Heart Defects,Congenital

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Editorial

The Challenge of Echocardiography in the Accurate Assessment of the Right Ventricle and Pulmonary Insufficiency

The current recognition of the importance of the right ventricle (RV) role in the evolution and prognosis of various cardiovascular conditions is indisputable and the proper assessment of its dimension and function has a direct impact on clinical management.

Pulmonary insufficiency (PI) is common after tetralogy of Fallot repair (POTF) and approach of congenital heart disease with pulmonary obstruction, being is an important determinant of outcome due to its consequences, causing RV dilation and dysfunction, exercise intolerance, increased risk of arrhythmias and sudden death.

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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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Clinicoradiological Correlation

Case 6/2020 – 16-Year-Old Adolescent with Severe Pulmonary Stenosis At Valvar Level, After Correction of Truncus Arteriosus using the Barbero-Marcial Technique in the First Month of Life

Edmar Atik et al. Miguel Barbero-Marcial

Clinical Data

The newborn, in heart failure with truncus arteriosus type I, underwent repair at 15 days of age, weighing 2800 g. of body weight, by the Barbero-Marcial technique. At that time, the right ventricular outflow tract was approached directly with the pulmonary trunk and a monocusp valve was placed in the pulmonary position.

The evolution was adequate, with heart failure control, and he remained asymptomatic and showed normal physical development. The clinical examination ruled out residual lesions, such as pulmonary valve insufficiency. Over time, while asymptomatic, a systolic murmur was identified in the pulmonary area, of progressive intensity, together with an increasing pressure gradient in the region of the pulmonary monocusp. At 2 years of age, it was 25 mmHg; at 5 years, 34 mm Hg; at 7 years, it was 40; at 13 years it was 90 and at 16 years it was 149 mmHg. The patient did not use any specific medications.

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Original Article

Predictors of Unfavourable Outcomes in Children and Adolescents Submitted to Surgical Mitral Valvuloplasty Secondary to Chronic Rheumatic Heart Disease

Renata Cristina Castro Cruz et al. Bruna Silva Cordeiro Felipe de Souza Santos Caroline Rodrigues Fernandes … Julia Maria Alves Gama Ana Marice Teixeira Ladeia

Abstract

Background:

Mitral valve repair in paediatric patients with chronic rheumatic heart disease is superior to valve replacement and has been used with good results.

Objective:

To identify predictors of unfavourable outcomes in children and adolescents submitted to surgical mitral valvuloplasty secondary to rheumatic heart disease.

Methods:

Retrospective study of 54 patients under the age of 16 operated at a tertiary paediatric hospital between March 2011 and January 2017. The predictors of risk for unfavourable outcomes were: age, ejection fraction, degree of mitral insufficiency, degree of pulmonary hypertension, presence of tricuspid insufficiency, left chamber dilation, preoperative functional classification, duration of cardiopulmonary bypass, duration of anoxia, presence of atrial fibrillation, and duration of vasoactive drug use. The outcomes evaluated were: death, congestive heart failure, reoperation, residual mitral regurgitation, residual mitral stenosis, stroke, bleeding and valve replacement. For all analyzes a value of p < 0.05 was established as significant.

Results:

Of the patients evaluated, 29 (53.7%) were female, with an average of 10.5 ± 3.2 years. The functional classification of 13 patients (25%) was 4. There was no death in the sample studied. The average duration of extracorporeal circulation was 62.7±17.8 min, and anoxia 50 ± 15.7 min. The duration of use of vasoactive drug in the immediate postoperative period has an average of 1 day (interquartile interval 1-2 days). The logistic regression model was used to evaluate the predictive variables for each unfavourable outcome. The duration of use of vasoactive drug was the only independent predictor for the outcomes studied (p = 0.007). Residual mitral insufficiency was associated with reoperation (p = 0.044), whereas tricuspid insufficiency (p = 0.012) and pulmonary hypertension (p = 0.012) were associated with the presence of unfavourable outcomes.

Conclusion:

The duration of vasoactive drug use is an independent predictor for unfavourable outcomes in the immediate and late postoperative period, while residual mitral regurgitation was associated with reoperation, and both tricuspid regurgitation and pulmonary hypertension were associated with unfavourable outcomes.

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The Challenge of Echocardiography in the Accurate Assessment of the Right Ventricle and Pulmonary Insufficiency

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

Introduction

Case 6/2020 – 16-Year-Old Adolescent with Severe Pulmonary Stenosis At Valvar Level, After Correction of Truncus Arteriosus using the Barbero-Marcial Technique in the First Month of Life

Clinical Data

Predictors of Unfavourable Outcomes in Children and Adolescents Submitted to Surgical Mitral Valvuloplasty Secondary to Chronic Rheumatic Heart Disease

Abstract

Background:

Objective:

Methods:

Results:

Conclusion:

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