Felipe Guimarães Machado, MD; Éric Guimarães Machado, MD; Maria Clara Menezes de Jesus Lisboa, MD; Léo Guimarães Soares, DDS, PhD; Paulo Sérgio Lopes Soares, MD; Gabriel Porto Soares, MD, PhD
Introduction
The Atrioventricular Septal Defect (AVSD) occurs in 2: 10.000 live births, accounting for 3% of Congenital Heart Disease (CHD), AVSD is more frequent in females and usually associated with carriers of the Down Syndrome (DS).[1,2] The AVSD is characterized by the absence of the atrioventricular septum, and five or more atrioventricular valve leaflets of varying sizes are usually present.[1,2]
The AVSD is characterized by the absence of the atrioventricular septum, and five or more atrioventricular valve leaflets of varying sizes are usually present.[1,2]
This clinical case is characterized by increased pulmonary blood flow in the first weeks of life. The natural history of this defect is survival of 54% at six months and 15% at two years.[1] If significant regurgitation of the common atrioventricular valve is present, a systolic cardiac murmur and gallop rhythm are frequently heard. Over time, pulmonary hypertension and congestive heart failure (CHF) are observed.[2]
The main cause of death in infants is CHF; after the first year of life, occlusive pulmonary vascular disease predominates.[1]
The diagnosis should be suspected in patients with CHF symptoms in the first months of life. Chest radiographs show cardiomegaly. The electrocardiogram may show left-axis deviation and bi-atrial and bi-ventricular enlargement, in addition to pressure and volume overload. Echocardiography is the main tool for both the diagnosis and anatomical classification of this malformation, according to Rastelli classification (Type A, B or C). The electrocardiogram also demonstrated the Atrial Septal Defect, which is commonly associated with AVSD. Echocardiography can still observe anatomical defects, such as atrial septal defect with the underlying common atrioventricular valve and Ventricular Septal Defect (VSD).
Medical treatment is aimed at improving the signs and symptoms of CHF. Medications used may be digoxin, diuretics (such as furosemide and spironolactone), and vasodilators (often angiotensin-converting enzyme inhibitors). Administration of these medications, pre-operatively, may result in an improved post-operative course and prognosis.
The surgery to repair AVSD involves the use of two patches (an atrial and a ventricular patch), which maintains the integrity of the valvular leaflets.[1]
FIGURE 1 Echocardiogram with Color Doppler. A: M mode or unidimensional left ventricle longitudinal section. B: M mode or one-dimensional aorta and left atrium longitudinal section. C: Apical 4-chamber view. D: Mitral flow demonstrated by Color Doppler. E: Aortic flow demonstratedby Color Doppler. F: Interatrial communication visualization.
Case Report
A 24-year-old woman was admitted to the cardiology clinic from the Hospital Universitário Sul Fluminense (HUSF), reporting effort dyspnea on exertion and chest pain.
The patient reported strong chest pain, which did not radiate, but was effort-related and was relieved by rest. The patient also presented with cyanosis of fingers and lips, numbness in left hemibody, more pronounced in the upper limb. Physical examination of the cardiovascular system showed a Grade 3/6 murmur at the left-midsternal border. The patient had no cognitive impairment, nor Down Syndrome stigmata.
An electrocardiogram was conducted, which showed sinus rhythm, 1st degree atrioventricular blockage, left-anterior hemiblock and change in lower and anteroseptal repolarization. Structural and functional study of the heart was requested through Doppler echocardiography.
On Color Dopper (Figure 1), right ventricular and right atrial enlargement were noted, as was the presence of large Atrial Septal Defects with left-to-right shunt, mild tricuspid insufficiency and mild pulmonary arterial hypertension. Other parameters were normal. Transesophageal echocardiography or cardiac MRI was recommended.
To read the full article, please go to the January 2016 Issue of CCT.