Takeshi Shinkawa

Impact of 22q11.2 deletion on the postoperative course of children after cardiac surgery.

The primary objective of this study was to describe the impact of 22q11.2 deletion (del22q11) on the clinical characteristics, postoperative course, and short-term outcomes of children undergoing surgery for congenital heart disease. The charts of all children ages 1xa0day–18xa0years who received cardiac surgery for interrupted aortic arch (IAA), tetralogy of Fallot (TOF), or truncus arteriosus (TA) repair from 1 January 2001 to 31 December 2011 were retrospectively reviewed. The patients were divided into two groups: the 22q11 group including children with del22q11 undergoing surgery for TOF, IAA, or TA and the non-22q11 or control group including children with no chromosomal or genetic abnormality undergoing surgery for TOF, IAA, or TA. Demographic information, cardiac diagnoses, noncardiac abnormalities, preoperative factors, intraoperative details, surgical procedures performed, postoperative complications, and in-hospital deaths were collected. The outcome data collected included days of inotrope use, need for dialysis, length of mechanical ventilation, intensive care unit (ICU) length of stay (LOS), hospital LOS, and mortality. The study enrolled 173 patients: 65 patients in the 22q11 group and 108 patients in the control group. Of the 65 patients in the 22q11 group, 36 (55xa0%) underwent repair for TOF, 13 (20xa0%) for IAA, and 16 (25xa0%) for TA. The two groups did not differ in terms of age or weight. The preexisting conditions were similar in the two groups. Unplanned noncardiac operations were more common in the children with del22q11, but delayed chest closure was similar in the two groups. The incidence of postoperative noncardiac complications such as reintubation, vocal cord paralysis, and diaphragmatic paralysis was similar in the two groups. However, increasing numbers of patients in del22q11 group needed dialysis in one form or the other during the immediate postoperative stay. The incidence of fungal infection and wound infection was higher in the del22q11 group than in the control group. Duration of mechanical ventilation, ICU LOS, and hospital LOS were similar in the two groups, except in certain subgroups. Mortality did not differ significantly between the two groups. In conclusion, children with del22q11 have a higher risk of postoperative complications after cardiac surgery, with no difference in length of mechanical ventilation, ICU LOS, hospital LOS, or mortality. However, short-term outcomes may differ in certain subgroups.

A Single-Center Experience of Extubation Failure in Infants Undergoing the Norwood Operation

BACKGROUNDnIdentify incidence, etiology, and predictors of extubation failure in neonates and infants who underwent Norwood operation with either a modified Blalock-Taussig shunt (mBTS) or a Sano shunt at a single tertiary care, academic childrens hospital.nnnMETHODSnExtubation failure for our study was defined as reintubation within 96 hours after extubation. We collected demographics, preoperative, intraoperative, postoperative, and peri-extubation data in a retrospective, observational format in patients who underwent Norwood operation with either a modified Blalock-Taussig shunt (mBTS) or a Sano shunt between January 2005 and March 2011. Clinical outcomes evaluated included the success or failure of extubation, cardiac intensive care unit (CICU) length of stay (LOS), hospital LOS, and mortality. Descriptive, univariate, and multivariate statistics were utilized to compare groups with extubation failure and extubation success.nnnRESULTSnOf 64 eligible patients during the study period, extubation failure occurred in 22% (14 of 64) of the patients. Eight patients failed extubation in the first 24 hours with an extubation failure rate of 12%. The median age of patients was 18 days (range 13.75 days to 22 days) and median weight of patients was 3.37 kg (range 3.11 kg to 3.86 kg). Twelve patients received a mBTS while 52 patients received a Sano shunt. All extubation failures occurred in patients receiving a Sano shunt. The most common risk factors for failed extubation were lung disease in 29% (4 of 14), cardiac dysfunction in 21% (3 of 14), diaphragmatic paralysis in 14% (2 of 14), airway edema in 14% (2 of 14), vocal cord paralysis in 14% (2 of 14), and laryngotracheomalacia in 7% (1 of 14). Patients with extubation failure had longer CICU LOS and hospital LOS. Overall mortality at the time of hospital discharge was 8%. Independent predictors of extubation failure included use of nitric oxide after surgery, increased days of mechanical ventilation prior to extubation, elevated inotrope score leaving the operating room, and presence of atelectasis prior to extubation.nnnCONCLUSIONSnExtubation failure in children after the Norwood operation is a slow and evolving process occurring as late as 96 hours after extubation and is not associated with an increase in in-hospital mortality. Causes of failed extubation are diverse. Successful weaning from positive pressure ventilation depends on adequate cardiovascular function, the presence of satisfactory ventilatory reserves, and favorable pulmonary mechanics.

Prophylactic amiodarone reduces junctional ectopic tachycardia after tetralogy of Fallot repair

OBJECTIVEnJunctional ectopic tachycardia is common after pediatric heart surgery. After tetralogy of Fallot repair, the incidence of junctional ectopic tachycardia may be as high as 15% to 20%. We introduced prophylactic amiodarone for tetralogy repair. This study was conducted to evaluate the effectiveness of the prophylactic amiodarone.nnnMETHODSnA continuous infusion of amiodarone was started in the operating room at the time of rewarming during cardiopulmonary bypass at a rate of 2 mg/kg/d and continued for 48 hours. Between November 2005 and November 2009, 63 consecutive patients underwent primary repair of tetralogy, of whom 20 had prophylactic amiodarone (amiodarone group) and 43 did not (control group). Variables studied included demographic and bypass data, surgical procedure details (transannular or nontransannular patch), preoperative and postoperative echocardiography findings, and postoperative inotropic support. Univariate and stepwise multivariate analyses were conducted to determine factors associated with the occurrence of junctional ectopic tachycardia.nnnRESULTSnThe incidence of junctional ectopic tachycardia was 37% in the control group and 10% in the amiodarone group. The groups were similar in age, weight, bypass time, rate of transannular patch usage, and preoperative and postoperative gradient through the right ventricular outflow tract. Prophylactic amiodarone was significantly negatively associated with junctional ectopic tachycardia by both univariate (P = .039) and multivariate (P = .027) analyses. There were no adverse events attributable to prophylactic amiodarone use.nnnCONCLUSIONSnProphylactic amiodarone is well tolerated and significantly associated with a decreased incidence of junctional ectopic tachycardia after tetralogy repair.

Valved Polytetrafluoroethylene Conduits for Right Ventricular Outflow Tract Reconstruction

BACKGROUNDnThe purposes of this study were to review our early outcomes using valved expanded polytetrafluoroethylene (ePTFE) conduits, with or without bulging sinus structure, for right ventricular outflow tract reconstruction and to examine the mechanical properties of the ePTFE material after bulging sinuses were created.nnnMETHODSnA retrospective review was performed of all patients who received the valved ePTFE conduit between 2008 and 2014 at a single institution. The surface morphologies and the mechanical strengths of the ePTFE conduit with bulging sinuses examined by scanning electron microscopy and unidirectional pull test were compared with those of the original ePTFE material.nnnRESULTSnThere were 120 operations with the valved ePTFE conduit (60 with bulging sinuses). The patients median age and weight were 6.9 years and 23.7 kg. The conduits were a median size of 22 mm. At 5 years, freedom from conduit reoperation was 92.7% (95% confidence interval, 82.7% to 97.0%), and freedom from severe conduit insufficiency or more than a 50 mm Hg gradient was 74.8% (95% confidence interval, 60.8% to 84.4%). No significant differences in the surface morphologies were observed by the scanning electron microscopy or in the maximum tolerated loads obtained by the pull test between the original ePTFE material and the ePTFE with bulging sinuses (121 and 122 N in longitudinal direction and 115 and 121 N in circumferential direction; p = 0.88 and p = 0.68).nnnCONCLUSIONSnThe valved ePTFE conduits demonstrated excellent early clinical outcomes. The mechanical property examinations showed no obvious difference after bulging sinuses were created on the ePTFE material.

Primary Translocation of Aberrant Left Subclavian Artery for Children With Symptomatic Vascular Ring

BACKGROUNDnThe standard operation for vascular ring with right aortic arch and aberrant left subclavian artery is ligamentum arteriosum division. A new surgical approach with primary translocation of the aberrant left subclavian artery to the left carotid artery, removal of the Kommerell diverticulum, and division of the ligamentum through left thoracotomy was recently applied. This study assessed the early outcomes of this approach.nnnMETHODSnThis is a retrospective review of all patients having an operation for right aortic arch with aberrant left subclavian artery and the Kommerell diverticulum between January 2001 and April 2011 at a single institution.nnnRESULTSnEight patients had a division of the ligamentum and 10 had a primary translocation of an aberrant left subclavian artery with diverticulum removal and ligamentum division. The median operative age and weight for each surgical technique group were, respectively, 2.2 and 1.8 years (p = 0.56) and 10.7 and 12.6 kg (p = 0.30). All patients were symptomatic preoperatively. No deaths occurred. One patient from each group had chylous effusion that was medically treated. The median hospital stay for each group was 4 and 5 days (p = 0.45). During the median follow-up of 47 months, 3 patients in the division of ligamentum group required albuterol aerosol or oral antiinflammatory medication for respiratory symptoms. All patients in the primary translocation group remained asymptomatic and required no medication (p = 0.069).nnnCONCLUSIONSnPrimary translocation of aberrant left subclavian artery with removal of the diverticulum and division of the ligamentum had excellent early outcomes and can potentially eliminate residual symptoms and late complications.

Outcome of Right Ventricle to Pulmonary Artery Conduit for Biventricular Repair

BACKGROUNDnThe objective of this study was to assess the outcomes of the right ventricle to pulmonary artery conduit for biventricular repair.nnnMETHODSnThis is a retrospective review of all right ventricle to pulmonary artery conduit operations for biventricular repair of congenital heart disease between 1982 and 2013 at a single institution. Results were compared among the conduit size and materials.nnnRESULTSnA total of 476 physiologic right ventricle to pulmonary artery conduit operations were identified, with 195 pulmonary homografts, 105 handmade valved expanded polytetrafluoroethylene conduits, 103 Medtronic Hancock (Minneapolis, MN) bioprosthetic valved conduits, 38 non-valved expanded polytetrafluoroethylene tubes, and 35 others. The actuarial survival was 92.4% and the freedom from conduit reoperation was 33.0% at 20 years. The freedom from conduit reoperation was significantly different among conduit materials (76.8%, 92.1%, 81.9%, 80.6%, and 63.8% for pulmonary homograft, valved expanded polytetrafluoroethylene conduit, Hancock conduit, non-valved expanded polytetrafluoroethylene tube, and others at 5 years, pxa0= 0.0001). The Cox proportional hazards model showed that age (p < 0.001 and pxa0= 0.04), preoperative diagnosis (p < 0.001 and p < 0.001), conduit size (p < 0.001 and p < 0.001), and conduit material (the valved expanded polytetrafluoroethylene conduit versus combined other materials; pxa0= 0.01 and pxa0= 0.02, respectively) were significant factors for the freedom from conduit reoperation both when treating conduit size as a categoric predictor and as a quantitative predictor.nnnCONCLUSIONSnThe handmade valved expanded polytetrafluoroethylene conduit showed excellent early outcome as a right ventricle to pulmonary artery conduit forxa0biventricular repair. A longer follow-up and a randomized study will be necessary to explore the advantages of the valved expanded polytetrafluoroethylene conduit.

The Midterm Outcomes of Bioprosthetic Pulmonary Valve Replacement in Children

The purpose of this study was to assess the outcomes of bioprosthetic pulmonary valve replacement (PVR) in children. This is a retrospective review of all bioprosthetic PVR in children (≤ 20-year old) between 1992 and 2013 at a single institution. Most outcomes studied included pulmonary valve reintervention and bioprosthetic valve function. A total of 136 bioprosthetic PVRs were identified for 123 patients. The median age and body weight at the time of operation were 13.2 years and 48.4 kg. There were 1 early death and 3 late deaths during the median follow-up of 7.2 years (0-22.0 years). The actuarial transplant-free survival was 97.6% at 10 years. There were 43 bioprosthesis reinterventions with 29 reoperations and 14 catheter-based interventions. The freedom from bioprosthesis reintervention was 89.6% and 55.0% at 5 and 10 years, respectively. Echocardiographic bioprosthesis dysfunction (≥ moderate bioprosthesis insufficiency, ≥ 50 mmHg peak gradient through bioprosthesis, or bioprosthesis endocarditis with vegetation) was found in 57 bioprostheses. The freedom from bioprosthesis dysfunction was 74.0% and 32.8% at 5 and 10 years, respectively. Results from the Cox proportional hazards models showed that age had significant association with freedom from bioprosthesis reintervention and freedom from bioprosthesis dysfunction (P < 0.001 and P = 0.03), whereas bioprosthesis type had nonsignificant association with freedom from bioprosthesis dysfunction (P = 0.068). Bioprosthetic PVR in children had excellent early outcomes but rapidly deteriorating midterm outcomes. Careful and close follow-up are necessary for children with bioprosthesis in the pulmonary position.

Single institutional experience of interrupted aortic arch repair over 28 years.

A single institutional outcome of the biventricular repair for congenital heart disease with interrupted aortic arch between 1982 and 2010 were retrospectively reviewed. There were 48 consecutive patients with a mean follow-up of 10.0 ± 7.9 years. The staged repair was applied in 27 patients, and primary complete repair was applied in 21. The actuarial survival was 79.0% at 10 years. There was a significant difference in survival between the patients operated before 2000 and after 2001 (65.2 vs. 100% at 10 years, P = 0.005), but not in survival between the staged repair and the primary complete repair (77.4 vs. 81.0% at 10 years, P = 0.793). There was no significant difference in freedom from unplanned reoperation between the staged repair and the primary complete repair (47.9 vs. 70.6% at 5 years, P = 0.249). No patients with primary complete repair had reoperation for left ventricular outflow tract obstruction, whereas five patients with staged repair did. The patients with interposition graft placement between ascending and descending aorta had significantly low freedom from reoperation for the aortic arch compared with other techniques (7.2 vs. 90.0% at 10 years, P = 0.001). In conclusion, surgical outcomes for interrupted aortic arch have been significantly improved in the last decade and the staged repair remains an effective option in selected patients.

New Transplant Technique With Hemiazygos Continuation and Interrupted Inferior Vena Cava

In the presence of left superior vena cava, cardiac transplantation is performed at the risk of left superior vena cava flow obstruction. In patients with hemiazygos continuation with interrupted inferior vena cava, the patency of left superior vena cava is vital. We introduced a new surgical technique to use a Fontan connection including the native central pulmonary artery as the systemic venous return. The pulmonary artery anastomosis was performed distal to the superior vena cava anastomosis sites. We used this treatment approach successfully to perform cardiac transplantation in two patients who developed severe heart failure after Fontan completion.

Incidence of Immediate Extubation After Pediatric Cardiac Surgery and Predictors for Reintubation

Objectives: The objectives were to assess the incidence of immediate tracheal extubation in the operating room after pediatric cardiac surgery and to investigate predictors for subsequent reintubation. Methods: This is a single institutional retrospective study including all patients who had a cardiac operation with cardiopulmonary bypass from 2011 to 2016. Patients who required preoperative ventilator support, postoperative open chest, or mechanical support were excluded. Predictors for reintubation after immediate extubation were analyzed only for patients with stage II palliation for single ventricle physiology. Results: Nine hundred nine qualifying operations were identified. Immediate extubation was performed in 590 (64.9%) operations. A multivariable logistic regression model showed that the identities of anesthesiologist (P = .0003), year of the operation performed (P < .001), cardiopulmonary bypass time (P < .001), and type of operations (P < .001) were significantly associated with immediate extubation. Reintubation was significantly less frequent in patients with immediate extubation compared to those without (6.1% vs 15.0%; P < .001). A subgroup analysis for stage II palliation showed that reintubation after immediate extubation was significant for younger age (0.42 vs 0.54 years, P = .044), lower Po2/Fio2 and Po2 at the last blood gas analysis (66 vs 98 mm Hg, P = .032 and 39 vs 47 mm Hg, P = .008), and higher inotropic score (2 vs 0, P = .034). A multivariable logistic regression model showed that only inotropic score was significantly associated with reintubation (P = .018). Conclusions: Immediate extubation in the operating room after pediatric cardiac surgery can be performed in most patients. Inotropic score is a predictor for reintubation in stage II palliation.