Congenital giant left ventricular aneurysm.

We report a case of congenital giant left ventricular aneurysm with severely depressed systolic cardiac function who underwent early surgical resection with subsequent recovery of left ventricular systolic function.

Genotype-Phenotype Correlation of a Rare Transthyretin Variant Causing Amyloidosis.

Left ventricular hypertrophy is a common entity with a broad differential diagnosis. We present a case of a middle-aged woman with left ventricular hypertrophy and neuropathy caused by a rare transthyretin variant in the absence of a family history or regional reports of hereditary transthyretin amyloidosis. This report outlines the diagnosis and management of patients with a mixed phenotype of hereditary transthyretin amyloidosis and enriches clinical data supporting the pathogenicity of a rare variant of transthyretin.

L'hypertrophie ventriculaire gauche est une entité clinique fréquente pour laquelle le diagnostic différentiel est vaste. Nous décrivons le cas d'une femme d'âge moyen présentant une hypertrophie ventriculaire gauche et une neuropathie, causées par un variant rare de la transthyrétine en l'absence d'antécédents familiaux ou de cas régionaux déclarés d'amylose héréditaire à transthyrétine. Le présent article décrit le diagnostic et la prise en charge des patients qui présentent un phénotype mixte d'amylose héréditaire à transthyrétine, et il alimente le bassin de données cliniques sur la pathogénicité d'un variant rare de la transthyrétine.

Patent ductus arteriosus stenting versus modified Blalock-Taussig shunt for palliation of duct-dependent pulmonary blood flow lesions.

BACKGROUND: Patent ductus arteriosus (PDA) stenting is an alternative to modified Blalock-Taussig shunt (MBTS) as first-stage palliation of duct-dependent lesions. The superiority of one approach over the other is still controversial. Our objective was to compare PDA  stent versusMBTS for palliation in regard to safety, efficacy, and efficiency. METHODS: From 2010 to 2021, 134 patients had first-stage palliation with either PDA stent (n = 83) or MBTS (n = 51). Twenty-seven patients failed the primary treatment and were converted to the other group. The study endpoints were hospital outcomes, interstage reintervention, and concomitant procedures at the second-stage palliation. RESULTS: Patients with PDA stent were significantly younger. The prevalence of antegrade pulmonary blood flow (PBF) was higher in patients who had MBTS and graft thrombosis was higher in the PDA stent. Hospital stay was significantly longer in patients who had MBTS. Predictors of prolonged mechanical ventilation were low-weight, MBTS, and conversion. Intensive care unit stay significantly increased with conversion, low-weight, and antegrade PBF. The interstage intervention was required more frequently in PDA-stent group. Predictors of reintervention were conversion and pulmonary atresia with the intact interventricular septum. Pulmonary artery plasty was required more frequently during the second-stage palliation in PDA-stent group. CONCLUSION: PDA stent is an alternative to MBTS for first-stage palliation. It is associated with shorter hospital stays and avoidance of surgery at the expense of a high rate of stent thrombosis and interstage reintervention. Conversion increased the risk of the procedure. More studies are needed to determine factors that affect PDA-stent outcomes and patient selection criteria.

Fick versus flow: a real-time invasive cardiovascular magnetic resonance (iCMR) reproducibility study.

BACKGROUND: Cardiac catheterization and cardiovascular magnetic resonance (CMR) imaging have distinct diagnostic roles in the congenital heart disease (CHD) population. Invasive CMR (iCMR) allows for a more thorough assessment of cardiac hemodynamics at the same time under the same conditions. It is assumed but not proven that iCMR gives an incremental value by providing more accurate flow quantification. METHODS: Subjects with CHD underwent real-time 1.5 T iCMR using a passive catheter tracking technique with partial saturation pulse of 40° to visualize the gadolinium-filled balloon, CMR-conditional guidewire, and cardiac structures simultaneously to aid in completion of right (RHC) and left heart catheterization (LHC). Repeat iCMR and catheterization measurements were performed to compare reliability by the Pearson (PCC) and concordance correlation coefficients (CCC). RESULTS: Thirty CHD (20 single ventricle and 10 bi-ventricular) subjects with a median age and weight of 8.3 years (2-33) and 27.7 kg (9.2-80), respectively,  successfully underwent iCMR RHC and LHC. No catheter related complications were encountered. Time taken for first pass RHC and LHC/aortic pull back was 5.1, and 2.9 min, respectively. Total success rate to obtain required data points to complete Fick principle calculations for all patients was 321/328 (98%). One patient with multiple shunts was an outlier and excluded from further analysis. The PCC for catheter-derived pulmonary blood flow (Qp) (0.89, p < 0.001) is slightly lower than iCMR-derived Qp (0.96, p < 0.001), whereas catheter-derived systemic blood flow (Qs) (0.62, p = < 0.001) was considerably lower than iCMR-derived Qs (0.94, p < 0.001). CCC agreement for Qp at baseline (C1-CCC = 0.65, 95% CI 0.41-0.81) and retested conditions (C2-CCC = 0.78, 95% CI 0.58-0.89) were better than for Qs at baseline (C1-CCC = 0.22, 95% CI - 0.15-0.53) and retested conditions (C2-CCC = 0.52, 95% CI 0.17-0.76). CONCLUSION: This study further validates hemodynamic measurements obtained via iCMR. iCMR-derived flows have considerably higher test-retest reliability for Qs. iCMR evaluations allow for more reproducible hemodynamic assessments in the CHD population.

Feasibility and safety of quantitative adenosine stress perfusion cardiac magnetic resonance imaging in pediatric heart transplant patients with and without coronary allograft vasculopathy.

BACKGROUND: Pediatric heart transplant patients require cardiac catheterization to monitor for coronary allograft vasculopathy. Cardiac catheterization has no safe and consistent method for measuring microvascular disease. Stress perfusion cardiac magnetic resonance imaging (MRI) assessing microvascular disease has been performed in adults. OBJECTIVE: To investigate the feasibility and safety of performing cardiac MRI with quantitative adenosine stress perfusion testing in pediatric heart transplant patients with and without coronary allograft vasculopathy. MATERIALS AND METHODS: All pediatric heart transplant patients with coronary vasculopathy at our institution were asked to participate. Age- and gender-matched pediatric heart transplant patients without vasculopathy were recruited for comparison. Patients underwent cardiac MRI with adenosine stress perfusion testing. RESULTS: Sixteen pediatric heart transplant patients, ages 6-22 years, underwent testing. Nine patients had vasculopathy by angiography. No heart block or other complications occurred during the study. The myocardial perfusion reserve for patients with vasculopathy showed no significant difference with comparison patients (median: 1.43 vs. 1.48; P=0.49). Values for both groups were lower than expected values based on previous adult studies. The patients were also analyzed for time after transplant and the number of rejection episodes. Patients within 6 years of transplantation had a nonsignificant trend toward a higher myocardial perfusion reserve (median: 1.57) versus patients with older transplants (median: 1.47; P=0.46). Intra- and interobserver reproducibility were 97% and 92%, respectively. CONCLUSION: Myocardial perfusion reserve is a safe and feasible method for estimating myocardial perfusion in pediatric heart transplant patients. There is no reliable way to monitor microvascular disease in pediatric patients. This method shows potential and deserves investigation in a larger cohort.

Accuracy of Routine 2D Echocardiography to Estimate Patent Ductus Arteriosus Type and Dimension and Predict Device Selection for Successful PDA Occlusion.

BACKGROUND: Assessment of the shape and dimensions of PDA is usually done angiographically and in the majority of cases need arterial access. Our aim was to evaluate the value of routine 2 D echocardiography (ECHO) in predicting type, dimensions of PDA and to anticipate device size to be used during the intervention. MATERIAL AND METHODS: The charts of all patients who underwent transcatheter closure of PDA between January 2015 and December 2020 were reviewed. Their pre-procedure ECHO and catheterization details at the time of device closure were analyzed. RESULTS: Total of 139 patients were reviewed and 8 were excluded because of lack of adequate echocardiographic or angiographic images. The mean age and weight of the study population were 2.6 ± 2.5 years (range 0.2-14 years) and 11.2 ± 7.8 kg (range: 1.5-57 kg), respectively. There was no statistically significant difference in PDA narrowest diameter (p = 0.99) and predicted device type (p = 0.54) between Echo and angiography. Echo slightly overestimated PDA length (p = 0.01) and aortic ampulla dimension (p = 0.047), while morphology of PDA was correctly identified in the majority of cases (82%). CONCLUSIONS: Pre-procedure echocardiography correlates well with angiographically obtained measurements and hence can be used to estimate PDA diameter, shape and guide device decide selection. Routine echocardiography can be used successfully to plan the intervention and in some cases to guide transcatheter closure.

Real-World Experience Measurement of Liver Iron Concentration by R2 vs. R2 Star MRI in Hemoglobinopathies.

BACKGROUND: Non-invasive determination of liver iron concentration (LIC) is a valuable tool that guides iron chelation therapy in transfusion-dependent patients. Multiple methods have been utilized to measure LIC by MRI. The purpose of this study was to compare free breathing R2* (1/T2*) to whole-liver Ferriscan R2 method for estimation of LIC in a pediatric and young adult population who predominantly have hemoglobinopathies. METHODS: Clinical liver and cardiac MRI scans from April 2016 to May 2018 on a Phillips 1.5 T scanner were reviewed. Free breathing T2 and T2* weighted images were acquired on each patient. For T2, multi-slice spin echo sequences were obtained. For T2*, a single mid-liver slice fast gradient echo was performed starting at 0.6 ms with 1.2 ms increments with signal averaging. R2 measurements were performed by Ferriscan analysis. R2* measurements were performed by quantitative T2* map analysis. RESULTS: 107 patients underwent liver scans with the following diagnoses: 76 sickle cell anemia, 20 Thalassemia, 9 malignancies and 2 Blackfan Diamond anemia. Mean age was 12.5 ± 4.5 years. Average scan time for R2 sequences was 10 min, while R2* sequence time was 20 s. R2* estimation of LIC correlated closely with R2 with a correlation coefficient of 0.94. Agreement was strongest for LIC < 15 mg Fe/g dry weight. Overall bias from Bland-Altman plot was 0.66 with a standard deviation of 2.8 and 95% limits of agreement -4.8 to 6.1. CONCLUSION: LIC estimation by R2* correlates well with R2-Ferriscan in the pediatric age group. Due to the very short scan time of R2*, it allows imaging without sedation or anesthesia. Cardiac involvement was uncommon in this cohort.

3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions.

Novel commercially available software has enabled registration of both CT and MRI images to rapidly fuse with X-ray fluoroscopic imaging. We describe our initial experience performing cardiac catheterisations with the guidance of 3D imaging overlay using the VesselNavigator system (Philips Healthcare, Best, NL). A total of 33 patients with CHD were included in our study. Demographic, advanced imaging, and catheterisation data were collected between 1 December, 2016 and 31 January, 2019. We report successful use of this technology in both diagnostic and interventional cases such as placing stents and percutaneous valves, performing angioplasties, occlusion of collaterals, and guidance for lymphatic interventions. In addition, radiation exposure was markedly decreased when comparing our 10-15-year-old coarctation of the aorta stent angioplasty cohort to cases without the use of overlay technology and the most recently published national radiation dose benchmarks. No complications were encountered due to the application of overlay technology. 3D CT or MRI overlay for CHD intervention with rapid registration is feasible and aids decisions regarding access and planned angiographic angles. Operators found intraprocedural overlay fusion registration using placed vessel guidewires to be more accurate than attempts using bony structures.

Feasibility of real-time cine cardiac magnetic resonance imaging to predict the presence of significant retrosternal adhesions prior to redo-sternotomy.

BACKGROUND: Injury to vital structures posterior to the sternum is a complication associated with redo sternotomy in congenital cardiac surgery. The goal of our study was a novel evaluation of real-time cine cardiovascular magnetic resonance (CMR) to predict the presence of significant retrosternal adhesions of cardiac and vascular structures prior to redo sternotomy in patients with congenital heart disease. METHODS: Twenty-three patients who had prior congenital heart surgery via median sternotomy had comprehensive CMR studies prior to redo sternotomy. The real time cine (RTC) sequence that was used is an ungated balanced steady-state free precession (bSSFP) sequence using SENSitivity Encoding for acceleration with real-time reconstruction. Spontaneously breathing patients were instructed to take deep breaths during the acquisition whilst increased tidal volumes were delivered to mechanically ventilated patients. All patients underwent redo cardiac surgery subsequently and the presence and severity of retrosternal adhesions were noted at the time of the redo sternotomies. RESULTS: Median age at the time of CMR and operation were 5.5 years (range, 0.2-18.4y) and 6.1 years (range, 0.3-18.8y) respectively. There were 15 males and 8 females in the study group. Preoperative retrosternal adhesions were identified on RTC in 13 patients and confirmed in 11 (85%) at the time of surgery. In only 2 patients, no adhesions were identified on CMR but were found to have significant retrosternal adhesions at surgery; false positive rate 15% (CI 0.4-29.6%), false negative rate 20% (CI 3.7-36.4%). The total classification error of the real time cine sequence was 17% (CI 1.7-32.4%) with an overall accuracy of 83% (CI 67.7-98.4%). Standard breath-hold cine images correlated poorly with surgical findings and did not increase the diagnostic yield. CONCLUSIONS: RTC imaging can predict the presence of significant retrosternal adhesions and thus help in risk assessment prior to redo sternotomy. These findings complement the surgical planning and potentially reduce surgical complications .

Vitamin D Status After Cardiopulmonary Bypass in Children With Congenital Heart Disease.

Deficiency in 25-hydroxyvitamin D (25OHD) is associated with increased morbidity and mortality in the critically ill. Children who underwent surgery for congenital heart disease under cardiopulmonary bypass (CPB) are typically deficient in 25OHD. It is unclear whether this deficiency is due to CPB. We hypothesized that CPB reduces the levels of 25OHD in children with congenital heart disease. We conducted a prospective observational study on children aged 2 months to 17 years who underwent CPB. Serum was collected at 3 time points: immediately before, immediately after surgery, and 24 hours after surgery. 25-Hydroxyvitamin D, 1,25-dihydroxyvitamin D, 1,25(OH)2D, vitamin D binding protein, and albumin levels were measured. Levels were compared using repeated measures analysis of variance. We enrolled 20 patients, 40% were deficient in 25OHD with levels <20 ng/mL prior to surgery. Mean (±standard deviation) of 25OHD at the 3 time points was 21.3 ± 8 ng/mL, 19 ± 5.8 ng/mL, and 19.5 ± 6.6 ng/mL, respectively ( P = .02). The decrease in 25OHD was observed primarily in children with sufficient levels of 25OHD, with mean levels at the 3 time points: 26.8 ± 4.2 ng/mL, 21.5 ± 5.7 ng/mL, and 23.0 ± 4.9 ng/mL, respectively ( P < .001). Calculated means of free fraction of 25OHD at the 3 time points were 6.2 ± 2.8 pg/mL, 5.8 ± 2.2 pg/mL, and 5.5 ± 2.4 pg/mL, respectively, ( P = .04). Mean levels of 1,25(OH)2D were 63.7 ± 34.9 ng/mL, 53.2 ± 30.6 ng/mL, and 67.7 ± 23.5 ng/mL ( P = .04). Vitamin D binding protein and albumin levels did not significantly change. Cardiopulmonary bypass decreases 25OHD by reducing the free fraction. Current investigations are geared to establish whether vitamin D deficiency is associated with outcomes and if treatment is appropriate.

Iatrogenic left pulmonary artery to left atrium fistula.

Trans-catheter balloon angioplasty is a well-established treatment modality for pulmonary artery (PA) stenosis in children with congenital heart disease. We report a case of an unusual complication where a fistula developed between the left PA and the left atrium during balloon angioplasty in a patient with history of tetralogy of Fallot. This was successfully treated with placement of a covered stent.

A pediatric critical care perspective on vitamin D.

The mechanisms of action of vitamin D are the subject of intense investigation. Evidence now suggests vitamin D affects immune function and cell proliferation, prompting interest in its role in critical illness and cardiac disease. Multiple studies demonstrate strong associations between vitamin D deficiency and severity of illness including need for higher inotrope support, more fluid resuscitation, and longer intensive care unit stay. The pediatric cardiac population may be at even more risk and nearly twice as likely to be deficient compared to the noncardiac population. Low vitamin D levels have been found in postoperative cardiac patients, where investigators speculate cardiopulmonary bypass alters levels directly or indirectly. Patients with congestive heart failure who are deficient also seem to benefit from vitamin D supplementation. This review summarizes recent studies in children that investigate the relation between vitamin D status and clinical outcomes in the critically ill including those with cardiac disease.