Early-Onset Late Gadolinium Enhancement is a Prognostic Factor for Duchenne Cardiomyopathy.

Dilated cardiomyopathy (DCM) is an inevitable complication of Duchenne muscular dystrophy (DMD). Late gadolinium enhancement (LGE) demonstrated by cardiac MRI occurs in DMD-related DCM, indicating myocyte death and remodeling. We conducted a retrospective chart review identifying DMD patients in our center between January 2009 and July 2013. Subjects were cohorted by presence of LGE before age 14. We excluded patients in whom we could not determine LGE status prior to age 14. We reviewed comprehensive clinical data. Of the 41 subjects with complete data, 15 demonstrated LGE before age 14 ("early LGE") and 26 had no LGE by age 14 ("controls"). Those with early LGE exhibited a more rapid decline in LV fractional shortening (p = 0.028). Patients with early LGE were younger at age of initiation of ACE inhibition (p = 0.025), mineralocorticoid receptor antagonism (p = 0.0024), and beta-blockade (p = 0.0017), suggesting aggressive clinical management in response to abnormal MRI findings. There were no significant differences in LV dilation between the two groups (p = 0.1547). Early LGE was not associated with obesity (p = 0.32), age at loss of ambulation (p = 0.31), or heart rate (p-value > 0.8). Early onset of myocardial fibrosis as indicated by LGE on cardiac MRI is associated with earlier progression of cardiomyopathic changes despite earlier medication therapy. Identifying this risk factor, observed in 34% of our cohort during preadolescence, may guide medical therapy and early counseling about cardiomyopathy progression. We advocate for obtaining at least one MRI in patients with DMD prior to age 14 to risk stratify patients.

Acute Cardiac Care for Neonatal Heart Disease.

This manuscript is one component of a larger series of articles produced by the Neonatal Cardiac Care Collaborative that are published in this supplement of Pediatrics. In this review article, we summarize the contemporary physiologic principles, evaluation, and management of acute care issues for neonates with complex congenital heart disease. A multidisciplinary team of authors was created by the Collaborative's Executive Committee. The authors developed a detailed outline of the manuscript, and small teams of authors were assigned to draft specific sections. The authors reviewed the literature, with a focus on original manuscripts published in the last decade, and drafted preliminary content and recommendations. All authors subsequently reviewed and edited the entire manuscript until a consensus was achieved. Topics addressed include cardiopulmonary interactions, the pathophysiology of and strategies to minimize the development of ventilator-induced low cardiac output syndrome, common postoperative physiologies, perioperative bleeding and coagulation, and common postoperative complications.

Mechanical tricuspid valve replacement in hypoplastic left heart syndrome: An institutional experience.

Objective: Atrioventricular valvar regurgitation in patients with single ventricles is associated with worse outcomes. Valve repair or replacement has been undertaken in an attempt to reduce mortality and morbidity. Current data on valve replacement in single ventricle patients are limited and derived from heterogenous populations. We describe our experience with repair and replacement of the tricuspid valve in children undergoing single ventricle palliation for hypoplastic left heart syndrome. Methods: We included 27 patients with hypoplastic left heart syndrome with at least moderate tricuspid regurgitation who underwent valve intervention between 2007 and 2021 at our institution; charts were retrospectively reviewed for data. Results: Eleven patients (73% male) underwent valve replacement (median age, 3 years). Preoperative ventricular systolic function was normal in 10 patients (91%). Median follow-up postoperatively was 4 years with no early mortality, 1 (9%) late mortality, and 1 heart transplant (9%). Morbidity consisted of complete heart block in 1 patient (9%), with no important hemorrhagic or thrombotic events. Among survivors of replacement (n = 9), ventricular function was preserved in all (n = 8) who had normal function preoperatively and improved in the remaining patient. Sixteen patients underwent valve repair (median age, 4 months) with no early mortality, 8 (50%) midterm mortalities, and 2 heart transplants (12%). Conclusions: Tricuspid valve replacement is a feasible option in hypoplastic left heart syndrome with significant tricuspid regurgitation, with favorable outcomes in the intermediate follow-up. When undertaken in the setting of normal function, ventricular function may be preserved in up to 80% of patients. Long-term follow-up is needed.

Missed Opportunity Encounters for Early Diagnosis of HIV Infection in Adolescents.

BACKGROUND: Early diagnosis of HIV infection improves patient outcomes and reduces transmission. Adolescents make up one-fifth of new HIV diagnoses in the United States. We sought to quantify the number of missed opportunity encounters (MOEs) before HIV diagnosis for adolescents at a pediatric hospital (PediHosp) and a proximate adult hospital which employs universal HIV screening in its emergency department (ED) (CountyHosp). METHODS: An observational study at 2 academic tertiary care hospitals in the United States that included all adolescents 13-20 years old with a new diagnosis of behaviorally-acquired HIV infection from 2006 to 2017. MOE were defined as any encounter at PediHosp or CountyHosp after the latter of the individual's 13th birthday or the date 3 months after the individual's most recent negative HIV screen, and before the encounter of HIV diagnosis. Comparisons were made by site of diagnosis and location of MOE. RESULTS: Two-hundred five subjects met inclusion criteria: 68% male, 76% Black and 81% men who have sex with men. There were 264 MOE, the proportion of adolescent ED encounters that were MOE at the PediHosp ED was 8.3 MOE per 10,000 encounters and the proportion at the CountyHosp ED was 1.2 (relative risk = 6.7; 95% CI: 4.1-11.0; P < 0.001). CONCLUSIONS: MOE for HIV diagnosis in adolescents occur frequently and are greater in number at a PediHosp as compared with a similar adult setting with universal screening. Universal HIV screening protocols at PediHosp may identify HIV-positive adolescents earlier.

Scratching Past Lymphadenopathy: A Case of Bartonella henselae Encephalitis.

A previously healthy 6-year-old boy presented with new onset seizure activity and altered mental status. His prehospital course included prolonged fever, vague abdominal complaints, and unusual behavior. Neurological testing was unrevealing, and his symptoms slowly improved without intervention. His primary pediatrician had ordered serum antibody titers to Bartonella henselae for testing of prolonged fever in the setting of exposure to a kitten; these were found to be positive for both immunoglobulin G and immunoglobulin M. Further examination for organ involvement revealed splenic and liver micro-abscesses. After completion of his antibiotic course, the patient returned to his cognitive and neurologic baseline with resolution of his abdominal abscesses. This case emphasizes the importance of obtaining a thorough exposure history when evaluating for infectious causes of encephalitis. [Pediatr Ann. 2020;49(8):e359-e362.].

Rationalising the use of cardiac catheterisation before Glenn completion.

Previous studies have shown that cardiac MRI can be used to evaluate the suitability for infants to undergo the Glenn operation after having undergone the Norwood procedure. We sought to analyse our institutional data retrospectively to identify whether such a policy would be advisable in the current era. We reviewed patients who underwent the Norwood procedure between 1 January, 2006 and 1 January, 2016. All patients undergoing evaluation for the Glenn procedure received clinical evaluation, echocardiography, and cardiac catheterisation. A total of 179 patients were identified; 154 patients (86%) survived to undergo cardiac catheterisation as part of evaluation for the Glenn, and all who were evaluated did not eventually receive the Glenn. Using said algorithm, if cardiac MRI or CT were to be used to rationalise the use of catheterisation, 26 of 154 patients would have required catheterisation after cross-sectional imaging identified vascular obstruction; 83 of 154 patients would have received cross-sectional imaging only; and 45 of 154 would have had catheterisation only. All cases that required intervention, excluding aortopulmonary collaterals, and all cases that were not suitable to progress would have been correctly identified using clinical and echocardiographic criteria in addition to cardiac cross-sectional imaging to rationalise the use of catheterisation. Thus, in cases with acceptable clinical, echocardiographic, and angiographic findings, the additional haemodynamic information from catheterisation is rarely of use for decision-making, and interventions can largely be predicted by angiographic imaging modalities.

A clinical combined gadobutrol bolus and slow infusion protocol enabling angiography, inversion recovery whole heart, and late gadolinium enhancement imaging in a single study.

BACKGROUND: The use of gadolinium contrast agents in cardiovascular magnetic resonance is well-established and serves to improve both vascular imaging as well as enable late gadolinium enhancement (LGE) imaging for tissue characterization. Currently, gadofosveset trisodium, an intravascular contrast agent, combined with a three-dimensional inversion recovery balanced steady state free precession (3D IR bSSFP) sequence, is commonly used in pediatric cardiac imaging and yields excellent vascular imaging, but cannot be used for late gadolinium enhancement. Gadofosveset use remains limited in clinical practice, and manufacture was recently halted, thus an alternative is needed to allow 3D IR bSSFP and LGE in the same study. METHODS: Here we propose a protocol to give a bolus of 0.1 mL/kg = 0.1 mmol/kg gadobutrol (GADAVIST/GADOVIST) for time-resolved magnetic resonance angiography (MRA). Subsequently, 0.1 mmol/kg is diluted up to 5 or 7.5 mL with saline and then loaded into intravenous tubing connected to the patient. A 0.5 mL short bolus is infused, then a slow infusion is given at 0.02 or 0.03 mL/s. Image navigated (iNAV) 3D IR bSSFP imaging is initiated 45-60 s after the initiation of the infusion, with a total image acquisition time of ~5 min. If necessary, LGE imaging using phase sensitive inversion recovery reconstruction (PSIR) is performed at 10 min after the infusion is initiated. RESULTS: We have successfully performed the above protocol with good image quality on 10 patients with both time-resolved MRA and 3D IR bSSFP iNAV imaging. Our initial attempts to use pencil beam respiratory navigation failed due to signal labeling in the liver by the navigator. We have also performed 2D PSIR LGE successfully, with both LGE positive and LGE negative results. CONCLUSION: A bolus of gadobutrol, followed later by a slow infusion, allows time-resolved MRA, 3D IR bSSFP using the iNAV navigation technique, and LGE imaging, all in a single study with a single contrast agent.