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BACKGROUND This study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure. METHODS The study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019. RESULTS The DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P < .001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P < .001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P < .001). CONCLUSIONS A primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function.

We aimed to explore the relation between temporal right heart growth and long-term outcomes in patients with pulmonary atresia with an intact ventricular septum (PA/IVS) who underwent balloon pulmonary valvuloplasty (BPV).We performed echocardiography to measure pulmonary valve diameter, right atrial end-systolic area (RAA), and right ventricular end-diastolic area (RVA) before BPV 1 and 5 years after BPV. Primary and secondary end points were to explore temporal changes in right heart structures and to determine echocardiographic parameters related to late adverse events (LAEs).In 31 patients, pulmonary valve diameter significantly increased after BPV, whereas tricuspid valve diameter remained unchanged throughout the 10.0-year follow-up (range, 5.8-14.0 years). After BPV, RAA temporally decreased, whereas RVA significantly increased. There were LAEs in 6 patients (19%), arrhythmias in 2, heart failure in 1, reintervention of the right ventricular outflow tract in 1, and reintervention for residual cyanosis in 2. The rate of freedom from LAEs at 5 and 10 years was 92% and 82%, respectively. Right atrial end-systolic area temporally decreased in patients without LAEs (P.01); however, RAA remained unchanged throughout the period in patients with LAEs (P = .16). Moderate or severe pulmonary regurgitation (hazard ratio = 23.0; 95% confidence interval, 1.3-385; P = .03) and the ratio of RVA to RAA at 1 year after BPV (hazard ratio = 6.3 × 10Disproportional right heart growth was observed in patients with pulmonary atresia with an intact ventricular septum after BPV. Pulmonary regurgitation and increased RAA are crucial for identifying the burden of LAEs among them.

Management of patients with hypertrophic obstructive cardiomyopathy (HOCM) and severe mitral annular calcification, can be challenging. Our cases highlight the importance of addressing all elements contributing to the left ventricular outflow tract (LVOT) obstruction in cases of HOCM: hypertrophic basal interventricular septum, abnormal papillary muscles, systolic anterior motion of the anterior mitral valve leaflet. Addressing the mitral valve repair through the aortotomy, by performing a septal myectomy, papillary muscle realignment, and a resection of aberrant chordae tendineae, allows LVOT obstruction and systolic anterior motion to be successfully corrected. The success of these procedures depended on pre-operative imaging and intra-operative provocation.

Catheter ablation (CA) of ventricular tachycardia (VT) after repair of congenital heart disease may be difficult because of complex anatomy and sometimes unmappable VT. Here, we report a 41-year-old woman with successful CA of unmappable VT in a patient with complete transposition of the great arteries after Rastelli repair. Clinical VT was induced by programmed electrical stimulation, when the mapping catheter was placed at the high anterior right ventricular outflow tract (RVOT). During VT, the local potential at the high anterior RVOT under the right ventricle (RV) – pulmonary artery (PA) conduit was equal to that at the timing of onset of QRS. The VT was unmappable because the hemodynamics deteriorated. Pace mapping was also tried at the aortic cusp and the left ventricular outflow tract (LVOT). Fractionated potential during sinus rhythm was observed at the noncoronary cusp, and the paced QRS morphology at this site was similar to that of the clinical VT, with a delay of 55 ms from pacing to the onset of QRS. However, mapping at the LVOT was impossible due to the difficulty of catheter manipulation. Radiofrequency energy was successfully applied at the noncoronary cusp and the high anterior RVOT under the RV-PA conduit.

Acute decompensated heart failure (ADHF) due to severe aortic stenosis (AS) and concomitant left ventricular outflow tract (LVOT) obstruction is a serious condition. Treatment with medication alone is sometimes difficult, and the efficacy of further interventional strategies has not been fully elucidated. In patients with high surgical risks, combination therapy using transcatheter aortic valve replacement (TAVR) and percutaneous alcohol septal ablation (ASA) is used as a non-surgical intervention. However, this treatment cannot be performed under unstable hemodynamic conditions. This report highlights the utility and efficacy of emergent balloon aortic valvuloplasty (BAV) in controlling heart failure and its potential to serve as a bridge to curative therapy, even in older patients with ADHF due to severe AS with concomitant LVOT obstruction. Furthermore, combination therapy with TAVR and percutaneous ASA could be safely performed after controlling for ADHF using BAV. Non-surgical management is a more feasible treatment option in older patients with ADHF who are at higher risk of complications during surgical intervention.

To compare in-hospital outcome of Evolut-R 34 mm vs. smaller Evolut-R devices and to identify predictors of paravalvular leak (PVL) and deep implantation specific for Evolut-R 34 mm.This single-center retrospective study included 359 consecutive patients undergoing transcatheter aortic valve replacement (TAVR) with Evolut-R 34 mm (N = 84,23.4%) and Evolut-R 23/26/29 mm (N = 275,76.6%) between 2016 and 2019.Patients in Evolut-R 34 mm group were more frequently males, had lower STS score, ejection fraction, and mean aortic gradient compared to the Evolut-R 23/26/29 mm group. Horizontal aorta and large LVOT were more frequent findings in the Evolut-R 34 mm group, whereas calcium volume was comparable among the groups. During TAVR, mean implantation depth and contrast volume were greater in the Evolut-R 34 mm group, compared to the Evolut 23/26/29 mm group. Post-procedurally, 30-day mortality, ≥moderate PVL, device success and pacemaker implantation (PM) rates were comparable between groups. Among independent predictors of ≥moderate PVL, calcium volume (OR:1.04; p0.001) was predictive with different thresholds in both groups, whereas aortic angulation (OR:1.40; p = 0.005) was predictive only in Evolut-R 34 mm group at a cutoff of 60° (AUC:0.73; p = 0.043). Body weight (OR:1.03; p = 0.027), left ventricular outflow tract (LVOT) diameter (OR:1.34; p = 0.001), and mean aortic gradient (OR:0.96; p = 0.006) were independent predictors of deep implantation (mean depth ≥ 6 mm), with LVOT27 mm being predictive specifically for Evolut-R 34 mm (AUC:0.66; p = 0.024).TAVR with Evolut-R 34 mm and Evolut-R 23/26/29 mm showed comparable in-hospital outcome. Aortic angulation60° and LVOT27 mm were predictive respectively of ≥moderate PVL and deep implantation specifically in Evolut-R 34 mm patients.

Background We aimed to determine left ventricular outflow tract (LVOT) calcification impact following transcatheter aortic valve replacement (TAVR) with contemporary transcatheter heart valves. Recent studies reported a higher rate of 2-year mortality with greater than moderate LVOT calcium, but they have not established a reliable and validated method to assess the degree of valve calcification and utilized first-generation valves for their analyses. Materials/methods We conducted a retrospective analysis of patients who underwent TAVR at our institution from 2013 through 2017 with available valves. LVOT calcification quantification was assessed as a continuous variable. Results We included 273 patients: 179 had a non-calcified LVOT (NOLVOTCA) and 96 had a calcified LVOT (LVOTCA). Balloon post-dilatation (BPD) was utilized in 31.3% of LVOTCA vs. 19% of NOLVOTCA (p = 0.029). The Evolut R valve was used in 40.6% vs. 23.4% ( p = 0.002), while the Sapien 3 was used in 59.4% vs. 76.6% (p = 0.004), for the LVOTCA and NOLVOTCA, respectively. Paravalvular leak (PVL) at hospital discharge was higher in LVOTCA (47.5%) versus NOLVOTCA (29.1%; p = 0.004). All-cause mortality (11.5% vs. 10.1%; p = 0.5) and need for permanent pacemaker implantation were similar between the groups. There was a positive trend between LVOT calcification volume and the probability of any PVL (OR 1.012; 95% CI, 0.99–1.02). Conclusions TAVR performed in patients with calcified LVOT is safe, but LVOT calcification adversely impacts TAVR outcomes, with a higher PVL rate despite greater usage of BPD. Calcium quantification did not predict any PVL degree post-TAVR.

Obesity is highly prevalent in patients with obstructive hypertrophic cardiomyopathy (HCM). In this study, we investigated the impact of body mass index (BMI) in patients undergoing septal myectomy (SM) for obstructive HCM.We reviewed 2746 patients who underwent transaortic SM for obstructive HCM from February 1993 through September 2018. Patients were stratified into 3 groups based on BMI (normal weight,25 kg/mPreoperatively, the median left ventricular outflow tract gradient was 58 mm Hg, and there was no difference in gradients across BMI strata (P = .35). The percentage of obese patients with moderate or greater mitral valve regurgitation was lower (45.8%) compared with normal weight (52.9%) and overweight (55.4%) patients (P.001). However, patients with a higher BMI were more likely to have New York Heart Association Functional Classification III/IV limitation at presentation (P.001). After myectomy, anteroseptal thickness (P = .115) and left ventricular outflow tract gradient (P = .210) did not differ between groups. There were 14 (0.5%) deaths within 30 days postoperatively, and the risk was similar across BMI strata (P = .448). Model-estimated changes in average BMI at 10 years postprocedure showed stratum-specific increases ranging from 0.60 to 1.56 kg/mSM is safe and effective in HCM patients with obesity, but the risk of late death increased with increasing BMI. Attention to risk factor management through weight loss may improve late results after SM.

OBJECTIVES It is not uncommon to observe some discrepancy in hemodynamic values characterizing left ventricular outflow tract (LVOT) obstruction preoperatively and in the operating room in patients with hypertrophic obstructive cardiomyopathy. Interpretation of this discrepancy can be challenging. To clarify the extent of the discrepancy, the authors compared hemodynamic variables in patients undergoing septal myectomy at the time of preoperative and intraoperative evaluation. DESIGN Retrospective study. SETTING Single academic medical center. INTERVENTIONS Medical records review, study group-173 patients. MEASUREMENTS AND MAIN RESULTS While there was no statistically significant difference in resting peak LVOT gradients by preoperative transthoracic echocardiography (TTE) compared to intraoperative transesophageal echocardiography (46 mmHg [19-87 mmHg] v 36 mmHg [16-71 mmHg], p = 0.231), the former were higher compared to direct needle-resting LVOT gradient measurements before myectomy (49 mmHg [19-88 mmHg] v 32 mmHg [14-67 mmHg], p = 0.0022). The prevalence of systolic anterior motion was high (94.6% v 91.6%, P = 1.000) both on pre- and intraoperative evaluation. The incidence of moderate/severe mitral was higher intraoperatively (p < 0.0001). Pulmonary artery systolic pressures measured by pulmonary artery catheter provided higher values compared to preoperative TTE estimate (39 mmHg [34-45 mmHg] v 34 mmHg [28-41 mmHg], p < 0.0001). CONCLUSIONS Discrepancy between hemodynamic measurements in the cardiac laboratory and operating room is common and generally should not affect planned patients' care. These changes in hemodynamics might be explained by preoperative fasting, anesthetic agents, volume shifts while supine, and positive-pressure ventilation, as well as the difference in measurement techniques.

We developed a handmade expanded polytetrafluoroethylene (ePTFE) pulmonary valvular conduit (PVC) with bulging sinuses and a fan-shaped ePTFE valve for right ventricular outflow tract (RVOT) reconstruction. We aimed to investigate the results of this device in this multicenter study. From 2001 to 2020, 1776 patients underwent RVOT reconstruction using ePTFE PVCs at 65 institutions in Japan. The median age and body weight were 4.1 years (range, 3 days to 67.1 years) and 13.3 (range, 1.8-91.3) kg, respectively. The median PVC size was 18 (range, 8-24) mm. The median Z-value of the ePTFE PVC was 1.1 (range, -3.8 to 5.0). The ePTFE PVC conditions were investigated by cardiac echocardiography and catheterization. The median follow-up period was 3.3 years (range, 0 day to 16.2 years). There were only 9 cases (0.5%) with PVC-related unknown deaths. Reintervention was performed in 283 patients (15.9%), and 190 patients (10.7%) required explantation. Freedom from reintervention and explantation at 5/10 years were 86.7/61.5% and 93.0/69.1%, respectively. At the latest echocardiography, PVC regurgitation grade was better than mild in 88.4% patients. The average peak RVOT gradient was 15.7 ± 15.9 mm Hg at the latest cardiac catheterization. ePTFE PVC infection was detected in only 8 patients (0.5%). Relative stenosis due to somatic growth was the most common cause of PVC explantation. The performance of ePTFE in terms of durability, valvular performance, and the resistance against infection is considerable and may replace conventional prosthetic materials. Further improvement of the ePTFE membrane is essential to prevent valvular dysfunction.

Background Landiolol enables us to treat the patients with rapid atrial fibrillation (AF) with acute decompensated heart failure (ADHF) efficiently. We sought to determine the role of echocardiography in predicting the prognosis. Methods Among 314 patients, a total 115 ADHF patients with reduced ejection fraction and rapid AF were enrolled. They received landiolol treatment to decrease the heart rate (HR) to 20% within 24 h. The dose of landiolol was increased every 2 h; then, we performed echocardiography repeatedly, at baseline, 2 h, and 24h. We followed the patients after discharge for 180 days, and checked cardiac death and HF hospitalization as major adverse cardiac events (MACE). Results During initial hospitalization, 5 patients (4%) died. During 180 days after discharge, 19 (16%) out of 115 patients experienced MACE (2 cardiac death, 17 HF rehospitalization, 5 in-hospital death). Multivariate analysis showed that the change in left ventricular outflow tract-velocity time integral (LVOT-VTI) at 2 h was the most significant predictor for MACE (hazard ratio =1.21, 95% confidence interval: 1.10-1.83, p=0.0001). Kaplan-Meier curves demonstrated the patients with deteriorated LVOT-VTI at minimum dose landiolol suggested the high-risk patients for MACE (χ2=30.9, p Conclusions During landiolol treatment, the patients with deteriorated LVOT-VTI predicted the poor prognosis. We may detect the high-risk patients by two-point echocardiography. UMIN000020084. Registered 1 November 2013 – prospective study https://upload.umin.ac.jp/cgi-open-bin/ctr/ctr.cgi?function=brows&action=brows&type=summary&language=J&recptno=R000023203

Transcatheter mitral valve replacement carries a risk of left ventricular outflow tract obstruction associated with mortality. We present a case of left ventricular outflow tract obstruction that resolved spontaneously when chords to the anterior mitral leaflet were found to have ruptured. (Level of Difficulty: Advanced.), Central Illustration

Infective endocarditis can have peri-annular spread and involve the valvular annulus and adjacent cardiac structures, leading to tissue necrosis and peri-annular abscess. This process may cause pseudoaneurysm formation and other rare and potentially life-threatening complications, so their identification and correct diagnosis are crucial. We describe a case of an 81-year-old woman, with a history of aortic valve replacement and worsening of symptoms, that presents at the imaging a pseudoaneurysm of the aortic root complicated at the same time by 2 life-threatening conditions: fistulization in the Right Ventricular Outflow Tract (RVOT) and the compression of Right Coronary Artery (RCA). This case underlines the importance of imaging, especially Coronary Computed Tomography Angiography (CCTA), in the diagnosis and follow-up of infective endocarditis and its complications, especially in a patient not eligible for surgery.

Hypertrophic obstructive cardiomyopathy requires surgical myectomy when heart failure symptoms persist despite best medical therapy. Minimally invasively myectomy with robotic surgery can be performed in experienced centers allowing for surgical correction of accompanying mitral valve pathologies. The extent of myectomy is important to relieve left ventricular outflow tract obstruction while care should be taken to prevent iatrogenic ventricular septal defects or heart blocks caused by excessive removal of septal tissue. We report the use of intracardiac ultrasound during robotic surgery to intraoperatively assess the myocardium before and after myectomy to increase the safety of this procedure. Hypertrophic obstructive cardiomyopathy (HOCM) is a genetic myocardial disease characterized by thickened myocardium and obstruction of the left ventricular outflow tract (LVOT). 1 Patients with symptoms of heart failure resistant to drug treatment are candidates for surgical septal myectomy. Conventionally performed through a midline sternotomy, septal myectomy can be carried out via transapical, transaortic, or transmitral approaches. The transmitral approach allows surgical treatment of the concomitant mitral valve pathology through a left atriotomy. Robotic septal myectomy can be performed in experienced centers with results comparable to the classical sternotomy approach with the added benefits of minimally invasive surgery. 2 Excessive removal of septal tissue can result in a heart block or a ventricular septal defect while an inadequate myectomy can leave a high gradient in the LVOT. Intracardiac ultrasound can assist the surgeon in performing a safe and adequate myectomy. We describe our technique for intracardiac ultrasound guided robotic septal myectomy in a patient with HOCM and infective endocarditis.

Background Residual or new left ventricular outflow tract (LVOT) obstruction after an aortic valve replacement poses special challenges with respect to operative techniques. Our study assesses this gap. Methods From January 1993 to May 2019, 18 patients underwent a septal myectomy at Mayo Clinic for subaortic obstruction after aortic valve replacement. We evaluated their demographics, clinical presentation, and echocardiograms, and the type of prior valve replacement, need for repeat replacement, and their short- and long-term outcomes. The data were analyzed using descriptive statistics. Results All patients underwent septal myectomy for LVOT obstruction at a median interval of 7 years (interquartile range, 3-15 years) from their prior aortic valve procedure. Preoperatively, the median left ventricular outflow tract gradient was 57 mm Hg (interquartile range, 44-77 mm Hg); 10 patients (55.5%) had systolic anterior motion (SAM) of the mitral leaflets. Repeat replacement of the aortic valve at the time of myectomy was needed in 14 patients, and septal myectomy alone was performed in 4 patients. One hospital death occurred 34 days after myectomy and aortic valve replacement, and 2 patients needed permanent pacemaker placement for complete heart block. Conclusions Septal myectomy after aortic valve replacement may be performed with repeat replacement of the valve, if there is coexisting prosthetic dysfunction, through a normally functioning bioprosthesis or through an apical approach when visualization through the aortic prosthesis is poor. The complexity of reoperation supports a liberal approach to myectomy at the time of aortic valve replacement when there is significant subaortic septal hypertrophy.

Background Electrocardiographic (ECG) criteria have been proposed to localize the site of origin of outflow region ventricular arrhythmias (VAs). Many factors influence the QRS morphology of VAs and may limit the accuracy of these criteria. Objective The purpose of this study was to assess the accuracy of ECG criteria that differentiate right from left outflow region VAs and localize VAs within the aortic sinus of Valsalva (ASV). Methods One hundred one patients (mean age 52 ± 16 years; 55 [54%] women) undergoing catheter ablation of right ventricular outflow tract (RVOT) or ASV VAs with a left bundle branch block, inferior axis morphology were studied. ECG measurements including V2 transition ratio, transition zone index, R-wave duration index, R/S amplitude index, V2S/V3R index, V1–3 QRS morphology, R-wave amplitude in the inferior leads were tabulated for all VAs. Comparisons were made between the predicted site of origin using these criteria and the successful ablation site. Results Patients had successful ablation of 71 RVOT and 38 ASV VAs. For the differentiation of RVOT from ASV VAs, the positive predictive values and negative predictive values for all tested ECG criteria ranged from 42% to 75% and from 71% to 82%, respectively, with the V2S/V3R index having the largest area under the curve of 0.852. Morphological QRS criteria in leads V1 through V3 did not localize ASV VAs. The maximum R-wave amplitude in the inferior leads was the sole criterion demonstrating a significant difference between right ASV, right-left ASV commissure, and left ASV sites. Conclusion ECG criteria for differentiating right from left ventricular outflow region VAs and for localizing ASV VAs have a limited accuracy.

Background Pacemapping (PM) is a useful maneuver for aiding premature ventricular complex (PVC) ablation. Its standalone clinical value is still to be defined. Objectives The purpose of this study was to analyze the efficacy of a predefined PM protocol for low-burden PVC ablation, regardless of their site of origin (SOO) and the presence of structural heart disease. Methods This was a prospective, nonrandomized, multicenter study. The PM protocol was performed when 94% correlation, and 3 radiofreqency (RF) applications were delivered. Results Of 185 patients, 105 (57%) underwent activation mapping, 60 (32%) were PM-guided, and 20 (11%) were canceled due to absence of PVCs. Baseline QRS, PVC burden, and outflow tract origin were independent predictors of PM-guided ablation. A higher proportion of right ventricular outflow tract SOO in the PM group (52% vs 40%; P = .03) was observed. Mean target area was 0.6 ± 0.9 cm2. Mean 10-ms isochronal area in local activation time (LAT)-guided procedures was higher (1.7 ± 2.3 cm2; P Conclusion When LAT mapping is precluded, application of a PM-guided ablation protocol directed to >94% matching correlation target area is a more efficient alternative with comparable clinical results.

Evaluation of prosthesis-patient mismatch (P-PM) after transcatheter aortic valve replacement (TAVR) by transthoracic echocardiography (TTE) has provided conflicting results regarding its impact on outcomes. Whether post-TAVR computed tomography angiography (CTA) evaluation of P-PM can improve our understanding is unknown. We aimed to evaluate the inter-modality (TTE vs. CTA) agreement, inter-valve platform (balloon-expanding valve [BEV] vs. self-expandable valve [SEV]) differences in P-PM severity, and outcomes related to P-PM after TAVR.We analyzed patients with both CTA and TTE before and after TAVR. Indexed effective orifice area was calculated using two methods: TTE-derived left ventricular outflow tract (LVOT) area from measured diameter and post-TAVR CTA-measured area. Body size specific cut-offs for P-PM severity were used: for body mass index (BMI) ​ ​30 ​kg/mA total of 447 patients were included (median age, 83 years; 54% male). The prevalence of P-PM (moderate or severe) was lower with CTA vs. TTE (3.5% vs. 19.5%, p ​ ​0.001). The prevalence of P-PM measured by TTE was more common in BEV compared to SEV (p ​= ​0.002), while CTA assessment showed no difference in P-PM incidence and severity between TAVR platforms (p ​= ​0.40). In multivariable analysis, CTA-defined but not TTE-defined P-PM was associated with mortality after TAVR (HR:3.97; 95%CI,1.55-10.2; p ​= ​0.004). Both CTA-defined and TTE-defined P-PM were associated with the composite of death and heart failure rehospitalization.Although post-TAVR CTA substantially downgraded the prevalence of P-PM compared to TTE, it identified a subset of patients with clinically relevant P-PM which associated with outcomes.

The Ross-Konno operation is effective for enlargement of a hypoplastic aortic annulus and left ventricular outflow tract. It is, however, an invasive operation with the potential for early and late cardiac morbidity. We propose a new technique of annular and outflow tract enlargement which avoids the septal incision while effectively opening up the outflow tract and aortic annulus.

Background Anomalous aortic origin of the left coronary artery (AAOCLA) with an extended transseptal course behind the right ventricular outflow tract (RVOT) is a rare variant that poses challenges not addressed by current surgical techniques. We utilized a novel transconal approach in 7 consecutive patients. Methods A retrospective review was made of a prospectively collected database for consecutive patients undergoing transconal unroofing of transseptal AAOLCA. Surgical repair entails transection of the RVOT, unroofing the septal course of the AAOLCA, followed by RVOT extension with a rectangular strip of autologous pericardium. Preoperative characteristics, operative details, and postoperative course were abstracted. Results All 7 patients identified were symptomatic. Median age was 48 years (range, 12 to 62). The AAOLCA with transseptal course was confirmed by computed tomography angiography. Three patients had provocative testing demonstrating anterolateral ischemia. Four patients underwent cardiac catheterization with intravascular ultrasound and indexed fractional flow reserve, confirming flow-limiting lesion. Importantly, 3 patients had negative provocative noninvasive testing for ischemia. Median postoperative hospital length of stay was 6 days (range, 4 to 12). No mortality or major complications occurred during a median follow-up of 0.75 years. Postoperative evaluation demonstrated anatomically patent unroofed AAOLCA with improved indexed fractional flow reserve compared with preoperative (0.59 ± 0.16 vs 0.90 ± 0.03, P = .05). Conclusions Complete unroofing of AAOLCA with transseptal course repaired with posterior extension of RVOT is an effective technique with excellent early outcome. Multimodality provocative testing is critical to evaluate these lesions as individual studies may be misleading. Intravascular ultrasound with indexed fractional flow reserve is clinically useful to confirm the hemodynamic significance of specific lesions.

Objectives This study sought to evaluate cardiac computed tomography (CCT) findings and their clinical impact among patients being considered for transcatheter mitral valve replacement (TMVR). Background CCT is used to evaluate whether patients are candidates for TMVR, but limited data exist on the yield of such tests. Methods Patients referred for pre-procedural CCT for TMVR planning in the context of failing mitral bioprosthetic valves, annuloplasty rings, and severe native valve disease with annular calcification were included in this study. CCT findings were analyzed to evaluate for suitability for TMVR. In the subset of patients who underwent TMVR, echocardiographic and procedural characteristics were recorded. Results Among 80 patients who underwent pre-procedural CCT, the mean age was 71.8 ± 11.4 years, 60% were women, and the mean Society of Thoracic Surgeon score was 9.4 ± 6.7. Most cases were referred for valve-in-native annular calcification planning (n = 43), followed by valve-in-valve (n = 29), and valve-in-ring procedures (n = 8). A total of 51 (64%) patients did not undergo TMVR, 37 of whom had high-risk features identified on CCT. The most common reason for exclusion was related to large annular size, followed by heightened risk of left ventricular outflow tract (LVOT) obstruction. Among 29 patients (36%) who underwent TMVR, the 30-day mortality rate was 17%. Five patients experienced LVOT obstruction, 4 of whom were predicted by CCT. Following TMVR, 5 patients had at least moderate peri-valvular regurgitation. Conclusions A minority of patients referred for TMVR planning ultimately undergo the procedure. CCT identifies unsuitable anatomy and leads to exclusion in a significant number of cases.

Objectives The aim of this study was to explore if right ventricular (RV) contractile function and its coupling to pulmonary circulation (PC) were associated with successful weaning from venoarterial−extracorporeal membrane oxygenation (VA-ECMO) at maintenance of pump flow. Background Limited data are available on predictors of successful weaning from VA-ECMO. Methods A total of 79 patients with cardiogenic shock underwent transthoracic echocardiography to evaluate weaning from ECMO and were prospectively enrolled between 2016 and 2019. The noninvasively measured RV-PC coupling index was acquired by indexing tricuspid annular S' velocity, tricuspid annular plane systolic excursion (TAPSE), fractional area change (FAC), and RV free-wall longitudinal strain (FWLS) to right ventricular systolic pressure (RVSP). Results Transthoracic echocardiography was performed at a median 3.0 days (range 1 to 6 days) after ECMO initiation at a median ECMO flow of 3.2 l/min (range 3.0 to 3.6 l/min). The RV-PC coupling matrix, tricuspid annular S'/RVSP, TAPSE/RVSP, and RV FWLS/RVSP exhibited satisfactory predictive performances for predicting successful weaning from ECMO. Using the best cutoff values derived from the area under the receiver-operator characteristic curve, tricuspid annular S'/RVSP demonstrated a significantly better predictive performance than conventional echocardiographic parameters (left ventricular ejection fraction >20%, left ventricular outflow tract time-velocity integral ≥10 cm, and mitral annular S' ≥6 cm/s). Conclusions Echocardiographic RV-PC coupling metrics exhibited a significantly better performance for predicting successful weaning from VA ECMO compared with conventional echocardiographic criteria at maintenance of pump flow.

Patient prosthesis mismatch (PPM) is an important factor of the outcome in transcatheter aortic valve implantation. However, the impact of PPM in transcatheter pulmonic valve implantation (TPVI) has not been studied. Based on the narrowest valve stent diameters in two views of fluoroscopy, internal geometric orifice area (GOA) of the valve stent was calculated and indexed by body surface area (BSA), deriving iGOA. To define PPM in TPVI, receiver operating characteristics (ROC) curve analysis for iGOA for predicting significant residual right ventricular outflow tract (RVOT) gradient was used to derive the optimal cut-off value of iGOA. Our cohort were divided into 2 groups: PPM versus non-PPM. The clinical data were compared between 2 groups. TPVI was performed using Melody valve in 101 patients. Significant RVOT residual pressure gradient (≥ 15 mmHg) was observed in 31 patients (39.6%). Over a mean follow up periods of 6.9 ± 2.7 years, 22 patients (21.8%) required repeat interventions (16 transcatheter, 11 surgical, and both in 5 patients). Based on the ROC analysis, the best cut-off value of iGOA was 1.25 cm2/m2 (area under the curve 0.873, p

Background Current evidence suggests that Brugada syndrome (BrS), far from being a purely electrical condition, is associated with subtle mechanical abnormalities primarily affecting the right ventricle (RV). We aimed to characterize RV function in BrS and investigate the echocardiographic profile of patients with arrhythmic events, with a special focus on parameters of RV dyssynchrony using speckle-tracking echocardiography (STE). Methods An echocardiogram was performed in 71 BrS patients and 25 healthy controls. STE was performed to assess regional and global RV mechanics, including RV outflow tract shortening (RVOTS). RVOT contraction time was considered to calculate the modified RV mechanical dispersion (RVMDm). Arrhythmic events were prospectively evaluated in the BrS cohort. Results Compared to controls, BrS patients showed subtle contractile abnormalities, including impaired RV longitudinal strain, higher RV index of myocardial performance (RIMP) and lower RVOTS. BrS patients also exhibited a greater contraction delay between the lateral and the septal aspect of the RV. After a median follow-up of 7.3 year (IQR 5.2–10.8), 6 patients presented malignant arrhythmic events. RIMP >0.50, RVOTS 42 ms showed high sensitivity for the identification of BrS patients with arrhythmic events during follow-up. Conclusions Subtle RV mechanical abnormalities were present in BrS patients. RIMP and RVOTS, a novel STE-derived parameter, were found to be sensitive markers of arrhythmic events. Adding the RVOT contraction time to the analysis of RVMD may help identify patients at higher risk, reflecting the importance of the RVOT mechanical substrate in the assessment of the arrhythmic risk in BrS.

Aims Left ventricular outflow tract(LVOT) obstruction after mitral valve replacement can be life-threatening once occur. We simulated mitral valve replacement preoperatively using dynamic, three-dimensional(3D) printed models to help predict LVOT obstruction in this study. Methods 56 patients who underwent mitral valve replacement were included. Prediction of LVOT obstruction in vitro was based on the data from 4 sources: digital, anatomical, flexible, and dynamic model. Digital 3D models were designed based on computed tomography (CT) image dataset and printed with photopolymer resin to create a 3D anatomical model, which contributed to the morphology display. Then, flexible models were made from specialized silicone, which is similar to cardiac tissue in terms of its softness and elasticity. Dynamic function was achieved by coupling flexible models to a mock circulatory system (MCS). Besides, surgery simulation and hemodynamic testing was done using dynamic 3D printed model and patients were regrouped based on hemodynamic change. Finally, different methods for prediction of LVOT obstruction as well as classification based on two-dimensional image data and dynamic model were compared with surgical results as golden standard. Results (1)Qualitatively, the prediction of LVOT obstruction using the dynamic 3D model was the most accurate and was consistent with clinical outcomes. In the four patients who developed LVOT obstruction after surgery, only two were at a high risk based on the other three models. (2)Quantitatively, the area of neo-LVOT predicted by the digital, anatomical, and flexible models was higher compared with the dynamic models and in-vivo after surgery. (3)Classification based on traditional criteria(two-dimensional image data) was different from surgical results. While the difference between dynamic model and surgical results was not statistically different. Conclusions After coupling the flexible model with the mock circulatory system, the dynamic 3D model predicted LVOT obstruction more accurately with hemodynamic testing compared with morphological evaluation. 3D printing can assist surgeons to better plan mitral valve replacement than traditional image data.

We describe a rare patient with a large pseudoaneurysm of the right ventricular outflow tract that had developed after placement of a right ventricle–pulmonary artery conduit. A 7-mm Amplatzer Septal Occluder device was used to exclude the pseudoaneurysm, allowing for safer subsequent surgical repair. (Level of Difficulty: Advanced.), Central Illustration

Surgery for truncus arteriosus has an early mortality of 3% to 20%, with a long-term survival of approximately 75% at 20 years. Nowadays, truncus arteriosus repair is mostly done in the neonatal period together with a single-staged repair of concomitant cardiovascular anomalies. There are many challenging subgroups of patients with truncus arteriosus, including those with clinically significant truncal valve insufficiency, an interrupted aortic arch, or a coronary artery anomaly. In fact, truncal valve competency appears to be the most important factor influencing the outcomes after truncus arteriosus repair. The use of a conduit during truncus arteriosus repair invariably requires reoperation on the right ventricular outflow tract. Through improvements in perioperative techniques over time, many children are now living well into adulthood after repair of truncus arteriosus, albeit with a high rate of reoperation. Despite this, the long-term outcomes of truncus arteriosus repair are good, with many patients being asymptomatic and with a quality of life comparable to the general population.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is pathologically characterized by fibrofatty replacements.1 It is reported that the subtricuspid/peritricuspid area and the left ventricular inferolateral wall may be the only affected regions in the early stages of ARVC.2 This lipomatous or lipofibromatous composition causes delayed excitation. This late conduction through diseased myocardium is described as an epsilon wave, which is included in the revised Task Force diagnostic criteria.3 Herein, we report a case of ARVC with epsilon wave, in which radiofrequency catheter ablation (RFCA) on left ventricular outflow tract (LVOT) eliminated the epsilon wave.

Graphical abstract, Highlights • Cardiac complications are the most common cause of death in dialysis patients. • Cardiac complications include coronary disease, arrhythmias, and valvular disease. • Valvular calcification can lead to valve dysfunction, conduction issues, and embolism. • With an increasingly dialysis-dependent and aged population, clinical prevalence is high.

With the advent of transcatheter mitral valve replacement (TMVR), the concept of the neo-left ventricular outflow tract (LVOT) was introduced and remains an essential component of treatment planning. This paper describes the LVOT anatomy and provides a step-by-step computed tomography methodology to segment and measure the neo-LVOT while discussing the current evidence and outstanding challenges. It also discusses the technical and hemodynamic factors that play a major role in assessing the neo-LVOT. A summary of expert-based recommendations about the overall risk of LVOT obstruction in different scenarios is presented along with the currently available methods to reduce the risk of LVOT obstruction and other post-procedural complications.

For decades, researchers have investigated the ideal material for clinical use in the cardiovascular field. Several substitute materials are used clinically, but each has drawbacks. Recently we developed biodegradable and elastic poly(ε-caprolactone-co-D,L-lactide) (P(CL-DLLA)) copolymers by adjusting the CL/DLLA composition, and evaluated the long-term efficacy and outcomes of these copolymers when used for right ventricular outflow tract (RVOT) replacement. This P(CL-DLLA) material was processed into a circular patch and used to replace a surgical defect in the RVOT of adult rats. Control rats were implanted with expanded polytetrafluoroethylene (ePTFE). Histologic evaluation was performed at 8, 24, and 48 weeks post-surgery. All animals survived the surgery with no aneurysm formation or thrombus. In all periods, ePTFE demonstrated fibrous tissue. In contrast, at 8 weeks P(CL-DLLA) showed infiltration of macrophages and fibroblast-like cells into the remaining material. At 24 weeks, P(CL-DLLA) was absorbed completely, and muscle-like tissue was present with positive staining for α-sarcomeric actinin and cardiac troponin T (cTnT). At 48 weeks, the cTnT-positive area had increased. The biodegradable and elastic P(CL-DLLA) induced cardiac regeneration throughout the 48-week study period. Future application of this material as a cardiovascular scaffold seems promising. STATEMENT OF SIGNIFICANCE: Biomaterials for reconstruction of tissue deficiencies in cardiovascular surgery require having suitable mechanical properties for cardiac tissue and biodegradation resulting in native tissue growth. Several biodegradable polymers such as poly-ε-caprolactone (PCL) and polylactic acid (PLA) have excellent biocompatibility and already been widely used clinically. In general, PCL and PLA are quite mechanically rigid. Meanwhile, significant elasticity is required in the high-pressure environment of the heart while the material is being replaced by new tissue. The present study provides a novel four-armed crosslinked poly(ε-caprolactone-co-D,L-lactide) (i.e., P(CL-DLLA)) material for cardiac patch, which was demonstrated properties including tissue-compatible, super-elastic nature, that made it suitable for long-term, in vivo RVOT repair. This super-elastic biomaterial could be useful for reconstruction of various muscular tissues deficiencies.

A 16-year-old boy was referred for an electrophysiological study for documented regular narrow complex tachycardia. A diagnosis of a concealed left lateral accessory pathway was made with an eccentric atrial activation sequence both during tachycardia and right ventricular (RV) pacing. The pathway was mapped at the left posterior mitral vestibule during RV pacing, performed through the distal tip of the His bundle catheter pushed into right ventricular outflow tract. An unusual response to ventricular stimulation with alternation of QRS complex width and morphology was noted. The possible mechanisms are hereby discussed.

In patients with interrupted aortic arch and ventricular septal defect (VSD) with a small left ventricular outflow tract (LVOT), either aortopulmonary amalgamation or a Ross-Konno type procedure can be performed to create stable systemic outflow. We sought to analyze factors associated with these different surgical approaches.We retrospectively identified patients who underwent surgical repair for interrupted aortic arch/VSD at our institution between 1998 and 2017. Of these, 43 patients had a small, native LVOT that was unsuitable for systemic outflow. Patient data were retrospectively collected for this cohort and analyzed.Aortopulmonary amalgamation was performed at 7 days (interquartile range [IQR], 5-10) in 30 patients (group I). Within group I a primary Yasui repair with ventricular septation was performed in 3 patients and a Norwood-type repair in the other 27. Of these 27, 19 underwent subsequent biventricular conversion at 9 months (IQR, 7-11). In contrast 13 patients underwent a Ross procedure at 12 days (IQR, 6-27) (group II). Compared with group I, group II patients had a smaller VSD (3.5 vs 5.1 mm, P.001) that was more often remote from the semilunar valves (38% vs 13%, P = .02). Operative mortality occurred in 1 group I patient (4%) at the time of biventricular conversion and 2 group II patients (15%) during the Ross procedure. After a 5.2-year (IQR, 3.2-7.4) follow-up there were 2 additional mortalities in each group, all unrelated to cardiac disease.When native LVOT in interrupted aortic arch/VSD is unsuitable for systemic outflow, size and location of the VSD can be used to tailor the surgical approach to establish biventricular circulation with favorable intermediate-term outcomes.

To address various problems with conventional surgical procedures for transposition of the great arteries with left ventricular outflow obstruction, the aortic translocation procedure has been reconsidered as an alternative surgical procedure. We developed another innovative translocation technique, the half-turned truncal switch operation, to make use of various anatomical characteristics. The truncal block involving both semilunar valves is harvested en bloc and anastomosed to the opposite ventricular outflow tract after a half-turn. Temporarily detached coronary arteries are re-anastomosed to the reversed aortic wall defects. The key advantage of this half-turned truncal switch operation is the creation of hemodynamically faultless right and left ventricular outflow tracts including competent valve functions and a coronary circulation ensured by the posteriorly translocated aortic valve, the maximally used autologous pulmonary valve, and anastomosis of the coronary arteries to the confronting aortic wall defects.

Background Alcohol septal ablation (ASA) in patients with hypertrophic cardiomyopathy (HCM) can lead to heart rhythm disturbances including complete heart block (CHB) and atrial and ventricular arrhythmias. Objective We aimed to evaluate the utility of long-term arrhythmia monitoring with an implantable cardiac monitor (ICM) after ASA. Methods Between February 2014 and March 2019, 56 patients with HCM undergoing ASA were enrolled in a prospective study and underwent ICM implantation. Kaplan-Meier survival analysis was used to assess the rate of ICM-detected arrhythmic events. Results The mean age was 59 ± 11 years, and 20 (36%) were women. The median (25th, 75th percentile) resting left ventricular outflow tract gradient obtained by echocardiography was 43 (22, 81) mm Hg. Greater than 1 septal perforating artery was injected in 48 patients (86%). The Kaplan-Meier cumulative rate of ICM-detected arrhythmic events at 18 months of follow-up was 71%, with an event rate of 43% occurring within 3 months of ASA. The cumulative rate of the ICM-detected first atrial fibrillation event at 18 months was 37%, and the corresponding rate of CHB was 19%. All atrial fibrillation and CHB events were actionable, leading to the initiation of anticoagulation and pacemaker implantation, respectively. No baseline demographic or procedural variables were identified as independent predictors of an increased risk of developing ICM-detected arrhythmic events. Conclusion After ASA, ICM is effective in capturing clinically actionable arrhythmic events in patients with HCM regardless of patient's baseline risk factors.

Aortic insufficiency sometimes occurs after arterial switch operation for transposition of the great arteries. Left ventricular outflow tract enlargement by Konno operation and right ventricular outflow tract reconstruction are necessary during the aortic valve replacement in case of small aortic annulus. A vertical incision from the anterior pulmonary artery to the right ventricular outflow tract in Konno operation can be applied if the coronary anatomy is Shaher type 1, 2, 6, and 8. However, a vertical incision jeopardizes the coronary artery which traverses on the right ventricular outflow tract in Shaher type 3a, 3c, 3d, 4, 5c, 7c, and 9. In this difficult situation, atrioventricular groove patch plasty as part of Konno operation is a choice of surgery.

Background Aortic valve area (AVA) is commonly determined from 2-dimensional transthoracic echocardiography (2D TTE) by the continuity equation; however, this method relies on geometric assumptions of the left ventricular outflow tract which may not hold true. This study compared mean differences and correlations for AVA by planimetric (2-dimensional transesophageal echocardiography [2D TEE], 3-dimensional transesophageal echocardiography [3D TEE], 3-dimensional transthoracic echocardiography [3D TTE], multi-detector computed tomography [MDCT], and magnetic resonance imaging [MRI]) with hemodynamic methods (2D TTE and catheterization) using pairwise meta-analysis. Method Ovid MEDLINE®, Ovid EMBASE, and The Cochrane Library (Wiley) were queried for studies comparing AVA measurements assessed by planimetric and hemodynamic techniques. Pairwise meta-analysis for mean differences (using random effect model) and for correlation coefficients (r) were performed. Results Forty-five studies (3014 patients) were included. Mean differences between planimetric and hemodynamic techniques were 0.12 cm2 (95%CI 0.10–0.15) for AVA (pooled r = 0.84; 95%CI 0.76–0.90); 1.36cm2 (95%CI 1.03–1.69) for left ventricular outflow tract area; and 0.13 cm (95%CI 0.07–0.20) for annular diameter (pooled r = 0.76; 95% CI 0.64–0.94); 0.67 cm2 (95%CI 0.59–0.76) for annular area (pooled r = 0.74; 95%CI 0.55–0.86). Conclusions Planimetric techniques slightly, but significantly, overestimate AVA when compared to hemodynamic techniques.

Double-chambered right ventricle [DCRV)] is an uncommon congenital anomaly in which anomalous muscle bands divide the right ventricle into a proximal high-pressure and distal lowpressure chamber. Very rarely DCRV presentation for diagnosis and treatment occurs during adulthood as most cases are diagnosed and treated during childhood and usually does not recur. This case report describes a rare etiology for an acquired recurrent DCRV that presented in adulthood. An aorto-right ventricular fistula following closure of a subaortic ventricular septal defect with right ventricular outflow tract muscle resection during childhood triggered the development of an acquired DCRV.

Background The initial depolarization vector of outflow tract (OT) ventricular arrhythmia (VA) varies in different origins, which may help to predict OT-VA origin more accurately. Objective The purpose of this study was to develop a more accurate electrocardiographic (ECG) criterion for differentiating between left and right OT-VA origins. Methods We studied 275 patients with successful ablation in the right ventricular outflow tract (RVOT) (n = 207) or left ventricular outflow tract (LVOT) (n = 68) in the development cohort. Amplitude of the QRS complex within initial 40 ms (QRSi40) in precordial leads was measured. A novel criterion for identifying OT-VA origin was developed based on the development cohort. Predictive performance of novel criterion was further validated by comparing with previous ECG criteria (V2S/V3R index, V2 transition ratio, and transition zone index) in the validation cohort with 107 patients (RVOT 75; LVOT 32). Results QRSi40 of identical precordial leads were significantly greater in the LVOT group than the RVOT group (P Conclusion V2QRSi40 is a novel and accurate ECG criterion to predict OT-VA origin that outperforms previous criteria.

Three canines were identified with aberrant drainage of the caudal vena cava to the left atrium, consistent with imperforate cor triatriatum dexter, and concurrent patent foramen ovale. All three had concurrent significant pulmonic stenosis with varying degrees of hypoplasia of the right ventricular outflow tract. Echocardiography, positive contrast studies, and angiography confirmed the diagnosis. Surgical redirection of the caudal vena cava bloodflow to the right atrium was attempted unsuccessfully in two symptomatic patients. The third patient was asymptomatic, and correction was not attempted. Possible embryologic causes and possible approaches in future cases are discussed.

Sex differences in the long-term prognosis of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients undergoing alcohol septal ablation (ASA) remain unclear, especially in the Chinese Han population.This cohort study included 320 HOCM Chinese Han patients who underwent ASA because of symptomatic left ventricular outflow tract (LVOT) obstruction. Patients were grouped according to sex: females (mean±standard deviation age [SD] 50.7±6.8 years) and males (mean±SD age 52.6±7.3 years). Individuals were followed over the long term.Pre-procedure, women had more symptoms (New York Heart Association [NYHA] class III-IV 67.3% vs 56.3%, p=0.03), more atrial fibrillation (23.5% vs 14.6%, p=0.047) than men. Transient complete atrioventricular block after ASA was more common in woman than in men (34.0 vs 23.4%; p=0.048). Residual LVOT gradient, post-procedural residual left ventricular wall thickness, NYHA functional class, and adverse arrhythmic events were comparable between the two groups. The 10-year survival rate (77% vs 89%, p=0.037) and the annual adverse arrhythmic event rate (1.3% vs 0.4%, p0.01) following ASA were significantly worse in women compared with men. Kaplan-Meier analysis showed a significantly lower survival in women compared with men (p=0.023). In multivariable modelling, female sex remained independently associated with higher all-cause mortality (hazard ratio, 1.12; 95% confidence interval, 1.08-1.27; p=0.03) when adjusted for age, NYHA class III-IV symptoms, and other cardiovascular comorbidities.Female patients with HOCM undergoing ASA tended to have more severe symptoms and adverse arrhythmic events. The 10-year survival rate after ASA was significantly worse in women compared with men with HOCM. Sex may need to be considered as an important factor in the clinical management of patients with symptomatic HOCM.

Pathophysiological differences in relief of pulmonary stenosis (PS) as opposed to pulmonary regurgitation (PR) by percutaneous pulmonary valve implantation (PPVI) remain elusive, but might impact current assessment of procedural success and ultimately indications.Invasive pressure measurements, cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed before pre-stenting (BMS), after BMS and after PPVI in patients with either PS or PR.In PS (n = 14), BMS reduced the right ventricular (RV) to systemic pressure ratio (0.8 ± 0.2 vs. 0.4 ± 0.1%; p .01), improved RF EF (53 ± 14 vs. 59 ± 12%; p = .01) but introduced free PR (PR fraction post 39 ± 12%; p .01) with no changes in effective RV stroke volume (SV). PPVI eliminated PR (PR fraction 5 ± 3%; p .01) and improved effective RV SV (p .01) with no changes in RV EF (p = .47). Peak VO2 improved significantly after BMS, with no changes following PPVI (26 ± 9 vs. 30 ± 11 vs. 31 ± 10 ml/kg*min). In PR (n = 14), BMS exaggerated PR (PR fraction post 47 ± 10) with reduction in effective RV SV (pre 43 ± 9 vs. post 38 ± 8 ml/mExercise capacity in patients with right ventricular outflow tract dysfunction is primarily afterload-dependent.

Balloon valvuloplasty is routinely performed in dogs with severe pulmonic stenosis using a transvenous approach. We report a novel transventricular approach to stent the right ventricular outflow tract. An 18-month old Havanese with frequent syncope was referred with a diagnosis of severe valvular pulmonic stenosis. Transvenous approaches were unsuccessful; therefore, two Palmaz XL 29 mm by 12 mm transhepatic biliary stents were deployed across the valve using a direct right ventricular approach. The echocardiographically derived systolic pressure gradient decreased from 133 mmHg to 39 mmHg with resolution of clinical signs. The patient continues to do well after 3 years with occasional syncope when extremely excited despite pressure gradient increases to 130 mmHg at 12 months and 174 mmHg at 22 months.

We present an exceedingly rare case of right ventricular outflow tract obstructing mass in an adult patient who presented with dyspnea and dizziness. Transthoracic echocardiography and computed tomography angiogram of the chest showed a large mass in the right ventricle obstructing the outflow tract. A dual right ventriculotomy and right atriotomy surgical approach was taken to completely resect the mass from the interventricular septum, which subsequently confirmed histopathologically the mass as a mature cystic teratoma.

The right ventricular outflow tract (RVOT) velocity time integral (VTI), an echocardiographic measure of stroke distance, correlates with cardiac index. We sought to determine the prognostic significance of low RVOT VTI on clinical outcomes among patients with acute pulmonary embolism (PE).We conducted a retrospective review of echocardiograms on Pulmonary Embolism Response Team (PERT) activations at our institution. The main outcome was a composite of death, cardiac arrest, or hemodynamic deterioration.Of 188 patients, 30 met the combined outcome (16%) and had significantly lower RVOT VTI measurements (9.0 cm v 13.4 cm, p 0.0001). The AUC for RVOT VTI at a cutoff of 10 cm was 0.78 (95% CI 0.67-0.90) with a sensitivity, specificity, negative predictive value, and positive predictive value of 0.72, 0.81, 0.94, and 0.42, respectively. Fifty-two patients of the cohort were classified as intermediate-high-risk PE and 21% of those met the combined outcome. RVOT VTI was lower among outcome positive patients (7.3 cm v 10.7 cm, p = 0.02).Low RVOT VTI is associated with poor clinical outcomes among patients with acute PE.

Objectives The aim of this study was to demonstrate the safety and functionality of the Alterra Adaptive Prestent and SAPIEN 3 transcatheter heart valve (THV) in patients with dysfunctional, dilated right ventricular outflow tract (RVOT) greater or equal to moderate pulmonary regurgitation (PR). Background Significant variations in the size and morphology of the RVOT affect the placement of transcatheter pulmonary valves. The Alterra Prestent internally reduces and reconfigures the RVOT, providing a stable landing zone for the 29-mm SAPIEN 3 THV. Methods Eligible patients had moderate or greater PR, weighed >20 kg, and had RVOT diameter 27 to 38 mm and length >35 mm. The primary endpoint was device success, a 5-item composite: 1 Alterra Prestent deployed in the desired location, 1 SAPIEN 3 THV implanted in the desired location within the Prestent, right ventricular–to–pulmonary artery peak-to-peak gradient Results Enrolled patients (N = 15) had a median age and weight of 20 years and 61.7 kg, respectively; 93.3% were in New York Heart Association functional class I or II. Device success was 100%. No staged procedures were necessary. No THV dysfunction was reported to 6 months. No serious safety signals were reported. Conclusions This early feasibility study demonstrated the safety and functionality of the Alterra Adaptive Prestent in patients with congenital RVOT dysfunction and moderate or greater PR. Durability and long-term outcome data are needed.

A 20-year-old male without any symptoms was referred for heart murmur on a medical examination. A thrill was palpable at the upper left sternal border. His cardiac murmur showed respiratory variation. The systolic murmur was louder (Levine grade IV/VI) during expiration and diminished during inspiration (Levine grade I/VI). He was thin and had a narrow thoracic cage in the anteroposterior direction due to straight back syndrome (SBS). An echocardiogram and a right ventriculogram showed changes in the diameter of the right ventricular outflow tract (RVOT) on respiration. During expiration, the RVOT was compressed and narrow, while it was expanded during inspiration. Cardiac catheterization demonstrated a 10-mmHg of pressure gradient across the RVOT during expiration but no pressure gradient during inspiration. Thus, respiratory compression to the RVOT by a narrow thoracic cage due to SBS was the cause of the cardiac murmur with respiratory alterations. Our case highlights the importance of physical examination, including an inspection of the patient’s physique.