Motion Of Mitral Leaflet Research Articles

Impact of Sinus Rhythm Restoration on Mitral Regurgitation in Patients With Atrial Fibrillation: A Comparative Echocardiographic Study.

Mitral regurgitation (MR) is a common valvular abnormality that can be exacerbated by atrial fibrillation (AF). Mitral regurgitation is classified based on mitral leaflet motion and can be either primary (organic) or secondary (functional). This study investigates the relationship between AF and functional MR, specifically assessing whether cardioversion to sinus rhythm influences MR severity and echocardiographic indices. This retrospective cohort study included 417 patients with AF and significant MR (Grade III or IV) who were hospitalized for cardioversion. Patients underwent echocardiography during AF and again within six months after successful cardioversion. The primary outcome was the change in MR severity post cardioversion. Secondary outcomes included changes in left atrial size, left ventricular ejection fraction, and mitral valve anatomy. Statistical analysis involved chi-square tests for categorical variables, Student's t-tests for continuous variables, and multivariate logistic regression to assess the impact of sinus rhythm restoration on MR severity. Of the 417 patients, 61% (n = 254) returned to sinus rhythm after cardioversion. Among these, 28.4% (n = 72) showed a two-grade or greater improvement in MR severity. Patients who returned to sinus rhythm also exhibited significant reductions in left ventricular and left atrial dimensions and improved transvalvular gradients. The multivariate analysis indicated that conversion to sinus rhythm was independently associated with MR improvement. Restoration of sinus rhythm is associated with significant improvement in functional MR, potentially reducing the need for surgical intervention. These findings suggest that rhythm control should be considered in managing patients with AF and significant MR, as it may lead to improved cardiac function and better clinical outcomes. Further large-scale prospective studies are needed to determine the most effective management strategy for functional MR in the context of AF.

Atrial Functional Mitral Regurgitation Subtypes Undergoing Transcatheter Edge-to-Edge Repair: Suboptimal Outcomes in Atriogenic Hamstringing.

Two subtypes of atrial functional mitral regurgitation (AFMR) have been described, one is characterized by Carpentier type I and the other by Carpentier type IIIb leaflet motion. The authors sought to analyze echocardiographic characteristics and outcomes of AFMR subtypes undergoing mitral valve transcatheter edge-to-edge repair (M-TEER). Of 1,047 consecutive patients who underwent M-TEER, the authors identified those with isolated mitral annulus dilation (Carpentier I), termed AFMR-IAD, and those with atriogenic hamstringing characterized by restricted posterior mitral leaflet motion (Carpentier IIIb), termed AFMR-AH. Echocardiographic baseline characteristics and outcomes up to 1-year were analyzed. A total of 128 patients (12.2%) met AFMR criteria; 75 (58.6%) were identified as AFMR-IAD and 53 (41.4%) as AFMR-AH. AFMR-AH displayed greater left atrial and left ventricular volumes, greater mitral annulus, shorter and steeper posterior mitral leaflet, and more pronounced MR (all P< 0.05). Technical success was achieved in 98.7% (AFMR-IAD) and 86.8% (AFMR-AH) of patients (P = 0.009). At discharge, device detachments were exclusively observed in AFMR-AH (10.0%). MR≤II was achieved in 95.6% and 78.6% at 30days (P=0.009) and in 93.0% and 74.1% at 1 year (P = 0.038) in patients with AFMR-IAD and AFMR-AH, respectively. AFMR-AH was associated with procedural failure (OR: 1.17 [95%CI: 1.00-1.38]; P = 0.045) at 30days (43.4% vs 24.0%; P = 0.023) and all-cause mortality (HR: 2.54 [95%CI: 1.09-5.91]; P = 0.031) at 1 year (77% vs 92%, Kaplan-Meier estimated 1-year survival; P = 0.017). AFMR-AH shows worse procedural and clinical outcomes following M-TEER than AFMR-IAD. Thus, vigilance regarding this pathology is warranted and alternative mitral valve therapies might need to be considered.

SCARY ECG IN TAKOTSUBO CARDIOMYOPATHY

Abstract Takotsubo cardiomyopathy (TTC) is characterized by a transient ventricular wall motion abnormality and shares common features with acute coronary syndrome (ACS) with a wide spectrum of triggers, such as both emotional or physical, activating common toxic catecholamines–mediated pathways. TTC might be associated with severe clinical complications. We report a case of a 74–year female patient with circulatory shock with mixed phenotype, distributive due to sepsis and cardiogenic due to TTC. The patient presented to the emergency department with severe hypotension, pathological qSOFA score and clinical and laboratory findings consistent with severe sepsis. ECG showed diffuse lambda ST–segment elevation, also called “shark fin”(fig.1). According to hemodynamically unstable scenario and ECG pattern, emergency coronary angiography was performed, showing absence of coronary artery disease(fig. 2). Echocardiography revealed severe LV systolic disfunction (EF 20–25%) with apical ballooning and severe systolic anterior motion (SAM) of the anterior mitral leaflet eliciting dynamic LVOTO with peak gradient greater than 90 mmHg; fig. 3) and severe MR. Patient was treated with cautious fluid administration, hemotrasfusion and empiric large spectrum antibiotic therapy. According to TTC working diagnosis, even if septic state was thought to contribute to cardiogenic shock, norepinephrine was not administered due to concerns for LVOTO and SAM. After overcoming of dramatic first phase and after hemodynamic steadiness was achieved, beta blockade and ACEi were progressively administered. Seriate transthoracic echocardiogram revealed progressive improvement of LV systolic function, regression of apical ballooning, SAM and LVOTO. ECG monitoring showed regression of ischemic ST elevation. ST elevation has been described in about 56% of TTC cases, however with amplitude tipically lower than in STEMI. Lambda–wave ST elevation has been rarely reported. Vasospasm, local intermittent microvascular constriction, catecholamine–induced metabolic toxicity, transmural systolic wall tension and its the mechano–electric feedback can explain scaring ECG–pattern. Despite its rare prevalence in TTC “shark fin” pattern appears to relate to adverse outcome and it should be used as an early marker of potential further clinical deterioration, suggesting more intensive monitoring to avoid delays in possible therapy escalation.

Comparison of cardiac amyloidosis and hypertrophic cardiomyopathy: retrospective analysis of cardiac and kidney lesion

Cardiac amyloidosis performance is in many ways similar to hypertrophic cardiomyopathy (HCM), and therefore it is extremely difficult to distinguish these two diseases in practice. The present study was performed to identify features that aid in the differential diagnosis by analyzing clinical, electrocardiographic, echocardiographic and laboratory data in patients with systemic amyloidosis and HCM. In the study, 38 patients with cardiac amyloidosis and 80 patients with HCM were examined. It was found that symmetrical left ventricular (LV) wall thickening, granular and sparkling appearance of the myocardium and thickened heart valves were the best cardiac predictors, and age above 63 years, decreased glomerular filtration rate and proteinuria were the best non-cardiac predictors of cardiac amyloidosis, whereas low QRS voltage and pericardial effusion were of less clinical significance. Systolic anterior motion of the anterior mitral leaflet, asymmetric LV wall thickening, and electrocardiographic signs of LV hypertrophy were highly suggestive of HCM. Thus, standard noninvasive methods may be useful for identifying myocardial infiltrative processes and differentiating cardiac amyloidosis from HCM.

Early outcomes of thoracoscopic trans-mitral myectomy in obstructive hypertrophic cardiomyopathy patients with left midventricular obstruction

Objective: To examine the early effect of thoracoscopic trans-mitral myectomy for hypertrophic cardiomyopathy patients with left midventricular obstruction. Methods: From April 2020 to July 2021, 10 hypertrophic cardiomyopathy patients with left midventricular obstruction underwent thoracoscopic trans-mitral myectomy at Guangdong Provincial People's Hospital. The whole group of patients consisted of 7 males and 3 females aged (52.0±16.4) years (range: 18 to 68 years). The EuroSCORE Ⅱ predicted mortality rate was 1.78% (1.20%) (M(IQR)) (range: 0.96% to 4.86%). The clinical data were collected and analyzed retrospectively to evaluate the clinical efficacy by comparing preoperative and postoperative echocardiographic parameters using paired t-test, paired Wilcoxon test or Fisher exact test, including left ventricular outflow tract peak pressure gradient, maximum interventricular septum thickness, systolic anterior motion of the anterior mitral leaflet and so on. The safety was determined by summarizing the incidence of perioperative and follow-up complications. Results: All the procedures successed with no conversion to median sternotomy, septal defect, ventricular rupture. There was no in-hospital 30-day death, neither serious complications like permanent pacemaker implantation, re-sternotomy for bleeding, low cardiac output syndrome, stroke, or multiple organ dysfunction syndrome. The left ventricular outflow tract obstruction was effectively relieved in all patients expect a patient developed residual obstruction. Compared with that of pre-operation, the thickness of the interventricular septum was significantly reduced from (22.1±4.0) mm to (10.3±1.7) mm (t=10.693, P<0.01), while the left ventricular outflow tract peak pressure gradient was significantly reduced from (81.7±21.1) mmHg to 12.3 (11.5) mmHg (Z=-2.805, P<0.01) (1 mmHg=0.133 kPa). Conclusion: Thoracoscopic trans-mitral myectomy is an effective and safe procedure for hypertrophic cardiomyopathy patients with left midventricular obstruction.

Hypertrophic Obstructive Cardiomyopathy Associated with Moderate Aortic Stenosis in Symptomatic 75-Year-Old Female: A Case Report

A 72-year-old female was found to have symptomatic hypertrophic obstructive cardiomyopathy associated with significant aortic stenosis. She was found to have severe left ventricular outflow tract obstruction, asymmetric septal hypertrophy, significant systolic anterior motion of the anterior mitral valve leaflet, and mitral regurgitation. She was also found to have moderate aortic stenosis that made the obstruction even worse which made the patient progressively symptomatic not responding to medical therapy. She eventually underwent cardiac surgery in form of left ventricular septal myomectomy and bioprosthetic aortic valve replacement (tissue AVR) with a size 23 tissue valve, aortic root enlargement with bovine pericardial patch. She is currently stable on optimal medical therapy, feels much better and stable cardiac-wise with much better quality of life.

Effect analysis of myectomy guided by personalized three-dimensional reconstruction and printing in the treatment of obstructive hypertrophic cardiomyopathy

Objective: To examine the clinical efficacy of myectomy guided by personalized three-dimensional reconstruction and printing for patients with obstructive hypertrophic cardiomyopathy. Methods: The clinical data of 28 patients with obstructive hypertrophic cardiomyopathy, who underwent septal myectomy guided by personalized three-dimensional reconstruction and printing in the Department of Cardiaovascular Surgery, Guangdong Provincial People's Hospital from May 2020 to December 2021, were retrospectively analyzed. There were 14 males and 14 females, aging (51.1±14.0) years (range: 18 to 72 years). Enhanced cardiac computed tomography images were imported into Mimics software for preoperative three-dimensional reconstruction. The direction of the short axial plane of each segment was marked perpendicularly to the interventricular septum on the long axial plane of the digital cardiac model, then the thickness was measured on each short axial plane. A figurative digital model was used to determine the extent of resection and to visualize mitral valve and papillary muscle abnormalities. Correlation between the length, width, thickness, and volume of the predicted resected myocardium and those of the surgically resected myocardium was assessed by Pearson correlation analysis or Spearman correlation analysis. The accuracy of detecting mitral valve and papillary muscle abnormalities of transthoracic echocardiography and three-dimensional reconstruction was also compared. Results: There was no death or serious complications like permanent pacemaker implantation, re-sternotomy for bleeding, low cardiac output syndrome, stroke, or multiple organ dysfunction syndromes in the whole group. Namely, the obstruction of the left ventricular outflow tract was effectively relieved. The systolic anterior motion of the anterior mitral valve leaflet was absent in all patients after myectomy. The length, width, and thickness of the predicted resected myocardium by three-dimensional reconstruction were significantly positively correlated with the length (R=0.65, 95%CI: 0.37 to 0.82, P<0.01), width (R=0.39, 95%CI: 0.02 to 0.67, P<0.01), and thickness (R=0.82, 95%CI: 0.65 to 0.92, P<0.01) of the surgically resected myocardium, while the relation of the volume of the predicted resected myocardium and the volume of the surgically resected myocardium was a strong positive correlation (R=0.88, 95%CI: 0.76 to 0.94, P<0.01). Importantly, the interventricular septal myocardial thickness measured by preoperative transthoracic echocardiography showed a moderate positive correlation with the volume of surgically resected myocardium (R=0.52, 95%CI: 0.19 to 0.75, P<0.01). During a follow-up of (14.4±6.8) months (range: 3 to 22 months), no death occurred, and 1 patient was readmitted for endocardial radiofrequency ablation due to atrial fibrillation. Conclusion: Personalized three-dimensional reconstruction and printing can not only visualize the intracardiac structure but also guide septal myectomy by predicting the thickness, volume, and extent of resected myocardium to achieve ideal resection.

Prevalence and Prognosis of Atenolol-Responsive Systolic Anterior Motion of the Septal Mitral Valve Leaflet in Young Cats with Severe Dynamic Left Ventricular Outflow Tract Obstruction.

Severe dynamic left ventricular outflow tract obstruction (DLVOTO) secondary to the systolic anterior motion of the septal mitral valve leaflet (SAM) can result either from congenital mitral valve disorders or left ventricular concentric hypertrophy of any cause, in cats commonly hypertrophic cardiomyopathy (HCM). Though HCM cannot be reversed, the question remains whether atenolol can cure cats with severe DLVOTO resulting from a presumed mitral valve dysplasia. In this retrospective case series, client-owned asymptomatic cats younger than 1.5 years with echocardiographic evidence of SAM and severe DLVOTO were included. Oral atenolol therapy and recheck echocardiography after 2-3 months were recommended. The owners and referring veterinarians were called for long-term follow-up information. Of the 28 included cats, 23 were treated with atenolol. Recheck echocardiography performed in 17 cats showed a resolution of SAM in 47%. In the long term, SAM remained absent in only 9% of the treated cats. Cardiac-related death occurred in 26% of the atenolol-treated cats. The long term benefit of twice-daily atenolol therapy was documented in 9% of cats. Whether the cats where atenolol failed to resolve DLVOTO on long-term had HCM, or a therapy-resistant congenital primary mitral valve disorder remains unclear.

Abstract 11157: A Rare Case of Concomitant Dual Genetic Cardiomyopathies in a Patient Complicated by Stroke

Background: Very little evidence exists on the prevalence of co-existing cardiomyopathies. We report in this case a highly atypical presentation of concurrent arrhythmogenic right ventricular cardiomyopathy (ARVC) and hypertrophic cardiomyopathy (HCM) likely attributable to simultaneous separate somatic mutations, complicated by stroke. Case: A 60-year-old male with a history of rheumatoid arthritis, sleep apnoea and obesity with no significant coronary disease referred for review of asymptomatic ECG abnormalities was found to have severe asymmetrical left ventricular (LV) wall thickening with apical segment akinesis and an LV apex mass on echocardiogram. She had a family history of early sudden cardiac death, with an otherwise unremarkable history and physical examination. Clinical Course: Cardiac MRI (CMR) subsequently demonstrated aneurysmal dilatation of LV apical segments with a 17x13mm thrombus present. Systolic anterior mitral leaflet motion was seen, and a dilated right ventricle (RV) with 240ml end-diastolic volume and 27% ejection fraction. "Accordion" sign was present in the sub-tricuspid RV free wall with dyskinesia, and ECGs confirmed presence of epsilon waves. One week after CMR, she suffered a large left middle cerebral artery (MCA) stroke in the setting of warfarin cessation due to echocardiography-suggestive absence of thrombus. Discussion: Genetic analysis revealed a mutated Plakophillin 2 (PKP2) gene, and FHOD3 gene associated with HCM of uncertain significance. The diagnosis was concomitant genetic cardiomyopathies of ARVC and HCM complicated by large LV apical aneurysm and thrombus culminating in left MCA stroke. PKP2 mutations to date have not been associated with hypertrophic phenotypes. This case therefore demonstrates the unlikely 1 in 2,500 incidence of coexistence of two separate cardiomyopathies likely attributable to simultaneous separate somatic mutations, while also highlighting the central role of CMR in investigation and diagnosis of RV pathology. In addition to this case, prior studies have also shown echocardiography can only identify 50% of ARVC cases identified by CMR, suggesting that selective use of CMR may double the number of identified cases of this life-threatening disease.

Unexpected systolic anterior motion of the mitral valve-related hypoxemia during transurethral resection of the prostate under spinal anesthesia: a case report

BackgroundDynamic obstruction of the left ventricular outflow tract resulting from systolic anterior motion of the mitral valve can be an unexpected cause of acute and severe perioperative hypotension in noncardiac surgery. We report a patient undergoing spinal anesthesia for transurethral resection of the prostate who experienced sudden hypoxemia caused by systolic anterior motion-induced mitral regurgitation but with a clinically picture simulating fluid overload.Case presentationAn 83-year-old man with a history of hypertension was scheduled for transurethral resection of the prostate. One hour after spinal anesthesia, he developed acute restlessness and dyspnea, with pink frothy sputum and progressive hypoxemia. Slight hypertension was noted, and an electrocardiogram showed atrial fibrillation with a rapid ventricular response. Furosemide and nitroglycerin were thus administered for suspected fluid overload or transurethral resection of the prostate syndrome; however, he then became severely hypotensive. After tracheal intubation, intraoperative transesophageal echocardiography was promptly performed, which revealed an empty hypercontractile left ventricle, significant mitral regurgitation and mosaic flow signal in the left ventricular outflow tract. Following aggressive fluid therapy, his hemodynamic changes stabilized. Repeat echocardiography in intensive care unit confirmed the presence of systolic anterior motion of the anterior mitral leaflet obstructing the left ventricular outflow tract. We speculate that pulmonary edema was induced by systolic anterior motion-associated mitral regurgitation and rapid atrial fibrillation, and the initial management had worsened his hypovolemia and provoked left ventricular outflow tract obstruction and hemodynamic instability.ConclusionsPulmonary edema caused by systolic anterior motion of the mitral valve can be difficult to clinically differentiate from that induced by fluid overload. Therefore, bedside echocardiography is paramount for timely diagnosis and prompt initiation of appropriate therapy in the perioperative care setting.

Evaluation of an Innovative Device for Mitral Valve Regurgitation: Experimental Acute In Vivo Results.

Objective: Currently, mitral prosthetic rings are intended only to reshape the annulus. We present in vivo results of an innovative device characterized by an intraventricular segment designed to enable artificial chordae implantation and simplify leaflets and subvalvular apparatus correction. Methods: Eight sheep were employed. The first 4 underwent solely device implantation. In the last 4, primary chordae of the anterior leaflet (A2) were torn to induce severe mitral regurgitation. The severed chordae were replaced by 2 pairs of 5-0 Gore-Tex artificial chordae previously measured and anchored to the device bridge. Ease of device and chordae implantation were evaluated, and postprocedural valve competence was verified by postoperative echocardiogram. Results: The procedure was completed in all 8 sheep. In the 4 sheep with induced severe mitral regurgitation, repair could be achieved by means of artificial chordae implantation. Length of the 2 chordae implanted was 21.6 ± 2 mm and 22 ± 3 mm, respectively. The time required to suture the artificial chordae was 2.5 ± 1.2 min. Postoperative echocardiograms showed normal left ventricular ejection fraction and free motion of the mitral leaflets. Mitral regurgitation was absent in 5 cases and trivial in 3. The transvalvular peak pressure gradient was 9.5 ± 6 mm Hg, and mean gradient was 3.7 ± 4 mm Hg. Postprocedural evaluation of the heart and mitral valve showed no damage to the left ventricle wall, valve leaflets, chordae, and papillary muscles. Conclusions: In vivo tests confirm safety of the device, ease of chordal length estimation prior to implantation, short operative time, and no negative impact of the device on mitral leaflet motion, function, and structure.

Surgical Management for Systolic Anterior Motion (SAM) of the Mitral Valve in Obstructive Hypertrophic Myopathy.

In patients with obstructive hypertrophic cardiomyopathy, left ventricular outflow tract (LVOT) obstruction can be created by the hypertrophic interventricular septum (IVS) as well as systolic anterior motion (SAM) of the anterior mitral leaflet (AML). Sufficient septal myectomy is a fundamental surgical technique to treat LVOT obstruction, however, direct surgical management for SAM is another key aspect. Besides the hypertrophic IVS, mitral valve, subvalvular apparatus, and papillary muscle may play important role for SAM and several surgical techniques have been proposed to treat SAM in literature. In this review, each surgical technique is classified by the anatomical structure on which the surgical procedure is applied. The AML is the main surgical site and is applied with plication (vertical plication, resection–plication–release strategy), extension (the AML extension, transverse incision of the AML), sutured (edge-to-edge repair, anterior leaflet retention plasty), or traction (floating stitch, papillary muscle-to-anterior annulus stitches, paradoxical stitches, transposition of a directed chorda tendinea to the AML). Height reduction of the posterior mitral valve leaflet and papillary muscle reorientation are other techniques. We should understand theoretical aspects of each technique on correction of anatomical and functional abnormalities of the structure and should apply them under proper indication.

Automatic morphological classification of mitral valve diseases in echocardiographic images based on explainable deep learning methods.

Carpentier's functional classification is a guide to explain the types of mitral valve regurgitation based on morphological features. There are four types of pathological morphologies, regardless of the presence or absence of mitral regurgitation: Type I, normal; Type II, mitral valve prolapse; Type IIIa, mitral valve stenosis; and Type IIIb, restricted mitral leaflet motion. The aim of this study was to automatically classify mitral valves using echocardiographic images. In our procedure, after the classification of apical 4-chamber (A4C) and parasternal long-axis (PLA) views, we extracted the systolic/diastolic phase of the cardiac cycle by calculating the left ventricular area. Six typical pre-trained models were fine-tuned with a 4-class model for the PLA and a 3-class model for the A4C views. As an additional contribution, to provide explainability, we applied the Gradient-weighted Class Activation Mapping (Grad-CAM) algorithm to visualize areas of echocardiographic images where the different models generated a prediction. This approach conferred a proper understanding of where various networks "look" into echocardiographic images to predict the four types of pathological mitral valve morphologies. Considering the accuracy metric and Grad-CAM maps and by applying the Inception-ResNet-v2 architecture to classify Type II in the PLA view and ResNeXt50 architecture to classify the other three classes in the A4C view, we achieved an 80% rate of model accuracy in the test data set. We suggest an explainable, fully automated, and rule-based procedure to classify the four types of mitral valve morphologies based on Carpentier's functional classification using deep learning on transthoracic echocardiographic images. Our study results infer the feasibility of the use of deep learning models to prepare quick and precise assessments of mitral valve morphologies in echocardiograms. According to our knowledge, our study is the first one that provides a public data set regarding the Carpentier classification of MV pathologies.

675 A rare case of tako-tsubo syndrome in a patient with hypertrophic cardiomyopathy

Abstract Aims We know that basal septal hypertrophy is a rare and unique anatomical finding associated with hypertrophic cardiomyopathy (HCM). Tako-Tsubo cardiomyopathy (TTC) is a transient left ventricular systolic dysfunction induced by high physical or emotional stress. Its occurrence with HCM is unusual. However, this presentation occurs more often with the classic asymmetrical septal hypertrophy compared with the apical variant. This case demonstrates that the coexistence of TTC with septal HCM in an elderly patient may lead to a severe haemodynamic instability picture. Methods and results A 81-year-old female presented to the emergency department (ED) complaining of dyspnoea and chest pain lasting for 1 day. She had hypertension and dyslipidemia associated with a familial history of sudden death. On physical exam, we found a severe hypotension (systolic blood pressure of 80 mmHg) associated with bilateral rales at chest auscultation. Cardiac auscultation revealed a harsh systolic murmur, best heard over the left sternal border. Heart rate was 60 b.p.m. in sinus rhythm. Labs were significant for HS-I troponin of 6.035 ng/L (NV: ≤ 12) and NT-proBNP of 7.640 pg/ml (NV: ≤1800). A 12-leads electrocardiogram (ECG) at admission revealed a STEMI-like ST segment elevation from V2 to V6 (Figure 1A). For this reason she was urgently taken to the cath-lab where she was found to have tortuous but normal coronary arteries. After coronary angiography, a trans-thoracic echocardiogram (TTE) revealed a pathological LV hypertrophy with a septal diastolic thickness of 19 mm, depressed LV ejection fraction (LVEF) due to a severe apical ballooning. At continuous wave (CW)-Doppler there was a dynamic obstruction across the LV outflow tract (LVOT), with a late peak velocity of 4.9 m/s and an estimated peak gradient of 98 mmHg. The gradient was increased by a systolic anterior motion (SAM) of anterior mitral leaflet causing a moderate mitral regurgitation (MR). All these findings were consistent with obstructive septal HCM associated with Takotsubo cardiomyopathy. After treatment with intravenous diuretics and metoprolol (5 + 5 mg i.v. bolus followed by oral dose of 100 mg daily), her clinical condition markedly improved. One week later, ECG demonstrated deeply inverted T waves on antero-lateral leads and QT prolongation (501 ms). Three weeks later, after a complete resolution of the LV apical dyskinesia, LVEF normalized. LVOT gradient decreased to 20 mmHg, with a dynamic increase to 70 mmHg during Valsalva manoeuvre. Conclusions It is well known that TTC may be complicated by a reversible LVOT obstruction by itself but the combination with obstructive HCM can lead to low cardiac output and acute heart failure. This combination has been found to be not common and the correct treatment of this unusual type of cardiogenic shock is still unclear. Careful initial evaluation and continuous monitoring must be warranted in such rare cases. Supportive care afterward with beta blockers, along with echocardiogram surveillance, are the mainstay of management. Cardiologists, intensivisits, and clinicians alike need to recognize and comprehend the pathophysiology behind this unique clinical manifestation so that they may adjust their management and treatment accordingly.

Abstract 11693: When a Parachute Fails: A Rocky Landing to Heart Transplant

A 71 year-old man with heart failure, porcelain aorta, and mitral regurgitation presented for heart transplant evaluation. He was in cardiogenic shock requiring inotropic support. An echocardiogram showed a dilated left ventricle, severely reduced systolic function, restricted mitral valve leaflet motion, an elevated mean transmitral gradient, and severe mitral regurgitation. The differential of mixed stenotic and regurgitant mitral valve pathology included rheumatic heart disease and congenital mitral valve anomaly. Hemodynamic management of cardiogenic shock in the setting of mixed mitral valve disease presented unique challenges due to a narrow therapeutic window of optimal preload, afterload reduction, inotropic support, and chronotropic control. He was not a candidate for percutaneous mitral valve interventions or surgery. A porcelain aorta made him high risk for vascular complications with mechanical circulatory support. His course was complicated by worsening shock requiring escalation of inotropes, rapid atrial fibrillation, and recurrent episodes of acute pulmonary edema. Ultimately, he underwent an orthotropic heart transplant. A transesophageal echocardiogram at the time of transplant suggested a parachute mitral valve, where all chordae appeared be originating from one papillary muscle. However, additional views revealed the presence of two papillary muscles with asymmetric, shortened anterior chordae and a hypertrophied anterior papillary muscle. Findings were consistent with a rare parachute-like asymmetric mitral valve complicated by mild mitral stenosis and severe mitral regurgitation. Examination of the explanted native heart confirmed these findings.This case highlights: 1) complex management of mixed valvular disease in the setting of cardiogenic shock, 2) echocardiographic evaluation of mixed valvular heart disease, and 3) a rare congenital malformation of a parachute-like mitral valve presenting in adulthood.

Cardiac Ultrasound for Pediatric Emergencies.

Cardiac point-of-care ultrasound (POCUS) provides real-time views of the heart to answer specific questions in a timely manner. This is a valuable tool for managing pediatric patients, from those with congenital heart disease to those who are critically ill. The main echocardiographic findings of pericardial tamponade consist of a pericardial effusion, diastolic right ventricular collapse, systolic right atrial collapse, and a plethoric inferior vena cava with minimal respiratory variation. The main echocardiographic findings of hypertrophic cardiomyopathy consist of increased wall thickness (concentric or eccentric), systolic anterior motion of the anterior mitral leaflet, and a dynamic sub-aortic left ventricular outflow tract obstruction. Additional uses of cardiac POCUS include assessment of dilated cardiomyopathy and the detection of pediatric congenital heart disease, including detection of a patent ductus arteriosus. The use of POCUS in the pediatric population is supported by societal position statements and is expected to develop further with increasingly robust education and training. [Pediatr Ann. 2021;50(10):e424-e431.].

SILENT GIANT LEFT ATRIUM

TOPIC: Cardiovascular Disease TYPE: Global Case Reports INTRODUCTION: Enlarged LA is a common finding in severe mitral stenosis(MS) and severe mitral regurgitation(MR). It can enlarge to giant proportions. The unusual finding in a thirty-year-old woman presenting with only mild dyspnea despite a hugely dilated LA prompted us to report this case CASE PRESENTATION: A thirty-year-old woman presented to the outdoor clinic with the complaint of dyspnea on exertion NYHA class I. Aside from mild shortness of breath during the past year, she had been asymptomatic all her life. The admission electrocardiogram showed atrial flutter with the variable block while her chest roentgenogram showed marked cardiac enlargement. The right heart border showed a double contour of atrial shadow (Fig.2). An echocardiogram showed a giant left atrium measuring 14.4 X 16.7 cm that encroached on the other cardiac chambers (Fig.3). Thickened mitral valves along with doming and restricted motion of anterior and posterior mitral leaflets were diagnostic of rheumatic heart disease(RHD) It was surprising to find a relatively asymptomatic patient despite the marked distortion of cardiac anatomy. DISCUSSION: Giant LA occurs due to many reasons but most commonly its result from long-standing rheumatic mitral valve regurgitation or mixed mitral valve disease with predominant regurgitation. Two factors implicated in its development are increased LA pressure and weakening of the LA wall by Rheumatic Pancarditis. When mitral valve disease persists for long, it leads to LA enlargement as a compensatory mechanism to ease the markedly increased LA pressure which could lead to pulmonary Congestion due to backpressure hemodynamic and hence protect from the development of pulmonary hypertension. These compensatory mechanisms help patients survive asymptomatically for a long time. Giant LA can lead to various complications including thromboembolism, pulmonary edema, pulmonary hypertension and it can compress the esophagus and airway causing Dysphagia. CONCLUSIONS: Progressive LA enlargement might be undetected and well-tolerated for a long time, even when it reaches massive proportions. When the LA enlarges to a gigantic size, a chest x-ray is insufficient to delineate the cause of cardiomegaly and even can be misleading. An echocardiographic study is the most useful method for evaluating these patients. Our case was unusual because the giant LA distorted the cardiac structures. This led to striking cardiomegaly on the chest x-ray, and it confused interpreting the cause of the enlarged cardiac silhouette. The echocardiogram was very helpful in demonstrating the severe MS and the giant LA. There is a poor correlation between the size of the left atrium and the severity of the MS. REFERENCE #1: Kawazoe K, Beppu S, Takahara Y, Nakajima N, Tanaka K, Ichinashi K, Fujita T, Manabe H: Surgical treatment of giant left atrium combined with mitral valvular disease. Plication procedure for reduction of compression to the left ventricle, bronchus, and pulmonary parenchyma. J Thorac Cardiovasc Surg 1983, 85: 885-892. REFERENCE #2: Hurst W: Memories of patients with a giant left atrium. Circulation2001, 104: 2630-2631. 10.1161/hc4701.100775 REFERENCE #3: Plaschkes J, Broman JB, Mercin G, Milwidsky H: Giant left atrium in rheumatic heart disease: a report of18 cases treated by mitral valve replacement. Ann Surg 1971, 174: 194-201. 10.1097/00000658-197108000-00004 DISCLOSURES: No relevant relationships by Vikash Jaiswal, source=Web Response no disclosure submitted for Akshyaya Pradhan; No relevant relationships by Prachi Sharma, source=Web Response No relevant relationships by Shweta Vohra, source=Web Response

ANALYSIS OF ECHOCARDIOGRAPHIC AND MRI ANATOMICAL CHARACTERISTICS AND HEMODYNAMIC CHARACTERISTICS OF LEFT VENTRICULAR OUTFLOW TRACT STENOSIS

Objective: To analyze the anatomical morphological and hemodynamic characteristics of left ventricular outflow tract stenosis (LVOTS) by echocardiography and MRI. Methods: The Echocardiography data of 112 patients with LVOTS were retrospectively analyzed by measuring the basal interventricular septal thickness (IVST-b), the left ventricle posterior wall thickness (LVPWT), and the peak pressure gradient of LVOTS, as well as by observing the characteristics of spectral pattern and the presence of systolic anterior motion of mitral valve leaflets. A Siemens 3.0T MRI scanner was used to scan the contrast-enhanced left ventricular (LV) volume of all cases. The obtained end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) of LV were compared with the Echocardiography results. Results: The 112 patients were divided into four groups: hypertrophic obstructive cardiomyopathy (Group I, 42 cases), hypertensive left ventricular hypertrophy (Group II, 40 cases), basal septal hypertrophy in the elderly (Group III, 21 cases), and the subaortic membrane (Group IV, 9 cases). The continuous wave (CW) Doppler characteristic of Groups I, II, and III was broadsword-shaped jet, and that of Group IV was equilateral triangle-like spectrum. The IVST-b, IVST-b/LVPWT ratio and peak pressure gradient of LVOTS of Group I was significantly higher than those in Groups II and III ([Formula: see text]). The LVPWT of the first three groups was slightly correlated with the LVOTS peak pressure gradient ([Formula: see text], respectively, [Formula: see text]). There were no statistically significant differences between Echocardiography and MRI results regarding the LV EDV, ESV, SV, and EF ([Formula: see text]), and no statistically significant differences were found between Echocardiography and MRI results regarding the myocardial thicknesses of septal, anterior, lateral, and inferior walls ([Formula: see text]). The Pearson’s correlation analysis demonstrated correlations between MRI and Echocardiography results for LV EDV, ESV, SV, and EF ([Formula: see text], 0.002, 0.009, and [Formula: see text], respectively). The MRI enhancement was shown as delayed enhancement in 52 cases, localized enhancement in 8 cases, diffuse enhancement in 6 cases, and transmural enhancement in 3 cases, with abnormal enhancement lesions distributed in the area of ventricular septum free wall junctions and ventricular septum. Conclusion: Using MRI to evaluate LV function of hypertrophic cardiomyopathy is reliable and accurate. MRI enhancement can be used for the quantitative measurement of myocardial fibrosis. Echocardiography can distinguish the stenosis types of LVOTS. The IVST-b and existence of SAM may be important anatomical characteristics determining the degree of dynamic stenosis, and MRI combined with Echocardiography can provide important detailed information.

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

Massive myocardial calcification: a heart of stone!

Abstract A 71-year-old female was admitted in the emergency room after effort-related syncope. She had past medical history of obesity and hypertension treated with lercanidipine. No relevant family history. Physical examination revealed systolic murmur (grade 2/6). ECG showed sinus rhythm and left bundle brunch block (Supplementary figure). Lab results were unremarkable including troponin I. Transthoracic echocardiography (TTE) revealed moderate left ventricular (LV) hypertrophy [septal thickness 14 mm (normal: 6–9mm)], extensive mitral annulus calcification (MAC) with exuberant myocardial calcification at the level of posterior leaflet that invaded adjacent LV walls, systolic anterior motion of the anterior mitral leaflet causing LV outflow tract (LVOT) obstruction (maximum gradient 34 mmHg) and moderate mitral regurgitation (MR) (Panel A). Exercise echocardiogram provoked LVOT gradient &amp;gt;100mmHg and severe MR. Continuous ECG monitoring during 13 days demonstrated no arrhythmic events. Cardiac CT showed multiple calcifications extending from mitral annulus to LV anterior and lateral walls and cardiac MRI was compatible with caseous MAC (Panels B-F). Coronary angiography showed 50% stenosis of mid left anterior descending artery. Normal thoracic CT and ACE, and negative IGRA excluded sarcoidosis and tuberculosis. Comprehensive study, including calcium metabolism and autoantibodies, excluded metabolic and inflammatory aetiologies of myocardial calcification, which was assumed as dystrophic in the context of MAC. No syncope occurred after bisoprolol 2.5 mg, oral rehydration and discontinuation of lercanidipine. However, rest and exercise TTE failed to show improvement of LVOT gradient or MR and patient remained on NYHA class II-III, despite maximal tolerated dose of bisoprolol (5 mg), being therefore referred to cardiac surgery.