Impact of atrial arrhythmia on survival in adults with tetralogy of Fallot.

BACKGROUND: Atrial arrhythmia is a late complication after tetralogy of Fallot (TOF) repair, but arrhythmia outcomes data are limited. OBJECTIVES: The purpose of the study was to describe atrial arrhythmia presentations, outcomes of antiarrhythmic therapy, and impact of arrhythmia on transplant-free survival. METHODS: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) Registry and identified 113 patients (age 49 ±â€¯13 years) with documented arrhythmia, and 302 patients without history of arrhythmia, 1990-2017. We classified arrhythmias into atrial fibrillation and atrial flutter/tachycardia based on the rhythm on the first abnormal electrocardiogram. RESULTS: At the time of first documented arrhythmia, 58(51%) had atrial fibrillation while 55(49%) had atrial flutter/tachycardia. Of the 113 patients, 14(12%) received rhythm control with class I/III antiarrhythmic drugs (AAD), 79(70%) had direct current cardioversion, 9(8%) received rate control with class II/IV AAD, and 11(10%) received only anticoagulation. Successful cardioversion occurred in 100(89%) patients, and arrhythmia recurrence rate was 16 per 100 patient-years. The multivariate risk factors for death and/or heart transplant were atrial fibrillation (HR 1.94, CI 1.10-3.15, P = .031) and older age (HR 1.63, CI 1.12-2.43, P = .019) per 5 year increment. CONCLUSIONS: Atrial fibrillation, but not atrial flutter, was associated with reduced survival in our repaired TOF cohort. Further studies are required to determine if more aggressive antiarrhythmic therapy will improve survival in patients with atrial fibrillation.

Exercise Capacity After Repair of Ebstein Anomaly in Adults.

Repair of Ebstein anomaly has evolved over the last decade, and timing of repair remains variable. There have been no studies of exercise or functional capacity in patients who have had tricuspid valve surgery for Ebstein anomaly in adulthood. We aimed to compare exercise capacity before and after tricuspid valve repair or replacement for Ebstein anomaly in adults at Mayo Clinic. We performed a retrospective chart review of all patients with Ebstein anomaly who underwent tricuspid valve surgery at Mayo Clinic between June 2007 and January 2015. We compared pre- and postoperative echocardiograms, exercise tests, and clinic visits. Tricuspid valve surgery was done for 322 patients, and 32 patients met criteria of native tricuspid valve repair or replacement at age 18 or older and had maximal pre- and postoperative exercise tests. Nineteen patients had valve repair, and 13 had valve replacement. Surgery for Ebstein anomaly resulted in significant reduction in tricuspid regurgitation and right ventricular size. There was a significant improvement in NYHA functional class after surgery; however, there was no significant improvement in functional aerobic capacity (FAC), metabolic equivalents (METs), exercise time, or [Formula: see text] after surgery. Patients who had an atrial shunt closed during surgery had improved minimum blood oxygen saturations during exercise, though no improvement in exercise capacity. In our cohort, patients who had tricuspid valve repair or replacement for Ebstein anomaly reported an improvement in functional capacity; however, this did not reflect improvement in measured exercise capacity, despite excellent surgical results by echocardiography.

Right Parasternal Lead Placement Increases Eligibility for Subcutaneous Implantable Cardioverter Defibrillator Therapy in Adults With Congenital Heart Disease.

BACKGROUND: The subcutaneous implantable cardioverter defibrillator (S-ICD) provides an attractive option for patients with congenital heart disease (CHD) in whom a transvenous defibrillator is contraindicated. Given the unusual cardiac anatomy and repolarization strain, the surface electrocardiogram (ECG) is frequently abnormal, potentially increasing the screen failure rate. METHODS AND RESULTS: We prospectively screened 100 adult CHD patients regardless of the presence of clinical indication for ICD utilizing a standard left sternal lead placement, as well as a right parasternal position. Baseline patient and 12-lead ECG characteristics were examined to assess for predictors of screen failure. Average patient age was 48±14 years, average QRS duration was 134±37 ms, and 13 patients were pacemaker dependent. Using the standard left parasternal electrode position, 21 patients failed screening. Of these 21 patients with screen failure, 9 passed screening with the use of right parasternal electrode positioning, reducing screening failure rate from 21% to 12%. QT interval and inverted T wave anywhere in V2-V6 leads were found to be independent predictors of left parasternal screening failure (P=0.01 and P=0.04, respectively). CONCLUSIONS: Utilization of both left and right parasternal screening should be used in evaluation of CHD patients for S-ICD eligibility. ECG repolarization characteristics were also identified as novel predictors of screening failure in this group. (Circ J 2016; 80: 1328-1335).

Choosing Between MRI and CT Imaging in the Adult with Congenital Heart Disease.

Improvements in the outcomes of surgical and catheter-based interventions and medical therapy have led to a growing population of adult patients with congenital heart disease. Adult patients with previously undiagnosed congenital heart disease or those previously palliated or repaired may have challenging echocardiographic examinations. Understanding the distinct anatomic and hemodynamic features of the congenital anomaly and quantifying ventricular function and valvular dysfunction plays an important role in the management of these patients. Rapid advances in imaging technology with magnetic resonance imaging (MRI) and computed tomography angiography (CTA) allow for improved visualization of complex cardiac anatomy in the evaluation of this unique patient population. Although echocardiography remains the most widely used imaging tool to evaluate congenital heart disease, alternative and, at times, complimentary imaging modalities should be considered. When caring for adults with congenital heart disease, it is important to choose the proper imaging study that can answer the clinical question with the highest quality images, lowest risk to the patient, and in a cost-efficient manner.

Outcomes of Surgical Repair of Aberrant Subclavian Arteries in Adults.

BACKGROUND: Symptoms, imaging characteristics, and early and midterm surgical outcomes for aberrant subclavian arteries (ASCA) are not well defined in the adult population. METHODS: A single-institution retrospective review was conducted of adults undergoing surgical repair of ASCA and descending aorta origin/Kommerell diverticulum (KD) from January 1, 2002, to December 31, 2021. Symptom improvement and differences in imaging characteristics between anatomic groups and the number of symptoms were assessed. RESULTS: Mean age was 46 ± 17 years. There were 23 of 37 left aortic arches with right ASCA (62%) and 14 of 37 right aortic arches with left ASCA (38%). Of these, 31 of 37 (84%) were symptomatic, and 19 of 37 (51%) had KD size/growth meeting criteria for surgical repair. KD aortic origin diameter was larger in more symptomatic patients: 20.60 mm (interquartile range [IQR], 16.42-30.68 mm) in patients with ≥3 symptoms vs 22.05 mm (IQR, 17.52-24.21 mm) for 2 symptoms vs 13.72 mm (IQR, 12.70-15.95 mm) for 1 symptom (P = .018). Aortic replacement was required in 22 of 37 (59%). There were no early deaths. Complications occurred in 11 of 37 (30%): vocal cord dysfunction (4 of 37 [11%]), chylothorax (3 of 37 [8%]), Horner syndrome (2 of 37 [5%]), spinal deficit (2 of 37 [5%]), stroke (1 of 37 [3%]), and temporary dialysis requirement (1 of 37 [3%]). Over a median follow-up of 2.3 years (IQR, 0.8-3.9 years), there was 1 endovascular reintervention and no reoperations. Dysphagia and shortness of breath resolved in 92% and 89%, respectively, whereas gastroesophageal reflux persisted in 47%. CONCLUSIONS: The KD aortic origin diameter correlates with the number of symptoms, and surgical repair of ASCA and descending aorta origin/KD effectively relieves symptoms, with low rates of reintervention. Given the operative complexity, surgical repair should be performed in patients meeting size criteria or with significant dysphagia or shortness of breath symptoms.

Incidence and Outcomes of Advanced Heart Failure in Adults With Congenital Heart Disease.

BACKGROUND: There are limited data about the stage D heart failure (advanced HF) in adults with congenital heart disease. Our study objectives were (1) to determine the incidence of new-onset advanced HF in patients and the relationship between advanced HF and all-cause mortality and (2) to determine the relationship between therapies for advanced HF and all-cause mortality. METHODS: Retrospective cohort study of adults with congenital heart disease at Mayo Clinic (2003-2019). We defined advanced HF using the European Society of Cardiology diagnostic criteria for advanced HF. Therapies received by the patients with advanced HF were classified into 3 mutually exclusive groups (treatment pathways): (1) conventional cardiac intervention, (2) transplant listing, and (3) palliative care. RESULTS: Of 5309 patients without advanced HF at baseline assessment, 432 (8%) developed advanced HF during follow-up (1.1%/y), and the incidence of advanced HF was higher in patients with severe or complex congenital heart disease. Onset of advanced HF was associated with 6-fold increase in the risk of mortality. Conventional cardiac intervention was associated with significantly higher risk of mortality as compared to transplant listing. The longer the interval from the initial onset of advanced HF to transplant evaluation, the lower the odds of being listed for transplant. CONCLUSIONS: Based on these data, we postulate that early identification of patients with advanced HF, and a timely referral for transplant evaluation (instead of conventional cardiac intervention) may offer the best chance of survival for these critically ill patients. Further studies are required to validate this postulation.

Prevalence and risk of progressive aortic aneurysm and dissection in adults with conotruncal anomalies.

AIMS: Conotruncal anomalies share common embryogenic defects of the outflow tracts and great arteries, which result in a predisposition to aortic aneurysms. The purpose of this study was to describe the prevalence and risk of progressive aortic aneurysms in adults with conotruncal anomalies. METHODS AND RESULTS: Retrospective study of adults with conotruncal anomalies that underwent cross-sectional imaging 2003-20. Aneurysm was defined as aortic root/mid-ascending aorta >2.1 mm/m2/>1.9 mm/m2, progressive aneurysm as increase by >2 mm, and severe aneurysm as dimension >50 mm. Of 2261 patients (38 ± 12 years; male 58%), 1167 (52%) had an aortic aneurysm, and 205 (14%) had a severe aortic aneurysm. Mean annual increase in aortic root/mid-ascending aorta was 0.3 ± 0.1 mm/0.2 ± 0.1 mm. The 3-, 5-, and 7-year cumulative incidence of the progressive aortic aneurysm was 4%, 7%, and 9%, respectively. The rate of aneurysm growth decreased with age, with no significant growth after age 40 years. There was an excellent correlation between aortic indices from cross-sectional imaging and echocardiography. Of 950 females, 184 had ≥1 pregnancy, and 81 (44%) of the 184 patients had aortic aneurysm prior to pregnancy. There was no aortic dissection or progression of the aortic aneurysm during pregnancy. Overall, there was no aortic dissection during 7984 patient-years of follow-up. CONCLUSIONS: Aortic aneurysm was common in patients with conotruncal anomalies. However, the risk of progressive aneurysm or dissection was low. Collectively, these data suggest a benign natural history and perhaps a less frequent need for cross-sectional imaging. Further studies are required to determine the optimal timing for surgical intervention in this population.

Single coronary artery giving rise to an intraseptal left coronary artery in a patient presenting with neurocardiogenic syncope.

BACKGROUND: Syncope occurs frequently in adolescents, and is often benign. Potential worrisome syncopal events include those occurring with exertion, concurrent chest pain, dyspnoea or palpitations, and those with focal or diffuse neurologic changes. CASE: A 16-year-old female was referred to our institution for a history of exercise-induced spells. She was diagnosed since the age of 2 years with neurocardiogenic syncope and postural orthostatic tachycardia syndrome. She had been evaluated at multiple institutions, and was followed by pediatric neurology for a diagnosis of migraines. Owing to recurrent worsening symptoms and a syncopal episode requiring resuscitation, an echocardiogram was performed. The right coronary was normal, but the left coronary artery ostium could not be identified well. Doppler patterns were suspicious of an abnormal left coronary artery, and computed tomography angiography was performed. This revealed a single coronary artery arising from the right aortic sinus, with the left coronary artery arising from the proximal coronary trunk and coursing through the infundibular septum. This was surgically treated utilising a left internal mammary artery bypass graft to the left anterior descending coronary artery. A year later, she has not experienced any recurrence of syncope, and has returned to athletic activity. CONCLUSION: This case highlights the index of suspicion that must be present when evaluating any patient with syncope, both clinically and via echocardiography. A computed tomography angiogram is indicated for better evaluation of coronary artery anatomy when an anomalous coronary cannot be ruled out by echocardiography.

Heightened Dependence of Left-Heart Filling Pressures on Right-Heart Failure in Congenital Heart Disease.

BACKGROUND: Pulmonary artery wedge pressure (PAWP) is often elevated in patients with right-sided congenital heart disease (CHD), raising the possibility of coexisting left-heart disease, but pressure-volume relationships in the left and right sides of the heart influence one another through interdependence, which may be amplified in patients with CHD. METHODS: We hypothesized that increases in PAWP in patients with CHD would be more strongly related to ventricular interdependence compared with patients who have isolated left-heart disease such as heart failure with preserved ejection fraction (HFpEF). Ventricular interdependence was assessed by the relationship between PAWP and right-atrial pressure (RAP), RAP/PAWP ratio, and the left-ventricular (LV) eccentricity index. RESULTS: PAWP was elevated (≥15 mm Hg) in 49% of patients with CHD (n = 449). There was a very strong correlation between RAP and PAWP in CHD (r = 0.81, P < 0.001) that greatly exceeded the respective correlation in HFpEF (n = 160; r = 0.58, P < 0.001; P < 0.001 between groups). RAP/PAWP ratio and LV eccentricity index were higher in CHD than HFpEF (1.26 ± 0.18 vs 1.05 ± 0.14, P = 0.007) and (0.80 ± 0.21 vs 0.59 ± 0.19, P < 0.001), respectively. RAP (but not PAWP) was an independent predictor of death/transplant (hazard ratio 1.86 per 5 mm Hg, 95% confidence interval, 1.39-2.45, P = 0.002). CONCLUSIONS: Left-heart filling pressures are commonly elevated in right-sided CHD, but this is related predominantly to right-heart failure and enhanced ventricular interdependence rather than left-heart disease. These data provide new insight into the basis of abnormal left-heart hemodynamics in patients with CHD and reinforce the importance of therapeutic interventions targeted to the right heart.

Persistent Hypertension and Left Ventricular Hypertrophy After Repair of Native Coarctation of Aorta in Adults.

[Figure: see text].

Adults With Tetralogy of Fallot: Early Postoperative Outcomes and Risk Factors for Complications.

OBJECTIVE: To report the early postoperative outcomes in adults with tetralogy of Fallot (TOF) undergoing cardiac surgery and to identify patient factors associated with complications. PATIENTS AND METHODS: We performed a single-institution retrospective review of adults with TOF who underwent cardiac surgery from January 8, 2008, through June 21, 2018. Patients' characteristics, preoperative imaging, surgical interventions, outcomes, and complications were analyzed. RESULTS: There were 219 adults with TOF (mean age, 40 years; range, 18-83 years; 88 [40%] female) in the study. Surgical interventions included repair or replacement of the pulmonary valve (n=199 [91%]), tricuspid valve (n=70 [32%]), mitral valve (n=13 [5.9%]), and aortic valve (n=8 [3.7%]). Three patients (1.4%) underwent first-time TOF repair. The 30-day mortality rate was 1.4% (n=3). Early postoperative complications occurred in 66 (30%) and included arrhythmias requiring treatment, dialysis requirement, liver dysfunction, respiratory failure, infection, reoperation, cardiac arrest, mechanical circulatory support, and death. Multivariate analysis found older age at current surgery (odds ratio [OR], 1.04 per year; 95% CI, 1.01 to 1.06; P<.001), longer cardiopulmonary bypass time (OR, 1.01 per minute; 95% CI, 1.01 to 1.02; P<.001), right ventricular systolic dysfunction (OR, 1.31; 95%, CI 1.02 to 1.69; P=.03), diabetes mellitus (OR, 3.50; 95% CI, 1.20 to 10.2; P=.02), and history of initial palliative surgery (OR, 1.99; 95% CI, 1.01 to 3.91; P=.05) as independent predictors of complications. CONCLUSION: Surgical interventions for adult patients with TOF can be performed with low early morbidity and mortality. Clinical characteristics and preoperative testing parameters can predict risk for complications in the postoperative period.

Early and Late Outcomes of Cardiovascular Surgery in Patients With Ehlers-Danlos Syndrome.

BACKGROUND: Cardiovascular surgical outcomes reports are few for vascular type IV of Ehlers- Danlos Syndrome (vEDS) compared to non-vascular types I-III (nEDS). METHODS: To define cardiovascular surgical outcomes among adult patients (≥18 years) with EDS types, a review of our institution's in-house STS Adult Cardiac Surgery Database-compliant software and electronic medical records from Mayo Clinic (1993-2019) was performed. Outcomes were compared for vEDS patients and nEDS patients. Demographics, baseline characteristics, operative, in-hospital complications and follow-up vital status were analyzed. RESULTS: Over the study time frame, 48 EDS patients underwent surgery (mean age 52.6 ± 14.6 years; 48% females). Of these, 17 patients had vEDS and 31 patients had nEDS. Six patients (12.5%) underwent prior sternotomy. Urgent or emergent surgery was performed in 10 patients (20.8%). Aortic (vEDS 76.5% vs. nEDS 16.1%) and mitral procedures (vEDS 11.8% vs. nEDS 48.4%) were the two most common cardiovascular surgeries performed (p < .01 and p = .007, respectively). Cardiopulmonary bypass time (CPB) (165 ± 18 vs. 90 ± 13 min; p = .015) and aortic cross clamp times (140 ± 14 vs. 62 ± 10 min; p < .001) were longer for vEDS patients. There was 1 (2.1%) early and 7 (14.6%) late deaths; 6 among vEDS and 2 among nEDS patients. Survival at 5 (80% vs. 93%), 10 (45% vs. 84%) and 15 years (45% vs. 84%) was lower in patients with vEDS (p = .015 for each comparison). CONCLUSION: Cardiovascular surgeries are significantly more complex with longer bypass and cross clamp times for type IV vEDS compared to nEDS patients. Reduced overall survival underscores the complexity and fragility of vEDS patients.

CT and MR imaging of the mitral valve: radiologic-pathologic correlation.

Computed tomography (CT) and magnetic resonance (MR) imaging are increasingly important adjuncts to echocardiography for the evaluation of mitral valve disease. The mitral valve may be involved in various acquired or congenital conditions with resultant regurgitation or stenosis, and many of these conditions can be identified with CT or MR imaging. In addition, CT is useful for detecting and monitoring postoperative complications after mitral valve repair or replacement. As the use of CT and MR imaging increases, awareness of the CT and MR imaging appearances of the normal mitral valve and the various disease processes that affect it may foster recognition of unsuspected mitral disease in patients undergoing imaging for other purposes. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.306105518/-/DC1.

Echocardiographic predictors of severe right ventricular diastolic dysfunction in tetralogy of Fallot: Relations to patient outcomes.

BACKGROUND: Previous studies have described echocardiographic indices of right ventricular (RV) diastolic function in patients with tetralogy of Fallot (TOF) but these indices have not been validated against invasive hemodynamic data. The purpose of this study was to determine echocardiographic predictors of severe RV diastolic dysfunction, and the impact of severe RV diastolic dysfunction on transplant-free survival. METHODS: Cohort study of TOF patients that underwent non-simultaneous cardiac catheterization and echocardiogram at Mayo Clinic. Based on prior studies we selected these indices for assessment: tricuspid E/A, E/e', deceleration time, pulmonary artery forward flow, dilated inferior vena cava (IVC), and hepatic vein diastolic flow reversal (HVDFR). RV diastolic function classes (normal, mild/moderate and severe dysfunction) were created using arbitrary cut-off points of the median values of right ventricular end-diastolic pressure (RVEDP) and right atrial pressure (RAP) for the cohort. RESULTS: Among 173 patients (age 40 ± 13 years), 68 patients were classified as normal (RVEDP≤14 and RAP≤10), 37 as mild/moderate dysfunction (either RVEDP>14 or RAP>10), and 69 as severe dysfunction (RVEDP>14 and RAP>10). Of the indices assessed, dilated IVC had the best sensitivity of 95% (area under the curve [AUC] 0.689) while HVDFR had the best specificity of 69% (AUC 0.648) for detecting severe RV diastolic dysfunction. Severe RV diastolic dysfunction was an independent risk factor for death/transplant (hazard ratio 2.83, p = 0.009). CONCLUSION: Severe RV diastolic dysfunction, as defined by invasive hemodynamic indices, was associated with poor prognosis. Echocardiographic indices can identify these high risk patients, and hence improve risk stratification in clinical practice.

Impact of Inferior Venae Cava Assessment in Tetralogy of Fallot.

BACKGROUND: Inferior vena cava (IVC) size and collapsibility provide a noninvasive estimate of right heart filling pressures, an important determinant of right heart hemodynamic performance that is not measured by cardiac magnetic resonance imaging (CMRI). We hypothesized that compared with CMRI risk model alone, a combined CMRI-IVC risk model will have better correlation with disease severity and peak oxygen consumption in patients with tetralogy of Fallot (TOF). METHODS: We performed a retrospective review of patients with TOF with moderate/severe pulmonary regurgitation who underwent CMRI and echocardiography. A CMRI risk model was constructed using right ventricular (RV) end-diastolic volume index, RV end-systolic volume index, RV ejection fraction, and left ventricular ejection fraction. We added IVC hemodynamic classification to the CMRI indices to create CMRI-IVC risk model, and IVC hemodynamics were modeled as a categorical variable: normal vs mild/moderately abnormal (dilated IVC or reduced collapsibility) vs severely abnormal IVC hemodynamics (dilated IVC and reduced collapsibility). We defined disease severity as atrial arrhythmias, ventricular arrhythmias, and heart failure hospitalization. RESULTS: Of 207 patients, 131 (63%), 72 (35%), and 4 (2%) had normal, mild/moderately abnormal, and severely abnormal IVC hemodynamics, respectively. Compared with the CMRI risk model, the CMRI-IVC risk model had a better correlation with disease severity (area under the curve, 0.62; 95% confidence interval, 0.51-0.74 vs area under the curve 0.84, 95% confidence interval, 0.78-0.91, P = 0.006) and peak oxygen consumption (r = 0.35, P = 0.042 vs r = 0.43, P = 0.031, Meng test P = 0.026). CONCLUSIONS: The combined CMRI-IVC risk model had a better correlation with disease severity compared with CMRI indices alone and can potentially improve risk stratification in the population with TOF.

CONTEXTE: Le diamètre et la collapsibilité de la veine cave inférieure (VCI) permettent d'estimer de façon non invasive les pressions de remplissage du cœur droit, un déterminant important de la capacité hémodynamique cardiaque droite que ne mesure pas l'imagerie par résonance magnétique cardiaque (IRMC). Notre hypothèse était que, comparativement au modèle de risque IRMC seul, un modèle de risque combiné IRMC-VCI présenterait une meilleure corrélation avec la gravité de la maladie et la consommation maximale d'oxygène chez les patients atteints de la tétralogie de Fallot (TF). MÉTHODOLOGIE: Nous avons effectué une étude rétrospective de cas de TF avec régurgitation pulmonaire modérée ou sévère où les patients ont subi un examen d'IRMC et une échocardiographie. Nous avons créé un modèle de risque IRMC intégrant l'indice du volume télédiastolique ventriculaire droit, l'indice du volume télésystolique ventriculaire droit, la fraction d'éjection ventriculaire droite et la fraction d'éjection ventriculaire gauche. Nous avons ajouté une classification hémodynamique de la VCI aux indices d'IRMC pour créer le modèle de risque IRMC-VCI, et les caractéristiques hémodynamiques de la VCI ont été modélisées en tant que variable nominale : état normal vs anomalie légère ou modérée (VCI dilatée ou collapsibilité réduite) vs anomalie sévère des caractéristiques hémodynamiques de la VCI (VCI dilatée et collapsibilité réduite). Nous avons défini la gravité de la maladie en distinguant les arythmies auriculaires, les arythmies ventriculaires et l'insuffisance cardiaque entraînant une hospitalisation. RÉSULTATS: Au sein d'un groupe de 207 patients, les caractéristiques hémodynamiques de la VCI présentaient un état normal, une anomalie légère ou modérée et une anomalie sévère dans 131 cas (63 %), 72 cas (35 %) et 4 cas (2 %), respectivement. Comparativement au modèle de risque IRMC, le modèle de risque IRMC-VCI a présenté une meilleure corrélation avec la gravité de la maladie (aire sous la courbe = 0,62 et intervalle de confiance à 95 % = 0,51-0,74 vs aire sous la courbe = 0,84 et intervalle de confiance à 95 % = 0,78-0,91, p = 0,006) et avec la consommation maximale d'oxygène (r = 0,35, p = 0,042 vs r = 0,43, p = 0,031, p = 0,026 pour le test de Meng). CONCLUSIONS: Le modèle de risque combiné IRMC-VCI a présenté une meilleure corrélation avec la gravité de la maladie comparativement aux indices d'IRMC seuls. Il pourrait améliorer la stratification du risque au sein de la population atteinte de la TF.

Pathophysiologic and Prognostic Implications of Right Atrial Hypertension in Adults With Tetralogy of Fallot.

Background Right atrial pressure (RAP), a composite metric of right ventricular diastolic function, volume status, and right heart compliance, is a predictor of mortality in patients with heart failure due to acquired heart disease. Because patients with tetralogy of Fallot (TOF) might have abnormal right atrial and ventricular mechanics caused by myocardial injury and remodeling, we hypothesized that RAP would be associated with disease severity and cardiovascular adverse events in this population. Methods and Results We performed a cohort study of adults with TOF who underwent right heart catheterization at the Mayo Clinic Rochester between 1990 and 2017. The objective was to determine the association between RAP and multiple domains of disease severity in TOF (percentage of predicted peak oxygen consumption, atrial or ventricular arrhythmia, and heart failure hospitalization), as well as cardiovascular adverse events, defined as sustained ventricular tachycardia, resuscitated or aborted sudden death, heart transplantation, or death. Among 225 patients (113 male; mean age: 39±14 years), mean RAP was 10.7±5.2 mm Hg and median was 10 mm Hg (interquartile range: 7-13 mm Hg). Increasing RAP was associated with atrial or ventricular arrhythmias (odds ratio: 5.01; 95% CI, 1.22-23.49; P<0.001), heart failure hospitalization (odds ratio: 1.47; 95% CI, 1.10-2.39; P=0.033) per 5 mm Hg, and worsening exercise capacity (peak oxygen consumption; R2=0.74, r=-0.86, P<0.001). RAP was a predictor of cardiovascular adverse events (hazard ratio: 1.28; 95% CI, 1.10-1.47; P=0.028) per 5 mm Hg. Conclusions In symptomatic patients with TOF, increasing RAP correlates with multiple domains of disease severity (risk stratification) and predicts future cardiovascular events (prognostication). These data have potential clinical implications in the target population of symptomatic TOF patients.

The placenta: A site of end-organ damage after Fontan operation. A case series.

BACKGROUND: Placental insufficiency may be the cause of the high preterm birth rate in women after Fontan operation. In this study we reviewed the clinical course and pregnancy outcome of women with Fontan physiology with a focus on placental pathology. METHODS: We reviewed clinical charts and placental pathology from 7 women with Fontan physiology who had pregnancies at Mayo Clinic, Rochester, Minnesota. The review was limited to cases where placental pathologic specimens were rigorously examined. RESULTS: Seven women had 13 deliveries between 2002 and 2018. Only 2 of 13 deliveries were at term (>37 weeks). Mean maternal age at time of last delivery was 27.5 ±â€¯3.2 years. Preeclampsia was noted during 2 pregnancies and 2 women had preterm premature rupture of membranes at 24 and 35 weeks gestation, respectively. Placental abruption with bleeding occurred in 2 pregnancies. An additional 4 pregnancies were complicated by intrauterine growth restriction (IUGR). Median placental weight was 441.5 g (IQR 305.5-622.5 g). Median placental weight percentile for gestational age was 10th to 25th, but varied greatly; two placentas were <10th percentile and 5 were >90th percentile for gestational age. Two umbilical cords contained a single umbilical artery. Prominent subchorionic fibrin deposition was a consistent feature in all placentas. Villous hypermaturity was noted in 4 placentas. CONCLUSIONS: Fontan physiology may be associated with poor placental health. High systemic venous pressure and low cardiac output may contribute to stagnation of placental blood flow and result in subchorionic fibrin deposition and variable villous hypoplasia. This may explain the high preterm birth rate in women with Fontan physiology. Preterm deliveries and small-for-gestational-age (SGA) newborns should be anticipated in this patient population. Analysis of placental pathology may help determine both candidacy for future pregnancy and long-term effects of pregnancy for women with Fontan physiology.