Parallel oxygenators in the same circuit for refractory hypoxemia on veno-venous extracorporeal membrane oxygenation. A 3-patient series.

Extracorporeal membrane oxygenator (ECMO) is a well-established therapy for respiratory failure. Refractory hypoxemia, despite the use of ECMO, remains a challenging problem. The ECMO circuit may not provide enough oxygenation support in the presence of high cardiac output, increased physiologic demand, and impaired gas exchange. Adding a second ECMO oxygenator using the same pump (sometimes needing a second drainage cannula) can improve oxygenation and facilitate lung-protective ventilation in selected patients. We describe a 3-patient series with severe ARDS secondary to SARS-CoV-2 infection and refractory hypoxemia during ECMO support successfully treated with this approach.

Outcomes of a modified, low-cost, veno-arterial extracorporeal membrane oxygenation (V-A ECMO) for elective, periprocedural support of high-risk percutaneous cardiac interventions: An experience from a latinamerican center.

INTRODUCTION: High-risk procedures in interventional cardiology include a wide spectrum of clinical and anatomical scenarios related to a higher periprocedural morbidity and mortality. The prophylactic use of short-term mechanical circulatory support (ST-MCS) may improve both the safety and efficacy of the intervention by leading to more stable procedural hemodynamics. However, the significant costs may limit its use in resource constrained settings. To overcome this limitation, we ideated a modified, low-cost, veno-arterial extracorporeal membrane oxygenator (V-A ECMO) setup. METHODS: We conducted an observational prospective study including all patients undergoing a high-risk interventional cardiology procedure at our institution under prophylactic ST-MCS using a modified, low-cost version of V-A ECMO, where some components of the standard V-A ECMO circuit were replaced by supplies used for cardiac surgical cardiopulmonary bypass, achieving a cost reduction of 72%. We assessed in-hospital and mid-term outcomes, including procedural success, post-procedure complications and mortality. RESULTS: Between March 2016 and December 2021, ten patients underwent high-risk IC procedures with prophylactic use of V-A ECMO. Isolated percutaneous intervention (PCI) was performed in six patients, isolated transcatheter aortic valve replacement (TAVR) in two, and a combined procedure (PCI + TAVR) in two. Mean ejection fraction was 34% (range 20-64%). Mean STS PROM was 16.2% (range 9.5-35.8%) and mean EuroScore was 23.7% (range 1.5-60%). The planned intervention was successfully performed in all cases. There were no reports of V-A ECMO malfunction. In nine patients the VA-ECMO was withdrawn immediately after the procedure but one patient required extended - 24 h - support with no significant issues. One patient experienced a periprocedural myocardial infarction and another developed a femoral pseudoaneurysm. In-hospital and 30-day survival were 100%, and 1-year survival was 80%. CONCLUSIONS: High-risk procedures in interventional cardiology can be successfully performed under prophylactic ST-MCS using a modified, low-cost V-A ECMO, suitable for limited-resource settings.

Case report: Multi-site perfusion strategy for type A acute aortic dissection complicated with cerebral malperfusion.

Acute type A dissection presenting with cerebral malperfusion has high morbidity and mortality. Given the complexity of underlying vascular involvement, it is a challenging clinical scenario. Many of these patients are not deemed surgical candidates. If surgery is considered, it often requires complex aortic arch and neck vessel reconstruction. We present a 48-year-old male with an acute type A aortic dissection that presented with paraplegia and decreased level of consciousness. A Computed Tomography showed occlusion of both common carotid arteries. He was successfully treated with a multi-site perfusion strategy and a Hybrid Frozen Elephant Trunk graft to achieve fast restoration of the cerebral circulation and minimize brain ischemia and permanent neurological damage. From this case, we learn that aggressive arch and neck vessel reconstruction supported by multi-site perfusion could help improve mortality and neurological outcomes in selected patients.

Venoarterial Extracorporeal Membrane Oxygenation as A Bridge to Surgery in Post-Myocardial Infarction Ventricular Septal Defect with Cardiogenic Shock: Case Report

ABSTRACT We describe a 60-year-old woman with post-myocardial infarction (MI) ventricular septal defect (VSD) and cardiogenic shock who was successfully stabilized with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge therapy for the surgical closure of her VSD. This case highlights the role of VA-ECMO in the management of post-MI VSD to improve the results of surgical repair and patient survival.

Venoarterial Extracorporeal Membrane Oxygenation as A Bridge to Surgery in Post-Myocardial Infarction Ventricular Septal Defect with Cardiogenic Shock: Case Report.

We describe a 60-year-old woman with post-myocardial infarction (MI) ventricular septal defect (VSD) and cardiogenic shock who was successfully stabilized with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge therapy for the surgical closure of her VSD. This case highlights the role of VA-ECMO in the management of post-MI VSD to improve the results of surgical repair and patient survival.

Cirugía convencional del aneurisma tóraco-abdominal / Open surgical treatment of thoracoabdominal aortic aneurysm. Experience in 45 patients

BACKGROUND: Thoracoabdominal aortic aneurysm (TAAA) is an infrequent disease and demands a highly specialized and experienced management. Open repair (OR) is the gold standard but it is associated with significant morbidity and mortality. Paraplegia and renal failure are the most important complications. AIM: To report our results with OR treatment of TAAA. MATERIAL AND METHODS: Descriptive study including all patients with TAAA operated electively and consecutively by OR between 1983 and 2019. Main outcomes are operative mortality, renal and neurological morbidity, and long-term survival. RESULTS: We report 45 operated patients aged 33 to 84 years, 74% males. Aneurysm extension according to Crawford classification was I in 18%, II in 18 %, III in 36% and IV in 29%. Operative mortality was 4%. The frequency of paraplegia or paraparesis at discharge was 9%. No patient was discharged on hemodialysis. Survival at 5 and 10 years were 60% and 40% respectively. CONCLUSIONS: OR of TAAA is a complex procedure. Our results show perioperative mortality rates comparable to highly experienced centers. Although being a major procedure, OR remains an alternative to treat this serious condition.

Extracorporeal membrane oxygenation for tuberculosis pneumonia with empyema.

Acute respiratory distress syndrome (ARDS) caused by Mycobacterium Tuberculosis (TB) is a rare entity. Extracorporeal membrane oxygenation (ECMO) therapy had been used as an effective therapy for this cases, but the evidence is scarce. We present a case that took place in the middle of SARS-CoV2 pandemic. A 33-year-old female presented with ARDS due to pulmonary TB infection (pneumonia with empyema and pneumothorax), which required invasive mechanical ventilation with poor response. Long term veno-arterio-venous (VAV) ECMO, overlapped with veno-venous ECMO, was used as a salvage therapy with a good response for a total of 26 days. This is an example of the effectiveness of this therapy in this scenario, never described before. The fact that this therapy was used in the middle of SARS-CoV2 pandemic, with limited resources available, was remarkable, but it was encouraged by previous successful experiences.

[Open surgical treatment of thoracoabdominal aortic aneurysm. Experience in 45 patients]. / Cirugía convencional del aneurisma tóraco-abdominal.

BACKGROUND: Thoracoabdominal aortic aneurysm (TAAA) is an infrequent disease and demands a highly specialized and experienced management. Open repair (OR) is the gold standard but it is associated with significant morbidity and mortality. Paraplegia and renal failure are the most important complications. AIM: To report our results with OR treatment of TAAA. MATERIAL AND METHODS: Descriptive study including all patients with TAAA operated electively and consecutively by OR between 1983 and 2019. Main outcomes are operative mortality, renal and neurological morbidity, and long-term survival. RESULTS: We report 45 operated patients aged 33 to 84 years, 74% males. Aneurysm extension according to Crawford classification was I in 18%, II in 18 %, III in 36% and IV in 29%. Operative mortality was 4%. The frequency of paraplegia or paraparesis at discharge was 9%. No patient was discharged on hemodialysis. Survival at 5 and 10 years were 60% and 40% respectively. CONCLUSIONS: OR of TAAA is a complex procedure. Our results show perioperative mortality rates comparable to highly experienced centers. Although being a major procedure, OR remains an alternative to treat this serious condition.

[Results of a national program of pediatric heart transplantation: strengths and weakness]. / Resultados de un programa nacional de trasplante cardiaco pediátrico: fortalezas y debilidades.

Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. OBJECTIVES: To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. PATIENTS AND METHODS: Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality. RESULTS: Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33.3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple association of immunosuppression. One patient died 16 days post transplant due to primary graft failure (7.1%). The average follow-up was 43 months (0.5-159 months). Two patients died later on (82 and 55 months), both due to secondary rejection because of voluntary cessation of immunosuppressive therapy. Survival at 1 and 5 years was 93% and 74%, respectively. CONCLUSIONS: Our program has successfully transplanted 50% of patients enrolled, with good medium-term survival. A significant proportion of patients were listed as a medical emergency and 34.5% died on the waiting list.

Resultados de un programa nacional de trasplante cardiaco pediátrico: fortalezas y debilidades / Results of a national program of pediatric heart transplantation: strengths and weakness

El trasplante cardiaco pediátrico es una terapia efectiva para tratar la insuficiencia cardiaca avanzada. Objetivos: Analizar los resultados inmediatos y a mediano plazo de niños enlistados para trasplante cardiaco. Pacientes y Método: Se analizó el registro de pacientes enlistados para trasplante, entre octubre de 2001 y julio de 2016, analizando datos demográficos, diagnósticos, status de enlistamiento, tiempo de espera, datos de donantes, uso de asistencia ventricular, complicaciones y mortalidad. Resultados: La serie abarca 30 pacientes con edad promedio de 9,4 años (1 mes a 15 años). El diagnóstico principal fue miocardiopatía dilatada en 24 pacientes (80%). El status de ingreso fue I (urgencia) en 19 casos y II (no urgencia) en 11. Fallecieron 10 en la lista de espera (33,3%) en un promedio de 52 días (13 a 139 días). Catorce pacientes fueron trasplantados (46.7%), con un tiempo de espera de 199,6 días (4 a 586 días). Requirieron asistencia ventricular 9 pacientes (30%). Todos recibieron inmunosupresión tri asociada. Un paciente falleció a los 16 días por falla primaria del injerto (7,1%). El seguimiento promedio fue de 43 meses (0,5 a 159 meses). Dos pacientes fallecieron alejadamente (55 y 82 meses) por rechazo secundario al abandono de tratamiento inmunosupresor. La supervivencia a 1 y 5 años fue 93% y 74%, respectivamente. Conclusiones: Nuestro programa ha trasplantado al 50% de los pacientes enlistados con buena supervivencia a mediano plazo. Una proporción significativa de pacientes se enlistó con carácter de urgencia y un 34.5% de los pacientes fallecieron en la lista de espera.

Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. Objectives: To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. Patients and Methods: Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality. Results: Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33.3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple association of immunosuppression. One patient died 16 days post transplant due to primary graft failure (7.1%). The average follow-up was 43 months (0.5-159 months). Two patients died later on (82 and 55 months), both due to secondary rejection because of voluntary cessation of immunosuppressive therapy. Survival at 1 and 5 years was 93% and 74%, respectively. Conclusions: Our program has successfully transplanted 50% of patients enrolled, with good medium-term survival. A significant proportion of patients were listed as a medical emergency and 34.5% died on the waiting list.

Veinte años de reparación de Ia válvula aórtica bicúspide insuficiente / Repair of the regurgitant bicuspid aortic valve: a twenty year experience

Introducción: La Insuficiencia Aórtica (IA) excepcionalmente es susceptible de reparación. Una de estas excepciones es la Válvula Aórtica Bicúspide (VAB). Objetivo: Analizar nuestros resultados de la reparación de la VAB insuficiente. Método: Se revisó la Base de Datos para el período enero 1994 a Julio 2014. Se identificaron 29 pacientes y se revisaron las fichas clínicas y protocolos operatorios. La supervivencia se certificó en el Registro Civil e Identificación de Chile. Resultados: Todos los pacientes fueron hombres. La edad promedio fue 39,4 años (19- 61 años). Cinco pacientes presentaban una endocarditis. El ecocardiograma preoperatorio demostró IA severa en 25 casos (86%) y moderada en 4. El diámetro sis-tólico fue 44 ± 7,1 mm y el diastólico 67,8 ± 6,7 mm. La fracción de acortamiento fue 35,96 ± 5,54%. En todos los casos la VAB presentaba fusión del velo coronariano izquierdo y derecho con rafe medio; en 3, el rafe era incompleto produciéndose un cleft. En 23 casos (79%) la IA era secundaria a prolapso del velo fusionado, en 3 a perforación de velo, en 1 a un cleft y en 2 a perforación y cleft. En 23 casos (79%) se efectuó una resección triangular y en 16 (55%) se complementó con una anuloplastía. En 3 se cerró una perforación y en otros 3 se efectuó un cierre primario de cleft. En 10 casos se realizó un procedimiento asociado. En todos los casos se realizó un ecocardiograma transesofágico intra-operato-rio. En 35% no hubo insuficiencia aórtica residual y en 65% esta fue mínima o leve. No hubo mortalidad operatoria. El seguimiento se completó en el 100%. Dos pacientes (7%) fallecieron por causas no cardiacas. Siete (24%) fueron re-operados, en promedio a los 7,14 años. La media de supervivencia fue 19,3 años (IC95% 17,6-21) y la supervivencia libre de re-operación 15,8 años (IC95% 13-18,7), a 20,6 años de seguimiento. El ecocardiograma efectuado en promedio a los 4,9 años demostró una reducción del diámetro sistólico de 6,15 ± 7,2 mm (p<0,05), del diastólico de 11,26 ± 8,7 mm (p<0,05) y de la fracción de acortamiento de 1,12 ± 5,57% (p<0,33). De los 22 pacientes no reoperados, 9 no tenían IA, en 6 esta era leve (1+) y en 3 leve a moderada (2+); 4 pacientes tenían una estenosis aórtica leve. Conclusión: La reparación quirúrgica de la válvula aortica bicúspide insuficiente tiene baja mortalidad peri-operatoria y excelente supervivencia alejada. Si bien el 24% de los pacientes requirió una re-operación, esta fue tardía en la mayoría de los casos.

Background: Aortic insufficiency (AI) is rarely amenable to surgical repair. One of the exceptions to that statement is the bicuspid aortic valve Aim: to analyze our results in the repair of a regur-gitant bicuspid aortic valve Method: A review of the cardiac surgery data base in the period January 1994 to July 2014 allowed the identification of 29 patients with AI and a bicuspid aortic valve submitted to surgical repair. The data from the clinical record and the surgical report was analyzed. Survival was established from the National Identification Service. Results: all patients were males. Mean age was 39.4 years (range 19-61). Five patients had infective endocarditis. Preoperative echocardiography revealed severe AI in 25 patients (86%) and moderate AI in 4. Left ventricular diastolic and systolic diameters were 67,8 ± 6,7 and 44 ± 7,1 mm, respectively. All patients presented fusion of the left and right leaflets with mid rafhe, and 3 patients had an incomplete rafhe with a cleft. AI was caused by prolapsed fu-sioned leaflet in 23 patients, leaflet perforation in 3, cleft in one and perforation plus cleft in 2 patients. Triangular resection was performed in 23 (79%) and complemented by annuloplasty in 16 (55%) patients. Three patients underwent closure of a perforation and 3 a repair of the cleft. An additional surgical procedure was performed in 10 patients. All patients underwent intra-operative TEE. 35% had no residual AI and 65% had minimal or mild AI. There was no surgical mortality. Follow up was completed in all patients. Two patients (7%) died from non cardiac causes. Seven patients (24%) had to be re-operated on, a mean of 7.1 years after the initial surgery. Mean survival rate was 19.3 (95% CI 17.6-21), and mean survival free from re-operation was 15.8 years (95%CI 13-18.7) at 20.6 years of follow up. Echo-cardiogram performed at a mean of 4.9 years after surgery showed a reduction of LV systolic diameter of 6,15 ± 7,2 mm (p<0,05), LV diastolic diameter of 11,26 ± 8,7 mm (p<0,05) and fractional shortening of 1,12 ± 5,57% (p<0,33). In the group of 22 patients who did not have a re-operation, AI was absent in 9, mild in 6 and moderate in 3; 4 patients had mild aortic stenosis. Conclusion: Surgical repair of the regurgitant bicuspid aortic valve has low peri-operative mortality rate and excellent late survival. Reoperation, required in 24% of patients, occurred late after the initial operation in most cases.

Gastrointestinal neuroendocrine tumor with unresectable liver metastases: an example of multimodal therapeutic approach.

Gastrointestinal neuroendocrine tumors (NET) frequently present with unresectable hepatic metastases, which poses a barrier for curative treatment. Resection of the primary tumor and subsequent orthotopic liver transplantation (OLT) has been proposed as a treatment approach but available data in this regard is limited. We present a clinical case of an otherwise asymptomatic 44-yo man complaining of abdominal pain and dyspepsia that was diagnosed of a 10 cm duodenal tumor with multiple hepatic metastases. A CT-guided biopsy confirmed a NET. He underwent first a Whipple's procedure, and then was listed for liver transplantation. During the waiting time a multimodal therapeutic approach was used including the use of radioactive 177lutetium-labeled somatostatin analogues, long-acting somastostatin analogues and antiangiogenic antibodies (bevacizumab) in order to keep neoplastic disease under control. Two years after Whipple's procedure and given disease stability he underwent OLT with an uneventful postoperative evolution. Patient condition and graft function are optimal after a 4-year follow-up period with no evidence of recurrence. This case report underscores how a multimodal approach involving careful patient selection, resective surgery as well as use of somatostatin analogues and antiangiogenic biological therapy followed by liver transplantation can achieve excellent long-term results in this difficult patient population.

[Esophageal aspergillosis in a patient with acute myelogenous leukemia and febrile neutropenia]. / Aspergilosis esofágica en una paciente con leucemia mieloide aguda y neutropenia febril.

Aspergillosis usually compromises the respiratory system, but can also affect others. We report a 46 yo female with acute myeloid leukemia, developed febrile neutropenia and dysphagia. Endoscopy revealed esophageal cytomegalovirus-like ulcers, but biopsies showed Aspergillus spp. It's important to consider aspergillosis in the differential diagnosis of esophageal lesions in high-risk patients.

[Crigler-Najjar syndrome. Report of one case with a long term follow up]. / Evolución prolongada en síndrome de Crigler-Najjar tipo I.

Crigler-Najjar Syndrome is an uncommon genetic disorder characterized by the elevation of unconjugated plasmatic bilirubin secondary to deficiency of the enzyme uridine diphosphate glucuronyltransferase (UDP-GT). We report a 19-years-old woman with the syndrome diagnosed during the neonatal period, when she developed a severe jaundice in the first 10 days of life, reaching unconjugated bilirubin levels of 29 mg/dl, with normal liver function tests. After transient response to phototherapy, the patient was referred to a tertiary medical center in which an extensive work up ruled out other etiologies and the diagnosis of type I Crigler-Najjar syndrome was established. Currently, the patient has a mild mental retardation. She is receiving homemade phototherapy 18 h per day with acceptable control of bilirubin levels. Many mutations have been associated with UDP-GT dysfunction resulting in a broad spectrum of the disease. When bilirubin rises above physiological limits, it permeates the hematoencephalic barrier, inducing bilirubin impregnation of basal ganglia with secondary neuronal damage and necrosis. The worst outcome, kernicterus, is characterized by mental retardation, central deafness, ophthalmoplegia, ataxia, athetosis, spasticity, seizures and death. First line therapy includes phototherapy, but definitive therapy is liver transplantation before the occurrence of neurological damage.

Evolución prolongada en síndrome de Crigler-Najjar tipo I / Crigler-Najjar syndrome. Report of one case with a long term follow up

Crigler-Najjar Syndrome is an uncommon genetic disorder characterized by the elevation of unconjugated plasmatic bilirubin secondary to deficiency of the enzyme uridine diphosphate glucuronyltransferase (UDP-GT). We report a 19-years-old woman with the syndrome diagnosed during the neonatal period, when she developed a severe jaundice in the first 10 days of life, reaching unconjugated bilirubin levels of 29 mg/dl, with normal liver function tests. After transient response to phototherapy, the patient was referred to a tertiary medical center in which an extensive work up ruled out other etiologies and the diagnosis of type I Crigler-Najjar syndrome was established. Currently, the patient has a mild mental retardation. She is receiving homemade phototherapy 18 h per day with acceptable control of bilirubin levels. Many mutations have been associated with UDP-GT dysfunction resulting in a broad spectrum of the disease. When bilirubin rises above physiological limits, it permeates the hematoencephalic barrier, inducing bilirubin impregnation of basal ganglia with secondary neuronal damage and necrosis. The worst outcome, kernicterus, is characterized by mental retardation, central deafness, ophthalmoplegia, ataxia, athetosis, spasticity, seizures and death. First line therapy includes phototherapy, but definitive therapy is liver transplantation before the occurrence of neurological damage.