Balloon valvuloplasty for annular- and leaflet-level tricuspid stenosis: Report of cases and literature review.

Congenital tricuspid valve stenosis is extremely rare. We describe 2 cases of patients with adult congenital heart disease with hypoplastic tricuspid valve annulus who were symptomatic from annular- and leaflet-level tricuspid stenosis. The patients underwent transcatheter balloon valvuloplasty with good clinical outcomes. An extensive literature review and analysis of various procedural strategies suggests that percutaneous balloon valvuloplasty may be a reasonable therapeutic choice as a first-line therapy or when open surgical repair is associated with prohibitively high mortality. This procedure can be performed either as a destination therapy or as a bridge to valve replacement.

QRS Duration After Pulmonary Valve Replacement in Adults with Repaired Tetralogy of Fallot: Association with Ventricular Arrhythmia and Correlation with Right Ventricular Size.

The aim of this study is to analyze the relationship between QRS duration after pulmonary valve replacement (PVR) and ventricular arrhythmias (VA) in patients with repaired tetralogy of Fallot (ToF). ToF patients may face complications such as heart failure and VA after primary repair, often mitigated by PVR. Prior studies have shown a decrease in QRS duration and right ventricular (RV) size following PVR. It remains unclear whether a lack of QRS duration reduction identifies patients at risk of VA. We retrospectively identified adult patients with repaired ToF who underwent surgical or transcatheter PVR. EKG data (pre-PVR, 30 days to 1-year post-PVR, and closest to CMR) was collected. The primary endpoint was sustained ventricular tachycardia (VT), ICD shock for sustained VT, or inducible VT on EP study. 85 patients were included (median follow-up 3.6 years; median age 34 years; 51% females). The primary outcome was noted in 8 patients. Mean QRS duration decreased by 5 ms following PVR (p = 0.0001). Increased age at PVR, QRS ≥ 180 ms post-PVR, no reduction in QRS after PVR, and a history of VT were associated with higher risk of the primary endpoint. The change in QRS was linearly correlated with the change in RVEDVi (R = 0.66). Adults with repaired ToF experience a reduction in QRS duration post-PVR that correlates with the change of the RV size. Patients with QRS ≥ 180 ms post-PVR, no reduction in QRS, increased age at repair, and a history of VT are at risk for recurrent VT and warrant closer monitoring/ICD consideration.

Kidney disease and congenital heart disease: Partnership for life.

The literature on the relationship between kidney and cardiovascular diseases is continuously expanding. Scientists have elucidated many of the neurohormonal and hemodynamic pathways involved in cardiorenal disease. However, little is known about kidney disease in patients with congenital heart disease. Given advances in the medical and surgical care of this highly complex patient population, survival rates have dramatically improved leading to a higher percentage of adults living with congenital heart disease. Accordingly, a noticeable increase in the prevalence of kidney disease is appreciated in these patients. Some of the main risk factors for developing chronic kidney disease in the adult congenital heart disease population include chronic hypoxia, neurohormonal derangements, intraglomerular hemodynamic changes, prior cardiac surgeries from minimally invasive to open heart surgeries with ischemia, and nephrotoxins. Unfortunately, data regarding the prevalence, pathophysiology, and prognosis of chronic kidney disease in the adult congenital heart disease population remain scarce. This has led to a lack of clear recommendations for evaluating and managing kidney disease in these patients. In this review, we discuss contemporary data on kidney disease in adults with congenital heart disease in addition to some of the gaps in knowledge we face. The article highlights the delicate interaction between disease of the heart and kidneys in these patients, and offers the practitioner tools to more effectively manage this vulnerable population.

Subjective and Objective Impact of Angiotensin Receptor-Neprilysin Inhibitors on Systemic Right Ventricle Patients.

INTRODUCTION: Patients with adult congenital heart disease (ACHD) who have an anatomic right ventricle supporting the systemic circulation have increased mortality and morbidity from heart failure (HF). Angiotensin receptor-neprilysin inhibitors (ARNI) have emerged as a standard of therapy for adults with HF. However, the effects of this therapy have not been extensively studied in ACHD patients, especially those with systemic right ventricle (SRV). HYPOTHESIS: ARNIs are associated with subjective and objective improvement in SRV patients. METHODS: Eighteen (18) SRV patients were prescribed ARNI at our institution in the last 5 years. Data before and during treatment, including demographics, medical history, New York Heart Association functional class (NYHA FC), labs, cardiac computed tomography (CT) or magnetic resonance imaging (MRI), echocardiographic measurements, cardiopulmonary stress test (CPET), and hospitalisation for HF were obtained by review of the electronic medical record. Statistical analysis was performed using paired t and Wilcoxon rank sum tests. RESULTS: Eighteen (18) SRV patients (mean age 40 yrs, 72% male) were treated with ARNI (median duration 13 mo) in addition to other HF medications. All patients tolerated ARNI without symptomatic or asymptomatic hypotension or worsening kidney function. High ARNI dose (97/103 mg) was achieved in three (17%) patients, and moderate (49/51 mg) in three (17%). At baseline, nine patients were NYHA FC 2, seven FC 3, and two FC 4. Mean baseline cardiopulmonary exercise testing (CPET) and echocardiographic data were: oxygen uptake (VO2) 18 mL/kg/min, minute ventilation/carbon dioxide (VE/VCO2) 38, right ventricular ejection fraction (RVEF) 32%, fractional area change (FAC) 21%. Significant tricuspid regurgitation was present in 33% (28% moderate, and 5% severe) and mean tricuspid annular plane systolic excursion (TAPSE) was 9.4 mm. With treatment, there was no statistically significant difference in blood pressure, labs, testing, or imaging. There was a statistically significant improvement in median NYHA FC (2 vs 2.5, p=0.005). When compared to an equal pre-ARNI median timeframe, there was a noted decrease in cardiac hospitalisation (4 vs 9) that did not reach statistical significance (p=0.313). CONCLUSION: In adult patients with failing systemic right ventricle, ARNI is safe and well tolerated. Their use is associated with improvement in functional status. Prospective studies on a larger group of patients are warranted to better understand the causes of this improvement.

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.

Case Report: Constrictive Pericarditis in a Patient With Isolated Anomalous Right Upper Pulmonary Venous Return.

Thirty-eight-year-old male presented for evaluation of abdominal swelling, lower extremity edema and dyspnea on exertion. Extensive work-up in search of the culprit etiology revealed the presence of an Anomalous Right Upper Pulmonary Venous Return (ARUPVR) into the Superior Vena Cava (SVC). During the attempted repair, the pericardium was found to be thickened and constrictive. Only one other case of co-existent partial anomalous pulmonary venous return and constrictive pericarditis (CP) has been reported. The patient underwent a warden procedure with pericardial stripping with good outcomes at 45 days post-operatively. Thus, the presence of severe heart failure symptoms in the setting of ARUPVR should prompt further investigations. Also, further cases are needed to help guide management in these patients.

Do we have the ACHD physician resources we need to care for the burgeoning ACHD population?

BACKGROUND: Delivery of care to the adult congenital heart disease (ACHD) population has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels. METHODS: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on the ACHA website were cross-referenced with ABIM to verify ACHD board certification status. RESULTS: There are 115 self-identified ACHD programs and 418 self-identified ACHD physicians listed in the ACHA website. There are 320 board-certified ACHD cardiologists in the United States today, including 161 not listed in the ACHA website. Regarding ratios of ACHD-certified physicians to patients, the best served metropolitan statistical area (MSA) is Raleigh-Cary, NC, and the worst served MSA is Riverside-San Bernardino-Ontario, CA. The best served State is Washington, DC, and the worst served State is Indiana. CONCLUSIONS: The ACHD population continues to grow, and the looming national physician shortage is likely to greatly affect the ability to meet the complex needs of this growing population. In order to bring the ACHD patient: physician ratio to 1000:1, a minimum of 170 additional ACHD board-certified physicians are needed now.

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary.

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.

Contemporary ACHD training and the reality of the field in the United States.

BACKGROUND: Care delivery for the growing population of adults living with congenital heart disease (CHD) has been met with challenges due to a shortage of physicians trained to care for this population. To meet this urgent need, restructuring and standardization of the training programs were implemented in 2015. The consequences of such a system on the graduating fellows have not been examined. METHODS: A 25-question electronic survey was distributed to early career physicians who graduated following training in adult CHD (ACHD) care between 2015 and 2017 and are currently practicing in the United States. The survey results were anonymous. RESULTS: Of the 30 physicians who trained in ACHD between 2015 and 2017 in the U.S., 21 (70%) responded to the survey. The majority completed a 2-year ACHD program, practice at an adult hospital, are happy with their current job, spend most of their time in ACHD-related activities, make on average around 250,000 USD for entry level positions, and prioritize supportive leadership and colleagues. Their training was adequate for their job requirements. However, the acquisition of an additional skill, in addition to clinical ACHD care, allowed them to secure a more ideal job. A sizeable number of jobs required program building or expansion with only 9.5% of trainees comfortable doing so immediately after graduation. CONCLUSIONS: The new ACHD training curriculum successfully meets most of the needs for ACHD jobs. Integration of specialty tracks, ensuring uniformity in the quality of training between programs, and promoting leadership skills may improve career prospects.

Relationship of Red Cell Distribution Width to Adverse Outcomes in Adults With Congenital Heart Disease (from the Boston Adult Congenital Heart Biobank).

Red cell distribution width (RDW), a measure of variability in red cell size, predicts adverse outcomes in acquired causes of heart failure. We examined the relation of RDW and outcomes in adults with congenital heart disease. We performed a prospective cohort study on 696 ambulatory patients ≥18years old enrolled in the Boston Adult Congenital Heart Disease Biobank between 2012 and 2016 (mean age 38.7 ± 13.5 years; 49.9% women). The combined outcome was all-cause mortality or nonelective cardiovascular hospitalization. Most patients had moderately or severely complex congenital heart disease (42.5% and 38.5%, respectively). Mean RDW was 14.0 ± 1.3%. RDW >15% was present in 81 patients (11.6%). After median 767days of follow-up, 115 patients sustained the primary combined outcome, including 31 who died. Higher RDW predicted both the combined outcome (hazard ratio [HR] for RDW >15% = 4.5, 95% confidence interval [CI] 3.0 to 6.6; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001) and death alone (HR for RDW >15% = 7.1, 95% CI 3.5 to 14.4; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001). RDW remained an independent predictor of the combined outcome after adjusting for age, cyanosis, congenital heart disease complexity, ventricular systolic function, New York Heart Association functional class, hemoglobin concentration, mean corpuscular volume, high-sensitivity C-reactive protein and estimated glomerular filtration rate (HR per + 1SD RDW = 1.5, 95% CI 1.2 to 1.9, p <0.0001). RDW also remained an independent predictor of mortality alone after adjustment for age plus each variable individually. In conclusion, elevated RDW is an independent predictor of all-cause mortality or nonelective cardiovascular hospitalization in adults with congenital heart disease. This simple clinical biomarker identifies increased risk for adverse events even among patients with preserved functional status.

The 745.5 issue in code-based, adult congenital heart disease population studies: Relevance to current and future ICD-9-CM and ICD-10-CM studies.

OBJECTIVE: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for "rule-out" congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age. DESIGN: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 were reviewed to validate the true incidence of an ASD. This observational, cross-sectional record review included patients between 11 and 64 years of age. RESULTS: Medical charts and echocardiograms of 190 patients (47.9% males) were reviewed. The number of falsely coded patients with 745.5 (no ASD) was high (76.3%). Forty-five (23.7%) patients had a true ASD. Among the 145 patients without an ASD, 100 (52.6%) were classified as having a PFO, 37 (19.5%) had a normal non-CHD echocardiogram, and 8 (4.2%) had some other CHD anomaly. The likelihood that 745.5 coded for a true ASD was higher in children aged 11-20 (64.3%) than adults aged 21-64 years (20.6%). CONCLUSIONS: This validation study demonstrates that 745.5 performed poorly across all ages. As 745.5 is widely used in population-level investigations and ICD-10-CM perpetuates the problem, future analyses utilizing CHD codes should consider separate analysis of those identified only through code 745.5.

The fluoro-less and contrast-less peripheral endovascular intervention: Halfway there.

INTRODUCTION: Percutaneous endovascular revascularization requires the use of fluoroscopic guidance and radiopaque contrast. We present a successful intervention without the use of iodinated contrast. CASE: A 92-year-old man with dry gangrene involving the second and fourth left toes had acute on chronic kidney injury. Arterial duplex showed severe stenosis in bilateral superficial femoral arteries (SFAs). Fluoroscopic and ultrasound guidance and intravascular imaging were used to avoid iodinated contrast. After right to left femoral crossover, the entire left SFA was imaged with ultrasound. The lesion was delineated with radiopaque measuring tapes then wired. Near-infrared spectroscopy and intravascular ultrasound (NIRS-IVUS) imaging were performed. Points of interest were correlated with corresponding radiopaque markings on the ruler. Stenting and post-dilation resulted in complete stent expansion and no evidence of dissection by IVUS. The total procedure time was 113min and the total radiation dose 813mGy. The day after the procedure, there was a palpable dorsalis pedis pulse. He was discharged to inpatient rehabilitation on dual antiplatelet therapy. DISCUSSION: Contrast and radiation continue to limit the feasibility of endovascular angiography and intervention. Carbon dioxide (CO2) digital subtraction angiography is an alternative for these patients but has several disadvantages. Previously proposed projects demonstrated the real potential of performing endovascular peripheral intervention without fluoroscopy or contrast. CONCLUSION: This case is a clear demonstration of a successful use of a combination of fluoroscopy, ultrasonography and intravascular imaging to achieve a successful endovascular intervention to treat critical limb ischemia, without the use of iodinated contrast.

The fluoro-less and contrast-less peripheral endovascular intervention: a concept for the future today.

INTRODUCTION: Percutaneous endovascular revascularization requires fluoroscopic guidance and radiopaque contrast use. This approach becomes problematic, especially in patients with advanced renal disease or allergies to iodinated contrast medium. The direct (exposure) and indirect (lead garment) burden of radiation affects patients and operators alike. PURPOSE: We propose a completely contrast-free, fluoroscopy-free approach to endovascular diagnostic arterial imaging and percutaneous intervention using available technologies, and outline a timeframe for its implementation. PROJECT DESCRIPTION/METHODOLOGY: Ultrasound imaging of the leg creates a roadmap of the vessel and identifies the lesion of interest. Device-based sensors using a low-powered electromagnetic field allow for wiring of the vessel. This is followed by the use of intravascular ultrasonography and near infrared spectroscopy to characterize the lesion dimensions and composition. After completion of the diagnostic phase of the process, the interventional portion with deployment of an angioplasty balloon and/or stent is performed using the electromagnetic field-guided sensors. FEASIBILITY: The project uses already available technologies. BENEFITS/ANTICIPATED OUTCOMES: This project demonstrates the real potential of performing endovascular peripheral intervention without fluoroscopy or contrast in a practical, user-friendly way with the currently available technology. The prospects in renal function preservation and radiation avoidance for both patients and operators are extremely attractive.

Red blood cell distribution width is a predictor of readmission in cardiac patients.

BACKGROUND: Three-quarters of rehospitalizations ($44 billion yearly estimated cost) may be avoidable. A screening tool for the detection of potential readmission may facilitate more efficient case management. HYPOTHESIS: An elevated red blood cell distribution width (RDW) is an independent predictor of hospital readmission in patients with unstable angina (UA) or non-ST-elevation myocardial infarction (NSTEMI). METHODS: The study is a retrospective observational cohort analysis of adults admitted in 2007 with UA or NSTEMI. Data were gathered by review of inpatient medical records. The rate of 30-day nonelective readmission and time to nonelective readmission were recorded until November 1, 2011, and compared by RDW group using the 95th percentile (16.3%) as a cutoff. RESULTS: The median follow-up time of the 503 subjects (average age, 65 ± 13 years; 56% male) was 3.8 years (interquartile range: 0.3-4.3 years). Those readmitted within 30 days were older, had more comorbidities and higher RDW and creatinine levels, and were more likely to have had an intervention. At 3.8 years of follow-up, subjects with high RDW (>16.3%) were more likely to be readmitted compared to those with normal RDW (≤16.3%) (72.28% vs 59.95%, P = 0.003). In multivariable analyses, high RDW was a statistically significant predictor of readmission in general (hazard ratio: 1.35 (95% confidence interval [CI]:1.02-1.79), P = 0.033) but not of 30-day rehospitalization (odds ratio: 1.34 (95% CI: 0.78-2.31), P = 0.292). Its area under the receiver operating characteristic curve was 0.54 (sensitivity 23% and specificity 85%). CONCLUSIONS: An elevated RDW is an independent predictor of hospital readmission in patients with UA or NSTEMI.