An Electrifying Case of a Broken Heart Syndrome.

Takotsubo syndrome (TTS) describes an acute and transient left ventricular (LV) dysfunction that, although not obligatory, is many times associated with an underlying emotional, physical, or combined trigger. We describe a rare case of an 80-year-old female who developed TTS after pacemaker implantation in the context of a complete atrioventricular block (CAVB). During the patient's workup, right ventricular (RV) lead dislodgment was found. She developed acute heart failure symptoms 12 hours after device implantation with transthoracic echocardiogram showing de novo severe systolic biventricular dysfunction with dyskinesia of the apical segments and hyperdynamic contractility of the basal segments of both ventricles. Coronarography was normal, and left ventriculography demonstrated apical ballooning. TTS was then considered the most probable diagnosis. The patient received supportive care with diuretics, beta-blocker, and angiotensin-converting enzyme inhibitor (ACEI); an RV lead repositioning was also done. After four weeks, left ventricular function had fully recovered, confirming the diagnosis. This is a rare case of a post-pacemaker implantation TTS with concomitant lead dislodgment that can be assumed as a likely contributing factor. This report emphasizes that, although rare, TTS should be considered in the differential diagnosis of patients with acute heart failure development after pacemaker implantation.

Recurrent myocarditis in the context of Behçet's disease: a case report.

BACKGROUND: Behçet's syndrome is a multisystemic vasculitis of unknown aetiology. Cardiac involvement is rare, with described prevalence between 1% and 46%, with pericarditis, valvular insufficiency, intracardiac thrombosis, and eventually sinus of Valsalva aneurysms being the most common findings. Although previously reported, myocarditis is a very rare complication of Behçet's syndrome. CASE SUMMARY: A 26-year-old man, smoker but otherwise healthy, was admitted to the emergency department with atypical chest pain, with no radiation, relation to efforts, position or deep inspiration, and dyspnoea, since the day before. His physical examination was unremarkable, including no fever, tachycardia, or pericardial friction rub. Electrocardiogram (ECG) revealed an early repolarization pattern, with no changes noted in subsequent exams. He had elevation of inflammatory parameters and an increased high-sensitivity troponin level of 3300 ng/L. Transthoracic echocardiography (TTE) was unremarkable. Coronary angiography showed no coronary stenosis. A presumed diagnosis of non-complicated viral myocarditis was established. The patient's condition improved with acetylsalicylic acid as needed and colchicine and he was discharged after 3 days. Cardiac magnetic resonance was performed, showing late epicardial enhancement in the apical segment of the lateral wall, supporting the diagnosis of myocarditis. Four months later, the patient returned with recurrence of chest pain. Additionally, he also complained of fever, odynophagia, and otalgia since the previous week. Oropharyngeal examination revealed tonsillar pillars aphthosis. The ECG was similar to the previous and TTE was normal. Bloodwork revealed once again elevation of inflammatory parameters and elevation of troponin. Recurrent myocarditis was diagnosed. Treatment with ibuprofen, colchicine, and antibiotic therapy was started with no significant improvement. After a more thorough physical examination, an ulcerated scrotal lesion, a left buttock folliculitis, and an axillary hidradenitis were found, which, according to the patient, were recurrent in the last year. Accordingly, the diagnosis of Behçet's syndrome with mucocutaneous and cardiac involvement was established. The patient was kept on colchicine and was also started on immunosuppressive therapy with corticosteroids and azathioprine, with resolution of the symptoms in the following day. A positron emission tomography (PET) was performed 2 days after discharge and showed a higher myocardial uptake in the left ventricular basal segments and both papillary muscles. Prednisolone tapering was started after 2 months, while maintaining azathioprine. At 1-year follow-up, the patient remained asymptomatic. A re-evaluation PET was performed, showing no images suggestive of metabolically active disease in the myocardium. DISCUSSION: This case highlights the importance of awareness of this rare but potentially serious entity and reinforces the significance of aetiology investigation in cases of recurrent myocarditis. It also shows the success of immunosuppressive therapy in a context where the optimal management is still considerably uncertain.

Short- and medium-term prognosis of Takotsubo syndrome in a Portuguese population.

INTRODUCTION: Takotsubo syndrome (TTS) is characterized by transient left ventricular (LV) systolic dysfunction. AIM: To characterize a Portuguese population with TTS and to determine their short- and medium-term prognosis. METHODS: We conducted a multicenter study in Portuguese hospitals that included all patients diagnosed with TTS, initially retrospectively and subsequently prospectively. Short- and medium-term clinical complications and mortality were assessed. Independent predictors of in-hospital complications and prognostic factors were determined. RESULTS: A total of 234 patients (210 female, age 68±12 years) were included. During hospitalization, 32.9% of patients had complications: acute heart failure (24.4%), atrial fibrillation (9.0%), ventricular arrhythmias (2.6%), complete atrioventricular block (2.1%), stroke/transient ischemic attack (1.7%), and LV thrombus (1.3%). Chronic kidney disease (CKD) (p=0.02), coronary artery disease (CAD) (p=0.027), lower LV ejection fraction (LVEF) on admission (p=0.003), and dyspnea at presentation (p=0.019) were predictors of in-hospital complications. In-hospital mortality was 2.2%. At the mean follow-up of 33±33 months, all-cause mortality was 4.4%, cardiovascular mortality was 0.9% and TTS recurrence was 4.4%. Prolonged QTc interval on admission was associated with complications in follow-up (p=0.001). CONCLUSION: TTS has a good short- and medium-term prognosis. However, the rate of in-hospital complications is high and should not be overlooked. Dyspnea at presentation, CKD, CAD and lower LVEF on admission were independent predictors of in-hospital complications. Prolonged QTc on admission was associated with complications in follow-up.

Light chain cardiac amyloidosis - a rare cause of heart failure in a young adult

SUMMARY Cardiac amyloidosis is an infiltrative cardiomyopathy, resulting from amyloid deposition within the myocardium. In primary systemic (AL-type) amyloidosis, the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia, and cardiac involvement occurs in up to 50% of the patients We present a case of a 43-year-old man, with complaints of periodical swollen tongue and xerostomia, bleeding gums and haematuria for two months. His blood results showed normocytic anaemia, thrombocytopenia and a high spontaneous INR, therefore he was referred to the Internal Medicine clinic. In the first visit, he showed signs and symptoms of overt congestive heart failure and was referred to the emergency department. The electrocardiogram showed sinus tachycardia and low voltage criteria. Echocardiography showed biventricular hypertrophy with preserved ejection fraction, restrictive physiology with elevated filling pressures, thickened interatrial septum and atrioventricular valves, small pericardial effusion and relative "apical sparing" on 2D longitudinal strain. Cardiac MRI showed diffuse subendocardial late enhancement. Serum protein electrophoresis was inconclusive, however urine analysis revealed nephrotic range proteinuria, positive Bence Jones protein and an immunofixation test with a monoclonal lambda protein band. Abdominal fat biopsy was negative for Congo red stain, nevertheless a bone marrow biopsy was performed, revealing lambda protein monoclonal plasmocytosis, confirming the diagnosis of primary systemic amyloidosis. This case represents a rare cause of heart failure in a young adult. Low-voltage QRS complexes and typical echocardiography features should raise the suspicion for cardiac amyloidosis. Prognosis is dictated by the level of cardiac involvement; therefore, early diagnosis and treatment are crucial.

RESUMO A amiloidose cardíaca corresponde a uma miocardiopatia infiltrativa, resultante do depósito da proteína amiloide no miocárdio. Na amiloidose sistêmica primária (tipo AL), a proteína amiloide é composta por cadeias leves que resultam de discrasia dos plasmócitos, havendo envolvimento cardíaco em até 50% dos doentes. Apresentamos o caso de um homem de 43 anos, com queixas de edema periódico da língua e xerostomia, hemorragia gengival e hematúria há dois meses. Analiticamente havia a destacar anemia normocítica, trombocitopenia e um INR alto espontâneo, pelo que foi referenciado à consulta de Medicina Interna. Na primeira consulta, apresentou-se com sinais de insuficiência cardíaca congestiva franca, pelo que foi referenciado ao Serviço de Urgência. O eletrocardiograma demonstrou taquicardia sinusal e critérios de baixa voltagem. O ecocardiograma revelou hipertrofia biventricular com fração de ejeção preservada, fisiologia restritiva com elevação das pressões de enchimento, espessamento do septo interauricular e das válvulas auriculoventriculares, derrame pericárdico ligeiro e padrão de apical sparing no strain longitudinal 2D. Realizou ainda ressonância magnética cardíaca, que mostrou realce tardio subendocárdico difuso. A eletroforese das proteínas foi inconclusiva, contudo a análise da urina revelou proteinúria no espectro nefrótico, presença de proteína de Bence Jones e um teste de imunofixação com uma banda monoclonal de cadeias lambda. A biópsia da gordura abdominal foi negativa. Não obstante, foi realizada uma biópsia da medula óssea, verificando-se plasmocitose monoclonal lambda, o que confirmou o diagnóstico de amiloidose primária sistêmica. Este caso representa uma causa rara de insuficiência cardíaca no jovem adulto. A baixa voltagem no eletrocardiograma e os achados ecocardiográficos típicos devem fazer suspeitar de amiloidose cardíaca. O prognóstico é ditado pelo nível de envolvimento cardíaco, motivo pelo qual o diagnóstico e o tratamento precoces são essenciais.

Light chain cardiac amyloidosis - a rare cause of heart failure in a young adult.

Cardiac amyloidosis is an infiltrative cardiomyopathy, resulting from amyloid deposition within the myocardium. In primary systemic (AL-type) amyloidosis, the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia, and cardiac involvement occurs in up to 50% of the patients We present a case of a 43-year-old man, with complaints of periodical swollen tongue and xerostomia, bleeding gums and haematuria for two months. His blood results showed normocytic anaemia, thrombocytopenia and a high spontaneous INR, therefore he was referred to the Internal Medicine clinic. In the first visit, he showed signs and symptoms of overt congestive heart failure and was referred to the emergency department. The electrocardiogram showed sinus tachycardia and low voltage criteria. Echocardiography showed biventricular hypertrophy with preserved ejection fraction, restrictive physiology with elevated filling pressures, thickened interatrial septum and atrioventricular valves, small pericardial effusion and relative "apical sparing" on 2D longitudinal strain. Cardiac MRI showed diffuse subendocardial late enhancement. Serum protein electrophoresis was inconclusive, however urine analysis revealed nephrotic range proteinuria, positive Bence Jones protein and an immunofixation test with a monoclonal lambda protein band. Abdominal fat biopsy was negative for Congo red stain, nevertheless a bone marrow biopsy was performed, revealing lambda protein monoclonal plasmocytosis, confirming the diagnosis of primary systemic amyloidosis. This case represents a rare cause of heart failure in a young adult. Low-voltage QRS complexes and typical echocardiography features should raise the suspicion for cardiac amyloidosis. Prognosis is dictated by the level of cardiac involvement; therefore, early diagnosis and treatment are crucial.

Diagnostic Challenges in Chronic Constrictive Pericarditis.

INTRODUCTION: Chronic constrictive pericarditis (CCP) is a disease that has multiple possible causes and is associated with variable clinical findings, depending on its severity. It develops insidiously, and in many cases, particularly in developed countries, no antecedent diagnosis can be found. These cases are termed idiopathic. Tuberculosis is the leading cause of constrictive pericarditis in developing nations but represents only a small minority in developed countries. METHODS: Here the authors describe two different case reports where tuberculosis was the probable cause of CCP. RESULTS: A 21-year-old man born in Cape Verde living in Europe for 4 years and a 24-year-old man born in Guiné Bissau were both admitted due to intense precordial pain and syncope after exertion. Interestingly both had fatigability, dyspnea, chest discomfort and palpitations on exertion, as well as progressive involuntary weight loss and decubitus cough. On physical examination they had tachycardia, jaundice, cachexia, elevated jugular venous pressure, hepatomegaly and ascites. Both electrocardiograms showed prominent P waves and chest X-ray showed bilateral pulmonary interstitial infiltrates and enlargement of the right cavities. Analytically, elevated bilirubin, leukopenia and thrombocytopenia was also found in both. Echocardiography revealed findings, in both cases, compatible with CCP including less common signs as annulus reversus and annulus paradoxus. Thoraco-abdomino-pelvic CT from both patients revealed chronic liver disease with congestion, pleural effusion, pericardial calcifications, ascites and massive mediastinal and abdominal adenopathies. Blood cultures and IGRA test were negative. However, given the presumptive diagnosis of tuberculosis (TB), anti-TB therapy was started. Despite the diagnosis of "end-stage" CCP with very high operative risk multidisciplinary team decided after informed consent, to perform total anterior pericardiectomy, that occurred without complications. Pericardial and mediastinal biopsies, pericardial/pleural fluid cultures/ immune-phenotyping were inconclusive. Anti- tuberculosis therapy was maintained. After surgery, the patients had a remarkable clinical improvement (NYHA I) that persisted in 6- month follow-up. CONCLUSION: These two case reports illustrate that despite the markedly elevated operative risk of pericardiectomy in "end-stage" forms of disease after patients informed consent must be a considered option. The other point to consider is that, despite rare, tuberculosis still is a possible diagnosis to consider in CCP in Portugal.

Coronary angiography after successful thrombolysis - Is the recommended time interval of 24h an important issue?

INTRODUCTION: Percutaneous coronary intervention (PCI) is currently considered the gold-standard treatment of acute coronary syndromes with ST-segment elevation (STEMI). However, this is not the reality of many European centers, where thrombolysis is performed as primary therapy. AIMS: To determine, in a STEMI population that performed successful fibrinolytic treatment, if the performance of coronary angiography after the first 24h was associated with more hospital complications, including higher mortality, compared with its performance in the recommended time. METHODS: Retrospective study, including 1065 patients with STEMI, who performed successful thrombolysis. The population was divided in three groups: A, patients who didn't undergo coronary angiography after successful thrombolysis (n=278; 26.1%); B, patients who underwent coronary angiography in the first 24h after successful thrombolysis (n=127; 11.9%); and C, patients who underwent angiography after the first 24h (n=660; 62.0%). Groups were compared regarding their characteristics and in-hospital complications. RESULTS: Groups B and C had more male patients and had younger patients than group A. Group A presented higher Killip classes at admission, more severe left ventricle dysfunction and a higher number of complications during hospitalization. Logistic regression revealed that: 1) the non-performance of coronary angiography after thrombolysis was an independent predictor of in-hospital mortality; and 2) the performance of angiography after the recommended time wasn't associated with higher mortality. CONCLUSIONS: Coronary angiography after thrombolysis constitutes an important strategy, whose non-performance carries worse prognosis. The time interval currently recommended of 24h seems clinically acceptable; however, its realization outside the recommended time doesn't seem to lead to higher mortality.

Native aortic valve pneumococcal endocarditis--fulminant presentation.

Pneumococcal endocarditis is a rare entity, corresponding to 1 to 3% of native valve endocarditis cases. It has a typically adverse prognosis, with high mortality. There is a reported predilection for the aortic valve; thus, a common presentation is acute left heart failure. We present a case of a 60-year-old woman with a history of sinusitis, who was admitted with the diagnosis of pneumonia. She rapidly deteriorated with signs of septic shock and was transferred to the critical care unit. The transesophageal echocardiogram revealed severe aortic regurgitation due to valve vegetations. Blood cultures were positive for Streptococcus pneumoniae. She underwent cardiac surgery and had multiple postoperative complications. Nonetheless, the patient made a slow and complete recovery. Infectious endocarditis should be ruled out if any suspicion arises, and echocardiography should be performed in an early stage in patients with poor response to vasopressors and inotropes. Patients with pneumococcal endocarditis benefit from an aggressive approach, with performance of early surgery.

Endocardite pneumocócica em válvula aórtica nativa - apresentação fulminante / Native aortic valve pneumococcal endocarditis - fulminant presentation

RESUMO A endocardite pneumocócica é uma entidade rara, cuja incidência se situa entre 1% e 3% dos casos de endocardite de válvula nativa. Esta patologia tem um prognóstico naturalmente adverso, com elevada mortalidade. Relata-se predileção pela válvula aórtica, de forma que é frequente que se apresente com insuficiência cardíaca. Apresentamos o caso de uma paciente do sexo feminino com 60 anos de idade e história pregressa de sinusite, admitida com diagnóstico de pneumonia. Após rápida deterioração, com sinais de choque séptico, ela foi transferida para a unidade de terapia intensiva. O ecocardiograma transesofágico revelou grave refluxo aórtico, devido à presença de vegetações valvares. As hemoculturas foram positivas para Streptococcus pneumoniae. A paciente foi submetida à cirurgia cardíaca e apresentou múltiplas complicações pós-operatórias. Apesar disso, apresentou lenta, porém completa recuperação. A endocardite infecciosa deve ser afastada em caso do surgimento de qualquer suspeita, e a ecocardiografia deve ser realizada precocemente nos pacientes com resposta insuficiente aos vasopressores e inotrópicos. Pacientes com endocardite pneumocócica se beneficiam de uma abordagem agressiva, com realização precoce da intervenção cirúrgica.

ABSTRACT Pneumococcal endocarditis is a rare entity, corresponding to 1 to 3% of native valve endocarditis cases. It has a typically adverse prognosis, with high mortality. There is a reported predilection for the aortic valve; thus, a common presentation is acute left heart failure. We present a case of a 60-year-old woman with a history of sinusitis, who was admitted with the diagnosis of pneumonia. She rapidly deteriorated with signs of septic shock and was transferred to the critical care unit. The transesophageal echocardiogram revealed severe aortic regurgitation due to valve vegetations. Blood cultures were positive for Streptococcus pneumoniae. She underwent cardiac surgery and had multiple postoperative complications. Nonetheless, the patient made a slow and complete recovery. Infectious endocarditis should be ruled out if any suspicion arises, and echocardiography should be performed in an early stage in patients with poor response to vasopressors and inotropes. Patients with pneumococcal endocarditis benefit from an aggressive approach, with performance of early surgery.