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TÍTULO DEL CASO First case of neonatal VA-ECMO with left sided venting in Peru. RESUMEN Hasta 150 palabras resumiendo presentación y resolución del caso We report a first case of neonatal VA-ECMO with left sided venting at the National Cardiovascular Institute, in Lima–Perú. The patient was a 27-days old infant, who underwent surgery for D-TGA (Arterial Switch) and developed cardiogenic shock due to severe left ventricular dysfunction in the postoperative state. Approximately 36 hours after surgery, we implant VA-ECMO with chest cannulation at operating room. An additional cannula was placed in the left atrium because distention of the left heart chambers. Stöckert Centrifugal Pump Console (SCPC, SoringGroup), Revolution centrifugal blood pump (SoringGroup), membrane oxygenator Lilliput 2 ECMO (Sorin Group) was used. Approximately 12 hours after ECMO was successfully initiated, the patient achieved hemodynamic stability and tissue perfusion was normal. After 16 hours of ECMO support, inotropic and vasopressor drugs (dopamine and epinephrine) were withdrawn and received only nitroprusside. The recovery of target organs was possible between the third and fifth day of ECMO support. At the fourth day we began the weaning process, successfully removing the support at the fifth day. The patient developed complications such as chylothorax, left phrenic nerve palsy and intercurrent infection. To this report date (25 days after ECMO explant), he remains at the pediatric intensive care unit, weaning from conventional mechanical ventilation and in improved condition. IMPORTANCIA DEL CASO ¿Porqué es importante este caso? ¿Porqué deseas presentarlo? Is the first successful case of extracorporeal membrane oxygenation with left sided venting in a neonate at our country, who additionally was a patient with corrective surgery of congenital heart disease (D-TGA). PRESENTACIÓN DEL CASO Presentación del paciente, antecedentes medicos/sociales/familiares A 23 days old male infant diagnosed with D-TGA, restrictive atrial septal defect and patent ductus arteriosus was referred to our institution from a general hospital, he was under mechanical ventilation and had not received prostanglandin E1 (Alprostadil) for last 12 hours. The patient was admitted to the pediatric intensive care unit in poor general condition, cyanotic, with pulse oximetry of 44%. Arterial blood gas analysis showed PaO2 28 mmHg, lactate 7 mMol/L, PaCO2 26 mmHg, bicarbonate 15mMol/L, arterial oxygen saturation 57% and hemoglobin 12.2 g/dL. Due to presence of restrictive atrial septal defect, we performed an ultrasound-guided balloon atrial septostomy. We noted insufficient oxygenation improvement (PaO2 33 mmHg and arterial oxygen saturation 60%); with additional and transitory elevation of lactate up to 11.2 mMol/L, bicarbonate at 14 mMol/L, pH 7.21, glucose 212 mg/dL and normal seric electrolytes. His hemoglobin level fell to 7.9 g/dL, which required packed red blood cells transfusion. He also required inotropic support with dopamine up to 10 microgram/kg/min and adrenaline 0.1 micrograms/kg/min, titratable nitroprusside for a mean arterial pressure of 40-45 mmHg, and increased PGE1 to 0.2 micrograms/kg/minute. Later, we noticed improvement in lactate level to 3-4 mmol/L, but PaO2 remained at 28-33 mmHg, with oxyhemoglobin saturation of 50-60%, and good spontaneous urine output. Approximately 48 hours after balloon atrial septostomy, lactate continued to improve to 1 mMol/L, with absence of metabolic acidosis, and arterial oxygen saturation was 65-70% and we decided to perform corrective surgery of D-TGA. Jatene procedure was performed after hemodynamic stabilization of the patient (arterial oxygen saturation of 65-70%, pO2 30-35 mmHg, good urine output and adequate mean arterial pressure). Arterial Switch was held on with cardiopulmonary bypass of 273 minutes and aortic cross clamp time of 104 minutes, custodiol was used for cardioplegia. Variation in coronary anatomy Jacoub D type was evidenced (circumflex arises from the right coronary artery and left anterior descending arises alone, and its initial course was intramural). Page 1 of 7 In the early postoperative state the urine output was mantained with furosemide infusion, we managed to reduce adrenaline infusion from 0.25 to 0.1 micrograms/kg/min, dopamine remained at 10 micrograms/kg/min and levosimendan at 0.1 micrograms/kg/min. In the next 24-36 hours the patient presented hemodynamic compromise with persistent tachycardia, drop in mean arterial pressure to 39 mmHg, lactate elevation up to 9 mmol/L, fall in central venous saturation to 60%; and increased inotropic-vasopressor support requirement, including adrenaline up to 0.2 micrograms/kg/min, dopamine to 10 micrograms/kg/min and hydrocortisone infusion. We also observed LV dysfunction with LVEF of 18% and SF 7%, elevated liver enzymes (AST 232 IU/L, ALT 966 IU/L, total bilirubin 2.43 mg/dL, direct bilirubin 0.99 mg/dL and INR 2.68), cardiac enzymes (troponin T 5.71 ng/mL, CK-MB 149 U/L) and impaired renal function (creatinine 0.77 mg/dL). Due to clinical deterioration we decided to start circulatory support with VA-ECMO, with chest cannulation. Venous cannula draining in right atrium 14 Fr (Medtronic) and arterial inflow cannula in aorta 10 Fr (SorinGroup). VA-ECMO was succesfully initiated with increasing flows up to 540 ml (150 ml/kg/min). However, immediate dilation of the left atrium with displacement of the interatrial septum was evidenced through epicardial echocardiography, so we decided to place a 12 Fr (Medtronic) cannula in the left atrium to decompress the left heart chambers. Stöckert Centrifugal Pump Console (SCPC, Soring Group), Revolution centrifugal blood pump (SoringGroup), extracorporeal membrane oxygenator polymethylpentene Lilliput 2 ECMO (Sorin Group) was used. As being in ECMO support during fixing cannulas, aortic cannula was out incidentally, but this incident was resolved properly. During the first hour of ECMO support, withdrawal of adrenaline was achieved. About 4 hours post ECMO implant, the patient was receiving only dopamine at 4 micrograms/kg/min. After 16 hours inotropic support was withdrawn and patient received only nitroprusside. Mechanical ventilation was given with protective parameters (PEEP 7 cm H2O, FiO2 0.35, inspiratory time 0.8 seconds, respiratory rate 20 per minute, PIP 22 cm H 2O) for a tidal volume of 6 ml/kg. After 12 hours of ECMO support, hemodynamic compensation was achieved with central venous oxygen saturation of 74%, lactate 1.8 mMol/L, adequate tissue perfusion and spontaneous urine output. After 48 hours of ECMO implant, the left atrium cannula was repositioned, because it was positioned into the left ventricle in curve "U" and was returned to left atrium, this procedure was performed bedside at the PICU without complications. Target organ damage recovery was achieved between the third and fifth day of ECMO support. After approximately 84 hours of ECMO support, we tested weaning it; decreasing 10 ml/kg/min flow pump every 10-15 minutes until 38 ml/kg/min (140 ml/min), with close monitoring of hemodynamic variables as heart rate, MAP, pulse wave pressure, central venous saturation, tissue perfusion and echocardiographic exams. Noticing a successful weaning test, slow weaning of ECMO support was initiated, reducing the flow pump 40 ml/min every 4 hours, it was necessary to place a bridge to maintain an adequate flow in the circuit, reaching a flow 210 ml/min (58 ml/kg/min). Twenty four hours after the initial weaning test from VA-ECMO, we did another try, reducing successfully the flow to 140ml/min. ECMO explant was programmed to be performed at operating room; where a ECMO off trial was performed, clamping arterial, left atrium and the right atrium cannulas, and the patient presented adequate hemodynamic status with stable heart rate, suitable MAP 50-55 mmHg, pulse pressure wave of 15 mmHg, with epinephrine support at 0.05 micrograms/kg/min and titratable nitroprusside. During the removal of the cannula from the aorta, the patient presented major bleeding and there were difficulties in aortic repairing, which conditioned increased requirement of adrenaline infussion and high need of blood components replacement. The patient remained on ECMO support for 5 days 1 hour 26 minutes. No mechanical complications related to ECMO circuit were observed. INVESTIGACIONES En caso de ser relevante Page 2 of 7 DIAGNÓSTICO DIFERENCIAL En caso de ser relevante TRATAMIENTO En caso de ser relevante RESOLUCIÓN Y SEGUIMIENTO After ECMO support was withdrawn the patient had the following complications: left upper limb arterial thrombosis, related to axillary access; chylothorax, which is being managed with nutritional support and octreotide; left phrenic nerve palsy, which underwent diaphragmatic plication. Currently, 25 days post ECMO explant, the patient is under weaning from mechanical ventilation, her left ventricle ejection fraction is 55%, and he does not receive inotropic-vasopressor support but has developed an intercurrent infection, which is being treated with appropriate antimicrobial drugs. DISCUSIÓN Pequeños resúmenes de casos similares ya publicados. The treatment of choice for D-TGA is corrective surgery, Arterial Switch. According to the literature, 70% of patients with D-TGA, have usual coronary anatomy; the remaining patients presented variants of these, of which more than half the circumflex arise from right coronary artery (1). In our patient circumflex artery arises from the right coronary artery and left anterior descending arises alone, and its initial course was intramural (Yacoub and Radley-Smith type D, Post circumflex 1AD; 2R, Cx in Leiden classification) (1,2). LV dysfunction due to deconditioned LV is seen in patients who underwent surgery for D-TGA, mainly in older infant (3). Failure of an unprepared LV is more likely to occur in older infant, our patient was 25 days old at the moment of surgery. These patients need more pharmacologic support and delayed closure of the sternum. The indications for VA ECMO are: failure in the operating room (unable to come off bypass), hemodynamic decompensation in ICU (more support with inotropic-vasopressor, metabolic acidosis, decreased urine output for 6 hours), ECLS in CPR, and others. Use ECMO as cardiovascular support in these patients may be lifesaving and give added time for ventricular recovery o retraining (3–5). Venoarterial ECMO is a mechanical circulatory support of election for infants and children with severe acute cardiopulmonary failure. Volume overload VI, increased afterload generated by the VA-ECMO perfusion, results in increased wall-stress, and poor LV decompression; it may result in dilatation of the left heart, raised LV end-diastolic pressures, and reduced subendothelial perfusion causing myocardial ischemia. For these reasons the addition of a cannula drainage into the left atrium is necessary. It can be showed by echocardiography or chest radiograph (3,6,7). Yasuhiro Kotani et. al. at the Hospital for Sick Children, University of Toronto, from 2005 to 2011, reported 23 of 178 (12.9%) patients with VA ECMO had cannula in left atrium for decompression the LA and LV; so to help improve left ventricular function, LA and LV dilatation, and relieve lung edema. Of the 23 cases with left sided venting: 16 was achieved with left cannulation, 3 with atrial septal defect created surgically, and 4 with balloon atrial septostomy. The duration of ECMO support was 5.9 4.5 days (6). In our patient we take an early decision to add a cannula to the left atrium, guided by epicardial echocardiography in the operating room and the duration of ECMO support was 5 days. According to ELSO register, that cyanotic patient with incremented pulmonary flow survive only 35% (8). Our patient has survived the explant ECMO, but still remains on mechanical ventilation in the PICU. PUNTOS DE APRENDIZAJE 3 a 5 puntos. Initial experience in the indication, management and care of patients with VA-ECMO with left sided venting. Multidisciplinary teamwork. Emphasize and verify proper cannulas position during ECMO implant. Experiment the usefulness of placing bridge in the ECMO circuit. REFERENCIAS 1. Hugh D. Allen, Robert E. Shaddy, Daniel J. Penny, Timothy F Feltes. Moss & Adams’ Heart Disease in Infants, Children, and Adolescents, Including the Fetus and Young Adult. Eight. Page 3 of 7 2. 3. 4. 5. 6. 7. 8. Wolters Kluwer; 1900 p. Richard A Jonas, James DiNardo, Laussen PC, Robert Howe, Robert LaPierre, Gregory Matte. Comprehensive Surgical Management of Congenital Heart Disease. Peter C. Lausenn, David L. Wessel. Pediatric Cardiac Intensive Care Handbook. Washintong D.C.: Pediatric Intensive Care Books; 2015. Extracorporeal Life Support Organization - ELSO. ELSO Pediatric Cardiac Failure Supplement to the ELSO General Guidelines [Internet]. 2013. Available from: www.elsonet.org Extracorporeal Life Support Organization - ELSO. General Guidelines for all cases [Internet]. Ann Arbor, MI, USA; 2013. Available from: www.elsonet.org Kotani Y, Chetan D, Rodrigues W, Sivarajan VB, Gruenwald C, Guerguerian A-M, et al. Left atrial decompression during venoarterial extracorporeal membrane oxygenation for left ventricular failure in children: current strategy and clinical outcomes. Artif Organs. 2013 Jan;37(1):29–36. Hacking DF, Best D, d’Udekem Y, Brizard CP, Konstantinov IE, Millar J, et al. Elective decompression of the left ventricle in pediatric patients may reduce the duration of venoarterial extracorporeal membrane oxygenation. Artif Organs. 2015 Apr;39(4):319–26. ELSO. Extracorporeal Life Support Organization - ECMO and ECLS > Registry > Statistics > International Summary [Internet]. [cited 2016 Sep 30]. Available from: http://www.elso.org/Registry/Statistics/InternationalSummary.aspx IMÁGENES O FIGURAS Las imagines o figuras no deben ir en el texto. Figure 1. VA-ECMO Page 4 of 7 Figure. VA-ECMO with bridge Page 5 of 7 Figure 3. Cannula position Abbreviations: TGA: Transposition of Great Arteries OR: operating room LVEF: left ventricular ejection fraction PGE1: Prostanglandin E1 PICU: Pediatric intensive care unit PIP: peak inspiratory pressure. PEEP: Positive end expiratory pressure SF: shortening fraction ECLS: extracorporeal life support. ECMO: extracorporeal membrane oxygenation VA: Venoarterial LA: left atrium LV: left ventricle CPR: Cardiopulmonary resuscitation SCPC: Stöckert Centrifugal Pump Console Fecha: September 30, 2016 Autor: Prado-Gómez, Tommy (1) Co-Autor: Alcarraz-Alcarraz, Brisa(1); Ortiz-Rojas, Rubén(1); Lescano-Alva, Miguel (2); León-Lázaro, Silvia (3); Angeles-De-Los-Santos, Tula(4). (1) Pediatric Intensive Care Service, National Cardiovascular Institute (2) Perioperative Cardiology Service, National Cardiovascular Institute (3) Cardio-pediatric Nursing Service, National Cardiovascular Institute (4) Surgical Nursing Service Center, National Cardiovascular Institute Page 6 of 7 Page 7 of 7