Creation of a Neo-Mitral Valve With Right Atrial Appendage Tissue in an Infant.

Mitral valve replacement in neonates and infants is a challenging operation with few good options. Neo-mitral valve reconstruction with right atrial appendage (RAA) may overcome some of the limitations of existing options.

Management Options for Congenitally Corrected Transposition: Which, When, and for Whom?

Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.

Senning With Aortic Translocation and Anatomic Repair for Congenitally Corrected Transposition.

BACKGROUND: Anatomic repair for congenitally corrected transposition of the great arteries with ventricular septal defect (VSD) and pulmonic stenosis has been accomplished with atrial switch and Rastelli. Aortic translocation offers a direct left ventricular outflow without an extraanatomic right ventricular-to-pulmonary conduit, which may lead to decreased reoperations. We reviewed our entire experience performing Senning with aortic translocation (SAT). METHODS: From 2007 to 2017, 8 patients (mean age, 14.1 months; size, 8.86 kg) underwent SAT. Associated anomalies included situs inversus (n = 2), dextrocardia (n = 6), multiple muscular VSDs (n = 2), abnormal or straddling atrioventricular valve chords (n = 5), and branch pulmonary artery stenosis (n = 3). Four of 8 had previous systemic arterial shunts. Mean cardiopulmonary bypass was 487 minutes, and mean cardiac ischemic time was 307 minutes. Additional procedures included repair of branch pulmonary artery stenoses and closure of multiple muscular VSDs. RESULTS: There was no hospital death. One patient was supported with extracorporeal membrane oxygenation because of junctional tachycardia on postoperative day 5. One patient required pacemaker placement for first-degree heart block. Median hospital length of stay was 31 days. Mean length of follow-up was 52 months. All patients remain well with mild or no aortic regurgitation. The first patient underwent a repeat surgical operation for pulmonary venous baffle obstruction 2 years after SAT. CONCLUSIONS: Despite the technical complexity, patient outcomes have been satisfactory. We believe SAT provides a superior anatomic repair in these complex defects. Longer-term follow-up is needed regarding late intervention.

Selective Use of the Blalock-Taussig Shunt and Right Ventricle-to-Pulmonary Artery Conduit During the Norwood Procedure.

BACKGROUND: The single ventricle reconstruction trial showed better one-year transplant-free survival for the right ventricle-to-pulmonary artery (RV-to-PA) conduit over the modified Blalock-Taussig shunt (mBTS) at Norwood operation. However, concerns remain about the long-term effects of a neonatal ventriculotomy. In our institution, we have used specific selection criteria for the use of mBTS in the Norwood operation. METHODS: We reviewed 122 consecutive neonates undergoing the Norwood procedure from December 2006 to December 2013. We used the following criteria to select our source of pulmonary blood flow: (1) presence of a dominant morphologic left ventricle; (2) presence of antegrade blood in an ascending aorta that is greater than 3 mm; and (3) presence of significant large "crossing coronaries" on ventricle. All patients who met any of the above 3 criteria underwent an mBTS while the remaining patients underwent an RV-to-PA conduit. RESULTS: Seventy-five (61.5%) patients had the RV-to-PA conduit and 47 (38.5%) patients had an mBTS. The overall surgical mortality was 9%. Mean follow-up interval was 23.5 months. Actuarial transplant-free survival was similar at 12, 24, 36, and 48 months in both the mBTS group and the RV-to-PA conduit group. In the RV-to-PA conduit group, actuarial transplant-free survival was 73% at 12 months, 71% at 24 months, 71% at 36 months, and 67% at 48 months, while in the mBTS group, actuarial transplant-free survival was 82% at 12 months, 75% at 24 months, 75% at 36 months, and 75% at 48 months. CONCLUSION: Our selection criteria for mBTS have allowed us to obtain equivalent transplant-free survival at 12, 24, 36, and 48 months when compared to the RV-to-PA conduit group.

Single-Center Experience With the Senning Procedure in the Current Era.

The Senning procedure is an operative technique for atrial inversion in congenital heart anomalies. We sought to evaluate our contemporary outcomes employing this technique. A retrospective analysis of all patients who underwent the Senning procedure at our institution was performed. Hospital records were reviewed, and follow-up data were obtained to evaluate outcomes. Overall, a total of 19 patients underwent a Senning procedure between August 2005 and July 2014. Median age at repair was 594 days (range: 5 days to 15 years). Of those, 7 patients underwent a combined Senning-Rastelli operation and 10 patients underwent a double switch operation (arterial switch or atrial switch). Primary diagnosis was congenitally corrected transposition of the great arteries (cc-TGA) in 17 patients. Associated lesions included ventricular septal defect (n = 10), pulmonary stenosis or atresia (n = 7), and Ebstein׳s anomaly of the tricuspid valve (n = 4). There was an operative death (5%). In all, 2 patients required superior vena cava baffle revision in the operating room. No new pulmonary venous or systemic venous baffle obstruction was observed during follow-up. A total of 5 (26%) patients developed heart block requiring pacemaker implantation. Actuarial survival was 81% at 5 years with a median follow-up of 38 months. The 2 patients in the cc-TGA group were subsequently transplanted. Surviving patients (n = 15) are all well at the last clinical follow-up, most with normal biventricular function. In conclusion, the Senning procedure offers excellent outcomes with regard to systemic and pulmonary venous baffle patency. Results in patients with cc-TGA, however, are affected by significant early mortality after Senning-Rastelli and incidence of left ventricular dysfunction after double switch; which should be considered during patient selection and surgical planning.

Early Outcomes With HeartWare HVAD as Bridge to Transplant in Children: A Single Institution Experience.

The HeartWare HVAD has been used as a bridge to cardiac transplantation in the pediatric population. We describe outcomes following HeartWare HVAD implantation at a single center. A retrospective chart review was performed of all HeartWare HVAD implants performed at our institution between May 2013 and March 2015. Eight children between the ages of 9 and 17 years underwent HVAD implantation as a bridge to transplant (N = 7 cardiomyopathy, N = 1 complex single ventricle). There was one operative death in the complex single ventricle patient. Seven patients (87%) were successfully bridged to transplant. Median time of support was 24.5 days (range, 6-91 days). All transplanted patients are alive and well at a median follow-up of 448 days. Our results demonstrated that mechanical support with HeartWare HVAD is feasible in patients of varying sizes (from older children to adolescents).

Thermally processed polymeric microparticles for year-long delivery of dexamethasone.

Dexamethasone-releasing poly(lactic-co-glycolic acid) (PLGA) microparticles were formulated using a solvent displacement technique with the addition of distillation aiming to increase drug delivery lifetime. Two PLGA copolymer ratios (50:50 and 75:25) were used to determine the influence of lactic acid and glycolic acid ratio on microparticle characteristics. The addition of distillation significantly slows the release of dexamethasone compared to traditional solvent removal via evaporation while still maintaining a therapeutic dosage. Microparticles formulated with PLGA 50:50 controllably release dexamethasone up to one year and 75:25 release up to two years in-vitro. The ratio of lactic acid to glycolic acid plays a significant role in microparticle stability, drug loading efficiency, and thermal properties. In all, this formulation technique offers new prospects for inflammation suppression in pediatric vascular and airway diseases.

Modified Senning Procedure for Correction of Atrioventricular Discordance With Total Anomalous Pulmonary Venous Return, Atrial Situs Inversus, Dextrocardia, and Bilateral Superior Venae Cavae.

The Senning and Mustard baffles remain important techniques for the treatment of congenitally corrected transposition (cc-TGA), isolated ventricular inversion, and D-transposition of the great arteries with delayed presentation. We describe the treatment of an 8-month old infant with atrioventricular discordance, ventriculoarterial concordance, and dextrocardia with atrial situs inversus. A modified Senning procedure was performed through the "left-sided" atrium. Modifications of the Senning and Mustard baffles remain important tools in the treatment of rare conditions like isolated ventricular inversion.

Management and outcomes of heterotaxy syndrome associated with pulmonary atresia or pulmonary stenosis.

BACKGROUND: Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death. METHODS: We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012. Death data were also confirmed with the Social Security Death Index. The log-rank test was done to assess six risk factors for death. RESULTS: We identified 42 patients with heterotaxy and PA/PS. Median age at the first operation was 6.5 days, and median follow-up was 3.5 years. Death data were complete for all patients. Overall mortality was 19% (8 of 42). The 30-day, 1-year, and 5-year mortality estimation was 4.76%, 12.3%, and 19.1% respectively, as determined by the Kaplan-Meier method. The log-rank test showed total anomalous pulmonary venous return (TAPVR) (p<0.05) and obstructed TAPVR requiring an operation at less than 30 days (p=0.001) were significant risk factors for death. CONCLUSIONS: In the current era, surgical treatment of heterotaxy and PA/PS can result in good outcomes. Associated TAPVR and obstructed TAPVR requiring neonatal correction were noted to be risk factors for death.

Fetal cardiac intervention: improved results of fetal cardiac bypass in immature fetuses using the TinyPump device.

OBJECTIVE: Fetal cardiac surgery is a potential innovative treatment for certain congenital heart defects that have significant mortality and morbidity in utero or after birth, but it has been limited by placental dysfunction after fetal cardiac bypass. We have used the TinyPump device for fetal cardiac bypass in sheep fetuses at 90 to 110 days gestation. METHODS: Ten mixed-breed pregnant ewes were used over a period of 6 months, and 10 fetuses were placed on bypass for 30 minutes. Five fetuses with a mean gestational age of 104 ± 4.5 days and mean weight of 1.4 ± 0.4 kg were placed on bypass using the TinyPump device, and 5 fetuses with a mean gestational age of 119 ± 4.5 days and mean weight of 3.4 ± 0.4 kg were placed on bypass using the roller head pump. The fetuses were monitored for up to 3 hours after bypass or until earlier demise. RESULTS: Progressive respiratory and metabolic acidosis developed in all fetuses. The TinyPump group had a lower gestational age and weight compared with the roller head pump group. However, the rate of postbypass deterioration in the TinyPump group, as measured with blood gases, was noted to be significantly slower compared with the roller head pump group. CONCLUSIONS: We demonstrate the feasibility of the TinyPump device for fetal cardiac bypass in a fetal sheep model. The TinyPump group showed improved results compared with the roller head group despite more immature fetuses. The TinyPump device seems to be a promising device for future studies of fetal cardiac bypass in immature fetal sheep and in primates.

Multiple live subconjunctival dipetalonema: report of a case.

Parasitic infestations of the eye have been reported since centuries, affecting various parts of the eye. Some are subtle, coexisting with vision, while many others damage and destroy, in part or totally, the gift of sight. This report describes a patient with live subconjunctival dipetalonema infestation of the right eye, with 22 parasites removed live in one sitting from one eye.

Cerebral near-infrared spectroscopy during cardiopulmonary bypass predicts superior vena cava oxygen saturation.

OBJECTIVE: Cerebral and flank near-infrared spectroscopy are used to monitor tissue oxygenation during cardiopulmonary bypass in pediatric patients. We sought to validate these noninvasive measurements as predictors of oxygen saturation in the superior and inferior venae cavae during cardiopulmonary bypass. METHODS: Eight patients underwent elective repair of congenital heart defects with bicaval cannulation. Ultrasonic flow probes and oximetric catheters were placed in the superior and inferior venae cavae limbs of the perfusion circuit. Cerebral and flank near-infrared spectroscopy and 12 additional variables were recorded each minute on cardiopulmonary bypass. Relationships between these variables and superior and inferior venae cavae oxygen saturation were analyzed by linear mixed modeling. The regression of superior vena cava oxygen saturation by current cerebral near-infrared spectroscopy and 1-minute lag cerebral near-infrared spectroscopy, which are equivalent to the regression of the superior vena cava saturation by the current cerebral near-infrared spectroscopy and the 1-minute change in cerebral near-infrared spectroscopy, were used to assess cerebral near-infrared spectroscopy as a trend monitor. RESULTS: The mean number of observation time points per patient was 86 (median 72, range 34-194) for 690 total observations. The root mean square percentage error was 6.39% for the prediction model of superior vena cava saturation by single-factor cerebral near-infrared spectroscopy. The root mean square percentage error was 10.8% for the prediction model of inferior vena cava saturation by single-factor flank near-infrared spectroscopy. CONCLUSIONS: Cerebral near-infrared spectroscopy accurately predicts superior vena cava oxygen saturation and changes in superior vena cava oxygen saturation on cardiopulmonary bypass. The relationship between flank near-infrared spectroscopy and inferior vena cava saturation is not as strong.

Heart transplantation techniques after hybrid single-ventricle palliation.

The Norwood procedure for hypoplastic left heart syndrome (HLHS) aims to provide an unobstructed systemic outflow tract, unrestrictive inter-atrial communication, controlled source of pulmonary blood flow, and reliable source of coronary blood flow. The hybrid palliative strategy of pulmonary artery banding and ductal stenting has emerged as an alternative treatment for neonates with HLHS. Neonates who have undergone a hybrid Norwood but are not candidates for the three-stage single-ventricle pathway may need heart transplantation. Patients who have undergone hybrid Norwood or those with visceral heterotaxy who have undergone ductal stenting and bilateral PA bands represent a technically challenging group of patients for heart transplantation, but it appears to be a favorable approach and we describe our experience with three patients who underwent heart transplant after a hybrid Norwood procedure.

Pyoderma gangrenosum associated with chronic idiopathic myelofibrosis after coronary artery bypass graft surgery.

Pyoderma gangrenosum (PG) is an ulceronecrotizing dermatosis that can occur after minor trauma or surgery and is rare after cardiac surgery. We report a case of PG after coronary artery bypass grafting (CABG) in a patient with chronic idiopathic myelofibrosis (CIMF). Diagnosis was made with punch skin biopsy and he was treated with systemic steroids. His lesions showed remarkable improvement with this therapy. Cardiothoracic surgeons need to consider this diagnosis in all rapidly expanding postoperative lesions, especially those that do not improve with debridement or antibiotics or conservative wound care.

Ministernotomy for repair of congenital cardiac disease.

We report our experience with repair of a variety of congenital heart defects utilizing a ministernotomy incision. A ministernotomy was used in 79 patients with a variety of congenital heart diseases from November 2004 to August 2007. Patients included 36 males and 43 females with ages ranging from 1 month to 122 months (median age, 22 months). The weight ranged from 3.5 kg to 40 kg (median weight, 10.9 kg). There were no deaths, and one conversion to full median sternotomy (1/79, 1.3%). The median cardiopulmonary bypass time was 59 min, and median aortic cross-clamp time was 38 min. One patient underwent atrial septal defect (ASD) repair with fibrillatory arrest time of 35 min. The operating time ranged from 103 min to 312 min (median operating time, 168 min). The intensive care unit (ICU) stay ranged from 1 to 21 days (median ICU stay, 1 day) and the hospital stay ranged from 2 to 56 days (median hospital stay, 4 days). There were no reinterventions for residual cardiac defects. We demonstrate the safety and efficacy of ministernotomy for the correction of a range of congenital heart defects with improved cosmetic results.

Intestinal obstruction and ileus: role of computed tomography scan in diagnosis and management.

A significant proportion of patients with intestinal obstruction will be evaluated with a CT scan of the abdomen. This study presents a group of 97 patients diagnosed with mechanical obstruction or ileus on CT scan over a 16-month period at a community based teaching hospital and follows the further management of these patients. Our study shows that 43.3 per cent of patients with mechanical obstruction, diagnosed by CT scan, eventually needed surgical treatment. On the other hand, even when CT indicates ileus, 20 per cent of these patients may still require surgical intervention.