September

Impact of trisomy 13 and 18 on airway anomalies and pulmonary complications after cardiac surgery. Swanson SK, Schumacher KR, Ohye RG, Zampi JD. J Thorac Cardiovasc Surg. 2020 Sep 1:S0022-5223(20)32473-9. doi: 10.1016/j.jtcvs.2020.08.082. Online ahead of print. PMID: 32981701   Take Home Points:   Cardiac surgery can be performed safely in the setting of Trisomy 13 and Trisomy 18. T13 and T18 patients undergoing cardiac operations have higher rates of airway complications and require longer ICU stays than matched controls. While operative interventions are likely to be successful, guidance for parents of these patients should include a discussion of postoperative respiratory support.     Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Trisomy 13 (Patau syndrome) and Trisomy 18 (Edwards syndrome) are both associated with high rates of congenital heart diseases, such as VSD, ASD and PDA.  More importantly, however, these chromosomal abnormalities are associated with a severely impaired long-term survival, with a median survival of less than 2 weeks.  As approximately 5-10% of patients survive over 1 year, it is perhaps not surprising that offering surgical treatment for cardiac malformations remains somewhat controversial.  However, as reports of long-term survival increase, more centers offer treatments, such as cardiac surgery, aimed at long-term survival.  Although multiple  have established that these patients can safely undergo surgery, fewer reports have described the postoperative courses of these patients, and the specific comorbidities which complicate postoperative recovery.  Because respiratory complications have been noted to be major complicators of recovery in previous series, this review was performed with a focus on identification and management of respiratory complications.  The authors performed a retrospective case-control study of all Trisomy 13 and Trisomy 18 (T 13/18) patients in a single center over 20 years undergoing cardiac surgery.  A 3:1 diagnosis-matched control group was identified, and individuals were further matched by age at surgery and then date of surgery.   In this study, 14 patients with T 13/18 were identified, and underwent surgical operations at a median age of 93 days. Trisomy 18 was more common than trisomy 13. The indications for operation  included VSD, coarctation, tetralogy of Fallot and complete AVSD.  64% of T 13/18 patients were admitted preoperatively , whereas only 13% of controls were.   Preoperatively, 3 of the T13/18 patients had airway evaluation by specialist physicians.  Patients with T13/18 were more likely to require preoperative respiratory support (71% vs 14%, P=0.001), including intubation and ventilation as well as noninvasive ventilatory assistance.   Operatively, there were no significant differences in cardiopulmonary bypass time, cross clamp time, or circulatory arrest time.   Postoperatively, 13 of the 14 T 13/18 patients experienced at least one complication, And all of these patients experienced at least one respiratory complication. Further, 58% of these patients had at least one non respiratory complication.  In addition to having higher rates of respiratory support preoperatively, T 13/18 patients also had high rates of post-operative respiratory complications, including prolonged mechanical ventilation prolonged noninvasive ventilation, reintubation , tracheostomy, and respiratory infections.   Some other notable complications, including infection (tracheitis and sepsis), need for gastrostomy tube placement, and increased need for vasoactive and inotropic support.   The authors conclude that although survival is good, hospital and ICU length of stay is longer in T13/18 patients than in matched controls.   This retrospective study demonstrates that those increases are at least correlated with an increased prevalence of respiratory and airway anomalies in these patients.   While not recommending against surgical repair for these patients, they suggest that awareness of these airway issue should be included in prenatal counseling, preoperative planning, and managing expectations for these patients.    

Ross Procedures in Children With Previous Aortic Valve Surgery. Buratto E, Wallace FRO, Fricke TA, Brink J, d'Udekem Y, Brizard CP, Konstantinov IE. J Am Coll Cardiol. 2020 Sep 29;76(13):1564-1573. doi: 10.1016/j.jacc.2020.07.058. PMID: 32972534   Take Home Points:   Initial Ross procedure in infancy may be associated with relatively higher mortality, justifying initial attempts to defer operation with aortic valve repairs, even if eventual replacement is necessary. Secondary Ross after initial valve repair is associated with superior long-term survival and freedom from autograft reoperation. Delaying the Ross operation by performing initial valve repair, when possible, may be preferred to initial Ross in infant patients.   Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Aortic valve disease requiring surgical repair in childhood can be a challenge, with limited options available for replacement.  Although the Ross procedure offers the best long-term freedom from reoperation in neonates and infants, the long term freedom from reintervention in some series remain disappointing, with high mortality rates in infants (16 – 22%).  Because of this, some centers advocate an approach that of initial aortic valve repair, whenever possible, followed by a secondary Ross procedure when necessary.  The goal of this retrospective analysis as to determine if there was a difference in outcome between the Ross operation when performed as a primary operation and when performed as a reoperation.   The authors retrospectively analyzed 541 aortic valve operations in their institution, 344 of which were aortic valve repairs, and 140 of which were Ross procedures.  68 (49%) of the Ross procedures took place after previous valve repairs, and 72 (51%) took place as the primary operation.   Patients undergoing a primary Ross were older (8.6 years vs 7 years), and had higher weights (28.9 kg vs. 19.7 kg) although these differences weren’t significant.  The early mortality was 5.6% (n = 4 of 72) in those undergoing primary Ross procedure, compared with 4.4% (n = 3 of 68) in those undergoing secondary Ross procedure.  This difference was not statistically significant (p = 1.0).  Freedom from reoperation at 10 and 15 years was 68.3% and 48.5% in those undergoing primary Ross procedure.  In those who underwent secondary Ross procedure, freedom from reoperation at 10 and 15 years was 62.2% and 47.0% respectively.  Again, this difference was not statistically significant.   In the  propensity-matched groups, however, survival at 10 and 15 years was 90.0% and 82.6% in the primary Ross procedure group, compared with 96.8% at both 10 and 15 years in the secondary Ross procedure group. In the propensity-matched analysis, this difference was statistically significant (p = 0.04).   The mechanisms for the improved survival and freedom from reoperation are discussed briefly, including possible stabilization of the autograft from postoperative scar and adhesion formation, but this was just postulated rather than investigated.  None were investigated in this retrospective review.   The authors concluded that secondary Ross procedure performed after initial aortic valve surgery achieves superior long-term survival and freedom from autograft reoperation compared with primary Ross procedure. A strategy of initial aortic valve surgery followed by delayed Ross procedure may provide better long-term survival and freedom from autograft reoperation in aortic valve disease in children.