• Title/Summary/Keyword: Ventricular function, right

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Light-Chain Cardiac Amyloidosis: Cardiac Magnetic Resonance for Assessing Response to Chemotherapy

  • Yubo Guo;Xiao Li;Yajuan Gao;Kaini Shen;Lu Lin;Jian Wang;Jian Cao;Zhuoli Zhang;Ke Wan;Xi Yang Zhou;Yucheng Chen;Long Jiang Zhang;Jian Li;Yining Wang
    • Korean Journal of Radiology
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    • v.25 no.5
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    • pp.426-437
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    • 2024
  • Objective: Cardiac magnetic resonance (CMR) is a diagnostic tool that provides precise and reproducible information about cardiac structure, function, and tissue characterization, aiding in the monitoring of chemotherapy response in patients with light-chain cardiac amyloidosis (AL-CA). This study aimed to evaluate the feasibility of CMR in monitoring responses to chemotherapy in patients with AL-CA. Materials and Methods: In this prospective study, we enrolled 111 patients with AL-CA (50.5% male; median age, 54 [interquartile range, 49-63] years). Patients underwent longitudinal monitoring using biomarkers and CMR imaging. At follow-up after chemotherapy, patients were categorized into superior and inferior response groups based on their hematological and cardiac laboratory responses to chemotherapy. Changes in CMR findings across therapies and differences between response groups were analyzed. Results: Following chemotherapy (before vs. after), there were significant increases in myocardial T2 (43.6 ± 3.5 ms vs. 44.6 ± 4.1 ms; P = 0.008), recovery in right ventricular (RV) longitudinal strain (median of -9.6% vs. -11.7%; P = 0.031), and decrease in RV extracellular volume fraction (ECV) (median of 53.9% vs. 51.6%; P = 0.048). These changes were more pronounced in the superior-response group. Patients with superior cardiac laboratory response showed significantly greater reductions in RV ECV (-2.9% [interquartile range, -8.7%-1.1%] vs. 1.7% [-5.5%-7.1%]; P = 0.017) and left ventricular ECV (-2.0% [-6.0%-1.3%] vs. 2.0% [-3.0%-5.0%]; P = 0.01) compared with those with inferior response. Conclusion: Cardiac amyloid deposition can regress following chemotherapy in patients with AL-CA, particularly showing more prominent regression, possibly earlier, in the RV. CMR emerges as an effective tool for monitoring associated tissue characteristics and ventricular functional recovery in patients with AL-CA undergoing chemotherapy, thereby supporting its utility in treatment response assessment.

Correlation between the Seoul Neuropsychological Screening Battery of the Parkinson's Disease Patient with Mild Cognitive Impairment and Change of the Cerebral Ventricle Volume in the Brain MRI (경도인지장애를 동반한 파킨슨병 환자의 서울신경심리검사와 뇌 자기공명영상에서 뇌실 체적 변화에 대한 상관관계)

  • Lee, Hyunyong;Kim, Hyeonjin;Im, Inchul;Lee, Jaeseung
    • Journal of the Korean Society of Radiology
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    • v.8 no.5
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    • pp.231-240
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    • 2014
  • The purpose of this study were to analyze that the Seoul neuropsychological screening battery (SNSB) for the evaluating cognitive assessment of the Parkinson's disease patients with mild cognitive impairment (PD-MCI) and the changes of the cerebral ventricle volume in the brain magnetic resonance imaging (MRI), and we has been bring forward the guideline to determine the diagnostic criteria for the PD-MCI. To achieve this, we was diagnosed with Parkinson's disease patients (PD-MCI group: 34 patients; Parkinson's disease with normal cognition, PD-NC group: 34 patients) to perform the SNSB test for the attention, language, memory, visuospatial, and frontal/executive functions and the brain MRI. Additionally, to compared the change of the cerebral ventricle volume, we performed the brain MRI for the 32 normal control (NC) group. The volumetric analysis for a specific cerebral ventricle performed by using Freesurfer Ver. 5.1 (Massachusetts general Hospital, Boston MA, USA). As a results, compared to the PD-NC group, the PD-MCI group were statistically significant reduction in the ability to perform the memory and the visuospatial function (p<0.05). The volumetric changes for a specific cerebral ventricle were statistically significant variation in the left and right lateral ventricle, left and right inferior lateral ventricle, and 3rd ventricle. Although, in order to compared the objectification, the normalized percentage applied to the volumetric changes showed to extend the PD-MCI group than the PD-NC group. Specially, the left and right ventricle extension for the PD-MCI patients conspicuously had showed a quantitative linear relationship between the memory and the visuospatial function for the SNSB (r>0.5, p<0.05). Therefore, we were able to judge the diagnostic criteria of the PD-MCI through that can observe the volumetric variation of the specific cerebral ventricle by using Freesurfer in brain MRI, and to analyze the correlation between the SNSB.

The Clinical Application and Results of Palliative Damus-Kaye-Stansel Procedure (고식적 Damus-Kaye-Stansel 술식의 임상적 적용 및 결과)

  • Lim, Hong-Gook;Kim, Soo-Jin;Kim, Woong-Han;Hwang, Seong-Wook;Lee, Cheul;Shinn, Sung-Ho;Yie, Kil-Soo;Lee, Jae-Woong;Lee, Chang-Ha
    • Journal of Chest Surgery
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    • v.41 no.1
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    • pp.1-11
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    • 2008
  • Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.

The alterations of $Ca^{2+}$-activated $K^+$ channels in coronary artery during cardiac hypertrophy

  • Kim, Nari;Lee, Sang-Kyeong;Chung, Joon-Yong;Seog, Dae-Hyun;Kim, Euiyong;Jin Han
    • Proceedings of the Korean Biophysical Society Conference
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    • 2003.06a
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    • pp.23-23
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    • 2003
  • It has been suggested that the impairment of smooth muscle cell (SMC) function by alterations in the $Ca^{2+}$-activated $K^{+}$ ( $K_{Ca}$ ) channels accounts for the reduction in coronary reserve during left ventricular hypertrophy (LVH). However, this hypothesis has not been fully investigated. The main goal of this study was to assess whether the properties of $K_{Ca}$ channels in coronary SMCs were altered during LVH. New Zealand white rabbits (0.8-1.0 kg) and Sprague-Dawley rats (300-400 g) were randomly selected to receive either an injection of isoproterenol (300 $\mu\textrm{g}$/kg body weight) or an equal volume of 0.9% saline (1 mL/kg body weight). The animals developed LVH 10 days after injection. In patch-clamp experiments, the unitary current amplitude and open probability for the $K_{Ca}$ channels were significantly reduced in LVH patches compared with control patches. The concentration-response curve of the $K_{Ca}$ channel to [C $a^{2+}$]$_{i}$ was shifted to the right. Inhibition of the $K_{Ca}$ channels with TEA was more pronounced in LVH cells than in the control cells. The whole-cell currents of $K_{Ca}$ channels were reduced during LVH. Western blot analysis indicated no differences in $K_{Ca}$ channel expression between the control and LVH coronary SM membranes. In contraction experiments, the effect of a high $K^{+}$concentration on the resting tension of the LVH coronary artery was greater than on that of the control. The effect of TEA on the resting tension of the LVH coronary artery was reduced as compared with the effect on the control. Our findings imply a novel mechanism for reduced coronary reserve during LVH.ing LVH.

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Histologic Changes of the Immunologically Untreated Xenogenic Valved Conduit (면역학적 처리 없는 이종 심장 판막 도관의 조직학적 변화에 관한 연구)

  • Sung, Ki-Ick;Seo, Jeong-Wook;Kim, Won-Gon
    • Journal of Chest Surgery
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    • v.40 no.1 s.270
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    • pp.1-7
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    • 2007
  • Backgound: It has been shown that the endothelium of cardiac valves and adjacent great vessels have a reduced immune reaction compared to other vessels. We investigated the clinical feasibility of using immunologically untreated xenogenic valves, in a pig-to-goat pulmonary valve conduit implantation model. Material and Method: Porcine pulmonary valve conduits were prepared without specific immunologic treatment and implanted into the right ventricular outflow tract of goats while undergoing cardiopulmonary bypass. Two goats each were assigned to the following observation time intervals: one day, one week, three months, six months and twelve months. Echo-cardiographic examinations were performed prior to sacrifice of the goat to evaluate pulmonary valve function. After the xenograft specimens were retrieved, histological changes were evaluated microscopically. Result: Ten of the twelve animals survived the predetermined observation time intervals. Aneurysmal dilatations, of the anterior wall of the implanted pulmonary artery, were observed at each of three and twelve month-survival animals. A variable degree of pulmonary valve regurgitation was observed on echocardiography. However, valve stenosis, thrombotic occlusion and vegetation were not seen. Microscopically, the nuclei of the donor tissue disappeared as a result of pyknosis and karyolysis; however the three components of the implanted xenografts (the pulmonary artery, the valve and the infundibulum) were gradually replaced by host cells over time, while maintaining their structural integrity. Conclusion: Immunologically untreated xenogenic pulmonary valve conduits were replaced by host cells with few observed clinical problems in a pig to goat pulmonary valve implantation model. Therefore, they might be an alternative bioprosthesis option.

Arterial Switch Operation in Patients with Intramural Coronary Artery: Early and Mid-term Results

  • Kim, Hyung-Tae;Sung, Si-Chan;Kim, Si-Ho;Chang, Yun-Hee;Ahn, Hyo-Yeong;Lee, Hyoung-Doo
    • Journal of Chest Surgery
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    • v.44 no.2
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    • pp.115-122
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    • 2011
  • Background: The intramural coronary artery has been known as a risk factor for early death after an arterial switch operation (ASO). We reviewed the morphological characteristics and evaluated the early and mid-term results of ASO for patients with an intramural coronary artery. Materials and Methods: From March 1994 to September 15th 2010, 158 patients underwent ASO at Dong-A and Pusan National University Hospitals for repair of transposition of the great arteries and double outlet right ventricle. Among these patients, 14 patients (8.9%) had an intramural coronary artery. Mean age at operation was $13.4{\pm}10.2$ days (4 to 39 days) and mean body weight was $3.48{\pm}0.33$ kg (2.88 to 3.88 kg). All patients except one were male. Eight patients had TGA/IVS and 4 patients had an aortic arch anomaly. Two patients (14.3%) had side-by-side great artery relation, of whom one had an intramural right coronary artery and the other had an intramural left anterior descending coronary artery. Twelve patients had anterior-posterior relation, all of whom had an intramural left coronary artery (LCA). The aortocoronary flap technique was used in coronary transfer in 8 patients, of whom one patient required a switch to the individual coronary button technique 2 days after operation because of myocardial ischemia. An individual coronary button implantation technique was adopted in 6, of whom 2 patients required left subclavian artery free graft to LCA during the same operation due to LCA injury during coronary button mobilization and LCA torsion. Results: There was 1 operative death (7.1%), which occurred in the first patient in our series. This patient underwent an aortocoronary flap procedure for coronary transfer combining aortic arch repair. Overall operative mortality for 144 patients without an intramural coronary artery was 13.2% (19/144). There was no statistical difference in operative mortality between the patients with and without an intramural coronary artery (p>0.1). There was no late death. The mean follow-up duration was $52.1{\pm}43.0$ months (0.5 to 132 months). One patient who had a subclavian artery free graft required LCA stenting 6.5 years after surgery for LCA anastomotic site stenosis. No other surviving patient needed any intervention for coronary problems. All patients had normal ventricular function at latest echocardiography and were in NYHA class 1. Conclusion: The arterial switch operation in Transposition of Great Arteries or Double Outlet Right Ventricle patients with intramural coronary can be performed with low mortality; however, there is a high incidence of intraoperative or postoperative coronary problems, which can be managed with conversion to the individual coronary button technique and a bypass procedure using a left subclavian free graft. Both aortocoronary flap and individual coronary button implantation techniques for coronary transfer have excellent mid-term results.

The Prognostic role of Electrocardiographic Signs of Cor Pulmonale in Chronic Obstructive Pulmonary Disease (폐성심을 시사하는 심전도 소견 유.무에 따른 만성폐쇄성 폐질환 환자의 예후)

  • Shin, Moo-Chul;Park, Jae-Yong;Bae, Moon-Seob;Bae, Nack-Cheon;Chae, Po-Hee;Kim, Chang-Ho;Jung, Tae-Hoon
    • Tuberculosis and Respiratory Diseases
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    • v.48 no.6
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    • pp.944-955
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    • 2000
  • Background : In patients with chronic obstructive pulmonary disease(COPD), several factors have been associated with a poor prognosis. These include old age, low $FEV_1$ low diffusing capacity, high alveolar-arterial oxygen pressure difference, and finally cor pulmonale. This study was done to investigate if the ECG signs suggesting cor pulmonale were independent prognostic factors in patients with COPD. Method : We analyzed ECG, pulmonary function data and arterial blood gas values in 61 patients who were admitted through the emergency department with an acute exacerbation of COPD. The ECG signs reflecting cor pulmonale were right strial overloading(RAO), right bundle branch block, right ventricular hypertrophy and low-voltage QRS. The 61 patients were divided into 2 groups ; group I with no ECG signs(n=36) and group II with one or more ECG signs(n=25) suggesting cor, pulmonale. Results : Poor, prognostic factors by univariate analysis were low $FEV_1$, $FEV_1$ % pred., VC % pred., DLco, DLco % pred., $PaO_2$ and $SaO_2$ high $PaCO_2$ presence of ECG signs reflecting cor pulmonale, presence of mental status change, use of mechanical ventilator, and long term use of glucocorticoid. A multivariate analysis indicated that age(risk ratio=1.13, 95% confidence interval 1.05-1.23), DLco % pred. (risk ratio=0,97. 95% confidence interval 0.94-0.99), $PaO_2$ (risk ratio=0.95, 95% confidence interval 0.90-0.99) and RAO(risk ratio=5.27, 95% confidence interval 1.40-19.85) were independent prognostic factors of survival. There was a significant difference in survival between the patients with and without RAO(p=0.038). The survival rates at 1, 2, and 5 years were 94.5%. 81.4%, and 50.0% in patients without RAO and 82.4%, 70.6%, and 27.5% in patients with RAO, respectively. Conclusion : These results suggest that the presence of ECG signs reflecting cor pulmonale is a predictor of survival and that RAO of these ECG signs is a significant independent predictor of survival in patients with COPD.

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Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up (수정 대혈관 전위 환자에서 예후에 영향을 주는 인자들에 대한 중기적 고찰)

  • Song, Young-Hwan;Kwon, Hyok-Joo;Kim, Gi-Beom;Kang, Soo-Jung;Bae, Eun-Jung;Noh, Chung-Il;Yun, Yong-Soo;Lee, Jeong-Ryul;Kim, Yong-Jin;Rho, Joon-Ryang
    • Clinical and Experimental Pediatrics
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    • v.46 no.2
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    • pp.154-161
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    • 2003
  • Purpose : The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. Methods : Medical records of 94 patients(males 58, females 36; mean age at last follow-up, $12{\pm}9$ years; mean follow-up duration, $9{\pm}6.4$ years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. Results : Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P=0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. Conclusion : TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

ROC Analysis of Visual Assessments Made in Gated Blood Pool Scans of Patients with Coronary Artery Disease (관상동맥질환에서 심장풀 스캔의 육안적 평가에 대한 ROC 분석)

  • Lee, Kyun-Han;Choi, Yoon-Ho;Lee, Bum-Woo;Moon, Dae-Hyuk;Koong, Sung-Soo;Chung, June-Key;Lee, Myung-Chul;Koh, Chang-Soon
    • The Korean Journal of Nuclear Medicine
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    • v.23 no.2
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    • pp.175-181
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    • 1989
  • Visual assessment of regional wall motion abnormality (RWMA) by gated blood pol scan (GBPS) serves as an useful parameter in the diagnosis, functional evaluation, and follow up in various clinical settings, but are still subject to some inherent limitations. On important problem may be the interobserver as well as intraobsever variation that may well be present due to the subjective nature of the interpretations. This study was carried out to determine the reliability and reproducibility of visual assessments made in GBPSs, and to observe the degree to which the results would be influenced by observer variation. Fifty two patients with coronary heart disease had resting GBPS and contrast ventriculography within 4 days appall. Contrast ventriculography-showed normal wall motion in 6 patients and the remaining 46 had RWMA in one or more segments. The anterior and left anterolateral views of all 52 GBPSs were analyzed by three independent observers, who selected from 5 scales, their level of confidence that there was RWMA in that segment. Reciever operating characteristic (ROC) curves for each analysis was plotted and the area under the curve $(\theta)$ was used as a parameter representing each observer's performance in his interpretations. The findings of contrast ventriculographies were used as the standard for RWMA. The apical and inferoapical segments showed the best correlation with contrast ventriculography ($\theta=0.90-0.94$, 0.81-0.94, respectively), and the inferior wall showed the poorest correlation $(\theta=0.70-0.74)$. The interpretations of the inferior, septal, apical, and posteroinferior, segments showed no difference between the observers, but there was significantly better performance in assessment by observer A compared to that by B or C for the anterolateral segments ($\theta=0.87$, 0.78, 0.76, respectively. p<0.01 for A vs B, p<0.05 for A vs C), as well as when all segments were considered altogether ($\theta=0.88$, 0.83, 0.82, respectively. both p<0.05). This was also true for the infero-apical segment between A and C ($\theta=0.09$, 0.81, p<0.05). The intraobserver variation, however, did not appear significant, with only the inferior segment for observer B showing any significant difference when observer A and B repeated the analysis 10 days latter. There was no difference in assessing dyskinesia, with all observers showing a high performance ($\theta=0.98$, 0.87, 0.97, respectively). The visual assessment of left ventricular ejection fraction by all three observers correlated well with the calculated value from a semiautomated method (Spearman's r = 0.91, 0.83, 0.83. p<0.01, p<0.05, p < 0.05). The assessment of LV and RV size also correlated well between the three observers (Kendall's w = 0.80, 0.51, p<0.01 for both left and right ventricles). The above findings suggest that RWMA visually assessed by GBPS correlates well with that done by contrast ventriculography. And although the observer's experience or skill may influence the results in certain segments, visual analysis of GBPS may serve as a reliable and reproducible means for evaluating ventricular function.

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Early Result of Surgical Management of the Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (관상동맥-폐동맥 이상 기시증에 대한 수술의 조기 결과)

  • Yoon Yoo Sang;Park Jeong Jun;Yun Tae Jin;Kim Young Hwue;Ko Jae Kon;Park In Sook;Seo Dong Man
    • Journal of Chest Surgery
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    • v.39 no.1 s.258
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    • pp.18-27
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    • 2006
  • Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, but is one of the most common causes of myocardial ischemia which would result in high mortality within the first year of life. This is our early result of the surgical management for these patients. Material and Method: From June 1989 to July 2003, 6 patients with ALCAPA and one patient with ARCAPA (Anomalous origin of the Right coronary artery from the pulmonary artery) underwent surgical repair. We have reviewed the all medical records, electrocardiogram, chest X-ray and echocardiography retrospectively. Result: Three of the patients were boys and four were girls. The median age at the operation was 5.4 months (Range: 3$\∼$33 months). The average body weight of at the operation was 6.7 kg (Range: 3.7$\∼$11.3 kg). A mean follow up period was 18 months. Only 3 patients were initially diagnosed as ALCAPA. And 3 patients had moderate mitral regurgitation. Immediate coronary artery reimplantation on diagnosis with the aim of restoring a two-coronary system circulation was done. The average bypass time was 114$\pm$37 minutes, and the average aortic cross clamping time was 55$\pm$22 minutes. The average stay of intensive care unit was 5$\pm$3 days, the mean mechanical ventilator time was 38$\pm$45 hours and the hospital stay after operation was 12$\pm$5 days. There were significant improvements in electrocardiogram and chest X-ray of the all patients except one late death patient. The ventricular function showed almost normal recovery after operation; the EF (Ejection Fraction) increased from 41.2$\pm$ 10.3$\%$ to 60.5$\pm$ 15.8$\%$ within 1 month and to 59.8$\pm$13.9$\%$ within 1 year after operation, the SF (Shortening Fraction) increased from 23.6$\pm$4.7$\%$ to 38.6$\pm$8.4$\%$ within 1 month and to 37.4$\pm$7.9$\%$ within 1 year after operation, LVEDDI (Left Ventricular End-diastolic Dimension Index) decreased from 100.8$\pm$25.6 mm/$m^{2}$ to 90.3$\pm$ 19.2 mm/$m^{2}$ within f month and to 79.3$\pm$ 15.8 mm/$m^{2}$ within 1 year after operation. Concomitant mitral repair was done in two patients with anterior mitral leaflet prolapse. In every patient, mitral valve showed less than mild regurgitation during follow up. One late death occurred in which patient Dor procedure was applied 10 months after initial operation due to the dilated cardiomyopathy Conclusion: In the management of this rare and could be fatal Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), early suspicion and correct diagnosis is of most important. But, after diagnosis, immediate restoration of 2 coronary systems could result in good outcome.