2020;114(4 suppl 1) – ABC CARDIOL

Case Report

Recurrent Atrial Myxoma in a Patient with Carney Complex. A Case Report and Literature Review

Laura A. Cervantes-Molina et al. David Ramírez-Cedillo Italo D. Masini-Aguilera Jaime G. López-Taylor … Michel Machuca-Hernández Dulman O. Pineda-De Paz

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.

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Case Report

Glycogen Storage Disease Type I (Von Gierke disease): Report of Two Cases with Severe Dyslipidemia

Julia Maria Avelino Ballavenuto et al. Jéssica D´Ório Dantas de Oliveira Renato Jorge Alves

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 and Cori analyzed liver biopsies of patients with similar symptoms, and observed a partial or total absence of glucose-6-phosphatase (G6Pase) enzyme activity – this entity became known as Von Gierke disease. Nordlie et al., in studies carried out in the 70’s, also using liver biopsies, observed normal levels of G6Pase enzyme, but with decreased activity. Thus, Glycogen Storage Disease Type I is characterized by G6Pase deficiency, a key enzyme in glycogen metabolism, which leads to the reduction in glycogenolysis and gluconeogenesis and, consequently, to hepatic accumulation of glucose-6-phosphate (G6P).

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Case Report

Giant Cell Arteritis with Aortic Involvement Leading to Cardio Vocal Syndrome (Ortner’s Syndrome)

Edgar Stroppa Lamas et al. Ricardo Luiz José Rogoni Bononi Paulo Augusto Cotta de Ávila Reis

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 multiple foci with intense lymphocytic presence. The occurrence of thoracic aortic aneurysms is reported in about 20% of patients.

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Anatomopathological Correlation

Case 1/2020 – A 56 Year-Old Woman Developed Heart Failure after a Presumed Diagnosis of Acute Myocardial Infarction and Mitral Valve Regurgitation with Rupture of Chordae Tendineae

Desiderio Favarato et al. Luiz Alberto Benvenuti

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.

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Clinicoradiological Correlation

Case 2/2020 – Anomalous Origin of the Left Coronary Artery from the Pulmonary Trunk, Under Natural Evolution in a 75-Year-old Asymptomatic Woman

Edmar Atik et al. Oliver Kligerman Luiz Kajita

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 rosuvastatin, levothyroxine and vitamin D.

Physical examination: good overall status, eupneic, acyanotic, normal pulses in the four limbs. Weight: 49.8 Kg, Height: 143 cm, BP:120 x 80 mmHg, HR: 74 bpm.

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Case Report

A Life-threatening Combination: Indomethacin and Dabigatran

Adem Adar et al. Orhan Onalan Fahri Cakan

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.

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Clinicoradiological Correlation

Case 3/2020 – Pulmonary Atresia, Interventricular Communication and Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta developing after Prior Left Central Shunt, in a Symptomatic 40-year-old Adult.

Edmar Atik et al. Maria Angélica Binotto Alessandra Costa Barreto

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 ( ).

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Case Report

Painful Left Bundle Branch Block Syndrome in a Patient Referred to Electrophysiologic Study: A Case Report

José Nunes de Alencar Neto et al. Marcel Henrique Sakai Saulo Rodrigo Ramalho de Moraes Elano Sousa da Frota … Claudio Cirenza Angelo Amato Vincenzo de Paola

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 effort. The chest pain in the case of the painful LBBB syndrome may range from a mild discomfort to a disabling condition.

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Case Report

Acute Decompensated Heart Failure due to Chikungunya Fever

Carolina Cunto de Athayde et al. Fabio Akio Nishijuka Márcia Cavalcanti de Campos Queiroz Monica Medeiros Luna … Jaime Lobo Figueiredo Nadia Matias de Albuquerque Sebastião Carlos Ribeiro de Castilho Renata R. T. Castro

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 common, decompensation may occur due to viral infections, which justifies vaccination against influenza in these patients.

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Clinicoradiological Correlation

Case 1/2020 – Very Accentuated Isthmic Coarctation of the Aorta in a Young Individual with Arterial Hypertension Relieved by Interventional Catheterization

Edmar Atik et al. Raul Santiago Arrieta Renata Cassar

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 since a few months before.

Physical examination: Good overall status, eupneic, acyanotic, wide pulses in the upper limbs and absent in the lower limbs. Weight: 45.5 Kg, Height: 163 cm, right upper limb BP and left upper limb BP = 155/80 mmHg, HR: 55 bpm. Aorta easily palpated at the suprasternal notch.

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Case Report

Transthyretin Amyloidosis (ATTR) – The Role of Multimodality in the Definitive Diagnosis

Tonnison de Oliveira Silva et al. Eduardo Sahade Darze Luiz Eduardo Fonteles Ritt André Luiz Cerqueira Almeida Antônio Ximenes

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.

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Case Report

Acute Myocardial Infarction as First Onset of Polycythemia Vera

Caroline Ferreira da Silva Mazeto Pupo da Silveira et al. Lívia Beatriz Santos Limonta Vitali Fabiana Garcia Faustino Alejandra Del Carmen Villanueva Maurício … Renato Teixeira Silméia Garcia Zanati Bazan

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 mean normal predicted value; bone marrow biopsy showing hypercellularity for age with trilineage growth; presence of JAK2V617F or JAK2 exon 12 mutation. A minor criterion is reduced serum erythropoietin level. Diagnosis requires meeting either all 3 major criteria or 2 major criteria and the minor criterion. The patient is also considered as at thrombosis risk; those aged over 60 or with thrombosis history are considered at considered high risk; if both risk factors are absent, low risk is considered.

Treatment includes cytoreductive drugs, such as hydroxyurea, antiplatelet agents and therapeutic sangrias.

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Case Report

Image Diagnosis: An Anomalous Origin of Left Coronary Artery from the Pulmonary Artery

Haisong Bu et al. Tianli Zhao

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 achieve, especially in patients undergoing reoperation.

Chronic ischemic mitral regurgitation (MR) develops as a consequence of coronary artery disease in the absence of primary leaflet abnormalities or chordal pathology: ischemic cardiopathy causes remodeling of left ventricular geometry, displacement of papillary muscles, leaflet tethering and annular dilatation, leading to functional mitral insufficiency. The outcome of these patients represents a challenging problem for both cardiologists and cardiac surgeons. In fact, the role of mitral valve surgery (MVS) associated with coronary artery revascularization is still debated.

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Case Report

Syncope as a Phenotypic Expression of Hereditary Transthyretin Amyloidosis Val142Ile (Val122Ile)

Nágela S. V. Nunes et al. João Paulo Moreira Carvalho Fernanda Salomão Costa Marcelo Souto Nacif … Joelma Dominato Claudio Tinoco Mesquita Evandro Tinoco Mesquita

Introduction

Transthyretin amyloidosis (ATTR) is a familial disease caused by one of more than 100 described mutations, where there is production of amyloids that are deposited in tissues. Phenocopies include neuropathy (autonomic and peripheral), cardiomyopathy, renal, gastrointestinal, vitreous and meningeal involvement, which vary according to the genetic mutation, ethnicity and geographical origin, even among individuals with the same mutation or within the same family.

Syncope (transient loss of consciousness caused by global cerebral hypoperfusion) in the presence of heart disease confers risk of fatal events. The Val142Ile mutation has heart failure with preserved ejection fraction (HFpEF) as the predominant clinical phenotype, with syncope being an uncommon symptom.^,

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