"Fontan Conduit Stent-Angioplasty and Progression of Fontan-Associated Liver Disease".

Patients with Fontan circulation insidiously develop congestive hepatopathy related to chronically reduced cardiac output and central venous hypertension, also known as Fontan-associated liver disease (FALD). Fontan pathway obstruction is increasingly detected and may accelerate FALD. The impact of conduit stent angioplasty on FALD is unknown. Retrospective, single-center review of patients with Fontan circulation who underwent conduit stent angioplasty at cardiac catheterization over 5-year period. Demographics and cardiac histories were reviewed. Labs, liver ultrasound elastography, echocardiogram, hemodynamic and angiographic data at catheterization were recorded pre- and post-stent angioplasty. Primary outcome was change in hepatic function via MELD-XI scores and liver stiffness (kPa), with secondary outcomes of ventricular function, BNP, and repeat catheterization hemodynamics. 33 patients underwent Fontan conduit stent angioplasty, 19.3 ± 7.0 years from Fontan operation. Original conduit diameter was 19.1 ± 1.9 mm. Prior to angioplasty, conduit size was reduced to a cross-sectional area 132 (91, 173) mm2 and increased to 314 (255, 363) mm2 post-stent. Subjects' baseline median MELD-XI of 11 (9, 12) increased to 12 (9, 13) at 19 ± 15.5 months post-angioplasty (n = 22, p = 0.053). There was no significant change in liver stiffness at 12.1 ± 8.9 months post-angioplasty (n = 15, p = 0.13). Median total bilirubin significantly increased (1.4 [0.9, 1.8]), from baseline 1.1 [0.7, 1.5], p = 0.04), as did median BNP (41 [0, 148] from baseline 34 [15, 79]; p = 0.02). There were no significant changes in ventricular function or repeat invasive hemodynamics (n = 8 subjects). Mid-term follow-up of Fontan subjects post-conduit stent angioplasty did not show improvements in non-invasive markers of FALD.

Design of a multi-institutional neurocognitive discovery study in adult congenital heart disease (MINDS-ACHD).

BACKGROUND: Neurocognitive dysfunction (NCD) is a common comorbidity among children with congenital heart disease (CHD). However, it is unclear how underlying CHD and its sequelae combine with genetics and acquired cardiovascular and neurological disease to impact NCD and outcomes across the lifespan in adults with CHD. METHODS: The Multi-Institutional Neurocognitive Discovery Study in Adults with Congenital Heart Disease (MINDS-ACHD) is a partnership between the Pediatric Heart Network (PHN) and the Adult Alliance for Research in Congenital Cardiology (AARCC) that examines objective and subjective neurocognitive function and genetics in young ACHD. This multicenter cross-sectional pilot study is enrolling 500 young adults between 18 and 30 years with moderate or severe complexity CHD at 14 centers in North America. Enrollment includes 4 groups (125 participants each): (1) d-looped Transposition of the Great Arteries (d-TGA); (2) Tetralogy of Fallot (TOF); (3) single ventricle (SV) physiology; and (4) "other moderately or severely complex CHD." Participants complete the standardized tests from the NIH Toolbox Cognitive Battery, the NeuroQoL, the Hospital Anxiety and Depression Scale, and the PROMIS Global QoL measure. Clinical and demographic variables are collected by interview and medical record review, and an optional biospecimen is collected for genetic analysis. Due to the COVID-19 pandemic, participation may be done remotely. Tests are reviewed by a Neurocognitive Core Laboratory. CONCLUSIONS: MINDS-ACHD is the largest study to date characterizing NCD in young adults with moderate or severely complex CHD in North America. Its results will provide valuable data to inform screening and management strategies for NCD in ACHD and improve lifelong care.

A transition clinic intervention to improve follow-up rates in adolescents and young adults with congenital heart disease.

BACKGROUND: Children with congenital heart disease (CHD) require lifelong cardiology follow-up. Many experience gaps in care around the age of transition to adult-oriented care with associated comorbidity. We describe the impact of a clinic-based intervention on follow-up rates in this high-risk population. METHODS: Patients ≥11 years seen in a paediatric outpatient CHD Transition Clinic completed self-assessment questionnaires, underwent focused teaching, and were followed on a clinic registry with assessment of care continuation. The cohort "lost to follow-up" rate, defined as absence from care at least 6 months beyond the recommended timeframe, was compared with a control group. Secondary outcomes included questionnaire scores and adult cardiology referral trends. RESULTS: Over 26 months, 53 participants completed an initial Transition Clinic visit; 43% (23/53) underwent a second visit. Median participant age was 18.0 years (interquartile range 16.0, 22.0). The cohort's "lost to follow-up" rate was 7.3%, which was significantly lower than the control rate (25.9%, p < 0.01). Multivariable regression analyses demonstrated clinic participation as the only factor independently associated with follow-up rates (p = 0.048). Transition readiness was associated with older age (p = 0.01) but not sex, univentricular heart, interventional history, or surgical complexity. One-third of adult participants transferred to adult care. CONCLUSIONS: A CHD Transition Clinic intervention can improve follow-up rates in adolescents and young adults. Age is an important factor in transition readiness, and retention of adults in paediatric care appears multi-factorial. We postulate that serial assessments of self-management, focused education, and registry utilisation may improve patient outcomes by reducing lapses in care.

QRS fragmentation versus QRS prolongation in predicting right ventricular enlargement and dysfunction in children and adults with repaired Tetralogy of Fallot.

Patients with repaired Tetralogy of Fallot (rTOF) have risks of late life-threatening sequelae, including right ventricular (RV) dilation and failure, arrhythmias, and sudden death. QRS prolongation is a well-known ECG predictor of these outcomes but has poor sensitivity for mortality. Growing evidence demonstrates QRS fragmentation (fQRS) as a better prognostic marker for mortality in adults with rTOF, though the two markers have not been directly compared as correlates for CMR abnormalities. Additionally, fQRS has never been studied in pediatric TOF. This single institution retrospectively reviewed 138 CMRs in rTOF patients (median age 21.7 years) who had a corresponding 12-lead ECG within 1 year. fQRS was defined as ≥3 R-waves/notches in the R/S complex (>2 in right bundle branch block) in ≥2 contiguous leads. QRS prolongation was defined as QRS ≥160 ms. Nearly half (46%) the sample had fQRS (42.1% of pediatric subgroup), and 26% had QRS prolongation. Both markers were significantly associated with reduced RV ejection fraction (EF%) (p < 0.01) and larger RV end-diastolic volumes (p < 0.01). QRS prolongation alone predicted lower LV EF% (p = 0.02). Regression analyses showed both QRS prolongation (p < 0.01) and fQRS (p < 0.01) independently associated with reduced RV EF%; QRS prolongation alone predicted RV dilation (p < 0.01). We concluded that both QRS prolongation and fQRS are equivalent as significant markers of RV dysfunction in rTOF patients. QRS prolongation may be a better surrogate for RV dilation specifically. fQRS was frequently seen in children with rTOF and was significantly associated with similar late structural sequelae.

Diastolic Dysfunction With Preserved Ejection Fraction After the Fontan Procedure.

Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross-sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e'/end-diastolic volume) was >90th percentile (≥0.26 mL-1). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO2. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P<0.01. DD patients had lower predicted peak VO2 compared with controls (52% [20] versus 67% [23], P<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7), P<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients.

Hemodynamic Impact of MitraClip Procedure for Systemic Tricuspid Regurgitation in Congenitally Corrected Transposition of Great Arteries: A Case Report.

Patients with congenitally-corrected transposition of the great arteries (ccTGA) commonly develop significant systemic tricuspid valve regurgitation and systemic right ventricular dysfunction in adulthood, both of which presenting a therapeutic dilemma for the care team. Here we describe the case of a 35-year-old male with congenitally-corrected transposition of the great arteries who presented with severe systemic tricuspid valve regurgitation, biventricular systolic failure, and pulmonary hypertension. Due to prohibitive surgical risk, he underwent percutaneous tricuspid valve repair via MitraClip placement. Post-procedure, he demonstrated rapidly improved symptoms and sustained echocardiographic and hemodynamic evaluations. Few reports exist describing the safety and feasibility of the MitraClip procedure on a systemic tricuspid valve, but to our knowledge, this is the first to describe invasive hemodynamic improvements in patients with this degree of cardiopulmonary sequelae from the congenital lesion. There may be optimism for the MitraClip procedure as "bridge to list" in patients with ccTGA otherwise initially ineligible for surgical valve intervention or transplant.

Imaging in Adult Congenital Heart Disease.

There has been tremendous growth in the population of adults with congenital heart disease (CHD) over the last few decades because of advances in medical care. Whereas some cases are cured during childhood, most patients instead undergo palliation, which leaves them at risk for late complications. Lifelong clinical follow-up involving serial multimodality imaging is helpful to monitor and guide the treatment of late complications. Imaging of these individuals is challenging because of their unique anatomy and therefore requires careful consideration on a case-by-case basis. Knowledge of late complications associated with various diseases and surgical palliations provides a basis for selection and interpretation of an appropriate imaging study. This review highlights the most common conditions in adult CHD and the clinical questions that imaging hopes to answer, as well as the advantages and disadvantages of available imaging modalities. We illustrate clinical scenarios and the use of noninvasive imaging modalities including echocardiography, computed tomography, and magnetic resonance imaging, as well as applications in the following CHDs: tetralogy of Fallot, transposition of the great arteries, coarctation of the aorta, shunts, functional single ventricle, and coronary anomalies.