Anterolateral Papillary Muscle Research Articles

Omnipolar mapping and vector mapping in premature ventricular contractions ablation: a comparison with bipolar mapping

Abstract Background Omnipolar Mapping, with HD Grid high-density mapping catheter, overcomes the dependency of EGM characteristics to wavefront orientation, reconstructing the highest peak-to-peak voltage attainable within the electric field and the real wavefront direction, displayed in the form of a local activation vector [1][2]. Methods Consecutive patients who underwent radiofrequency ablation of PVCs from October 2021 to August 2023 at the Hospital Clinic of Barcelona were included in the study. EAM was performed with EnsiteX mapping system, Advisor HDgrid was used as mapping catheter. An activation map was performed if enough PVC burden. The area of interest was determined by earliest activation time combined with the morphology of unipolar electrogram during activation mapping. RF was delivered at the area of interest with target temperature of 45°C and maximum power of 50W. Both bipolar and omnipolar maps were analysed retrospectively. The reviewer was blinded to the ablation set for this analysis. A window of interest was created uniformally for all maps by defining a caliper within 15 ms before the onset of PVC. Activation vectors were displayed on the map and visually analysed. Three specific patterns were identified: Starburst, Homogeneous and Chaotic. Area of the earliest activation was measured. Earliest site of bipolar and omnipolar maps and specific location of star burst pattern were tagged. The site of the effective RF ablation was identified and the distance between points was determined. Results 18 patients were included. Overall, 11(61%) were males; age: 57,9±15,2 ys, 6(33%) patients haven't structural heart disease, 4(22%) presented PVC-induced cardiomyopathy. Mean left ventricular ejection fraction was 46±10. Mean PVC burden was 27±10%. In 9(50%) patients, PVCs were ablated from RVOT, 4(22%) in LVOT, 1(5%) in the left superior fascicle, 1(5%) in the antero-lateral papillary muscle, 2(10%) in the perihissian area and 1(5%) in the aortomitral continuity. Compared with bipolar, omnipolar mapping showed a smaller area of earliest activation(2,13±1,9mm2 vs 3,34±2,1 mm2,p<0,001), a shorter distance of the earliest point to starburst region(4,2±4,1mm vs 7,8±4,2mm,p<0,001) and shorter distance to the effective ablation site(6,1±3,9 mm vs 8,33±4,2mm, p<0,07). No significative difference was found between the effective RF ablation site and the starburst area compared to the distance between the effective RF ablation site and the omnipolar earliest deflection (6,11±3,9mm vs 6,5±4,3mm, p=0,6). No statistical significative difference was found between the timing of the earliest deflection in both settings (33,2±12,5 ms vs 31,5±12 ms, p=0,2) Conclusions Omnipolar mapping allows a better identification of the area of interest in PVC’s ablation compared to bipolar mapping. Vector mapping is a valuable asset to identify the earliest activation point to guide PVCs catheter ablation.Retrospective EAM analysisArea/distance measurements

Parachute mitral valve and mid-aortic syndrome – unusual associations of Alagille syndrome

Background: Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome. Case description: Case 1: A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms. Case 2: A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13–14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management. Conclusion: PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients.

Anterolateral papillary muscle suction causing low flow in a COVID-19 patient without medical history: a case report of central extracorporeal life support with left ventricular apex decompression.

Left ventricular (LV) decompression is an essential strategy for improving early survival in patients with refractory cardiogenic shock. Low pump flow in patients on extracorporeal life support (ECLS) with LV apex decompression is a life-threatening issue. However, identifying the underlying causes of low flow can be challenging. A 38-year-old woman with COVID-19-related fulminant myocarditis was treated with central ECLS with LV apex decompression. The pump flow in the intensive care unit (ICU) was intermittently low, and low flow alerts were frequent. The initial evaluation based on pressure monitor waveforms and transthoracic echocardiography failed to identify the underlying cause. Prompt bedside transesophageal echocardiography (TEE) revealed that the anterolateral papillary muscle was suctioned into the vent cannula of the LV apex during systole. The patient underwent a repeat sternal midline incision in the operating room, and the cannula at the LV apex was repositioned. There were no further suction events after the repositioning, and the patient was weaned from ECLS 12days after admission to the ICU. The patient was discharged in a stable condition and without neurological deficits. TEE is an important diagnostic tool to identify the underlying cause of low flow flow in patients undergoing ECLS with LV apex decompression.

Cardiac Computed Tomography Assessment of the Variation of Papillary Muscle Morphology before Mitral Valve Surgery

Background. The morphology of the papillary muscles of the left ventricle is highly variable. Few studies have investigated papillary muscle morphology using imaging. Objective. This study aimed to assess papillary muscle morphology with primary mitral regurgitation (MR) using cardiac computed tomography (CT). Methods. We examined 116 patients who underwent robotic mitral valve repair for primary MR using preoperative cardiac CT. Papillary muscle morphology was assessed using CT images and compared with operative findings. Results. CT images of papillary muscles were consistent with the operative findings during robotic mitral valve repair in all cases. Both the anterolateral papillary muscle (APM) and posteromedial papillary muscle (PPM) groups were identified in all cases, and the middle papillary muscle (MPM) group was identified in 24.1% of cases. The PPM group had a higher proportion of complex morphologies with more heads and bases than the APM group (head: p<0.001 and base: p<0.001). The PPM group had smaller papillary muscle sizes than the APM group. The MPM group in most patients had one base and one head (78.6%). Papillary muscle sizes were significantly smaller in the order of the APM, PPM, and MPM groups (p<0.001). Conclusions. Cardiac CT allowed clear visualization and accurate assessment of papillary muscle morphology in the left ventricle. It may be useful to obtain the papillary muscle variations preoperatively using CT imaging in procedures involving the papillary muscles such as mitral valve repair.

Abstract 15299: Cardiogenic Shock and Flash Pulmonary Edema in an Uncommon Nonischemic Nonmyxomatous Spontaneous Rupture of Anterolateral Mitral Valve Papillary Muscle

Introduction: Mitral valve papillary muscle rupture is a rare condition that presents a cardiac emergency requiring rapid identification and treatment. Here we present a case of flash pulmonary edema and cardiogenic shock from severe mitral regurgitation (MR) secondary to spontaneous rupture of the anterolateral papillary muscle. Case Presentation: A 48-year-old female with hypertension presented to the ED with acute shortness of breath. In the ED, she was tachycardiac, hypertensive, tachypnic and saturating 75% on room air. On physical exam a 3/6 systolic murmur was present at the apex. ECG had no signs of ischemia. TTE showed severe MR with a fail anterior mitral leaflet. The patient was intubated and taken emergently to the cath lab for intra-aortic balloon pump placement, TEE (Figure 1A) and coronary angiogram, which was normal (Figure 1B, 1C). She was taken to the OR for successful mitral valve replacement surgery. Discussion: The rapid clinical decline and emergent management of patients who develop cardiogenic shock with pulmonary edema from acute non-ischemic papillary muscle rupture presents a significant challenge for clinicians. The cause of papillary muscle rupture most often occurs due an ischemic event, but can occur spontaneously as in this case. Rapid identification of the etiology of the clinical symptoms is of upmost importance to increase chances of survival. On review, 11 cases have been published between 2000-2015 discussing nonischemic nonmyxomatous spontaneous papillary muscle rupture of which the majority had a posterior muscle rupture. In our case, the 48-year old patient only had mild chordal calcification and essential hypertension and presented with nonischemic nonmyxomatous rupture of the anterolateral muscle. With prompt identification and urgent multidisciplinary team intervention, the patient was eventually discharged after replacement and is doing well at 6 months follow-up.

Prevalence of papillary muscle hypertrophy in fabry disease

Background and aimsFabry disease (FD) is an X-linked genetic lysosomal disease, in which a deficit in the alpha-galactosidase A enzyme results in lysosomal build-up of globotriaosylceramide in several organs, causing cardiac, renal and cerebrovascular complications. The aim of this study was to assess the prevalence of papillary muscle hypertrophy (PMH) in patients with FD.MethodsA group of 63 patients with FD and a positive genetic diagnosis were studied and were divided into two groups: one included 24 patients with FD and LVH and another group included 39 patients with FD and without LVH. Papillary muscles were measured from the left parasternal short axis view, defining PMH as a diastolic thickness greater than 11 mm in any diameter.ResultsPatients with FD and LVH had a high prevalence of anterolateral PMH (66.6%), and such prevalence was lower for the posteromedial PMH (33.3%). However, patients who had not yet developed LVH had a high prevalence of anterolateral PMH (33.3%).ConclusionsPatients with FD in the pre-clinical stage (without LVH) have a high prevalence of PMH, especially involving the anterolateral papillary muscle. This finding could be an early marker for the development of LVH, allowing to suspect the disease during its early stages, and begin enzyme replacement therapy in the appropriate patients.

Anterolateral Papillary Muscle Rupture Predicted by Post-Infarction Inflammatory Markers.

BACKGROUND The incidence of papillary muscle rupture (PMR), a mechanical complication of acute myocardial infarction, has decreased in the reperfusion era; however, its fatality rate remains high. Timely recognition and prompt initiation of treatment for PMR are important to avoid prolonged cardiogenic shock; however, the symptoms of PMR are nonspecific, and early diagnosis is often difficult. CASE REPORT A 72-year-old woman with nausea for 2 days presented with ST-segment elevation myocardial infarction with obstruction of the obtuse marginal branch and 75% stenosis of the first diagonal branch. Percutaneous coronary intervention was performed to revascularize the obtuse marginal lesion, which was over thrombolysis in myocardial infarction grade 2 flow. After percutaneous coronary intervention, the patient developed fever, an elevated C-reactive protein level, and an increased neutrophil-to-lymphocyte ratio (NLR). The patient showed no signs of infection but elevated inflammatory marker levels, with C-reactive protein rising to 39.32 mg/dL and NLR to 15. On postoperative day 4, the patient's clinical condition rapidly deteriorated, resulting in circulatory failure. Transthoracic echocardiography showed anterolateral PMR, and urgent surgical mitral valve replacement was performed. On day 32, the patient was discharged from the hospital, and at the 1-year follow-up, she remained in good health. CONCLUSIONS When there are multiple lesions, including the obtuse marginal and diagonal branches, anterolateral PMR should be suspected as the cause of cardiogenic shock. Performing point-of-care echocardiography and closely monitoring C-reactive protein levels and NLR can be helpful to detect PMR early.

Traumatic rupture of anterolateral papillary muscle in a patient with congenital deficiency of left hemi-pericardium: case report

Abstract Congenital absence or deficiency of the pericardium or hemi-pericardium is uncommon. When it happens, the protection given to the heart is greatly reduced, and blunt or penetrating trauma to the chest is transmitted directly to the heart. Valvular injuries from these traumas are however rare with a case of tricuspid valve rupture reported. Traumatic papillary muscle rupture in all patients group is also rare. Co-existing rupture of papillary muscle in patients with congenital absence of the pericardium is extremely rare. We report the rare case of a patient with deficient left hemi-pericardium who had a traumatic rupture of the anterolateral papillary muscle from a road traffic accident.

Is catheter-tissue contact force value important for ablation of ventricular arrhythmias originating from the left ventricular papillary muscles?

Good catheter-tissue contact is mandatory to create effective ablation lesions. The minimal contact force value for ablation of arrhythmias originating from the left ventricle is 8.0-10.0 grams but is not known for arrhythmias arising from papillary muscles. To analyze contact force values during successful ablation procedures of arrhythmias originating from the left ventricular papillary muscles. 24 consecutive patients (mean age 57.9 ± 11.9 years, 16 males) underwent ablation of premature ventricular complexes originating from left ventricular papillary muscles with the use of CARTO electro-anatomical system and intracardiac echocardiography. Acute complete abolition of ventricular ectopy was obtained in 23 (96%) patients. The fluoroscopy time was 3.9 ± 3.5 min and procedure duration - 114.8 ± 37.9 min. The mean contact force during successful ablations was 3.0 ± 1.1 grams and 3.18 ± 1.8 grams for antero-lateral and postero-medial papillary muscle, respectively (NS). The mean contact force during a single unsuccessful ablation was 3.0 grams. At control Holter ECG, the mean Ectopy Burden was Reduced in the Antero-Lateral Papillary Muscle Group from 18.0% ± 7.9% to 2.6% ± 2.9% (p = 0.005415) and in the Postero-Medial Papillary Muscle Group - from 34.8% ± 13.7%-1.7% ± 1.3% (p = 0.012694). During Median 27 (IQR: 17-34) Months of Follow-up There one Recurrence of Arrhythmia. The values of contact force for successful ablation of ventricular ectopy originating from the left ventricular papillary muscles may be much lower than those for ablation of other foci which questions the role of contact force measurement when ablating these arrhythmias.

P155 CT EVIDENCE OF PARTIAL ISCHEMIC PAPILLARY MUSCLE RUPTURE

Abstract A 77–year–old man was referred for coronary CT following an episode of prolonged chest pain that occurred about 30 days earlier, with subsequent onset of dyspnea on moderate exertion. Coronary CT demonstrated right dominance, occlusion of the right coronary artery, as well as severe stenosis of the distal segment of the left anterior descending artery and of the first obtuse marginal branch. CT scan also showed an intraventricular image consistent with partial rupture of the posteromedial papillary muscle (Figure 1). The patient was transferred to the emergency department. An ECG showed signs of inferolateral necrosis. Transthoracic echocardiography confirmed akinesia of the inferior and inferolateral walls and of the basal segment of the inferior septum, with severe mitral valve insufficiency from anterior leaflet prolapse (A3 scallop) from partial rupture of the posteromedial papillary muscle (Figure 2). Transesophageal echocardiography confirmed the diagnosis (Figure 3). The patient was admitted to the CCU and underwent coronarography, which confirmed the severe triple vessel coronary artery disease with occlusion of the right coronary artery, and then urgent mitral valve replacement with bioprosthesis and aortocoronary bypass with saphenous vein grafting to the posterior descending artery. The postoperative course was uneventful. Ischemic papillary muscle rupture is a rare cause of mitral insufficiency. Papillary rupture typically occurs within 5 days of an acute myocardial infarction, and causes mitral insufficiency by prolapse and flail of one or, more rarely, both mitral valve leaflets. The posteromedial papillary muscle is most commonly affected, since the anterolateral papillary muscle has dual supply from the anterior interventricular artery and the circumflex artery. Papillary muscle rupture can be total or partial; however, most cases of partial rupture progress to complete rupture and therefore require urgent surgical treatment. The peculiarity of this case lies in the fact that it is the first case described in the literature of a papillary muscle rupture documented on CT.

C34 AN UNUSUAL CASE OF CARDIAC ARREST DUE TO TRAUMA–INDUCED QT PROLONGATION

Abstract Background QT prolongation is a well–known cause of sudden cardiac death due to polymorphic ventricular tachycardia triggered by early afterdepolarizations. Acquired long QT is a common finding in several clinical scenarios and invariably represents a high risk feature. Case Report An 84 years–old woman presented to our emergency department (ED) after experiencing a blunt chest trauma due to a car accident. She reported mild dyspnea and chest pain exacerbated by breathing and movement while denied palpitations and loss of consciousness. Baseline ECG showed atrial fibrillation with normal ventricular rate and a prolonged QT interval (QTc 640, Fig 1A) not present at previous ECGs. She did not assume any QT–prolonging drug and laboratory tests at admittance revealed troponin I values slightly elevated (29,6 ng/ml; n.v.: <11,6 ng/l) and no other significant abnormalities. A CT scan demonstrated a displaced fracture of the sternal body. Due to the QT prolongation, the patient was admitted for ECG monitoring.. During the stay in our ED the patient suffered a cardiac arrest. A torsade des pointes triggered by a late–coupled premature ventricular complex (PVC) was documented (Fig 1B). The arrhythmia was interrupted with external DC shock and magnesium solfate infusion was started. The coronary angiography demonstrated normal coronary arteries. The patient underwent cardiac magnetic resonance (CMR) four days later which showed transmural necrosis of the middle third of the lateral wall of the left ventricle at the insertion of the anterolateral papillary muscle (Fig 2). No further ventricular arrhythmias were observed and the patient was discharged after QTc normalization. Cardiac arrhythmias are a rare complication of cardiac contusion ranging from isolated PVCs to atrial or ventricular fibrillation. However, the role of the ECG in this peculiar subset is unclear. The most common ECG abnormalities are non specific ST–T wave changes, whereas in our case the marked QTc prolongation resulted in a closer monitoring of the patient allowing a prompt recognition of cardiac arrest. On the other hand CMR proved to be an effective method to identify a cardiac injury showing the typical pattern of myocardial damage associated with cardiac contusion (stress–induced lesions at the insertions of papillary muscles due to sudden increase of left ventricular pressure) and, thus, could be adopted as a useful tool to identify patients at higher risk for arrhythmic complications.

Analysis of heart displacement during thoracic radiotherapy based on electrocardiograph-gated 4-dimensional magnetic resonance imaging.

Periodic cardiac movement may expose the heart to radiation field induced damage, leading to radiation-induced heart disease (RIHD). Studies have proven that delineation of the heart based on planning CT fails to show the real margin of the substructures and a compensatory margin should be applied. The purpose of this study was to quantify the dynamic changes and the compensatory extension range by breath-hold and electrocardiogram gated 4-dimensional magnetic resonance imaging (4D-MRI), which had the advantage of discriminating soft tissues. Eventually, 15 patients with oesophageal or lung cancers were enrolled, including one female and nine males aged from 59 to 77 years from December 10th, 2018, to March 4th, 2020. The displacement of the heart and its substructures was measured through a fusion volume and the compensatory expansion range was calculated by expending the boundary on the planning CT to that of the fusion volume. The differences were tested through the Kruskal-Wallis H test and were considered significant at a two-side P<0.05. The extent of movement of heart and its substructures during one cardiac cycle were approximately 4.0-26.1 millimetre (mm) in anterior-posterior (AP), left-right (LR), and cranial-caudal (CC) axes, and the compensatory margins should be applied to planning CT by extending the margins by 1.7, 3.6, 1.8, 3.0, 2.1, and 2.9 centimetres (cm) for pericardium; 1.2, 2.5, 1.0, 2.8, 1.8, and 3.3 cm for heart; 3.8, 3.4, 3.1, 2.8, 0.9, and 2.0 cm for interatrial septum; 3.3, 4.9, 2.0, 4.1, 1.1, and 2.9 cm for interventricular septum; 2.2, 3.0, 1.1, 5.3, 1.8, and 2.4 cm for left ventricular muscle (LVM); 5.9, 3.4, 2.1, 6.1, 5.4, and 3.6 cm for antero-lateral papillary muscle (ALPM); and 6.6, 2.9, 2.6, 6.6, 3.9, and 4.8 cm for postero-medial papillary muscle (PMPM) in anterior, posterior, left, right, cranial, and caudal directions, respectively. Periodic cardiac activity causes obvious displacement of the heart and its substructures, and the motion amplitude of substructures differs. Extending a certain margin as the compensatory extension to represent the organs at risk (OAR) and then limiting the dose-volume parameters could be performed in clinical practice.

Apical papillary muscle displacement is a prevalent feature and a phenotypic precursor of apical hypertrophic cardiomyopathy.

Papillary muscle (PM) abnormalities are considered part of the phenotypic spectrum of hypertrophic cardiomyopathy (HCM). The aim of this study was to evaluate the presence and frequency of PM displacement in different HCM phenotypes. We retrospectively analysed cardiovascular magnetic resonance (CMR) findings in 156 patients (25% females, median age 57 years). Patients were divided into three groups: septal hypertrophy (Sep-HCM, n = 70, 45%), mixed hypertrophy (Mixed-HCM, n = 48, 31%), and apical hypertrophy (Ap-HCM, n = 38, 24%). Fifty-five healthy subjects were enrolled as controls. Apical PM displacement was observed in 13% of controls and 55% of patients, which was most common in the Ap-HCM group, followed by the Mixed-HCM and Sep-HCM groups (respectively: inferomedial PM 92 vs. 65 vs. 13%, P < 0.001; anterolateral PM 61 vs. 40 vs. 9%, P < 0.001). Significant differences in PM displacement were found when comparing healthy controls with patients with Ap- and Mixed-HCM subtypes but not when comparing them with patients with the Sep-HCM subtype. T-wave inversion in the inferior and lateral leads was more frequent in patients with Ap-HCM (100 and 65%, respectively) when compared with Mixed-HCM (89 and 29%, respectively) and Sep-HCM (57 and 17%, respectively; P < 0.001 for both). Eight patients with Ap-HCM had prior CMR examinations because of T-wave inversion [median interval 7 (3-8) years], and in the first CMR study, none showed apical hypertrophy [median apical wall thickness 8 (7-9) mm], while all of them presented with apical PM displacement. Apical PM displacement is part of the phenotypic Ap-HCM spectrum and may precede the development of hypertrophy. These observations suggest a potential pathogenetic, mechanical link between apical PM displacement and Ap-HCM.

Does papillary muscle free strain has predictive value in risk stratification of patients with hypertrophic cardiomyopathy?

Papillary muscle free strain has not been evaluated previously in hypertrophic cardiomyopathy (HCMP) patients. Our aim was to evaluate free papillary muscle free strain in HCMP patients and to find whether it has a value for prediction of sudden cardiac death (SCD) risk score. Transthoracic echocardiography with tissue Doppler imaging, 2-D speckle tracking imaging (STI) of 55 HCMP patients and 45 controls were performed. HCMP patients were further divided into two groups according to their SCD risk score. Patients with risk score of less than 6 points constituted low/intermediate risk group, whereas patients with risk score of greater or equal to 6 points constituted high risk group. Interventricular septum, posterior wall, and left ventricular mass index were significantly higher, whereas mitral E/A ratio was significantly lower in HCMP patients compared to controls. Longitudinal apical 4C, 2C, 3C, global longitudinal LV strain, anterolateral papillary muscle (ALPM), posteromedial papillary muscle (PMPM) free strain were significantly reduced in HCMP group compared to control group. Global longitudinal strain and ALPM free strain were significantly lower in patients with high SCD risk score (-14.6 (-17.4 - -13.1) vs -11.6 (-13.2 - -10.1), p = 0.001 and -17.1 (-20.3 - -14.0) vs -9.2 (-12.6 - -7.5), p<0.001, respectively. Global longitudinal strain and ALPM free strain were statistically significantly correlated with SCD risk score (r = 0.480, p<0.001 and r = 0.462, p<0.001, respectively). Global longitudinal strain value of -12.60% had a sensitivity of 73.3% and specificity of 82.5% for predicting high SCD risk score (AUC: 0.787, 95% CI: 00.643-0.930, p = 0.001). ALPM free strain value of -12.95% had 66.7% sensitivity and 77.5% specificity for predicting high SCD risk score (AUC: 0.766, 95% CI: 0.626-0.905, p = 0.003). Papillary muscle free strain was reduced in HCMP patients. It might be used in risk stratification of these patients.

991 A PATIENT WITH INFERIOR Q WAVES AND CHEST PAIN: WHEN HOOF BEATS ARE NOT HORSES

Abstract Background Cardiac masses are commonly classified as neoplastic (primary and secondary) or non-neoplastic (e.g., thrombus or vegetation). Cardiac tumors are rare, and primary tumors are even rarer, with an estimated autopsy prevalence of &amp;lt; 0.1%. Differentiating between these masses is of paramount importance as treatment options greatly differ. Case presentation A 46-year-old male patient, current smoker, with family history of CAD and new onset hyperlipidemia and hypertension, underwent electrocardiogram (ECG) screening for an episode of chest pain. His ECG showed sinus rhythm with Q waves in inferior leads. The laboratory tests did not reveal any abnormality. His transthoracic echocardiogram showed normal biventricular function, and a lobular mass of 30×20 mm, that seemed in continuity with the antero-lateral (A-L) papillary muscle. For the presence of multiple cardiovascular risk factor, the patient underwent diagnostic coronary angiography which showed normal coronary arteries. Differential diagnoses were: hypertrophic cardiomyopathy with isolated hypertrophy of the A-L papillary muscle and LV mass. Cardiac magnetic resonance (CMR) was performed and demonstrated a pedunculated mobile non-enhancing 30×18mm mass within the LV anterior apex, that in the systolic phase contracts close relationships with the A-L papillary muscle which appears dislocated. This encapsulated formation determined a mild systolic obliteration of the LV cavity at the mid-apical portion. T1 imaging with and without suppression of adipose tissue, were compatible with cardiac intraventricular lipoma. The case was discussed in a multi-disciplinary heart team, and in accordance with the cardiac surgeon the patient underwent conservative management and follow up with echocardiography at 6 months and CMR at 1 year. Discussion Cardiac lipomas are rare benign primary cardiac tumors. They are usually indolent, asymptomatic and incidentally-discovered. Symptoms such as dyspnea, syncope or palpitations can occur if the tumor grows and causes LV obstruction, LV dysfunction or invasion of the conduction system. Sudden cardiac death has been reported, but the true incidence is unknown given its rarity. The most common location for cardiac lipomas is the inter-atrial septum, followed by endocardium of RA and LV. CMR is the imaging modality of choice when available and lipomas have a homogeneous appearance of increased signal intensity on T1-weighted imaging, with a reduction in signal intensity in fat-saturated sequences. As the prevalence of cardiac lipomas is very low, there are no randomized clinical trials or large prospective cohorts to provide guidance for optimal treatment. Embolization or infiltration of the myocardium are not common. For large lesions causing obstruction, surgical resection is usually indicated. Conclusion Cardiac lipoma is a rare primary tumor of the heart. The comprehensive evaluation is highly dependent on multimodality imaging methods. The best treatment strategy is still not clear and should be individualized.

Three-year Follow-up of Unresected Large Anterolateral Papillary Muscle Lipoma

To our knowledge, this is the largest unresected anterolateral papillary muscle lipoma, in an asymptomatic patient, followed up for three years since diagnosis.

Abstract 13963: Short-Coupled Variant of Torsade de Pointes: A Rare Entity With Unknown Etiology and Malignant Phenotype

Introduction: Torsade de Pointe (TdP) is defined as a polymorphic VT (pVT) typically associated with QTc prolongation. However, not all pVT occur in the presence of QTc prolongation and respond to different forms of therapy. Hence defining their etiology is important. Case: A 48-yo Female with H/O resolved peripartum cardiomyopathy, hypertension &amp; obesity presented to the hospital following a syncopal episode. Her BP was 207/125 mmHg, and ECG showed sinus rhythm with a QTc of 436. No history of heart disease or SCD in her family. Physical exam was unremarkable, labs showing K-3.2 without troponin elevation. On day 2, she developed a pVT requiring defibrillation and amiodarone. Telemetry revealed a PVC causing R on T phenomenon with a coupling interval of 260 msec prior to initiation of SVT with QTc 484 and K-2.8. On day 3, despite electrolyte correction, she had multiple non-sustained pVTs with QTc 482. Amiodarone was switched to lidocaine and QTc normalized to 447. TTE and LHC were unremarkable with a small LV aneurysm on cMRI. On day 4, the patient continued to have incessant runs of pulseless pVT requiring defibrillation and started on verapamil. A 12 lead ECG during VT demonstrated left bundle branch mimicry with a left axis consistent with a VT exit site along the RV moderator band. The patient underwent emergent ablation with targets along the right anterolateral papillary muscle. AICD was placed prior to discharge. Discussion: The critical timing of PVC falling on the peak of the preceding T wave differentiates ‘Short-coupled TdP’ from other malignant VTs with normal QTc. These PVCs typically originate from distal ramifications of Purkinje fibers in both ventricles. PVC morphology with LBBB pattern, left axis and late precordial R-S transition points to the moderator band as the source of idiopathic VT. Contrasting with typical TdP, medical management with Quinidine and Verapamil may be efficacious. Catheter ablation is curative in most cases.

A papillary fibroelastoma with myxoma camouflage: a case report

BackgroundBenign cardiac tumours are infrequent in clinical practice and, of these, cardiac myxoma is the one with the highest incidence. Given that a left intraventricular presentation is rare, other differential diagnoses such as papillary fibroelastoma should be considered.Case summaryA 73-year-old woman patient with cardiac mass detected in transthoracic echocardiography (TTE) after a transient ischaemic attack. At TTE 2D–3D, a left intraventricular mass anchored at the level of the anterolateral papillary muscle was detected. Subsequently, cardiac magnetic resonance (CMR) was performed for mass characterization. This revealed behaviour in T1 (isointense with respect to myocardium), T2 (hyperintense), very prolonged T1-mapping (1848 msg), and T2-mapping (161 msg) values, without gadolinium uptake in the first-pass perfusion sequence, but with intense uptake in late enhancement sequences. Previous findings were compatible with a diagnosis of papillary fibroelastoma. The mass was resected intraoperatively and, although its macroscopic appearance pointed to a diagnosis of cardiac myxoma, it was finally confirmed to be a papillary fibroelastoma by pathological anatomy.DiscussionIn cases where the size of the mass and its mobility allow tissue characterization by CMR, a diagnosis of papillary fibroelastoma and its differentiation with cardiac myxoma are feasible by this cardiac imaging technique.

Leaflet plication with neochordae implantation: A novel technique for mitral valve repair.

Leaflet plication with neochordae implantation: A novel technique for mitral valve repair.

Visualization and Quantification of the Unrepaired Complete Atrioventricular Canal Valve Using Open-Source Software

Repair of complete atrioventricular canal (CAVC) is often complicated by residual left atrioventricular valve regurgitation. The structure of the mitral and tricuspid valves in biventricular hearts has previously been shown to be associated with valve dysfunction. However, the three-dimensional (3D) structure of the entire unrepaired CAVC valve has not been quantified. Understanding the 3D structure of the CAVC may inform optimized repair. Novel open-source work flows were created in SlicerHeart for the modeling and quantification of CAVC valves on the basis of 3D echocardiographic images. These methods were applied to model the annulus, leaflets, and papillary muscle (PM) structure of 35 patients (29 with trisomy 21) with CAVC using transthoracic 3D echocardiography. The mean leaflet and annular shapes were calculated and visualized using shape analysis. Metrics of the complete native CAVC valve structure were compared with those of normal mitral valves using the Mann-Whitney U test. Associations between CAVC structure and atrioventricular valve regurgitation were analyzed. CAVC leaflet metrics varied throughout systole. Compared with normal mitral valves, the left CAVC PMs were more acutely angled in relation to the annular plane (P<.001). In addition, the anterolateral PM was laterally and inferiorly rotated in CAVC, while the posteromedial PM was more superiorly and laterally rotated, relative to normal mitral valves (P<.001). Lower native CAVC atrioventricular valve annular height and annular height-to-valve width ratio before repair were both associated with moderate or greater left atrioventricular valve regurgitation after repair (P<.01). It is feasible to model and quantify 3D CAVC structure using 3D echocardiographic images. The results demonstrate significant variation in CAVC structure across the cohort and differences in annular, leaflet, and PM structure compared with the mitral valve. These tools may be used in future studies to catalyze future research intended to identify structural associations of valve dysfunction and to optimize repair in this vulnerable and complex population.