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.
Adolescent ; Aorta ; Aortic Valve Insufficiency ; Discrete Subaortic Stenosis* ; Female ; Heart Ventricles* ; Humans ; Vena Cava, Superior

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.
Constriction, Pathologic ; Discrete Subaortic Stenosis ; Echocardiography ; Heart ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular ; Humans ; Membranes ; Thoracic Surgery

BACKGROUNDSinus 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.

METHODSBetween 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.

RESULTSThere 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.

CONCLUSIONSSurgical 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.

Adolescent ; Adult ; Discrete Subaortic Stenosis ; pathology ; surgery ; Female ; Humans ; Male ; Middle Aged ; Sinus of Valsalva ; pathology ; surgery ; Treatment Outcome ; Young Adult

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.
Aortic Coarctation ; Aortic Stenosis, Subvalvular ; Aortic Valve Insufficiency ; Cerebral Infarction ; Constriction, Pathologic ; Discrete Subaortic Stenosis* ; Follow-Up Studies ; Humans ; Male ; Membranes ; Mortality ; Recurrence ; Reoperation ; Retrospective Studies ; Risk Factors

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.
Aortic Stenosis, Subvalvular* ; Aortic Valve ; Cardiomyopathy, Hypertrophic ; Diagnosis ; Discrete Subaortic Stenosis ; Early Intervention (Education) ; Echocardiography* ; Echocardiography, Transesophageal ; Humans ; Membranes ; Ventricular Outflow Obstruction

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.
Adult ; Aortic Valve Insufficiency/etiology ; Discrete Subaortic Stenosis/*complications/surgery/ultrasonography ; Echocardiography ; Female ; Heart Defects, Congenital/*complications/surgery/ultrasonography ; Humans ; Tetralogy of Fallot/*complications/surgery/ultrasonography ; Time Factors

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.
Adult* ; Aged ; Aortic Valve ; Bicuspid ; Bundle-Branch Block ; Child ; Discrete Subaortic Stenosis ; Ductus Arteriosus, Patent ; Echocardiography ; Echocardiography, Doppler ; Electrocardiography ; Female ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Hypertension, Pulmonary ; Infant ; Male ; Sex Characteristics ; Social Security

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.
Aorta, Thoracic ; Aortic Coarctation ; Aortic Valve Insufficiency ; Cardiac Catheterization ; Cardiac Catheters ; Child ; Diagnosis ; Discrete Subaortic Stenosis* ; Echocardiography ; Endocarditis ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Humans ; Male ; Thorax ; Ventricular Outflow Obstruction