Cardiovascular Abnormalities

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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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Crawford 2 Thoracoabdominal Aneurysm Associated with Bicarotid Trunk and Aberrant Right Subclavian Artery

Milton Sérgio Bohatch Junior et al. Fernando Reis Neto Daniel Gustavo Miquelin Andre Rodrigo Miquelin … Henrique Amorim Santos Amanda Fernandes Vidal da Silva Luiz Fernando Reis Jose Maria Pereira de Godoy

A 73-year-old woman with a history of hypertension was referred to the vascular surgery department for severe chest pain and signs of shock, including tachycardia and a tendency toward hypotension. An angiotomography revealed a thoracoabdominal aneurysm (Crawford Classification type 2 – ) with rupture signs ( and ) and anatomical variations in the supra-aortic trunks, including an aberrant right subclavian artery (ARSA) ( and ) and a bicarotid trunk (). She underwent emergency surgery with a hybrid approach, which included visceral branching via laparotomy and endovascular repair of the aneurysm. Unfortunately, she developed hemodynamic instability and refractory shock during the procedure, resulting in death. Her family authorized the publication and signed the consent form.

An ARSA is a rare anomaly with a 0.2% to 13.3% prevalence.^, It is more common among females, individuals with congenital anomalies (up to 3%), and those with Down syndrome (up to 35%). ARSA may be associated with a bicarotid trunk in 0.8% to 20% of cases, where common carotid arteries originate from the same point on the aortic arch. Thoracoabdominal Aortic Aneurysms (TAAAs) represent 10% of all aortic aneurysms. These aneurysms are potentially fatal if not treated early, as they may progress to rupture or dissection. In a São Paulo epidemiological study, the mortality rate for emergency TAAA repairs was nearly double that of elective repairs (42.10% vs. 26.78%). This difference is often attributed to the hemodynamic instability of ruptured aneurysms and the lack of timely preoperative preparation in urgent cases. Anatomical assessment is essential for treating TAAAs, whether through open surgery, endovascular surgery, or hybrid techniques. Anatomical variations in the supra-aortic trunks increase surgical complexity, regardless of the chosen technique.

[…]

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Case Report

Uhl's Anomaly: Case Report and Differential Diagnosis in an Adult

Isaura Elaine Goncalves Moreira Rocha et al. Paulo Henrique Benevides Siqueira Beatriz Gonçalves Rocha Bruno Farias Oliveira … George Elias Luna Martins Simone Cristina Soares Brandão

Introduction

Uhl’s anomaly (UA) is a disorder exclusive to the right ventricle (RV) characterized by the absence of total or partial myocardium, in such a way that the ventricular wall is comprised of the overlapping of the endocardium and the epicardium, with no fatty tissue, or inflammatory or infiltrative process. The muscles of the atria, the interventricular septum and the left ventricle (LV) are not involved in the process.^,

The clinical manifestations are due to right ventricular failure (RVF). Forms with partial involvement may be mildly symptomatic and remain undiagnosed for long periods, and may be confused with other more frequent pathologies that affect the right heart in adult life.^,

[…]

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Original Article

Incidence and Morphological Study of Myocardial Bridge in the State of Ceará: A Cadaveric Study

Jalles Dantas de Lucena et al. Hudson Martins de Brito João Victor Souza Sanders Jonathan Barros Cavalcante … Michelly Carneiro Collyer Cecília de Lima Leite Helson Freitas da Silveira Julio Cesar Campos Ferreira Filho

Abstract

Background

Myocardial bridges (MB) are anatomical anomalies with possible clinical repercussions; hence, their understanding deserves attention.

Objective

To determinate the prevalence and characterize MB in human hearts from the state of Ceará. Methods: Fifty hearts of adult human cadavers from the Medicine School of Federal University of Ceará, Brazil. The hearts were dissected to identify MBs that pass over part of the coronary artery. The segment of the artery (proximal, middle, and distal) with a bridge was identified. The external diameter of the artery at the proximal and distal points of the MB was measured. The length and thickness of the MB were also measured with an electronic caliper. The muscle index (MMI) of the MB was calculated as the product of length and thickness expressed in millimeters. The significance level adopted in the statistical analysis was 5%.

Results

MB was confirmed in 40% of sample. Approximately one third of the sample had only 1 MB. MB was most frequently found over the anterior interventricular branch of the left coronary artery (59.25%, p=0.02), and its prevalence in other branches was much lower (22.23%). The most affected segments of arteries were the superior (44.44%) and medium (40.74%). The mean diameter of arteries proximal to the MB was 2.38±0.97mm (range=0.78-5.15mm), and the diameter distal to the MB was 1.71±0.75mm (range=0.42-3.58mm). The length was measured as mean=8.55±5.27mm, while the mean thickness was 0.89±0.33mm.

Conclusion

A high prevalence of MB is more likely to affect the left coronary artery system with larger MMI than other affected branches.

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Case Report

Diagnosis of Coronary Anomaly of the Circumflex Artery With Retroaortic Course by Transthoracic Echocardiography (Rac Sign) Confirmed by Computed Tomography Coronary Angiography: A Case Series

Raul Serra Valério et al. Alfredo A. Eyer Rodrigues Carlos Eduardo Suaide Silva Marly Uellendahl Luciano de Figueiredo Aguiar Filho

Introduction

Congenital coronary artery anomaly (CCAA) is a relatively rare condition with an estimated population prevalence of 0.21–5.79%. CCAA presents a wide spectrum of variations and can be classified into origin and course, intrinsic, and termination anomalies. ^, The CCAA with the highest prevalence is the anomalous origin of the circumflex artery (CxA) from the right coronary sinus (RCS) with an estimated prevalence of 0.3–0.8%.

CCAA usually travels along a retroaortic course and may present different originations, such as from a common ostium with the right coronary artery (RCA), from separate ostia in the RCS, or from the proximal segment of the RCA.

[…]

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Intracavitary Right Coronary Artery: An Incidental Finding with Potential Implications for Invasive Cardiac Procedures

Sara Cristina da Silva Borges et al. Catarina Isabel Ribeiro Carvalho Miguel Eduardo Teixeira Moz Gonçalves Ana Isabel Santos Baptista José Ilídio Moreira

A 66-year-old man with a history of palpitations suggestive of paroxysmal supraventricular tachycardia was referred for CT angiography (CTA) for exertional dyspnea etiology investigation. ECG-gated cardiac CT was performed using the 64-slices dual-source Somatom Go Scanner.

CTA showed the normal origin of the right and left main coronary arteries, and there was no evidence of obstructive coronary artery disease. The proximal right coronary artery (RCA) had a normal epicardial course, but mid-RCA was noted to penetrate the right atrial wall for a 30 mm course within the right atrium, exiting to its usual course in the posterior atrioventricular groove, as demonstrated via the multiplanar reconstruction CT images at maximum intensity projection ( ) as well as 3-dimensional reconstructions ( ).

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Original Article

Echocardiography: The Examination Request by the Pediatriciant Achieving the Pediatric Cardiologist

Lene Garcia Barbosa et al. Amanda Bentes Rios de Freitas Mariana Aparecida Brunossi Moura Proença Célia Maria Camelo Silva

Summary

Background

Echocardiography is a diagnostic tool used to evaluate cardiac anomalies and clarify doubts about the benign nature of certain findings of cardiovascular clinical examination.

Objectives

To identify the main reasons for the request of echocardiography by pediatrician and evaluate the degree of anxiety of the family generated by this conduct.

Methods

We included patients younger than 18 years without previous diagnosis of heart disease and referred for the first echocardiogram. Family members responsible for the patients answered a questionnaire to assess the degree of their anxiety from order to the performance of the echocardiogram, with scores between 0 and 19. Data were analyzed using percentages, standard deviation and Student’s t test.

Results

Studied 30 patients with a mean age of 4.45 years. The most prevalent reason for referral was heart murmur (23 cases), of which 70% patients had no congenital heart disease hypothesis confirmed, after echocardiography. The average score on the anxiety questionnaire was 11 ± 6 significantly associated with the presence of heart disease, with a mean of 13.0 ± 5.3 vs. 9.3 ± 5.2 in the group without the possibility of it (p = 0.007).

Conclusion

The murmurs findings was the main reason for echocardiography request by the pediatrician. Confirmation of heart disease was higher in children under 1 year and with murmurs findings. Despite the anxiety level was higher in the group of patients who were diagnosed with congenital heart disease, this was not negligible in the family in the group of patients without heart disease. (Arq Bras Cardiol: Imagem cardiovasc. 2017;30(2):39-45)

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Original Article

HDI, Technological and Human Resources in the Diagnosis and Treatment of Malformations of the Circulatory System in Brazil

Thais Rocha Salim et al. Thayanne Mendes Andrade Carlos Henrique Klein Gláucia Maria Moraes de Oliveira

Abstract

Background

In 2015, the number of infants with congenital malformations (CMs) per 100 000 live births (LBs) was 2368 (7.6%) worldwide, of whom 10.6% died in the first year of life, 43% due to malformations of the circulatory system (MCSs), a scenario similar to what occurs in Brazil.

Objective

To assess, per Brazilian macroregion, whether diagnosis of MCS at birth and death due to MCS in the first year of life associate with human development index (HDI) and with technological and human resources.

Methods

Ecological study including data available in 2000–2015. Data of LBs, deaths and availability of echocardiography devices were obtained from the DATASUS website. The HDI was obtained from the Atlas of Human Development in Brazil, while other variables were obtained from medical demographic data. Correlation measures between the variables were performed using the Kendall index.

Results

The CM rate was 660.8/100 000 LBs, of which 18 444 were due to MCS (diagnosis rate, 38.55/100 000 LBs). Of all Brazilian macroregions, the Southern and Southeastern regions, with the highest HDI values and resources, had the highest MCS diagnosis rates (56.94/100 000 and 62.83/100 000 LBs, respectively). The Northern and Northeastern regions, with the lowest HDI values and resources, had the lowest MCS diagnosis rates (9.77/100 000 and 13.43/100 000 LBs, respectively). The MCS diagnosis rate was 6.4-fold higher in the Southeastern region as compared to the Northern region, but mortality rates were similar in both regions.

Conclusion

Of the CMs, the MCS accounted for the highest number of deaths in children under the age of 1 year in Brazil.

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Case Report

Cardiovascular magnetic resonance in unsuspected interrupted inferior vena cava

Andre Mauricio Fernandes et al. Vikas Rathi June Yamrozik Ronald Willians Robert W. Biederman

Interrupted inferior vena cava (IVC) is a rare anomaly. Anomalies of IVC are clinically important for cardiologists and radiologists who plan to intervene in the right heart. We describe three cases of IVC interruption diagnosed by cardiac magnetic resonance imaging study.

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