Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk ...for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management.
Carcinoid heart disease is a frequent occurrence in patients with carcinoid syndrome and is responsible for substantial morbidity and mortality. The pathophysiology of carcinoid heart disease is ...poorly understood; however, chronic exposure to excessive circulating serotonin is considered one of the most important contributing factors. Despite recognition, international consensus guidelines specifically addressing the diagnosis and management of carcinoid heart disease are lacking. Furthermore, there is considerable variation in multiple aspects of screening and management of the disease. The aim of these guidelines was to provide succinct, practical advice on the diagnosis and management of carcinoid heart disease as well as its surveillance. Recommendations and proposed algorithms for the investigation, screening, and management have been developed based on an evidence-based review of the published data and on the expert opinion of a multidisciplinary consensus panel consisting of neuroendocrine tumor experts, including oncologists, gastroenterologists, and endocrinologists, in conjunction with cardiologists and cardiothoracic surgeons.
Background There are limited studies of thrombotic and embolic complications (TEC) in the adult Fontan population. The purpose of the study was to determine the prevalence, risk factors, and outcomes ...of TECs in this population. Methods Retrospective review of adults with a previous Fontan operation, with follow-up at Mayo Clinic, 1994–2014. Systemic TEC was defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus. Non-systemic TEC was defined as Fontan conduit/right atrial thrombus or pulmonary embolus. Results We identified 387 patients; mean (±SD) age 28 (7) years and mean follow-up 8 (2) years. An atriopulmonary connection (APC) was done for 286 patients (74%). Atrial arrhythmias were present in 278 (72%). There were 121 TECs (systemic n = 36, non-systemic n = 85) in 98 patients (25%). Risk factors for systemic TEC were atrial arrhythmia (hazard ratio HR, 2.28; P = .001) and APC (HR, 1.98; P = .02); non-systemic TEC also had similar risk factors. All 98 patients received warfarin. Warfarin was discontinued in 10/98 because of bleeding; and 8 of these 10 subsequently had a second TEC. Among the 82 patients that had follow-up imaging, 16 (20%) had resolution of thrombus. In total, 24/98 patients had a second TEC, most of whom had inadequate anticoagulation. Conclusions TEC was not uncommon; risk factors for TEC were APC and atrial arrhythmias. Most patients were treated successfully with warfarin alone. A second TEC occurred in most patients whose anticoagulation was discontinued because of bleeding events.
Bioprosthetic valve thrombosis (BPVT) is considered uncommon; this may be related to the fact that it is often unrecognized. Recent data suggest that BPVT responds to vitamin K antagonists, ...emphasizing the need for reliable diagnosis.
This study sought to determine the diagnostic features of BPVT and to formulate a diagnostic model for BPVT.
Cases of BPVT occurring between 1997 and 2013 were identified from the Mayo Clinic pathology database. Patients with BPVT were matched 1:2 for age, sex, and prosthesis position with patients whose valves were explanted for structural failure. We formulated a diagnostic model for BPVT using multivariate linear logistic regression and receiver operating characteristic.
Among 397 consecutive cases of explanted bioprostheses, there were 46 cases of BPVT (11.6%; aortic 29, mitral 9, tricuspid 7, pulmonary 1), mean age was 63 years, and 68% were male. Thirty (65%) cases occurred >12 months post-implantation; median bioprosthetic valve longevity was 24 months (cases) versus 108 months (controls) (p < 0.001). Independent predictors of BPVT were >50% increase in mean echo-Doppler gradient from baseline within 5 years (odds ratio OR: 12.7), paroxysmal atrial fibrillation (OR: 5.19), subtherapeutic international normalized ratio (OR: 7.37), increased cusp thickness (OR: 12.2), and abnormal cusp mobility (OR: 6.94). Presence of all 5 diagnostic features was predictive of BPVT with 76% sensitivity, 93% specificity, 85% positive predictive value, and 89% negative predictive value (p < 0.001).
BPVT is not uncommon and can occur several years after surgery. A combination of clinical and echocardiographic features can reliably diagnose BPVT.
Ebstein anomaly has heterogeneous anatomy and numerous operative techniques are described. Cone repair provides a near anatomic tricuspid valve repair. The purpose of this study was to examine our ...experience with cone repair.
Cone repair was performed in 235 consecutive patients with Ebstein anomaly, 134 children (57%) and 101 adults (43%), from June 2007 to October 2015. Median age was 15.6 years (range, 6 months to 73 years). Cone repair was the first operation in 192 patients (82%), the second in 41 (17%), and the third in 2 (1%). Previous tricuspid valve repair had been performed in 27 (12%). Echocardiograms were obtained preoperatively and at hospital dismissal for all patients and for a subgroup of patients at least 6 months after cone repair (n = 81).
Leaflet augmentation was done in 67 patients (28%), Sebening stitch in 57 (24.2%), neochordae in 49 (21%), and annuloplasty band in 158 (67%). Bidirectional cavopulmonary shunt was performed in 46 patients (20%). There was 1 early death (0.4%). Early reoperation was required in 14 patients (5.9%); re-repair was possible in 7 (50%). The majority of early reoperations (11 of 14; 79%) occurred in the first third of the series. Mean follow-up was 3.5 ± 2.5 years. There was sustained reduction in tricuspid regurgitation (p < 0.0001), a progressive decline in right ventricle size (p < 0.0001), and late increase in right ventricle fractional area change after initial decline (p < 0.0001). Freedom from late reoperation was 97.9% at 6 years.
Cone repair is safe, and the learning curve is significant. Sustained reduction in tricuspid regurgitation and favorable changes in the right ventricle at follow-up suggest that cone repair has an advantageous impact on right ventricular remodeling.
Display omitted
Ebstein Anomaly: We Should Do Better Connolly, Heidi M.; Dearani, Joseph A.; Miranda, William R.
Journal of the American College of Cardiology,
06/2023, Letnik:
81, Številka:
25
Journal Article
Left ventricular ejection fraction (LVEF) is reduced in a subset of patients with severe aortic stenosis (AS).
The authors sought to determine the temporal course of reduced LVEF, its predictors, and ...its impact on prognosis in severe AS.
Serial echocardiograms of 928 consecutive patients with first-time diagnosis of severe AS (aortic valve area AVA ≤1 cm2) who had at least 1 echocardiogram before the diagnosis were evaluated. A total of 3,684 echocardiograms (median 3 studies per patient) within the preceding 10 years were analyzed.
At the initial diagnosis, 196 (21%) patients had an LVEF <50% (35.1 ± 9.7%) and 732 (79%) had an LVEF ≥50% (64.2 ± 6.1%). LVEF deterioration had begun before AS became severe for those with an LVEF <50% and accelerated after AVA reached 1.2 cm2, whereas mean LVEF remained >60% in patients with LVEF ≥50% at initial diagnosis. The strongest predictor for LVEF deterioration was LVEF <60% at 3 years before AS became severe (odds ratio: 0.86; 95% confidence interval: 0.83 to 0.89; p < 0.001). During the median follow-up of 3.3 years, mortality was significantly worse, not only for patients with an LVEF <50%, but for patients with an LVEF of 50% ≤ LVEF <60% compared with patients with an LVEF ≥60% even after aortic valve replacement (p < 0.001).
In patients with severe AS and reduced LVEF, a decline in LVEF began before AS became severe and accelerated after AVA reached 1.2 cm2. LVEF <60% in the presence of moderate AS predicts further deterioration of LVEF and appears to represent abnormal LVEF in AS.
Display omitted
Objective: Cardiovascular disease is the major cause of pregnancy-related maternal mortality in the United States, and congenital heart disease (CHD) is the most common form of structural heart ...disease affecting women of childbearing age. Most females born with CHD will reach childbearing age and consider pregnancy. Adult CHD and maternal-fetal medicine (MFM) specialists managing women with CHD should provide preconception counseling, cardiovascular risk assessment prior to pregnancy that estimates maternal and fetal risk, management during pregnancy, and in the peripartum period and also know the potential complications and special circumstances that may occur in the post-partum period.
This chapter will review the population at risk, patient risk prior to pregnancy, management during pregnancy, management in the peripartum and post-partum periods, and outline specific cardiovascular complications. The chapter will also briefly review some common or high-risk congenital cardiovascular lesions commonly encountered.
Management of patients with most forms of CHD encountered during pregnancy requires a multidisciplinary approach and careful team-based care to facilitate safe and appropriate management and pregnancy success.
Pulmonary valve replacement (PVR) is associated with improvement in symptoms and right ventricular remodeling in patients with tetralogy of Fallot (TOF). There are limited population-based data about ...outcomes after PVR. We therefore hypothesized a temporal increase in annual volume of PVR and decrease in in-hospital mortality after PVR in the United States.
We reviewed the National Inpatient Sample (NIS) for PVR performed in adults (>18 years) with TOF, 2000–2014. The primary outcome was trends in admissions for PVR, in-hospital mortality after PVR, and age at time of PVR. In order to assess trends, we divided the study period into tertiles: early era (2000–2004), mid era (2005–2009) and late era (2010–2014).
There were 18,353 admissions in adults with TOF diagnosis, of which PVR was performed in 1230 (6.7%), and 90 (7.3%) were transcatheter PVRs. The median age at PVR was 34 years and in-hospital mortality was 1.5%. Comparisons by study era showed temporal increase in the proportion of admissions for PVR (3.7% vs 6.3% vs 9.6%, p < 0.001), decrease in in-hospital mortality (4.1% vs 1.2% vs 0.8%, p = 0.002), and a decrease in age at the time of PVR (35.8 vs 33.8 vs 31.0 years, p < 0.001).
The proportion of admissions for PVR increased while in-hospital mortality and age at time of PVR decreased over time. A younger age at the time of PVR highlights important concerns and knowledge gaps about the cumulative lifetime risk of reinterventions and prosthetic valve endocarditis. Further studies are required to address these knowledge gaps.
•Proportion of admissions for valve replacement (PVR) increased over time.•In-hospital mortality and age at time of PVR decreased over time.•Results highlight potential risk of cumulative lifetime risk of reinterventions.
PDF
