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• W3120496680 abstract "KOTHARI ET AL. presented, in their E-Challenge, a woman in her late 50s recently diagnosed with a congenital heart defect who underwent successful repair.1Kothari P Nguyen Q Pagel P et al.Gradually progressive dyspnea and exercise intolerance in an otherwise active elderly woman: Why was the presentation of congenital heart disease delayed.J Cardiothorac Vasc Anesth. 2021; 35: 1225-1230Abstract Full Text Full Text PDF Scopus (1) Google Scholar Their case highlighted a growing phenomenon: adult congenital heart disease (CHD) patients presenting to the operating room. Currently, adults with CHD are outnumbering pediatric cardiac patients.2Moodie D. Adult congenital heart disease: Past, present, and future.Tex Heart Inst J. 2011; 38: 705-706PubMed Google Scholar This is the result of improved surgical, cardiopulmonary bypass (CPB) and anesthetic techniques treating or palliating children and allowing them to survive to adulthood. Congenital cardiac surgery has advanced to the point where it has become routine, and it is easy to take for granted the difficult path that has led us here. This accomplishment may cause one to pause and wonder: How did we reach this achievement? Descriptions of congenital lesions were described in the 17th century, but it was not until the 1900s at Johns Hopkins University that the field of pediatric cardiology was created, in Dr Helen Taussig's pediatric cardiology clinic.3Blalock A Taussig HB. Landmark article May 19, 1945: The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia.JAMA. 1984; 251: 2123-2138Crossref PubMed Scopus (81) Google Scholar,4Murphy J. Breakthrough!: How three people saved blue babies and changed medicine forever. Houghton Mifflin Harcourt, Boston, MA2019Google Scholar The first congenital cardiac surgery occurred in 1938 at Boston Children's Hospital, with Dr Robert Gross ligating a patent ductus arteriosus in a seven-year-old child. Few details regarding the anesthetic were documented other than it being delivered by a nurse, using a facemask and cyclopropane.5Gross RE Hubbard JP. Surgical ligation of a patent ductus arteriosus: Report of first successful case.JAMA. 1939; 112: 729-731Crossref Scopus (370) Google Scholar In 1946, Drs Harmel and Lamont published a detailed report of the anesthesia provided for 100 patients operated on by Dr Blalock for pulmonary stenosis or atresia using the Blalock shunt. This provided tremendous insight into the anesthetic practices at that time and was the first paper published about pediatric cardiac anesthesia. Despite its novelty, the authors highlighted a vital tenet of pediatric anesthesia, preoperative bonding. They discussed the importance of the preoperative visit, often bringing a mask to play with so it would not be foreign or frightening the day of surgery. Patients were premedicated with intramuscular morphine and atropine prior to surgery to the point of producing sleep. Inhalation cyclopropane was used for induction. All patients, except for six, were intubated with a tight-fitting uncuffed endotracheal tube. The authors mentioned the difficulty in finding tubes for infants and that they much preferred to use a tube rather than a mask. The thought of masking a patient for the duration of a cardiac surgery should fill us with gratitude for the technology and standards that we enjoy today. Either cyclopropane, ether, or a combination was used for anesthetic maintenance. The paper was filled with comments that today's anesthesiologists can appreciate and commiserate with, such as the observation that with increasing skill, surgeons were less bothered by slight movements in the mediastinum. They went on to comment that in one patient the phrenic nerve was crushed accidentally, which facilitated a quieter surgical field for the surgeon, and they postulated that, in the future, applying local anesthetic to the phrenic nerve would aid the surgeons. Cardiac surgery in its infancy was extremely hazardous, with 23 out of 100 patient deaths occurring in this series.6Harmel MH Lamont A. Anesthesia in the surgical treatment of congenital pulmonic stenosis.Anesthesiology. 1946; 7: 477-498Crossref PubMed Scopus (29) Google Scholar Anesthetic care was further refined as documented by Dr McQuiston at Children's Memorial Hospital in Chicago, who published about anesthetizing pediatric patients for the Potts-Smith aortic pulmonary anastomosis. Heavy premedication was used to reduce agitation and the resulting cardiovascular collapse due to limited cardiac reserve. Most patients were intubated, and anesthesia was maintained with cyclopropane. The author comments on the importance of using physical examination skills during the operation provided clues about hypoxia or hypercarbia leading to impending cardiovascular collapse, as pulse oximetry and end-tidal carbon dioxide monitoring were not available. Consequently, he and his colleagues avoided administering neuromuscular blockade.7McQuiston WO. Anesthetic problems in cardiac surgery in children.Anesthesiology. 1949; 10: 590-600Crossref PubMed Scopus (19) Google Scholar Pulse oximetry was not available in the operating room until the 1980s; hence, there were many years that anesthesiologists relied on observation methods to discern oxygen saturation.8Yelderman M New W. Evaluation of pulse oximetry.Anesthesiology. 1983; 59: 349-351Crossref PubMed Scopus (368) Google Scholar He also discussed the importance of monitoring temperature and the protective effects of hypothermia in cardiac surgery.7McQuiston WO. Anesthetic problems in cardiac surgery in children.Anesthesiology. 1949; 10: 590-600Crossref PubMed Scopus (19) Google Scholar A milestone in pediatric cardiac surgery was the introduction of CPB in 1954, allowing repair of intracardiac lesions such as atrioventricular canal (AVC) defects. Initial use of CPB was associated with high morbidity, markedly worse among pediatric patients. Keats et al. wrote about the anesthetic experience of 200 patients undergoing cardiac surgery with CPB at Texas Children's Hospital. Patients were premedicated with pentobarbital, meperidine, and scopolamine, and anesthesia was induced with cyclopropane or thiopental. Ether-oxygen was administered to maintain anesthesia until commencement of CPB at which point only d-tubocurare was given. They avoided adding any volatile anesthetics during bypass due to the potential explosion hazards and, surprisingly, reported no cases of recall. Children requireing frequent blood transfusions and having arrhythmias were common. The mortality rate was 22.5%; infants undergoing the procedure had a mortality of 47.5%.9Keats AS Kurosu Y Telford J et al.Anesthetic problems in cardiopulmonary bypass for open heart surgery; experiences with 200 patients.Anesthesiology. 1958; 19: 501-514Crossref PubMed Google Scholar In comparison, the mortality rate VSD repair today is 1%.10Scully BB Morales DCL Zafar F et al.Current expectations for surgical repair of isolated ventricular septal defects.Ann Thorac Surg. 2010; 89: 544-551Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar The high mortality rate of early CPB led surgeons to pursue alternative and novel methods, such as deep hypothermic circulatory arrest, for repair of intracardiac lesions. Avoiding the CPB machine, Horiuchi et al. published a series of ventricular septal defect (VSD) repairs in 1963. After anesthetizing the patient, they were cooled in an ice-water bath to 28°C, and then they were placed on the operating table with a cold-water blanket and surgery commenced under circulatory arrest. After completion of the repair, the child was brought to normothermia with warm water, during which time arrhythmias, such as atrioventricular block, ventricular tachycardia, or ventricular fibrillation, occurred. This process took about 2.5 hours, and they reported an 11% mortality rate among the infants who underwent cardiac surgery.11Horiuchi T. Radical operation for ventricular septal defect in infancy.J Thorac Cardiovasc Surg. 1963; 46: 180-190Abstract Full Text PDF PubMed Google Scholar Faced with the initial poor outcomes of CPB, American surgeons took notice. Dr Bill Norwood, then at Boston Children's Hospital, tasked anesthesiologist Dr Dolly Hansen to recreate this technique. This well-known pioneer in pediatric cardiac anesthesia was left to figure out how to perform the anesthetic with little guidance, and recalls purchasing a dishwashing bucket to facilitate cooling. Her experiences led to great advancements in the care of patients with congenital heart defects.12Odegard KC Rockoff MA. The “mother of pediatric cardiac anesthesia”: An interview with Dr Dolly D. Hansen, a pioneering woman in medicine.Paediatr Anaesth. 2020; 30: 964-969Crossref PubMed Scopus (1) Google Scholar The following E-Challenge by Kothari et al. was notable for an increasingly rare phenomenon; they described an adult patient with undiagnosed CHD presenting in her sixth decade of life.1Kothari P Nguyen Q Pagel P et al.Gradually progressive dyspnea and exercise intolerance in an otherwise active elderly woman: Why was the presentation of congenital heart disease delayed.J Cardiothorac Vasc Anesth. 2021; 35: 1225-1230Abstract Full Text Full Text PDF Scopus (1) Google Scholar Given the advances in prenatal screening and surgical technique, most AVC defects are identified early and successfully repaired. Prior to the 1950s and advent of CPB, this patient would not have undergone repair and would have lived her life until the development of intractable heart failure. During the 1950s, her survival would have been tenuous given the novel surgical, anesthetic, and perfusion techniques. However, today her outcome would not be questioned thanks to care provided by congenital cardiac centers. Children with heart disease are not only surviving but thriving into adulthood; this is a testament to how far we have come. The sacrifices of the patients who faced uncertain outcomes and high mortalities with experimental procedures should be exalted. Thanks to their sacrifice, and the ingenuity of our predecessors, we can provide safe care for these complicated patients. None. Gradually Progressive Dyspnea and Exercise Intolerance in an Otherwise Active Middle-Aged Woman: Why Was the Presentation of Congenital Heart Disease Delayed?Journal of Cardiothoracic and Vascular AnesthesiaVol. 35Issue 4PreviewPatients with congenital heart disease (CHD) increasingly are surviving into adulthood. In the United States alone, there are more than one million adult patients living with CHD with the number increasing about 5% each year. With more than 85% of infants with CHD surviving into adulthood with their disease, encounters with these patients in the operating room for cardiac and noncardiac operative procedures is becoming more commonplace. Most of these patients receive corrective surgery early in life, although some may live with uncorrected CHD with no-to-relatively mild symptoms and present at a later time in life with symptoms of heart failure or pulmonary hypertension. Full-Text PDF" @default.
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