Annals of Pediatric Cardiology最新文献

We present the case of a 4-year-old girl with tetralogy of Fallot associated with an incidentally detected isolation of the left brachiocephalic artery, with no communication between it and the pulmonary artery or ductus arteriosus. The case highlights the unusual association and hemodynamic consequences of the condition.

Background: Bronchopulmonary dysplasia (BPD) poses a challenge in neonatal care. Previous literature recommended a hypothetical role for patent ductus arteriosus (PDA) in the development of BPD. This study explores the possible link between PDA and BPD, aiming to illuminate demographic and clinical factors influencing BPD development within the context of PDA.

Methods: This retrospective cohort analysis employed data from the National Inpatient Sample (NIS) spanning from 2016 to 2020. The study focused on patients diagnosed with PDA and BPD, identified through International Classification of Diseases 10^th Revision codes Q250 and P271, respectively. Utilizing STATA ×15, descriptive and inferential statistics, encompassing univariate and multivariate regression analyses, were conducted to examine the association between PDA and BPD.

Results: A total of 9737 patients were included: 5133 without PDA and 4604 with PDA. The mortality rate was significantly higher among patients with PDA (3.80%) compared to those without PDA (2.53%) (P < 0.0001). Univariate and multivariate regression analyses identified a significant association between PDA and BPD, with odds ratios of 14.62 and 2.43, respectively (both P < 0.0001). BPD patients with PDA also exhibited a significantly higher prevalence of extremely preterm birth (76.24% vs. 58.31%, P < 0.0001) and extremely low birth weight (65.57% vs. 42.70%, P < 0.0001) compared to BPD patients without PDA. In addition, significant associations were observed between BPD and factors such as preterm birth category, neonatal sepsis, race, hospital status, and region (all P < 0.0001).

Conclusions: This research confirms the connection between PDA and BPD, stressing the importance of continued investigation and prospective studies. The findings highlight the need to consider several factors in understanding the etiology of the disease, which could lead to more targeted interventions and improved patient care.

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.