EMBOLIZATION OF PERCUTANEOUS LEFT ATRIAL APPENDAGE OCCLUSION DEVICE: RESCUE SURGERY.

59-year-old women with permanent atrial fibrillation and previous haemorrhagic stroke was admitted for percutaneous left atrial appendage occlusion. Shortly after, AmplatzerTM device migration into the left ventricular outflow tract was confirmed. Unsuccessful transcatheter mobilization led to an emergent surgery for device retrieval and LAA closure. No events at 6-month follow-up.

EXTRACORPOREAL MEMBRANE OXYGENATION SUPPORT FOR POSTCARDIOTOMY SHOCK: SINGLE CENTER EXPERIENCE.

OBJECTIVES: We aim to report our experience on venoarterial extracorporeal membrane oxygenation (VA-ECMO) for postcardiotomy shock (PCS). METHODS: Single center, retrospective study of all patients on VA-ECMO for PCS, from November 2006 to July 2019. Pediatric and adult patients were analysed separately. Primary outcomes were survival to discharge and one-year survival. RESULTS: Twenty-nine patients were included. Pediatric group (group PED) (62%, n=18): mean age 1,3±2,1 years and 39% male. Adults (group AD) (38%, n=11): mean age 55,6±15,9 years and 64% male. Indications in group PED were complex congenital heart surgery (94%) and heart transplant (6%), with 27% being reoperations; in group AD valvular surgery (45%), aortic surgery (21%), coronary artery bypass grafting (18%) and pulmonary endarterectomy (9%); 45% were reoperations. ECMO support was initiated intraoperatively due to failure to wean from cardiopulmonary bypass in 28% of group PED and 73% of group AD. Central cannulation was performed in all pediatric patients and 82% adults. Bleeding was the most common complication in both groups (group PED 39%, group AD 45%). Mean ECMO support time was respectively 6,2±4,9 and 6,2± 3,6 days for group PED and group AD. Weaning rate was 44% in group PED (with 2 patients bridged to LVAD) and 45% in group AD. Survival to discharge as well as one-year survival were both 28% in group PED and 18% in group AD. CONCLUSION: Despite low survival and high complication rates, VA ECMO support provides a survival benefit in refractory cases, with a dismal prognosis, that would otherwise die.

Metastatic endometrial stromal sarcoma: A rare cause of right-sided intracardiac mass.

The endometrial stromal sarcoma (EES) is a rare uterine malignancy and its intracardiac metastasis are exceedingly rare. We report a case of a 53-year-old female patient diagnosed with a metastatic tumor of a ESS in the right side of the heart, who underwent successful surgical resection and initiated chemotherapy with docetaxel and gemcitabine. At a 9-month follow-up, the patient was in New York Heart Association-Class I, without any further complications.

Quadricuspid Aortic Valve with Mild Stenosis and Servere Insufficiency.

The quadricuspid aortic valve is a very rare congenital malformation and usually presents with aortic insufficiency. Other associated congenital malformations have been described and particularly coronary anomalies have to be considered and investigated. We report the case of an intraoperatively diagnosed quadricuspid aortic valve in a 70 years old patient and discuss the clinical relevance of this rare congenital malformation.

A válvula aórtica quadricúspide é uma malformação congénita muito rara e, geralmente, apresenta-se como uma insuficiência aórtica. Outras malformações congénitas podem estar associadas e devem ser excluídas, em particular anomalias das artérias coronárias. Apresentamos o caso clínico de um homem de 70 anos de idade submetido a cirurgia da válvula aórtica quadricúspide diagnosticada intra-operatoriamente e discutimos a sua relevância clínica.

Cardiac Fibroelastoma: 10-Year Experience.

INTRODUCTION: Papillary fibroelastoma is one of the most common types of primary cardiac tumour. Though a rare pathology, its importance relates to its form of presentation, with stroke and sudden death, and the questions regarding its management. OBJECTIVES: To review the casuistic of cardiac tumours in our service, in special the papillary fibroelastomas. METHODS: clinical case series overviewing the period from 1st January 2008 to 30th September 2017. We analysed all patients submitted to cardiac surgery due to cardiac masses or tumours and verified all the pathology results to confirm the diagnosis. RESULTS: In the period selected, around 6500 surgeries were performed. Of those, 59 patients were operated on due to cardiac tumours. 81.4% were myxomas, 13.6% were papillary fibroelastomas, 3.4% were cardiac sarcomas and 1.7% were metastasis. Of the patients that had papillary fibroelastomas (n=8), 5 were male, and the mean age was 51.5 ± 16.2 years. 62.5% (n=5) had origin in the aortic valve and 37.5% (n=3) in the mitral valve. Regarding presentation, 3 patients presented with stroke, 2 patients had episodes of syncope and 3 patients were asymptomatic. After surgery, all patients remained asymptomatic and there was no evidence of recurrence. At the end of follow-up all patients were alive. CONCLUSION: Although a rare pathology, papillary fibroelastomas remain important due to their potential for embolization and cardiac chamber obstruction, therefore should be primarily treated with surgery.

Fulminant myocarditis caused by Legionella pneumophila: case report.

Myocarditis is defined as inflammation of the myocardium. The clinical manifestations of myocarditis vary from flu-like symptoms to fatal fulminant forms. We report the case of a 39-year-old woman with a diagnosis of cardiogenic shock caused by fulminant myocarditis. Extracorporeal membrane oxygenation was used as a bridge to recovery. Etiological study revealed Legionella pneumophila serogroup 1 infection. Recovery of biventricular function was seen after treatment with azithromycin.

Iatrogenic aortic pseudoaneurysm: a forgotten complication.

Pseudoaneurysms of the ascending aorta are a rare complication of cardiac surgery. However, the poor prognosis associated with this condition if untreated makes early diagnosis and treatment important. We present the case of a 66-year-old woman who had undergone mitral valvuloplasty 12 days previously, who was admitted with a diagnosis of new-onset atrial fibrillation. The transthoracic echocardiogram showed a clot in the right atrium and anticoagulation was initiated, followed by antibiotic therapy. After further investigation, the patient was diagnosed with a pseudoaneurysm of the ascending aorta and underwent surgical repair, followed by six weeks of antibiotic therapy. She was readmitted six months later for an abscess of the lower sternum and mediastinum. After a conservative approach with antibiotics and local drainage failed, recurrence of a large pseudoaneurysm compressing the superior vena cava was documented. A third operation was performed to debride the infected tissue and to place an aortic allograft. There were no postoperative complications.

[Right atrial thrombus: a rare presentation of plasminogen activator inhibitor deficiency]. / Trombo na aurícula direita: apresentação rara da deficiência do inibidor do ativador do plaminogénio.

Free-floating right atrial thrombi are rare but associated with high mortality. Although advances in echocardiography have improved diagnosis, their management is still the subject of debate. A 24-year-old woman with a history of smoking, obesity and oral contraceptive use presented to the emergency department with dyspnea, cough and hemoptysis. Transthoracic echocardiography revealed a large free-floating cardiac mass occupying the right atrial chamber and restricting tricuspid valve opening. In view of recurrent pulmonary embolism, she was referred for cardiac surgery and the cardiac mass was excised. Anatomopathological analysis revealed an organized and calcified thrombus. Genetic study showed her to be homozygous for the 4G/4G allelic variant of plasminogen activator inhibitor-1 and heterozygous for the allelic variant A1298C of 5,10-methylenetetrahydrofolate reductase.

Long-term survival with heart transplantation for fibrosarcoma of the heart.

Primary sarcoma of the heart is a rare disease that has an ominous prognosis with either medical or surgical therapy. We report a case of a 25-year-old woman with sarcoma of the heart who received a transplant and is clinically well after 7 years. We believe that transplantation must be considered in this kind of pathology for selected cases.

Primary idiopathic chylopericardium.

Chylopericardium is a rare entity which is usually associated with trauma (thoracic duct lesions), neoplasia or filaria infection. Primary forms are even rarer and are caused by lymphatic circulation malformations. We present the case of a 52-year-old male patient with primary chylopericardium. We discuss the diagnostic approach, as well as the favorable clinical evolution with conservative management.

Caseous calcification of the mitral annulus. A review of six cases.

Caseous calcification of the mitral annulus is a rare form of mitral annular calcification, whose etiology is not completely understood and which can lead to an erroneous diagnosis of intracardiac tumor. The authors describe the cases of six patients, five of them female, mean age 74.8 +/- 6.4 years (65-81). Four patients presented with heart failure, two with atrial fibrillation and five with hypertension. Round, echogenic images, 18-26 mm in their largest diameter with a central echolucent area, were identified by transthoracic echocardiography on the lateral and posterior segments of the mitral annulus. Severe mitral regurgitation was also found in four patients. Only three patients with severe mitral regurgitation and heart failure were operated on, and one patient refused surgical treatment. A caseous mass, similar to toothpaste, was obtained from the mitral annulus zone during surgery.

Isolated cleft of the anterior mitral valve leaflet.

Isolated anterior mitral leaflet cleft (not associated with atrio-ventricular septal defect) is a rare cause of congenital mitral regurgitation, and the treatment consists of direct suturing of the cleft. We present a clinical case with this entity.

[Anomalous origin of anterior descending coronary artery, originated in the right coronary]. / Anomalias coronárias, a propósito de um caso clínico com origem da artéria descendente anterior a partir da coronária direita.

We describe a very rare case of anomalous origin of the LAD from the RCA and inter arterial course. Diagnosis was made by CAT scan following angiography for pre operative evaluation for aortic valve replacement (bicuspid aortic valve). This patient received a LIMA graft to the LAD. An extensive literature review is provided with special emphasis on risk stratification and therapeutical indication.

[New prosthesis for cardiac valve replacement]. / Novas próteses para substituição valvular cardíaca.

This paper focus on the most common used prosthesis for replacement of diseased heart valves, when repair is not feasible. A brief historical review is made. New prosthesis and the trends for the future are also addressed.

[Mitral valve repair in children for acquired valvular disease]. / Cirurgia de reparação mitral em crianças com valvulopatia adquirida.

BACKGROUND: Valve surgery in children is aimed at restoring correct hemodynamics with few reoperations and limited resort to prostheses, which would imply early deterioration or definitive hypocoagulation. OBJECTIVES: Report a series of paediatric pts with acquired mitral valve disease, mostly due to rheumatic disease, in whom it was possible, for the great majority, to repair the damaged valve. DEMOGRAPHICS: Fifty children with predominant mitral valve disease, 47 rheumatic (94%) and 3 after endocarditis were consequently operated by the same surgical team over the last five years. Ages were 12.5+/-3.1 yrs and weights 33.2+/-8.4 Kg, 30 pts presented with predominant mitral regurgitation and 20 pts had significant stenosis. In 8 pts there also moderate to severe aortic regurgitation and in 2 pts severe tricuspid regurgitation was present. Patients were not operated during the acute phase of the disease. Five pts were reoperations and from those, all but one received mechanical prosthesis. RESULTS: In all operations the intention was to repair the mitral valve. In 46 pts complex mitral valvuloplasties were performed extended comissurotomies, shortening of chordae, chordal replacement with PTFE, and reconstruction of valve leaflefts by direct patching or pericardial extension of the retracted posterior leaflet (78.2% cases), plus reshaping of the annulus by using a fixed prosthetic CE ring (sizes 26 to 32) in every case. Ring sizes correlated poorly with body weights, but correlation was close and positive for the use of pericardial advancement of the posterior leaflet (p<0.01). There was no operative mortality, but one pt died early from sepsis and there was no late mortality. Maximum follow up extends now to 50 months (median 28 months) and functional evaluation, at latest follow up, as assessed by Doppler Echocardiography, showed residual mitral regurgitation, mild-moderate in 4 pts and LA-LV gradients mild in 5 and moderate in 2 pts. NYHA functional class, at present follow-up is class I for 43 pts (88%) and class II in the remaining 6 pts. Along the follow-up period 2 pts had to be reoperated for early repair failures and other three for late failures, presently freedom for reoperation is 91.8% at 5 years. CONCLUSIONS: Mitral valve repair in children with rheumatic lesions can be achieved for the great majority of cases by using different techniques. Pericardial extension of the retracted posterior leaflet allowed the use of a bigger size prosthetic ring. Intermediate functional results are good with fair functional classes and few reoperations but follow-up is short and does not allow us to draw conclusions about the long-term results of the repair in these rheumatic patients.