scholarly journals P1720 Hammock mitral valve, a challenging echocardiographic diagnosis

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D Rodrigo Carbonero ◽  
U Estandia ◽  
C Perez ◽  
R Voces ◽  
P Perez ◽  
...  

Abstract We report a 43 year-old female with a past TTE echocardiography of rheumatic valve disease performed in her district hospital , ( No clear symptomatology of rheumatic fever in the past). She was transferred to our tertiary hospital for elective cardiac surgery. Preoperative echocardiogram showed a non-dilated left ventricle with preserved contractility, mild-moderate left atrium enlargement with severe mitral regurgitation and basal displacement of papillary muscles and severe tricuspid regurgitation.All of it resembling a hammock mitral valve instead of former echocardiogram described as rheumatic valve disease. Preoperative cardiac study showed severe pulmonary hypertension with increased pulmonary vascular resistances. Preserved biventricular cardiac output and increased proto and telesystolic pressures. During surgery , ifindings were described as a mitral valve with a large papillary muscle inserted in the distal third of the left ventricle with none tendinous cords at the anterior leaflet and without cords in the posterior leaflet with an isolated papillary muscle with cords at A3 and P3 scallops, compatible with hammock mitral valve. A tendinous muscle/fibrous or fibromuscular band connecting the septum to the posterior wall of the left ventricle was described. Moreover over, there was an enlarged tricuspid ring with very short tendinous cords on the septal leaflet, although the leaflet was bigger than usual. Surgery consisted of resection of the mitral valve preserving A3 and P3 scallops with a 29mm Bicarbon Sorin mechanical mitral prosthesis and a 32mm Carpentier tricuspid ring implantation and pulmonary veins ablation combined with occlusion of left atrial appendage. After 112 minutes of cross-clamping time, the patient was weaned from cardiopulmonary bypass. She had important left ventricle dysfunction which improved with dobutamine and AAI pacemaker at 90lpm. Postoperative TEE showed moderate dysfunction of right ventricle, mild left ventricular dysfunction, moderate tricuspid regurgitation and a good functioning of the prosthesis. TTE before discharge showed good function of mitral valve prosthesis, good left ventricle function, mild tricuspid regurgitation, mild-moderate right ventricular enlargement, although less than preoperatively. Conclusion Congenital mitral valulophaty is a rare condition in the adulthood. The estimated prevalence is 0,5%. The hammock mitral valve is a more uncommon pathology which affects the mitral valve and subvalvular apparatus. This anomaly, was first described in 1967 and it is characterised by anomalous papillary muscles directly connected to the anterior mitral valve by a fibrous bridge without chordae tendineae in between them. This fibrous bridge hampers the opening and closure of the mitral valve. Diagnosis requires a high index of suspicion, both ultrasound studies and medical history, to avoid misdiagnosis. Abstract P1720 Figure.

Author(s):  
Kazem Rahimi

Mitral stenosis is obstruction to inflow of blood from left atrium to left ventricle at the level of the mitral valve. Non-valvar causes of left ventricular inflow obstruction include left atrial tumours and cor triatriatum.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D Rodrigo ◽  
U Estandia ◽  
P Perez ◽  
C Perez ◽  
A Cortes ◽  
...  

Abstract We report a 62-year-old man with a past medical history of dyslipidemia, paranoid schizophrenia and permanent atrial fibrillation. A ATTE performed at his district hospital revealed rheumatic mitral valve disease with double lesion: severe regurgitation and mild stenosis, plus moderate tricuspid regurgitation and a mean PAP of 32mm Hg. Cardiac catheterization showed no abnormalities of the coronary arteries. He was transferred to our hospital and scheduled for mitral valve replacement and tricuspid ring valvuloplasty. Preoperative transesophageal echocardiography showed an abnormal subvalvular mitral apparatus, with false tendons and multiple papillary muscles, resembling a hammock mitral valve. Most cordae tendinae arose from a single dominant papillary muscle at a posterior medial region, which provoke severe mitral regurgitation due to coaptation defect and mild subvalvular mitral stenosis. It could also be appreciated hypertrabeculation in the lateral medial, basal and apical segments. This suggested no-compaction cardiomyopathy associated with hammock mitral valve. Left ventricular systolic function was preserved. No evidence of rheumatic mitral valve disease was found in transesophageal echocardiographic study performed at our hospital. On the 30th April 2019 he underwent mechanic mitral valve replacement (Bicarbon 29mm) and tricuspid ring valvuloplasty (Edwards Physio 32mm) surgery. Once the patient was weaned from cardiopulmonary bypass, severe left ventricle systolic dysfunction ensued, predominantly localized in the anterior, inferior septal, inferior lateral basal and medial segments. Apical segments had preserved mobility An adrenalin infusion prior weaning from CBP was initiated. Preserved mobility of the mitral prosthesis discs was observed. The patient developed cardiogenic shock in spite of high doses of dobutamin and adrenaline infused. IACB was implanted with 1:1 assistance. The patient was transfered to the hemodynamic room in order to rule out coronary complications. Cardiac catheterization showed no significant angiographic lesions. During the first postoperative hours, the patient was stabilized allowing progressive lowering of the drugs (adrenaline, dobutamine). TTE showed normally functioning prosthetic mitral valve and preserved left ventricle systolic function. An MRI was performed demostrating no-compaction cardiomyopathy Conclusion This case report describes a rare presentation of simultaneous ocurrence of hammock mitral valve and no-compaction cardiomyopathy. Perioperative left ventricle dysfunction in no-compaction cardiomyopathy is related to subendocardial ischemia caused during extracorporeal circulation in the multiple prominent ventricular trabeculations with deep intertrabecular recesses corresponding to non-compacted myocardium .This must be taken account in those patients with no-compaction cardiomyopathy scheduled for cardiac surgery in order to take preventive measures. Abstract 89 Figure. non - compacted myocardium


PLoS Genetics ◽  
2021 ◽  
Vol 17 (9) ◽  
pp. e1009726
Author(s):  
Erik Axelsson ◽  
Ingrid Ljungvall ◽  
Priyasma Bhoumik ◽  
Laura Bas Conn ◽  
Eva Muren ◽  
...  

Selective breeding for desirable traits in strictly controlled populations has generated an extraordinary diversity in canine morphology and behaviour, but has also led to loss of genetic variation and random entrapment of disease alleles. As a consequence, specific diseases are now prevalent in certain breeds, but whether the recent breeding practice led to an overall increase in genetic load remains unclear. Here we generate whole genome sequencing (WGS) data from 20 dogs per breed from eight breeds and document a ~10% rise in the number of derived alleles per genome at evolutionarily conserved sites in the heavily bottlenecked cavalier King Charles spaniel breed (cKCs) relative to in most breeds studied here. Our finding represents the first clear indication of a relative increase in levels of deleterious genetic variation in a specific breed, arguing that recent breeding practices probably were associated with an accumulation of genetic load in dogs. We then use the WGS data to identify candidate risk alleles for the most common cause for veterinary care in cKCs–the heart disease myxomatous mitral valve disease (MMVD). We verify a potential link to MMVD for candidate variants near the heart specific NEBL gene in a dachshund population and show that two of the NEBL candidate variants have regulatory potential in heart-derived cell lines and are associated with reduced NEBL isoform nebulette expression in papillary muscle (but not in mitral valve, nor in left ventricular wall). Alleles linked to reduced nebulette expression may hence predispose cKCs and other breeds to MMVD via loss of papillary muscle integrity.


2012 ◽  
Vol 93 (3) ◽  
pp. 490-493
Author(s):  
Yu M Belozerov ◽  
Sh M Magomedova ◽  
Z S Abakarova ◽  
I M Osmanov

Aim. To assess the role of subvalvular structures in the development of mitral valve prolapse in children. Methods. Conducted was an echocardiography investigation with comparative analysis of structural and functional parameters of subvalvular structures in healthy children (50 individuals) and children with mitral valve prolapse divided in two groups: first group - without regurgitation (34 children), the second group - with regurgitation (16 children). Results. In healthy children identified were strong correlation links between echocardiographic indices of the subvalvular structures of the mitral valve and the functional parameters of the left ventricle. The diameter of the anterolateral group of papillary muscles directly correlated with the thickness of the interventricular septum, while the diastolic distance between the papillary muscles - with the end diastolic diameter of the left ventricle. It was established that the following factors may contribute to the pathogenesis of mitral valve prolapse: (1) long papillary muscles and the corresponding chords of the valve, (2) a weak (relative to the norm) contractile ability of the papillary muscles, (3) hyperkinesis of the left ventricular apex, and (4) no relationship between the apical and the global ejection fraction. These factors contribute to the fact that the papillary muscles and chords constrain the mitral valve cusps to a lesser extent during systole, which in turn easily bulge into the left atrium under the influence of systolic blood pressure. Apical hyperkinesis contributes to even closer approximation of the subvalvular structures with the cusps. Conclusion. Mitral valve prolapse is attributed to desynchronization of the function of papillary muscles and of the left ventricle; the development of functional mitral regurgitation during mitral valve prolapse is associated with dyssynchrony of the papillary muscles’ activity.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Bettella ◽  
M De Lazzari ◽  
A Zorzi ◽  
T Vessella ◽  
A Cipriani ◽  
...  

Abstract Aims To evaluate by cardiac magnetic resonance (CMR) if left ventricle papillary muscle abnormalities, such as hypertrophy and abnormal location, may be the anatomo-functional substrates responsible for TWI inversion in lateral or infero-lateral leads in otherwise healthy athletes. Methods We included competitive athletes with TWI in lateral or infero-lateral leads in the absence of cardiac diseases detected by CMR. The control population included healthy athletes with normal ECG, matched for age and gender. We compared thickness, volume (both absolute and relative to the cardiac mass) and position of the papillary muscles between cases and controls. Results We included 53 athletes with apparently unexplained TWI in the lateral or infero-lateral leads (median age 20 years (17–42), 86.8% males) and 53 athletes with no TWI matched for age and gender. 4 patients (7.6%) had family history for cardiomyopathy or sudden cardiac death. Athletes with TWI showed more hypertrophic papillary muscles compared to controls, with statistically significant difference in diameter, area and volume (p<0.01). The median ratio between the papillary muscles and the left ventricular mass was 4.4% among athletes with TWI versus 3% among those without TWI (p<0.001). Papillary muscles showed apical displacement in 47% of cases, compared to 17% in the control group (p=0.001). Conclusions Idiopathic TWI in lateral or infero-lateral leads is associated with left ventricle papillary muscle hypertrophy and their apical displacement detected by CMR. The comprehension of clinical and prognostic significance of papillary muscle abnormalities responsible for these ventricular repolarization alterations requires further studies. Example Funding Acknowledgement Type of funding source: None


2018 ◽  
Vol 22 (4) ◽  
pp. 54
Author(s):  
V. V. Bazylev ◽  
A. I. Mikulyak ◽  
R. M. Babukov ◽  
V. A. Karnakhin

<p><strong>Background.</strong> Enlargement of the left ventricular chamber and displacement of papillary muscles in the apical and lateral directions increase the tethering forces. Left ventricular and papillary muscle desynchrony and reduced myocardial contractility reduce the closing forces, thus leading to impaired leaflet coaptation and appearance of mitral regurgitation. Therefore, treatment of mitral insufficiency requires an integrated approach, affecting all aspects of the pathogenesis of mitral regurgitation recurrence. Recent publications show that adjunctive subvalvular repair during mitral annuloplasty for secondary mitral regurgitation is effective in preventing recurrent regurgitation. One of these procedures is papillary muscle approximation. However, the safety and the positive impact of this method are still open to question. <br /><strong>Aim.</strong> This study focused on the assessment of mid-term results of papillary muscles approximation and comparison of the obtained results with those of isolated mitral annuloplasty.<br /><strong>Methods.</strong> Two hundred and twelve patients with ischemic cardiomyopathy and ischemic mitral regurgitation were enrolled in this retrospective single-center study. The patients were randomised to 2 groups by using propensity score matching (a “neighbor” method) according to the following parameters: end diastolic volume, end systolic volume, stroke volume and ejection fraction. The first group included 112 patients with ischemic cardiomyopathy and mitral regurgitation, who underwent coronary artery bypass grafting, mitral annuloplasty and papillary muscle approximation. The second group included 112 patients with ischemic cardiomyopathy who underwent coronary artery bypass grafting and mitral valve annuloplasty. We evaluated early and mid-term results.<br /><strong>Results.</strong> Two patients in group 1 and three patients in group 2 died of heart failure progression during 31.3±10.4 month follow-up. According to the Kaplan-Meier analysis, no statistically significant differences were noted between the groups (log-rank test = 0.8). Approximation of papillary muscles in patients with ischemic mitral regurgitation improved mitral valve leaflet coaptation as evidenced by the values of coaptation depth, coaptation line and tenting area (p&gt;0.05). During follow-up, 3 cases (2.7%) of mitral insufficiency recurrence were recorded in group 1 and 16 (14.3%) in group 2. The Kaplan-Meier analysis of cumulative probability showed a significant difference in freedom from recurrence of mitral regurgitation ≥2 between groups in the mid-term postoperative period (log-rank test = 0.041).<br /><strong>Conclusion.</strong> Adjunctive papillary muscle approximation performed at the time of mitral annuloplasty improves the durability of mitral valve repair.</p><p>Received 18 April 2018. Revised 12 October 2018. Accepted 18 October 2018.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p>


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
H El Jattari ◽  
E Snijders ◽  
S Laga ◽  
A Van Berendoncks

Abstract A 52-year-old man was admitted to the emergency department of the University Hospital after being involved in a car crash. At first he was alert with stable vital parameters. There was bilateral rales on lung auscultation, without further prominent aberrant findings at the initial clinical examination. Shortly after, he became agitated with respiratory distress, resulting into respiratory insufficiency. Computed tomography (CT) scan of the thorax showed multiple rib fractures, a translated sternal fracture and pulmonary contusion. After intubation and stabilization he was transferred to the Intensive Care Unit (ICU), where his respiratory and cardiovascular parameters progressively worsened, leading to cardiogenic shock. An urgent chest X-ray revealed a tension pneumothorax with mediastinal shift to the right, for which a chest tube was inserted in the left hemithorax. No or little improvement was seen after this intervention. Therefore, an emergency transthoracic echocardiography (TTE) was performed. Subcostal view showed a severe mitral regurgitation with a mass in the left atrium. Because of a limited transthoracic window, we proceeded to a transoesophageal echocardiogram (TEE). A completely ruptured anterolateral papillary muscle of the mitral valve was seen, protruding in the left atrium and resulting in severe mitral regurgitation (Figure). Other echocardiographic findings were a hyperdynamic systolic function of the left ventricle, a hypo-contractile right ventricle with a mild-to-moderate quantity of pericardial effusion. Taking into account the increased cardiovascular risk profile of the patient, a coronary angiography was performed and showed normal coronary anatomy. Consequently, urgent surgical intervention was performed. Perioperative assessment confirmed the earlier described echocardiographic findings. Due to severe laceration of the left ventricular wall at the level of the attachment site of the anterolateral papillary muscle, reconstructive surgery was not possible. The anterolateral papillary muscle head with the attached mitral valve leaflet was fully excised with repair of the concealed myocardial rupture site. A mechanical mitral valve prosthesis and an intra-aortic balloon pump for hemodynamic support were placed. After four days the balloon pump was removed and the tenth day after admission the patient was extubated. Conclusion Acute severe mitral regurgitation due to papillary muscle rupture is a rare but potential fatal complication of a blunt trauma. TOE is essential in early diagnosis. Abstract 505 Figure 1


2018 ◽  
Vol 3 (4) ◽  

Papillary muscles develop separately from mitral valve leaflet and chordae, they origin from myocardial ridge of the anterior wall and to the posterior wall of the left ventricle (LV). While chordae and mitral valve leaflets origin from a cushion tissue. The myocardial ridge gradually loosens from the ventricular wall and meanwhile the cushion tissue transforms into leaflet and chordae1. Abnormal papillary muscle formation may present a muscle bridge and divide the LV into two chambers. The most rare form is the superior inferior LV subdivision which our cases represent.


Author(s):  
Jess L. Thompson ◽  
Rakesh M. Suri ◽  
Stephen H. McKellar ◽  
Hartzell V. Schaff

Objective Delivery of a collapsable aortic valve prosthesis via the left ventricular (LV) apex has proven problematic clinically. We describe our experience with a novel technique for a laparoscopic transdiaphragmatic approach to the LV outflow tract (LVOT). Methods Laparoscopic transabdominal access to the left ventricle was achieved in four live swine and one human cadaver using three abdominal laparoscopic trocars. Pericardioscopy and cardioscopy were performed. A hemostatic purse-string suture was placed laparoscopically at the apex of the left ventricle in one pig, and an instrument passed through the purse-string and into the LVOT. After the procedure, a thoracotomy was performed and the heart explanted for gross examination. Results Transdiaphragmatic exposure of the LV apex was accomplished at a mean of 23 minutes. Hemodynamic stability was maintained in all animals and the pleural cavities were not violated. Pericardioscopy allowed visualization of both right and left pulmonary veins along with the left atrial appendage. In situ cardioscopy confirmed that access to the LVOT and the aortic annulus was possible from the LV apex entry site. Conclusions Laparoscopic transdiaphragmatic approach to the LVOT is a possible route for minimally invasive beating heart aortic valve implantation. This strategy permits a straight transapical line of access to the aortic valve; avoiding the potential for cardiac torsion via a thoracotomy.


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