Ilídio Moreira

Effect of Obstructive Sleep Apnea in Acute Coronary Syndrome

The effect of obstructive sleep apnea (OSA) on clinical outcomes after acute coronary syndrome (ACS) is incompletely defined. We sought to determine the prevalence of OSA in patients with ACS and evaluate prognostic impact of OSA and continuous positive airway pressure (CPAP) therapy in these patients. This was a prospective longitudinal cohort study of 73 patients admitted on cardiac intensive care unit for ACS. Cardiorespiratory sleep study and/or polysomnography were performed in all patients. CPAP was recommended if Apnea-Hypopnea Index ≥5. The main study outcome was a composite of death for any cause, myocardial infarction, and myocardial revascularization. OSA was diagnosed in 46 patients (63%). Age and cardiovascular risk factors were not significantly different between groups. OSA was classified as mild (m-OSA) in 14 patients (30%) and as moderate-to-severe (s-OSA) in 32 patients (70%). After a median follow-up of 75 months (interquartile range 71 to 79), patients with s-OSA had lower event-free survival rate. After adjustment for gender, patients with s-OSA showed a significantly higher incidence of the composite end point (hazard ratio 3.58, 95% CI 1.09 to 17.73, p = 0.035). Adherence to CPAP occurred in 19 patients (41%), but compliance to CPAP therapy did not reduce the risk of composite end point (hazard ratio 0.87, 95% CI 0.31 to 2.46, p = 0.798). In conclusion, OSA is an underdiagnosed disease with high prevalence in patients with ACS. It is urgent to establish screening protocols because those have high diagnostic yield and allow identifying a group of patients with manifestly unfavorable prognosis.

Screening for Fabry Disease in Left Ventricular Hypertrophy: Documentation of a Novel Mutation

Background Fabry disease is a lysosomal storage disease caused by enzyme α-galactosidase A deficiency as a result of mutations in the GLA gene. Cardiac involvement is characterized by progressive left ventricular hypertrophy. Objective To estimate the prevalence of Fabry disease in a population with left ventricular hypertrophy. Methods The patients were assessed for the presence of left ventricular hypertrophy defined as a left ventricular mass index ≥ 96 g/m2 for women or ≥ 116 g/m2 for men. Severe aortic stenosis and arterial hypertension with mild left ventricular hypertrophy were exclusion criteria. All patients included were assessed for enzyme α-galactosidase A activity using dry spot testing. Genetic study was performed whenever the enzyme activity was decreased. Results A total of 47 patients with a mean left ventricular mass index of 141.1 g/m2 (± 28.5; 99.2 to 228.5 g/m2] were included. Most of the patients were females (51.1%). Nine (19.1%) showed decreased α-galactosidase A activity, but only one positive genetic test − [GLA] c.785G>T; p.W262L (exon 5), a mutation not previously described in the literature. This clinical investigation was able to establish the association between the mutation and the clinical presentation. Conclusion In a population of patients with left ventricular hypertrophy, we documented a Fabry disease prevalence of 2.1%. This novel case was defined in the sequence of a mutation of unknown meaning in the GLA gene with further pathogenicity study. Thus, this study permitted the definition of a novel causal mutation for Fabry disease - [GLA] c.785G>T; p.W262L (exon 5).

Enfarte agudo do miocárdio após a toma de zolmitriptano

Zolmitriptan is a drug used in the acute treatment of migraine, which should not be used in high cardiovascular risk individuals because of its potential to induce vasospasm. We report a rare case of myocardial infarction precipitated by taking zolmitriptan.

Severe aneurysmal coronary artery disease in a patient with ulcerative colitis

Formation of coronary artery aneurysms in the setting of systemic inflammatory conditions is rare but has been described. We report a case of severe aneurysmal coronary artery disease leading to an acute coronary event in a patient with ulcerative colitis. A 67-year-old Caucasian man with a medical history of ulcerative colitis and hypertension presented with chest pain and diaphoresis ongoing for 12 hours. His physical examination was unremarkable; electrocardiography revealed inverted T waves in leads I, aVL and V4--V6, and troponin I

Transpulmonary resection of an interventricular septal fibroma in an adult patient

A 39-year-old woman was referred to our institution for a cardiac mass discovered incidentally during transthoracic echocardiography performed for unrelated reasons. She was asymptomatic and the physical examination was normal. To further characterize the mass, she underwent magnetic resonance imaging (MRI). The study revealed a solid nodular intramyocardial tumor (3.2 cm×2.2 cm×1.6 cm) located within the basal interventricular septum (Figure 1A). The lesion was isointense in T1-weighted sequences and hypointense in T2-weighted sequences (Figure 1C and D). There was no perfusion within the tumor on firstpass gadolinium perfusion images and heterogeneous late gadolinium enhancement was evident (Figure 1B). Computed tomography excluded calcifications within the tumor. A diagnosis of fibroma was considered. Taking into account the uncertainty concerning the nature of the tumor, right ventricular outflow tract (RVOT) obstruction and high risk of malignant arrhythmias, the patient underwent surgical resection. The tumor was resected through a pulmonary artery approach (Figure 1E and F), avoiding right ventricular (RV) myectomy. The intraoperative and postoperative course was uneventful. Pathologic examination confirmed the diagnosis of fibroma.

Fibroelastoma condicionando obstrução coronária intermitente e enfarte do miocárdio. Quando o ecocardiograma poderia ter feito a diferença

Este caso remete-se a uma doente de 65 anos trazida ao hospital pela emergência médica por quadro de dor torácica constritiva com uma hora de evolução. O eletrocardiograma pré-hospitalar mostrava elevação do segmento ST nas derivações inferiores (Figura 1A). Na admissão a doente já se encontrava assintomática e o eletrocardiograma mostrava resolução das alterações descritas. Foram observados na telemetria períodos intermitentes de elevação de ST assintomáticos, enquanto a doente era preparada para cateterismo urgente. Neste visualizou-se um stop de contraste no ostium da artéria coronária direita que condicionava elevados gradientes intracoronários durante a angiografia seletiva da mesma (Figura 1B). Inicialmente colocou-se a hipótese de se tratar de uma possível disseção,

Trombo mural e aorta torácica aneurismática – uma associação rara

A 72-year-old woman was referred for surgery following fracture of the humerus in a fall, without syncope. She had a history of dementia, hypertension and aortic aneurysm with mural thrombus diagnosed eight years previously, not referred for surgical treatment due to the patient’s refusal. On preoperative cardiovascular assessment, she was asymptomatic, with controlled blood pressure and no pressure difference between limbs. The transthoracic echocardiogram showed mild dilatation of the ascending aorta (36 mm) and aneurysm of the aortic arch (69 mm) and the descending aorta (43 mm), with what appeared to be a large (35

Lesão tumoral gigante na aurícula esquerda: um caso incomum de sarcoma cardíaco indiferenciado

Primary cardiac tumors are rare, with an incidence ranging from 0.0001% to 0.030%; 80% are benign, while sarcomas account for 95% of malignant tumors. The authors report the case of a 75-year-old patient with a giant mass in the left atrium. The final diagnosis was of an undifferentiated cardiac sarcoma. This tumor represents a real challenge not only for timely diagnosis, but especially the therapeutic approach to adopt.

Stent Thrombosis eight years past drug-eluting stent placement: a case report

Stent thrombosis (ST) is a major concern in the drug-eluting stents (DES) era. There are several reports of stent thrombosis occurring up to five years after stent implantation. We report the case of a sixty eight-year-old woman with very late ST presenting as acute ST-elevation myocardial infarction (STEMI) after stopping antiplatelet therapy eight years past DES placement. The long time-to-event of this case brings new questions to the controversial aspects of optimal followup time and the safety of antiplatelet therapy cessation after recommended times are met in patients with DES.

Lesão cardíaca no contexto de carcinoma hepatocelular metastático

Hepatocellular carcinoma with extension or metastasis to the right atrium is an uncommon form of cardiac malignancy. The authors report the case of a 51-year-old patient with hepatocellular carcinoma and thrombi in the portal and mesenteric veins, which histopathology revealed to be metastatic. Echocardiography showed a right atrial mass which in this context has to be considered as a possible cardiac metastasis.Resumo O carcinoma hepatocelular com extensao ou mestastases para a auricula direita e uma forma de doenca cardiaca maligna pouco frequente. Os autores relatam o caso de um doente de 51 anos de idade a quem foi diagnosticado carcinoma hepatocelular com trombos a nivel das veias porta e mesenterica, cuja analise histopatologica revelou ser compativel com metastases. O ecocardiograma revelou uma massa na auricula direita que, neste contexto, deve ser considerada como possivel metastase cardiaca.