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1.
Opinion statement  
–  Transposition of the great arteries (TGA) is a lethal condition without intervention.
–  Cross-sectional echocardiography is the diagnostic investigation of choice.
–  Intravenous infusion of prostaglandin is employed to maintain ductal patency and allow mixing of blood, thus improving tissue oxygenation.
–  Balloon atrial septostomy is recommended once the diagnosis is made.
–  The arterial switch is accepted as the best option for simple TGA.
–  Late follow-up includes survivors of the intra-atrial repair (Mustard and Senning operations), and the emerging cohort of survivors of the arterial switch procedure.
–  Arrhythmia, baffle stenosis, tricuspid valve dysfunction, systemic ventricular dysfunction, and sudden death may occur late during follow-up after the Mustard or Senning procedure.
–  There are less data for late follow-up after arterial switch; however, late death is rare, usually is related to reoperation, and important arrhythmias are uncommon. The long-term fate of the coronary circulation is unknown but coronary arterial obstruction has been reported.
–  Continuing long-term surveillance is essential to detect the development of late problems in all groups of survivors.
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2.
Opinion statement  
–  Balloon angioplasty or early surgical correction is recommended once coarctation of the aorta has been diagnosed. Medical management is not a long-term option.
–  The rate of restenosis is higher in infants and children than in adults, but the incidence of residual or late hypertension increases with age at initial intervention.
–  Current angioplasty and stent placement techniques have reduced the rate of aneurysm formation and expanded the types of complex anatomic configurations amenable to nonsurgical intervention.
–  Patients require long-term follow-up for restenosis and late or residual hypertension.
–  Coarctation treatment is straightforward in patients with simple isolated coarctation. In neonates with associated lesions and diffuse arch hypoplasia, aggressive (albeit higher-risk) arch-enlargement procedures can be done at the time of open-heart correction of intracardiac defects.
–  Changes in aortic compliance, vascular reactivity, and vascular homeostasis mechanisms in patients who have late surgery are associated with a higher incidence of residual hypertension.
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3.
Opinion statement  
–  Endoscopic sphincterotomy followed by retrieval balloons or baskets is 90% to 95% effective in removing common bile duct stones, and should be first-line therapy.
–  Mechanical lithotripsy should be available for stones that are difficult to extract.
–  Failure to clear the duct can be managed with temporary stenting, which may facilitate stone extraction at a later date; if not, either extracorporeal shock-wave lithotripsy, or intraductal laser lithotripsy is successful in the majority of cases.
–  Balloon sphincter dilation should be considered in patients who are at high risk of bleeding from sphincterotomy due to coagulopathy.
–  Surgical common bile-duct exploration can be performed as a last resort for removal of common duct stones.
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4.
Opinion statement  
–  Most symptomatic internal hemorrhoids, grade 1 through 3, can be treated successfully with office-based procedures.
–  Anorectal suppurative diseases must be treated surgically. Control of sepsis with subsequent fistula surgery as necessary is the goal.
–  New nonoperative methods of anal fissure therapy are directed at reducing anal sphincter pressures. These methods have shown significant reduction in the need for sphincterotomy—a proven surgical technique with some risk of impaired continence.
–  Surgery, using an advancement flap and partial internal sphincterotomy, remains the primary treatment for anal stenosis.
–  Solitary rectal ulcer remains a difficult problem to manage medically and surgically.
–  Multiple surgical techniques can effectively treat rectal prolapse. A minimal technique using Silastic wrap (Wright Medical Technologies; Arlington, TX), perineal resection (Altemeier procedure), and sigmoidectomy-rectopexy, or Ripstein suspension, has been the most favored method in selected patients.
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5.
Amebiasis     
Opinion statement  
–  More than 80% of cases of amebic liver abscess can be managed with a 14-day course of intravenous or oral metronidazole. In cases of suspected amebic liver abscess, treatment should be started before diagnostic confirmation.
–  If no clinical improvement is evident by 72 to 96 hours, treatment should be changed to dehydroemetine and chloroquine.
–  Invasive treatment is necessary only in patients in whom medical treatment fails within 5 days or in whom signs of clinically severe disease are present.
–  A 10-day course with a luminal agent such as paromomycin to eliminate intestinal cysts, which are resistant to imidazoles, should always follow treatment of the liver abscess.
–  Percutaneous catheter drainage is indicated in patients with impending rupture, with a lesion 6 cm or more in diameter, with an abscess located in the left lobe or high in the dome of the right lobe, or in whom medical treatment fails.
–  Although sympathetic pleural effusion is not an indication for drainage, direct pulmonary involvement or spread to pleural or lung tissues requires drainage.
–  Intraperitoneal rupture and peritonitis necessitate open surgical drainage.
–  Only a small minority of amebic liver abscesses are secondarily infected by other organisms.
–  Because relapses are possible, feces should be checked for cysts monthly for several months after therapy.
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6.
Opinion statement  
–  Fulminant ulcerative colitis necessitates immediate hospitalization.
–  Supportive therapy such as aggressive rehydration, restriction of oral intake, and consideration of parenteral nutrition should be initiated.
–  High-dose intravenous steroids should be started in almost all cases.
–  Antibiotics and cyclosporine should be considered, especially in disease refractory to steroid therapy.
–  Indications for surgery should always be kept in mind, and early involvement of the surgical team is always encouraged.
–  Avoidance of life-threatening complications such as toxic megacolon, hemorrhage, and perforation is the goal of any treatment for fulminant ulcerative colitis.
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7.
Opinion statement  
–  Subvalvular aortic stenosis (SAS) is a congenital heart defect that causes fixed form of hemodynamically significant left ventricular outflow tract (LVOT) obstruction with progressive course. It has a spectrum of anatomy.
–  It appears usually beyond infancy, causes left ventricular hypertrophy and myocardial dysfunction, and tends to involve the aortic and mitral valves in its progressive course. Although most of the patients are asymptomatic, careful monitoring is essential.
–  Moderate to severe SAS requires surgical resection and septal myomectomy. There is a high rate of postoperative recurrence of the lesion. Recurrent lesions and the complex type of lesions with aortic valve involvement should have aortoseptoplasty (to enlarge the outflow tract) and Ross procedure (removal of the damaged aortic valve and placement of a pulmonary autograft in the aortic position and a pulmonary homograft in the pulmonary position).
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8.
Opinion statement  
–  The treatment of sarcoid cardiomyopathy can be considered in part the treatment of the systemic disorder, and in part cardiac involvement, the manifestations of which may differ greatly. Therapy for the systemic disease is corticosteroid. Therapy for cardiac involvement includes prednisone, but because treatment must ameliorate or abolish many differing manifestations, therapy differs among patients.
–  Asymptomatic patients (the majority) who are free from serious manifestations of the disease do not require pharmacologic or interventional treatment.
–  Patients with dilated cardiomyopathy require treatment for congestive heart failure.
–  High-grade atrioventricular conduction delay usually necessitates a permanent electronic pacemaker.
–  Life-threatening arrhythmia usually requires implantation of an automatic implantable cardiac defibrillator (AICD). Antiarrhythmic drugs may also be needed.
–  Cardiac tamponade should be treated by drainage of pericardial fluid.
–  Pericardiectomy is usually the appropriate treatment for patients who develop significant constrictive pericarditis.
–  Calcium channel blockers may be helpful for severe diastolic dysfunction that occurs in those with restrictive cardiomyopathy.
–  Therapy should be given to those few patients who manifest hypertrophic cardiomyopathy to relieve left ventricular outflow obstruction.
–  Cardiac transplantation for intractable heart failure or arrhythmia may be needed.
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9.
Opinion statement  
–  An oral calcium supplement (1000 mg/day) is recommended.
–  Regular exercise should be performed.
–  Ethanol intake should be moderate.
–  Protein intake should be moderate.
–  The patient’s vitamin D status should be determined and corrected with an oral supplement when deficiency is present.
–  Baseline and yearly bone density measurement should be taken.
–  Alendronate, 10 mg/d orally, or risedronate, 5 mg/d orally, should be given to patients with osteopenia.
–  Use of corticosteroids, cyclosporin, tacrolimus, and methotrexate should be limited to the short term when possible.
–  Estrogen replacement therapy is recommended in postmenopausal women unless contraindications exist.
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10.
Opinion statement  
–  Dysplasia is the most important marker of progression to invasive cancer in Barrett’s esophagus.
–  Intensive endoscopic surveillance with biopsy may identify invasive cancer in a patient with high-grade dysplasia (HGD).
–  Close relationship with an experienced gastrointestinal pathologist and thoracic surgeon will improve treatment decisions and patient outcomes.
–  No intervention is required in patients with low-grade dysplasia (LGD); continued surveillance is recommended.
–  Surgical resection is the currently accepted therapy for high-grade dysplasia. Endoscopic ablative therapy remains experimental.
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11.
Opinion statement  |
–  After a significant coronary artery abnormality is recognized in a pediatric patient, surgery or appropriate transcatheter intervention should be performed. The risk of fatality from a congenital coronary abnormality far outweighs the small risks of surgical or transcatheter intervention.
–  Angiography, although considered the state-of-the-art method of diagnosis, has significant spatial limitations and is not always diagnostic of aberrant coronary origins from the contralateral aortic sinus. In the hands of an experienced coronary imager, color flow Doppler echocardiography is one of the best diagnostic tools for congenital coronary abnormalities.
–  Symptoms of a coronary abnormality vary from none to a sudden coronary event that may result in death. Awareness of subtle as well as obvious symptoms is essential for a timely intervention.
–  Surgical or transcatheter intervention in an asymptomatic child with a coronary abnormality is controversial, but it is becoming more acceptable due to a better understanding of the risks involved in unrepaired congenital coronary abnormalities.
–  Surgical reimplantation is the treatment of choice for a patients with a pulmonary origin of a coronary artery. Surgical unroofing of the intramural segment is preferable in an aberrant coronary origin from the contralateral aortic sinus. Surgical enlarge-ment of a stenotic ostium is recommended for ostial stenosis. Transcatheter coil embolization is becoming the treatment of choice of large coronary artery fistula.
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12.
Opinion statement  
–  There is no definitive therapy for this disorder.
–  Heliobacter pylori eradication therapy is only useful in areas with a high prevalence of H. pylori-related ulcer disease
–  Proton pump inhibitors are effective in short-term treatment for dyspepsia with a predominant symptom of epigastric pain
–  Prokinetic agents may be useful in some patients, particularly those with dysmotility-like dyspepsia, but serious side effects limit their usefulness.
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13.
Hemochromatosis     
Opinion Statement  
–  The mainstay of treatment for hemochromatosis is therapeutic phlebotomy. The procedure is safe, effective, and relatively cheap.
–  Deferoxamine (iron chelation) is necessary only in iron-loaded individuals who cannot undergo therapeutic phlebotomy.
–  If therapy is initiated before complications develop, it is expected that no complications will ever develop.
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14.
Opinion Statement  
–  Early diagnosis permits preventive therapy to preempt development of organ damage.
–  In all diagnosed patients, both symptomatic and asymptomatic, pharmacologic therapy is lifelong, and maintenance treatment to prevent copper toxicity is mandatory.
–  Patients with either fulminant hepatic failure or hepatic insufficiency unresponsive to medical therapy should be considered for orthotopic liver transplantation, which effectively cures Wilson’s disease.
–  Prognosis is excellent for compliant patients receiving pharmacologic therapy.
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15.
Opinion statement  
–  Esophageal malignancies presenting with dysphagia from luminal obstruction generally are not resectable for cure, and palliative therapy is the primary focus.
–  Self-expandable metal stents (SEMS) have replaced plastic stents as a primary mode of palliation for malignant esophageal obstruction because of the relative ease of insertion, lower initial morbidity, and larger stent diameter.
–  Self-expandable metal stents are ideal for patients with midesophageal tumors.
–  A majority of patients experience relief of dysphagia with SEMS and dietary modification, but the initial cost is high and early morbidity may be significant.
–  The placement of SEMS across the gastroesophageal junction may result in free reflux that may improve with a stent containing a one-way gastric flap valve.
–  The placement of SEMS in the cervical esophagus, although more difficult, less effective, and less well tolerated, also may be successful.
–  Coated SEMS are a treatment of choice for individuals with tracheoesophageal fistula.
–  Delayed complications occur in up to 40% of patients and include stent migration, bleeding, perforation, fistula formation, and occlusion. Most complications can be managed endoscopically and additional stents may be placed for tumor overgrowth.
–  The comparison of three currently available SEMS for esophageal malignancy show no statistically significant differences with regard to ease of placement, effectiveness, complications, and mortality.
–  The use of SEMS for patients with benign disease is still considered experimental.
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16.
Opinion statement  
–  Autoimmune hepatitis can be treated effectively with either prednisone alone or a lower dose of prednisone in combination with azathioprine.
–  All types of autoimmune hepatitis should be treated similarly.
–  The combination schedule of prednisone and azathioprine is preferred since it is associated with fewer side effects than a higher dose of prednisone alone.
–  Lifestyle changes are essential if overeating, under-exercising, excessive alcohol consumption, or overexposure to hepatotoxic medication or chemicals are common.
–  Adjunctive therapies, including calcium supplementation, vitamin D, and hormonal replacement, are important in postmenopausal women to preserve skeletal integrity.
–  Initial treatment should be continued until complete clinical, laboratory and histologic resolution. Relapse after attainment of an “ideal” end point tempers the goals of subsequent treatments.
–  Relapse after a second treatment and drug withdrawal is associated with a diminishing benefit-risk ratio for retreatment. Long-term, indefinite therapy with low dose prednisone or azathioprine alone is the appropriate treatment strategy.
–  Deterioration despite compliance with conventional therapy (treatment failure), incomplete response, and drug toxicity are adverse outcomes that warrant modifications in the conventional treatment protocol. High dose corticosteroid regimens, longterm low dose maintenance schedules, and investigational drugs can be considered.
–  Liver transplantation is an effective strategy for patients who have decompensated disease despite corticosteroid therapy.
–  Tacrolimus and budesonide are the most promising new therapies for autoimmune hepatitis and controlled treatment trials are sorely needed.
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17.
Opinion statement  
–  Venous malformations of blue rubber bleb nevus syndrome (BRBNS) may involve any area of the gastrointestinal tract.
–  Gastrointestinal blood loss and anemia brings these patients to the attention of gastroenterologists.
–  Effective treatment of these malformations throughout the gastrointestinal tract requires aggressive management to ultimately decrease blood loss and restore the patient’s hemoglobin to a near-normal level.
–  Treatment of patients with BRBNS includes supportive measures, endoscopic ablation, and surgery.
–  Supportive therapy consists of proton pump inhibitors and octreotide to decrease blood loss, iron replacement, and blood transfusions.
–  The effective management of patients with anemia demands aggressive treatment of venous malformations in the small bowel. This requires a collaboration between the surgeon and the therapeutic endoscopist, ie, laparotomy and excision of larger lesions with surgically assisted enteroscopy and thermal ablation of smaller lesions via enterotomy.
–  There is no effective systemic therapy for treatment of the vascular malformations in patients with BRBNS.
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18.
Hepatic cysts     
Opinion statement  
–  Treatment of hepatic cysts should be considered only for those patients who are symptomatic.
–  For simple cysts, percutaneous aspiration invariably leads to recurrence; laparoscopic deroofing is usually curative.
–  Open deroofing (fenestration) should be reserved for cysts inaccessible by laparoscopy.
–  Percutaneous instillation of sclerosing agents (ethanol, iophendylate, minocycline) into nonbiliary and nonparasitic cysts is an alternative therapeutic option in certain cases.
–  Due to increased morbidity, hepatic resection should be reserved for polycystic liver disease, diffuse hepatic involvement, or recurrence after a deroofing procedure.
–  Patients with congenital fibropolycystic disorders (eg, congenital hepatic fibrosis) with evidence of hepatic decompensation, should be considered for liver transplantation.
–  For hepatic hydatid cysts, simple cystectomy or the PAIR (puncture, aspirate, inject, and reaspirate) technique with albendazole treatment have been shown to be equally successful.
–  In the case of alveolar echinococcosis, hepatic resection and liver transplantation are the only effective modalities for localized and extensive hepatic disease, respectively.
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19.
Gallstones     
Opinion statement  
–  Gallstone disease is a common clinical problem that poses particular difficulties for our aging and increasingly obese population.
–  Exciting new research suggests an infectious cause for most cholesterol stones.
–  The keys to managing patients with gallstone disease are recognition of the wide spectrum of clinical disease resulting from gallstones and a flexible, multidisciplinary approach to diagnosis and treatment.
–  The biliary tract has been a proving ground in the development of minimally invasive technology.
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20.
Opinion statement  
–  Untreated, the prognosis for patients with tricuspid atresia (TA) is poor. Recent advances in medical and surgical therapy, particularly the application of Fontan principle, have markedly improved the long-term outlook for children with this condition.
–  Palliative procedures to normalize the pulmonary blood flow and to relieve interatrial or interventricular obstruction should be undertaken promptly.
–  Staged total cavopulmonary connection to bypass the right atrium and right ventricle by an initial bidirectional Glenn procedure and followed by extracardiac conduit diversion of inferior vena caval flow into the pulmonary arteries appears to be the current procedure of choice in the surgical management of TA.
–  Total cavopulmonary diversion appears to be superior to conventional Fontan-Kreutzer operations, but long-term follow-up results are needed to confirm this impression.
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