Giant Cardiac Rhabdomyoma with Mixed Atrial Tachycardia and Nonsustained Ventricular Tachycardia in a Newborn with Tuberous Sclerosis / 이화의대지

Cardiac rhabdomyomas are typically presented in the tuberous sclerosis. Although benign and often associated with spontaneous regression, in rare circumstances huge mass size and critical location can lead to heart failure, ventricular outflow tract obstruction and refractory tachyarrhythmias. An 1-day-old girl was diagnosed as cardiac tumor during perinatal period. At birth, transthoracic echocardiography revealed huge cardiac mass located in septal area of both ventricle measuring 34×30 mm. It protruded into the left ventricular (LV) outflow tract, potentially obstructing it. A surface ECG revealed atrial tachycardia with nonsustained ventricular tachycardia with heart rate of 217 beats per min. The tachyarrhythmias were controlled with intravenous amiodarone. Reduction of the giant cardiac mass was treated with mammalian target of rapamycin pathway inhibitor sirolimus. However, she unfortunately died at 10 days-old because of sudden cardiac arrest maybe due to LV outflow tract obstruction during therapy. Gene analysis revealed TSC2 mutation after death. (Ewha Med J 2022;45(3):e5)

Embarrassed Radiofrequency Catheter Ablation of Supraventricular Tachycardia in Pectus Excavatum / 이화의대지

A 16-year-old patient with pectus excavatum visited our hospital because of palpitation. He underwent first Nuss operations at the age of 3. When he was 13 years old, the slow-fast type atrioventricular nodal reentrant tachycardia was documented during electrophysiology study. However, the catheter ablation was not conducted because of recurrent atrial fibrillation during procedure. At that time, second Nuss operation was performed due to progressive chest wall deformity. And then, atrioventricular nodal reentrant tachycardia was successfully treated by radiofrequency catheter ablation at the higher position than usual slow pathway zone under the modified fluoroscopic view with the cranial angle although distorted right atrial geometry and radiographic obstacle of Nuss operation bar. The concern about abnormal cardiac and electrical anatomy, and the accurate and modified procedure technique are essential in patients with pectus excavatum. (Ewha Med J 2022;45(3):e6)

Benign arrhythmias in pediatric patients

An irregular heart rhythm is a common concern among children. Recognizing the cause of the irregular rhythm is crucial for the proper diagnosis and management by primary physicians as well as pediatric specialists. The cause of pediatric arrhythmias can be identified based on the clinical history, physical examination, presenting symptoms, and electrocardiogram. Pediatric arrhythmias are classified as benign and non-benign. Both benign and non-benign arrhythmias can originate from the sinoatrial node, atrial myocardium, atrioventricular node, and ventricle myocardium. The common form of benign arrhythmias, including sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and Wenckebach atrioventricular block, have no clinical significance or need for therapy. On the other hand, non-benign arrhythmias, such as supraventricular tachycardia, ventricular tachycardia, high-degree atrioventricular conduction abnormalities, and genetic arrhythmia, can affect the hemodynamic state and may cause a serious condition in pediatric patients. Most cases of benign arrhythmia are asymptomatic, and the prognosis is favorable for the normal heart. In this article, we review our current understanding of the electrocardiographic characteristics, clinical presentation, etiology, and natural history of benign arrhythmias in pediatric patients.

Discrimination of Kawasaki disease with concomitant adenoviral detection differentiating from isolated adenoviral infection / 소아과

PURPOSE: Human adenovirus infection mimics Kawasaki disease (KD) but can be detected in KD patients. The aim of this study was to determine the clinical differences between KD with adenovirus infection and only adenoviral infection and to identify biomarkers for prediction of adenovirus-positive KD from isolated adenoviral infection. METHODS: A total of 147 patients with isolated adenovirus were identified by quantitative polymerase chain reaction. In addition, 11 patients having KD with adenovirus, who were treated with intravenous immunoglobulin therapy during the acute phase of KD were also evaluated. RESULTS: Compared with the adenoviral infection group, the KD with adenovirus group was significantly associated with frequent lip and tongue changes, skin rash and changes in the extremities. In the laboratory parameters, higher C-reactive protein (CRP) level and presence of hypoalbuminemia and sterile pyuria were significantly associated with the KD group. In the multivariate analysis, lip and tongue changes (odds ratio [OR], 1.416; 95% confidence interval [CI], 1.151–1.741; P=0.001), high CRP level (OR, 1.039; 95% CI 1.743–1.454; P= 0.021) and sterile pyuria (OR 1.052; 95% CI 0.861–1.286; P=0.041) were the significant predictive factors of KD. In addition, the cutoff CRP level related to KD with adenoviral detection was 56 mg/L, with a sensitivity of 81.8% and a specificity of 75.9%. CONCLUSION: Lip and tongue changes, higher serum CRP level and sterile pyuria were significantly correlated with adenovirus-positive KD.

Comparison of Clinical Manifestation and Laboratory Findings between Adenoviral Infection with or without Kawasaki Disease / 이화의대지

OBJECTIVES: Adenovirus infection, which has been known to mimic Kawasaki disease (KD), is one of the most frequent conditions observed during differential diagnosis when considering KD. Accordingly, it is essential to being able to differentiate between these two diseases. Therefore, we performed multiplex reverse transcriptase-polymerase chain reaction and tissue-Doppler echocardiography to distinguish between adenovirus patients and KD patients. METHODS: A total of 113 adenoviral infection patients (female 48, male 65) diagnosed from January 2010 to June 2016 were evaluated. We divided adenoviral infection patients into two groups: group 1, which consisted of individuals diagnosed with KD according to the KD American Heart Association criteria (n=62, KD with adenovirus infection); and group 2, which comprised individuals only diagnosed with adenovirus infection (n=51). Laboratory data were obtained from each patient including N-terminal pro-brain natriuretic peptide. Echocardiographic measurements were compared between two groups. In addition, reverse transcriptase-polymerase chain reaction was performed using nasopharyngeal secretions to diagnose adenoviral infection. RESULTS: Conjunctival injection, cervical lymphadenopathy, polymorphous skin rash, abnormalities of the lip or oral mucosa and abnormalities of extremities were significantly higher in group 1 than group 2. Moreover, group 1 had significantly higher C-reactive protein and alanine aminotransferase levels, as well as lower platelet counts and albumin levels than group 2. Coronary artery diameter was significantly greater in group 1 than group 2. CONCLUSION: In patients with adenoviral infection with unexplained prolonged fever, echocardiography and C-reactive protein can be used to differentiate KD with adenoviral infection from adenoviral infection alone.

Tracheal Bronchus with Persistent Pulmonary Hypertension of the Newborn: A Case Report

Tracheal bronchus is an uncommon anomaly in which an ectopic bronchus originates directly from the supracarinal trachea. It is usually an asymptomatic anatomical variant incidentally found on computed tomography or bronchoscopy. However, it can present with symptoms, such as chronic cough, wheezing, atelectasis, and recurrent pneumonia. We report a case of tracheal bronchus diagnosed in the neonatal period, in which the term baby presented with respiratory distress and persistent pulmonary hypertension of the newborn after birth, but no other congenital anomaly was found on further evaluation.

Neonatal arrhythmias: diagnosis, treatment, and clinical outcome / 소아과

Arrhythmias in the neonatal period are not uncommon, and may occur in neonates with a normal heart or in those with structural heart disease. Neonatal arrhythmias are classified as either benign or nonbenign. Benign arrhythmias include sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and junctional rhythm; these arrhythmias have no clinical significance and do not need therapy. Supraventricular tachycardia, ventricular tachycardia, atrioventricular conduction abnormalities, and genetic arrhythmia such as congenital long-QT syndrome are classified as nonbenign arrhythmias. Although most neonatal arrhythmias are asymptomatic and rarely life-threatening, the prognosis depends on the early recognition and proper management of the condition in some serious cases. Precise diagnosis with risk stratification of patients with nonbenign neonatal arrhythmia is needed to reduce morbidity and mortality. In this article, I review the current understanding of the common clinical presentation, etiology, natural history, and management of neonatal arrhythmias in the absence of an underlying congenital heart disease.

Intractable Atrial Flutter Successfully Treated with Flecainide and Propranolol in a Premature Infant / 이화의대지

Although Atrial flutter (AFL) in newborn infant with normal cardiac anatomy has benign clinical course, an intractable AFL is associated with an increased risk of development of heart failure and sudden death, and is still difficult to manage. It requires multiple external electrical cardioversions, and it shows a poor response to antiarrhythmic drug therapy. We report a case of a premature infant with an intractable AFL, which we successfully treated with oral flecainide and propranolol in spite of recurred AFL. A 1-month-old, 34-week gestation, premature baby presented with an irregular heart beat and irritability. An AFL with 2:1 atrioventricular conduction was documented. Because of the intractable AFL, repeated electrical cardioversion and amiodarone were continued for 14 days. However, amiodarone was discontinued in favour of flecainide and propranolol because of the recurrent AFL and newly developed transient hypothyroidism. During 1-year follow-up period, in which oral flecainide and propranolol were continued, no AFL was observed.

Serum periostin levels and squamous cell carcinoma-related antigen levels in children with atopic dermatitis

PURPOSE: It was found that periostin and squamous cell carcinoma-related antigens (SCCAs) were strongly interleukin-13-inducible gene products. This study measures the serum periostin and SCCA levels in children suffering from atopic dermatitis (AD) and to evaluate the association between the severity of AD and their values. METHODS: Seventy AD children aged 1 month to 10 years were included in our study. Subjects were characterized as having atopic eczema (AE; n=55) or non-AE (NAE; n=15) by atopic sensitization. Serum SCCA and periostin levels were measured. RESULTS: The serum periostin levels were significantly higher in children with AE than in those with NAE (geometric mean [95% confidence interval]: 80.47 ng/mL [75.06–85.93 ng/mL] vs. 67.45 ng/mL [59.99–75.64] ng/mL, P=0.020). The serum concentrations of both SCCA1 and SCCA2 were significantly higher in children with AE than in those with NAE (geometric mean [95% confidence interval]: 1.401 [1.198–1.643] ng/mL vs. 0.969 [0.723–1.268] ng/mL, P=0.039 for SCCA1) (1.178 [0.974–1.455] ng/mL vs. 0.711 [0.540–0.994] ng/mL, P=0.025 for SCCA2). The serum periostin levels were significantly correlated with disease severity and with peripheral blood eosinophil counts. The SCCA levels were not significantly correlated with disease severity. Both SCCA1 and SCCA2 were significantly correlated with serum periostin levels and blood eosinophil counts. CONCLUSION: Serum periostin levels may be significantly correlated with disease severity and blood eosinophil counts in children with AD. Serum SCCA levels can be significantly correlated with serum periostin levels and blood eosinophil counts in children with AD.

Relationship between atopy and bronchial hyperresponsiveness to indirect stimuli in asthmatic children

PURPOSE: Both atopy and bronchial hyperresponsiveness (BHR) are characteristic features of asthma. Several BHR studies comparing groups of atopic and nonatopic asthmatics have reported conflicting results. The aim of this study was to compare BHR to indirect stimuli, such as mannitol or exercise, between atopic and nonatopic asthmatics in children. METHODS: We performed a retrospective analysis of data from 110 children with asthma, aged 6–18 years using skin prick tests, and serum total and specific IgE levels. Atopy degree was measured using the sum of graded wheal size or the sum of the allergen-specific IgE. Bronchial provocation tests (BPTs) using methacholine were performed on all subjects. BPTs using indirect simuli, including exercise and mannitol, were also performed. RESULTS: Asthma cases were classified as atopic asthma (n=83) or nonatopic asthma (n=27) from skin prick or allergen-specific IgE test results. There was no significant difference in the prevalence of BHR to mannitol or exercise between atopic and nonatopic asthmatics. Atopic asthma had a significantly lower postexercise maximum decrease in % forced expiratory volume in 1 second (FEV1) (geometric mean [95% confidence interval]: 31.9 [22.9–40.9] vs. 14.0 [9.4–18.6], P=0.015) and a methacholine PC20 (provocative concentration of methacholine inducing a 20% fall in FEV1) than nonatopic asthmatics (geometric mean [95% confidence interval]: 1.24 [0.60–1.87] ng/mL vs. 4.97 [3.47–6.47]) ng/mL, P=0.001), whereas mannitol PD15 (cumulative provocative dose causing a 15% fall in FEV1) was not significantly different between the 2 groups. CONCLUSION: There was no significant difference in the prevalence of BHR to mannitol or exercise between atopic and nonatopic asthmatics in children.

Swallowing-Induced Atrial Tachyarrhythmias Successfully Ablated at the Left Posterior Interatrial Septum in Patient with Wolff-Parkinson-White Syndrome

We reported a case of a 55-year-old patient who presented with palpitation after swallowing. Initial surface electrocardiogram revealed ventricular preexcitation utilizing a left lateral bypass tract. The orthodromic atrioventricular reentrant tachycardia (AVRT) was induced during electrophysiologic studies. After successful ablation of the AVRT utilizing a left lateral free wall bypass tract, 2 different atrial tachycardias (ATs) were induced under isoproterenol infusion. When the patient swallowed saliva or drank water, 2 consecutive beats of atrial premature complexes (APCs) preceded another non-sustained AT repeatedly, which was coincident with the patient's symptom. The preceding APC couplet had the same activation sequence with one induced AT, and the subsequent non-sustained AT had the same activation sequence with the other induced AT, respectively. We first targeted the preceding 2 consecutive APCs at the left posterior interatrial septum. The following non-sustained AT was also eliminated following ablation of the APCs. After ablation, the patient remained free from the swallowing-induced atrial tachyarrhythmias during the one year follow-up.

Electrophysiological Characteristics Related to Outcome after Catheter Ablation of Idiopathic Ventricular Arrhythmia Originating from the Papillary Muscle in the Left Ventricle

BACKGROUND AND OBJECTIVES: The electrophysiological properties associated with favorable outcome of radiofrequency catheter ablation (RFCA) for idiopathic ventricular arrhythmia (VA) originating from the papillary muscle (PM) remain unclear. The purpose of this study was to investigate the relationships of electrophysiological characteristics and predictors with the outcome of RFCA in patients with VAs originating from PM in the left ventricle (LV). SUBJECTS AND METHODS: Twelve (4.2%) of 284 consecutive patients with idiopathic VAs originating from LV PM were assessed. The electrophysiological data were compared between the patients in the successful group and patients in the recurrence group after RFCA. RESULTS: In 12 patients with PM VAs, non-sustained ventricular tachycardias (VTs, n=6), sustained VTs (n=4) and premature ventricular complexes (n=2) were identified as the presenting arrhythmias. Seven of eight patients showing high-amplitude discrete potentials at the ablation site had a successful outcome (85.7%), while the remaining four patients who showed low-amplitude fractionated potentials at the ablation site experienced VA recurrence. The mean duration from onset to peak downstroke (Deltat) on the unipolar electrogram was significantly longer in the successful group than in the recurrence group (58+/-8 ms vs. 37+/-9 ms, p=0.04). A slow downstroke >50 ms of the initial Q wave on the unipolar electrogram at ablation sites was also significantly associated with successful outcome (85.7% vs. 25.0%, p=0.03). CONCLUSION: In PM VAs, the high-amplitude discrete potentials before QRS and slow downstroke of the initial Q wave on the unipolar electrogram at ablation sites were related to favorable outcome after RFCA.

Bivalvation Valvuloplasty for Common Atrioventricular Valve Regurgitation in Functional Single Ventricle; Early and Mid-term Results / 대한흉부외과학회지

BACKGROUND: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. MATERIAL AND METHOD: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvation valvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was 6.9+/-7.0 months (median 4 months, 24 days~21 months) and mean body weight was 6.2+/-2.8 kg (3.1~11.3 kg). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. RESULT: There was one hospital death after surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months (4.3~114 months). Mean preoperative CAVVR score was 3.3+/-0.6, which decreased to 1.9+/-0.7 postoperatively (p<0.0001). This residual regurgitation slightly increased to 2.2+/-0.4 (no statistical significance) after a mean follow-up of 14.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectional cavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. CONCLUSION: Bivalvation valvuloplasty for CAVVR in FSV patients is an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.

A Case of Successful Bronchial Artery Embolization for Bronchial Artery to Pulmonary Artery Shunt with Massive Hemoptysis / 소아알레르기및호흡기학회지

A 13-year-old girl was admitted with massive hemoptysis and hematemesis. An estimated volume was about 400 cc. The blood was frothy and mixed with sputum and food. She had no history of epistaxis, choking, joint pain or trauma. Her vital sign was stable and physical examination was normal except for bilateral crackles and ronchi on auscultation of the lungs. Laboratory data on arrival including blood counts, liver enzyme, urinalysis and electrolytes were all normal. Chest CT showed bilateral extensive centrilobular ground glass opacity nodules and there were suspicious blood clots in the right bronchus. There was no evidence of pleural effusion, increased vascular markings, abscess or brochiectasis. We performed an upper GI endoscopy and bronchoscopy, it was all normal. On cytology from bronchoalveolar lavage, cell count was 1,660/microL with 63% of macrophages laden with erythrocytes and 35% lymphocytes. Tuberculosis PCR from this fluid was negative and AFB stain, Gram stains were all negative. She has had a second attack on day seven, emergency bronchial angiography was performed. There was dysplastic bronchial artery with a tortuous change, shunting to the pulmonary artery. Bronchial artery embolization was performed successfully using embolization particles. Follow-up blood counts, electrolytes and chest radiogram were all normal. There was no further episode of hemoptysis twelve months after embolization.

Replacement of Obstructed Extracardiac Conduits with Autologous Tissue Reconstructions (Peel operation); Early and Midterm Results / 대한흉부외과학회지

BACKGROUND: Reoperation is usually required for a right ventricle to pulmonary artery conduit obstruction caused by valve degeneration, conduit peel formation or somatic growth of the patient. An autologous tissue reconstruction (peel operation), where a prosthetic roof is placed over the fibrotic tissue bed of the explanted conduit, has been used to manage conduit obstructions at our institute since May 2002. Herein, the early and midterm results are evaluated. MATERIAL AND METHOD: Between May 2002 and July 2006, 9 patients underwent obstructed extracardiac conduit replacement with an autologous tissue reconstruction, at a mean of 5.1 years after a Rastelli operation. The mean age at reoperation was 7.5+/-2.4 years, ranging from 2.9 to 10.1 years. The diagnoses included 6 pulmonary atresia with VSD, 2 truncus arteriosus and 1 transposition of the great arteries. The preoperative mean systolic gradient was 88.3+/-22.2 mmHg, ranging from 58 to 125 mmHg. The explanted conduits were all Polystan valved pulmonary conduit (Polystan, Denmark). A bioprosthetic valve was inserted in 8 patients, and a monocusp ventricular outflow patch (MVOP) was used in 1 patient. The anterior wall was constructed with a Gore-Tex patch (n=7), MVOP (n=1) and bovine pericardium (n=1). Pulmonary artery angioplasty was required in 5 patients and anterior aortopexy in 2. The mean cardiopulmonary bypass time was 154 minutes, ranging from 133 to 181 minutes; an aortic crossclamp was not performed in all patients. The mean follow-up duration was 20 months, ranging from 1 to 51 months. All patients were evaluated for their right ventricular outflow pathway using a 3-D CT scan. RESULT: There was no operative mortality or late death. The mean pressure gradient, assessed by echocardiography through the right ventricular outflow tract, was 20.4 mmHg, ranging from 0 to 29.6 mmHg, at discharge and 26 mmHg, ranging from 13 to 36 mmHg, at the latest follow-up (n=7, follow-up duration >1 year). There were no pseudoaneurysms, strictures or thrombotic occlusions. CONCLUSION: A peel operation was concluded to be a safe and effective re-operative option for an obstructed extracardiac conduit following a Rastelli operation.

Can Peritoneal Dialysis Remove Endothelin-1 after Cardiopulmonary Bypass for Repair of Congenital Heart Disease? / 대한흉부외과학회지

BACKGROUND: Lung injury that follows bypass has been well described. It is manifested as reduced oxygenation and lung compliance and, most importantly, increased pulmonary vascular resistance reactivity; this is a known cause of morbidity and mortality after repair of congenital heart disease. Injury to the pulmonary vascular endothelium, and its associated alterations of endothelin-1, is considered to be a major factor of bypass-induced lung injury. Removing endothelin-1 after bypass may attenuate this response. This study measured the concentration of serum and peritoneal effluent endothelin-1 after performing bypass to determine if endothelin-1 can be removed via peritoneal dialysis. MATERIAL AND METHOD: From March 2005 to March 2006, 18 patients were enrolled in this study. Peritoneal catheters were placed at the end of surgery. Serum samples were obtained before and after bypass, and peritoneal effluents were obtained after bypass. Endothelin-1 was measured by enzyme linked immunosorbent assay (ELISA). RESULT: In the patients with a severe increase of the pulmonary artery pressure or flow, the mean preoperative plasma endothelin-1 concentration was significantly higher than that in the patients who were without an increase of their pulmonary artery pressure or flow (4.2 vs 1.8 pg/mL, respectively, p<0.001). The mean concentration of plasma endothelin-1 increased from a preoperative value of 3.61+/-2.17 to 5.33+/-3.72 pg/ml immediately after bypass. After peritoneal dialysis, the mean plasma endothelin-1 concentration started to decrease. Its concentration at 18 hours after bypass was significantly lower than the value obtained immediately after bypass (p=0.036). CONCLUSION: Our data showed that the plasma endothelin-1 concentration became persistently decreased after starting peritoneal dialysis, and this suggests that peritoneal dialysis can remove the circulating plasma endothelin-1.

A case of isolated congenitally corrected transposition of the great arteries with complete atrioventricular block / 대한내과학회지

Patients with isolated congenitally corrected transposition of the great arteries (CCTGA) comprise 1% of all CCTGA patients. They are usually asymptomatic until functional abnormalities such as atrioventricular block or systemic atrioventricular valve regurgitation become complicated. We report here a case of a 33 year-old man with isolated CCTGA and complete AV block that underwent implantation of a permanent pacemaker using endocardial leads. During a 12 month follow-up period, no symptoms developed and lead-related parameters have been stable.

Analysis of the Causes of and Risk Factors for Mortality in the Surgical Repair of Interrupted Aortic Arch / 대한흉부외과학회지

BACKGROUND: Interrupted aortic arch is a rare congenital heart anomaly which still shows high surgical mortality. In this study, we investigated the causes of and the risk factors for mortality to improve the surgical outcomes for this difficult disease entity. MATERIAL AND METHOD: From 1984 to 2004, 42 patients diagnosed as IAA were reviewed retrospectively. Age, body weight at operation, preoperative diagnosis, preoperative PGE 1 requirement, type of interrupted aortic arch, degree of left ventricular outflow stenosis, CPB time, and ACC time were the possible risk factors for mortality. RESULT: There were 14 hospital deaths. Preoperative use of PGE1, need for circulartory assist and aortic cross clamp time proved to be positive risk factors for mortality on univariate analysis. Preoperative left ventricular outflow stenosis was considered a risk factor for mortality but it did not show statistical significance (p-value=0.61). Causes of death included hypoxia due to pulmonary banding, left ventricular outtract stenosis, infection, mitral valve regurgitation, long cardiopulmonary bypass time and failure of coronary transfer failure in TGA patients. CONCLUSION: In this study, we demonstrated that surgical mortality is still high due to the risk factors including preoperative status and long operative time. However preoperative subaortic dimension was not related statistically to operative death statistically. Adequate preoperative management and short operation time are mandatory for better survival outcome.

One-stage Repair of Aortic Arch Anomaly and Associated Complex Intracardiac Defects:Recent 5-year's Result

PURPOSE: The optimal surgical approach for aortic arch anomaly associated complex intracardiac defects is not universally agreed upon. We reviewed our recent experience of one-stage repair for the aortic arch anomaly associated with complex intracardiac defects. METHODS: We retrospectively analyzed the medical records of 15 consecutive patients with arch anomaly associated with complex intracardiac defects underwent going a one-stage repair through median sternotomy between September 2000 and May 2004. Transposition of the great artery or Taussig-Bing was associated in 9 patients, truncus arteriosus in 2, Shone' complex in 2, aorticopulmonary window in 1, and double outlet right ventricle in 1 patient. Aortic arch anomalies were coartation in 9 patients and interruption in 6. Age at operation ranged from 3 days to 23.4 months (median 12.5 days) and body weight ranged from 2.3 to 10.3 kg (mean 3.7+/-1.9 kg). RESULTS: There were 2 early deaths (13.3%) in the patients with Taussig-Bing anomaly and Shone's complex respectively. The causes of deaths were sepsis with right heart failure and pulmonary edema respectively. There were 2 late deaths. A patient with truncus arteriosus died suddenly of unknown cause and the other patient with Taussig-Bing anomaly died of ventricular dysfunction. One of 2 patients who died lately underwent balloon aortoplasty with success. There was no residual or recurrent coarctation in the rest of the patients. A patient required reoperation for left pulmonary artery stenosis. All survivors were in NYHA functional class I. CONCLUSION: One-stage repair of aortic arch with complex intracardiac defects can be performed with acceptable operative mortality. This procedure is strongly recommended as considering their grave prognosis and adverse effects of staged repair.

Early and mid-term results of pulmonary valve reconstruction in surgical repair of tetralogy of Fallot; comparison with other techniques of right ventricular outflow reconstruction / 소아과

PURPOSE: The purpose of this study is to determine whether the new pulmonary valve reconstruction technique prevents short-term postoperative pulmonary regurgitation and improves early and mid-term clinical outcome. METHODS: We reviewed postoperative echocardiographic variables and chest X-ray films from 31 patients who had undergone valve reconstruction(pulmonary valve reconstruction group:PVR) for the repair of TOF between April 2000 and August 2004. We compared the clinical data of these patients with those from 47 patients who had right ventricular outflow tract reconstruction with a monocusp valve(monocusp ventricular outflow patch group:MVOP) and 22 patients who had a transannular patch repair without a monocusp valve(transannular patch group:TAP). RESULTS: In the PVR group, 25 patients(81 percent) had trivial or mild pulmonary regurgitation in their early post operative echocardiogram. Only 12 patients(26 percent) in the MVOP group had mild pulmonary regurgitation; and no patient in the TAP group had it. Pulmonary valve function was good in 96 percent of the PVR group, 36 percent of the MVOP group, and none in the TAP group in early post-operative echocardiogram. Follow-up echocardiogram(1, 2, 3, 4 years later) of the MVOP and TAP groups showed moderate pulmonary regurgitation and severely decreased valve function in almost all cases. However, in the PVR group 54 percent(16/28), 50 percent(14/28), 37 percent(9/24), and 31 percent(5/16) of the patients had trivial or mild pulmonary regurgitation 1, 2, 3 and 4 years after operation, respectively. The valve function remained good in 80 percent(24/30), 64 percent(18/28), 57 percent(12/21), and 31 percent(5/16) of the patients 1, 2, 3 and 4 years after operation respectively. CONCLUSION: Pulmonary valve reconstruction is effective in reducing pulmonary regurgitation and right ventricular dilatation in the repair of TOF, even though regurgitation increases with time. Further study is needed to determine long-term results.