A CASE OF RARE INHERITED RESTRICTIVE CARDIOMYOPATHY CAUSED BY A NOVEL MUTATION IN MYH7 GENE

Introduction: Inherited restrictive cardiomyopathy (RCM) is a rare cause of RCM associated with cytoskeletal and sarcoma gene mutations. We describe a case of inherited RCM due to MYH7s genetic mutation.Case description: A 66 year-old-woman was admitted for acute global heart failure. She had a family history of RCM with a mutation of MYH7 gene: sons sudden death at 30, one of her daughters who is 40 and grandson who is 1. The transthoracic cardiac ultrasound (TTE) showed a bi-atrial dilation, a non-dilated left ventricle (LV) non-hypertrophied. Genetic investigation found the same pathogenic missense mutation (c. 1477A>G in heterozygous state) in our patient and her daughter who has a non-obstructive hypertrophy cardiomyopathy (HCM).A few weeks later, our patient had a syncope on complete atrioventricular block. A triple chamber pace maker was installed. Discussion: Familial RCMs mutations are characterized by high allelic, genetic and phenotypic variability, with autosomal dominant inheritance and variable penetrance. This mutation is rarely found in RCM, it is usually reported in HCM (OMIM 160760). Genetic screening should be considered to identify patients at risk in families with suspected familial transmission. MYH7 mutations seem to be associated with severe phenotypes, earlier age of onset and more pejorative evolution than other mutations. Conclusion:The evaluation of familial RCM requires an understanding of its variable phenotypic expression and incomplete penetrance. RCM and HCM may coexist in the same family. Genetic testing for hereditary RCM should be considered when secondary causes have been excluded.

Down’s Syndrome with Congenital Heart Disease: Our Surgical Experience

Background: Our objective was to analyze the outcome of patients of Down’s syndrome with congenital heart diseases undergoing cardiac surgery. Methods: This was a retrospective study conducted between January 2013 and June 2019. 49 consecutive patients with Down’s syndrome with congenital heart disease admitted in pediatric cardiac surgery unit at National Institute of Cardiovascular Diseases (NICVD). Patients were followed up postoperatively for in-hospital outcome. Results: Among 49 patients the heart lesion ranked in incidence as follows- VSD 24(48.97%), AV canal defect 12(24.48%), TOF 6(12.24%), PDA 6(12.24%) and ASD 1(2.04%). Pulmonary hypertension was found in 63.25% patients. Moderate pulmonary hypertension was most common, found in 18(38.66%) patients. Severe and mild pulmonary hypertension was found in 10(32.38%) and 3(9.67%) patients respectively. All the patients had undergone surgical correction. The postoperative period was complicated in 44.89% of patients. The most frequent complication was pulmonary infection 20.40%, Wound infection 6.12% and low output syndrome 6.12% were the next. One patient had postoperative heart block, needed permanent pace maker implantation. In-hospital mortality was 12.24%. Conclusion: Patients with Down’s syndrome with congenital heart disease undergoing surgical correction had an acceptable postoperative morbidities and mortality. Cardiovasc. j. 2020; 13(1): 35-39

A case of atrioventricular junction ablation and permanent left bundle branch pacing for patient with refractory atrial fibrillation and hypertrophic cardiomyopathy

Atrioventricular node ablation (AVNA) combined with His bundle pacing (HBP) are feasible, safe, and effective in patients with refractory atrial fibrillation (AF), however, the pacing parameters of sensing and capture threshold maybe sometimes unsatisfactory. Left bundle branch pacing (LBBP) provides obvious advantage in patients with conduction diseases at the distal His bundle for its better sensing, a lower and more stable capture threshold. Among hypertrophic cardiomyopathy (HCM) patients, AF is a common sustained arrhythmia, primarily caused by left atrial dilatation and remodeling. Few is known about the feasibility of electrophysiological performance, safety and clinical effectiveness of atrioventricular junction ablation (AVJA) combined with LBBP in patient with refractory AF and HCM. Here, we report a case of a 56-year-old woman suffering from refractory AF and HCM, however HBP was failed for its unsatisfactory sensing, a high and unstable capture threshold for her, therefore, ablation and LBBB were accepted by her to achieve better rate control. Improvement in symptoms, quality of life, and exercise capacity has been observed during the 1.5-year follow-up. To our knowledge, our case originally confirmed that the combination of AVJA and LBBP, without the defect of AVNA combined with HBP, is a better strategy with feasibility and safety for refractory AF patients with comorbidity of HCM, additionally, it may make LBBP more applicable and valuable among patients suffering from HCM meanwhile pace maker treatments are essential.

Valve repair in patients with infective endocarditis

Background Valve repair is considered the treatment of choice for native mitral and tricuspid valve regurgitation but the rate of feasibility when the defect is caused by acute infective endocarditis (IE) is debated. We report the experience of valve repair versus replacement following IE in a high-volume surgical center. Methods We retrospectively analyzed 351 consecutive patients (123 women) admitted to our department with definite diagnosis of IE. IE occurred on native valves in 219 patients (62%) and on prosthetic valves in 132 (38%). Among native valves, IE involved the aortic valve in 105 cases (48%), mitral valve in 97 cases (44%) and tricuspid valve in 17 (8%). Since only native mitral and tricuspid valves are elegible for surgical repair, we limited the analysis to this subset (114 patients); of them, 92 (80.7%) underwent surgery (the final cohort) and 22 were treated conservatively, 13 due to absence of surgical indication and 9 due to prohibitive surgical risk. Long-term follow-up was obtained by structured telephone interviews. Primary endpoints were mortality and freedom from recurrent endocarditis. Secondary endpoints were the postoperative incidence of major adverse events (hospitalization for any cause, pace-maker implantation, new onset of atrial fibrillation, sternal dehiscence). Results Mean age at surgery was 61.9 years (SD 14.5). Mean vegetation length was 11.6 mm (SD 8.8). Endocarditis was left-sided in 80 (87%). Among the 92 surgical patients, 58 (63%) underwent valve replacement and 34 valve repair (37%). Mortality was similar between valve repair and replacement (15 vs 12%). Adverse events rate (19% vs 16%) and relapse were not statistically different between repair and replacement procedures. Conclusions The present study shows that a sizeable subgroup of consecutive patients with native mitral or tricuspid IE are amenable to valve repair in expert hands. Outcome of repair in IE is comparable to valve replacement mid-term, and should be considered whenever possible, as in degenerative valve disease. Funding Acknowledgement Type of funding source: None

Urodynamic Studies for the Diagnosis of Lower Urinary Tract Symptoms: Experience in BSMMU

Objectives: The aim of urodynamic testing is to obtain objective information regarding urinary bladder storage and voiding function. This investigation provides information of the underlying causes depending on the individual situation and findings. Materials and Methods: This Retrospective study of the UDS was conducted at Urology department of Bangabandhu Sheikh Mujib Medical University, Dhaka from January 2012 to April 2017. A total of 403 cases of urodynamic studies done. Patient age range was 10-85 years. Female Patient was only 41 and post operative cases was only six. Bladder dairy, Ultrasonography of Kidney, ureter, bladder with maximum cystometric capacity (MCC)and post void residue (PVR), Urine culture were done for most of the patient. Patient with indwelling catheter was remove and clean intermittent catheterization was demonstrated. There was no patient with pace maker or valve replacement. We didn’t use routine prophylactic antibiotics, Patients was advice to contact to UDS room physically or over telephone if they feel fever, retention, and other complications. Urodynamic tests include uroflowmetry, postvoid residual measurement, cystometric test, leak point pressure measurement, pressure flow study, and few cases with electromyography. Results: Among them most cases are equivocal and obstructive cases are ranked second about 68 cases (16.87 These were Benign Prostatic Hyperplasia (BPH), stricture urethra, atonic bladder, and dysfunctional voiding. Only five patients had urinary tract infections (1.2%) with rise of temperature but only two had cultural positive and treated with sensitive antibiotics. Organism were streptococcus aureus and pseudomonus. One female and one male patient develop retention and relived by continuous catheterisation and after 7 days removed catheter. Conclusions: Urodynamics could help most diagnosis of LUTSs in most of the cases. We are not yet in using video urodynamics instead urethrocystoscopy may help in assessing the urethra and bladder neck. Bangladesh Journal of Urology, Vol. 21, No. 1, January 2018 p.8-13

A Leadless Pace Maker

In this case a cervico facial cancer patient scheduled for tracheostomy followed by an endoscopy, and known to have a pacemaker, had hemodynamic instability at the start of general anaesthesia which lasted several hours. The anaesthetist in charge used the magnet to override the pace maker which did not respond. Indeed, it was a leadless pacemaker. These devices will be used more frequently and should be carefully scrutinized at the anaesthetic consultation.

P957Characteristics of atrial tachycardia after mitral surgey via biatrial trans-septal approach using high density mapping system

Background The biatrial trans-septal approach (BTSa) ameliorates mitral valve (MV) exposure in difficult cases when routine left atriotomy doesnt"t allow it. Main steps are an oblique incision on the right atrium (RA), reaching medially the right pulmonary veins (PV), a septal incision from the fossa ovalis, extended up to reach the first incision, then on the left atrium (LA). Purpose We aim to study the arrhythmic burden in this post-surgical context, focusing on atrial tachycardia (AT), to investigate the complexity of several possible circuits. Methods All patients (>18yo) with previous MV surgery via BTSa for MV repair or replacement, who underwent ablation of AT from January 2017 to September 2019, were enrolled. Patients ablated for persistent or paroxysmal AF, or with AF during the index procedure were excluded. Patients with associated surgery on other valves or congenital defects, coronary, surgical or percutaneous rhythm interventions weren’t excluded. Electroanatomical mapping was created using 2 different high-density mapping system. Substrate and activation map and radio-frequency (RF) ablation (25-50W, Ablation Index target 400) were realized. Cartographies were analysed to evaluate AT re-entry circuit, critical isthmus (CI) location and characterization, atrial vulnerability. Procedural outcomes (AT termination, sinus rhythm (SR) restoration, anti-arrhythmic drugs (AAD) withdrawal), and peri-procedural complications were also evaluated. Results We enrolled 49 patients (median age 57 ± 15), finding a maximum of 5 AT per procedure (2 ± 1). A total of 112 AT were mapped: the majority (72%) were persistent AT, 8,2% common atrial flutter. Cycle length was 314 ± 74 msec, with proximal-distal activation of coronary sinus (78%). A multiple re-entry circuit was observed in 70% of index AT. We identified 152 critical isthmus (maximum 5 per procedure). Only 27,9% of our patients had a single CI; CTI was the most frequent one (n = 37), envolved in 33% of all AT, while BTS scars altogether were envolved in 65% AT. A complete AT circuit was mapped in the RA, the LA and both atria in respectively 49%, 11,5% and 39%AT. The distribution of CIs is shown in figure 1. Biatrial and left AT leads to superior procedure, RF and fluoroscopy duration (p <0,05). SR was restored in 93,4%of patients, requiring a DC shock in 4 cases. Immediate AAD withdrawal was achieved after 41%procedures. No pericardial, oesophageal, vascular or phrenic complication occurred. 4 pace-maker implantations were realized because of 3 interatrial, 2 AV block and a sinus node dysfunction. Conclusions AT occurring after a BTSa have a high prevalence of multiple re-entry circuits with multiple critical isthmus. Ablation in this context is feasible and safe but often requires a left atrial access. Mapping of both atria should be considered to identify critical isthmus and tailored ablation strategy. Abstract Figure 1. Critical Isthmus Distribution

863Leadless endocardial pacing improves symptoms in patients with failed conventional CRT implant in long term follow up

Funding Acknowledgements EBR Systems, Inc OnBehalf WiSE-CRT and LV-SELECT study and POST-M REGISTRY Background The WiSE-CRT (Wireless stimulation endocardial) system has advantages over conventional epicardial CRT. Whenever conventional CRT failed to implant or failed to echocardiographic response, the WiSE-CRT was implanted as part of the WiSE CRT study (N = 13), as part of the LV-SELECT study (N = 35) or as part of the POST-M REGISTRY (N = 117) over the last 8 years. All these studies have reported high rates of clinical and echocardiographic response compared to conventional CRT. Objectives The purpose of this analysis was to determine the safety and clinical response in the largest available number of implanted patients (pts) with long term follow up of 2 years and the first, second and third generation of WiSE-CRT devices. Method All pts undergoing a WiSE-CRT implantation as part of the WiSE CRT study (N = 13), as part of the LV-SELECT study (N = 35) or as part of the POST-M REGISTRY (N = 117) were analysed (N = 165). Pts were followed-up for 24 months and considered CRT responders if an improvement in NYHA ≥ 1 class from baseline (pre-implant) was achieved. Results In total, 165 pts were implanted, demographics include: 68.2 ± 9.6 year’s old, 81.8% male, 49.7% with history of AFib and 54.5% non-ischaemic aetiology. The mean intrinsic QRS duration was 165.0 ± 32.3 msec (28 pts pace-maker dependent). 161 pts had the system successfully implanted with no major complications, 3 (1.8%) pts developed a pericardial effusion and 1 (0.6%) electrode was lost during implantation and recovered surgically. During the 24-month follow-up period, 20 (12.1%) pts died from any cause, 4 (2.4%) pts developed TIA or Stroke and 15 (9.1%) pts had pocket or transmitter infection. There was a significant improvement in NYHA functional class in 63.6% pts and an average improvement of -26.1 (-45.1, -7.1) msec in QRS duration. Conclusion Despite a history of failed conventional CRT implantation, pts undergoing CRT upgrades with a WiSE-CRT have a high success rate and a complication rate similar to previously described. In addition endocardial LV pacing led to symptomatic improvements in 64% of patients reaching the 24 month of follow up. Abstract Figure 1: Forest Plot NYHA Responder Rat