Intervenção Paliativa Endovascular no Lactente com Tetralogia de Fallot: Uma Série de Casos / Palliative Endovascular Intervention in Infants with Tetralogy of Fallot: A Case Series

Resumo Fundamento Tendo em vista os casos de lactentes sintomáticos com Tetralogia de Fallot (TF), baixo peso ao nascimento e anatomia complexa, o implante de stent na via de saída do ventrículo direito (VSVD) tem sido indicado alternativamente à cirurgia de Blalock-Taussig (BT). Objetivo Avaliar o implante endovascular de stent na VSVD como abordagem primária no lactente com TF e não candidato à cirurgia de BT, bem como relatar seus resultados a médio prazo e até a retirada do stent na cirurgia corretiva. Métodos Entre outubro de 2015 e abril de 2018, uma série de seis lactentes portadores de TF receberam stents para desobstrução da VSVD. Os parâmetros hemodinâmicos foram comparados em períodos pré e pós-implante. Resultados As medianas de idade e peso no momento do procedimento foram de 146,5 dias e 4,9 kg, respectivamente. O gradiente sistólico máximo diminuiu de 63,5 mmHg para 50,5 mmHg, enquanto o diâmetro dos ramos pulmonares direito e esquerdo aumentou de 3,5 mm para 4,9 mm e 4,3 mm, respectivamente. O índice de Nakata aumentou de 96,5 mm para 108,3 mm; assim como o peso, de 4,9 kg para 5,5 kg. A saturação de oxigênio aumentou de 83,5% para 93%. Houve um caso de migração do stent e dois óbitos, um deles devido à embolização do stent e o outro não teve relação com o procedimento. Conclusões O implante de stent na VSVD como procedimento paliativo na TF se mostra uma alternativa promissora para o tratamento de lactentes com má anatomia e baixo peso ao nascimento.

Abstract Background Endovascular stent placement in the right ventricular outflow tract (RVOT) has been an alternative to Blalock-Taussig (BT) surgery in the treatment of Tetralogy of Fallot (TOF) in symptomatic infants with low birth weight and complex anatomy. Objective To evaluate endovascular stent placement in the RVOT as a primary treatment for infants with TOF who are not candidates for BT surgery, and evaluate medium-term outcomes until the stent is removed during corrective surgery. Methods Six infants with TOF were treated with RVOT stenting from October 2015 to April 2018. Hemodynamic parameters were compared between the pre- and post-stenting periods. Results At the time of stenting, participants had a median age and weight of 146.5 days and 4.9 kg, respectively. Peak systolic gradient decreased from 63.5 mm Hg to 50.5 mm Hg, while the diameter of the left and right pulmonary arteries increased from 3.5 mm to 4.9 mm and 4.3 mm, respectively. The Nakata index increased from 96.5 mm to 108.3 mm; weight increased from 4.9 kg to 5.5 kg; and oxygen saturation, from 83.5% to 93%. There was one case of stent migration and two deaths, one caused by stent embolization and the other unrelated to study procedures. Conclusions RVOT stenting is a promising alternative for the palliative treatment of TOF in infants with low birth weight and complex anatomy.

Informe de caso y revisión bibliográfica: Tetralogía de Fallot / Case report and bibliographic review: Tetralogy of Fallot

La Tetralogía de Fallot es la enfermedad cardiaca congénita cianótica más común y requiere una corrección quirúrgica temprana; se presenta el caso de una paciente que a los seis meses es sometida a cirugía paliativa de derivación sistémico pulmonar con fistula Blalock Taussing modificada con in-jerto de Goretex, su cuadro no mejora con el paso de los años debiéndose realizar cirugía de corrección total. Al ingreso presenta disnea de medianos esfuerzos, astenia, cianosis peribucal y distal que ceden con el reposo; en tórax presencia de cicatriz infra axilar compatible con cirugía paliativa previa, corazón: R1 y R2 rítmicos, normo fonéticos, presencia de soplo holosistólico en foco pulmonar y soplo infraclavicular izquierdo de fístula sistémico pulmo-nar. En la radiografía de tórax se observa aumento de índice cardiotorácico e hilios vasculares, el ecocardiograma pre quirúrgico transtorácico reporta estenosis infundibular por hipertrofia severa del miocardio, gradiente máximo de 90 mm/Hg. La cirugía de corrección total de Tetralogía de Fallot luego de procedimientos paliativos previos, con control por ecocardiograma transesofágico transoperatorio mejora la calidad de vida del paciente.

Tetralogy of Fallot is the most common cyanotic congenital heart disease and requires early surgical correction, this is a case of a patient who at six months undergoes palliative pulmonary systemic bypass surgery with modified Bla-lock Taussing fistula with Goretex graft, his condition does not improve with the passing of the years and a total correction surgery must be performed.Upon admission, the patient presents: dyspnea of medium efforts, asthenia, peribucal and distal cyanosis that yield with rest; in thorax presence of infra axillary scar compatible with previous palliative surgery, heart: rhythmic R1 and R2, phonetic norm, presence of holosystolic murmur in pulmonary focus and left infraclavicular murmur of pulmonary systemic fistula.The chest radiograph shows an increase in cardiothoracic index and vascu-lar congenital and prominent hilum, transthoracic pre-surgical echocardio-graphy is performed where infundibular stenosis is observed due to severe myocardial hypertrophy, maximum gradient of 90 mm / Hg.Tetralogy of Total Fall correction surgery after previous palliative procedures, with transoperative transesophageal echocardiogram control, improves the patient's quality of life.

Superior vena cava obstruction after the Glenn procedure

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Caso Clínico: Tetralogía de Fallot Extrema / Case Report: Extreme Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot representa la cardiopatía congénita cianógena más frecuente después del primer año de vida, ocurre aproximadamente en 1 de cada 3 500 nacimientos y representa el 7 al 10% de todas las Enfermedades Cardiacas Congénitas. Su variante extrema que se acompaña de atresia pulmonar representa el 1 a 2% de las cardiopatías congénitas. Presentamos una Tetralogía de Fallot Extrema Ductus Dependiente de manejo complejo. CASO CLÍNICO: Se trata de un neonato a término de 12 días de vida, adecuado para la edad gestacional, sin factores de riesgo prenatales, ni antecedentes patológicos familiares, el cual es diagnosticado de Tetralogía de Fallot Extrema por ecocardiograma. EVOLUCIÓN: Paciente inicialmente tratado con infusión de prostaglandina E1. Posteriormente mediante fístula Blalock-Taussig izquierda. CONCLUSIONES: Para mejorar el pronóstico y sobrevida de un paciente con Tetralogía de Fallot Extrema el diagnóstico prenatal oportuno y el tratamiento apropiado es de vital importancia, sin embargo el tratamiento definitivo con derivaciones pulmonares sistémicas representan complicaciones con riesgo de mortalidad. El presente caso tuvo un desenlace desfavorable.

BACKGROUND: The Tetralogy of Fallot represents the most common cyanogenic heart disease after the first year of life. It occurs in 1 of 3 500 live births and accounts as 7 to 10% of all Congenital Heart Diseases. Its extreme variant is accompanied by pulmonary atresia and accounts 1 to 2% of the Congenital Heart Diseases. We present an Extreme Tetralogy of Fallot­ Ductus Dependent of complex management. CLINICAL CASE: A full term 12 day neonate, adequate for gestational age, without prenatal risk factors or pathologic family antecedents, is diagnosed with Extreme Tetralogy of Fallot by echocardiogram. EVOLUTION: The patient was treated initially with an infusion of prostaglandin E1. Then, a left Blalock-Taussig shunt was performed. CONCLUSIONS: To improve the prognosis and survival rates of patients with Extreme Tetralogy of Fallot , a timely diagnosis and treatment are of vital importance. Definitive treatment with systemic pulmonary shunts carries high risk complications. The present case had an unfavorable evolution.

Tetralogy of Fallot repair in patients presenting after Infancy: a single surgeon experience

Objective: To determine the early surgical outcomes of Tetralogy of Fallot [TOP] repair in children and young adults operated after the age of one year

Methods: In this retrospective study, 307 cases of primary repair of Tetralogy of Fallot were done between September 2012 to February 2017, at CPE Institute of cardiology, Multan. Out of 307 operated patients, 4 [1.3%] patients had previous modified Blalock Taussig shunts, 2 [0.6%] associated ASD with TOF, 3 [0.9%] co-association of TOF with PDA, 2 [0.6%] had large conal arterial branch crossing the annulus, 3 [0.9%] had dextrocardia with situs inversus, 12 [3.9%] TOF with double outlet right ventricle [DORV], 2 [0.6%] were associated with complete AV canal defect, 8 [2.60%] with absent pulmonary valve syndrome, 15 [5.5%] I with left pulmonary artery stenosis. Data of post-operative complications and operative parameters was recorded for all patients

Results: Mean age of operated patients was 9.56+/-4.89 years. Post-operative complications occurred in 7.8% of patients. Most common post-operative complications were pleural effusion with a frequency of 12[3.9%] patients, and complete heart block in one patient. Insignificant small residual VSD was diagnosed in 8 [2.6%] patients. One moderately large VSD was closed surgically after one year of 1st surgery. Moderate to severe pulmonary valve regurgitation was diagnosed in 114 [37.1%] patients. Mild to moderate tricuspid regurgitation in 15 [4.8%] patients and moderate right ventricular outflow tract obstruction [RVOT] in 16 [5.2%] patients. Thirty-day mortality was only four [1.3%]

Conclusion: Surgical correction of Tetralogy of Fallot [TOF] in children after one year carries good operative outcomes with minimum morbidity and mortality

Indicações atuais para anticoagulacão em crianças com Cardiopatias Congênitas / Current indications for anticoagulation in children with Congenital Heart Disease

Os avanços nas técnicas cirúrgicas e manejo clínico aumentaram a sobrevida das crianças com cardiopatia congênita. Eventos tromboembólicos representam importante causa de morbimortalidade nestes pacientes, principalmente no pós-operatório da cirurgia de Fontan e implante de próteses, acrescido do risco adicional do seu tratamento. Particularidades próprias da idade, fatores hemodinâmicos e limitações na investigação de trombose contribuem para a dificuldadede definir diretrizes para anticoagulação em crianças. Os medicamentos mais usados na fase aguda são: heparina não fracionada e de baixo peso molecular, enquanto em longo prazo os antagonistas da vitamina K, principalmente a varfarina. O manejo seguro de anticoagulação nesta faixa etária é dificultado pela estreita faixa terapêutica, interação alimentar, uso simultâneo de vários medicamentos, além da dificuldade de punção venosa frequente e, com frequência os níveis desejáveis não são atingidos. Ainda faltam dados que deem suporte para recomendação de anticoagulação em crianças. Os novos anticoagulantes como os inibidores diretos da trombina e os inibidores seletivos do fator Xa, são empregados em adultos, principalmente com fibrilação atrial, o uso pediátrico ainda não está definido. Esta revisão sumariza as indicações atuais do uso de anticoagulantes e antiagregantes plaquetários em crianças com cardiopatia congênita.

Advances in surgical techniques and clinical management have led to increased survival ofchildren with congenital heart disease. Thromboembolic events represent a major cause of morbidity and mortality in these patients, particularly following Fontan surgery and the implantation of prosthesis, added to the risk of their treatment. Characteristics such as age, hemodynamic factors and limitations in the investigation of thrombosis have contributed to the difficulty of defining guidelines for anticoagulation in children. The most commonly used medications in the acute phase are and low molecular weight heparin, and in the long term, vitamin K antagonists, particularly warfarin. The safe management of anticoagulation in this age range is hindered by the narrow therapeutic range, food interactions, simultaneous use of other medications, and the difficulty of frequent venous puncture. As a result, the desired levels are often not reached. There is also a lack of data to support the recommendation of anticoagulation in children. The pediatric use of the new anticoagulants, such as direct thrombin inhibitors and selective factor Xa inhibitors used in adults, particularly with atrial fibrillation, is not yet defined. This review summarizes the current indications of the use of anticoagulants and platelet antiaggregants in children with congenital heart disease.

Resultados imediatos e de médio prazo da intervenção com stent ductal em recém-nascidos e lactentes / Immediate and medium-term outcomes of ductal stenting in neonates and infants

O implante de stents para manter o ducto arterial patente na cardiopatia congênita cianótica éuma alternativa à cirurgia de Blalock-Taussig modificada (BTm) em pacientes de alto risco. Descrevemos osresultados imediatos e de médio prazo do implante de stent em neonatos e lactentes com circulação pulmonarducto-dependente. Métodos: Trata-se de estudo descritivo e prospectivo, que incluiu diferentes cardiopatias congênitas cianóticas tratadas entre 2014 e 2015.Resultados: Avaliamos 14 pacientes, com média de idade de 46 dias e pesando 4,5 kg, sendo a atresia pulmonar associada à comunicação interventricular a cardiopatia mais tratada. A abordagem pela artéria femoral ocorreu em 70% dos procedimentos e, nos demais, por via carotídea. Stents de 3,5 × 12 mm foram usados na maioria dos casos, e o sucesso do implante foi obtido em 78% das intervenções (11/14). Os casos de insucesso foram encaminhados para cirurgia − uma delas em situação de urgência, que resultou em óbito. Ocorreu espasmo ductal < 48 horas em três pacientes que necessitaram de BTm, com evolução favorável. Complicações após a alta e nos primeiros 30 dias incluíram trombose de stent (2/11), uma delas controlada com redilatação e outra que evoluiu para óbito, e uma morte súbita (1/11). A mortalidade total foi de 21,4% (3/14). A patência do ducto arterial nos primeiros 6 meses foi obtida em 5 casos que foram submetidos à cirurgia paliativa.Conclusões: A experiência inicial de implante de stent ductal mostrou resultados imediatos favoráveis, e, emmédio prazo, mais de um terço dos pacientes com circulação pulmonar ducto-dependente manteve seus canaispatentes...

The implantation of stents to keep the ductus arteriosus patent in cyanotic congenital heart disease is an alternative to the modified Blalock-Taussig surgery (mBT) in high-risk patients. This study describes theimmediate and medium-term outcomes of stent implantation in neonates and infants with duct-dependentpulmonary circulation. Methods: This was a descriptive and prospective study including different cyanotic congenital heart diseases treated between 2014 and 2015. Results: Fourteen patients with a mean age of 46 days, and mean weight of 4.5 kg were assessed, andpulmonary artresia with interventricular communication was the most treated condition. The femoral artery approach was used in 70% of procedures; carotid approach was used in the remaining cases. Stents of 3.5 x 12 mm were used in most cases, and implant success was achieved in 78% of interventions (11/14). The failed cases were referred to surgery – one of them was an emergency, which resulted in death. Ductal spasm occurred in < 48 hours in three patients who required mBT, with favorable outcome. Complicationsafter discharge and within the first 30 days included stent thrombosis (2/11), one of which was controlled with redilation, another progressed to death, and one sudden death (1/11). The overall mortality was 21.4% (3/14). A patent ductus arteriosus in the first 6 months was present in five cases, which underwent palliative surgery.Conclusions: The initial experience of ductal stenting showed favorable immediate outcomes, but in the mediumterm, little more than a third of the cases maintained a patent ductus arteriosus within 6 months...

Laparoscopic colectomy in an adult with single ventricle physiology: Anesthetic implications and management.

Increasing numbers of adult patients with complex congenital heart conditions are presenting for noncardiac surgery later in life. These disorders can present challenges for surgical and anesthesia providers. Specifically, single ventricle lesions offer anatomic and physiologic concerns during the perioperative period. Single ventricle physiology represents a delicate balance between systemic and pulmonary blood flow. Any alterations in blood flow through these systems can produce undesirable hemodynamic changes, especially during the perioperative period. We present a case of an adult patient with a single left ventricle who presented for laparoscopic total colectomy due to inflammatory bowel disease. His abnormal anatomy coupled with the hemodynamic disruptions caused by laparoscopy presented significant anesthetic challenges. We highlight the anesthetic concerns of single ventricle physiology, specifically pertaining to laparoscopic surgery. We provide recommendations for safely managing these patients perioperatively. With detailed preoperative evaluation and close hemodynamic monitoring during the perioperative period, these patients can experience successful surgical and anesthetic outcomes.

Modified Blalock Taussig shunt: Comparison between neonates, infants and older children.

Objective: The aim was to compare various pre-and post-operative parameters and to identify the predictors of mortality in neonates, infants, and older children undergoing Modifi ed Blalock Taussig shunt (MBTS). Materials and Methods: Medical records of 134 children who underwent MBTS over a period of 2 years through thoracotomy were reviewed. Children were divided into three groups-neonates, infants, and older children. For analysis, various pre-and post-operative variables were recorded, including complications and mortality. Results: The increase in PaO2 and SaO2 levels after surgery was similar and statistically signifi cant in all the three groups. The requirement of adrenaline, duration of ventilation and mortality was signifi cantly higher in neonates. The overall mortality and infant mortality was 4.5% and 8%, respectively. Conclusion: Neonates are at increased risk of complications and mortality compared with older children. Age (<30 days), weight (<3 kg), packed red blood cells transfusion >6 ml/kg, mechanical ventilation >24 h and post shunt increase in PaO2 (PDiff) <25% of baseline PaO2 are independent predictors of mortality in children undergoing MBTS.

vitro flow visualization of the pulmonary circulation

Flow field of the pulmonary circulation has been investigated by in vitro pulsatile and steady flow visualization in simulation models. A couple of counter-rotating secondary flows were symmetric about the centerline in the normal valve. As the pulmonic valve became more stenotic, the two counter-rotating secondary flows in both the left pulmonary arteries [LPA] and right pulmonary arteries [RPA] were no longer symmetric. With a normal Hancock porcine aortic valve inside the extracardiac conduit, the flow of the proximal conduit was spiral, and that of the distal portion was axial. In stenosed Hancock porcine aortic valve loaded conduit, the flow was a continuous spiral. Studies on cavopulmonary connection models showed that energy savings were more evident at the 50:50 right / left pulmonary artery ratio, and the energy losses increased in proportion to total flow rates. A 60° to 90° anastomotic angle between the subclavian artery and the graft of Blalock-Taussig shunt could result in favorable pulmonary artery flow distribution and peak pressure. Simulations in the Norwood circulation model showed that larger shunts rendered an increased cardiac output to the lungs. In order to determine the idealistic cardiac surgical technical conditions, in vitro flow visualization study is a primarily useful tool in optimizing the flow and diminishing the energy losses

Right Ventricle Exclusion in Severe Neonatal Ebstein's Anomaly / 대한흉부외과학회지

A one-day-old baby was transferred for cyanosis and heart murmur. Echocardiographic evaluation revealed that he had severe neonatal Ebstein's anomaly (Carpentier type C), pulmonary atresia, and pulmonary circulation via patent ductus arteriosus. Because the wall of the atrialized right ventricle was very thin, showed decreased contractility, and the small right ventricle showed pulmonary atresia, we decided that a two-ventricular repair was impossible. When the patient was one-month-old, he underwent right atrium reduction-plasty, a right ventricular exclusion procedure (including atrialized right ventricle resection and functional right ventricle plication), and right modified Blalock-Taussig shunt. He was discharged without specific problems. He received a bidirectional cavopulmonary shunt successfully at 4 months later.

Outcomes of ventricular septal defect repair in various anomalies: a single institute experience and literature review

This is a single institute retrospective study [from 2007 to 2008] to evaluate how patient related factors [age, weight] and type of anatomical location of ventricular septal defect [VSD] could affect the outcome of surgery. Patients with any diagnosis who had undergone ventricular septal defect repair [a total of 25 patients] from 2007 till early 2008 at our center were evaluated retrospectively for factors which might be responsible for developing residual ventricular septal defects and heart block. Data were analyzed through univariate and multivariate analysis. There was 2 deaths among 252 patients [0.8%]. The incidence of postoperative residual ventricular septal defect was 28.2% +/- 2.8* [71 of 252], but only 3 of them [4.2%] needed reoperation. Neither patch material [p=0.572], nor type [p=0.349] or size [p=0.599] of ventricular septal defect had any effect on this complication. The mean age and weight of patients who had residual ventricular septal defect compared to those who did not were not significantly different, although they were somewhat lower [4.7 +/- 0.7 vs. 5.2 +/- 0.4 years, p=0.537; and 15.4 +/- 1.7 vs. 17.9 +/- 1.1 kg, p=0.222, respectively]. There were five patients [2.05] with postoperative complete heart block [CHB] and again this was independent of the patients' age, weight and surgical approach [transatrial or transventricular]. Patients with history of previous Blalock-Taussig [BT] shunt proved to have postoperative bleeding more commonly [13%, 6 of 46 patients] than patients who had not [3.4%, or 7 of 206 patients, p=0.009]. Also in patients with a history of BT shunt compared to those without it, postoperative pericardial effusion [6.5% versus 1.5%, P=0.04] and pneumonia [4.35 versus 0.5%, P=0.025] were more common. It seems that for VSD repair, there are no limitations such as wight or age to proceed with the definitive surgery. Also the incidence of complications is independent of the type of anomaly or approaches for closing the defect. Finally, BT shunt has its own complications which are neither rare nor minor, so it is advisable to proceed with the definitive surgery at the first time to avoid the complications associated with BT shunt

Clinical features and results of recent neonatal cardiac surgery: A review of 82 cases in one hospital / 소아과

PURPOSE: The purpose of this study was to investigate the clinical features and outcome in newborns undergoing cardiac surgery. METHODS: Eighty two neonates underwent heart surgery for congenital heart defect at Kyungpook National University Hospital between March 2000 and February 2006. Patient characteristics (sex, age, diagnosis), pre-operative conditions, operation type, postoperative complications and mortality were reviewed retrospectively. RESULTS: In 82 patients, 41 (50%) were male. The mean age and weight at operation were 12 days and 3.2 kg, respectively. The common cardiac anomalies were complete transposition of the great arteries (TGA), Tetralogy of Fallot (TOF), pulmonary atresia with intact ventricular septum, and single ventricle variants. Fifty seven operations were performed with cardiopulmonary bypass and corrective surgery was done on 54 patients. Arterial switch operation and modified Blalock-Taussig shunt were most frequently performed as corrective and palliative operations, respectively. The early hospital mortality rate was 7%; the late mortality was 3.9%. Complications were acute renal insufficiency, delayed sternal closure, wound infection, arrhythmia, and brain hemorrhage. CONCLUSION: During the last 6 years, the outcomes of cardiac surgery for congenital heart defects in neonates improved by progress in perioperative, anaesthetic, surgical, and postoperative care.

Risk Factor Analysis for SaO2 Instability after Systemic-pulmonary Shunt / 대한흉부외과학회지

BACKGROUND: Arterial oxygen saturation (SaO2) instability frequently takes place after systemic-pulmonary shunt without shunt occlusion. We analyzed actual incidence and risk factors for SaO2 instability after shunt operations, and possible mechanisms were speculated on. MATERIAL AND METHOD: Ninety three patients, who underwent modified Blalock-Taussig shunt from January 1996 to December 2000, were enrolled in this study. Adequacy of shunt was verified in all patients, either by ensuing one ventricle or biventricular repair later on or by appropriate pulmonary artery growth on postoperative angiogram. Age, body weight, hemoglobin level at operation were 3 day to 36 years (median: 1.8 months), 2.5 kg to 51 kg (median: 4.1 kg) and 10.7~24.3 gm/dL (median: 15.2 gm/dL) respectively. Preoperative diagnoses were functional single ventricle with pulmonary stenosis or atresia in 39, tetralogy of Fallot in 38 and pulmonary atresia with intact ventricular septum in 16. Pulmonary blood flow (PBF) was maintained pre-operatively by patent ductus or previous shunt in 64 and by forward flow through stenotic right ventricular outflow tract (RVOT) in 29. SaO2 instability was defined as SaO2 less than 50 % for more than 1 hour with neither anatomic obstruction of shunt nor respiratory problem. RESULT: 10 patients (10.7%) showed SaO2 instability after shunt operation. After shunt occlusion was ruled out by echocardiogram, they received measures to lower pulmonary vascular resistance (PVR), which worked within a few hours in all patients. Risk factors for SaO2 instability included older age at operation (p=0.039), lower preoperative SaO2 (p=0.0001) and emergency operation (p=0.001). PBF through stenotic RVOT showed marginal statistical significance (p=0.065). CONCLUSION: SaO2 instability occurs frequently after shunt operation, especially in patients with severe hypoxia pre-operatively or unstable clinical condition necessitating emergency operation. Temporary elevation of pulmonary vascular resistance is a possible mechanism in this specific clinical setting.

Modified Blalock-Taussig Shunt for the Patients with Complex Congenital Heart Defects in Early Infancy / 대한흉부외과학회지

BACKGROUND: This retrospective review examines the preoperative condition, postoperative course, mortality and cause of death for the patients who underwent modified Blalock-Taussig shunt for complex congenital heart defects in early infancy. MATERIALS AND METHOD: Fifty eight patients underwent modified Blalock-Taussig shunts from January 2000 to November 2003. The mean age at operation was 23.1+/-16.2 days (5~81 days), and the mean body weight was 3.4+/-0.7 kg (2.1~4.3 kg). Indications for surgery were pulmonary atresia with ventricular septal defect in 12 cases, pulmonary atresia with intact ventricular septum in 17, single ventricle (SV) in 18, and hypoplastic left heart syndrome (HLHS) in 11. Total anomalous pulmonary venous return (TAPVR) was associated with SV in 4 cases. RESULT: There were 11 (19.0%) early, and 5 (10.6%) late deaths. Causes of early death included low cardiac output in 9, arrhythmia in 1, and multiorgan failure in 1. Late deaths resulted from pneumonia in 2, hypoxia in 1, and sepsis in 1. Risk factors influencing mortality were preoperative pulmonary hypertension, metabolic acidosis, use of cardiopulmonary bypass, HLHS and TAPVR. Twenty four patients (41.4%) had hemodynamic instability during the 48 postoperative-hours. Six patients underwent shunt revision for occlusion, and 1 shunt division for pulmonary overflow. CONCLUSION: Modified Blalock-Taussig shunt for complex congenital heart defects in early infancy had satisfactory results except in high risk groups. Many patients had early postoperative hemodynamic instability, which means that continuous close observation and management are mandatory in this period. Aggressive management may appear warranted based on understanding of hemodynamic changes for high risk groups.

A Method of Preventing Perigraft Leak from a Polytetrafluoroethylene Blalock-Taussig Shunt

PURPOSE: Perigraft seroma and excessive fluid leaks are well known complications after the use of expanded polytetrafluoroethylene(ePTFE, Gore-Tex(R)) graft. The purpose of this study is to evaluate the effectiveness of precoating the grafts with fibrin glue in the patients underwent a modified Blalock-Taussig shunt. METHODS: Among 51 consecutive infants underwent modified Blalock-Taussig shunt between June 2000 and August 2003, 49 patients underwent 53 shunt procedures through thoracotomy were included for analysis. Their median age was 0.9 months(2 days-5.9 months), median body weight was 3.5(2.2-7.9) kg. The ePTFE grafts precoated with fibrin glue were used in 20 procedures. Perioperative variables of the precoated group were compared with those of a control group(n=33) using t-test and Fisher's exact test. RESULTS: There was no operative mortality in the both groups. No significant difference between the groups could be found in the preoperative variables, postoperative morbidity, and the incidence of perigraft seroma, which developed only in 1 case in the control group. The size of the grafts was significantly smaller in the precoated group(P<0.01). The total amount of pleural drainage per body weight of the precoated group was significantly less than that of the control group(19.7+/-25.2 versus 5.5+/-4.9 mL/kg; P<0.01). CONCLUSION: Precoating an ePTFE graft with fibrin glue may reduce perigraft leak after a modified Blalock-Taussig shunt, although the impact of the size of the graft used and the native pulmonary artery requires further investigation.

Mid-term Results of Neonatal Surgical Management of Pulmonary Atresia with Intact Ventricular Septum / 대한흉부외과학회지

BACKGROUND: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. MATERIAL AND METHOD: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. RESULT: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8+/-1.50 (-3.2~1.2) and -2.5+/-0.5 (-3~-2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. CONCLUSION: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.