BACKGROUND: With an increasing awareness of the limitations of both mechanical prostheses and bioprostheses, aortic valvuloplasty has gained attention as an alternative procedure for aortic valve disease. MATERIAL AND METHOD: Eight consecutive patients underwent aortic valvuloplasty caused by leaflet prolapse between June 1999 to June 2000. Mean age of the patients was 18.4+/- 12.6 year. Four paitents(50%) were male. Six patients had tricuspid valves and ventricular septal defect and two patients had bicuspid valves. The extent of aortic insufficiency was 3.5+/- 0.5 by preoperative Doppler echocardiography. The technique involved triangular resection of the free edge of the prolapsed leaflet, annular plication at the commissure, and resection of a raphe when present in bicuspid valves. RESULT: There was no in-hospital mortality or morbidity. Mean follow-up was complete at 11.9+/- 3.6months. There was no late mortality or morbidity. The amount of the severity of aortic insufficiency, as assessed by echocardiography preoperatively, postoperatively and at late follow-up was 3.5+/- 0.5, 0.6+/- 0.5 and 0.8+/- 0.6, respectively(p value = 0.01). There was one patient with grade 2/4 aortic insufficiency and in the other patients, grade 1/2 or trivial aortic insufficiency were detected with late echocardiograms. CONCLUSION: Triangular resection in the patients with aortic leaflet prolapse offers a good early clinical result, but long-term follow-up is necessary.
Aortic Valve ; Bioprosthesis ; Echocardiography ; Echocardiography, Doppler ; Follow-Up Studies ; Heart Septal Defects, Ventricular ; Hospital Mortality ; Humans ; Male ; Mitral Valve ; Mortality ; Prolapse ; Prostheses and Implants ; Tricuspid Valve

Twenty-one cases of mitral valve prolapse (MVP) diagnosed by M-mode echocardiograms were studied in regard to the underlying or associated conditions, types of MVP, and dimensions of the cardiac structures and parameters reflecting cardiac performance determined by echocardiograms. In 9 cases, MVP was thought to be idiopathic origin. Associated conditions in the remainder were 5 cases of congenital heart disease, 2 cases of rheumatic myocarditis, 1 case each of aortic valve disease, ischemic heart disease and hyperthyroidism, and 2 cases of undiagnosed cardiac conditions. There were 2 cases of buckling type of MVP and in the remainder hammock type. In these 2 patients with hammock type of MVP, mitral valve echoes were seen in the left atrium during systole in 1 case, and diastolic anterior displacement of posterior mitral leaflet in the other. In 9 cases of idiopathic MVP, cardiac dimensions and parameters reflecting cardiac performance as determined from echocardiograms showed no significant differences from those of 27 normal controls.
Aortic Valve ; Echocardiography* ; Heart Atria ; Heart Defects, Congenital ; Humans ; Hyperthyroidism ; Mitral Valve Prolapse* ; Mitral Valve* ; Myocardial Ischemia ; Myocarditis ; Systole

We experience a case of 34-year-old Turner's syndrome(45, XO) associated with atrial septal defect(secondum type) and mitral valve prolapse who was admitten due to moderate exertional dyspnea. It is well know hat chromosomal abnormality is one of the etiology of congenital heart disease. In case of Turner's syndrome, coarctation of aorta or bocuspid aortic valve is frequently combined, but atrial septal defect simultaneously with mitral valve prolapse is not reported till now in Krea. We report this case with a brief review of the literature.
Adult ; Aortic Coarctation ; Aortic Valve ; Chromosome Aberrations ; Dyspnea ; Heart Defects, Congenital ; Heart Septal Defects, Atrial* ; Humans ; Mitral Valve Prolapse* ; Mitral Valve* ; Turner Syndrome*

BACKGROUND: Surgery for mitral valve disease in children carries both technical and clinical difficulties that are due to both the wide spectrum of morphologic abnormalities and the high incidence of associated cardiac anomalies. The purpose of this study is to assess the outcome of mitral valve surgery for treating congenital mitral regurgitation in children. MATERIAL AND METHOD: From 1997 to 2007, 22 children (mean age: 5.4 years) who had congenital mitral regurgitation underwent mitral valve repair. The median age of the patients was 5.4 years old and four patients (18%) were under 12 months of age. 15 patients (68%) had cardiac anomalies. There were 13 cases of ventricular septal defect, 1 case of atrial septal defect and 1 case of supravalvar aortic stenosis. The grade of the preoperative mitral valve regurgitation was II in 4 patients, III in 15 patients and IV in 3. The regurgitation was due to leaflet prolapse in 12 patients, annular dilatation in 4 patients and restrictive leaflet motion in 5 patients. The preoperative MV Z-value and the regurgitation grade were compared with those obtained at follow-up. RESULT: MV repair was possible in all the patients. 19 patients required reduction annuloplasty and 18 patients required valvuloplasty that included shortening of the chordae, papillary muscle splitting, artificial chordae insertion and cleft closure. There were no early or late deaths. The mitral valve regurgitation after surgery was improved in all patients (absent=10, grade I=5, II=5, III=2). MV repair resulted in reduction of the mitral valve Z-value (2.2+/-.1 vs. 0.7+/-.3, respectively, p<0.01). During the mid-term follow-up period of 3.68 years, reoperation was done in three patients (one with repair and two with replacement) and three patients showed mild progression of their mitral regurgitation. CONCLUSION: Our experience indicates that mitral valve repair in children with congenital mitral valve regurgitation is an effective and reliable surgical method with a low reoperation rate. A good postoperative outcome can be obtained by preoperatively recognizing the intrinsic mitral valve pathophysiology detected on echocardiography and with the well-designed, aggressive application of the various reconstruction techniques.
Aortic Stenosis, Supravalvular ; Child ; Dilatation ; Echocardiography ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Mitral Valve ; Mitral Valve Insufficiency ; Papillary Muscles ; Prolapse ; Reoperation

BACKGROUND: Intracardiac pathology resulting in embloic phenomena is a well-recognized cause of cerebral ischemia and infarction. Recently, the use of transesophageal echocardiography(TEE) has gained wide acceptance because of its superior resolution of basal structures such as the left atrium, left atrial appendage, mitral valvular apparatus, atrial septum, and aorta. The purposes of this study are to evaluate the effectiveness of TEE for detection of intracardiac source of cerebral emboli. METHOD: From 1991 to 1995, 215 patients were included in this study. All patients underwent both transthoracic and transesophageal imaging with saline contrast administration and Doppler color flow imaging. The study group consisted of 132 men and 83 women with a mean age of 51 years(range 15-74). We also reviewed TEE result of all patients according to two groups, which were divided by the presence of clinical cardiac abnormalities. RESULT: 1) TEE identified a potential cardiac source of embolism in 43.7%(94 to 215) of the overall study group compared with only 3.7%(8 to 215) by TTE. 2) Success rate of TEE was 93.5%(201 to 215). 3) Abnormalities noted by TEE included 23 patients with LA spontaneous echo contrast, 20 patients with patient foramen ovale, 16 patients with aortic atheroma, 16 patients with LA thrombi, 8 patients with atrial septal aneurysm, 5 patients with aortic valve prolapse, 4 patients with mitral valve prolapse, and 2 patients with LV thrombi. 4) In the 62 patients with cardiac disease, TEE identified 16 patients with LA spontaneous echo contrast, 12 patients with LA thrombi, 2 patients with patent foramen ovale, 1 patient with aortic atheroma, 1 patient with atrial septal aneurysm and 1 patients with aortic valve prolapse. In the 152 patients with no cardiac disease, TEE identified 18 patients with patent foramen ovale, 15 patients with aortic atheroma, 7 patients with artial septal aneurysm, 7 patients with spontaneous echo contrast, 4 patients with aortic valve prolapse, 1 patient with mitral valve prolapse and 1 patient LA thrombi. CONCLUSION: TEE was very useful method in investigating potential intracardiac source of cerebral emboli. Thus, the use TEE combined with TTE in patients with unexplained stroke should be recommended.
Aneurysm ; Aorta ; Aortic Valve Prolapse ; Atrial Appendage ; Atrial Septum ; Brain Ischemia ; Echocardiography, Transesophageal ; Embolism ; Female ; Foramen Ovale ; Foramen Ovale, Patent ; Heart Atria ; Heart Diseases ; Humans ; Infarction ; Intracranial Embolism ; Male ; Methods ; Mitral Valve Prolapse ; Pathology ; Plaque, Atherosclerotic ; Stroke

We descrive a 23-year-old female of 46, XXqi Turner's syndrome associated with large atrial sepatal defect(secundum type) and mitral valve prolapse who was admitted due to amenorrhea, sexual infantilism and exertional dyspnea. This patient had only one spontaneous menstrual period at the age of 15 and had a short stature without webbed neck. Chromosomal aberrations cause primarily structural defects of cardiovasculaqr system, and a variety of structural aberrations involving the X chromosome and cause partial or complete Turner's syndrome. In Turner's syndrome, bicuspid aortic valve or coarctaton of aorta is frequently combined, also aortic root dilatation, partial anomalous venous drainage, hypoplastic left heart and ventricular septal defect, atrial septal defect has been reported. However, this patient had not abnormalities in aortic valve and whole aorta. Atrial septal defect simultaneously with mitral valve prolapse in 46 XXqi Turner's syndrome have not been reported in Korea. We report this case with a brief review of the literature.
Amenorrhea ; Aorta ; Aortic Valve ; Bicuspid ; Chromosome Aberrations ; Dilatation ; Drainage ; Dyspnea ; Female ; Heart ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Korea ; Mitral Valve Prolapse ; Mitral Valve ; Neck ; Sexual Infantilism ; Turner Syndrome ; X Chromosome ; Young Adult

STUDY DESIGN: Retrospective study. PURPOSE: To identify the incidence of congenital cardiac abnormalities in patients who had scoliosis and underwent surgical treatment for scoliosis. OVERVIEW OF LITERATURE: Congenital and idiopathic scoliosis (IS) are associated with cardiac abnormalities. We sought to establish and compare the incidence of congenital cardiac abnormalities in patients with idiopathic and congenital scoliosis (CS) who underwent surgical treatment for scoliosis. METHODS: Ninety consecutive scoliosis patients, who underwent surgical correction of scoliosis, were classified as CS (55 patients, 28 female [51%]) and IS (35 patients, 21 female [60%]). The complete data of the patients, including medical records, plain radiograph and transthoracic echocardiography were retrospectively assessed. RESULTS: We found that mitral valve prolapse was the most common cardiac abnormality in both patients with IS (nine patients, 26%) and CS (13 patients, 24%). Other congenital cardiac abnormalities were atrial septal aneurysm (23% of IS patients, 18% of CS patients), pulmonary insufficiency (20% of IS patients, 4% of CS patients), aortic insufficiency (17% of IS patients), atrial septal defect (11% of IS patients, 13% of CS patients), patent foramen ovale (15% of CS patients), dextrocardia (4% of CS patients), bicuspid aortic valve (3% of IS patients), aortic stenosis (2% of CS patients), ventricular septal defect (2% of CS patients), and cardiomyopathy (2% of CS patients). CONCLUSIONS: We determined the increased incidence of congenital cardiac abnormalities among patients with congenital and IS. Mitral valve prolapse appeared to be the most prevalent congenital cardiac abnormality in both groups.
Aneurysm ; Aortic Valve ; Aortic Valve Stenosis ; Bicuspid ; Cardiomyopathies ; Dextrocardia ; Echocardiography ; Female ; Foramen Ovale, Patent ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Medical Records ; Mitral Valve Prolapse ; Retrospective Studies* ; Scoliosis*

STUDY DESIGN: Retrospective study. PURPOSE: To identify the incidence of congenital cardiac abnormalities in patients who had scoliosis and underwent surgical treatment for scoliosis. OVERVIEW OF LITERATURE: Congenital and idiopathic scoliosis (IS) are associated with cardiac abnormalities. We sought to establish and compare the incidence of congenital cardiac abnormalities in patients with idiopathic and congenital scoliosis (CS) who underwent surgical treatment for scoliosis. METHODS: Ninety consecutive scoliosis patients, who underwent surgical correction of scoliosis, were classified as CS (55 patients, 28 female [51%]) and IS (35 patients, 21 female [60%]). The complete data of the patients, including medical records, plain radiograph and transthoracic echocardiography were retrospectively assessed. RESULTS: We found that mitral valve prolapse was the most common cardiac abnormality in both patients with IS (nine patients, 26%) and CS (13 patients, 24%). Other congenital cardiac abnormalities were atrial septal aneurysm (23% of IS patients, 18% of CS patients), pulmonary insufficiency (20% of IS patients, 4% of CS patients), aortic insufficiency (17% of IS patients), atrial septal defect (11% of IS patients, 13% of CS patients), patent foramen ovale (15% of CS patients), dextrocardia (4% of CS patients), bicuspid aortic valve (3% of IS patients), aortic stenosis (2% of CS patients), ventricular septal defect (2% of CS patients), and cardiomyopathy (2% of CS patients). CONCLUSIONS: We determined the increased incidence of congenital cardiac abnormalities among patients with congenital and IS. Mitral valve prolapse appeared to be the most prevalent congenital cardiac abnormality in both groups.
Aneurysm ; Aortic Valve ; Aortic Valve Stenosis ; Bicuspid ; Cardiomyopathies ; Dextrocardia ; Echocardiography ; Female ; Foramen Ovale, Patent ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Medical Records ; Mitral Valve Prolapse ; Retrospective Studies* ; Scoliosis*

Axenfeld-Rieger syndrome is a rare autosomal dominant inherited developmental disorder characterized by ocular and systemic abnormalities. In ocular anomaly, it includes a prominent and anteriorly displaced Schwalbe line and an iridocorneal synechiae, iris hypoplasia, corectopia and secondary glaucoma. Extraocular developmental abnormalities of the syndrome are hearing loss, congenital heart disease, dental anomalies, developmental delay, and a characteristic facial appearance. We herein reported a familial case of Axenfeld-Rieger syndrome that had a mitral valve prolapse with severe mitral regurgitation finally requiring mitral valve replacement.
Glaucoma ; Hearing Loss ; Heart Defects, Congenital ; Iris ; Mitral Valve ; Mitral Valve Insufficiency* ; Mitral Valve Prolapse

Authors reviewed and analyzed 108 cases of secundum ASD(1–15 years old), which had been diagnosed bycineangiography in Seoul Natinal University Children's Hospital from 1979 to 1985. 1) The incidence of theasociation of MVP with secundum ASD was 48%(Male 43%, Female 52%). 2) The incidnece of this association incresedwith age(1–5 years old: 39%, 6–10 : 50%, 11–15: 59%). 3) The incidence of concurrent mitral regurgitation in MVPassociated with ASD was 48%. No special correlation was not found between the degree or incidence of MVP and thatof MR. 4) The incedence of the association of MVP with ASD increased with the increaseing amount of leftventricular geomatry attendant on left to right shunt (QS/QS < 2:33%, 2<=QP/QS < 4:52%, QP/QS >= 4: 100%). Thissuggested that abnormal left ventricular geometry attendant on left to ring shunt would contribute much to theassociation of MVP with secundum ASD.
Female ; Heart Septal Defects, Atrial ; Humans ; Incidence ; Mitral Valve Insufficiency ; Mitral Valve Prolapse ; Mitral Valve ; Seoul