Early Outcomes of the Norwood Procedure in a Reference Center in Brazil. / Resultados Precoces do Procedimento de Norwood em um Centro de Referência no Brasil.

BACKGROUND: Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil. OBJECTIVES: We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality. METHODS: Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled. Demographic, anatomical, and surgical data were analyzed. Endpoints were early mortality (within the first 30 days after surgery), intermediate mortality (from early survival up to the Glenn procedure) and the need for postoperative ECMO support. Univariate and multivariate analyses were performed, and odds ratios, with 95% confidence intervals, were calculated. A p-value <0.05 was considered statistically significant. RESULTS: A total of 80 patients with HLHS underwent the stage I Norwood procedure. The 30-day survival rate was 91.3% and the intermediate survival rate 81.3%. Fourteen patients (17.5%) required ECMO support. Lower weight (p=0.033), aortic stenosis (vs aortic atresia; p=0.036), and the need for postoperative ECMO support (p=0.009) were independent predictive factors for 30-day mortality. Mitral valve stenosis (vs mitral valve atresia; p=0.041) was an independent predictive factor for intermediate mortality. CONCLUSION: The present study includes the largest Brazilian cohort of patients with HLHS undergoing the stage I Norwood procedure in the recent era. Our survival rates were comparable to the highest survival rates reported globally. Low body weight, aortic valve stenosis, and the need for postoperative ECMO support were independent predictors for 30-day mortality. Mitral valve stenosis was the only independent predictive factor for intermediate mortality.

FUNDAMENTO: Apenas dois artigos abordam os resultados precoces de pacientes com síndrome do coração esquerdo hipoplásico (SHCE) submetidos à operação de Norwood, no Brasil. OBJETIVOS: Avaliamos pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood para identificar os fatores preditivos de mortalidade precoce (nos primeiros 30 dias após a cirurgia) e intermediária (desde a sobrevida precoce até o procedimento de Glenn). MÉTODOS: Foram incluídos pacientes com SHCE submetidos em nosso serviço ao primeiro estágio da operação de Norwood de janeiro de 2016 a abril de 2019. Dados demográficos, anatômicos e cirúrgicos foram analisados. Os desfechos foram mortalidade precoce (nos primeiros 30 dias após a cirurgia), mortalidade intermediária (desde a sobrevida precoce até o procedimento de Glenn) e a necessidade de suporte pós-operatório com ECMO. Foram realizadas análises univariadas e multivariadas e calculados odds ratios, com intervalos de confiança de 95%. Um valor de p < 0,05 foi considerado estatisticamente significativo. RESULTADOS: Um total de 80 pacientes com SHCE foram submetidos ao primeiro estágio da operação de Norwood. A taxa de sobrevida em 30 dias foi de 91,3% e a taxa de sobrevida intermediária foi de 81,3%. Quatorze pacientes (17,5%) necessitaram de suporte com ECMO. Menor peso (p=0,033), estenose aórtica (vs atresia aórtica; p=0,036) e necessidade de suporte pós-operatório com ECMO (p=0,009) foram fatores preditivos independentes para mortalidade em 30 dias. A estenose da valva mitral ( vs atresia da valva mitral; p=0,041) foi um fator preditivo independente para mortalidade intermediária. CONCLUSÃO: O presente estudo inclui a maior coorte brasileira de pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood na era recente. Nossas taxas de sobrevida foram comparáveis às mais altas taxas de sobrevida relatadas globalmente. Baixo peso corporal, estenose valvar aórtica e necessidade de suporte pós-operatório com ECMO foram preditores independentes para mortalidade em 30 dias. A estenose da valva mitral foi o único fator preditivo independente para mortalidade intermediária.

Extracorporeal Membrane Oxygenation After Norwood Surgery in Patients With Hypoplastic Left Heart Syndrome: A Retrospective Single-Center Cohort Study From Brazil.

Background: Extracorporeal membrane oxygenation (ECMO) is increasingly being used to support patients after the repair of congenital heart disease. Objective: We report our experience with patients with a single functional ventricle who were supported by ECMO after the Norwood procedure, reviewing the outcomes and identifying risk factors for mortality in these patients. Methods: In this single-center retrospective cohort study, we enrolled 33 patients with hypoplastic left heart syndrome (HLHS) who received ECMO support after the Norwood procedure between January 2015 and December 2019. The independent variables evaluated in this study were demographic, anatomical, and those directly related to ECMO support (ECMO indication, local of initiation, time under support, and urinary output while on ECMO). The dependent variable was survival. A p < 0.05 was considered statistically significant. Results: The ECMO support was applied in 33 patients in a group of 120 patients submitted to Norwood procedure (28%). Aortic atresia was present in 72.7% of patients and mitral atresia in 51.5%. For 15% of patients, ECMO was initiated in the operating room; for all other patients, ECMO was initiated in the intensive care unit. The indications for ECMO in the cardiac intensive care unit were cardiac arrest in 22 (79%) of patients, low cardiac output state in 10 (18%), and arrhythmia in 1 patient (3%). The median time under support was 5 (2-25) days. The median follow-up time was 59 (4-150) days. Global survival to Norwood procedure was 90.9% during the 30-day follow-up, being 33.3% for those submitted to ECMO. Longer ECMO support (p = 0.004) was associated with a higher risk of death in the group submitted to ECMO. Conclusions: The mortality of patients with HLHS who received ECMO support after stage 1 palliation was high. Patients with low urine output were related to worse survival rates, and longer periods under ECMO support (more than 9 days of ECMO) were associated with 100% mortality. Earlier ECMO initiation before multiorgan damage may improve results.

Resultados Precoces do Procedimento de Norwood em um Centro de Referência no Brasil / Early Outcomes of the Norwood Procedure in a Reference Center in Brazil

Resumo Fundamento Apenas dois artigos abordam os resultados precoces de pacientes com síndrome do coração esquerdo hipoplásico (SHCE) submetidos à operação de Norwood, no Brasil. Objetivos Avaliamos pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood para identificar os fatores preditivos de mortalidade precoce (nos primeiros 30 dias após a cirurgia) e intermediária (desde a sobrevida precoce até o procedimento de Glenn). Métodos Foram incluídos pacientes com SHCE submetidos em nosso serviço ao primeiro estágio da operação de Norwood de janeiro de 2016 a abril de 2019. Dados demográficos, anatômicos e cirúrgicos foram analisados. Os desfechos foram mortalidade precoce (nos primeiros 30 dias após a cirurgia), mortalidade intermediária (desde a sobrevida precoce até o procedimento de Glenn) e a necessidade de suporte pós-operatório com ECMO. Foram realizadas análises univariadas e multivariadas e calculados odds ratios, com intervalos de confiança de 95%. Um valor de p < 0,05 foi considerado estatisticamente significativo. Resultados Um total de 80 pacientes com SHCE foram submetidos ao primeiro estágio da operação de Norwood. A taxa de sobrevida em 30 dias foi de 91,3% e a taxa de sobrevida intermediária foi de 81,3%. Quatorze pacientes (17,5%) necessitaram de suporte com ECMO. Menor peso (p=0,033), estenose aórtica (vs atresia aórtica; p=0,036) e necessidade de suporte pós-operatório com ECMO (p=0,009) foram fatores preditivos independentes para mortalidade em 30 dias. A estenose da valva mitral ( vs atresia da valva mitral; p=0,041) foi um fator preditivo independente para mortalidade intermediária. Conclusão O presente estudo inclui a maior coorte brasileira de pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood na era recente. Nossas taxas de sobrevida foram comparáveis às mais altas taxas de sobrevida relatadas globalmente. Baixo peso corporal, estenose valvar aórtica e necessidade de suporte pós-operatório com ECMO foram preditores independentes para mortalidade em 30 dias. A estenose da valva mitral foi o único fator preditivo independente para mortalidade intermediária.

Abstract Background Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil. Objectives We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality. Methods Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled. Demographic, anatomical, and surgical data were analyzed. Endpoints were early mortality (within the first 30 days after surgery), intermediate mortality (from early survival up to the Glenn procedure) and the need for postoperative ECMO support. Univariate and multivariate analyses were performed, and odds ratios, with 95% confidence intervals, were calculated. A p-value <0.05 was considered statistically significant. Results A total of 80 patients with HLHS underwent the stage I Norwood procedure. The 30-day survival rate was 91.3% and the intermediate survival rate 81.3%. Fourteen patients (17.5%) required ECMO support. Lower weight (p=0.033), aortic stenosis (vs aortic atresia; p=0.036), and the need for postoperative ECMO support (p=0.009) were independent predictive factors for 30-day mortality. Mitral valve stenosis (vs mitral valve atresia; p=0.041) was an independent predictive factor for intermediate mortality. Conclusion The present study includes the largest Brazilian cohort of patients with HLHS undergoing the stage I Norwood procedure in the recent era. Our survival rates were comparable to the highest survival rates reported globally. Low body weight, aortic valve stenosis, and the need for postoperative ECMO support were independent predictors for 30-day mortality. Mitral valve stenosis was the only independent predictive factor for intermediate mortality.

[Ebstein's anomaly: results of the conic reconstruction of the tricuspid valve]. / Anomalia de Ebstein: resultados com a reconstrução cônica da valva tricúspide.

OBJECTIVE: To assess the results of a technical modification of tricuspid valvuloplasty in Ebstein's anomaly. METHODS: From November 1993 to August 2002, 21 patients with Ebstein's anomaly of the tricuspid valve, with ages ranging from 20 months to 37 years (mean, 23 years), underwent a new technique of tricuspid valvuloplasty. This technique consisted of total or almost total detachment of the anterior tricuspid megaleaflet from the ventricular wall and valvular ring, transforming it into a cone, whose vertex remained fixed in the right ventricular tip, and the base was sutured to the tricuspid ring, after its plication, adjusting it to the size of the base of the cone (tricuspid ring), including the septal region. RESULTS: One (4.7%) patient with cardiomyopathy caused by chronic hypoxia died in the hospital due to low cardiac output. The mean follow-up lasted 4 years, and the recent echocardiograms showed good morphology of the right ventricle and tricuspid valve with mild or minimal insufficiency in 18 patients and moderate insufficiency in 2 patients. In 2 of the 3 patients with an anomalous bundle, it could be located and sectioned during surgery. No atrioventricular block occurred. CONCLUSION: The technique used was efficient in repairing tricuspid insufficiency and restoring right ventricular morphology, being applicable to all anatomic types of Ebstein's anomaly, except for Carpentier classification type D.

Horizontal right axillary minithoracotomy: aesthetic and effective option for atrial and ventricular septal defect repair in infants and toddlers.

INTRODUCTION: Congenital heart defects treatment shows progressive reduction in morbidity and mortality, however, the scar, resulting from ventricular (VSD) and atrial septal defect (ASD) repair, may cause discomfort. Right axillary minithoracotomy approach, by avoiding the breast growth region, is an option for correction of these defects that may provide better aesthetic results at low cost. Since October 2011, we have been using this technique for repairing VSD and ASD defects as well as associated defects. OBJECTIVES: To evaluate the efficacy of this method in children undergoing correction of VSD and ASD, to compare perioperative clinical outcomes with those repaired by median sternotomy, and to evaluate the aesthetic result. METHODS: Perioperative clinical data of 25 patients submitted to axillary thoracotomy were compared with data from a paired group of 25 patients with similar heart defects repaired by median sternotomy, from October 2011 to August 2012. RESULTS: Axillary approach was possible even in infants. There was no mortality and the main perioperative variables were similar in both groups, except for lower use of blood products in the axillary group (6/25) vs. control (13/25), with statistical difference (P =0.04). The VSD size varied from 7 to 15 mm in axillary group. Cannulation of the aorta and vena cavae was performed through the main incision, whose size ranged from 3 to 5 cm in the axillary group, with excellent aesthetic results. CONCLUSION: The axillary thoracotomy was effective, allowing for a heart defect repair similar to the median sternotomy, with more satisfactory aesthetic results and reduced blood transfusion, and it can be safely used in infants.

Lack of tight association between quality of life and exercise capacity in pulmonary arterial hypertension.

BACKGROUND: In pulmonary arterial hypertension (PAH) health-related quality of life (HRQOL) has been investigated over the short-term (weeks) but little is known about patient's perspective over the medium and long term. OBJECTIVE: To analyze how patients on specific PAH therapies do over one year of observation in terms of HRQOL, and to investigate if possible associations between the exercise capacity (EC) and HRQOL persist over the medium term. METHODS: Thirty-four patients on PAH therapies (bosentan and/or sildenafil) were enrolled (age 14 to 58 years, median 35.5 years, functional class II or III), and evaluated at baseline, and 3, 6, 9 and 12 months subsequently using the six-minute walk test and the SF-36 HRQOL questionnaire. RESULTS: The six minute walked distance did not change over the follow-up (387-432 meters, median values, p=0.2775), the same for the functional class and peripheral oxygen saturation. The SF-36 scores also remained stable, with physical health always worse than mental health. Of 40 possible associations between EC and HRQOL, only 12 were significant (30%, p<0.05). Prediction of severely depressed HRQOL based on a walked distance of <235 meters was >90% specific but <43% sensitive. CONCLUSION: Patients with PAH who remain stable in terms of EC also seem to do so in terms of HRQOL. However, EC and HRQOL are not consistently tied over time, and should be analyzed as different perspectives in the individual patient.

Pulmonary root translocation in malposition of great arteries repair allows right ventricular outflow tract growth.

OBJECTIVE: Optimal surgical treatment of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) remains a matter of debate. This study evaluated the clinical outcome and right ventricle outflow tract performance in the long-term follow-up of patients subjected to pulmonary root translocation (PRT) as part of their surgical repair. METHODS: From April 1994 to December 2010, we operated on 44 consecutive patients (median age, 11 months). All had malposition of the great arteries as follows: TGA with VSD and PS (n = 33); double-outlet right ventricle with subpulmonary VSD (n = 7); double-outlet right ventricle with atrioventricular septal defect (n = 1); and congenitally corrected TGA with VSD and PS (n = 3). The surgical technique consisted of PRT from the left ventricle to the right ventricle after construction of an intraventricular tunnel that diverted blood flow from the left ventricle to the aorta. RESULTS: The mean follow-up time was 72 ± 52.1 months. There were 3 (6.8%) early deaths and 1 (2.3%) late death. Kaplan-Meier survival was 92.8% and reintervention-free survival was 82.9% at 12 years. Repeat echocardiographic data showed nonlinear growth of the pulmonary root and good performance of the valve at 10 years. Only 4 patients required reinterventions owing to right ventricular outflow tract problems. CONCLUSIONS: PRT is a good surgical alternative for treatment of patients with TGA complexes, VSD, and PS, with acceptable operative risk, high long-term survivals, and few reinterventions. Most patients had adequate pulmonary root growth and performance.

Occlusive venopathy phenotype in hereditary pulmonary arterial hypertension.

A 33-year-old male with severe hereditary pulmonary arterial hypertension had a confirmed diagnosis of occlusive venopathy and microvasculopathy. He remained stable for three and a half years on oral sildenafil, 75 mg t.i.d. (six-minute walked distance of 375 m vs 105 m at baseline), but required addition of bosentan (125 mg b.i.d.), subsequently. Despite the fatal outcome at five years post-diagnosis, the observations suggest a potential usefulness of vasodilators as a bridge for lung transplant in selected cases with significant venous/capillary involvement. The occurrence of veno-occlusive and capillary lesions in the familial form of pulmonary arterial hypertension reinforces the difficulties with the current classification of the disease.

Cone reconstruction in Ebstein's anomaly repair: early and long-term results.

BACKGROUND: The main Ebstein anomaly (EA) repairs are based on the monocusp reconstruction of the tricuspid valve and are limited by the frequent need for replacement or the high recurrence of valve regurgitation. OBJECTIVE: To evaluate the feasibility and effects of anatomical repair of Ebstein's anomaly using the cone reconstruction technique on patients' clinical evaluation, tricuspid valve function and right ventricular morphology. METHODS: We compared the clinical, echocardiographic and radiological data of 52 consecutive patients, with a mean age of 18.5 ± 13.8 years, submitted to the cone reconstruction technique, obtained in the preoperative, early postoperative (EPO) and long-term (LPO) periods. RESULTS: There were two in-hospital deaths (3.8%) and two more during the follow-up. Mean functional class of pre-operative heart failure improved from 2.2 to 1.2 after 57 months of mean follow up of 97% of patients (p <0.001). The mean degree of preoperative tricuspid regurgitation decreased from 3.6 to 1.6 in the EPO (p <0.001), remaining at 1.9 in LPO period (p> 0.05). The indexed RV functional area increased from 8.53 ± 7.02 cm²/m² preoperatively to 21.01 ± 6.87 cm²/m² in the EPO (p <0.001) and remained unchanged at 20.28 ± 5.26 cm²/m² in LPO period (p> 0.05). The mean cardiothoracic ratio was decreased from 0.66 ± 0.09 to 0.54 ± 0.06 (p <0.001) in the long term. CONCLUSION: The cone technique showed low in-hospital mortality, resulting in an effective and long-lasting repair of tricuspid regurgitation, restoring the functional area of the right ventricle and allowing reverse remodeling of the heart and clinical improvement in most patients in the long term.

The hypoplastic left heart syndrome is not a risk factor for Fontan operation.

OBJECTIVE: To show the mortality rate of children undergoing to Fontan operation and determine whether the hypoplastic left heart syndrome (HLHS) is a risk factor for hospital mortality. METHODS: From August 2001 to June 2008, 76 patients underwent Fontan operation and were divided into two groups: group A with 54 patients, 31 (40.7%) patients with tricuspid atresia and variants, six (7.8%) of double-inlet left ventricle, four (5.3%) and atrioventricular septal defect and total 13 (17.1%) of other complex congenital heart disease and group B all patients with HLHS a total of 22 (28.9%) patients. RESULTS: Group A patients had a mean age of 6.47 years ± 4.83 and group B of 2.08 years ± 0.24 P <0.001, the average weight was 22.42 ± 11.04 against 12.99 ± 1.2 P = 0.016, the mean CPB time was 119.5 min versus 113.3 min P = 0.0, with a mean clamping time of 74.8 min and 73.5 min p = 0.75. The mean ICU stay was 4.1 days for group A versus 7.52 days for group B p = 0.0003. In total (group A + B) three patients died, with hospital mortality of 3.9%, and one patient with HLHS (4.54%) (P <0.001, 95% CI 0.001 to 0.228). CONCLUSION: Our study shows that despite higher morbidity in HLHS is not a risk factor for hospital mortality.

[Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil]. / Seguimento de dois anos em pacientes com hipertensão arterial pulmonar sob tratamento com sildenafila.

BACKGROUND: The long-term effects of drugs developed for the control of pulmonary arterial hypertension (PAH) are little known, since multicenter studies usually last 12 to 16 weeks. OBJECTIVE: To evaluate the two-year outcome of PAH patients receiving monotherapy with sildenafil (a phosphodiesterase-5 inhibitor), with regard to their functional capacity. METHODS: Twenty four patients (ages between 8 and 54 years) with idiopathic PAH (IPAH, n = 9) or congenital heart disease-associated PAH (CHD-PAH, n = 15) were treated with sildenafil for two years, with daily oral doses ranging from 60 to 225 mg (tid). Physical capacity was assessed by the distance walked in the 6-minute walk test (DW6M) and by the degree of dyspnea at the end of the walk (Borg scale); peripheral oxygen saturation was also recorded (SpO(2)6M, pulse oximetry). RESULTS: In the 18 patients who completed the two-year follow-up, there was a progressive and sustained increase in DW6M, both in the IPAH group (from 239 +/- 160 m to 471 +/- 66 m, p = 0.0076) and in the CHD-PAH group (from 361 +/- 144 m to 445 +/- 96 m, p = 0.0031), with improvement of dyspnea at the end of the walk (p<0.05 for both groups). No decrease in SpO(2)6M was observed in the groups; in patients with CHD-PAH, in particular, SpO(2)6M went from 77 +/- 20% to 79 +/- 16% (p = 0.5248). Five deaths occurred (three in the IPAH group) and one patient was lost to follow-up during the study period. CONCLUSION: In a two-year follow-up, sildenafil proved useful in the control of the functional status of PAH patients, with significant improvement in both groups considered.

Horizontal right axillary minithoracotomy: aesthetic and effective option for atrial and ventricular septal defect repair in infants and toddlers / Minitoracotomia axilar direita horizontal: opção estética e eficaz para correção de comunicação interventricular e interatrial em lactentes e crianças

Introduction: Congenital heart defects treatment shows progressive reduction in morbidity and mortality, however, the scar, resulting from ventricular (VSD) and atrial septal defect (ASD) repair, may cause discomfort. Right axillary minithoracotomy approach, by avoiding the breast growth region, is an option for correction of these defects that may provide better aesthetic results at low cost. Since October 2011, we have been using this technique for repairing VSD and ASD defects as well as associated defects. Objectives: To evaluate the efficacy of this method in children undergoing correction of VSD and ASD, to compare perioperative clinical outcomes with those repaired by median sternotomy, and to evaluate the aesthetic result. Methods: Perioperative clinical data of 25 patients submitted to axillary thoracotomy were compared with data from a paired group of 25 patients with similar heart defects repaired by median sternotomy, from October 2011 to August 2012. Results: Axillary approach was possible even in infants. There was no mortality and the main perioperative variables were similar in both groups, except for lower use of blood products in the axillary group (6/25) vs. control (13/25), with statistical difference (P =0.04). The VSD size varied from 7 to 15 mm in axillary group. Cannulation of the aorta and vena cavae was performed through the main incision, whose size ranged from 3 to 5 cm in the axillary group, with excellent aesthetic results. Conclusion: The axillary thoracotomy was effective, allowing for a heart defect repair similar to the median sternotomy, with more satisfactory aesthetic results and reduced blood transfusion, and it can be safely used in infants. .

Introdução: O tratamento dos defeitos cardíacos apresenta progressiva redução da morbimortalidade, porém, a cicatriz, sequela aparente do tratamento da comunicação interatrial (CIA) e interventricular (CIV), pode causar incômodo. A abordagem por minitoracotomia axilar é opção para correção destes defeitos, com possível melhor estética e baixo custo, além de evitar a região de crescimento da mama. Desde outubro de 2011, empregamos esta técnica para correção de CIA, CIV e defeitos associados. Objetivo: Avaliar a eficácia do método em crianças submetidas à correção de CIV e CIA, avaliar a evolução clínica perioperatória em comparação à esternotomia mediana e avaliar o resultado estético. Métodos: Dados clínicos perioperatórios de 25 pacientes submetidos à minitoracotomia axilar foram comparados com dados de grupo de 25 pacientes submetidos à correção de defeitos cardíacos semelhantes por esternotomia mediana, de outubro de 2011 a agosto de 2012. Resultados: A correção dos defeitos via axilar foi factível inclusive em lactentes. Não houve mortalidade e as principais variáveis perioperatórias foram similares em ambos os grupos, exceto pelo menor uso de hemoderivados no grupo axilar (6/25) x controle (13/25), com diferença estatística (P=0,04). A sequela ventricular nos pacientes do grupo axilar variou de 7 a 15 mm. A canulação da aorta e veias cavas foi realizada através da incisão principal, cujo tamanho variou de 3 a 5 cm no grupo axilar, com resultado estético excelente. Conclusão: A minitoracotomia axilar foi eficaz, permitiu correção do defeito cardíaco semelhante à esternotomia mediana, com resultado estético mais ...

Ausência de estreita associação entre qualidade de vida e capacidade de exercício na hipertensão arterial pulmonar / Lack of tight association between quality of life and exercise capacity in pulmonary arterial hypertension

FUNDAMENTO: Na hipertensão arterial pulmonar (HAP) a qualidade de vida relacionada saúde (QVRS) tem sido investigada em curtos períodos de tempo (semanas), mas pouco se sabe sobre a perspectiva do paciente no médio e longo prazo. OBJETIVO: Analisar o estado de pacientes em terapias específicas de HAP durante um ano de observação, em termos de QVRS, e investigar se possíveis associações entre a capacidade de exercício (CE) e a QVRS persistem no médio prazo. MÉTODOS: Trinta e quatro pacientes em terapias para a HAP (bosentan e/ou sildenafil) foram selecionados (idade de 14 a 58 anos, mediana de 35,5 anos, classe funcional II ou III), e avaliados no momento basal, e 3, 6, 9 e 12 meses depois, usando o teste de caminhada de 6 minutos e questionário SF-36 de QVRS. RESULTADOS: A distância percorrida nos seis minutos não mudou durante o acompanhamento (387 - 432 metros, valores da mediana, p=0.2775), o mesmo para a classe funcional e saturação periférica de oxigênio. Os escores SF-36 também se mantiveram estáveis, com a saúde física sempre pior que a saúde mental. Das 40 possíveis associações entre a CE e QVRS, apenas 12 foram significativas (30%, p<0,05). A previsão de uma QVRS severamente deprimida com base em uma distância percorrida de 235 metros foi específica em >90%, mas sensível em <43%. CONCLUSÃO: Os pacientes com HAP que se mantêm estáveis em termos da CE também parecem fazê-lo em termos de QVRS. Contudo, CE e QVRS não têm ligação consistente com o tempo, e devem ser analisadas como diferentes perspectivas no paciente individual.

BACKGROUND: In pulmonary arterial hypertension (PAH) health-related quality of life (HRQOL) has been investigated over the short-term (weeks) but little is known about patient's perspective over the medium and long term. OBJECTIVE: To analyze how patients on specific PAH therapies do over one year of observation in terms of HRQOL, and to investigate if possible associations between the exercise capacity (EC) and HRQOL persist over the medium term. METHODS: Thirty-four patients on PAH therapies (bosentan and/or sildenafil) were enrolled (age 14 to 58 years, median 35.5 years, functional class II or III), and evaluated at baseline, and 3, 6, 9 and 12 months subsequently using the six-minute walk test and the SF-36 HRQOL questionnaire. RESULTS: The six minute walked distance did not change over the follow-up (387-432 meters, median values, p=0.2775), the same for the functional class and peripheral oxygen saturation. The SF-36 scores also remained stable, with physical health always worse than mental health. Of 40 possible associations between EC and HRQOL, only 12 were significant (30%, p<0.05). Prediction of severely depressed HRQOL based on a walked distance of <235 meters was >90% specific but <43% sensitive. CONCLUSION: Patients with PAH who remain stable in terms of EC also seem to do so in terms of HRQOL. However, EC and HRQOL are not consistently tied over time, and should be analyzed as different perspectives in the individual patient.

Hypoplastic left heart syndrome: the report of a surgical strategy and comparative results of Norwood x Norwood-Sano approach.

OBJECTIVES: To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch. To compare the results of the modified Blalock-Taussig (mBT) shunt and the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow. METHOD: Retrospective study of 78 newborns consecutively operated between March, 1999 and June 2006. One technique for reconstruction of the neoaorta and two different techniques for reestablishment of the pulmonary blood flow: the mBT shunt in the first 37 newborns and RV-PA conduit in the last 41. Cannulation of the ductus arteriosus for systemic perfusion was the main part of the surgical strategy to reduce the hypothermic circulatory arrest time. RESULTS: In-hospital survival for the entire cohort was 74,35%, or 67.57% for the mBT shunt and 80,49% for RV-PA conduit groups (p=0,21). Hypothermic circulatory arrest times were 45.79+/-1.99 min and 36,8+/-1,52 min (p=0,0012), respectively. Mortality rates between first and second stages were 40% for the mBT shunt and 6,9% for RV-PA conduit groups (p=0,007). Late coarctation of the aorta occurred in five patients Actuarial survival curves(Kaplan-Meier) comparison showed better results with VD-AP conduit (p=0,003). CONCLUSIONS: This surgical strategy resulted in short circulatory arrest time, low mortality and low incidence of aortic coarctation. Although the higher rate of survival to first palliation stage with the RV-PA conduit was not significant, the lower interstage mortality and superior medium-term survival in RV-AP group were statistically advantageous.

The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results.

OBJECTIVES: We sought to describe a new technique for tricuspid valve repair in Ebstein's anomaly and to report early echocardiographic results, as well as early and midterm clinical outcomes. METHODS: From November 1993 through August 2005, 40 consecutive patients with Ebstein's anomaly (mean age, 16.8 +/- 12.3 years) underwent a new surgical repair modified from Carpentier's procedure, the principal details of which are as follows. The anterior and posterior tricuspid valve leaflets are mobilized from their anomalous attachments in the right ventricle, and the free edge of this complex is rotated clockwise to be sutured to the septal border of the anterior leaflet, thus creating a cone the vertex of which remains fixed at the right ventricular apex and the base of which is sutured to the true tricuspid valve annulus level. Additionally, the septal leaflet is incorporated into the cone wall whenever possible, and the atrial septal defect is closed in a valved fashion. RESULTS: There was 1 (2.5%) hospital death and 1 late death. Early postoperative echocardiograms have shown good right ventricular morphology and reduction in tricuspid regurgitation grade from 3.6 +/- 0.5 to 1.2 +/- 0.5 (P < .0001). After mean follow-up of 4 years, the functional class (New York Heart Association) improved from 2.6 +/- 0.7 to 1.2 +/- 0.4 (P < .0001). Two patients required late tricuspid valve re-repair, and there was neither atrioventricular block nor tricuspid valve replacement at any time. CONCLUSIONS: This surgical technique for Ebstein's anomaly can be performed with low mortality and morbidity. Early echocardiograms showed significant reduction of tricuspid insufficiency, and the follow-up showed improvement in patients' clinical status and low incidence of reoperation.

Hypoplastic left heart syndrome: the influence of surgical strategy on outcomes.

OBJECTIVES: To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch, and to compare the results of the modified Blalock-Taussig (mBT) shunt with the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow. METHODS: Retrospective study of 71 newborns with HLHS consecutively operated between March, 1999 and February, 2006. One technique for reconstruction of the neoaorta and two different techniques for reestablishment of the pulmonary blood flow were used: the mBT shunt in the first 37 newborns and RV-PA conduit in the last 34. Cannulation of the ductus arteriosus for arterial perfusion was the main part of the surgical strategy to reduce the hypothermic circulatory arrest time. RESULTS: In-hospital survival for the entire cohort was 74.64%, or 67.57% and 82.35% for the mBT shunt and RV-PA conduit groups, respectively (p=0.1808). Mortality rates between the first and second palliation stages were 40% and 4.4% for the mBT shunt and RV-PA conduit groups, respectively (p=0.0054). Hypothermic circulatory arrest times were 45.79+/-1.99 min and 36.62+/-1.62 min (p=0.0012), respectively. Late coarctation of the aorta occurred in five patients (7.2%). CONCLUSION: This surgical strategy resulted in short circulatory arrest time, low mortality and favorable morphology of the neoaorta, with low incidence of late coarctation of the aorta. The higher rate of survival to first palliation stage with the RV-PA conduit was not significant, but interstage mortality was statistically lower when compared with the modified Blalock-Taussig shunt procedure.

Hipertensão arterial pulmonar hereditária apresentando-se como venopatia oclusiva / Occlusive venopathy phenotype in hereditary pulmonary arterial hypertension / Hipertensión arterial pulmonar hereditaria presentándose como venopatía oclusiva

Um homem de 33 anos com hipertensão arterial pulmonar hereditária teve um diagnóstico confirmado de venopatia oclusiva e microvasculopatia. O paciente permaneceu estável por 3 anos e meio recebendo sildenafila via oral, 75 mg 3x/dia (teste de caminhada de seis minutos de 375 m vs 105 m basal), mas necessitou da adição de bosentana (125 mg 2x/dia) posteriormente. A despeito do desfecho fatal após 5 anos, as observações sugerem um utilidade potencial dos vasodilatadores como uma ponte para o transplante de pulmão em casos selecionados com envolvimento venocapilar significante. A ocorrência de lesões veno-oclusivas e capilares na forma familiar da hipertensão arterial pulmonar enfatiza as dificuldades com a atual classificação da doença.

A 33-year-old male with severe hereditary pulmonary arterial hypertension had a confirmed diagnosis of occlusive venopathy and microvasculopathy. He remained stable for three and a half years on oral sildenafil, 75 mg t.i.d. (six-minute walked distance of 375 m vs 105 m at baseline), but required addition of bosentan (125 mg b.i.d.), subsequently. Despite the fatal outcome at five years post-diagnosis, the observations suggest a potential usefulness of vasodilators as a bridge for lung transplant in selected cases with significant venous/capillary involvement. The occurrence of veno-occlusive and capillary lesions in the familial form of pulmonary arterial hypertension reinforces the difficulties with the current classification of the disease.

Un hombre de 33 años con hipertensión arterial pulmonar hereditaria tuvo un diagnóstico confirmado de venopatía oclusiva y microvasculopatía. El paciente permaneció estable 3,5 años recibiendo sildenafila vía oral, 75mg 3x/ día (test de caminata de seis minutos de 375m vs. 105m basal), pero necesitó adición de bosentánana (125mg 2x/día) posteriormente. A despecho del desenlace fatal después de 5 años, las observaciones sugieren una utilidad potencial de los vasodilatadores como un puente para el transplante de pulmón en casos seleccionados con compromiso venocapilar significativo. La ocurrencia de lesiones veno-oclusivas y capilares en la forma familiar de la hipertensión arterial pulmonar enfatiza las dificultades con la actual clasificación de la enfermedad.