Incidence, risk factors, and management following cardiac catheterization via carotid and axillary artery approaches: A single-center experience on pseudoaneurysms in young infants.

BACKGROUND: Use of alternate access for complex neonatal interventions has gained acceptance with carotid and axillary artery access being used for ductal and aortic interventions. METHODS: This study was a retrospective, single-center study at Cincinnati Children's Hospital Medical Center. The study included infants, aged ≤90 days, who underwent cardiac catheterization with either carotid or axillary artery access between 2013 and 2022. Data encompassing demographics, clinical information, catheterization data, and the incidence of pseudoaneurysm as a procedural complication were collected. RESULTS: Among 29 young infants (20 males, 69%), 4 out of 15 patients (27%) who underwent the carotid approach developed pseudoaneurysms, while 1 out of 14 patients (7.1%) who underwent the axillary approach developed one. Two patients required transcatheter intervention due to enlargement of pseudoaneurysms, involving the placement of transarterial flow-diverting stent and occlusion of left common carotid artery. Longer sheath in-to-out time (135 vs. 77 min, p = 0.001), and higher closing activated clotting times (ACT) (268 vs. 197 s, p = 0.021) were observed among patients with pseudoaneurysms compared to those without. CONCLUSIONS: Young infants with alternative access via the carotid and axillary arteries may be at risk of pseudoaneurysm formation during longer procedures and with higher ACTs for closure. Ultrasound-guided compression can be employed to prevent the progression and in resolution of these lesions.

Left ventricle hypertrophy and re-modeling in children with essential hypertension: does the race matter?

BACKGROUND: This is the first study to report on the impact of race on differences in the prevalence of echocardiographic left ventricular hypertrophy and left ventricular adaptation at the time of diagnosis of essential hypertension in children. METHODS: This cross-sectional, single-centre study included patients aged 3-18 years who had newly diagnosed essential hypertension. Echocardiography was used to assess left ventricular mass index and left ventricular relative wall thickness. An left ventricular mass index > the 95th percentile for age and gender, and an left ventricular relative wall thickness > 0.42, were used to diagnose left ventricular hypertrophy and concentric adaptation. Various echocardiographic parameters were compared between African Americans and Caucasians. RESULTS: The study included 422 patients (289 African Americans and 133 Caucasians) diagnosed with essential hypertension at a median age of 14.6 (interquartile range; 12.1-16.3) years. Eighty-eight patients (20.9%) had left ventricular hypertrophy. There was no statistically significant difference in the prevalence of left ventricular hypertrophy between African Americans and Caucasians (22.5% versus 17.3%, p=0.22). The median left ventricular relative wall thickness was 0.35 (0.29-0.43), and 114 patients (27.0%) had an left ventricular relative wall thickness > 0.42. The presence of an left ventricular relative wall thickness > 0.42 was significantly higher among African Americans compared to Caucasians (30.1% versus 20.3%, p = 0.04). The African American race was a strong predictor for an left ventricular relative wall thickness > 0.42 (odds ratio 1.7, p = 0.04), but not for left ventricular mass index > the 95th percentile (p = 0.22). Overweight/obesity was a strong predictor for an left ventricular mass index > the 95th percentile. CONCLUSIONS: There was no difference in the prevalence of left ventricular hypertrophy in children with essential hypertension of different races. Obesity, rather than being African American, is associated with left ventricular hypertrophy.

Exercise Capacity After Arterial Switch Operation in Patients with D-Transposition of Great Arteries: Does the Coronary Artery Anatomy Matter?

Variant coronary anatomy (VarCA) is frequent in D-Transposition of the great arteries (d-TGA). There are a paucity of data on the effect of the VarCA on the exercise capacity (XC) in patients with repaired d-TGA. This retrospective study included patients with d-TGA who underwent an arterial switch operation (ASO) and had at least one cardiopulmonary exercise test (CPET). Data from the treadmill CPET and simultaneously performed spirometry were collected. The parameters of CPET were compared between patients with usual anatomy vs. VarCA. Longitudinal changes in XC in patients with ASO were also analyzed. A total of 44 patients with either usual coronary anatomy (n = 27, 61%) or VarCA (n = 17, 39%) met inclusion criteria. There was no significant difference in oxygen consumption (%VO2) at initial CPET (104 vs. 100%, p = 0.53) between the two groups. Abnormal %VO2 (< 85%) was uncommon in both groups (n = 2, 7.4% vs. n = 4; 23.5%; p = ns). For longitudinal changes, there was no significant decline in %VO2 in either group: (i) usual coronary anatomy (n = 15, median follow-up 4.8 years, %VO2 111 vs. 108%; p = 0.306) and (ii) VarCA (n = 10, median follow-up 6.6 years, %VO2 106 vs. 92%; p = 0.441). Spirometry was abnormal in 25 (59.5%) patients [restrictive (n = 8, 19.0%), obstructive (n = 15, 35.7%), and mixed (n = 2, 4.8%)] butabnormal spirometry had no impact on the XC. Patients with d-TGA who underwent neonatal ASO uniformly exhibited good XC without any longitudinal decline on medium-term follow-up, regardless of coronary artery anatomy. Although frequent (60%), abnormal spirometry was not associated with reduced exercise capacity.

An echocardiographic finding mimicking tricuspid atresia in a neonate with dilated cardiomyopathy.

We report a neonate with dilated cardiomyopathy and have echocardiographic findings consistent with "functional" tricuspid atresia. There was an echo-bright, plate-like tissue at the tricuspid valve position with no forward flow across it. This report underscores the role of right ventricle intracavitary haemodynamic influence on the tricuspid valve leaflet excursion and demonstrates a phenomenon of "pseudo or functional tricuspid atresia" mimicking tricuspid atresia in a patient with acute presentation of cardiomyopathy.

Blood Pressure Response to Treadmill Cardiopulmonary Exercise Test in Children with Normal Cardiac Anatomy and Function.

OBJECTIVES: To describe blood pressure (BP) responses during cardiopulmonary exercise testing (CPET) and propose criteria for abnormal BP responses in children with normal hearts. STUDY DESIGN: Treadmill CPET data of children <18 years with normal hearts were retrospectively analyzed to evaluate BP responses to exercise at the Children's Hospital of Michigan over an 8-year period (2011-2018). RESULTS: Our cohort included 1085 children (boys, 59%) aged 7-17 years. Analysis of variance for systolic BP at peak exercise (peak SBP) and SBP change (Δ) showed significant age and sex effects and age-sex interaction effects (P < .01). In the multiple linear regression model (P < .001, R square 0.298, standard error of the estimate 17.15), peak SBP (mm Hg) was predicted as 132.27 + 1.37 × age (years) + (3.31 × age (years) - 31.88) × sex [boys 1, girls 0]. The following criteria for abnormal BP responses in children were proposed: hypertensive BP response was defined by peak SBP with a 90% CI upper limit of reference values based on age and sex, and blunted BP response as ΔSBP <10 mm Hg for aged 7-11 years, <20 mm Hg for boys aged 12-17 years, and ΔSBP <10 mm Hg in girls. CONCLUSIONS: BP responses to treadmill CPET depend on age and sex in children with normal hearts. The proposed criteria for abnormal BP responses in children during CPET will need to be verified in a large pediatric cohort.

Pneumocystis pneumonia with hypogammaglobulinemia in a pediatric patient who underwent heart transplantation.

A 23-month-old Caucasian female who had heart transplantation (HT) at 11 days of age was diagnosed with Pneumocystis pneumonia (PCP) in the setting of secondary hypogammaglobulinemia (HGG). She was diagnosed with HGG at 5 months of age and had been receiving monthly intravenous immunoglobulin infusion. This is the first case report describing the clinical course of PCP in a pediatric patient with HGG. She developed PCP 23 months after HT even when she was off steroids and was receiving timely IVIG. The case posed some clinical questions regarding PCP prophylaxis and HGG management.

Ductus arteriosus aneurysm with left pulmonary artery obstruction.

The echogenic mass in the pulmonary artery is a rare finding in newborns. Differential diagnoses include pulmonary artery thrombosis, ductal aneurysm, and malignant tumor. We report a newborn case who presented with mild desaturation and was found to have a large echogenic mass at the pulmonary bifurcation on echocardiography, which caused partial flow obstruction in the proximal left pulmonary artery. Along with the findings of cardiac computed tomography and magnetic resonance imaging, the diagnosis of ductus arteriosus aneurysm (DAA) was made. This DAA spontaneously regressed without any intervention at 4 months follow-up.

The Identification and Impact of Abnormal Spirometry Patterns on Exercise Capacity in Pediatric Patients with Fontan Palliation.

Reduced exercise capacity and restrictive lung physiology are common in patients after Fontan palliation (FP). However, there is paucity of data regarding the association between specific spirometry patterns and key exercise parameters in this population. This is a single-center, cross-sectional, study correlating pulmonary function and exercise parameters in children with FP. Patients who were ≤ 18 years of age and underwent a comprehensive cardiopulmonary treadmill exercise stress test (CPT) and spirometry at the same time, were included. Patients were categorized as (i) normal or (ii) abnormal based on the results of spirometry. The abnormal group was subdivided into (a) restrictive, (b) obstructive, and (c) mixed patterns. Demographic and key exercise parameters were compared between groups. Our study included 82 patients who underwent CPT at 13.6 (IQR, 11.3-15.4) years of age. A reduced exercise capacity (%VO2 ≤ 85%) was noted in the majority (n = 50, 61%). Spirometry was abnormal in 47 (57%) patients [restrictive (n = 25, 30%), obstructive (n = 12, 15%), and mixed (n = 10, 12%)]. The abnormal spirometry group had significant lower %VO2 (77% vs. 92%, p = 0.01) and METS (8.4 vs. 9.6, p = 0.02). Subgroup analysis revealed that obstructive (p = 0.04) and mixed (p = 0.02) patterns were associated with a significant decrease in % VO2. Majority of the children demonstrated an abnormal spirometry pattern post-FP. Abnormal pulmonary function was associated with the reduced exercise capacity. Identification and treatment of the abnormal lung function may improve the exercise capacity in these patients and improve the morbidity.

Longitudinal Changes in Exercise Capacity in Patients Who Underwent Ross Procedure and Mechanical Aortic Valve Replacement: Does the Type of Surgery Matter?

The surgical options for significant aortic valve disease include either Ross procedure (RP) or aortic valve replacement (AVR). The exercise stress test is routinely performed in these patients to assess the objective functional capacity. This retrospective study was conducted to evaluate the differences and the longitudinal changes of exercise capacity in patients following the RP and AVR for aortic valve disease. This is an IRB approved retrospective study and included patients who had either RP or AVR performed for aortic valve disease and had at least one exercise stress test performed after the surgical procedure. Patients with other congenital heart disease, pacemaker or defibrillators, and those with inadequate data were excluded. Demographic data including age at surgery, type of surgery and type of aortic valve was collected. Data regarding treadmill cardiopulmonary exercise test (CPET) was also collected. A total of 47 patients met inclusion criteria and were equally represented in each group, i.e. RP [n = 23, 73.9% male, age at surgery 11.2 (4.5-15.9) years] vs. AVR [n = 24, 88% mechanical AVR, 60.9% male, age at surgery 15.1 (12.8-19.4) years]. There was a significant decline in predicted oxygen consumption (%VO2) at time of first post-operative CPET in patients after AVR compared to RP (79 vs. 88%, p = 0.048) over a similar accrued median interval follow-up (4.6 vs. 6.2 years, p = 0.2). The longitudinal follow-up analysis of following AVR (n = 11, 54.5% male, median inter-test duration of 5 years) showed significant decline in peak exercise capacity or VO2 (34.2 vs. 26.2 vs., p = 0.006). In contrast, after RP (n = 12 patients [58.3% male, median inter-test duration 7.1 of years], exercise capacity and other key parameters remained preserved. In this small sentinel study, we report a better initial exercise capacity among patients after RP compared to AVR over an intermediate follow-up. During longitudinal follow-up in a subset of patients, exercise capacity remained preserved amongst the RP group while it further declined in the AVR group.

Left ventricular outflow tract obstruction in a patient with pulmonary atresia with intact ventricle septum following Fontan procedure: a rare complication.

Left ventricular outflow tract obstruction in patients with pulmonary atresia with intact ventricular septum has been rarely reported. Data are lacking on the impact and management of systemic ventricular outflow tract obstruction that developed following the Fontan procedure. We report a case of an 8-year-old male who developed left ventricular outflow tract obstruction 6 months after the Fontan procedure.

Severe conduit stenosis in a patient with Fontan circulation with a Y-shaped Dacron conduit.

Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.

A rhabdomyoma in the right ventricle presenting as hemodynamics of hypoplastic right heart.

Rhabdomyomas are the most common paediatric cardiac tumours. The natural history of these tumours is mostly benign, and the tumour usually regresses spontaneously. Although surgical resection of these tumours is one of the considerations in patients with ventricular outflow obstruction, a palliation with Blalock-Taussig shunt is an alternative approach with the hope of regression of the tumour over time. We report a case of prenatally diagnosed rhabdomyomas in the right ventricle and its outflow presenting as hemodynamic simulating hypoplastic right ventricle in a newborn. She required prostaglandin and Blalock-Taussig shunts palliation for pulmonary flow and subsequent regression of tumours.

Silent in situ thrombus within a hypoplastic left ventricle in a teenager with Fontan palliation.

Thrombosis, especially in the Fontan pathway, is one of the major concerns in patients who underwent Fontan surgery, with reported prevalence of 5-33%. We report a case of thrombus in a rudimentary left ventricle in teenager with no arrhythmia or neurological complications. We also report the special concerns of silent thrombus and role of cardiac MRI in diagnosing an intracardiac thrombus.

Recurrent infective endocarditis in a patient after a second Melody valve placement.

A 16-year-old Caucasian male was diagnosed with recurrent infective endocarditis associated with his second Melody valve placed inside a previously treated infected Melody valve. The replacement of Melody valve after the first infective endocarditis episode might increase the risk of the second episode of infective endocarditis.

Community-acquired lobar pneumonia caused by Pseudomonas aeruginosa infection in Japan: a case report with histological and immunohistochemical examination.

Pseudomonas aeruginosa is a common pathogen in nosocomial and/or healthcare-associated pneumonia, but is rare in community-acquired pneumonia. A 50-year-old previously healthy woman was taken to the emergency department because of rapidly progressing dyspnea. Chest radiograph showed consolidation of the entire right upper lobe, a finding suggestive of lobar pneumonia. The patient died of respiratory failure with bronchial bleeding, on the same day of admission. Autopsy revealed that the alveoli throughout the upper right lobe were filled with dense inflammatory cells mainly consisting of macrophages and neutrophils. Immunoreactive bacilli by using an anti-P. aeruginosa antibody were localized within macrophages accumulated in the alveoli as well in the vessel walls. Lobar pneumonia composed of dense neutrophils and bacteria-laden macrophages with total lung congestion and edema may be characteristic for community-acquired P. aeruginosa pneumonia in a healthy adult.

Subclavian Steal Phenomenon Associated With Vascular Ring in an Infant Who Had a Prenatally Diagnosed Right Aortic Arch With an Atretic Aberrant Left Subclavian Artery.

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.

Rapid progression of aortic stenosis in a 3-month-old infant with bicuspid aortic valve and DeSanto-Shinawi syndrome.

A 3-month-old female was diagnosed at 1 month of age with DeSanto-Shinawi syndrome (DSS) and bicuspid aortic valve with trivial stenosis. The aortic valve stenosis progressed to severe within 2 months and required balloon aortic valvuloplasty. This is the first case of aortic stenosis (AS) associated with DSS, and the syndrome may be the reason for the rapid worsening of AS in this case.

Impact of Patient Prosthesis Mismatch on the Outcome of Transcatheter Pulmonic Valve Implantation.

Patient prosthesis mismatch (PPM) is an important factor of the outcome in transcatheter aortic valve implantation. However, the impact of PPM in transcatheter pulmonic valve implantation (TPVI) has not been studied. Based on the narrowest valve stent diameters in two views of fluoroscopy, internal geometric orifice area (GOA) of the valve stent was calculated and indexed by body surface area (BSA), deriving iGOA. To define PPM in TPVI, receiver operating characteristics (ROC) curve analysis for iGOA for predicting significant residual right ventricular outflow tract (RVOT) gradient was used to derive the optimal cut-off value of iGOA. Our cohort were divided into 2 groups: PPM versus non-PPM. The clinical data were compared between 2 groups. TPVI was performed using Melody valve in 101 patients. Significant RVOT residual pressure gradient (≥ 15 mmHg) was observed in 31 patients (39.6%). Over a mean follow up periods of 6.9 ± 2.7 years, 22 patients (21.8%) required repeat interventions (16 transcatheter, 11 surgical, and both in 5 patients). Based on the ROC analysis, the best cut-off value of iGOA was 1.25 cm2/m2 (area under the curve 0.873, p < 0.001) to define PPM. PPM was present in 42 patients (42%). On the Kaplan-Meier survival analysis, PPM was associated with the need of repeat intervention (p = 0.02). In conclusion, in TPVI, PPM was a strong predictor for the need of re-intervention. Considering PPM, target diameter of valve stent would depend on the patient body size and should be taken into account for optimal outcome of TPVI.

Refeeding Syndrome with Hypoglycemia in a Severely Malnourished Infant.

A 5-mo-old severely malnourished 3.5 kg boy was brought to the emergency department with hypoglycemia, bradycardia, bradypnea, and hypothermia. His findings were likely due to severe malnutrition secondary to parental neglect. Resuscitation with dextrose containing intravenous fluids was promptly started. On day 2 of admission, refeeding was initiated. From that time, he had multiple hypoglycemic episodes along with hypophosphatemia, hypomagnesemia, and hypokalemia. Hypoglycemia was associated with the initiation of enteral feeding and an increase in calories and amounts of enteral feeding. Hypoglycemia associated with refeeding syndrome in infant has not been previously reported.