Heart Septal Defects, Atrial – ABC CARDIOL

ABCIC

Review Article

My Approach to Transcatheter Closure of Atrial Septal Defect: Step-by-Step and Current Contraindications

Angele Azevedo Alves Mattoso et al. Joberto Pinheiro Sena Gilson Soares Feitosa Filho Maria Lúcia Duarte

Abstract

All patients who are candidates for transcatheter closure of an ostium secundum atrial septal defect (ASD) should undergo a detailed echocardiographic assessment using 2D transesophageal echocardiography (TEE) prior to the procedure. It is essential for a comprehensive analysis of interatrial septal anatomy and for determining defect eligibility for the procedure. The type, number, size, location and morphology of the ASD, as well as the quality of the surrounding septal tissue and its relationship with adjacent cardiac structures, should be carefully assessed. 3D TEE may complement this assessment by providing additional relevant anatomical information. During the procedure, TEE plays a fundamental role in real-time guidance, allowing proper deployment of the septal occluder device as well as immediate assessment of the result and early detection of potential complications. This article presents a practical approach to transcatheter closure of ostium secundum ASD, with emphasis on echocardiographic aspects in the pre-procedural, intraprocedural, and post-procedural phases.

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ABCIC

Case Report

Percutaneous Closure of Multiple Atrial Septal Defects with Hemodynamic Repercussions: Report of Two Cases in First-Degree Related Adult Patients

Débora Freire Ribeiro Rocha et al. Henrique Lima Guimarães Maurício Prudente Lopes João Batista Masson Silva Giulliano Gardenghi

Introduction

Atrial septal defects (ASD) account for 7–10% of congenital heart anomalies.^, ASD are classified as three anatomical types: ostium secundum (OS) (70% of cases), ostium primum (15–20% of cases), and sinus venosus (5–10% of cases) defects. OS involves the fossa ovalis region and results from poor embryological development of septum secundum or excessive absorption of septum primum resulting in the lack of a septum in the fossa ovalis.

Affected patients are usually asymptomatic. Exercise intolerance in the form of dyspnea or fatigue is the most common initial presentation. Echocardiography is a fundamental tool in the diagnosis and management of patients with ASD. Transthoracic echocardiography (TTE) identifies ASD type and size, shunt direction, and the presence of anomalous sinus venous drainage. Three-dimensional transesophageal echocardiography (3DTEE) details the morphology and anatomical relationships of the atrial septum, providing fundamental information for choosing treatment.^,

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ABCIC

Case Report

Lutembacher Syndrome: Case Report and Literature Review

Susan Caroline Azevedo Aredes et al. Gabriela da Silva Scopel Stella de Souza Carneiro Josana Azevedo Aredes … Aline Valério de Lima Patrick Ventorim Costa

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ABCIC

Case Report

Cor Triatriatum Sinistrum in Symptomatic Adult: Case Report

Francilayne Moretto dos Santos et al. Laura Gomes Flores Felipe Rodrigues Dias Leandro Shigueru Ikuta Ueda Eduardo Frederico Philippi

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ABCHF

Case Report

Heart Failure Due to Anomalous Pulmonary Vein Connection Associated with Atrial Septal Defect

Marcely Gimenes Bonatto et al. Ana Karyn Ehrenfried de Freitas Letícia dos Santos de Oliveira Rocha Claudinei Collatusso … Rafael de Almeida Torres Gustavo Gavazzoni Blume Lídia Zytynski Moura

Introduction

Anomalous pulmonary vein dreinage (APVD) is a rare disease, accounting for nearly 1 to 3% of congenital heart diseases. It may be total, when all PVs are involved, or partial, when only some PVs drain anomalously into one or more systemic veins or directly into the right atrium (RA).^, This condition occurs alone or in association with an interatrial septal defect, more often sinus venosus interatrial communication (IAC) (which accounts for nearly 5% of all IAC cases).

Patients with this condition may remain asymptomatic until adulthood. However, overload of right chambers may trigger symptoms, especially reduced functional capacity and progressive dyspnea, in addition to the development of pulmonary hypertension (PH) and ventricular dysfunction, which is the most severe disease scenario. Recognizing these symptoms and establishing early diagnosis and treatment is crucial to avoid disease progression and its prognostic implications.

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ABCIC

Case Report

Amplatzer™ Prosthesis Embolization After Percutaneous Atrial Septal Defect Closure: A Case Report

Raul Serra Valério et al. Alfredo Augusto Eyer Rodrigues Artur José da Silva Raoul Marly Maria Uellendahl … Carlos Eduardo Suaide Silva Luciana Braz Peixoto

Introduction

Atrial septal defect (ASD) is the most common congenital heart disease in adults; in specific cases, it has indications for treatment. The percutaneous approach has been highlighted as a less invasive method to close this defect, specifically in cases of ostium secundum ASD; however, it can have complications as described in the following report.

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ABC

Review Article

Genetic Syndromes Associated with Congenital Cardiac Defects and Ophthalmologic Changes – Systematization for Diagnosis in the Clinical Practice

Priscila H. A. Oliveira et al. Beatriz S. Souza Eimi N. Pacheco Michele S. Menegazzo … Ivan S. Corrêa Paulo R. G. Zen Rafael F. M. Rosa Claudia C. Cesa Lucia C. Pellanda Manuel A. P. Vilela

Abstract

Background:

Numerous genetic syndromes associated with heart disease and ocular manifestations have been described. However, a compilation and a summarization of these syndromes for better consultation and comparison have not been performed yet.

Objective:

The objective of this work is to systematize available evidence in the literature on different syndromes that may cause congenital heart diseases associated with ocular changes, focusing on the types of anatomical and functional changes.

Method:

A systematic search was performed on Medline electronic databases (PubMed, Embase, Cochrane, Lilacs) of articles published until January 2016. Eligibility criteria were case reports or review articles that evaluated the association of ophthalmic and cardiac abnormalities in genetic syndrome patients younger than 18 years.

Results:

The most frequent genetic syndromes were: Down Syndrome, Velo-cardio-facial / DiGeorge Syndrome, Charge Syndrome and Noonan Syndrome. The most associated cardiac malformations with ocular findings were interatrial communication (77.4%), interventricular communication (51.6%), patent ductus arteriosus (35.4%), pulmonary artery stenosis (25.8%) and tetralogy of Fallot (22.5%).

Conclusion:

Due to their clinical variability, congenital cardiac malformations may progress asymptomatically to heart defects associated with high morbidity and mortality. For this reason, the identification of extra-cardiac characteristics that may somehow contribute to the diagnosis of the disease or reveal its severity is of great relevance.

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Clinicoradiological Session

Case 5 / 2017 – Scimitar Syndrome and Pulmonary Sequestration in Natural Progression in a 68-Year-Old Woman

Edmar Atik et al. Álvaro Francisco Gudiño Marcelo Lopes Montemor

Clinical data

Tiredness and palpitations during physical work for approximately 20 years, without progression. Some episodes of small-volume hemoptysis during this period. Taking enalapril for hypertension and metformin for diabetes.

Physical examination: good general condition, eupneic, acyanotic. Weight: 56 Kg, Height: 160 cm, blood pressure (right arm): 140/90 mm Hg, HR: 95 bpm, oxygen saturation = 99%.

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ABC

Anatomopathological Session

Case 1/2016 – 56-Year-Old Male with Atrial Septal Defect, Pulmonary Arterial Hypertension, Hospitalized Due to Eisenmenger Syndrome

Carolina Santana et al. Antonio Augusto B. Lopes Antonio Fernando Lins de Paiva Luiz Alberto Benvenuti

Postmortem examination

The heart was enlarged and globoid, weighing 832 g. On gross examination, the RV was prominent. The opening of the cavities evidenced eccentric hypertrophy of the four chambers, mainly the right ones, which showed marked dilation. A large ASD was identified in the fossa ovalis (ostium secundum type), measuring 3.5 x 3.0 cm (). The ventricular septum was intact, no other heart malformation being identified. The microscopic examination revealed hypertrophy of the cardiomyocytes and myocardial interstitial fibrosis in both ventricles, with no evidence of ischemic lesions (infarction). The pulmonary trunk was dilated (diameter of 4.0 cm) and showed complicated atherosclerosis, with calcified areas. The right pulmonary artery showed calcified atherosclerosis, organized partial thrombosis, and markedly dilation of its parenchymatous branches. The process was similar in the left lung, but the thrombosis was more extensive, affecting numerous arterial branches, in an occlusive way, including the pulmonary hilum (). The microscopic examination of the lungs confirmed the extensive organizing arterial thrombosis detected on gross examination, in addition to revealing: organized and rechanneled arteriolar thrombosis (); recent focal thrombosis of a small artery and small hemorrhagic infarction in the lower lobe of the right lung (); areas of alveolar hemorrhage; anthracosis; microscopic evidence of chronic bronchitis; and diffuse pulmonary emphysema. In addition, a small in situ adenocarcinoma was found in the right lung, measuring 9 mm. Moderate aorta atherosclerosis was identified, with calcified plaques. Neither pneumothorax nor bronchopneumonia were evidenced.

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ABC

Clinicoradiological Session

Case 1/2016 – Aortic Coarctation and Atrial Septal Defect submitted to Percutaneous Repair in Adult Patient

Edmar Atik et al. Raul Arrieta Roberto Kalil Filho

Complementary Examinations

The Electrocardiogram showed sinus rhythm, first-degree atrioventricular block and complete right bundle-branch block. A P: +50º, AQRS: +115º, AT: 0º. QRS Duration: 0.14″, PR 0.21 ms ().

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ABC

Artigo Original

Results of simplified single-patch repair for complete atrioventricular septal defect

Ana Paula Tagliari et al. Daniel Augusto Schröder Guaracy Teixeira Filho Paulo Roberto Prates … João Ricardo M. Sant'Anna Ivo A. Nesralla Renato A. K. Kalil

BACKGROUND: Since Wilcox’s description of the simplified single-patch technique for atrioventricular septal defect (AVSD) repair in 1997, several studies have compared that technique with the two-patch technique. OBJECTIVE: To report the mid- and long-term results of the simplified single-patch technique for complete AVSD repair. METHODS: Retrospective study of 16 consecutive cases between January 2001 and December 2011. The patients’ mean age was 18.31 ± 34.19 months (2 months – 11 years), and their mean weight, 7.80 ± 6.12 kg (3.77 – 25.0 kg). Six patients were males and 14 had Down syndrome. Mean follow-up duration was 54.97 ± 47.79 months. RESULTS: Mean cardiopulmonary bypass time was 74.63 ± 18.48 min (49 – 112 min), and mean aortic cross-clamp time, 46.44 ± 11.89 min (34 – 67 min). Two patients died during hospitalization (12.5%), both of cardiovascular causes. Three patients underwent reoperation due to left atrioventricular (AV) valve regurgitation, and two had third-degree VA block, requiring permanent pacemaker implantation. No patient had left ventricular outflow tract obstruction. The 14 surviving patients remain asymptomatic, ten of whom with mild left VA valve regurgitation (71.42%). CONCLUSION: The simplified single-patch technique for complete AVSD repair proved to be feasible, providing adequate correction of the defects and favorable clinical and echocardiographic outcome in the mean 57.97-month follow-up.

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ABC

Artigo Original

Percutaneous versus surgical closure of atrial septal defects in children and adolescents

Rodrigo Nieckel da Costa et al. Marcelo Silva Ribeiro Fabricio Leite Pereira Simone Rolim Fontes Pedra … Marcelo Biscegli Jatene Ieda Biscegli Jatene Carlos R. Ferreiro Maria Virginia Tavares Santana Valmir Fernandes Fontes Carlos Augusto Cardoso Pedra

BACKGROUND: There is a scarcity of data comparing percutaneous and surgical closure of the secundum atrial septal defect (ASD). OBJECTIVES: Assessment of safety and efficacy of both methods of treatment in a referral center affiliated with the Ministry of Health. METHODS: Observational, prospective, non-randomized study of two cohorts of children and adolescents younger than 14 years, treated by catheterization or surgery. Data was collected prospectively in the percutaneous group (A) and retrospectively in the surgical group (B). RESULTS: A total of 75 patients (pts) were enrolled in group A from April 2009 to October 2011 and 105 pts were treated in group B from January 2006 to January 2011. Age was older and weight was higher in group B and the ASD diameter was similar in both groups. Technical success was achieved in all procedures and there were no deaths. Complications (most minor) occurred in 68% of group B and 4% of A (p < 0.001). Rates of total occlusion or non-significant residual shunts were similar in both groups. Median hospitalization time was 1.2 days in group A and 8.4 days in group B (p < 0.001). CONCLUSION: Both treatment modalities are safe and effective, showing excellent outcomes. However, the percutaneous treatment has lower morbidity and shorter in-hospital stay length. These observations support the concept that percutaneous treatment of atrial septal defects should be regarded as the method of choice to manage selected patients with this condition.

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Artigo Original

Mitral valve and atrial septal defect surgery: minimally invasive or sternotomy approach

Josué V. Castro Neto et al. Emanuel Melo Juliana Fernandes Regina Gomes … Caroline Freitas João Machado Francisco Martins Aloísio Barbosa Bernardo Oliveira Cesar Gondim

BACKGROUND: To decrease the surgical trauma in heart procedures, minimally invasive (MI) techniques were alternatively introduced. OBJECTIVE: To compare MI surgical access with median sternotomy (MS) for the treatment of mitral valve (MV) disease and atrial septal defect (ASD). METHODS: Forty patients underwent surgery for correction of MV disease or ASD. Patients were divided into group A (GA) (n=20), access by right minithoracotomy and video-assistance; and group B (GB) (n=20), access by full MS. Aortic cross-clamp and cardiopulmonary bypass time, intensive care unit (ICU) time, hospital stay and morbidity were compared in this prospective study . RESULTS: Fifteen patients were submitted to MV procedures and five to ASD corrections in each group. There were 9 mitral replacements (7 bioprostetic and 2 mechanical) and 6 repairs in GA, and 10 (all bioprostetic) and five in GB. The mean aortic cross-clamp and cardiopulmonary bypass time, in minutes, were 65.1 ± 29.3 in GA and 50.2 ± 21.4 in GB (p=0.074); and 91.8±35 in GA and 63.7±27.3 in GB ( p=0.008). The mean ICU time, in hours, were 51.7 ± 16.3 in GA and 55.8±17.5 in GB (p=0.45). The in hospital stay, in days, were 5.2 ± 1 in GA and 6.4±1.5 in GB (p=0.009). CONCLUSION: MI access for correction of the MV disease and ASD implicated in a longer cardiopulmonary bypass time for finalization of the main procedure, nevertheless it didn´t affect patient’s recuperation. MI treated patients were discharged earlier than sternotomy treated patients.

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Case Report

Atrial septal defect and pulmonary hypertension in professional soccer player

Fernando Bianchini Cardoso et al. Marcos Bianchini Cardoso Roberto Itiro Nishimura Georgiane Crespi Ponta … Gustavo Calado de Aguiar Ribeiro Cledcyson Eloy Costa

This study reports a rare case of late diagnosis of atrial septal defect (ASD) with pulmonary hypertension (PH) through two-dimensional echocardiography (ECHO) in a professional soccer player who resumed high-performance sports activity after surgery.

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Artigo Original

Congenital heart disease in adults: outpatient clinic profile at the Hospital das Clínicas of Ribeirão Preto

Fernando Amaral et al. Paulo Henrique Manso João Antonio Granzotti Walter Villela de Andrade Vicente Andre Schmidt

BACKGROUND: Service experiences for adults with congenital heart disease have not been reported in our country. OBJECTIVE: To describe the basic clinical profile of adults with congenital heart disease in an outpatient tertiary care center. METHODS: We compiled data on age, gender, place of residence, primary diagnosis, and secondary diagnoses of 413 patients treated for seven years. RESULTS: G1 (untreated): 195 patients, 51% women, 57% between 14 and 30 years, 80% living in the region. The most frequent heart diseases were ventricular septal defect (VSD) (31%), atrial septal defect (ASD) (29%), and pulmonary stenosis (7%). The predominant secondary diagnoses were hypertension (9%) and arrhythmias (5%). G2 (treated): 218 patients, 56% women, 57% between 14 and 30 years, 81% living in the region. The most frequently treated heart diseases were: ASD (36%), tetralogy of Fallot (14%), coarctation of the aorta (12%), and VSD (11%). Sixty-nine (32%) patients were operated on for congenital heart diseases in adulthood. Sixteen (7%) underwent an interventional catheterization. The predominant secondary diagnoses were hypertension (18%) and arrhythmias (8%). CONCLUSION: In the study, most patients were treated invasively, all of them were residents in the region, and most of them were under 40 years of age. Defects such as ASD, VSD, and pulmonary stenosis predominated in the untreated group, whereas in the treated group, most patients had undergone surgical correction of ASD, tetralogy of Fallot, aortic coarctation, and VSD. Hypertension and arrhythmias were relevant in both groups, and a large variety of other comorbidities were also observed.

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Artigo Original

Morphological basis for the study of the interatrial septum in the human fetus

Hugo Becker Amaral et al. Paulo Zielinsky Aron Ferreira da Silveira Ijoni Costabeber … Luiz Henrique Nicoloso Olmiro Cezimbra de Souza Filho Marcelo Salum João Luiz Manica Juliana Silveira Zanettini Ane Micheli Costabeber

OBJECTIVE: To describe morphological features of the interatrial septum in normal fetuses, especially foramen ovale (FO) and septum primum (SP), in order to compare septum primum excursion with foramen ovale diameter. METHODS: Septum primum excursion (SPE) toward the left atrium (LA) and foramen ovale diameter (FOD) were measured in the hearts of ten formaldehyde-fixed human fetuses ranging from 28 to 36 weeks of gestation. Histological sections were obtained from the foramen ovale (FO), septum primum (SP), septum secundum (SS), left atrium (LA), and right atrium (RA). RESULTS: FOD and SPE measurements were the following: FOD 3.1-3.5 mm and SPE 2.8-3.1 mm in three fetuses with presumed gestational age (GA) of 28 weeks; FOD 3.3-3.5 mm, and SP excursion 4.0-5.0 mm in four fetuses with presumed GA of 34 weeks, plus FOD 3.3-4.5 mm and SPE 6.0-9.0 in three fetuses with presumed GA of 36 weeks. Cardiac muscular fibers were identified in both the septum primum and secundum. CONCLUSION: Based on its muscular components, it may be suggested that SP is active in character, influencing blood flow through the FO, SP mobility, and its excursion into the LA.

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Case Report

Right to left shunt through a patent foramen ovale without pulmonary hypertension

Thiago Costa Lisboa et al. Clarice Daniele Oliveira Costa Thiago Quedi Furian Silvia Regina Rios Vieira

This article describes a patient presenting with dyspnea and platypnea and whose only clinical finding was presence of patent foramen ovale with a right to left shunt, without pulmonary hypertension, characteristic of the rare Platypnea-Orthodeoxya Syndrome, with very interesting pathophysiological findings and with therapeutic alternatives not yet defined.

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