Which criteria should be used to select patients for the Fontan operation?

The Fontan operation has improved the survival of children born with single ventricle physiology. Selecting candidates for the Fontan operation may be difficult on borderline cases. No clear criterion has been established on the risk for staged Fontan palliation. Another aspect that remains controversial is the indications for fenestration. Intraoperative pulmonary flow study may identify high-risk patients for the procedure. In this report, the authors describe their results with Fontan procedures in children with pulmonary pressure >15 mmHg.

Dilated cardiomyopathy in children: moving beyond traditional pharmacologic therapy.

PURPOSE OF REVIEW: Dilated cardiomyopathy (DCM) is a rare myocardial disorder characterized by a dilated left ventricle and systolic dysfunction. Globally, it affects around 1 in every 100 000 children. The prognosis is generally poor, with 40% either failing traditional medical therapy within the first 2 years or requiring a heart transplant. This article will address the basic cause, epidemiology, pathobiology, and historical treatment approach of DCM and introduce novel contemporary medical and surgical strategies that may reduce the need for heart transplantation. RECENT FINDINGS: In the last 15 years, there has been a significant emphasis on identifying alternative treatment strategies in managing the child with a DCM and heart failure symptoms. New therapies have evolved to help bridge these critically ill children to transplant or have these therapeutic modalities serve as end-points in themselves. Thus subsequently, we will highlight contemporary as well as novel medical and procedural therapies that are being used for the treatment of pediatric DCM. SUMMARY: The child with a DCM and severe left ventricular dysfunction has a number of options available beyond simple diuretics and afterload reduction. Novel pacing strategies and mechanical assist device may provide not only a more stable clinical bridge environment but also may actually serve as an endpoint itself.

Eighteen years of paediatric extracorporeal membrane oxygenation and ventricular assist devices: insight regarding late outcomes.

BACKGROUND: We reviewed all patients who were supported with extracorporeal membrane oxygenation and/or ventricular assist device at our institution in order to describe diagnostic characteristics and assess mortality. METHODS: A retrospective cohort study was performed including all patients supported with extracorporeal membrane oxygenation and/or ventricular assist device from our first case (8 October, 1998) through 25 July, 2016. The primary outcome of interest was mortality, which was modelled by the Kaplan-Meier method. RESULTS: A total of 223 patients underwent 241 extracorporeal membrane oxygenation runs. Median support time was 4.0 days, ranging from 0.04 to 55.8 days, with a mean of 6.4±7.0 days. Mean (±SD) age at initiation was 727.4 days (±146.9 days). Indications for extracorporeal membrane oxygenation were stratified by primary indication: cardiac extracorporeal membrane oxygenation (n=175; 72.6%) or respiratory extracorporeal membrane oxygenation (n=66; 27.4%). The most frequent diagnosis for cardiac extracorporeal membrane oxygenation patients was hypoplastic left heart syndrome or hypoplastic left heart syndrome-related malformation (n=55 patients with HLHS who underwent 64 extracorporeal membrane oxygenation runs). For respiratory extracorporeal membrane oxygenation, the most frequent diagnosis was congenital diaphragmatic hernia (n=22). A total of 24 patients underwent 26 ventricular assist device runs. Median support time was 7 days, ranging from 0 to 75 days, with a mean of 15.3±18.8 days. Mean age at initiation of ventricular assist device was 2530.8±660.2 days (6.93±1.81 years). Cardiomyopathy/myocarditis was the most frequent indication for ventricular assist device placement (n=14; 53.8%). Survival to discharge was 42.2% for extracorporeal membrane oxygenation patients and 54.2% for ventricular assist device patients. Kaplan-Meier 1-year survival was as follows: all patients, 41.0%; extracorporeal membrane oxygenation patients, 41.0%; and ventricular assist device patients, 43.2%. Kaplan-Meier 5-year survival was as follows: all patients, 39.7%; extracorporeal membrane oxygenation patients, 39.7%; and ventricular assist device patients, 43.2%. CONCLUSIONS: This single-institutional 18-year review documents the differential probability of survival for various sub-groups of patients who require support with extracorporeal membrane oxygenation or ventricular assist device. The indication for mechanical circulatory support, underlying diagnosis, age, and setting in which cannulation occurs may affect survival after extracorporeal membrane oxygenation and ventricular assist device. The Kaplan-Meier analyses in this study demonstrate that patients who survive to hospital discharge have an excellent chance of longer-term survival.

Factors Associated With Mortality in Neonates Requiring Extracorporeal Membrane Oxygenation for Cardiac Indications: Analysis of the Extracorporeal Life Support Organization Registry Data.

OBJECTIVES: Survival among neonates supported with extracorporeal membrane oxygenation for cardiac indications is 39%. Previous single-center studies have identified factors associated with mortality, but a comprehensive multivariate analysis is not available for this population. Understanding factors associated with mortality may help design treatment strategies, determine optimal timing for cannulation, and inform patient selection. This study identifies factors associated with mortality in neonates supported with extracorporeal membrane oxygenation for cardiac indications. DESIGN: Retrospective cohort study. SETTING: Two hundred and thirty U.S. and international centers reporting extracorporeal membrane oxygenation data to the Extracorporeal Life Support Organization. SUBJECTS: Four thousand and four seventy one neonates with congenital and acquired cardiac disease supported with extracorporeal membrane oxygenation for cardiac indications during 2001-2011. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: The primary outcome measure was mortality prior to hospital discharge. Overall hospital mortality was 59%. Demographic and preextracorporeal membrane oxygenation factors associated with mortality were evaluated in a multivariable model. Factors associated with death prior to hospital discharge included lower body weight, earlier era, single ventricle physiology, lower preextracorporeal membrane oxygenation arterial pH, and longer time from intubation to extracorporeal membrane oxygenation cannulation. Lower pH was associated with increased mortality regardless of cardiac diagnosis and surgical complexity. The majority of survivors separated from extracorporeal membrane oxygenation less than 8 days after extracorporeal membrane oxygenation deployment. CONCLUSIONS: Mortality for neonates supported with extracorporeal membrane oxygenation for cardiac indications is high. Severity of preextracorporeal membrane oxygenation acidosis was independently associated with increased risk of mortality. Earlier initiation of extracorporeal membrane oxygenation may reduce the degree and duration of acidosis and may improve survival. Further studies are needed to determine optimal timing of cannulation in this population.

Training fellows and residents in paediatric cardiac critical care.

As pediatric cardiac critical care becomes more sub-specialized it is reasonable to assume that dedicated units may provide a better infrastructure for improved multidisciplinary care, cardiac-specific patient safety initiatives, and dedicated training of fellows and residents. The knowledge base required to optimally manage pediatric patients with critical cardiac disease has evolved sufficiently to consider a standardized training curriculum and board certification for pediatric cardiac critical care. This strategy would potentially provide consistency of training and healthcare and improve quality of care and patient safety.

Extracorporeal membrane oxygenation for the support of adults with acute myocarditis.

OBJECTIVES: To characterize survival outcomes for adult patients with acute myocarditis supported with extracorporeal membrane oxygenation and identify risk factors for in-hospital mortality. DESIGN: Retrospective review of Extracorporeal Life Support Organization registry database. SETTING: Data reported to Extracorporeal Life Support Organization by 230 extracorporeal membrane oxygenation centers. PATIENTS: Patients 16 years old or older supported with extracorporeal membrane oxygenation for myocarditis during 1995 to 2011. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 150 separate runs of extracorporeal membrane oxygenation for 147 patients with a diagnosis of acute myocarditis in the Extracorporeal Life Support Organization database from 1995 through 2011. Survival to hospital discharge was 61%. Nine patients underwent heart transplantation, and transplant-free survival to discharge was 56%. Extracorporeal membrane oxygenation was deployed during extracorporeal cardiopulmonary resuscitation in 31 patients (21% of the cohort). In a multivariate model evaluating pre-extracorporeal membrane oxygenation and extracorporeal membrane oxygenation support factors, pre-extracorporeal membrane oxygenation arrest (adjusted odds ratio, 2.4; 95% CI, 1.1-5.0) and need for higher extracorporeal membrane oxygenation flows at 4 hours post-extracorporeal membrane oxygenation cannulation (odds ratio, 2.8; 95% CI, 1.1-7.3) were associated with increased odds of in-hospital mortality. In a second multivariate model evaluating adverse events while on extracorporeal membrane oxygenation, central nervous system injury (odds ratio, 26.5; 95% CI, 7.3-96.6), renal failure (odds ratio, 3.6; 95% CI, 1.4-9.3), arrhythmia (odds ratio, 5.8; 95% CI, 2.2-15.1), and hyperbilirubinemia (odds ratio, 9.1; 95% CI, 2.6-31.8) were associated with increased odds of in-hospital mortality. CONCLUSIONS: Extracorporeal membrane oxygenation can be used effectively in adults with myocarditis to support the circulation while awaiting myocardial recovery. Early extracorporeal membrane oxygenation deployment prior to cardiac arrest may be associated with better outcomes.

Extracorporeal membrane oxygenation-supported cardiopulmonary resuscitation following stage 1 palliation for hypoplastic left heart syndrome.

OBJECTIVES: To report on survival from a large multicenter cohort of neonates with hypoplastic left heart syndrome requiring extracorporeal membrane oxygenation-assisted cardiopulmonary resuscitation after stage 1 palliation operation. DESIGN: Retrospective analysis of data from the Extracorporeal Life Support Organization data registry (1998 through 2013). We computed the survival to hospital discharge for neonates (age < 30 d) who required extracorporeal membrane oxygenation after stage 1 palliation and evaluated factors associated with mortality using multivariate logistic regression analysis. SETTING: Multicenter data reported to Extracorporeal Life Support Organization registry. PATIENTS: Infants with hypoplastic left heart syndrome after stage 1 palliation who received extracorporeal membrane oxygenation-assisted cardiopulmonary resuscitation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 307 extracorporeal membrane oxygenation runs in the setting of extracorporeal membrane oxygenation-assisted cardiopulmonary resuscitation in 293 neonates with hypoplastic left heart syndrome following stage 1 palliation operation. The median age at cannulation was 9 days (interquartile range, 5-14 d). Survival to hospital discharge was 36%. In univariate analysis, gestational age, weight, extracorporeal membrane oxygenation duration, presence of air embolism, hemorrhagic complications, renal failure, and pulmonary complications (pulmonary hemorrhage and pneumothorax) were all associated with nonsurvival. In multivariate analysis, lower body weight at cannulation (odds ratio, 3.9; 95% CI, 1.9-8.3), duration of the extracorporeal membrane oxygenation (odds ratio, 3.4; 95% CI, 1.9-7.3), and renal failure while on extracorporeal membrane oxygenation (odds ratio, 2; 95% CI, 1.2-3.5) increased odds of mortality. CONCLUSIONS: Mortality for neonates with hypoplastic left heart syndrome supported with extracorporeal membrane oxygenation-assisted cardiopulmonary resuscitation after stage 1 palliation is high. Lower body weight, increased duration of extracorporeal membrane oxygenation support, and renal failure increased mortality.

Femoral artery catheterization in neonates and infants.

OBJECTIVE: To determine the incidence of perfusion-related complications associated with indwelling femoral artery monitoring catheters in neonates and infants following introduction of a 2.5-F diameter, 5-cm length, polyethylene catheter (Cook Medical, Bloomington, IN) to our unit. DESIGN: Prospective observational cohort study. SETTING: Pediatric cardiac intensive care unit in a university-affiliated children's hospital. PATIENTS: All patients <2 yrs old with an indwelling femoral artery catheter during a 3-yr period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Two hundred eighty-two patients (including 98 neonates), median (range) age 10 wks (0.1-84), weight 4.1 kg (2.0-11.1) were enrolled; outcomes in 249 were evaluable. Pulse strength in dorsalis pedis arteries and pulse discrepancies between feet were assessed hourly by the cardiac intensive care unit nurse and recorded on a flow sheet. Nonpalpable pulses were assessed as "absent" or "present" with ultrasonic Doppler. Following removal of the catheter, assessments of pulse strength continued until resolution of any discrepancies. Median (range) duration of catheterization was 4 days (1-23). Catheters of 2.5-F diameter were used in 227 patients and larger catheters in 55 patients. The incidence of pulse strength discrepancies between feet was 20%, loss of pulse was 3.4% (6.7% in neonates, 1.4% in older infants) when extracorporeal membrane oxygenation patients were excluded, and resolution of pulse discrepancy or loss was 100%. Duration of catheterization and use of a catheter larger than 2.5 Fr were significant predictors of loss of pulse. CONCLUSIONS: Loss of pedal pulse distal to small-bore monitoring femoral artery catheters in neonates and infants is directly related to the duration of catheterization and is less frequent when 2.5-F, 5-cm polyethylene catheters are used instead of larger catheters.

Outcomes and risk factors for mortality in premature neonates with critical congenital heart disease.

We sought to describe contemporary outcomes and identify risk factors for hospital mortality in premature neonates with critical congenital heart disease who were referred for early intervention. Neonates who were born before 37 weeks' gestation with critical congenital heart disease and admitted to our institution from 2002 to 2008 were included in this retrospective cohort study. Critical congenital heart disease was defined as a defect requiring surgical or transcatheter cardiac intervention or a defect resulting in death within the first 28 days of life. Logistic regression analyses were performed to identify risk factors for mortality before hospital discharge. The study included 180 premature neonates, of whom 37 (21%) died during their initial hospitalization, including 6 (4%) before cardiac intervention and 31 (17%) after cardiac intervention. For the 174 patients undergoing cardiac intervention, independent risk factors for mortality were a 5 min Apgar score ≤ 7, need for preintervention mechanical ventilation, and Risk Adjustment in Congenital Heart Surgery category ≥ 4 or not assignable. Mortality for premature infants with critical congenital heart disease who are referred for early intervention remains high. Patients with lower Apgar scores who receive preintervention mechanical ventilation and undergo more complex procedures are at greatest risk.

Perioperative care of the infant with single ventricle physiology.

OPINION STATEMENT: Among patients with congenital heart defects, neonates with single ventricle disease continue to challenge clinicians despite significant improvements in survival over the past 30 years. The cardiac anatomical variants associated with the term "single ventricle" are characterized by severe hypoplasia (or absence) of either ventricle, typically in association with obstruction or atresia of either the pulmonary or systemic outflow tracts. Physiologically, the single ventricle receives both pulmonary and systemic venous blood and ejects simultaneously into the pulmonary and systemic circulations, a pattern commonly referred to as single ventricle physiology. Medical and surgical management strategies, though palliative, are aimed at achieving the optimal balance of systemic blood flow and pulmonary blood flow to maximize oxygen delivery. Patients with single ventricle physiology have a greater risk of dying than those with biventricular circulations and are generally committed to multiple palliative interventions throughout childhood with considerable risk. Surgical intervention in the newborn period involves Norwood Stage I palliation, placement of a systemic-to-pulmonary artery shunt, or banding of the pulmonary artery, depending on the status of the outflow tracts. Heart transplantation is offered as the initial approach in some centers. The management strategy and the actual delivery of care from the time of birth (or at time of diagnosis) through the postoperative period is crucial to optimize the short-term and long-term outcomes. Whereas survival following initial palliation in experienced centers is as high as 95%, emphasis is now appropriately shifting toward the control of in-hospital morbidity and optimizing long-term functional outcome. Centers are continually striving to gather and apply new knowledge related to the underlying anatomical and physiologic problems while seeking to improve decision making and care of the patient with single ventricle physiology.

Anesthesia considerations for children with pulmonary hypertension.

Children with pulmonary arterial hypertension undergoing anesthesia pose a challenge. The prevalence of morbidity and mortality in this subgroup is substantially greater than that in the general population. In this article, we attempt to describe the adverse events that occur and also identify some of the factors that may precipitate them. We also suggest mechanisms to attenuate or prevent these crises.

Applying Educational Theory to Interdisciplinary Education in Pediatric Cardiac Critical Care.

At the 14th Annual International Meeting for the Pediatric Cardiac Intensive Care Society, the authors presented a simulation workshop for junior multidisciplinary providers focused on cardiopulmonary interactions. We provide an overview of educational theories of particular relevance to curricular design for simulation-based or enhanced activities. We then demonstrate how these theories are applied to curriculum development for individuals to teams and for novice to experts. We review the role of simulation in cardiac intensive care education and the education theories that support its use. Finally, we demonstrate how a conceptual framework, SIMZones, can be applied to design effective simulation-based teaching.

Inadequate oxygen delivery index dose is associated with cardiac arrest risk in neonates following cardiopulmonary bypass surgery.

AIM: To evaluate the Inadequate oxygen delivery (IDO2) index dose as a predictor of cardiac arrest (CA) in neonates following congenital heart surgery. METHODS: Retrospective cohort study in 3 US pediatric cardiac intensive units (1/2011- 8/2016). Calculated IDO2 index values were blinded to bedside clinicians and generated from data collected up to 30 days postoperatively, or until death or ECMO initiation. Control event data was collected from patients who did not experience CA or require ECMO. IDO2 dose was computed over a 120-min window up to 30 min prior to the CA and control events. A multivariate logistic regression prediction model including the IDO2 dose and presence or absence of a single ventricle (SV) was used. Model performance metrics were the odds ratio for each regression coefficient and receiver operating characteristic area under the curve (ROC AUC). RESULTS: Of 897 patients monitored during the study period, 601 met inclusion criteria: 29 patients had CA (33 events) and 572 patients were used for control events. Seventeen (59%) CA and 125 (26%) control events occurred in SV patients. Median age/weight at surgery and level of monitoring were similar in both groups. Median postoperative event time was 0.73 days [0.05-22.39] in CA patients and 0.82 days [0.08 25.11] in control patients. Odds ratio of the IDO2 dose coefficient was 1.008 (95% CI: 1.006-1.012, p = 0.0445), and 2.952 (95% CI: 2.952-3.258, p = 0.0079) in SV. The ROC AUC using both coefficients was 0.74 (95% CI: 0.73-0.75). These associations of IDO2 dose with CA risk remained robust, even when censored periods prior to arrest were 10 and 20 min. CONCLUSION: In neonates post-CPB surgery, higher IDO2 index dose over a 120-min monitoring period is associated with increased risk of cardiac arrest, even when censoring data 10, 20 or 30 min prior to the CA event.

Correlation of abdominal site near-infrared spectroscopy with gastric tonometry in infants following surgery for congenital heart disease.

OBJECTIVE: Splanchnic oximetry, as measured by near-infrared spectroscopy (NIRS), correlates with gastric tonometry as a means of assessing regional (splanchnic) oxygenation and perfusion. DESIGN: Prospective, data-gathering study. SETTING: Pediatric cardiac intensive care unit in a tertiary care children's hospital. SUBJECTS: Neonates and infants with congenital heart disease who underwent catheter intervention or surgical repair requiring cardiopulmonary bypass. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Twenty neonates and infants were studied within 48 hrs of surgery. We measured somatic saturation (rSO2) via NIRS sensors placed over the anterior abdomen (splanchnic bed) and dorsal lateral flank (renal bed). Somatic rSO2 readings were paired with simultaneous points of intramucosal gastric pH (pHi), measured by tonometry. The rSO2 readings were paired with serum lactate and measurements of systemic mixed venous saturation (SVO2). There was strong correlation between the abdominal rSO2 and pHi (r = .79; p < .0001) as well as between abdominal rSO2 and SVO2 (r = .89; p < .0001). There was also significant negative correlation between the abdominal rSO2 and serum lactate (r = .77; p < .0001). Correlations between the dorsal lateral (renal) rSO2 measurements and serum lactate and SVO2 were also significant but not as strong. CONCLUSIONS: Abdominal site rSO2, measured in infants with either single or biventricular physiology, exhibits a strong correlation with gastric pHi as well as with serum lactate and SVO2. The results indicate that rSO2 measurements over the anterior abdominal wall correlate more strongly than flank rSO2 with regard to systemic indices of oxygenation and perfusion. This study suggests that the NIRS monitor is a valid modality to obtain an easy, immediate, and noninvasive measurement of splanchnic rSO2 in infants following cardiac surgery for congenital heart disease.

Risk Factors for Survival After Heart Transplantation in Children and Young Adults: A 22-Year Study of 179 Transplants.

BACKGROUND: This article reviews all patients who underwent heart transplantation (HTx) within a single institution (172 patients underwent 179 HTx [167 first-time HTxs, 10 second HTxs, 2 third HTxs]) to describe diagnostic characteristics, management protocols, and risk factors for mortality. METHODS: Descriptive analysis was performed for the entire cohort using mean, standard deviation, median, interquartile range, and overall range, as appropriate. Univariable and multivariable Cox proportional hazards models were performed to identify prognostic factors for outcomes over time. The primary outcome of interest was mortality, which was modeled by Kaplan-Meier analysis. RESULTS: Median age at HTx was 263 days (range, 5 days to 24 years; mean = 4.63 ± 5.95 years; 18 neonates, 79 infants). Median weight at HTx was 7.5 kg (range, 2.2-113 kg; mean = 19.36 ± 23.54). Diagnostic categories were cardiomyopathy (n = 62), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 33), transplantation after cardiac surgery for HLHS or HLHS-related malformation (n = 17), non-HLHS congenital heart disease (n = 55), and retransplant (n = 12). Operative mortality was 10.1% (18 patients). Cumulative total follow-up is 1,355 years. Late mortality was 18.4% (33 patients). Overall Kaplan-Meier five-year survival was 76.2%. One hundred twenty-one patients are alive with a mean follow-up of 7.61 ± 6.46 years. No survival differences were seen among the five diagnostic subgroups ( P = .064) or between immunosensitized patients (n = 31) and nonimmunosensitized patients (n = 141; P = .422). CONCLUSIONS: Excellent results are expected for children undergoing HTx with comparable results among diagnostic groups. Pretransplant mechanical circulatory support and posttransplant mechanical circulatory support are risk factors for decreased survival. Survival after transplantation for HLHS or HLHS-related malformation is better with primary HTx in comparison to HTx after prior cardiac surgery.

Hypothyroidism in Infants With Congenital Heart Disease Exposed to Excess Iodine.

Thyroid hormone is critical for neonatal brain development, and even transient hypothyroidism can cause adverse neurocognitive outcomes. Infants exposed to excess iodine are at risk of developing hypothyroidism, especially those with congenital heart disease (CHD), because they are routinely exposed to excess iodine from intravenous iodinated contrast media and topical antiseptics. The aim of the present study was to identify the proportion of neonates with CHD exposed to iodine who developed hypothyroidism and to identify the associated risk factors. This was a retrospective study of neonates undergoing cardiac catheterization at Boston Children's Hospital during a 3-year period, some of whom also underwent cardiac surgery. Hypothyroidism was defined as an elevated thyroid-stimulating hormone level (>20 mIU/L at 24 to 96 hours of age and >15 mIU/L at >96 hours of age by heel-stick sampling and >9.1 mIU/L at 1 to 20 weeks of age by serum testing). Multivariate logistic regression was performed to predict the odds of developing hypothyroidism. Hypothyroidism was diagnosed incidentally in 46 of 183 infants (25%) with CHD after iodine exposure. Controlling for baseline cardiac risk, postnatal age, and gestational age, we found a fourfold increase in odds of developing hypothyroidism in neonates with serum creatinine >0.9 mg/dL and a fourfold increase in those who underwent more than three procedures. Hypothyroidism in neonates with CHD exposed to excess iodine is associated with multiple procedures and impaired renal function. Routine serial monitoring of thyroid function in these neonates is warranted. Future studies should examine the association between hypothyroidism and neurocognitive function in this population.

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification.

BACKGROUND: The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO). We reviewed our experience with DORV patients and two ventricles that underwent repair, applying this nomenclature. METHODS: Between January 2000 and January 2005, 50 patients with DORV and two viable ventricles underwent surgical intervention: 44 patients had biventricular repair, 3 had 1.5 ventricular repair, 2 underwent a Fontan, and 1 died prior to corrective surgery. Median age at repair was 9.1 months (range: 4D-4Y). Eighteen patients (36%) were DORV-Fallot (including 5 with AVSD and heterotaxy), 9 (18%) were DORV-TGA (Taussig-Bing), 12 (24%) were DORV-VSD, and 11 (22%) were DORV non-committed VSD. Corrective surgery included 35 repairs with a VSD-aorta baffle+/-RVOTO procedure and 12 arterial switches with a VSD-PA baffle (9 Taussig-Bing and 3 DORV-ncVSD). Associated procedures included 13 VSD enlargements, 8 subaortic resections, 9 arch repairs, 5 AVSD repairs, and 7 others. RESULTS: There were three deaths in the 50 patients studied (overall mortality of 6%). Excluding one patient that died prior to corrective surgery and the two patients palliated with a Fontan procedure, the actual surgical mortality for a corrective repair was 4.3% (2/47 patients). Two surgical deaths occurred following, respectively, one repair of a Taussig-Bing with an interrupted arch and a Swiss cheese VSD and one repair of ncVSD-type with pulmonary atresia that had undergone a previous cavo-pulmonary anastamosis. No late deaths occurred. Two late reoperations included a heart transplant in a DORV-Fallot patient with Swiss cheese VSD and subaortic resection in a DORV-ncVSD patient. Angioplasties were needed for PA stenosis (n=2) and aortic arch obstruction (n=2). Four patients had LV to aorta baffle gradients between 10 and 20 mmHg. All patients were of NYHA class I/II. CONCLUSIONS: The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality. Biventricular repair can be safely achieved on selected DORV, including DORV-ncVSD and DORV with AVSD and heterotaxy lesions traditionally indicated for a single ventricle palliative approach.

Theoretical and Evidence-Based Benefit of Afterload Reduction in the Postoperative Pediatric Heart.

Rational use of vasodilators to induce afterload reduction is predicated on a thorough knowledge of the constituents of afterload and of the role ventriculoarterial coupling plays in determining their effects. Afterload reduction therapy should be goal directed with the intent to improve stroke volume and tissue oxygen delivery rather than to decrease blood pressure per se. This review will summarize the components comprising circulatory system afterload and will use ventriculoarterial coupling concepts to demonstrate the variable but predictable effects of vasodilator therapy on hemodynamics and tissue oxygen delivery. This article addresses the third of eight topics comprising the special issue entitled "Pharmacologic strategies with afterload reduction in low cardiac output syndrome after pediatric cardiac surgery".

A Review of Systemic Vasodilators in Low Cardiac Output Syndrome Following Pediatric Cardiac Surgery.

Following surgery for congenital heart disease, patients develop a predictable and progressive decline in cardiac output known as low cardiac output syndrome. During low cardiac output states, a compensatory response to increase systemic perfusion occurs both innately and as part of the postoperative pharmacologic support strategies intended to increase or sustain adequate oxygen delivery. The result typically involves a rise in systemic vascular resistance and heart rate. These and other responses may actually limit the ability of the recently operated heart to provide sufficient cardiac output to meet the oxygen demands of the body. In order to improve systemic oxygen delivery, clinicians have increasingly employed systemic vasodilator therapy to reduce afterload and improve ventriculoarterial coupling. This review will summarize currently utilized pharmacologic agents that promote systemic vasodilation and improve cardiac output through afterload reduction. This article addresses the fourth of eight topics comprising the special issue entitled "Pharmacologic strategies with afterload reduction in low cardiac output syndrome after pediatric cardiac surgery".