Background: From January 1989 to December 1996, we analyzed 22 cases of ventricular septal defect associated(VSD) with aortic valvular prolapse. Material and Method: The mean age of the patients was 7 years with a range of 6 months to 22 years . Thirteen patients were male and 9 were female. The types of VSD were Kirklin type I in 13 , Kirklin type II in 8 and Kirklin type I+II in one. Result: The preoperative echocardiographic findings were aortic valvular prolapse in 10 patients, aortic valvular prolapse associated with aortic regurgitation in 6, and only aortic regurgitation in 2. Aortic valvular prolapse were found in operation field in 4 that was not be in preoperative echcardiography. Preoperative mean Qp/Qs, systolic PAP, systolic RVP were 1.48${\pm}$0.42, 27.9${\pm}$9.87, 32.9${\pm}$10.87 mmHg, respectively. Twenty patients underwent patch closure of VSD, and two patients with moderate aortic regurgitation and prolapsed of the aortic valve underwent patch closure of VSD and aortic valvuloplasty. Short and long term echocardiographic follow-up in 8 patients who had preoperative aortic regurgitation were found to have improved or not aggravated by performing VSD patch closure only and patch closure with valvuloplasty in 2. Twelve patients who had only preoperative aortic valvular prolapse had no change in prolapsed valve in postoperative echocardiography. Conclusion: Early closure of VSD with patch is necessary in VSD with aortic valvular prolapse even in associated with mild regurgitation. But in moderate regurgitation, VSD closure with valvuloplasty is recommended.
Chae, Yong Hwa;Kwak, Dong Wook;Kim, Moon Young;Park, So Yeon;Lee, Bom Yi;Lee, Yeon Woo;Lee, Young Ho;Song, Mi Jin;Ryu, Hyun Mee
Journal of Genetic Medicine
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v.10
no.2
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pp.99-103
/
2013
Purpose: This study was designed to determine the frequency and echocardiographic findings of 22q11.2 deletions in fetuses with cardiac defects on fetal ultrasound or familial backgrounds of 22q11.2 deletions. Materials and methods: We retrospectively reviewed the medical and ultrasonographic records of 170 fetuses that underwent fluorescence in situ hybridization (FISH) analysis for chromosome 22q11.2 deletions between February 2001 and April 2013. Results: Among 145 fetuses with cardiac defects, six (4.1%) had 22q11.2 deletions. Deletions of 22q11.2 were detected in 6 (5%) of the 120 fetuses with conotruncal defects: 5 (8.9%) of 56 with tetralogy of Fallot (TOF) and 1 (5.9%) of 17 with double outlet right ventricle (DORV). No deletions were found in cases of pulmonary atresia, truncus arteriosus, right aortic arch, or transposition of the great arteries. No 22q11.2 deletions were found in non-conotruncal cardiac malformations. Among 25 fetuses with familial backgrounds of 22q11.2 deletions, one (4%) had a maternally inherited 22q11.2 deletion with no cardiac findings. Conclusion: Knowledge of the frequency and echocardiographic findings of 22q11.2 deletions might be helpful for prenatal genetic counseling. It is advisable to perform FISH analysis for 22q11.2 deletions in pregnancies exhibiting conotruncal cardiac defects such as TOF or DORV.
Proceedings of the Korean Society of Medical Physics Conference
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2002.09a
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pp.314-317
/
2002
The purpose of this study was to evaluate the clinical application of Patlak tool on GE PET workstation for quantitative analysis of dynamic PET images in cardiac patients. Three patients including coronary artery disease (CAD), myocardial infarction (MI), and angina were studied. All subjects underwent dynamic cardiac PET scan using a GE Advance scanner. After 10 min transmission scan for attenuation correction using two rotating $\^$68/Ge rod sources, three patients with cardiac disease were performed dynamic cardiac PET scan after the administration of approximately 370 MBq of FDG. The dynamic scan consisted of 36 frames with variable frame length (12${\times}$10s, 6${\times}$20s, 6${\times}$60s, 12${\times}$300s) for a total time of 70 min. Blood samples were obtained to determine the plasma substrate concentration. Region of interest of circular and rectangular shape to acquire input functions and tissue data were placed on left ventricle and myocardium. A value of 0.67 was used for lumped constant. Mean plasma substrate concentrations for three patients were 100 mg/dl (CAD), 100 mg/dl (MI), 132 mg/dl (angina), respectively. Regional MMRGlc values (mean${\pm}$SD) at lateral myocardium area for CAD, MI, and angina were 8.43${\pm}$0.24, 4.08${\pm}$0.16, and 6.15${\pm}$0.23 mg/min/100ml, respectively. Patlak tool on GE PET workstation appeared to be useful for quantitative analysis of dynamic PET images in cardiac patients, although further studies may be required for absolute quantitation.
This study was performed to assess the efficacy of surgical treatment with flexible alligator forceps (FAF) on heartworm infected dogs. Twenty dogs (10 males and 10 females) with heartworm infection, age $5.3\pm3.0\;(mean{\pm}S.D.)$ years and weight $9.3\pm6.5(mean{\pm}S.D.)kg$ were treated with FAF. Adult heartworms were removed by fluoroguided technique with FAF under inhalation anesthesia with isoflurane. For evaluation of the efficacy, removal rate and surgical time were measured, and removal sites were found. Antigen ELISA kit test and autopsy were performed to examine remained adult heartworms. Removal rate of total heartworms was $91.4\%$ including 15 complete retrieval cases and surgical time was $30.0\pm7.6(mean{\pm}S.E.)$ minutes. Heartworms were removed from the right ventricle, main pulmonary artery, and right and left pulmonary artery. Surgical treatment with FAF is an effective method with high removal rate, short surgical time, and various removal sites and it could be considered for the removal of adult heartworms in a dog.
This study was carried out to investigate the amino acid and protein concentrations in amniotic fluid and the potency of the teratogenic effect of ethylenethiourea(2-imidazolidinethione, ETU) in the fetuses due to different dose amounts of this compound. The S.P.F. Sprague-Dawley female rats(10 weeks) were used in this study and these animals were divided into four groups; control group(25pregnant female rats), group I (dosed ETU from day 7 to day 17 of gestation at 10mg/kg/day), group II (dosed ETU from day 7 to day 17 of gestation at 30mg/kg/day), group III (dosed ETU from day 7 to day 17 of gestation at 50mg/kg/ day). 250mg/100mg ETU in group I, 750mg/100ml ETU in group II and 1,250mg/100ml ETU in group III were administered 4ml/kg 13.W by oral route. The results obtained were summarized as follows; 1. The anomalies of the external examination werf meningocele in the head, kinky tail, clubfoot and sharp tail.(Meningocele, in group III, significantly increased from control value at p<0.001). 2. The skeletal variations and delayed ossification were Lumbar ribs, asymmetric sternebrae, asymmetric 13th rib and delayed ossification of skull. Asymmetric sternebrae(group III ) was significantly increased from control value at p<0.05 and delayed ossification of skull (group II and III ) were significantly increased from control value at p<0.05 and p<0.01, respectively. 3. The internal soft tissue anomalies were hydroencephaly of 3th lateral ventricle, dilatation of ureter, dilatation of renal pelvis and cleft palate. (Hydroencephaly, 28.1% in group I, 88.3% in group II and 100% in group III ). 4. Protein values in amniotic fluids are not significantly decreased in 10mg/kg group but significantly(p<0.05) decreased in 30mg/kg group and 50mg/kg group from control group. 5. In the levels of amino acid in amniotic fluids, the levels of glntamic acid, iso-lencine, leucine, tyrosine and phenylalanine of 10mg/kg group are significantly decreased from control group. In 50mg/kg group, except for glycine, valine and methionine, all amino acid levels are significantly(p<0.05) decreased from control group.
Between August 1996 and August 1997, 22 patients underwent extracardiac Fontan operations. The basic diagnoses included univentricular heart of the right ventricular type (n=12); univentricular heart of the left ventricular type (n=4); tricuspid atresia (n=4); left isomerism, transposition of great arteries, ventricular septal defect and pulmonary stenosis (n=1); and criss-cross heart with uneven ventricle (n=1). The median age of the 14 men and 8 women was 29 months (range from 21 months to 26 years). Previous procedures included bidirectional cavopulmonary shunt (n=15, interval=15.6$\pm$3.4 months), Kawashima operation (n=4, interval=37.5$\pm$20 months), and classic Glenn shunt (n=1, interval=14 years). In 2 patients, extracardiac Fontan operations were done without any previous procedures. A 16- to 22-mm flexible Gore-Tex tube graft (n=18), Hemashield graft (n=3), or, alternatively, a nonvalved aortic allograft (n=1) was cut and anastomosed end-to-end between inferior vena cava and undersurface of pulmonary artery using Gore-Tex or Prolene suture in a running fashion. In risk Fontan patients (n=12), a communication between the extracardiac conduit and the right atrium was constructed. In the most 13 recent patients, the procedures were done without cross-clamping of the aorta and with a beating heart. Operative mortality was 9.1% (n=2). Complications included persistent chest tube drainage for more than 7 days (n=5), chorea (n=2), and low cardiac output (n=1). There were no late deaths. Follow-up echocardiogram (mean: 6 months) demonstrated satisfactory hemodynamic results in the surviving 20 patients. Potential advantages of this technique consist of minimization of surgical manipulation of atrial tissue, reduction or elimination of myocardial ischemia, creationof a uniform and stable inferior vena cava-to-pulmonary artery conduit, and increased flexibility and safety in certain high-risk patients such as those with increased pulmonary vascular resistance, pulmonary hypertension, and impaired ventricular function. Further investigations during a longer follow-up are needed to confirm the intermediate and long-term results, especially the reduction of late atrial arrhythmias.
Background: Recently, cell transplantation has been extensively investigated to improve heart function in dysfunctional heart. This study was designed to compare the effects of smooth muscle cells (SMC) and heart cells (HC) transplantation in dilated cardiomyopathic hamsters. Material and Method: HC and SMC were isolated from heart and ductus deferens of BIO 53.58 hamsters, and cultured for transplantation. HC and SMC or culture medium were transplanted into the left ventricle of 17 weeks old adult hamsters in HC transplanted (HCTx), SMC transplantation (SMCTX), and control groups (Con) (N = 10 each). Cyclosporine (5 mg/Kg) was administered subcutaneously for HCTx. Sham operated hamsters (N=10) underwent the surgery but did not receive an injection. At 4 weeks after transplantation, heart function was evaluated in all groups using a Langendorff perfusion apparatus. Result: Histology showed severe focal myocardial necrosis in all groups. HCTx and SMCTx formed huge muscle tissue in dilated myocardium. SMCTx and HCTx had better heart function than Con and sham (p<0.01). And SMCTx had better peak systolic pressure (p<0.05) antral developed pressure (p<0.05) than HCTx. But sham and Con did not any statistical make difference. Conclusion: SMCTx and HCTx formed muscle tissue and improved ventricular function in hamsters with dilated cardiomyopathy And SMCTx showed better heart function in peak systolic pressure and developed pressure than HCTx.
Congenital aortic stenosis in children is characterized by "excessive" left ventricular hypertrophy with reduced left ventricular systolic wall stress that allows for supernormal ejection performance. We hypothesized that left ventricular wall stress was decreased immediately after surgical correction of pure congenital aortic stenosis. Also measuring postoperative left ventricular wall stress was a useful noninvasive measurement that allowed direct assessment for oxygen consumption of myocardium than measuring the peak systolic pressure gradient between ascending aorta and left ventricle for the assessment of surgical results. Material and Method: Between September 1993 and August 1999, 8 patients with isolated congenital aortic stenosis who underwent surgical correction at Yonsei cardiovascular center were evaluated. There were 6 male and 2 female patients ranging in age from 2 to 11 years(mean age, 10 years). Combined Hemodynamic-Ultrasonic method was used for studying left ventricular wall stress. We compared the wall stress peak systolic pressure gradient and ejection fraction preoperatively and postoperatively. Result: After surgical correction peak aortic gradient fell from 58.4${\pm}$17.6, to 23.7${\pm}$17.7 mmHg(p=0.018) and left ventricular ejection fraction decreased but it is not statistically significant. In the consideration of some factors that influence left ventricular end-systolic wall stress excluding one patient who underwent reoperation for restenosis of left ventricular outflow tract left ventricular end-systolic pressure and left ventricular end-systolic dimension were fell from 170.6${\pm}$24.3 to 143.7${\pm}$27.1 mmHg and from 1.78${\pm}$0.4 to 1.76${\pm}$0.4 cm respectively and left ventricular posterior wall thickness was increased from 1.10${\pm}$0.2, to 1.27${\pm}$0.3cm but it was not statistically singificant whereas left ventricular end-systolic wall stress fell from 79.2${\pm}$24.9 to 57.1${\pm}$27.6 kdynes/cm2(p=0.018) in 7 patients. For one patient who underwent reoperation peak aortic gradient fell from 83.0 to 59.7 mmHg whereas left ventricular end-systolic wall stress increased from 67.2 to 97.0 kdynes/cm2 The intervals did not change significnatly. Conclusion ; We believe that probably some factors that are related to left ventricular geometry influenced the decreased left ventricular wall stress immediately after surgical correction of isolated congenital aortic stenosis. Left ventricular wall stress is a noninvasive measurement and can allow for more direct assesment than measuring peak aortic gradient particularly in consideration of the stress and oxygen consumption of the myocardium therefore we can conclude it is a useful measurement for postoperative assessment of congenital aortic stenosis.
According to the 2007 International Society for Heart and Lung Transplantation (ISHLT) report, a congenital diagnosis, infantile transplantation and being on extracorporeal membrane oxygenation (ECMO) at the time of transplant are risk factors for mortality for the patients who undergo a heart transplant, and a large body weight ratio also increases the risk of mortality. The patient of this case underwent a Ross operation and mitral valve repair due to left ventricle outflow track obstruction and mitral regurgitation. But the baby was treated with ECMO due to heart failure after the operation. When he was 3-months-old and had been. on 30 days of ECMO, he underwent a heart transplant with a heart that had a high donor-recipient weight ratio (4.42). We present this case from a technical standpoint and we include a review of the relevant literature.
Song, Jin Hwa;Park, Eun-Ah;Lee, Whal;Chung, Jin Wook;Park, Jae Hyung
Investigative Magnetic Resonance Imaging
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v.17
no.1
/
pp.33-40
/
2013
Purpose : To evaluate late gadolinium enhancement (LGE) pattern of left ventricular (LV) myocardium and presence or absence of LGE in other regions of the heart on cardiac magnetic resonance (CMR) imaging in patients diagnosed with cardiac amyloidosis. Materials and Methods: From 2009 to 2011, 9 patients who were suspected cardiac amyloidosis underwent CMR. We retrospectively analyzed the presence or absence of LGE and enhancement pattern in LV myocardium, and the presence or absence of LGE in other chambers as well. Also we measured interatrial septal thickness (IST), relative signal intensities of atrial septum and epicardial fat over the left atrial (LA) cavity on delayed enhanced images. MRI parameters in these patients were compared to those of control group of patients with ischemic heart disease by Wilcoxon rank sum test. Results: Of nine patients, LGE were found in 8; subendocardial circumferential pattern in 4 and diffuse pattern in 4. LGE in right ventricle was observed in 7. IST was significantly increased in patients with cardiac amyloidosis (P = 0.02). Ratio of atrial septum to LA cavity and ratio of epicardial fat to LA cavity showed a significant difference (P = 0.0002 and P = 0.0006, respectively). Conclusion: In LGE CMR, subendocardial or diffuse enhancement pattern is a typical finding for patients with cardiac amyloidosis. Atrial septum and epicardial fat show relatively increased signal intensities over LA blood cavity.
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