Surgical Repair of Secondary Subaortic Stenosis in Congenital Heart Disease Without Initial Subaortic Obstruction.

BACKGROUND: Secondary subaortic stenosis (SSS) is a rare heart disease of the left ventricular outflow tract (LVOT). It usually occurs after cardiovascular correction with or without initial left ventricular outflow tract obstruction (LVOTO). Because most patients with SSS are asymptomatic, many do not realize the need for reoperation until the obstruction worsens. Few studies suggest the characteristics and reasons of SSS without initial SAS. We conducted a retrospective study to describe the characteristics and surgical outcomes of these patients. METHODS: In this study, we examined a single-center retrospective cohort of SSS patients without initial SAS undergoing resection from 2010 to 2019. Patients are defined as secondary subaortic obstruction requiring surgery after cardiovascular correction. Demographics, perioperative findings, and clinical data were analyzed. RESULTS: Twenty-three patients had undergone secondary cardiac surgery for SSS without initial SAS during 10 years in our center. The median age at operation was 7.3 (4.0-13.5) years. In this study, the most commonly associated cardiac lesions were ventricular septal defect (VSD), atrioventricular septal defect (AVSD), patent ductus arteriosus (PDA), and coarctation arch hypoplasia (COA). The surgical techniques included membranous resection of five patients, fibromuscular resection of 17 patients, and reconstruction of the intraventricular baffle of one patient. The results of surgery in these patients are satisfied. The average LVOT gradient at the last follow up was 14.9 (7.8-26.2) mmHg. There was no operative mortality. Two patients had postoperative complications. The median follow-up period was 2.9 (1.1-4.3) years with one late death. Two patients (8.7%) had recurrence of stenosis. CONCLUSIONS: Secondary subaortic stenosis is an uncommon heart disease. The reason is related to several causes, including missed diagnosis, unnoticed abnormalities of LVOT, and further changes of geometric morphology by intracardiac surgery. The results of surgery in these patients are satisfied. However, the recurrence of stenosis is still frequent.

Intraoperative transesophageal echocardiographic predictors of recurrent left ventricular outflow tract obstruction in children undergoing subaortic stenosis resection.

BACKGROUND: Intraoperative transesophageal echocardiography (iTEE) is used to assess for residual left ventricular outflow tract obstruction (LVOTO) after surgical resection of subaortic membrane causing subaortic stenosis (sub-AS). We aimed to identify the iTEE features associated with recurrence of LVOTO. METHODS: We conducted a retrospective study of children undergoing sub-AS resection from June 2006 to June 2014. Doppler assessment of the flow velocity and the anatomical features of the left ventricular outflow tract were analyzed from stored echocardiograms. Recurrent LVOTO was defined as an increase in the mean pressure gradient across the left ventricular outflow tract of > 15 mm Hg on the most recent follow-up echocardiogram from the mean pressure gradient on the predischarge echocardiogram or as doubling of the mean pressure gradient to a value ≥20 mm Hg. RESULTS: Thirty-five patients were included, with median age at surgery was 8.1 years (range: 0.7-29 years) and median follow-up was 47 months (2-91 months). Ten patients (29%) had recurrent LVOTO, which was associated with a shorter distance between the narrowest diameter of the outflow tract and the aortic valve on iTEE [median 0.59 cm (range 0.39-0.74) vs 0.98 cm (0.75-1.5), P = .03]. No patients with more than mild residual LVOTO on iTEE regressed to mild or no LVOTO on follow-up echocardiograms. CONCLUSIONS: LVOTO recurrence after sub-AS resection is common, and residual LVOTO remains the same or increases over time. Proximity of the LVOTO to the aortic valve is a risk factor for recurrent LVOTO. These findings may be useful in counseling patients and to guide the frequency of postoperative follow-up.

Congenital discrete subaortic stenosis in pregnancy: case report and literature review.

Today's growing numbers of pregnant patients with congenital heart defects presents challenges for cardiologists treating adult patients. The case is presented of a 19-year-old woman who was admitted with a threatened miscarriage in the third trimester and was diagnosed with an incomplete atrioventricular canal, cleft mitral valve with severe regurgitation, and severe congenital subaortic stenosis caused by a subaortic membrane. She was managed conservatively, delivered via cesarean section, and then underwent surgical repair of all defects with good outcome. Here, a review is provided of the literature on the natural course, complications, and treatment strategies of subaortic stenosis, together with a summary of recommendations for the management of the condition.

Surgical management of supravalvar aortic stenosis with coronary artery involvement.

Supravalvar aortic stenosis is characterized by an obstruction of the left ventricular outflow tract distal to the aortic valve. Its association with left main coronary artery stenosis is well known but right coronary artery involvement has not been reported. We describe two cases of supravalvar aortic stenosis with coronary artery stenosis and its surgical management.

[Epidemiological, clinical and echographic profile of patients operated for subvalvular aortic stenosis in the region of Sfax (Tunisia) and factors associated with postoperative recurrence: an observational study]. / Profil épidémiologique, clinique et échographique des patients opérés pour un rétrécissement aortique sous-valvulaire dans la région de Sfax (Tunisie) et facteurs associés à la récidive post-opératoire: étude observationnelle.

Subvalvular aortic stenosis is difficult to manage due to the evolutionary unpredictability of stenosis and a high recurrence rate after surgical treatment. The purpose of this study is to describe the profile of patients undergoing surgery for the treatment of aortic subvalvular stenosis and to investigate factors associated with post-operative recurrence of the subaortic obstacle. We conducted an observational study of all patients operated for subvalvular aortic stenosis, whose data were collected in the Department of Cardiology of the Sfax University Hospital between January 2010 and December 2020. The study involved 28 patients, with predominance of male sex (64.29%, n=18). At diagnosis, the mean age was 6.82 (±4.84) years and 19 patients (67.85%) had symptoms. On echocardiography, maximal subaortic gradient ≥50 mmHg was found in 23 patients (82.14%). Cardiovascular malformations associated with subvalvular stenosis were found in 16 patients (57.14%). The average age of patients at the time of surgery was 10.43 (±7.08) years. Subaortic membrane resection was the most commonly used technique (46.4%, n=13). It was associated with septal myomectomy in 8 patients (28.6%). Postoperative mortality rate was zero. Residual gradient ≥30 mmHg was reported in 8 patients (28.6%) after surgery. Recurrences were observed in 7 patients (25%) of whom 6 underwent reintervention. In multivariate analysis, only postoperative residual gradient was significantly associated with recurrence (p=0.030, OR=33.785, 95% CI: 1.398-816.754). Despite old age at diagnosis and surgery, favorable short-term outcomes were reported, but recurrences were frequent in the long term. This highlights the role of regular, perioperative and postoperative, clinical and echographic monitoring of these patients.

The dilemma of subaortic stenosis--a single center experience of 15 years with a review of the literature.

OBJECTIVE: The term "subaortic stenosis" includes a variety of obstructions of the left ventricular outflow tract (LVOT), ranging from a short (discrete) subvalvular membrane to long, tunnel-like narrowing. An association with other congenital lesions is frequent. We reviewed the reported literature and describe our results, analyzing the nomenclature of and risk factors for restenosis after surgical treatment. METHODS: From 1994 to 2009, 81 children (53 males, 28 females; median age: 57 months, range [ R]: 5-204) underwent surgical relief of a subaortic stenosis. Patients were divided, according to pathology, into short segment (group A, n = 42) and complex obstructions (group B, n = 39), with the latter including long segment stenosis and/or associated anomalies such as aortic coarctation, interrupted aortic arch or Shone's complex. RESULTS: Surgery resulted in a significant reduction of the gradient between the left ventricle and the aorta in both groups (Δ P group A: 51 ± 28 mmHg, group B: 46 ± 25 mmHg). There was no operative mortality. One patient died in the early postoperative period due to pericardial tamponade. Median follow-up was 90 months (R = 0.5-187). Twenty-five (31%) patients required reoperation because of recurrent stenosis after a median of 43 months (R = 0.5-128). Seven (16%) patients belonging to group A developed restenosis, and 18 (46%) in group B. Freedom from reoperation for all patients was 60% after 10 years. 10 (40%) of the patients of group B were ultimately treated with a Ross-Konno reconstruction of the LVOT. CONCLUSION: Despite adequate surgical resection, recurrence of subaortic stenosis within several years after initial surgical treatment is frequent, especially in patients with complex lesions. In cases requiring reoperation, the surgical therapy is often extensive, and even includes Ross-Konno reconstruction of the LVOT.

Treatment of severe valvular aortic stenosis and subvalvular discrete subaortic stenosis and septal hypertrophy with Percutaneous CoreValve Aortic Valve Implantation.

BACKGROUND: Percutaneous aortic valve implantation (PAVI) is a procedure gaining popularity and becoming more widely used for the treatment of patients with severe aortic stenosis who are at high risk for surgery. Here we show, for the first time, that a successful and complete elimination of both valvular and subvalvular pressure gradients can be achieved with a slight modification of the valve implantation technique. METHODS AND RESULTS: A 91-year-old woman presented with shortness of breath at rest, effort angina, and pulmonary congestion. Echocardiography revealed calcified aortic stenosis with a peak gradient of 75 mm Hg across the valve, and discrete subaortic stenosis (DSS) and marked hypertrophy of the basal septum with systolic anterior motion of the mitral valve (SAM). The intra ventricular gradient had a dynamic pattern across the DSS and the septal hypertrophy and measured 75 mm Hg. The total gradient across the left ventricular outflow (valvular and subvalvular) was 125 mmHg. PAVI with a 23 mm CoreValve was performed with an intentional lower positioning of the valve towards the LV outflow tract; so that the valve struts cover the subaortic membrane and part of the thickened basal septum. At the end of the procedure, the SAM disappeared, and the left ventricular ouflow was widely open. At 1 month follow up the patient was asymptomatic, no pressure gradient was measured between the LV apex and the aorta. CONCLUSIONS: This is the first report of successful treatment of severe valvular aortic stenosis and combined subvalvular aortic stenosis due to DSS and septal hypertrophy with SAM with percutaneous aortic valve implantation.

Left main coronary artery and supravalvular aortic stenosis in adult: treatment with ostial patchplasty and modified Brom procedure.

Left main coronary artery (LMCA) stenosis accompanying to supravalvular aortic stenosis is a very uncommon, serious congenital abnormality. Aortic valve leaflet fusions and intimal thickening of the aortic valve leaflets and coronary artery are the underlying pathologies for the LMCA stenosis. We operated on a 21-year-old male patient for supravalvar aortic stenosis with LMCA ostial stenosis. We enlarged the LMCA with a pericardial patch (ostial plasty) and reconstructed the aortic root with a modified Brom procedure. Postoperative course was uneventful; echocardiographic evaluation revealed a normal functioning aortic valve with a normal left ventricular function. Gradient at left ventricular outflow tract was decreased a great deal. Although supravalvular aortic stenosis with LMCA stenosis is a very rare congenital abnormality, this clinical entity can be successfully corrected with detailed and selected surgical procedures.

Subvalvular aortic stenosis: a review of current literature.

Subvalvular aortic stenosis (SAS) is one of the common adult congenital heart diseases, with a prevalence of 6.5%. It is usually diagnosed in the first decade of life. Echocardiography is the test of choice to diagnose SAS. Surgical correction is the best treatment modality, and the prognosis is usually excellent. In this review, we describe the pathophysiology, diagnosis, prognosis, and management of SAS with a focus on different pathophysiologic mechanisms, diagnostic approach, and prognosis of the disease by reviewing the current literature.

Anomalous papillary muscle insertion contributing to obstruction in discrete subaortic stenosis.

A case of discrete subvalvular aortic stenosis with anomalous insertion of a papillary muscle to the base of the anterior mitral valve leaflet and continuous with the discrete subaortic stenosis is described. Two-dimensional echocardiographic and pathologic data showing the contribution of the anomalous papillary muscle to left ventricular outflow tract obstruction are presented.

"Acquired" discrete subvalvular aortic stenosis: natural history and hemodynamics.

Discrete subvalvular aortic stenosis is a progressive lesion. Most reported cases have had a measurable left ventricular-aortic gradient that progressed in severity. This report describes 35 patients in whom no significant left ventricular-aortic obstruction was noted at initial cardiac catheterization, but who later were shown to have significant subvalvular aortic stenosis. In 24 of the 35 cases, absence of a significant left ventricular-aortic gradient (less than or equal to 10 mm Hg) was documented at initial cardiac catheterization. In 11 patients, a left ventricular-aortic pressure gradient was not obtained or not sought in the absence of clinical evidence of an obstructive lesion. In each case, discrete subaortic stenosis was not noted on angiography. Associated lesions included ventricular septal defect in 7, patent ductus arteriosus in 12, coarctation of the aorta in 8, pulmonary stenosis in 3, atrioventricular canal in 2 and miscellaneous lesions in 3 cases. The 35 patients had documentation of subvalvular aortic stenosis 3 months to 19 years after their initial study based on repeat catheterizations in 26, echocardiography in 6 or discovery at surgery in 3 cases. There were eight children with coarctation and no left ventricular-aortic gradient who developed significant subvalvular stenosis at a median of 2 years 9 months after initial cardiac catheterization. Of 30 patients with associated lesions, 23 had surgical intervention before development of subvalvular aortic stenosis, but only 5 of 17 patients with ventricular septal defect had surgical repair or palliation specifically for the interventricular communication before development of subvalvular aortic stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Malaligned outlet septum with subpulmonary ventricular septal defect and abnormal ventriculoarterial connection: a morphologic spectrum defined echocardiographically.

A retrospective analysis of 62 echocardiograms from 22 patients with malaligned outlet septum, subpulmonary ventricular septal defect and abnormal ventriculoarterial (VA) connection was performed and the results were confirmed by intraoperative inspection, cardiac catheterization and angiography, or pathologic specimen analysis in 21 of the 22 patients. The purpose of this study was to determine the relative incidence of the primary morphologic features; the correlation between the presence or absence of mitral valve to pulmonary valve fibrous continuity and the type of VA connection; the types and incidence of additional cardiovascular anomalies; and the correlation between the determination of the VA connection by echocardiography versus that ascertained at intraoperative and autopsy inspection. A spectrum of VA connections was found, ranging from double outlet right ventricle (23%) at one extreme, through an intermediate type (64%) in which the pulmonary artery was overriding the ventricular septum, thereby having no consistent and predominant connection to either ventricle, to a discordant VA connection (13%) at the opposite end of the spectrum. All three types of VA connection occurred whether or not there was mitral valve to pulmonary valve fibrous continuity. Additional cardiovascular anomalies were present in 95% of patients, the most common being aortic arch obstructive lesions in 59%, anomalous attachments of the atrioventricular valve tension apparatus in 55% and subaortic stenosis in 50%. A method of echocardiographic determination of the VA connection that correlated with that determined intraoperatively and from pathologic specimen analysis is described.

[Subaortic stenosis associated with perimembranous ventricular septal defect. Clinical follow-up of 36 patients]. / Estenose subaórtica associada a comunicação interventricular perimembranosa. Acompanhamento clínico de 36 pacientes.

OBJECTIVE: To study the clinical pattern of subaortic stenosis associated with perimembranous ventricular septal defect. METHODS: From January 1979 to June 2000, 36 children with perimembranous ventricular septal defect and fixed subaortic stenosis were followed-up regarding anatomic characteristics, evolvement, and clinical events. RESULTS: Age at diagnosis of subaortic stenosis ranged from 6 months to 170 months, and it was less than 1 year in only 2 children. Regarding sex, the distribution was 2:1 with a greater predominance of males. Ventricular septal defect was small in 61.0% of cases, medium in 30.56%, and large in 8.40%; the size of the septal defect decreased during follow-up in 30.56% (11 cases). In all patients, subaortic stenosis was membranous and fixed. During follow-up, 23 patients experienced evolvement of the stenosis. Surgical treatment was performed in 21 cases, and one patient underwent surgery for restenosis. Infectious endocarditis occurred in 2 patients; one of the patients died. CONCLUSION: Subaortic stenosis occurs in the natural history of ventricular septal defect usually after the first year of life, and it is progressive and requires surgery in most cases.