Dose-Blinded Myosin Inhibition in Patients With Obstructive Hypertrophic Cardiomyopathy Referred for Septal Reduction Therapy: Outcomes Through 32 Weeks.

BACKGROUND: Septal reduction therapy (SRT) in patients with intractable symptoms from obstructive hypertrophic cardiomyopathy (oHCM) is associated with variable morbidity and mortality. The VALOR-HCM trial (A Study to Evaluate Mavacamten in Adults with Symptomatic Obstructive Hypertrophic Cardiomyopathy Who Are Eligible for Septal Reduction Therapy) examined the effect of mavacamten on the need for SRT through week 32 in oHCM. METHODS: A double-blind randomized placebo-controlled multicenter trial at 19 US sites included patients with oHCM on maximal tolerated medical therapy referred for SRT with left ventricular outflow tract gradient ≥50 mm Hg at rest or provocation (enrollment, July 2020-October 2021). The group initially randomized to mavacamten continued the drug for 32 weeks, and the placebo group crossed over to dose-blinded mavacamten from week 16 to week 32. Dose titrations were based on investigator-blinded echocardiographic assessment of left ventricular outflow tract gradient and left ventricular ejection fraction. The principal end point was the proportion of patients proceeding with SRT or remaining guideline eligible at 32 weeks in both treatment groups. RESULTS: From the 112 randomized patients with oHCM, 108 (mean age, 60.3 years; 50% men; 94% in New York Heart Association class III/IV) qualified for week 32 evaluation (56 in the original mavacamten group and 52 in the placebo cross-over group). After 32 weeks, 6 of 56 patients (10.7%) in the original mavacamten group and 7 of 52 patients (13.5%) in the placebo cross-over group met SRT guideline criteria or elected to undergo SRT. After 32 weeks, a sustained reduction in resting left ventricular outflow tract gradient (-33.0 mm Hg [95% CI, -41.1 to -24.9]) and Valsalva left ventricular outflow tract gradient (-43.0 mm Hg [95% CI, -52.1 to -33.9]) was observed in the original mavacamten group. A similar reduction in resting (-33.7 mm Hg [95% CI, -42.2 to -25.2]) and Valsalva (-52.9 mm Hg [95% CI, -63.2 to -42.6]) gradients was quantified in the cross-over group after 16 weeks of mavacamten. After 32 weeks, improvement by ≥1 New York Heart Association class was observed in 48 of 53 patients (90.6%) in the original mavacamten group and 35 of 50 patients (70%) after 16 weeks in the cross-over group. CONCLUSIONS: In severely symptomatic patients with oHCM, 32 weeks of mavacamten treatment showed sustained reduction in the proportion proceeding to SRT or remaining guideline eligible, with similar effects observed in patients who crossed over from placebo after 16 weeks. REGISTRATION: URL: https://www. CLINICALTRIALS: gov; Unique identifier: NCT04349072.

Comparison of Early and Mid-Term Outcomes After Classic and Modified Morrow Septal Myectomy in Patients with Hypertrophic Obstructive Cardiomyopathy.

INTRODUCTION: The aim of our study is to compare the early and mid-term outcomes of patients with hypertrophic obstructive cardiomyopathy who underwent classic and modified Morrow septal myectomy. METHODS: Between 2014 and 2019, 48 patients (24 males; mean age 49.27±16.41 years) who underwent septal myectomy were evaluated. The patients were divided into two groups - those who underwent classic septal myectomy (n=28) and those who underwent modified septal myectomy (n=20). RESULTS: Mitral valve intervention was higher in the classic Morrow group than in the modified Morrow group, but there was no significant difference (P=0.42). Mortality was found to be lower in the modified Morrow group than in the classic Morrow group (P=0.01). In both groups, the mean immediate postoperative gradient was significantly higher than the mean of the 3rd and 12th postoperative months. The preoperative and postoperative gradient difference of the modified Morrow group was significantly higher than of the classic Morrow group (P<0.001). CONCLUSION: Classic Morrow and modified Morrow procedures are effective methods for reducing left ventricular outflow tract obstruction. The modified Morrow procedure was found to be superior to the classic Morrow procedure in terms of reducing the incidence of mitral valve intervention with the reduction of the left ventricular outflow tract gradient.

Comparison of Early and Mid-Term Outcomes After Classic and Modified Morrow Septal Myectomy in Patients with Hypertrophic Obstructive Cardiomyopathy

ABSTRACT Introduction: The aim of our study is to compare the early and mid-term outcomes of patients with hypertrophic obstructive cardiomyopathy who underwent classic and modified Morrow septal myectomy. Methods: Between 2014 and 2019, 48 patients (24 males; mean age 49.27±16.41 years) who underwent septal myectomy were evaluated. The patients were divided into two groups - those who underwent classic septal myectomy (n=28) and those who underwent modified septal myectomy (n=20). Results: Mitral valve intervention was higher in the classic Morrow group than in the modified Morrow group, but there was no significant difference (P=0.42). Mortality was found to be lower in the modified Morrow group than in the classic Morrow group (P=0.01). In both groups, the mean immediate postoperative gradient was significantly higher than the mean of the 3rd and 12th postoperative months. The preoperative and postoperative gradient difference of the modified Morrow group was significantly higher than of the classic Morrow group (P<0.001). Conclusion: Classic Morrow and modified Morrow procedures are effective methods for reducing left ventricular outflow tract obstruction. The modified Morrow procedure was found to be superior to the classic Morrow procedure in terms of reducing the incidence of mitral valve intervention with the reduction of the left ventricular outflow tract gradient.

The use of two Amplatzer "Cribriform" Septal Occluders to close multiple postinfarction ventricular septal defects.

Rupture of the interventricular septum is an uncommon and often fatal complication of myocardial infarction. Herein, we report the successful deployment of the Amplatzer Multi-Fenestrated Septal Occluder-"Cribriform" (AGA Medical Corporation; Plymouth, Minn) during 2 procedures to close multiple postinfarction ventricular septal defects in a severely ill patient. We show that, in selected patients who have multi-fenestrated multiple postinfarction ventricular septal defects, transcatheter implantation of the Amplatzer Cribriform Occluder can be a good therapeutic option.

Morfología de las paredes ventriculares en fetos humanos con defectos septales

Introducción: las cardiopatías congénitas constituyen defectos estructurales y/o funcionales del corazón y los grandes vasos, como consecuencia de un error en la embriogénesis de estas estructuras. Los defectos septales suelen ser las cardiopatías congénitas más frecuentes. Objetivo: identificar la asociación entre la morfología de las paredes ventriculares y los defectos septales en fetos humanos. Materiales y métodos: se realizó un estudio observacional, descriptivo y transversal en la Facultad de Ciencias Médicas de Pinar del Río Dr. Ernesto Guevara de la Serna y el Departamento de Anatomía Patológica del Hospital General Docente Abel Santamaría Cuadrado, en el período de enero de 2019 a diciembre de 2020, con 54 fetos producto de interrupciones de embarazo indicadas por genética. Resultados: predominaron los defectos de septación en el sexo masculino. La comunicación interventricular fue el defecto de septación más frecuente. El grosor del ventrículo derecho mostró diferencias lineales y estadísticas significativas en relación con la edad gestacional en los tres defectos estudiados. Similares características presentó el grosor del tabique interventricular en los defectos troncoconales. Conclusiones: ante la presencia de defectos septales se observan modificaciones del grosor de los tabiques cardiacos.

Introduction: congenital heart diseases are structural and/or functional defects of the heart and large vessels, as a consequence of an error in the embryogenesis of these structures. Septal defects are usually the most common congenital heart diseases. Objective: to identify the association between ventricular walls morphology and septal defects in human fetuses. Materials and methods: an observational, descriptive and cross-sectional study was carried out in the Medical Sciences Faculty of Pinar del Rio Dr. Ernesto Guevara de la Serna and the Department of Pathological Anatomy of the General Teaching Hospital Abel Santamaría Cuadrado, in the period from January 2019 to December 2020, with 54 fetuses resulting from pregnancy interruptions indicated by genetics Results: septation defects predominated in males. Interventricular communication was the most frequent septation defect. The thickness of the right ventricle showed significant linear and statistical differences in relation to gestational age in the three studied defects. Similar characteristics showed the thickness of the inter-ventricular septum in truncoconal defects. Conclusions: in the presence of septal defects, modifications of the heart septum thickness are observed.

The "1st septal unit" in hypertrophic obstructive cardiomyopathy: a newly recognized anatomo-functional entity, identified during recent alcohol septal ablation experience.

In hypertrophic obstructive cardiomyopathy, selective and asymmetric hypertrophy results in a stenotic subaortic channel, which is further narrowed by a Venturi effect (suctioning of the anterior leaflet, manifested by systolic anterior motion of the mitral valve). Better understanding of these essential pathophysiologic mechanisms has led to the definition of a new anatomo-functional entity, the 1st septal unit, which consists of the basal interventricular septal hypertrophy and its related septal arterial branches. As an alternative to surgical myomectomy, alcohol septal ablation is an effective method of reducing subaortic stenosis and improving mitral valve function. After alcohol ablation, global negative remodeling of the hypertrophied left ventricle eventually ensues. This review presents specific anatomic and functional features of a newly identified pathophysiologic entity (the 1st septal unit) in relation to the clinical manifestations and natural history of hypertrophic obstructive cardiomyopathy. This relationship is also relevant during the performance of alcohol septal ablation interventions: related operative suggestions are provided for optimizing subaortic stenosis relief during septal ablation and for preventing complications.

Clinical variables determining the success of adenotonsillectomy in children with Down syndrome.

OBJECTIVES: To evaluate the evolution of polysomnographic parameters of children with Down syndrome and obstructive sleep apnea syndrome submitted to adenotonsillectomy and the interaction of comorbidities on therapeutic outcome. METHODS: Ninety patients with Down syndrome and habitual snoring were identified between 2005 and 2015 in a Pediatric Otorhinolaryngology Clinic. Parent's complaints were evaluated by the test of equality of two proportions. Wilcoxon test was used to examine pre- and post-operative polysomnographic differences. Mann-Whitney test evaluated the influence of comorbidities. A p < 0.05 was considered significant. RESULTS: A total of 27 patients met the inclusion criteria (55.6% patients were males; mean (SD) age were 6.7 (3.6) years (range, 1.5-16 years). Significant improvement of parent's complaints (p < 0.001), arousal index (p = 0.045), and minimum oxygen saturation were observed post-adenotonsillectomy (p = 0.034). Adenotonsillectomy was able to resolve obstructive sleep apnea syndrome in 29.6% of children with Down syndrome. Nineteen patients (70.4%) remained with obstructive sleep apnea syndrome and 44.4% showed a reduction of at least 50% of obstructive apnea-hypopnea index. Central apnea index post-adenotonsillectomy was worse in patients with heart disease (p = 0.022). Sleep efficiency (p = 0.031), N1 sleep stage (p = 0.001), apnea-hypopnea index (p = 0.023), and central apnea index (p = 0.008) were worse after surgery in patients with hypothyroidism. Patients with severe OSAS showed significant improvement in polysomnographic parameters after surgery. CONCLUSION: Although adenotonsillectomy improved symptoms and objective sleep data in children with Down syndrome, it was not able to resolve obstructive sleep apnea syndrome in most patients. Congenital heart diseases and hypothyroidism may affect the outcome.

Hypertrophic obstructive cardiomyopathy: review of surgical treatment.

Hypertrophic cardiomyopathy ranks among the most common congenital cardiac diseases, affecting up to 1 in 200 of the general population. When it causes left ventricular outflow tract obstruction, treatment is guided to reduce symptoms and the risk of sudden cardiac death. Pharmacologic therapy is the first-line treatment, but when it fails, surgical myectomy or percutaneous ablation of the hypertrophic myocardium are the standard therapies to eliminate subaortic obstruction. Both surgical myectomy and percutaneous ablation are proven safe and effective treatments; however, myectomy is the gold standard with a significantly lower complication rate and more complete and lasting reduction of left ventricular outflow tract obstruction.

Aneurysm of the sinus of Valsalva dissecting into the intraventricular septum.

Aneurysm of the sinus of Valsalva is an uncommon entity and especially rare when it dissects into the intraventricular septum. This uncommon clinical manifestation often takes a drastic clinical course with life-threatening arrhythmia, coronary ischemia, rupture, and heart failure. We present the case of a 36-year-old man with an aneurysm of the sinus of Valsalva dissecting into the intraventricular septum, which induced severe aortic insufficiency and heart block.

Transseptal Approach versus Left Atrial Approach to Mitral Valve: A Propensity Score Matching Study.

BACKGROUND: Many patients with mitral valve diseases need surgical procedures for the repair or replacement of their mitral valve. There is a great deal of controversy over the outcomes of the transseptal (TS) and left atrial (LA) approaches to the mitral valve. We sought to evaluate the outcomes of each approach more accurately by eliminating the possible biases in case selection and matching. METHODS: This retrospective study included patients who had surgery for mitral valve diseases via either the TS approach or the LA approach between 2004 and 2011 in Tehran Heart Center. Patients with surgical approaches other than the TS and LA were excluded. To control for the confounding effects, a propensity score matching technique was applied and the patients were matched for 14 demographic and preoperative variables. After the selection of controls, the effect of the TS approach (163 patients) versus the LA approach (652 patients) on the outcomes was presented through odds ratio (OR) with 95% confidence intervals (CI). RESULTS: The mean age of the patients was 53.15 ± 12.02 years in the TS group and 52.93 ± 13.56 years in the LA group. Females comprised 119 (73.0%) patients in the TS group and 462 (70.9%) in the LA group. There was a significant association in the prevalence of new postoperative atrial fibrillation in the two groups (OR = 1.539, 95%CI: 1.072-2.210; p value = 0.019). Temporary pacemaker placement had no statistically significant difference between the two groups (p value = 0.418). The TS patients had significantly longer pump (p value < 0.001) and cross-clamp (p value < 0.001) times. The mortality rate was 4.1% (27 patients) in the LA group and 6.1% (10 patients) in the TS group (p value = 0.274). CONCLUSION: In our study population, the TS approach was associated with higher pump and cross-clamp times as well as risk of postoperative atrial fibrillation, but it did not increase the rates of permanent pacemaker placement, re-operations, and mortality.

Giant right atrial lipoma mimicking tamponade.

Cardiac lipomas are rarely encountered. They are mostly asymptomatic and may be discovered incidentally. We describe the case of a 56 year-old man with a presentation similar to tamponade. He had decreased heart sounds, global cardiomegaly, and oligemic lung fields. Echocardiography showed a 110 × 75-mm mass attached to the interatrial septum, almost completely occupying the right atrium. Chest computed tomography showed a large homogeneous low-attenuation mass with thin septa, originating from interatrial septum and filling the right atrium, consistent with lipoma. The patient underwent surgery for resection of the tumor. Pathologic examination was consistent with cardiac lipoma.

Acute Left Atrial Thrombus Formation on Resected Residual Cribriform Septum after Atrial Septal Defect Surgery.

Acute left atrial thrombosis at the site of the resection of the primary cribriform septum is an exceedingly rare and important complication after atrial septal defect (ASD) closure with a pericardial or synthetic patch. This case report presents a mobile thrombus noted on the left atrium at the raw surface site of a resected cribriform primary septum that was not caught in the suture line with the pericardial patch for the closure of the ASD in a 30-year-old woman with an uncomplicated ASD surgery. The patient had no symptoms in the postoperative period, and routine postoperative transesophageal echocardiography revealed a large pedunculated and mobile mass (thrombosis) at the left atrial side of the interatrial septum at the level of the implanted pericardial patch. The thrombus was successfully treated with surgery. The patient had an uneventful recovery in the postoperative period and was discharged from the hospital 15 days after admission. One-year follow-up showed no evidence of clot recurrence in the left or right atrium.

Defecto ventricular septal aislado. Reporte de un caso / Isolated ventricular septal defect. A case report / Isolated ventricular septal defect. A case report

RESUMEN Las cardiopatías congénitas son las anomalías congénitas más frecuentes. El defecto ventricular septal (DVS) es la cardiopatía congénita más común en los recién nacidos que afecta entre 25 y 30% de los neonatos con defecto cardiaco; los de tipo medio muscular son más comunes que los perimembranosos. La incidencia de anomalías cromosómicas con DVS aislado es relativamente baja. El cierre espontáneo del DVS aislado ocurre en aquellos que son pequeños, y el DVS muscular medio es el que más comúnmente se cierra comparado con el membranoso o perimembranoso. El diagnóstico de DVS muscular aislado sin otra anomalía puede ser considerado un hallazgo benigno.

ABSTRACT Congenital heart disease is the most common congenital anomaly. Ventricular septal defect (VSD) is a frequent congenital heart disease in newborns, affecting 25 to 30% neonates with cardiac defects. Muscular VSDs are more frequent than perimembranous VSDs. The association of cases with chromosomal anomalies and isolated VSD is relatively low. Spontaneous closure of isolated VSD is higher with small VSD cases, and the muscular VSD is more likely to close spontaneously than the membranous or perimembranous types. Therefore, diagnosis of isolated muscular VSD with no other anomalies can be considered a benign finding.

Surgical outcome of hypoplastic left heart syndrome with intact atrial septum.

BACKGROUND: The surgical outcomes of hypoplastic left heart syndrome with intact atrial septum remains very poor in spite of the introduction of prenatal diagnosis before the Norwood operation. The hybrid operation consisting of bilateral pulmonary artery banding and balloon atrioseptectomy is one of the treatment strategies to potentially improve this patient population, however, the long-term outcomes are unknown. METHODS: Six consecutive patients with hypoplastic left heart syndrome with intact atrial septum, who underwent the hybrid operation between October 2006 and July 2014, were retrospectively reviewed. Hypoplastic left heart syndrome with highly restrictive atrial communication was excluded. RESULTS: Three patients died after the hybrid operation, due to sepsis, cerebral bleeding, and heart failure. Three patients underwent the modified Norwood operation. One of these died due to severe hypoxia while awaiting the bidirectional Glenn operation. The others underwent a bidirectional Glenn operation and subsequent Fontan completion but died due to lung dysfunction and sudden hemoptysis. CONCLUSIONS: The hybrid operation for hypoplastic left heart syndrome with intact atrial septum may have improved the neonatal outcome and Fontan completion rate in this extremely high-risk group of patients, but the long-term outcome remains dismal. Considering the late mortality related to pulmonary complications, aggressive fetal intervention to create a nonrestrictive atrial septal communication to promote normal development of the pulmonary vessels may be the last resort to improve the long-term outcome.

Factores asociados a hipertrofia sigmoidea en personas adultas que habitan en altura / Factors associated with sigmoidea hypertrophy in adults who live in altitude

RESUMEN Objetivo: Determinar los factores asociados con hipertrofia sigmoidea (HS) en adultos que habitan en la altura. Material y métodos: Estudio observacional, analítico, de casos y controles. Se realizó en el laboratorio de Ecocardiografía del Hospital Regional Docente Clínico Quirúrgico Daniel Alcides Carrión de Huancayo, entre enero del 2017 y julio del 2019. La muestra estuvo conformada por 74 personas que viven a más de 3000 m.s.n.m. Para el diagnóstico de HS se consideró el incremento del grosor septal basal (> 13 mm en varones y > 12 mm en mujeres), y tener el grosor > a 50% de la pared septal media. Los datos se recolectaron mediante un cuestionario. Se realizó el análisis estadístico con X2, t-student, y regresión logística considerándose significativo p<0,05. Resultados: Se incluyeron 74 pacientes. El promedio de edad fue 63 ± 16 años (rango: 23-94), 41 (55,4 %) fueron mujeres. La HS tipo 2 fue dos veces más frecuente que el tipo 1. Se conformaron dos grupos: n1=37 casos y n2=37 controles. En el análisis multivariado se encontró que la diabetes mellitus (OR=23,76 IC 95%: 1,61-350,7); la edad de 60 a más años (OR=9,97; IC 95%: 1,93-51,48) y la hipertensión arterial (OR=5,18 IC 95%: 1,19-22,59) estuvieron asociados con la mayor frecuencia de presentación de HS (p<0.05). Conclusiones: Se concluye que en el poblador que habita en la altura la diabetes mellitus, la edad avanzada y la hipertensión arterial son factores asociados a HS.

SUMMARY Objective: To determine factors associated to sigmoidal hypertrophy (SH) in adults living at high altitude. Methods: A case-control study was carried-out in the Ultrasound laboratory at Hospital Regional Docente Clínico Quirúrgico Daniel Alcides Carrión, Huancayo from January 2017 to July 2019. 74 persons were included who lived at altitudes above 3000 m.a.s.l. SH was defined considering the septal thickening (> 13 mm for males and > 12 mm for females) and having a septal median thickening > a 50%. Data were collected in a questionnaire, chi-square and student´s t-test were performed and a logistic regression analysis was carried-out including variables with statistical significance at <0.05. Results: Mean age was 63 ± 16 years (range: 23-94); 41 (55.4%) were females. SH type 2 was twice more common than SH type 1; 37 patients were cases and 37 were controls. The multivariate analysis found that diabetes mellitus (OR=23.76; 95% CI: 1.61-350.7); age above 60 years old (OR=9.97; 95% CI: 1.93-51.48) and blood hypertension (OR=5.18; 95% CI: 95%: 1.19-22.59) were associated with HS (p<0.05). Conclusions: In people living at high altitude, diabetes mellitus, advanced age and blood hypertension are associated to SH.

Surgical approach to huge cardiac hydatid cyst of the interventricular septum.

Cardiac echinococcosis is a rare but potentially fatal condition. The cysts are frequently located in the left or right ventricle; involvement of the interventricular septum is exceptional. We report the case of a 29-year-old woman who presented with palpitation and generalized T-wave inversion on her electrocardiogram. Transthoracic echocardiography, magnetic resonance imaging, and contrast-enhanced computed tomography showed a huge hydatid cyst originating from the interventricular septum. She was started on albendazole tablets, and surgical excision on cardiopulmonary bypass was carried out after 5 days of treatment. She had an uneventful postoperative recovery, and was continued on albendazole tablets for 4 months.

A case of ventricular septal defect and mitral insufficiency after blunt trauma.

Few reports have described traumatic heart injury in children. We describe a case of acute mitral regurgitation associated with papillary muscle rupture, traumatic ventricular septal defect, and impending left ventricular free wall rupture due to blunt trauma in a 2-year-old girl. The papillary muscle was sutured to the left ventricular free wall. The septal defect and surrounding ruptured muscle were covered with a pericardial patch, and a Hemashield patch was used to close the ventriculotomy. A residual defect caused by dehiscence of the pericardial patch necessitated reoperation 10 months later. The patient is currently being observed on an outpatient basis.