Sonia Meiken Franchi

One-year follow-up of the effects of sildenafil on pulmonary arterial hypertension and veno-occlusive disease

We hypothesized that chronic oral administration of the phosphodiesterase-5 inhibitor sildenafil could improve the exercise capacity and pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH) on the basis of previous short-term studies. We tested this hypothesis in 14 subjects with PAH, including seven patients with the idiopathic form and seven patients with atrial septal defects, but no other congenital heart abnormalities. Patients were subjected to a 6-min walk test and dyspnea was graded according to the Borg scale. Pulmonary flow and pressures were measured by Doppler echocardiography. Patients were given sildenafil, 75 mg orally three times a day, and followed up for 1 year. Sildenafil therapy resulted in the following changes: increase in the 6-min walk distance from a median value of 387 m (range 0 to 484 m) to 462 m (range 408 to 588 m; P < 0.01), improvement of the Borg dyspnea score from 4.0 (median value) to 3.0 (P < 0.01), and increased pulmonary flow (velocity-time integral) from a median value of 0.12 (range 0.08 to 0.25) to 0.23 (range 0.11 to 0.40; P < 0.01) with no changes in pulmonary pressures. In one patient with pulmonary veno-occlusive disease diagnosed by a lung biopsy, sildenafil had a better effect on the pulmonary wedge pressure than inhaled nitric oxide (15 and 29 mmHg, respectively, acute test). He walked 112 m at baseline and 408 m at one year. One patient died at 11 months of treatment. No other relevant events occurred. Thus, chronic administration of sildenafil improves the physical capacity of PAH patients and may be beneficial in selected cases of veno-occlusive disease.

Anomalia de Ebstein: resultados com a reconstrução cônica da valva tricúspide

OBJECTIVE: To assess the results of a technical modification of tricuspid valvuloplasty in Ebsteins anomaly. METHODS: From November 1993 to August 2002, 21 patients with Ebsteins 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 Ebsteins anomaly, except for Carpentier classification type D.

Síndrome do coração esquerdo hipoplásico: estratégia cirúrgica e comparação de resultados com técnicas de Norwood x Sano

Objectives: To report a surgical strategy for the Norwood procedure in hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest and aortic arch reconstruction using autologous pericardium. Additionally, the work compares the results of the modified BlalockTaussig (mBT) shunt and the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow. Method: A retrospective study was performed of 78 newborns consecutively operated on between March 1999 and June 2006. One technique to reconstruct the neoaorta and two different techniques, to reestablish the pulmonary blood flow, were utilized - mBT shunts in the first 37 newborns and RV-PA conduits in the last 41. Cannulation of the ductus arteriosus for systemic perfusion was the main part of the surgical strategy used 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 the RVPA conduit Groups (p=0.21). Hypothermic circulatory arrest times were 45.79 ± 1.99 minutes and 36.8 ± 1.52 minutes (p=0.0012), respectively. Mortality rates between first and second stages were 40% for the mBT shunt and 6.9% for the RV-PA conduit Groups (p=0.007). Late coarctation of the aorta occurred in five patients. A comparison of the actuarial survival curves (Kaplan-Meier) showed that the results were better with the VD-AP conduit (p=0.003). Conclusions: This surgical strategy resulted in a short circulatory arrest time, low mortality and a low incidence of aortic coarctation. Although the higher rate of survival in the first palliation stage with the RV-PA conduit was not significant, the lower interstage mortality and superior medium-term survival in the RV-AP Group were statistically advantageous.

Síndrome da hipoplasia do coração esquerdo: a influência da estratégia cirúrgica nos resultados

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.

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.

Operação de Glenn bidirecional no tratamento estagiado da síndrome de hipoplasia do coração esquerdo: resultados imediatos e tardios

OBJETIVO: Relatar os resultados imediatos e tardios da operacao de Glenn bidirecional como segundo estagio do tratamento da Sindrome de Hipoplasia do Coracao Esquerdo (SHCE) e descrever a tecnica de miniesternotomia utilizada. METODO: Entre marco de 1998 e fevereiro de 2004, 15 pacientes com operacao de Norwood previa foram submetidos eletivamente a derivacao cavopulmonar. As idades variaram de 2 a 6 meses (media 3,46 ± 0,83 meses), sendo seis pacientes do sexo masculino. Foram realizadas miniesternotomias em 11 (73,3%) casos. Para adequada oxigenacao sanguinea inicial foi associado enxerto sistemico-pulmonar de 3 mm em nove casos e manutencao do enxerto VD-TP em um caso. Acompanhamento clinico e ecocardiografico foi realizado em todos os pacientes. RESULTADOS: A sobrevida hospitalar foi de 86,6%, ocorrendo um obito por sangramento e outro por hipoxia. O ecocardiograma imediato mostrava fluxo pelo enxerto de PTFE nos dez pacientes em que foi utilizado, ocorrendo seu fechamento no controle tardio. Ocorreram dois (13,3%) obitos tardios, um por complicacao de traqueostomia e outro por meningite bacteriana. Sete pacientes aguardam o terceiro estagio, estando assintomaticos. Quatro foram submetidos ao terceiro estagio com sucesso. O ecocardiograma dos 11 pacientes sobreviventes tardios mostra boa funcao do ventriculo direito, sem insuficiencia tricuspide e bom fluxo pela anastomose cavo-pulmonar, num seguimento medio de 2 anos e 5 meses. CONCLUSOES: A operacao de Glenn na SHCE apresenta baixa mortalidade hospitalar, com resultados satisfatorios em longo prazo, podendo ser realizada atraves de miniesternotomia A associacao de fluxo sistemico-pulmonar acessorio em criancas de baixa idade parece melhorar a saturacao de oxigenio.

Ausência de estreita associação entre qualidade de vida e capacidade de exercício na hipertensão arterial pulmonar

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 patients 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.

A síndrome do coração esquerdo hipoplásico não constitui fator de risco para operação de Fontan

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.

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.

Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil

Resumen Fundamento: Los efectos a largo plazo de las drogas desarrolladas para el control de la hipertension arterial pulmonar (HAP) son poco conocidos, ya que los estudios multicentricos en general tienen una duracion de 12 a 16 semanas.Objetivo: Evaluar la evolucion a dos anos, en pacientes con HAP sometidos a monoterapia con sildenafil (inhibidor de la fosfodiesterasa-5), con respecto a la capacidad funcional.Metodos: Veinticuatro pacientes (edades entre 8 y 54 anos) con HAP idiopatica (HAPI, n = 9) o asociada a cardiopatias congenitas (HAP-CCg, n = 15) fueron tratados con sildenafil durante dos anos, con dosis diarias que variaron de 60 a 225 mg (tres tomas), por via oral. La capacidad fisica fue evaluada por la distancia caminada en el test de 6 minutos (DC6M) y por el grado de disnea al final de la caminata (escala de Borg), siendo tambien registrada la saturacion periferica de oxigeno (SpO26M, oximetria de pulso).Resultados: En los 18 pacientes que completaron dos anos de seguimiento, hubo un incremento progresivo y sostenido en la DC6M, tanto en el grupo HAPI (de 239 ± 160 m a 471 ± 66 m, p = 0,0076) como en el grupo HAP-CCg (de 361 ± 144 m a 445 ± 96m, p = 0,0031), con mejora de la disnea al final de la caminata (p < 0,05 en ambos). No hubo disminucion de la SpO26M en los grupos considerados; en particular, pacientes con HAP-CCg evolucionaron de 77 ± 20% a 79 ± 16% (p = 0,5248). Hubo 5 obitos (tres en el grupo HAPI) y una perdida de seguimiento en el periodo.Conclusion: En dos anos de seguimiento, el sildenafil demostro ser util en el control de la condicion funcional de pacientes con HAP, con mejora significativa en las dos etiologias consideradas. (Arq Bras Cardiol 2010;94(5):653-659)Palabras clave: Inhibidores de fosfodiesterasa/analisis, hipertension, hipertension pulmonar, capacidad vital, vasodilatadores.mas alla de 12-16 semanas en la mayoria de las vecesBACKGROUND 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.