First name
Stephanie
Last name
Fuller

Title

Comparison of outcomes of pulmonary valve replacement in adult versus paediatric hospitals: institutional influence†.

Year of Publication

2019

Date Published

2019 Apr 08

ISSN Number

1873-734X

Abstract

OBJECTIVES: Controversy exists in ascertaining the ideal location for adults with congenital heart disease requiring surgical intervention. In this study, we sought to compare the perioperative management between our paediatric and adult hospitals and to determine how clinical factors and the location affect the length of stay after pulmonary valve replacement.

METHODS: A retrospective analysis of patients, ≥18 years of age, undergoing pulmonary valve replacement was conducted at our paediatric and adult hospitals between 1 January 2000 and 30 October 2014. Patients with previous Ross or concomitant left heart procedures were excluded. Descriptive statistics were used to assess demographics and clinical characteristics. Inverse probability weight-adjusted models were used to determine differences in the number of surgical complications, duration of mechanical ventilation and postoperative length of stay between paediatric and adult hospitals. Additional models were calculated to identify factors associated with prolonged length of stay.

RESULTS: There were altogether 98 patients in the adult (48 patients) and paediatric (50 patients) hospitals. Patients in the adult hospital were older with more comorbidities (arrhythmia, hypertension, depression and a history of cardiac arrest, all P < 0.05). Those at the paediatric hospital had better preoperative right ventricular function and less tricuspid regurgitation. The cardiopulmonary bypass time, the length of intubation and the length of stay were higher at the adult hospital, despite no difference in the number of complications between locations. Factors contributing to the increased length of stay include patient characteristics and postoperative management strategies. There were no deaths.

CONCLUSIONS: Pulmonary valve replacement may be performed safely with no deaths and with a comparable complication rate at both hospitals. Patients undergoing surgery at the adult hospital have longer intubation times and length of stay. Opportunities exist to streamline management strategies.

DOI

10.1093/ejcts/ezz102

Alternate Title

Eur J Cardiothorac Surg

PMID

30957859

Title

Impact of Transcatheter Pulmonary Artery Intervention Following Superior Cavopulmonary Connection on Pulmonary Artery Growth.

Year of Publication

2021

Number of Pages

635-642

Date Published

2021 Sep

ISSN Number

2150-136X

Abstract

<p><strong>INTRODUCTION: </strong>Balloon and stent angioplasty of the pulmonary arteries (PAs) are frequently performed following superior cavopulmonary connection (SCPC), not only to normalize the caliber of the affected PA but also in hopes of maximizing downstream growth over time. There are limited data on the impact on subsequent PA growth prior to total cavopulmonary connection (TCPC).</p>

<p><strong>METHODS: </strong>A single-center, retrospective cohort study was performed on children who underwent transcatheter (TC) PA intervention following SCPC between January 1, 2010, and December 31, 2018. Growth of treated and contralateral PAs was measured at the lobar bifurcation (distal branch PA [DBPA]) and in the proximal lower lobe (lower lobe branch [LLB]) on serial angiograms. Growth rate was evaluated using a mixed-effect model clustered by individual patient with an interaction term for treated PA and time to evaluate for differential growth rates between treated and contralateral PAs.</p>

<p><strong>RESULTS: </strong>Thirty-five patients underwent TC PA intervention following SCPC, at a median of 70 days (interquartile range: 19-297 days) postoperatively. Significant growth was seen at both DBPA and LLB for raw (0.8 mm/year, 95% CI: 0.6-1.0, &lt; .001 for both) and body surface area (BSA) adjusted measures (8.4mm/m/year, 95% CI: 5.6-11.2, &lt; .001; 7.9 mm/m/year, 95% CI: 5.5-10.2, &lt; .001). The growth rate of the treated vessel was not significantly different from that of the contralateral vessel at the DBPA or LLB positions for raw ( = .71, .70) or BSA-adjusted measurements ( = .86, .64).</p>

<p><strong>CONCLUSION: </strong>Transcatheter PA intervention was associated with normal distal PA growth rate relative to the untreated side.</p>

DOI

10.1177/21501351211033238

Alternate Title

World J Pediatr Congenit Heart Surg

PMID

34597205

Title

Cumulative Effect of Preoperative Risk Factors on Mortality after Pediatric Heart Transplantation.

Year of Publication

2018

Date Published

2018 Apr 20

ISSN Number

1552-6259

Abstract

<p><strong>BACKGROUND: </strong>Risk assessment in heart transplantation is critical for candidate selection, but current models inadequately assess individual risk of postoperative mortality. We sought to identify risk factors and develop a scoring system to predict mortality following heart transplantation in children.</p>

<p><strong>METHODS: </strong>The records of patients undergoing heart transplantation at our institution from 2010 - 2016 were reviewed. Clinical characteristics were recorded and compared between survivors and non-survivors. Using Cox proportional hazard modeling, a risk factor score was developed using factors associated with postoperative mortality.</p>

<p><strong>RESULTS: </strong>Seventy-four patients underwent heart transplantation at a mean age of 8.8 ± 6.6 years. Congenital heart disease was the most common indication, comprising 48.6% of the cohort. Overall mortality was 18.9%, with 10/14 dying ≤30 days of operation or during initial postoperative admission (early mortality). The following preoperative factors were associated with overall mortality: single ventricle congenital heart disease (HR 3.2, p = 0.042), biVAD (HR 4.8, p = 0.043), history of ≥4 sternotomies (HR 3.9, p = 0.023), panel reactive antibody &gt; 10% (HR 4.4, p = 0.013), any previous surgery at an outside institution (HR 3.2, p = 0.038), and pulmonary vein disease (HR 4.7, p = 0.045). Each risk factor was assigned a point value, based on similar magnitude of the hazard ratios. A score of ≥4 predicted mortality with 57% sensitivity and 90% specificity.</p>

<p><strong>CONCLUSIONS: </strong>In this single-center pediatric cohort, post-heart transplantation mortality could be predicted using patient-specific risk factors. The cumulative effect of these risk factors predicted mortality with high specificity.</p>

DOI

10.1016/j.athoracsur.2018.03.044

Alternate Title

Ann. Thorac. Surg.

PMID

29684375

Title

Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation.

Year of Publication

2017

Date Published

2017 Sep

ISSN Number

1941-7632

Abstract

<p><strong>BACKGROUND: </strong>There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation.</p>

<p><strong>METHODS AND RESULTS: </strong>All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P=0.006) and lateral tunnel Fontan (HR, 4.3; P=0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P=0.007), Norwood operation (HR, 2.3; P=0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P=0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P&lt;0.001) and hypoplastic left heart syndrome (HR, 2.0; P=0.01).</p>

<p><strong>CONCLUSIONS: </strong>In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.</p>

DOI

10.1161/CIRCINTERVENTIONS.116.004924

Alternate Title

Circ Cardiovasc Interv

PMID

28851719

Title

Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.

Year of Publication

2017

Date Published

2017 Mar 16

ISSN Number

1879-1913

Abstract

<p>Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3&nbsp;years (interquartile range 1.4 to 11.2), and 30% had follow-up &gt;10&nbsp;years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20&nbsp;years (95% confidence interval [CI] 38 to 55). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (hazard ratio [HR] 1.7, 95% CI 1.2 to 2.4, p&nbsp;= 0.001), pleural drainage &gt;14&nbsp;days (HR 1.5, 95% CI 1.01 to 2.2, p&nbsp;= 0.04), and longer cross-clamp time (HR 1.2 per 10&nbsp;minutes, 95% CI 1.06 to 1.3, p&nbsp;= 0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17 to 76, p &lt;0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20&nbsp;years (95% CI 96 to 99). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging.</p>

DOI

10.1016/j.amjcard.2017.03.004

Alternate Title

Am. J. Cardiol.

PMID

28385177

Title

Long-term survival after the Fontan operation: Twenty years of experience at a single center.

Year of Publication

2017

Date Published

2017 Mar 06

ISSN Number

1097-685X

Abstract

<p><strong>OBJECTIVE: </strong>Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort.</p>

<p><strong>METHODS: </strong>All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013.</p>

<p><strong>RESULTS: </strong>Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1&nbsp;year (95% confidence interval [95% CI], 92%-95%), 90% at 10&nbsp;years (95% CI, 88%-92%), 85% at 15&nbsp;years (95% CI, 82%-88%), and 74% at 20&nbsp;years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1&nbsp;year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P&nbsp;&lt;&nbsp;.001), intensive care unit stay&nbsp;&gt;1&nbsp;week (HR, 2.4; P&nbsp;&lt;&nbsp;.001), Fontan before 1997 (HR, 3.3; P&nbsp;&lt;&nbsp;.001), preoperative atrioventricular valve regurgitation (HR, 2.0; P&nbsp;&lt;&nbsp;.001), and longer crossclamp time (HR, 1.3 per 10&nbsp;minutes; P&nbsp;&lt;&nbsp;.001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P&nbsp;≤&nbsp;.001), and post-Fontan ICU stay&nbsp;&gt;1&nbsp;week (HR, 2.4; P&nbsp;&lt;&nbsp;.001).</p>

<p><strong>CONCLUSIONS: </strong>Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.</p>

DOI

10.1016/j.jtcvs.2017.01.056

Alternate Title

J. Thorac. Cardiovasc. Surg.

PMID

28341469

Title

Mechanical Circulatory Support as Bridge to Transplantation for the Failing Single Ventricle.

Year of Publication

2016

Date Published

2016 Jul 14

ISSN Number

1552-6259

Abstract

<p><strong>BACKGROUND: </strong>Circulatory failure necessitating cardiac transplantation will ultimately develop in many patients with functional single-ventricle physiology. Interest in the use of mechanical circulatory support (MCS) in this population is growing.</p>

<p><strong>METHODS: </strong>This was a retrospective case series of patients with functional single-ventricle physiology who underwent MCS with a ventricular assist device or a total&nbsp;artificial heart as a bridge to cardiac transplantation between January 2006 and December 2014. Baseline demographics, intraoperative data, postoperative complications, and outcome data were collected from the medical record.</p>

<p><strong>RESULTS: </strong>MCS was used in 5 patients: HeartWare ventricular assist device (HeartWare International, Framingham, MA) in 1 patient, SynCardia total artificial heart (SynCardia Systems, Tucson, AZ) in 1, Thoratec Paracorporeal ventricular assist device (Thoratec Corp, Pleasanton, CA) in 1, and the Berlin Heart EXCOR (Berlin Heart Inc, The Woodlands, TX) in 2. The mean age at MCS was 12 ± 8 years. There were 2 early deaths at 12 and 28 days after MCS: 1 patient died of multiorgan system failure and 1 of neurologic injury. Overall, neurologic complications occurred in 3 patients (60%), and 1 patient (20%) required renal replacement therapy. Three patients (60%) underwent successful cardiac transplantation. The median time on the waiting list was 59 days (interquartile range, 18 to 126 days), and the median duration of MCS was 60 days (interquartile range, 28 to 93 days). At the time of transplant, all 3 patients were ambulatory, without the need for mechanical ventilation, and end-organ dysfunction had resolved. The 3 patients who received transplants were discharged from the hospital and were alive at an average follow-up of 9 ± 14 months.</p>

<p><strong>CONCLUSIONS: </strong>MCS can be successfully used as a bridge to transplantation in patients with a failing single-ventricle circulation. Use of MCS can allow for resolution of end-organ dysfunction and rehabilitation, leading to improved outcomes in this difficult population.</p>

DOI

10.1016/j.athoracsur.2016.05.015

Alternate Title

Ann. Thorac. Surg.

PMID

27424467

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