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.