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18 impatto dell’ecocardiografia fetale

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18 impatto dell’ecocardiografia fetale

  1. 1. IMPATTO DELL’ECOCARDIOGRAFIA FETALE SULL’OUTCOME CHIRURGICO DELLE CARDIOPATIE CONGENITE Prof Fabio Miraldi
  2. 2. Fetal Echocardiography  Two forms: basic and extended  “Four chamber view” the most basic  Color-flow and spectral Doppler imaging  M-mode available  Sensitivity from 60-100%  Limited by body habitus and fetal age (11 to 14 weeks  transvaginal ultrasound). 16 weeks by abdominal.  Reported specificity up to 100% in babies without congenital heart disease
  3. 3. IMPROVING  In 1992, only 8% of infants with congenital heart disease undergoing cardiac surgery had a prenatal diagnosis. With fetal echo, this number rose to 57% in 2002.(Mohan)  In one study assessing use of early fetal echo (prior to 14 weeks gestation) in a high-risk population, sensitivity of 70% and specificity of 98%  This resulted in 79% of patients terminating their pregnancy prior to their 18-20 week follow up.  Diagnosis of congenital heart disease is 3.5/1000 in II trimester
  4. 4. Fetal echocardiography: advantages  Counseling  In some cases, life saving procedures may be undergone on mother/fetus before delivery or during pregnancy  Delivery planning in a special institute and close to a pediatric cardiac surgery centre
  5. 5. Counseling  Provide an accurate diagnosis of the malformation  Provide a clear and truthful picture of the prognosis  Outline management and treatment options that are available  Help parents to reach decisions concerning the form of management that is best for them
  6. 6. Diagnosis and Treatment of Fetal Cardiac Disease by Mary T. Donofrio, Anita J. Moon-Grady, Lisa K. Hornberger, Joshua A. Copel, Mark S. Sklansky, Alfred Abuhamad, Bettina F. Cuneo, James C. Huhta, Richard A. Jonas, Anita Krishnan, Stephanie Lacey, Wesley Lee, Erik C. Michelfelder, Gwen R. Rempel, Norman H. Silverman, Thomas L. Spray, Janette F. Strasburger, Wayne Tworetzky, and Jack Rychik Circulation Volume 129(21):2183-2242 May 27, 2014
  7. 7. Standardized transverse scanning planes for fetal echocardiography include an evaluation of the 4-chamber view (1), arterial outflow tracts (2, 3), and 3-vessel trachea view (4). Mary T. Donofrio et al. Circulation. 2014;129:2183-2242
  8. 8. Sagittal views of the superior and inferior vena cavae (1), aortic arch (2), and ductal arch (3). Mary T. Donofrio et al. Circulation. 2014;129:2183-2242
  9. 9. Low and high short-axis views of the fetal heart. Mary T. Donofrio et al. Circulation. 2014;129:2183-2242
  10. 10. Transabdominal view of the 4 chambers of the heart at 13 weeks' gestation.
  11. 11. Transvaginal view of atrioventricular septal defect at 13 weeks' gestation in a fetus with trisomy 21
  12. 12. Transvaginal 3-vessel view of the great arteries at 13 weeks' gestation. PA, Main pulmonary artery; Ao, aorta; SVC, superior vena cava; BPA, right branch pulmonary artery. McAuliffe F.
  13. 13. (A) A full examination of the fetal heart may be obtained by five transverse sections through the abdomen and chest of the fetus. The first section shows abdominal situs (B) with the aorta (Ao) to the left of the spine and the inferior caval vein (IVC) anterior and to the right. The normal fetal stomach (St) and heart lie on the left side. The second section (C) illustrates the four chambers of the heart with the left atrium (LA) in front of the spine and the right ventricle (RV) just below the sternum. The third cut (D) shows the aorta arising centrally in the heart from the left ventricle (LV) and the fourth the pulmonary trunk (PV) arising from the anteriorly placed right ventricle and crossing to the fetal left over the ascending aorta (E). The fifth section shows the anteriorly positioned ductal arch (D) and the transverse aortic arch (Ao) to be of equal size traversing back to the fetal spine (F). A normal variant "three vessel" view is shown with a right sided aortic arch and persistent left superior caval vein (LSVC). The trachea (T) can be seen lying between the aortic (Ao) and ductal (D) arches (G). Gardiner.
  14. 14. Secondary lesions develop during following 30-32 gestation weeks Flow related theory Cardiac Embryogenesis Rational of primary early correction
  15. 15. Fetal echocardiography: advantages  Counseling  In some cases, disease modifying or lifesaving procedures may be undergone on mother/fetus before delivery or during pregnancy  Delivery planning in a special institute and close to a pediatric cardiac surgery centre
  16. 16. Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart SyndromeCLINICAL PERSPECTIVE by Lindsay R. Freud, Doff B. McElhinney, Audrey C. Marshall, Gerald R. Marx, Kevin G. Friedman, Pedro J. del Nido, Sitaram M. Emani, Terra Lafranchi, Virginia Silva, Louise E. Wilkins-Haug, Carol B. Benson, James E. Lock, and Wayne Tworetzky Circulation Volume 130(8):638-645 August 19, 2014 Copyright © American Heart Association, Inc. All rights reserved.
  17. 17. Kaplan–Meier curves depicting intention-to-treat analyses from the date of fetal intervention. Lindsay R. Freud et al. Circulation. 2014;130:638-645 Copyright © American Heart Association, Inc. All rights reserved.
  18. 18. Flow diagram summarizing postnatal management and outcomes for the entire 100-patient cohort. Lindsay R. Freud et al. Circulation. 2014;130:638-645 Copyright © American Heart Association, Inc. All rights reserved.
  19. 19. Preintervention fetal and neonatal echocardiograms of a patient who underwent technically unsuccessful fetal aortic valvuloplasty (FAV) and was managed with a univentricular strategy (A and B), and a patient who underwent technically successful FAV and was managed as biventricular postnatally (C and D). Lindsay R. Freud et al. Circulation. 2014;130:638-645 Copyright © American Heart Association, Inc. All rights reserved.
  20. 20. Kaplan–Meier curves depicting survival from the time of birth. Lindsay R. Freud et al. Circulation. 2014;130:638-645 Copyright © American Heart Association, Inc. All rights reserved.
  21. 21. Diagnosis and Treatment of Fetal Cardiac Disease Circulation Volume 129(21):2183-2242 May 27, 2014
  22. 22. Fetal echocardiography: advantages  Counseling  In some cases, life saving procedures may be undergone on mother/fetus before delivery or during pregnancy  Delivery planning in a special institute and close to a pediatric cardiac surgery centre
  23. 23. Prostaglandins The response of the ductus arteriosus to prostaglandins. Coceani F, Olley PM. Can J Physiol Pharmacol. 1973 Mar;51(3): 220-5 Arterial duct
  24. 24. Prostaglandins The response of the ductus arteriosus to prostaglandins. Coceani F, Olley PM. Can J Physiol Pharmacol. 1973 Mar;51(3): 220-5 PGE1 Arterial duct
  25. 25. CHD classification Difetto interatriale Difetto interventricolare Dotto arterioso Canale atrioventricolare Iperafflusso polmonare Coartazione aortica Stenosi aortica Stenosi polmonare Ostruzione degli efflussi ventricolari Acianotiche Tetralogia di Fallot Atresia tricuspide Atresia polmonare Ipoafflusso polmonare Trasposizione delle G.A. Rit. venoso polm. anom. tot. Truncus arteriosus Sindrome cuore sx ipoplasico Mixing Cianotiche Cardiopatie congenite
  26. 26. Difetto interatriale Difetto interventricolare Dotto arterioso Canale atrioventricolare Iperafflusso polmonare Coartazione aortica Stenosi aortica Stenosi polmonare Ostruzione degli efflussi ventricolari Acianotiche Tetralogia di Fallot Atresia tricuspide Atresia polmonare Ipoafflusso polmonare Trasposizione delle G.A. Rit. venoso polm. anom. tot. Truncus arteriosus Sindrome cuore sx ipoplasico Mixing Cianotiche Cardiopatie congenite Dotto-dipendenze (polmonare o sistemica) CHD classification PGE1
  27. 27. ( ( ( ( AD AS VENTRICOLO AP AO Parallel circulations: u.h. with single outlet PDA 62 1.5 Qp/Qs = 0.5 ( ( ( ( AD AS VENTRICOLO AP AO 75 2.0 Qp/Qs = 1.0 PDA ( ( ( ( AD AS VENTRICOLO AP AO PDA 90 3.5 Qp/Qs = 3.0 Restrictive ASD neonate 4-5 days PVRs decrease PDA closure SO2 Work load (X normal)
  28. 28. HLHS: rational of perioperative treatment NORMALIZE Qp/Qs Personalized ventilatory treatment TO INCREASE PVRs: • decrease FiO2 (21%) • hypoventilation (PCO2 40 mmHg) • addition of CO2 • increase insp. Pressures (PEEP) • avoid anemia (Ht >40%) • minimal sedation TO DECREASE PVRs: • increase FiO2 (100%) • hyperventilation(PCO2 25-30 mmHg) • NO • decrease insp. pressures • avoid multiple transfusions • deep sedation
  29. 29. Timing chirurgico Evitare di operare il paziente nel giorno della sua morte!
  30. 30. Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis Pediatrics International Volume 41, Issue 6, pages 728-732, 1 MAR 2002 DOI: 10.1046/j.1442-200x.1999.01154.x
  31. 31. Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis Pediatrics International Volume 41, Issue 6, pages 728-732, 1 MAR 2002 DOI: 10.1046/j.1442-200x.1999.01154.x
  32. 32. Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis Pediatrics International Volume 41, Issue 6, pages 728-732, 1 MAR 2002 DOI: 10.1046/j.1442-200x.1999.01154.x
  33. 33. Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis
  34. 34. Prostaglandin E1 keeps the ductus arteriosus open. If the ductus arteriosus shows a closing tendency in the hemodynamics of HLHS, the blood flow ejected from the right ventricle cannot go through the ductus because of ductal stenosis and systemic circulation cannot be established. Most of the blood flow also goes through the pulmonary artery instead of the systemic circulation. The patient’s condition suddenly deteriorates with tachypnea, low cardiac output, peripheral cyanosis and renal shutdown and blood pressure goes down to shock level. This is the so-called ‘ductal shock’. Once ductal shock occurs in a patient with HLHS preoperatively, the patient’s general condition deteriorates and the surgical outcome may be affected. In the present study group, there were no patients who had ductal shock in the prenatally diagnosed group; however, four of 10 patients had ductal shock in the non-prenatally diagnosed group. Prevention of ductal shock is the most important issue in preoperative management of patients with HLHS. Thus, we can conclude that in terms of prevention of ductal shock, prenatal diagnosis of HLHS is quite beneficial for the patients, although surgical outcomes did not show a significant difference between the two groups Ductal shock
  35. 35. CHD diagnosis  Counseling  Treatment options -Termination of pregnancy (chromosomal anomaly, major defects) -During pregnancy treatment (medical, interventional) -Time, type and site of delivery -Appropriate treatment (which operation) immediately after birth
  36. 36. Diagnosis and Treatment of Fetal Cardiac Disease  TGA  HLHS  TOF  EBSTEIN  Shunt lesions, most ductal dependent lesions
  37. 37. CONCLUSIONI  L’ecocardio fetale non ha cambiato sicuramente l’outcome del trattamento chirurgico di nessuna cardiopatia congenita di per sé se non in quelle trattate con metodica interventistica in età fetale, ma ha anticipato/migliorato il trattamento postnatale influendo quindi sul successivo decorso
  38. 38. Conclusioni “La selezione naturale determina la sopravvivenza del più forte” (Darwin) La medicina moderna deve impegnarsi per la sopravvivenza e la qualità di vita del più debole.

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