Scimitar syndrome: is the adult always asymptomatic?

INTRODUCTION: Scimitar syndrome (CS) is a rare congenital cardiac malformation that occurs in 2/100,000 live births with a 2:1 predominance in females, it belongs to the group of partial anomalous pulmonary venous connections, which has numerous described variations, being a subtype that constitutes only 3-5% of congenital cardiac anomalies. It is classified as a heart disease with increased pulmonary and cyanogenic flow, its diagnosis is usually early, but we will highlight the importance of attention to this pathology in adult patients without a previous diagnosis. CASE REPORT: Female patient, 39 years old, with dyspnea for 3 years, with progressive worsening of functional class. On physical examination, pulmonary auscultation was normal with 98% saturation. On cardiac auscultation, regular rhythm, with fixed split S2 and ejective systolic murmur 2+/4+ in the middle right sternal border. A chest X-ray was performed during the investigation, which showed a slightly arched tubular structure towards the right atrium with dextrocardia. In transthoracic echocardiogram evidenced anomalous pulmonary venous connections: the right pulmonary veins drain into the right atrium at the mouth of the inferior vena cava, with free flow, and the left into the left atrium. . Ostium secundum interatrial communication of 13.5 mm with bidirectional flow, significant dilation of the right cavities, with right ventricular myocardium with mild hypertrophy, with preserved function. PSVD 51mmHg and hypoplastic right pulmonary artery. DISCUSSION SC is a rare disease characterized by anomalous venous drainage from the lung directly into the inferior vena cava. The disease may be associated with right pulmonary hypoplasia, dextrocardia, cardiac abnormalities, and systemic pulmonary collaterals. The present case report draws attention to the late discovery of a congenital heart disease, with the patient already symptomatic and with possible important hemodynamic repercussions, highlighting the importance of always evaluating the clinical picture of dyspnea and heart failure in adults during the routine investigation. the presence of a congenital heart disease.

Anomalous pacemaker lead placement ­ a report of extrinsic compression of the left pulmonary artery

INTRODUCTION: The Atrioventricular Septal Defect (AVSD) corresponds to 3% of congenital heart diseases, being associated with genetic syndromes and other congenital malformations. One of the anatomical alterations present in AVSD is the posterior displacement of the atrioventricular node, located between the coronary sinus and the annulus of the atrioventricular valve, increasing the risk of injury to the same during mitral cleft repair, generating atrioventricular block (AVB), Such a complication may evolve with the need for definitive implantation of a pacemaker. ABSTRACT: A 27-year-old female patient with complex heart disease (left atrial isomerism, partial AVSD, muscular interventricular communication, single atrium, pulmonary hypertension), underwent atrial septation surgery, mitral cleft repair and ventriculoseptoplasty in the first year of life, evolving in the postoperative period with total AVB, requiring implantation of a permanent pacemaker 1 month after the surgical procedure. In follow-up, she demonstrated heart rate reversibility, being submitted to extraction of the pacemaker generator and plasty of the abdominal pocket at age 22, remaining with the endocardial electrode. The patient evolved with symptoms of right heart failure (dyspnea, with worsening of the functional class). During the current diagnostic investigation, a significant pulmonary gradient was evidenced by the transthoracic echocardiogram, but without valvular lesion. An angiotomography of the heart and basal vessels was performed, which showed extrinsic compression of the right pulmonary artery by the pacemaker wire, which generated the pulmonary stenosis evidenced by the echocardiogram. CONCLUSION: Extrinsic compression by endocardial pacemaker lead is a rare phenomenon that can be difficult to recognize. In the case reported, due to the left atrial isomerism and the need to implant a permanent pacemaker during childhood in the abdominal cavity, the electrode was implanted through the azygos venous system. With the development and growth of the patient, the fixed electrode implanted in the right ventricle pulled the azygos vein under the right pulmonary artery, generating extrinsic compression and pulmonary stenosis evidenced both by imaging methods and by the patient's clinical condition. It is clear the importance of late follow-up of patients who still have implanted electrodes, even without the use of a pacemaker, in complex anatomical situations.

Ischemic cardiomyopathy due to late diagnosis of alcapa syndrome

INTRODUCTION: ALCAPA Syndrome (Anomalous Left Coronary from the Pulmonary Artery) is the anomalous origin of the left coronary artery from the pulmonary artery. Also known as BlandWhite-Garland Syndrome, it is a rare congenital heart defect, present in 0.25-0.5% of patients with congenital heart defects. It can occur in isolation or be associated with other birth defects. Usually the diagnosis is early, made in the prenatal period and the need for its correction is immediate. CASE REPORT: A 63-year-old female patient sought emergency care with chest pain, dyspnea on moderate exertion, and palpitations. Myocardial scintigraphy was positive for ischemia, corresponding to the territory of the anterior descending artery. Cardiac catheterization was requested, in which an anomalous left coronary artery was observed in the pulmonary artery trunk. The transthoracic echocardiogram showed the following findings: akinesia of the apical and anteroapical septum, with hypokinesia of the anterolateral wall and of the mid-basal segments of the anterior wall, moderate left ventricular systolic dysfunction (ejection fraction of 37 %), pulmonary artery systolic pressure of 62 mmHg. It has magnetic resonance imaging of the heart, which shows the areas of infarction mentioned in the echocardiogram, without myocardial viability. DISCUSSION: ALCAPA syndrome is difficult to diagnose, and should be suspected in children with dilated cardiomyopathy. About 85% of cases manifest up to 2 months of age, but symptoms can be misinterpreted and the disease may be underdiagnosed. The disease requires surgical treatment to restore the flow of oxygenated blood to the myocardium. The procedure of choice consists of reimplanting the coronary artery in the aorta and it is estimated that the surgical mortality is less than 5-10%, with a good prognosis after reimplantation. The peculiarity of the case reported is due to the fact that the diagnosis was extremely late and with important irreversible hemodynamic REPERCUSSIONS: the patient has an ischemic cardiomyopathy without myocardial viability, a condition usually related to chronic coronary artery disease or acute coronary syndromes, rarely related to ALCAPA, since due to the severity of the syndrome, its diagnosis is early. Currently, due to the lack of myocardial viability, surgical correction would not bring benefits or recovery of heart muscle, opting for clinical treatment for cardiac remodeling.