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One of the major determinants of exercise intolerance and limiting symptoms among patients with obstructive hypertrophic cardiomyopathy (HCM) is an elevated intracardiac pressure resulting from left ventricular outflow tract obstruction. Aficamten is an oral selective cardiac myosin inhibitor that reduces left ventricular outflow tract gradients by mitigating cardiac hypercontractility. In this phase 3, double-blind trial, we randomly assigned adults with symptomatic obstructive HCM to receive aficamten (starting dose, 5 mg; maximum dose, 20 mg) or placebo for 24 weeks, with dose adjustment based on echocardiography results. The primary end point was the change from baseline to week 24 in the peak oxygen uptake as assessed by cardiopulmonary exercise testing. The 10 prespecified secondary end points (tested hierarchically) were change in the Kansas City Cardiomyopathy Questionnaire clinical summary score (KCCQ-CSS), improvement in the New York Heart Association (NYHA) functional class, change in the pressure gradient after the Valsalva maneuver, occurrence of a gradient of less than 30 mm Hg after the Valsalva maneuver, and duration of eligibility for septal reduction therapy (all assessed at week 24); change in the KCCQ-CSS, improvement in the NYHA functional class, change in the pressure gradient after the Valsalva maneuver, and occurrence of a gradient of less than 30 mm Hg after the Valsalva maneuver (all assessed at week 12); and change in the total workload as assessed by cardiopulmonary exercise testing at week 24. A total of 282 patients underwent randomization: 142 to the aficamten group and 140 to the placebo group. The mean age was 59.1 years, 59.2% were men, the baseline mean resting left ventricular outflow tract gradient was 55.1 mm Hg, and the baseline mean left ventricular ejection fraction was 74.8%. At 24 weeks, the mean change in the peak oxygen uptake was 1.8 ml per kilogram per minute (95% confidence interval [CI], 1.2 to 2.3) in the aficamten group and 0.0 ml per kilogram per minute (95% CI, -0.5 to 0.5) in the placebo group (least-squares mean between-group difference, 1.7 ml per kilogram per minute; 95% CI, 1.0 to 2.4; P<0.001). The results for all 10 secondary end points were significantly improved with aficamten as compared with placebo. The incidence of adverse events appeared to be similar in the two groups. Among patients with symptomatic obstructive HCM, treatment with aficamten resulted in a significantly greater improvement in peak oxygen uptake than placebo. (Funded by Cytokinetics; SEQUOIA-HCM ClinicalTrials.gov number, NCT05186818.).

A total of 35 patients completed the study. Mean age was 19.8 (±4.2) years, with a male-to-female ratio of 4:3. A total of 25 patients had uncorrected TOF, and 10 had undergone intracardiac repair. After the procedure, patients with uncorrected TOF showed a significant increase in oxygen saturation from 84.7% (±1.4%) to 94.6% (±1.2%). Procedural success was 91.4%, with 3 patients experiencing significant restenosis. No procedural complications were observed. There were no arrhythmic events until the first year of follow-up. At 1-year follow-up, the mean RVOT pressure gradient was significantly decreased, and all patients remained symptom-free. In conclusion, combined balloon pulmonic valvuloplasty and conal artery occlusion is a safe and effective method for alleviating RVOT obstruction in patients with TOF, showing promising intermediate-term outcomes with minimal complications.

Background Dynamic left ventricular outflow tract obstruction (LVOTO) is an increasingly recognized life-threatening condition in critically ill patients, with ICU mortality rates reaching 53% in septic shock populations. Bedside transthoracic echocardiography (TTE) has become indispensable for early detection due to its diagnostic sensitivity and real-time monitoring capabilities in ICU settings. Cases We present three prototypical cases managed at our tertiary center: (1) A 66-year-old male with volume depletion-induced LVOTO (peak gradient 29 mmHg) reversed by fluid resuscitation; (2) A 71-year-old female developing inotrope-induced obstruction (36 mmHg gradient) requiring β-blockade; and (3) A septic shock patient with severe LVOTO managed through combined fluid resuscitation, sedation, and vasopressor optimization. Management Pathophysiology-guided interventions included: fluid resuscitation for hypovolemia (Case 1), analgesia/sedation for hypercontractility (Case 2), and norepinephrine for preload and afterload augmentation in sepsis (Case 3). Serial TTE monitoring confirmed gradient reductions. Outcomes Early TTE-directed therapy achieved: (1) vasopressor weaning in 48 h (Case 1), (2) hemodynamic stabilization within 24 h (Case 2), and (3) septic shock reversal (Case 3). Conclusion LVOTO is a life-threatening yet underrecognized complication in critical care, requiring prompt and tailored management based on its diverse etiologies. Bedside TTE serves as an essential tool for rapid diagnosis, guiding targeted interventions, and optimizing outcomes in these high-risk patients. It should be stressed that almost all hypotensive patients in contemporary ICUs should receive bedside TTE as a standard of care, given its critical role in identifying underlying causes and informing timely treatment decisions.

Objective The purpose of this research was to explore the application value of a three-dimensional (3D)-printed heart in surgery for left ventricular outflow tract (LVOT) obstruction. Methods From August 2019 to October 2021, 46 patients with LVOT obstruction underwent surgical treatment at our institution. According to the treatment method, 22 and 24 patients were allocated to the experimental and control groups, respectively. In the experimental group, each patient’s 3D-printed heart model was used for simulated preoperative surgery, and then the Morrow operation was performed. In the control group, only the Morrow operation was performed, without simulated preoperative surgery using a 3D-printed heart model. The intraoperative and postoperative data of patients in the two groups were recorded, and the clinical data of patients were compared between the two groups. Results The operation time, cardiopulmonary bypass time, intraoperative blood loss, hospitalization time, LVOT pressure difference (LVP), postoperative interventricular septal thickness (IST), aortic regurgitation (AR), systolic anterior motion (SAM), and postoperative left ventricular flow velocity (LVFV) were significantly lower in the experimental group than in the control group ( P  < 0.05). The inner diameter of the left ventricular outflow tract (IDLV) was larger in the experimental group than in the control group ( P  < 0.05). There was no significant difference in the postoperative ejection fraction, atrioventricular block rate or complication rate between the two groups ( P  > 0.05). Conclusion A 3D-printed heart model for simulated surgery in vitro is conducive to formulating a more reasonable surgical plan and reducing the trauma and duration of surgery, thereby promoting the recovery and maintenance of the heart.

Background To investigate the characteristics of right ventricular outflow tract obstruction (RVOTO) in recipient twins of twin-to-twin transfusion syndrome (TTTS), including its prevalence, perinatal outcomes, and the impact of fetoscopic laser coagulation (FLC) on postnatal RVOTO status. Methods This retrospective study included recipient twins of TTTS treated with FLC at the Asan Medical Center between January 2011 and December 2023. Among those diagnosed with RVOTO, the recipient twins were categorized into two groups based on postnatal outcomes: RVOTO improvement versus persistence. Prenatal ultrasound findings and neonatal outcomes were compared between the groups. To identify the predisposing factors for RVOTO, the entire recipient population was divided into RVOTO and non-RVOTO groups, followed by univariate and multivariable logistic regression analyses. Results Among the 284 TTTS cases, 24 recipient twins (8.5%) were diagnosed with RVOTO. RVOTO improved following FLC in 7 cases (29.2%). In the remaining 17 cases with persistent RVOTO, six were stillbirths. In the 11 survivors, five had mild RVOTO and required no intervention, while six underwent intervention (four with balloon valvuloplasty and two with surgical correction). Multivariate analysis identified the initial Quintero stage (odds ratio [OR] 2.64), pulsatile umbilical vein (OR 3.44), and tricuspid regurgitation (OR 30.66) as significant independent risk factors for RVOTO. Conclusions RVOTO is a relatively common cardiac complication in advanced TTTS. Timely FLC may lead to postnatal improvement. Serial ultrasound assessment is essential for early detection, risk stratification, and individualized counseling.

Accessory mitral valve tissue is a rare congenital cardiac anomaly that can lead to left ventricular outflow tract obstruction. We present the case of an older female patient with accessory mitral valve tissue, a subaortic membrane, an unruptured aneurysm of the aortic sinus, and left ventricular outflow tract obstruction. Successful excision of the accessory mitral valve tissue and repair of the aortic sinus were performed. Postoperative echocardiography showed complete removal of the subaortic membrane, with a residual accessory mitral valve tissue (5 mm). Color Doppler imaging revealed a significant reduction in mosaic flow signals in the left ventricular outflow tract, with the peak blood flow velocity decreasing to 1.6 m/s. The postoperative course was uneventful, and the patient was followed up with echocardiography at 3 days, 5 days, 1 month, 3 months, and 6 months after the surgery. Accessory mitral valve tissue is a rare congenital defect frequently associated with other cardiovascular congenital malformations. This report also provides a comprehensive clinical review of accessory mitral valve tissue, covering anatomical classification, associated cardiac anomalies, pathophysiology, diagnostic approaches, and treatment strategies to offer an improved clinical understanding of the condition.

Isolated native pulmonary valve endocarditis is rare. We present a rare case of isolated native pulmonary valve endocarditis resulting in severe right ventricular outflow tract obstruction in an immunocompetent patient with surgically repaired ventricular septal defect caused by Burkholderia cepacia.

This study aimed to identify the phenotypic features contributing to the development of left ventricular outflow tract obstruction (LVOTO) in patients with hypertrophic cardiomyopathy (HCM) and to evaluate the genotype‒phenotype relationship. This cross-sectional study included 96 patients diagnosed with HCM (mean age: 56.9 ± 13.5 years, 32.3% female). The patients were divided into hypertrophic nonobstructive cardiomyopathy (HNCM; n = 60) and hypertrophic obstructive cardiomyopathy (HOCM; n = 36) groups. All patients underwent CMR. Patients (n = 77) who had previously provided formal approval underwent a genetic examination that included 18 genes. The anterior mitral leaflet (AML) length/LVOT diameter ratio, posterior mitral leaflet (PML) length/LVOT diameter ratio, and anterolateral papillary muscle (AL-PM) mobility were associated with LVOTO, independent of the basal IVS thickness, abnormal chordal attachment, and bifid PM. An AML length/LVOT diameter ratio of ≥ 2.30, a PML length/LVOT diameter ratio of ≥ 1.83, and an AL-PM mobility of ≥ 57.7% were predictors of LVOTO, with good sensitivity and specificity. Positive variants (VUS, LP, and P) were detected in 37.7% (29 of 77) of the patients who underwent genetic testing. The LP/P variant was detected in 20.8% (16 of 77) of patients. Three groups (variant-negative, VUS, and LP/P groups) had significant differences in the LVOT diameter (median 14, 12, and 10 mm, respectively; p = 0.021), AML length (mean 25.3, 26.5, and 27.5 mm, respectively; p = 0.029), AML length/LVOT diameter ratio (median 1.74, 2.33, and 2.85, respectively; p = 0.006), PML length/LVOT diameter ratio (median 1.29, 1.82, and 2.10, respectively; p = 0.045), and abnormal chordal attachment (6.3%, not observed, and 31.3%, respectively; p = 0.009). The AML length/LVOT diameter ratio, PML length/LVOT diameter ratio, and AL-PM mobility were associated with LVOTO. In addition, genetic testing results may provide information regarding the phenotypic expression of patients with HCM.

For over 50 years, surgical septal myectomy has been the preferred treatment for drug-refractory heart failure symptoms in obstructive hypertrophic cardiomyopathy (HCM). However, given the relatively youthful adult ages at which HCM surgery is usually performed, it is informative to evaluate longer-term results of myectomy after ≥10 years. We identified 139 consecutive obstructive HCM patients (50 ± 15 years of age; 55% men) who underwent surgical myectomy, 2003 to 2010 at Tufts HCM Center and followed 11.3 ± 2.7 years (range to 17). Operative mortality was low (0.6%) and left ventricular (LV) outflow gradients at rest were reduced from 56 ± 40 mm Hg preoperatively to 1 ± 7 mm Hg postoperatively, durable over the study period, with no patient requiring reoperation for the residual gradient. Over follow-up, 129 of 139 patients (93%) were alive ≥10 years after myectomy, including 17 patients ≥15 years. Of 118 patients with complete long-term clinical follow-up data, 109 (92%) experienced clinical improvement to New York Heart Association classes I or II. In 9 patients (8%) refractory class III/IV symptoms reoccurred 6.6 ± 3.9 years postoperatively, including 4 who ultimately underwent a heart transplant. After myectomy, there were 2 late HCM-related deaths, but none suddenly; notably 6 patients (12%) with prophylactic implantable cardioverter-defibrillators experienced appropriate therapy terminating ventricular tachycardia/ventricular fibrillation after myectomy. Survival following myectomy was 91% at 10 years (95% confidence interval: 85, 96%) not different from the age- and gender-matched general United States population (log-rank p = 0.64). In conclusion, myectomy provides permanent abolition of outflow gradients with reversal of heart failure and highly favorable long-term survival, representing a low-risk:high-benefit option when performed in experienced HCM centers. Myectomy did not protect absolutely against arrhythmic sudden death events, underscoring the importance of risk stratification in operative patients.

In selected patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO), the arterial switch operation (ASO) may be the procedure of choice. This study reviews the clinical outcomes of TGA-VSD-LVOTO patients after ASO and compares mechanisms of LVOTO in this patient group to a historical series of cardiac specimens. This retrospective analysis included all cases with TGA-VSD-LVOTO who underwent ASO between January 1977 and December 2023. Additionally, a series of non-operated cardiac specimens with TGA-VSD-LVOTO was selected and examined for morphological comparison. Eleven patients with TGA-VSD-LVOTO underwent ASO. Eight of them had TGA-VSD, and three had Taussig-Bing anomaly. LVOTO mechanisms were multifactorial, including posteriorly deviated infundibular septum and fibrous tissue masses. Median age at ASO was 0.4 (0.07-1.8) years. Ten patients underwent primary LVOTO relief during ASO; no in-hospital mortality occurred. Two patients died >30 days post-ASO at 3.1 months and 6.0 years. Median follow-up was 19.0 (11.1-26.8) years, all survivors in NYHA class I. The patient without initial LVOTO relief did require reoperation during follow-up for progressive LVOTO at 3.5 months post-ASO. Two patients had moderate residual LVOTO at latest follow-up (gradient 30-50 mmHg). No significant neoaortic valve regurgitation was observed. From the anatomical specimen series, 10 of 33 TGA-VSD-LVOTO specimen were deemed eligible for ASO, revealing similar LVOTO mechanisms as the clinical cases. ASO is feasible in selected patients with TGA-VSD-LVOTO showing good long-term outcomes with preserved neoaortic valve function and no reoperations for LVOTO after initial relief.