Submaximal Exercise Response is Associated with Future Hypertension in Patients with Coarctation of the Aorta.

Hypertension (HTN) is common in patients with a history of coarctation of the aorta (CoA) and remains underrecognized and undertreated. Studies in the non-coarctation otherwise healthy adult population have correlated an exaggerated blood pressure response during mild to moderate exercise with subsequent diagnosis of HTN. The goal of this study was to determine if blood pressure response to submaximal exercise in normotensive CoA patients correlated with development of HTN.Retrospective chart review was performed in individuals ≥ 13 years old with CoA and no diagnosis of HTN at time of cardiopulmonary exercise testing (CPET). Systolic blood pressure (SBP) during CPET at rest, submax 1 (stage 1 Bruce or minute 2 bicycle ramp), submax 2 (stage 2 Bruce or minute 4 bicycle ramp), and peak were recorded. The primary composite outcome was HTN diagnosis or initiation of anti-hypertensive medications at follow up.There were 177 patients (53% female, median age 18.5 years), of whom 38 patients (21%) met composite outcome during a median follow up of 46 months. Men were more likely to develop hypertension. Age at repair and age at CPET were not significant covariates. At each stage of CPET, SBP was significantly higher in those who met the composite outcome. Submax 2 SBP ≥ 145 mmHg was 75% sensitive, 71% specific in males and 67% sensitive, 76% specific in females for development of composite outcome.Our study shows an exaggerated SBP response to submaximal exercise may portend an increased risk of developing hypertension during short- to mid-term follow up.

Hepatic abnormalities in youth with Turner syndrome.

BACKGROUND & AIMS: Liver disease in children with Turner Syndrome (TS) is poorly understood relative to associated growth, cardiac and reproductive complications. This study sought to better characterize hepatic abnormalities in a large national cohort of youth with TS. METHODS: Using electronic health record data from PEDSnet institutions, 2145 females with TS were matched to 8580 females without TS on eight demographic variables. Outcomes included liver enzymes (AST and ALT) stratified as normal, 1-2 times above the upper limit of normal (ULN), 2-3 times ULN and >3 times ULN, as well as specific liver disease diagnoses. RESULTS: Fifty-eight percent of youth with TS had elevated liver enzymes. Patients with TS had higher odds of enzymes 1-2 times ULN (OR: 1.7, 95% CI: 1.4-1.9), 2-3 times ULN (OR: 2.7, 95% CI: 1.7-3.3) and >3 times ULN (OR: 1.7, 95% CI: 1.3-2.2). They also had higher odds of any liver diagnosis (OR: 2.4, 95% CI: 1.7-3.3), fatty liver disease (OR: 1.9, 95% CI: 1.1-3.2), hepatitis (OR: 3.7, 95% CI: 1.9-7.1), cirrhosis/fibrosis (OR: 5.8, 95% CI: 1.3-25.0) and liver tumour/malignancy (OR: 4.8, 95% CI: 1.4-17.0). In a multinomial model, age, BMI and presence of cardiovascular disease or diabetes significantly increased the odds of elevated liver enzymes in girls with TS. CONCLUSIONS: Youth with TS have higher odds for elevated liver enzymes and clinically significant liver disease compared with matched controls. These results emphasize the need for clinical screening and additional research into the aetiology and treatment of liver disease in TS. LAY SUMMARY: Turner Syndrome, a chromosomal condition in which females are missing the second sex chromosome, is often associated with short stature, infertility and cardiac complications. Liver abnormalities are less well described in the literature. In this study, nearly 60% of youth with TS have elevated liver enzymes. Furthermore, patients with TS had a diagnosis of liver disease more often than patients without TS. Our results support the importance of early and consistent liver function screening and of additional research to define mechanisms that disrupt liver function in paediatric TS females.

Individuals aged 1-64 years with documented congenital heart defects at healthcare encounters, five U.S. surveillance sites, 2011-2013.

BACKGROUND: Many individuals born with congenital heart defects (CHD) survive to adulthood. However, population estimates of CHD beyond early childhood are limited in the U.S. OBJECTIVES: To estimate the percentage of individuals aged 1-to-64 years at five U.S. sites with CHD documented at a healthcare encounter during a three-year period and describe their characteristics. METHODS: Sites conducted population-based surveillance of CHD among 1 to 10-year-olds (three sites) and 11 to 64-year-olds (all five sites) by linking healthcare data. Eligible cases resided in the population catchment areas and had one or more healthcare encounters during the surveillance period (January 1, 2011-December 31, 2013) with a CHD-related ICD-9-CM code. Site-specific population census estimates from the same age groups and time period were used to assess percentage of individuals in the catchment area with a CHD-related ICD-9-CM code documented at a healthcare encounter (hereafter referred to as CHD cases). Severe and non-severe CHD were based on an established mutually exclusive anatomic hierarchy. RESULTS: Among 42,646 CHD cases, 23.7% had severe CHD and 51.5% were male. Percentage of CHD cases among 1 to 10-year-olds, was 6.36/1,000 (range: 4.33-9.96/1,000) but varied by CHD severity [severe: 1.56/1,000 (range: 1.04-2.64/1,000); non-severe: 4.80/1,000 (range: 3.28-7.32/1,000)]. Percentage of cases across all sites in 11 to 64-year-olds was 1.47/1,000 (range: 1.02-2.18/1,000) and varied by CHD severity [severe: 0.34/1,000 (range: 0.26-0.49/1,000); non-severe: 1.13/1,000 (range: 0.76-1.69/1,000)]. Percentage of CHD cases decreased with age until 20 to 44 years and, for non-severe CHD only, increased slightly for ages 45 to 64 years. CONCLUSION: CHD cases varied by site, CHD severity, and age. These findings will inform planning for the needs of this growing population.

Outcomes in hospitalisations of women with Turner syndrome compared to women without Turner syndrome.

OBJECTIVE: To evaluate outcomes in patients with Turner Syndrome, especially those with cardiac conditions, compared to those without Turner syndrome. DESIGN: Retrospective cohort study utilising hospitalisation data from 2006 to 2012. Conditional logistic regression models are used to analyse outcomes of interest: all-cause mortality, increased length of stay, and discharge to home. PARTICIPANTS: We identified 2978 women with Turner syndrome, matched to 11,912 controls by primary diagnosis. RESULTS: Patients with Turner syndrome were more likely to experience inpatient mortality (odds ratio 1.44, 95% confidence interval 1.02-2.02, p = 0.04) and increased length of stay (OR 1.31, CI 1.18-1.46, p = 0.03) than primary diagnosis matched controls, after adjusting for age, race, insurance status, and Charlson comorbidity index. Patients with Turner syndrome were 32% less likely to be discharged to home (OR 0.68, CI 0.60-0.78, p < 0.001). When restricting the sample of patients to those admitted with a cardiac diagnosis, the likelihood of mortality (OR 3.10, CI 1.27-7.57, p = 0.01) and prolonged length of stay (OR 1.42, CI 1.03-1.95, p = 0.03) further increased, while the likelihood of discharge to home further decreased (OR 0.55, CI 0.38-0.80, p = 0.001) in Turner syndrome compared to primary diagnosis matched controls. Specifically, patients with congenital heart disease were more likely to have prolonged length of stay (OR: 1.53, CI 1.18-2.00, p = 0.002), but not increased mortality or decreased discharge to home. CONCLUSIONS: Hospitalised women with Turner syndrome carry a higher risk of adverse outcomes even when presenting otherwise similarly as controls, an important consideration for those treating them in these settings.

Estimating the prevalence of congenital heart disease among adolescents and adults in Colorado adjusted for incomplete case ascertainment.

BACKGROUND: Congenital heart defects (CHDs), the most common type of birth defect in the United States, are increasing in prevalence in the general population. Though CHD prevalence at birth has been well described in the United States at about 1%, little is known about long-term survival and prevalence of CHDs beyond childhood. This study aimed to estimate the prevalence of CHDs among adolescents and adults in Colorado. METHODS: The prevalence of CHDs among adolescents and adults residing in Colorado during 2011 to 2013 was estimated using log-linear capture-recapture methods to account for incomplete case ascertainment. Five case-finding data sources were used for this analysis including electronic health record data from 4 major health systems and a state-legislated all payer claims database. RESULTS: Twelve thousand two hundred ninety-three unique individuals with CHDs (2481 adolescents and 9812 adults) were identified in one or more primary data sources. We estimated the crude prevalence of CHDs in adolescents and adults in Colorado to be 3.22 per 1000 individuals (95% CI 3.19-3.53). After accounting for incomplete case ascertainment, the final capture-recapture model yielded an estimated total adolescent and adult CHD population of 23,194 (95% CI 22,419-23,565) and an adjusted prevalence of 6.07 per 1000 individuals (95% CI 5.86-6.16), indicating 47% of the cases in the catchment area were not identified in the case-identifying data sources. CONCLUSION: This statewide study yielded new information on the prevalence of CHDs in adolescents and adults. These high prevalence rates underscore the need for additional specialized care facilities for this population with CHDs.

Interatrial pressure gradients during simulated obstructive sleep apnea: a catheter-based study.

OBJECTIVES: We set to measure the interatrial pressure gradient during simulated obstructive sleep apnea (OSA). BACKGROUND: OSA occurs when a sleeping patient attempts to inhale against an obstructed airway. How this event affects the interatrial pressure gradient has not been defined. We hypothesized that simulated OSA in a conscious subject (Mueller maneuver [MM], inspiration against obstruction) would promote increased right-to-left pressure gradient, and then the substrate for right-to-left atrial shunting. METHODS: Selected patients underwent simultaneous measurement of airway and atrial pressures (both left and right atrium [LA, RA]) using high-fidelity micromanometry at rest, during MM, and during VM, during right heart catheterization. RESULTS: Ten patients (age 55 ± 11 years, two women) were successfully studied. During the onset of MM, RA pressure transiently but consistently exceeded LA pressure in response to the steep decline in intrathoracic pressure (maximum RA-LA pressure gradient increased from 0.1 ± 1.4 mm Hg at baseline to 7.0 ± 4.3 mm Hg during MM, P < 0.001). The maximum right-to-left atrial pressure gradient during Mueller maneuver was higher than that achieved during the Valsalva maneuver release (P < 0.007). CONCLUSIONS: The onset of MM increased right-to-left pressure gradient across the atrial septum, likely as a result of greater blood return to the RA from extrathoracic veins. The RA-LA pressure gradient achieved during MM was greater than that observed during VM. These findings delineate the hemodynamic substrate for right to left shunting during OSA.

Preoperative factors associated with adverse outcome after tricuspid valve replacement.

BACKGROUND: Preoperative factors associated with increased mortality and worse outcome after tricuspid valve replacement in patients with severe tricuspid regurgitation are poorly understood. METHODS AND RESULTS: We retrospectively analyzed 189 patients (37% men; age, 67.5±11.3 years) who underwent tricuspid valve replacement for severe tricuspid regurgitation. Operative mortality rate was 10%, and was associated with intra-aortic balloon pump (odds ratio, 3.2; 95% confidence interval, 1.9 to 5.6; P<0.0001) or the presence of severe symptoms (New York Heart Association class IV relative to classes II and/or III) at the time of surgery (1.7; 95% confidence interval, 1.05 to 2.8; P=0.02). At the end of follow-up (29.3±27.1 months), 70 patients (37%) died, 6 (3%) needed tricuspid reoperation, and 41 (21.7%) were readmitted for heart failure. Seventy-eight patients (41.3%) were free from cardiovascular events (death, tricuspid reoperation, or heart failure admissions). The only echocardiographic parameter independently associated with adverse outcomes was a decrease in the right index of myocardial performance ratio. All-cause mortality was independently associated with a higher Charlson index (hazard ratio, 1.18; 95% confidence interval, 1.01 to 1.36; P=0.03), shorter right index of myocardial performance ratio (0.91; 95% confidence interval, 0.87 to 0.96; P=0.005), and preoperative New York Heart Association IV class (1.71; 95% confidence interval, 1.3 to 2.2; P<0.0001). In 68 patients with isolated tricuspid valve replacement, the associations between short right index of myocardial performance ratio, high Charlson index, New York Heart Association class IV, and increased mortality remained significant. CONCLUSIONS: Tricuspid valve replacement for severe tricuspid regurgitation can be performed with an acceptable operative mortality if patients undergo surgery before the onset of advanced heart failure symptoms. Late mortality is associated with a high preoperative Charlson index, short right index of myocardial performance ratio, and advanced New York Heart Association class.

Post-traumatic stress disorder, anxiety, and depression among adults with congenital heart defects.

BACKGROUND: Due to invasive treatments and stressors related to heart health, adults with congenital heart defects (CHDs) may have an increased risk of post-traumatic stress disorder (PTSD), anxiety, and/or depressive disorders. Our objectives were to estimate the prevalence of these disorders among individuals with CHDs. METHODS: Using IBM® MarketScan® Databases, we identified adults age 18-49 years with ≥2 outpatient anxiety/depressive disorder claims on separate dates or ≥1 inpatient anxiety/depressive disorder claim in 2017. CHDs were defined as ≥2 outpatient CHD claims ≥30 days apart or ≥1 inpatient CHD claim documented in 2007-2017. We used log-binomial regression to estimate adjusted prevalence ratios (aPR) and 95% confidence intervals (CI) for associations between CHDs and anxiety/depressive disorders. RESULTS: Of 13,807 adults with CHDs, 12.4% were diagnosed with an anxiety or depressive disorder. Adults with CHDs, compared to the 5,408,094 without CHDs, had higher prevalence of PTSD (0.8% vs. 0.5%; aPR: 1.5 [CI: 1.2-1.8]), anxiety disorders (9.9% vs. 7.5%; aPR: 1.3 [CI: 1.3-1.4]), and depressive disorders (6.3% vs. 4.9%; aPR: 1.3 [CI: 1.2-1.4]). Among individuals with CHDs, female sex (aPR range: 1.6-3.3) and inpatient admission (aPR range 1.1-1.9) were associated with anxiety/depressive disorders. CONCLUSION: Over 1 in 8 adults with CHDs had diagnosed PTSD and/or other anxiety/depressive disorders, 30-50% higher than adults without CHDs. PTSD was rare, but three times more common in women with CHDs than men. Screening and referral for services for these conditions in people with CHDs may be beneficial.

Incidence of aortic complications in patients with bicuspid aortic valves.

CONTEXT: Bicuspid aortic valve (BAV), the most common congenital heart defect, has been thought to cause frequent and severe aortic complications; however, long-term, population-based data are lacking. OBJECTIVE: To determine the incidence of aortic complications in patients with BAV in a community cohort and in the general population. DESIGN, SETTING, AND PARTICIPANTS: In this retrospective cohort study, we conducted comprehensive assessment of aortic complications of patients with BAV living in a population-based setting in Olmsted County, Minnesota. We analyzed long-term follow-up of a cohort of all Olmsted County residents diagnosed with definite BAV by echocardiography from 1980 to 1999 and searched for aortic complications of patients whose bicuspid valves had gone undiagnosed. The last year of follow-up was 2008-2009. MAIN OUTCOME MEASURE: Thoracic aortic dissection, ascending aortic aneurysm, and aortic surgery. RESULTS: The cohort included 416 consecutive patients with definite BAV diagnosed by echocardiography, mean (SD) follow-up of 16 (7) years (6530 patient-years). Aortic dissection occurred in 2 of 416 patients; incidence of 3.1 (95% CI, 0.5-9.5) cases per 10,000 patient-years, age-adjusted relative-risk 8.4 (95% CI, 2.1-33.5; P = .003) compared with the county's general population. Aortic dissection incidences for patients 50 years or older at baseline and bearers of aortic aneurysms at baseline were 17.4 (95% CI, 2.9-53.6) and 44.9 (95% CI, 7.5-138.5) cases per 10,000 patient-years, respectively. Comprehensive search for aortic dissections in undiagnosed bicuspid valves revealed 2 additional patients, allowing estimation of aortic dissection incidence in bicuspid valve patients irrespective of diagnosis status (1.5; 95% CI, 0.4-3.8 cases per 10,000 patient-years), which was similar to the diagnosed cohort. Of 384 patients without baseline aneurysms, 49 developed aneurysms at follow-up, incidence of 84.9 (95% CI, 63.3-110.9) cases per 10,000 patient-years and an age-adjusted relative risk 86.2 (95% CI, 65.1-114; P <.001 compared with the general population). The 25-year rate of aortic surgery was 25% (95% CI, 17.2%-32.8%). CONCLUSIONS: In the population of patients with BAV, the incidence of aortic dissection over a mean of 16 years of follow-up was low but significantly higher than in the general population.

Patients with bicuspid aortic valves may be associated with infra-hisian conduction disease requiring pacemakers.

BACKGROUND: Bicuspid aortic valves (BAVs) are associated with accelerated valvular dysfunction. Increasing rates of conduction system disease are seen in patients with calcific tricuspid aortic valves (TAVs). However, little is known regarding the extent of conduction disorders in BAV patients. We sought to determine the extent of infra-hisian conduction pathology among patients with BAVs undergoing EP studies. METHODS: We prospectively analyzed patients presenting to the EP laboratory from 2006 to 2017 at our institution. Thirty-three BAV patients had measured HV intervals. Each individual was matched by age and gender to two control patients. Clinical characteristics were collected and compared, and patients followed for outcomes. RESULTS: The BAV cohort had a mean age of 47.8 ± 17.2 years (range 19-76 years). Indications for referral to the EP lab in the BAV cohort included SVT ablation (n = 16), VT ablation (n = 10), and EP study for syncope, pre-syncope, or palpitations (n = 29). Patients with BAVs had a mean HV interval of 58.7 ms ± 18.6 ms, compared to a mean of 47.2 ms ± 9.6 ms for controls (p value = 0.0001). Over a 10-year follow-up period, 9 BAV patients (27%) went on to require permanent pacing compared to 6 patients (9%) in the control group (p value = 0.03). CONCLUSION: Compared to patients with TAVs presenting for EP evaluation, individuals with BAVs have longer HV intervals and a significantly increased requirement for pacemaker therapy over long-term follow-up. Closer monitoring of progressive conduction system disease in BAV patients may be warranted.

Prevalence of Mental Illness in Adolescents and Adults With Congenital Heart Disease from the Colorado Congenital Heart Defect Surveillance System.

The aim of this study was to estimate the prevalence of the full spectrum of mental illness in adolescents (aged 11 to 17) and adults (aged 18 to 64) with congenital heart defects (CHDs) in the population-level Colorado Congenital Heart Disease Surveillance System. Further we sought to investigate whether severity of the defect, frequency of recent cardiac procedures or underlying genetic disorders influence these estimates. The cohort included patients in clinical care for CHDs between January 1, 2011 and December 31, 2013, identified across multiple healthcare systems and insurance claims. Of 2,192 adolescents with CHDs, 20% were diagnosed with a mental illness with the most prevalent categories being developmental disorders (8%), anxiety disorders (6%), attention, conduct, behavior, impulse control disorders (6%), and mood disorders (5%). Of 6,924 adults with CHDs, 33% were diagnosed with a mental illness with the most prevalent categories being mood disorders (13%), anxiety disorders (13%), and substance-related disorders (6%). Greater lesion complexity was associated with a higher likelihood of anxiety and developmental disorders in both adolescents and adults. Adolescents and adults who had ≥2 cardiac procedures in the 3-year surveillance period had a 3- and 4.5-fold higher likelihood of a mental illness diagnosis, respectively, compared with those who had fewer than 2 cardiac procedures. Finally, patients with a genetic syndrome were more likely to have a mental illness diagnosis. In conclusion, mental illness is a prevalent co-morbidity in the adolescent and adult population with CHDs, thus comprehensive care should include mental health care.

An Approach to Endovascular Ventricular Pacing in a Patient with Ebstein Anomaly and a Mechanical Tricuspid Valve.

In the presence of a mechanical tricuspid valve, endocardial right ventricular pacing is contraindicated, and permanent pacing is usually achieved via a surgically implanted epicardial lead. In a patient with Ebstein anomaly, a mechanical tricuspid valve, and complete heart block, transvenous pacing was achieved by implantation of a pace-sense lead in a coronary sinus ventricular branch. Noninvasive cardiac imaging can provide information regarding anatomic variation in patients with congenital heart disease or when there are challenges to lead placement. With careful planning and execution, endovascular pacing in patients with a mechanical tricuspid valve is feasible and can safely be performed.

Benchmark Outcomes for Pulmonary Valve Replacement Using The Society of Thoracic Surgeons Databases.

BACKGROUND: As less invasive alternatives to surgical pulmonary valve replacement (PVR) are being refined and evaluated, there is a need for benchmark data concerning outcomes from surgical PVR. METHODS: We examined in-hospital outcomes from surgical PVR in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and Adult Cardiac Surgery Database (STS-ACSD) between 2007 and 2013, with a focus on patients likely to be eligible for transcatheter PVR (ie, ≥ 5 years age and ≥ 30 kg). Patient characteristics, morbidity, and mortality were described. RESULTS: The STS-CHSD included 6,431 eligible patients with a median age of 17 years (interquartile range [IQR], 14-25 years). Preoperative comorbidities were uncommon: arrhythmia (1.7%), renal failure (0.1%), endocarditis (0.3%), neurologic deficit (0.8%), and diabetes (0.5%). In-hospital mortality was 0.9%. A major complication occurred in 2.2%. The STS-ACSD included 3,352 eligible patients; the median age was 41 years (IQR, 30-55 years). Preoperative comorbidities were more common: arrhythmia (24.3%), renal failure (3.8%), endocarditis (12.2%), cerebrovascular disease (7.9%), and diabetes (10.9%). In-hospital mortality was 4.1%. A major complication occurred in 20.9%. CONCLUSIONS: Contemporary outcomes from surgical PVR include a low risk of in-hospital death or major complications. Patients in the STS-ACSD are older and have an increased prevalence of preoperative factors, which may contribute to higher morbidity and mortality.

Cardiopulmonary exercise test in patients with subacute pulmonary emboli.

OBJECTIVE: Patients presenting with suspected pulmonary embolism (PE) may present a challenge, particularly if diagnostic testing is not immediately available or clinically not indicated (iodine allergy, pregnancy, renal dysfunction). These patients have abnormal regional gas exchange that can be recognized by a cardiopulmonary exercise test (CPET), which may become helpful in their evaluation. METHODS: A retrospective analysis was performed of outpatients evaluated for subacute exertional dyspnea of 2 to 12 weeks duration with a test for PE and CPET. A total of 108 patients met inclusion criteria. Thirty patients (27.8%) had confirmed PE. RESULTS: The patients with PE had increased nadir ventilatory equivalent ratio for carbon dioxide (VE/VCO(2)), decreased peak oxygen uptake/predicted, and decreased end exercise saturation (P < .005 for all). All patients but 1 had normal breathing reserve (>15%). A normal nadir VE/VCO(2) excluded PE with 100% sensitivity. By using a "flow chart strategy," the exercise test had 92.8% sensitivity and 92.1% specificity for PE. Eight patients with PE died during follow-up (3.8 ± 4.6 years), 6 of PE-related causes. Peak VO(2)/kg was the best predictor of all-cause mortality and nadir VE/VCO(2) for PE-related mortality. There were no serious complications from any of the exercise tests. CONCLUSION: PE may be excluded by a normal nadir VE/VCO(2) in patients presenting with subacute dyspnea. A combination of decreased peak VO(2)/kg, increased nadir VE/VCO(2), normal breathing reserve, and exercise-induced desaturation may be sensitive and specific for PE. CPET may assist in identifying subacute PE in patients with contraindications to use of computed tomography angiography or ventilation perfusion scans.

Single-center experience with implantable cardioverter-defibrillators in adults with complex congenital heart disease.

Adults with congenital heart disease are at risk of lethal ventricular arrhythmias and are candidates for implantable cardiac defibrillator (ICD) therapy, yet implant risks, long-term outcomes, and rates of appropriate and inappropriate ICD therapies are not well characterized. We reviewed clinical, implantation, and follow-up data on all transvenous ICDs in adults with congenital heart disease at the Mayo Clinic from 1991 through 2008. Seventy-three adults with congenital heart disease received 85 ICDs. Implantation diagnoses included tetralogy of Fallot (44%) and congenitally corrected transposition of the great arteries (17%). Implantation indication was occurrence of sustained ventricular arrhythmias (secondary prevention) in 36% and prophylactic (primary prevention) in the remainder. There were no major implant-related complications. During follow-up (2.2 ± 2.8 years, range 0 to 15) 11 patients died and 4 patients received heart or heart/lung transplants. An appropriate shock for a ventricular arrhythmia was observed in 19% of patients and an inappropriate shock was observed in 15% of patients. Likelihood of an appropriate shock was associated with increased subpulmonic ventricular pressure. In conclusion, implantation of transvenous ICDs in adults with congenital heart disease is associated with a low risk of implant complications. In this high-risk adult population the rate of inappropriate ICD shocks is low, whereas the likelihood of appropriate therapy for potentially lethal ventricular arrhythmias is high. These data suggest overall benefit of ICD therapy in adults with congenital heart disease.

Composite hodgkin and non-hodgkin lymphoma of the mitral and aortic valves.

A 58-year-old man with a remote history of diffuse large B-cell lymphoma (DLBCL), status post chemotherapy, radiation, and peripheral blood stem cell transplantation, presented with splenic nodular sclerosis classical Hodgkin lymphoma. He was found to have aortic and mitral valve mass lesions. The mitral valve mass showed typical histologic and immunophenotypic features of nodular sclerosis classical Hodgkin lymphoma, whereas the aortic valve mass and aortic mitral curtain tissue showed DLBCL with necrosis. Both tumors were Epstein-Barr virus positive and were clonally related; however, they were not related to his DLBCL from 14 years prior. This is the first case report of a patient with a composite lymphoma affecting two cardiac valves.