Long-Term Functional Results of Patients Operated for Isolated Discrete Subaortic Stenosis.

BACKGROUND: This study aimed to examine the long-term functional results of patients with isolated discrete subaortic stenosis who underwent subaortic membrane resection and myectomy, using transthoracic M-mode echocardiography and 2D speckle-tracking echocardiography. METHODS: Twenty patients operated for isolated discrete subaortic stenosis and 31 controls were included in the study. Patients underwent subaortic membrane resection and myectomy. During the long-term follow up, patients were evaluated with transthoracic M-mode echocardiography and 2D speckle-tracking echocardiography for functional assessment. RESULTS: The mean age at operation and mean duration of follow up was 8.1±5.6 years and 7.2±3.3 years, respectively. Interventricular septal thickness at diastole (0.9±0.1 vs. 0.8±0.1 cm, P = 0.001), ejection time (285.7±26.2 vs. 261.2±24.3 msec, P = 0.001), and aortic strain (15.6±3.7 vs. 10.5±4.0, P < 0.001) were significantly higher in patients. On the other hand, ejection fraction (64.9±6.1 vs. 75.1±5.4 %, P < 0.001), fractional shortening (35.0±5.1 vs. 43.7±5.1, P < 0.001), and corrected velocity circumferential fiber shortening (0.12±0.02 vs. 0.17±0.03, P < 0.001) were significantly lower, when compared with the controls. Longitudinal strain value significantly differed among the groups, with patients having significantly lower strain (18.8±1.8 vs. 20.1±2.1, P = 0.021). CONCLUSION: In patients operated for isolated discrete subaortic stenosis, aortic gradient seems to continue in the long-term, with the persistence of low longitudinal strain.

Subaortic Membranes in Patients With Hereditary Hemorrhagic Telangiectasia and Liver Vascular Malformations.

Background Patients with hereditary hemorrhagic telangiectasia have liver vascular malformations that can cause high-output cardiac failure (HOCF). Known sequelae include pulmonary hypertension, tricuspid regurgitation, and atrial fibrillation. Methods and Results The objectives of this study were to describe the clinical, echocardiographic, and hemodynamic characteristics and prognosis of hereditary hemorrhagic telangiectasia patients with HOCF who were found to have a subaortic membrane (SAoM). A retrospective observational analysis comparing patients with and without SAoM was performed. Among a cohort of patients with HOCF, 9 were found to have a SAoM in the left ventricular outflow tract by echocardiography (all female, mean age 64.8±4.0 years). The SAoM was discrete and located in the left ventricular outflow tract 1.1±0.1 cm below the aortic annular plane. It caused turbulent flow, mild obstruction (peak velocity 2.8±0.2 m/s, peak gradient 32±4 mm Hg), and no more than mild aortic insufficiency. Patients with SAoM (n=9) had higher cardiac output (12.1±1.3 versus 9.3±0.7 L/min, P=0.04) and mean pulmonary artery pressures (36±3 versus 28±2 mm Hg, P=0.03) compared with those without SAoM (n=19) during right heart catheterization. Genetic analysis revealed activin receptor-like kinase 1 mutations in each of the 8 patients with SAoM who had available test results. The presence of a SAoM was associated with a trend towards higher 5-year mortality during follow-up. Conclusions SAoM with mild obstruction occurs in patients with hereditary hemorrhagic telangiectasia and HOCF. SAoM was associated with features of more advanced HOCF and poor outcomes.

Left Ventricle to Right Atrial Shunt Associated With Ventricular-Atrial Malalignment, Double Outlet Right Atrium, and Subaortic Membrane.

We describe an asymptomatic 7-year-old boy who was taken to the operating room for repair of a subaortic membrane and possible Gerbode's defect. He was found to have a double outlet right atrium associated with an accessory atrioventricular valve in addition to a small atrial septal defect and subaortic membrane. Regurgitant flow through this accessory valve led to the left ventricle to right atrial shunt that was seen on preoperative ECHO. The atrial septal defect was repaired and a baffle was used to isolate blood flow across the accessory valve from the left atrium to the left ventricle. The patient was discharged on postoperative day 4 and has been doing well 2 years postoperatively.

56-year-old lady with transaortic gradient.

CLINICAL INTRODUCTION: A 56-year-old lady with a background of hypertension was admitted to our institution with acute pulmonary oedema. She reported gradual and increasingly severe dyspnoea on exertion over the preceding 12 months and, prior to presentation, her exercise tolerance was restricted to one flight of stairs. On transthoracic echocardiography during the index admission, left ventricular size and systolic function were normal, and peak and mean transaortic gradients were 67 mm Hg and 33 mm Hg, respectively, with a peak velocity of 3.9 m/s. No aortic incompetence or other significant valvular abnormality was noted. A transoesophageal echocardiogram was performed. Figure 1 depicts the mid-oesophageal parasternal long-axis view. What is the explanation behind the significant transaortic gradient?heartjnl;104/12/1036/F1F1F1Figure 1Transoesophageal echocardiogram, mid-oesophageal long-axis view at 135 degrees. QUESTION: What is the explanation behind the significant transaortic gradient?Ventricular septal defect Supravalvular aortic stenosisAortic valvular stenosisSubaortic membraneHypertrophic obstructive cardiomyopathy.

Outcomes of Subaortic Obstruction Resection in Children.

BACKGROUND: Studies of long-term outcomes of discrete subaortic stenosis are rare. Therefore, we reviewed the long-term outcomes of fibromuscular resection in children with subaortic stenosis over 26 years from a single institution. METHODS: We conducted a retrospective review of all children (n=72) who underwent resection of subaortic obstruction for discrete subaortic stenosis between 1989 and 2015. RESULTS: Median age at surgery was 5.0 years (2.7-7.6 years). There were no operative deaths but three late deaths (4.2%, 3/72). Overall Kaplan-Meier survival at 10 years was 93.0 ± 3.9% (95% CI: 79.6, 97.7). Peak instantaneous left ventricular outflow tract Doppler gradient decreased from 74.2±36.7mmHg (16.0-242.0mmHg) preoperatively to 12.8±7.4mmHg (2.6-36.0mmHg) postoperatively (p<0.001). Mean left ventricular outflow tract Doppler gradient decreased from 42.4±17.2mmHg (12.0-98.0) preoperatively to 7.5±2.7mmHg (1.4-19.3mmHg) postoperatively (p<0.001). However, over the mean follow-up period of 7.8±6.1 years (0.1-25.2 years), 29.0% (20/69) of patients had recurrence and 18.8% (13/69) required reoperation at median time of 4.8 years (3.1-9.1 years) after the initial repair. Freedom from reoperation at 10 years was 71.1±7.1% (95% CI: 54.6, 82.3). Risk factors for reoperation were age less than five years at initial repair (p=0.036) and extension of the membrane to the aortic valve (p=0.001). Aortic insufficiency was present in 54.2% (39/72) of patients preoperatively. Progression of aortic insufficiency occurred in 38.9% (28/72). Involvement of the aortic valve at initial repair was associated with need for subsequent aortic valve repair or replacement (p=0.01). CONCLUSIONS: Resection of subaortic obstruction is associated with low mortality and morbidity. Recurrence and reoperation rates are high and progression of aortic insufficiency following subaortic resection is common. Therefore, these patients warrant close follow-up into adult life.

Estenosis subglótica en granulomatosis con poliangitis (granulomatosis de Wegener): presentación de 4 casos / Subglottic stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): report of 4 cases

Introducción. La estenosis subglótica (ESG) en la granulomatosis con poliangitis (GPA) puede ser consecuencia de la enfermedad activa o de procesos inflamatorios repetitivos. Nuestro objetivo es describir las características clínicas y el tratamiento de los pacientes con ESG. Métodos. Estudio descriptivo retrospectivo de los casos diagnosticados durante el período comprendido entre el 1 de enero del 2000 y el 1 de junio del 2015. Resultados. Presentamos 4 casos; la ESG se presentó entre los 2 y 144 meses del diagnóstico de la GPA, los síntomas de presentación fueron disnea de esfuerzo y el estridor laríngeo, 3 desarrollaron ESG en ausencia de actividad sistémica. Dos sujetos con ESG grado i fueron tratados con dilatación traqueal, 2 casos presentaron reestenosis y en 3 casos fue necesario la realización de traqueostomía. Conclusión. La ESG presenta una alta morbilidad. La dilatación endoscópica proporciona alivio sintomático; sin embargo, suelen existir recidivas de la estenosis. La obstrucción grave de la vía aérea a menudo requiere de traqueostomía (AU)

Introduction. Subglottic stenosis (SGS) in granulomatosis with polyangiitis (GPA) may result from active disease or from chronic recurrent inflammation. The objective of the study was to describe the clinical features and treatment of patients with subglottic stenosis. Methods. We retrospectively reviewed the medical records of all patients with SGS due to GPA diagnosed at Rheumatology deparment between January 2000 and June 2015. Results. We present 4 cases of SGS at our department during a period of 15 years. The interval between the presentation of the GPA and SGS varied between 2 and 144 months. The leading symptoms of SGS were dyspnoea on exertion and stridor. Three patients presented SGS without evidence of systemic activity. Two patients presented SGS grade i and received tracheal dilatation; two recurred and three needed a tracheostomy due to severe airway-limiting stenosis. Conclusion. SGS presents high morbidity. Even though subglottic dilatation provides symptomatic relief, recurrences may present. Severe airway-limiting stenosis often requires tracheostomy (AU)

Long-term outcomes and risk factors for aortic regurgitation after discrete subvalvular aortic stenosis resection in children.

OBJECTIVES: To characterise long-term outcomes after discrete subaortic stenosis (DSS) resection and to identify risk factors for reoperation and aortic regurgitation (AR) requiring repair or replacement. METHODS: All patients who underwent DSS resection between 1984 and 2009 at our institution with at least 36 months' follow-up were included. Demographic, surgical and echocardiographic data were reviewed. Outcomes were reoperation for recurrent DSS, surgery for AR, death and morbidities, including heart transplant, endocarditis and complete heart block. RESULTS: Median length of postoperative follow-up was 10.9 years (3-27.2 years). Reoperation occurred in 32 patients (21%) and plateaued 10 years after initial resection. Survival at 10 years and 20 years was 98.6% and 86.3%, respectively. Aortic valve (AoV) repair or replacement for predominant AR occurred in 31 patients (20%) during or after DSS resection. By multivariable analysis, prior aortic stenosis (AS) intervention (HR 22.4, p<0.001) was strongly associated with AoV repair or replacement. Risk factors for reoperation by multivariable analysis included younger age at resection (HR 1.24, p=0.003), preoperative gradient ≥60 mm Hg (HR 2.23, p=0.04), peeling of membrane off AoV or mitral valve (HR 2.52, p=0.01), distance of membrane to AoV <7.0 mm (HR 4.03, p=0.03) and AS (HR 2.58, p=0.01). CONCLUSIONS: In this cohort, the incidence of reoperations after initial DSS resection plateaued after 10 years. Despite a significant rate of reoperation, overall survival was good. Concomitant congenital AS and its associated interventions significantly increased the risk of AR requiring surgical intervention.

Diagnosis and surgical management of subaortic stenosis and mitral valve systolic anterior motion.

The case is reported of a patient with a previously undiagnosed cause of severe congestive heart failure (CHF) caused by the presence of a discrete subaortic stenosis (SAS) from a subvalvular membrane (SVM). The clinical decision making was complicated by the concurrent presence of systolic anterior motion (SAM) of the mitral valve leaflet. Due to the limitations and eventual failure of physiologically opposing medical management strategies, the patient eventually required an open-heart surgical approach and underwent intraoperative SVM resection. A persistent intraoperative left ventricular outflow tract (LVOT) gradient of 50 mmHg due to SAM prompted mitral valve replacement, which resulted in a complete resolution of the LVOT gradient and symptoms. In this extremely rare scenario of SAS and SAM, when SVM resection is thought to be inadequate to relieve LVOT obstruction due to the concurrent presence of SAM, mitral valve replacement represents a reasonable therapeutic approach.

The role of shear stress in the pathogenesis of discrete subaortic stenosis: implications for surgical treatment.

Discrete subaortic stenosis (DSS) is characterized by the presence of an obstructing membrane in the left ventricular outflow tract (LVOT). Evidence suggests that the formation of DSS represents a fibroproliferative reaction of the endocardium occurring in response to alterations in shear stress caused by geometric abnormalities within the LVOT. The aim of this review is to discuss the role of altered LVOT shear stress in the pathogenesis of DSS, and its implications in surgical decision making.

Subaortic stenosis associated with systolic anterior motion.

Left ventricular outflow tract obstruction in children is classified according to the site of the obstruction into a supra-aortic type, valvular type, and subaortic type (subaortic stenosis). Subaortic stenosis, in turn, is classified into two major subtypes, i.e., a discrete type, which accounts for most cases and a tunnel type, and one minor subtype, the accessory mitral tissue type, which is rare. Systolic anterior motion (SAM) is a phenomenon that is commonly observed in hypertrophic cardiomyopathy. We report a rare case of subaortic stenosis associated with SAM, which was caused by cleft anterior mitral leaflet and an accessory papillary muscle. Surgical treatment was successful, and there were no complications.

Discrete subaortic stenosis in elderly women.

Discrete subaortic stenosis (DSS) is likely an acquired cardiac disorder which requires anatomic precursors and a genetic background. DSS occurs usually within the first decade, provoking rapidly progressive left ventricular outflow tract obstruction and secondary aortic regurgitation. DSS has been considered for a long time exclusively a disease of infancy and childhood and few reports and small series have described DSS in adulthood and only two cases are reported in elderly. Our case describes a discrete subaortic membranous ridge in an elderly woman with recent onset of dyspnea.

[Discrete subaortic stenosis with pressure recovery: a case report].

A 54-year-old woman with subvalvular aortic stenosis was admitted to our hospital. The pressure gradient across the left ventricular outflow tract was estimated as 88 mmHg (peak) and 45 mmHg (mean) by Doppler echocardiography, but only 14 mmHg (peak to peak) and 31 mmHg (mean) by cardiac catheterization. We considered this discrepancy attributable to the presence of moderate aortic regurgitation and the pressure recovery phenomenon. Pressure recovery has clinical relevance particularly in a patient with tunnel-like stenosis, with gradual lumen re-expansion beyond the limiting orifice. Therefore, if Doppler echocardiography shows significant left ventricular outflow tract gradient, precise evaluation of the stenosis geometry is required to investigate the effect of pressure recovery.