Ventricular Outflow Tract Obstruction Research Articles

Aficamten for Symptomatic Obstructive Hypertrophic Cardiomyopathy.

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.).

Application of Transcatheter Electrosurgery in Structural Heart Interventions: A Report of Three Different Techniques

Transcatheter electrosurgery is a novel technique to traverse or cut tissue, within blood-filled spaces, using alternating current directed by guidewires and catheters. We report the first series of transcatheter electrosurgery procedures in Asia including transcaval access for transcatheter aortic valve implantation, intentional aortic leaflet laceration to mitigate the risk of coronary occlusion in transcatheter aortic valve implantation and intentional laceration of anterior mitral valve leaflet to mitigate the risk of left ventricular outflow tract obstruction. These procedures are potentially valuable in Asian patients because of their smaller body build compared to Caucasians posing a higher risk of coronary or left ventricular outflow tract obstruction during transcatheter valve implantation. There is a need to adopt transcatheter electrosurgery in Asia to treat more surgically inoperable patients, including proper simulation training, bench training and clinical proctorship.

Comparing clinical profiles, in-hospital complications and one-year cerebrovascular events in typical and midventricular TTS-results from a retrospective study between two third-level centers

Abstract Funding Acknowledgements None. Background Apical ballooning is considered the typical form of Takotsubo syndrome (TTS) and occurs in about 75-80% of cases. Midventricular (MV) is the most frequent atypical variant and represents about 15% of TTS. Clinical and analytical characteristics, early complications and the risk of cerebrovascular events (CVEs) between these two specific groups are poorly studied. Purpose of the study To investigate the clinical profile, laboratory variables, in-hospital complications and one-year CVEs between typical and MV variants of TTS. Methods we retrospectively examined the patients admitted to two third-level hospitals with a diagnosis of TTS. A total of 248 TTS met InterTAK diagnostic criteria. Clinical features, myocardial enzymes, echocardiographic variables, early complications and one-year CVEs were assessed. Results Of 248 patients diagnosed with TTS, a total of 205 (83%) patients presented with typical and 43 (17%) with MV variant of TTS. Patients with MV form were younger (age mean ± SD 68 ± 12 vs 74 ± 11 years, p value = 0.002) and had a lower incidence of hypertension (46% vs 73%; p value &amp;lt; 0.001) than those with apical variant. On admission, patients with MV form presented a lower degree of left ventricular dysfunction (EF mean ± SD, 44 ± 9% vs 41 ± 9%, p value = 0.018) and a tendence not to present left ventricular outflow tract obstruction (LVOTO) (0 vs 9%, p value = 0.049). Lower N-terminal pro-brain natriuretic peptide (NT-proBNP) levels on admission (median [IQR], 881 [99 – 3049] pg/mL vs 3466 [677 - 9067] pg/mL, p value &amp;lt;0.001) and at peak (3150 [947- 4437] pg/mL vs 4622 [1886 - 13163] pg/mL, p value = 0.005) were observed in MV group compared to the typical form. In-hospital cardiovascular (CV) complications were frequently observed (46% in the overall population) and appeared to be more frequent in the typical variant (51% vs 33%, p value= 0.035); in particular, we reported a higher prevalence of AF (16% vs 2%, p value = 0.022). The number of CS and VT cases is remarkable in both the subgroups (13% vs 7%; 5% vs 2%), with a higher prevalence in the typical form, even if not reaching a statistically significant difference between the two groups (p= 0.437; p= 0.695). No significant differences were observed in LV thrombosis, and in on admission and one-year CVEs between the two groups, although with a higher prevalence in the typical variant (respectively 3 vs 0, 10 vs 1 and 5 vs 0). Conclusions MV TTS presents some differences in clinical characteristics, analytical values and echocardiographic parameters compared to the typical variant. In-hospital CV complications are remarkable, and more frequently observed in typical variant, although not reaching a statistically significant difference in CS and VT. Similarly, more CVE were observed in the typical TTS group, although in the absence of a statistical significance. The importance of close monitoring in the acute phase is confirmed for both variants under study.

Utility of Pulmonary Angiography by 128-Slice Computed Tomographic Scanner in Diagnosis of Tetralogy of Fallot Cases

Tetralogy of Fallot (TOF) is a significant cause of cyanotic congenital heart disease (CHD) encountered in childhood with few cases manifesting in adulthood. It has four classical features (ventricular septal defect, overriding of aorta, hypertrophy of right ventricular hypertrophy, and right ventricular outflow tract obstruction), but the clinical presentation and course can be variable. Due to various anatomical variations and complex anatomy, presurgical planning and postoperative follow-up by pulmonary computed tomographic angiography (CTA) have a very important role. With continued technological advances and the availability of 128-slice computed tomographic (CT) scans, they now play an important role in TOF preoperative evaluation and workup, assisting by minimizing routine invasive digital subtraction catheter angiography. The fast scan of a 128-slice CTA with very sensitive detectors is a very useful modality for studying the complex anatomy and variations as well as its utilization for postoperative management. In this article, we report four cases of TOF where we used a 128-slice scan for performing pulmonary angiography (Optima 660, GE 128, 2180 Premier Row, Orlando, FL 32809, U.S.A.) for preoperative diagnosis and management of three cases and work up for revision surgery for an already operated case with a nonfunctional modified Blalock-Taussig shunt with additional lung parenchymal findings simultaneously. This study will explain the advantageous role of the 128-slice CT scanner over the lesser-slice CT scanners with the ability of pulmonary CTA to facilitate accurate diagnosis and postoperative management.

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

Retrofitting the Heart: Explaining the Enigmatic Septal Thickening in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy is the most common genetic cardiac disease and is characterized by left ventricular hypertrophy. Although this hypertrophy often associates with sarcomeric gene mutations, nongenetic factors also contribute to the disease, leading to diastolic dysfunction. Notably, this dysfunction manifests before hypertrophy and is linked to hypercontractility, as well as nonuniform contraction and relaxation (myofibril asynchrony) of the myocardium. Although the distribution of hypertrophy in hypertrophic cardiomyopathy can vary both between and within individuals, in most cases, it is primarily confined to the interventricular septum. The reasons for septal thickening remain largely unknown. In this article, we propose that alterations in muscle fiber geometry, present from birth, dictate the septal shape. When combined with hypercontractility and exacerbated by left ventricular outflow tract obstruction, these factors predispose the septum to an isometric type of contraction during systole, consequently constraining its mobility. This contraction, or more accurately, this focal increase in biomechanical stress, prompts the septum to adapt and undergo remodeling. Drawing a parallel, this is reminiscent of how earthquake-resistant buildings are retrofitted with vibration dampers to absorb the majority of the shock motion and load. Similarly, the heart adapts by synthesizing viscoelastic elements such as microtubules, titin, desmin, collagen, and intercalated disc components. This pronounced remodeling in the cytoskeletal structure leads to noticeable septal hypertrophy. This structural adaptation acts as a protective measure against damage by attenuating myofibril shortening while reducing cavity tension according to Laplace Law. By examining these events, we provide a coherent explanation for the septum's predisposition toward hypertrophy.

The results of modified Konno procedure for complex left ventricular outflow tract obstruction

Background: Complex left ventricular outflow tract obstruction (c- LVOTO) necessitates an diagnosis, follow – up and operation to void severe left ventricular outflow tract obstruction. There are some procedures, for example: Aor–ventriculoplasty (the Konno-Rastan procedure), apicoaortic conduit… However, all procedures require a prosthetic or allogeneic valve as well as postoperative anticoagulation therapy, reoperation, or both, the modified Konno procedure which avoid that problems. Methods: This was a review of seri c-LVOTO  cases who underwent modified Konno procedure from 1/2014 to 12/2022. There were 8 patients ( 5 males and 3 females). The mean age was 5,6 years (range from 1 – 10 years) and the mean weight was 12,6  kilograms. The gradient of left ventricular outflow tract was 43 mmHg. All aortic valve were competents. Results:  The time of cardiopulmonary bypass, aortic clamping were: 76 mins; 56 mins. There was no mortality.  The gradient postopration of LVOT and RVOT were 14 mmHg and 8 mmHg. During follow-up time 61 months after surgery, there was no late death. The gradient  of LVOT and RVOT were 10 mmHg and 6 mmHg Conclusion: The modified Konno procedure had  good results with low gradient of  LVOT and RVOT

Successful management of ischaemic symptoms in a patient with asymmetric septal hypertrophy: a grand round case report

Abstract Background Hypertrophic cardiomyopathy (HCM) is a genetic heart disease that can lead to heart failure, atrial fibrillation, and ischaemic symptoms. Managing patients with HCM and ischaemic symptoms is challenging, and several treatment options have been proposed. Case summary A 30-year-old male patient presented with severe chest pain that had been ongoing for more than 30 min at rest. He was diagnosed with HCM and had periodic chest pain since the age of 14. He underwent two separate ethyl alcohol ablations of the first septal branches of the left anterior descending and posterior descending arteries, which relieved his symptoms. Discussion This case report highlights the challenges in managing patients with HCM and ischaemic symptoms. In this patient, the use of ethyl alcohol ablation was effective in reducing left ventricular outflow tract obstruction and improving symptoms. Ethyl alcohol ablation is a minimally invasive procedure that has been shown to be effective in symptomatic patients with HCM. Overall, this case report emphasizes the importance of individualized treatment for patients with HCM and the potential benefits of alcohol ablation in this population.

Perioperative management and outcomes for posterior spinal fusion in patients with Friedreich ataxia: A single-center, retrospective study.

Friedreich ataxia is a rare genetic disorder associated with progressive mitochondrial dysfunction leading to widespread sequelae including ataxia, muscle weakness, hypertrophic cardiomyopathy, diabetes mellitus, and neuromuscular scoliosis. Children with Friedreich ataxia are at high risk for periprocedural complications during posterior spinal fusion due to their comorbidities. To describe our single-center perioperative management of patients with Friedreich ataxia undergoing posterior spinal fusion. Adolescent patients with Friedreich ataxia presenting for spinal deformity surgery between 2007 and 2023 were included in this retrospective case series performed at the Children's Hospital of Philadelphia. Perioperative outcomes were reviewed along with preoperative characteristics, intraoperative anesthetic management, and postoperative medical management. Seventeen patients were included in the final analysis. The mean age was 15 ± 2 years old and 47% were female. Preoperatively, 35% were wheelchair dependent, 100% had mild-to-moderate hypertrophic cardiomyopathy with preserved systolic function and no left ventricular outflow tract obstruction, 29% were on cardiac medications, and 29% were on pain medications. Intraoperatively, 53% had transesophageal echocardiography monitoring; 12% had changes in volume status on echo but no changes in function. Numerous combinations of total intravenous anesthetic agents were used, most commonly propofol, remifentanil, and ketamine. Baseline neuromonitoring signals were poor in four patients and one patient lost signals, resulting in 4 (24%) wake-up tests. The majority (75%) were extubated in the operating room. Postoperative complications were high (88%) and ranged from minor complications like nausea/vomiting (18%) to major complications like hypotension/tachycardia (29%) and need for extracorporeal membrane oxygenation support in one patient (6%). Patients with Friedreich ataxia are at high risk for perioperative complications when undergoing posterior spinal fusion and coordinated multidisciplinary care is required at each stage. Future research should focus on the utility of intraoperative echocardiography, optimal anesthetic agent selection, and targeted fluid management to reduce postoperative cardiac complications.

Simultaneous transapical transcatheter aortic and mitral valve replacement in patients with severe valve dysfunction: initial experience.

Simultaneous transcatheter mitral valve in valve (VIV) replacement and aortic valve replacement experience is limited. We report our initial experience with simultaneous transapical transcatheter aortic and mitral valve replacement in patients with severe valve dysfunction. A total of 8 patients had simultaneous transcatheter heart valve implants for severe mitral bioprosthesis failure (VIV), with a second valve procedure that included native aortic regurgitation (n = 3) or degenerated bioprostheses in the aortic position (n = 5). All patients were treated with a self-expandable J-valve transcatheter valve, using the transapical approach. The mean age of the patients was 73.1 ± 6.2years. The mean Society of Thoracic Surgeons score was 13.8 ± 6.3%. Device success was 100% according to Valve Academic Research Consortium-2 criteria. No other procedure-associated complications occurred, including left ventricular outflow tract obstruction and valve migration. The mean hospital lengths of stay after the procedure were 11.5 ± 8.0days. No deaths occurred at 30days. At a median follow-up period of 28.7 ± 22.3months, no patients died. All patients were in New York Heart Association functional classes I-II. Echocardiographic parameters at follow-up showed a normofunctioning J valve in the mitral position and a mean max mitral flow velocity of 2.0 ± 0.5m/s; the J valve in the aortic position was also normofunctioning, and the mean max aortic flow velocity was 2.3 ± 0.5m/s. Simultaneous transapical transcatheter aortic and mitral valve replacement using the self-expandable J valve appears to be a feasible and effective alternative to redo surgery.

Management of Left Ventricular Intramural Hematoma- an Unusual Complication of Complete Atrioventricular Septal Defect Repair in a Child.

Postoperative survival for complete atrioventricular septal defect (cAVSD) is excellent. Common complications of surgery include valvular stenosis/regurgitation, left ventricular outflow tract obstruction, arrythmias, and residual defects. We report a challenging case of a 7-month old girl with Trisomy 21, preoperative obstructive sleep apnea with severe pulmonary hypertension, who underwent AVSD repair and required veno-arterial extracorporeal membrane oxygenation (V-A ECMO) while unable to come off cardiopulmonary bypass and developed left ventricular intramural hematoma during the course. This case highlights the challenges in management of an unusual complication.

Effectiveness of pulmonary valve-sparing strategy for transatrial-transpulmonary repair of tetralogy of Fallot: a single institution experience.

We report the midterm results of our strategy utilizing transatrial-transpulmonary repair for tetralogy of Fallot at a single institution in a low-middle income country. Medical records were retrospectively reviewed for 532 consecutive patients who underwent definitive repair of tetralogy of Fallot at our institution from 2010 to 2020. The median age and weight of patients in the study patients were 11.6 months (interquartile range, 8.6-17.2 months) and 7.5 kg (interquartile range, 6.8-8.8 kg). The pulmonary valve annulus was preserved (no transannular patch) in 398 patients (75%) and a mini-transannular patch was utilized for 134 patients (25%). The overall survival was 98% at 1 year, and 97% at 10-years follow-up, respectively. Longer postoperative ventilation time was the only risk factor correlated to early death (p = 0.004; Odds Risk, 1.04; 95% confidence intervals, 1.01-1.07). Fourteen patients required pulmonary valve replacement (2.6%, 14/532), four required surgical resection to relieve right ventricular outflow tract obstruction (0.8%, 4/532), and freedom from reoperation of the right ventricular outflow tract was 87% at 10 years. The only risk factor for right ventricular outflow tract reoperation was a postoperative systolic pressure gradient through the right ventricular outflow tract of greater than 50 mmHg (p < 0.001; HR, 47; 95% confidence intervals, 9.1-244). In total, 94.6% (471/489) of the patients were asymptomatic at the latest follow-up without significant arrhythmia. At our institution in an low-middle income country, the transatrial-transpulmonary repair for tetralogy of Fallot has excellent midterm results with few reoperations required. Close long-term follow-up is essential for patients who undergo repair with a mini-transannular patch and may eventually require pulmonary valve replacement.

Exposure to Endocrine-Disrupting Chemicals and Congenital Heart Diseases: The Pooled Results Based on the Current Evidence.

The relationships between maternal exposure to endocrine-disrupting chemicals (EDCs) and congenital heart diseases (CHD) are not elucidated yet. The exposure levels of EDCs are generally estimated based on self-reported questionnaires or occupational exposure evaluations in the literature. Therefore, a study based on epidemiological data from human biospecimens is required to provide stronger evidence between maternal exposure to EDC and CHD. Embase, Pubmed, Scopus, and the Cochrane Library databases were searched for related research which provided risk estimates regarding the relationships between maternal EDC exposure and CHD in human offspring. Baseline characteristics and outcomes of CHD were extracted from each included study. Odds ratios (ORs) with 95% confidence intervals (CIs) were pooled to calculate the overall estimates of CHD. Subgroup and meta-regression analyses were performed to identify the sources of heterogeneity. Bootstrapping techniques were used in analyses where several studies originated from a similar population. A total of seventeen studies were involved in the meta-analyses. Maternal EDC exposure was significantly related to CHD in offspring (OR 2.15; 95%CI 1.64 to 2.83). EDC exposure was significantly associated with septal defects (OR 2.34; 95%CI 1.77 to 3.10), conotruncal defects (OR 2.54; 95%CI 1.89 to 3.43), right ventricular outflow tract obstruction (OR 2.65; 95%CI 1.73 to 4.07), left ventricular outflow tract obstruction (OR 3.58; 95%CI 2.67 to 4.79), anomalous pulmonary venous return (OR 2.31; 95%CI 1.34 to 4.00), and other heart defects (OR 2.49; 95%CI 1.75 to 3.54). In addition, maternal exposure to heavy metals, which included lead (OR 2.19; 95%CI 1.29 to 3.71), cadmium (OR 1.81; 95%CI 1.28 to 2.56), mercury (OR 2.23; 95%CI 1.13 to 4.44), and manganese (OR 2.65; 95%CI 1.48 to 4.74), increased risks for CHD significantly. In conclusion, based on the latest evidence, maternal EDC exposure may increase CHD risks in human offspring, especially in heavy metal exposure conditions.

Anesthesia management of laparoscopic right colectomy in an older patient with postoperative tetralogy of Fallot with residual anomaly

BackgroundDiversity in hemodynamics of adult congenital heart disease necessitates a case-by-case selection of appropriate surgical and anesthetic options. However, previous case reports regarding the management of laparoscopic surgery in adult patients with congenital heart disease are limited.Case presentationA 72-year-old man who underwent a laparoscopic right colectomy for colon cancer had a residual ventricular septal defect and right ventricular outflow tract obstruction despite post-repair of tetralogy of Fallot. Pulmonary hypertension or right ventricular dysfunction was not observed. The preoperative pulmonary to systemic blood flow ratio (Qp/Qs) was 2.3. After positive pressure ventilation and insufflation, the amount of left-to-right ventricular shunting decreased, and the Qp/Qs approached 1.0, as calculated from pulmonary arterial and systemic arterial blood gas analysis.ConclusionsLaparoscopic surgery might be tolerable in patients with tetralogy of Fallot who have preserved the right ventricular function, left-to-right ventricular shunting, and no high pulmonary vascular resistance.

Repair of complex transposition of great arteries: What is the best technique to avoid outflow tract obstructions?

This study aimed to evaluate the short-/mid-term outcome of patients with complex dextro-/levo-transposition of the great arteries (d-/l-TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstructions (LVOTO). A single-center, retrospective review of all complex d-TGA (n = 85) and I-TGA (n = 22) patients undergoing different surgeries (Arterial switch operation (ASO)+LVOTO-resection (ASO-R), half-turned truncal switch/Mair (HTTS), Nikaidoh and Rastelli) between 05/1990-09/2022 was performed. Groups were analyzed using Kruskal-Wallis test with post-hoc pairwise comparison and Kaplan-Meier time-to-event models. A total of 107 patients (ASO-R (n = 20), HTTS (n = 23), Nikaidoh (n = 21), Rastelli (n = 43)) were included, with a median age of 1.0 year [0.5-2.5] at surgical repair median follow-up was 3.8 years [0.3-10.5]. Groups did not differ in respect to early postoperative complications/early mortality. 5-year overall survival curves were comparable: ASO-R 78.9% [53.2-91.5], HTTS 75.3% [46.8-89.9], Nikaidoh 85% [60.4-94.9], and Rastelli 83.9% [67.5-92.5], p = 0.9. Highest rates of right ventricular outflow tract (RVOT) reinterventions (33.3%, and 32.6% (p = 0.04)) and reoperations (28.6%, and 32.6% (p = 0.02)) occurred after Nikaidoh and Rastelli procedures. However, overall freedom from RVOT reinterventions and RVOT reoperations at 5 years did not differ statistically significantly between the groups (ASO-R, HTTS, Nikaidoh and Rastelli): 94.4% [66.6-99.2], 69.1% [25.4-90.5], 67.8% [34-86.9], 64.4% [44.6-78.7], p = 0.2, and 90.0% [65.6-97.4], 91% [50.8-98.7], 65.3% [32.0-85.3] and 67.0% [47.4-80.6], p = 0.3. Surgical repair of complex d-TGA/l-TGA can be performed with satisfying early/mid-term survival. RVOT reinterventions/reoperations were frequent, with highest rates after Nikaidoh and Rastelli procedures. LVOTO reoperations were rare with zero events after Nikaidoh and HTTS procedures.

Long-Term Safety and Efficacy of Mavacamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Interim Results of the PIONEER-OLE Study.

The phase 2 PIONEER-HCM (Phase 2 Open-label Pilot Study Evaluating Mavacamten in Subjects With Symptomatic Hypertrophic Cardiomyopathy and Left Ventricular Outflow Tract Obstruction) study showed that mavacamten improved left ventricular outflow tractgradients, exercise capacity, and symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM), but the results of longer-term treatment are less well described. We report interim results from the PIONEER-OLE (PIONEER Open-Label Extension) study, the longest-term study of mavacamten in patients with symptomatic obstructive HCM. Patients who previously completed PIONEER-HCM (n=20) were eligible to enroll in PIONEER-OLE. Patients received oral mavacamten, 5 mg once daily (starting dose), with individualized dose titration at week 6. Evaluations included serial monitoring of safety, echocardiography, Kansas City Cardiomyopathy Questionnaire-Overall Summary Score, and serum NT-proBNP (N-terminal pro-B-type natriuretic peptide) levels. Thirteen patients enrolled and received mavacamten (median study duration at data cutoff, 201 weeks). Most patients (92.3%) received β-blockers concomitantly. Treatment-emergent adverse events were predominantly mild/moderate. One patient had an isolated reduction in left ventricular ejection fraction to 47%, which recovered and remained normal with continued treatment at a reduced dose. At week 180, mavacamten was associated with New York Heart Association class improvements from baseline (class II to I, n=9; class III to II, n=1; and unchanged, n=2), sustained reductions in left ventricular outflow tract gradients (mean [SD] change from baseline: resting, -50 [55] mm Hg; Valsalva, -70 [41] mm Hg), and serum NT-proBNP levels (median [interquartile range] change from baseline: -498 [-2184 to -76] ng/L), and improved Kansas City Cardiomyopathy Questionnaire-Overall Summary Score (mean [SD] change from baseline: +17 [16]). This long-term analysis supports the continued safety and effectiveness of mavacamten for >3 years in obstructive HCM. URL: https://www.clinicaltrials.gov; Unique identifier: NCT03496168.

Individualised surgical treatment strategy for subaortic stenosis.

The clinical data of patients with subaortic stenosis who underwent surgical treatment in our centre in the past 12 years were reviewed. The short-term and long-term clinical outcomes were analyzed, and the long-term outcomes of different surgical methods for subaortic stenosis were compared to determine the optimal surgical treatment strategy for subaortic stenosis. From December 2010 to December 2022, 90 patients undergoing surgical treatment for subaortic stenosis in our hospital were enrolled. There were 55 males and 35 females with a median age of 72 (46,132) months and an average surgical weight of (21.35 ± 15.84) kg. According to the operation method, 90 patients were divided into group A (50 patients with simple subaortic membrane resection) and group B (40 patients with subaortic membrane and muscle resection or modified Konno procedure). There were three early deaths (3.33%). One late death occurred in group B. There was no significant difference in long-term survival rate between the two groups (p = 0.904). The preoperative left ventricular outflow tract pressure gradient in group B was (91.56 ± 36.98) mm Hg, which was higher than that in group A(51.13 ± 36.04)mm Hg(p < 0.001). There was no significant difference in immediate postoperative left ventricular outflow tract pressure gradient between group B [(5.44 ± 8.43) mm Hg] and group A [(7.82 ± 13.44) mm Hg] (p = 0.343). In the long-term follow-up, left ventricular outflow tract pressure gradient in group B was (5.86 ± 9.53) mm Hg, which was not statistically significant compared with group A (8.83 ± 14.52) mm Hg (p = 0.294). Eleven patients with moderate or greater aortic regurgitation (group A/group B: 3/8) underwent simultaneous aortic valvuloplasty. In group B, moderate or greater aortic regurgitation was significantly improved immediately after operation (p = 0.013) and was not significantly aggravated in long-term follow-up (p = 0.083), and there was no significant improvement in group A after operation and long-term follow-up. According to the different anatomical lesions of left ventricular outflow tract, the individualised surgical treatment strategy for patients with subaortic stenosis can achieve good long-term outcomes. The long-term survival rate and freedom from reoperation due to late left ventricular outflow tract obstruction after simple subaortic membrane resection and extended left ventricular outflow tract resection are comparable. For patients with moderate or greater aortic regurgitation before extended left ventricular outflow tract resection, simultaneous aortic valvuloplasty is beneficial to improve postoperative aortic valve function.

Postnatal Cerebral Hemodynamics and Placental Vascular Malperfusion Lesions in Neonates With Congenital Heart Disease

BackgroundNeonates with congenital heart disease (CHD) have smaller brain volume at birth. High rates of placental vascular malperfusion lesions may play a role in disrupted brain development. MethodsThis is a single-center retrospective cohort study of infants born between 2010 and 2019 who were diagnosed with a major cardiac defect requiring surgery in the first year of life. Doppler ultrasound RI of the middle cerebral artery (MCA) and anterior cerebral artery were calculated within the first 72 hours of life. Placentas were evaluated using a standardized approach. ResultsOver the study period, there were 52 patients with hypoplastic left heart syndrome (HLHS), 22 with single-ventricle right ventricular outflow tract obstruction (SV-RVOTO), 75 with a two-ventricle cardiac defect (2V), and 25 with transposition of the great arteries (TGA). MCA Doppler RI were significantly higher for all subgroups of CHD compared with control subjects (0.68 ± 0.11 in control subjects compared with 0.78 ± 0.13 in HLHS, P = 0.03; 0.77 ± 0.10 in SV-RVOTO, P = 0.002; 0.78 ± 0.13 in 2V, P = 0.03; and 0.80 ± 0.14 in TGA; P = 0.001) with the highest average MCA RI in the TGA group. In subgroup analyses, placental fetal vascular malperfusion in the 2V group was associated with higher MCA RI, but this relationship was not present in other subgroups, nor in regards to maternal vascular malperfusion. ConclusionsMajor forms of CHD are associated with significantly higher cerebral artery RI postnatally, but placental vascular malperfusion lesions may not contribute to this hemodynamic adaptation.

Mitral Regurgitation and Left Ventricular Outflow Tract Obstruction: Confluence of Challenges for Transcatheter Treatment

Mitral Regurgitation and Left Ventricular Outflow Tract Obstruction: Confluence of Challenges for Transcatheter Treatment

Transcatheter Aortic Valve Embolization Complicated by Inversion and Left Ventricular Outflow Tract Obstruction

Transcatheter Aortic Valve Embolization Complicated by Inversion and Left Ventricular Outflow Tract Obstruction