Outcomes of recurrent laryngeal nerve injury following congenital heart surgery: A contemporary experience.

OBJECTIVE: Injury to the recurrent laryngeal nerve can lead to significant morbidity during congenital cardiac surgery. The objective is to expand on the limited understanding of the severity and recovery of this iatrogenic condition. DESIGN: A six-year retrospective review of all congenital heart operations at a single institution from January 1, 2008 to December 31, 2013 was performed. All patients with documented vocal cord paralysis on laryngoscopic examination comprised the study cohort. Evaluation of time to vocal cord recovery and need for further surgical intervention was the primary focus. RESULTS: The incidence of post-operative vocal cord paralysis was 1.1% (32 out of 3036 patients; 95% confidence interval: 0.7-1.5%). The majority were left-sided injuries (71%). Overall rate of recovery was 61% with a median time of 10 months in those who recovered, and a total follow up of 46 months. Due to feeding complications, 45% of patients required gastrostomy tube after the injury, and these patients were found to have longer duration of post-operative days of intubation (median 10 vs. 5 days, p = 0.03), ICU length of stay (50 vs. 8 days, p = 0.002), and hospital length of stay (92 vs. 41 days, p = 0.01). No pre-operative variables were identified as predictive of recovery or need for gastrostomy placement. CONCLUSION: Recurrent laryngeal nerve injury is a serious complication of congenital heart surgery that impacts post-operative morbidity, in some cases leading to a need for further intervention, in particular, gastrostomy tube placement. A prospective, multi-center study is needed to fully evaluate factors that influence severity and time to recovery

Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit.

Background: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. Methods: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children’s National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. Results: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support. All patients identified manifested persistent severe bleeding, despite appropriate management of anticoagulation and blood product administration based on the established protocols. Detailed hemostatic testing including quantitative von Willebrand factor (vWF) multimer analysis revealed decreased high-molecular-weight multimers (HMWMs) and absent ultra-HMWM, consistent with AvWS in all patients. Eight patients received treatment with vWF concentrate, one patient with desmopressin, and five recovered without specific treatment. Bleeding ceased in all but one patient. Conclusions: Acquired von Willebrand syndrome is an uncommon but important cause of bleeding in pediatric patients with cardiac disease. A high index of clinical suspicion with knowledge of the characteristic clinical scenario in addition to low levels of vWF multimers is required to manage and diagnose AvWS. Although the optimal management of AvWS in this patient population is unclear, vWF concentrates are available and appear to be efficacious for controlling life-threatening bleeding. </jats:sec

Novel, 3D Display of Heart Models in the Postoperative Care Setting Improves CICU Caregiver Confidence.

Background: Postoperative care delivered in the pediatric cardiac intensive care unit (CICU) relies on providers’ understanding of patients’ congenital heart defects (CHDs) and procedure performed. Novel, bedside use of virtual, three-dimensional (3D) heart models creates access to patients’ CHD to improve understanding. This study evaluates the impact of patient-specific virtual 3D heart models on CICU provider attitudes and care delivery. Methods: Virtual 3D heart models were created from standard preoperative cardiac imaging of ten patients with CHD undergoing repair and displayed on a bedside tablet in the CICU. Providers completed a Likert questionnaire evaluating the models’ value in understanding anatomy and improving care delivery. Responses were compared using two-tailed t test and Mann-Whitney U test and were also compared to previously collected CICU provider responses regarding use of printed 3D heart models. Results: Fifty-three clinicians (19 physicians, 34 nurses/trainees) participated; 49 (92%) of 53 and 44 (83%) of 53 reported at least moderate to high satisfaction with the virtual 3D heart’s ability to enhance understanding of anatomy and surgical repair, respectively. Seventy-one percent of participants felt strongly that virtual 3D models improved their ability to manage postoperative problems. The majority of both groups (63% physicians, 53% nurses) felt that virtual 3D heart models improved CICU handoffs. Virtual 3D heart models were as effective as printed models in improving understanding and care delivery, with a noted provider preference for printed 3D heart models. Conclusions: Virtual 3D heart models depicting patient-specific CHDs are perceived to improve understanding and postoperative care delivery in the CICU. </jats:sec

Mitral Annular Disjunction

Mitral annular disjunction (MAD) is a rare and under-recognized entity in the pediatric population. We present 2 cases of MAD in previously healthy pediatric patients and highlight clinical scenarios where MAD should be suspected