Sheng-Ling Jan

Correctness of multi-detector-row computed tomography for diagnosing mechanical prosthetic heart valve disorders using operative findings as a gold standard

The purpose was to compare the findings of multi-detector computed tomography (MDCT) in prosthetic valve disorders using the operative findings as a gold standard. In a 3-year period, we prospectively enrolled 25 patients with 31 prosthetic heart valves. MDCT and transthoracic echocardiography (TTE) were done to evaluate pannus formation, prosthetic valve dysfunction, suture loosening (paravalvular leak) and pseudoaneurysm formation. Patients indicated for surgery received an operation within 1 week. The MDCT findings were compared with the operative findings. One patient with a Björk-Shiley valve could not be evaluated by MDCT due to a severe beam-hardening artifact; thus, the exclusion rate for MDCT was 3.2% (1/31). Prosthetic valve disorders were suspected in 12 patients by either MDCT or TTE. Six patients received an operation that included three redo aortic valve replacements, two redo mitral replacements and one Amplatzer ductal occluder occlusion of a mitral paravalvular leak. The concordance of MDCT for diagnosing and localizing prosthetic valve disorders and the surgical findings was 100%. Except for images impaired by severe beam-hardening artifacts, MDCT provides excellent delineation of prosthetic valve disorders.

Isolated neonatal ductus arteriosus aneurysm

OBJECTIVE A prospective study was performed to evaluate the incidence, clinical manifestations and outcome of ductus arteriosus aneurysm (DAA) in full-term neonates. BACKGROUND Ductus arteriosus aneurysm has been considered to be a rare congenital lesion and a potentially fatal abnormality. METHODS A total of 548 full-term neonates received echocardiographic screening. RESULTS There were 48 (8.8%) patients (28 boys and 20 girls) with DAA detected by echocardiography. The maximal diameter of the DAA ranged from 6.5 to 11.2 mm (8.2 +/- 1.2 mm). All cases were asymptomatic. There were no significant differences in gender, gestational age, maternal age or Apgar score between the newborns with or without DAA. Newborns with DAA had a higher birth body weight, higher incidence of maternal gestational diabetes mellitus and more mothers with blood group A, compared with newborns without DAA (p < 0.05). Follow-up echocardiograms showed spontaneous closure of the ductus arteriosus in all patients except those without DAA. The DAA became progressively smaller after ductal closure in 33 patients (70.2%) and completely disappeared by 7 to 35 days of life. The other 14 patients (29.8%) with DAA had echocardiographic evidence of progressive formation of thrombi between the third and tenth day of life. The DAA and thrombi spontaneously disappeared in all patients by one month after birth. CONCLUSIONS There is a higher incidence of DAA with a good outcome in our series compared with previous reports. We speculate that the presence of DAA may be a normal variant of the ductal bump and part of a normal process of spontaneous ductal closure in full-term neonates.

Prediction of ductus arteriosus closure by neonatal screening echocardiography

AIMS We reported the incidence, clinical manifestations and outcome of patent ductus arteriosus (PDA) in full-term infants by screening echocardiography. METHODS AND RESULTS Total 1230 infants received echocardiographic screening and periodic follow-up. On the third day of life, there were 109 infants with isolated ductus arteriosus aneurysm (DAA), those with persistent patency of the ductus arteriosus (DA) in 26. The DA closed within 48 h in 828 infants, and persisted beyond 48 h in 180. The incidence of PDA in full-term infants was 0.91, 0.83 and 0.66% at 1, 4 and 6 months follow-up, and 0.82, 0.73 and 0.64% if infants with DAA were excluded. Infants with PDA had lower birth body weight (BBW) than those with closed DA (p = 0.02). The DA width on the third day of life ranged from 0.23 to 0.43 cm (0.32+/-0.08 cm) and 0.08 to 0.44 cm (0.16+/-0.06 cm) in infants with or without PDA, respectively, which was significantly different (p < 0.05). The sensitivity, specificity, predictive positive and negative values were 87.5, 92.5, 36.8 and 99% respectively for the cuff-off point at 0.25 cm of DA width. CONCLUSIONS Risk factors in infants with PDA included lower BBW and larger measured DA width at the third day of life. We predicted the arterial duct would close if DA width <0.25 cm, and suggested follow up earlier if >0.25 cm on the third day of life.Aims: We reported the incidence, clinical manifestations and outcome of patent ductus arteriosus (PDA) in full-term infants by screening echocardiography. Methods and results: Total 1230 infants received echocardiographic screening and periodic follow-up. On the third day of life, there were 109 infants with isolated ductus arteriosus aneurysm (DAA), those with persistent patency of the ductus arteriosus (DA) in 26. The DA closed within 48 h in 828 infants, and persisted beyond 48 h in 180. The incidence of PDA in full-term infants was 0.91, 0.83 and 0.66% at 1, 4 and 6 months follow-up, and 0.82, 0.73 and 0.64% if infants with DAA were excluded. Infants with PDA had lower birth body weight (BBW) than those with closed DA (p= 0.02). The DA width on the third day of life ranged from 0.23 to 0.43 cm (0.32 ± 0.08 cm) and 0.08 to 0.44 cm (0.16 ± 0.06 cm) in infants with or without PDA, respectively, which was significantly different (p < 0.05). The sensitivity, specificity, predictive positive and negative values were 87.5, 92.5, 36.8 and 99% respectively for the cuff-off point at 0.25 cm of DA width. Conclusions: Risk factors in infants with PDA included lower BBW and larger measured DA width at the third day of life. We predicted the arterial duct would close if DA width <0.25 cm, and suggested follow up earlier if 0.25 cm on the third day of life.

Epidemiologic features of Kawasaki disease in acute stages in Taiwan, 1997–2010: Effect of different case definitions in claims data analysis

Background Kawasaki disease is the leading cause of pediatric acquired cardiac disease in many industrialized countries. The aim of this study was to estimate the incidence of Kawasaki disease in acute stages in Taiwan, by linking the diagnosis code to medication and comparing the differences in epidemiological features with those of previous reports that used the diagnosis code alone. Methods We searched the National Health Insurance Research Database from 1997 to 2010. For the International Classification of Diseases, Ninth Revision (ICD‐9) set, all inpatients with a main diagnosis of Kawasaki disease (ICD‐9, 446.1) were retrieved. For the ICD‐9 + intravenous immunoglobulin (IVIG) set, Kawasaki disease in acute stages was defined as the disease stages requiring IVIG. The epidemiologic features were calculated and compared by both methods. Results The incidence rates for children under 5 years ranged from 21.5 to 68.5 per 100,000 person‐years (average 49.1) for the ICD‐9 + IVIG set and from 48.5 to 82.8 per 100,000 person‐years (average 74.9) for the ICD‐9 set. Significant discrepancy in peak season estimation occurred in summer. The 5‐year recurrence rate was 1.1% for the ICD‐9 + IVIG set and 4.5% for the ICD‐9 set. The coronary complication rates were around 7.24% (ICD‐9 + IVIG) and 6.48% (ICD‐9). Conclusion Discrepancies occurred when different case definitions were used in claims data analysis. Previous reports might have overestimated the incidence, recurrence rate, and complication rate in older children. The new method might slightly underestimate them. The true incidence might lie in between.

Comparative effectiveness of intravenous immunoglobulin for children with Kawasaki disease: a nationwide cohort study.

Introduction Different immunoglobulin manufacturing processes may influence its effectiveness for Kawasaki disease. However, nationwide studies with longitudinal follow-up are still lacking. The aim of this study was to evaluate the comparative effectiveness of immunoglobulin preparations from a nationwide perspective. Materials and Methods This is a nationwide retrospective cohort study with a new user design. Data came from the National Health Insurance Research Database of Taiwan. From 1997 to 2008, children under 2 years old who received immunoglobulin therapy for the first time under the main diagnosis of Kawasaki disease were enrolled. The manufacturing processes were divided into β-propiolactonation, acidification and those containing IgA. The endpoints were immunoglobulin non-responsiveness, acute aneurysm, prolonged use of anti-platelets or anti-coagulants, and recurrence. Results In total, 3830 children were enrolled. β-propiolactonation had a relative risk of 1.45 (95% CI 1.08∼1.94) of immunoglobulin non-responsiveness, however, the relative risks for acidification and containing IgA were non-significant. For acute aneurysms, acidification had a relative risk of 1.49 (95% CI 1.17∼1.90), however the relative risks for β-propiolactonation and containing IgA were non-significant. For prolonged use of anti-platelets or anti-coagulants, β-propiolactonation had a relative risk of 1.44 (95% CI 1.18∼1.76), and acidification protected against them both with a relative risk of 0.82 (95% CI 0.69∼0.97), whereas the relative risk for containing IgA was non-significant. For recurrence, all three factors were non-significant. Conclusions The effectiveness of immunoglobulin may differ among different manufacturing processes. β-propiolactonation had a higher risk of treatment failure and prolonged use of anti-platelets or anti-coagulants. Acidification may increase the risk of acute coronary aneurysms.

The association between Kawasaki disease and allergic diseases, from infancy to school age.

Kawasaki disease (KD) is the most common acquired heart disease among preschool children in most industrialized countries. An atopic trend after KD illness has been observed in epidemiological studies. This is consistent with the findings of elevated IgE levels and increased IL-4 in KD patients. However, studies on the early allergic association among children with KD are still limited. This study aimed to evaluate the association between KD and allergic diseases, from infancy to school age. Allergic diseases included atopic dermatitis, allergic rhinitis (AR), asthma, and urticaria. This matched case-control study used the National Health Insurance Research Database of Taiwan as its data source. Patients born between 1997 and 2004 and with a main diagnosis of KD were retrieved for analysis. A 1:4 matched control group was selected by zip code, gender, and age. The prevalence rates and progression sequence of allergic manifestations were analyzed. During the first 5 years of life, children with KD had higher rates of allergic manifestations. Both groups have similar atopic march. In 2010, at the age of 6-13 years, there were 7072 children with KD and 27,265 children without KD. Children with KD had more AR (odds ratio [OR], 1.30; 95% confidence interval [CI], 1.22-1.38) and asthma (OR, 1.16; 95% CI, 1.05-1.27) than controls. Children with KD have a higher allergic susceptibility recognized from their 1st year of life. The atopic tendency persists until school age. Additional studies are needed to elucidate the underlying determinants of this distinct immune phenotype.

Transcatheter implantation of intravascular stents for postoperative residual stenosis of peripheral pulmonary artery stenosis.

This is a prospective study of transcatheter implantation of 11 intravascular stents in 7 patients with status/post (S/P) surgical correction of major cardiovascular lesions. The safety and efficacy of balloon-expandable stents for treatment of peripheral pulmonary artery stenosis (PPAS) is evaluated and analyzed. Although the transcatheter implantation of intravascular stents has been reported as a possible treatment for stenotic peripheral pulmonary arteries, the results of intermediate follow-up studies on patients with S/P surgical correction for residual PPAS need to be evaluated. From June 1998 to December 2001, a total of 15 patients with PPAS having S/P surgery for major cardiovascular lesions were enrolled in this study. Eight of them had redo surgery after complete evaluation and the other 7 patients who might be at higher risk of mortality or morbidity from redo surgery, underwent transcatheter implantation of stents to dilate significant PPAS. Tetralogy of Fallot, S/P total correction, was done in 6 and transposition of great vessels, S/P Jatene operation, was done in 1. There were 10 stents (P 308 Palmaz stent ×8 and Intrastent™ ×2) implantation for 10 sites of the stenotic PPAS in these 7 patients, who were aged from 3.6 to 17.3 (10.1 ±5.6) years and had body weights ranging from 17 to 72.5 (37.1 ±23.0) kg. The narrowest diameter of the stenotic peripheral pulmonary arteries and pressure gradients across the stenosis were measured before and after implantation of stents. A follow-up catheterization and pulmonary angiography was performed 1 year later to evaluate the intermediate efficacy of stents implantation. All the stenotic peripheral pulmonary arteries of these 7 patients had a significant reduction of pressure gradients immediately after the procedure. The narrowest mean diameter of pulmonary arteries increased from 6.7 ±3.4 to 11.3 ±3.0 mm (p<0.001), and the mean pressure gradient dropped from 31 ±9.9 to 11.4 ±4.6 mm Hg (p<0.001). The follow-up catheterization 1 year later revealed a persistent effect in all but 1 patient. Only a young male presented with a recurrent stenosis with a pressure gradient of ≥20 mm Hg, which was relieved by redilation with implantation of another stent. There was no immediate or intermediate complication. Transcatheter stent implantation for treatment of a significant residual PPAS after surgical correction of complicated congenital heart disease is a safe and effective procedure. Since children are growing with age, a long-term follow-up study to evaluate the effects and possible problems of stent implantation is mandatory.

Dislodgment of Port-A-Cath Catheters in Children

BACKGROUND Port-a-cath catheters are frequently used in children with malignancies. Their dislodgment is rare, but carries potentially serious risks. This study analyzed our 11-year experience of this important issue. METHODS Between June 1997 and January 2008, 290 ports of different brands were implanted in children by pediatric surgeons. Among the patients, 12 children with catheter dislodgement were retrospectively studied. Their ages ranged from 2-16 years, with a median of 6.4 years. Their body weights ranged from 12-39 kg, with a median of 20 kg. Ten patients presented with a port-a-cath dysfunction, while the other two patients were identified incidentally during surgery for removal of their ports. RESULTS The downstream ends of dislodged catheters were located in the right ventricle (five patients), right atrium (four), main pulmonary artery (one), left pulmonary artery (one) and right pulmonary artery (one). Eleven catheters were broken, and one catheter was disconnected from the port. Most (10/11) catheters were broken at the site of anastomosis to the port. All dislodged catheters were successfully retrieved without complications by transcatheter retrieval using a gooseneck snare. CONCLUSION The dislodgment rate of port-a-cath catheters in children in our series was 4.1%. Most (83%) catheters were broken at the site of anastomosis to the port. All dislodged catheters could be successfully retrieved by transcatheter retrieval using a gooseneck snare.

Transcatheter Therapy of Lutembacher Syndrome

Lutembacher syndrome is a combination of congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). The combination of these 2 diseases has hemodynamic influences on each other and the degree of MS may be underestimated. Traditionally, Lutembacher syndrome is corrected by surgical treatment. Nowadays, these 2 diseases are amenable to transcatheter treatment without the need for surgery. Here, we describe a 28-year-old female with Lutembacher syndrome who benefited from combined transcatheter therapy of balloon valvuloplasty for MS and device closure for ASD with an Amplatzer septal occluder.

Congestive heart failure as presentation of acute lymphoblastic leukaemia with eosinophilia

A previously healthy 17-year-old girl presented with the sudden onset of chest tightness accompanied by dyspnoea on exertion. Physical examination showed splinter haemorrhages and a grade III/VI systolic murmur at the left sternal border with no radiation. Laboratory data showed a white cell count of 49AE8 · 10/l with 63% eosinophils (absolute eosinophil count of 31AE4 · 10/l). The eosinophils showed no vacuolation or degranulation. Biochemical studies showed normal levels of creatine kinase and creatine kinase MB, but an elevated troponin-I of 2AE19 (normal <0AE034 ng/ml). Transthoracic echocardiography in the parasternal long-axis view disclosed an intracardiac thrombus (arrows), seen attached to the mitral valve at the left ventricular posterior wall, associated with mild mitral regurgitation (left panel: AO, aorta; LA, left atrium; LV, left ventricle, RV, right ventricle). The multidetector-row computed tomography image disclosed diffuse mural thrombus that occurred more in association with the posterior wall (arrows, right panel). A bone marrow biopsy demonstrated precursor B lymphoblastic leukaemia with 40% lymphoblasts and an increase of eosinophils and their precursors. Cytogenetic analysis of the bone marrow aspirate showed t(3;6)(p22;p24). Further studies relevant to a hypereosinophilic disorder disclosed normal levels of interleukin 5, no FIP1L1-PDGFRA fusion gene and negative stool examination for parasites and ova. The patient received chemotherapy according to a high-risk protocol for acute lymphoblastic leukaemia, which resulted in bone marrow remission and a return of the peripheral eosinophil count to normal by 8 weeks. Subsequent echocardiography showed resolution of the left ventricular endocardial thrombosis.