Long-Term Aortic Valve Function in Patients With or Without Surgical Treatment for Discrete Subaortic Stenosis.

The purpose of this study was to evaluate the long-term function of the aortic valve in patients with discrete subaortic stenosis (DSS), and its prognostic implications. 34 patients with the diagnosis of isolated DSS were treated at our pediatric heart center between 1992 and 2019. Demographic, echocardiographic and surgical data were analyzed. 26 patients had surgery to remove the subaortic membrane. The primary endpoint of this study was a change in the aortic valve function, secondary outcomes included functional class, aortic valve replacement, and reoperation due to recurrence of DSS. The mean post-operative follow-up time was 7.07 years (1-22.5). At the time of the most recent follow up, in the surgical group 11 patients (46%) had echocardiographic evidence of deterioration in their aortic valve insufficiency (AI) (p < 0.002). The deterioration was associated with a pre-operative left ventricular outflow tract (LVOT) gradient greater than 49 mmHg (p < 0.022). 8 patients with DSS were followed for a mean of 12.9 years (6.8-21.3), without meeting the criteria for surgical intervention, none showed any change in the aortic valve function during follow-up time. DSS resection may not prevent worsening of aortic valve insufficiency over time. Patients with stable AI and low LVOT gradient may not require surgery. Given these findings, it may be appropriate to carefully follow patients with new onset AI and low LVOT gradient, delaying surgical intervention.

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.

Echocardiographic predictors of reoperation for subaortic stenosis in children and adults.

OBJECTIVES: Subaortic stenosis (SAS) can present as various types of obstruction of the left ventricular outflow tract (LVOT) below the level of the aortic valve. Even though corrective surgery has been identified as the most effective treatment, SAS more frequently reoccurs requiring reoperation in a significant proportion of the patients. Previous studies have focused on predictors of recurrence in various subgroups of patients with SAS, but rarely in the overall population of patients with SAS. The aim of this study was to determine the predictors of recurrence of SAS after initial corrective surgery. METHODS: Patients from the database of the Congenital Cardiology Department of the University Hospital of Southampton with significant SAS requiring corrective surgery were included in the study. Data retrieved were obtained and used to determine the predictors of SAS recurrence after the initial corrective surgery. RESULTS: Eighty-two patients (paediatric, n = 72 and adult, n = 10) who underwent initial successful resection were included in the analysis. Thirty patients required reoperation for recurrent SAS. These were significantly younger (median age 3.0 vs 6.7 years, P = 0.002). The recurrence of SAS was more common in patients with an interrupted aortic arch (23.3% vs 3.8%, P = 0.010) and unfavourable left ventricle geometry (43.3% vs 7.6%, P < 0.001), with steeper aortoseptal angle (131.0° ± 8.7° vs 136.1° ± 8.6°, P = 0.030), shorter distance between the point of obstruction of the LVOT and the aortic valve annulus in systole and diastole (median 4.30 vs 5.90 mm, P = 0.003 and 3.65 vs 4.95 mm, P = 0.006, respectively) and in those who had higher residual peak and mean LVOT gradients postoperatively (29.3 ± 16.0 vs 19.8 ± 10.7 mmHg, P = 0.006 and 15.9 ± 8.3 vs 10.1 ± 5.8 mmHg, P = 0.002, respectively). Overall, the presence of an interrupted aortic arch [odds ratio (OR) 10.34, 95% confidence interval (CI) 1.46-73.25; P < 0.019] and unfavourable left ventricle geometry (OR 10.42, 95% CI 1.86-58.39; P < 0.008) could independently predict reoperation for SAS after initial successful resection. CONCLUSIONS: Patients who have initial corrective surgery for SAS at a younger age, unfavourable left ventricle geometry, an interrupted aortic arch and higher early postoperative LVOT gradients are more likely to have recurrent SAS requiring reoperation.

Correction of left ventricular outflow tract obstruction caused by anomalous papillary muscle and subaortic membrane

Abstract This paper presents a case study of a 30-year-old male patient with dyspnea on exertion had echocardiographic diagnosis of aortic subvalvar stenosis. Discrete mitral regurgitation and aortic valve dysplasia with mild to moderate insufficiency and hypertrophic cardiomyopathy were also noted. During surgery, a rare condition was identified: presence of papillary muscle anomaly associated with the subaortic membrane as a cause of obstruction of the left ventricular outflow tract. With the resection of these structures and a mitral valve annuloplasty, the patient evolved with a significant improvement of clinical condition and heart failure, with no residual mitral insufficiency.

Membranous subaortic stenosis coexisting with hypertrophic cardiomyopathy: A case report.

Subaortic stenosis (SAS) is a congenital heart disease, and its association with hypertrophic cardiomyopathy is very rare and clinically underappreciated. We report here a case of a 45-year-old female who was admitted to our hospital with chest tightness and shortness of breath. Both transthoracic and transesophageal echocardiography revealed asymmetric left ventricular hypertrophy and a membrane-like echo below the level of the aortic valve. This patient was diagnosed with membranous SAS with hypertrophic cardiomyopathy. Screening of her immediate family members revealed that her son also had hypertrophic cardiomyopathy. The patient chose conservative treatments and has been closely followed-up after discharge.

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.

Correction of Left Ventricular Outflow Tract Obstruction Caused by Anomalous Papillary Muscle and Subaortic Membrane.

This paper presents a case study of a 30-year-old male patient with dyspnea on exertion had echocardiographic diagnosis of aortic subvalvar stenosis. Discrete mitral regurgitation and aortic valve dysplasia with mild to moderate insufficiency and hypertrophic cardiomyopathy were also noted. During surgery, a rare condition was identified: presence of papillary muscle anomaly associated with the subaortic membrane as a cause of obstruction of the left ventricular outflow tract. With the resection of these structures and a mitral valve annuloplasty, the patient evolved with a significant improvement of clinical condition and heart failure, with no residual mitral insufficiency.

[Subaortic membrane in adults. A brief review of literature apropos of one case]. / Membrana subaórtica en adultos. Breve revisión de la literatura a propósito de un caso.

A 54-year-old man, ex smoker with high blood pressure and a history of possible Wolff-Parkinson-White syndrome was admitted for presenting an episode suggestive of acute coronary syndrome with immediate syncope and left bundle branch block, while performing physical activity. Angioplasty and a drug-eluting stent were performed in the left circumflex artery. Subsequently, Doppler echocardiography disclosed an image suggestive of a subaortic membrane. Given these findings, the patient underwent a 3D transesophageal echocardiogram and a magnetic resonance study, which confirmed the diagnosis of a subaortic membrane. In turn, in the Holter monitoring a paroxysmal pattern of Wolff-ParkinsonWhite was observed. The patient presented three possible causes of syncope. A stress echocardiogram elicited a gradient of 126 mm Hg, which could be possibly related to the syncopal episode that the patient suffered.

Paciente masculino de 54 años, ex fumador, hipertenso y con el antecedente no confirmado de síndrome de Wolff-Parkinson-White, que ingresó por haber presentado, mientras realizaba actividad física, un cuadro sugestivo de síndrome coronario agudo con inmediato episodio de síncope y bloqueo de rama izquierda, por lo que se le realizó una angiografía coronaria con posterior angioplastia y la colocación de un stent liberador de drogas en la arteria circunfleja. En el ecocardiograma Doppler se observó una imagen compatible con membrana subaórtica. Ante estos hallazgos se realizó un ecocardiograma transesofágico 3D y una resonancia magnética cardiaca que confirmaron el diagnóstico. A su vez se evidenció en el monitoreo Holter y de forma paroxística el patrón de Wolff-Parkinson-White. De esta manera, el paciente presentó tres posibles causas de síncope. Se realizó un ecocardiograma de esfuerzo, en el que el gradiente intraesfuerzo alcanzó los 126 mm Hg, lo que podría justificar el episodio del síncope.

Resection of subaortic membrane for discrete subaortic stenosis.

BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%. CONCLUSIONS: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence.

Acquired discrete subaortic stenosis late after mitral valve replacement.

Although acquired left ventricular outflow obstruction has been reported in a variety of conditions, there are scant reports of its occurrence following mitral valve replacement (MVR). This study describes two female patients, who developed severe discrete subaortic stenosis, five years following MVR. In both cases, the mitral valve was replaced by a porcine Carpentier-Edwards 27-mm bioprosthesis with preservation of mitral valve leaflets. The risk of very late left ventricular outflow tract obstruction after bio-prosthetic MVR with preservation of subvalvular apparatus needs to be kept in mind in symptomatic patients.