Severe Left Ventricular Outflow Tract Obstruction Research Articles

Primary vs Staged Biventricular Repair for Neonatal IAA with VSD and LVOTO

BackgroundThis study sought to determine the safety of primary and staged biventricular repair in neonates with interrupted aortic arch (IAA), ventricular septal defect (VSD), and severe left ventricular outflow tract obstruction (LVOTO). MethodsPatients with a fundamental diagnosis of IAA and VSD between 2015 and 2020 were extracted from The Society of Thoracic Surgeons National Database by using a Participant User File. The objective was to compare outcomes for neonates undergoing primary and staged Yasui and Ross operations. Primary end points were operative morbidity and mortality. ResultsDuring the study period, 11.4% (123 of 1079) of neonates with a fundamental diagnosis of IAA and VSD underwent operations indicative of severe LVOTO. Of these patients, 42 (34%) underwent primary biventricular repair (Yasui or Ross/Ross-Konno), and 81 underwent a potential staging procedure (Norwood or hybrid stage I). No differences were observed in preoperative patient characteristics between groups. Neonates undergoing staged repair experienced fewer major complications (0 vs 1; P = .04) and total complications (2 vs 4; P = .02), but similar operative mortality (5% vs 12%; P = .27) as neonates undergoing primary repair. A total of 58 patients undergoing Rastelli, biventricular repair, Yasui, or Ross/Ross-Konno operations with a diagnosis of IAA and VSD and history of neonatal Norwood or hybrid stage I procedures were also identified. Operative mortality for second-stage biventricular conversion operations was 2% (1 of 58). Only 4 centers performed 1 or more complex biventricular repairs for IAA and VSD with LVOTO per year. ConclusionsPrimary and staged biventricular repairs for IAA and VSD with LVOTO are associated with low operative mortality in the modern era and may be favorable to long-term single-ventricle palliation.

The Yasui operation: A single institutional experience over 30 years

ObjectiveThe Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation. MethodsWe retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR). ResultsTwenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention. ConclusionsThe Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

Management of a Critically Ill Patient With Severe Hypertrophic Obstructive Cardiomyopathy Presenting for Emergent Craniotomy Due to Subdural Hemorrhage

Hypertrophic obstructive cardiomyopathy (HOCM) is an inherited cardiac disorder characterized by asymmetric thickening of the myocardium, most commonly of the interventricular septum. Perioperative considerations for patients with HOCM undergoing surgical procedures are discussed, so as to avoid worsening the existing left ventricular outflow tract (LVOT) obstruction, leading to potential cardiovascular collapse. Of particular interest is managing these patients when confronted with vascular aneurysmal disease or hemorrhagic comorbidities with conflicting hemodynamic goals. In this case report, we present the case of a 77-year-old female with known HOCM and severe LVOT obstruction, presenting with intracranial hemorrhage (ICH), acute on chronic subdural hematoma, and potential cerebral aneurysm, undergoing decompressive craniectomy and hematoma evacuation. Anesthetic management of a patient with HOCM presenting for emergent ICH can be challenging given the complex hemodynamic management goals, often conflicting with a patient's comorbidities. Here we describe the prioritization of the patient's underlying HOCM pathology and favored maintenance of afterload given the potentially lethal risk of cardiovascular collapse if LVOT obstruction was encountered.

Abstract 11687: Management of Hypertrophic Obstructive Cardiomyopathy Using Septal Coiling

Introduction: Hypertrophic obstructive cardiomyopathy (HOCM) is a genetic disorder associated with dynamic left ventricular outflow tract obstruction (LVOTO). Medical management of HOCM aims at decreasing the outflow tract gradient and improving symptoms. In a subset of patients, septal reduction is necessary to achieve symptomatic relief. Alcohol Septal Ablation (ASA) is an accepted method for septal reduction, though it carries risks of complications including unpredictable ethanol diffusion, complete heart block requiring pacemaker and death. We sought to evaluate septal coil embolization (CE) as another method to achieve hemodynamic improvement in HOCM patients with severe LVOTO. Method: We identified 53 HOCM patients that underwent CE at our institution. Baseline septal thickness and LVOT gradients were measured at rest and with Valsalva during echocardiogram (ECHO) and catheterization. Immediate and 2-6 month follow-up post coiling gradients were measured using both modalities and results were compared to baseline. Septal thickness was compared before and after CE. Paired t-test was used to compare pre and post values. Results: Mean age was 68, range (31-90) years, 60% were females, 5.6% had prior permanent pacemaker (PPM). Invasive mean gradient (MG) at rest was 56.6 mmHg and immediately post CE was 16.5 mmHg with mean difference (MD) of 40 mmHg (95% CI 10.6-22.4) (p<0.01). Invasive MG following PVC was 123 mmHg pre and 34 mmHg post with MD of 79 mmHg (95% CI 65-111) (p<0.01). ECHO MG at rest was 79 mmHg and 35 mmHg on follow up with MD 44 mmHg (95% CI 27-60) (p<0.01). Septal thickness at baseline was 17.4 mm and was 14.6 mm on follow up with average reduction of 2.8 mm (95% CI 1.5-4.1) (p<0.01). Ninety three percent of patients reported class III-IV NYHA symptoms at baseline, compared to 14% on follow up. A total of 4 patients required new PPM placement and 6 patients required recoiling. Conclusion: Our study demonstrated a safe and effective alternative compared to ASA for treatment of severely symptomatic HOCM patients. The use of coils compared to alcohol ablation for septal reduction affords more precise tissue ablation with a concomitant reduction in the need for permanent pacemaker insertion with a similar hemodynamic and symptomatic success rate.

Clinical characteristics and natural history of pre-adolescent non-syndromic hypertrophic cardiomyopathy

Abstract Background The clinical presentation and natural history of pre-adolescent sarcomeric hypertrophic cardiomyopathy (HCM) has not been systematically characterised. The aim of this study was to describe the clinical characteristics and outcomes of a large, international, multicentre cohort of children diagnosed with non-syndromic HCM below the age of 12. Methods Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children meeting diagnostic criteria for HCM below 12 years of age (pre-adolescent) were collected and compared with 568 diagnosed aged 12–16 years. Patients with syndromic and metabolic HCM were excluded. Results Of 639 (male n=417, 65.3%) children with pre-adolescent HCM, 339 (53.1%) had a family history of HCM and 57 (8.9%) a family history of sudden cardiac death (SCD). At the time of baseline assessment; 132 (20.7%) had heart failure symptoms and 39 (6.1%) reported unexplained syncope. Median maximal left ventricular wall thickness on echocardiogram was 13.6mm (IQR 10–19) with a corresponding median z-score of 8.7 (5.3–14.4). 145 (22.7%) had left ventricular outflow tract obstruction (LVOTO) (maximal LVOT gradient≥30mmHg) and 35 (5.5%) had severe LVOTO (gradient≥90mmHg). Over a median follow up 5.6 years (IQR 2.3–10), 10.5% underwent a myectomy and 23.2% implantable cardiac defibrillator (ICD) implantation for primary (81.8%) or secondary (14.2%) prevention. 42 (6.7%) patients died [SCD 4.9%, heart failure death 0.8%, other 1%] and 21 (3.3%) underwent cardiac transplantation. 69 (10.8%) patients had an arrhythmic event (SCD n=31, resuscitated cardiac arrest n=17, appropriate ICD therapy n=14, sustained VT with haemodynamic compromise n=7). Compared to those presenting after 12 years, those under 12 were less likely to have a family history of SCD (8.9% vs 13%, p:0.047) or report unexplained syncope (6.1% vs 12.3%, p<0.00). The degree of hypertrophy did not differ but a higher proportion of pre-adolescent patients had LVOTO (22.7% vs 14.4%, p<0.00). A higher proportion of pre-adolescent underwent a myectomy (10.5% vs 7.2%, p:0.045) but a lower proportion received a primary prevention ICD (18.9% vs 30.1%, p:0.041). The overall proportion of patients reaching the mortality or arrhythmic end-points did not differ, but SCD and resuscitated cardiac arrest events were more frequent in pre-adolescent patients (4.9% vs 3.9% and 2.7 vs 1.6% respectively). Conclusion Pre-adolescent HCM is associated with a high symptom burden and variable cardiac phenotype, comparable to those presenting later in childhood. Despite baseline similarities and equal overall survival, younger patients were less likely to receive a primary prevention ICD despite being more likely to experience a SCD or resuscitated cardiac arrest. This study suggests that younger patients should not be considered a distinct entity for risk stratification and that similar management strategies to older HCM patients should be employed. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): BHF (British Heart Foudnation) MRC (Medical Research Council)

Abstract 13876: Squat-to-stand Provocation of Dynamic Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

Background: Left ventricular outflow tract (LVOT) obstruction is an important determinant in management of hypertrophic cardiomyopathy (HCM). Valsalva maneuver and amyl nitrite are used in clinical practice during resting echocardiography to induce latent obstruction (LVOT gradient <30 mmHg at rest, ≥30 mmHg with maneuver). With a nationwide shortage of amyl nitrite in 2019, we implemented a “repetitive squat-to-stand” maneuver to provoke LVOT obstruction during echocardiography. Methods: Patients with known or suspected HCM without resting obstruction who underwent repetitive squat-to-stand maneuver during echocardiography between February and September 2019 were included. Maximal instantaneous gradients were recorded. Provocable gradients were compared utilizing a two-sided t-test. Amyl nitrite was not available during the study period. Results: Squat-to-stand assessments were available in 125 patients without resting obstruction (age 58 ± 15 years, 67% male); 114 also performed Valsalva maneuver. 47 of 125 patients (37.6%) demonstrated provocable obstruction. 46 (36.8%) demonstrated latent obstruction provoked by squat-to-stand, which was severe (gradient ≥50 mmHg) in 29 (23.2%). Of the 46 patients, 19 demonstrated latent obstruction only with squat-to-stand but not with Valsalva, 13 of which demonstrated severe obstruction. Among patients with latent obstruction who performed both Valsalva and squat-to-stand maneuver (n=41), squat-to-stand resulted in a higher gradient than Valsalva maneuver (p<0.0001, Figure). Conclusion: Repetitive squat-to-stand maneuver is a clinically useful adjunct to the traditional echocardiographic exam in HCM. This maneuver appears to be more sensitive than the Valsalva maneuver for identifying severe dynamic LVOT obstruction. Use of repetitive squat-to-stand warrants further study in comparison to amyl nitrite, particularly regarding cost savings and diagnostic performance.

Emergent surgical removal of a migrated atrial septal defect occluder: case report

BackgroundAtrial septal defect (ASD) closure has been widely accepted and is now routinely performed using a percutaneous approach under especially echocardiographic guidance Transesophageal echocardiography (TEE). One major complication is dislocation of occluder device during or after the device implantation. Surgical removal may be required, especially when the device stuck in the left ventricular outflow tract (LVOT).Case introductionA 21-year-old female was admitted to our department for percutaneous closure of secundum ASD. Percutaneous closure under the guidance of TEE was recommended for the patients. During device implantation, the TEE showed dislocation of the 22 mm ASD occluder device, stucked into the LVOT and behind the anterior mitral leaflet, producing severe LVOT obstruction Fig. 1). We herein present a safe and quick technique for surgical removal of an ASD occlude device located in the LVOT.ConclusionThis technique provides a safe method for surgical removal of malposition and migration ASD occluder device.

Reverse remodeling after percutaneous transluminal septal myocardial ablation in severe but asymptomatic LVOT obstruction (RASTA) study: Rationale and design of transcatheter septal reduction in asymptomatic patients with severe hypertrophic obstructive cardiomyopathy.

The aim of this study is to evaluate the impact of percutaneous transluminal septal myocardial ablation (PTSMA) on remodeling in asymptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) and severe left ventricular outflow tract (LVOT) obstruction. Symptoms justify invasive treatment in HOCM patients with LVOT obstruction. Adverse structural and functional changes (remodeling) in the heart occur preceding heart failure and sudden cardiac death. Early invasive treatment in asymptomatic patients may reverse adverse remodeling to the same extent as in symptomatic patients. Reverse remodeling after PTSMA in severe but asymptomatic LVOT obstruction (RASTA) study is a prospective single-blind randomized trial (ClinicalTrials.gov number: NCT04230551). Ten asymptomatic HOCM patients with an exertional LVOT gradient ≥50 mmHg (or >30 mmHg in rest) are randomized 1:1 to PTSMA versus conservative therapy, in the absence of mitral valve disease or other indications for cardiac surgery. Five symptomatic (reference group) will undergo PTSMA according to the current guidelines. Remodeling is assessed using extensive cardiac imaging with transthoracic echocardiography and late gadolinium enhancement cardiac magnetic resonance at baseline and during follow-up at 1, 12, and 24 months. Extracellular volume fraction, global, and regional strain analysis, geometry, pressure gradients and changes in four-dimensional velocity mapping are primary parameters to study (reversal of) adverse remodeling. The RASTA study gives insight in cardiac remodeling that may occur in asymptomatic patients after PTSMA. It will provide arguments whether to pursue (or not) a larger trial with clinical endpoints in asymptomatic HOCM patients with severe LVOT obstruction.

Multidisciplinary evaluation and management of obstructive hypertrophic cardiomyopathy in 2020: Towards the HCM Heart Team.

Patients with hypertrophic cardiomyopathy (HCM) exhibit a variable phenotype with ventricular hypertrophy as the cardinal manifestation and left ventricular (LV) outflow tract obstruction (LVOTO) as a key pathophysiologic determinant. Patients with severe LVOTO usually present with exertional dyspnea, exertional syncope, and heart failure symptoms, while successful relief of LVOTO by pharmacological or invasive interventions leads to a dramatic improvement in clinical status. Proper management of obstructive HCM remains challenging and poses numerous clinical dilemmas. Since the development of surgical myectomy over half a century ago, progress in the management of LVOTO in HCM has paralleled technological advances in genetic testing, cardiac imaging, arrhythmic prophylaxis, cardiac surgery and interventional cardiology. These changes have been incorporated in dedicated scientific guidelines on both sides of the Atlantic. However, either the 2011 American guidelines or the 2014 European guidelines remain largely based on expert consensus for lack of recommendations with level of evidence A regarding any of the treatment options commonly employed in HCM. Consequently, management of obstructive HCM patients remains largely subjective and dependent on clinical judgment, local expertise, and patient preference. Following the trend that has emerged for other cardiac diseases amenable to invasive interventions, adequate evaluation and management of obstruction in HCM today requires a multidisciplinary team capable of optimizing referral, choosing the best available options, minimizing complications and ensuring state-of-the-art results. The concept of an HCM Heart Team is coming of age. This review aims to provide an update of available pharmacologic and invasive options for the management of LVOTO in HCM, either in adulthood or in childhood, highlighting areas for multidisciplinary integration and future development.

Balloon assisted translocation of the mitral anterior leaflet to prevent left ventricular outflow obstruction (BATMAN): A novel technique for patients undergoing transcatheter mitral valve replacement.

Transcatheter mitral valve replacement (TMVR) is an option for patients at high risk for mitral valve replacement or repair via sternotomy or left thoracotomy approach. TMVR carries up to 22% risk of left ventricular outflow tract (LVOT) obstruction. Severe LVOT obstruction can have devastating hemodynamic and clinical consequences. We previously presented a novel technique to prevent LVOT obstruction during transapical retrograde mitral valve replacement, by penetrating and ballooning the anterior mitral leaflet (AML), resulting in creation of a "hole" and posterior translocation of AML, then deploying the valve. Three patients underwent TMVR at Saint Louis University for severe mitral regurgitation after being deemed too high risk for surgery, and not candidates for a Mitra-clip procedure. These patients were deemed to be at risk for LVOT obstruction based on the preprocedural evaluation. Via transapical approach, a needle was advanced "through," perforating the AML and wire was placed in the left atrium. Over the wire, an 20-mm valvuloplasty balloon was positioned "within" the anterior leaflet and inflated leading to translocation of the AMVL. Then the valve was deployed. This novel technique has been performed on three patients at our institution. Sapien S3 transcatheter valves were used in all three patients, with 100% procedural success rate. Intraoperative TEE demonstrated no significant LVOT obstruction, cardiopulmonary bypass time was 42-44 min. The balloon assisted translocation of the mitral anterior leaflet to prevent left ventricular outflow obstruction technique described here may offer the option of transcatheter mitral valve implantation in patients at high risk of LVOT obstruction. A variation of this technique to allow application in cases with transseptal approach is under investigation.

Erratum: A novel case of late left ventricular outflow tract obstruction post mitral valve surgery.

[This corrects the article DOI: 10.1093/jscr/rjy357.].

Surgical septal myectomy for relief of dynamic obstruction in Anderson-Fabry Disease

Patients with Anderson-Fabry Disease (AFD) and severe left ventricular hypertrophy complicated by left ventricular outflow tract (LVOT) obstruction may benefit from surgical septal myectomy (SSM). Mid- and late outcomes following surgery have not been established, and we sought to better characterize postoperative outcomes following septal myectomy. Between January 2011 and June 2017, 7 patients (6 females) with AFD underwent SSM. The median (range) age at the time of surgery was 53 (37–72) years; 4 patients had a positive family history of AFD and a preoperative diagnosis of AFD. Extracardiac features suggestive of AFD were present in 3 patients and all but 1 (female) had reduced α-galactosidase A activity. All patients had severe left ventricular hypertrophy and LVOT obstruction on transthoracic echocardiography. Preoperatively, all patients were severely symptomatic with New York Heart Association (NYHA) class III symptoms. There was no early mortality following surgery. The median in-hospital length of stay was 5 (4–7) days with 6 patients reporting NYHA class II or less symptoms at 3 month follow-up. Long-term outcomes were favorable with 4 patients reporting sustained NYHA class II or less symptoms, but 2 patients had recurrence of NYHA class III symptoms without evidence of recurrent LVOT obstruction. In conclusion, SSM appears to provide favorable short- and long-term relief of severe, symptomatic LVOT obstruction but may not alter progression of Fabry cardiomyopathy.

A case report: haemodynamic instability due to true dynamic left ventricular outflow tract obstruction and systolic anterior motion following resuscitation: reversal of haemodynamics on supportive veno-arterial extracorporeal membrane oxygenation.

BackgroundObstruction of the left ventricular outflow tract (LVOT) as seen in hypertrophic cardiomyopathy is a dynamic condition with a wide range of clinical presentations and symptoms.Case summaryWe report the use of veno-arterial extracorporeal membrane oxygenation in a female patient who was resuscitated after out-of-hospital cardiac arrest. Soon after admission the patient developed critical haemodynamic compromise due to severe obstruction of the left ventricle outflow tract and systolic anterior motion (SAM) of the mitral valve. Veno-arterial extracorporeal membrane oxygenation restored haemodynamics and was weaned after 4 days without any haemodynamic compromise due to SAM. The patient was discharged from the intensive care unit at Day 13, and after 3 days at the coronary care unit, she was discharged to ambulatory follow-up with no sequelae.DiscussionVeno-arterial extracorporeal membrane oxygenation restored haemodynamic stability in this patient with dynamic severe LVOT obstruction following cardiac arrest.

Outcomes of Ebstein's Anomaly Patients Treated with Tricuspid Valvuloplasty or Tricuspid Valve Replacement: Experience of a Single Center.

Background:The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).Methods:TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.Results:The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.Conclusions:Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.

Modified Ross-Konno procedure in children: subcoronary implantation technique with Konno incision for annular and subannular hypoplasia.

The technique of subcoronary autograft implantation for the Ross procedure has shown excellent durability of aortic valve function in adults. However, its use in children with hypoplastic aortic annulus or multilevel left ventricular outflow tract obstruction (LVOTO) was traditionally precluded. We combined this technique with a Konno incision and evaluated LVOTO relief and durability of the autograft function in growing patients. Between January 2012 and January 2017, 13 patients with severe LVOTO and hypoplastic aortic annulus underwent Ross-Konno procedure with subcoronary autograft implantation. The median age at operation was 14 months. Six were infants. Concomitant procedures included resection of endocardial fibroelastosis (n = 9), mitral valvuloplasty (n = 2) and aortic arch repair (n = 1). The Konno incision was enlarged with a patch. The autograft was implanted beneath the ostia of the coronary arteries, retaining external support by the native aortic annulus. A newborn with hypoplastic left heart complex and Turner syndrome died in hospital: early mortality of 7.7%. No residual LVOTO or autograft regurgitation was observed at discharge. At a median follow-up of 20 months, no death had occurred. No catheter or surgical reintervention on the aortic valve or any LVOT site was needed. All peak pressure gradients across the LVOT were <10 mmHg. No autograft regurgitation was detected. The Ross procedure using subcoronary implantation technique combined with LVOT Konno enlargement is feasible even in patients with multilevel LVOTO and marked size discrepancy between the autograft and native aortic annulus. Longer follow-up is indicated to confirm the expected durability of the autograft function.

Rationale and design of the TRICHAMPION trial: Triple Chamber Pacing in Hypertrophic Obstructive Cardiomyopathy Patients.

Dual-chamber (DDD) pacing has been used for treatment of patients with hypertrophic obstructive cardiomyopathy (HOCM). Due to inconclusive results in prior trials, current guidelines assign DDD pacing a class IIb indication in selected patients. Prior observations indicate that lack of clinical improvement may result from suboptimal effect of DDD pacing with non-physiological AV intervals due to fusion of intrinsic and paced QRS complex. The Triple Chamber Pacing in Hypertrophic Obstructive Cardiomyopathy Patients (TRICHAMPION) trial is a prospective, randomized, single-blinded, multicenter study to investigate the benefit of atrial synchronous biventricular pacing (CRT-P) in highly symptomatic HOCM patients with severe left ventricular outflow tract (LVOT) obstruction who are not candidates for ablative therapies. AV node ablation is used as key tool to optimize AV intervals in patients with QRS fusion. The primary endpoint is the percentage of patients with symptomatic improvement at 12months, defined as improvement of New York Heart Association functional class, in the Minnesota Living with Heart Failure Questionnaire score and increased cardiopulmonary exercise endurance. The aim of the TRICHAMPION trial is to investigate the benefit of optimized atrial synchronous biventricular pacing in absence of QRS fusion in highly symptomatic HOCM patients with severe LVOT obstruction who are not candidates for ablative therapies.

Short-term results of alcohol septal ablation as a bail-out strategy to treat severe left ventricular outflow tract obstruction after transcatheter mitral valve replacement in patients with severe mitral annular calcification.

To evaluate the outcomes of the early experience of percutaneous alcohol septal ablation in patients with severe left ventricular outflow tract (LVOT) obstruction post transcatheter mitral valve replacement (TMVR). Severe LVOT obstruction with hemodynamic compromise is a complication of TMVR associated with high mortality. Percutaneous alcohol septal ablation has recently been described as a therapeutic option in this setting. Multicenter retrospective review of clinical outcomes of patients undergoing alcohol septal ablation to treat LVOT obstruction after TMVR for severe mitral stenosis with severe mitral annular calcification. Six patients underwent percutaneous alcohol septal ablation to treat LVOT obstruction post-TMVR at six different centers. Five patients had immediate significant reduction in LVOT obstruction with improvement in hemodynamic status while one had persistent LVOT gradient but hemodynamic instability improved. The first patient died on postoperative day 4 due to complete heart block. One patient had initial improvement in LVOT gradient with recurrence on postoperative day 1 thought to be secondary to septal edema, was treated with surgical removal of the transcatheter valve and resection of the anterior mitral leaflet followed by transatrial TMVR and died 3 weeks later due to multi-organ failure. The remaining four patients improved clinically after alcohol septal ablation, were discharged from the hospital and were clinically stable at 30-day follow-up. Percutaneous alcohol ablation provides acute relief of TMVR-induced LVOT obstruction when septal hypertrophy is a contributing factor. This may be a safer alternative to bail-out surgery in this extremely high-risk patient population. © 2017 Wiley Periodicals, Inc.

Role of Perioperative Echocardiography in Revision of Assessment: A Condition of Severe Aortic Stenosis leading to Left Ventricular Dysfunction and Apical Clot

ABSTRACT The prevalence of discrete subaortic stenosis (SAS) in adults with congenital heart disease (CHD) is 8 to 20%, with a male to female ratio of 2:1. Fixed SAS may be due to a discrete fibrous membrane, a muscular narrowing, or a combination of the two. The discrete form of fibromuscular SAS is most frequently encountered (90%), but the tunnel-type lesions are associated with a greater degree of stenosis. We report the case of a 16-year-old boy scheduled for double valve replacement (DVR) based on the preoperative echocardiographic report of rheumatic heart disease (RHD) with severe aortic stenosis (AS), severe aortic regurgitation (AR) and moderate mitral stenosis (MS), and moderate mitral regurgitation (MR) with severe left ventricular (LV) systolic dysfunction and LV apical clot. Preoperative transthoracic echocardiography (TTE) in the operation theater revealed discrete subaortic membrane (SAM) causing severe LV outflow tract obstruction (LVOTO). The patient underwent open heart surgery with resection of the discrete membrane and removal of apical clot. How to cite this article Bhat IH, Mandal B, Damodaran S, Kumar R. Role of Perioperative Echocardiography in Revision of Assessment: A Condition of Severe Aortic Stenosis leading to Left Ventricular Dysfunction and Apical Clot. J Perioper Echocardiogr 2017;5(2):77-81.

Computed tomography imaging to quantify the area of the endocardial subvalvular apparatus in hypertrophic cardiomyopathy – Relationship to outflow tract obstruction and symptoms

BackgroundAbnormalities of the endocardial subvalvular apparatus (SVA), which includes the papillary muscles directly attached to the mitral leaflet and left ventricular apical-basal muscle bundles, are occasionally identified in hypertrophic cardiomyopathy (HCM). Their associations with left ventricular outflow tract (LVOT) obstruction are unknown. MethodsWe retrospectively reviewed cardiac computed tomography image data sets of 107 consecutive patients with HCM [56 obstructive (HOCM) and 51 non-obstructive (HNOCM)] as well as 53 controls. We evaluated anomalies of the SVA, measured the cross-sectional area of the SVA at the level of the LVOT, and subsequently assessed its correlation with the LVOT pressure gradient with and without medication. ResultsThe area of the SVA was greater in HOCM than in HNOCM patients and in the control group (2.5 ± 1.3 cm2, 1.4 ± 0.8 cm2, and 0.9 ± 0.6 cm2, respectively; p < 0.0001). Anomalies in the SVA were more often observed in the HOCM group than in the HNOCM patients and controls (abnormal papillary muscles, 14%, 8%, and 0%, respectively; P = 0.010; LV apical–basal muscle bundles, 73%, 65%, and 45%, respectively; P = 0.0094). Among HOCM patients, logistic regression analysis demonstrated that an SVA area of 2.2 cm2 was an independent risk factor of residual severe LVOT obstruction (≥50 mmHg) after medication (odds ratio, 10.1; 95% confidence interval, 2.05–49.80). ConclusionAn increased area of the endocardial subvalvular apparatus could be an independent risk factor for clinically relevant LVOT obstruction refractory to medication.

Comparison of sublingual isosorbide dinitrate and Valsalva maneuver for detection of obstruction in hypertrophic cardiomyopathy.

IntroductionA left ventricular outflow tract (LVOT) obstruction assessment with a provoking test should be a routine part of the evaluation of patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to compare the utility of the Valsalva maneuver (VM) and sublingual spray application of isosorbide dinitrate (ISDN) for detection of an obstruction.Material and methodsWe prospectively evaluated 81 consecutive HCM patients without severe rest LVOT obstruction (defined as peak rest pressure gradient (PG) ≥ 50 mm Hg). We measured PG at rest, during the VM, after sublingual ISDN spray, and during the VM after ISDN. An obstruction was defined as a PG ≥ 30 mm Hg.ResultsAn obstruction was present in 15 patients (19%) at rest (median and interquartile range of PG 16 (7–26) mm Hg), in 38 patients (47%) during the VM (PG 28 (12–49) mm Hg), in 50 (62%) patients after ISDN (PG 50 (12–79) mm Hg), and in 55 patients (68%) during the VM after ISDN (PG 59 (20–87) mm Hg). The difference in occurrence of obstruction among different provoking tests was statistically significant for all comparisons (p < 0.001, except for the comparison of the ISDN test with the VM during ISDN, p = 0.025).ConclusionsThe ISDN test and the VM are useful screening methods for the detection of an HCM obstruction. Although ISDN appears to be more precise than the VM, the best option is a combination of both methods, which maximizes inducement of LVOT obstruction in patients with HCM.