Contemporary sequential segmental approach to congenital heart disease using four-dimensional magnetic resonance imaging with ferumoxytol: an illustrated editorial.

The ferumoxytol-enhanced 4D MR angiography with MUSIC (Multiphase Steady State Imaging with Contrast) technique provides a single data set that captures dynamic cardiovascular anatomy and ventricular function at the same time. Homogeneous opacification of all cardiovascular structures within the imaging volume allows full sequential segmental approach to the congenital heart diseases without any blind spots. The complex systemic and pulmonary venous anatomy is particularly well captured in the MUSIC. Cinematographic display of multiplanar sectional and 3D volume images is helpful in the morphological identification of the cardiac chambers, the assessment of the dynamic nature of the ventricular outflow tracts, and the assessment of the coronary arterial origins and courses.

Assessment of liver fibrosis using a 3-dimensional high-resolution late gadolinium enhancement sequence in children and adolescents with Fontan circulation.

OBJECTIVES: To assess abnormal liver enhancement on 15-20 min delayed 3D high-resolution late gadolinium enhancement (3D HR LGE) sequence in patients with Fontan circulation. METHODS: Retrospective study of pediatric Fontan patients (< 18 years old) with combined cardiac-liver MRI from January 2018 to August 2021. Abnormal hepatic enhancement was graded (0-3) for each lobe, summed for a total liver enhancement score (0-6), and compared to repaired tetralogy of Fallot (rTOF) patients. Correlations with other hepatic imaging biomarkers were performed. Temporal relationships of enhancement compared to traditional early portal venous and 5-7-min delayed phase liver imaging were analyzed. RESULTS: The Fontan group (n = 35, 13 ± 3.4 years old, median time from Fontan 10 (9-12) years) had 23/35 (66%) with delayed 3D HR LGE total liver enhancement score > 0 (range 0-5), with greater involvement of the right lobe (1 (0-1) vs 0 (0-1), p < 0.01). The rTOF group (n = 35, 14 ± 2.6 years old) had no abnormal enhancement. In the Fontan group, total liver enhancement was 3 (2-4) in the early portal venous phase, lower at 1 (1-2) in the 5-7-min delayed phase (p < 0.01), and lowest at 1 (0-2) in the 15-20-min delayed phase (p = 0.03). 3D HR LGE enhancement correlated inversely with portal vein flow (rs = - 0.42, p = 0.01) and positively with left lobe stiffness (rs = 0.51, p < 0.01). The enhancement score decreased in 13/35 (37%) between the 5-7- and 15-20-min delayed phases. CONCLUSIONS: Liver fibrosis can be assessed on 3D HR LGE sequences in patients with Fontan circulation, correlates with other imaging biomarkers of Fontan liver disease, and may add information for hepatic surveillance in this population. KEY POINTS: • Abnormal liver enhancement on 3D HR LGE sequences in Fontan patients likely represents liver fibrosis and is seen in up to 66% of children and adolescents with variable distribution and severity. • The degree of 3D HR LGE liver enhancement correlates with decreased portal vein flow and increased left hepatic lobe stiffness.

Myocardial late gadolinium enhancement using delayed 3D IR-FLASH in the pediatric population: feasibility and diagnostic performance compared to single-shot PSIR-bSSFP.

BACKGROUND: This study compares three-dimensional (3D) high-resolution (HR) late gadolinium enhancement (LGE; 3D HR-LGE) imaging using a respiratory navigated, electrocardiographically-gated inversion recovery gradient echo sequence with conventional LGE imaging using a single-shot phase-sensitive inversion recovery (PSIR) balanced steady-state free precession (bSSFP; PSIR-bSSFP) sequence for routine clinical use in the pediatric population. METHODS: Pediatric patients (0-18 years) who underwent clinical cardiovascular magnetic resonance (CMR) with both 3D HR-LGE and single-shot PSIR-bSSFP LGE between January 2018 and June 2020 were included. Image quality (0-4) and detection of LGE in the left ventricle (LV) (per 17 segments), in the right ventricle (RV) (per 3 segments), as endocardial fibroelastosis (EFE), at the hinge points, and at the papillary muscles was analyzed by two blinded readers for each sequence. Ratios of the mean signal intensity of LGE to normal myocardium (LGE:Myo) and to LV blood pool (LGE:Blood) were recorded. Data is presented as median (1st-3rd quartiles). Wilcoxon signed rank test and chi-square analyses were used as appropriate. Inter-rater agreement was analyzed using weighted κ-statistics. RESULTS: 102 patients were included with median age at CMR of 8 (1-13) years-old and 44% of exams performed under general anesthesia. LGE was detected in 55% of cases. 3D HR LGE compared to single-shot PSIR-bSSFP had longer scan time [4:30 (3:35-5:34) vs 1:11 (0:47-1:32) minutes, p < 0.001], higher image quality ratings [3 (3-4) vs 2 (2-3), p < 0.001], higher LGE:Myo [23.7 (16.9-31.2) vs 5.0 (2.9-9.0), p < 0.001], detected more segments of LGE in both the LV [4 (2-8) vs 3 (1-7), p = 0.045] and RV [1 (1-1) vs 1 (0-1), p < 0.001], and also detected more cases of LGE with 13/56 (23%) of patients with LGE only detectable by 3D HR LGE (p < 0.001). 3D HR LGE specifically detected a greater proportion of RV LGE (27/27 vs 17/27, p < 0.001), EFE (11/11 vs 5/11, p = 0.004), and papillary muscle LGE (14/15 vs 4/15, p < 0.001). Inter-rater agreement for the recorded variables ranged from 0.42 to 1.00. CONCLUSIONS: 3D HR LGE achieves greater image quality and detects more LGE than conventional single-shot PSIR-bSSFP LGE imaging, and should be considered an alternative to conventional LGE sequences for routine clinical use in the pediatric population.

Modified Warden Procedure for Partial Anomalous Pulmonary Venous Drainage to Promote Growth.

Extreme forms of partially anomalous pulmonary venous drainage with high entrance of the pulmonary veins into the superior caval vein can be challenging to correct without obstructing both systemic and pulmonary venous pathways. We report an unusual morphologic subform of this malformation and a surgical technique to address its specific anatomic peculiarities while maintaining growth potential of the venous pathways. We highlight that even such complex reconstructions can be performed minimally invasively.

Hemodynamic effects of pulmonary regurgitation in one and one-half ventricle repair.

The pulmonary circulation after one and one-half ventricle repair is complex because of the direct connection of two sources of blood flow. Associated pulmonary regurgitation further complicates the pulmonary circulation. We report the complex hemodynamics depicted by phase-contrast magnetic resonance imaging in a patient with one and one-half ventricle circuit and severe pulmonary regurgitation. Antegrade superior vena caval flow occurred almost exclusively during diastole and regurgitated into the right ventricle. Consequently, the entire systemic venous return reached the right ventricle during diastole and is pumped back into the pulmonary arteries in systole. The case highlights the importance of analyzing flow patterns phase-by phase throughout the cardiac cycle instead of relying on the net flow volumes through each source of pulmonary blood flow.

Long-term course after pediatric right ventricular outflow tract reconstruction.

OBJECTIVES: Valved homografts are commonly used for right ventricular outflow tract reconstruction. However, despite good early results, they lack durability. This study was designed to compare single-center results of implantation of 3 types of right ventricular outflow tract conduit, in terms of patient survival, graft failure, reoperation, and risk factors for reoperation. METHODS: One hundred and forty-three pediatric patients who underwent right ventricular outflow tract conduit implantation between January 2006 and December 2018 were reviewed. We stratified conduits by aortic, pulmonic homograft, and Contegra; 74 aortic homografts, 61 pulmonic homografts, and 8 Contegra conduits were implanted. Median age at implantation was 3 years. The primary diagnosis was truncus arteriosus in 41.3%. We analyzed the role of sex, age, diagnosis, and graft size. Endpoints included freedom from graft failure, freedom from reoperation, and survival. RESULTS: The survival rate was 83.2% at 10 years. Freedom from graft failure at 2, 5, and 10 years was 100%, 97.9%, and 63.4%, respectively. Freedom from reoperation was 85.8% for pulmonic homografts and 74.9% for aortic homografts at 10 years, and 100% for Contegra at 6 years. Multivariable analysis identified conduit diameter <18 mm as a risk factor for reoperation (hazard ratio: 3.16, 95% confidence interval: 1.38-7.23, p = 0.007). CONCLUSION: Homograft valves used for right ventricular outflow tract reconstruction provided excellent long-term durability and late survival. The only factor that adversely affected graft longevity was small graft size (diameter <18 mm). Reoperation for conduit failure was not significantly different among the groups.

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Recurrent Takotsubo syndrome in a boy with Duchenne muscular dystrophy: A case report.

Takotsubo syndrome is a rare cause of reversible ventricular dysfunction that imitate an acute coronary syndrome. The entity is unusual among pediatric populations and a recurrent episode is extremely rare. We report a case of recurrent takotsubo syndrome in an eight-year-old boy with Duchenne muscular dystrophy (DMD). His chest pain episodes were aggravated by a strong emotional stimuli. During episodes of chest pain, electrocardiograms (ECG) showed ST elevation while echocardiograms showed left ventricle apical ballooning; however, a coronary angiography was normal. Serial ECG and echocardiogram revealed a spontaneous resolution of ST elevation and normalized apical contraction which were compatible with the diagnosis of takotsubo syndrome. Interestingly, serial cardiac magnetic resonance imaging demonstrated increasing subepicardial enhancement which was compatible with progression of cardiac involvement in DMD. .

Genotype and clinical characteristics of congenital long QT syndrome in Thailand.

BACKGROUND: Congenital long QT syndrome (LQTS) is an inheritable arrhythmic disorder which is linked to at least 17 genes. The clinical characteristics and genetic mutations may be variable among different population groups and they have not yet been studied in Thai population. METHODS: Clinical characteristics were retrospectively reviewed from children and young adults with congenital long QT syndrome whose blood samples were sent for genotyping during 1998-2017. Sangers sequencing was used to sequentially identify KCNQ1 or KCNH2 genetic variants. Whole exome sequencing (WES) was used to identify variants in all other known LQTS genes. RESULTS: Of the 20 subjects (17 families), 45% were male, mean QTc was 550.3 ±â€¯68.8 msec (range 470-731 msec) and total Schwartz's score was 5.6 ±â€¯1.2 points (range 3-8 points). Fifty percent of patients had events at rest, 30% had symptoms after adrenergic mediated events, and 20% were asymptomatic. We discovered pathogenic and likely pathogenic genetic variants in KCNQ1, KCNH2, and SCN5A in 6 (35%), 4 (24%), and 2 (12%) families, respectively. One additional patient had variance of unknown significance (VUS) in KCNH2 and another one in ANK2. No pathogenic genetic variant was found in 3 patients (18%). Most patients received beta-blocker and 9 (45%) had ICD implanted. LQT1 patients were either asymptomatic or had stress-induced arrhythmia. Most of the LQT2 and LQT3 patients developed symptoms at rest or during sleep. CONCLUSIONS: Our patients with LQTS were mostly symptomatic at presentation. The genetic mutations were predominantly in LQT1, LQT2, and LQT3 genes.

Clinical presentation and course of long QT syndrome in Thai children.

BACKGROUND: Congenital long QT syndrome (LQTS) is a genetically transmitted cardiac channelopathy that can lead to lethal arrhythmia and sudden cardiac death in healthy young people. The clinical characteristics of LQTS are variable and depend on the subtype of long QT syndrome, which differ among populations. This single hospital-based case review study examined the clinical presentation of long QT syndrome and the outcomes of its treatment in 20 Thai children at King Chulalongkorn Memorial Hospital in Bangkok, Thailand. METHODS: Inpatient and outpatient records of children (aged 0-14 years) diagnosed with long QT syndrome from January 1, 1998, to September 30, 2013, were retrospectively reviewed. Presentation at diagnosis, treatments, and clinical courses were collected and analyzed. In the 20 subjects, total Schwartz scores totaled 5.2±0.9 points, and mean age at diagnosis was 7.6±4.4 years (range, 1 day-13.8 years). The patients were assigned to one of 3 groups based on trigger events: 50% of patients had events at rest (sleep or at rest), 35% experienced adrenergic-mediated events (e.g., stress, exercise, startle), and 15% were asymptomatic. Excluding the 3 patients who died at first presentation, 100% of patients received a beta blocker, and 47.1% were treated with an automatic implantable cardioverter-defibrillator (AICD). RESULTS: At follow-up (median=959 days; range, 1-4170 days), 4 patients (20%) were known to have died, 3 of whom died shortly after the diagnosis. Among patients who survived the initial event, 52.9% (9 of 17) experienced cardiac events (appropriate AICD shock, death, and/or syncope) during the follow-up period. The mean duration from diagnosis to cardiac event was 1420±759 days (range, 497-2499 days). CONCLUSIONS: All 20 patients with LQTS were mostly symptomatic at presentation. Owing to the geographical region and ethnicity of the Thai population, we conclude that the ratio of patients who develop cardiac symptoms at rest or during sleep might be higher than in other Asian countries.

The presence of epsilon waves in all precordial leads (V1 -V6 ) in a 13-year-old boy with arrhythmogenic right ventricular dysplasia (ARVD).

Electrocardiographic feature is included in the diagnostic criteria for arrhythmogenic right ventricular dysplasia (ARVD) based on the Revised Task Force criteria 2010. The epsilon wave, which reflects delayed conduction of the right ventricle, is considered to be one of the major diagnostic criteria. We reported a 13-year-old Thai boy with ARVD who presented with ventricular tachycardia. The presence of epsilon wave in all precordial leads (V1 -V6 ) was observed in standard 12-lead EKG. Extensive scarring of the right and left ventricle was seen on cardiac MRI. The extensive Epsilon wave found in this patient may reflect the extensive ventricular wall involvement.