Serial evaluation of myocardial function using the myocardial performance index in Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myocarditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV) dysfunction in patients with KD based on the myocardial performance index (MPI) using pulse Doppler (PD) and tissue Doppler imaging (TDI), from the acute to convalescent phases. METHODS: We retrospectively studied 89 children diagnosed with KD from January 2010 to August 2012. We assessed the presence of coronary artery lesions (CALs) and the LV ejection fraction, PD-MPI, and TDI-MPI at diagnosis, and 2, 14, and 56 days after intravenous immunoglobulin (IVIG) treatment. We enrolled 70 healthy children as a control group. RESULTS: The ejection fraction in patients with KD at diagnosis (67.3 ± 0.9%) was lower than that in the control group (69.8 ± 0.8%, P = 0.035), and the LV TDI-MPIs for patients with KD at diagnosis (0.49 ± 0.01) and 2 days after IVIG treatment (0.48 ± 0.01) were higher than those in the control group (0.45 ± 0.01, P = 0.002, P = 0.033, respectively). No significant differences were found in the LV dysfunction between the patients with complete and incomplete KD. Septal TDI-MPIs in patients with KD with CAL at diagnosis (0.52 ± 0.02) were higher than those in patients with KD without CAL (0.47 ± 0.01, P = 0.019). CONCLUSIONS: Transient LV dysfunction occurred in patients with complete and incomplete KD in the acute stage. In patients with KD with CAL at diagnosis, the LV dysfunction was more prominent. The PD-MPI and TDI-MPI are useful parameters for assessing LV function in patients with KD.

Differences in perioperative femoral and radial arterial blood pressure in neonates and infants undergoing cardiac surgery requiring cardiopulmonary bypass / Diferenças na pressão arterial femoral e radial no perioperatório em neonatos e bebês submetidos a cirurgia cardíaca com bypass cardiopulmonar

Abstract Objective: Several reports claim that blood pressure (BP) in the radial artery may underestimate the accurate BP in critically ill patients. Here, the authors evaluated differences in mean blood pressure (MBP) between the radial and femoral artery during pediatric cardiac surgery to determine the effectiveness of femoral arterial BP monitoring. Method: The medical records of children under 1 year of age who underwent open-heart surgery between 2007 and 2013 were retrospectively reviewed. Radial and femoral BP were measured simultaneously, and the differences between these values were analyzed at various times: after catheter insertion, after the initiation of cardiopulmonary bypass (CPB-on), after aortic cross clamping (ACC), after the release of ACC, after weaning from CPB, at arrival in the intensive care unit (ICU), and every 6 h during the first day in the ICU. Results: A total of 121 patients who underwent open-heart surgery met the inclusion criteria. During the intraoperative period, from the beginning to the end of CPB, radial MBPs were significantly lower than femoral MBPs at each time-point measured (p < 0.05). Multivariate analysis showed that longer CPB time (>60 min, odds ratio: 7.47) was a risk factor for lower radial pressure. However, discrepancies between these two values disappeared after arrival in the ICU. There was no incidence of ischemic complications associated with the catheterization of both arteries. Conclusion: The authors suggest that femoral arterial pressure monitoring can be safely performed, even in neonates, and provides more accurate BP values during CPB-on periods, and immediately after weaning from CPB, especially when CPB time was greater than 60 min.

Resumo Objetivo: Diversos relatos alegam que a pressão arterial (PA) na artéria radial poderá subestimar a PA precisa em pacientes gravemente doentes. Aqui, avaliamos diferenças na pressão arterial média (PAM) entre a artéria radial e femoral durante cirurgia cardíaca pediátrica para determinar a eficácia do monitoramento da PA da artéria femoral. Método: Realizamos uma análise retrospectiva de prontuários médicos de crianças com menos de 1 ano de idade submetidas a cirurgia de coração aberto entre 2007 e 2013. As PAs radial e femoral foram auferidas simultaneamente, as diferenças entre esses valores foram analisadas diversas vezes: após a inserção do cateter, após o início do bypass cardiopulmonar (CPB-on), após pinçamento cruzado da aorta (ACC), após a liberação do ACC, após desmame do CPB, na entrada na unidade de terapia intensiva (UTI) e a cada 6 horas durante o primeiro dia na unidade de terapia intensiva (UTI). Resultados: Um total de 121 pacientes submetidos a cirurgia de coração aberto atenderam aos nossos critérios de inclusão. Durante o transoperatório, do início ao término do CPB, as PAMs da artéria radial foram significativamente menores do que as PAMs da artéria femoral em cada ponto de medição (p < 0,05). A análise multivariada mostrou que a duração mais longa do CPB (> 60 minutos, Razão de Chance = 7,47) representou um fator de risco de pressão radial mais baixa. Contudo, as diferenças entre esses dois valores desapareceram após a entrada na UTI. Não houve incidência de complicações isquêmicas associadas à cateterização de ambas as artérias. Conclusão: Sugerimos que o monitoramento da pressão arterial femoral pode ser realizado com segurança, mesmo em neonatos, e fornece valores da PA mais precisos durante períodos de CPBon e imediatamente após o desmame do CPB, principalmente nos casos em que a duração do CPB foi superior a 60 minutos.

Differences in perioperative femoral and radial arterial blood pressure in neonates and infants undergoing cardiac surgery requiring cardiopulmonary bypass.

OBJECTIVE: Several reports claim that blood pressure (BP) in the radial artery may underestimate the accurate BP in critically ill patients. Here, the authors evaluated differences in mean blood pressure (MBP) between the radial and femoral artery during pediatric cardiac surgery to determine the effectiveness of femoral arterial BP monitoring. METHOD: The medical records of children under 1 year of age who underwent open-heart surgery between 2007 and 2013 were retrospectively reviewed. Radial and femoral BP were measured simultaneously, and the differences between these values were analyzed at various times: after catheter insertion, after the initiation of cardiopulmonary bypass (CPB-on), after aortic cross clamping (ACC), after the release of ACC, after weaning from CPB, at arrival in the intensive care unit (ICU), and every 6h during the first day in the ICU. RESULTS: A total of 121 patients who underwent open-heart surgery met the inclusion criteria. During the intraoperative period, from the beginning to the end of CPB, radial MBPs were significantly lower than femoral MBPs at each time-point measured (p<0.05). Multivariate analysis showed that longer CPB time (>60min, odds ratio: 7.47) was a risk factor for lower radial pressure. However, discrepancies between these two values disappeared after arrival in the ICU. There was no incidence of ischemic complications associated with the catheterization of both arteries. CONCLUSION: The authors suggest that femoral arterial pressure monitoring can be safely performed, even in neonates, and provides more accurate BP values during CPB-on periods, and immediately after weaning from CPB, especially when CPB time was greater than 60min.

Rapid Regression of Obstructive Cardiac Rhabdomyoma in a Preterm Neonate after Sirolimus Therapy.

Cardiac rhabdomyoma can be subclinical or fatal depending on the onset age, involving site, and the size and degree of invasion. Although most rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction induces hemodynamic instability. Mammalian target of rapamycin (mTOR) inhibitors have been used to treat neonates and children with hemodynamically obstructive cardiac rhabdomyoma. Herein, we report a premature neonate at the gestational age of 30 + 4 weeks with severe left ventricular outflow tract obstructive cardiac rhabdomyoma who was successfully treated with the mTOR inhibitor sirolimus. To the best of our knowledge, this is the first recorded case of a premature neonate with obstructive cardiac rhabdomyoma who was successfully treated with an mTOR inhibitor. Therefore, sirolimus could be considered as an alternative medical option for managing premature neonates with obstructive cardiac rhabdomyoma.

Hashimoto thyroiditis with an unusual presentation of cardiac tamponade in Noonan syndrome.

Noonan syndrome is an autosomal dominant, multisystem disorder. Autoimmune thyroiditis with hypothyroidism is an infrequent feature in patients with Noonan syndrome. A 16-year-old boy was admitted because of chest discomfort and dyspnea; an echocardiogram revealed pericardial effusion. Additional investigations led to a diagnosis of severe hypothyroidism due to Hashimoto thyroiditis. The patient was treated with L-thyroxine at 0.15 mg daily. However, during admission, he developed symptoms of cardiac tamponade. Closed pericardiostomy was performed, after which the patient's chest discomfort improved, and his vital signs stabilized. Herein, we report a case of an adolescent with Noonan syndrome, who was diagnosed with Hashimoto thyroiditis with an unusual presentation of cardiac tamponade.

Clinical Utility of Echocardiography for the Diagnosis and Prognosis in Children with Bronchopulmonary Dsyplasia.

BACKGROUND: Bronchopulmonary dysplasia (BPD) may result in chronic pulmonary artery hypertension and right ventricular (RV) dysfunction. Various echocardiographic assessments of RV dysfunction have been used to determine whether echocardiographic measurements of premature infants with BPD could provide sensitive measures of RV function that correlates with BPD severity. METHODS: Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and tissue Doppler imaging (TDI) measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and TDI measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. RESULTS: None of the standard echocardiographic findings was significantly different between the control group and BPD groups. However, mean septal TDI-MPI of the severe BPD group (0.68 ± 0.06) was significantly (p < 0.01) higher than that of the non-BPD (0.58 ± 0.10) or the mild BPD group (0.59 ± 0.12). In addition, mean RV TDI-MPI of the severe BPD group (0.71 ± 0.13) was significantly (p < 0.05) higher than that of the non-BPD group (0.56 ± 0.08) or the mild BPD group (0.60 ± 0.125). Linear regression showed a good correlation between the severity of BPD and RV TDI-MPI (p = 0.01, R = 0.30) or septal TDI-MPI (p = 0.04, R = 0.24). CONCLUSION: Echocardiographic evaluation of RV function based on an assessment of RV TDI-MPI can provide RV dysfunction parameter in premature infants with BPD.

Radiodensity on serial chest X-rays for the diagnosis of foreign body aspiration in children.

OBJECTIVE: To evaluate the utility of measuring lung radiodensity from chest X-ray for the diagnosis of foreign body aspiration. METHODS: Records of 59 children with foreign body aspiration were retrospectively reviewed. Lung radiodensity and radiodensity ratio (right/left lung radio density) before and after foreign body removal were measured. Radiodensity was calculated as the relative score compared with the tenth thoracic vertebra body (100 points) and the background (0 point). The change of radiodensity ratio (difference in radiodensity ratio of the second X-ray from that of first X-ray) was compared between 22 patients (foreign body group) and 22 normal subjects (control group). RESULTS: In the group of foreign body in the left bronchus, the mean (SD) radiodensity of the left lung [53.5 (12.8)] was lower than that of the right lung [60.8 (7.7), P<0.01] and it increased after foreign body removal [60.0 (6.9), P=0.02]. The radiodensity ratio decreased from 1.20 (0.30) to 0.96 (0.09) (P<0.01) after foreign body removal. In the group with a foreign body in the right bronchus, the radiodensity of the right lung [51.8 (12.8)] was lower than that of left lung [62.0 (11.7), P=0.03], and it also increased after foreign body removal [58.4 (9.6), P=0.03]. The change of radiodensity ratio in the foreign body group [15.7 (17.8)%] was higher than the control group [5.4 (4.3) %, P=0.01] and the cutoff value was 7.5%. CONCLUSIONS: Radiodensity from chest X-ray could be a useful tool for diagnosing foreign body aspiration in children.

Nationwide survey of coronary aneurysms with diameter >6 mm in Kawasaki disease in Korea.

BACKGROUND: We investigated the course of coronary aneurysm with diameter > 6 mm in Kawasaki disease (KD), as well as related therapeutic trends and prognosis in Korea. METHODS: A nationwide questionnaire survey was carried out in 77 hospitals, to investigate, retrospectively, patients with KD who had coronary aneurysms with a diameter > 6 mm between 1990 and 2011. RESULTS: The median age of onset was 3 years (range, 2 months-16 years) in a total of 239 patients. During the acute stage of KD, most patients received i.v. immunoglobulins and aspirin. In addition, 27 patients received steroid therapy. In the current study, the mean coronary aneurysm size was 8.7 ± 3.2 mm (range, 6-21 mm). Twenty-two patients underwent interventional catheterization. Procedures included percutaneous transluminal coronary balloon angioplasty (n = 10), stent placement (n = 9), and percutaneous transluminal coronary rotational ablation (n = 3). Fourteen patients underwent coronary artery bypass graft surgery. Of the 239 patients who had coronary aneurysms with diameter > 6 mm, 13 (5.4%) presented with findings suggestive of myocardial infarction. Five patients died during the follow-up period. CONCLUSIONS: Severe stenosis or occlusion of the coronary artery may occur in some patients who develop coronary aneurysms with diameter > 6 mm; early management such as coronary interventions or surgery should be considered in such cases.

Chemical pleurodesis using a Viscum album extract in infants with congenital chylothorax.

UNLABELLED: Congenital chylothorax is a rare condition, but it is the most common cause of pleural effusion in neonates and infants. Here, we report on the first trials of the intrapleural instillation of an extract of Viscum album (European mistletoe) (Abnobaviscum Q®) in two infants with congenital chylothorax that was refractory to standard conservative management and thoracic duct ligation. CONCLUSION: The clinical course of both children improved with no side effects related to the extract after following up the children for 2 years and 9 months, respectively. Randomized multicenter prospective studies will help determine the effectiveness of pleurodesis with a V. album extract and the occurrence of long-term side effects with this agent.

Timing in resolution of left heart dilation according to the degree of mitral regurgitation in children with ventricular septal defect after surgical closure / Momento da resolução da dilatação do coração esquerdo segundo o grau de regurgitação mitral em crianças submetidas a fechamento cirúrgico de defeito do septo ventricular

OBJECTIVE: Children with ventricular septal defects (VSD) can have chronic volume overload, which can result in changes of left heart echocardiographic parameters. To evaluate the changes before and after surgical closure, the children were divided into three groups according to the degree of mitral regurgitation (MR), and their echocardiographic characteristics were reviewed at serial follow-up after surgical closure. METHODS: The preoperative, and one-, three-, and 12-month postoperative echocardiographic data of 40 children who underwent surgical closure of VSD were retrospectively reviewed. Left ventricular end-diastolic volume (LVEDV), left ventricular end-diastolic dimension (LVEDD), left ventricular end-systolic dimension (LVESD), mitral valvular characteristics, including degree of MR and mitral valve annulus, and left atrial (LA) characteristics, including volume and dimensions, were observed. RESULTS: Preoperative LVEDV, LVEDD, LVESD, mitral valvular annulus, LA volume, and LA dimensions were significantly larger in children with MR. Additionally, there were significant decreases in LVEDV, LVEDD, LA volume, and LA dimensions at one, three, and 12 months postoperatively. The degree of MR also improved to a lower grade after surgical closure of the VSD without additional mitral valve repair. CONCLUSION: The echocardiographic parameters of left heart dilation and MR in children with VSD improved within the first year after surgical closure without additional mitral valve repair. Furthermore, in all of the patients with VSD, regardless of MR, LA dilation was reduced within three months after surgical closure of the VSD; however, LV and mitral valve annular dilatation decreased within 12 months. .

OBJETIVO: Crianças com defeito do septo ventricular (DSV) podem apresentar sobrecarga devolume crônica, que pode resultar em mudanças nos parâmetros ecocardiográficos do curacao esquerdo. Para avaliar as mudanças antes e depois do fechamento cirúrgico, as crianças foram divididas em 3 grupos segundo o grau de regurgitação mitral (RM) e suas características eco-cardiográficas foram analisadas com acompanhamento em série após o fechamento cirúrgico. MÉTODO: Revisamos retrospectivamente os dados ecocardiográficos de 40 crianças submetidas afechamento cirúrgico de DSV antes da cirurgia e nos meses 1, 3 e 12 após a cirurgia. Observamos o volume diastólico final do ventrículo esquerdo (VDFVE), dimensão diastólica final do ventrículo esquerdo (DDFVE) e dimensão sistólica final do ventrículo esquerdo (DSFVE), características da válvula mitral, incluindo grau de RM e o anel da válvula mitral, e características do átrio esquerdo (AE), incluindo volume e dimensões. RESULTADOS: Os resultados para VDFVE, DDFVE, DSFVE, anel da válvula mitral, volume do AE e dimensões do AE foram significativamente maiores em crianças com RM. Além disso, não houveredução significativa no VDFVE, DDFVE, volume do AE e nas dimensões do AE nos meses 1, 3e 12 após a cirurgia. O grau de RM também apresentou melhoria para um grau menor após o fechamento cirúrgico do DSV sem reparo adicional da válvula mitral. CONCLUSÃO: Os parâmetros ecocardiográficos de dilatação do coração esquerdo e a RM em crianças com DSV haviam apresentado melhora no primeiro ano após o fechamento cirúrgicos em reparo adicional da válvula mitral. Além disso, em todos os pacientes com DSV, independentemente ...

Timing in resolution of left heart dilation according to the degree of mitral regurgitation in children with ventricular septal defect after surgical closure.

OBJECTIVE: Children with ventricular septal defects (VSD) can have chronic volume overload, which can result in changes of left heart echocardiographic parameters. To evaluate the changes before and after surgical closure, the children were divided into three groups according to the degree of mitral regurgitation (MR), and their echocardiographic characteristics were reviewed at serial follow-up after surgical closure. METHODS: The preoperative, and one-, three-, and 12-month postoperative echocardiographic data of 40 children who underwent surgical closure of VSD were retrospectively reviewed. Left ventricular end-diastolic volume (LVEDV), left ventricular end-diastolic dimension (LVEDD), left ventricular end-systolic dimension (LVESD), mitral valvular characteristics, including degree of MR and mitral valve annulus, and left atrial (LA) characteristics, including volume and dimensions, were observed. RESULTS: Preoperative LVEDV, LVEDD, LVESD, mitral valvular annulus, LA volume, and LA dimensions were significantly larger in children with MR. Additionally, there were significant decreases in LVEDV, LVEDD, LA volume, and LA dimensions at one, three, and 12 months postoperatively. The degree of MR also improved to a lower grade after surgical closure of the VSD without additional mitral valve repair. CONCLUSION: The echocardiographic parameters of left heart dilation and MR in children with VSD improved within the first year after surgical closure without additional mitral valve repair. Furthermore, in all of the patients with VSD, regardless of MR, LA dilation was reduced within three months after surgical closure of the VSD; however, LV and mitral valve annular dilatation decreased within 12 months.

Total anomalous pulmonary venous return in siblings.

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.

Procalcitonin levels in patients with complete and incomplete Kawasaki disease.

Incomplete Kawasaki disease (iKD) is considered to be a less complete form of Kawasaki disease (cKD), and several differences in the laboratory presentations of iKD and cKD have been noted. We investigated serum procalcitonin levels in patients with iKD, cKD, and other febrile diseases (a control group). Seventy-seven patients with cKD, 24 with iKD, and 41 controls admitted to our hospital from November 2009 to November 2011 were enrolled in the present study. We obtained four measurements of serum procalcitonin levels and those of other inflammatory markers from each patient. Samples were taken for analysis on the day of diagnosis (thus before treatment commenced; D0) and 2 (D2), 14 (D14), and 56 days (D56) after intravenous immunoglobulin infusion. We obtained control group data at D0. The mean D0 serum procalcitonin levels of cKD patients (0.71 ± 1.36 ng/mL) and controls (0.67 ± 1.06 ng/mL) were significantly higher than those of iKD patients (0.26 ± 0.26 ng/mL) (P = 0.014 and P = 0.041, resp.). No significant difference in mean procalcitonin level was evident among groups at any subsequent time. In conclusion, the serum procalcitonin level of patients with acute-stage cKD was significantly higher than that of iKD patients.

Right ventricular failure in congenital heart disease.

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling. Pressure-overload RV failure caused by RV outflow tract obstruction after total correction of tetralogy of Fallot, pulmonary stenosis, atrial switch operation for transposition of the great arteries, congenitally corrected transposition of the great arteries, and systemic RV failure after the Fontan operation. Volume-overload RV failure may be caused by atrial septal defect, pulmonary regurgitation, or tricuspid regurgitation. Although the measurement of RV function is difficult because of many reasons, the right ventricle can be evaluated using both imaging and functional modalities. In clinical practice, echocardiography is the primary mode for the evaluation of RV structure and function. Cardiac magnetic resonance imaging is increasingly used for evaluating RV structure and function. A comprehensive evaluation of RV function may lead to early and optimal management of RV failure in patients with CHD.

Right ventricular myocardial performance index is decreased with severe pressure-overload cardiac hypertrophy in young rats.

Although the right ventricular (RV) myocardial performance index (MPI) usually is increased in the presence of RV dysfunction and pressure overload, debate continues over the correlation between the RV MPI and functional derangement in patients with RV pressure-overload congenital heart disease (CHD). To address this controversy, this study took serial measurements of the RV MPI in addition to invasive RV hemodynamic measurements during the acute stage of mild to severe pressure overload. Right ventricle pressure overload was induced by partial pulmonary arterial banding (PAB) in 3-week-old rats. The rats were divided into two groups: mild pulmonary stenosis (PS) group (20-40 % stenosis; n = 20) and severe PS group (40-70 % stenosis; n = 28). Sham-treated animals (sham group; n = 30) underwent the same surgical procedure without PAB. Pressure-overload RV hypertrophy was documented by weighing the heart, by evaluating echocardiograms, and by evaluating cardiac hypertrophy-associated gene expression. The RV MPI was checked 1, 2, 3, 5, and 8 weeks after PAB. The MPI was calculated as the sum of the isovolumic contraction time and the isovolumic relaxation time (IRT) divided by the ejection time. The RV MPI of the mild PS group did not differ significantly from that of the sham group. The RV MPI of the severe PS group, however, was lower than that of the sham group (0.27 ± 0.01 vs 0.29 ± 0.01) 2 to 8 weeks after PAB: 0.19 ± 0.01 at 2 weeks (P < 0.001), 0.16 ± 0.01 at 3 weeks (P < 0.001), 0.20 ± 0.01 at 5 weeks (P = 0.021), and 0.18 ± 0.01 at 8 weeks (P < 0.001) after PAB. The decreased RV MPI was associated with decreased IRT and increased ejection time. RV hypertrophy contributes to the decrease in the RV MPI in the severe pressure-overload condition.

Transvenous proximal closure of large congenital coronary arteriovenous fistula using the single Amplatzer vascular plug in a 3-year-old girl.

Congenital coronary arteriovenous fistulas (CCAFs) are rare coronary artery abnormalities in which blood is shunted into a cardiac chamber or great vessel. If the fistula itself is large and tortuous, it is generally recommended to occlude the fistula to prevent several complications. In approaches of transcatheter occlusion, the transvenous approach is preferred over the transarterial approach. The transvenous approach would enable the cannulation of a relatively larger catheter or sheath without potential damage to the femoral vessels or normal coronary arteries, which can occur in the transarterial approach. The transvenous approach may also minimize the blind pouch after releasing the devices. Herein, we report the success of transvenous proximal closure of a CCAF using an Amplatzer vascular plug (AVP) in a 3-year-old patient with cardiomegaly. Complete occlusion was achieved by a single AVP and thrombus formation of the distal aneurysmal portion of the fistula. We suggest that this strategy of closing the proximal end with a dilated fistula using a single AVP by the transvenous approach may be a good option in treating CCAFs in a young child.

Recurrent plastic bronchitis in a child with 2009 influenza A (H1N1) and influenza B virus infection.

Plastic bronchitis is an uncommon disorder characterized by the formation of bronchial casts. It is associated with congenital heart disease or pulmonary disease. In children with underlying conditions such as allergy or asthma, influenza can cause severe plastic bronchitis resulting in respiratory failure. A review of the literature showed nine cases of plastic bronchitis with H1N1 including this case. We report a case of a child with recurrent plastic bronchitis with eosinophilic cast associated with influenza B infection, who had recovered from plastic bronchitis associated with an influenza A (H1N1) virus infection 5 months previously. To the best of our knowledge, this is the first case of recurrent plastic bronchitis related to influenza viral infection. If patients with influenza virus infection manifest acute respiratory distress with total lung atelectasis, clinicians should consider plastic bronchitis and early bronchoscopy should be intervened. In addition, management for underlying disease may prevent from recurrence of plastic bronchitis.

Mosaic ring chromosome 6 in an infant with significant patent ductus arteriosus and multiple congenital anomalies.

The clinical features of ring chromosome 6 include central nervous system anomalies, growth retardation, facial dysmorphism and other congenital anomalies. Ring chromosome 6 occurs rarely and manifests as various phenotypes. We report the case of mosaic ring chromosome 6 by conventional karyotyping in a 7-day-old male infant diagnosed with a large patent ductus arteriosus (PDA) with hypoplasia of aortic valve and aortic arch. These have not been previously reported with ring chromosome 6. He recovered from heart failure symptoms after ligation of the PDA. He showed infantile failure to thrive and delayed milestone in a follow-up evaluation. To the best of our knowledge, this is the first report of a Korean individual with ring chromosome 6 and hemodynamically significant PDA.

Partial anomalous pulmonary venous connection with intact atrial septum in a child with ventricular septal defect: a case report.

Partial anomalous pulmonary vein connection (PAPVC) is a rare congenital abnormal cardiac defect involving the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Ninety percent of PAPVCs are accompanied by atrial septal defect (ASD). To our knowledge, there is no previous report of PAPVC with ventricular septal defect (VSD) without ASD in Korea, and in this paper, we report the first such case. A 2-day-old girl was admitted into the Chonnam National University Hospital for evaluation of a cardiac murmur. An echocardiogram revealed perimembranous VSD without ASD. She underwent patch closure of the VSD at 5 months of age. Although the VSD was completely closed, she had persistent cardiomegaly with right ventricular volume overload, as revealed by echocardiography. Three years later, cardiac catheterization and chest computed tomography revealed a PAPVC, with the right upper pulmonary vein draining into the right SVC. Therefore, correction of the PAPVC was surgically performed at 3 years of age. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, but without ASD.

Clinical significance of pleural effusion in the new influenza A (H1N1) viral pneumonia in children and adolescent.

BACKGROUND: Parapneumonic effusion has been reported to develop either in typical bacterial infection or in viral pneumonia with bacterial co-infection and to cause death. Swine-origin influenza A (H1N1) virus infection can be accompanied with pleural effusion; however, there are no reports about the significance of pleural effusion in H1N1 pneumonia. We retrospectively analyzed both the clinical characteristics and the significance of pleural effusion associated with H1N1 pneumonia in children and adolescent. METHOD: Eighty-nine patients who were admitted with H1N1 pneumonia were divided into two groups: 17 patients with pleural effusion (i.e., the effusion group), and 72 patients without pleural effusion (the non-effusion group). RESULTS: Lymphopenia (P = 0.030), elevation of the C-reactive protein (P = 0.026), and positive rate of anti-sptreptolysin O titer (P = 0.040) were significantly increased in the effusion group than in the non-effusion group. In addition, the need for treatment with both oxygen (P < 0.001) and oseltamivir (P = 0.013) was significantly increased in the effusion group. However, there was no significant difference between the two investigated groups in the duration of the treatment with intravenous antibiotics, the time of fever remission calculated from admission, and the days of hospital stay. Also, there was no documented bacterial co-infection in any of the studied groups. CONCLUSION: This result suggested that pleural effusion in H1N1 pneumonia could develop without bacterial co-infection and had mild clinical course.