A Parent-child yoga intervention for reducing attention deficits in children with congenital heart disease: the Yoga for Little Hearts Feasibility Study Protocol.

INTRODUCTION: Preschoolers and school-aged children with congenital heart disease (CHD) are at higher risk of attention deficit hyperactivity disorder (ADHD) compared with the general population. To this day, no randomised controlled trial (RCT) aiming to improve attention has been conducted in young children with CHD. There is emerging evidence indicating that parent-child yoga interventions improve attention and reduce ADHD symptoms in both typically developing and clinical populations. METHODS AND ANALYSIS: This is a single-blind, two-centre, two-arm trial during which 24 children with CHD and their parents will be randomly assigned to (1) a parent-child yoga intervention in addition to standard clinical care or (2) standard clinical care alone. All participants will undergo standardised assessments: (1) at baseline, (2) immediately post-treatment and (3) 6 months post-treatment. Descriptive statistics will be used to estimate the feasibility and neurodevelopmental outcomes. This feasibility study will evaluate: (1) recruitment capacity; (2) retention, drop-out and withdrawal rates during the yoga programme and at the 6-month follow-up; (3) adherence to the intervention; (4) acceptability of the randomisation process by families; (5) heterogeneity in the delivery of the intervention between instructors and use of home-based exercises between participants; (6) proportion of missing data in the neurodevelopmental assessments and (7) SD of primary outcomes of the full RCT in order to determine the future appropriate sample size. ETHICS AND DISSEMINATION: Ethical approval has been obtained by the Research Ethics Board of the Sainte-Justine University Hospital. The findings will be disseminated in peer-reviewed journals and conferences and presented to the Canadian paediatric grand round meetings. TRIAL REGISTRATION NUMBER: NCT05997680.

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.

Gross Motor Development of Children with Congenital Heart Disease Receiving Early Systematic Surveillance and Individualized Intervention: Brief Report.

Purpose: This retrospective study aims to describe the gross motor development of children aged 4 to 24 months with congenital heart disease (CHD) enrolled in a systematic developmental follow-up program and to describe the frequency of physical therapy sessions they received between 4 and 8 months of age. Methods: Twenty-nine infants with CHD underwent motor evaluations using the AIMS at 4 months, and the Bayley-III at 12 and 24 months. Results: Based on AIMS, 79% of 4-month-old infants had a gross motor delay and required physical therapy. Among these, 56.5% received one to two physical therapy sessions, and 43.5% received three to six sessions. Infants who benefited from regular interventions tended to show a better improvement in motor scores from 12 to 24 months. Conclusion: This study highlights the importance of early motor screening in infants with CHD and suggests a potential benefit of early physical therapy in at-risk children. Abbreviations: CHD: Congenital heart disease; AIMS: Alberta Infant Motor Scales; Bayley-III: Bayley Scales of Infant and Toddler Development, Third edition; Bayley-III/GM: Gross Motor section of the Bayley Scales of Infant and Toddler Development, Third edition.

Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. OBJECTIVES: This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission). METHODS: The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression. RESULTS: Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index. CONCLUSIONS: In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.

Transvenous Lead Extraction in Adults With Congenital Heart Disease: Insights From a 20-Year Single-Center Experience.

BACKGROUND: Safety and feasibility data on transvenous lead extraction (TLE) in the challenging population of adults with congenital heart disease (A-CHD) are limited. Herein, we report the results of TLE in A-CHD during a 20-year period. METHODS AND RESULTS: All consecutive TLE procedures in A-CHD were included in a monocentric prospective registry from 1996. A total of 121 leads were extracted in 49 A-CHD (median age, 38 years; 51% men) during 71 TLE procedures. Twenty-four (49%) patients had transposition of the great arteries. Main indications for extraction were infection in 34 (48%) and lead failure in 22 (31%). A laser sheath was required for 56 (46%) leads and a femoral approach for 10 (8%). Complete TLE was achieved for 111 leads (92%). In multivariable analysis, lead duration (odds ratio, 1.02; 95% confidence interval, 1.00-1.04; P<0.01) and number of previous cardiac surgeries (odds ratio, 2.65; 95% confidence interval, 1.52-4.67; P<0.01) were predictive of TLE failure. No perioperative death or pericardial effusion was observed. Subpulmonary atrioventricular valve regurgitation increased in 8 patients (5 with transposition of the great arteries) and was independently associated with an implantable cardioverter defibrillator lead (odds ratio, 9.69; 95% confidence interval, 1.31-71.64; P=0.03) and valvular vegetation (odds ratio, 7.29; 95% confidence interval, 1.32-40.51; P=0.02). After a median of 54 (19-134) months of follow-up after the first TLE, 3 deaths occurred independently from lead management. CONCLUSIONS: Despite complex anatomic issues, TLE can be achieved successfully in most A-CHD using advanced extraction techniques. Subpulmonary atrioventricular valve regurgitation is a prevalent complication, particularly in patients with transposition of the great arteries.

Successful repair of a bicuspid aortic valve with anomalous chordal attachment to the aortic wall.

The congenitally bicuspid aortic valve with anomalous chordal attachment of the fused cusp to the aortic wall is a rare and unappreciated cause of aortic insufficiency. We report the case of a 43-year-old male patient who presented with severe aortic insufficiency caused by this anomaly and in whom surgical aortic valve repair was successfully performed. To our knowledge, this is the first report to describe the successful surgical repair of this rare variant of bicuspid aortic valve.

What is the role of the Ross procedure in today's armamentarium?

The Ross procedure, which consists of replacing the aortic root with a pulmonary autograft, is the only surgery that ensures long-term viability of the aortic valve substitute. The presence of a living valve in the aortic position was theorized to translate into improved clinically relevant outcomes. This has now been confirmed by a number of recently published studies, which highlight the benefits associated with this surgery in terms of survival, quality of life, hemodynamic characteristics, and freedom from valve-related complications. Nevertheless, despite the evidence, the Ross procedure remains largely underused and has even been abandoned by a large number of groups based on some real and sometimes justifiable concerns, and a number of unfounded biases. The aim of this article is to provide a comprehensive, evidence-based review of the current literature pertaining to the Ross procedure, with an emphasis on the rationale, results, technical key points, alternatives, and future directions.

Retrospective cohort analysis of 926 tricuspid valve surgeries: clinical and hemodynamic outcomes with propensity score analysis.

BACKGROUND: The objectives were to describe morbidity and mortality after tricuspid valve (TV) surgery, to compare outcomes after repair versus replacement, and to assess risk factors for mortality and tricuspid regurgitation (TR) recurrence. METHODS: A retrospective cohort study including 926 consecutive cases of TV surgery (792 repairs and 134 replacements) performed at the Montreal Heart Institute was conducted. Median follow-up was 4.3 years (4,657 patient-years). Median age was 62 years (interquartile range 53-69 years), and 72% of patients were women. RESULTS: Operative mortality was 14% (128 patients: 1977-1998 20%, 1999-2008 7%, P < .001). Independent risk factors for operative mortality in the 1999 to 2008 period were hypertension (odds ratio [OR] 6.03, P = .02), daily furosemide dose (by 10 mg) (OR 1.06, P = .05), weight (by 10 kg) (OR 0.36, P < .01), and cardiopulmonary bypass time (by 10 minutes) (OR 1.29, P < .001). Ten-year survival was 49% ± 2% and 38 ± 5% in the repair and replacement groups, respectively (P = .012). At discharge, severity of TR was ≥3/4 in 13% and 2% of patients in the repair and replacement groups, respectively (P = .01). Propensity score analysis showed that tricuspid repair was associated with higher rates of TR ≥3/4 at follow-up compared with replacement (hazard ratio 2.15, P = .02). Forty-eight reoperations (7% of patients at risk) were performed during follow-up (repair group, 6%; replacement group, 15%; P = .01). At last follow-up, New York Heart Association functional class was improved compared with baseline in both groups (P < .001). CONCLUSION: Tricuspid valve surgery is associated with substantial early and late mortalities but with significant functional improvement. Replacement is more effective in early and late corrections of regurgitation, but it does not translate into better survival outcomes.

Treatment of isolated ventricular septal defects in children: Amplatzer versus surgical closure.

BACKGROUND: Isolated hemodynamically significant ventricular septal defects (VSD) were previously treated surgically. Since the introduction of percutaneous (PC) devices, the management of isolated VSD has evolved. In our center, Amplatzer devices have been implanted for selected isolated perimembranous VSD since 2002. METHODS: The charts of all isolated PC perimembranous VSD closures and all surgical closures performed since 2002 were reviewed retrospectively. Clinical, electrocardiographic, and echocardiographic data were analyzed. The preclosure, immediate postclosure, and 1-month, 6-month, and 12-month postclosure results were assessed. RESULTS: Thirty-seven patients underwent PC closure, and 34 had surgical treatment. Mean follow-up was 42.1 ± 26.0 months. The PC group was significantly older (p < 0.01) and larger in size (p < 0.001). Surgical patients had more severe congestive heart failure and a significantly lower VSD gradient (p < 0.004). At follow-up, there were no differences in the incidence of residual shunting between the two groups (p = 0.92). All valvular regurgitations improved over time, except for 3 aortic regurgitations (5.4%) in the PC group that got worse. Two permanent pacemakers were implanted for early complete heart block in the PC group, and one was implanted in the surgical group (p = 0.94). CONCLUSIONS: The surgical results in our population were excellent. The selection of patients with perimembranous VSD remains a challenge to avoid post-PC intervention complications such as heart block and aortic insufficiency. For isolated VSD, PC closure, which avoids the morbidity of open heart surgery, should be considered as part of the therapeutic armamentarium.

Clinical assessment of arrhythmias in tetralogy of Fallot.

As a major success story of an early surgical era, corrective surgery transformed the prognosis of patients born with tetralogy of Fallot. With lifelong care, healthy survival well into adulthood is now the norm, albeit somewhat blemished by long-term sequelae. Arrhythmias are an important source of morbidity and a leading cause of mortality. This succinct review provides a contemporary summary of the prevalence and types of arrhythmias encountered in tetralogy of Fallot, including conduction disorders, atrial and ventricular tachyarrhythmias, and sudden death. Current prognostic markers and existing diagnostic tools are discussed, and available therapeutic options are elaborated.

Safety and efficiency of recombinant activated factor VII in postcardiotomy massive hemorrhage.

Postoperative massive hemorrhage is a difficult clinical situation after cardiac surgery. Recombinant activated factor VII (rf-VIIa) can be a useful adjunct to surgical hemostasis and blood product transfusion. Four cases of massive hemorrhage treated with rf-VIIa after complex cardiac surgery are reported. A review of the literature and possible guidelines for the use of rf-VIIa in cardiac surgery are provided.

Late myocardial revascularization in patients with tetralogy of Fallot.

Since surgical correction of congenital heart diseases began in the early 1950s, a growing number of patients are reaching older age and becoming a risk for developing coronary artery disease. This study presents the clinical experience of myocardial revascularization in 4 symptomatic coronary artery disease patients with unrepaired or corrected tetralogy of Fallot. Three patients had surgery and 1 patient had percutaneous angioplasty. One corrected tetralogy of Fallot patient developed symptomatic coronary artery disease and responded favorably to medical therapy.

Cardiac transplantation for hypertrophic cardiomyopathy: a valid therapeutic option.

BACKGROUND: Hypertrophic cardiomyopathy is a rare indication for cardiac transplantation, with only anecdotal reports in the literature. Transplantation has been proposed to patients with hypertrophic cardiomyopathy who remained symptomatic despite optimal medical treatment or who progressed to congestive heart failure, and when conventional surgical correction was not or was no longer possible. In this report, we analyze the clinical results of cardiac transplantation in these patients. METHODS: We retrospectively reviewed complete clinical data and prospectively gathered complete follow-up data for 14 patients who underwent orthotopic cardiac transplantation for hypertrophic cardiomyopathy at the Montreal Heart Institute and Ste-Justine Hospital between 1984 and 2001. RESULTS: Ten male (71.4%, 5 adults and 5 children) and 4 female (28.6%, 2 adults and 2 children) patients underwent heart transplantation for hypertrophic cardiomyopathy. The median age of the recipients in the pediatric group was 13 years (range, 6-16) and was 40 years (range, 22-46) in the adult group. Median duration of follow-up was 9.5 years (mean, 8.8 +/- 4.8 years; range, 1-18) and was 100% complete. We found no operative mortality and found 2 late deaths related to coronary graft atherosclerosis. Long-term survival at 5, 10, and 15 years was 100%, 85%, and 64%, respectively. Freedom from acute rejection at 1, 5, and 10 years was 73%, 32%, and 9%, respectively. The remaining 11 survivors exhibit few symptoms and are currently in New York Heart Association Class I or II. CONCLUSIONS: Cardiac transplantation is a valid therapeutic option for patients with symptomatic hypertrophic cardiomyopathy who do not respond to optimal medical management and who are not candidates for conventional surgical treatment. The long-term outcome is excellent in these patients.

Selective timing for the arterial switch operation.

BACKGROUND: To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status. METHODS: A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. RESULTS: From July 1993-April 2001, 117 infants underwent an arterial switch operation before 90 days of age. Seventy-five patients (64%) had transposition of the great arteries with intact ventricular septum with the aim of operation before 14 days of age; however, 8 of these patients had delayed presentation (range 15-46 days). Thirty-five patients (30%) had transposition with a ventricular septal defect (30 patients) or double outlet right ventricle (5 patients) and normal arch anatomy and were repaired within the first 90 days of life depending on the severity of heart failure at a median of 12 days of age (range 3-83 days). Seven patients (6%) had associated aortic coarctation (5 patients) or interrupted aortic arch (2 patients). One patient died during hospitalization (0.85% hospital mortality) and one patient died from noncardiac causes during a median follow-up of 35 months (1.7% total mortality). Four patients required intervention during follow-up (3.4%) for new aortic coarctation (2 patients), supravalvar pulmonic stenosis (1 patient), or right hemi-diaphragm paralysis (1 patient). CONCLUSIONS: Individualized timing for the arterial switch operation within the first ninety days of life produces excellent survival rates for all types of transposition physiology with the expectation of a satisfactory course during follow-up.

Long-term results of left ventricular reconditioning and anatomic correction for systemic right ventricular dysfunction after atrial switch procedures.

OBJECTIVES: Systemic right ventricular failure after atrial switch procedures for transposition of the great arteries has been addressed at Melbourne's Royal Children's Hospital (1981-1993) and the Cleveland Clinic Foundation (1993-2001) with reconditioning of the morphologically left ventricle by means of pulmonary artery banding followed by an arterial switch operation and an atrial reseptation. METHODS: Thirty-nine patients (Royal Children's Hospital, 19; Cleveland Clinic Foundation, 20) with a median age of 10.8 years (range, 13 months-24 years) entered this protocol a median of 10.3 years (range, 0.5-24 years) after an atrial switch procedure. RESULTS: The median duration of pulmonary artery banding was 13 months (range, 0.5-5.4 years). Ten (28%) patients responded unfavorably to morphologically left ventricular reconditioning (5 mortalities: 4 transplantations and 1 PAB still in place). Twenty-four (83%) of the 29 patients who underwent an atrial switch operation and atrial reseptation survived. During a median follow-up period of 8.2 years (range, 1-16 years), 3 patients had cardiac-related deaths. All 18 long-term survivors are asymptomatic. At last echocardiographic evaluation, the morphologically left ventricular function was normal or mildly decreased in 16 (89%) patients, and all had normal or mildly decreased systemic right ventricular function with no or mild tricuspid regurgitation. Age greater than 12 years was associated with a greater probability of morphologically left ventricular failure and not completing the protocol (P =.02) and a higher operative mortality at anatomic correction (P =.02). CONCLUSIONS: Morphologically left ventricular reconditioning and an anatomic correction protocol should be integrated into a cardiac transplantation program when treating patients with morphologically right ventricular failure after Mustard and Senning procedures. It is an alternative to cardiac transplantation in selected patients, with good long-term results. The response to morphologically left ventricular reconditioning past adolescence is inconsistent.

Tricuspid valve replacement: an analysis of 25 years of experience at a single center.

BACKGROUND: Tricuspid valve replacement is seldom used in clinical practice, but the choice between mechanical and biologic prostheses remains controversial. METHODS: Between 1977 and 2002, 97 patients underwent tricuspid valve replacement and were followed at the Montreal Heart Institute Valve Clinic. Patients underwent replacement with bioprostheses (n = 82) and mechanical valves (n = 15). RESULTS: Patients with bioprosthetic tricuspid replacements averaged 53 +/- 13 years of age compared with 48 +/- 11 years in those with tricuspid mechanical valve replacements (p = 0.2). Isolated tricuspid valve replacement was performed in 11 patients (73%) in the mechanical valve group compared with 31 patients (38%. p = 0.01) in the bioprosthetic replacement group. In patients undergoing bioprosthetic tricuspid replacement, 51 (62%) underwent multiple associated valve replacements. The 5-year survival after tricuspid replacement averaged 60% +/- 13% in the mechanical valve group and 56% +/- 6% in the biologic replacement group (p = 0.8). The 5-year freedom rate from tricuspid valve reoperation averaged 91% +/- 9% in patients with mechanical valves and 97% +/- 3% in those with biologic valves (p = 0.2). CONCLUSIONS; Patient survival after tricuspid valve replacement is suboptimal but related to the clinical condition at operation. The use of biologic prostheses for tricuspid valve replacement remains a good option in young patients because of limited life expectancy unrelated to the type of tricuspid prostheses at long-term follow-up.

Long-term results with triple valve surgery.

BACKGROUND: Whether to use biological or mechanical prostheses and whether to repair or replace the tricuspid valve during primary and reoperative triple valve surgery remains controversial. The objective of the present study was to review our experience with primary and reoperative triple valve surgery using CarboMedics (CM) and Carpentier-Edwards (C-E) heart valves. METHODS: All 73 patients undergoing triple valve surgery since 1982 were prospectively followed at the Montreal Heart Institute valve clinic. Aortic valve replacement was performed with CM prostheses (57 patients) and with C-E prostheses (16 patients). Mitral valve replacement was performed with mechanical prostheses (56 patients) and with biological valves (14 patients). Mitral valve repair was done in 3 patients. Tricuspid valve annuloplasty or commissurotomy or both were performed in 66 patients and the tricuspid valve was replaced in 7 patients. Patient survival, complications, and the type of valve procedures were analyzed. RESULTS: Thirty patients averaging 62+/-10 years of age underwent primary triple valve surgery and 43 patients averaging 60+/-10 years of age underwent reoperative triple valve surgery (p = 0.5). Tricuspid repair consisted of annuloplasty with the Bex linear reducer (n = 47), the C-E ring (n = 13), or the De Vega technique (n = 5). Tricuspid valve replacement was done using the C-E pericardial prostheses. The 30-day mortality was 17% and 12% in patients with primary and reoperative surgery, respectively (p = 0.5) and patient survival averaged 80%+/-7%, 75%+/-8%, and 41%+/-15%, and 70%+/-7%, 57%+/-9%, and 50%+/-10%, respectively 1, 5, and 10 years following surgery (p = 0.5). The freedom rate from thromboembolism and from bleeding complications were 87%+/-6% and 95%+/-3% in primary and reoperative patients, respectively, 5 years following surgery. CONCLUSIONS: Triple valve surgery, either as a primary or a reoperative procedure, results in acceptable long-term survival with both mechanical and biological prostheses.

Left ventricular reconditioning and anatomical correction for systemic right ventricular dysfunction.

The morphologically right ventricle (mRV) fails after managing systemic workload for a variable period of time in some patients with transposition of the great arteries (TGA) who have undergone an atrial switch operation and in patients with congenitally corrected transposition of the great arteries (cc-TGA). Conventional therapy for progressive mRV failure, including tricuspid valve replacement and cardiac transplantation, has been disappointing. Anatomical correction, reincorporating the morphologically left ventricle (mLV) into systemic circulation, was performed in a total of 84 patients (39 TGA, 45 cc-TGA) in 2 institutions (Royal Children Hospital 1981-1993; Cleveland Clinic Foundation 1993-1999). The mVL was retrained to generate systemic pressure by means of pulmonary artery band in 43 patients. The overall mortality for patients entering this program is 15.4% (8 early and 5 late operative deaths). All of the operative deaths were patients with TGA and a prior atrial switch operation. Four patients (5%) have been transplanted or are on a transplantation waiting list. In patients with TGA and previous atrial switch operation, older age and abnormal coronary anatomy was associated with a higher operative mortality. None of the measured parameters of the mLV and mRV were predictors of failure of mLV reconditioning. Follow-up echocardiographic evaluations of the 46 survivors of anatomical correction showed normal mRV function in 41 (89%) and normal mLV function in 42 patients (91%). mLV retraining and anatomical correction produces good results in prepubescent patients. The response of older patients is less predictable and associated with a higher early and late mortality. Copyright 2000 by W.B. Saunders Company