Cardiomiopatia periparto associada à síndrome do QT longo e síncope por taquicardia ventricular polimórfica: relato de caso / Peripartum cardiomyopathy associated with long QT syndrome and polymorphic ventricular tachycardia syncope: a case report

A cardiomiopatia periparto é uma causa rara de insuficiência cardíaca no período entre o último mês de gestação e os cinco meses após o parto. A síndrome do QT longo caracteriza-se pelo atraso da repolarização ventricular e pode se manifestar com síncope e morte súbita devido a um tipo de taquicardia ventricular polimórfica conhecida como torsades de pointes. Descrição do caso: J.S., 26 anos, sexo feminino, natural e procedente de São Paulo. Paciente puérpera - 40º dia (G3P3A0), procurou o pronto-socorro com queixa de síncope durante amamentação e dispneia em moderados esforços. Durante a avaliação no PS, evoluiu para desconforto torácico e agitação psicomotora, sendo notada taquicardia ventricular não sustentada no monitor cardíaco ( torsades de pointes), que foi controlada com cardioversão elétrica e sulfato de magnésio intravenoso. O eletrocardiograma mostrou ritmo sinusal, alteração difusa da repolarização ventricular e intervalo QTc de 580 ms. O ecocardiograma mostrou disfunção sistólica moderada, com fração de ejeção do ventrículo esquerdo de 43% à custa de hipocinesia difusa. Após avaliação da equipe de arritmologia chegou-se ao diagnóstico de cardiomiopatia periparto associado à síndrome do QT longo. Foi iniciado tratamento otimizado para insuficiência cardíaca e implantado cardiodesfibrilador por causa de episódios recorrentes de arritmia durante a internação. Discussão: A cardiomiopatia periparto é uma doença rara, porém, tem taxa de mortalidade elevada, entre 18% e 56%. A paciente descrita satisfez os quatro critérios para o diagnóstico: sintomas de insuficiência cardíaca nos primeiros 5 meses depois do parto, ausência de cardiomiopatia prévia, etiologia desconhecida e disfunção sistólica com FEVE < 45%. A síndrome do QT longo é uma doença genética de apresentações variáveis. Os fatores que desencadeiam as taquiarritmias são situações de instabilidade elétrica por hiperatividade do sistema simpático e também situações raras, como a cardiomiopatia periparto. Em casos de arritmias ventriculares graves, o tratamento é o implante de cardiodesfibrilador. Conclusão: A associação da cardiomiopatia periparto com a síndrome do QT longo é rara. A gravidade associada a essas condições torna importante o diagnóstico precoce e tratamento imediato pelo potencial risco de morte associado a ambas as condições clínicas

Peripartum cardiomyopathy is a rare cause of heart failure during the period between the last month of pregnancy and five months after delivery. Long QT syndrome is characterized by a delay in ventricular repolarization and may manifest with syncope and sudden death due to a type of polymorphic ventricular tachycardia known as torsades de pointes. Case description: J.S., 26-years-old, female, born and residing in São Paulo, Puerperal - 40th day (G3C3A0), went to the emergency room complaining of syncope during breastfeeding and dyspnea on moderate exertion. During evaluation in the ER, the patient developed thoracic discomfort and psychomotor agitation, with non-sustained ventricular tachycardia on the cardiac monitor (torsades de pointes), which was controlled with electrical cardioversion and intravenous magnesium sulfate. The electrocardiogram showed sinus rhythm, diffuse alteration of ventricular repolarization and QTc interval of 580 ms. The echocardiogram showed moderate systolic dysfunction, with a left ventricular ejection fraction of 43% influenced by diffuse hypokinesia. After evaluation by the arrhythmology team, the diagnosis of peripartum cardiomyopathy associated with long QT syndrome was made. Optimized treatment for heart failure was initiated and a cardioverter-defibrillator was implanted due to recurrent episodes of arrhythmia during hospitalization. Discussion: Peripartum cardiomyopathy is a rare disease, but it has a high mortality rate, between 18% and 56%. The patient described met the 4 diagnostic criteria: symptoms of heart failure in the first 5 months after delivery, absence of prior cardiomyopathy, unknown etiology, and systolic dysfunction with LVEF<45%. Long QT syndrome is a genetic disease of varying presentations. The factors that trigger the tachyarrhythmias are situations of electrical instability due to sympathetic system hyperactivity and rare situations, such as peripartum cardiomyopathy. In cases of severe ventricular arrhythmias, the treatment is a cardioverter-defibrillator implant. Conclusion: The association of peripartum cardiomyopathy with long QT syndrome is rare. The severity associated with these conditions points out early diagnosis and immediate treatment important because of the potential risk of death associated with both clinical conditions

Effect of Balloon Catheter Angioplasty Alone for Recurrent Bilateral Renal Artery Restenosis and Quick Deterioration for Hemodialysis: An Alternative for in-Stent Restenosis

Aim: Atherosclerotic renal artery stenosis is one of the main causes of secondary hypertension and results in a progressive loss of renal function. This case reports recurrent hypertensive pulmonary edema and worsening renal function successfully treated with multiple percutaneous renal interventions. Presentation of the Case: A 62-year-old female patient was admitted for carotid endarterectomy. She had uncontrolled blood pressure and worsened renal function. Bilateral renal artery stenosis was diagnosed and endovascular procedure plus stent was performed in both renal arteries. She was discharged with an improvement in renal function and blood pressure. Three months later, she presented a sudden onset of pulmonary hypertensive edema. Renal arteriography confirmed bilateral in-stent restenosis and a new bilateral angioplasty plus stent was fulfilled. Six months later, new worsening of dyspnea, severe renal dysfunction, and new hospitalization were indicated renal replacement therapy. Renal arteriography showed again bilateral renal artery in-stent restenosis and bilateral balloon catheter angioplasty with no stent was performed. She was discharged, with no need for hemodialysis, improved blood pressure and proposal for further discussion about surgical revascularization. Discussion: When severe reduction of glomerular filtration rate occurs in hypertensive patients with high risk for atherosclerotic disease, it seems prudent to examine renal arteries. Once a diagnosis is made, if blood pressure control cannot be achieved, mainly associated with progressive renal dysfunction, it is recommended restoration of renovascular supply. Conclusion: Percutaneous renal intervention with no-stenting in bilateral in-stent restenosis and recent onset of hemodialysis may contribute to reversion of renal dysfunction even in patients undergoing to previous interventions.

Intermittent claudication and severe renal artery stenosis are independently associated in hypertensive patients referred for renal arteriography

OBJECTIVE: The purpose of this study was to evaluate the association between the presence of clinical symptoms of peripheral artery disease and severe renal artery stenosis in patients referred for renal angiography. METHOD: We included 82 patients with clinical suspicion of renovascular hypertension and performed an imaging investigation (renal Doppler ultrasound and/or renal scintigraphy) for possible renal artery stenosis. All patients underwent renal arteriography and were examined for peripheral artery disease based on the presence of intermittent claudication and ankle-brachial index test results. Severe renal artery stenosis was defined as a lesion causing 70% obstruction. RESULTS: Severe renal artery stenosis was present in 32 of 82 (39%) patients. Patients with severe renal artery stenosis were older (63±12 vs 56±12 years, p=0.006), had more intermittent claudication (55 vs 45%, p=0.027), and had a greater prevalence of an ankle-brachial index <0.9 (44% vs 20%, p=0.021) than patients without severe renal artery stenosis. Multivariate logistic regression analysis showed that the presence of intermittent claudication was independently associated with renal artery stenosis ≥70% (OR: 3.33; 95% CI 1.03-10.82, p=0.04), unlike the ankle-brachial index, which showed no association (OR: 1.44; 95% CI 0.37-5.66, p=0.60). CONCLUSION: Intermittent claudication is independently associated with severe renal artery stenosis (≥70%) in patients clinically suspected of having renovascular hypertension.

Carcinoid Heart Disease: A Review.

Carcinoid tumors are rare malignancies able to spread and produce bioactive humoral products, mainly serotonin, which is responsible for the Carcinoid Syndrome (CS); its features are: flushing, diarrhea, bronchospasm and valvular heart disease. The Carcinoid Heart Disease (CHD) importantly worsens prognosis and it is found in up to 50% of patients with CS. After being produced by liver implants, serotonin finds its way straight into the right heart cavities, leading to valve tissue aggression and ventricular dysfunction. Early CHD diagnosis is still a challenge due to the asymptomatic initial stage, until right heart failure develops along with ascites, swelling and hepatomegaly. Echocardiography is still the main tool for diagnosis, especially due to its ability to appropriately evaluate ventricular and valve function, cardiac morphology and hemodynamics. Tricuspid regurgitation, pulmonic stenosis and dilated cardiomyopathy are the main impairments found in CHD. Magnetic resonance imaging has also developed well in this field, and it is now believed to be essential, due to accurate right cavities evaluation and fibrosis detection. For better prognosis, early diagnosis must be pursued, which has impact on clinical management and valve repair surgical decision.

Splenic Rupture after Infective Endocarditis by Enterococcus faecalis: Case Report.

Aims: In patients with infective endocarditis, with risk of embolization, early identification of parenchymal changes may suggest the risk of splenic rupture. Presentation of Case: A 68-year-old male presented with a history of 2 months of fever and also left upper quadrant pain initiated 2 days before admission. Transesophageal echocardiogram demonstrated the presence of two mobile vegetations on the ventricular side of the aortic valve; the largest diameter being 2.1 cm. Enterococcus faecalis was isolated in blood culture after a diagnosis of subacute aortic valve infective endocarditis. He complained of abdominal pain. An abdominal computed tomography scan revealed infarction of the upper region of the spleen (septic embolism). Therapy with penicillin and gentamicin was initiated, but the patient developed symptoms of heart failure that led to a surgical treatment, and aortic bioprosthesis was implanted on day 14. On day 5 postoperatively, the patient developed sudden hemorrhagic shock signs due to splenic rupture and underwent emergency splenectomy. A pathological examination revealed areas of splenic laceration of the capsule, splenic infarction areas, and the absence of abscesses. Splenic rupture is a complication much rarely occurring due to infectious endocarditis caused by E. faecalis. Conclusion: This case highlights the importance of conducting serial imaging, particularly in symptomatic patients, for the early detection of parenchymal changes that may suggest the risk of rupture.

Estudo dos determinantes de doença arterial coronária grave em pacientes hipertensos com indicação de arteriografia renal por suspeita de estenose de artéria rena / Determinants of severe coronary artery disease in hypertensive patients with indications of renal angiography for suspected renal artery stenosis

INTRODUÇÃO: Em pacientes hipertensos com suspeita de doença arterial coronária (DAC), indicações para a realização de arteriografia renal no mesmo procedimento da cinecoronariografia estão bem estabelecidas. Entretanto, em hipertensos com suspeita de estenose de artéria renal (EAR) com indicação de arteriografia renal, não estão bem definidos os determinantes da presença de DAC grave. OBJETIVOS: Avaliar a prevalência e os determinantes de DAC grave em pacientes hipertensos com indicação de arteriografia renal por suspeita de EAR. METODOLOGIA: Oitenta e dois pacientes com suspeita clínica de EAR foram submetidos à cinecoronariografia e arteriografia renal no mesmo procedimento. Lesão arterial significativa em artérias renal e/ou coronária foi definida por obstrução luminal maior ou igual a 70 por cento. RESULTADOS: Obstrução luminal significativa, tanto em artéria coronária quanto em renal, foi encontrada em 39 cento dos pacientes. Os pacientes com DAC grave apresentaram idade mais avançada (63±12 vs. 56±13 anos; p=0,03), maior prevalência de angina (41 vs. 16 cento; p=0,013), maior diâmetro do átrio esquerdo (44,7 vs. 40,6mm; p=0,005) e maior velocidade de onda de pulso (12,6 vs. 10,7 m/s, p=0,02), comparados com os pacientes sem DAC grave. A presença de EAR significativa esteve associada a uma maior prevalência de DAC grave comparada a pacientes sem a lesão (66 cento vs. 22 cento; p<0,001). A análise multivariada demonstrou que EAR70 cento esteve associada com DAC grave de maneira independente (OR: 11,48; 95 centoCI 3,2-40,2; p<0,001), mesmo em pacientes sem angina (OR: 13,48; 95 centoCI 2,6-12,1; p<0,001). CONCLUSÃO: Há elevada prevalência de doença coronária grave em pacientes hipertensos com estenose de artéria renal significativa. A presença de estenose maior ou igual a 70 cento, verificada em arteriografia renal, é preditor forte e independente para a presença de DAC grave, mesmo na ausência de angina...

INTRODUCTION: In patients with suspected coronary artery disease (CAD), indications for performing coronary and renal angiography at the same setting are well established. However, in hypertensive patients with suspected renal artery stenosis (RAS) with indication for renal angiography, it is not well defined the determinants of the presence of severe CAD. OBJECTIVES: We aimed to evaluate the prevalence and determinants of severe CAD in hypertensive patients referred to renal angiography for the diagnosis of RAS. METHODS: Eighty-two consecutive patients with high clinical risk for RAS systematically underwent renal angiography and coronary angiography at the same procedure. Significant RAS and CAD were defined as arterial luminal obstruction 70 percent. RESULTS: Either significant RAS or significant CAD were present in 32/82 patients (39 percent). Patients with severe CAD were older (63±12 vs. 56±13 years, p=0.03), had more angina (41 vs. 16 percent; p=0.013), higher left atrial diameter (44.7 vs. 40,6mm; p=0.005), and higher pulse wave velocity (12.6 vs. 10.7 m/s); p=0.02) compared to patients without significant CAD. Significant RAS was associated with an increased prevalence of severe CAD compared to patients without the lesion (66 percent vs. 22 percent, respectively; p<0.001). Binary logistic regression analysis showed that RAS 70 percent was independently associated with severe CAD (OR: 11.48; 95 percentCI 3.2-40.2; p<0.001), even in patients without angina (OR: 13.48; 95 percentCI 2.6-12.1; p<0.001). CONCLUSION: The prevalence of severe CAD in hypertensive patients with significant RAS is high. The presence of RAS 70 percent, diagnosed by renal angiography, is a strong and useful predictor to identify severe CAD, independently of the presence of angina...

Associação de estenose de artéria renal e doença coronária em pacientes com hipertensão e aterosclerose / Association between renal artery stenosis and coronary disease in patients with hypertension and atherosclerosis

Existem fortes evidências da associação de estenose de artéria renal em pacientes portadores de doença arterial coronária, pois ambas as situações apresentam fatores em comum para aterosclerose. Neste artigo foram revisadas as principais evidências da associação e a importância do diagnóstico dessas duas entidades na prática clínica.

Strong evidences support the association of renal arterystenosis in patients with coronary artery disease, since both conditions share similar risk factors for atherosclerosis. In this review we address the principal evidences for this association and the importance of the correct diagnosis for the clinical practice.

Alterações vasculares da hipertensão arterial / Vascular modifications of arterial hypertension

As principais complicações da hipertensão arterial são consequências de lesões vasculares. As alterações da estrutura e função das pequenas artérias de resistência em reposta ao aumento de tensão na parede da artéria incluem espessamento arterial, diminuição do lúmen e vasoconstrição aumentada. As grandes artérias que apresentam capacidade de condução e de amortecimento respondem ao aumento de pressão com aumento da rigidez e diminuição da distensibilidade, que piora com o envelhecimento. O aumento da rigidez no paciente hipertenso está associado a maior risco cardiovascular, assim como o remodelamento inadequado das pequenas artérias. As principais manifestações clínicas das lesões vasculares da hipertensão arterial incluem a hipertensão maligna, a doença arterial periférica, o auneurisma e a dissecção da aorta. O tratamento adequado da pressão arterial pode reverter as lesões vasculares e consequentemente melhorar o prognóstico cardiovascular do paciente hipertenso.

Tumor de Franz: relato de um caso / Frantz's tumor: report of a case

O tumor de Frantz é uma neoplasia rara do pâncreas. Ocorre principalmente em pacientes jovens e apresenta umbom prognóstico. Pouco mais de 300 casos foram relatados na literatura mundial. Os autores apresentam um casode Tumor de Frantz, em uma paciente do sexo feminino cuja apresentação clínica foi de massa abdominal palpável.A tomografia computadorizada (TC) associada à ultrassonografia (US) evidenciaram presença de massa sólidocística.Dois terços destes tumores ocorrem no corpo e cauda do pâncreas, e apesar do crescimento excessivopara fora dos limites pancreáticos, raramente invadem estruturas vasculares ou órgãos adjacentes, o que correspondeuaos achados intraoperatórios do caso. A paciente foi submetida a pancreatectomia corpo-caudal com preservaçãodo baço. À microscopia foram evidenciadas cavidades císticas com ocasionais estruturas papilíferas. A pacienteencontra-se sem evidência da doença 24 meses após a operação. Enfatiza-se a necessidade de se considerar otumor de Frantz no diagnóstico diferencial de massa abdominal em pacientes jovens.

Frantz´s tumor is a rare neoplasia of the pancreas that occur mainly in teenage patients with a good prognosis. Alittle over 300 cases have been reported in the literature worldwide. The autors present a case of Frantz's tumor ona female patient, whose presentation was palpable abdominal mass. CT scans and ultrasound imaging showed asolid cystic mass. Two thirds of these tumors occur at the body or tail of the pancreas, and in spite of the excessivegrowth around the pancreas, it rarely invades vascular structures or adjacent organs, what could have been seenintraoperatively in this case. The patient was submitted to a partial pancreatectomy of body and tail with preservation of the spleen. Microscopic examination displayed evident cystic cavities with occasional papillary structures. The patient is disease-free 24 months after the operation. We emphasize the need to consider tumor of Frantz as a differential diagnosis for abdominal masses in youngsters.