• Title/Summary/Keyword: Heart defects

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Postoperative Assessment of Residual Oefects Following Surgical Closure of Ventricular Septal Defects (심실중격결손의 개심술 후 잔류단락에 관한 임상적 고찰)

  • 조준용;허동명
    • Journal of Chest Surgery
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    • v.29 no.2
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    • pp.147-152
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    • 1996
  • his study was undertaken to assess the residual interventricular shunt following surgical closure of the isolated ventricular septal defect. From January 1989 through December 1993, 211 patients underwent surgical closure of the isolated ventricular septal defect. All patients had 2D-Echocardlo-graphic study after operation to rule out residual ventricular septal defect. There was a 9.5% incidence of a definite residual shunt. The type of ventricular septal defect, closure method of the defect and cardiopulmonary bypass time showed no significant differences between two groups. The sue of ventricular septal defect (6.3 $\pm$ 3.7mm versus 10.6 $\pm$ 5.8mm : p : 0.0034), aortic cross-clamping time(32.6 $\pm$ 15.0 minutes versus 48.5 $\pm$ 20.0 minutes, p : 0.0003), pulmonary-to-systemic pressure ratio(0.31 $\pm$ 0.22 versus 0.51 $\pm$ 0.33, p=0.019) and mean pulmonary artery pressure(20.3 $\pm$ 11.9 mmHg versus 29.1 $\pm$ 16.2 mmHg, p : 0.009) were meaningfully different between two groups. There were 9 insta ces of spontaneous closure of the residual shunts at mean 21 months of following up (ranged 1 ~43 months). In conclusion, we suggest that the size of ventricular septal defect, aortic cross-clamping time and mean pulmonary artery pressure may play an important role in occurance of residual ventricular septal defect.

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Phenotypic and Cytogenetic Delineation of Six Korean Children with Kabuki Syndrome (한국인 Kabuki 증후군 환아들의 임상적 표현형 및 세포유전학적 양상)

  • Ko, Jung-Min;Hwang, Jeong-Min;Kim, Hyon-Ju
    • Journal of Genetic Medicine
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    • v.7 no.1
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    • pp.37-44
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    • 2010
  • Purpose : Kabuki syndrome is a multiple congenital malformation syndrome with mental retardation. It was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. To date, six Korean cases of Kabuki syndrome have ever been reported. Here, we present the phenotypic and genetic characteristics of six patients with Kabuki syndrome. Materials and Methods : Between 2003 and 2009, six Korean girls have been diagnosed and followed up as Kabuki syndrome at Center for Genetic Diseases of Ajou University Hospital. Their clinical and laboratory data were collected and analyzed by the retrospective review of medical records. Results : All six patients showed the characteristic facial dysmorphism and developmental delay. Persistent fingertip pads were also found in all patients. Most patients showed postnatal growth retardation (83.3%) and hypotonia (83.3%). Opthalmologic problems were common, particularly for strabismus (83.3%). Congenital heart defects were present in three patients (50%). Skeletal abnormalities including 5th finger shortening (83.3%), clinodactyly (50%), joint hypermobility (50%) and hip dislocation (16.7%) were also observed. There was no patient who had positive family history for Kabuki syndrome. Cytogenetic and molecular cytogenetic analyses including karyotyping and array CGH could not reveal any underlying genetic cause of Kabuki syndrome. Conclusion : Korean patients with Kabuki syndrome showed a broad spectrum of clinical features affecting multiple organ systems. Although clinical manifestations of Kabuki syndrome have been well established, our results failed to detect recurrent chromosome aberrations which could cause Kabuki syndrome. Its natural history and genetic background remains to be further studied for providing appropriate management and genetic counseling.

Automated Functional Morphology Measurement Using Cardiac SPECT Images (SPECT 영상을 사용한 기능적 심근형태의 자동 계측법 개발)

  • Choi, Seok-Yoon;Ko, Seong-Jin;Kang, Se-Sik;Kim, Chang-Soo;Kim, Jung-Hoon
    • Journal of radiological science and technology
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    • v.35 no.2
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    • pp.133-139
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    • 2012
  • For the examination of nuclear medicine, myocardial scan is a good method to evaluate a hemodynamic importance of coronary heart disease. but, the automatized qualitative measurement is additionally necessary to improve the decoding efficiency. we suggests the creation of cardiac three-dimensional model and model of three-dimensional cardiac thickness as a new measurement. For the experiment, cardiac reduced cross section was obtained from SPECT. Next, the pre-process was performed and image segmentation was fulfilled by level set. for the modeling of left cardiac thickness, it was realized by applying difference equation of two-dimensional laplace equation. As the result of experiment, it was successful to measure internal wall and external wall and three-dimensional modeling was realized by coordinate. and, with laplace formula, it was successful to develop the thickness of cardiac wall. through the three-dimensional model, defects were observed easily and position of lesion was grasped rapidly by the revolution of model. The model which was developed as the support index of decoding will provide decoding information to doctor additionally and reduce the rate of false diagnosis as well as play a great role for diagnosing IHD early.

Single-Stage Repair of Coarctation of the Aorta and Ventricular Septal Defect in Infants Younger than 6 Months (생후 6개월 이하 환아에서 대동맥 축착증과 심실중격결손의 일차 완전교정)

  • 백만종;김웅한;이영탁;한재진;이창하
    • Journal of Chest Surgery
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    • v.34 no.10
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    • pp.733-744
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    • 2001
  • Background: The optimal therapeutic strategies for patients with coarctation of the aorta(CoA) and ventricular septal defect(VSD) remain controversial. This study was undertaken to determine the outcome and the need for reintervention following single-stage repair of coarctation with VSD in infants younger than 6 months. Material and Method: Thirty three consecutive patients who underwent single-stage repair of CoA with VSD, from January 1995 to December 2000, at Sejong General Hospital were reviewed retrospectively. Mean age and body weight at repair were 54$\pm$37 days(12 days-171 days) and 3.9$\pm$1.1 kg(1.5~6 kg), respectively. The surgical repair of CoA was performed under deep hypothermic circulatory arrest(CA) in the early period of the study and under regional cerebral perfusion through a direct innominate arterial cannulation without CA in the later period. The technique used in the repair of the CoA was resection and extended end-to-end anastomosis(EEEA; n=16) and extended side-to-side anastomosis(ESSA; n=2) in the early period, and resection and extended end-to-side anastomosis(EESA; n= 15) in the later period. The simultaneous closure of VSD was done with a Dacron patch(n= 16) and autologous pericardium(n=17). Aortic arch hypoplasia was present in 29 patients(88%) and its types were distal(n=18), complete(n=5), and complex(n=6)

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Application of Intraoperative Neurophysiological Monitoring in Aortic Surgery (대동맥수술에서의 수술 중 신경계감시의 적용)

  • Jang, Min Hwan;Chae, Ji Won;Lim, Sung Hyuk
    • Korean Journal of Clinical Laboratory Science
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    • v.54 no.1
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    • pp.61-67
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    • 2022
  • Intraoperative neurophysiological monitoring (INM) ensures the stability and safety of specific surgeries in high-risk groups. As part of INM, intensive tests are conducted during the surgical process. When INM tests are applied during surgery, a delay in notifying the operating surgeon in cases of neurological defects can cause serious irreversible sequelae to the patient. Aortic replacement, which is necessitated due to aortic aneurysms and aortic dissection, is a complicated procedure that blocks the blood flow to the heart. When arteries that branch out from the aorta and supply blood to the spinal cord are replaced, blood flow to the spinal cord decreases, resulting in spinal ischemia. In aortic surgery, INM plays an important role in preventing spinal ischemia and serious complications by quickly detecting the early signs of spinal ischemia during cross-clamping and reporting it to the surgeon. Therefore, this paper was prepared to help examiners who conduct INM by detailing the process, method, time, and warning criteria for INM. This paper identifies the need for INM in aortic surgery and the process flow for a smooth test, accurate and rapid examination, and subsequent reporting.

Relationship Between Adenosine-Induced ST Segment Depression During $^{99m}Tc$-MIBI Scintigraphy and The Severity of Coronary Artery Disease (Adenosine 부하 $^{99m}Tc$-MIBI 심근 관류스캔도중 나타나는 ST절 하강과 관상동맥 질환의 중증도와의 관계)

  • Cho, Jung-Ah;Choi, Chung-Il;Kwak, Dong-Suk;Kim, Jeong-Gyun;Bae, Sun-Kun;Chung, Byung-Cheon;Lee, Jae-Tae;Lee, Kyu-Bo;Kang, Seung-Wan;Woo, Eon-Jo;Kim, Sin-Woo;Sohn, Sang-Kyun;Chae, Shung-Chull
    • The Korean Journal of Nuclear Medicine
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    • v.28 no.2
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    • pp.177-185
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    • 1994
  • Pharmacologic coronary vasodilation in conjunction with myocardial perfusion scintigraphy has become an alternative to dynamic exercise test for the diagnosis and risk stratification of coronary artery disease, especially in patients who are unable to perform adequate exercise. Dipyridamole and adenosine have been used for pharmacologic stress testing with myocardial perfusion imaging. Adenosine is a potent coronary vasodilator with rapid onset of action, short half-life, near maximal coronary vasodilation and less serious side effects. ST segment depression has been reported in about 7-15% of patients with coronary artery disease receiving dipyridamole in conjunction with myocardial perfusion imaging. The exact cause and clinical significance are not known. In order to evaluate the relationship between adenosine-induced ST segment depression during $^{99m}Tc$-MIBI myocardial perfusion scintigraphy and the severity of coronary artery disease, we performed $^{99m}Tc$-MIBI imaging after intravenous Infusion of adenosine In 120 patients with suspected coronary artery disease. Of the 120 patients, 28 also performed coronary angiography. There were 24 patients with ST segment depression during $^{99m}Tc$-MIBI scintigraphy and 96 patients without ST segment depression. Adenosine was infused Intravenously at a dose of 0.14mg/kg per minute lot 6minutes and $^{99m}Tc$-MIBI was injected at 3 minute. We then com-pared the hemodynamic changes, side effects, scintigraphic and angiographic findings. Heart rate increased $90{\pm}19$ beats/minute in the group with ST depression compared with $80{\pm}16$ beats/minute in the group without ST depression(p<0.05). Baseline systolic blood pressure was significantly higher in the group with ST depression($152{\pm}27$ mmHg) than in the group without 57 depression($140{\pm}21$mmHg, p<0.05). Double product at baseline($10.90{\pm}2.77$ versus $9.55{\pm}2.34\;beats/minute{\times}mmHg$) and during adenosine infusion($12.72{\pm}3.89$ versus $10.83{\pm}2.98\;beats/minute{\times}mmHg$) were significantly higher in the group with ST depression(p<0.05). The incidence of anginal chest pain was also significantly higher in the group with ST depression(ST versus 29%, p<0.0001). The $^{99m}Tc$-MIBI images were abnormal in 23(96%) patients with ST segment depression and 66(69%) patients without ST segment depression(p<0.05). In patients with ST segment depression, there were more reversible perfusion defects than in patients without ST segment depression(83 versus 55%, p<0.05). The number of abnormal segments were significantly higher in the group with ST depression($3.05{\pm}2.01$ versus $1.51{\pm}1.45$, p<0.005). In patients with ST segment depression, there were more segments of reversible perfusion defects than in patients without segment depression($2.15{\pm}2.11$ versus $0.89{\pm}1.24$, p<0.05). There were no differences in the angiographic severity by vessel(p ; NS). We concluded that ST segment depression during $^{99m}Tc$-MIBI myocardial perfusion scintigraphy with Intravenous adenosine is related to the severity of coronary artery disease.

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Cost-Benefit Analysis on Rubella Vaccination Policy (풍진 예방접종사업의 비용-편익분석)

  • Shin, Young-Jeon;Choi, Bo-Youl;Park, Hung-Bae;Moon, Ok-Ryun;Yoon, Bae-Joong
    • Journal of Preventive Medicine and Public Health
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    • v.27 no.2 s.46
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    • pp.337-365
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    • 1994
  • Rubella is a viral disease with mild constitutional symptoms and generalized rashes. In childhood, it is an inconsequential illness, but when it occurs during early pregnant period, there are significant risks of heart defects, cataract, mental retardation to the fetus. The series of congenital defects induced by rubella is called 'congenital rubella syndrome'. Many research have been performed to find out more effective prevention program on rubella. The objectives of this study are, first, to calculate the incidence rate of acute rubella infection and congenital rubella syndrome in Korea, second, to evaluate economic efficiency of several rubella vaccination policies and to offer data for the most reasonable decision on vaccination policy. Study populations are 663,312 children of one year-old in 1992. The author has performed cost-benefit analyses according to the three vaccination policies-U.S.A.'s. U.K.'s and Sweden's. In this Study, the author got the incidence rate of acute rubella infection using the catalytic model. In the meantime, the author used 50 per 100,000 live births as the incidence rate of congenital rubella syndrome. The discount rate used in this study was 5 percent per annum. The sensitivity analyses were done with different discount rates (4%, 7%) and different incidence rate of congenital rubella syndrome (10,100 per 100,000 live births) : The study results are as follows: 1. Without vaccination, lifetime expenditures per patient for acute rubella infeciton amount to 14,822 won and the total expenditures to about 3.1 billion won. Meanwhile, lifetime expenditures per patient for congenital rubella syndrome amount to about 91 million won and the total expenditures to about 16.3 billion won without vaccination. 2. The cost of vaccination for a child of one year old was 2,322 won and the total cost for the one year old children was about 1.5 billion won (American style). The cost for vaccination of female children at fifteen was about 339 million won (Birtish style). And the cost of vaccination at one for both sex and female children at fifteen was about 1.9 billion won (Swedish style). 3. The benefit to cost ratios of vaccination of female children at fifteen that is the british mode of rubella vaccination, was 60.0 at the level of 80% population coverage and 48.6 at 100% coverage. It shows much higher benefit to cost ratio than those of the other two vaccination policies. 4. Both net benefits of vaccination at one (American style) and that of vaccinations at one and fifteen (Swedish style) range from about 17.0 billion to 17.8 billion won, those were larger than that of vaccinations of female children at fifteen (Birtish style, about 16.0 billion). 5. In marginal cost-benefit analysis of only additional program of revaccination, the benefit to cost ratios were 3.6 (80% coverage rate) or 0.6 (100% coverage rate). It implies that additional program was less efficient or inefficient. 6. In sensitivity analysis with different discount rates(4% or 7%) and different incidence rates of congenital rubella syndrome (10 or 100 per 100,000 live births), the benefit to cost ratios has fluctuated in wide range. However, all the ratios of vaccination of female children at fifteen were higher than those of the others. Even under the most conservative assumption, the benefit to cost ratios of all the rubella vaccination policies were higher than 3.3. In conclusion, all the rubella vaccination policies found to be cost-effective and particularly the vaccination of female children at fifteen was strongly recommended.

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Clinical Study of the Patients, in Whom Pulmonary Embolism was Suspected by Lung Perfusion Scan (폐 관류주사검사상 폐동맥 색전증 소견을 보인 환자의 임상적 고찰)

  • Lee, Gwi-Lae;Kim, Jae-Yeol;Park, Jae-Suk;Yoo, Chul-Gyu;Kim, Young-Whan;Shim, Young-Soo;Han, Sung-Koo
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.4
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    • pp.889-898
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    • 1997
  • Pulmonary Embolism can develop in variable conditions, and presents with nonspecific symptoms and signs. If diagnosis is delayed, it can be resulted in catastrophic results. Therefore, early diagnosis and adequate treatment is crucial in Pulmonary Embolism. Lung Perfusion Scan is useful screening test. Negative result can exclude pulmonary embolism. But, perfusion defects don't always mean pulmonary embolism. To find the better methods of interpretation of lung perfusion scan and To evaluate the clinical course and outcomes of the patients, in whom pulmonary embolism was suspected by lung perfusion scan, we reviewed the clinical records of 49 cases suspected by lung perfusion scan at Seoul National University Hospital during the period of January, 1995 to July, 1996. The results are as follows. First impression of cases in which PE was present at time of admission were pulmonary embolism (63%), heart diseases (26%), and pneumonia (11%) in orders. Underlying diseases of cases in which PE developed during admission were malignancy (36.5%), ICH (22.7%), sepsis (13.7%), and SLE (9.1%) in orders. The predisposing factors were operation (20%), cancer (16%), immobility (16%), connective tissue disease (16%), heart dis. (10%), old age (10%), and preg/pelvic dis. (8%) The results, of lung perfusion scan were HPPE 40 cases(26.8 %), IPPE 21 cases(14.1%), LPPE 88 cases (59.1%), and cases(%) of treatment in these cases were HPPE 34 cases(85%), IPPE 9 cases(42.9%), LPPE 0 case(0.0%). Treatments were heparin and warfarin (69.5%), heparin alone (8.2%), warfarin alone (2.0%), embolectomy (4.1%), thrombolytics (2.0%), IVC filter (2.0%), and no treatment (12.2%) In 34 cases (69.4%), follow up could be done, and 5 cases were recurred (10.2%). The causes of recurrence was incomplete anticoagulant therapy (3 cases) and recurrence of predisposing factor (2 cases). Expired case due to pulmonary embolism was one who was expired just before trial of thrombolytic therapy. Conclusion : Efforts should be made to shorten the interval from onset of Sx to Dx, ie, high index of suspision.

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Surgical and Long Term Results for Double Outlet Right Ventricle by the Type of Ventricular Septal Defect (심실중격결손의 형태에 따른 양대혈관 우심실기시증의 수술 및 장기 결과)

  • Yu Song Hyeon;Park Han Ki;Cho Bum Koo;Park Young Hwan
    • Journal of Chest Surgery
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    • v.38 no.3 s.248
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    • pp.181-190
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    • 2005
  • The results of biventricular repair for double outlet right ventricle have been improved in recent series. We studied the surgical and long term results for total correction of double outlet right ventricle by the type of ventricular septal defect. Material and Method: Between November 1979 and December 2003, 126 patients had biventricular repair for double outlet right ventricle. The mean age was 1.8 years (range 1$\~$44) and 86 patients ($68.3\%$) were male. We classified and studied this disease by the type of VSD. Result: The locations of VSD were subaortic in 79 ($62.7\%$), subpulmonary in 17 ($13.5\%$), doubly committed in 16 ($12.7\%$) and noncommitted in 14 ($11.1\%$). 28 patients had palliative operation before total correction and the mean interval to total correction was 41.0$\pm$45.1 months. The methods of total correction were intraventricular baffling in 37 ($29.4\%$), intraventricular baffling with patch enlargement of right ventricular outflow tract in 49 ($38.9\%$), intraventricular baffling with Rastelli procedure in 15 ($11.9\%$), arterial switch operation in 8 ($6.3\%$) and REV procedure in 4 ($3.2\%$), etc. Hospital mortality rate was $10.3\%$ (13 patients) and 25 reoperations were performed in 24 patients ($19.0\%$). The risk factors for hospital mortality and reoperation were cardiopulmonary bypass time (p=0.020) and previous palliative operation (p=0.013), respectively. Follow up was possible in 98 patients and mean follow up period was 118.9$\pm$70.7 months. The percent survival and survival for freedom from reoperation at 15 years were $82.5\%$ and $66.7\%$, respectively. The survival rate was significantly lower (p=0.003) in transposition of great artery type and remote type than in simple ventricular septal defect type and tetralogy of Fallot type, but there was no statistical differences in survival rate for freedom from reoperation. Conclusion: It is thought to be that acceptible surgical and long term results can be obtained with application of appropriate methods of repair for double outlet right ventricle.

Aoric Valve Lesion in Type I Ventricular Septal Defect (제1형 심실중격결손에서 대동맥판막 병변)

  • 김관창;임홍국;김웅한;김용진;노준량;배은정;노정일;윤용수;안규리
    • Journal of Chest Surgery
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    • v.37 no.6
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    • pp.492-498
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    • 2004
  • Background: In this study, we investigated the risk factors for the development or progression of aortic regurgitation(AR) in patients with type I ventricular septal defect (VSD) to determine the optimal surgical timing and strategy. Material and Method: Three-hundred and ten patients with type I VSD with or without AR were included. The mean of age was 73.7$\pm$114.7 (1-737) months. One hundred and eighty six patients (60%) had no AR, 83 (27%) had mild AR, 25 (8%) had moderate AR and 16 (5%) had severe AR. Aortic valve was repaired in 5 patients and replaced in 11 patients with closure of VSD in the first operation. Four patients required redo aortic valve repair and 11 patients required redo aortic valve replacement. Age at operation, association with aortic valve prolapse, Qp/Qs, systolic pulmonary arterial pressure, VSD size and systolic pulmonary artery to aortic pressure ratio(s[PAP/AP]) were included as risk factors analysis for the development of AR. The long-term result of aortic valve repair and aortic valve replacement were compared. Result: Older age at operation, association with aortic valve prolapse, high Qp/Qs, and s[PAP/AP] were identified as risk factors for the development of AR (p<0.05, Table 2). The older the patient at the time of operation, the higher the severity of preoperative AR and the incidence of postoperative AR (p<0.05, Table 1, Fig. 1). For the older patients at operation, aortic valve repair had higher occurrence of AR compared to those who had aortic valve replacement (p<0.05, Fig. 2). Conclusion: From the result of this study, we can concluded that early primary repair is recommended to decrease the progression of AR. Aortic valve repair is not always a satisfactory option to correct the aortic valve pathology, which may suggest that aortic valve replacement should be considered when indicated.