Annals of Pediatric Cardiology最新文献

The interventional cardiac catheterization for treating congenital heart disease has evolved. Complications following interventional procedures might need emergency surgery as a bailout. Here, we report a case of cardiac perforations in three different sites following device closure of atrial septal defect (ASD). In literature, the major sites of ASD device erosion are at the roof of the right atrium (RA), left atrium (LA), or at the atrial junction with the aorta. In our patient, the device eroded at all three sites: the roof of the LA, RA, and the aorta, causing hemopericardium.

Pseudoaneurysm formation within the right ventricular outflow tract (RVOT) is a rare but serious complication following cardiac surgeries involving the RVOT. This report presents the case of a 3-month-old, 4 kg infant with tetralogy of Fallot and pulmonary atresia, previously treated with ventricular septal defect closure and right ventricle (RV)-pulmonary artery homograft placement. The patient presented critically ill with septic shock and suprasystemic RV pressure. A high-risk surgical approach was averted through the endovascular exclusion of the pseudoaneurysm using a Gore VBX^® balloon expandable covered stent, in addition to stenting of bilateral proximal branch pulmonary arteries to alleviate RV hypertension. This case underscores the multifactorial nature of RVOT pseudoaneurysm formation and the importance of a high index of suspicion for diagnosis. Management options, including surgical and transcatheter strategies, are discussed, focusing on the successful use of a covered stent for stabilization in a critically ill patient.

Background: Extracorporeal membrane oxygenation (ECMO) is a vital mechanical circulatory support used with increasing frequency in complex congenital cardiac surgeries. This study evaluated the outcomes of a protocol-based venoarterial (VA) ECMO program following congenital heart surgeries.

Methods and results: This was a retrospective review of 198 patients who underwent VA-ECMO after congenital cardiac surgeries at our institute between 2004 and 2023. Patients were divided into pre-ECMO protocol (2004-2017) and post-ECMO protocol (2018-2023) implementation. There were 107 patients in the preprotocol era and 91 in the postprotocol era. We compared weaning from ECMO and survival to hospital discharge between the two eras. An analysis of the factors influencing survival to hospital discharge was also done. ECMO was initiated through the central cannulation technique through median sternotomy in all patients. The median age and weight at initiation were 4 months (interquartile range [IQR] 1-33.5 months) and 4.4 kg (IQR 3.3-10.1 kg), respectively. The successful weaning of the ECMO (n = 67/91, 73.6%) and survival to discharge (n = 43/91, 47.3%) were higher in patients of the postprotocol era. However, it was not statistically significant. Higher risk adjustment for congenital heart surgery-1 >3 and acute kidney injury were independent predictors of poorer survival to hospital discharge.

Conclusions: A protocol-based ECMO program may improve outcomes of successful weaning and survival to discharge in patients undergoing congenital cardiac surgeries.

Partial cavopulmonary shunt is an established procedure for patients with abnormal right ventricular (RV) physiology. Late failure of the Glenn shunt is usually due to further progression of the primary disease process. However, there may be surprises, as in our patient. We report an 8-year-old child who was diagnosed with tricuspid valve (TV) dysplasia with borderline RV hypoplasia. He underwent TV repair with a right cavopulmonary shunt. He presented with features of heart failure after 6 years of the surgery. An echocardiographic evaluation suggested a restrictive filling pattern of the RV with preserved size and function. Computed tomography and cardiac magnetic resonance imaging revealed a normal pericardial thickness with no evidence of myocardial involvement. Despite the contradicting clinical and imaging features, endomyocardial biopsy confirmed a diagnosis of desmin cardiomyopathy. Restrictive cardiomyopathy may remain an elusive diagnosis despite extensive imaging. In our case, the altered loading conditions due to a cavopulmonary shunt further masked pathophysiological hemodynamic changes.

Berry et al. (1982) described a rare syndrome associated with distal aortopulmonary window, aortic origin of the right pulmonary artery, intact ventricular septum, and interruption or coarctation of the aorta. Here, we present the first neonatal case of single-stage repair of Berry syndrome in India. Timely surgery and skilled postoperative care define the short-term and long-term outcomes. Single-staged repair is preferred wherever feasible.

Aorto-pulmonary window (APW) is a rare congenital heart disease. The non-restrictive types of APWs present at an early age and require early surgical correction. The transcatheter device closure in young infants pose significant challenges that include hemodynamic instability during arteriovenous loop formation and due to potential encroachment of the occlusive device on vital adjacent structures, and sizing of introducer sheaths and delivery systems. We report a successful transcatheter closure of an AP window in a 1.35 Kg preterm infant with heart failure requiring mechanical ventilation. To the best of our knowledge this is the smallest and youngest baby , wherein transcatheter device closure has been attempted for a non-restrictive aorto-pulmonary window. The procedure was done through a 4 French right femoral venous access, thus avoiding an arterial access. A 0.025 Terumo wire was maneuvered through the pulmonary artery across the APW without formation of an arteriovenous loop. The APW was successfully occluded with a Konar multi-functional device delivered via a 4-French delivery system. The baby could be successfully weaned off the ventilator and could be discharge. On 1 month follow up, baby was in good health and gaining adequate weight. Thus we conclude that careful selection of patients allows successful closure of APW, even in the smallest baby.

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.

Introduction: This study aimed to systematically analyze and describe the main challenges of congenital heart diseases (CHDs) in the countries in the Amazon region.

Methods: The methodology followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews checklist utilizing the Medline, Embase, Lilacs, and Google Scholar databases. The inclusion criteria were articles addressing any topic involving CHD in the Amazon region. Incomplete articles, book chapters, lectures, conference abstracts, and editorials were excluded.

Results: Nine studies were identified, 7 of which were published in the last decade and were of Colombian and Brazilian origin. The methodology of the studies was cross-sectional and ecological, evaluating the regional and epidemiological factors, challenges to diagnosis and treatment, multidisciplinary team challenges, and the impact of the COVID-19 pandemic. Studies carried out by surgeons demonstrate more existing data regarding the challenges of the health-care system.

Conclusions: There is growing interest in analyzing the situation of CHD in the region. However, only a few studies are available, mostly on ecological and cross-sectional analysis. These records show the lack of hospital infrastructure and multidisciplinary teams for the diagnosis and treatment of CHD in the Amazon region; we see an initiative by specialists from two countries (Colombia and Brazil) to demonstrate the difficulties by seeking international training programs and government aid to improve the health system situation.