Volume : 6, Issue : 11, November - 2017

Early and Long-term Outcomes after Aortic Surgery in Patients with Marfan Syndrome

Ali El Sayed Ahmad, Nestoras Papadopoulos, Anton Moritz, Andreas Zierer

Abstract :

<p>&nbsp;<span style="font-size: 12pt; line-height: 200%; font-family: &quot;Times New Roman&quot;, serif;">Background:</span><span style="font-size: 12pt; line-height: 200%; font-family: &quot;Times New Roman&quot;, serif;">The aim of this study is to determine the early- and long-term results after aortic surgery in Marfan syndrome patients.</span></p> <p class="MsoNormal" style="line-height:200%"><span style="font-size:12.0pt;&#10;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:EN-US;&#10;mso-bidi-font-weight:bold;mso-bidi-font-style:italic">Methods:</span><span style="font-size:12.0pt;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;&#10;mso-ansi-language:EN-US">Between January 2000 and December</span><span lang="EN-GB" style="font-size:12.0pt;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;&#10;mso-ansi-language:EN-GB"> 2015</span><span style="font-size:12.0pt;line-height:&#10;200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:EN-US">, 36 patients affected by Marfan syndrome underwent open surgical aortic and root repair.</span><span lang="EN-GB" style="font-size:12.0pt;line-height:200%;&#10;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:EN-GB;mso-bidi-font-weight:&#10;bold"> Isolated</span><span style="font-size:12.0pt;line-height:200%;&#10;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:EN-US"> ascending replacement, hemiarch replacement and total arch replacement was performed in 5 patients (14%), 14 patients (39%) and 17 patients (47%), respectively. Elephant trunk technique and frozen elephant trunk technique was performed in 10 patients (28%) and 3 patients (8%), respectively. The surgery was performed urgently in 16 patients (44%) and electively in 20 patients (56%). Hundred twenty-seven imaging studies were analyzed for segmental aortic diameter at our institution. Clinical data were prospectively entered into our institutional database. Mean late follow up for late survival was 5 &plusmn; 3 years and was 98 % complete.<o:p></o:p></span></p> <p class="MsoNormal" style="line-height:200%"><span style="font-size:12.0pt;&#10;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:EN-US;&#10;mso-bidi-font-weight:bold;mso-bidi-font-style:italic">Results:</span><span lang="EN-GB" style="font-size:12.0pt;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;&#10;mso-ansi-language:EN-GB">Thirty-day mortality was 6% (n=2). One patient died in the hospital. Intensive care unit stay was 3 &plusmn; 4 days. One patient (3%) experienced permanent neurologic deficits and 4 patients (11%) transient neurologic deficits. 5 Patients (14%) underwent redo surgery. Late survival and freedom from re-operation/re-intervention were 89 &plusmn; 2 % and 81 &plusmn; 3% at 6 years.<o:p></o:p></span></p> <p class="MsoNormal" style="line-height:200%"><span lang="EN-GB" style="font-size:&#10;12.0pt;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:&#10;EN-US">&nbsp;</span><span style="font-size:&#10;12.0pt;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:&#10;EN-US;mso-bidi-font-weight:bold;mso-bidi-font-style:italic">Conclusions:</span><span lang="EN-GB" style="font-size:12.0pt;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;&#10;mso-ansi-language:EN-GB">Marfan syndrome patients </span><span style="font-size:12.0pt;line-height:200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;&#10;mso-ansi-language:EN-US">seem to benefit the most from total arch replacement with elephant trunk</span><span lang="EN-GB" style="font-size:12.0pt;line-height:&#10;200%;font-family:&quot;Times New Roman&quot;,&quot;serif&quot;;mso-ansi-language:EN-GB">. We recommend rigorously adjust the systolic blood pressure less than 120 mmHg and a postoperatively treatment with &szlig;-blockers and ACE inhibitors. The time of re-operation and the increase of the aortic size still unpredictably therefore a yearly follow-up lifelong is recommended.&nbsp;<o:p></o:p></span></p>