, more evidence is essential for establishing the effectiveness of endoscopic techniques.

Long-Term Complications and Treatment in Patients with Ventriculo-Peritoneal Shunt Surgery

The purpose of treatment hydrocephalus is to maintain intracranial pressure, as well as to create a favorable psychomotor developmental environment, particularly in pediatric cases. Various complications associated with shunt surgery require long-term care. When a shunt is performed for neonates and infants with hydrocephalus, proper management is required thereafter during whole their life. The most common complication that can occur as a result of shunt surgery is obstruction of the ventricular catheter. Ventricular catheter obstruction can be avoided by placing that the tip of the catheter is placed in an appropriate position. A further measure would include preventing the ventricular catheter from being pulled out as the skull expands. The next common complication is obstruction of the peritoneal catheter. The risk of occlusion can be reduced by making that the tip of the peritoneal catheter is open-ended as opposed to having side slits. Isolated ventricles and slit ventricle syndrome are peculiar shunt-related conditions that are difficult to treat. When shunt dysfunction occurs, hydrocephalus can progress slowly, but in many cases the patient's condition deteriorates rapidly and requires immediate medical intervention. Recently, neuroendoscopic surgery has been actively performed for cases of shunt dysfunction and prophylactic replacement of peritoneal catheters, and attempts have been made to remove the shunt device. Shunt infection is another troublesome complication in the management of hydrocephalus and has not yet been resolved. However, if all staff involved in shunt surgery have a common understanding and adopt fixed protocols related to preoperative, intraoperative, and postoperative management, the incidence of infection can be significantly reduced. Furthermore, the development of new and improved catheters may also contribute to a reduction in shunt infection. Neurosurgeons should be cognizant that a delay in the diagnosis and treatment of shunt-related complications in patients with hydrocephalus could result in death. Shunt surgery for the treatment of pediatric hydrocephalus is merely the beginning, as neurosurgeons must be responsible for management until the child reaches adulthood.

Machine learning-based phenotyping and risk assessment of hypertrophic cardiomyopathy - linking ECGs, morphology and genotypes

Abstract Funding Acknowledgements Type of funding sources: None. Background Integrating clinical data to distinguish hypertrophic cardiomyopathy (HCM) phenotypes is relevant in clinical practice. Machine learning (ML) can help - deep learning (DL) networks can automate detection and segmentation of 12-lead electrocardiograms (ECGs), whereas unsupervised learning can group patients to compare ECG, imaging and genetic characteristics. The aim is to automate ECG morphology analysis from all 12 ECG leads and multiple beats, and relate this to HCM genotypes and imaging phenotypes. Methods The single-center cohort included phenotype- and genotype-positive (G+) HCM patients (n = 104) and their phenotype-negative relatives (n = 50, 42% G+). All patients had a digital 12-lead ECG, echocardiography, and a magnetic resonance (CMR) study performed. The workflow is shown in Fig 1. A U-Net DL network was used for ECG delineation (P, QRS, T onsets/offsets) for all cardiac cycles. Three heartbeats were selected for each patient based on their morphology, with the aim of capturing beat-to-beat variability. An unsupervised representation learning algorithm was used to fuse ECG data and assess inter-patient similarities. Patients were clustered based on similarities of ECG biomarkers, and compared with regards to genotypes, family history of sudden cardiac death (SCD), history of ventricular arrhythmias/syncope/aborted SCD, implanted defibrillators (ICD), left ventricular (LV) obstruction, maximal wall thickness, late gadolinium enhancement (LGE), and HCM risk-SCD score. Results ML based on ECG biomarkers provided a good separation of HCM patients and relatives (Fig 1A), also showing G- and patients with variants of uncertain significance grouping together (Fig 1B). Clustering resulted in 6 ECG phenogroups (C1-6). C1 and 2 were related to less comorbidities, cardiac remodeling, and HCM risk score, capturing the majority of G- patients. C3 and 4 were related to LV obstruction – where C4 consisted of symptomatic patients with high ICD implantation and event rates, high LGE, and impaired systolic function. C5 captured patients with high comorbidities, extremely remodeled hearts, but no obstruction, whereas C6 patients with positive family history and high arrhythmic events (Fig 1C, Table 1). The average ECG morphology is shown side-by-side for C1 and C5 in Fig 1D – negative T waves, increased R/S wave amplitudes, left axis deviation (LAD) and ST elevation can be recognized as macro-biomarkers in C5 (yellow arrows). Conclusion ML can automate the analysis of complex clinical data, simultaneously taking into account the morphology of all ECG components in all 12-leads, throughout multiple beats, compare it with clinical and imaging data, and identify clinically sensible phenogroups as validated by structural and functional findings, as well as with genotypes and clinical information. Automated and comprehensive cardiac data analysis has diagnostic and research potential to help screen populations and phenotype disease etiologies. Abstract Figure 1: analysis pipeline Abstract Table 1: clinical variables

Hypertrophic Cardiomyopathy and Atrial Fibrillation: A Review.

Hypertrophic cardiomyopathy (HCM) is an inherited cardiological condition that exhibits various clinical symptoms. The leading cause of atrial fibrillation (AF) in patients with HCM is advanced diastolic dysfunction and left atrial dilatation and remodeling. In addition to the gradual symptomatic and functional decline caused by AF, there is an increased risk of thromboembolic disease and mortality, especially if there is a rapid ventricular rate or obstruction of the left ventricular outflow tract. The mainstay of management of AF in HCM is a combination of non-pharmacological lifestyle and risk factor modification, long-term anticoagulation, and rhythm control with anti-arrhythmic medications, septal ablation, and radiofrequency catheter ablation. This article has examined the development of AF in HCM, its clinical symptomatology, and its impact, highlighting its management and the mortality associated with AF in HCM.

Therapeutic benefits of distal ventricular pacing in mid-cavity obstructive hypertrophic cardiomyopathy

Introduction:Hypertrophic cardiomyopathy (HCM) patients with left ventricular (LV) mid-cavity obstruction (LVMCO) often experience severe drug-refractory symptoms thought to be related to intraventricular obstruction. We tested whether ventricular pacing, guided by invasive haemodynamic assessment, reduced LVMCO and improved refractory symptoms.Methods:Between December 2008 and December 2017, 16 HCM patients with severe refractory symptoms and LVMCO underwent device implantation with haemodynamic pacing study to assess the effect on invasively defined LVMCO gradients. The effect on the gradient of atrioventricular (AV) synchronous pacing from sites including right ventricular (RV) apex and middle cardiac vein (MCV) was retrospectively assessed.Results:Invasive haemodynamic data were available in 14 of 16 patients. Mean pre-treatment intracavitary gradient was 77 ± 22 mmHg (in sinus rhythm) versus 21 ± 21 mmHg during pacing from optimal ventricular site (95% CI: −70.86 to −40.57, p < 0.0001). Optimal pacing site was distal MCV in 12/16 (86%), RV apex in 1/16 and via epicardial LV lead in 1/16. Pre-pacing Doppler-derived gradients were significantly higher than at follow-up (47 ± 15 versus 24 ± 16 mmHg, 95% CI: −37.19 to −13.73, p < 0.001). Median baseline NYHA class was 3, which had improved by ⩾1 NYHA class in 13 of 16 patients at 1-year post-procedure (p < 0.001). The mean follow-up duration was 4.6 ± 2.7 years with the following outcomes: 8/16 (50%) had continued symptomatic improvement, 4/16 had symptomatic decline and 4/16 died. Contributors to symptomatic decline included chronic atrial fibrillation (AF) (n = 5), phrenic nerve stimulation (n = 3) and ventricular ectopy (n = 1).Conclusion:In drug-refractory symptomatic LVMCO, distal ventricular pacing can reduce intracavitary obstruction and may provide long-term symptomatic relief in patients with limited treatment options. A haemodynamic pacing study is an effective strategy for identifying optimal pacing site and configuration.

Ventricular Tachycardia Unveiling an Obstructive Cardiomyopathy Complicated with an Apical Aneurysm

Background: We report the case of a 58-year-old hypertensive patient under treatment who presented with a ventricular tachycardia unveiling an obstructive cardiomyopathy complicated with an apical aneurysm. Aim: Highlight the rarity of the case and the difficulty of management. Case Presentation: This patient was transferred from Regional Hospital of Ziguinchor in southern Senegal for a brutal dizzy spell without loss of consciousness. The electrocardiogram showed a wide monomorphic QRS complex regular tachycardia with a rate of 215 beats/min. An external electrical cardio version at 300 joules was applied which led to the recovery of a sinus rhythm on the electrocardiogram. The Doppler echocardiography showed an asymetricalmedio-ventricular hypertrophy with a maximum left intraventricular gradient at 10 mm Hg at rest. The coronarography via the radial artery was normal. The evolution was labeled with a recurrence of the ventricular tachycardia. The patient was then put on amiodarone 200 mg, beta-blocker (bisoprolol 10 mg) and anti-vitamin K (acenocoumarol 4 mg). Facing rhythmic instability, an implantable automatic defibrillator was fitted. No complication has been reported after one year of evolution. Conclusion: HCM with medio ventricular obstruction and apical aneurysm complicated with ventricular arrhythmias is a rare entity. Its management is difficult and controversial.

Congenital heart defects in molecularly confirmed KBG syndrome patients.

Congenital heart defects (CHDs) are known to occur in 9%-25% of patients with KBG syndrome. In this study we analyzed the prevalence and anatomic types of CHDs in 46 personal patients with KBG syndrome, carrying pathogenetic variants in ANKRD11 or 16q24.3 deletion, and reviewed CHDs in patients with molecular diagnosis of KBG syndrome from the literature. CHD was diagnosed in 15/40 (38%) patients with ANKRD11 variant, and in one patient with 16q24.3 deletion. Left ventricular outflow tract obstructions have been diagnosed in 9/15 (60%), subaortic or muscular ventricular septal defect in 5/15 (33%), dextrocardia in 1/15 (8%). The single patient with 16q24.3 deletion and CHD had complete atrioventricular septal defect (AVSD) with aortic coarctation. Review of KBG patients from the literature and present series showed that septal defects have been diagnosed in 44% (27/61) of the cases, left ventricular tract obstructions in 31% (19/61), AVSD in 18% (11/61). Septal defects have been diagnosed in 78% of total patients with 16q24.3 deletion. Valvar anomalies are frequently diagnosed, prevalently involving the left side of the heart. A distinctive association with AVSD is identifiable and could represent a marker to suggest the diagnosis in younger patients. In conclusion, after precise molecular diagnosis and systematic cardiological screening the prevalence of CHD in KBG syndrome seems to be higher than previously reported in clinical articles. In addition to septal defects, left-sided anomalies and AVSD should be considered. Clinical management of KBG syndrome should include accurate and detailed echocardiogram at time of diagnosis.

587 Exercise induced pulmonary hypertension in hypertrophic cardiomyopathy: a combined cardiopulmonary exercise test-echocardiographic study

Abstract Aims Pulmonary arterial hypertension (PAH) has been described in patients with hypertrophic cardiomyopathy (HCM) and it was associated with a worse prognosis. Nevertheless in most HCM patients, despite normal pulmonary pressures at rest, congestive symptoms are elicited by exercise. In the present study, combining cardiopulmonary exercise test (CPET) with echocardiography, we aimed to evaluate the presence of exercise-induced pulmonary hypertension (EiPAH) its role in functional limitation and its prognostic significance in a cohort of patients with obstructive and non-obstructive HCM. Methods and results 182 HCM patients (35% females, mean age 47.5 ± 15.9) undergoing CPET. During CPET, LVOT velocities and trans-tricuspid gradient were measured. Thirty-seven patients (20%) developed sPAP &amp;gt; 40 mmHg at peak exercise (EiPAH). EiPAH was associated with an lower exercise performance, larger left atrial volumes, higher left ventricular gradient and higher VE/VCO2 slope .At multivariable model baseline sPAP (P &amp;lt; 0.0001) and baseline left ventricular obstruction (LVOT) (P = 0.028) were significantly associated with EiPAH .Kaplan-Meier curve analysis showed EiPAH was a significant predictor of HCM –related morbidity (hazards ratio: 6.21, 95% CI: 1.47–26.19; P = 0.05; 4.21, 95% CI: 1.94–9.12; P &amp;lt; 0.001) for the primary and the secondary endpoint respectively. Conclusions EiPAH was present in about one fifth of HCM patients without evidence of elevated pulmonary pressures at rest, and was associated with adverse clinical outcome. Diagnosing EiPAH by exercise echo/CPET may help physicians to detect early stage of PAH requiring a closer clinical monitoring and individualized treatment strategies.

Extended myectomy for apical hypertrophic cardiomyopathy: a case report

BackgroundApical hypertrophic cardiomyopathy is a variant of hypertrophic cardiomyopathy that predominantly affects the apex of the left ventricle and rarely involves the right ventricular apex or both apexes. Heart transplantation is the traditional treatment for apical hypertrophic cardiomyopathy. Although surgical myectomy approaching the apex has been available for decades, its safety and accuracy greatly depend on the surgeon’s skills and experience.Case presentationThe first case involved a 63-year-old man with apical hypertrophic cardiomyopathy, wherein preoperative contrast computed tomography findings revealed apical hypertrophy and complete apical cavity obliteration. The patient underwent extended myectomy, which revealed the apex cavity filled with abnormal muscles. Using the transaortic approach, the location of the bilateral papillary muscle was confirmed, thereby providing the required orientation. The abnormal muscle mass was successfully resected, and the postoperative end-diastolic volume was extremely increased. The second case involved a 43-year-old man with an apical left ventricular aneurysm and mid-hypertrophic cardiomyopathy obstruction. The thin-walled apical aneurysm contained a large apical-basal band. Upon detecting the bilateral papillary muscle, mid-ventricular myectomy was performed from the apex. During postoperative catheterization, there was no pressure gradient between the left ventricle and aorta.ConclusionsWe reviewed two cases of apical hypertrophic cardiomyopathy, efficiently treated using extended apical myectomy. Although it is an uncommon procedure, the cases presented show how it can be used to successfully manage cases of apical hypertrophic cardiomyopathy. However, it is important to secure the postoperative left ventricular end-diastolic volume.

The first 200 septal myectomies: Ensuring gold standard outcomes.

Septal myectomy remains the gold standard treatment for symptomatic left ventricular outflow tract obstruction refractory to medical treatment. It is recommended that this operation be performed in dedicated hypertrophic obstructive cardiomyopathy centres by experienced surgeons. The septal myectomy option remains unavailable to many patients based solely on geography, including those who would clearly benefit more substantially from surgery than other therapeutic options. Here, we share our experience in starting new hypertrophic cardiomyopathy programmes. We retrospectively reviewed initial septal myectomy experiences at two hypertrophic cardiomyopathy programmes starting in 2014. Two-hundred septal myectomies were performed. Mean age was 58.8 years and 51% were females. Advanced heart failure symptoms were present in 95.5% of patients and 23.5% had experienced syncope. Mean maximal intraventricular gradient was 89 mmHg and 48.5% underwent concomitant procedures at the time of septal myectomy. There was no perioperative (in-hospital or 30 days) mortality. Ninety-two per cent had provoked left ventricular gradients of ≤ 15 mmHg and 97% had none/mild mitral regurgitation at post-operative assessment. In our contemporary cohort, there were 2 (1%) intraoperative ventricular septal defects and 5% required a permanent pacemaker. Our early septal myectomy experience targeted a complex population, frequently in need of concomitant procedures. Abolition of left ventricular obstruction and resolution of systolic anterior motion mediated mitral regurgitation can be expected. The safety and efficacy of septal myectomy carried at hypertrophic cardiomyopathy centres by properly trained surgeons achieved the desired outcomes established by recent hypertrophic cardiomyopathy guidelines.

Caudal Regression Syndrome: A Case Report

Abstract Introduction: Lumbosacral agenesis or caudal regression syndrome (CRS) is a rare congenital malformation that is represented with symmetrical sacrococcygeal or lumbosacrococcygeal agenesis with a varied incidence between 1 per 25000 live birth to 2.5 per 100000 live birth. Additionally, manifold abnormalities may associate CRS e.g. spinal cord malformations, cardiac malformations, lipomyelomeningocele, orthopaedic deformities, renal agenesis and neurogenic bladder, tethered-cord, sacral agenesis, and anorectal atresia Case Presentation: We report a case of a male neonate delivered to a 28-year-old diabetic mother at 38 weeks’ gestation diagnosed with CRS. In this case lumbosacral agenesis, hip dislocation, and club foot deformities along with cardiac abnormalities including, small patent dactus arteriosus (PDA), atrial septal defect (ASD), hypertrophic cardiomyopathy (HCM) without left ventricular outlet obstruction were presented. Conclusions: Having the 200-fold increased relative risk of developing CRS in infants of diabetic mothers in mind, this case report provides evidence that uncontrolled diabetes might increase the chance of CRS in the infants.

Ventricular outflow tract obstruction: An in-silico model to relate the obstruction to hemodynamic quantities in cardiac paediatric patients.

BackgroundRight (R) or left (L) ventricular outflow tract (VOT) obstruction can be either a dynamic phenomenon or a congenital anatomic lesion, which requires a prompt and optimal timing of treatment to avoid a pathological ventricular remodelling.ObjectiveTo develop a simple and reliable numerical tool able to relate the R/L obstruction size with the pressure gradient and the cardiac output. To provide indication of the obstruction severity and be of help in the clinical management of patients and designing the surgical treatment for obstruction mitigation.MethodsBlood flow across the obstruction is described according to the classical theory of one-dimensional flow, with the obstruction uniquely characterized by its size. Hemodynamics of complete circulation is simulated according to the lumped parameter approach. The case of a 2 years-old baby is reproduced, with the occlusion placed in either the R/ or the L/VOT. Conditions from wide open to almost complete obstruction are reproduced.ResultsBoth R/LVOT obstruction in the in-silico model resulted in an increased pressure gradient and a decreased cardiac output, proportional to the severity of the VOT obstruction and dependent on the R/L location of the obstruction itself, as it is clinically observed.ConclusionThe in-silico model of ventricular obstruction which simulates pressure gradient and/or cardiac output agrees with clinical data, and is a first step towards the creation of a tool that can support the clinical management of patients from diagnosis to surgical treatments.

Upright exercise stress echocardiography may unmask dynamic left ventricular obstruction also beyond hypertrophic cardiomyopathy

Abstract Background Dynamic left ventricular outflow obstruction (LVOTO) during exercise stress echocardiography (ESE) is recommended in hypertrophic cardiomyopathy (HCM) to identify the obstructive phenotype. Aim To assess left ventricular outflow gradient (LVOTG) during ESE in different conditions. Methods In a single-group, prospective, observational study, we performed peak and/or post-treadmill ESE with systematic assessment of LVOTG in the orthostatic position by continuous-wave Doppler in 1333 subjects (837 males, mean age 38,2±20 ranging from 6 to 87 years) recruited over a period of twenty years, from 2001 to 2021. Peak LVOTG ≥30 mm Hg was considered abnormal for LVOTO during ESE. We enrolled 7 different populations: asymptomatic healthy controls (n=35); HCM (n=81); genotype-positive, phenotype negative asymptomatic HCM (n=6); patients with chest pain symptoms, suspected myocardial ischemia and either normal coronary arteries (INOCA, n=131,or with very low pre-test probability of coronary artery disease (probable INOCA, n=416) and; fatigue and suspected heart failure with preserved ejection fraction (HFpEF, n=206); amateur athletes with ischemia-like ECG changes during exercise-test or symptoms such as near syncope or chest pain or dizziness (n=457); aborted sudden death and with negative screening (n=1). Results Technical success rate of LVOTG assessment was 1333/1333 at rest and at peak stress (feasibility 100%). Imaging and analysis time were &amp;lt;1 minute. LVOTG at rest was present in 25 pts (2.8%) of the overall population: 23 HCM, 1 INOCA, and 1 HFpEF. Overall prevalence during ESE was 432/1333 (32%). During ESE, LVOTO (see Figure 1 and 2) was 0% (0/35) in normals, 58% (47/81) in HCM (23 with obstruction at rest), 33% (2/6) in genotype-positive, phenotype negative HCM, 37% (33/131) in INOCA, 40% (135/416) in athletes and 1/1 in the patient with aborted sudden death on strenuous exercise. Conclusion LVOTO in orthostatic position is detectable during treadmill ESE in several cardiovascular conditions associated with symptoms such as dyspnea, chest pain or near syncope, and even in asymptomatic patients with genotype-positive, phenotype-negative HCM. The identification of the obstructive phenotype is easy to capture during ESE without any significant additional imaging and analysis burden and can be important also outside HCM. Funding Acknowledgement Type of funding sources: None. Figure 1Figure 2

LEFT VENTRICULAR DYSFUNCTION SECONDARY TO LARGE HIATAL HERNIA COMPLICATED BY GASTRIC OUTLET OBSTRUCTION

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: A hiatal hernia is an anatomical abnormality in which abdominal contents displace into the thoracic cavity through a diaphragmatic defect and can result in GERD, bronchiectasis, and rarely cardiac compression. We describe a case of a large hiatal hernia in an advanced age female in whom the hernia compressed the left ventricle resulting in significant cardiac dysfunction. CASE PRESENTATION: An advanced age female with a past history of diabetes, hypertension, hypothyroidism, and large hiatal hernia repaired in 2017 presented with nausea and bloody/bilious vomiting with food intolerance and was found to have a gastric outlet obstruction. The patient's case was complicated by aspiration pneumonia and cardiac sequelae. Patient exhibited soft pressures and sinus tachycardia in the 120 to 140 range with frequent atrial and ventricular ectopy and a left bundle branch block. Echocardiogram noted that her left ventricle was small and compressed secondary to bowel from the hiatal hernia. Given patient's advanced age, the plan was to treat conservatively with beta blockers. Patient then had rhythmic change to new atrial fibrillation with rapid ventricular response. Ultimately patient had cardiorespiratory compromise secondary to decreased preload from cardiac compression from her large hiatal hernia and expired. DISCUSSION: There are a few case reports of cardiac sequelae such as ST elevation and decreased preload from left atrial and ventricular compression due to large hiatal hernias, especially in the post prandial state in which the mass effect increases. In this patient's case, the patient's mass effect of her large hiatal hernia was exacerbated by her gastric outlet obstruction. She had recurrence of her hiatal hernia despite two repair procedures in the past and given her advanced age, she was a poor surgical candidate for surgery of her hernia. She had conservative management with beta blockers and NG tube placement for gastric decompression, but she ultimately had complicated course with increased ectopy, new arrhythmia, and left ventricular compression caused by mass effect of the massive hernia. CONCLUSIONS: The patient's mass effect of her large hiatal hernia was exacerbated by her gastric outlet obstruction which led to increased ectopy, new arrhythmia, and decreased preload from left ventricular compression. In an advanced age patient with gastric outlet obstruction and a history of large hiatal hernia, the treatment options were limited. In cases of patients who are able to undergo more aggressive management, it is advisable to closely monitor and intervene on large hiatal hernias and relieve the cause of gastric outlet obstructions as they can have significant cardiopulmonary effects secondary to their mass effect as seen in this case. REFERENCE #1: Umemura, A. et al. Cardiopulmonary Impairments Caused by a Large Hiatal Hernia with Organoaxial Gastric Volvulus Showing Upside-Down Stomach: A Case Report. The American journal of case reports, [s. l.], v. 20, p. 1530–1535, 2019. DOI 10.12659/AJCR.918191 REFERENCE #2: Gnanenthiran, S. et al. Feeding Inducses Left Atrial Compression and Impedes Cardiac Filling in Patients with Large Haital Hernia. Journal of the American Society of Echocardiography. V.32, iss. 3, p. 375-384, 2019. DOI 10.1016/j.echo.2018.09.017 REFERENCE #3: Harada, K. et al. Left ventricular obstruction caused by a large hiatal hernia. Echocardiography (Mount Kisco, N.Y.), [s. l.], v. 34, n. 8, p. 1254–1256, 2017. DOI 10.1111/echo.13563. Disponível em: https://search-ebscohost-com.ezproxy.libraries.wright.edu/login.aspx?direct=true&db=mnh&AN=28497506&site=eds-live. Acesso em: 11 set. 2020. DISCLOSURES: No relevant relationships by Anas Ahmed, source=Web Response No relevant relationships by Amie Kim, source=Web Response No relevant relationships by Steven Young, source=Web Response

Echocardiographic Profile of Hypertrophic Cardiomyopathy – A Single-Centre, Observational study

Background: Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease of left ventricular hypertrophy (LVH). Phenotypic expression varies widely from subclinical hypertrophy to gross asymmetric septal hypertrophy causing left ventricular outflow tract (LVOT) obstruction. On top of genetic and phenotypic heterogeneity, the prevalence of different types of HCM may have geographical, as well as, ethnic variation. Methods: This observational study was carried out during 2010 to 2020 to determine the echocardiographic profile of HCM in Bangladeshi population. All patients undergoing transthoracic echocardiography (TTE) in a private consultation centre of Dhaka, Bangladesh were included. HCM was defined as the presence of a maximal end-diastolic wall thickness of e”15 mm anywhere in the left ventricle (LV), in the absence of another cause of hypertrophy in adults. HCM was further classified according to the pattern of myocardial hypertrophy and presence or absence of LVOT, or mid-left ventricular cavity obstruction. Results: Out of 76 cases, non-obstructive HCM was the commonest type (65.8%), followed by HCM causing LVOT obstruction (13.2%), HCM causing mid-LV cavity obstruction (10.5%), and the apical variety ( 10.5%). Asymmetric septal hypertrophy (ASH) was found in 42.1%, systolic anterior motion (SAM) of anterior mitral leaflet (AML) in 14.5%, mitral regurgitation (MR) in 50%, left ventricular systolic dysfunction in 5.3%, and raised pulmonary artery systolic pressure (PASP) in 15.8% of cases. Maximum LV wall thickness ≥30 mm was found in 66 out of 76 cases. Conclusion: The study highlights the clinically useful profile of HCM in Bangladeshi population based on conventional echocardiography. Further studies involving clinical, newer echocardiographic modalities and genetic analyses are warranted to discover the additional information in this ethnicity. Cardiovasc j 2021; 14(1): 5-11

Use of transesophageal echocardiography for transcatheter valve-in-valve implantation for patients with prior bioprosthetic surgical aortic, mitral, tricuspid, and pulmonic valves.

Valve-in-valve (ViV) transcatheter procedures are the preferred option for redo valve replacement in patients who otherwise would be high risk for surgery. Transesophageal echocardiography (TEE) is an integral imaging modality for both peri-procedural and intra-procedural guidance during transcatheter ViV replacement. When intentional leaflet laceration is needed, such as with the BASILICA (Bioprosthetic or native Aortic Scallop Intentional Laceration to prevent Iatrogenic Coronary Artery obstructions during TAVR) or LAMPOON (Laceration of the Anterior Mitral valve leaflet to Prevent left ventricular Outlet ObstructioN) procedures, TEE is critical to proper guidewire positioning and achieving a successful laceration. In this paper we detail the role of TEE in ViV transcatheter valve replacement in patients with prior surgical aortic, mitral, tricuspid, and pulmonic valves.