Determinants of Advanced Liver Fibrosis in Adult Patients After Fontan Palliation: Usefulness of Ultrasound Transient Elastography.

BACKGROUND: Fontan-associated liver disease is an increasing concern. Our aim was to assess prevalence and predictors of advanced liver fibrosis with a specific focus on utility of liver stiffness measurement by ultrasound transient elastography. METHODS: A total of 97 adult Fontan patients (55% male, median age: 23.1 years, interquartile range [IQR]: 18.7-30.6); 92 (95%) were evaluated with transient elastography, and 50 (52%) underwent transjugular liver biopsy. Advanced liver fibrosis was defined as congestive hepatic fibrosis score 3 or 4. RESULTS: Only 4 patients (4%) had liver stiffness values < 10 kilopascal (kPa). Liver-stiffness measurements correlated weakly with peak oxygen uptake on exercise testing and Fontan pressure but not with Model for End-Stage Liver Disease excluding INR (MELD-XI) score or spleen size. Serial follow-up liver stiffness measurements in 73 clinically stable patients showed large variability among individual patients. Advanced liver fibrosis was present in 35 of 50 (70%) patients on liver biopsy and was associated to MELD-XI-Score ≥ 11 and splenomegaly but not to liver-stiffness measurements. Advanced liver fibrosis was not associated with patient age or time since Fontan operation but with younger age at completion of Fontan (3.7 years, IQR: 2.3-6.3 vs 6.8 years; IQR: 3.5-12.1; P = 0.037). CONCLUSIONS: In our cohort, advanced liver fibrosis was present in the majority of adult Fontan patients. Liver stiffness as measured by transient elastography was not associated with the degree of liver fibrosis. Because of its high variability on serial measurements, it seems not to be useful for clinical decision making. The unexpected finding that younger age at completion of Fontan was associated with advanced liver fibrosis merits further evaluation.

High Variability of Right Ventricular Volumes and Function in Adults with Severe Pulmonary Regurgitation Late After Tetralogy of Fallot Repair.

Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventricular ejection fraction (RVEF) in asymptomatic adults with repaired tetralogy of Fallot, with native right ventricular outflow tract and severe pulmonary regurgitation by serial cardiac magnetic resonance imaging (CMR). The study included 23 asymptomatic adults who underwent ≥3 CMR studies (total of 88 CMR studies). We compared changes in RVEDVi and RVEF between first and last study (median follow-up: 8.8 years, interquartile range: 6.3 to 13.1 years) and between all study pairs. Variability of measurements between study pairs (65 consecutive and 139 nonconsecutive CMR study pairs) were assessed using Bland-Altman analysis and intraclass correlation coefficients. On average, there were no significant changes of RVEDVi or RVEF over the study period (change in RVEDVi: +0.4 ± 17.8 ml/m2, change in RVEF: -1.0 ± 5.5%). Assessment of variability of measurements between study pairs demonstrated no systematic change in RVEDVi and RVEF between study pairs with limits of agreement within the range of previously published studies (RVEDVi -29.1 to +27.2 ml/m2; RVEF -11.5% to 10.2%). High intraclass correlation coefficients for RVEDVi (0.943, 95% CI 0.906 to 0.965, p <0.001) and RVEF (0.815, 95% CI 0.697 to 0.887, p <0.0001) indicate high reliability of reported measurements. In conclusion, in asymptomatic adults with repaired tetralogy of Fallot with native right ventricular outflow tracts and severe pulmonary regurgitation, CMR measurements of RV volumes and RVEF remain stable during follow-up with variability between CMR studies in individual patients, as expected for interobserver and interstudy variability. Measurements derived from a single CMR study or changes occurring between 2 CMR studies should be used with caution for clinical decision-making.

Arrhythmic Burden of Adult Survivors With Repaired Total Anomalous Pulmonary Venous Connection.

Background: The long-term outcome of adults with repaired total anomalous pulmonary venous connection (TAPVC) is poorly documented. Therefore, the present study aims to provide current clinical data on adult survivors with repaired TAPVC focusing on arrhythmia. Methods: Clinical and imaging data (prevalence and type of arrhythmias, symptoms, surgical and medical treatment, echocardiographic and cardiac magnetic resonance haemodynamic parameters) were retrospectively collected from 8 European centres and compared between patients with and without arrhythmias. Results: Fifty-seven patients were included (age 20 [16-67] years [female 28, 49%]). At the last follow-up, that is, 21 (8-51) years after surgery, 79% and 93% of patients were free of symptoms and cardiac medication, respectively. The prevalence of late arrhythmias was 21%; 9 (16%) patients showed intra-atrial re-entrant tachycardia (IART) and 2 (4%) ventricular arrhythmias. Patients with IART were older (P = 0.018) and 4 (7%) required antiarrhythmic medication. Three patients (5%) underwent an electrophysiological study, and another 3 (5%) underwent pacemaker implantation within 36 months after surgical correction, which were removed in 2 patients after 7 years. Early postoperative arrhythmias (P = 0.005), right ventricular dilatation (P = 0.003), and valvulopathy (P = 0.009) were more often present in patients with late IART. Conclusions: Adult survivors after isolated-TAPVC repair presented a high prevalence of arrhythmias. Age, right ventricular dilatation, early arrhythmias, and valvular lesions are risk factors for IART. Long-term follow-up is important as some of these currently asymptomatic patients will probably develop arrhythmias in the future.

Contexte: Les résultats de santé à long terme chez les adultes ayant subi la correction d'un retour veineux pulmonaire anormal total (RVPAT) sont mal connus. Notre étude vise donc à recueillir des données cli-niques au sujet des patients ayant subi cette intervention et ayant survécu jusqu'à l'âge adulte, en particulier pour ce qui est des arythmies. Méthodologie: Les données d'observation clinique et d'imagerie (la prévalence et le type d'arythmies, les symptômes, les traitements chirurgicaux et médicaux, et les paramètres hémodynamiques obtenus par échographie et par résonance magnétique cardiaque) ont été recueillies de façon rétrospective dans huit centres européens et comparées selon que les patients présentaient ou non une arythmie. Résultats: Cinquante-sept patients ont été retenus (âge médian : 20 [16-67] ans; 28 [49 %] femmes). Au dernier suivi, soit 21 (8-51) ans après l'intervention chirurgicale, 79 % des patients ne présentaient pas de symptômes et 93 % des patients ne prenaient pas de médicaments pour des troubles cardiaques. La prévalence d'arythmies tardives s'élevait à 21 %; neuf patients (16 %) présentaient une tachycardie par réentrée intra-atriale (TRIA) et deux patients (4 %) présentaient des arythmies ventriculaires. Les patients qui présentaient une TRIA étaient plus âgés (P = 0,018) et quatre d'entre eux (7 %) devaient prendre des médicaments antiarythmiques. Trois patients (5 %) avaient subi des études électrophysiologiques et trois autres patients (5 %) avaient subi l'implantation d'un stimulateur cardiaque au cours des 36 mois suivant la correction chirurgicale; le stimulateur cardiaque a été retiré sept ans plus tard dans deux de ces cas. Les arythmies postopératoires précoces (P = 0,005), la dilation du ventricule droit (P = 0,003) et la valvulopathie (P = 0,009) étaient plus fréquentes chez les patients qui présentaient une TRIA tardive. Conclusions: La prévalence d'arythmies chez les patients survivant jusqu'à l'âge adulte après la correction isolée d'un RVPAT était élevée. L'âge, la dilation du ventricule droit, les arythmies précoces et les lésions valvulaires sont des facteurs de risque de TRIA. Il est important d'effectuer un suivi à long terme des patients ayant subi une RVPAT puisque certains d'entre eux, asymptomatiques pour le moment, présenteront sans doute des arythmies dans les années à venir.

Recommendations for advance care planning in adults with congenital heart disease: a position paper from the ESC Working Group of Adult Congenital Heart Disease, the Association of Cardiovascular Nursing and Allied Professions (ACNAP), the European Association for Palliative Care (EAPC), and the International Society for Adult Congenital Heart Disease (ISACHD).

Survival prospects in adults with congenital heart disease (CHD), although improved in recent decades, still remain below expectations for the general population. Patients and their loved ones benefit from preparation for both unexpected and predictable deaths, sometimes preceded by a prolonged period of declining health. Hence, advance care planning (ACP) is an integral part of comprehensive care for adults with CHD. This position paper summarizes evidence regarding benefits of and patients' preferences for ACP and provides practical advice regarding the implementation of ACP processes within clinical adult CHD practice. We suggest that ACP be delivered as a structured process across different stages, with content dependent upon the anticipated disease progression. We acknowledge potential barriers to initiate ACP discussions and emphasize the importance of a sensitive and situation-specific communication style. Conclusions presented in this article reflect agreed expert opinions and include both patient and provider perspectives.

Myocardial blood flow and cardiac sympathetic innervation in young adults late after arterial switch operation for transposition of the great arteries.

BACKGROUND: The arterial switch operation (ASO) for repair of transposition of the great arteries (TGA) requires transection of the great arterial trunks and re-implantation of the coronary arteries into the neoaortic root resulting in cardiac sympathetic denervation which may affect myocardial blood flow (MBF) regulation. The aims of the present study were to evaluate sympathetic (re-)innervation in young adults after ASO and its impact on MBF. METHODS: Twelve patients (age 22.5 ±â€¯2.6 years) after ASO for TGA in the neonatal period and ten healthy controls (age 22.0 ±â€¯1.7 years) were included. Positron emission tomography (PET) was used for measuring cardiac sympathetic innervation with [11C]meta-hydroxyephedrine (mHED) and MBF with [15O]H2O PET at rest, during adenosine stimulation, and during sympathetic stimulation with cold pressor test. Cold pressor-induced MBF response capacity was calculated as maximal global MBF over peak rate-pressure product multiplied by 10'000. RESULTS: Global [11C]mHED uptake was significantly lower in patients compared to controls (7.0 ±â€¯2.3 versus 11.8 ±â€¯2.1%/min, p < 0.001). Global MBF was lower in patients compared to controls at rest and during adenosine-induced hyperemia (0.66 ±â€¯0.08 versus 0.82 ±â€¯0.15 ml/min/g, p = 0.005; 2.23 ±â€¯1.19 versus 3.36 ±â€¯1.04 ml/min/g, p = 0.030, respectively). Interestingly, MBF during cold pressor test did not differ between patients and controls (0.99 ±â€¯0.20 versus 1.07 ±â€¯0.16 ml/min/g, p = 0.330). However, cold pressor-induced MBF response capacity was significantly lower for patients as compared to controls (1.09 ±â€¯0.35 versus 1.44 ±â€¯0.39 ml/g/10,000 mmHg, p = 0.040). CONCLUSIONS: With only partial sympathetic re-innervation of the coronary arteries, maximal dilator capacity of the coronary microvasculature and cold pressor-induced MBF response capacity remain substantially impaired in young adults after ASO compared to healthy controls.

State-of-the-art multimodality approach to assist ablations in complex anatomies-From 3D printing to virtual reality.

Imaging of the heart anatomy plays an important role, especially in catheter ablation for the treatment of arrhythmias in adults with congenital heart disease (ACHD). We present a comprehensive overview of the current state-of-the-art modalities available to plan and guide catheter ablation in an ACHD patient. In addition to the clinical assessment of the computed tomography and the integration of 3D reconstructions into the electroanatomical mapping system, 3D printing and virtual reality assessment showed its value in preprocedural planning of the intervention.

Interrupted right-sided aortic arch: performance of umbilical xenograft after primary neonatal corrective surgery.

Interrupted aortic arch (IAA) associated with right-sided aorta descendens is a rare congenital malformation. We present a case of a female patient who was diagnosed with type B IAA and received primary corrective surgery as a neonate. A bovine umbilical vascular graft was used to establish aortic arch continuity. Thirty-nine years later the same patient presents with graft-dilatation and a distal aneurysm requiring a surgery. This case shows a late complication of a biological xenograft in term of long-term material performance. Moreover, it highlights the necessity for regular follow-ups in order to detect and treat late onset complications respectively.

The 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery: week in review - adults with CHD.

For the first time in 2017 adults with CHD was included in the World Congress of Pediatric Cardiology & Cardiac Surgery. With growing numbers and more complex patients reaching adulthood, there is a growing need for more attention to this subspecialty. Although survival is excellent and now over 90% of patients reach adulthood, many have residual problems and complications. Heart failure and arrhythmias are most commonly encountered. Life-expectancy is nearly normal for mild lesions, but remains reduced in moderate-complex lesions and re-interventions are often needed. As most patients want to live a normal life, sports participation and pregnancy become very important issues. Finally, although innovative treatments are being developed, including for end-stage heart failure, we have to refine strategies for optimal care, including during the end-stage lives of our patients. This article provides an overview of a selection of topics in the field of adults with CHD presented during the 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery (WCPCCS) in Barcelona.

Case Report: Emergency awake craniotomy for cerebral abscess in a patient with unrepaired cyanotic congenital heart disease.

We report the case of a 39-year-old male with complex cyanotic congenital heart disease undergoing emergency craniotomy for a cerebral abscess. Maintenance of intraoperative hemodynamic stability and adequate tissue oxygenation during anesthesia may be challenging in patients with cyanotic congenital heart disease. In this case, we decided to perform the surgery as an awake craniotomy after interdisciplinary consensus. We discuss general aspects of anesthetic management during awake craniotomy and specific concerns in the perioperative care of patients with congenital heart disease.

Increasing mortality burden among adults with complex congenital heart disease.

BACKGROUND: Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. METHODS: Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. RESULTS: Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. CONCLUSIONS: Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.

Inestabilidad posterior en edad pediátrica: abordaje artroscópico / Posterior instability in pediatric age: arthroscopic approach

Presentamos una inestabilidad posterior pura post traumática, en un paciente de 14 años. Luego de discutir el examen físico y la paraclínica se decide realizar la estabilización artroscópica, posteriormente a la rehabilitación presente un excelente resultado al año de seguimiento. Esta es una patología rara a esta edad y muchas veces se duda ante la idea de realizar intervenciones en estos pacientes. La cirugía artroscópica permite un correcto diagnostico y tratamiento de estos pacientes.

We present a 14 years old patient, who has a pure traumatic posterior shoulder instability. After discussing the physical examination and paraclinical we decided to perform an arthroscopic stabilization, with subsequent rehabilitation. He had an excellent outcome at one year follow up. This is a rare pathology at this age and usually there is doubt about the idea of a surgical intervention. Arthroscopic surgery allows an accurate diagnosis and treatment for these patients.

Osteotomía de apertura anterior de tibia bilateral en recurvatum de rodilla: a propósito de un caso / Anterior tibial osteotomy in bilateral knee recurvatum. Case report

En los recurvatum de rodilla, existen varios métodos de corrección, la osteotomía descripta por Brett y Lexer E. modificada es una opción para su tratamiento. El objetivo de este trabajo es describir la técnica como una solución para los recurvatum de rodilla tibiales y mostrar la evolución de un caso clínico. El paciente presentaba un recurvatum bilateral de rodilla de origen tibial idiopático, con valores angulares de 26 grados a derecha y 20 grados a izquierda de ángulo diafiso-epifisario. Con el objetivo de corregir la deformación para calmar el dolor de características mecánicas por sobrecarga articular y evitar la pérdida del equilibrio articular, se decidió realizar esta técnica. Las cirugías fueron realizadas comenzando con la derecha y un año y medio después la rodilla izquierda. Para la rodilla derecha se logró corregir la deformidad llevando el platillo tibial de 26° de inclinación anterior a 6°. En cuanto a la rodilla izquierda de 20° se corrigió a 0°. Resulta una técnica reproducible, que soluciona los recurvatum de origen tibial epifiso-metafisarios, que no altera la biomecánica del aparato extensor como otras osteotomías. Esta técnica no permite corregir las deformidades diafisarias. Creemos que es una de las mejores opciones para la corrección del recurvatum, que a pesar de no lograr corrección total de la alteración ósea, mejora la función de la rodilla y la sintomatología, sin alterar la mecánica del aparato extensor.

In the knee recurvatum, there are several methods of correction osteotomy, the modified Brett and Lexer E. osteotomy is an option for this type of pathology. The aim of this paper is to describe the technique as a solution to the tibial recurvatum and show the outcome of a case. The patient had a bilateral tibial idiopathic knee recurvatum, with angular values of 26 degrees right and 20 degrees left of diafiso-epiphyseal angle. In order to correct the deformation and release pain secondary to mechanical overload and prevent loss of articular balance, we decided to perform this technique. The surgeries were performed first the right and a year and a half after his left knee. His right knee, we managed to correct the tibial plateau deformity from 26° of anterior tilt, to 6°. As for the left knee was corrected from 20° to 0°. It is a reproducible technique that solves tibial epiphyseal-metaphyseal origin recurvatum, which does not alter the biomechanics of the extensor mechanism as other osteotomies. This technique does not allow correction of diaphyseal deformities. We believe it is one of the best options for the correction of recurvatum, which despite not achieveing full correction of bone alteration improves knee function and symptoms without altering the extensor mechanism.

Facilitators of and barriers to advance care planning in adult congenital heart disease.

BACKGROUND: Most adults with congenital heart disease (CHD) are interested in discussing matters related to advance care planning (ACP) early in the disease course, yet few such conversations actually occur. We aimed to evaluate factors that impact these discussions between patients and adult CHD providers. METHODS: Two hundred adult CHD outpatients completed a survey that included factors that might impact ACP discussions with their doctors. In parallel, forty-eight providers within the Canadian Adult Congenital Heart Network completed a similar online survey. Responses were compared between the groups. RESULTS: Most providers (85%) worried that they were unable to reliably estimate life expectancy and believed that patients were not ready for end-of-life discussions if their estimated life expectancies were beyond 5 years (63%) or beyond 10 years (79%). In contrast, only 24% of patients, independent of disease complexity, thought they were not ready to talk about ACP. Most providers (83%) reported that greater certainty about patients' prognoses would help them discuss ACP. Patients thought that such discussions were best facilitated when they had trust in their doctors (85%) and believed their doctors are good at taking care of patients with CHD (78%). CONCLUSION: Despite the fact that challenges to prognostication exist, discussions about ACP should not be reserved for patients with a severely reduced life expectancy. Most patients want these discussions regardless of the complexity of their disease. The trusting and close patient-doctor relationship in adult CHD, often evolving over many years, may provide an excellent platform from which to initiate such discussions.

Fracturas de eminencia intercondílea tibial: tratamiento artroscópico / Tibial intercondylar eminence fractures: arthroscopic treatment

El término “fracturas de eminencia inter-condilea” parece ser más conveniente ya que el sector comprometido no son las espinas tibiales en si, sino la superficie pre-espinosa donde se encuentra la inserción del ligamento cruzado anterior. En 1959, Meyers y Mc Keever, en un intento de clasificar las fracturas para aportar elementos en la toma de decisiones para su manejo, propusieron tres tipos de fracturas de la espina tibial basados en el grado de desplazamiento de la misma. En cuanto al manejo de las fracturas no desplazadas tipo I y II, la evidencia disponible actualmente menciona que el tratamiento cerrado presenta buenos resultados. En los tipo III, el consenso actual recomienda el manejo quirurgico, ya sea de forma abierta o artroscópica, mediante fijación con tornillos o mediante la técnica de pull cut. El objetivo principal de este trabajo es mostrar una técnica de reducción y fijación de estas fracturas, guiado por artroscopía, utilizando como ejemplo un caso clínico. Pensamos que es el tratamiento ideal para reducción y fijación de las fracturas de eminencia tibial tipo II en adelante, incluso las tipo III A y B.

Cardiac outcomes in adults with supravalvar aortic stenosis.

AIMS: Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis. METHODS AND RESULTS: This is a multicentre retrospective study of cardiac outcomes in adults (≥18 years) with supravalvar aortic stenosis. We examined: (i) adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and (ii) the need for cardiac surgery in adulthood. One hundred and thirteen adults (median age at first visit 19 years; 55% with Williams-Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams-Beuren syndrome had more severe supravalvar aortic stenosis and more often associated left ventricular outflow tract obstructions (P < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams-Beuren syndrome. Eighty-five per cent of adults (96/113) had serial follow-up information (median follow-up 6.0 years). Of these patients, 13% (12/96) had an adverse cardiac event and 13% (12/96) had cardiac operations (7 valve repair or replacements, 4 supravalvar aortic stenosis repairs, 1 other). Cardiac surgery was more common in adults without Williams-Beuren syndrome (P = 0.007). Progression of supravalvar aortic stenosis during adulthood was rare. CONCLUSION: Adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperations, while progression of supravalvar aortic stenosis in adulthood is rare. Valve surgery is the most common indication for cardiac surgery in adulthood.

Supportive and palliative care for adults dying from congenital heart defect.

PURPOSE OF REVIEW: Adult survivors with congenital heart disease (CHD) are a novel group of adults with chronic disease. Due to the nature of their surgical repair, the majority is not cured. This review focuses on changing demographics and mortality of adults with CHD, available knowledge and data on end-of-life (EOL) and palliative care in this patient group and outlines needs for future clinical practice and research. RECENT FINDINGS: Recent findings underscore that survival to adulthood has become the rule for the majority of patients born with CHD. Many remain at increased risk of premature death from their disease. Chronic circulatory failure has been identified as one of the leading causes of death and, thus, patients may benefit from incorporation of palliative care principles in their management. Recent studies show that this practice has not yet been widely adopted. Nonetheless a survey of adult outpatients has shown that many patients prefer to be involved in EOL decisions early in the course of their disease. SUMMARY: More research is needed to learn how we can best implement principles of palliative care in the growing population of adult survivors with CHD. This includes improved communication about EOL issues and a more comprehensive approach for symptom control.

Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries.

OBJECTIVES: We sought to determine cardiac outcomes in young adults with complete transposition of the great arteries (TGA) after the arterial switch operation (ASO). BACKGROUND: Although cardiac outcomes in the pediatric population with TGA after ASO have been well described, outcomes in the adult population have not to our knowledge been studied. METHODS: We determined late survival in all operative survivors with TGA after ASO performed before 1991 at our local pediatric referring hospital. In the subset of adults (n = 65) followed in our adult congenital cardiac clinic, we examined cardiac outcomes in adulthood. RESULTS: Survival of the 132 infants discharged from hospital after ASO was 97% (70% confidence interval [CI]: 95.0% to 98.1%) at 20 years. In the 65 patients (mean age 21 +/- 3 years, 62% male) followed at our institution, 17% (11 of 65) had at least 1 clinically significant cardiac lesion, including ventricular dysfunction, valvular dysfunction, or arrhythmias. Residual lesions were more common in those who had had cardiac reinterventions in childhood (odds ratio: 10.7, 95% CI: 2.1 to 55). In adulthood, 5 patients (8%) had arrhythmia requiring treatment and 7 patients (11%) required reinterventions (5 reoperations and 2 pacemaker implantations). Intervention for aortic valve regurgitation and aortic root dilation were not observed. Exercise capacity was reduced in most adults (82%) after ASO. CONCLUSIONS: Although most adults after ASO are well, and few have residual defects, there are subgroups, particularly those who needed further cardiac intervention in childhood, who are at higher risk for ventricular and valve dysfunction and arrhythmias.

A reminder from the past: a 49-year-old patient with transposition of the great arteries palliated with Dr. Thomas Baffes' and Dr. William Mustard's original operations.

The authors report the case of a 49-year-old woman with transposition of the great arteries and an unusual surgical history. At the time she was born, most patients with this condition did not survive into adulthood. This patient was palliated with the Baffes procedure and subsequently with the modified Mustard operation. Both operations were performed by their inventors and have allowed her to live a normal life for almost half a century. This unusual case is illustrated with a series of echocardiographic and cardiac computed tomographic images.

Artroplastia de rodilla postosteotomia: revision casuistica banco de protesis 1986-2001 / Total knee replacement after osteotomy: revision between 1986 to 2001

Introduccion: Las artroplastias postosteotomia son cirugias de mayor complejidad, por presentar cicatrices previas, defectos oseos y un aparato extensor modificado. Material y metodos: Se evaluaron 2630 artroplastias entre 1986 y 2001 (6 por ciento de las cirugias). Valguizantes 142 y varizantes 11,6. Seguimiento promedio de 5,3 años. Se realizo evaluacion con protocolo y rx, utilizando el score del Hospital for Special Surgery (HSS). Resultados: El tiempo promedio de sobrevida de las osteotomias fue de 8 años, en un rango de 1 a 30 años. Se realizo, Snip de Insall en 26 casos y osteotomia de Whiteside en 3 casos; 1 alargamiento y 1 liberacion de cuadriceps. El 6,5 por ciento de las cirugias requirio protesis especiales. 78 casos tenian un HSS preoperatorio malo, 68 regular y 4 buenos. 103 casos mostraron un HSS postoperatorio excelente, 28 buenos, 3 regulares y 9 malos. Complicaciones: 11 por ciento, 9 casos de infeccion profunda (5,8 por ciento), 1 aguda y 8 tardias. Necrosis de piel y dificultad de cicatrizacion, 2 casos (1,3 por ciento). Desinsercion tendon rotuliano, 2 casos (1,3 por ciento). Falsa ruta, 2 casos (1,3 por ciento). Aflojamiento aseptico, 2 casos (1,3 por ciento). Conclusiones: Indice de complicaciones similar a las cirugias de revision. Se debe realizar una incision apropiada, asi como elegir el tipo de osteotomia osea a realizar, teniendo en mente la futura eventual artroplastia.