Dorry Segev, M.D., Ph.D.

• Associate Vice Chair for Research
• Professor of Surgery

https://www.hopkinsmedicine.org/profiles/results/directory/profile/0008001/dorry-segev

Selection of Data in the Event of Multiple Records in a Window Depending on the statistical analysis method allergy or sinus buy genuine benadryl, single values may be required for each analysis window savannah ga allergy forecast order generic benadryl canada. For example allergy medicine without antihistamine order benadryl 25mg without a prescription, change from baseline by visit usually requires a single value allergy medicine zantac benadryl 25mg with amex, whereas a time-to-event analysis would not require 1 value per analysis window allergy medicine clortrimitime discount 25mg benadryl free shipping. If there are 2 visits equidistant from the nominal day within the analysis window allergy medicine congestion buy 25 mg benadryl overnight delivery, take the latest. If there is more than 1 record on the selected day, take the average (for continuous data) or the worst (for categorical data). If there are 2 values on the same day, the second may be a retest because there was a problem with the first test (eg, specimen hemolyzed). The denominator for this calculation will be the number of subjects in the safety analysis set. Similarly, the number and percentage of subjects randomized and treated in each stratum and the number of subjects who completed/did not complete study drug within each stratum will be summarized. The denominator for percentage of subjects in the stratum will be all subjects randomized and treated and the denominator for completed or did not complete study drug will be subjects randomized and treated within the stratum. Disposition of Subjects A summary of subject disposition will be provided by randomized treatment group and overall. The denominator for the percentages of subjects in each category will be the number of subjects in the safety analysis set. A data listing of date of informed consent, first dose date, last dose date of any study drug (study day), date completed study (study day), completed study treatment (yes/no), completed study (yes/no) and reasons for premature study treatment/study discontinuation will be provided. Extent of Exposure Duration of Exposure to Study Regimen Duration of exposure to study regimen will be defined as (last dose date of any study drug - first dose date of any study drug + 1), regardless of temporary interruptions in study drug administration and will be expressed in weeks (recorded to 1 decimal place, eg, 12. The 12-week group (Group 1) will be additionally summarized for Week 10 (Day 70) and Week 12 (Day 84). Calculation of Number of Tablets Prescribed for Study Drug For subjects who complete study drug or prematurely discontinue study drug for a reason other than virologic failure (ie, met virologic failure stopping criteria) number of tablets prescribed is displayed in Table 4. Subjects who prematurely discontinue study drug for lack of efficacy (ie, virologic failure) will have prescribed study drugs calculated for the number of study days at the first date when virologic stopping criteria were met (ie, substitute number of days to virologic failure [date of first of 2 consecutive measurements or a last available value meeting criteria] for 84 days [Group 1] or 56 days [Groups 2 and 3]). For subjects who discontinue for virologic failure, bottles dispensed after the subject first met virologic failure criteria will not be included in calculations. If a bottle is dispensed, and the bottle is returned empty, then the number of tablets returned will be entered as zero. If a bottle is dispensed but not returned (missing), the number of tablets taken from that bottle will be counted as zero. Categorical displays will be presented for the number of subject who are at least 80% adherent to their drug regimen (ie, adherence is 80% for each drug). Protocol Deviations A summary of major protocol violations will be provided by the Clinical Operations group for subjects in the safety analysis set. Subjects who received study drug other than their randomized treatment assignment will be listed with the start and stop dates that they received incorrect study treatment. The summary of demographic data and baseline characteristics will be provided for the safety analysis set. For continuous variable of demographic and baseline characteristics, a Kruska-Wallis test will be used to evaluate the overall difference across all treatment groups. Medical History General medical history (ie, conditions not specific to the disease being studied) data will be listed only. Interferon Classification A summary of categories for interferon classification will be provided by randomized treatment group and overall for the safety analysis set. This summary will present the number of subjects that are interferon eligible and ineligible, as determined by the opinion of the principle investigator at each site, with reasons for interferon ineligible. If both non-inferiority tests are statistically significant, the key secondary analysis of non-inferiority in which Group 3 is compared to Group 2 will be performed at the 0. As previously described, the adjusted historical control rate is estimated to be 65%. A non-inferiority margin of 12% is very conservative based on statistical consideration. Primary Analysis of the Primary Efficacy Endpoint the 2-sided 1-sample binomial test will be used to test the primary statistical hypotheses described above. Key Secondary analysis of the Primary Efficacy Endpoint Assessments of the key secondary analyses are gated on results from the primary analysis and are structured to ensure strong control of the family-wise type I error rate at the 0. If non-inferiority for both comparisons between Group 2 and Group 1, and between Group 3 and Group 1 are established, non-inferiority test of Group 3 versus Group 2 will be performed at the 0. Subgroup Analysis of the Primary Efficacy Endpoint Subgroup analysis will be performed for the subgroups outlined in Section 3. The 2-sided 95% exact confidence interval based on Clopper-Pearson method will be provided for the proportion within each treatment group. Virologic failure will be descriptively summarized as "on-treatment virologic failure" and relapse (which will be broken down by study drug completed yes/no). Drug resistant substitutions will be analyzed as part of the Virology Study Report. To identify or validate genetic markers that may predict the natural history of disease, response to therapy, and/or tolerability of medical therapy through genetic discovery research. A Wilcoxon rank sum test will be used to explore differences between treatment groups in change in status from baseline to each of the timepoints. Results (p-values) will be presented, but should be interpreted with caution as multiple endpoints are being tested, and the study has not been powered to test these exploratory endpoints. For imputation of missing data in the quality of life data, please refer to Section 3. This more inclusive definition allows subjects without a genotype, as determined per protocol by the central laboratory, to be included in the efficacy analysis. All safety data (except for laboratory tests with results that were cancelled by the lab) will be included in data listings based on the safety analysis set. Adverse Event Severity Adverse events are graded by the investigator according to the Gilead Grading Scale for Severity of Adverse Events and Laboratory Abnormalities as specified in the clinical study protocol. The severity grade of events for which the investigator did not record severity will be categorized as "missing" for tabular summaries and data listings, and will be considered the least severe for the purposes of sorting for data presentation. Events for which the investigator did not record the relationship to study drug will be considered to be related to study drug for summary purposes. The event is treatment emergent if the month and year of onset (or year of onset) of the event meets both of the following criteria: the same as or after the month and year (or year) of the first dose of any study drug the same as or before the month and year (or year) of the 30th day after the last dose of any study drug Summaries of Adverse Events and Deaths 7. Laboratory results cancelled by the central laboratory will not be included in analysis. The Week 4 safety follow-up visit will be presented as an additional separate visit. Graded Laboratory Values the Gilead Grading Scale for Severity of Adverse Events and Laboratory Abnormalities will be used for assignment of toxicity grades to laboratory results for purposes of analysis as Grade 0, Grade 1 (mild), Grade 2 (moderate), Grade 3 (severe) or Grade 4 (potentially life threatening). Grade 0 includes all values that do not meet criteria for an abnormality of at least Grade 1. Some laboratory tests have laboratory toxicity criteria for both increased and decreased levels; analyses for each direction (ie, increased, decreased) will be presented separately. If the relevant baseline laboratory data are missing, then any abnormality of at least Grade 1 will be considered treatment emergent. This listing will include the complete laboratory test profile for each laboratory test with the graded result throughout the study. Values falling outside of the relevant reference range and/or meeting Gilead Grading Scale will be flagged, as appropriate, in the data listings. For individual laboratory tests, subjects will be counted once based on the most severe postbaseline values when the criterion is met. The denominator will be the number of subjects in the safety analysis set with at least one nonmissing postbaseline value for the test. In the case of multiple values in an analysis window, data will be selected for analysis as described in Section 3. The summary will be sorted alphabetically by drug class and then by decreasing total frequency within a class. For purposes of programming, any medication with a stop date that is on/prior to first dosing date or start date that is after the last dose of any study drug will be excluded from this summary. If a partial stop date is entered, then the month and year (if day is missing) or year (if day and month are missing) that is prior to the study drug start date will be excluded from the summary. If a partial start date is entered, then the month and year (if day is missing) or year (if day and month are missing) that is after the study drug stop date will be excluded from the summary. A listing of all concomitant medications reported during the study will be provided. The number and percent of subjects in each cross-classification group will be presented (subjects with a missing value at baseline or on- treatment will not be included in the denominator for percent calculation). Other Safety Measures A data listing will be provided for subjects who become pregnant during the study. Data from this study will be combined with data from other studies in a meta-population analysis using mixed-effect modeling techniques. Each score is calculated only if at least half of corresponding items are not missing. To achieve this, scores for following 23 questions are reversed by taking the difference from 4. Diseases of Ear, Nose and Throat, which represents otorhinolaryngology Head and Neck Surgery in all of its diversity, is created to fill the need of contemporary definitive book. The reader will find boxes, tables, flow charts, line diagrams and photographs, which serve to enhance learning. The book is comprehensive and of broader scope and is designed for students, residents and practitioners alike. It offers a balanced presentation of content and emphasizes the practical features of clinical diagnosis and patient management. Each chapter includes clear, compelling, and up-to-date discussions and expertly executed and generously sized art. The brevity, conciseness, readable format and easy accessibility of key information will facilitate efficient use in any practice setting. Each page is carefully laid out to place related text, figures, and tables near one another to minimize the need for page turning. To provide an overview, each chapter begins with the list of its content and ends with further Reading section. Each chapter has clinical Highlights section for the quick revision of the students. This section has been especially prepared for answering frequently asked McQs, short-answer questions and oral/viva questions. The Appendix contains Top 101 clinical Secrets and problem-oriented cases which will be of immense use and interest to the readers. I would like to acknowledge my parents, late Shri Ramchandra and Smt Kalawati Devi Bansal, for enabling me to survive comfortably during my seemingly endless years of education. My family has unswervingly endorsed the time required for this mission, so heartfelt love and thanks go to my wife, Sushma, as well as our children Tejal and Mohit and his wife Astha. My loyal assistant for the last 10 years, Tejal patel, has provided amounts of all-round care to cover for my time. I wish to thank my professor friends who spared their valuable time in reviewing the chapters. I hope that my quest to document significant and up-to-date information has been successful. I invite readers and educators to send their suggestions so that I can include them in the next edition. The structure, content, and production values of this book will be shaped by its relationship with educators and readers. I would like to recognize and thank the members of the book team, who indeed worked hard, to bring this book to you. Shri Jitendar p Vij (chairman and Managing Director), Jaypee Brothers Medical publishers, illuminated the path for this book with his creative ideas and dedication. The insights and skills of Dr Richa Saxena (Editor-in-chief) helped in polishing this book to best meet the needs of students and faculty alike. Mr Ankit Vij (Managing Director), the young and dynamic leader, took personal interest and laid out each page of the book to achieve the best possible placement of text, figures, and other elements. The suggestions from Mr Saket Budhiraja (Director-Sales and Marketing) were very practical and meaningful. Mr Tarun Duneja (Director- publishing) demonstrated his untiring expertise during each step of the production process. I would like to thank Ms Sunita Katla (publishing Manager) for her efforts towards the finalisation of the book. Dr Rimpal chauhan, chandani, priti, falguni, Rina, Rashmi, Tejal, Bimal and Hansika, my students, have collaborated on the illustrations for this book. So I am thankful to prof Ravi Tiwari, prof Girish Mishra, prof Yojana Sharma, Dr Hiren Soni, Dr Siddharth Shah, Dr Nimesh patel and pG students for their valuable and meaningful discussions. I wish to especially thank several of my academic colleagues for their helpful contribution to this book. Majority of them generously provided their time and expertise and reviewed the chapters. Anatomy and Physiology of Nose and Paranasal Sinuses Anatomy of Nose 30 External Nose 30; Internal Nose 30; Anatomy of Paranasal Sinuses 37 physiology of Nose 39 Respiration 39; Air-Conditioning of Inspired Air 40; Protection of Airway 40; Vocal Resonance 41; Nasal Reflexes 41; Olfaction 41 physiology of paranasal Sinuses 41 Functions 41; Ventilation of Sinuses 42 29 3.

Note the widening of thyroid cartilage and healed tracheostomy opening Computed Tomography or Magnetic Resonance Imaging allergy forecast denton tx generic benadryl 25 mg otc. Any cartilage removed at tracheostomy should be submitted for histopathologic examination allergy testing mobile al order benadryl american express. Microlaryngoscopy: Small lesions of vocal cords are better visualized under operative microscope allergy forecast college station purchase 25 mg benadryl with mastercard. The complete excisional biopsy of small suspected lesion with border of healthy tissue helps in determining the depth of invasion allergy testing seattle buy generic benadryl 25 mg line, which is difficult with small biopsy specimen of early lesions allergy forecast waco tx order genuine benadryl line. An intense gag reflex and difficult patient and anatomy may require supplemental examination allergy testing antibiotics cheap 25 mg benadryl with visa. Laryngeal cancer: the most common malignancy of the larynx is squamous cell carcinoma. Squamous cell carcinoma with various grades of differentiation accounts for 95% of all laryngeal malignancies. The staging and classification help to analyze outcomes of different modalities of treatment, and assists in predicting prognosis of disease. T4b Subglottis T1 T2 T3 T4a Surgery To prove negative surgical margins, the specimen should be submitted for histopathological examination. Hypopharynx tumor size in greatest dimension T1 T2 T3 T4a Tumor 2 cm and limited to one subsite Tumor > 2 cm but 4 cm or invading > 1 subsite and without hemilarynx fixation Tumor > 4 cm or with hemilarynx fixation. Note the incision and skin changes after postoperative radiotherapy w Malignant tumors of larynx Distant metastasis (M) associated hemithyroidectomy or subtotal thyroidectomy is indicated in following conditions: Palpable thyroid abnormality Subglottic extension or tumors T4 glottic tumors T4 pyriform sinus tumors Positive Delphian nodes Cartilage destruction Stage grouping this N0 M0 T1 N0 M0 T2 N0 M0 T3 N0 M0 or T1-3 N1 M0 T4a N0-1 M0 or T1-4a N2 M0 T4b N0-3 M0 or T1-4 N3 M0 T1-4 N0-3 M1 combined therapy Surgery may be combined with pre or postoperative radiation in a planned way to decrease the incidence of recurrence. Overall survival is better with postoperative radiotherapy, which is delivered within 7 weeks of surgery. Partial horizontal laryngectomy: Excision of supraglottis, which include epiglottis, aryepiglottic folds, false cords and ventricle. Hemithyroidectomy or Subtotal Thyroidectomy: the Endoscopic significant debulking with laser or surgical forceps temporarily relieves the airway. Spread Local: Fixation of vocal cord is a bad prognostic sign and indicates involvement of thyroarytenoid muscle. Normal cord mobility suggests growth limited to the surface whereas impaired mobility indicates invasion into intrinsic laryngeal muscles or paraglottic space. In a case of postradiation edema, which persists for longer than 6 months, deep invasive recurrence must be ruled out. In cases of radiation failure, laryngectomy or partial vertical laryngectomy is indicated. Anterior commissure and/or arytenoids involved or cord mobility impaired: Vertical hemi-laryngectomy or frontolateral laryngectomy. Anterior: Growths of infrahyoid epiglottis and anterior ventricular band extend into preepiglottic space, and penetrate the thyroid cartilage. Those who have complete response (some include even partial response) are given complete radiotherapy. Those who have no response (some include even partial response) are treated with surgery, usually followed by radiotherapy. No difference in survival was found in these two groups; those who went for radiotherapy or surgery after the course of chemotherapy. SubglottIc cancEr Subglottic region lies below the glottic area, and extends up to the lower border of cricoid cartilage. Spread Local: One side growth of subglottis spreads around the anterior wall, and to the opposite side. Hoarseness of voice due to infiltration of thyroarytenoid muscle or recurrent laryngeal nerve at the cricoarytenoid joint. Erythrocyte leakage and endothelial damage of vessels cause ischemic necrosis of tumor tissue. T3 and T4: Total laryngectomy and postoperative radiation, which include superior mediastinum. Esophageal Speech Patient learns how to swallow air, and hold it in the upper esophagus. Some patients learn to store air in stomach, which greatly improves duration of speech. After Artificial Larynx Artificial larynx is useful when patients fail to learn esophageal speech. They are least socially acceptable because they draw 508 unwanted attention to the speaker. It produces low-pitched sound which is further modulated into speech by the tongue, lips, teeth and palate. The vibrating disc of artificial larynx is held against the soft tissues of the neck or oral cavity. Transoral Pneumatic Device: the expired air from the tracheostome vibrates the diaphragm of this pneumatic device. These rubber diaphragm vibrations are carried by a plastic tube into the back of the oral cavity where modulators convert the sound into speech. Supraglottic cancer: It is the most aggressive of laryngeal cancers and has highest incidence of cervical nodal metastases. Synchronous second primary: In cases of carcinoma larynx the most common site of second primary (synchronous second primary) is bronchus. Near total laryngectomy in advanced cancers of the larynx and pyriform fossa-a much suited procedure for the Indian Milieu. Survival patterns in treated cases of carcinoma larynx in north India: A 10-year follow up study. Failure to achieve a patent airway in cases of impaired airway, inevitably results in hypoxic brain injury and death. The tracheotomy means opening the trachea, which is a step of tracheostomy operation. Tracheostomy facilitates suctioning, feeding, mobility, early return of speech, and decrease work of breathing. Suction: Suction clearance of tracheobronchial secretions avoids the need for repeated bronchoscopy or intubation. Indications the indications can be categorized in three groups: upper respiratory obstruction, retained secretions, and respiratory insufficiency. Other indications include easier management of secretions and an adjuvant procedure during chest surgery, and major head and neck operations. Retained secretions Inability to cough: Coma due to head injuries, cerebrovascular strokes, accidents, narcotic poisoning. Paralysis of respiratory muscles due to spinal injuries, polio, Guillain-Barre syndrome and myasthenia gravis. Respiratory insufficiency: Emphysema, chronic bronchitis, bronchiectasis and atelectasis. The thyroid isthmus is dissected down (even cut with knife or diathermy) to expose upper three tracheal rings. Urgent (awake) Tracheostomy: this is done in the operation theater under local anesthesia with minimal sedation. It is performed where all operative surgical facilities such as endotracheal intubation, local and general anesthesia, are available. Therapeutic: It relieves not only the respiratory obstruction but also allows removing tracheobronchial secretions, and provides assisted ventilation. The indications include extensive surgery of tongue, floor of mouth, mandibular resection or laryngofissure. Temporary Tracheostomy: Elective tracheostomy is usually temporary, and is closed when causative disease is cured. In cases of laryngectomy and laryngopharyngectomy, a tracheostome is created where lower tracheal stump is stitched to the surface skin. The high tracheostomy is generally avoided because of the postoperative risk of perichondritis of the cricoid cartilage and subglottic stenosis. In cases of carcinoma larynx with stridor when total laryngectomy would be done, high tracheostomy is indicated. Mid Tracheostomy: It is done through the second and third tracheal rings, and needs either division of the thyroid isthmus or its retraction upwards. Low Tracheostomy: It is done below the level of isthmus where trachea becomes deep, and lies close to large vessels. The surgeon tends to displace trachea, and go deep or lateral to it; possibly injuring recurrent laryngeal nerve and common carotid artery. Therefore, tracheostomy in infants and children is done under general endotracheal anesthesia. Steps of Operation (figs 1 and 2) Skin Incision: A vertical midline cervical incision that extends from cricoid cartilage to just above the sternal notch is the most frequently used skin incision. Strap Muscles: Strap muscles are first separated in the midline and then retracted laterally. Thyroid Isthmus: the thyroid isthmus is either displaced upwards or divided between the clamps. Trachea: After injecting few drops of 4% lignocaine into the trachea that suppresses the cough, trachea is incised with a vertical incision in the region anywhere between second to fourth tracheal rings. When making incision in the tracheal wall, sharp tracheal hook is applied to the lower border of cricoid cartilage to stabilize the trachea. Proper humidification that prevents crusting is achieved by using humidifier, steam tent, ultrasonic nebulizer, or keeping a boiling kettle in the room. Tracheostomy tube: Inner cannula is removed, and cleaned regularly for the first 3 days to prevent respiratory distress. The patient requires endoscopic examination of the larynx, trachea and bronchi using telescopes or a flexible endoscope. Soft silastic and portex tubes are preferred over metallic tubes, which cause more traumas. Inability to tolerate the resistance of the upper airways (physiological dependence). Complications Complications of tracheostomy (Box 1) are traditionally categorized into three groups: immediate (intraoperative), intermediate (few hours to days), and late (weeks and months). Tracheoesophageal fistula is the result of injury to posterior wall of trachea and esophagus. Then, cricothyroid membrane is cut with a transverse incision, and subglottic larynx is entered. A small tracheostomy tube or any hollow tube like thing can be inserted to maintain airway. It is followed by tracheostomy, which is done at the earliest to avoid complications of cricothyrotomy such as perichondritis, subglottic edema and laryngeal stenosis. Clinical features the newborn develops intermittent, low-pitched inspiratory stridor within the first 2 weeks of life. Gastroesophageal reflux is probably secondary to increased negative intrathoracic pressure. Technique There are different systems and approaches to perform a percutaneous tracheostomy; the details of which are beyond the scope of this book. Cleft and craniofacial anomalies: the care of these patients needs many medical and surgical subspecialties and requires a team management. The incomplete recanalization of larynx results in the web which is seen between the vocal cords, and has a concave posterior margin. Sharp pointed foreign bodies (such as pins, needles, or fish bones) may stick in either larynx or tracheobronchial tree. All patients need genetic screening and cardiovascular evaluation especially of aortic arch. Thick web needs excision via laryngofissure, silicon keel placement, and subsequent dilatations. Direct Laryngoscopy with Telescope or Microscope: Reddish-blue mass below the vocal cords can be seen. They have a tendency to put the things in their mouth, which can go in either the food or the air passages. Laryngofissure: Surgical resection of circumferential or bilateral subglottic hemangiomas. Differential Diagnoses Laryngotracheobronchitis (see chapter Infections of Larynx). Diagnosis the detailed history, physical examination, and radiographs confirm the diagnosis. Larynx, Trachea and bronchus management Medical: Antibiotics, steroids and oxygen are administered immediately, and continued after the bronchoscopy. Pathology Depending on the mode and severity of injury, the following lesions can occur: Bruises: External cervical bruises. Hyoid bone Thyroid cartilage (vertical or transverse): Fracture of upper part of thyroid cartilage may cause avulsion of epiglottis and false cords. Fractures of lower part of thyroid cartilage can disrupt true vocal cords Cricoid cartilage Upper tracheal cartilages Hematoma and edema of supraglottic or subglottic region.

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Frequently allergy symptoms in ears 25 mg benadryl mastercard, these origins are far apart such that they may not be seen in one frame allergy symptoms cold symptoms order online benadryl. A combination of parasternal short- axis view and suprasternal views may have to be used to demonstrate each of the two branch pulmonary arteries allergy shots tendonitis purchase 25mg benadryl with visa. Paying careful attention to presence or absence of head and neck branches may help to avoid such misstep allergy guidelines 2015 benadryl 25mg without a prescription. Catheter Course In current clinical practice allergy medicine while pregnant second trimester purchase 25 mg benadryl fast delivery, the diagnosis of truncus arteriosus is usually known at the time of catheterization allergy treatment singapore purchase benadryl overnight. Cannulating each branch pulmonary artery for pressure measurement is necessary to evaluate any stenosis at their origin. Hemodynamic Evaluation Measurement of pulmonary artery pressures is important in the overall evaluation. Evaluation of pulmonary vascular resistance and demonstration of its reversibility becomes a crucial entity in older unoperated children being considered 611 8 cyAnoTic HeArT diseAses for surgical correction. Administration of 100 percent oxygen and/or inhaled nitric oxide is used to determine reversibility of pulmonary arterial hypertension. In order to estimate relative blood flow to each of the lungs, nuclear quantitative lung perfusion scans may be used. However, this technique has not been formally studied or validated for this application. Truncal Root Angiography Truncal root angiography will delineate truncal valve, number of cusps, truncal valve regurgitation, anatomy of truncus arteriosus, pulmonary artery relationships and probably origins and coronary arteries. When a common pulmonary artery is present, it is usually noted at the leftward and posterior part of the common arterial trunk. Confirming the position of each of the aortic arch branches helps to correctly interpret aortic arch anomalies. Selective Pulmonary Angiograms Special views may be needed if origins of the pulmonary arteries are to be shown. The ideal camera angles will vary in each patient and need to be determined based on various Pulmonary Venous Wedge Angiogram Pulmonary venous wedge angiogram may become necessary to identify native pulmonary arteries when these are not visualized otherwise. When there is increased pulmonary blood flow and congestive heart failure, therapy is indicated to address heart failure. High calorie diet may be necessary in infants who show signs of increased pulmonary blood flow and heart failure. For infants and children who are slightly cyanotic and not have features of increased pulmonary blood flow, active therapy may not be necessary. But, surgery should preferably be performed in early infancy for the fear of development of pulmonary vascular changes. Ventricular septal defect closure, which is part of this surgery is not shown in this figure. Banding individual pulmonary arteries is fraught with difficulty in getting the appropriate size band for each vessel. Exact timing of surgery may vary depending upon the specific features in each patient. Since the only approximately 10 percent of patient survive first year without surgery and there is a risk of rapid development pulmonary vascular obstructive changes, surgical repair usually is performed in early infancy if not as newborn. Usually, truncal valve does not require any surgical intervention, unless there is significant stenosis or regurgitation. When there is associated interrupted aortic arch, surgical repair has to be performed as newborn after initial stabilization on prostaglandin infusion. Surgical mortality is relatively high, when a combined repair of truncus arteriosus and interrupted aortic arch need to be performed. Presence of truncal valve regurgitation at presentation greatly worsens the prognosis for surgical outcome and survival. Surgical options include truncal valve repair, truncal valve replacement using homografts or mechanical valves; these have not produced good results, but may be necessary. Postoperative Management In addition to the usual postoperative care, immediate postoperative treatment includes management of episodes of pulmonary hypertensive crises. Considerable precautions are taken to avoid pulmonary hypertensive crisis by keeping the baby sedated with ample analgesics and sedatives. Some institutions routinely use muscle relaxants at least for the first night of surgery. Ventilatory strategy to maintain alkalosis and avoiding hypoxia and hypercarbia is instituted. There is also significant morbidity from pulmonary artery stenosis, usually at the distal anastamosis of the conduit- in the postoperative period. Such stenosis may need cardiac catheterization and angiography to demonstrate the stenosis. Balloon angioplasty of such conduit/pulmonary artery stenosis is not advisable due to fresh suture lines. Freedom from reoperation at 1 month was 69 percent, at 3 years 54 percent, at 5 years 30 percent, at 10 years 11 percent and 0 percent at 15 years. Survival after hospital discharge was 90 percent at 5 years, 85 percent at 10 years and 83 percent in 15 years. Independent risk factor for nonsurvival was preoperative presence of moderate or severe truncal valve regurgitation. In addition, 107 patients underwent 133 conduit replacements and 26 patients required 30 truncal valve replacements. Freedom from truncal valve surgery in the absence of any prior truncal valve repair was 95 percent at 10 years. There were 46 patients in the cohort with a median age of 62 days and a median weight of 3. Among associated anomalies, coronary anomalies were the commonest (n = 16); truncal regurgitation was noted in 12 and interrupted aortic arch in five. Two hospital deaths were noted (both with severe truncal regurgitation and interrupted aortic arch) and one death at 4 months after surgery. The predictor of reoperation among the patients was use of a smaller homograft size. Successful pregnancies have occurred in patients who have had truncus repair in the past-especially when the individual is in good functional state. Prepregnancy counseling should include checking the status of 22q11 microdeletion. Relief of bilateral branch pulmonary artery stenosis enabled weaning from positive pressure ventilator and postoperative recovery in this infant. Truncus arteriosus repair along with truncal valve repair was performed in 37 babies. Repair of truncus arteriosus and interrupted aortic arch was performed in 38 babies in whom a mortality of 60 percent was noted. In four babies, valve surgery was performed as second surgery after an initial truncus repair. Whether or not this advantage at short-term follow-up33 holds up at a longer-term follow-up remains to be seen. Freedom from conduit reoperation at 1 year was 92 percent and at 2 years, 76 percent. Balloon angioplasty35 and stents36,37 appear to prolong the life of the conduit and lengthen the interval between conduit replacements. Recently, older candidates with conduit stenosis and/or regurgitation have an option of transcatheter valves such as Bonhoeffer,38 Melody (Medtronic, Inc. Following stent implantation (arrow in B) the right ventricular pressure decreased and the gradient across this region abolished. The diagnosis is relatively simple and can often be suspected on clinical features and confirmed by echocardiography with a rare need for cardiac catheterization and selective cineangiography. Outcome depends on defect type, pulmonary artery anatomy, aortic arch anomalies and truncal valve function. Risk of recurrence of the genetic and cardiac defect in the offspring depends on genetic testing in the mother. Appropriate counseling should be provided to prospective mothers with repaired truncus arteriosus. The physician should look upon the patient as a besieged city and try to rescue him with every means that art and science place at his command. The anatomy of common aorticopulmonary trunk (Truncus arteriosus communis) and its embryologic implications. Pax3 is required for neural crest migration in the mouse: evidence from the splotch (Sp2H) mutant. Truncal Root Dilatation and Truncal Valve Regurgitation Truncal root dilatation and truncal valve regurgitation occur in most of the patients. In a recent study of a cohort of 78 patients, the mean truncal root diameter Z-score was 5. Arrhythmias Arrhythmias though rare can occur, also an important disorder after surgical repair. Progressive Pulmonary Vascular Disease 616 In patients with delayed detection, pulmonary vascular obstructive disease may develop. Selection of patients with truncus arteriosus for surgical correction: Anatomic and hemodynamic considerations. Surgical repair of truncus arteriosus associated with interrupted aortic arch: Long-term outcomes. Wide-angle twodimensional echocardiographic profiles of conotruncal abnormalities. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the society of thoracic surgeons congenital heart surgery database. Long-term follow-up of truncus arteriosus repaired in infancy: a twentyyear experience. Early reintervention on pulmonary arteries and right ventricular outflow tract after neonatal or early infant repair of truncus arteriosus using homograft conduits. Stents in the management of congenital heart disease in the pediatric and adult patients. Its management has been one of the amazing success stories as far as the treatment of various complex congenital heart defects in the last two decades is concerned. This treatise will discuss pure or isolated transposition of the great arteries, i. Transposition of the great arteries means that the origin of the great arteries from the heart is reversed. The genetic mechanisms and several mutations have been implicated as the cause of discordant ventriculoarterial connections. The genes which maybe involved are the growth differentiation factor-1 gene, the thyroid hormone receptorassociated protein-2 gene and the gene encoding the cryptic protein. The associated risk factors, namely gestational diabetes mellitus, maternal exposure to rodenticides, herbicides, and maternal use of antiepileptic drugs have been postulated. This infundibulum pushes the Simple transposition of great arteries usually presents in the neonatal age group with severe cyanosis. For the pediatrician handling a neonate presenting with severe cyanosis, the other differential diagnosis are pneumonia and/or sepsis. Because of the reversed connections, the venous blood the neonate, who escapes detection of cyanosis will present (deoxygenated blood) returning from the body goes back in infancy/early childhood with failure to thrive. Similarly, the subpulmonary valvar obstruction, a murmur may be the oxygenated or pure blood returning from the lungs via the presenting sign. The several anatomic and sufficient mixing will show a retarded physical and mental functional factors and the extent of intercirculatory mixing growth and succumb to ill effects of hypoxia and polycythemia. The most common variation is origin of the circumflex artery from the right coronary artery (16% incidence). Almost all anatomic features and even coronary arterial anomalies may be picked up by this imaging modality. An anomalous coronary artery can be suspected if in any view, a coronary is seen coursing posterior to the pulmonary artery. We describe the coronary arteries in terms of arising from sinus 1 (leftward sinus) and sinus 2 (rightward sinus). A description in words of what is seen on echocardiography most often conveys the anatomy better than complicated classification systems. This is especially true, when images are suboptimal and there is a suspicion of anomalous coronary. The only practical indication seems to be to assess pulmonary artery pressure and response to vasodilators in cases of late presentation when a palliative repair is being comtemplated. In fact, severe cyanosis accompanied by (paradoxically) increased pulmonary vascular markings on chest radiograph is a pointer to the diagnosis. Gradually, right ventricular hypertrophy manifests as upright T waves in the right precordial leads along with right axis deviation. If this does not help, balloon atrial the management of transposition of great arteries in a neonate septostomy is performed on an emergency basis. Occasionally,giving Neonatal care involves ensuring that the peripheral oxygen to the neonate may improve oxygenation as well. Monitoring and maintenance of the aim is to regulate the pulmonary and systemic vascular peripheral temperature, pulses, capillary refill time and circuits so that adequate mixing is achieved. Laboratory tests to assess brain the surgical corrective procedure of choice in the neonatal (ultrasound), kidneys (blood urea nitrogen, creatinine) and period and early infancy in simple and most of the complex liver function tests should be performed. The Lecompte maneuver brings the main pulmonary axis length of the ventricular cavity obtained from the 4 artery anterior and the ascending aorta posteriorly.

Anomalous origin of the left cornary artery from the pulmonary trunk with special reference to the occurence of mitral insufficiency allergy symptoms every morning purchase 25mg benadryl otc. Endocardial fibroelastosis: a clinical and anatomic study of 47 patients with emphasis on its relationship to mitral insufficiency allergy bed cover buy discount benadryl 25mg line. Dilated and contracted forms of primary endocardial fibroelastosis: A single fetal disease with two stages of development allergy forecast huntsville tx purchase benadryl uk. Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block allergy medicine l612 cheap 25mg benadryl with amex. Right and left heart catheterization and angiocardiographic findings in idiopathic cardiac hypertrophy with endocardial fibroelastosis allergy medicine ok to take when breastfeeding buy discount benadryl on-line. Endocardial fibroelastosis: A factor in heart disease of obscure etiology: A study of 20 autopsied cases in children and adults allergy testing tuscaloosa al generic benadryl 25 mg with visa. Endocardial fibroelastosis: Etiologic and pathogenetic considerations in children. Prospective single-arm protocol of carvedilol in children with ventricular dysfunction. Cardiac Transplantation in a Neonate with Endocardial Fibroelastosis, Tex Heart Inst J. In 2003, the American Academy of Pediatrics/ American College of Physicians presented a consensus statement on health care transition for young adults with special heath care needs. The following highlights key elements that should be included in a transitional plan of care in order to ensure an effective transfer program from childhood into adulthood (Box 1). The main problem with this model is that pediatric providers lack the experience of handling acquired heart diseases and addressing adult care issues. A second model proposes adult cardiologists as primary providers since they are well prepared to deal with acquired diseases and address adult issues. Adult-centered care is also more patient-centered and disease oriented thus it takes for granted that the patient has the necessary skills to self-manage his/her care. Throughout childhood, the pediatric cardiologist 10 Congenital Heart DiSeaSe in aDultS has served, in most cases, not only as the cardiologist, but as the primary care provider. As the child grows, parents need to be encouraged to identify a primary care physician (general physician) who assumes not only the responsibility of coordinating the care, but is also willing to work closely with cardiology and other subspecialities. The surgical team must be informed as to the relative risk of operation, including risks of bleeding, hypotension, hypovolemia and infective endocarditis. For females, gynecologic and reproductive issues are the leading non-cardiac health issues that health care providers must address. A number of gynecologic issues such as menstrual complaints and contraception frequently present in adolescents and require age appropriate counseling and referral. Over the years, legislation in many parts of the world has been enacted to reduce employment discrimination of individuals with preexisting disabilities. It is therefore important for health care providers to give career and vocational counseling that assists the patient in selecting a career. Career counseling and vocational guidance should be offered in middle and high schools so that based upon their intellectual abilities and interests, adolescents are encouraged to achieve higher education and skills necessary for employment in occupations that are manageable based on their work capacity. Even in developed countries, patients and their families often have to travel longdistances for specialized care and follow-up. While most countries have national health care systems that will provide care to this special population, many of these centers are not within the patients reach. It is important, therefore, to permit frank and open discussion on sexuality, childbearing and contraception for females. Introducing the topic early in the transition process sends the message to both teenagers and their parents that these are safe and welcome topics to discuss as part of their clinic visit. Early studies reported that only 50 percent of their population could name one situation considered at risk for acquiring endocarditis. The patients should be made aware of questions to ask with respect to needle sterilization before getting the tattoos. While the standards of needle sterilization and safety techniques have improved, the frequency of procedurerelated infections remains a concern. As adolescents enter high school and look to participate in higher level or competitive sports, exercise testing is helpful in gauging safety. Over the years, the American Heart Association and others have published recreational and sport recommendations for young persons with heart disease. Although oral contraceptives are effective when used by teenagers, it is estimated that 13 percent of teen users between ages of 15 to 17 years will miss an average of three pills per month. Injectables which are also safe still require regular return visits thus placing a certain level of responsibility on the teen to return to clinic. It should be presented in a sensitive and culturally appropriate manner that is in alignment with their individual level of emotional maturity and the value system of their society. It is important for health care providers to proactively address questions and concerns regarding their sexuality, the ability to conceive/bear children, the safety and availability of contraceptives based upon their underlying defects. Its goal is to outline routine health care recommendations and specific information on diagnoses, procedures, operations, medications, allergies, endocarditis prophylaxis, exercise, contraception and the recommended frequency of medical and dental follow-up. They should also carry a list of their cardiologist/health providers and personal emergency contacts. Therefore, such a summary is referred to as a health passport in that it is similar to a travel passport, because it carries vital information to ensure safe health care when one is away from his/her own providers. Patients are also encouraged to carry scanned copies of their medical reports on a password encrypted flash drive so that more detailed medical records are readily available during unforeseen circumstances. Even though this list is not complete, through these contacts more information can be sought at the local and regional levels by the health care providers and then passed on to the patients. Contraceptives the prevention of unplanned pregnancy in adolescent/young adult requires a collaboration between providers, patients and sometimes, their parents. Additionally, the adolescent health care provider should be familiar with the relation between oral contraception, particularly the estrogenic type and venous thromboembolism that makes this form of contraception hazardous for females who are cyanotic and/or in those with conditions such as right-to-left shunts, pulmonary vascular disease, prosthetic valves/conduits. These measures are to ensure a smooth transition into the adult heath care system and avoid the complications resulting from lack of proper long-term care. Transition to adult health care for adolescents and young adults with chronic conditions: Position paper of the Society for Adolescent Medicine. Transition and transfer from pediatric to adult care of the young adult with complex congenital heart disease. Prevalence and correlates of successful transfer from pediatric to adult health care among a cohort of young adults with complex congenital heart defects. Outcomes of noncardiac surgical procedures in children and adults with congenital heart disease. Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease. Best Practices in Managing Transition to Adulthood for Adolescents With Congenital Heart Disease: the Transition Process and Medical and Psychosocial Issues. Transitional care issues influencing access to health care: employability and insurability. Employability and career counseling for adolescent and adults with congenital heart disease. Sexual behavior and reproductive concerns among adolescents and young adults with congenital heart disease. Adults with congenital heart disease: Patient knowledge of endocarditis prophylaxis. Piercing and tattooing in patients with congenital heart disease: Patient and physician perspectives. Self-efficacy and physical activity in adolescents with trivial, mild, or moderate congenital cardiac malformations. A prospective comparison of bone density in adolescent girls receiving depot medroxyprogesterone acetate (Depo-Provera), levonorgestrel (Norplant), or oral contraceptives. Little did he know then that two of his female disciples would generate a new wave in the field of medicine. Maude Abbott, initially barred from studying medicine because she was a woman, with her undying persistence and perseverance acquired medical knowledge from universities around the world. She returned to Montreal, Canada where she soon became the curator of the McGill Pathological Museum in 1901. Categorizing in detail the various congenital heart defects over the course of decades, she published her work in the "Atlas of Congenital Cardiac Disease" in 1936. Her contributions have served as a foundation for understanding the pathology of congenital heart disease. She led the pioneering "blue baby operation" along with Vivien Thomas and Alfred Blalock in 1944. This set the stage for the advent of cardiac surgeries assisted by artificial circulation (bypass machine). Born in India and raised in Australia, Dr Wood became well-known for describing the Eisenmenger syndrome and for writing the first single author cardiology textbook, "Diseases of the Heart and Circulation". Deeply influenced by the chapters from this book on "Physical examination" and "Congenital affections of the heart" another disciple, Dr Joseph Perloff went on to write individual textbooks on these two topics. Advances in all aspects of medicine and surgery have allowed the majority of the infants born with congenital heart defects, 10 Congenital Heart Disease in aDults who have access to healthcare, to survive into adulthood. While most individuals born with congenital heart defects are diagnosed in infancy and childhood, some go undiagnosed until adulthood. There has been organized global effort to integrate multidisciplinary services devoted to this special population. An adequate Valsalva maneuver is essential, while assessing the presence of a right-to-left shunt by an agitated saline contrast study with transthoracic or transesophageal echocardiogram. In most studies, the clinical diagnosis of paradoxical embolism was presumptive and was based on the presence of a rightto left shunt in the absence of a left-sided thromboembolic source. A recent randomized trial (Closure I) did not detect any benefit of closure over medical treatment alone. Palpitations due to atrial arrhythmias and dyspnea are the most common presenting symptoms. Characteristic physical findings are a prominent right ventricular parasternal lift, persistent or wide fixed splitting of the second heart sound and a pulmonary systolic ejection murmur, at the left upper sternal edge due to increased pulmonary flow. Pulmonary hypertension leads to an accentuation of the pulmonary component of the second heart sound. A sinus venosus defect with associated anomalous pulmonary venous return is suspected, when there is right heart enlargement in the absence of a clearly defined interatrial shunt. The consensus is to close defects in symptomatic patients and in those with right heart enlargement. In the presence of pulmonary hypertension, the defect may be safely closed if pulmonary artery systolic pressure is less than 50 percent of the systemic arterial pressure. Right heart catheterization to assess the pulmonary vascular resistance may be required for those with higher pulmonary artery pressures. In general, all defects should be considered for early closure unless there are specific contraindications. Endocarditis/endarteritis prophylaxis is recommended for 6 months after device closure. It was later defined by Dr Paul Wood in 1958 as Eisenmenger syndrome with its distinctive clinical and physiologic characteristics. Although aware of a murmur all through their lives, many asymptomatic individuals are only diagnosed in adulthood. Physical examination is characteristic of a pansystolic murmur (and sometimes precordial). The pulmonic component of the second heart sound may be accentuated in the presence of pulmonary hypertension. The direction and the volume of an interventricular shunt are dictated by the size of the defect and the ratio of pulmonary vascular resistance to the systemic vascular resistance. The left heart experiences volume load and depending on the size of the defect, the right heart may experience a pressure load. In a large unrestrictive ventricular septal defect there is equalization of right and left ventricular pressures due to free communication between the two chambers. This leads to near systemic pressures in the pulmonary arteries that causes irreversible pulmonary vascular disease and pulmonary hypertension. Patent ductus arteriosus is a residual fetal communication between the proximal left pulmonary artery and the descending aorta distal to the left subclavian artery. During fetal life, it allows the diversion of blood from the right ventricle to the descending aorta, thus bypassing the pulmonary circulation. Physical examination is remarkable for a continuous machinery murmur at the left upper sternal border with radiation to the back. The risk of endarteritis is present in all patients, therefore antibiotic prophylaxis and early closure are recommended. Patients, who have increased pulmonary vascular resistance at the time of closure may present with late pulmonary hypertension. There is a higher likelihood of pulmonary artery dilatation and aneurysms due to an associated connective tissue disorder. Surgical valvotomy or pulmonary valve replacement is considered, when there is significant calcification, a thickened stiff dysplastic valve and/ or concomitant pulmonary artery aneurysm. Pulmonary valve replacement (transcatheter or surgical) is indicated for severe pulmonary regurgitation before it affects the right ventricular function.