Mortality and Morbidity of Heart Failure Hospitalization in Adult Patients With Congenital Heart Disease.

BACKGROUND: Little is known about outcomes following heart failure (HF) hospitalization among adults with congenital heart disease (CHD) in the United States. We aim to compare the outcomes of HF versus non-HF hospitalizations in adults with CHD. METHODS AND RESULTS: Using a national deidentified administrative claims data set, patients with adult congenital heart disease (ACHD) hospitalized with and without HF (ACHDHF+, ACHDHF-) were characterized to determine the predictors of 90-day and 1-year mortality and quantify the risk of mortality, major adverse cardiac and cerebrovascular events, and health resource use. Cox proportional hazard regression was used to compare ACHDHF+ versus ACHDHF- for risk of events and health resource use. Of 26 454 unique ACHD admissions between January 1, 2010 and December 31, 2020, 5826 (22%) were ACHDHF+ and 20 628 (78%) were ACHDHF-. The ACHD HF+ hospitalizations increased from 6.6% to 14.0% (P<0.0001). Over a mean follow-up period of 2.23 ± 2.19 years, patients with ACHDHF+ had a higher risk of mortality (hazard ratio [HR], 1.86 [95% CI, 1.67-2.07], P<0.001), major adverse cardiac and cerebrovascular events (HR, 1.73 [95% CI, 1.63-1.83], P<0.001) and health resource use including rehospitalization (HR, 1.09 [95% CI, 1.05-1.14], P<0.001) and increased postacute care service use (HR, 1.56 [95% CI, 1.32-1.85], P<0.001). Cardiology clinic visits within 30 days of hospital admission were associated with lower 90-day and 1-year all-cause mortality (odds ratio [OR], 0.62 [95% CI, 0.49-0.78], P<0.001; OR, 0.69 [95% CI, 0.58-0.83], P<0.001, respectively). CONCLUSIONS: HF hospitalization is associated with increased risk of mortality and morbidity with high health resource use in patients with ACHD. Recent cardiology clinic attendance appears to mitigate these risks.

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.

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

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

[Figure: see text].

A Comparison of Hemodynamic and Clinical Outcomes After Transcatheter Versus Surgical Therapy in Adults in Coarctation of Aorta.

BACKGROUND: Transcatheter stent therapy provides similar acute reduction in coarctation of aorta (COA) gradient and systolic blood pressure (SBP) as compared with surgery. However, there are limited data comparing mid-term outcomes after transcatheter vs surgical therapy for COA. The purpose of this study was to compare temporal changes in Doppler COA gradient and SBP after transcatheter stent therapy versus surgical therapy for COA. METHODS: A retrospective study of COA patients (≥18 years old) undergoing transcatheter stent therapy or surgical therapy at Mayo Clinic in Rochester, Minnesota from 2000-2018 was performed. Linear regression analyses were used to compare temporal changes in Doppler gradient and SBP between the 2 groups. Propensity matching was used to adjust for between-group differences in clinical and anatomic characteristics. RESULTS: A total of 44 and 128 patients underwent transcatheter and surgical therapy, respectively; there were no significant between-group differences in the anatomy of the thoracic aorta. Both groups had similar acute reduction in Doppler peak gradient (P=.66), mean gradient (P=.41), SBP (P=.22), and upper-to-lower extremity SBP (ULE-SBP) gradient (P=.69). The median follow-up was 46 months (interquartile range, 27-81 months) and 63 months (interquartile range, 41-94 months) in the transcatheter and surgical groups, respectively. There were no significant between-group differences in the temporal change in Doppler peak gradient (P-interaction=.06), mean gradient (P-interaction=.15), SBP (P-interaction=.20), and ULE-SBP gradient (P-interaction=.51). CONCLUSIONS: These favorable short- and mid-term outcome data support the use of transcatheter therapy as an alternative to surgery in adults with COA. Further studies are required to determine if these favorable outcomes are maintained on long-term follow-up.

Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes.

BACKGROUND: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. METHODS: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. RESULTS: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months. CONCLUSIONS: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Repair of Partial Atrioventricular Septal Defects in Adults: A Single Center Experience.

Limited data are available on long-term outcome after repair of partial atrioventricular septal defects (pAVSD) in adults. We sought to review our experience. Between January 1, 1957 and December 31, 2016, 179 adult patients [median age (IQR) 34 (18, 72) years] underwent primary repair of pAVSD. The most common associated defects were secundum atrial septal defect (n = 38) and ventricular septal defect (VSD) (n = 7). Left atrioventricular valve (LAVV) zone of apposition (ZOA) was complete in 47 patients and LAVV regurgitation (≥moderate) was present in 73 patients. Autologous pericardium (n = 79, 45%) and polytetrafluoroethylene felt (n = 56, 32%) were mainly used for pAVSD closure.  Repair techniques for LAVV regurgitation included: ZOA suture closure (n =  142), suture annuloplasty (n = 10) and posterior band annuloplasty (n = 9). Six had LAVV replacement. There were 61 deaths over a median follow-up of 21 years (IQR 10, 38), with only 4 early deaths. In a limited subset of patients with 80 paired measurements (n = 40), median right ventricular systolic pressure declined from 43 mm Hg (IQR 35-51) to 33 mm Hg (IQR 30-44) postoperatively (P < 0.001), and this improvement was sustained over long-term follow-up (P = 0.513). A total of 34 patients underwent a reoperation (recurrent LAVV regurgitation, n = 26; left ventricular outflow-tract obstruction, n = 7; LAVV stenosis, n = 4; patch dehiscence, n = 1) with cumulative incidence of 6% and 16% at 10 and 15 years, respectively. Repair of pAVSD in adults can be done safely with low early mortality and good long-term outcomes. Postrepair reduction of pulmonary artery pressure is significant. Despite the low re-operation rates, long-term surveillance remains essential.

Hemodynamics in Adults With the Shone Complex.

Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.

Cardiovascular Considerations in Caring for Pregnant Patients: A Scientific Statement From the American Heart Association.

Cardio-obstetrics has emerged as an important multidisciplinary field that requires a team approach to the management of cardiovascular disease during pregnancy. Cardiac conditions during pregnancy include hypertensive disorders, hypercholesterolemia, myocardial infarction, cardiomyopathies, arrhythmias, valvular disease, thromboembolic disease, aortic disease, and cerebrovascular diseases. Cardiovascular disease is the primary cause of pregnancy-related mortality in the United States. Advancing maternal age and preexisting comorbid conditions have contributed to the increased rates of maternal mortality. Preconception counseling by the multidisciplinary cardio-obstetrics team is essential for women with preexistent cardiac conditions or history of preeclampsia. Early involvement of the cardio-obstetrics team is critical to prevent maternal morbidity and mortality during the length of the pregnancy and 1 year postpartum. A general understanding of cardiovascular disease during pregnancy should be a core knowledge area for all cardiovascular and primary care clinicians. This scientific statement provides an overview of the diagnosis and management of cardiovascular disease during pregnancy.

Management of the Adult Patient With Congenitally Corrected Transposition: Challenges and Uncertainties.

Congenitally corrected transposition (ccTGA) is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Patients with ccTGA usually have associated congenital cardiovascular conditions; less than 1% have no associated lesions. Generally, ccTGA is identified during infancy or childhood with features of heart failure or cyanosis when there are associated lesions such as ventricular septal defect and/or pulmonic stenosis. Presentation later in life generally occurs when there are either mild or no associated lesions. Presentation during adulthood may be prompted by symptoms or signs of cardiovascular disease or due to abnormal findings on cardiac testing. Management of patients with ccTGA depends on presentation, symptoms, and associated defects. In this review, we will focus on the management of adult patients with ccTGA.

Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings.

OBJECTIVE: To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction. BACKGROUND: ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited. METHODS: Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP). RESULTS: Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP <15 mmHg. None of those with a baseline LVEDP <15 mmHg had a LAP/PAWP >15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg. CONCLUSION: LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

Echo-Doppler assessment of ventricular filling pressures in adult Fontan patients.

BACKGROUND: Diastolic dysfunction is felt to be part of the natural history of patients with prior Fontan operation. Despite that, data on noninvasive assessment of diastolic function and ventricular filling pressures in Fontan patients are limited. METHODS: We included 45 adult Fontan patients who underwent right heart catheterization with pulmonary artery wedge pressure (PAWP) measurement and transthoracic echo-Doppler assessment within 7 days. Offline measurement of systemic atrioventricular valve pulsed-wave (PW) and medial/lateral atrioventricular annular tissue Doppler velocities was performed and correlated to PAWP. RESULTS: Median age was 31.4 years (IQR 24.8-37); 51% of patients were females. Median ventricular ejection fraction was 55% (IQR 50.3-57.5) and median PAWP was 10 mm Hg (IQR 8-11). PW Doppler E velocity (r = 0.64, p ≤ 0.0001), deceleration time (r = -0.40, p = 0.008), and E/A ratio (r = 0.33, p = 0.03) correlated with PAWP. Median medial and lateral E/e' ratios also correlated to PAWP (r = 0.71, p = 0.002 and r = 0.75, p < 0.0001; respectively). An E velocity >75 cm/s had 88% sensitivity and 86% specificity [97% negative predictive value (NPV); 58% positive predictive value (PPV)], E/A ratio >1.7 had 100% sensitivity and 61% specificity (100% NPV; 26% PPV), and deceleration time <135 ms had 88% sensitivity and 83% specificity (97% NPV; 58% PPV) for predicting PAWP >12 mm Hg. CONCLUSION: Our data suggest that PW Doppler atrioventricular E velocity, E/A ratio, and deceleration time could be used to identify adult Fontan patients with normal filling pressures. However, PPVs for PAWP >12 mm Hg were poor. Further studies, particularly prospective, simultaneous echo-catheterization correlation, are critically needed.