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Aims Assessing reversibility of pulmonary vascular changes through vasoreactivity testing (VRT) optimizes end‐stage heart failure patient selection for heart transplant. All efforts should be made to unload the left ventricle and reduce pulmonary vascular resistance to effectively exclude irreversible pulmonary hypertension. Methods and results We reviewed our centre's cardiac transplant registry database (2009–2017) for VRT and compared haemodynamic responses with 40 ppm inhaled NO (n = 14), 14–17 μg inhaled iloprost (n = 7), and 24 h 0.1 μg/kg/min intravenous levosimendan (n = 14). Response to levosimendan was assessed by repeat right heart catheterization within 72 h. Baseline clinical and haemodynamic features were similar between groups. VRT was well tolerated in all patients. All drugs effectively reduced pulmonary artery pressures and transpulmonary gradient while increasing cardiac index, although levosimendan had a greater impact on cardiac index increase (P = 0.036). Levosimendan was the only drug that reduced pulmonary artery wedge pressure (P = 0.004) and central venous pressures (P

BACKGROUND: Heart failure (HF) is a growing public health problem. Sacubitril/valsartan is now recommended to be used in persistently symptomatic patients with left ventricular ejection fraction (LVEF)

Infective endocarditis still has significant morbimortality, despite diagnostic and therapeutic improvements. We describe a case of a 67-year-old man with recurrent prosthetic aortic valve infective endocarditis complicated by massive local tissue damage, making valve replacement unfeasible. Although rarely performed in this setting, heart transplantation was a needed salvage strategy, highlighting the need to make bold decisions for complex clinical scenarios.

Introduction: Since 2011, the European guidelines have included a specific low-density lipoprotein cholesterol (LDL-C) target

Introduction: Biomarkers in dilated cardiomyopathy (DCM) reflect various pathobiological processes, including neurohormonal activation, oxidative stress, matrix remodeling, myocyte injury and myocyte stretch. We assessed the role of biomarkers in clinical and echocardiographic parameters and in left ventricular (LV) reverse remodeling (LVRR). Methods: In this prospective study of 50 DCM patients (28 men, aged 59±10 years) with LV ejection fraction (LVEF) 10 U in LVEF after optimal medical therapy. Results: Baseline LVEF was 25.4±9.8% and LV end-diastolic diameter (LVEDD)/body surface area (BSA) was 34.2±4.5 mm/m2. LVRR occurred in 34% of patients within 17.6±15.6 months. No correlation was found between B-type natriuretic peptide (BNP), 25-hydroxyvitamin D (25(OH)D), CA-125, high-sensitivity C-reactive protein (hs-CRP), lipoprotein(a) [Lp(a)], noradrenaline, adrenaline, renin or aldosterone and LVRR. Patients in NYHA class III or IV, with pulmonary congestion or ankle edema, had higher CA-125, cystatin C, BNP and hs-CRP levels (p10 U da LVEF, após a terapêutica médica otimizada. Resultados: A LVEF basal foi de 25,4±9,8% e o diâmetro do VE (LVD)/BSA de 34,2±4,5 mm/m2. A LVRR ocorreu em 34%, em 17,6±15,6 meses. Não houve correlação entre BNP, 25-OH-vit D, CA 125, hsCRP, Lp(a), noradrenalina, adrenalina, renina, aldosterona e LVRR. Doentes em classe NYHA (III-IV), com congestão pulmonar ou edema periférico apresentaram níveis mais elevados de CA 125, cistatina C, BNP e hsCRP (p

Introduction Vasodilator challenge (VC) during right heart catheterization in heart transplant (HTx) candidates is warranted whenever pulmonary artery (PA) systolic pressure ≥50 mmHg and either transpulmonary gradient (TPG) ≥15 mmHg or pulmonary vascular resistance (PVR) >3 WU as long as systolic arterial blood pressure >85 mmHg. Nitric oxide (NO) remains the mainstay but in doubtful cases a 24–48h course of diuretics, inotropes and vasoactive agents may be required. Our aim is to report our centre's experience with levosimendan (LEVO) as alternative to NO in VC in HTx candidates due to advanced heart failure (HF). Methods VC records with either NO (20 ppm for 5–10 mins) or within 72h of LEVO infusion (12 mg/kg/min for 24–48h) carried out between 2009 and September 2018 were retrieved from the centre's database. Analysis was carried out with Fisher's exact test or Student's t-test for categorical and continuous variables, respectively, or the equivalent non-parametric test for non-normal distribution variables. Data are presented as counts and percentage, or mean ± standard deviation and median, percentile 25–75, for categorical and continuous variables, respectively. Results Baseline demographic and clinical characteristics from 26 patients (NO=13; LEVO=13) were similar between groups (12% female; 54±10 years of age; left ventricular ejection fraction 20±7%; BNP 1550±1090 pg/mL; 88% on NYHA III-IV). Although no differences were observed in baseline cardiac index (CI, 1.6±0.3 vs 1.4±0.4 L/min.m-2, in NO and LEVO, respectively), LEVO patients showed higher right ventricular systolic (70±10 vs 60±13 mmHg; p=0.036) and diastolic pressures (16±4 vs 11±5 mmHg; p=0.009) and lower PA compliance (0.9±0.2 vs 1.3±0.4 ml/mmHg; p=0.007) as well as a trend for increased PA wedge pressure (26±4 vs 21±4 mmHg; p=0.09), translating worse hemodynamics. Upon VC only LEVO decreased PA pressure and the increase in CI was higher compared with NO (2.5±0.8 vs 1.9±0.5 L/min.m-2, p=0.004) thus PVR reduction was comparable between groups (7.8±2.7 to 4.7±1.8 vs 6.3±2.3 to 3.6±2.1 WU, respectively). Also, only LEVO increased right (497, 387–837 to 791, 570–946 mmHg.mL.m-2; p=0.006) and left ventricular stroke work index (895, 807–1364 to 1257, 1107–2957 mmHg.mL.m-2; p=0.005) and cardiac power output (0.4±0.1 to 0.6±0.1 W; p Conclusion LEVO is a safe and effective alternative in PVR reduction for VC. Its positive inotropic effect and long-lasting hemodynamic improvement may improve clinical status before HTx and allow better scrutiny of suitable candidates.

Introduction and Objectives: Dilated cardiomyopathy (DCM) is a myocardial disease that can progress to a terminal stage, requiring heart transplantation. In this work we aim to contribute to knowledge of genetic variants in adult patients undergoing heart transplantation due to end-stage DCM, reporting the results obtained in our single-center tertiary hospital series using target next-generation sequencing (NGS). Methods and Results: Genetic variants were screened in 15 genes, preselected based on variants previously identified in DCM patients. Thirteen unrelated patients were included, nine (69%) male, mean age at diagnosis 33±13 years, eight (62%) with familial DCM. Nine genetic variants were identified in six (46%) patients: five in LMNA, two in LBD3, one in TNNT2 and one in TCAP. These variants were new in most patients. The majority were classified as of uncertain significance. Two patients were double and triple heterozygotes in the LBD3 and LMNA genes, respectively. Conclusion: Our results highlight the potential of NGS in the genetic characterization of DCM patients. LMNA is one of the most frequently mutated genes and should be included in all target gene assessments of end-stage DCM patients until more data are available. Resumo: Introdução e objetivos: A miocardiopatia dilatada é uma doença miocárdica que pode evoluir para um estádio terminal, requerendo transplante cardíaco. Neste trabalho, pretendemos contribuir para o conhecimento das variantes genéticas presentes em pacientes adultos submetidos a transplante cardíaco, descrevendo os resultados obtidos utilizando técnicas de sequenciação de ADN de nova geração. Métodos e resultados: Variantes genéticas foram pesquisadas em 15 genes pré-selecionados com base em variantes previamente identificadas em pacientes com miocardiopatia dilatada. Foram incluídos 13 pacientes não relacionados, nove (69%) do sexo masculino, com idade média na altura do diagnóstico de 33±13 anos, oito (62%) com doença familiar. Foram identificadas nove variantes genéticas em seis (46%) pacientes: LMNA-5, LBD3-2, TNNT2-1 e TCAP- 1. A maioria das variantes genéticas foi classificada como de significado incerto. Dois pacientes eram heterozigotos duplos e triplos nos genes LBD3 e LMNA, respetivamente. Conclusões: Os nossos resultados reforçam o potencial das novas tecnologias de sequenciação na caracterização genética de doentes com miocardiopatia dilatada. Até que mais dados estejam disponíveis, o gene LMNA é um dos mais frequentemente envolvidos e deverá ser incluído na avaliação de pacientes com miocardiopatia dilatada em fase terminal. Keywords: Dilated cardiomyopathy, Heart transplantation, Next-generation sequencing, Genetic variants, Palavras-chave: Miocardiopatia dilatada, Transplante cardíaco, Sequenciação de nova geração, Variantes genéticas

Introduction: Cardiac remodeling is manifested as changes in size, shape and function of the heart. We studied the prevalence, prognosis and predictors of left ventricular reverse remodeling (LVRR) in idiopathic dilated cardiomyopathy (IDCM) after optimized medical therapy. Methods: A total of 113 IDCM patients were followed for 7.1±5.6 years. LVRR was defined as an increase of 10 units in ejection fraction (EF) and decrease in left ventricular diastolic diameter (LVDD), in the absence of resynchronization therapy. Results: Baseline EF was 27±8% and LVDD index was 37.1±6.3 mm/m2. LVRR occurred in 34.5% within 22.6 months. Final EF was 47.5±10.1%, LVDD index was 30.2±3.9 mm/m2. LVRR was associated with better NYHA class (I–II) and lower BNP (p

This chapter looks at employee’s perceptions regarding the type of family-friendly work practices available to them by their employing organizations and, at the same time, it conveys their views on the organizational support received on these matters. The empirical research is based on a case study design that includes four companies belonging to different industries in Portugal . Overall, we conducted 24 in-depth interviews with both operational employees and human resource managers . The findings show that the HR managers in all the companies admit not having formal procedures on family-friendly work practices but only a set of informal practices that vary according to the functional level and the employee rank . The company size and resources, the type of operational activities or the work schedules are important explanatory factors for the scant adoption and implementation of family-friendly practices in these companies, and seem to play a more influential role than institutional forces. Immediate supervisor and co-worker support are perceived by the employees as fundamental sources of organizational support in the work–family life conciliation . The study limitations and future research suggestions are also presented.

Objective: To assess the prognostic significance of iron deficiency (ID) in a chronic heart failure (CHF) outpatient population. Methods and Results: We prospectively evaluated 127 patients with stable CHF and left ventricular ejection fraction ≤45%. Clinical and analytical data as well as information regarding the occurrence of the composite endpoint of overall mortality and nonfatal cardiovascular events were assessed. Among the 127 patients enrolled [81% men, median age: 62 years (25th-75th percentile: 53-68)], 46 (36%) patients had ID. Women, patients with higher plasma brain natriuretic peptide levels (>400 pg/ml) and with right ventricular systolic dysfunction presented ID more frequently (p < 0.05 for all). At 225 ± 139 days of follow-up, the composite endpoint occurred in 15 (12%) patients. It was more frequent in ID (24 vs. 5%, p = 0.001) and anemic patients (25 vs. 8%, p = 0.014). In a Cox regression analysis, ID was associated with a higher likelihood of composite endpoint occurrence (HR 5.00, 95% CI 1.59-15.78, p = 0.006). In a multivariable analysis adjusted for clinical variables, including the presence of anemia, ID remained a significant predictor of the composite endpoint (HR 5.38, 95% CI 1.54-18.87, p = 0.009). Conclusion: In a CHF outpatient population, ID carried a higher risk of unfavorable outcome, irrespectively of the presence of anemia.

Heart transplantation is the treatment of choice in severe heart failure despite maximal medical therapy, which has no other surgical alternatives and exhibiting no contraindications. The aim of this study was to analyze the prevalence of late complications and survival of patients undergoing cardiac transplantation at our Hospital Center.We evaluated 78 patients (mean age 43 ± 15 years) transplanted at our center between February 1987 and December 2011, with a mean follow-up of 6 years.Of late complications after heart transplantation, allograft vascular disease was detected in 10 patients ( 12.8%), was the one with impact on mortality, being responsible for four deaths. The most frequent complication was hypertension in 54.6% of cases, followed by dyslipidemia (47.4%), renal failure (47.4%), diabetes mellitus (21.8%) and neoplasms (11.5%). Atrial tachyarrhythmias was observed in eight patients (10.3%). The overall survival of our population at first and tenth year after heart transplantation was 81% and 69%, respectively. The mean survival of patients was 15.6 years (Cl 95%: [12,6-18,7]).. There were 23 deaths (29.5%), nine (11.5%) of which occurred within the first 30 days after transplantation.Cardiac transplantation remains a valid therapeutic option for patients with end-stage heart disease. Our center had a heart transplant survival rate and incidence of late complications similar to those seen in international registries.

Resumo: Os autores apresentam um caso clínico de um homem de 68 anos com clínica de insuficiência cardíaca predominantemente direita, no contexto de estenose aórtica grave associada a hipertensão pulmonar. Concomitantemente, foi diagnosticada anemia que, após estudo endoscópico, se concluiu ser secundária a angiodisplasias intestinais, tendo sido feito o diagnóstico de síndrome de Heyde.Após cirurgia de substituição valvular houve resolução do quadro, com melhoria dos sintomas de insuficiência cardíaca prévia e estabilização dos valores de hemoglobina.Com este caso pretende‐se mostrar a necessidade do conhecimento de associações menos frequentes na estenose aórtica grave para uma atuação terapêutica imediata e adequada. Abstract: The authors present the case of a 68‐year‐old man with predominantly right heart failure in the context of severe aortic stenosis associated with pulmonary hypertension. Anemia was diagnosed which, after endoscopic study, was considered to be secondary to angiodysplasia and a diagnosis of Heyde syndrome was made.After valve replacement surgery the patient's heart failure improved and hemoglobin levels stabilized.We present this case to show the need to recognize less common associations of severe aortic stenosis, in order to provide immediate and appropriate treatment. Palavras‐chave: Estenose aórtica grave, Hemorragia gastrointestinal, Angiodisplasias, Síndrome de Heyde, Hipertensão pulmonar, Keywords: Severe aortic stenosis, Gastrointestinal bleeding, Angiodysplasia, Heyde syndrome, Pulmonary hypertension

The authors present the case of a 68-year-old man with predominantly right heart failure in the context of severe aortic stenosis associated with pulmonary hypertension. Anemia was diagnosed which, after endoscopic study, was considered to be secondary to angiodysplasia and a diagnosis of Heyde syndrome was made. After valve replacement surgery the patient's heart failure improved and hemoglobin levels stabilized.We present this case to show the need to recognize less common associations of severe aortic stenosis, in order to provide immediate and appropriate treatment. Resumo: Os autores apresentam um caso clínico de um homem de 68 anos com clínica de insuficiência cardíaca predominantemente direita, no contexto de estenose aórtica grave associada a hipertensão pulmonar. Concomitantemente, foi diagnosticada anemia que, após estudo endoscópico, se concluiu ser secundária a angiodisplasias intestinais, tendo sido feito o diagnóstico de síndrome de Heyde.Após cirurgia de substituição valvular houve resolução do quadro, com melhoria dos sintomas de insuficiência cardíaca prévia e estabilização dos valores de hemoglobina.Com este caso pretende-se mostrar a necessidade do conhecimento de associações menos frequentes na estenose aórtica grave para uma atuação terapêutica imediata e adequada. Keywords: Severe aortic stenosis, Gastrointestinal bleeding, Angiodysplasia, Heyde syndrome, Pulmonary hypertension, Palavras-chave: Estenose aórtica grave, Hemorragia gastrointestinal, Angiodisplasias, Síndrome de Heyde, Hipertensão pulmonar

We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.

Familial dilated cardiomyopathy (FDCM) is characterized by clinical and genetic heterogeneity. There are still few survival studies concerning this subgroup of patients.To determine the prognosis of patients with FDCM on optimal medical therapy and attending a heart failure clinic.This is a prospective study including patients with FDCM, defined according to the guidelines of the European Society of Cardiology. Cardiovascular morbidity and all-cause mortality were evaluated.Thirty-six patients, 23 (64%) men, were followed for 3.8 +/- 2.5 years. Age at baseline was 42 +/- 14 years and 67% were in NYHA class II. In 22% heart failure symptoms first occurred after a respiratory infection, and in 6%, after pregnancy/delivery. Most patients were in sinus rhythm (89%) and 33% had left bundle branch block (LBBB). Baseline left ventricular (LV) ejection fraction was 28 +/- 9%, LV end-diastolic diameter was 68 +/- 8 mm and left atrial dimension was 46 +/- 9 mm. Baseline serum sodium was 140 +/- 3 mEq/l. All patients were taking angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin-II receptor blockers (ARBs), 81% beta-blockers and 47% spironolactone. During follow-up, 5 patients died, 4 underwent heart transplantation and one received an implantable cardioverter-defibrillator. Five-year survival was 68%.Five-year survival of our patients with FDCM, under optimal medical therapy, was similar to that of other forms of nonischemic DCM reported in the literature.

In previous randomized studies levosimendan improved hemodynamics and clinical course, with a still unclear effect on prognosis. There are, however, few data regarding its effects when used in daily practice.We evaluated the clinical effectiveness and safety of levosimendan in the treatment of acute systolic heart failure (SHF) in daily practice conditions.In this prospective, multicenter, nonrandomized trial, a continuous infusion of levosimendan (0.05 microg/kg/min-0.2 microg/kg/min) was administered for 24 hours. An optional loading dose of 12 microg/kg over 10 minutes was used. The primary combined endpoint of clinical effectiveness (as defined by a eight-variable clinical score) and safety (defined by the absence of serious adverse events) was assessed at 24 hours after the beginning of treatment; a second similar primary combined endpoint was assessed at 5 days.One hundred and twenty-nine consecutive patients requiring inotropes despite optimal oral background heart failure therapy were recruited. The primary endpoint was reached in 80.6% at 24 hours and in 79.7% at 5 days. During the six months before levosimendan the number of patient days of hospitalization for heart failure was 14.9 +/- 14.6 versus 3.1 +/- 7.6 during the six months following levosimendan (p0.001).In daily practice, levosimendan was clinically effective and safe in 80.6% and 79.7% of patients with acute SHF at 24 hours and 5 days respectively after the beginning of treatment. A marked reduction in the number of days of hospitalization for heart failure was also seen during the subsequent six months.

The pathological significance of myocardial adrenergic activity in patients with heart failure is well documented. No previous study has assessed the usefulness of I123-metaiodobenzylguanidine (123I-MIBG) cardiac uptake imaging for the evaluation of familial dilated cardiomyopathy (DCM).To evaluate cardiac adrenergic activity, using 123I-MIBG cardiac uptake imaging, in members of a genotyped family with DCM.Clinical evaluation, 12-lead ECG, 2D echocardiogram, heart rate variability analysis by 24h Holter, plasma B-type natriuretic peptide (BNP) measurements and 123I-MIBG cardiac imaging were performed in all participants. Anterior projection planar images and single photon emission computed tomographies of the thorax were obtained 20 min and 4 hours after the intravenous administration of 370 MBq of 123I-MIBG (early and late images). Heart/mediastinal (H/M) ratio and myocardial washout (MW) rate were obtained based on the anterior planar images. In polar maps, segmental uptake of 123I-MIBG was evaluated using a 4-grade visual score: grade 1 - uptake75% of maximum myocardial uptake (MMU); grade 2 - uptake 51-75% of MMU; grade 3 - uptake 26-50% of MMU; grade 4 - uptakeor = 25% of MMU.Eleven adults were included: 4 with DCM, 4 with isolated left ventricular enlargement (LVE), and 3 with normal echocardiogram. Patients with DCM and LVE presented higher MW rates, lower H/M ratios and higher visual score grades than those with normal 2D echocardiograms. One patient with a normal echocardiogram but carrying the disease locus also presented an abnormal MIBG cardiac scintigram.Patients with the phenotypic expression of the disease (DCM and LVE) and even carriers of the DCM gene with normal echocardiograms may present an abnormal MIBG cardiac scintigram, probably reflecting cardiac adrenergic hyperactivity. If confirmed in larger numbers, this method may be useful for the evaluation of DCM families.

Cardiac troponin I (cTnI) is a specific marker which allows detection of minor myocardial cell damage. In patients with severe pulmonary embolism (PE), the rise in pulmonary artery pressure can lead to progressive right ventricular dysfunction (RVD), and clinical studies have demonstrated the presence of ischemia and even right ventricular infarction. Our aims were to determine the prevalence and diagnostic utility of cTnI in identifying patients with RVD and to ascertain whether it correlates with severity of PE.We studied 77 patients with PE diagnosed by pulmonary angiography, ventilation-perfusion lung scan, spiral computed tomography scan or a combination of abnormal echocardiogram with clinical presentation suggestive of PE or with positive subsidiary exams (d-dimers, venous Doppler of the lower limbs, ECG, blood gas analysis). We further classified the PE according to the European Society of Cardiology severity levels, the PE being: 1) massive, if there was shock and/or hypotension; 2) submassive, if we found right ventricular hypokinesis on the echocardiogram; and 3) non-massive, in the remaining cases. We considered the highest cTnI serum value from the admission to 24 hours and a normal value of0.10 ng/ml.Among the 60 patients with cTnI measurements, 42 had elevated values. Among those with RVD, 26 (81.3%) had increased cTnI levels and only 14 (35%) with elevated cTnI values did not have RVD, indicating that positive cTnI tests were significantly associated with RVD (p = 0.038). Patients with positive cTnI tests had earlier onset of symptoms (24.0 vs. 144.0 hours, p=0.02), higher prevalence of emboli in proximal vessels (pulmonary trunk and right or left main pulmonary arteries) (OR = 12, CI= 1.6-88.7), and received more thrombolytic therapy (OR = 5.4, CI = 1.1-26.8) than those with normal cTnI tests. cTnI levels were higher among patients with submassive PE (median: 0.77 ng/ml) and lower in those with non-massive PE (0.08 mg/ml, p0.05).Around 70% of patients with PE have elevated cTnI values and this test is significantly associated with RVD. cTnI measurements provide additional information in the evaluation of patients with PE by identifying more severe cases and those at increased risk of hemodynamic deterioration, who can benefit from more aggressive therapeutic strategies.

Corrective surgery for tetralogy of Fallot (TF) has led to excellent survival. However; several years after surgery, the majority of patients have right ventricular (RV) dilatation, and 10% will need reoperation of the RV outflow tract due to limited exercise capacity, ventricular arrhythmias or symptoms of heart failure (HF). Our aim was to identify predictive factors of adverse outcome: moderate to severe RV dilatation, HF, reoperation of the RV outflow tract and cardiac death.Eighty-eight adult patients with TF were operated between January 1977 and July 2001; 22 were lost to follow-up and 66 were followed for 18 +/- 6 years. We analyzed clinical, electrocardiographic and echocardiographic variables. RV dilatation was considered to exist if the inlet measurement at end-diastole in 4-chamber apical view was more than 35 mm, being classified as moderate whenor = 50 and60 mm and severe whenor = 60 mm.Of the 66 patients, 25 (37.9%) had undergone previous palliative shunt (PS) at the age of 4 +/- 5 years. Mean age at surgical correction was 10 +/- 8 years (range:1 to 38 years; median: 6.5 years). Transannular patching was used in 65% of patients, patch closure of a right ventriculotomy in 91%, and in 53% of patients a pulmonary commissurotomy was performed. At the end of follow-up, 3 patients were in NYHA class III-IV and one patient was successfully reoperated with implantation of a biological pulmonary valve. Prevalence of RV dilatation was 97% (57/59), being moderate to severe in 69% (36/52). In patients with moderate to severe RV dilatation we found previous PS (18.8 vs. 50.0%; p = 0.03), transannular patching (37.5 vs. 75.0%; p 0.01) and wide QRS (160 ms) (6.7 vs. 45.7%, p = 0.01) to be more frequent. These patients reported more palpitations (0 vs. 22.2%; p 0.05), but there were no differences in arrhythmic events (18.8 vs. 33.3%; p = 0.28); maximal heart rate on exercise was lower (86.2 +/- 10.9 vs. 79.9 +/- 8.6; p = 0.04), but exercise time and functional capacity were similar between the groups. Follow-up time and use of RV patching were similar. Transannular patching was associated with previous PS at an older age (0.9 +/- 0.7 vs. 4.9 +/- 5.7 years; p = 0.01), a higher grade of pulmonary regurgitation (III-IV) (22.7 vs. 57.5%; p = 0.01), wide QRS (160 ms) (9.5 vs. 41.0%, p = 0.01), and greater RV dilatation. No mortality was reported.Transannular patching and performance of previous PS were predictive factors of severe RV dilatation, and pulmonary regurgitation seems to be its physiological mechanism. Despite this, long-term prognosis is favorable and patients have good functional capacity.

Introdução: A correcção cirúrgica da Tetralogia de Fallot (TF) permitiu obter uma excelente sobrevivência. A longo prazo após cirurgia, a maioria dos doentes tem dilatação do ventrículo direito (VD) e ± 10% necessitam de re-operação do tracto de saída do VD, por baixa capacidade funcional, arritmias ventriculares ou sintomas de insuficiência cardíaca (IC). Procuramos analisar os preditores de eventos adversos: dilatação moderada ou severa (mod-sev) do VD, IC e re-operação e morte cardiovascular. Métodos: Oitenta e oito doentes adultos com TF efectuaram correcção cirúrgica entre 1977 e 2001; 22 foram perdidos para seguimento; 66 foram seguidos em média 18 ± 6 anos, com análise de variáveis clínicas, electrocardiográficas e ecocardiográficas. A dilatação do VD existia quando o diâmetro por via apical, ao nível do anel tricúspide, era superior a 35 mm, sendo de grau moderado quando 50 e < 60 mm e de grau severo quando 60 mm. Resultados: De 66 doentes, 25 (37,9 %) foram submetidos a shunt sistémico-pulmonar (SP) aos 4 ± 5 anos. A idade da correcção foi 10 ± 8 anos (< 1 até 38 anos, mediana: 6,5 anos); 65 % com patch transanular; 91 % com patch no VD; 53 % com comissurotomia pulmonar. Registou-se IC classe II-III em 3 doentes; apenas um foi re-operado com implantação de válvula pulmonar biológica com sucesso. A prevalência de dilatação do VD foi de 97 % (n = 57/59), sendo mod-sev em 69 % (n = 36/52). Nos doentes com dilatação mod-sev do VD foi mais frequente o shunt SP prévio (18,8 vs 50,0 %; p = 0,03), o uso de patch transanular (37,5 vs 75,0 %; p = 0,01) e existência de QRS alargado (> 160 ms) (6,7 vs 45,7 %, p < 0,01). Estes doentes referiram com maior frequência palpitações (0 vs 22,2 %; p = 0,05), sem maior número de eventos arrítmicos documentados (18,8 vs 33,3 %; p = 0,28). A frequência cardíaca máxima no esforço foi inferior (86,2 ± 10,9 vs 79,9 ± 8,6; p = 0,04), mas o tempo de prova e a capacidade funcional foram semelhantes entre os grupos. O tempo de seguimento e o uso de patch no VD também foi semelhante. A colocação de patch transanular esteve associada à realização de shunt SP numa idade mais tardia (0,9 ± 0,7 vs 4,9 ± 5,7 anos; p = 0,01). O seu uso originou um maior grau de insuficiência pulmonar (III-IV) (22,7 vs 57,5 %; p = 0,01), QRS muito alargados (> 160 ms) (9,5 vs 41,0 %, p < 0,01), para além de maior dilatação do VD. Não houve mortes. Conclusão: O patch transanular e a realização de um shunt SP prévio são preditores de dilatação grave do VD no adulto, parecendo ser a insuficiência pulmonar o seu mecanismo fisiopatológico. Apesar disso o prognóstico é bom e os doentes têm boa capacidade funcional a longo prazo. Tetralogy of Fallot: Prognostic Factors after Surgical Repair Introduction and Objective: Corrective surgery for tetralogy of Fallot (TF) has led to excellent survival. However, several years after surgery, the majority of patients have right ventricular (RV) dilatation, and 10% will need reoperation of the RV outflow tract due to limited exercise capacity, ventricular arrhythmias or symptoms of heart failure (HF). Our aim was to identify predictive factors of adverse outcome: moderate to severe RV dilatation, HF, reoperation of the RV outflow tract and cardiac death. Methods: Eighty-eight adult patients with TF were operated between January 1977 and July 2001; 22 were lost to follow-up and 66 were followed for 18 ± 6 years. We analyzed clinical, electrocardiographic and echocardiographic variables. RV dilatation was considered to exist if the inlet measurement at end-diastole in 4-chamber apical view was more than 35 mm, being classified as moderate when 50 and < 60 mm and severe when 60 mm. Results: Of the 66 patients, 25 (37.9 %) had undergone previous palliative shunt (PS) at the age of 4 ± 5 years. Mean age at surgical correction was 10 ± 8 years (range: < 1 to 38 years; median: 6.5 years). Transannular patching was used in 65 % of patients, patch closure of a right ventriculotomy in 91 %, and in 53 % of patients a pulmonary commissurotomy was performed. At the end of follow-up, 3 patients were in NYHA class III-IV and one patient was successfully reoperated with implantation of a biological pulmonary valve. Prevalence of RV dilatation was 97 % (57/59), being moderate to severe in 69 % (36/52). In patients with moderate to severe RV dilatation we found previous PS (18.8 vs. 50.0 %; p = 0.03), transannular patching (37.5 vs. 75.0 %; p0.01) and wide QRS (160 ms) (6.7 vs. 45.7 %, p = 0.01) to be more frequent. These patients reported more palpitations (0 vs. 22.2%; p0.05), but there were no differences in arrhythmic events (18.8 vs. 33.3 %; p = 0.28); maximal heart rate on exercise was lower (86.2 ± 10.9 vs. 79.9 ± 8.6; p = 0.04), but exercise time and functional capacity were similar between the groups. Follow-up time and use of RV patching were similar. Transannular patching was associated with previous PS at an older age (0.9 ± 0.7 vs. 4.9 ± 5.7 years; p = 0.01), a higher grade of pulmonary regurgitation (III-IV) (22.7 vs. 57.5 %; p = 0.01), wide QRS (160 ms) (9.5 vs. 41.0 %, p = 0.01), and greater RV dilatation. No mortality was reported. Conclusion: Transannular patching and performance of previous PS were predictive factors of severe RV dilatation, and pulmonary regurgitation seems to be its physiological mechanism. Despite this, longterm prognosis is favorable and patients have good functional capacity.

Cardiac transplantation is the gold standard therapy for patients below 60 years presenting with severe heart failure (HF) despite maximal medical therapy, who have no other surgical option and no contraindications to this procedure. We evaluated our experience with this important form of heart failure therapy. Between February 1987 and December 2002, 32 patients, aged 37 +/- 16 years, 19 males, with ejection fraction of 18 +/- 7%, underwent heart transplantation in our center. Seven (22%) patients were in NYHA class IV with hemodynamic support. Seventeen (53%) patients had idiopathic dilated cardiomyopathy (DCM), 7 (22%) had ischemic DCM, 3 (9%) had valvular DCM and the remainder had other causes of left ventricular dysfunction. Overall survival rate was 68% at first year post-transplantation, 59% at 5 years and 59% at 10 years. One year after cardiac transplantation, 95% of patients were in NYHA class I and the rest were in NYHA class II. Among the 13 patients who died, in five (18%) death occurred during the first month: the most frequent cause was hemodynamic failure. Causes of late death were: allograft vasculopathy (n = 3), allograft rejection (n = 1), infection (n = 1), sudden death (n = 1), hemodynamic failure (n = 1) and bradyarrhythmia (n = 1). Among the patients followed for more than one year, only three died. Early complications were: infection (8 episodes, 7 of respiratory location), right heart failure (3 patients), pericardial effusion (5 patients) and others (7 patients). Late complications were: a) allograft rejection: 17 (53%) patients, 72 episodes (10 ISHLT grade 3, 6 of whom were treated with intravenous corticotherapy, 8 grade 2 and 54 grade 1); b) infections: 19 (59%) patients; 35 episodes, 25 requiring hospitalization: 10 (28%) involving the respiratory tract, 6 (17%) the oropharynx, 5 (14%) the urinary tract, 4 (11%) the skin and 10 (28%) of undetermined location; c) chronic allograft rejection: 6 (19%) patients; d) arterial hypertension: 14 (45%) patients; d) renal failure: 5 (16%) patients; e) diabetes: 2 (6%) patients; f) cancer: 2 (6%) patients. Patients with severe heart failure and a very poor prognosis who underwent cardiac transplantation in our hospital showed marked improvement in functional capacity and quality of life and had an overall survival similar to the results of international heart transplantation registries. Complications during follow-up were similar to those usually described in the literature.