Characteristics of Hospitalizations for the Glenn Procedure in Those With Isomerism Compared to Those Without.

Isomerism, also known as heterotaxy, is a unique clinical entity in which there are mirror imaged findings in the thoracic. In the abdomen, the arrangement of organs can be random. The anatomic findings also have functional consequences. Isomerism has been known to increase morbidity and mortality in those with functionally univentricular hearts. The aim of this study was to determine the impact of isomerism on the Glenn hospitalization. Data from the 1997 to 2012 Kids' Inpatient Database were utilized for this cross-sectional study. Admissions during which a Glenn procedure was done were identified. Next, these admissions were separated into those with and without isomerism. Admission characteristics were then compared in a univariate fashion as well as by regression analysis. Length of hospitalization, cost of hospitalization, extracorporeal membrane oxygenation, and inpatient mortality were the outcomes of interest. A total of 4959 admissions with a Glenn procedure were identified. Of these, 450 were associated with isomerism. The median age at which the Glenn procedure was done was 10 and 13 months in those without and with isomerism, respectively. Neither univariate nor regression analysis demonstrated any significant difference in length of hospitalization, cost of hospitalization, need for extracorporeal membrane oxygenation, and inpatient mortality between those with and without isomerism. Isomerism does not impact the characteristics of the Glenn hospitalization. Those with isomerism did tend to be older when they underwent the Glenn procedure.

Association of Body Mass Index With Clinical Features and Outcomes in Adults With Fontan Palliation.

Background With improving survival of patients with single ventricle physiology who underwent Fontan palliation, there is also an increase in the prevalence of overweight and obesity in these patients. This tertiary care single-center study aims to determine the association of body mass index (BMI) with the clinical characteristics and outcomes in adults with Fontan. Methods and Results Adult patients (aged ≥18 years) with Fontan who were managed at a single tertiary care center between January 1, 2000, and July 1, 2019, and had BMI data available were identified via retrospective review of medical records. Univariate and multivariable (after adjusting for age, sex, functional class, and type of Fontan) linear and logistic regression, as appropriate, were utilized to evaluate associations between BMI and diagnostic testing and clinical outcomes. A total of 163 adult patients with Fontan were included (mean age, 29.9±9.08 years), with a mean BMI of 24.2±5.21 kg/m2 (37.4% of patients had BMI ≥25 kg/m2). Echocardiography data were available for 95.7% of patients, exercise testing for 39.3% of patients, and catheterization for 53.7% of patients. Each SD increase in BMI was significantly associated with decreased peak oxygen consumption (P=0.010) on univariate analysis and with increased Fontan pressure (P=0.035) and pulmonary capillary wedge pressure (P=0.037) on multivariable analysis. In addition, BMI ≥25 kg/m2 was independently associated with heart failure hospitalization (adjusted odds ratio [AOR], 10.2; 95% CI, 2.79-37.1 [P<0.001]) and thromboembolic complications (AOR, 2.79; 95% CI, 1.11-6.97 [P=0.029]). Conclusions Elevated BMI is associated with poor hemodynamics and worse clinical outcomes in adult patients with Fontan. Whether elevated BMI is the cause or consequence of poor clinical outcomes needs to be further established.

Fetal diagnosis of superior vena cava aneurysm.

Superior vena cava aneurysm is a rare intrathoracic vascular lesion with only 27 cases reported in the literature. The majority are fusiform and can be associated with cystic hygroma due to the close embryonic relationship between lymphatic vessels and systemic veins. This is the first report of superior vena cava aneurysm diagnosed with fetal echocardiography in a fetus with a cystic hygroma. There is a need of a prospective registry to further delineate all aspects of this condition and establish the most appropriate therapeutic approach.

Journey Toward Improved Long-Term Outcomes After Norwood-Sano Procedure: Focus on the Aortic Arch Reconstruction.

The disadvantage of right ventricle-to-pulmonary artery (RV-PA) shunt is the need for more unplanned interventions to address stenosis in the shunt or branch pulmonary arteries, as compared to the modified Blalock-Taussig shunt group. Ring-enforced RV-PA PTFE conduit and dunk technique minimized these complications and right ventricle (RV) damage. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation; therefore, most surgeons prefer to use homograft, autologous pericardium, or bovine pericardium to reconstruct the neoaorta. Artificial materials decrease the elastic properties, increase wall stiffness, and decrease the distensibility of the aorta; and as a result, RV function gradually deteriorates. This inelastic reconstructed aorta may be one of the reasons why long-term outcomes after the Fontan procedure are worse in hypoplastic left heart syndrome (HLHS) patients, in comparison to non-HLHS. Reconstruction of the neoaorta without any patch materials, or at least techniques that largely minimize the use of non-autologous materials, will offer a further refinement of our ability to optimize ventriculoarterial coupling and thereby long-term RV function.

Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle.

OBJECTIVE: This tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle. METHODS: This retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death. RESULTS: 160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8-21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration. CONCLUSIONS: In this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

Experience with the Levitronix CentriMag in the pediatric population as a bridge to decision and recovery.

Short-term mechanical circulatory support in the pediatric population with acute cardiac failure has traditionally been limited to extracorporeal membrane oxygenation given the limited availability of pediatric-sized pumps. The Levitronix CentriMag system (Thoratec Corporation, Pleasanton, CA, USA) offers expanded options for short-term support for this population. We report our experience with the successful use of the CentriMag in the pediatric population as a bridge to decision after postcardiotomy ventricular failure and as a bridge to recovery after heart transplantation. The first patient was bridged to a long-term HeartMate II (Thoratec Corporation) as a bridge to potential recovery. The second patient was supported after severe graft failure post heart transplantation, with a full recovery. The Levitronix CentriMag has proven to be a versatile, safe, and effective short-term circulatory support system for our pediatric patients.

Management of deep wound complications with vacuum-assisted therapy after Berlin Heart EXCOR ventricular assist device placement in the pediatric population.

Wound complications after ventricular assist device (VAD) placement remain a formidable challenge to surgeons. The Berlin Heart EXCOR VAD is a versatile pulsatile system that has been successful in pediatric patients of all ages and sizes. Prevention of device-related complications such as infection, particularly in pediatric patients, remains an essential issue in minimizing patient morbidity and mortality. The introduction of vacuum-assisted wound closure (VAC) therapy and its application in VAD-related wound complications provide an efficient and effective method for wound healing. We report our experience in the management of deep wound complications in two pediatric patients after placement of the Berlin Heart EXCOR VAD. The wound VAC system proved to achieve complete wound healing without any infectious complications.

360-Degree Cone Reconstruction for Ebstein's Anomaly.

Several modifications of the cone procedure as first described by da Silva have been reported. We have used a complete 360-degree leaflet detachment coupled with a 60-degree counterclockwise rotation in performing the cone procedure in children and adults. We believe that complete leaflet detachment coupled with counterclockwise rotation provides both for better alignment of the leaflets and subvalvular apparatus and for a more symmetric distribution of tension after leaflet reattachment, thus potentially resulting in better valve function and longevity of repair.

S-Nitrosohemoglobin Levels and Patient Outcome After Transfusion During Pediatric Bypass Surgery.

Banked blood exhibits impairments in nitric oxide (NO)-based oxygen delivery capability, reflected in rapid depletion of S-nitrosohemoglobin (SNO-Hb). We hypothesized that transfusion of even freshly-stored blood used in pediatric heart surgery would reduce SNO-Hb levels and worsen outcome. In a retrospective review (n = 29), the percent of estimated blood volume (% eBV) replaced by transfusion directly correlated with ventilator time and inversely correlated with kidney function; similar results were obtained in a prospective arm (n = 20). In addition, an inverse association was identified between SNO-Hb and postoperative increase in Hb (∆Hb), reflecting the amount of blood retained by the patient. Both SNO-Hb and ∆Hb correlated with the probability of kidney dysfunction and oxygenation-related complications. Further, regression analysis identified SNO-Hb as an inverse predictor of outcome. The findings suggest that SNO-Hb and ∆Hb are prognostic biomarkers following pediatric cardiopulmonary bypass, and that maintenance of red blood cell-derived NO bioactivity might confer therapeutic benefit.

Scimitar Syndrome-Complex Surgical Revision 3 Decades After Repair.

We present a case of a 39-year-old woman with scimitar syndrome who had a 2-patch repair 3 decades previously and presented with a right-to-left shunt of the inferior vena cava (IVC) to the left atrium resulting from baffle dehiscence. We discuss details of our reoperative repair.

Towards the development of a pediatric ventricular assist device.

The very limited options available to treat ventricular failure in children with congenital and acquired heart diseases have motivated the development of a pediatric ventricular assist device at the University of Pittsburgh (UoP) and University of Pittsburgh Medical Center (UPMC). Our effort involves a consortium consisting of UoP, Children's Hospital of Pittsburgh (CHP), Carnegie Mellon University, World Heart Corporation, and LaunchPoint Technologies, Inc. The overall aim of our program is to develop a highly reliable, biocompatible ventricular assist device (VAD) for chronic support (6 months) of the unique and high-risk population of children between 3 and 15 kg (patients from birth to 2 years of age). The innovative pediatric ventricular assist device we are developing is based on a miniature mixed flow turbodynamic pump featuring magnetic levitation, to assure minimal blood trauma and risk of thrombosis. This review article discusses the limitations of current pediatric cardiac assist treatment options and the work to date by our consortium toward the development of a pediatric VAD.

The PediaFlow pediatric ventricular assist device.

The very limited options available to treat ventricular failure in patients with congenital and acquired heart diseases have motivated the development of a pediatric ventricular assist device (VAD). Our effort involves a consortium consisting of the University of Pittsburgh, Carnegie Mellon University, Children's Hospital of Pittsburgh, World Heart Corporation, and LaunchPoint Technologies, LLC. The overall aim of our program is to develop a highly reliable, biocompatible VAD for chronic support (6 months) of the unique and high-risk population of children between 3 kg and 15 kg (patients from birth to 2 years of age). The innovative pediatric VAD we are developing (PediaFlow) is based on a miniature mixed-flow turbodynamic pump featuring magnetic levitation, with the design goal being to assure minimal blood trauma and risk of thrombosis. This article discusses the limitations of current pediatric cardiac assist treatment options and the work to date by our consortium toward the development of a pediatric VAD.

Venous Myocardial Infarction in an Infant with Obstructed Totally Anomalous Pulmonary Venous Drainage and Coronary Sinus Ostial Atresia.

We report a rare causal association between obstructed supracardiac totally anomalous pulmonary venous drainage and coronary sinus ostial atresia. Our 12-week-old patient developed venous myocardial infarction secondary to coronary venous hypertension because her sole route of coronary venous drainage was obstructed. She recovered after the obstruction was relieved by balloon dilation. Surgical repair then included anastomosis of the pulmonary venous confluence to the left atrium, ligation of the vertical vein, and unroofing of the coronary sinus. Coronary sinus ostial atresia is rarely diagnosed before autopsy.

Role of nitric oxide in heparin-induced attenuation of hypoxic pulmonary vascular remodeling.

Heparin and nitric oxide (NO) attenuate changes to the pulmonary vasculature caused by prolonged hypoxia. Heparin may increase NO; therefore, we hypothesized that heparin may attenuate hypoxia-induced pulmonary vascular remodeling via a NO-mediated mechanism. In vivo, rats were exposed to normoxia (N) or hypoxia (H; 10% O(2)) with or without heparin (1,200 U x kg-1 x day-1) and/or the NO synthase (NOS) inhibitor Nomega-nitro-L-arginine methyl ester (L-NAME; 20 mg x kg-1 x day-1) for 3 days or 3 wk. Heparin attenuated increases in pulmonary arterial pressure, the percentage of muscular pulmonary vessels, and their medial thickness induced by 3 wk of H. Importantly, although L-NAME alone had no effect, it prevented these effects of heparin on vascular remodeling. In H lungs, heparin increased NOS activity and cGMP levels at 3 days and 3 wk and endothelial NOS protein expression at 3 days but not at 3 wk. In vitro, heparin (10 and 100 U x kg-1 x ml-1) increased cGMP levels after 10 min and 24 h in N and anoxic (0% O2) endothelial cell-smooth muscle cell (SMC) coculture. SMC proliferation, assessed by 5-bromo-2'-deoxyuridine incorporation during a 3-h incubation period, was decreased by heparin under N, but not anoxic, conditions. The antiproliferative effects of heparin were not altered by L-NAME. In conclusion, the in vivo results suggest that attenuation of hypoxia-induced pulmonary vascular remodeling by heparin is NO mediated. Heparin increases cGMP in vitro; however, the heparin-induced decrease in SMC proliferation in the coculture model appears to be NO independent.

Pyloric exclusion for treatment of complicated duodenal atresia.

Duodenal atresia (DA) is a well-described congenital anomaly that usually responds well to surgical correction. Associated defects are common, and these confounding variables often influence outcome. The authors present a case of a newborn female with an unusual constellation of problems including DA with annular pancreas, trisomy 21, and coarctation of the aorta. She developed protracted complications postoperatively and was treated with an innovative surgical strategy.

Long-term Outcome and Risk of Heart Block After Surgical Treatment of Subaortic Stenosis.

Although mortality following repair of subaortic obstruction is low, aggressive resection may increase morbidity. We sought to evaluate outcomes and risk of atrioventricular heart block (AVB) after subaortic resection in the current era. Simple obstruction was defined as a discrete subaortic membrane and complex as multilevel or diffuse narrowing. Limited resection included membranectomy and limited myomectomy. Aggressive resection included Konno, modified Konno, and Ross. Specified variables were obtained from a chart review. The 185 consecutive patients (1991-2008) ranged in age from 1 day to 21.8 years (5.1 ± 5.1 years) with 2 early and 4 late deaths. Actuarial survival was 97%, 95%, and 95% at 1, 5, and 10 years, respectively. Reoperations were required in 29 of 185 patients (15.7%); 2 required a third operation (1%). Freedom from reoperation in all patients was 97%, 83%, and 73% at 1, 5, and 10 years, respectively. Accessory mitral valve tissue (P < .001) and age <3 months (P = .004) predicted the need for reoperation. Transient or permanent high-degree AVB was documented in 33 of 185 patients (17.8%). Complex anatomy (P = .01) and aggressive resection (P < .001) increased the risk of acquiring AVB. The AVB was permanent in 21 of 185 (11.4%) patients, and pacemaker implantation was undertaken in 20 of 185 (10.8%) patients. Complex anatomy (P = .04) and modified Konno procedure (P = .03) increased the risk of acquiring a pacemaker. Aggressive resection lowered the frequency of recurrence but increased the risk of AVB. When aggressive resection is considered for long-term relief of subaortic obstruction, the risk of reobstruction must be balanced with the risk of AVB and the need for pacemaker implantation.