Outcomes of aortic coarctation surgical treatment in adults.

OBJECTIVE: The aim of this study is to describe our experience in aortic coarctation surgery in adult patients by assessing the immediate and mid-term outcomes. METHODS: From January 1987 to March 2000, 50 consecutive adult patients underwent surgery for correction of aortic coarctation, through left lateral thoracotomy. Of these, forty two (84%) patients presented high blood pressure, with mean systolic arterial pressure of 170.56 mmHg (125-220 mmHg). The mean of pressure gradient in the coarctation area was 51.4 mmHg (18-123 mmHg). Other associated surgical cardiovascular diseases were not treated in the same operative act, except in two cases of patent ductus arteriosus (PDA). Three different techniques were used: aortic coarctation resection with end-to-end anastomosis was performed in 20 (40%) patients, coarctation enlargement with bovine pericardial patch was performed in 22 (44%) patients and synthetic tube interposition was performed in eight (16%) patients. RESULTS: Operative morbidity was low; there was one case of bleeding who required reoperation. The most common immediate postoperative event was high blood pressure (98%), but it was easily controlled by intravenous drugs. There was no hospital death. Mean residual pressure gradient was 18.7 (8-33 mmHg). Patients were discharged in 9.5 days (5-30). Postoperative follow-up mean was 46.8 months (1-145 months) in 45 (91.8%) patients. Forty one (91.1%) of these followed-up patients had normal blood pressure, whereas 75.6% of them without drugs intake. 93.3% of these followed-up patients were asymptomatic. Four of them required further surgical operation, one needed a pacemaker implant, other two patients needed a cardiac valve replacement and one had endocarditis. There was one related death due to sepsis secondary to endocarditis. CONCLUSION: Surgical treatment of aortic coarctation, even in adult patients, is an efficient therapeutic choice, regardless of the applied surgical technique, with low morbidity and mortality. It reduces efficiently the arterial pressure levels in both immediate and mid-term follow-up.

Resultados do tratamento cirúrgico da coarctação de aorta em adultos / Outcomes of aortic coarctation surgical treatment in adults

OBJETIVO: Reportar a experiência no tratamento cirúrgico da coarctação da aorta (CoAo) em pacientes adultos, avaliando os resultados imediatos e a médio prazo. MÉTODOS: Entre janeiro de 1987 e março de 2000, 50 pacientes consecutivos adultos foram submetidos a tratamento cirúrgico da coarctação de aorta, por toracotomia lateral esquerda. Destes, 42 (84 por cento) eram hipertensos, com pressão arterial sistólica média de 170,6 mmHg (125-220 mmHg). O gradiente médio no local da coarctação era de 51,4 mmHg (18-123 mmHg). A abordagem de doenças cardiovasculares associadas não foi realizada no mesmo tempo cirúrgico, com exceção de dois casos de persistência do canal arterial (PCA). Ressecção da CoAo e anastomose término-terminal foi realizada em 20 (40 por cento) pacientes, ampliação da área de CoAo com retalho de pericárdio bovino em 22 (44 por cento) e interposição de um tubo sintético em oito (16 por cento). RESULTADOS: A morbidade operatória foi baixa, ocorrendo apenas uma reoperação por sangramento; a ocorrência mais frequente nas primeiras horas de pós-operatório foi hipertensão, observada em 98 por cento dos pacientes, controlada com medicamentos endovenosos. Não houve óbito hospitalar. O gradiente residual médio foi de 18,7 mmHg (8-33 mmHg). O tempo médio de internação hospitalar foi de 9,5 dias (5-30 dias). O tempo médio de seguimento foi de 46,8 meses (1145 meses) em 45 (91,8 por cento) pacientes; destes, 41 (91,1 por cento) encontravam-se normotensos, sendo que 75,6 por cento sem medicamentos. Em 93,3 por cento dos pacientes, não ocorreram sintomas de qualquer natureza. Quatro pacientes foram reoperados neste período (um para implante de marca-passo definitivo, dois para troca valvar, e outro por endocardite), ocorrendo um óbito tardio por endocardite e sepse. CONCLUSÃO: O tratamento cirúrgico da CoAo, mesmo em pacientes adultos, impõe-se como método terapêutico eficaz, nesta série, independentemente da técnica cirúrgica utilizada, ...

OBJECTIVE: The aim of this study is to describe our experience in aortic coarctation surgery in adult patients by assessing the immediate and mid-term outcomes. METHODS: From January 1987 to March 2000, 50 consecutive adult patients underwent surgery for correction of aortic coarctation, through left lateral thoracotomy. Of these, forty two (84 percent) patients presented high blood pressure, with mean systolic arterial pressure of 170.56 mmHg (125-220 mmHg). The mean of pressure gradient in the coarctation area was 51.4 mmHg (18-123 mmHg). Other associated surgical cardiovascular diseases were not treated in the same operative act, except in two cases of patent ductus arteriosus (PDA). Three different techniques were used: aortic coarctation resection with end-to-end anastomosis was performed in 20 (40 percent) patients, coarctation enlargement with bovine pericardial patch was performed in 22 (44 percent) patients and synthetic tube interposition was performed in eight (16 percent) patients. RESULTS: Operative morbidity was low; there was one case of bleeding who required reoperation. The most common immediate postoperative event was high blood pressure (98 percent), but it was easily controlled by intravenous drugs. There was no hospital death. Mean residual pressure gradient was 18.7 (8-33 mmHg). Patients were discharged in 9.5 days (5-30). Postoperative follow-up mean was 46.8 months (1-145 months) in 45 (91.8 percent) patients. Forty one (91.1 percent) of these followed-up patients had normal blood pressure, whereas 75.6 percent of them without drugs intake. 93.3 percent of these followed-up patients were asymptomatic. Four of them required further surgical operation, one needed a pacemaker implant, other two patients needed a cardiac valve replacement and one had endocarditis. There was one related death due to sepsis secondary to endocarditis. CONCLUSION: Surgical treatment of aortic coarctation, even in adult patients, is an ...

[B-type natriuretic peptide assessment in the diagnosis of rejection after pediatric heart transplant].

BACKGROUND: Rejection is one of the major causes of mortality following pediatric heart transplant. B-type natriuretic peptide (BNP) has been studied as a method for the diagnosis of acute rejection, especially in adult patients undergoing heart transplant. OBJECTIVE: To correlate serum BNP levels with acute rejection as diagnosed by endomyocardial biopsy in patients of the pediatric heart transplant group. METHODS: A total of 50 BNP samples were collected from 33 children in the postoperative period of heart transplant, and data on age, gender, skin color, blood group, immune panel, follow-up time after transplant, functional class, immunosuppressive regimen used and number of rejections were analyzed. RESULTS: Thirty three children with median age of 10.13 years were analyzed; of these, 54% were females and 78% were Caucasians. BNP levels were determined at a mean time from transplant of 4.25 years. Nine episodes of rejection were diagnosed in eight patients (27%) by means of endomyocardial biopsy; of these, three were grade 3A, five were grade 2, and one had humoral rejection. At the moment of biopsy, most patients were asymptomatic. The mean serum BNP level was 77.18 pg/ml, with 144.22 pg/ml in the group with rejection and 62.46 pg/ml in the group without rejection, with p = 0.02. CONCLUSION: Asymptomatic children can present acute rejection in the postoperative period of heart transplant. Serum BNP levels show a statistically significant difference in the group with rejection and thus can be an additional method in the diagnosis of cardiac rejection.

Avaliação do peptídeo natriurético tipo B no diagnóstico de rejeição após transplante cardíaco pediátrico / B-type natriuretic peptide assessment in the diagnosis of rejection after pediatric heart transplant / Evaluación del péptido natriurético tipo B en el diagnóstico de rechazo tras transplante cardiaco pediátrico

FUNDAMENTO: A rejeição constitui-se em uma das principais causas de mortalidade após o transplante cardíaco pediátrico. O peptídeo natriurético tipo B (BNP) tem sido estudado como método no diagnóstico de rejeição aguda, especialmente em pacientes adultos submetidos a transplante cardíaco. OBJETIVO:Correlacionar o nível sérico de BNP à rejeição aguda diagnosticada pela biópsia endomiocárdica em pacientes do grupo de transplante cardíaco pediátrico. MÉTODOS:Foram coletadas 50 amostras de BNP de 33 crianças em pós-operatório de transplante cardíaco e analisados dados de idade, sexo, cor, grupo sangüíneo, painel imunológico, tempo de evolução após o transplante, classe funcional, imunossupressão utilizada e número de rejeições. RESULTADOS:Foram 33 crianças com idade mediana de 10,13 anos, predomínio do sexo feminino (54%) e da cor branca (78%). No momento da dosagem de BNP o tempo médio de transplante foi 4,25 anos. A biópsia endomiocárdica diagnosticou nove rejeições em oito pacientes (27%), sendo três com grau 3 A, cinco com grau 2 e um com rejeição humoral. No momento da biópsia, a maioria dos pacientes encontrava-se assintomática. O nível sérico de BNP foi em média 77,18 pg/ml, sendo 144,22 pg/ml no grupo com rejeição e 62,46 pg/ml no grupo sem rejeição, com p = 0,02. CONCLUSÃO: Crianças assintomáticas podem apresentar rejeição aguda no pós-operatório de transplante cardíaco. O nível sérico de BNP apresentou diferença estatisticamente significante no grupo com rejeição, podendo ser método adicional no diagnóstico de rejeição cardíaca.

BACKGROUND: Rejection is one of the major causes of mortality following pediatric heart transplant. B-type natriuretic peptide (BNP) has been studied as a method for the diagnosis of acute rejection, especially in adult patients undergoing heart transplant. OBJECTIVE: To correlate serum BNP levels with acute rejection as diagnosed by endomyocardial biopsy in patients of the pediatric heart transplant group. METHODS: A total of 50 BNP samples were collected from 33 children in the postoperative period of heart transplant, and data on age, gender, skin color, blood group, immune panel, follow-up time after transplant, functional class, immunosuppressive regimen used and number of rejections were analyzed. RESULTS: Thirty three children with median age of 10.13 years were analyzed; of these, 54% were females and 78% were Caucasians. BNP levels were determined at a mean time from transplant of 4.25 years. Nine episodes of rejection were diagnosed in eight patients (27%) by means of endomyocardial biopsy; of these, three were grade 3A, five were grade 2, and one had humoral rejection. At the moment of biopsy, most patients were asymptomatic. The mean serum BNP level was 77.18 pg/ml, with 144.22 pg/ml in the group with rejection and 62.46 pg/ml in the group without rejection, with p = 0.02. CONCLUSION: Asymptomatic children can present acute rejection in the postoperative period of heart transplant. Serum BNP levels show a statistically significant difference in the group with rejection and thus can be an additional method in the diagnosis of cardiac rejection.

FUNDAMENTO: El rechazo constituye una de las principales causas de mortalidad tras el transplante cardiaco pediátrico. El péptido natriurético tipo B (BNP) ha sido estudiado como método en el diagnóstico de rechazo agudo, sobre todo en pacientes adultos sometidos a transplante cardiaco. OBJETIVO:Correlacionar el nivel sérico de BNP al rechazo agudo diagnosticado por biopsia endomiocárdica en pacientes del grupo de transplante cardiaco pediátrico. MÉTODOS:Se recolectaron 50 muestras de BNP de 33 niños en postoperatorio de transplante cardiaco, y se analizaron datos como edad, sexo, color, grupo sanguíneo, cuadro inmunológico, tiempo de evolución tras el transplante, clase funcional, inmunosupresión utilizada y número de rechazos. RESULTADOS:Se seleccionaron a 33 niños con edad promedio de 10,13 años, predominio del sexo femenino (54%) y de color blanca (78%). Al momento de la dosificación de BNP, el tiempo promedio de transplante fue de 4,25 años. La biopsia endomiocárdica diagnosticó nueve rechazos en ocho pacientes (27%), de ellos tres presentaron grado 3A, cinco grado 2 y uno rechazo humoral. Ya al momento de la biopsia, la mayoría de los pacientes se encontraba asintomática. El nivel sérico de BNP tuvo como promedio, 77,18 pg/ml; 144,22 pg/ml en el grupo con rechazo y 62,46 pg/ml en el grupo sin rechazo, con p = 0,02. CONCLUSIÓN: Niños asintomáticos pueden presentar rechazo agudo en el postoperatorio de transplante cardiaco. El nivel sérico de BNP presentó diferencia estadísticamente significante en el grupo con rechazo, lo que lo convierte en posible método adicional en el diagnóstico de rechazo cardiaco.

Off-pump correction of coronary fistula: the single suture technique approach.

The single suture technique was developed to obtain stabilization and exposure of all all coronary branches during off-pump coronary artery bypass, while maintaining hemodynamic stabilily during the procedure. We describe the use of this technique during an off-pump correction of a coronary artery fistula associated with right coronary dilatation. The suggested technique is quicker and less complex than on-pump surgery. Furthermore, it can be a useful tool for congenital fistula correction in select cases, stimulating the practice of less invasive heart surgery in these patients.

[Intrapericardic surgical treatment of teratoma in an infant]. / Tratamento cirúrgico de teratoma intrapericárdico em lactente.

The intrapericardic cardiac tumors are infrequent; however, the clinical manifestations can be serious, even with symptoms of low cardiac output or cardiogenic shock, depending on the localization of the tumor. We report the case of a 3-month-old infant who progressed to cardiogenic shock due to an intrapericardic tumor compressing the right atrium and the vena cava superior. Emergent surgery for resection of the tumor mass was recommended. The patient had a 6-month uneventful postoperative course.

Tratamento cirúrgico de teratoma intrapericárdico em lactente / Intrapericardic surgical treatment of teratoma in infant

Os tumores cardíacos intrapericárdicos são pouco freqüentes, porém, as manifestações clínicas podem ser graves, até com sintomas de baixo débito ou choque cardiogênico, dependendo da localização do tumor. Relatamos o caso de um lactente com três meses de idade, que apresentou na evolução choque cardiogênico, em decorrência de um tumor intrapericárdico, comprimindo o átrio direito e a veia cava superior. Indicada operação de urgência para ressecção da massa tumoral, apresentou adequada evolução até seis meses de pós-operatório.

The intrapericardic cardiac tumors are infrequent; however, the clinical manifestations can be serious, even with symptoms of low cardiac output or cardiogenic shock, depending on the localization of the tumor. We report the case of a 3-month-old infant who progressed to cardiogenic shock due to an intrapericardic tumor compressing the right atrium and the vena cava superior. Emergent surgery for resection of the tumor mass was recommended. The patient had a 6-month uneventful postoperative course.

A novel adjustable pulmonary artery banding system for hypoplastic left heart syndrome.

OBJECTIVE: Hypoplastic left heart syndrome remains a challenge for worldwide surgeons. Initial palliation employing bilateral pulmonary artery banding along with ductal stent implantation and atrial septostomy has been proposed as an alternative approach. However, the surgically placed bands are fixed and may become inadequate after sternum closure or with somatic growth of the patient. We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini banding system that allows for fine percutaneous adjustments of pulmonary blood flow. METHOD: Through a mid sternotomy, a 5 day-old neonate underwent bilateral pulmonary artery banding using this new system combined with placement of a main pulmonary artery to innominate artery shunt. RESULTS: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75%-85% range. On the 48th day of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. The Norwood operation and the bidirectional Glenn shunt were carried out on the 106th day of life. The bands were removed with no distortion of the pulmonary arteries. CONCLUSIONS: The clinical use of this innovative pulmonary artery banding system was feasible, safe and effective. It allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

Nova bandagem ajustável das artérias pulmonares na Síndrome de Hipoplasia de Câmaras Esquerdas / A novel adjustable pulmonary artery banding system for hypoplastic left heart syndrome

OBJETIVO: A Síndrome de Hipoplasia de Câmaras Esquerdas representa um grande desafio para cirurgiões do mundo inteiro. Atualmente, tem sido proposto procedimento paliativo alternativo, por meio da bandagem bilateral das artérias pulmonares associada à colocação de stent no canal arterial e atrioseptostomia. No entanto, as bandagens utilizadas são fixas, podendo tornar-se inadequadas após o fechamento do esterno ou com o rápido crescimento somático do paciente. Descrevemos a primeira aplicação clínica do novo dispositivo miniaturizado de bandagem ajustável das artérias pulmonares em neonato portador da síndrome de hipoplasia de câmaras esquerdas, o qual permitiu ajustes percutâneos precisos do fluxo sangüíneo pulmonar. MÉTODO: Através de esternotomia mediana, neonato de 5 dias de vida foi submetido à bandagem pulmonar bilateral, usando este novo dispositivo, combinada com interposição de tubo de PTFE entre o tronco pulmonar e o tronco braquiocefálico. RESULTADOS: O paciente apresentou boa evolução pós-operatória. Três ajustes percutâneos das bandagens foram necessários para manter a saturação arterial de oxigênio entre 75-85 por cento. No 48° dia de vida, o paciente foi submetido a atrioseptostomia com colocação de stent (6 mm) para tratamento de comunicação interatrial restritiva. No 106° dia de vida, realizou-se operação de Norwood associada à anastomose cavopulmonar bilateral. As bandagens foram removidas, sem distorção das artérias pulmonares. CONCLUSÕES: O uso clínico deste sistema inovador de bandagem ajustável das artérias pulmonares mostrou-se factível, seguro e eficaz. Permitiu o ajuste fino do fluxo pulmonar de acordo com as necessidades clínicas, proporcionando um equilíbrio preciso entre as circulações pulmonar e sistêmica.

OBJECTIVE: Hypoplastic left heart syndrome remains a challenge for worldwide surgeons. Initial palliation employing bilateral pulmonary artery banding along with ductal stent implantation and atrial septostomy has been proposed as an alternative approach. However, the surgically placed bands are fixed and may become inadequate after sternum closure or with somatic growth of the patient. We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini banding system that allows for fine percutaneous adjustments of pulmonary blood flow. METHOD: Through a mid sternotomy, a 5 day-old neonate underwent bilateral pulmonary artery banding using this new system combined with placement of a main pulmonary artery to innominate artery shunt. RESULTS: The patient had an uneventful postoperative course. Three percutaneous adjustments of the banding system were necessary to keep the arterial oxygen saturation in the 75 percent-85 percent range. On the 48th day of life, she was submitted to stent placement (6 mm) within the atrial septum to treat a restrictive atrial septal defect. The Norwood operation and the bidirectional Glenn shunt were carried out on the 106th day of life. The bands were removed with no distortion of the pulmonary arteries. CONCLUSIONS: The clinical use of this innovative pulmonary artery banding system was feasible, safe and effective. It allowed for customization of the pulmonary blood flow according to the underlying clinical needs, resulting in a more precise balance between the pulmonary and systemic circulations.

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.

Bandagem ajustável do tronco pulmonar: comparação de dois métodos de hipertrofia aguda do ventrículo subpulmonar / Adjustable pulmonary trunk banding: comparison of two methods of acute subpulmonary ventricle hypertrophy

OBJETIVO: Este estudo compara a sobrecarga contínua versus intermitente do ventrículo direito (VD) de cabritos, para induzir a hipertrofia ventricular. MÉTODO: Foram utilizados três grupos de sete cabritos jovens (controle, contínuo, intermitente). A sobrecarga sistólica foi imposta por 96 horas, no contínuo e por quatro períodos de 12 horas, alternados com 12 horas de descanso, no intermitente. Avaliações ecocardiográficas e hemodinâmicas foram feitas diariamente. Os animais foram, então, mortos para avaliar o conteúdo de água e peso das massas cardíacas. RESULTADOS: O Intermitente mostrou aumento dos pesos de VD e de septo, em relação ao controle (p<0,05), enquanto o contínuo aumentou apenas a massa do VD (p<0,05). Houve maior aumento da espessura do VD no Intermitente (p<0,05). O volume diastólico final do VD mostrou diferença significativa entre os grupos (p=0,01), com maior dilatação do VD do grupo contínuo, no momento 24 horas de sobrecarga sistólica (p<0,03). A fração de ejeção do VD se manteve dentro da normalidade nos dois grupos ao longo do protocolo. Foi observado menor perímetro do VD no intermitente, após 96 horas de treinamento (p<0,05). Não houve diferença significante entre os grupos de estudo e o controle quanto ao conteúdo de água do miocárdio do VD. CONCLUSÃO: A bandagem ajustável do tronco pulmonar permitiu um rápido processo hipertrófico do VD em ambos os grupos, sendo, porém, mais eficiente no intermitente. Nosso estudo sugere que a preparação do ventrículo subpulmonar de forma intermitente poderá proporcionar melhor resultado para a operação de Jatene em dois estágios.

OBJECTIVE: This study compares ventricular hypertrophy induced by continuous versus intermittent systolic overload of the pulmonary ventricle (RV) of young goats. METHODS: Three groups of seven goats were used (control, continuous, and intermittent). Systolic overload was maintained for 96 hours in the continuous group, while the intermittent group suffered four 12-hour periods of systolic overload, alternating with 12-hour resting periods. Echocardiographic and hemodynamic evaluations were performed every day. The animals were then killed for myocardial water content and weight evaluation. RESULTS: Both study groups achieved significant increases in RV mass (p<0.05). However, significant increases of the septum mass were observed only in the Intermittent Group (p<0.05). A greater increase in the RV wall thickness was observed in the Intermittent Group (p<0.05). There was a significant difference in RV diastolic volume between the two groups (p= 0.01), with a greater RV dilation in the Continuous Group after 24 hours of continuous overload (p< 0.03). In both groups, the RV ejection fraction was maintained within the normal range throughout the protocol. A smaller RV perimeter was observed in the Intermittent Group after 96 hours of systolic overload (p<0.05). There was no significant difference in RV myocardial water content between the study groups and the Control Group. CONCLUSIONS: Adjustable pulmonary artery bandages permit rapid RV hypertrophy in both groups. Nevertheless, it is more efficient in the Intermittent Group. This study suggests that preparation of the pulmonary ventricle with intermittent systolic overload might provide better results for the 2-stage arterial switch operation.

Post-transplantation lymphoproliferative disorder in pediatric patient.

Immunosuppressive therapy for transplanted patients exposes them to a high risk of developing posttransplantation lymphoproliferative disorders (PTLD). We report the case of a child undergoing heart transplantation at seven months of age who developed PTLD at nine years of age, diagnosed by resection of a pulmonary nodule.

Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery.

OBJECTIVE: Unilateral pulmonary artery agenesis is an uncommon isolated anomaly and since 1978 only 119 cases have been reported. In general the condition presents as pulmonary hypertension (PH) in children and hemoptysis in adults. Interventions such as pulmonary artery reconstruction and lobectomies were performed in 17% of the cases. We analyzed four of these cases, two in natural evolution and two with late term PH regression after surgical correction. METHODS: Three 22,10 and 35 month old male children and one 20 month old female child were included in the study. The 22 month old presented right-sided heart failure (RHF) and cyanosis; the 10 month old presented RHF and the other two presented exertion fatigue. All had PH symptoms, right ventricular strain on the EKG and cardiomegaly. Cardiac catheterization showed systemic pressures in the contralateral pulmonary artery, with right-sided agenesis in three of the children and left-sided agenesis in one child. RESULTS: Surgical correction of pulmonary artery continuity was possible in the 22 month old and 10 month old using a 7 mm diameter Goretex conduit between the pulmonary arteries up to the hypoplastic contralateral pulmonary hilum. There was early and late regression of the PH signs and the children remained stable during follow-up to the ages of 7 and 2.5 years, respectively. The pressure ratio between the left and right ventricles was 30 and 40%, in both cases. Pulmonary perfusion increased from 8 to 44% and from 8 to 23%, in the two cases. The same procedure was scheduled for the other patients. CONCLUSION: This technique has become the operation of choice for similar cases, that are rarely described in literature, even in the presence of severe PH and contralateral pulmonary artery hypoplasia.

Agenesia isolada da artéria pulmonar direita ou esquerda: avaliação da evolução natural e a longo prazo, após intervenção corretiva / Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery / Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery

OBJETIVO: Agenesia da artéria pulmonar, direita ou esquerda, rara como anomalia isolada, foi relatada em 119 casos desde 1978. Apresenta-se clinicamente com hipertensão pulmonar (HP) na infância e com hemoptise no adulto. Intervenções como reconstrução arterial pulmonar e lobectomia foram realizadas em 17 por cento dos casos. Analisamos quatro destes casos, sendo dois em evolução natural e dois com regressão da HP, após longo tempo da correção operatória. MÉTODOS: Quatro crianças, três do sexo masculino, com 22,10 e 35 meses de idade e uma do sexo feminino com 20 meses, com insuficiência cardíaca direita (ICD) e cianose na primeira e ICD na segunda e cansaço aos esforços nas outras duas. Todas tinham sinais de HP, sobrecarga ventricular direita no ECG e cardiomegalia. Cateterismo cardíaco mostrou pressões sistêmicas na artéria pulmonar contralateral na agenesia à direita em três e na agenesia à esquerda em um caso. RESULTADOS: Restabelecimento cirúrgico da continuidade arterial pulmonar foi possível em dois casos, na criança de 22 e na de 10 meses de idade, pela interposição de Goretex de diâmetro de 7 mm entre as artérias pulmonares até o hilo pulmonar contralateral hipoplásico. Houve regressão dos sinais de HP em período imediato e tardio, com 7 e 2,5 anos de idade respectivamente. A relação das pressões entre os ventrículos direito e esquerdo era de 30 e 40 por cento, nos dois casos. A perfusão pulmonar aumentou de 8 para 44 por cento e de 8 para 23 por cento, nos dois casos. O mesmo procedimento foi programado para os outros pacientes. CONCLUSÃO: Esta técnica se torna a operação de escolha para casos similares, raramente descritos na literatura, mesmo em presença de acentuada HP e hipoplasia arterial pulmonar contralateral.

OBJECTIVE: Unilateral pulmonary artery agenesis is an uncommon isolated anomaly and since 1978 only 119 cases have been reported. In general the condition presents as pulmonary hypertension (PH) in children and hemoptysis in adults. Interventions such as pulmonary artery reconstruction and lobectomies were performed in 17 percent of the cases. We analyzed four of these cases, two in natural evolution and two with late term PH regression after surgical correction. METHODS: Three 22,10 and 35 month old male children and one 20 month old female child were included in the study. The 22 month old presented right-sided heart failure (RHF) and cyanosis; the 10 month old presented RHF and the other two presented exertion fatigue. All had PH symptoms, right ventricular strain on the EKG and cardiomegaly. Cardiac catheterization showed systemic pressures in the contralateral pulmonary artery, with right-sided agenesis in three of the children and left-sided agenesis in one child. RESULTS: Surgical correction of pulmonary artery continuity was possible in the 22 month old and 10 month old using a 7mm diameter Goretex conduit between the pulmonary arteries up to the hypoplastic contralateral pulmonary hilum. There was early and late regression of the PH signs and the children remained stable during follow-up to the ages of 7 and 2.5 years, respectively. The pressure ratio between the left and right ventricles was 30 and 40 percent, in both cases. Pulmonary perfusion increased from 8 to 44 percent and from 8 to 23 percent, in the two cases. The same procedure was scheduled for the other patients. CONCLUSION: This technique has become the operation of choice for similar cases, that are rarely described in literature, even in the presence of severe PH and contralateral pulmonary artery hypoplasia.

Desordem linfoproliferativa pós-transplante em paciente pediátrico / Post-transplantation lymphoproliferative disorder in pediatric patient / Post-transplantation lymphoproliferative disorder in pediatric patient

Terapias de imunossupressão, a que pacientes transplantados devem ser submetidos, os expõe a um alto risco de desenvolver desordens linfoproliferativas pós-transplante (PTLD). Descrevemos o caso de uma criança submetida a transplante cardíaco aos sete meses de idade e que acabou desenvolvendo PTLD, aos nove anos, diagnosticada por meio de retirada de nódulo pulmonar.

Immunosuppressive therapy for transplanted patients exposes them to a high risk of developing posttransplantation lymphoproliferative disorders (PTLD). We report the case of a child undergoing heart transplantation at seven months of age who developed PTLD at nine years of age, diagnosed by resection of a pulmonary nodule.

Avaliação experimental de novo eletrodo para implante de marca-passo em fetos / Experimental assessment of a new electrode for fetal pacing

OBJETIVO: O bloqueio atrioventricular total (BAVT) fetal apresenta mau prognóstico quando associado à hidropisia. O implante de marca-passo pode melhorar as chances de sobrevida fetal. Este estudo analisa as características eletrofisiológicas do novo eletrodo de marca-passo fetal, a hemodinâmica e metabolismo fetal sob freqüências cardíacas variadas. MÉTODO: O novo eletrodo (formato de "T") foi implantado no coração de cinco fetos de cabras. O débito cardíaco fetal foi medido com fluxômetro. O BAVT foi obtido por meio da crioablação do nó atrioventricular fetal. Os parâmetros eletrofisiológicos, hemodinâmica e metabolismo fetal foram analisados sob diversas freqüências cardíacas. RESULTADOS: O eletrodo fetal apresentou baixos limiares agudos, sem falhas no comando do estímulo. A curva do limiar de estimulação permaneceu relativamente constante para larguras de pulso acima de 0,5 mseg. A resistência foi de 1050,4 ± 76,6 ohms; e a onda R foi de 8,6 ± 5,6 mV. A freqüência cardíaca fetal abaixo de 60 bpm produziu baixo débito cardíaco fetal e hipotensão arterial (p<0,05). A bradicardia fetal ocasionou queda da saturação de oxigênio paralela à queda do débito cardíaco fetal, sendo mais grave abaixo de 60 bpm. CONCLUSÃO: O novo eletrodo permite um procedimento menos invasivo e uma fixação miocárdica estável, evitando-se o deslocamento do eletrodo após a recuperação da atividade fetal. Os limiares obtidos são compatíveis com uma estimulação crônica estável e segura. Este estudo sugere a freqüência cardíaca inicial de 80 bpm pós-implante, com posterior aumento gradual, o que permitirá um aumento adequado do débito cardíaco e uma situação mais adaptativa.

OBJECTIVE: The prognosis of congenital complete heart block (CHB) is very poor when manifested with fetal hydrops. Fetal pacing may improve the survival rate. This study aims to assess the electrophysiological characteristics of a new lead, as well as fetal hemodynamic and metabolism under different fetal heart rates. METHODS: The new lead (T-shaped) was deployed into the myocardium of five fetal goats. Fetal cardiac output was measured by a flow transducer. CHB was achieved by cryosurgical ablation of fetal AV node. Electrophysiological parameters, hemodynamic and metabolic behavior of the fetus under different fetal heart rates were evaluated. RESULTS: The acute stimulation thresholds were consistently low. The voltage strength-duration curve remained relatively constant at pulse widths > 0.5 msec. The stimulation resistance was 1050.4 ± 76.6 ohms, and the sensed fetal R wave was 8.6 ± 5.6 mV. Fetal heart rate bellow 60 bpm was associated to low cardiac output and low blood pressure (p<0.05). Fetal bradycardia also determined oxygen saturation drop parallel to low cardiac output, with severe hypoxia below 60 bpm. CONCLUSION: The new lead allows for a less invasive procedure and stable fixation to the myocardium that may prevent lead dislodgement after fetal recovery. It seems to be compatible with safe chronic stimulation. This study suggests that a gradual increase in the fetal rate beginning on 80 bpm after implantation would be more adaptive and adequately augment fetal cardiac output.

Defeito completo do septo atrioventricular com cianose / Complete repair in total atrioventricular canal defect with cyanosis / Complete repair in total atrioventricular canal defect with cyanosis

Os defeitos do septo atrioventricular total (DSAVT) representam 4 por cento das mal formações cardíacas e acima de 50 por cento dos defeitos observados na síndrome de Down (SD)¹. A apresentação clínica é de insuficiência cardíaca precoce na infância e hipertensão pulmonar por hiperfluxo. Raramente a cianose é observada e sugere hipertensão pulmonar ou associação à tetralogia de Fallot³, dupla via de saída de ventrículo direito², anomalia de Ebstein4, drenagem anômala de cava esquerda persistente em átrio esquerdo (Barbero Marcial, comunicação pessoal). Crianças com SD são particularmente difíceis de avaliação por apresentarem obstrução de vias aéreas superiores, que podem contribuir com o aumento da resistência pulmonar observada no cateterismo cardíaco. A presença de cianose pré-operatória constitui-se um desafio ao tratamento cirúrgico devido ao risco de hipertensão pulmonar irreversível com falência ventricular direita com, a correção dos defeitos intracardíacos.

Atrioventricular septal defects account for 4 percent of congenital cardiac malformations and over 50 percent of cardiac defects seen in Down syndrome¹. Clinical presentation is marked by congestive heart failure early in infancy. Cyanosis is rarely found in infants and suggests irreversible pulmonary hypertension or associated cardiac defects as tetralogy of Fallot, double outlet right ventricle², Ebstein anomaly³, persistent left superior vena cava draining in the left atrium (Barbero Marcial, personal communication). Children with Down's syndrome is particularly difficult to assess because they often suffer from upper airways obstruction4, which may contribute to the increased pulmonary vascular resistance determined at cardiac catheterization. This association of factors becomes a challenge for operability and, we will report one such case.

Tratamento cirúrgico da embolização de coil para artéria pulmonar após tentativa de fechamento percutâneo do canal arterial / Surgical treatment of coil embolization to the pulmonary artery after an attempt at percutaneous closure of patent ductus arteriosus

A embolização de uma das três molas de Gianturco usadas para o fechamento de grande canal arterial, em criança de 5 anos e 6 meses, alojada na artéria pulmonar esquerda, requereu intervenção operatória 6 meses após, em face da diminuição da perfusão para esse pulmão. Foi realizada secção e sutura do canal arterial e, sob hipotermia profunda através a arteriotomia pulmonar esquerda, foi retirada a mola obstrutiva, localizada na artéria lobar inferior, aderida à parede arterial e coberta por endotélio. Não havendo trombo no local, a perviabilidade arterial pulmonar foi totalmente restabelecida.

[Total anomalous drainage of the pulmonary veins-- surgical therapy for the infradiaphragmatic and mixed anatomical types]. / Drenagem anômala total das veias pulmonares: terapêutica cirúrgica dos tipos anatômicos infracardíaco e misto.

OBJECTIVE: To study the in-hospital evolution of patients with the infradiaphragmatic and mixed types of total anomalous pulmonary venous drainage (TAPVD), who underwent surgical therapy. METHODS: Of the 65 patients diagnosed with isolated TAPVD and operated on from December 1993 to March 2002, 7 (10.8%) patients with the mixed and infradiaphragmatic forms were retrospectively selected. Their ages ranged from 5 days to 19 months (mean of 7 months), 5 (71.4%) were males, and their clinical diagnosis was established with 2-dimensional echocardiography. Four (57.1%) patients had the mixed form, which was intrinsic obstructive in 1 patient, with mild stenosis of the left inferior vein. The remaining 3 (42.9%) patients had the obstructive infradiaphragmatic form, extrinsic at the level of the diaphragm. All surgeries were performed through median sternotomy with hypothermic extracorporeal circulation, and total circulatory arrest was required in 2 patients. RESULTS: In-hospital death occurred in 1 patient with infradiaphragmatic TAPVD with connection of the inferior vertical vein with the portal vein. The cause of death was related to multisystem organ failure. In 4 (57.1%) patients, the postoperative period was characterized by the presence of low cardiac output and pulmonary hypertension. CONCLUSION: The result of the surgical correction of this anomaly is associated with acceptable morbidity and mortality, depending on early referral and surgery, without progression of the pulmonary vascular hypertension findings.