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Competing Interests: Conflict of interestThe authors declare that they have no conflicts of interest.

An autosomal recessively inherited noninflammatory arthropathy known as camptodactyly, arthropathy, coxa vara, and pericarditis (CACP) syndrome was discovered in 1999. It is distinguished by synoviocyte hyperplasia and subcapsular fibrosis of the synovial capsule, which results in a shortage of lubricin production. The resulting lack of joint lubrication induces increased mechanical stress, causing progressive deformities that become evident with weight-bearing and heightened joint activity. Animal models with a lubricin gene knock-out display similar traits, underscoring the impact of mechanical stress on disrupting type II collagen on the articular surface. The gradual development of pericarditis and constriction often results in misdiagnosis as juvenile rheumatoid arthritis with cardiac involvement, but the defining feature remains the noninflammatory nature of the disease. Early recognition is pivotal, as interventions such as pericardiectomy and recombinant human lubricin hold promise for altering the disease's natural course. In our familial case of CACP, two siblings exhibited distinct phenotypic variations - one with fibrosis-dominant features and pericardial constriction and the other displaying synovial hyperplasia without pericardial involvement., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Annals of Pediatric Cardiology.)

Competing Interests: Conflict of interestThe authors declare that they have no conflict of interest.

Alzheimer's disease (AD) is a multifactorial, progressive, neurodegenerative disease typically characterized by memory loss, personality changes, and a decline in overall cognitive function. Usually manifesting in individuals over the age of 60, this is the most prevalent type of dementia and remains the fifth leading cause of death among Americans aged 65 and older. While the development of effective treatment and prevention for AD is a major healthcare goal, unfortunately, therapeutic approaches to date have yet to find a treatment plan that produces long-term cognitive improvement. Drugs that may be able to slow down the progression rate of AD are being introduced to the market; however, there has been no previous solution for preventing or reversing the disease-associated cognitive decline. Recent studies have identified several factors that contribute to the progression and severity of the disease: diet, lifestyle, stress, sleep, nutrient deficiencies, mental health, socialization, and toxins. Thus, increasing evidence supports dietary and other lifestyle changes as potentially effective ways to prevent, slow, or reverse AD progression. Studies also have demonstrated that a personalized, multi-therapeutic approach is needed to improve metabolic abnormalities and AD-associated cognitive decline. These studies suggest the effects of abnormalities, such as insulin resistance, chronic inflammation, hypovitaminosis D, hormonal deficiencies, and hyperhomocysteinemia, in the AD process. Therefore a personalized, multi-therapeutic program based on an individual's genetics and biochemistry may be preferable over a single-drug/mono-therapeutic approach. This article reviews these multi-therapeutic strategies that identify and attenuate all the risk factors specific to each affected individual. This article systematically reviews studies that have incorporated multiple strategies that target numerous factors simultaneously to reverse or treat cognitive decline. We included high-quality clinical trials and observational studies that focused on the cognitive effects of programs comprising lifestyle, physical, and mental activity, as well as nutritional aspects. Articles from PubMed Central, Scopus, and Google Scholar databases were collected, and abstracts were reviewed for relevance to the subject matter. Epidemiological, pathological, toxicological, genetic, and biochemical studies have all concluded that AD represents a complex network insufficiency. The research studies explored in this manuscript confirm the need for a multifactorial approach to target the various risk factors of AD. A single-drug approach may delay the progression of memory loss but, to date, has not prevented or reversed it. Diet, physical activity, sleep, stress, and environment all contribute to the progression of the disease, and, therefore, a multi-factorial optimization of network support and function offers a rational therapeutic strategy. Thus, a multi-therapeutic program that simultaneously targets multiple factors underlying the AD network may be more effective than a mono-therapeutic approach.

Competing Interests: Conflict of interestThe authors declare no competing interests.

Images of vascular ring-right aortic arch, mirror image branching with Kommerell's diverticulum and a brief comment on its management., (Copyright (c) 2009 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier B.V. All rights reserved.)

We present a case of left anterior descending (LAD) coronary artery aneurysm at the site of previous stent placement 3 years previously. The patient presented with recent worsening of angina. Angiography and 64 slice CT angiography confirmed the presence of 6mm aneurysm of LAD at the site of previous stent involving the origin of diagonal, with thrombus proximal and distal to the stent. This patient was successfully managed by taking the posterior wall of the anterior descending artery while suturing the heel of the left internal mammary artery (LIMA)-LAD anastomosis. The idea was to create severe stenosis upstream to prevent distal embolisation from the site of aneurysm. The diagonal was grafted with a saphenous venous graft. Follow-up angiogram at 3 months demonstrated successful exclusion of the aneurysm and unobstructed flow through the grafts.

Objective: This study investigated whether postoperative low cardiac output and mortality in obstructed total anomalous pulmonary venous connection could be reduced by selective vertical vein patency., Methods: Fifty-eight patients undergoing rechanneling of total anomalous pulmonary venous connection between 1997 and 2006 were studied. The vertical vein was left patent in 27 patients (group I) and ligated in 31 (group II). Mean ages were 1.49 +/- 1.63 and 4.37 +/- 3.38 months for groups I and II, respectively., Results: Operative mortalities were 29.1% and 7.4% for ligated and unligated groups, respectively (relative risk 1.75, 1.16-2.64, P = .036). Age younger than 1 month, obstructive total anomalous pulmonary venous connection, hypoplastic pulmonary veins, pulmonary hypertensive crisis, low cardiac output, and vertical vein ligation were significant risk factors for death according to logistic regression analysis. Patients with obstructed total anomalous pulmonary venous connection undergoing vertical vein ligation demonstrated predominant right ventricular dysfunction (relative risk 2.93, 1.28-6.73, P = .011), pulmonary hypertensive crisis (relative risk 2.90, 1.25-6.75, P = .013), and 3.28 times the risk of death (95% confidence interval 1.08-9.99, P = .032) relative to the unligated group., Conclusions: In a subset of patients with obstructed total anomalous pulmonary venous connection, an unligated vertical vein reduces pulmonary arterial pressure, decreases perioperative pulmonary hypertensive crises, provides a temporary pop-off valve during pulmonary hypertensive crisis, and improves survival by providing superior hemodynamics. The high mortality in the ligated group suggests that patients with obstructed total anomalous pulmonary venous connection with postbypass moderate pulmonary hypertension possibly should not undergo vertical vein ligation. We propose routine use of an adjustable ligature around the vertical vein in all patients with more than moderate post-bypass pulmonary hypertension, allowing gradual tightening in increments without multiple reoperations.

An unusual case of a sewing needle in the heart is reported for its rarity. The relevant literature is briefly reviewed.

Five patients of right pulmonary artery-to-left atrium communication presented with special emphasis on the clinical presentation, anatomic variations, diagnostic considerations, and management options. An individualized management algorithm is recommended.

This is a collective review of 59 published cases of right pulmonary artery to left atrium communication in the English-language literature. In this article, we review the literature on the clinical presentation, anatomic variations, diagnostic features, and management considerations of previously reported cases. Two-dimensional echocardiography and cardiac catheterization including selective angiography provided the necessary diagnostic information and defined the anatomy before surgery in all patients. Various management options including medical, surgical, and transcatheter closure have been reported. The lesions have been classified into four types (I, II, III, and IV) that have a bearing on the appropriate surgical approach and techniques of repair that are discussed in the text. Use of extracorporeal circulation may be considered in selected instances. The operative mortality rate during the earlier era (1950 to 1979) was high, at 22%. More recently, the mortality rate has approached zero. An individualized surgical approach depending on the type of surgical connections is recommended.

Background: The aim of this study is to investigate the feasibility of chordal-sparing mitral valve replacement procedures in a rheumatic population, and to determine the early and late effects of different chordal preservation procedures on the clinical outcome and left ventricular mechanics., Methods: Various techniques of chordal preservation during mitral valve replacement for rheumatic heart disease in 451 patients between 1996 and 1999 are described. The mean age was 35.6 +/- 19.0 years (range, 15 to 55 years). Seventy patients had complete excision of the subvalvular apparatus (group I), 124 had preservation of the posterior chordopapillary apparatus (group II), and 257 had total chordal preservation (group III). Echocardiography was performed preoperatively, at discharge, at 1 year, and at 4 years., Results: Chordal preservation groups (II and III) demonstrated significant improvement (p < 0.05) in left ventricular function immediately and late postoperatively. Reduction of left ventricular end-systolic and end-diastolic volume was significant in the chordal preservation groups (II and III) as compared to the nonchordal group (group I; p < 0.005). The total chordal group demonstrated greater fractional change of left ventricular end-systolic volume as compared to the posterior chordal and nonchordal group. The left ventricular ejection fraction and fractional shortening continued to decline over time in the nonchordal cohort (p = 0.05 and p = 0.001, respectively) and did not improve by 4 years. Statistically significant change in ejection fraction occurred in the chordal preservation groups as compared to the nonchordal group., Conclusions: We conclude that total chordal preservation is possible in the large majority of rheumatic patients and confers significant long-term advantage by preserving left ventricular function. The surgical technique should be individualized.