Introduction Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly with a mortality of 90% by 1 year of age without surgical intervention. Nowadays the procedure of choice for correction of ALCAPA depends on the establishment of a dual coronary artery system by direct reimplantation of the anomalous left coronary artery (LCA) into the ascending aorta. However, anatomic variations of the origin of the anomalous LCA often make this aim difficult to […]

Introduction Transthyretin amyloidosis (ATTR) is a rare cause of restrictive cardiomyopathy and/or peripheral polyneuropathy, of a progressive, irreversible and fatal nature, underdiagnosed and with its definitive diagnosis performed late. Early diagnosis, characterization of the type of amyloidosis and subsequent establishment of specific therapy are fundamental for a better prognosis of this disease. We present a case of ATTR where clinical suspicion associated with multimodality diagnosis — nuclear medicine — was able to safely deliver diagnosis, without the need for biopsy. […]

Introduction Although rare, several bleeding complications may occur in patients receiving dabigatran. The risk of bleeding is particularly high in patients with impaired kidney functions or in patients who are on concomitant nephrotoxic drugs. We report a case of massive pleuropericardial effusion developed after the initiation of indomethacin treatment in a patient who was receiving dabigatran for deep venous thrombosis. […]

Introduction Giant cell arteritis or temporal arteritis is the most common large-vessel arteritis in Western countries, generally affecting patients over 50 years of age. In most cases cranial symptoms are present, however, exclusively extracranial manifestations can occur in up to 22% of cases. The involvement of aortic and other large vessels (carotid, subclavian) is frequent in this context. The involvement of the adventitia of the artery is observed mainly by activation of dendritic cells generating a granulomatous inflammatory process in […]

Introduction Heart failure (HF) is a chronic condition with worldwide high and growing prevalence. – It is extremely important to identify the cause of decompensated HF in order to manage cases correctly, given that identification makes it possible to implement specific treatment and prevent new hospitalizations. In Brazil, the main causes are poor adherence to medical treatment (30%) and infections (23%), mainly pulmonary bacterial ones. For this reason, patients with HF should receive the pneumococcal vaccine. Although it is less […]

Introduction Glycogen storage diseases are a group of disorders caused by inherited errors of metabolism, resulting in abnormal glycogen concentration and/or structure in various body tissues. Nowadays, there are 14 types of glycogen storage disease, which are classified according to enzyme or transporter deficiency and to the different organ distribution of these defects. In 1929, Edgar von Gierke described an increased deposition of glycogen in body tissues in the autopsy reports of young individuals with hemorrhagic manifestations. In 1952, Gerty […]

Introduction Polycythemia vera (PV) is a chronic clonal progressive myeloproliferative neoplasm characterized by an absolute increase in erythrocytes and, usually, leukocytosis, thrombocytosis, and splenomegaly. Its incidence rates around 2.8/100,000 people per year. Diagnosis is confirmed using the criteria defined by the 2016 revised World Health Organization (WHO) guidelines. Major criteria include hemoglobin levels over 16.5 and 16.0 g/dL or hematocrit over 49 and 48% in men and women, respectively, or increased red cell mass of more than 25% above the […]

Introduction Primary cardiac tumors are uncommon. They have an incidence between 0.0017% and 0.28% corresponding to 17 and 2,800 of primary heart tumors per 1 million autopsies. Cardiac Myxoma (CM) is a benign neoplasm and represents the most common one among primary cardiac tumors in adults. CMs have an annual incidence of 0.5–0.7 of surgically resected cases per one million; with the majority of cases showing sporadic appearance and less than 10%, a family inheritance pattern. […]

Introduction The development of chest pain associated with intermittent left bundle branch block (LBBB) in the absence of coronary artery disease has been described in the literature as painful left bundle branch block syndrome. The mechanism responsible for the chest pain is unknown, but the main current hypothesis is related to acute cardiac dyssynchrony. In this syndrome, the LBBB occurs when the cycle length is equal to or less than the refractory period of the left bundle, mainly during physical […]

Clinical data Arterial hypertension had been detected 6 months before, after study-related stress in a 16-year-old individual. At the time, diagnostic images (echocardiography and angiotomography) confirmed the presence of accentuated isthmic coarctation of the aorta, with many collaterals that filled the descending aorta. Blood pressure was 170/80 mmHg, which decreased to 130 to 150/80 mmHg with propranolol-80 mg/day. He had been previously submitted to surgery for atrial septal defect closure at 4 years of age. He reported fatigue at exertion […]

Clinical data Patient evolves without symptoms in her daily routine as a domestic worker. She denies any symptoms of palpitations, precordial pain or fatigue. When she was aged 62, a cardiac catheterization revealed the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA, or Bland-White-Garland syndrome), after an altered routine exercise stress test. Since then, the patient reports her well-being, although she is aware of the existence of this anomaly. She is being treated with […]

Complementary examinations Electrocardiogram: Sinus rhythm, signs of right cavity overload, with apiculate P wave + 70º, QRS complex showing predominance of S waves from V4 to V6 and axis deviated to the right (AQRS= +110°). The T wave was negative in the precordial leads with diffuse ventricular repolarization changes in all other leads ( ). […]

Clinical aspects A 55-year-old patient with arterial hypertension and heart failure for one year and with chest pain, had a cardiorespiratory arrest five months before, developed heart failure and mitral regurgitation due to mitral valve prolapse and rupture of chordae tendineae. The diagnostic impression is that heart failure due to mitral valve disease preceded the episode of precordial pain and severe dyspnea. […]