Abnormal Origin of the Pulmonary Artery from the Ascending Aorta in the Neonate

ABSTRACT The anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital heart disease, generally diagnosed based on the clinical information and on echocardiographic and computed tomography angiography findings. Here we report two neonates successfully treated with surgery early in life.

Abnormal Origin of the Pulmonary Artery from the Ascending Aorta in the Neonate.

The anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital heart disease, generally diagnosed based on the clinical information and on echocardiographic and computed tomography angiography findings. Here we report two neonates successfully treated with surgery early in life.

Impact of pulmonary artery reduction during arterial switch operation: 14 years follow-up.

OBJECTIVES: Pulmonary artery/aorta (PA/Ao) size discrepancy plays an important role in the development of neoaortic root growth and valve regurgitation. Since 2004, we started using PA reduction to manage severe great vessels root mismatch at the time of arterial switch operation. The purpose of this study is to evaluate the impact of this technique in the mid- and long-term follow-up. METHODS: Patients considered to have severe PA/Ao mismatch (>2:1 ratio) underwent resection of a 3- to 4-mm flap of the posterior PA wall. Patients submitted to this technique were followed up with clinical and image examinations. Echocardiographic findings were reviewed, and Z-scores were recorded to evaluate the incidence and progression of neoaortic root dilatation and valve regurgitation. RESULTS: The median (Q1-Q3) follow-up time was 8 years (3-11). Before arterial switch operation, the median (Q1-Q3) Z-score of the PA annulus was 2.90 (2.75-3.75). At the latest follow-up, the median Z-score of the neoaortic annulus was 1.34 (0.95-1.66). The mean difference between the Z-scores of PA annulus and neoaortic annulus was 1.56 (P < 0.0001). The mean value of the sinus of Valsalva was +0.29 ± 1, that of sinotubular junction was +0.71 ± 0.6 and that of ascending Ao was +1.09 ± 0.7. There was no severe dilatation of the neoaortic annulus, neoaortic root or ascending Ao during follow-up. Neoaortic valve regurgitation was none or mild in 93% of patients. CONCLUSIONS: PA reduction proved to be a feasible and low-risk procedure to approach PA/Ao mismatch in arterial switch operation. Mid- and long-term follow-up showed a tendency towards stabilization of the neoaortic root dilatation and satisfactory valve performance after the procedure. Further investigation is required with a larger population and longer-term follow-up.

Operação de Fontan: uma técnica em evolução / Fontan operation: a technique in evolution

Objetivo: Estudos recentes de fluxo com modelos experimentais de anastomoses cavopulmonares totais (ACPTs) baseados em ressonância magnética e angiografia demonstram que este é um procedimento bem estabelecido para o tratamento de várias cardiopatias, mas o melhor arranjo espacial continua controverso. Nosso intuito é apresentar os resultados imediatos com três diferentes técnicas de ACPTs. Métodos: Ensaio clínico de ACPTs realizadas no período de janeiro de 2005 a julho de 2008 com 40 pacientes, com idade média de 6,4 ± 3,2 anos, com Glenn prévio. Os pacientes foram divididos em três grupos, dependendo da técnica cirúrgica empregada: Grupo 1 (G1) - túnel lateral; Grupo 2 (G2) - conduto extracardíaco; Grupo 3 (G3) - conduto intracardíaco dirigido para o ramo esquerdo de artéria pulmonar, todos com fenestração. Foram avaliadas variáveis pré e pós-operatórias. Resultados: Foram incluídos 11 pacientes no G1, 10 no G2 e 19 no G3. As variáveis pré-operatórias foram semelhantes nos três grupos (P>0,05). A mortalidade foi maior nos Grupos 1 e 2 (9,1 por cento e 10,0 por cento, respectivamente), comparadas ao Grupo 3 (zero), porém sem significância estatística (P=0,3841). Efusão pleural foi ausente no Grupo 3, diferença significativa (P=0,0128) em relação aos outros grupos (40,0 por cento e 33,3 por cento). A mediana do tempo de hospitalização pós-operatória foi menor no Grupo 3 (8 dias), em relação aos grupos 1 e 2 (18 e 13 dias, respectivamente) (P=0,0164). Conclusão: A técnica de conduto intracardíaco foi associada a menor morbidade pós-operatória, sendo a opção atual do nosso serviço na anastomose cavopulmonar total.

Objectives: Recent experimental flow studies based on angiography and magnetic resonance have shown that total cavopulmonary anastomosis (TCPA) is a valid concept for surgical treatment of many congenital heart defects, but there is not agreement of the best surgical arrangement. Our purpose is to analyze the immediate results with three different techniques of TCPA. Methods: We sought to analyze retrospectively all TCPA realized from January 2005 to July 2008; there were 40 patients, all with previous Glenn anastomosis, with mean age of 6.4 ± 3.2 years. Three different techniques were employed: Group 1 (G1) lateral tunnel, Group 2 (G2) extracardiac conduits, Group 3 (G3) intracardiac conduit directed to the left pulmonary artery. All patients had a fenestration done. Results: G1 had 11 patients, G2 10 patients and G3 19 patients. Preoperative data were similar in the 3 groups (P>0.05). Surgical mortality was higher in Groups 1 and 2 (9.1 percent and 10 percent) compared to Group 3 (0 percent) but there was no statistical significance (P=0.3841). Pleural effusion was absent in Group 3 (0 percent), which was statistically significant in relation to the other groups (P=0.0128). The hospitalization time was also significantly lower in G3 (8 days) in relation to G1 (18 days) and G2 (13 days) (P=0.0164). Conclusion Intracardiac TCPA was associated with lower postoperative morbidity and is currently our preferred technique.

Fontan operation: a technique in evolution.

OBJECTIVES: Recent experimental flow studies based on angiography and magnetic resonance have shown that total cavopulmonary anastomosis (TCPA) is a valid concept for surgical treatment of many congenital heart defects, but there is not agreement of the best surgical arrangement. Our purpose is to analyze the immediate results with three different techniques of TCPA. METHODS: We sought to analyze retrospectively all TCPA realized from January 2005 to July 2008; there were 40 patients, all with previous Glenn anastomosis, with mean age of 6.4 +/- 3.2 years. Three different techniques were employed: Group 1 (G1) lateral tunnel, Group 2 (G2) extracardiac conduits, Group 3 (G3) intracardiac conduit directed to the left pulmonary artery. All patients had a fenestration done. RESULTS: G1 had 11 patients, G2 10 patients and G3 19 patients. Preoperative data were similar in the 3 groups (P>0.05). Surgical mortality was higher in Groups 1 and 2 (9.1% and 10%) compared to Group 3 (0%) but there was no statistical significance (P=0.3841). Pleural effusion was absent in Group 3 (0%), which was statistically significant in relation to the other groups (P=0.0128). The hospitalization time was also significantly lower in G3 (8 days) in relation to G1 (18 days) and G2 (13 days) (P=0.0164). CONCLUSION: Intracardiac TCPA was associated with lower postoperative morbidity and is currently our preferred technique.

Surgical strategy in transposition of the great arteries with aortic arch obstruction.

OBJECTIVE: To analyze our experience in the surgical correction of transposition of the great arteries associated with aortic arch obstruction. METHOD: From January 1998 to December 2005 we performed 223 arterial switch operations for transposition of the great arteries; 21 (9.4%) patients had associated aortic arch obstruction. Aortic arch anatomy showed: localized aortic coarctation (n=10) and coarctation with hypoplastic aortic arch (n=6) and interrupted aortic arch (n=5). Ventricular septal defect was present in 19 (90.5%) patients. Size discrepancy between the aorta and pulmonary artery and complex coronary artery anatomy were common findings. Surgical correction was performed in either one (14) or two stages (7). Aortic arch reconstruction was achieved either by resection and extended anastomoses (13) or by relocation of the ascending aorta (8). RESULTS: Hospital mortality was 23.8% (n=5); with only one death (11.1%) among the last nine patients. Reoperations in the immediate post-operative period included: bleeding (5), residual ventricular septal defect and unrecognized coarctation (1) or residual stenosis of the aortic arch (1). There were two late deaths caused by fungal infections and reoperation for severe aortic regurgitation. Three patients underwent procedures to relieve right ventricular outflow tract obstruction. Two patients have slight to moderate aortic regurgitation. CONCLUSION: The surgical treatment of transposition of the great arteries with aortic arch obstruction is complex with high morbidity. Our present choice is one-stage treatment for all patients without using homologous or heterologous tissue for aortic arch reconstruction. We recommend resection and extended anastomoses for localized coarctation and relocation of the ascending aorta for hypoplastic or interrupted aortic arch.

Estratégia cirúrgica na transposição das grandes artérias associada à obstrução do arco aórtico / Surgical strategy in transposition of the great arteries with aortic arch obstruction

OBJETIVO: Analisar nossa experiência no tratamento cirúrgico da transposição das grandes artérias (TGA) associada à obstrução do arco aórtico. MÉTODO: Entre janeiro de 1998 e dezembro de 2005, realizamos 223 operações de Jatene para correção de TGA: 21 (9,4 por cento) pacientes apresentavam obstruções do arco aórtico. A anatomia do arco aórtico evidenciou: coarctação da aorta localizada (n=10); coarctação com hipoplasia tubular do arco aórtico (n=6); interrupção do arco aórtico (n=5). Comunicação interventricular (CIV): 19 pacientes (90,5 por cento), sendo 11 do tipo Taussig-Bing. Desproporção importante entre aorta e artéria pulmonar e anomalias coronárias foram achados freqüentes. Houve 7 correções em dois estágios e 14 correções em um único estágio. A reconstrução do arco foi realizada por ressecção e anastomose término-terminal ampliada (13) ou por translocação da aorta ascendente (8). RESULTADO: Houve cinco (23,8 por cento) óbitos hospitalares; apenas um (11,1 por cento) nos últimos nove casos consecutivos. Reoperações no período hospitalar: revisão de hemostasia (5), CIV residual + coarctação não identificada (1), estenose residual de arco aórtico (1). Após a alta, houve dois óbitos e três pacientes foram submetidos a reintervenções para estenose da via de saída do ventrículo direito. CONCLUSÃO: O tratamento da transposição das grandes artérias associada à obstrução do arco aórtico apresenta alta complexidade e morbi-mortalidade. Empregamos as correções em um e em dois estágios, obtendo resultados comparáveis. Nossa preferência atual é pela correção precoce em um único estágio para todos os pacientes, independente de sua configuração anatômica.

OBJECTIVE:To analyze our experience in the surgical correction of transposition of the great arteries associated with aortic arch obstruction. METHOD: From January 1998 to December 2005 we performed 223 arterial switch operations for transposition of the great arteries; 21 (9.4 percent) patients had associated aortic arch obstruction. Aortic arch anatomy showed: localized aortic coarctation (n=10) and coarctation with hypoplastic aortic arch (n=6) and interrupted aortic arch (n=5). Ventricular septal defect was present in 19 (90.5 percent) patients. Size discrepancy between the aorta and pulmonary artery and complex coronary artery anatomy were common findings. Surgical correction was performed in either one (14) or two stages (7). Aortic arch reconstruction was achieved either by resection and extended anastomoses (13) or by relocation of the ascending aorta (8). RESULTS: Hospital mortality was 23.8 percent (n=5); with only one death (11.1 percent) among the last nine patients. Reoperations in the immediate post-operative period included: bleeding (5), residual ventricular septal defect and unrecognized coarctation (1) or residual stenosis of the aortic arch (1). There were two late deaths caused by fungal infections and reoperation for severe aortic regurgitation. Three patients underwent procedures to relieve right ventricular outflow tract obstruction. Two patients have slight to moderate aortic regurgitation. CONCLUSION: The surgical treatment of transposition of the great arteries with aortic arch obstruction is complex with high morbidity. Our present choice is one-stage treatment for all patients without using homologous or heterologous tissue for aortic arch reconstruction. We recommend resection and extended anastomoses for localized coarctation and relocation of the ascending aorta for hypoplastic or interrupted aortic arch.

[Surgical strategy for transposition of the great arteries with intact ventricular septum after the neonatal period]. / Estratégia cirúrgica na transposição das grandes artérias com septo interventricular intacto após o período neonatal.

OBJECTIVE: To assess the surgical results in patients with transposition of the great arteries and intact ventricular septum undergoing surgery after the neonatal period. METHODS: From January 1998 to March 2004, 121 children with transposition of the great arteries with intact ventricular septum were treated, 29 (24%) of whom after the neonatal period. Selection for surgical treatment was based on echocardiographic assessment by use of the calculation of left ventricular mass and configuration of the ventricular septum. Of the 29 children, 12 were selected for primary anatomic correction, 12 for 2-stage correction after surgical preparation of the left ventricle, and 5 underwent atrial correction. RESULTS: In the group undergoing primary anatomic correction, one (8.3%) patient died at the hospital due to sepsis. In the group undergoing 2-stage correction, 5 patients underwent slow preparation with correction 3-6 months after the first stage, 4 of whom died after the first stage. This fact caused a change in our protocol, with adoption of the rapid preparation technique in the other 7 patients, of whom all achieved the second stage. Of the 8 children undergoing the second stage, one died at the hospital and another died later. Late clinical evolution of the children in both groups is excellent. CONCLUSION: The echocardiographic selection allowed a safe choice of the best surgical approach for patients with transposition of the great arteries and intact ventricular septum after the neonatal period.

Estratégia cirúrgica na transposicão das grandes artérias com septo interventricular intacto após o período neonatal / Surgical strategy for transposition of the great arteries with intact ventricular septum after the neonatal period

OBJETIVO: Analisar o resultado cirúrgico em portadores de transposicão das grandes artérias com septo interventricular intacto, operados após o período neonatal. MÉTODOS: Entre janeiro de 1998 e marco de 2004 foram atendidas 121 criancas com transposicão das grandes artérias com septo interventricular intacto, sendo 29 (24 por cento) após o período neonatal. A selecão para tratamento cirúrgico foi baseada na avaliacão ecocardiográfica pelo cálculo da massa do ventrículo esquerdo e da configuracão do septo interventricular. Das 29 criancas, 12 foram selecionadas para correcão anatômica primária, 12 para correcão em dois estágios, após preparo cirúrgico do ventrículo esquerdo e 5 submetidas a correcão atrial. RESULTADOS: No grupo submetido à correcão anatômica primária houve 1 (8,3 por cento) óbito hospitalar por sepsis. No grupo de correcão em dois estágios, 5 pacientes foram submetidos ao preparo lento, com correcão 3-6 meses após o 1º estágio, ocorrendo 4 óbitos após o 1º estágio. Este fato determinou mudanca no nosso protocolo, adotando-se a técnica de preparo rápido nos outros 7 pacientes, tendo todos atingido o 2º estágio. Das 8 criancas submetidas ao 2º estágio houve 1 óbito hospitalar e outro óbito tardio. A evolucão clínica tardia das criancas de ambos os grupos é excelente. CONCLUSAO: A selecão ecocardiográfica adequada da transposicão de grandes artérias com septo interventricular intacto quando abordada após o período neonatal, permitiu uma orientacão segura da escolha da melhor abordagem cirúrgica nestes pacientes.

Long-term results with the Biocor-SJM stentless porcine aortic bioprosthesis.

BACKGROUND AND AIM OF THE STUDY: The study aim was to update the clinical analysis of hemodynamic performance, structural failure and survival in patients undergoing aortic valve replacement (AVR) with a composite aortic, aldehyde tanned, stentless porcine bioprosthesis. METHODS: Between January 1990 and March 2001, 247 patients underwent AVR with aortic stentless valves. Patient demographic and clinical analysis included age, sex, valve lesion, valve size, pre- and postoperative NYHA class, hospital morbidity, mortality, operative data and duration of hospitalization. RESULTS: Mean patient age was 47.3 years; 71% of patients were males, and 45% had aortic insufficiency. The incidence of rheumatic heart disease requiring surgery was 41.7%. In 23% of patients surgery was indicated due to aortic stented bioprosthetic dysfunction. Preoperatively, 81% of patients were in NYHA classes III and IV. Mean follow up was 5.9+/-2.8 years (range: 1 month to 11.4 years); total follow up was 1,392 patient-years (98% complete). The valve size used was < or =25 mm in 75.3% of patients. The mean intensive care unit stay was 2.6 days; mean hospital stay was 10.7 days. Hospital mortality was 4.0% and late death 6.1%. There were no valve-related deaths. Postoperatively, the mean aortic effective orifice area (EOA) was 1.71 cm2, the mean peak transvalvular gradient 17.1 mmHg, and the mean transvalvular gradient 9.0 mmHg; the left ventricular mass index (g/m2) was 174 and 117 before and after surgery respectively. The rate of leaflet tissue degeneration was 0.9%, and seen as mild by echocardiographic follow up. Actuarial survival at almost 12 years was 91%, and freedom from reoperations was approximately 99%. CONCLUSION: Patients with aortic stentless valves have hemodynamic benefits seen as larger aortic EOA, low transvalvular gradients, satisfactory left ventricular remodeling with significant reduction of left ventricular mass, low complication rate, low reoperation rate, lower leaflet tissue degeneration rate, and no valve-related mortality. A longer follow up is required to confirm these benefits.

Valva mitral heteróloga sem suporte: resultados clínicos a médio prazo / Heterologous mitral stentless valva: mid-term clinical results

Objetivo: O uso da prótese mitral porcina sem suporte("Stentless") propicia manutençao das características de fluxo e contratilidade do ventrículo esquerdo. No presente estudo, sao analisados os resultados a médio prazo com o uso desse substituto valvar. Casuística e Métodos: No período de março de 1992 a dezembro de 1995, 108 pacientes foram submetidos a implante de valva mitral "Stentless". A idade variou de ll a 65 anos (média 35,22 + 14,98). A etiologia predominante foi a doença reumática (94 casos), seguida da disfunçao de bioprótese mitral (6), degeneraçao mixomatosa (5), endocardite infecciosa (2) e lesao isquêmica (l). Vinte e seis (24,1 por cento) tinham estenose mitral, 24 (22,2 por cento) insuficiência mitral e 58 (53,7 por cento) dupla lesao. Operaçoes cardíacas prévias haviam sido realizadas em 21,3 por cento dos pacientes. Procedimentos associados foram necessários em 10 (9,3 por cento)casos. Resultados: A mortalidade hospitalar foi de 6,5 por cento (7 pacientes); em apenas l caso a endocardite precoce foi relacionada à valva. Dos l01 restantes, 3 foram reoperados, 2 devido a erro na medida da valva e 1 devido a deiscência da fixaçao ao músculo papilar. Com 2 pacientes perdidos no seguimento, 96 foram seguidos por 3,2 a 45 meses. No seguimento tardio ocorreram 6 óbitos devidos a: endocardite (l), infarto agudo do miocárdio (1), pancreatite (l), acidente vascular cerebral (l) e reoperaçoes para retroca valvar (2). Foram reoperados tardiamente 12 pacientes, 8 devido a insuficiência mitral (l óbito), 2 devido a diminuiçao da área valvar mitral e 2 a endocardite protética (l óbito). Atualmente, 80 pacientes têm sido avaliados trimestralmente. Os estudos ecodopplercardiográficos têm mostrado 63 pacientes com valvas funcionalmente normais, 15 com insuficiência mitral discreta e estável e 2 com reduçao da área valvar. Com exceçao destes 2 últimos pacientes, todos os outros têm mostrado melhora da funçao ventricular esquerda, com reduçao dos volumes sistólico e diastólico final, em avaliaçoes ecocardiográficas seriadas. Conclusao: As valvas mitrais porcinas sem suporte têm mostrado melhor performance hemodinâmica, com maior possibilidade de manutençao da funçao e do tamanho do ventrículo esquerdo. Embora este estudo tenha demonstrado uma curva de aprendizado bem definida relacionada a um novo substituto valvar e à técnica cirúrgica, estes fatores sao superados com treino e aderência à técnica atualmente em uso.