Dominant Ventricular Morphology and Early Postoperative Course After the Fontan Procedure.

BACKGROUND: Single ventricle heart disease comprises a wide variety of critical heart defects that lead to the provision of systemic cardiac output by one dominant ventricle. It requires staged surgical palliation that culminates in Fontan circulation. Dominant ventricular morphology in single ventricle patients reportedly has an impact on postoperative morbidity and mortality with varying results. The objectives of this study were to examine the association between ventricular morphology and the early postoperative course after the Fontan procedure. METHODS: A retrospective cohort study in a tertiary referral pediatric medical center that included 98 consecutive patients who underwent Fontan procedure between October 2009 and May 2016. Postoperative outcomes were compared between patients with left ventricular morphology and those with right ventricular morphology (crude effect and regression analysis). RESULTS: Patients with right ventricular morphology had longer postoperative hospitalizations compared to patients with left ventricular morphology (26.5 days vs 18.2 days, respectively, P = .028), higher postoperative maximal vasoactive-inotropic scores (25.6 vs 12.4, P = .02), higher serum lactate levels (7.7 mmol/L vs 6.4 mmol/L, P = .03), higher proportions of ventilation throughout 24 h or more (16 patients [38%] vs 8 patients [14%], P = .009), higher proportions of ventricular dysfunction (12 patients [29%] vs 5 patients [9%], P = .0001), and lower blood oxygen saturation levels at discharge (87% vs 92%, P = .03). CONCLUSIONS: The Fontan procedure in patients with right ventricular morphology is associated with longer postoperative hospitalization and worse early postoperative characteristics (ventricular dysfunction and atrioventricular valve regurgitation) as well as higher rates of early, transient signs of sub-optimal postoperative hemodynamics compared to those with left ventricular morphology.

Evaluation of a Health Care Performance Improvement Initiative to Facilitate Optimal Clinical Outcomes in Patients Receiving Ventricular Assist Device Support.

BACKGROUND: Ventricular assist device (VAD) patients are at high risk for morbidities and mortality. One potentially beneficial component of the Joint Commission VAD Certification process is the requirement that individual VAD programs select 4 performance measures to improve and optimize patients' clinical outcomes. PROBLEM STATEMENT: Review of patient data after our program's first certification visit in 2008 showed that, compared to national recommendations and published reports, our patients had suboptimal outcomes in 4 areas after device implantation: length of hospital stay, receipt of early (<48 hours) postsurgical physical therapy, driveline infection incidence, and adequacy of nutritional status (prealbumin ≥18 mg/dL). METHODS: Plan-Do-Study-Act processes were implemented to shorten length of stay, increase patient receipt of early physical therapy, decrease driveline infection incidence, and improve nutritional status. With 2008 as our baseline, we deployed interventions for each outcome area across 2009 to 2017. Performance improvement activities included staff, patient, and family didactic, one-on-one, and hands-on education; procedural changes; and outcomes monitoring with feedback to staff on progress. Descriptive and inferential statistics were examined to document change in the outcomes. OUTCOMES: Across the performance improvement period, length of stay decreased from 40 to 23 days; physical therapy consults increased from 87% to 100% of patients; 1-year driveline infection incidence went from 38% to 23.5%; and the percentage of patients with prealbumin within the normal range increased from 84% to 90%. IMPLICATIONS: Performance improvement interventions may enhance ventricular assist device patient outcomes. Interventions' sustainability should be evaluated to ensure that gains are not lost over time.

An International Study on Ventricular Assist Device Program Models.

BACKGROUND: Advanced technology and improved outcomes have led to rapid growth of ventricular assist devices (VADs) throughout the world, but little exists regarding their structure. We sought to study trends in VAD programs on a global level. METHODS: We distributed a 26-question online survey to 321 individuals who work within those programs. Four categories of questions were formed: patient management, coordinator role, multidisciplinary support, and leadership. RESULTS: Fifty-eight surveys (47 United States, 11 international) were analyzed. The majority of programs cared for 26 to 100 device-assisted patients (62%), 26% cared for ≤25 patients, and 12% cared for ≥100 patients. Advanced practice providers (APPs) were used in 69% of programs as a device coordinator. In-hospital rounding was performed equally among the APPs and registered nurses. Most programs used a social worker (90%), nutritionist (74%), pharmacist (72%), palliative care (66%), and finance coordinator (64%). Less than half (43%) included a case manager and only 33% used a pharmacist. The program leader was identified as a cardiologist (31%) or surgeon (26%) or both equally (43%). CONCLUSION: This study demonstrates differences and similarities between VAD program structures. Additional research is warranted to evaluate the effect of program structure on outcomes, job satisfaction, and retention regions.

Endovascular repair of left ventricular assist device outflow graft defect.

Outflow graft complications after left ventricular assist device placement are infrequent but highly morbid. In this case report, we describe endovascular repair of multiple outflow graft defects with external hemorrhage in a complex patient using overlapping stent grafts. This approach successfully stopped the outflow graft hemorrhage and temporized the patient for subsequent cardiac transplantation.

Intraventricular Hemorrhage and Posthemorrhagic Ventricular Dilation: Current Approaches to Improve Outcomes.

Intraventricular hemorrhage (IVH) and posthemorrhagic ventricular dilation (PHVD) are important complications of prematurity with short- and long-term implications for the patient and for nursing care. Several approaches have been shown to reduce the incidence of IVH and, more recently, mitigate the impact of IVH on long-term neurodevelopment. This article discusses the pathophysiology of IVH, with a focus on prevention strategies. Posthemorrhagic ventricular dilation is a common complication of severe IVH and has implications for neurodevelopmental sequelae. Both surgical and nonsurgical interventions for PHVD are described.

Atrioventricular Valve Repair in Single Ventricle Physiology: Timing Matters.

OBJECTIVES: Atrioventricular valve (AVV) regurgitation in patients with single ventricle (SV) physiology severely impacts prognosis; the appropriate timing for surgical treatment is unknown. We sought to study the results of surgical treatment of AVV regurgitation in SV patients and evaluate risk factors for mortality. METHODS: Medical records of 81 consecutive patients with moderate or severe AAV regurgitation who were submitted to AVV repair or replacement during any stage of univentricular palliation between January 2013 and May 2017 were examined. We studied demographic data and perioperative factors looking for predictors that might have influenced the results. Binary logistic regression was used to assess the impact on postoperative ventricular dysfunction and mortality. RESULTS: Median age and weight were seven months (interquartile range [IQR]: 3-24) and 5.2 kg (IQR: 3.7-11.2), respectively. Seventy (86.4%) patients underwent AVV repair, and 11 (13.6%) patients underwent AVV replacement. There was an association between AVV repair effectiveness and timing of intervention (P = .004). Atrioventricular valve intervention at the time of initial surgical palliation was associated with more ineffective repairs (P = .001), while AVV replacement was more common between Glenn and Fontan procedures (P = .004). Overall 30-day mortality was 30.5% (25 patients). In-hospital mortality was 49.4%, and it was higher when AVV repair was performed concomitant with initial (stage 1) palliation (64.1% vs 35.7%; P = .01) and when an effective repair was not achieved (75% vs 41%; P = .008). Multivariable analysis identified timing concomitant with stage 1 palliation as an independent risk factor for mortality (P = .01); meanwhile, an effective repair was a protective factor against in-hospital mortality (P = .05). CONCLUSION: Univentricular physiology with AVV regurgitation is a high-risk group of patients. Surgery for AVV regurgitation at stage 1 palliation was associated with less effective repair and higher mortality in this initial experience. On the other hand, effective repair determined better outcomes, highlighting the importance of experience and the learning curve in the management of such patients.

Utility of High-Sensitivity and Conventional Troponin in Patients Undergoing Transcatheter Aortic Valve Replacement: Incremental Prognostic Value to B-type Natriuretic Peptide.

High-sensitivity Troponin (hs-Tn) has emerged as a useful marker for patients with myocardial injury or heart failure. However, few studies have compared intermediate and hs-Tn in patients undergoing transcatheter aortic valve replacement (TAVR). Moreover, there remains uncertainty of which thresholds are the most useful for discriminating ventricular dysfunction or outcome. In this study we prospectively enrolled 105 patients with severe aortic stenosis (AS) who underwent TAVR as well as blood sampling for high-sensitivity (hs-TnI) and conventional troponin I (EXL-LOCI and RXL) assessment. Patients underwent comprehensive pre-procedure echocardiography. Ventricular dysfunction was defined using left ventricular mass index (LVMI), LV global longitudinal strain (LVGLS) and LV end-diastolic pressure. The mean age was 84.0 ± 8.7 years old and 60% were male sex with mean transaortic pressure gradient of 50.1 ± 16.0 mmHg and AVA of 0.63 ± 0.19 cm2. When using a threshold of 6 ng/L, 77% had positive hs-TnI while 27% had positive hs-TnI using recommended thresholds (16 ng/L for female and 34 ng/L for male). Troponin levels were higher in the presence of abnormal LV phenotypes. The strongest correlate of troponin was LVMI. During median follow-up of 375 days, 21 patients (20%) died. Lower threshold of hs-TnI and EXL-TnI was more discriminatory for overall mortality (Log-rank P = 0.03 for both), while higher threshold of hs-TnI (p = 0.75) and RXL-TnI were not (p = 0.30). Combining hs-TnI and BNP improved to predict long-term outcome (p = 0.004). In conclusion, hs-TnI levels correlated with the degree of LV dysfunction phenotypes. Furthermore, applying a lower threshold for hs-TnI performed better for outcome prediction than a recommended threshold in patients undergoing TAVR. Combining hs-TnI with BNP helped better risk stratification.

Results of primary biventricular support: an analysis of data from the EUROMACS registry.

OBJECTIVES: The purpose of this study was to describe pre- and postoperative data from the EUROMACS registry with regard to indications, for and survival and complication rates of patients with primary continuous flow and pulsatile biventricular long-term assist devices (BiVADs) versus total artificial hearts (TAHs) or left ventricular assist devices (LVADs) + short-term right ventricular assist device (RVAD) implants. METHODS: We investigated patients who received implants between 1 January 2011 and 21 October 2017. Clinical baseline information about comorbidities, laboratory results, medical and device therapies and echocardiographic, haemodynamic and right ventricle (RV) parameters were evaluated along with the rates of deaths and complications. RESULTS: A total of 413 of 3282 patients (12.5%) needed a biventricular pump. We investigated 37 long-term BiVADs, 342 LVAD + short-term RVAD implants and 34 TAHs. Minor differences were found in the baseline characteristics of our population, which had an overall high morbidity profile. The 1-year survival rate was 55% for patients with a continuous flow BiVAD; 52% for patients with an LVAD + short-term RVAD; 37% for patients with pulsatile BiVADs; and 36% for patients with a TAH. No statistical difference was observed among the groups. Over 50% of patients with BiVAD support were classified as INTERMACS profiles 1 and 2. The percent of patients with ambulatory heart failure (INTERMACS 4‒7) undergoing BiVAD implants was modest at <15%. No patients with a pulsatile BiVAD (n = 15) or a TAH (n = 34) were implanted as destination therapy, but 27% of the patients with continuous flow BiVADs (n = 6) and 23% of the patients with LVAD + short-term RVAD (n = 342) were implanted as 'destination'. The adverse events profile remained high, with no significant difference among pump types. The right ventricular stroke work index and right heart failure scores indicated poor RV function in all groups. After 3 months of LVAD + short-term RVAD support, 46.7% still required ongoing support, and only 18.5% were weaned from RVAD support; 33.1% died. CONCLUSIONS: The mortality rate after BiVAD support was high. Survival rates and adverse events were statistically not different among the investigated groups. In the future, composite study end points examining quality of life and adverse events beyond survival may help in shared decision-making prior to general mechanical circulatory support, particularly in patients with BiVAD implants.

Valor pronóstico del índice de riesgo nutricional para los candidatos a implante de un dispositivo de asistencia ventricular izquierda de flujo continuo / Prognostic value of the nutritional risk index in candidates for continuous flow left ventricular assist device therapy

Introducción y objetivos: La desnutrición influye en la evolución clínica de los pacientes con insuficiencia cardiaca. El objetivo es analizar el impacto del estado nutricional preoperatorio evaluado mediante el índice de riesgo nutricional (IRN) en el pronóstico de los pacientes que recibieron dispositivos de asistencia ventricular izquierda de flujo continuo (DAVI-fc). Métodos: Estudio retrospectivo de 279 pacientes tratados con implante de DAVI-fc entre 2009 y 2015 en el centro. Se calculó el IRN preoperatorio y se realizó un seguimiento del primer año tras el implante. Se analizó mediante regresión la asociación entre el IRN preoperatorio y los eventos clínicos posoperatorios. Resultados: Las prevalencias de riesgo nutricional grave (IRN<83,5), moderado (83,5 ≤ IRN<97,5) y leve (97,5 ≤ IRN <100) fueron del 5,4, el 21,5 y el 9,3%. Las tasas de mortalidad a 1 año después del implante en estas 3 categorías fueron del 53,3, el 31,7 y el 23,1%, frente al 18,0% (p <0,001) de los pacientes con IRN normal. Un IRN preoperatorio normal se identificó como predictor independiente de riesgo de muerte por cualquier causa durante el seguimiento (HRa por unidad=0,961; IC95%, 0,941-0,981; p <0,001) y predictor de menor riesgo de infección (ORa=0,968; IC95%, 0,946-0,991; p=0,007), insuficiencia respiratoria (ORa=0,961; IC95%, 0,936-0,987; p=0,004) e insuficiencia cardiaca derecha (ORa=0,963; IC95%, 0,934-0,992; p=0,014) posoperatorias. Conclusiones: Los pacientes desnutridos tienen mayor riesgo de complicaciones posoperatorias y muerte después del implante de un DAVI-fc. La evaluación del riesgo nutricional puede contribuir a mejorar la selección de pacientes e iniciar un apoyo nutricional precoz

Introduction and objectives: Malnutrition has been shown to affect clinical outcomes in patients with heart failure. The aim of this study was to analyze the impact of preoperative nutritional status assessed by the nutritional risk index (NRI) on the prognosis of patients with a continuous-flow left ventricular assist device (cf-LVAD). Methods: We performed a retrospective study of 279 patients who underwent cf-LVAD implantation between 2009 and 2015 in our center. Preoperative NRI was calculated and the patients were followed-up for 1 year. The association between preoperative NRI and postoperative clinical events was analyzed using multivariable logistic regression. Results: The prevalence of severe (NRI <83.5), moderate (83.5 ≤ NRI <97.5) and mild (97.5 ≤ NRI <100) nutritional risk was 5.4%, 21.5%, and 9.3%. Mortality rates 1 year after cf-LVAD implantation in these 3 categories were 53.3%, 31.7%, 23.1% vs 18.0% (P <.001) in patients with a normal IRN. A normal preoperative NRI value was an independent predictor of lower risk of death from any cause during follow-up (aHR per 1 unit, 0.961; 95%CI, 0.941-0.981; P <.001) was and a predictor for a lower risk of postoperative infections (aOR, 0.968; 95%CI, 0.946-0.991; P=.007), respiratory failure (aOR, 0,961; 95%CI, 0.936-0.987; P=.004), and right heart failure (aOR, 0.963; 95%CI, 0.934-0.992; P=.014). Conclusions: Malnourished patients are at increased risk for postoperative complications and death after cf-LVAD implantation. Assessment of nutritional risk could improve patient selection and the early initiation of nutritional support

Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease.

Outcomes for patients with single ventricle congenital heart disease (SV-CHD) continue to improve over time. However, the prognosis for patients who develop heart failure immediately after surgery is poorly understood. We conducted a single-center, retrospective cohort study of patients with SV-CHD, who suffered postoperative heart failure. Of 1038 cardiac surgeries performed on 621 SV-CHD patients between 2004 and 2010, 125 patients met inclusion criteria, including non-septatable anatomy, stage 1 surgery, and verified low cardiac output or heart failure state per STS definition. Overall survival was 73.2% at 2 months, 64.9% at 1 year, 60.5% at 2 years, and 54.6% at 4 years. Inotrope dependence beyond 7 days post-op yielded 45% 2-year survival versus 68% for those who weaned from inotropes within 7 days (p = 0.02). Atrioventricular valve regurgitation (AVVR) influenced survival, and patients who developed renal failure or required ECMO fared poorly, even when they survived their hospitalization. Patients with postoperative heart failure and low cardiac output syndrome constitute a high-risk population beyond the term of the initial hospitalization and have an overall mid-term survival of 55% at 4 years. Wean from inotropic therapy is not completely reassuring in this population, as they have ongoing elevated risk of cardiac failure and death in the medium term. Ventricular dysfunction, AVVR, renal failure, and need for ECMO are all important prognostic factors for mid-term mortality. Inotrope dependence for > 7 days has important implications reaching beyond the hospitalization.

Survival to Stage II with Ventricular Dysfunction: Secondary Analysis of the Single Ventricle Reconstruction Trial.

Ventricular dysfunction affects survival in patients with single right ventricle (RV), and remains one of the primary indications for heart transplantation. Since it is challenging to predict the capacity of patients with ventricular dysfunction to proceed to the stage II procedure, we sought to identify factors that would be associated with death or heart transplantation without achieving stage II for single RV patients with ventricular dysfunction after Norwood procedure. The Single Ventricle Reconstruction (SVR) trial public-use database was used. Patients with a RV ejection fraction less than 44% or a RV fractional area of change less than 35% on the post-Norwood echocardiogram were included. Parametric risk hazard analysis was used to identify risk factors for death or transplantation without achieving stage II. Of 365 patients with ventricular function measurements on the post-Norwood echocardiogram, 123 (34%) patients had RV dysfunction. The transplantation-free survival was significantly lower for those with ventricular dysfunction compared to those with normal function (log rank Chi-square = 4.23, p = 0.04). Furthermore, having a Blalock-Taussig (BT) shunt, a large RV, a post-Norwood infectious complication, and a surgeon who performs five or less Norwood per year were independent risk factors for death or transplantation without achieving stage II. The predicted 6-month transplantation-free survival for patients with all four identified risk factors was 1% (70% CI 0-13%). Early heart transplantation referral might be considered for post-Norwood patients with BT shunt and RV dysfunction, especially if other high-risk features are present.

Multimodality Noninvasive Imaging in the Monitoring of Pediatric Heart Transplantation.

Orthotopic heart transplantation is a well-established and effective therapeutic option for children with end-stage heart failure. Multiple modalities, including noninvasive cardiac imaging, cardiac catheterization, angiography, and endomyocardial biopsy, are helpful to monitor these patients for graft dysfunction, rejection, and vasculopathy. Because of morbidities associated with invasive monitoring, noninvasive imaging plays a key role in the surveillance and evaluation of symptoms in pediatric transplant recipients. Echocardiography with or without stress augmentation may provide serial data on systolic and diastolic function, ventricular deformation, and tissue characteristics in children after transplantation. Although not perfectly sensitive or specific, advanced two- and three-dimensional echocardiographic detection of functional changes in cardiac grafts may allow early recognition of allograft rejection. Magnetic resonance imaging has shown promise for characterization of edema and scar and myocardial perfusion reserve, as well as potential application for the detection of microvasculopathic changes in the transplanted heart. Cardiac computed tomography is particularly well suited for the demonstration of coronary artery dimensions and anatomic residual lesions. In combination, these noninvasive imaging techniques help the transplantation cardiologist screen for graft dysfunction, detect critical graft events, and identify situations that require invasive testing of the transplanted heart. Advanced multimodality imaging techniques are likely to increasingly shape the monitoring practices for children following heart transplantation.

Fluorescein-Guided Neuroendoscopy for Intraventricular Lesions: A Case Series.

BACKGROUND: The benefits of neuroendoscopy in the pathological diagnosis of intra- and paraventricular tumors have already been shown in many neurosurgical studies. However, most authors agree that neuroendoscopic biopsies are not infrequently inconclusive due to small or inadequate samples, prompting the need for new diagnostic strategies. OBJECTIVE: To describe a technique not previously reported in the literature, combining neuroendoscopy with angiofluorescein guidance for the pathological diagnosis of intra- and paraventricular tumors. METHODS: The 4-mm steerable fiberscope used was equipped with dual observation modes for white light and fluorescein. Access was by the classical precoronal burr hole. After inspecting the ventricular system in white light, a 10-mg/kg dose of fluorescein sodium (FS) was administered intravenously to the patient. The endoscope was then switched to the blue light fluorescent mode to better localize the pathological tissue. The protocol had been submitted to the local ethics committee. RESULTS: From September 2011 to March 2015, 9 consecutive patients (aged 1-56 yr) harboring intra- and paraventricular lesions prospectively underwent angiofluorescein-guided endoscopy. In all cases, a pathological diagnosis was obtained without complications. In 5 patients, an endoscopic third ventriculostomy, and, in 1 patient, a septostomy was performed during the same procedure. Fluorescein guidance definitely modified our site of biopsy in 4 cases. CONCLUSION: In our experience, FS has proven to be a strong enhancer of all ventricular lesions presenting with a disrupted blood-brain barrier, including inflammatory processes. Fluorescein-guided neuroendoscopy appears to be a safe, economic method to improve diagnostic potential in ventricular lesions.

Experiencia con una asistencia ventricular pulsátil de larga duración como puente al trasplante cardiaco en adultos / Experience with a long-term pulsatile ventricular assist device as a bridge to heart transplant in adults

Introducción y objetivos: La mayoría de los dispositivos de asistencia ventricular (DAV) de larga duración utilizados actualmente son intracorpóreos y de flujo continuo. Sus principales inconvenientes son el coste elevado y la imposibilidad de ofrecer soporte biventricular. El objetivo de este estudio es describir los resultados de una estrategia de implante de un DAV paracorpóreo de flujo pulsátil como puente al trasplante (PAT) en pacientes adultos. Métodos: Análisis retrospectivo de las características, complicaciones y resultados de una serie unicéntrica de pacientes consecutivos tratados con el DAV EXCOR como PAT entre 2009 y 2015. Resultados: En este periodo se implantaron 25 DAV, 6 de ellos biventriculares. En 12 pacientes la indicación fue directamente PAT y en 13 puente a la decisión debido a la presencia de contraindicaciones potencialmente reversibles o posibilidad de recuperación. Veinte pacientes (80%) alcanzaron el objetivo del trasplante cardiaco tras una mediana de soporte de 112 días (rango 8-239). Las principales complicaciones fueron: infecciosas (52% de los pacientes), neurológicas (32%, la mitad de ellas mortales), hemorrágicas (28%) y fallo del DAV que obligó a cambiar algún componente de este (28%). Conclusiones: El 80% de los pacientes tratados con el DAV EXCOR como PAT alcanzaron el objetivo tras una mediana de soporte de casi 4 meses. Las complicaciones más frecuentes fueron las infecciosas y las más graves las neurológicas. El empleo de estos DAV de flujo pulsátil como PAT, en nuestro medio, es una estrategia factible que consigue resultados similares a los DAV de flujo continuo (AU)

Introduction and objectives: Most long-term ventricular assist devices (VADs) that are currently implanted are intracorporeal continuous-flow devices. Their main limitations include their high cost and inability to provide biventricular support. The aim of this study was to describe the results of using paracorporeal pulsatile-flow VADs as a bridge to transplant (BTT) in adult patients. Methods: Retrospective analysis of the characteristics, complications, and outcomes of a single-center case series of consecutive patients treated with the EXCOR VAD as BTT between 2009 and 2015. Results: During the study period, 25 VADs were implanted, 6 of them biventricular. Ventricular assist devices were indicated directly as a BTT in 12 patients and as a bridge to decision in 13 due to the presence of potentially reversible contraindications or chance of heart function recovery. Twenty patients (80%) were successfully bridged to heart transplant after a median of 112 days (range, 8-239). The main complications included infectious (52% of patients), neurological events (32%, half of them fatal), bleeding (28%), and VAD malfunction requiring component replacement (28%). Conclusions: Eighty percent of patients with the EXCOR VAD as BTT achieved the goal after an average of almost 4 months of support. The most frequent complications were infectious, and the most severe were neurological. In our enivonment, the use of these pulsatile-flow VAD as BTT is a feasible strategy that obtains similar outcomes to those of intracorporeal continuous-flow devices (AU)

Psychiatric Disorders in Adolescents With Single Ventricle Congenital Heart Disease.

BACKGROUND AND OBJECTIVES: Mental health outcomes for survivors of critical congenital heart disease (CHD) remain under-investigated. We sought to examine psychiatric disorders and psychosocial functioning in adolescents with single ventricle CHD and to explore whether patient-related risk factors predict dysfunction. METHODS: This cohort study recruited 156 adolescents with single ventricle CHD who underwent the Fontan procedure and 111 healthy referents. Participants underwent comprehensive psychiatric evaluation including a clinician-rated psychiatric interview and parent- and self-report ratings of anxiety, disruptive behavior, including attention-deficit/hyperactivity disorder (ADHD), and depressive symptoms. Risk factors for dysfunction included IQ, medical characteristics, and concurrent brain abnormalities. RESULTS: Adolescents with single ventricle CHD had higher rates of lifetime psychiatric diagnosis compared with referents (CHD: 65%, referent: 22%; P < .001). Specifically, they had higher rates of lifetime anxiety disorder and ADHD (P < .001 each). The CHD group scored lower on the primary psychosocial functioning measure, the Children's Global Assessment Scale, than referents (CHD median [interquartile range]: 62 [54-66], referent: 85 [73-90]; P < .001). The CHD group scored worse on measures of anxiety, disruptive behavior, and depressive symptoms. Genetic comorbidity did not impact most psychiatric outcomes. Risk factors for anxiety disorder, ADHD, and lower psychosocial functioning included lower birth weight, longer duration of deep hypothermic circulatory arrest, lower intellectual functioning, and male gender. CONCLUSIONS: Adolescents with single ventricle CHD display a high risk of psychiatric morbidity, particularly anxiety disorders and ADHD. Early identification of psychiatric symptoms is critical to the management of patients with CHD.