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.

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.

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.).

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.

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.

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.

Scimitar Syndrome in Children and Adults: Natural History, Outcomes, and Risk Analysis.

BACKGROUND: Scimitar syndrome involves congenital anomalous pulmonary venous return to the inferior vena cava. Optimal management remains controversial. We describe the natural history of disease, nonsurgical and surgical outcomes, and risk factors for poor outcomes at our institution. METHODS: Patients with anomalous pulmonary venous return to the inferior vena cava documented on echocardiography at our institution between January 1994 and January 2015 were reviewed retrospectively. The study protocol IRB-AAAO1805 was approved. RESULTS: Forty-seven patients were identified, including 20 infants with significant associated congenital heart defects (42.6%, including 7 with single ventricle physiology), and 10 infants (21.3%) and 16 noninfants (34.0%) with isolated scimitar syndrome. Median follow-up was 3.55 years. Noninfants exhibited lower incidences of right pulmonary artery hypoplasia (p < 0.001), aortopulmonary collaterals (p = 0.004), and scimitar vein obstruction at the caval confluence (p = 0.032). Eighteen patients (38.3%) underwent surgical repair for scimitar syndrome. Overall mortality after baffle repair or scimitar vein reimplantation was 37.5% (3 of 8) for infants and 0% (0 of 6) for noninfants (p = 0.209). Overall mortality for medically managed infants was 46.7% (7 of 15) compared with 0% (0 of 8) for noninfants (p = 0.052). Multivariable analyses identified infantile onset as an independent risk factor for stenosis or obstruction after repair (hazard ratio 9.34, p = 0.048), and single ventricle physiology as an independent risk factor for mortality among unrepaired patients (hazard ratio 29.8, p = 0.004). CONCLUSIONS: The severity of scimitar syndrome depends on presenting age and associated congenital heart disease. Nonsurgical and surgical outcomes are suboptimal for infantile disease, which is a risk factor for stenosis after repair. Single ventricle physiology is associated with poor prognosis.

Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion.

BACKGROUND: The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified. METHODS AND RESULTS: A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and liver size independently using post-gadolinium contrast sequences. Twenty-seven patients met inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean splenic diameter (P = .001). Serial post-gadolinium imaging was available in 25/27 (93%) patients of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies. Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV diameter was the only anatomic variable significantly correlated with time from Fontan completion (P = .004). CONCLUSIONS: Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a significant number of adult Fontan patients and may be associated with ventricular enlargement and decreased ventricular function by CMR.

Procedural Success and Adverse Events in Pulmonary Artery Stenting: Insights From the NCDR.

BACKGROUND: Risk factors associated with outcomes for pulmonary artery (PA) stenting remain poorly defined. OBJECTIVES: The goal of this study was to determine the effect of patient and procedural characteristics on rates of adverse events and procedural success. METHODS: Registry data were collected, and 2 definitions of procedural success were pre-specified for patients with biventricular circulation: 1) 20% reduction in right ventricular pressure or 50% increase in PA diameter; and 2) 25% reduction in right ventricular pressure or 50% decrease in PA gradient or post-procedure ratio of in-stent minimum to pre-stent distal diameter >80%. A separate definition of procedural success based on normalization of PA diameter was pre-specified for patients with single ventricle palliation. RESULTS: Between January 2011 and January 2014, a total of 1,183 PA stenting procedures were performed at 59 institutions across 1,001 admissions; 262 (22%) procedures were performed in patients with a single ventricle. The rate of procedural success was 76% for definition 1, 86% for definition 2, and 75% for single ventricle patients. In the multivariate analysis, ostial stenosis was significantly associated with procedural success for biventricular patients according to both definitions. The overall complication rate was 14%, with 9% of patients experiencing death or a major adverse event (MAE). According to multivariate analysis, weight <4 kg, having a single ventricle, and emergency status were significantly associated with death or MAEs. CONCLUSIONS: In our analysis, success was >75% across all definitions, and adverse events were relatively common. Biventricular patients with an ostial stenosis had a higher probability of a successful outcome. Patients who had a single ventricle, weight <4 kg, or who underwent an emergency procedure had a higher risk of death or MAE. These findings may help inform patient selection for PA stenting.

Postoperative tricuspid regurgitation after adult congenital heart surgery is associated with adverse clinical outcomes.

OBJECTIVE: Many patients with adult congenital heart disease will require cardiac surgery during their lifetime, and some will have concomitant tricuspid regurgitation. However, the optimal management of significant tricuspid regurgitation at the time of cardiac surgery remains unclear. We assessed the determinants of adverse outcomes in patients with adult congenital heart disease and moderate or greater tricuspid regurgitation undergoing cardiac surgery for non-tricuspid regurgitation-related indications. METHODS: All adult patients with congenital heart disease and greater than moderate tricuspid regurgitation who underwent cardiac surgery for non-tricuspid regurgitation-related indications were included in a retrospective study at the Schneeweiss Adult Congenital Heart Center. Cohorts were defined by the type of tricuspid valve intervention at the time of surgery. The primary end point of interest was a composite of death, heart transplantation, and reoperation on the tricuspid valve. RESULTS: A total of 107 patients met inclusion criteria, and 17 patients (17%) reached the primary end point. A total of 68 patients (64%) underwent tricuspid valve repair, 8 patients (7%) underwent tricuspid valve replacement, and 31 patients (29%) did not have a tricuspid valve intervention. By multivariate analysis, moderate or greater postoperative tricuspid regurgitation was associated with a hazard ratio of 6.12 (1.84-20.3) for the primary end point (P = .003). In addition, failure to perform a tricuspid valve intervention at the time of surgery was associated with an odds ratio of 4.17 (1.26-14.3) for moderate or greater postoperative tricuspid regurgitation (P = .02). CONCLUSIONS: Moderate or greater postoperative tricuspid regurgitation was associated with an increased risk of death, transplant, or reoperation in adult patients with congenital heart disease undergoing cardiac surgery for non-tricuspid regurgitation-related indications. Concomitant tricuspid valve intervention at the time of cardiac surgery should be considered in patients with adult congenital heart disease with moderate or greater preoperative tricuspid regurgitation.

Usefulness of magnetic resonance imaging to guide referral for pulmonary valve replacement in repaired tetralogy of Fallot.

The aim of this study was to determine if adult patients with repaired tetralogy of Fallot are being referred for pulmonary valve replacement (PVR) earlier on the basis of cardiac magnetic resonance imaging (CMR) parameters despite the absence of CMR-based recommendations in the American College of Cardiology and American Heart Association joint guidelines. Variables defined by the guidelines were analyzed in conjunction with CMR-based parameters across 3 groups defined by the release of the guidelines: (1) patients referred before the guidelines, (2) patients referred 0 to 3 years after the guidelines, and (3) patients referred ≥3 years after the guidelines. Seventy-nine patients were identified. No significant trend was observed in guideline-defined variables. Significant trends in indexed right ventricular end-diastolic volume (p = 0.034), indexed right ventricular end-systolic volume (p = 0.001), and the right ventricular ejection fraction (p = 0.005) were observed across groups. By multivariate regression, patients who underwent PVR ≥3 years after the release of the guidelines had a 29 ml/m(2) smaller indexed right ventricular end-diastolic volume (p = 0.01) and a 33 ml/m(2) smaller indexed right ventricular end-systolic volume (p <0.001) compared with patients who underwent PVR before the release of the guidelines. PVR 0 to 3 years after the guidelines was not a significant predictor of either indexed right ventricular end-diastolic volume (p = 0.93) or indexed right ventricular end-systolic volume (p = 0.18). Patients referred for PVR ≥3 years after the guidelines had significantly smaller CMR-based right ventricular volumes without significant trends in guideline-defined variables. Given the increased use of CMR to guide PVR referral, revisiting the guidelines to address appropriate use of CMR derived thresholds is indicated.

Validation of SPECT equilibrium radionuclide angiographic right ventricular parameters by cardiac magnetic resonance imaging.

BACKGROUND: Recent advances in the treatment of primary pulmonary hypertension (PPH), and in surgery to correct tetralogy of Fallot (TOF), have rekindled interest in evaluating right ventricular (RV) volume and ejection fraction (EF). The purpose of this investigation was to determine the accuracy of RV functional parameters assessed by single photon emission computed tomography (SPECT) equilibrium radionuclide angiography (ERNA). METHODS AND RESULTS: Twenty-eight patients with PPH (n = 15) or TOF (n = 13) (aged 28 +/- 14 years; 57% male) were analyzed by means of SPECT ERNA algorithms that automatically identified mid-RV tomographic planes, generated regions isolating the right ventricle from other structures, and presented RV-segmented regions as a cinematic display. RV EF and volumes were computed and compared with values obtained by magnetic resonance imaging (MRI). Mean values were not different between SPECT ERNA and MRI for RV EF, end-diastolic volume, and end-systolic volume (42% +/- 11% vs 41% +/- 10%, 135 +/- 67 mL vs 139 +/- 91 mL, and 87 +/- 54 mL vs 85 +/- 61 mL, respectively; P = not significant for all comparisons). Significant linear correlation (P <.0001) was found between SPECT ERNA and MRI for RV EF, end-diastolic volume, and end-systolic volume (r = 0.85, r = 0.94, and r = 0.93, respectively). No statistically significant trends or biases for RV EF were found. Intraobserver and interobserver comparisons demonstrated good reproducibility. As expected, RV volume was significantly higher and RV EF was significantly lower for patients with PPH and TOF than were values for individuals at low likelihood for coronary artery disease or other cardiac disease. CONCLUSIONS: SPECT ERNA provides accurate, reproducible assessment of RV volumes and EF and should prove useful in evaluating the magnitude of RV dysfunction in patients and in providing an objective means with which to assess the results of therapeutic interventions.