TGA + IVS + LVOTO: patterns of practice and outcomes.

PURPOSE: Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). METHODS: All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected. RESULTS: These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8). CONCLUSION: TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.

Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Steroids as a possible effective therapy in the management of large isolated chylopericardium following open heart surgery.

BACKGROUND: Chylopericardium is the collection of lymph fluid inside the pericardial cavity. The incidence of chylopericardium is very low, as this diagnosis is rarely reported following cardiac procedures in children. While some reports were published worldwide on isolated chylopericardium after cardiac surgeries for diverse reasons, it has never been reported after repair for partial anomalous pulmonary venous return. In addition, management of this diagnosis ends up being surgical with minimal concentration on medical treatment which proved unsuccessful. We present a medical approach with corticosteroids as an effective method to treat isolated chylopericardium. CASE PRESENTATION: In this manuscript, we present an approach to treat isolated post-operative chylopericardium in a child following repair of partial anomalous pulmonary venous return. Chylous drainage responded to corticosteroids and completely ceased. There was no need for surgical intervention. CONCLUSION: Until now, isolated chylopericardium has never been reported to occur with partial anomalous pulmonary venous return repair. A review of the literature showed that most patients follow a conservative approach consisting of diuretics and non-steroidal anti-inflammatory agents with some of them undergoing surgical re-intervention. With future research on the topic still needed, we hope that this will encourage physicians worldwide to consider administering a trial of corticosteroids as an option to treat chylopericardium.

The Ross Procedure: Imaging, Outcomes and Future Directions in Aortic Valve Replacement.

The Ross procedure is gaining recognition as a significant option for aortic valve replacement (AVR), and is particularly beneficial in specific patient groups. Although categorized as a class IIb recommendation in the 2020 American College of Cardiology (ACC)/American Heart Association (AHA), and the European Society of Cardiology (ESC) management guidelines on valvular heart disease, recent studies bolster its credibility. Research, including a propensity-matched study, underlines the Ross procedure's association with enhanced long-term survival and reduced adverse valve-related events compared to other AVR types. This positions the Ross procedure as a primary option for AVR in young and middle-aged adults within specialized centers, and potentially the only choice for children and infants requiring AVR. This review meticulously examines the Ross procedure, covering historical perspectives, surgical techniques, imaging, and outcomes, including hemodynamic performance and quality of life, especially focusing on pediatric and young adult patients. It explores contemporary techniques and innovations like minimally invasive approaches and tissue engineering, underscoring ongoing research and future directions. A summarization of comparative studies and meta-analyses reiterates the Ross procedure's superior long-term outcomes, valve durability, and preservation of the left ventricular function, accentuating the crucial role of patient selection and risk stratification, and pinpointing areas for future research.

Rupture of sinus of Valsalva aneurysm with aorto-biventricular fistulas and right-ventricular outflow tract obstruction: a unique association.

Sinus of Valsalva aneurysms are a rare entity. Rupture of such aneurysms is a major cause of aortocardiac fistulas usually occurring between the right sinus of Valsalva and right cardiac chambers. We report an exceptional case of a ruptured congenital sinus of Valsalva aneurysm with fistulas involving both the right- and left-ventricular outflow tracts and causing RVOT obstruction. We also demonstrate the utility of computed tomography angiography and transesophageal echocardiography in diagnosing these fistulas.

Successful heart transplantation in an infant with phosphoglucomutase 1 deficiency (PGM1-CDG).

We report successful heart transplantation in a phosphoglucomutase 1 deficient (PGM1-CDG) patient. She presented with facial dysmorphism, bifid uvula and structural heart defects. Newborn screening was positive for classic galactosemia. The patient was on a galactose-free diet for 8 months. Eventually, whole exome sequencing excluded the galactosemia and revealed PGM1-CDG. Oral D-galactose therapy was started. Rapid deterioration of the progressive dilated cardiomyopathy prompted heart transplantation at the age of 12 months. Cardiac function was stable in the first 18 months of follow-up, and hematologic, hepatic, and endocrine laboratory findings improved during D-galactose therapy. The latter therapy improves several systemic symptoms and biochemical abnormalities in PGM1-CDG but does not correct the heart failure related to cardiomyopathy. Heart transplantation has so far only been described in DOLK-CDG.

Impact of Chronic Kidney Disease on Clinical Outcomes during Hospitalization and Five-Year Follow-Up after Coronary Artery Bypass Grafting.

Background: Chronic kidney disease (CKD) is often associated with multiple comorbidities including diabetes mellitus, and each has its own complications and impact after cardiac surgery including coronary revascularization. The objective of this work was to study the impact of CKD on clinical outcomes after coronary artery bypass grafting (CABG) and to compare outcomes in patients with different grades of renal functions. We retrospectively reviewed all patients who underwent CABG from January 2016 to August 2020 at our tertiary care hospital using electronic medical records. Results: The study included 410 patients with a median age of 60 years, and 28.6% of them had CKD and hospital mortality of 2.7%. About 71.4% of the patients had GFR > 60 mL/min per 1.73 m2, 18.1% had early CKD (GFR 30-60), 2.7% had late CKD (GFR < 30), and 7.8% of them had end-stage renal disease (ESRD) requiring dialysis. The CKD group had significantly more frequent hospital mortality (p = 0.04), acute cerebrovascular stroke (p = 0.03), acute kidney injury (AKI) (p < 0.001), longer ICU stay (p = 0.002), post-ICU stay (p = 0.001), and sternotomy wound debridement (p = 0.03) compared to the non-CKD group. The frequencies of new need for dialysis were 2.4% vs. 14.9% vs. 45.5% (p < 0.001) in the patients with GFR > 60 mL/min per 1.73 m2, early CKD, and late CKD, respectively. Acute cerebral stroke (OR: 10.29, 95% CI: 1.82-58.08, and p = 0.008), new need for dialysis (OR: 25.617, 95% CI: 13.78-85.47, and p < 0.001), and emergency surgery (OR: 3.1, 95% CI: 1.82-12.37, and p = 0.036) were the independent predictors of hospital mortality after CABG. The patients with CKD had an increased risk of strokes (HR: 2.14, 95% CI: 1.20-3.81, and p = 0.01) but insignificant mortality increase (HR: 1.44, 95% CI: 0.42-4.92, and p = 0.56) during follow-up. Conclusion: The patients with CKD, especially the late grade, had worse postoperative early and late outcomes compared to non-CKD patients after CABG. Patients with dialysis-independent CKD had increased risks of needing dialysis, hospital mortality, and permanent dialysis after CABG.

Variation in Case-Mix Across Hospitals: Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database was queried to document variation of patient characteristics, procedure types, and programmatic case-mix. METHODS: All index cardiac operations in patients less than 18 years of age in the STS Congenital Heart Surgery Database (July 2016 to June 2020) were eligible for inclusion except patients weighing ≤2.5 kg undergoing isolated patent ductus arteriosus closure. At the hospital level, we describe variations in patient and procedural characteristics known from previous analyses to be associated with outcomes. We also report variations across hospitals of programmatic case-mix. RESULTS: Data were analyzed from 117 sites (90 322 total operations, 87 296 total index cardiac operations eligible for STAT [STS-European Association for Cardio-Thoracic Surgery] 2020 Mortality Score). The median annual total index cardiac operations eligible for STAT 2020 Mortality Score per hospital was 157 (interquartile range [IQR], 94-276). Wide variability was documented in total annual index cardiac operations eligible for STAT 2020 Mortality Score per hospital (ratio 90th/10th percentile = 9.01), operations in neonates weighing <2.5 kg (ratio 90th/10th percentile = 4.09), operations in patients with noncardiac anatomic abnormalities (ratio 90th/10th percentile = 3.46), and operations in patients with preoperative mechanical ventilation (ratio 90th/10th percentile = 3.97). At the hospital level, the median percentage of all index cardiac operations in STAT 2020 Mortality Category 5 was 3.7% (IQR, 1.7%-4.9%), the median percentage of all index cardiac operations in STAT 2020 Mortality Category 4 or 5 was 24.4% (IQR, 19.0%-28.4%), the median hospital-specific mean STAT Mortality Category was 2.39 (IQR, 2.20-2.47), and the median hospital-specific mean STAT Mortality Score was 0.86 (IQR, 0.73-0.91). CONCLUSIONS: Substantial variation of patient characteristics, procedure types, and case-mix exists across pediatric and congenital cardiac surgical programs. Knowledge about programmatic case-mix augments data about indirectly standardized programmatic observed-to-expected (O/E) mortality. Indirectly standardized O/E ratios do not provide a complete description of a given pediatric and congenital cardiac surgical program. The indirectly standardized programmatic O/E ratios associated with a given program apply only to its specific case-mix of patients and may represent a quite different case-mix than that of another program.

Does Secondary Antibiotic Prophylaxis Improve Clinical Outcomes in Adult Rheumatic Heart Disease Patients Post-Valve Replacement?

BACKGROUND: Secondary prevention of recurrent rheumatic fever in individuals with rheumatic heart disease (RHD) requires continuous antibiotic prophylaxis. However, the impact of antibiotic prophylaxis on the outcome of patients with severe RHD who underwent heart valve replacement is unknown. The objective of the study was to assess the relationship between the use of antibiotics as secondary prophylaxis in RHD patients who underwent valve replacement and clinical outcomes including mortality, reoperation, and valve-related hospitalization. METHODS: We retrospectively compared outcomes of adult patients who underwent heart valve replacement for RHD at our institution from 1990 through 2014 and who received secondary antibiotic prophylaxis (prophylaxis group) with those who did not receive prophylaxis (nonprophylaxis group) using propensity score matching analysis. RESULTS: A total of 1094 patients (56% females, median age 40 years, range 31-53 years) were included with a median follow-up of 9.6 years (range 2.9-12.6 years). Antibiotic prophylaxis was prescribed in 201 patients (18%). Propensity score matching analysis demonstrated no significant difference in overall survival (95% [92%-98%] vs 97% [95%-99%], respectively; P = .7), valve-related hospitalization-independent survival (72% [range 65%-78%] vs 81% [range 76%-88%]; P = .25), and redo valve surgery-independent survival [76% [range 70%-83%] vs 75% [range 72%-79%]; P = .41) at 10-year follow-up in the nonantibiotic prophylaxis versus the antibiotic prophylaxis group. CONCLUSION: Secondary antibiotic prophylaxis among adult RHD patients following valve replacement is not associated with improved clinical outcomes.

Incidence, Risk Factors and Effective Treatment Strategies for Chylothorax After Pediatric Heart Surgeries: Retrospective Analysis of Large Database in Single Institution.

Background: Risk factors for postoperative chylothorax in children who had cardiothoracic procedures are not always clear. Due to complex course in post-operative care, It's always challenging to find the risk factors, and their management. Objective: The aim of our study was to identify the incidence, risk factors and effective treatment approaches for chylothorax after pediatric heart surgery. Methods: Children who had the cardiac surgery and subsequently developed chylothorax were included in the study. The ratio of the experimental group to the control group was 1:2. Decannulations of extracorporeal membrane oxygenation (ECMO) were not included in the analysis of patient outcomes. For each patient, we keep track of their age, weight, gender, syndrome, RACH-1 scoring, fluid balance, bypass time, clamp time, redo operations, open or close heart surgeries, and rhythm difficulties. Care logs were kept for every single therapy that was administered. Primary outcome was chylothorax, with secondary outcomes included time in the intensive care unit (ICU), length of hospital stay (LOS), and death. Results: 5210 surgeries were performed in six years. 96 patients developed the chylothorax with incidence of 1.8%. In chylothorax group, mean weight was 6.7 ± 4.2, while mean age was 11.7 ± 15.2. Clamp time was 74.5 ± 53.5 versus 39.9 ± 13.7. Mean bypass time was 128.34(76.25) versus 84.3 ± 25.1 with an odds ratio 1.02 (Z test 0.0001). Six (6.3%) children with chylothorax had redo cardiac surgeries in the same admission (p-value 0.01) while none in other. Five (5.2%) cases got operated by thoracotomy, three from left side. Mean Chest tube duration was 10±7.8 days versus 3.8±2.4 in control group. (p-value 0.02). chylothorax resolved (mean resolving time = 4 days) in 76 (79.2%) children with monogen formula. Two patients receive midodrine with no significant effect. Four children underwent surgical repair for chylothorax. Conclusion: Bypass time linearly increases incidence of chylothorax. Younger age, low weight, syndromic children, redo operations, non-open-heart surgeries, and arrhythmias also contribute to this. Gender, fluid balance, and RACHS-1 Scoring were not significant. While further research and testing are required for the use of midodrine. However, the low-fat formula of Monogen has proven to be an effective treatment.

Transcriptomal Insights of Heart Failure from Normality to Recovery.

Current management of heart failure (HF) is centred on modulating the progression of symptoms and severity of left ventricular dysfunction. However, specific understandings of genetic and molecular targets are needed for more precise treatments. To attain a clearer picture of this, we studied transcriptome changes in a chronic progressive HF model. Fifteen sheep (Ovis aries) underwent supracoronary aortic banding using an inflatable cuff. Controlled and progressive induction of pressure overload in the LV was monitored by echocardiography. Endomyocardial biopsies were collected throughout the development of LV failure (LVF) and during the stage of recovery. RNA-seq data were analysed using the PANTHER database, Metascape, and DisGeNET to annotate the gene expression for functional ontologies. Echocardiography revealed distinct clinical differences between the progressive stages of hypertrophy, dilatation, and failure. A unique set of transcript expressions in each stage was identified, despite an overlap of gene expression. The removal of pressure overload allowed the LV to recover functionally. Compared to the control stage, there were a total of 256 genes significantly changed in their expression in failure, 210 genes in hypertrophy, and 73 genes in dilatation. Gene expression in the recovery stage was comparable with the control stage with a well-noted improvement in LV function. RNA-seq revealed the expression of genes in each stage that are not reported in cardiovascular pathology. We identified genes that may be potentially involved in the aetiology of progressive stages of HF, and that may provide future targets for its management.

Predictors and clinical outcomes of post-coronary artery bypass grafting cerebrovascular strokes.

BACKGROUND: Despite the improved medical and surgical managements, still there is a significant risk of developing acute cerebrovascular strokes after coronary artery bypass grafting (CABG). Our objectives were to study the immediate and long-term outcomes after CABG and to identify the possible predictors of post-CABG strokes. RESULTS: Between January 2016 and August 2020, 410 adult patients, mostly males (82.2%), were retrospectively enrolled after CABG. Acute postoperative strokes occurred in 31 (7.5%) patients; of them, 30 (96.8%) patients had ischemic stroke, while 1 (3.2%) had hemorrhagic stroke. Mechanical thrombectomy was done in two cases. The patients who developed acute cerebral stroke had significantly higher admission (p = 0.02) and follow-up (p < 0.001) SOFA scores, higher arterial blood lactate level (p < 0.001), longer hospitalization (p < 0.001) and more hospital mortality (p < 0.001) compared with the patients who did not develop stroke. Kaplan-Meier curves for 5-year mortality showed increased risk in those patients with postoperative stroke (HR: 23.03; 95% CI: 6.10-86.92, p < 0.001). After multivariate regression, the predictors of early postoperative stroke were carotid artery stenosis (CAS), postoperative atrial fibrillation, cardiopulmonary bypass time, prior cerebral stroke, admission SOFA score and chronic kidney disease (CKD). The predictors of late cerebrovascular stroke were CAS, combined CABG and valve surgery, CKD, atrial fibrillation, prior stroke and HbA1c. CONCLUSIONS: The development of post-CABG acute cerebrovascular stroke is associated with longer hospitalization, multiple morbidities and increased mortality. Careful assessment and management of risk factors especially atrial fibrillation and carotid artery stenosis should be implemented to decrease this substantial complication after CABG.

Heyde's Syndrome - An Enigma.

Heyde's syndrome (HS) is described as the association between recurrent bleeding from angiodysplasia of the gastrointestinal tract and aortic stenosis. Aortic valve replacement has been reported to stop the bleeding. In unfit patients, the options available are interventional or conservative management. We hereby report an elderly obese patient with severe comorbidity with complicated HS involving a narrow aortic root. She underwent left ventricular outlet myomectomy and aortic root replacement to promote better forward flow and prevent restenosis and recurrence of symptoms. She was discharged home symptom-free despite being on coumadin anticoagulants.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Determinants of short- and long-term outcomes following triple valve surgery.

BACKGROUND AND AIM OF THE STUDY: Triple valve surgery (3VS) is required in some cardiac disorders that simultaneously involve the aortic valve (AV), mitral valve (MV), and tricuspid valve (TCV). Herein are reported details of the authors' experience with 3VS, and their attempts to identify those factors associated with a poor clinical outcome. METHODS: The medical records of patients who underwent 3VS between 1985 and 2005 were reviewed. A competing-risks methodology was used to determine the time-related prevalence and associated factors for three mutually exclusive end states after 3VS, namely death, valve reoperation, and survival without subsequent valve reoperation. RESULTS: A total of 206 consecutive patients (124 females, 82 males; median age 34 years; range: 12-82 years) underwent 3VS. The most common underlying pathology for all valves was rheumatic, followed by repair failure and prosthesis dysfunction for AV and MV, and functional regurgitation for TCV. In total, 72 patients (35.0%) had undergone prior valve surgery. At 3VS, the MV and AV were usually replaced (> 70%), whereas the TCV was usually repaired (91%). The operative mortality (prior to hospital discharge) was 11%, and 15-year survival 68%. At 15 years after 3VS, 21% of the patients had died, without subsequent valve reoperation, 50% had undergone valve reoperation, and 30% remained alive without valve reoperation. Baseline factors associated with early mortality after 3VS were older age at operation, TCV replacement, and the use of a mechanical MV during 3VS. A late risk of mortality was associated with poor left ventricular function and renal impairment at the time of the initial 3VS, and MV prosthesis prior to the initial 3VS. Younger age at 3VS and MV repair during the index 3VS were associated with a higher constant risk of valve reoperation, while female gender, MV prosthesis prior to index 3VS, MV replacement with a bioprosthesis during 3VS, and rheumatic TCV disease were associated with an increased late risk of valve reoperation. CONCLUSION: Both, primary and reoperative 3VS can be performed with acceptable operative mortality and long-term survival. However, patients continue to be at risk of valve reoperation, which steadily increases over time.

Mitral valve replacement with the Quattro stentless pericardial bioprosthesis: mid-term clinical and echocardiographic follow up.

BACKGROUND AND AIM OF THE STUDY: Preservation of the subvalvar apparatus during mitral valve replacement (MVR) is associated with improved ventricular function and patient outcome. The Quattro valve is a chordally supported stentless mitral valve bioprosthesis that undergoes anticalcification treatment and is sutured to both annulus and papillary muscles heads, thus preserving annuloventricular continuity. The study aim was to assess the mid-term hemodynamic and functional results following MVR using the Quattro valve. METHODS: Between 1998 and 2005, a total of 76 patients received the Quattro valve at the authors' institution. A competing-risks methodology was used to determine the time-related prevalence of four mutually exclusive end-states: death; subsequent valve removal for dysfunction; subsequent valve removal for endocarditis; and survival without valve reoperation. Serial echocardiograms (n = 321) were collected, and regression models adjusted for repeated measures were used to model the longitudinal progression of the prosthesis mitral valve area (MVA), peak gradient (PG), mean gradient (MG), mitral regurgitation (MR) and ejection fraction (EF) over time. RESULTS: The median patient age was 33 years, and the most common underlying pathology rheumatic fever (46%). Of the patients, 35 (46%) had undergone a prior mitral surgery. A competing-risks analysis showed that, at 10 years after Quattro implantation, 11% of patients had died without subsequent replacement, 57% underwent valve replacement for dysfunction, 7% for endocarditis, and 26% remained alive without reoperation. Overall, 24 valves were explanted (five for endocarditis and 19 for valve malfunction). On multivariable analysis, risk factors for reoperation were prior mitral surgery (p = 0.03), especially prior MVR (p = 0.04). Serial echocardiographic data showed progressive increases in PG and MR, a decrease in MVA, and stable MG and EF. CONCLUSION: In a fairly young population, the longevity of the Quattro valve was limited by a high risk of endocarditis and reoperation requirement. In addition to valve degeneration, malfunction without structural change was a frequent cause of prosthesis replacement. This may be due to an unpredictable geometric ventricular adaptation to the prosthesis, with subsequent development of prosthesis insufficiency or subvalvar stenosis. Further evaluation is required to identify the best candidates to receive the Quattro bioprosthesis.

Simultaneous aortic and mitral valve replacement in children: time-related outcomes and risk factors.

BACKGROUND AND AIM OF THE STUDY: Valve replacement in children is problematic, and associated with high anticoagulation-related complications and increased reoperation requirements. Multiple valve replacement may further increase morbidity and worsen outcome. The results are reported of combined aortic valve replacement (AVR) and mitral valve replacement (MVR) in children. METHODS: The medical records of children who underwent simultaneous AVR and MVR between 1984 and 2004 were reviewed, and the short-term and long-term results and variables affecting outcomes explored. The mean duration of follow up was 9.7 +/- 6.6 years. RESULTS: A total of 84 patients (62 males, 22 females; mean age 15.0 +/- 2.2 years) was identified. The underlying pathology was mainly rheumatic (94%) and endocarditis (4%). Among the patients, 21 (25%) had undergone a prior cardiac surgery. The implanted valves were either mechanical (n = 71) or bioprosthetic (n = 13). The average aortic and mitral valve sizes were 22 mm and 29 mm, respectively. In total, 21 patients had concomitant cardiac surgery, most commonly tricuspid valve repair (n = 18). The mean cardiopulmonary bypass time and ischemic time were 142 +/- 47 min and 107 +/- 33 min, respectively. Survival at 30 days and at one year was 96% and 94%, respectively. The overall 15-year survival was 78% (bioprosthesis 92% versus mechanical 76%; p = 0.4). The 15-year freedom from cardiac reoperation was 59%, and 68% and 75% for mitral and aortic reoperation, respectively. Significant risk factors for reoperation were the use of a bioprosthetic valve (p = 0.003) and female gender (p = 0.03). Freedom rates from endocarditis, thromboembolic and bleeding complications at 15 years were 90%, 92%, and 96%, respectively. Among survivors, 95% were in NYHA class I/II. CONCLUSION: Children with rheumatic fever and endocarditis may require simultaneous AVR and MVR. Although the operative mortality is acceptable, patients continue to have constant attrition with time, especially those who have received mechanical prostheses. The risk of cardiac reoperation requirement is high in all patients. Despite the greater need for reoperation, bioprosthetic valves could be offered to selected patients, such as females and those who are non-compliant with anticoagulation regimens.

Superior results following the Ross procedure in patients with congenital heart disease.

BACKGROUND AND AIM OF THE STUDY: The Ross procedure is a versatile operation that can be applied for aortic valve replacement (AVR) in patients with congenital heart disease (CHD), including small infants and those with complex left ventricular outflow tract (LVOT) obstruction. Herein, the clinical outcome is reported following the Ross procedure in patients with CHD at the authors' institution. METHODS: The medical records of patients who underwent the Ross procedure for CHD between 1991 and 2007 were reviewed. A competing-risks methodology was used to determine the time-related prevalence and associated factors for three mutually exclusive end states after the Ross procedure, namely death prior to subsequent cardiac reoperation, cardiac reoperation, and survival without subsequent reoperation. RESULTS: A total of 151 patients (98 males, 53 females) was identified. The median age at the time of surgery was 8.6 years (range: 4 days to 33 years). Previously, 103 patients (68%) had undergone cardiac interventions, and 43 (28%) required LVOT enlargement (modified Ross-Konno procedure). A competing-risk analysis showed that, at 10 years after the Ross procedure, 8% of patients had died without subsequent reoperation, 26% underwent cardiac reoperation, and 66% remained alive without further reoperation. The 10-year freedom from autograft and homograft reoperation was 95% and 71%, respectively. Factors associated with early risk of mortality were age < 1 year and no prior surgical/percutaneous intervention at the time of the Ross procedure. Surgical factors associated with cardiac reoperation were concurrent cardiac surgery and the use of fresh homografts. There were no bleeding or thromboembolic complications, and the 15-year freedom from endocarditis was 95%. Ultimately, 99% of the survivors were in NYHA class I or II. CONCLUSION: The Ross procedure remains the authors' procedure of choice for AVR in patients with CHD. Outcomes in infants aged < 1 year may improve with better patient selection and palliative surgical/percutaneous interventions prior to valve replacement. The late survival was excellent and valve-related complications were minimal. The high autograft longevity led to few patients requiring late reoperation for graft replacement.

Results of left atrioventricular valve reoperations following previous repair of atrioventricular septal defects.

OBJECTIVES: We report results of left atrioventricular valve reoperations (LAVVR) following atrioventricular septal defect (AVSD) repair and examine variables predictive of outcome. METHODS: Multiple demographics and operative variables were analyzed to determine factors affecting survival and reoperation. RESULTS: Forty patients following partial (n = 9) or complete (n = 31) AVSD repair underwent 47 LAVVR (1992-2005). Median age was 0.87 years (24 days-7.7 years) at initial AVSD repair and 3.15 years (84 days-13.6 years) at subsequent LAVVR with median interval between AVSD repair and LAVVR of 1.76 years (1 day-12.9 years). First LAVVR included repair (n = 20) or replacement (n = 20). Operative mortality was 10% and five-year survival was 76 +/- 6%. Significant risk factors were complete AVSD (p < 0.001), valve replacement (p < 0.001) for early death, and young age at time of LAVVR (p = 0.03) for late death. Five-year freedom from LAVV re-intervention was 100% for replacement versus 55 +/- 13% for repair (p = 0.006). Overall, ejection fraction increased to 61 +/- 3% versus 42 +/- 2% preoperatively (p < 0.01), and left-ventricle end-diastolic dimension Z-score decreased to 0.05 +/- 0.36 versus 3.1 +/- 0.3 preoperatively (p < 0.01). Eighty-seven percent of children were in New York Heart Association class I/II at latest follow-up. CONCLUSIONS: LAVVR results in significant clinical improvement and lasting recovery in ventricular chamber function and size. Valve repair offers survival advantage and should be aggressively attempted; however, it is only achievable in 50% of cases. Valve replacement is necessary in cases associated with complex LAVV morphology or following repair failure. At intermediate follow-up, patients continue to be at risk of major valve-related morbidity, requirement for re-intervention, and cardiac death.

Off-pump hypoplastic aortic arch ± aortic coarctation repair via median sternotomy using a temporary shunt.

An off-pump technique was developed to surgically manage the problem of aortic arch hypoplasia frequently associated with coarctation of the aorta and borderline size left-sided structures. The technique was successfully utilized in 5 patients adequately repairing the arch without any side effects.