Newborn – ABC CARDIOL

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Original Article

Management of Ductus-Dependent Systemic Circulatory Lesions in the Neonatal Period: A Ten-Year Experience

Dilek Dilli et al. Hasan Akduman Rumeysa Çitli Utku Arman Örun … Vehbi Doğan Mehmet Taşar Tamer Yoldaş Nurdan Dinlen Fettah Ahmet Özyazıcı Ayşegül Zenciroğlu

Abstract

Background

In ductus-dependent left-sided heart lesions, systemic circulation depends on right-to-left flow via the ductus arteriosus. These lesions may occur as an isolated defect or complex disease.

Objective

In this study, we aimed to investigate the neonatal outcomes of duct-dependent systemic circulatory lesions, especially aortic coarctation (CoA) and interrupted aortic arch (IAA).

Methods

A total of 159 patients with duct-dependent systemic lesions were followed up in the Cardiac NICU of our institution from 2012 to 2022. We retrospectively reviewed the medical charts of all patients from the hospital database. They were analyzed for clinical and surgical outcomes. A p-value of <0.05 was considered statistically significant.

Results

Of 159 patients, CoA was detected in 120 (75.4%) and IAA in 39 (24.5%) patients. Cardiac catheterization was performed in 74 (61.6%) patients with CoA in the neonatal period; 49 (40.8%) underwent therapeutic and 25 (20.8%) diagnostic procedures. One hundred one patients with CoA (84.1%) underwent surgery at a median age of 14 days (9-23). Thirty-four of 39 patients with IAA (87.1%) underwent surgery; single-stage repair was performed on 13 patients (38.2%), while two-stage repair was applied to 21 (61.7%) patients. The overall neonatal mortality rate was 19.5% (n=31). In multivariate analysis, the higher STAT categories (OR:2.3, CI:95%, 1.1-5.1, p=0.03) and the presence of major postoperative complications (OR:9.8, CI:95%, 2.1-35.1, p=0.003) have increased the risk of neonatal mortality.

Conclusion

Newborns with congenital duct-dependent aortic anomalies necessitate meticulous perioperative care due to their heightened risk of morbidity and mortality.

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IJCS

Original Article

Prevalence of Critical Congenital Heart Disease Detected in the Pulse Oximetry Test in Asymptomatic Newborns, ≥35 Gestational Weeks, in a Maternity in Southern Brazil

Sandra Mara Witkowski et al. Rafael Bertoldi Torres Aline Fernanda Pereira Carolina Marchi Guerra … Marco Otílio Rodrigues Wilde Leonardo Fonseca Sene Marcelo França Soares Thor Ferreira da Cruz

Abstract

Background

Congenital heart diseases (CHD) are one of the most prevalent malformations, and the screening tests to identify critical congenital heart disease (CCHD) is the pulse oximetry test, with subsequent investigation and treatment.

Objective

To quantify positive pulse oximetry tests and verify the prevalence of CCHD detected by it in asymptomatic newborns, ≥35 gestational weeks, in a Brazilian maternity hospital.

Methodology

This is an observational, retrospective, quantitative, analytical and cross-sectional study, conducted from October 2020 to May 2022, in a maternity hospital in southern Brazil, through the collection of records of positive oximetry pulse tests, following the norms of screening CCHD test of the Brazilian Society of Pediatrics (SBP), after they were evaluated with echocardiography for confirmation or exclusion of CHD.

Results

A total of 5,667 newborns were evaluated in this study, according to the inclusion criteria; 0.17% (n = 10) had a positive pulse oximetry test. Regarding the results of the echocardiography of the neonates with a positive test, two were normal, seven were cases of patent foramen ovale (PFO), and one was a case (0,017%) with interatrial communication (IAC) diagnosis. In the same period, five neonates with CCHD were born, symptomatic, diagnosed by physical examination, and referred to neonatal intensive care units (NICU) before taking the neonatal cardiac screening (< 24 hours of life).

Conclusion

The prevalence of positive pulse oximetry tests was 0.17% and none CCHD was detected. Five cases of CCHD were born in this period, but they were diagnosed before the recommend time to perform the screening test.

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ABC

Original Article

Cytogenomics Investigation of Infants with Congenital Heart Disease: Experience of a Brazilian Center

Marcília Sierro Grassi et al. Marília Montenegro Evelin Aline Zanardo Antonio Carlos Pastorino … Mayra Barros Dorna Chong Kim Marcelo Jatene Nana Miura Leslie Kulikowski Magda Carneiro-Sampaio

Abstract

Background

Some syndromes have specific and easily recognizable features, while others may be more complex to identify and may present different phenotypic manifestations, for example. An etiological diagnosis is important to understand the nature of the disease, to establish the prognosis and to start the treatment, allowing the inclusion of patients in society and reducing the financial cost of such diseases.

Objective

The initial proposal of this study was cytogenetic screening for the detection of the 22q11.2 deletion syndrome in consecutive newborns and infants with congenital heart disease using the multiplex ligation-dependent probe amplification (MLPA) technique. Therefore, throughout our research, other genomic alterations were identified in these cardiac patients. Thus, our objective was extended to investigate these other cytogenetic alterations.

Methods

We investigated 118 neonates with congenital heart diseases born consecutively during one year using the MLPA technique.

Results

The MLPA technique allowed the detection of 22q11.2DS in 10/118 patients (8.5%). Other genomic alterations were also identified in 6/118 patients (5%): 1p36 del, 8p23 del (2 cases), 7q dup, 12 dup and 8q24 dup.

Conclusion

This study highlights the relevance of detecting genomic alterations that are present in newborns and infants with congenital cardiac diseases using cytogenomic tools.

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ABCIC

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Mixed Variety of Total Anomalous Pulmonary Venous Return in an Asymptomatic Newborn

Cláudio Henriques et al. Andreia Palma Patrícia Silva Helena Andrade António Pires

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IJCS

Case Report

Catheter Ablation in Neonate with Heart Failure Due to Incessant Atrioventricular Reentrant Tachycardia

Sissy Lara de Melo et al. José Nilo de Carvalho Neto Nathalia Maria Segovia Monge Italo Bruno dos Santos Sousa … Cristiano Faria Pisani Mauricio Scanavacca

Abstract

The atrioventricular (AV) reentrant tachycardia (AVRT) is the most common cause of supraventricular tachycardia (SVT) in the young pediatric population. Some newborns might present with congestive heart failure and require interventional treatment. Catheter ablation in small infants (<6 months and <5 kg) is still poorly performed and controversial due to high complications rate in this group of patients.¹ We report a case of a 28 days old infant (3,5 kg) with a drug-refractory left accessory pathway mediated tachycardia and severe hemodynamic compromise, who underwent catheter ablation. Radiofrequency ablation should be part of the therapeutic arsenal in a context of drug-resistant supraventricular tachycardia with hemodynamic compromise, despite the greater risks of complications in this special population.

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Artigo Original

Congenital heart diseases in the newborn: from the pediatrician's request to the cardiologist's evaluation

Ivan Romero Rivera et al. Maria Alayde Mendonça da Silva José Maria Gonçalves Fernandes Ana Claire Pimenteira Thomaz … Cláudio Fernando Rodrigues Soriano Maria Goretti Barbosa de Souza

OBJECTIVE: To analyze the importance of symptoms as a reason for referral to pediatric cardiologists in the diagnosis of congenital heart diseases (CHD) in the newborn (NB). METHODS: Prospective study on live NB referred for cardiac evaluation, with performance of electrocardiogram, chest radiography and echocardiography. Cardiology consultation was requested by means of a multiple-choice form including signs and symptoms suggestive of CHD. Patent ductus arteriosus (PDA) without clinical and/or hemodynamic consequences was not considered a heart disease. RESULTS: From 1999 to 2002, 358 out of 3716 NB were studied, and 49 cases of CHD and 128 of PDA were found. The prevalence of CHD was 13.2:1000 NB. The main reason for referral to the cardiologist was heart murmur in 256 (72%) NB, of which 39 (15%) had CHD, and in 91% of the 128 cases of PDA. In 14 (4%) NB, the reason for referral was cyanosis, and eight of these patients (57%) had a CHD. Heart failure was the reason for referral in 37 (10%) NB, of whom 17 (46%) had CHD. Arrhythmia, associated congenital malformations, or chromosome disorders were the reasons for referral in 14% of the cases. CONCLUSION: The main reason for referral was detection of a heart murmur on cardiac auscultation. Although cyanosis and heart failure were uncommon reasons for referral, their presence indicated a high probability of the diagnosis of heart disease. Pediatric screening plays a key role in this diagnosis.

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