Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.

Improving Results of Surgery for Ebstein Anomaly: Where Are We After 235 Cone Repairs?

BACKGROUND: Ebstein anomaly has heterogeneous anatomy and numerous operative techniques are described. Cone repair provides a near anatomic tricuspid valve repair. The purpose of this study was to examine our experience with cone repair. METHODS: Cone repair was performed in 235 consecutive patients with Ebstein anomaly, 134 children (57%) and 101 adults (43%), from June 2007 to October 2015. Median age was 15.6 years (range, 6 months to 73 years). Cone repair was the first operation in 192 patients (82%), the second in 41 (17%), and the third in 2 (1%). Previous tricuspid valve repair had been performed in 27 (12%). Echocardiograms were obtained preoperatively and at hospital dismissal for all patients and for a subgroup of patients at least 6 months after cone repair (n = 81). RESULTS: Leaflet augmentation was done in 67 patients (28%), Sebening stitch in 57 (24.2%), neochordae in 49 (21%), and annuloplasty band in 158 (67%). Bidirectional cavopulmonary shunt was performed in 46 patients (20%). There was 1 early death (0.4%). Early reoperation was required in 14 patients (5.9%); re-repair was possible in 7 (50%). The majority of early reoperations (11 of 14; 79%) occurred in the first third of the series. Mean follow-up was 3.5 ± 2.5 years. There was sustained reduction in tricuspid regurgitation (p < 0.0001), a progressive decline in right ventricle size (p < 0.0001), and late increase in right ventricle fractional area change after initial decline (p < 0.0001). Freedom from late reoperation was 97.9% at 6 years. CONCLUSIONS: Cone repair is safe, and the learning curve is significant. Sustained reduction in tricuspid regurgitation and favorable changes in the right ventricle at follow-up suggest that cone repair has an advantageous impact on right ventricular remodeling.

Novel Applications of Modified Ultrafiltration and Autologous Priming Techniques to Reduce Blood Product Exposure on ECMO.

Patients needing the assistance of extracorporeal membrane oxygenation (ECMO) are at risk of hemodilution and, in some instances, may require exposure to large amounts of allogeneic blood products. Patient outcomes can be improved by taking steps to reduce transfusions and hemodilution. Currently, modified ultrafiltration (MUF) is used across the world to reduce hemodilution after cardiopulmonary bypass (CPB). Another common technique during bypass initiation is autologous priming. By applying modified versions of these techniques, ECMO patients may potentially benefit. Usually, patients requiring immediate transition from CPB to ECMO are not stable enough to tolerate MUF. Through alterations of the CPB and ECMO circuit tubing, MUF can be performed once on ECMO. Another technique to potentially lower the transfusion requirements for ECMO patients is a complete circuit blood transfer during an ECMO circuit exchange. While selective component changes are preferred if possible, occasionally a complete circuit change must be done. To minimize hemodilution or prevent priming with blood products, the original ECMO circuit's blood can be transferred to the new ECMO circuit before connecting to the patient. Both of these techniques, in our opinion, helped to reduce the number of transfusions that our ECMO patients have seen during these critical time periods.

Using Daily Plasma-Free Hemoglobin Levels for Diagnosis of Critical Pump Thrombus in Patients Undergoing ECMO or VAD Support.

Patients supported with extracorporeal membrane oxygenation (ECMO) or short-term centrifugal ventricular assist devices (VADs) are at risk for potential elevation of plasma-free hemoglobin (pfHb) during treatment. The use of pfHb testing allows detection of subclinical events with avoidance of propagating injury. Among 146 patients undergoing ECMO and VAD from 2009 to 2014, five patients experienced rapid increases in pfHb levels over 100 mg/dL. These patients were supported with CardioHelp, Centrimag, or Pedimag centrifugal pumps. Revolutions per minute of the pump head and flow in the circuit in three of the patients did not change, to maintain patient flow during the period that pfHb level spiked. Two patients had unusual vibrations originating from the pump head during the pfHb spike. Four patients had pump head replacement. Following intervention, trending pfHb levels demonstrated a rapid decline over the next 12 hours, returning to baseline within 48 hours. Two of the three patients who survived to discharge also experienced acute kidney injury, which was attributed to pfHb elevations. The kidney injury resolved over time. The architecture of centrifugal pumps may have indirectly contributed to red blood cell damage due to thrombus, originally from the venous line or venous cannula, being snared in the pump fins or pump head.

Extracorporeal Membrane Oxygenation Support in Postcardiotomy Elderly Patients: The Mayo Clinic Experience.

BACKGROUND: We conducted a retrospective study to assess whether providing extracorporeal membrane oxygenation (ECMO) support to elderly patients (aged 70 years or more) who failed separation from cardiopulmonary bypass after cardiac surgery was a viable option. METHODS: From 2003 to 2013, 45 patients aged 70 years or more underwent 47 runs of ECMO postoperatively. RESULTS: There were 31 men (68.9%). The mean age was 76.8 years. Five patients were in cardiogenic shock preoperatively. Forty-four patients required venoarterial ECMO support for cardiogenic shock. Mean duration of support was 103.8 ± 74.3 hours. Twenty-one patients (46.6%) died while on ECMO support. Twenty-four patients (53.3%) were weaned off ECMO initially, and 11 patients were discharged from hospital. Inhospital mortality was 75.6%. Postoperative complications included acute kidney injury in 30 patients (44.4%), pneumonia in 12 (26.7%), and sepsis in 11 (24.4%). There were 30 deaths (88.2%) attributable to cardiac causes. Preoperative atrial fibrillation, chronic kidney injury, lactic acidosis on ECMO support, and persistent coagulopathy were associated with higher mortality. CONCLUSIONS: Postcardiotomy ECMO support in elderly patients is associated with high postoperative morbidity and mortality. Nevertheless, it often provides the last line of therapy for these critically ill patients and may provide positive outcomes in selected subgroups.

Strategies for tricuspid re-repair in Ebstein malformation using the cone technique.

BACKGROUND: Successful tricuspid repair for Ebstein malformation (EM) is challenging. Cone repair has recently emerged as the most anatomic repair technique. There are no data on tricuspid re-repair in EM. We reviewed our experience with tricuspid valve (TV) re-repair using a cone technique. METHODS: Between June 2007 and October 2012, 134 patients underwent cone repair. Prior TV repair was performed in 20 patients (10 female, 50%). Median age was 15 years (range, 4 to 68 years). Four patients (20%) had prior bidirectional cavopulmonary anastomosis (BDCPA). Preoperative heart failure was present in 8 patients (40%). Recurrent tricuspid regurgitation (TR) was due to incomplete leaflet coaptation with tethered anterior leaflet in all patients; and 10 patients (50%) had diminutive septal leaflet. Prior repair techniques consisted of annuloplasty maneuvers in all patients with no or incomplete surgical delamination in all patients. RESULTS: The TV re-repair using cone technique was possible in all patients. Plication of atrialized right ventricle was done in 6 patients (30%). Modifications included annuloplasty band in 16 patients (80%) and TV leaflet augmentation in 8 (40%). The BDCPA was performed in 3 patients (15%) and modified Cox-maze procedure was done in 10 (50%). There were no mortalities or reoperations. Mean follow-up was 7.7 ± 10.7 months; during follow-up, 18 patients had no or mild TR and 2 had moderate TR. CONCLUSIONS: TV re-repair in EM using the cone technique is feasible, particularly when there has been no surgical leaflet delamination at initial operation. Leaflet augmentation facilitates and annuloplasty band stabilizes a competent TV re-repair. Longer follow-up is required to assess durability.