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

2017

2017 Sep

1941-7632

<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>

10.1161/CIRCINTERVENTIONS.116.004924

Circ Cardiovasc Interv

28851719

Systemic Atrioventricular Valve Excision and Ventricular Assist Devices in Pediatric Patients.

2018

170-174

2018 Jan

1552-6259

<p><strong>BACKGROUND: </strong>Continuous-flow ventricular assist devices (CF VADs) designed for adults are increasingly used in pediatric patients. However, there is greater risk of device inflow obstruction as a result of size and anatomy.</p>

<p><strong>METHODS: </strong>We reviewed all cases of systemic atrioventricular valve (AVV) excision with HeartWare HVAD (HeartWare, Framingham, MA) implantation in the systemic ventricle performed at our institution from November 2015 to May&nbsp;2016.</p>

<p><strong>RESULTS: </strong>AVV excision with CF VAD implantation was undertaken in 3 patients. Patient 1 was palliated in infancy, resulting in biventricular physiology with a systemic right ventricle, and presented at age 15 years with worsening ventricular dysfunction. After CF VAD implantation in the systemic ventricle and discharge to home, tricuspid valve obstruction to VAD inflow developed, and the patient and underwent tricuspid valve excision on postoperative day 52. Patients 2 and 3 were aged younger than 4 years, with a body surface area of 0.62 m(2) and 0.58 m(2), respectively, and had undergone Fontan palliation, with subsequent systemic ventricular dysfunction and AVV regurgitation. In both Fontan patients, the CF VAD was implanted in the right atrium with simultaneous excision of the AVV. None have had evidence of elevated atrial pressures or recalcitrant pulmonary edema. At a mean follow-up of 359 days (range, 304 to 422 days), there have been no concerns for inflow obstruction or low flow.</p>

<p><strong>CONCLUSIONS: </strong>CF VAD implantation with AVV excision can successfully support complex pediatric patients in a wide range of size and anatomy (small chambers, systemic right ventricles). This technique may allow for CF VAD implantation in patients previously deemed too small for such support.</p>

10.1016/j.athoracsur.2017.05.038

28821330

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

2017

2017 Mar 16

1879-1913

<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>

10.1016/j.amjcard.2017.03.004

Am. J. Cardiol.

28385177

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

2017

2017 Mar 06

1097-685X

<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>

10.1016/j.jtcvs.2017.01.056

J. Thorac. Cardiovasc. Surg.

28341469

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

2016

2016 Jul 14

1552-6259

<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>

10.1016/j.athoracsur.2016.05.015

Ann. Thorac. Surg.

27424467