First name
Meryl
Middle name
S
Last name
Cohen

Title

Sinus venosus defect of the pulmonary vein-type: An easily missed diagnosis.

Year of Publication

2022

Date Published

2022 Feb 15

ISSN Number

1540-8175

Abstract

<p>Atrial septal defects are one of the most common forms of congenital heart disease, however sinus venosus communications, particularly pulmonary vein-type defects, are rare and are easily misdiagnosed. Patients with pulmonary vein-type sinus venosus defects often present earlier than those with ostium secundum defects with significant right heart dilation. Correct diagnosis has important implications for management. We discuss the clinical courses and review multimodality imaging of three patients correctly diagnosed with pulmonary vein-type defects after an initial diagnosis of an ostium secundum atrial septal defect, in order to promote understanding of the unique anatomy of this entity.</p>

DOI

10.1111/echo.15310

Alternate Title

Echocardiography

PMID

35170076

Title

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

Year of Publication

2021

Date Published

2021 Feb 15

ISSN Number

1097-6795

Abstract

<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>

DOI

10.1016/j.echo.2021.02.007

Alternate Title

J Am Soc Echocardiogr

PMID

33600926

Title

Dynamic Annular Modeling of the Unrepaired Complete Atrioventricular Canal Annulus.

Year of Publication

2020

Date Published

2020 Dec 23

ISSN Number

1552-6259

Abstract

<p><strong>BACKGROUND: </strong>Repair of complete atrioventricular canal (CAVC) is often complicated by atrioventricular valve regurgitation, particularly of the left-sided valve. Understanding the three-dimensional (3D) structure of the atrioventricular canal annulus prior to repair may help to inform optimized repair. However, the 3D shape and movement of the CAVC annulus has yet to be quantified nor has it been rigorously compared to a normal mitral valve annulus.</p>

<p><strong>METHODS: </strong>The complete annuli of 43 patients with CAVC were modeled in 4 cardiac phases using transthoracic 3D echocardiograms and custom code. The annular structure was compared to the annuli of 20 normal pediatric mitral valves using 3D metrics and statistical shape analysis (Procrustes analysis).</p>

<p><strong>RESULTS: </strong>The unrepaired CAVC annulus varied in shape significantly throughout the cardiac cycle. Procrustes analysis visually demonstrated that the average normalized CAVC annular shape is more planar than the normal mitral annulus. Quantitatively, the annular height to valve width ratio of the native left CAVC atrioventricular valve was significantly lower than that of a normal mitral valve in all systolic phases(p&lt;0.001).</p>

<p><strong>CONCLUSIONS: </strong>The left half of the CAVC annulus is more planar than that of a normal mitral valve with an annular height to valve width ratio similar to dysfunctional mitral valves. Given the known importance of annular shape to mitral valve function, further exploration of the association of 3D structure to valve function in CAVC is warranted.</p>

DOI

10.1016/j.athoracsur.2020.12.013

Alternate Title

Ann Thorac Surg

PMID

33359720

Title

Preoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect.

Year of Publication

2019

Date Published

2019 May 07

ISSN Number

1432-1971

Abstract

<p>In complete atrioventricular canal defect (CAVC), there are limited data on preoperative clinical and echocardiographic predictors of operative timing and postoperative outcomes. A retrospective, single-center analysis of all patients who underwent primary biventricular repair of CAVC between 2006 and 2015 was performed. Associated cardiac anomalies (tetralogy of Fallot, double outlet right ventricle) and arch operation were excluded. Echocardiographic findings on first postnatal echocardiogram were correlated with surgical timing and postoperative outcomes using bivariate descriptive statistics and multivariable logistic regression. 153 subjects (40% male, 84% Down syndrome) underwent primary CAVC repair at a median age of 3.3 (IQR 2.5-4.2) months. Median postoperative length of stay (LOS) was 7 (IQR 5-15) days. Eight patients (5%) died postoperatively and 24 (16%) required reoperation within 1&nbsp;year. On multivariable analysis, small aortic isthmus (z score &lt; -&nbsp;2) was associated with early primary repair at &lt; 3&nbsp;months (OR 2.75, 95% CI 1.283-5.91) and need for early reoperation (OR 3.79, 95% CI 1.27-11.34). Preoperative ventricular dysfunction was associated with higher postoperative mortality (OR 7.71, 95% CI 1.76-33.69). Other factors associated with mortality and longer postoperative LOS were prematurity (OR 5.30, 95% CI 1.24-22.47 and OR 5.50, 95% CI 2.07-14.59, respectively) and lower weight at surgery (OR 0.17, 95% CI 0.04-0.75 and OR 0.55, 95% CI 0.35-0.85, respectively). Notably, preoperative atrioventricular valve regurgitation and Down syndrome were not associated with surgical timing, postoperative outcomes or reoperation, and there were no echocardiographic characteristics associated with late reoperation beyond 1 year after repair. Key preoperative echocardiographic parameters helped predict operative timing and postoperative outcomes in infants undergoing primary CAVC repair. Aortic isthmus z score &lt; -&nbsp;2&nbsp; was associated with early surgical repair and need for reoperation, while preoperative ventricular dysfunction was associated with increased mortality. These echocardiographic findings may help risk-stratified patients undergoing CAVC repair and improve preoperative counseling and surgical planning.</p>

DOI

10.1007/s00246-019-02116-0

Alternate Title

Pediatr Cardiol

PMID

31065759

Title

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

Year of Publication

2019

Date Published

2019 Mar 15

ISSN Number

1552-6259

Abstract

<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>

DOI

10.1016/j.athoracsur.2019.02.017

Alternate Title

Ann. Thorac. Surg.

PMID

30885849

Title

Cardiovascular Disease in the Young Council's Science and Clinical Education Lifelong Learning Committee: Year in Review.

Year of Publication

2018

Number of Pages

e010617

Date Published

2018 Nov 06

ISSN Number

2047-9980

DOI

10.1161/JAHA.118.010617

Alternate Title

J Am Heart Assoc

PMID

30571390

Title

Accuracy of transesophageal echocardiography in the identification of postoperative intramural ventricular septal defects.

Year of Publication

2016

Number of Pages

688-95

Date Published

2016 09

ISSN Number

1097-685X

Abstract

<p><strong>BACKGROUND: </strong>Intramural ventricular septal defects (VSDs), residual interventricular communications occurring after repair of conotruncal defects, are associated with poor postoperative outcomes. The ability of intraoperative transesophageal echocardiography (TEE) to identify intramural VSDs has not yet been evaluated.</p>

<p><strong>METHODS: </strong>Intraoperative TEE and postoperative transthoracic echocardiography (TTE) data in all patients undergoing all biventricular repair of conotruncal anomalies in our hospital between January 1, 2006, and June 30, 2013, were reviewed. The ability of TEE to accurately identify residual defects was assessed using postoperative TTE as the reference imaging modality.</p>

<p><strong>RESULTS: </strong>Intramural VSDs occurred in 34 of 337 patients evaluated; 19 were identified by both TTE and TEE, and 15 were identified by TTE only. Sensitivity was 56% and specificity was 100% for TEE to identify intramural VSDs. Peripatch VSDs were identified in 90 patients by both TTE and TEE, in 53 by TTE only, and in 15 by TEE only, yielding a sensitivity of 63% and specificity of 92%. Of the VSDs requiring catheterization or surgical reintervention, 6 of 7 intramural VSDs and all 5 peripatch VSDs were identified by intraoperative TEE. TEE guided the intraoperative decision to return to cardiopulmonary bypass (CPB) in an attempt to close residual defects in 12 patients with intramural VSDs and in 4 patients with peripatch VSDs seen after initial CPB; of these, 10 intramural VSDs and all 4 peripatch VSDs resolved or became smaller on final intraoperative TEE.</p>

<p><strong>CONCLUSIONS: </strong>TEE has modest sensitivity but high specificity for identifying intramural VSDs and can detect most defects requiring reintervention. Repeat attempts at closure in the index operation may successfully correct intramural VSDs identified by TEE.</p>

DOI

10.1016/j.jtcvs.2016.04.026

Alternate Title

J. Thorac. Cardiovasc. Surg.

PMID

27183884

Title

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

Year of Publication

2018

Date Published

2018 Jun 05

ISSN Number

1432-1971

Abstract

<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>

DOI

10.1007/s00246-018-1921-9

Alternate Title

Pediatr Cardiol

PMID

29872881

Title

Prevalence and Risk Factors for Pericardial Effusions Requiring Readmission After Pediatric Cardiac Surgery.

Year of Publication

2016

Date Published

2016 Nov 30

ISSN Number

1432-1971

Abstract

<p>Pericardial effusion (PE) may require readmission after cardiac surgery and has been associated with postoperative morbidity and mortality. We sought to identify the prevalence and risk factors for postoperative PE requiring readmission in children. A retrospective analysis of the Pediatric Health Information System database was performed between January 1, 2003, and September 30, 2014. All patients ≤18&nbsp;years old who underwent cardiac surgery were identified by ICD-9 codes. Those readmitted within 1&nbsp;year with an ICD-9 code for PE were identified. Logistic regression analysis was performed to determine risk factors for PE readmissions. Of the 142,633 surgical admissions, 1535 (1.1%) were readmitted with PE. In multivariable analysis, older age at the initial surgical admission [odds ratio (OR) 1.17, p&nbsp;&lt;&nbsp;0.001], trisomy 21 (OR 1.24, p&nbsp;=&nbsp;0.015), geographic region (OR 1.33-1.48, p&nbsp;≤&nbsp;0.001), and specific surgical procedures [heart transplant (OR 1.82, p&nbsp;&lt;&nbsp;0.001), systemic-pulmonary artery shunt (OR 2.23, p&nbsp;&lt;&nbsp;0.001), and atrial septal defect surgical repair (OR 1.34, p&nbsp;&lt;&nbsp;0.001)] were independent risk factors for readmission with PE. Of readmitted patients, 44.2% underwent an interventional PE procedure. Factors associated with interventions included shorter length of stay (LOS) for the initial surgical admission (OR 0.85, p&nbsp;=&nbsp;0.008), longer LOS for the readmission (OR 1.37, p&nbsp;&lt;&nbsp;0.001), and atrial septal defect surgery (OR 1.40, p&nbsp;=&nbsp;0.005). In this administrative database of children undergoing cardiac surgery, readmissions for PE occurred after 1.1% of cardiac surgery admissions. The risk factors identified for readmissions and interventions may allow for improved risk stratification, family counseling, and earlier recognition of PE for children undergoing cardiac surgery.</p>

DOI

10.1007/s00246-016-1540-2

Alternate Title

Pediatr Cardiol

PMID

27900408

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