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.

Alterações congênitas do coração e dos grandes vasos em cães / Congenital abnormalities of the heart and large vessels of dogs

As alterações congênitas do coração e dos grandes vasos (ACCGV) são definidas como defeitos morfológicos associados ao nascimento e são as anomalias congênitas mais frequentes dos animais domésticos. O objetivo deste trabalho foi determinar a frequência de diagnósticos de ACCGV em cães no Rio Grande do Sul, com ênfase na Região Metropolitana de Porto Alegre, no período de janeiro de 2000 a dezembro de 2016. Do total de 7.903 necropsias de cães, 27 morreram espontaneamente ou foram submetidos à eutanásia devido às ACCGV, representando 0,3% dos casos. Em 11,1% dos cães apresentaram dois distúrbios congênitos no coração ou nos grandes vasos, totalizando 30 alterações. A idade variou de um dia a 12 anos, com a idade mediana de quatro meses. Em 81,5% acometeu cães com raça, e 18,5% sem raça definida. Em relação ao sexo, 51,8% dos cães eram machos e 48,2%, fêmeas. Estenose subaórtica foi a alteração mais frequente, seguido por defeito do septo atrial, persistência do arco aórtico direito, persistência do ducto arterioso, estenose pulmonar e defeito do septo interventricular, e fibroelastose endocárdica. Dos casos múltiplos, as combinações encontradas foram: persistência do ducto arterioso associado com defeito do septo atrial, estenose subaórtica com defeito do septo interventricular, e defeito do septo atrial e ventricular.(AU)

Congenital alterations of the heart and large vessels (CAHLV) are defined as morphological defects associated with birth and are the most frequent congenital anomalies of domestic animals. The aim of this study was to determine the frequency of CAHLV in dogs in Rio Grande do Sul, with emphasis in the Metropolitan Region of Porto Alegre, from January 2000 to December 2016. Of the 7,903 necropsied dogs, 27 died spontaneously or were submitted to euthanasia due to CAHLV, representing 0.3% of the cases. In 11.1% of the dogs, there were two congenital disorders in the heart or in the great vessels, totaling 30 changes. The age of affected dogs ranged from one day to 12 years, with the median age of four months. In 81.5% it affected dogs with breed, and 18.5%, without breed defined. In 51.8% were males, and 48.2%, females. Subaortic stenosis was the most frequent alteration, followed by atrial septal defect, persistent right aortic arch, patent ductus arteriosus, pulmonic stenosis, ventricular septal defect, and endocardial fibroelastosis. Of the multiple cases, the combinations found were: patent ductus arteriosus associated with atrial septal defect, subaortic stenosis with ventricular septal defect, and atrial and ventricular septal defect.(AU)

Value of morrow procedure in surgery of discrete membranous subaortic stenosis

Subuortic stenosis constitutes up to 20% of all farms of left ventricular outflow had [LVOT] obstruction in children. Although the resulting significant left ventricular hypertrophy and aortic insufficiency are clear indications for surgery, controversy persists whether to remove the membrane in symptom-free patients with an isolated membrane or not. In addition to the timing of surgical repair, the surgical technique remains an area of discussion. In this study, we try to evaluate the impact of early surgical interference as well as surgical technique on the early and mid-term outcome. 44 patients with a mean age of 9.26 years [range, 2.4-28 years] underwent surgical resection for discrete membranous subaortic stenosis [DMSS]. For the purpose of the study, the patients were divided into two groups. Group I consisted of 31 patients who underwent membranectomy combined with routine transoortic myectomy. Group II consisted 13 patients to whom only excision of the subaortic membrane was done [membranectomy] without any myotomy or myectomy. Group II was done in Assuit University Hospital between 2001 and 2003. Group I was done in Paediatric cardiothoracic surgery unit [Children Hospital. Assuit University] between 2009 and 2013, in early postoperative results, the mean LVOT gradient in group I was statistically significantly lower than that of group II [p=0.000]. Aortic regurge [AR] improved in all patients of group I but progressed in five patients of group H. Mid-term follow up showed better results in group I than group II early resection of DMSS, before development of significant left ventricular hypertrophy and aortic insufficiency is advisable. Resection of the obstructing membrane together with generous myectomy of the LVOT is a safe and effective treatment in terms of more significant reduction of LVOT gradient; both early and in mid-term follow up, with lower incidence of recurrence and significant improvement of associated aortic incompetence

Outcome of surgery for sinus of Valsalva aneurysm with discrete membranous subaortic stenosis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly, and SVA with discrete membranous subaortic stenosis is even rarer. The aim of the study was to make sure the incidence of SVA with discrete membraneous subaortic stenosis in SVA and their surgical results. We retrospectively analyzed 234 patients receiving surgical repair of SVA and reported the incidence of ventricular septal defect, aortic regurgitation, and discrete membranous subaortic stenosis. We also reported seven cases of SVA combined with discrete membranous subaortic stenosis and their surgical results.</p><p><b>METHODS</b>Between January 1999 and December 2009, seven patients of SVA with discrete membranous subaortic stenosis underwent surgical repair of SVA and resection of subaortic discrete membrane. There were six male and one female patients. The mean age was (33.71 ± 13.25) years (range 16 - 52 years). Associated cardiovascular lesions were aortic regurgitation (n = 7), ventricular septal defect (n = 5), coarctation of aorta (n = 1), bicuspid aortic valve (n = 1), patent ductus arteriosus (n = 1), and aortic valve stenosis (n = 1). The aortic valve was replaced in four patients and valvuloplasty was done in three. The other co-existing anomalies were corrected at the same time. All the seven patients were followed up from 18 to 125 months (mean (63.14 ± 39.54) months). Among 234 SVA patients who underwent surgical repair, the number of cases with coexisting ventricular septal defect, aortic regurgitation, and discrete membranous subaortic stenosis was 129, 108, and 7, respectively.</p><p><b>RESULTS</b>There was neither early death after operation nor late death during the follow-up period. All the seven patients were in the New York Heart Association (NYHA) functional classes I and II. There was no recurrence of discrete subaortic membrane during the follow-up period. The incidence of ventricular septal defect, aortic valve incompetence, and discrete membranous subaortic stenosis among 234 SVA patients was 55.13%, 46.15%, and 2.99%, respectively.</p><p><b>CONCLUSIONS</b>Surgical repair of SVA with discrete membranous subaortic stenosis showed good mid-term results. Resection of discrete subaortic membrane should be done actively while repairing SVAs. Long-term results need to be followed up.</p>

Subaortic Membrane Late after Surgical Correction of Tetralogy of Fallot

We herein report a rare case of subaortic stenosis in association with a previous tetralogy of Fallot (TOF) surgical repair, which was not taken into account as a differential diagnosis. Echocardiography plays a pivotal role in identification of this rare combination. Therefore, echocardiography should be performed periodically during follow-up of patients with surgically corrected TOF. Given the clinical complications that can result from subaortic stenosis (i.e., aortic regurgitation and infective endocarditis), early and aggressive management of this rare combination should be performed.

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.

Comportamiento in vitro de un anillo mitral ajustable para cirugía de reparación mitral / In vitro behavior of an adjustable mitral ring for mitral repair surgery

Introducción. La reducción anular mitral es una de las técnicas quirúrgicas utilizadas en la reparación de la insuficiencia mitral funcional. Para su realización, se implanta un anillo protésico restrictivo para lograr la coaptación de los velos mitrales. Los anillos protésicos disponibles tienen tamaño fijo y existe el riesgo de implantar un anillo de tamaño inadecuado que resulte en una reparación imperfecta. Además algunos pacientes con disfunción ventricular severa no toleran la corrección de la insuficiencia mitral. Por esto, hemos diseñado un nuevo anillo regulable desde un dispositivo extracardíaco que podría resolver algunas deficiencias actuales en el tratamiento de dicha afección. Objetivo. Evaluar el comportamiento in vitro del anillo mitral regulable en cuanto a su capacidad de modificar sus dimensiones, corregir la insuficiencia mitral y producir voluntariamente nuevamente la insuficiencia. Material y métodos. Se utilizó un modelo de corazón porcino aislado al que se produjo insuficiencia mitral por dilatación anular. Se implantó el anillo regulable y se realizaron maniobras de reducción y dilatación observando su comportamiento y su efecto sobre la insuficiencia valvular. Resultados. Las maniobras de reducción y dilatación anular permitieron variar las dimensiones protésicas proporcionalmente sin modificar la forma anular. Se logró corregir y reproducir la insuficiencia mitral desde el control extracardíaco. Conclusión. In vitro el anillo regulable es capaz de modificar sus dimensiones proporcionalmente con el fin de corregir o reproducir la insuficiencia mitral

Background. Mitral ring downsizing is one of the techniques used in ischemic mitral regurgitation (MR) and MR due to dilated cardiopathy. In this technique, a prosthetic ring smaller than the native one is implanted in order to achieve mitral leaflet coaptation. Mitral ring prosthesis available up to date have fixed sizes and there is a risk that the chosen size turns out to be inadequate at follow-up. Some patients with severe ventricular dysfunction don’t tolerate mitral valve repair and the increase in afterload that accompanies it. In order to overcome these difficulties, we designed a new mitral ring adjustable through an extra-cardiac device and tested it in vitro. Methods. Mitral regurgitation was obtained in a porcine isolated heart after forced dilatation of the mitral annulus. The mitral ring prototype was sutured to the mitral annulus using the conventional technique. Using the extra-cardiac device, the mitral annulus could be reduced and dilated obtaining annular diameters from 24 to 32 mm. Results. Proportional modification of annulus dimensions maintaining the characteristic shape of the mitral ring and its proportions was achieved. Mitral regurgitation was corrected and the optimal annulus dimension was determined after various dilatation and reduction maneuvers. Conclusions. Through this isolated porcine heart model of MR, we demonstrated that our prototype can reduce and dilate the mitral annulus proportionately without changing the native mitral annulus shape through the use of an extra-cardiac device

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.

Estenose subaortica: revisão e avaliação da casuística do Instituto Dante Pazzanese / Subaortic stenosis: review and evaluation of the sample of Dante Pazzanese Institute

A obstrução a ejeção ventricular pode ocorrer em vários níveis ao longo do segmento compreendido entre a cavidade ventricular esquerda e a aorta

Clinical Analysis of Surgical Results for Discrete Subaortic Stenosis / 대한흉부외과학회지

BACKGROUND: Discrete subaortic stenosis is known to recur frequently even after surgical resection. We retrospectively reviewed the preoperative and postoperative changes in pressure gradient through left ventricular outflow tract, and the recurrence rate. MATERIAL AND METHOD: Between September 1984 and December 2004, 34 patients underwent surgical treatment. Mean age of patients was 17.1+/-15.2 years and 19 patients (55.9%) were male. 16 patients (47.1%) had previous operations and associated diseases were aortic regurgitation (11), coarctation of aorta (3), and others. RESULT: Immediate postoperative peak pressure gradient was significantly lower than preoperative peak pressure gradient (21.8 mmHg vs 75.8 mmHg, p<0.01). Peak pressure gradient measured after 50.3 months of follow up was 20.2 mmHg, which was also significantly lower than that of preoperative value but not significantly different from that of immediate postoperative value. There was no surgical mortality but one patient developed cerebral infarction. Mean follow up duration was 69.8+/-54.6 months. During this period, 5 patients (14.7%) had reoperation, 3 (8.8%) of whom were due to recurred subaortic stenosis. We found no risk factors for recurrence and survival for free from reoperation was 76.4%. CONCLUSION: Excision of subaortic membrane combined with or without myectomy in discrete subaortic stenosis showed sufficient relief of left ventricular outflow tract obstruction with low mortality and morbidity, but careful long term follow up is necessary for recurrence, since it is not predictable.

Clinical Observation on Congenital Heart Disease in Adult

BACKGROUND: With the advance of the techniques of echocardiography and cardiovascular surgery, early detection and successful cardiovascular surgery of congenital heart disease is possible in infant as well as in child. And with the advance of the social insurance, the new case of adult congenital heart disease with mild cardiovascular symptom or frank symptom of the pulmonary hypertension is decreasing. We statistically analyze the new case of adult congenital heart disease. METHOD: 92 patients who were diagnosed to congenital heart disease by echocardiography from January 1993 to June 1998 were studied. 2.25 MHz probe for two-dimensional and Doppler echocardiography and biplane 5 MHz phased-array probe for transesophageal echocardiography(Ultramark-9) were used. RESULT: Among 92 patients, 45 patients(48.9%) were male and 47 patients(51.1%) were female and 6 patients(male : 2, female : 4) had multiple congenital heart disease. 61 patients(66.3%) had no definitive cardiovascular symptom and right bundle branch block was most common electrocardiographic abnormality. 32 cases(32.8%) were atrial septal defect, 21 cases(21.5%) ventricular septal defect, 12 cases(12.3%) patent ductus arteriosus, 8 cases(8.2%) congenial bicuspid aortic valve and so on. Female predominance was noted in ventricular septal defect and endocardial septal defect, while male predominance in bicuspid aortic valve and discrete subaortic stenosis. Atrial septal defect and patent ductus arteriosus were no sex difference. 23 cases(25.0%) were between 20 29, 17 cases(18.5%) between 15-19, and 2 cases(2.2%) over 70 years old. CONCLUSION: Our analysis shows similarity to previous report. With advance of the technique of echocardiography and cardiovascular surgery, a few new case of adult congenital heart disease can be diagnosed hereafter.

Multiplane Transesophageal Echocardiographic Findings of Two Cases of Discrete Subvalvular Aortic Stenosis

Discrete subvalvular aortic stenosis is a relatively uncommon cause of the left ventricular outflow obstruction, requiring early intervention. Conventional transthoracic echocardiography may fail in some patients due to insufficient imaging quality. In particular, in patients with a discrete fibrous membrane close to the aortic valve without narrowing of the left ventricular outflow tract, the echocardiographic detection of the membrane may be difficult. Transesophageal echocardiography allows a clear visualization of the aortic valve and the left ventricular outflow tract in virtually all patients, it can be performed rapidly with almost no risk, and it may therfore be helpful in establishing the diagnosis of discrete subaortic stenosis, in particular in patients where the conventional transthoracic approach fails. We have experienced two cases of discrete subaortic stenosis. One case of them was combined with hypertrophic obstructive cardiomyopathy in this report we discussed the utility of multiplane transesophageal echocardiography in patients with discrete subvalvular aortic stenosis.

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.

A Case of Combined Double Chambered Right Ventricle and Discrete Subaortic Stenosis

Combined double chambered right ventricle(DCRV) and discrete subaortic stenosis(DSAS) is a rare entity on which only 12 cases have been reported in the literature. We presented a case of combined DCRV and DSAS in an 18 year old girl. She had type II(thin membranous type) DSAS and the peak systolic prssure gradient between aorta and left ventricle was 38 mmHg. Aberrant muscle bundle was found on the right ventriculography and the pressure gradient in the right ventricle was 35 mmHg. She also had aortic regurgitation, persistent left sided superior vena cava and extracardiac malformations such as kyphoscoliosis and congenital cloacal anomaly.