• Clinical and Experimental Pediatrics
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• v.59 no.2
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• pp.59-64
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• 2016

Purpose: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. Methods: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. Results: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. Conclusion: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.

• Journal of Chest Surgery
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• v.21 no.5
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• pp.893-898
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• 1988

A number of centers have recorded a significant incidence of primary tissue valve failure with the Ionescu-Shiley pericardial valve. Clinically, Endothelialization and host tissue ingrowth on the cloth and the leaflets at the edge of the frame greatly reduced the amounts of abrasion and the incidence of tissue failure. In most cases severe regurgitation was caused by leaflet tears adjacent to the edge of the cloth-covered stent. We report a case of spontaneous disruption of one cusp on the Ionescu-Shiley pericardial xenograft in mitral position at 6years and its successful management.

• Journal of Chest Surgery
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• v.38 no.2 s.247
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• pp.132-138
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• 2005

Background: Mitral valve repair (MVP) is the optimal procedure for mitral regurgitation (MR), however, failure and subsequent reoperations are the limitations. The current study assessed the procedure in relation to the primary valve related causes of recurrent MR. Material and Method: MR was treated in 493 patients undergoing MVP from January of 1994 to January of 2002. The causes of MR were degenerative $(n=252,\;51.5\%),$ rheumatic $(n=156,\; 31.6\%),$ and others $(n=85,\; 16.9\%).$ Surgery comprised 446 ring annuloplasties $(90.5\%),$ 227 new chordae formations $(46\%),$ 125 quadriangular resections $(25.3\%),$ 28 chordae transfers $(5.7\%),$ and 8 Alfieri's stitches $(1.6\%).$ The mean follow up was $29.04\pm22.81$ months. Result: There were 5 early $(1.01\%)$, and 5 late deaths $(1.01\%).$ The reoperation rate was $1.42\%$. There were 45 $(9.1\%)$ recurrent MR (grade III or IV). Of these, 24 were procedure related including incomplete repair (n=14), discordant new chordae length (n=8) and others (n=2). In 21 patients, the cause was valve related including rheumatic disease progression (n=10), recurrent chordae elongation or prolapse (n=5) and others (n=6). Severe MR was higher after incomplete repair (p < 0.001), and valve related failure strongly correlated with rheumatic progression (p < 0.05). Conclusion: Since completeness of operation is the prime risk factor that determine the repair durability, intra-operative assessment of the initial repair with trans-esophageal echocardiography is essential.

• Journal of Korean Neurosurgical Society
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• v.30 no.1
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• pp.73-77
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• 2001

Object : To determine whether to use surgical or medical therapy in treatment of infectious intracranial aneurysms, we reviewed two recent cases of infectious intracranial aneurysms and others known previous reports of aforementioned cases. Hence, we attempted to compare the validity and effectiveness of surgical and medical treatment. Method : Recently, we treated two cases of ruptured infectious intracranial aneurysms. In former case, the aneurysm was located distal to the middle cerebral artery in a patient with mild mitral regurgitation of the heart. In latter case, the aneurysm was multiple with varying hemorrhage. The hemorrhage was located bilaterally and a moderate mitral regurgitation and infective endocarditis were accompanied in this patient. Result : Due to the large size of the intracranial hematoma, stable medical condition, and easy resectability, we treated the former patient surgically. And, because of successive hemorrhage by multiple aneurysmal rupture, and the risk of heart failure, we treated the latter patient medically with serial follow-up angiography. Both patients are at present in good health. Conclusion : Because of the variability in associated factors, such as the patient's health, the number of lesions, location, anatomy of the aneurysms and the causative organism, each patient's care must be individualized and tailored to the patient's particular clinical situation.

• Journal of Chest Surgery
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• v.48 no.6
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• pp.407-410
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• 2015

A two-month-old infant presented with coarctation of the aorta, severe left ventricular dysfunction, and moderate to severe mitral regurgitation. Through median sternotomy, the aortic arch was repaired under cardiopulmonary bypass and regional cerebral perfusion. The patient was postoperatively supported with a left ventricular assist device for five days. Left ventricular function gradually improved, eventually recovering with the concomitant regression of mitral regurgitation. Prompt surgical repair of coarctation of the aorta is indicated for patients with severe left ventricular dysfunction. A central approach for surgical repair with a back-up left ventricular assist device is a safe and effective treatment strategy for these patients.

• Journal of Chest Surgery
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• v.52 no.1
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• pp.9-15
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• 2019

Background: Although aortic valve repair can reduce prosthesis-related complications, rheumatic aortic regurgitation (AR) caused by leaflet restriction is a significant risk factor for recurrent AR. In this study, we evaluated the long-term results of the leaflet extension technique for rheumatic AR. Methods: Between 1995 and 2016, 33 patients underwent aortic valve repair using the leaflet extension technique with autologous pericardium for rheumatic pure AR. Twenty patients had severe AR and 9 had combined moderate or greater mitral regurgitation. Their mean age was $32.2{\pm}13.9$ years. The mean follow-up duration was $18.3{\pm}5.8$ years. Results: There were no cases of operative mortality, but postoperative complications occurred in 5 patients. Overall survival at 10 and 20 years was 93.5% and 87.1%, respectively. There were no thromboembolic cerebrovascular events, but 4 late deaths occurred, as well as a bleeding event in 1 patient who was taking warfarin. Twelve patients underwent aortic valve reoperation. The mean interval to reoperation was $13.1{\pm}6.1$ years. Freedom from reoperation at 10 and 20 years was 96.7% and 66.6%, respectively. Conclusion: The long-term results of the leaflet extension technique showed acceptable durability and a low incidence of thromboembolic events and bleeding. The leaflet extension technique may be a good option for young patients with rheumatic AR.

• Journal of Chest Surgery
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• v.55 no.1
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• pp.69-76
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• 2022

Background: While the use of bioprosthetic valves for mitral valve replacement (MVR) is increasing, very few studies have compared bovine pericardial and porcine valves in the mitral position to help guide bioprosthetic selection. Methods: In the present study, patients who underwent MVR using bovine pericardial valves were compared with those who underwent MVR with porcine bioprostheses between January 1996 and July 2018. Those with prior MVR, infective endocarditis, congenital mitral valve disease, or ischemic mitral regurgitation were excluded. The primary outcomes were structural valve deterioration (SVD) and mitral valve reoperation from any cause, and death was regarded as a competing risk. Competing risk analysis and propensity score-matching were used for comparisons. Results: Among the 388 patients enrolled, pericardial and porcine bioprostheses were implanted in 217 (55.9%) and 171 (44.1%), respectively. Propensity score-matching yielded 122 pairs of patients that were well-balanced for all baseline covariates. No significant differences were observed between the groups in unadjusted (p=0.09) and adjusted overall survival (hazard ratio [HR], 1.13; 95% confidence interval [CI], 0.72-1.76; p=0.60). Competing risk analysis revealed no significant differences in the risks of mitral reoperation (HR, 1.07; 95% CI, 0.50-2.27; p=0.86) and development of SVD (HR, 1.57; 95% CI, 0.56-4.36; p=0.39) between the groups. Matched population analysis confirmed similar results regarding reoperation (HR, 0.99; 95% CI, 0.40-3.22; p=0.98) and SVD (HR, 1.39; 95% CI, 0.41-4.73; p=0.60). Conclusion: No significant differences in survival or valve durability were observed between bovine pericardial and porcine bioprosthetic MVR. These findings require further validation through studies with larger sample sizes.

• Journal of Chest Surgery
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• v.15 no.1
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• pp.53-60
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• 1982

M-mode echocardiographic studies of left ventricular function in mitral valve disease were evaluated in 19 surgically treated patients before and one year after operation (mean 12.7 months). Twelve patients had mitral stenosis (MS) and seven patients had mitral regurgitation (MR). Before surgery, average end-diastolic and end systolic dimensions (EDD, and ESD) and left atrial dimension were significantly greater than normal in subject with MR. After surgery, EDD fell significantly from $66.5{\pm}8.4$ (SD)mm to $52.7{\pm}6.3$mm (P 0.01) at the time of late follow up study; ESD fell significantly from $46.5{\pm}9.7$mm to $36.4{\pm}8.6$ (P 0.05) on early follow up study; left atrial dimension fell significantly from $60.5{\pm}6.8$mm to $48.1{\pm}7.2$mm (P 0.01) at the time of the late follow up study. In patients with MS, EDD and ESD were normal and did not change significantly at any time after surgery. The left ventricular ejection fraction (E.F.) was normal in both groups. preoperatively([MR: $64.2{\pm}15.1$, MS: $65.7{\pm}12.3$). After surgery, E.F. did not change significantly at any time after surgery in both groups, but de-creased from $64.2{\pm}15.1$% to $59.5{\pm}11.2$% in MR patients at the time of early follow up study.

• Journal of Chest Surgery
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• v.42 no.2
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• pp.175-183
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• 2009

Background: Mitral valve abnormalities in the pediatric population are rare. Mitral valve replacement or pediatric mitral lesions can cause problems such as a lack of growth potential. There re only limited experiences with mitral valve repair at any institution, so the purpose of his study is to evaluate the outcomes of mitral valve repair n pediatric patients. Material and Method: Sixty-four consecutive children (28 males and 36 females) with a mean age of $5.5{\pm}4.7$ years underwent mitral valve repair for treating their congenital mitral valve disease between January 1996 and December 2005. The patients were divided into two groups: group 1 (34 patients (53.1%)) had isolated disease (mitral anomaly with or without trial septal defect or patent ductus arteriosus) and group 2 (30 patients (46.9%)) had complex disease (mitral anomaly with concurrent intracardiac disease, except atrioventricular septal defect). Result: The overall in-hospital mortality was 6.3%; group 1 had 5.9% mortality and group 2 had 10.0% mortality. The postoperative morbidity was 18.8%; group 1 and 2 had 14.7% and 23.3% postoperative morbidity, respectively, and there as no significant difference among the groups. The median follow-up was 4.6 years range: $0.5{\sim}12.2$ years). The 10-year survival rate was 95.3%. The 10-year freedom from re-operation rate was 76.1% with 10 re-operations. The majority of the functional classifications were annular dilatation and leaflet prolapse. A mean of $2.1{\pm}1.1$ procedures per patient were performed. The echocardiography that was done at the immediate postoperative period showed a significant improvement in the mitral valve function. The follow-up echocardiographic results were significantly improved. However, mitral stenosis newly developed over time, and there ere significant differences according to the repair strategies. Conclusion: The patients who underwent mitral valve repair for congenital mitral anomalies showed good results. The follow-up echocardiography revealed satisfactory short-term and long-term results. Close follow-up is necessary to detect the development of postoperative mitral stenosis or regurgitation.

• Journal of Chest Surgery
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• v.40 no.12
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• pp.855-858
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• 2007

A left ventricular rupture might be one of the most disastrous complications after a mitral valve replacement. An acute atrioventricular groove rupture (type I) was detected in a 54-year-old female diagnosed with a mitral stenosis combined with severe tricuspid regurgitation. She had a prior medical history of an open mitral commissurotomy in Japan at 30 years ago. The surgical findings suggested that the previous procedure was not a simple commissurotomy but a commissurotomy combined with a posteromedial annuloplasty procedure. After a successful mitral valve replacement and a measured (De Vega type) tricuspid annuloplasty, the weaning from a cardiopulmonary bypass was uneventful. However, copious intraoperative bleeding from the posterior wall was detected and the cardiopulmonary bypass was restarted. Exposure of the posterior wall of the left ventricle showed bleeding from the atrioventricular groove 3 cm lateral to the left atrial auricle. Under the impression of a Type I left ventricular rupture, epicardial repair (primary repair of the Teflon felt pledgetted suture, continuous sealing suture using auto-pericardial patch and application of fibrin-sealant) was attempted. Successful local control was made and the patient recovered uneventfully. The patient was discharged at 14 postoperative days without complications. We report this successful epicardial repair of an acute type I left ventricular rupture after mitral valve replacement.