Regina Ribeiras

Uncommon acquired Gerbode defect following extensive bicuspid aortic valve endocarditis

Gerbode defect is a rare type of left ventricle to right atrium shunt. It is usually congenital in origin, but acquired cases are also described, mainly following infective endocarditis, valve replacement, trauma or acute myocardial infarction. We report a case of a 50-year-old man who suffered an extensive and complex infective endocarditis involving a bicuspid aortic valve, the mitral-aortic intervalvular fibrosa and the anterior leaflet of the mitral valve. After dual valve replacement and annular reconstruction, a shunt between the left ventricle and the right atrium - Gerbode defect, and a severe leak of the mitral prosthesis were detected. Reintervention was performed with successful shunt closure with an autologous pericardial patch and paravalvular leak correction. No major complications occurred denying the immediate post-surgery period and the follow-up at the first year was uneventful.

Thrombus entrapment: the clue for coronary embolism

A 78-year-old obese and hypertensive female presented to the emergency department with acute chest pain. On physical examination blood pressure was 90/50 mmHg and her heart rate was 110 b.p.s. The electrocardiogram ( Panel A ) was notable for right-axis deviation and ST-segment elevation in DI, aVL and …

Recurrent myocardial infarction in a patient with papillary fibroelastoma.

This report describes a case of a 62 year-old man admitted for recurrent myocardial infarction and normal coronary arteries, caused by coronary embolism from aortic papillary fibroelastoma. Other conditions causing acute coronary syndrome and normal coronary arteries are discussed. A careful evaluation by transthoracic and transesophageal echocardiography is required in this clinical setting. Surgical excision of the tumor is safe and curative.This report describes a case of a 62 year-old man admitted for recurrent myocardial infarction and normal coronary arteries, caused by coronary embolism from aortic papillary fibroelastoma. Other conditions causing acute coronary syndrome and normal coronary arteries are discussed. A careful evaluation by transthoracic and transesophageal echocardiography is required in this clinical setting. Surgical excision of the tumor is safe and curative.

Rare presentation of sarcoidosis: Multimodal imaging diagnosis of cardiac involvement

We report a case of sarcoidosis with an unusual presentation, initially manifesting as bilateral pulmonary embolism and then as a cardiac form of the disease with an ominous clinical event consisting of sustained ventricular tachycardia. The diagnosis was established by clinical and magnetic resonance criteria despite normal conventional echocardiographic study. Detailed functional assessment provided by tracking techniques (speckle tracking echocardiography and cardiac magnetic resonance tissue tracking) enabled the detection of regional deformation abnormalities, indicating prominent circumferential strain and epicardial layer alterations, partly matching the structural changes depicted by distribution of delayed enhancement. We find this case notable for various issues it raises concerning diagnosis and management of cardiac sarcoidosis. These are mainly related to recent developments in imaging modalities that enable non-invasive identification of structural and functional abnormalities in this condition early, before overt deterioration in left ventricular ejection fraction. Information from different imaging modalities and tools provide information that could potentially assist preclinical diagnosis, with possible prognostic implications.

Um caso de endocardite infecciosa de válvula nativa em doente imunocomprometido

Infective endocarditis continues to be associated with high mortality, despite the medical and surgical therapeutic options available. Surgical intervention is indicated in cases of heart failure or uncontrolled infection and sometimes for the prevention of embolic phenomena. The authors present the case of a 56-year-old male patient, with fibro-calcific mitral-aortic valve disease, splenectomized and with recently relapsed Hodgkins lymphoma, who was admitted with infective endocarditis due to Streptococcus dysgalactiae. On the thirtieth day of directed antibiotic therapy, the mitral vegetation showed a significant increase in size and mobility. Surgery was considered at this point. However, given the patients clinical stability and laboratory results, it was decided to adopt a conservative approach and to extend antibiotic therapy. The vegetation had regressed considerably seven days later. Given this atypical vegetation behavior, with slower than usual regression for the causative agent, the authors suggest that antibiotic therapy should be extended in patients with some degree of immunosuppression.

Left atrial function and left ventricular diastolic dysfunction – Just the marionette and its master?

Left ventricular diastolic dysfunction (LVDD) usually precedes systolic dysfunction in the early phase after acute myocardial infarction (MI). Previous studies have shown that in MI patients, LVDD may promote progression of heart failure (HF) from stage B to stage C, even in HF with preserved left ventricular (LV) ejection fraction (HFpEF). In about one fifth of patients after MI, the left atrium undergoes significant remodeling, defined as increase in left atrial (LA) volume of ≥8 ml/m over a 12-month period (although it may be evident after one month). It has been reported that after MI, increased LV enddiastolic pressure (LVEDP) induces higher LA afterload and hence mechanical stress on the atrium that leads to higher LA volumes, mechanical dysfunction and increased stiffness, which in turn, by a tandem effect, reduce LA emptying, LV filling and cardiac output during exertion. The backward effects of LA enlargement and dysfunction are pulmonary venous congestion and vascular pulmonary vasoconstriction that may eventually trigger right ventricular overload and dysfunction. Accordingly, the evolving stages of HFpEF are associated with right ventricle-to-pulmonary circulation

Documento de Consenso e Recomendações para a realização de Ecocardiografia Transtorácica em Portugal

Echocardiography is the most widely used imaging technique in modern cardiological clinical practice, since it is readily available, portable and safe, and provides a comprehensive morphological and functional assessment at low cost compared to other imaging modalities. Recent technological advances have introduced new echocardiographic techniques and widened the clinical applications of echocardiography. However, these developments have also led to an increase in information, rendering interpretation of the data provided by the exam more complex; this may result in assessment errors by less experienced operators. Standardization of procedures and training in echocardiography is therefore essential to ensure quality and safety for patients. The present document aims to contribute to this end, recommending quality requirements for operators and echocardiography laboratories in Portugal.

Surgical pathology of subaortic septal myectomy: histology skips over clinical diagnosis

BACKGROUND Subaortic septal myectomy is usually performed to mitigate obstruction in patients with the obstructive form of hypertrophic cardiomyopathy (HCM) or in those with congenital subaortic stenosis. Moreover, it is combined with aortic valve replacement in patients with severe aortic valve stenosis (SAS) and asymmetrical septal hypertrophy causing concomitant left ventricular outflow tract obstruction. When both conditions coexist, it is conceptually difficult to identify a cardiomyopathy beyond an adaptive myocardial hypertrophy, strictly related to pressure overload. Myectomy histopathology might be useful to enlighten the cause of the obstruction and establish the diagnosis. AIM The aim was to describe the pathological findings of surgical septal myectomy specimens obtained from a group of patients with diverse clinical diagnosis, including HCM, severe aortic stenosis, and asymmetrical septal hypertrophy. METHODS This was a retrospective study of 56 patients undergoing septal myectomy along a 10-year period at a tertiary cardiac surgical center. Clinical, interventional, and anatomopathological findings between patients with and without a preoperative diagnosis of HCM were analyzed and compared. RESULTS Mean age at intervention was 67.5±20.5 years; 37 (66.1%) were female Preoperative diagnosis of sarcomeric obstructive HCM was assumed in 23 (41.1%) patients. All the other patients (58.9%) were referred for surgery with preoperative diagnosis of asymmetric septal hypertrophy, mainly in the context of severe aortic stenosis (24 patients). Twenty-seven (48.2%) patients had a greater than 30 mmHg intraventricular gradient at rest. Patients with presumed HCM were significantly younger (56.5±15.8 vs. 70.2±13.3 years, P<.001), had higher prevalence of significant intraventricular obstruction at rest [20 (87.0%) vs. 8 (34.8%), P<.001], and more frequently had moderate or severe mitral regurgitation [9 (39.1%) vs. 5(15.1%), P=.043]. All patients with aortic valve stenosis underwent both aortic valve replacement and septal myectomy. Twelve (52.1%) of the patients with obstructive HCM had isolated septal myectomy, while in the remaining 11, the procedure was combined with intervention on the mitral valve. Histopathological final diagnosis was of nonspecific reactive myocardial hypertrophy in all but 4 (92.2%) patients. In those, 2 (3.6%) had the final diagnosis of HCM and 2 (3.6%) the diagnosis of congenital subaortic membranous stenosis with reactive myocardial hypertrophy. Different grades of subendocardial fibroelastosis and myocardial fibrosis, mainly interstitial, were present [27 (48.2%) and 18 (32%) patients, respectively]. When microscopic data were compared between patients with or without a preoperative clinical diagnosis of HCM, no significant differences were found. CONCLUSION In patients submitted to surgical septal myectomy, histology was mostly indistinctive among different clinical entities. Since different myocardial hypertrophy etiologies may share similar pathological expression, there is a need for detailed clinical assessment when trying to define the best strategy for clinical management.

Transcatheter tricuspid valve-in-valve replacement: one-year results Alternative to surgery in high-risk patients

Although rheumatic heart disease is becoming uncommon in industrialized countries, its global burden is still significant. We report the case of a 70-year-old male with rheumatic heart disease, who underwent 4 previous heart valve replacement surgeries, and presented to our hospital with refractory heart failure (NYHA functional class IV) due to severe stenosis of a previously implanted tricuspid bioprosthesis. The Heart Team deemed the patient as inoperable/high-risk for surgery. As an alternative, a transcatheter tricuspid valve-in-valve replacement was decided upon and later executed through the right femoral vein, with the insertion of an Edwards SAPIEN XT 29 no. (Edwards Lifesciences, Irvine, CA, USA) through the inferior vena cava, towards the RV, followed by direct implantation in the tricuspid bioprosthesis (valve-in-valve), under rapid pacing, without complications. A substantial clinical and echocardiographic improvement was noted after the procedure and the patient was subsequently discharged in NYHA functional class II. These favourable outcomes persisted through the 1-year follow-up period. This case report adds to the current body of evidence that tricuspid valve implantation stands as a viable and reliable alternative in the treatment of degenerated bioprosthesis in high-surgical-risk patients.

Transposição de grandes vasos congenitamente corrigida e endocardite de eletrocateter

Homem 56 anos, referenciado ao nosso serviço em 1978, com o diagnóstico de cardiopatia congénita não cianótica operada: encerramento de comunicações interventricular (CIV) e interauricular (CIA) associado a correção de estenose valvular pulmonar. A radiografia do tórax mostrava mesocárdia. A partir de 1992 verificaram-se sintomas/sinais de insuficiência cardíaca, com sopro sistólico no bordo esternal esquerdo. Nos ecocardiogramas transtorácicos (ETT) seriados detetava-se CIV residual alta, tendo uma ressonância magnética cardíaca (RM), em 1999, colocado o diagnóstico de transposição das grandes artérias sem presença de CIV/CIA. Em 2005, foi-lhe implantado um cardioversor desfibrilhador (CDI) por taquicardia ventricular sincopal (Figura 1). Em 2011, foi internado no Luxemburgo por quadro febril, cefaleias, náuseas e alterações neurológicas não focais (estado estuporoso). Uma TAC-CE mostrou um abcesso cerebral na região frontal mediana esquerda e o ecocardiograma transesofágico (ETE) identificou vegetações no eletrocateter. Apresentava hemoculturas positivas para Streptococcus constellatus e Micromonas micros.