Arrhythmia Burden Among Adult Patients With Congenital Heart Disease: A Population-Based Study.

BACKGROUND: A significant percentage of patients with congenital heart disease surviving into adulthood will develop arrhythmias. These arrhythmias are associated with an increased risk of adverse events and death. We aimed to assess arrhythmia prevalence, risk factors, and associated health care usage in a large national cohort of patients with adult congenital heart disease. METHODS AND RESULTS: Adults with a documented diagnosis of congenital heart disease, insured by Clalit and Maccabi health services between January 2007 and December 2011, were included. We assessed the associations between arrhythmia and subsequent hospitalization rates and death with mixed negative binomial and Cox proportional hazard models, respectively. Among 11 653 patients with adult congenital heart disease (median age, 47 years [interquartile range, 31-62]), 8.7% had a tachyarrhythmia at baseline, 1.5% had a conduction disturbance, and 0.5% had both. Among those without a baseline arrhythmia, 9.2% developed tachyarrhythmias, 0.9% developed a conduction disturbance, and 0.3% developed both during the study period. Compared with no arrhythmia (reference group), arrhythmia in the previous 6 months was associated with a higher multivariable adjusted hospitalization rate, 1.33-fold higher than the rate of the reference group (95% CI, 1.00-1.76) for ventricular arrhythmia, 1.27-fold higher (95% CI, 1.17-1.38) for atrial arrhythmias, and 1.33-fold higher (95% CI, 1.04-1.71) for atrioventricular block. Atrial tachyarrhythmias were associated with an adjusted mortality hazard ratio (HR) of 1.65 (95% CI, 1.44-2.94), and ventricular tachyarrhythmias with a >2-fold increase in mortality risk (HR, 2.06 [95% CI, 1.44-2.94]). CONCLUSIONS: Arrhythmias are significant comorbidities in the adult congenital heart disease population and have a significant impact on health care usage and survival.

Long-Term Outcomes and Characteristics Associated With Mortality of Adult Patients Post Fontan Surgery: 27-Year Single-Center Experience.

Data on the characteristics and long-term outcomes of patients who underwent Fontan surgery and surviving into adulthood are limited. We aimed to describe our center's long-term experience with this unique patient population. Included were adult patients who had undergone Fontan surgery and were followed up at our Adult Congenital Heart Disease clinic between the years 1994 and 2021. We describe cardiac and noncardiac morbidities, medical treatment, laboratory data, echocardiographic characteristics, and all-cause mortality. The primary outcome was a composite of heart failure hospitalizations or death. A total of 107 patients who underwent Fontan surgery were followed up during the study period; 46.7% were male. The mean age at time of Fontan was 7.4 ± 6.2 years and the mean age at the last follow-up or at the time of an outcome event was 35.0 ± 8.0 years (range 21.1 to 62.8). At the last documented follow-up, 74.7% of the cohort were in New York Heart Association functional class I/II. The common morbidities included atrial arrythmias (37%) and stroke (17%). The primary outcome occurred in 17.7%. By the end of the study period, 9.3% of the patients in the cohort died. In a multivariate logistic regression analysis, controlling for gender, age, and Fontan type, worse functional class at the last follow-up (New York Heart Association III/IV vs I/II) was significantly associated with the risk of the primary outcome (odds ratio 34.57, 95% confidence interval 6.728 to 177.623, p <0.001). In conclusion, long-term outcomes of patients surviving into adulthood with a Fontan circulation is encouraging. Most of these patients achieve good functional cardiovascular status, despite the complex anatomy and a substantial burden of co-morbid conditions, specifically, atrial arrythmias and thrombotic events. Functional class was independently associated with heart failure hospitalizations and mortality.

Self-Reported Mental and Physical Measures in Adult Fontan Patients.

Introduction: The Fontan procedure is a palliative operation for patients with single functional ventricles, arising from a heterogeneous group of heart defects. There is a considerable gap in evidence regarding the self-reported physical and mental health of these patients surviving to adulthood. Methods and Results: We administered the PROMIS® Global Short Form (v 1.2) to Fontan patients during their scheduled clinic visits during 2017−2018. The raw PROMIS scores were subsequently converted to standardized T-scores, where the mean performance was 50 for the general population. We used Cronbach's alpha to assess reliability, with >0.8 considered good. A total of 42 patients were included. The median age was 30 (IQR: 24−34) years and 59% (95% CI: 43−74%) were female. The median time from birth to operation was 4.5 (IQR: 3−8) years, with 55% having an extracardiac Fontan. The questionnaire had good internal reliability with an alpha of 0.87. Seventy-one percent of respondents rated their overall health as "excellent" or "good". The mean T-score for physical health was 46.6, lower than the age-group mean (51.6, p < 0.001). The mean T-score for mental health was 53.3, higher than the age-group mean (48.5, p < 0.001). T-scores showed strong correlation with each other (r = 0.7) and weak correlation with age and time from procedure. There was no association of T-score with diagnosis or operation type. Conclusions: Adult Fontan patients report better mental health despite worse reporting physical health compared with the age group means. Patient-reported measures can provide clinically meaningful insights about the care of patients with complex congenital heart disease.

Long-term outcomes of catheter-based intervention for clinically significant paravalvular leak.

BACKGROUND: In most centres, clinically significant percutaneous paravalvular leak (PVL) closure following valve replacement surgery is reserved for those considered high-risk for surgery. There is a paucity of data regarding the long-term outcomes of these patients. AIMS: Our goals were to assess the long-term outcomes of patients undergoing percutaneous PVL closure. METHODS: A total of 100 consecutive transcatheter PVL closure procedures (74 mitral, 26 aortic) were performed in 95 patients between February 2005 and August 2019 at our hospital. Data collected included procedural success rates, indication-specific outcomes and mortality. RESULTS: Mean follow-up was 5.6±6.1 years, mean age 62.6±15.2 years, and 45.4% were female. The device was successfully implanted in 88 procedures (88.0%). Patients who presented with heart failure (n=57) had a significant improvement in NYHA classification (29.2% Class III/IV versus 100.0%, p<0.001). For patients who presented with haemolytic anaemia (n=38), haemoglobin increased (11.94±1.634 vs 9.72±1.49, p<0.001) and LDH levels were reduced (1,354.90±1,225.55 vs 2,039.40±1,347.20, p<0.001) following the procedure. Rates of mortality were 3.8% at 90 days, 15.6% after 1 year, and 27.2% after 5 years. CONCLUSIONS: For patients who are deemed intermediate- to high-risk for repeat surgery, transcatheter PVL closure shows reasonable clinical success rates, with a significant improvement in symptoms, and a relatively low rate of periprocedural complications.

Health Service Utilization Patterns Among Adults With Congenital Heart Disease: A Population-Based Study.

Background Several studies have examined hospitalizations among patients with adult congenital heart disease (ACHD). Few investigated other services or utilization patterns. Our aim was to study service utilization patterns and predictors among patients with ACHD. Methods and Results We identified 11 653 patients with ACHD aged ≥18 years (median, 47 years), through electronic records of 2 large Israeli healthcare providers (2007-2011). The association between patient, disease, and sociogeographic characteristics and healthcare resource utilization were modeled as recurrent events accounting for the competing death risk. Patients with ACHD had high healthcare utilization rates compared with the general population. The highest standardized service utilization ratios (SSRs) were found among patients with complex congenital heart disease including primary care visits (SSR, 1.53; 95% CI, 1.47-1.58), cardiology outpatient visits (SSR, 5.17; 95% CI, 4.69-5.64), hospitalizations (SSR, 6.68; 95% CI, 5.82-7.54), and days in hospital (SSR, 15.37; 95% CI, 14.61-16.12). Adjusted resource utilization hazard increased with increasing lesion complexity. Hazard ratios (HRs) for complex versus simple disease were: primary care (HR, 1.14; 95% CI, 1.06-1.23); cardiology outpatient visits (HR, 1.40; 95% CI, 1.24-1.59); emergency department visits (HR, 1.19; 95% CI, 1.02-1.39); and hospitalizations (HR, 1.75; 95% CI, 1.49-2.05). Effects attenuated with age for cardiology outpatient visits and hospitalizations and increased for emergency department visits. Female sex, geographic periphery, and ethnic minority were associated with more primary care visits, and female sex (HR versus men, 0.89 [95% CI, 0.84-0.94]) and periphery (HR, 0.72 [95% CI, 0.58-0.90] for very peripheral versus very central) were associated with fewer cardiology visits. Arab minority patients also had high hospitalization rates compared with the majority group of Jewish or other patients. Conclusions Healthcare utilization rates were high among patients with ACHD. Female sex, geographic periphery, and ethnicity were associated with less optimal service utilization patterns. Further research should examine strategies to optimize service utilization in these groups.

Depression and anxiety are associated with high health care utilization and mortality among adults with congenital heart disease.

BACKGROUND: The significance of depression/anxiety among ACHD patients in terms of health care utilization is unknown and data on the association with mortality are scarce. METHODS: Analyses comprised 8334 ACHD patients, age ≥ 18 years, insured by a large healthcare organization (2007-2011). Depression/anxiety were determined by diagnoses and treatments recorded in the organization database. Adjusted utilization relative rates (RRs) were estimated with negative binomial models and mortality hazard ratios (HRs) with the Cox proportional hazard model. RESULTS: ACHD patients with depression/anxiety (N = 2950, 35%) were more likely to be older (mean ±â€¯SD: 54 ±â€¯17 vs. 45 ±â€¯18 years), women (61% vs. 45%), and have comorbidities than counterparts without depression/anxiety. Following multivariable adjustment, patients with depression/anxiety had more primary care and cardiology clinic visits, more emergency department visits and more hospitalizations. RRs (95% confidence interval) were: 1.31 (1.27-1.35); 1.07 (1.01-1.13); 1.60 (1.46-1.77); and 1.18 (1.08-1.29) respectively, for diagnosis before the study period, and 1.36 (1.31-1.42); 1.22 (1.14-1.30); 1.43 (1.24-1.60) and 1.47 (1.33-1.64), respectively, for diagnosis during the study. Stratifying by age, the highest adjusted primary care and cardiology visit RRs were found among 18-24 years old patients and the lowest among patients ≥65 years. Between 2007 and 2017, 905 patients died. Depression/anxiety were associated with increased mortality risk with adjusted HRs: 1.10 (95% CI: 0.94-1.29) for past diagnosis and 1.40 (1.17-1.67) for study period depression/anxiety diagnosis. CONCLUSIONS: Depression/anxiety in ACHD patients is associated with increased health-care utilization and a higher risk of death. The efficacy of addressing patients' psychosocial needs in optimizing health-care utilization and improving prognosis needs further evaluation.

Pulmonary valve replacement in repaired tetralogy of Fallot: determinants of early postoperative adverse outcomes.

OBJECTIVE: We sought to examine the frequency of early postoperative complications and preoperative predictors of prolonged hospitalization in adults with repaired tetralogy of Fallot undergoing pulmonary valve replacement. METHODS: This was a retrospective study of consecutive adult patients undergoing surgical intervention between 1995 and 2006. A multivariate logistic regression model was used to identify determinants of prolonged hospitalization. RESULTS: One hundred sixteen patients (mean age, 36 +/- 11 years) underwent 118 pulmonary valve replacements. Most (95%) operations included additional procedures, such as pulmonary artery/outflow tract reconstruction or tricuspid valve annuloplasty. The early postoperative mortality (<30 days) was 2.5%. The majority of the patients (60%) had no postoperative complications. The postoperative adverse events included postoperative arrhythmias (19%), respiratory complications (13%), reoperation during admission (13%), renal dysfunction (13%), and myocardial infarction (3%). Postoperative adverse events were associated with prolonged hospitalization (14 +/- 12 vs 7 +/- 3 days, P = .001). In the multivariate analysis age at reoperation of greater than 45 years (odds ratio, 6.1; 95% confidence interval, 1.6-23.6; P = .009), the number of previous sternotomies (odds ratio, 3.8; 95% confidence interval, 1.4-10; P = .007), and the need for urgent surgical intervention (odds ratio, 5.7; 95% confidence interval, 1.1-27.8; P = .03) were predictors of prolonged hospitalization. CONCLUSION: Pulmonary valve replacement in adults with repaired tetralogy of Fallot has a low mortality risk. The most common early postoperative complications are arrhythmias and respiratory and renal complications. Although most early postoperative complications do not result in long-term sequelae, they are associated with prolonged hospitalization. Patients undergoing urgent interventions, older patients, and those with multiple previous sternotomies are at the highest risk for prolonged hospitalization.

Impact of ethnicity and MTHFR genotype on age at onset of coronary artery disease in women in Israel.

BACKGROUND: The C677T mutation in the methylenetetrahydrofolate reductase (MTHFR) gene is associated with early onset of coronary artery disease in some populations with certain ethnic backgrounds. However, data on its effect on CAD development in women are limited and conflicting. OBJECTIVES: To investigate the effects of the MTHFR C677T mutation and ethnicity on the development and age at onset of CAD in women in Israel. METHODS: The sample included 135 Jewish women with well-documented CAD (62 Ashkenazi, 44 Oriental and 29 of other origins) in whom CAD symptoms first developed at age < or = 65 years. DNA samples from 235 women served as the control. RESULTS: CAD symptoms developed later in Ashkenazi than in Oriental women or women of other origins (51.0 +/- 7.0 years vs. 48.3 +/- 7.5 and 46.3 +/- 7.7 years, respectively, P= 0.024). Among Ashkenazi women, the T/T genotype was less common in patients in whom CAD symptoms appeared after age 50 (6.4%) than in patients with earlier CAD symptoms (25.8%, P= 0.037) and Ashkenazi control subjects (23.3%, P= 0.045). Among women from other origins, these differences were not significant. On logistic regression analysis, the T/T genotype was associated with a nearly fourfold increase in the risk of early onset (age < 45 years) of CAD (odds ratio 3.87, 95% confidence interval 1.12-13.45, adjusted for risk factors and origin) and a trend towards an influence of ethnicity (P= 0.08). Compared to Ashkenazi women, the risk of early development of CAD associated with the T/T genotype among Oriental ones was 0.46 (95%CI 0.189-1.114) and in women of other origins, 5.84 (95%CI 1.76-19.34). Each additional risk factor increased the risk of earlier onset of CAD by 42% (OR 1.42, 95%CI 1.06-1.89). CONCLUSIONS: The age at onset of CAD in Israeli women is influenced by the MTHFR genotype, ethnic origin and coronary risk factors.

Once-daily versus twice-daily intravenous administration of vancomycin for infections in hospitalized patients.

The efficacy and toxicity of once-daily (od) versus twice-daily (bd) dosing of vancomycin was compared in 121 hospitalized patients. Eighteen patients were then withdrawn from the study. Clinical and bacteriological responses were evaluated in all patients (n = 103). Nephrotoxicity was assessed in patients who did not receive nephrotoxic agents (n = 76). Ototoxicity was assessed in patients who completed two audiograms and were not receiving ototoxic agents (n = 63). No significant difference was found between the two groups for favourable clinical response: 47/51 (92.1%) and 49/52 (94.2%) in the od and bd groups, respectively. In 34 patients vancomycin was the only effective antibiotic. Fifteen of 18 (83.3%) evaluated episodes in the od and 12/16 (75.0%) evaluated episodes in the bd group showed a favourable bacteriological response. There were no significant differences between the od and bd groups for all adverse events. Nephrotoxicity developed in 4/37 (10.8%) and 3/39 (7.7%) patients, respectively. Hearing loss developed in 1/31 (3.2%) and 5/32 (15.6%). Phlebitis occurred in 7/51 (13.7%) and 12/52 (23.0%). Red man syndrome occurred in 7/51 (13.7%) and 5/52 (9.6%) in od and bd groups, respectively. The efficacy and safety profile of od administration of vancomycin is similar to that of the customary, but less convenient, bd administration.