Timing of pulmonary valve replacement: can we use the same volumetric thresholds in repaired tetralogy of Fallot and pulmonary stenosis?

The timing of pulmonary valve replacement in patients with pulmonary regurgitation following treatment of pulmonary stenosis is undefined. Although cardiac magnetic resonance-based right ventricular volumes in tetralogy of Fallot patients have been used as a guide in pulmonary stenosis patients, anatomic differences between tetralogy of Fallot and pulmonary stenosis patients complicate their application to pulmonary stenosis patients and could result in late referral for pulmonary valve replacement. We sought to determine if pulmonary stenosis patients referred for pulmonary valve replacement were at greater risk for morbidity or need for tricuspid valve intervention at the time of pulmonary valve replacement. A retrospective cohort study was performed on all adult patients with a diagnosis of pulmonary stenosis or tetralogy of Fallot followed at our centre. Clinical and imaging-based exposures were collected. Pre-specified endpoints included need for concomitant tricuspid valve repair or replacement and pre- and post-pulmonary valve replacement cardiac magnetic resonance-based volumetric measurements. Between 1/1999 and 1/2020, 235 patients underwent pulmonary valve replacement for pulmonary regurgitation (52 with pulmonary stenosis, 183 with tetralogy of Fallot). Pulmonary stenosis patients were more likely to have at least moderate tricuspid regurgitation (p = 0.010), undergo concomitant tricuspid valve intervention (p = 0.003), and require tricuspid valve repair or replacement secondary to annular dilation (p = 0.027) compared to tetralogy of Fallot patients. There was no difference in pre-pulmonary valve replacement right ventricular size between pulmonary stenosis and tetralogy of Fallot patients. These findings suggest that referral for pulmonary valve replacement may be occurring later in the disease course for pulmonary stenosis patients.

Cardiac transplantation in adult congenital heart disease with prior sternotomy.

BACKGROUND: Adult congenital heart disease (ACHD) patients who require orthotopic heart transplantation are surgically complex due to anatomical abnormalities and multiple prior surgeries. In this study, we investigated these patients' outcomes using our institutional database. METHODS: ACHD patients who had prior intracardiac repair and subsequent heart transplant were included (2008-2018). Adult patients without ACHD were extracted as a control. A comparison of patients with functional single ventricular (SV) and biventricular (BV) hearts was performed. RESULTS: There were 9 SV and 24 BV patients. The SV group had higher central venous pressure/pulmonary capillary wedge pressure (P = .028), hemoglobin concentration (P = .010), alkaline phosphatase (P = .022), and were more likely to have liver congestion (P = .006). Major complications included infection in 16 (48.5%), temporary dialysis in 12 (36.4%), and graft dysfunction requiring perioperative mechanical support in 7 (21.2%). Overall in-hospital mortality was 15.2%. Kaplan-Meier analysis showed a higher, but not statistically significant, survival after 10 years between the ACHD and control groups (ACHD 84.9% vs. control 67.5%, P = .429). There was no significant difference in 10-year survival between SV and BV groups (78% vs. 88%, P = .467). CONCLUSIONS: Complex ACHD cardiac transplant recipients have a high incidence of early morbidities after transplantation. However, long-term outcomes were acceptable.

The Miracle Baby Grows Up: Hypoplastic Left Heart Syndrome in the Adult.

PURPOSE OF REVIEW: Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the mitral valve, left ventricle, and aorta and is ultimately palliated with a single-ventricle repair. Universally fatal in infancy prior to the advent of modern surgical techniques, the majority of HLHS patients will now reach adulthood. However, despite improvements in early survival, the HLHS population continues to face significant morbidity and early mortality. This review delineates common sources of patient morbidity and highlights areas in need of additional research for this growing segment of the adult congenital heart disease population. RECENT FINDINGS: It has become increasingly clear that palliated adult single ventricle patients, like those with HLHS, face significant life-long morbidity from elevated systemic venous pressures as a consequence of the Fontan procedure. Downstream organ dysfunction secondary to elevated Fontan pressures has the potential to significantly impact long-term management decisions, including strategies of organ allocation. Because of the presence of a morphologic systemic right ventricle, HLHS patients may be at even higher risk than other adult patients with a Fontan. Because the adult HLHS population continues to grow, recognition of common sources of patient morbidity and mortality is becoming increasingly important. A coordinated effort between patients and providers is necessary to address the many remaining areas of clinical uncertainty to help ensure continued improvement in patient prognosis and quality of life.

Outcomes of Adult Patients With Congenital Heart Disease After Heart Transplantation: Impact of Disease Type, Previous Thoracic Surgeries, and Bystander Organ Dysfunction.

BACKGROUND: Adults with congenital heart disease (CHD) are at increased risk for adverse outcomes after heart transplantation (HT). However, small cohorts have constrained the identification of factors associated with poor prognosis. We hypothesized that number of sternotomies and bystander organ dysfunction would be associated with an increased risk for early death after HT. METHODS AND RESULTS: We performed a retrospective observational study of all adult CHD patients who underwent HT at our institution from January 1997 to January 2014. Forty-eight adult CHD patients were followed for a mean of 5 years. Diagnoses included tetralogy of Fallot/pulmonary atresia/double-outlet right ventricle in 15 (31%), D-transposition of the great arteries (TGA) in 10 (21%), tricuspid atresia/double-inlet left ventricle in 9 (19%), ventricular or atrial septal defect in 4 (8%), heterotaxy in 3 (6%), congenitally corrected TGA in 2 (4%), and other diagnoses in 5 (10%). Survival at both 1 and 5 years was 77%. According to multivariate analysis, ≥3 sternotomies (hazard ratio [HR] 8.5; P = .02) and Model for End-Stage Liver Disease Excluding International Normalized Ratio (MELD-XI) score >18 (HR 6.2; P = .01) were significant predictors of mortality. Failed Fontan surgery was not a significant predictor of death (P = .19). CONCLUSIONS: In our cohort of adult CHD patients undergoing HT, ≥3 sternotomies and MELD-XI score >18 were significantly associated with death. These findings may be important in patient selection and decision regarding tolerable number of CHD surgeries before considering HT.

Comparative outcomes and risk analysis after cone repair or tricuspid valve replacement for Ebstein's anomaly.

Objective: Ebstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on the outcomes of patients with Ebstein's anomaly who underwent a cone repair or tricuspid valve replacement. Methods: A total of 85 patients who underwent a cone repair (mean age, 16.5 years) or tricuspid valve replacement (mean age, 40.8 years) between 2006 and 2021 were included. Univariate, multivariate, and Kaplan-Meier analyses were used to evaluate operative and long-term outcomes. Results: Residual/recurrent greater than mild-to-moderate tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement (36% vs 5%; P = .010). However, at last follow-up, the risk of greater than mild-to-moderate tricuspid regurgitation was not different between groups (35% in the cone group vs 37% in the tricuspid valve replacement group; P = .786). The tricuspid valve replacement group had a higher risk of tricuspid valve reoperation (37% vs 9%; P = .005) and tricuspid stenosis (21% vs 0%; P = .002) compared with the cone repair group. Kaplan-Meier freedom from reintervention was 97%, 91%, and 91% at 2, 4, and 6 years after cone repair, respectively, and 84%, 74%, and 68% at 2, 4, and 6 years after tricuspid valve replacement, respectively (P = .0191). At last follow-up, right ventricular function was significantly worse from baseline in the tricuspid valve replacement group (P = .0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group. Conclusions: The cone procedure offers excellent results, with stable tricuspid valve function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement, but this did not expose the patient to a higher risk of reoperation or death at last follow-up. Tricuspid valve replacement was associated with a significantly higher risk of tricuspid valve reoperation and tricuspid valve stenosis, and worse right ventricular function at last follow-up.

Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation.

BACKGROUND: The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown. OBJECTIVES: The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients. METHODS: We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses. RESULTS: A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011]). CONCLUSIONS: Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2.

Morbidity and Mortality in Adult Fontan Patients After Heart or Combined Heart-Liver Transplantation.

BACKGROUND: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited. OBJECTIVES: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population. METHODS: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of "failing" Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, "failing Fontan" diagnosis by treating cardiologist, or admission for heart failure. RESULTS: A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade. CONCLUSIONS: In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality.

COVID-19-Related Thrombotic and Bleeding Events in Adults With Congenital Heart Disease.

Background: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications. Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients. Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression. Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P < 0.0001) and were in more advanced physiological stage (P = 0.032) and NYHA functional class (P = 0.01), had lower baseline oxygen saturation (P = 0.0001), and more frequently had a history of atrial arrhythmia (P < 0.0001), previous hospitalization for heart failure (P < 0.0007), and were more likely hospitalized for COVID-19 (P < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P < 0.0001) were independently associated with increased risk of TE/bleeding complications. Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants.

Heart rate response during exercise and pregnancy outcome in women with congenital heart disease.

BACKGROUND: Cardiopulmonary exercise testing is often used to evaluate exercise capacity in adults with congenital heart disease including women who are considering pregnancy. The relationship between cardiopulmonary exercise testing parameters and pregnancy outcome has not been defined. METHODS AND RESULTS: We conducted a multicenter retrospective observational study of women with congenital heart disease who had undergone cardiopulmonary exercise testing within 2 years of pregnancy or during the first trimester. Cardiopulmonary exercise testing variables included peak oxygen consumption and measures of chronotropic response: peak heart rate, percentage of maximum age predicted heart rate, heart rate reserve (peak heart rate-resting heart rate), and chronotropic index [(peak heart rate-resting heart rate)/(220-age-resting heart rate)]. We identified 89 pregnancies in 83 women. There were 4 spontaneous abortions and 1 termination. One or more adverse cardiac events occurred in 18%; congestive heart failure in 14%, and sustained arrhythmia in 7%. Peak heart rate (odds ratio [OR] 0.71; 95% confidence interval [CI] [0.53, 0.94]; P=0.02), percentage of maximum age predicted heart rate (OR 0.93; 95% CI [0.88, 0.98]; P=0.01), and chronotropic index (OR 0.65; 95% CI [0.47, 0.90]; P=0.01) were associated with a cardiac event. Neonatal events occurred in 20%. Peak heart rate (OR 0.75; 95% CI [0.58, 0.98]; P=0.04), percentage of maximum age predicted heart rate (OR 0.94; 95% CI [0.89, 0.99]; P=0.02), heart rate reserve (OR 0.8; 95% CI [0.64, 0.99]; P=0.04), and chronotropic index (OR 0.73; 95% CI [0.54, 0.98]; P=0.04) correlated with a neonatal event. Peak oxygen consumption was not associated with an adverse pregnancy outcome. CONCLUSIONS: Abnormal chronotropic response correlates with adverse pregnancy outcomes in women with congenital heart disease and should be considered in refining risk stratification schemes.

Interventions for Congenital Atrioventricular Valve Dysfunction: JACC Focus Seminar.

Innovation and creativity have led to tremendous advancements in the care and management of patients with congenital heart disease (CHD) that have resulted in considerably increased survival. Catheter-based interventions have contributed significantly to these advancements. However, catheter-based interventions for congenital lesions of the atrioventricular (AV) valves have been limited in scope and effectiveness mainly because of patient size and anatomical challenges. Thus, surgical repair and replacement for congenital AV valve lesions have remained the preferred therapy. However, the ongoing transcatheter heart valve revolution has led to techniques and technologies that are changing the landscape, particularly for adult CHD patients. Many devices for AV valve repair and replacement are being studied in adult patients without CHD, and translation of select practices to CHD patients has begun, with many more to come. Transcatheter AV valve interventions represent exciting opportunities for the growing numbers of adult CHD patients.

Cardiac MRI predictors of adverse outcomes in adults with a systemic right ventricle.

AIMS: Predicting risk in individuals with a systemic right ventricle (SRV) remains difficult. We assessed the value of cardiac MRI (CMR) for predicting death, heart transplantation (HT), or need for a ventricular assist device (VAD) in adults with D-transposition of the great arteries (DTGA) post Mustard/Senning and in adults with congenitally corrected transposition of the great arteries (ccTGA) at two large academic centres. METHODS AND RESULTS: Between December 1999 and November 2020, 158 adult patients with an SRV underwent CMR. Indexed right ventricular end-diastolic volume (RVEDVI), indexed right ventricular end-systolic volume (RVESVI), right ventricular ejection fraction (RVEF), and right ventricular mass (RV mass) were determined by a core laboratory. Receiver operating curves, area under the curve (AUC), and cut-points maximizing sensitivity and specificity for the endpoint for each CMR parameter were calculated. Over a median of 8.5 years, 21 patients (13%) met a combined endpoint of HT referral, VAD, or death. Each CMR parameter was significantly associated with the endpoint in both cohorts. The AUCs for RVEDVI, RVESVI, RVEF, and RV mass to predict the endpoint were 0.93, 0.90, 0.73, and 0.84 for DTGA and 0.76, 0.74, 0.71, and 0.74 for ccTGA, respectively. Optimized cut-points for RVEDVI were calculated for DTGA and ccTGA and were 132 and 126 mL/m2 , respectively. RVEDVI cut-points were simplified to 130 mL/m2 for survival analysis, which was significantly associated with survival in both cohorts. CONCLUSIONS: Cardiac MRI parameters are associated with an increased risk of death, HT, or VAD in patients with an SRV and should be considered to facilitate risk stratification.

Cardiac Arrest in a Softball Player Following a Collision: Catching the Correct Diagnosis.

Commotio cordis is a rare cause of sudden cardiac arrest from blunt chest trauma; however, it is a diagnosis of exclusion. We present a case of sudden cardiac arrest in a collegiate athlete initially attributed to commotio cordis but in whom further history and workup revealed another rare condition. (Level of Difficulty: Advanced.).

Congenital cardiac liver cirrhosis with combined hepatocellular-cholangiocarcinoma-a case report.

Background: Cardiac liver cirrhosis secondary to Fontan procedure has been associated with hepatocellular carcinoma at a younger age. However, Fontan associated liver disease and combined hepatocellular-cholangiocarcinoma has not been previously reported. Combined hepatocellular-cholangiocarcinoma is a rare cancer that accounts for 2-5% of primary liver tumors and poses significant diagnostic and treatment challenges. This case highlights these needs and potential screening and treatment considerations. Herein we describe a case of combined hepatocellular-cholangiocarcinoma in a patient with autism, congenital heart disease, and Fontan procedure. Case Description: The patient is a 27-year-old male who presented with a liver mass detected on MRI performed in the context of a rising alpha-fetoprotein during a screening visit. Biopsy of the mass revealed a combined hepatocellular-cholangiocarcinoma which was staged as localized. Due to the COVID-19 pandemic and subsequent halt of all elective surgeries, the patient received local therapy with chemoembolization followed by pembrolizumab. The disease progressed though, and therapy was changed to gemcitabine plus cisplatin. Patient received 2 cycles of therapy, after which he and his family decided to transfer medical care to Memorial Sloan Kettering. Next generation sequencing of the tumor revealed TP53 and FGFR2 mutations. By then patient was also found to have lung metastasis. To help address the hepatocellular carcinoma, lenvatinib was added. Patient had sustainable disease control for about a year, yet eventually developed thrombocytopenia complicated by an episode of gastrointestinal bleeding. With a worsening performance status, adverse events of the treatment, and recurrent hospitalizations, a goals of care discussion with his family led to the discontinuation of active cancer therapy and patient was started on best supportive care. Patient remained in active follow-up until the time of this report and passed away less than a year from initiating best supportive care alone. Conclusions: This challenging case raises awareness towards screening and monitoring all patients with Fontan procedure for Fontan associated liver disease and liver cancers, including combined hepatocellular-cholangiocarcinoma. To the best of our knowledge, this is the first description of combined hepatocellular-cholangiocarcinoma occurring in the context of cardiac cirrhosis. The management difficulties that led to altering the goals of care, is another reminder of the dynamic nature of the care oncologists would provide.

COVID-19 in Adults With Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. OBJECTIVES: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. METHODS: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.

Effect of Ventricular Pacing on Morbidity in Adults After Fontan Repair.

Implantation of a permanent pacemaker is a negative prognostic marker in patients with Fontan palliation; however, data delineating outcomes in adult patients with pacemaker requirements are lacking. We hypothesize that high ventricular pacing burden is associated with adverse outcomes in adult Fontan patients. We performed a retrospective review comprising adult patients with history of Fontan repair. A high burden of ventricular pacing was defined as ≥40% pacing. Major adverse clinical events (MACE) were defined as all-cause mortality or need for advanced cardiac therapies (ventricular assist device or heart transplant). A total of 145 adult patients with Fontan were studied for a median of 3.1 years. Twenty (14%) patients had implanted pacemakers with ≥40% ventricular pacing. Twelve events occurred in those with ≥40% ventricular pacing (incidence 60.0%) versus 11 in those without (incidence 8.8%). In multivariable analysis, ≥40% ventricular-pacing (odds ratio 12.51, confidence interval [CI] 3.56 to 43.83, p <0.001) was associated with MACE independent of initial Fontan type, New York Heart Association functional class at baseline, or history of atrial tachyarrythmia. In survival analysis, patients with ≥40% ventricular pacing had nearly 8 times the risk of MACE compared with those with a lower ventricular pacing burden (hazard ratio 7.79, 95% CI 2.56 to 23.66, p <0.001), whereas patients with atrial-only or <40% ventricular pacing burden had a trend toward higher hazard of MACE compared with those without permanent pacemaker (hazard ratio 3.38, 95% CI 0.92 to 12.47, p = 0.07) that did not meet statistical significance. These findings suggest that high ventricular pacing burden contributes to poor outcomes in the adult Fontan patients and bear consideration when determining optimal treatment of tachyarrhythmias in this population.

Inappropriate implantable cardioverter-defibrillator shocks in repaired tetralogy of fallot patients: Prevalence and electrophysiological mechanisms.

BACKGROUND: Patients with Tetralogy of Fallot (TOF) are at increased risk for sudden cardiac death, often undergo implantable cardioverter defibrillator (ICD) implantation at younger ages, and are at greater risk of experiencing inappropriate shocks. We investigated occurrences of ICD shocks in TOF patients to identify prevalence, characteristics associated with inappropriate shocks, and therapeutic interventions after inappropriate shocks. METHODS: Records of patients with repaired TOF and ICD implantation who were followed at Columbia University Irving Medical Center between 1/1/2000 and 5/1/2019 were analyzed. RESULTS: 44 patients with repaired TOF and ICD implantation were reviewed. Mean age at implantation was 39 ± 13 years. Eight (18%) patients received both appropriate and inappropriate shocks, 6 (14%) received only appropriate shocks, and 3 (7%) received only inappropriate shocks. Three patients received inappropriate shocks for sinus tachycardia, 7 for atrial arrhythmias, and 1 for noise artifact. Inappropriately shocked patients had lower beat per minute (bpm) cutoff values for ICD therapy (mean = 162 ± 24 bpm vs. 182 ± 16 bpm, p = 0.007). After inappropriate shocks, 1 patient underwent lead replacement, 1 had the VT cutoff increased, and 6 were treated with medications. CONCLUSIONS: One quarter of TOF patients with ICDs experienced inappropriate shock therapy, the timing of which was most often clustered within the first two years after implant or years later. Lower shock therapy zones were associated with increased risk for inappropriate shocks, and the majority of inappropriate shocks resulted from atrial arrhythmias with rapid ventricular response. Treatments for inappropriate shocks included increasing VT therapy bpm and rhythm and/or rate control medications.

Impact of Coronavirus Disease 2019 (COVID-19) on Patients With Congenital Heart Disease Across the Lifespan: The Experience of an Academic Congenital Heart Disease Center in New York City.

Background We sought to assess the impact and predictors of coronavirus disease 2019 (COVID-19) infection and severity in a cohort of patients with congenital heart disease (CHD) at a large CHD center in New York City. Methods and Results We performed a retrospective review of all individuals with CHD followed at Columbia University Irving Medical Center who were diagnosed with COVID-19 between March 1, 2020 and July 1, 2020. The primary end point was moderate/severe response to COVID-19 infection defined as (1) death during COVID-19 infection; or (2) need for hospitalization and/or respiratory support secondary to COVID-19 infection. Among 53 COVID-19-positive patients with CHD, 10 (19%) were <18 years of age (median age 34 years of age). Thirty-one (58%) had complex congenital anatomy including 10 (19%) with a Fontan repair. Eight (15%) had a genetic syndrome, 6 (11%) had pulmonary hypertension, and 9 (17%) were obese. Among adults, 18 (41%) were physiologic class C or D. For the entire cohort, 9 (17%) had a moderate/severe infection, including 3 deaths (6%). After correcting for multiple comparisons, the presence of a genetic syndrome (odds ratio [OR], 35.82; P=0.0002), and in adults, physiological Stage C or D (OR, 19.38; P=0.002) were significantly associated with moderate/severe infection. Conclusions At our CHD center, the number of symptomatic patients with COVID-19 was relatively low. Patients with CHD with a genetic syndrome and adults at advanced physiological stage were at highest risk for moderate/severe infection.

Predictors and rates of recurrence of atrial arrhythmias following catheter ablation in adults with congenital heart disease.

BACKGROUND: Catheter ablation is commonly performed to treat atrial arrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, predictors of recurrence remain poorly defined. OBJECTIVE: We sought to determine predictors of arrhythmia recurrence in ACHD patients following catheter ablation for atrial arrhythmias. METHODS: We performed a retrospective study of all catheter ablations for atrial arrhythmias performed in ACHD patients between January 12, 2005 and February 11, 2015 at our institution. Prespecified exposures of interest and time from ablation to recurrence were determined via chart review. RESULTS: Among 124 patients (mean age: 45 years) who underwent catheter ablation, 96 (77%) were treated for macro-reentrant atrial tachycardia, 10 (7%) for focal atrial tachycardia, 9 (7%) for atrial fibrillation, 7 (6%) for atrioventricular nodal reentrant tachycardia, and 2 (2%) for atrioventricular reentrant tachycardia. 15 (12%) required transseptal/transbaffle puncture. Fifty-one percent of patients recurred with a median time to recurrence of 1639 days. By univariate and multivariable analysis, body mass index (BMI) and Fontan status were the only variables associated with recurrence. Dose-dependent effect was observed with overweight (HR = 2.37, P = .012), obese (HR = 2.67, P = .009), and morbidly obese (HR = 4.23, P = .003) patients demonstrating an increasing risk for arrhythmia recurrence postablation. There was no significant different in recurrence rates by gender, age, non-Fontan diagnosis, or need for transseptal puncture. CONCLUSIONS: In our cohort of ACHD patients, BMI was a significant risk factor for arrhythmia recurrence postablation, independent of Fontan status. These findings may help guide treatment decisions for persistent arrhythmias in the ACHD population.

Catheter Ablation for Atrial Tachycardia in Adults With Congenital Heart Disease: Electrophysiological Predictors of Acute Procedural Success and Post-Procedure Atrial Tachycardia Recurrence.

OBJECTIVES: This study sought to determine the electrophysiological predictors of acute procedural success and of post-ablation recurrence of atrial tachyarrhythmias (ATs) in our adult congenital heart disease (ACHD) population undergoing catheter ablation for treatment of AT. BACKGROUND: Catheter ablation is frequently performed to treat persistent AT in ACHD. The predictors of post-ablation AT recurrence have not been well studied in the ACHD population. METHODS: The authors performed a retrospective study of all catheter ablations for treatment of AT performed in ACHD patients between December 1, 2005, and July 20, 2017, at Columbia University Medical Center. Pre-specified clinical and procedural data of interest and the time from ablation to recurrence were determined by chart and procedure report review. RESULTS: A total of 140 patients (mean age: 45 years) underwent catheter ablation for 182 AT. Of the AT, 179 (93%) were intra-atrial macro-re-entrant tachycardia, and 12 (7%) had a focal origin. The presence of a single mechanism was a predictor of acute procedural success that could be achieved in 89% of the patients. At a median of 49.9 months, 62 patients (44%) had recurrent AT. Time to recurrence was significantly shorter (12.5 months) for recurrent AT in 13 of the 20 patients with previous Fontan procedure. By multivariable analysis, acute procedural success was a positive predictor and prior surgical maze procedure was a negative predictor of AT-free survival. Of the 62 patients with recurrent AT, 42 (68%) had a second catheter ablation procedure, and in 22 of these, the AT mechanism was different than previously observed. CONCLUSIONS: Catheter ablation for AT in ACHD patients is an effective method of arrhythmia control. More than 1 AT mechanism per patient is common. Acute procedural success is a predictor of freedom from AT recurrence. The majority of patients achieve multiple arrhythmia-free years.