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.

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.

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.

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.

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.

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.

Estimation of modified concordance ratio in sib-pairs: effect of consanguinity on the risk of congenital heart diseases.

Family studies are widely used for research into genetic and environmental influences on human traits. In this paper, we establish statistical methodology for the estimation of a new measure of sib similarity with respect to dichotomous traits measured on each member of within family sib-pair. We call this parameter "excess risk." For inference problems involving a single sample, we construct a large sample confidence interval on the concerned parameter. It has long been suspected that consanguinity is a risk factor for many genetic defects. Therefore, we establish a procedure to test the significance of the difference between excess risk parameters in a sample of consanguineous marriages and another sample of non-consanguineous marriages. We apply the methodology to data from a hospital-based congenital heart defects registry in Saudi Arabia, a population in which consanguinity is quite common.

Pericardial adhesions simulating myocardial bridging.

A 48-year-old man with aortic valve disease was diagnosed to have myocardial bridging on preoperative coronary angiography. During surgical exploration, only pericardial adhesions were found, with a normal epicardial coronary course.

Neoaortic bicuspid valve in arterial switch operation: mid-term follow-up.

BACKGROUND: We sought to identify the prevalence of bicuspid pulmonary valve among patients with transposition of the great arteries undergoing the arterial switch operation and evaluate functional integrity of that valve in the neoaortic position. METHODS: Between October 1985 and December 2001, 391 patients had an arterial switch operation for transposition and its variants. Perioperative information and follow-up data were available for 342 patients. The serial echocardiograms of patients with bicuspid pulmonary valve were reviewed. The neoaortic valve was serially assessed, focusing on aortic insufficiency, annulus diameter, and pressure gradients. RESULTS: Twenty-four patients (7%) had a bicuspid pulmonary valve. Age at operation was 5 days to 12 years. Two patients were lost to follow up, and 22 patients had mean follow-up of 5.3 years (range, 2 months to 13 years), of which 21 patients were alive and 1 died late. At least two postoperative echocardiogram reports were available on 19 patients. Seven patients had no neoaortic regurgitation, and 10 had trivial regurgitation. Severe aortic regurgitation developed in 1 patient with endocarditis and in another with repair of Taussig-Bing anomaly. Neoaortic valve size indexed to body surface area showed an increase in annular diameter over time proportional to somatic growth. No significant valve stenosis developed. CONCLUSIONS: Encountering a bicuspid pulmonary valve at the time of an arterial switch operation is not uncommon. The integrity of a bicuspid pulmonary valve in the neoaortic position is maintained at a mean follow-up of 5.3 years. We believe that the presence of a bicuspid pulmonary valve is not a contraindication to an arterial switch operation.

Exertional dizziness and syncope caused by anomalous left coronary artery origin from the right sinus of Valsalva.

Coronary anomalies are generally rare, but has fascinating congenital cardiac disease entities, recognized in less than 1.3% of all coronary angiograms. Left coronary artery arising from right sinus of valsalva RSOV represents an extremely uncommon subtype. Initial presentations include chest pain, myocardial infarction, arrhythmias, sudden death, and rarely exertional syncope. We report a case of exertional dizziness and syncope, diagnosed to have anomalous origin of left main coronary artery from RSOV. Surgical intervention was curative.

Intrapericardial paraganglioma.

We report a case of intrapericardial paraganglioma accidentally discovered during surgery for mixed mitral valve disease. The 6 cm tumor was located in the aorta-caval groove, adherent to the roof of the left atrium and compressing the superior vena cava. A biopsy of the mass established the histological diagnosis of a non pressor secreting paraganglioma. The tumor was resected 2 months after the original mitral valve surgery. The patient made an uneventful recovery and remains well 2 years after surgery.

Arterial complications following cardiac catheterization in children less than 10 kg.

We sought to determine if a higher dose of heparin would reduce arterial complications in patients weighing 10 kg or less undergoing cardiac catheterization to investigate congenital heart disease. Sixty patients were given either 100 (group A) or 150 (group B) IU x kg(-1) of heparin in a double-blinded randomized manner. Initial arterial access was established using a 4F cannula in all patients. Mean activated clotting time measured 20 minutes following heparin administration was significantly lower in group A than in group B (199 versus 251 seconds). Only 3 out of 60 patients (5%) required treatment for loss of femoral pulse. The age, weight, activated clotting time, length of catheterization procedure, time taken to establish arterial access, and the duration of arterial cannulation were comparable between the groups. Weight under 4 kg, age under 1 month, and cannula size larger than 4F were identified as independent risk factors for the development of arterial complications. Arterial access using a 4F cannula is a safe procedure in children weighing 10 kg or less. The incidence of significant arterial complications is low, and they do not appear to be preventable by a higher dose of heparin.