Differential Adaptation of Biventricular Myocardial Kinetic Energy in Patients With Repaired Tetralogy of Fallot Assessed by MR Tissue Phase Mapping.

BACKGROUND: The myocardial kinetic energy (KE) and its association with pulmonary regurgitation (PR) have yet to be investigated in repaired tetralogy of Fallot (rTOF) patients. PURPOSE: To evaluate the adaptation of myocardial KE in rTOF patients by tissue phase mapping (TPM). STUDY TYPE: Prospective. POPULATION: A total of 49 rTOF patients (23 ± 5 years old; male = 32), 47 normal controls (22 ± 1 year old; male = 29). FIELD STRENGTH/SEQUENCE: 3-T/2D dark-blood three-directional velocity-encoded gradient-echo sequence. ASSESSMENT: Left and right ventricle (LV, RV) myocardial KE in radial (KEr ), circumferential (KEø ), longitudinal (KEz ) directions. The proportions of KE in each direction to the sum of all KE (KErøz ): %KEr , %KEø , %KEz . PR fraction. STATISTICAL TEST: Student's t test, multivariable regression. Statistical significance: P < 0.05. RESULTS: In rTOF group, LV KEz remained normal in systole (P = 0.565) and diastole (P = 0.210), whereas diastolic LV %KEz (62% ± 14% vs. 72% ± 7%) and systolic LV %KEø (9% ± 6% vs. 20% ± 7%) were significantly decreased. The KEr and %KEr of both ventricles significantly increased in the rTOF group (RV in diastole: 6 ± 3 vs. 3 ± 1 µJ and 54% ± 13% vs. 27% ± 7%). The rTOF group exhibited significantly higher RV/LV ratios of %KEr (systole: 1.3 ± 0.3 vs. 1.0 ± 0.3) and %KEø (systole: 1.6 ± 0.8 vs. 1.0 ± 0.3) and significantly lower ratios of %KEz in systole (0.7 ± 0.2 vs. 1.0 ± 0.1) and diastole (0.5 ± 0.2 vs. 0.9 ± 0.1). In multivariable regression analysis, the RV peak systolic KErøz , RV systolic KEz , and LV diastolic %KEø were independently associated with PR fraction in the rTOF group (adjusted R2  = 0.479). DATA CONCLUSION: In rTOF patients, the adaptation of the KE proportion occurred earlier than that of the KE amplitude, and the biventricular balance of %KE was disrupted. PR may cause differential KE adaptation in RV and LV. TPM-derived KE may be useful in investigation of myocardial adaptation in rTOF patients. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 3.

Comparable long-term results of the extracardiac conduit total cavopulmonary connection in heterotaxy to non-heterotaxy patients.

BACKGROUND: Fontan operation in heterotaxy patients has been associated with high mortality. We studied whether adoption of the extracardiac conduit (EC) total cavopulmonary connection (TCPC) in heterotaxy demonstrated comparable results to non-heterotaxy population. METHODS: A retrospective medical record review of 35 consecutive patients with heterotaxy and 70 consecutive patients without heterotaxy syndrome who underwent EC TCPC between 2000 and 2018 was performed. RESULTS: In the 35 heterotaxy patients, 30 were right and 5 were left atrial isomerism. Anomalies of venous return included bilateral superior vena cava in 20 (57.1%), separated hepatic vein in 8 (22.9%), interrupted inferior vena cava in 3 (8.6%), total anomalous pulmonary venous return in 7 (20%), and partial in 2 patients (5.7%). All patients underwent EC TCPC under beating-heart cardiopulmonary bypass except in four patients (11.4%) cardioplegic arrest was needed for cardiac repair. The surgical mortality rate was lower in heterotaxy patients (0% vs. 5.7%; p = 0.299) but statistically not significant. The follow-up ranged from 2 months to 17.8 years (mean 9.4 ± 5.6 years). At 15 years, there was no significant difference between the heterotaxy and non-heterotaxy patients regarding the long-term survival (70% vs. 78.6%; p = 0.443), freedom from reoperation (81.9% vs. 96.5%; p = 0.057), and postoperative arrhythmia (17.1% vs. 7.1%; p = 0.174). CONCLUSIONS: EC TCPC can be performed in heterotaxy patients with comparable early and late results to the non-heterotaxy population. However, the late morbidities regarding the Fontan circulation needs careful follow-up.

Arterial stiffness late after Kawasaki disease in children: Assessment by performing brachial-ankle pulse wave velocity.

BACKGROUND: Whether low-risk Kawasaki disease (KD) patients are at increased risk of cardiovascular disease later in life remains controversial. The purpose of this study is to examine the arterial stiffness and exercise performance of KD patients in chronic stage. METHODS: This study included 158 subjects. They were divided into three groups: 37 KD patients with regressed coronary artery lesions (CALs) (M/F 23/14, 13.6 ± 6.5 years) (group I), 43 KD patients without CALs (M/F 26/17, 13.9 ± 6.2 years) (group II), and 78 age- and gender-matched normal controls (M/F 44/34, 13.2 ± 6.9 years) (group III). They all underwent brachial-ankle pulse wave velocity (baPWV), an exercise test, and blood sampling to measure the levels of high-sensitivity C-reactive protein (hs-CRP), triglycerides (TG), high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, and total cholesterol (TC). The differences among the groups were compared. RESULTS: There were significant differences among the three groups in terms of right and left baPWV (p < 0.01 respectively), HDL level (p < 0.05), TC/HDL ratio (p < 0.05), and oxygen consumption (VO2) peak (p < 0.05). Moreover, group I subjects had significantly higher right and left baPWV (p < 0.05 respectively), lower HDL level (p < 0.05), and lower VO2 peak (p < 0.05) than group II subjects. Furthermore, baPWV was significantly correlated with TG level (r = 0.326, p < 0.05), TC/HDL ratio (r = 0.483, p < 0.01), LDL level (r = 0.386, p < 0.01), and VO2 peak (r = -0.385, p < 0.05) in group I subjects. Only the TC/HDL ratio was found to be a significant correlating factor for an increase of baPWV (beta = 0.68, p < 0.05) in KD patients after multiple linear regression. CONCLUSION: Our results suggest that arterial stiffness is present late after KD and may adversely affect exercise performance, especially in patients with regressed CALs. Regular measurement of baPWV may be indicated in the long-term follow-up of KD patients.

Biventricular myocardial adaptation in patients with repaired tetralogy of Fallot: Mechanistic insights from magnetic resonance imaging tissue phase mapping.

BACKGROUND: The myocardial adaptive mechanism in patients with repaired tetralogy of Fallot (rTOF) is less understood. We aimed to investigate biventricular myocardial adaptive remodeling in rTOF patients. METHODS: We recruited 32 rTOF patients and 38 age- and sex-matched normal controls. The pulmonary stenosis of rTOF patients was measured using catheterized pressure gradient between right ventricle (RV) and pulmonary artery (PGRVPA). rTOF patients with PGRVPA < 15 mmHg and ≥15 mmHg were classified as low pulmonary stenosis (rTOFlow, n = 19) and high pulmonary stenosis (rTOFhigh, n = 13) subgroups, respectively. Magnetic resonance imaging tissue phase mapping was employed to evaluate the voxelwise biventricular myocardial motion in longitudinal (Vz), radial (Vr), and circumferential (Vφ) directions. RESULTS: The rTOFlow subgroup presented higher pulmonary regurgitation fraction than rTOFhigh subgroup (p < 0.001). Compared with the normal group, only rTOFlow subgroup presented a decreased RV ejection fraction (RVEF) (p < 0.05). The rTOFlow subgroup showed decreased systolic and diastolic Vz in RV and LV, whereas rTOFhigh subgroup showed such change only in RV. In rTOFlow subgroup, RVEF significantly correlated with RV systolic Vr (r = 0.56, p < 0.05), whereas LVEF correlated with LV systolic Vz (r = 0.51, p = 0.02). Prolonged QRS correlated with RV systolic Vr (r = -0.58, p < 0.01) and LV diastolic Vr (r = 0.81, p < 0.001). No such correlations occurred in rTOFhigh subgroup. CONCLUSIONS: The avoidance of unfavorable functional interaction in RV and LV in rTOFhigh subgroup suggested that adequate pulmonary stenosis (PGRVPA ≥ 15 mmHg in this sereis) has a protective effect against pulmonary regurgitation.

Adenovirus infection and subsequent risk of Kawasaki disease: A population-based cohort study.

BACKGROUND: The relationship between adenovirus infection and Kawasaki disease (KD) is unclear. The purpose of this study was to determine the relationship between adenovirus infection and KD using a cohort study in Taiwan. METHODS: We used Taiwan National Health Insurance data (from 2000 to 2008) to conduct a population-based cohort study, analyzing children that was under 18 years of age. In total, 5280 children had adenovirus infection, and 5280 children without adenovirus infection were matched and followed up. Subsequent KD was the major outcome event. The Cox proportional hazards model was used to estimate the hazard ratio (HR) with 95% confidence intervals (CIs) of developing KD associated with adenovirus infection. RESULTS: There was a significantly higher cumulative incidence of KD in the adenovirus-infected cohort than that in the control cohort (log-rank test, p < 0.001). In the adenovirus-infected cohort, overall incidence of KD was 5.29 times higher than that of the control cohort (adjusted HR 5.29, 95% CI: 2.48-11.3). Increased KD risk was associated with previous adenovirus infection in children aged 3-5 years, in female patients, in those with a low urbanization level, and in those with allergies. CONCLUSION: An association between previous adenovirus infection and KD was identified in Taiwanese children, but other potential risk factors were not fully analyzed. The relationship between infection and KD requires further study.

Update on association between Kawasaki disease and infection.

The relationship between infection and Kawasaki disease (KD) remains unclear. Infection has long been considered a key predisposing factor for KD. Bacterial and viral agents may be related to the onset of KD because of superantigen and cytokine production. Various bacterial and viral infections have been reported to be associated with KD, but the actual mechanism remains unknown. The higher association between KD and enterovirus has been well documented by using Taiwan National Health Insurance Research Database. However, no evidence has been obtained that various bacterial and viral infections induce KD. Comprehensive research, including infectious agents, should be conducted to elucidate the pathogenesis of KD.

Traumatic Ventricular Septal Defect in a 1-Year-Old Boy After Accidental Chest Compression by an Adult.

We report a 1-year-old boy with a delayed diagnosis of traumatic ventricular septal defect (VSD) related to chest compression. His cardiac function was stable after adequate medical treatment. Spontaneous closure of traumatic VSD occurred to this patient at the age of 4 years. This is a rare case of traumatic VSD associated with accidental chest compression, which is similar to rupture of the ventricular septum after blunt chest trauma. It should be kept in mind that traumatic VSD and concomitant thoracic injuries can develop during chest compression. The clinician should pay attention to the potential risk of traumatic VSD in patients experiencing chest compression. Echocardiography is a convenient and effective tool for serial follow-up examination and avoiding the delayed diagnosis. Troponin I level can be a useful screening test. Conservative management of traumatic VSD with hemodynamic stability is recommended because of possible spontaneous closure.

Successful repair of neonatal tricuspid regurgitation due to chordae rupture.

Neonatal rupture of the chordae of tricuspid valve with severe regurgitation is rare and disastrous. We report on a full-term female neonate presented with cyanosis caused by severe tricuspid regurgitation (TR) due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion, successful early repair was achieved with artificial chordae implantation. The unique pathophysiology and the therapeutic strategy of this situation will be described.

Enterovirus Infection and Subsequent Risk of Kawasaki Disease: A Population-based Cohort Study.

BACKGROUND: The relationship of enterovirus (EV) infection and Kawasaki disease (KD) is still unclear. The purpose of this study was to conduct a population-based cohort study to determine the relationship between KD and EV infection in Taiwan. METHODS: A population-based cohort study was conducted to analyze the children file (age < 18 years) of the Taiwan National Health Insurance program between 2000 and 2008. In total, 285,636 children with EV infection and 285,636 children without EV infection were included and followed up. The subsequent KD was the major outcome event. RESULTS: The cumulative incidence of KD was significantly higher in the EV-infected cohort than in the non-EV-infected cohort (log-rank test, P < 0.001). The overall incidence of KD was 56% higher in the EV-infected cohort than in the non-EV-infected cohort, with an adjusted hazard ratio of 1.56 (95% confidence interval: 1.44-1.69). Stratified analysis showed higher KD risk associated with previous EV infection in children 3-5 years old, in girls, in children living in less urbanization levels, in children with parental low-income occupation, and in children with allergic diseases. CONCLUSIONS: There is a higher association between KD and previous EV infection in Taiwanese children, especially in those 3-5 years old, with female sex, with less urbanization level, with low-income parental occupation, and with allergy.

Abnormal biventricular performance in asymptomatic adolescents late after repaired Tetralogy of Fallot: Combined two-dimensional speckle tracking and three-dimensional echocardiography study.

BACKGROUND: The aim of this prospective study was to assess biventricular performance in asymptomatic adolescents with repaired tetralogy of Fallot (TOF) using 2D speckle tracking and real time 3D echocardiography simultaneously. METHODS: We studied 31 patients with repaired TOF (M/F: 22/9, age: 16.1 ± 6.1 yrs) who had history of cardiac surgery with mean follow-up duration of 12.8 years, and 32 age- and sex-matched normal individuals (M/F: 23/9, age: 16.6 ± 5.1 yrs). All subjects underwent speckle tracking and 3D echocardiography, electrocardiogram, treadmill, and blood sampling for measurement of brain natriuretic peptide (BNP). RESULTS: Compared to the control group, the TOF group had higher BNP level (31.8 ± 21.4 vs 14.1 ± 12.4 pg/ml, p < 0.01), lower peak oxygen consumption (8.4 ± 1.7 vs 9.9 ± 1.6 ml/kg/min, p < 0.05), and longer QRS duration (126 ± 30 vs 82 ± 9 ms, p < 0.01). Patients with repaired TOF had significantly impaired right ventricle (RV) global and all six regional longitudinal strain and strain rate than normal controls. Left ventricle (LV) global and mainly apical regional longitudinal strain and strain rate were reduced in patients with repaired TOF. There was a significant correlation of global longitudinal strain (r = 0.456, p = 0.01) and global time to peak longitudinal strain (r = 0.484, p < 0.01) between LV and RV in patients with repaired TOF. In terms of 3D echo cardiographic volume data, patients with repaired TOF had lower LV stroke volume index (p < 0.05), but higher RV end diastolic volume index (p < 0.01), RV end systolic volume index (p < 0.01), RV stroke volume index (p < 0.01), and pulmonary regurgitation fraction (p < 0.01) than normal controls. CONCLUSION: Our results suggest asymptomatic adolescents with repaired TOF had abnormal biventricular myocardial performance, as demonstrated by combined 2D speckle-tracking and 3D echocardiography. The implications of these findings for management of adolescents late after repaired TOF remain to be determined.

The effects of maternal smoking exposure during pregnancy on postnatal outcomes: A cross sectional study.

BACKGROUND: The purpose of this article was to evaluate the effect of maternal smoking exposure during pregnancy on postnatal outcomes. METHODS: This prospective study enrolled 278 pregnant women in the third trimester, who were asked to complete a questionnaire which included inquires about the nature and extent of smoking exposure during their pregnancy. In addition to the questionnaire, each study subject provided urine sample for the measurement of cotinine. Using data generated from this inquiry, we analyzed the association between maternal smoking exposure and birth outcomes. RESULTS: From the 278 enrollees in this study, a minority of subjects (7.2%) smoked, while 40.6% of the study subjects were exposed to environmental tobacco smoke during pregnancy. There was significantly higher birth weight (3205.2 ± 373.1 vs 3089.7 ± 363.0 vs 2959.0 ± 403.7 g, p = 0.004), larger chest size (33.1 ± 1.7 cm vs 32.7 ± 1.5 cm vs 32.0 ± 1.7 cm, p = 0.009), higher bilirubin on postnatal day 3 (8.9 ± 1.6 vs 8.6 ± 1.5 vs 7.8 ± 1.4 mg/dL, p = 0.015), but lower maternal urinary cotinine level (83.7 ± 132.4 vs 153.2 ± 96.0 vs 800.5 ± 1027.8 µg/g creatinine, p < 0.001) in smoking-free status than in passive or active smoking status. Significant risks of birth weight < 2500 g (AOR 3.93 (95% CI 1.61-9.59), p = 0.003) and maternal urinary cotinine ≥ 143 µg/g creatinine (AOR 3.38 (95% CI 2.02-5.66), p < 0.001) were observed as smoking exposure increased. There was significantly higher birth weight (p = 0.048), larger chest size (p = 0.045), and higher bilirubin level on postnatal day 3 (p < 0.001) in the group with cotinine <143 µg/g creatinine than in the group with cotinine ≥ 143 µg/g creatinine. CONCLUSION: Our results demonstrated that maternal smoking exposure during pregnancy is associated with low birth weight and small chest circumference. Although the incidence of active smoking in Taiwanese pregnant women is low, most of them are exposed to passive smoking environment. Further studies are required to evaluate useful interventions to enhance a smoking-free environment during pregnancy.

Fontan Operation in a Patient with Severe Hypoplastic Right Pulmonary Artery, Single Ventricle, and Heterotaxy Syndrome.

Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances, some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For theses patients, completion of a Fontan operation will be challenging. We reported a 17-year-old girl with a single ventricle and heterotaxy syndrome and only her left lung functioning, who underwent one-lung Fontan operation with a satisfactory result.

Echocardiographic Diagnosis of Incidentally Found Left Coronary Artery to Pulmonary Artery Fistula in an 11-Year-Old Girl.

We report on a healthy 11-year-old girl who presented to our facility with sudden onset of fainting in a strenuous running course. Transthoracic echocardiography at short-axis view showed a diastolic flow into the main pulmonary artery (PA). The diagnosis of left anterior descending artery (LAD) to PA fistula was documented by cardiac computed tomography and catheterization. Interventional therapy of LAD to the main PA fistula was not performed because of no evidence of myocardial ischemia or significant hemodynamic change. Presently, the patient remains asymptomatic. Coronary fistula with an incidence of about 0.1-0.8% is very rare and may be undetected, particularly in pediatric patients without cardiac murmur. We herein describe the diagnostic approach and discuss the current treatment modalities.

Early and intermediate-term results of the extracardiac conduit total cavopulmonary connection for functional single-ventricle hearts.

BACKGROUND/PURPOSE: Fontan operation has evolved from atriopulmonary connection to total cavopulmonary connection (TCPC) due to its advantages in terms of hemodynamics and reduction of atrium-related complications. We analyzed the early and intermediate-term results of extracardiac conduit TCPC (EC-TCPC) procedure in patients with functional single ventricle to investigate the risk factors of surgical mortality and intermediate failure. METHODS: Retrospective review of the medical records of 88 consecutive patients with functional single ventricle who underwent EC-TCPC from 2000 to 2013 was conducted. RESULTS: The follow-up was 100% complete, ranging from 3 months to 13 years (mean 7.0 ± 3.8 years). There were two (2.3%) hospital and 18 (20.4%) late deaths. The estimated event-free survival rates at 1 year, 5 years, and 10 years were 90.6%, 89.3%, and 77.2%, respectively. On univariate analysis, fenestration was the only risk factor for surgical mortality (p = 0.027). On multivariate analysis, the significant atrioventricular valve regurgitation was the only risk factor for intermediate failure (p = 0.017). CONCLUSION: The clinical results of EC-TCPC in patients with functional single ventricle were satisfactory. The patients who needed fenestration during operation had higher risk of surgical mortality. Significant atrioventricular valve regurgitation had negative impact on intermediate survival.

Assessment of branch pulmonary artery stenosis in children after repair of tetralogy of Fallot using lung perfusion scintigraphy comparison with echocardiography.

PURPOSE: The aim of this study was to compare the usefulness of lung perfusion scintigraphy and echocardiogram in the evaluation of the branch pulmonary arteries stenosis in children with tetralogy of Fallot (TOF). MATERIALS AND METHODS: From February 2006 to November 2008, 74 children (mean age 7.8 years, range 1­18 years) who underwent repair of TOF at ages from 10 months to 13 years were suspected to have unilateral or bilateral branch pulmonary artery stenosis. In all patients, cardiac angiography was performed to confirm the diagnosis of branch pulmonary artery stenosis. Lung perfusion scintigraphy and two-dimensional transthoracic echocardiography were performed in all patients to compare their abilities to diagnose branch pulmonary artery stenosis. RESULTS: Of the 74 patients, 51 cases were found to have branch pulmonary artery stenosis by cardiac angiography. There was agreement between the scintigraphic and angiographic findings in 44 (86%) patients and there were discrepancies in 11 (15%) patients. The positive predictive value of our lung perfusion scintigraphy in detecting the branch pulmonary artery stenosis was 92 %. The positive and negative likelihood ratios of lung perfusion scintigraphy were 4.96 and 0.17, respectively. There was conformity between the echocardiographic and angiographic findings in 40 (78%) patients with discrepancies in 16 (21%) patients. The positive predictive value of our echocardiography in detecting the branch pulmonary artery stenosis was 89%. The positive and negative likelihood ratios of echocardiography were 3.61 and 0.28, respectively. CONCLUSIONS: Lung perfusion scintigraphy is a valuable, non-invasive screening tool in the assessment of branch pulmonary artery stenosis in children after TOF.

Myeloperoxidase genetic polymorphisms and susceptibility to Kawasaki disease in Taiwanese children.

BACKGROUND/PURPOSE: The aim of this study was to investigate the myeloperoxidase (MPO) -463G>A polymorphism in Kawasaki disease (KD) patients, and the relationship between gene polymorphism and MPO levels. METHODS: A total of 334 KD children and 492 sex-matched controls were assayed for polymorphism analysis. TaqMan assays were used for genotyping. MPO was measured in 37 KD patients and 42 febrile controls. RESULTS: A significant linear trend of KD risk was found to be related to the G/G genotype (plinear trend = 0.032). The combined genotypes (G/A and A/A) of MPO -463G>A were associated with a significantly decreased KD risk compared to the G/G genotype [adjusted odds ratios (AOR) = 0.71, 95% confidence interval (CI): 0.52-0.99, p = 0.040]. In addition, KD patients with A allele were associated with a significantly decreased KD risk as compared to those with G allele (AOR = 0.73, 95% CI: 0.54-0.98, p = 0.033). MPO levels were significantly elevated in KD patients in preintravenous immunoglobulin (pre-IVIG) stage compared to febrile controls (p = 0.002). KD patients in pre-IVIG stage had significantly higher MPO levels than febrile controls in terms of G/G genotype (p = 0.003) and G allele (p < 0.001). KD patients with A allele had significantly lower MPO levels than those with G allele in post-IVIG acute stage (p = 0.042). However, there was no significant difference of individual MPO change for KD patients from pre- to post-IVIG stage in terms of genotypes (p = 0.837) or alleles (p = 0.631). CONCLUSION: Our results suggest that G allele of MPO -463G>A polymorphism is a potential genetic marker for KD risk in Taiwanese children.