Optical Detection of Intracranial Pressure and Perfusion Changes in Neonates With Hydrocephalus.

2021

2021 May 15

1097-6833

<p><strong>OBJECTIVE: </strong>To demonstrate that a novel non-invasive index of intracranial pressure (ICP) derived from diffuse optics-based techniques is associated with intracranial hypertension.</p>

<p><strong>STUDY DESIGN: </strong>We compared non-invasive and invasive ICP measurements in infants with hydrocephalus. Infants born term and preterm were eligible for inclusion if clinically determined to require cerebrospinal fluid (CSF) diversion. Ventricular size was assessed preoperatively via ultrasound measurement of the fronto-occipital (FOR) and fronto-temporal (FTHR) horn ratios. Invasive ICP was obtained at the time of surgical intervention with a manometer. Intracranial hypertension was defined as invasive ICP ≥15 mmHg. Diffuse optical measurements of cerebral perfusion, oxygen extraction, and non-invasive ICP were performed preoperatively, intraoperatively, and postoperatively. Optical and ultrasound measures were compared with invasive ICP measurements, and their change in values after CSF diversion were obtained.</p>

<p><strong>RESULTS: </strong>We included 39 infants; 23 had intracranial hypertension. No group difference in ventricular size was found by FOR (p=0.93) or FTHR (p=0.76). Infants with intracranial hypertension had significantly higher non-invasive ICP (p=0.02) and oxygen extraction fraction (p=0.01) compared with infants without intracranial hypertension. Increased cerebral blood flow (p=0.005) and improved oxygen extraction fraction (P &lt; .001) after CSF diversion were only observed in infants with intracranial hypertension.</p>

<p><strong>CONCLUSIONS: </strong>Non-invasive diffuse optical measures (including a non-invasive ICP index) were associated with intracranial hypertension. The findings suggest impaired perfusion from intracranial hypertension was independent of ventricular size. Hemodynamic evidence of the benefits of CSF diversion was seen in infants with intracranial hypertension. Non-invasive optical techniques hold promise for aiding the assessment of CSF diversion timing.</p>

10.1016/j.jpeds.2021.05.024

J Pediatr

34004191

Venous flow variation predicts preoperative pulmonary venous obstruction in children with total anomalous pulmonary venous connection.

2021

2021 Feb 15

1097-6795

<p><strong>OBJECTIVE: </strong>Identifying preoperative pulmonary venous obstruction in total anomalous pulmonary venous connection (TAPVC) is important to guide treatment-planning and risk prognostication. No standardized echocardiographic definition of obstruction exists in the literature. Definitions based on absolute velocities are affected by technical limitations and variations in pulmonary venous return. We developed a metric to quantify pulmonary venous blood flow variation: pulmonary venous variability index (PVVI). We aimed to demonstrate its accuracy in defining obstruction.</p>

<p><strong>METHODS: </strong>All patients cared for with TAPVC at our institution were identified. Echocardiograms were reviewed, and maximum (V), mean (V), and minimum velocities (V) along the pulmonary venous pathway were measured. PVVI was defined as (V-V)/V. These metrics were compared to pressures measured by cardiac catheterization. Echocardiographic measures were then compared between the patients with and without clinical preoperative obstruction (defined as a need for preoperative intubation, catheter-based intervention, or surgery within one day of diagnosis), as well as pulmonary edema by chest X-ray and markers of lactic acidosis. 137 patients were included with 22 having catheterization pressure recordings.</p>

<p><strong>RESULTS: </strong>Maximum and mean velocity were not different between patients with catheter gradients ≥4 mmHg and &lt;4 mmHg, while PVVI was significantly lower and minimum velocity higher in those with gradients ≥4 mmHg. The composite outcome of preoperative obstruction occurred in 51 patients (37%). Absolute velocities were not different between patients with and without clinical obstruction, while PVVI was significantly lower in patients with obstruction. All metrics except maximum velocity were associated with pulmonary edema; none were associated with blood gas metrics.</p>

<p><strong>CONCLUSIONS: </strong>We developed a novel quantitative metric of pulmonary venous flow, which was superior to traditional echocardiographic metrics. Decreased PVVI was highly associated with elevated gradients measured by catheterization and clinical preoperative obstruction. These results should aid risk assessment and diagnosis preoperatively in patients with TAPVC.</p>

10.1016/j.echo.2021.02.007

J Am Soc Echocardiogr

33600926

Interventricular septal hematoma complicating placement of a ventricular assist device in an infant and support with bi-atrial cannulation.

2020

90-92

2020 Mar

2666-2507

<p>Interventricular septal hematoma is a rare complication of pediatric cardiac surgery, usually in the context of ventricular septal defect closure.&nbsp;We present intraoperative echocardiography of a large interventricular septal hematoma secondary to the placement of a left ventricular assist device (VAD) in an infant who required conversion to biventricular support with bi-atrial cannulation. As the patient later died of neurologic complications, we present correlation with pathologic findings.</p>

10.1016/j.xjtc.2019.10.001

JTCVS Tech

33103126

Echocardiographic Assessment of Diastolic Function in Children with Incident Systemic Lupus Erythematosus.

2019

2019 Apr 30

1432-1971

<p>The timing and etiology of diastolic impairment in pediatric-onset systemic lupus erythematosus (SLE) are poorly understood. We compared echocardiographic metrics of left ventricular diastolic function in children at SLE diagnosis to controls and identified factors associated with diastolic indices. Echocardiograms of children aged 5-18&nbsp;years within 1&nbsp;year of SLE diagnosis and age-/sex-matched controls were retrospectively read by blinded cardiologists. Clinical characteristics were abstracted separately. Z-scores for diastolic indices (E/A, e', E/e', and isovolumetric relaxation time (IVRT)) were calculated using published normative data and study controls, and compared using linear mixed-effects models adjusted for blood pressure. Pericardial effusions and valvular disease were also evaluated. Linear regression was used to identify factors associated with diastolic measures. 85 children with incident SLE had echocardiograms performed a median of 6&nbsp;days after diagnosis (interquartile range (IQR) 1-70). Prior cumulative prednisone exposure was minimal (median 60&nbsp;mg, IQR 0-1652). SLE cases had lower E/A, lower e', higher E/e', and longer IVRT compared to controls. Though none met criteria for Grade I diastolic dysfunction, Z-scores for e', E/e', and IVRT were abnormal in 30%, 25%, and 6% of SLE cases, respectively. Greater disease activity was associated with lower septal e' (p &lt; 0.01), higher E/e' (p = 0.02), and longer IVRT (p &lt; 0.01). Children with incident SLE have worse diastolic indices at diagnosis compared to peers without SLE, independent of blood pressure and prior to significant prednisone exposure. Longitudinal studies will determine whether diastolic dysfunction develops in this population over time.</p>

10.1007/s00246-019-02107-1

Pediatr Cardiol

31041461

Echocardiographic Assessment of Right Ventricular Function in Clinically Well Pediatric Heart Transplantation Patients and Comparison With Normal Control Subjects.

2019

537-544.e3

2019 Apr

1097-6795

<p><strong>BACKGROUND: </strong>Echocardiographic follow-up after pediatric heart transplantation is important because of the lifelong risk for rejection and resultant ventricular dysfunction. Although adult studies have shown that echocardiographic measures of right ventricular function are changed after transplantation, similar results have not been reported in the pediatric population.</p>

<p><strong>METHODS: </strong>A single-center retrospective study of echocardiograms obtained among pediatric heart transplant recipients was conducted. All echocardiograms were selected remote from transplantation, rejection, or graft vasculopathy. These criteria identified 127 patients. Right ventricular systolic function was measured using tricuspid annular plane systolic excursion, fractional area change (FAC), and peak systolic tricuspid annular tissue velocity (S'). Results were compared with those in 380 healthy age-matched echocardiographic control subjects.</p>

<p><strong>RESULTS: </strong>Tricuspid annular plane systolic excursion values in pediatric heart transplant recipients were significantly lower than in control subjects at all ages (P&nbsp;&lt;&nbsp;.0001), with a mean Z score of -3.38. FAC and S' did not vary by age in control patients &gt;6&nbsp;months of age. FAC values in transplantation patients were significantly decreased compared with those in control subjects (P&nbsp;&lt;&nbsp;.0001), but 83% of transplantation patients had FAC values within the control-derived normal range. S' values were also significantly lower in transplantation patients than control subjects (P&nbsp;&lt;&nbsp;.0001).</p>

<p><strong>CONCLUSIONS: </strong>Heart transplantation patients have significantly decreased quantitative metrics of right ventricular function relative to healthy control subjects; longitudinal shortening (tricuspid annular plane systolic excursion and S') is particularly affected. FAC is relatively preserved and may be a better metric in this population. These results establish nomograms of RV function in pediatric heart transplantation patients and in normal pediatric control subjects, which may allow quantification of changes in this vulnerable population.</p>

10.1016/j.echo.2019.01.015

J Am Soc Echocardiogr

30954122

Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction.

2019

2019 Mar 15

1552-6259

<p><strong>BACKGROUND: </strong>Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) results in substantial morbidity and mortality. Risk factors for postoperative obstruction remain ambiguous. Additionally, the existing literature has no standard definition for preoperative obstruction making patient counseling difficult.</p>

<p><strong>METHODS: </strong>All patients undergoing repair of TAPVC at our institution from 1/1/06 to 10/23/17 were identified. The primary outcome was the development of postoperative obstruction, analyzed as a time-to-event outcome. Clinical information was extracted to assess risk factors. Degrees of preoperative obstruction were defined based on echocardiographic, catheterization, and clinical findings. Univariable and multivariable Cox proportional hazard regression methods were used to identify factors associated with the primary outcome.</p>

<p><strong>RESULTS: </strong>During the study interval, 119 patients underwent repair of TAPVC (40% single-ventricle), and 25 patients (21%) developed postoperative obstruction. Risk factors associated with obstruction were heterotaxy syndrome, single-ventricle heart disease, additional surgeries at the time of vein repair, mixed-type TAPVC, and preoperative obstruction. Having even mild preoperative obstruction (≥1.2 m/s by Doppler echocardiography) was predictive of postoperative obstruction. A multivariable model showed mixed-type TAPVC and the presence of preoperative obstruction were associated with over 2-fold greater hazards of obstruction.</p>

<p><strong>CONCLUSIONS: </strong>TAPVC in the setting of heterotaxy and single-ventricle remains challenging with high rates of postoperative obstruction. Mixed-type TAPVC is an independent risk factor for postoperative obstruction, particularly in patients with isolated TAPVC. Even mild preoperative obstruction is a risk factor for postoperative obstruction. These results may help risk-stratify TAPVC patients.</p>

10.1016/j.athoracsur.2019.02.017

Ann. Thorac. Surg.

30885849

Postoperative Obstruction of the Pulmonary Veins in Mixed Total Anomalous Pulmonary Venous Connection.

2018

2018 Jun 05

1432-1971

<p>Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease in which the pulmonary veins drain by various pathways to the right atrium instead of the left atrium. Postoperative obstruction of the pulmonary veins is a known complication. Identifying risk factors for morbidity and mortality is important for counseling and monitoring. We describe a pattern of postoperative obstruction in a specific arrangement of mixed TAPVC. Five patients with a type of mixed TAPVC, namely, three pulmonary veins connecting to the coronary sinus and the left upper pulmonary vein (LUPV) connecting to the innominate vein, were identified over an 11-year period at our institution. Two additional patients with this TAPVC arrangement were cared for at our institution after having surgery at other institutions. Of these, one patient received only comfort care at birth due to other clinical issues. The six other patients underwent surgical unroofing of the coronary sinus. The anomalous LUPV was not addressed during the initial surgery in any of these cases. Following repair, one patient died from non-cardiac reasons. The remaining five patients all developed obstruction of the repaired pulmonary veins with decompression through the unrepaired LUPV, requiring surgical revision. Three patients underwent a second reoperation as well. Three of the six repaired patients also developed refractory atrial arrhythmias. This cohort suggests that this mixed TAPVC pattern predisposes patients to obstruction after surgical repair. Further investigation may aid pediatric cardiologists in risk-stratifying and counseling these patients. Alternative surgical approaches may need to be considered.</p>

10.1007/s00246-018-1921-9

Pediatr Cardiol

29872881