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.

Carcinoma Hepatocelular Metastático: Massa Auricular Direita como Manifestação Clínica Primária. Caso Clínico

Nos doentes com carcinoma hepatocelular a incidência de metástases cardíacas é de 0.67-3%. Mesmo na ressecção com intuito curativo, o prognóstico é reservado, sendo a sobrevida aos 5 anos de 12-39%. Descrevemos um caso clínico, pouco habitual, de um indivíduo do sexo masculino, de 51 anos de idade, que apresentava uma massa localizada à aurícula direita, diagnosticada por exame histopatológico, como sendo metástase de um carcinoma hepatocelular, tendo sido necessária a ressecção cirúrgica urgente, devido a instabilidade cardiovascular.

Operação de Ross: Resultados a Médio Prazo

The Ross procedure has been used in children and young adults for aortic valve replacement and the correction of complex obstruction syndromes of the left ventricular outflow tract. We report the mid-term results of the Ross procedure in a single institution and performed by the same surgical team. Population: Between March 1999 and December 2005, 18 patients were operated on using the Ross procedure. The mean age at the time of surgery was 12 years, being 12 patients male (67%). The primary indication for surgery was isolated aortic valve disease, being the predominant abnormality in 58% of cases aortic regurgitation and in 42% left ventricular outflow tract obstruction. Associated lesions included sub-aortic membrane in 3 patients (16%), small VSD in 2 patients (11%), bicuspid aortic valve in 4 patients (22%) and severe left ventricular dysfunction and mitral valve regurgitation in 1 patient (6%). Ten of the 18 patients (56%) had been submitted to previous surgical procedures or percutaneous interventions. Results: Early post-operative mortality was not seen, but two patients (11%), had late deaths, one due to endocarditis, a year after the Ross procedure, and the other due to dilated cardiomiopathy and mitral regurgitation. The shortest time of follow-up is 6 months and the longest 72 months (median 38 months). Of the 16 survivors, 14 patients are in class I of the NYHA and 2 in class II, without significant residual lesions or need for re-intervention. The 12 patients with more than a year of follow up revealed normal coronary perfusion in all patients and no segmental wall motion abnormalities. Nevertheless, two of the 12 patients developed residual dynamic obstruction of LVOT and in three patients aortic regurgitation of a mild to moderate degree was evident. Significant gradients were not verified in the RVOT. Conclusions: The Ross procedure, despite its complexity, can be undertaken with excellent immediate results. Aspects such as the dilation of the neo aortic root and homograft evolution can not be considered in a study of this nature, seeing that the mean follow up time does not exceed 5 years.

Cirurgias de Derivação do Ventrículo Pulmonar

Cavopulmonary connections have been extensively used in the palliation of complex forms of congenital heart disease requiring some form of right heart bypass. We examine the mid term outcomes of pulmonary ventricle bypass operations in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and April 2006, 62 patients underwent pulmonary ventricle bypass operations: bidirectional cavopulmonary anastomosis (Glenn procedure), total cavopulmonary connections (Fontan procedure) and one and a half ventricle correction in two cases. Age at operation averaged three years (range: 0.42-25 years) for the Glenn procedure and seven years (range: 3-14 years) for the Fontan procedure. There were 36 male patients (58%) and 26 female patients (42%). The most common indication for surgery was the single ventricle defect, present in 66% of patients. Associated lesions included: transposition of the great arteries in 16 patients (35.6%), bilateral superior vena cava in four patients (8.9%), situs ambigus in five patients (11%), situs inversus in another patient (2.2%), Ebstein disease in one patient (2.2) and coronary fistula in another patient (2.2%). Sub-aortic stenosis was present in one patient (2.2%). Palliative surgery was performed in all, but three patients (5%), before the Fontan procedure. RESULTS: Thirty two patients underwent bidirectional cavopulmonary anastomosis and thirty patients underwent cavopulmonary connections, total or 2nd stage. Mean cardiopulmonary bypass times were 50.6+/-21.9 minutes for the Glenn procedure and 88.5+/-26.3 minutes for the Fontan procedure. There was no intra-operative mortality, but two patients (3.2% (died in the first month after surgery; one due to failure of the Glenn circuit and sepsis and the other due to a low cardiac output syndrome and multi-organ dysfunction. Mean ventilation time was 5.2+/-1.7 hours for the Glenn operation and 6.2+/-3.2 hours for the Fontan operation. The mean length of stay in ICU was 3.4+/-2.8 days for patients undergoing the Glenn operation and 4.6+/-3.1 days for patients undergoing the Fontan operation and the mean length of hospital stay was 10.6+/-5.8 days for the Glenn operation and 19.1+/-12.6 days for the Fontan operation respectively. The mean follow up time was 4+/-2.1 years (minimum 0 years and maximum seven years), most patients being in NYHA class I. Epicardiac pacemakers were implanted in three patients due to arrhythmias. Two re-operations (6.7%) were needed, both in the same patient, after the Fontan procedure, this patient eventually died a few years after surgery. CONCLUSIONS: The immediate and mid term outcomes of pulmonary ventricle bypass operations can have excellent results. From our point of view there has been an improvement, namely in the use of the extracardiac conduit technique in the 2nd stage of the Fontan operation.

Endocardite a Nocardia após Cirurgia de Substituição Valvular Mitral: Caso Clínico e Revisão da Literatura

A Nocardia é responsável por diversos tipos de infecção quer em receptores imunocompetentes, quer imunocomprometidos e pode afectar qualquer órgão. A endocardite a Nocardia spp é muito rara e tem mau prognóstico. Segundo o nosso conhecimento e após revisão da literatura, foram reportados apenas 12 casos de endocardite a Nocardia, a maioria tratada com substituição valvular. Reportamos o primeiro caso descrito em Portugal de endocardite protésica a Nocardia, tratado com sucesso apenas com terapêutica antimicrobiana (trimetoprimsulfametoxazol), sem necessidade de substituição valvular.

Angioplastia Coronária no Enfarte Agudo do Miocárdio: Impacto do Timing da Revascularização na Alternância da Onda Ta

Encontra-se bem estabelecido que, na abordagem terapêutica do enfarte agudo do miocárdio (EAM), a trombólise e a angioplastia coronária percutânea (ACTP) permitem diminuir a mortalidade e melhorar o prognóstico, sendo esse benefício tanto maior quanto menor o tempo decorrido entre o evento isquémico e o procedimento terapêutico. No entanto, não está ainda estabelecido qual o impacto que o atraso da implementação dessas terapêuticas tem no desenvolvimento de taquidisritmias ventriculares e por consequência no resultado da alternância da onda T(TWA), que representa um método reconhecido para avaliação da vulnerabilidade ventricular às referidas arritmias. Objectivo: Analisar os resultados da TWA por microvoltagem numa população submetida a ACTP na sequência de EAM e avaliar a influência do timing de revascularização miocárdica nos resultados da TWA. Métodos: Estudámos 79 doentes (67 do sexo masculino; 57 ± 11 anos) consecutivos, admitidos por EAM e sujeitos a revascularização miocárdica por ACTP durante o internamento. A TWA foi avaliada utilizando um aparelho da HeartTwave System (Cambridge Heart, Inc., Bedford, Massachusetts) nos 30 dias pós-EAM. Durante a realização de uma prova de esforço em tapete rolante com protocolo manual, com o objectivo de elevar a frequência cardíaca até aos 110 batimentos por minuto, realizou-se o registo electrocardiográfico através da aplicação de sete eléctrodos standard e outros sete eléctrodos sensores de alta-resolução, especialmente concebidos para redução do «ruído», dispostos segundo as derivações ortogonais de Frank(X,Y, Z). A TWA foi considerada positiva quando se verificou a presença de alternância da onda T de magnitude ³ 1,9 μV de forma consistente e mantida (> 1 minuto), com início para frequências cardíacas < 110 batimentos/minuto ou quando esta alternância se verificou em doentes em repouso. Foi considerada negativa quando se conseguiu obter dados sem artefactos durante pelo menos um minuto, com frequências > 105 batimentos/minuto, sem atingir critério de positividade e indeterminada se não podia ser classificada como positiva ou negativa. Foram excluídos doentes com EAM ou revascularização miocárdica (cirúrgica ou percutânea) prévios, os que apresentavam insuficiência cardíaca congestiva, com fibrilhação auricular, > 10 extrassístoles por minuto, pacemaker definitivo, bradicárdia < 40 bpm, complexos QRS com duração > 130 ms ou sob terapêutica anti-arrítmica. Considerámos como marcador de risco para a ocorrência de eventos arrítmicos ventriculares malignos a presença de TWA positiva ou indeterminada. (TWA «nãonegativa»). Os resultados da TWA foram comparados entre o grupo de doentes submetidos a ACTP nas primeiras 24 horas pós-EAM (Grupo A; n = 45) e o grupo de doentes submetidos a ACTP > 24 horas pós-EAM. (Grupo B; n = 34) Resultados: A TWA foi positiva em 16 doentes (20,2%) e negativa em 56 (70,9%). Em 7 casos (8,9%), o resultado do teste foi considerado indeterminado. A TWA foi «não-negativa» em 29,1% da população. No grupo A a TWA foi “não-negativa” em 9 doentes (20 %) (6 com TWA positiva e três com TWA indeterminada) e negativa em 36 doentes (80 %) e no grupo B foi «não-negativa» em 14 doentes (41%) (10 com TWA positiva e quatro com TWA indeterminada) e negativa em 20 (59 %) (p < 0,05). Não se encontraram diferenças entre os dois grupos no que respeita à fracção de ejecção ventricular esquerda. No seguimento até aos 60 dias após a alta hospitalar não foram documentados eventos arrítmicos ventriculares, síncopes ou óbito. Foram reinternados cinco doentes (7 %) por recorrênciade angor. Conclusões: Numa população de sobreviventes de EAM encontrámos uma prevalência de TWA não negativa de 29 %, apesar da revascularização miocárdica com ACTP. A ACTP, quando efectuada nas primeiras 24 horas após o início do EAM, reduz de forma significativa o número de doentes com TWA não negativa,sugerindo que esta intervenção precoce poderá baixar o risco arrítmico destes doentes e influenciar favoravelmente o prognóstico pós-EAM. O impacto da morte súbita na mortalidade pós-EAM justifica estudos prospectivos de maiores dimensões

Results of Surgery for Ebstein Anomaly: a Multicenter Study from the European Congenital Heart Surgeons Association

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

Derrame Pericárdico Recidivante. Um Caso de Angiosarcoma Cardíaco

A case of angiosarcoma of the right atrium in a 59-year-old woman is reported, presenting as a recurrent hematic pericardial effusion. The usefulness of echocardiography in making the diagnosis is emphasized. The tumor was already disseminated at the time of the diagnosis. The patient was on palliative therapy, dying a few months later. The histological examination was done at autopsy. This case highlights the difficulty in diagnosis and the various modalities available for an accurate diagnosis of a rare clinical entity. It has a rapid and aggressive course with a very poor prognosis.

Effects of Acute Autonomic Modulation on Atrial Conduction Delay and Local Electrograms Duration in Paroxysmal Atrial Fibrillation

Slowed atrial conduction may contribute to reentry circuits and vulnerability for atrial fibrillation (AF). The autonomic nervous system (ANS) has modulating effects on electrophysiological properties. However, complex interactions of the ANS with the arrhythmogenic substrate make it difficult to understand the mechanisms underlying induction and maintenance of AF. AIM: To determine the effect of acute ANS modulation in atrial activation times in patients (P) with paroxysmal AF (PAF). METHODS AND RESULTS: 16P (9 men; 59±14years) with PAF, who underwent electrophysiological study before AF ablation, and 15P (7 men; 58±11years) with atrioventricular nodal reentry tachycardia, without documentation or induction of AF (control group). Each group included 7P with arterial hypertension but without underlying structural heart disease. The study was performed while off drugs. Multipolar catheters were placed at the high right atrium (HRA), right atrial appendage (RAA), coronary sinus (CS) and His bundle area (His). At baseline and with HRA pacing (600ms, shortest propagated S2) we measured: i) intra-atrial conduction time (IACT, between RAA and atrial deflection in the distal His), ii) inter-atrial conduction time (interACT, between RAA and distal CS), iii) left atrial activation time (LAAT, between atrial deflection in the distal His and distal CS), iv) bipolar electrogram duration at four atrial sites (RAA, His, proximal and distal CS). In the PAF group, measurements were also determined during handgrip and carotid sinus massage (CSM), and after pharmacological blockade of the ANS (ANSB). AF was induced by HRA programmed stimulation in 56% (self-limited - 6; sustained - 3), 68.8% (self-limited - 6; sustained - 5), and 50% (self-limited - 5; sustained - 3) of the P, in basal, during ANS maneuvers, and after ANSB, respectively (p=NS). IACT, interACT and LAAT significantly lengthened during HRA pacing in both groups (600ms, S2). P with PAF have longer IACT (p<0.05), a higher increase in both IACT, interACT (p<0.01) and electrograms duration (p<0.05) with S2, and more fragmented activity, compared with the control group. Atrial conduction times and electrograms duration were not significantly changed during ANS stimulation. Nevertheless, ANS maneuvers increased heterogeneity of the local electrograms duration. Also, P with sustained AF showed longer interACT and LAAT during CSM. CONCLUSION: Atrial conduction times, electrograms duration and fractionated activity are increased in PAF, suggesting a role for conduction delays in the arrhythmogenic substrate. Acute vagal stimulation is associated with prolonged interACT and LAAT in P with inducible sustained AF and ANS modulation may influence the heterogeneity of atrial electrograms duration.

Metastização Pulmonar na Apresentação de Angiossarcoma Cardíaco. Caso Clínico e Discussão

Apresenta-se um caso clínico referente a doente de 35 anos, do sexo masculino sem antecedentes pessoais relevantes, admitido no serviço de urgência por quadro de toracalgia e tosse produtiva com alterações electrocardiográficas sugestivas de pericardite. Inicialmente admitido pelo Serviço de Cardiologia, com melhoria do quadro clínico após terapêutica anti- -inflamatória; contudo, no internamento houve como intercorrência pneumonia de provável etiologia bacteriana, complicada por derrame pleural. Após a alta,foi referenciado à consulta de pneumologia, onde se manteve o estudo etiológico do derrame persistente, tendo vindo a complicar-se o seu quadro com alterações das cavidades cardíacas e múltiplos nódulos pulmonares, sugestivos de endocardite subaguda com embolização séptica pulmonar. Internado no serviço de Pneumologia e submetido a videotoracoscopia, foi-lhe diagnosticado angiossarcoma cardíaco com metastização pulmonar. Assistiu-se a uma rápida evolução do quadro clínico, quase fulminante, com falência cardíaca e óbito do doente sem ter iniciado radioterapia ou quimioterapia adjuvante.

Primary Angiosarcoma of the Pericardium. Case Report and Review of the Literature

Primary cardiac tumours are rare entities and angiosarcoma is the most frequent primary cardiac malignant tumour. Mean survival is six months and the tumour responds poorly to chemotherapy. We present the case of a 50 year-old patient with localised pericardial angiosarcoma who survived 23 months after diagnosis with a combined approach of chemotherapy and surgery.

Mitral Valve Myxomas: an Unusual Entity

Primary tumours of the heart are uncommon entities, cardiac myxomas being the most frequent. However, mitral valve myxomas are exceptionally rare. In the last 12 years, there have been 25 myxomas diagnosed at our institution, with only two of them originating from the mitral valve. Both patients were female, the first, 25, and the second, 72 years old. The younger patient was very symptomatic with a large mass, 4 cm long, which involved both leaflets causing significant obstruction to the left ventricular inflow. The second one had a smaller mass located at the atrial side of the posterior leaflet that only produced some flow divergence. Neither of them had constitutional nor embolic symptoms. Both patients were submitted to emergent surgical resection that in the first case involved the mitral valve and replacement with mechanical prosthesis. The macroscopic appearance of these tumours suggested a malignant aetiology which may represent somewhat different features of the myxomas when originating from the cardiac valves. Both patients are well reflecting the good prognosis of this illness after resection, although the younger patient was re-operated because of prosthetic valve obstruction and suspicion of recurrence that was not confirmed. Because of the illustrative images and different presentations, we found it interesting to report and discuss them together.