Secondary subaortic stenosis following ventricular septal defect closure: A retrospective study in a single center / 中国胸心血管外科临床杂志

@#Objective     To summarize the characteristics of children diagnosed with secondary subaortic stenosis after the surgical closure for ventricular septal defect and explore its potential mechanism. Methods     We retrospectively collected patients aged from 0 to 18 years, who underwent ventricular septal defect closure and developed secondary subaortic stenosis, and subsequently received surgical repair from 2008 to 2019 in Fuwai Hospital. Their surgical details, morphological features of the subaortic stenosis, and the follow-up information were analyzed. Results     Six patients, including 2 females and 4 males, underwent the primary ventricular septal defect closure at the median age of 9 months (ranging from 1 month to 3 years). After the first surgery, patients were diagnosed with secondary subaortic stenosis after 2.9 years (ranging from 1 to 137 months). Among them, 2 patients underwent the second surgery immediately after diagnosis, and the other 4 patients waited 1.2 years (ranging from 6 to 45 months) for the second surgery. The most common type of the secondary subaortic stenosis after ventricular septal defect closure was discrete membrane, which located underneath the aortic valve and circles as a ring. In some patients, subaortic membrane grew along with the ventricular septal defect closure patch. During the median follow-up of 8.1 years (ranging from 7.3 to 8.9 years) after the sencond surgery, all patients recovered well without any recurrence of left ventricular outflow tract obstruction. Conclusion     Regular and persistent follow-up after ventricular septal defect closure combining with or without other cardiac malformation is the best way to diagnose left ventricular outflow tract obstruction in an early stage and stop the progression of aortic valve regurgitation.

A Case of Biventricular Repair with Damus-Kaye-Stansel (DKS) Take-Down after DKS Anastomosis for Interruption of the Aortic Arch and Ventricular Septal Defect with Subaortic Stenosis / 日本心臓血管外科学会雑誌

The patient in this case was a boy aged 2 years and 9 months. The patient was transferred to our hospital with ductal shock, and bilateral pulmonary artery banding was performed on the 9th day after the diagnosis of interruption of the aortic arch, ventricular septal defect, subaortic stenosis, and bicuspid aortic valve. Left ventricular outflow tract stenosis due to aortic annulus diameter and subaortic stenosis after repair was suspected. Damus-Kaye-Stansel (DKS) anastomosis, extended aortic arch anastomosis, and a right modified Blalock-Taussig operation were performed. Preoperative examination of the intracardiac repair showed growth of the aortic annulus and confirmed that biventricular repair was possible after DKS take-down. The patient's native aortic and pulmonary valves were preserved, and an intracardiac repair was performed without using an extracardiac conduit. The postoperative course was uneventful, and the patient is currently in a good condition at the age of 6 years. Three and a half years after surgical intervention, echocardiography and cardiac catheterization showed improvement of subaortic stenosis and enlargement of the aortic annulus. Our findings indicate that the most appropriate surgical procedure can be selected by detailed examination of the preoperative condition at each stage of the staged operation.

Successful Percutaneous Balloon Dilatation of Membranous Sub-aortic Stenosis in a 22 Year Old Pregnant Patient

Congenital obstruction of the left ventricular outflow tract comprises a heterogeneous group of disorders, with obstruction potentially occurring below, above, or at the level of the aortic valve. Subvalvular stenosis is the second most common type of left ventricular outflow tract obstruction, of which discrete membranous type is the most common. Although surgical resection of the subaortic membrane is the treatment of choice in discrete membranous subaortic stenosis, in selected patients with isolated membranous subaortic stenosis, without significant aortic insufficiency, percutaneous balloon tearing of the membrane results in reduction in the degree of left ventricular outflow tract obstruction and symptomatic relief. We report a case of 22 year old pregnant patient admitted with NYHA class III breathlessness, found to have discrete membranous subaortic stenosis. Balloon aortic valvuloplasty was performed in the patient with good result. Patient underwent normal vaginal delivery at 38 weeks. Both mother and newborn were asymptomatic. Patient is asymptomatic on subsequent follow-ups.

Surgical treatment results of secondary tunnel-like subaortic stenosis after congenital heart disease operations / 中华胸心血管外科杂志

Objective To summarize surgical results of secondary tunnel-like subaortic stenosis( STSS) after congenital heart disease( CHD) operations, analyze the pathogenesis of STSS, evaluate the operative effect and prognosis.Methods We analyzed clinical data of 25 patients who underwent surgical repair for STSS in Fuwai Hospital from January 2009 to December 2015.There were 17 males and 8 females.CHD types included double outlet right ventricle, ventricular septal defect and par-tial atrioventricular septal defect, et al.The median age of the patients when they received their first CHD operations was 1 year (1 month to 42 years).The median age of the patients when they received TSS repair was 5 years and 8 months(2 years and 10 months to 48 years) .The surgical types contained modified Konno procedure , Konno procedure, resection of the conal sep-tum, resection of fibromuscular ridge and myectomy, removal and reconstruction of the intraventricular baffle.Results All the patients successfully received their TSS repair .There was no surgical death in this study .The preoperative gradient of aortic valve pressure was 81(43 to 159)mmHg(1 mmHg=0.133 kPa), and the postoperative gradient was 8.2(4.0 to 46.2) mm-Hg.Ⅲ degree atrioventricular block was occurred in 2 patients after operation and both 2 patients needed to set permanent pacemaker.All the patients were followed up after discharge for a median duration of 2 years( 4 months to 6 years) .During follow-up, none of the patients had any clinical symptom or subaortic restenosis , and there was no late death .Conclusion The pathogenesis of STSS is perhaps related to turbulent flow and shear stress to the left ventricular outflow tract , resulting in the proliferation of fibromuscular marterials and stenosis in the left ventricular outflow tract .The modified Konno procedure and resection of the conal septum are safe and effective.

Surgical management of secondery subaortic stenosis after repair of congenital heart disease: a ten-year experience / 中华胸心血管外科杂志

Objective A retrospective study of patients in the past ten years who underwent surgeries because of secondary subaortic stenosis(SSS) after previous repair of congenital heart disease(CHD) was performed.The anatomy,indication,and surgical outcomes were studied.Methods Between January 2004 and October 2013,forty-eight patients underwent surgeries because of SSS.Thirty-five patients were males,and 13 were females.The mean age was(54.0 ± 34.9) months (12-156 months).The mean weight was(16.1 ±6.2)kg(6.0-37.5 kg).And the mean interval between the first and second surgery was(39.6 ±25.0) months(11-115 months).The primary diagnosis of CHD included ventricular septal defect,double outlet of right ventricle,corctation,interruption of aortic arch,patent trucus arteriosus,atrio-ventricular defect,tetralogy of fallot,transposition of great arteries,and univentricle.Results Twenty-five patients were discrete subaortic stenosis,and had the fibromuscular ridge resected.Twenty-three patients were diffuse type of subaortic stenosis.Myectomy,reconstruction of the intraventricular baffle,and modified Konno procedure were done in 8,5,and 10 patients separately.One patient died of cardiac failure postoperatively.One developed complete left bundle branch block,and another one developed complete atrioventricular block.The pressure gradients dropped from(76.8 ±20.4)mmHg to(12.4 ±8.4)mmHg,P ＜0.001.One patient was lost in follow-up and the left 46 patients had been followed up for 27 ～ 144 months [mean (59.6 ± 28.3) months] with no late death.The recent echocardiography results showed that the pressure gradients were(16.3 ± 15.2) mmHg.Five patients required reoperations due to recurrence of stenosis and the freedom from reoperation was 82.8％ at 10 years.Conclusion Earlier diagnosis and intervention is recommended for SSS,and excellent relief can be achieved.

Surgical management of secondery subaortic stenosis after repair of congenital heart disease: a ten-year experience / 中华胸心血管外科杂志

Objective A retrospective study of patients in the past ten years who underwent surgeries because of secondary subaortic stenosis(SSS) after previous repair of congenital heart disease(CHD) was performed.The anatomy,indication,and surgical outcomes were studied.Methods Between January 2004 and October 2013,forty-eight patients underwent surgeries because of SSS.Thirty-five patients were males,and 13 were females.The mean age was(54.0 ± 34.9) months (12-156 months).The mean weight was(16.1 ±6.2)kg(6.0-37.5 kg).And the mean interval between the first and second surgery was(39.6 ±25.0) months(11-115 months).The primary diagnosis of CHD included ventricular septal defect,double outlet of right ventricle,corctation,interruption of aortic arch,patent trucus arteriosus,atrio-ventricular defect,tetralogy of fallot,transposition of great arteries,and univentricle.Results Twenty-five patients were discrete subaortic stenosis,and had the fibromuscular ridge resected.Twenty-three patients were diffuse type of subaortic stenosis.Myectomy,reconstruction of the intraventricular baffle,and modified Konno procedure were done in 8,5,and 10 patients separately.One patient died of cardiac failure postoperatively.One developed complete left bundle branch block,and another one developed complete atrioventricular block.The pressure gradients dropped from(76.8 ±20.4)mmHg to(12.4 ±8.4)mmHg,P ＜0.001.One patient was lost in follow-up and the left 46 patients had been followed up for 27 ～ 144 months [mean (59.6 ± 28.3) months] with no late death.The recent echocardiography results showed that the pressure gradients were(16.3 ± 15.2) mmHg.Five patients required reoperations due to recurrence of stenosis and the freedom from reoperation was 82.8％ at 10 years.Conclusion Earlier diagnosis and intervention is recommended for SSS,and excellent relief can be achieved.

Balloon valvuloplasty for severe subaortic stenosis in a Pomeranian dog

A nine-month-old Pomeranian dog with exercise intolerance and syncope was presented. The dog was depressed with grade 4 systolic murmur on cardiac auscultation. Based on cardiac examination, the dog was diagnosed with severe subaortic stenosis with involvement of the anterior mitral valve. β-blocker administration was initiated and clinical signs were improved, but not fully resolved. Balloon valvuloplasty was performed and the dog survived for nearly one year without clinical sign and the cardiac troponin I level was normalized. This case describes successful management of severe subaortic stenosis in a small breed dog through balloon valvuloplasty.

Tratamiento mediante dilatación con balón de un paciente con estenosis subaórtica por membrana: a propósito de un caso / Percutaneous balloon dilatation in a patient with membrane subaortic stenosis: a propos of a case

La estenosis subaórtica es una lesión cardiaca poco frecuente, en el cual el tracto de salida del ventrículo izquierdo está estrechado por la presencia de un anillo fibroso o fibromuscular. Constituye 8 a 20% de todas las formas de obstrucción al tracto de salida del ventrículo izquierdo. La cirugía ha sido considerada el tratamiento de elección cuando el gradiente pico es mayor de 50 mmHg o en pacientes que, con gradientes menores, presentan progresión de la insuficiencia aórtica o dilatación de los diámetros ventriculares, disfunción ventricular o síntomas. Presentamos el caso de un paciente menor de edad, portador de estenosis subaórtica por membrana, con síntomas de insuficiencia cardiaca y función ventricular izquierda muy comprometida, en la que se realizó el tratamiento percutáneo, alternativo al quirúrgico, con resultado exitoso. Constituye el primer caso reportado en el país de tratamiento percutáneo con balón y con evolución favorable.

Discrete subaortic stenosis is a rare cardiac lesion with left ventricle outflow narrowed by the presence of a muscular or fibrous ring. It accounts for 8-20% of all forms of left ventricle outflow tract obstruction. Surgery is considered the treatment of choice when the peak gradient is over 50 mmHg or in patients with lower gradients and either progression of aortic regurgitation, dilated ventricular diameters, left ventricular dysfunction or symptoms. We report the case of a child with membrane subaortic stenosis and symptoms of heart failure and compromised left ventricular function, in whom percutaneous balloon dilatation was performed as an alternative to surgery. This is the first case reported in the country with successful outcome.

Síndrome de Shone incompleto: a propósito de un caso diagnosticado en la adultez / Incomplete Shone's syndrome. Apropos of a case diagnosed in adulthood

El síndrome de Shone constituye una rara entidad cardíaca congénita que consiste en la presencia de lesiones estructurales obliterantes de hemicardio izquierdo que incluyen: anillo supravalvular, válvula mitral en paracaídas, estenosis sub-aórtica y coartación aórtica. Es poco frecuente que curse en su forma incompleta. El caso que a continuación se presenta, describe el síndrome de Shone incompleto detectado en un adulto

Shone's syndrome is a rare congenital heart disease consisting in the presence of obliterating structural lesions on the left side of the heart, including supravalvular ring, parachute mitral valve, subaortic stenosis and aortic coarctation. The incomplete form of the disease is not common. The presentation describes a case of incomplete Shone's syndrome diagnosed in adulthood

Ross operation in a neuro-cardio-facial-cutaneous syndrome patient.

Congenital heart diseases are a major part of Costello and cardio-facio-cutaneous syndromes. Subaortic stenosis was reported rarely and Ross operation never in these syndromes. We reported a girl patient whose manifestations were consistent with these syndromes. Distinction between these syndromes was not possible as genetic testing was not carried out. She developed severe neoaortic regurgitation 2.5 years after the Ross operation and died due to the complications of aortic valve replacement. Ross operation may be an unsuitable option in these syndromes due to the possibility of subtle pulmonic valve pathology.

Secondary Subaortic Stenosis after Patch Closure of Subarterial Ventricular Septal Defect

Subaortic stenosis usually occurs without a previous heart operation, however, it can occur after heart surgery as well, with a condition known as a secondary subaortic stenosis (SSS). SSS has been reported after surgical repair of several congenital heart defects. There are only a few recorded cases of SSS after repair of ventricular septal defect (VSD). Here we report a rare case of SSS that occurred 3 years after surgical repair of subarterial VSD. A follow-up echocardiogram is essential for detecting SSS caused by the newly developed subaortic membrane in patients who had cardiac surgery.

Accessory mitral valve: report of four cases and literature renew / 中华内科杂志

Objective To describe the echocardiographic and clinical findings of patients with accessory mitral valve(AMV).Methods Four adult patients were diagnosed to have AMV by Doppler echocardiography.Results All the four patients had cardiac murmur but 2 were asymptomatic.Echocardiogram showed that two cases had isolated AMV with significant left ventricular outflow tract (LVOT)obstruction.Another patient was complicated by idiopathic hypertrophic subaortic stenosis and his systolic pressure gradient across the LVOT reduced to normal after taking negative inotropic drugs.The fourth patient was associated with complex congenital cardiac anomalies but without LVOT obstruction.Conclusions AMV may not be very rare as previously reported.An echocardiographic examination Can detect AMV and difierentiate it from other causes of LVOT obstruction.

Surgical treatment of congenital subaortic stenosis / 第三军医大学学报

Objective To review retrospectively the clinical experience in surgical treatment of congenital subaortic stenosis in 21 cases. Methods A total of 21 cases of congenital subaortic stenosis, including 12 cases of diaphragmatic type of stenosis and 9 cases of cast constriction, underwent intracardiac surgery from January 1999 to June 2003. Simple resection of stenosis membrane was performed in 12 cases, simple resection of stenosis membrane plus resection of myocardium of left ventricle outflow tract in 9 cases, simple resection of stenosis membrane plus resection of myocardium of left ventricle outflow tract plus treatment of united cardiac monstrosity in 16 case. Results No operative death and complications were found in all patients. Disappearance of cardiac murmur was found in 15 cases and diminishment of cardiac murmur in the rest of the patients after operation. A follow up time for 6 months to 2 years revealed that there were no obvious clinical symptoms, and the surgical outcomes were satisfactory. The cardiac ultrasound suggested that the left ventricular outflow tract was fluent without recurrent constriction. Conclusion Early treatment of congenital subaortic stenosis should be conducted when the disease was diagnosed. Better knowledge of pathological anatomy, proper operation, and prevention of complications are essential.

Two Cases of Left Ventricular Tumors Complicating Subaortic Stenosis in Newborn Infants

The most cornmon obstruction of left ventricular outflow in childhood is produced either by a fibrous ring below the aortic valve, or by localized or diffuse muscular hypertrophy of the inter-ventricular septum. Cardiac tumors causing subaortic stenosis in the newborn infant are extremely rare. This report describes two cases of subaortic stenosis caused by a tumor of the left ventricle in newborn infants, both diagnosed by 2-dimensional echocardiography. In one patient, the obstructive portion of the tumor was successfully resected from its attachment to the ventricular septum by aortotomy on day 5. Histologic examination confirmed the diagnosis of rhabdomyoma. This patient developed cutaneous, neurologic and radiologic abnormalities of tuberous sclerosis during the follow-up of 42 months.

Resultados da valvoplastia percutânea por cateter baläo na estenose subaórtica em membrana / Results of percutaneous balloon valvuloplasty for membranous subaortic stenosis

OBJETIVO - Avaliar os resultados da valvoplastia percutânea por cateter baläo (VPCB) na estenose sub-aórtica em membrana (ESAM). MÉTODOS - Sete pacientes com diagnóstico de ESAM foram submetidos a VPCB por via retrógada. Estudos hemdinâmicos e angiográficos foram realizados antes e após o procedimento. A relaçäo baläo/anel variou de 0,9 a 1,05. RESULTADOS - O gradiente sistólico (GS) entre o ventrículo esquerdo (VE) e a aorta foi reduzido de 57,8ñ 11,6 para 22,0ñ8,6mmHg (p<0,001) e a pressäo sistólicaem VE de 154,5ñ30,3 para 118,7ñ18,4mmHG(p<0,001). Um paciente paresentou oclusäo arterial necessitando de embolectomia cirúrgica e um outro apresentou insuficiência mitral severa aliada a insuficiência aórtica (IAo), requerendo plastia cirúrgica. Näo tivemos mortalidade. Quatro pacientes tiveram seguimento de 11ñ10 meses: o GS pelo ecocardiograma foi de 34ñ7mmHG e näo houve progressäo de o. CONCLUSÄO: A VPCB é um procedimento eficaz na reduçäo imediata de gradiente subaórtico, com morbidade aceitável e com manutençäo dos resultados a curto prazo. Consideramos como uma alternativa válida na abordagem inicial dos pacientes com ESAM.

Fixed Subaortic Stenosis in Children

PURPOSE: This study attempted to describe the Doppler and two-dimensional echocardiographic features of fixed subaortic stenosis, the de novo occurrence and progression of this lesion and to review its etiology, treatment and course. METHODS: We studied 17 patients of fixed subaortic stenosis who, between Jan. 1993 and Jan. 1996, ware diagnosed by echocardiographic examination at Kyungpook National University Hospital. The patients' charts, the Doppler and echocardiographic findings as well as the operative reports were retrospectively reviewed. RESULTS: 1) There were 17 patients (14 boys and 13 girls, male/female ratio 1:1) of fixed subaortic stenosis which comprised 1.1% of congenital heart disease (17/1609) and 15% of congenital left ventricular outflow tract (LVOT) obstruction (17/113). 2) Ages of first detection ranged from 3 days to 11 years (mean : 2.74 years), and most of them (14/17) were under 5 years, and 8 under 1. 3) All of them had other major cardiac diseases and subaortic stenosis was diagnosed incidentally during the evaluation (initial and follow-up) of major cardiac diseases. Mean duration between diagnosis of major cardiac diseases and that of subaortic stenosis (11 cases) was 2.83 years (4 months-10 9/12 years), and the other 6 cases were diagnosed coincidentally. 4) The types of fixed subaortic stenosis were membranous in 10 cases (58.8%) and fibromuscular in 7 cases (41.2%). 5) Mean left ventricular to aortic peak systolic pressure gradient (Doppler) at initial diagnosis (9 cases) was 26.6 mmHg and in 6 cases underwent surgical resection 29.17 mmHg but there were no gradient in 8 cases. 6) Aortic insufficiency was found in 4 cases (23.5 %). 7) There were some evidences of progression of the lesion in 4 patients inspite of relatively short follow-up period (mean : 2.08 years). 8) Resection of fixed subaortic stenosis (membranectomy) was performed in 6 cases. CONCLUSIONS: Our data that most cases were coexisting with congenital heart diseases, and under 5 years of age suggest subaortic stenosis is a congenital lesion but also does not rule out it is an acquired lesion in the presence of some evidences of progression in our four patients.

Echocardiographic Study of the Ventricular Septal Defect with Subaortic Ridge

PURPOSE: The easy recognition and characterization of a discrete or fixed subaortic ridge by echocardiography provides a method for prospective study of the coexistence of ventricular septal defect (VSD) and subaortic ridge. In the presence of a VSD, a subaortic ridge may be clinically silent, but the obstruction can progress if the VSD is surgically or spontaneously closed. This study was carried out to test the hypothesis that the presence of a subaortic ridge associated with a VSD is related abnormal shunt flow through the septal defect. METHODS: Serial two-dimensional echocardiographic diagnosis of the coexistence of VSD and subaortic ridge was done prospectively in 271 patients. The VSD was morphologically characterized as perimembranous, muscular, or subarterial according to the components of its borders. Also, defect size of the VSD was characterized. The presence of septal malalignment was established when the outlet septum was deviated anteriorly or posteriorly. The subaortic ridge was recognized when an echo-bright localized protusion into the left ventricular outflow tract extending from the margin of the septal defect. RESULTS: The prevalence of a subaortic ridge was 8.86% (24/271). The mean age of patients at the initial detection of a subaortic ridge was 11.3+/-9.8 months. Among the 24 patients with a subaortic ridge 14 patients (p<0.01) had more than moderate defect in size of VSD. 16 patients (16/24) had malalignment VSD (p<0.01). A subaortic ridge was found in 20 (9.9%) patients with perimembranous VSD, 4 (8.3%) with subarterial VSD. CONCLUSIONS: The presence of a subaortic ridge associated with a VSD is related to a more than moderate sized defect and/or a malaligned ventricular septum. It is possible that the increased turbulence adjustance to the area of the VSD could favor the development of an abnormal fibrous tissue below the aortic valve.

Papel do septo atrioventricular e da separação mitro-aórtica na patogênese da estenose subaórtica fixa / Role of the atrioventricular septum and mitro-aortic separation in the pathogenesis of fixed subaortic stenosis

PURPOSE: To analyze, by cross-sectional echocardiography, morphological features of the atrial atrioventricular and ventricular septum potentially involved in the genesis of fixed subaortic stenosis. METHODS: Forty three children with fixed subaortic stenosis were compared with 86 normal children, matched by age, sex and body surface, and 43 children with congenital heart defects without fixed subaortic stenosis. RESULTS: The groups did not differ in as age, sex or body surface. The atrioventricular septum was significantly smaller in children with subaortic stenosis than in normal children or patients with other congenital heart diseases. The ventricular septum was significantly more aligned with the atrial septum in cases than in normal children and in patients with other congenital heart diseases. The odds ratio for the development of fixed subaortic stenosis was statistically significant in the presence of a short atrioventricular septum and with alignment of the ventricular and atrial septum, when analyzed in isolation or when controlled by perimembranous ventricular septal defect. CONCLUSION: It was concluded that in fixed subaortic stenosis the atrioventricular septum length is decreased and that this alteration may be a risk factor for its development

Objetivo - Estudar, através do ecocardiograma bidimensional, aspectos morfológicos dos septos atrial, atrioventricular e ventricular, potencialmente envolvidos na gênese da estenose subaórtica fixa. Métodos - Foram avaliadas 172 crianças divididas em três grupos: 43 casos com estenose subaórtica fixa, 86 controles normais e 43 controles cardiopatas. Foram estudadas a distância mitro-aórtica, a distância mitrotricuspídea e o índice de desalinhamento dos septos atriale ventricular. Resultados - Os grupos não diferiram quanto a idade, o sexo e a superfície corpórea. A média da distância mitro-aórtica mostrou-se aumentada no grupo de casos em relação aos controles. As médias da distância mitrotricuspídea e do índice de desalinhamento mostraram-se diminuídas no grupo de casos em comparação com os grupos controles. Houve correlação entre as variáveis, que mostraram razão de chances significativamente altas para o desenvolvimento da doença. Conclusão - Concluiu-se que, na estenose subaórtica fixa, a separação mitro-aórtica encontra-se aumentada e o septo atrioventricular diminuído e que estas alterações podem constituir risco para o seu desenvolvimento

A case of discrete subaortic stenosis

Discrete subaortic stenos is one of the subtype of congenital left ventricular outflow obstruction and frequently associated with other defect such as ventricular septal defect, coarctation of the aorta, inturrupted aortic arch, double-chambered right ventricle and atrioventricular canal. It is progressive disease from the neonatal period and can be a cause of secondary endocarditis or aortic regurgitation. The authors have experienced a case of discrete subaortic stenosis without other associated lesions in 8 years old male patient who has complained chest discomfort. The diagnosis was established by the findings of echocardiography and cardiac catheterization. We report this case with a brief review of related literatures.