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

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