Cases reported "Heart Defects, Congenital"

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1/19. Ultra fast track in elective congenital cardiac surgery.

    BACKGROUND: Changes in healthcare delivery have affected the practice of congenital cardiac surgery. We recently developed a strategy of limited sternotomy, early extubation, and very early discharge, and reviewed the perioperative course of 198 pediatric patients undergoing elective cardiovascular surgical procedures, to assess the efficacy and safety of this approach. methods: One hundred ninety-eight patients aged 0 to 18 years (median 3.2 years) underwent 201 elective cardiovascular surgical procedures over a 1-year period. All patients were admitted on the day of surgery. Patients were divided into six diagnostic groups: group 1, complex left-to-right shunts (n = 14, 7.0%); group 2, simple left-to-right shunts (n = 83, 41.3%); group 3, right-to-left shunts with pulmonary obstruction (n = 33, 16.4%); group 4, isolated, nonvalvular obstructive lesions (n = 30, 14.9%); group 5, isolated valvular anomalies (n = 20, 10.0%); and group 6, miscellaneous (n = 21, 10.4%). RESULTS: After 201 procedures, 175 patients (87.1%) were extubated in the operating room and 188 (93.6%) within 4 hours from operation. Four patients (2.0%) were extubated more than 24 hours from completion of the procedure, and 2 (1.0%) died while on respiratory support (never weaned). Five patients (2.6%) failed early extubation (<4 hours). Early discharge was achieved for the vast majority of patients. overall median length of stay (LOS, including day of surgery as day 1) was 2.0 days, with a median LOS of 3.0 days for those patients requiring circulatory arrest duration exceeding 20 minutes. Of 195 patients, 43 (24.6%), 121 (74.0%), and 159 (81.5%) were discharged, respectively, at <24, <48, <72 hours from admission. Longest and shortest mean postoperative LOS were in group 6 (9.9 /-14.5 days) and group 2 (1.6 = 0.7 days), respectively. Six patients (2.9%) died, and 11 (5.5%) suffered in-hospital complications. Thirty patients (15.4%) were either treated as outpatients (n = 11, 5.7%) or readmitted (n = 19, 9.7%) within 30 days from the time of surgery. Only 8 of 195 patients (4.1%) were readmitted with true surgical complications requiring invasive therapeutic procedures. CONCLUSIONS: Selected patients with a broad spectrum of congenital heart disease may enjoy same-day admission, limited sternotomy, immediate extubation, and very early discharge with excellent outcomes and acceptable morbidity.
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2/19. Successful staged-Fontan operation in a patient with ectopia cordis.

    ectopia cordis is a very rare and often fatal disease. We report a successful surgery case of thoracoabdominal ectopia cordis with univentricular heart. This patient underwent a three-stage fontan procedure, a right-modified Blalock-Taussig shunt at the age of 1 month, bidirectional Glenn shunt and pulmonary arterioplasty at 2 years 8 months, and finally a total cavopulmonary connection at 4 years. This patient was discharged from the hospital in good condition and has been doing well since. Thus, ectopia cordis is not a contraindication for a Fontan operation.
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3/19. pulmonary edema in 6 children with down syndrome during travel to moderate altitudes.

    OBJECTIVE: Children with down syndrome (DS) are living longer and are increasingly participating in recreational activities. When a child with DS was diagnosed with high-altitude pulmonary edema (HAPE), this study was undertaken to determine whether and under what circumstances children with DS develop HAPE. DESIGN: A retrospective review of the medical records of Children's Hospital, Denver, colorado was performed for children with a discharge diagnosis of HAPE. Diagnostic criteria for HAPE included the presence of crackles or frothy sputum production on examination, hypoxemia, chest radiograph findings consistent with pulmonary edema, and rapid clinical improvement after descent or oxygen therapy. RESULTS: A total of 52 patients with HAPE were found of whom 6 also had DS. The age range of the children with DS was 2 to 14 years. HAPE developed at altitudes ranging from 1738 to 3252 m. Four children developed HAPE within 24 hours of arrival to altitude. Three children had chronic pulmonary hypertension, and 4 had either an existing cardiac defect with left-to-right shunt or previously had a defect with left-to-right shunt that had been repaired. One child had Eisenmenger syndrome with chronic right-to-left shunting of blood. Five children had preexisting illnesses before travel to altitude. CONCLUSION: Children with DS often have medical problems such as chronic pulmonary hypertension, frequent infections, and pulmonary vascular overperfusion and injury from existing or previous cardiac defects. These problems all may be viewed as risk factors for HAPE and thus result in the rapid development of HAPE at low altitudes. Care should be taken when traveling to even moderate altitudes with children with DS.
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4/19. Closure of the fenestration in the extracardiac Fontan with the Amplatzer duct occluder device.

    We report the successful use of the Amplatzer duct occluder for the delayed closure of the fenestration in three patients who underwent an extracardiac modified fontan procedure. At the moment of closure, the patients were 5.5, 2.7, and 3 years old (29 months, 3 months, and 14 months after the fontan procedure, respectively). Immediate full occlusion was achieved in all cases. In addition, arterial saturation increased significantly (> 5%) with no hemodynamic deterioration. There were no complications during or after the procedure, and the patients were discharged in good conditions the day after and with uneventful follow-up. In conclusion, the Amplatzer duct is safe and effective for the closure of the fenestration in the extracardiac Fontan. Cathet Cardiovasc Intervent 2001;54:88-92.
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5/19. Aortico-left ventricular tunnel: two new cases with a long-term follow-up.

    Aortico-left ventricular tunnel is a rare congenital communication between the ascending aorta and the left ventricle. Its hemodynamic effect is severe aortic incompetence. Surgery is the only treatment and should be performed before aortic incompetence or ventricular dilation develops. Two neonates with aortico-left ventricular tunnel were operated on at our institution, with closure of the aortic end of the tunnel with a Gore-Tex patch. The 2 patients were discharged in good conditions, and at 112 and 42-month follow-up respectively they are in good health, without medication and with a normal echocardiographic pattern. Aortico-left ventricular tunnel should be treated surgically as soon as possible in order to prevent any damage to the aortic valve and the left ventricle. The operative risk is not low, but results are very encouraging.
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6/19. Corrected transposition of the great arteries with several associated anomalies in a 68-year-old patient.

    Few patients with corrected transposition of the great arteries survive past 50 years of age because of the association with congenital defects, development of total atrioventricular block, and right ventricular dysfunction. We report the case of a male patient with dextrocardia in situs solitus and corrected transposition of the great arteries associated with a wide atrial septal defect and severe pulmonary valvar and subvalvar stenoses. The patient also developed a large aneurysm on the pulmonary artery, total atrioventricular block diagnosed 8 years earlier, symptoms of dysfunction of the systemic ventricle in the previous 2 years, insufficiency of the left atrioventricular valve, and aortic regurgitation. Despite all these associated anomalies, the patient developed class III cardiac decompensation only at the age of 68 years, which makes this case a rarity. The patient was clinically treated, and was discharged from the hospital in good condition.
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7/19. Independent lung ventilation in a patient with complex congenital heart disease.

    Independent lung ventilation (ILV) is recognized as a method of treating unilateral lung disease. We report the use of ILV in a 22-year-old woman with acute respiratory failure and complex congenital heart disease with different sources of left and right pulmonary blood flow. She had a palliated single-ventricle circulation with pulsatile pulmonary blood flow from an aorto-pulmonary central shunt to her left lung and nonpulsatile pulmonary blood flow via a classic Glenn shunt (superior vena cava to right pulmonary artery). On admission she was hypoxemic and hypotensive. Her chest radiograph revealed opacification of the left lung and hyperinflation of the right lung, which was more compliant than the left lung. Following placement of a double-lumen endotracheal tube, synchronized ILV was instituted. ILV allowed us to deliver lower ventilator pressure to the right lung, which alleviated the over-distention of the right lung (to which pulmonary blood flow was supplied by the nonpulsatile Glenn shunt) while higher airway pressures were delivered to the diseased left lung, to facilitate re-expansion. There was immediate improvement in gas exchange and blood pressure. After 3 days the double-lumen endotracheal tube was changed to a single-lumen tube. She was extubated on day 6 and discharged on day 13. This case demonstrates the advantage of ILV in a patient with abnormal pulmonary blood flow and different lung mechanics in the left and right lungs.
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8/19. Using the resiliency model to deliver culturally sensitive care to Chinese families.

    In 1998, 4% of U.S. children were Asian/Pacific Islander; of these, 23% were Chinese. The incidence of infants with congenital heart defects (CHD) in Chinese children approximates that of Caucasian children in the united states. parents of children with CHD are faced with caring for the children at home after surgery. parents' roles and responsibilities expand significantly as their children return home with complex care needs. In pediatric nursing, we not only take care of the individual child but also the family as a whole. The family is the primary institution in society that preserves and transmits culture. Understanding Chinese culture and its association with health care practice helps nurses to establish culturally sensitive nursing interventions; develop appropriate discharge plans; decrease family stress; reduce future medical hospitalization costs; increase positive coping, adaptation, and family function; and improve health outcomes of the child and the family. The Resiliency Model was used to examine its applicability in caring for Chinese families in this paper.
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9/19. coloboma and other ophthalmologic anomalies in Kabuki syndrome: distinction from charge association.

    Kabuki (Niikawa-Kuroki) syndrome is associated with growth retardation, developmental delay, congenital heart disease, cleft palate, and characteristic facial features. Although the external appearance of the eyes has been well-described, the type and frequency of structural and functional eye anomalies has not been emphasized. We report three children with Kabuki syndrome who also had a retinal coloboma. A diagnosis of CHARGE association was initially suggested in two of the patients before the typical facial features of Kabuki syndrome emerged. A detailed review of reported cases of Kabuki syndrome shows that a variety of eye anomalies are associated with Kabuki syndrome. The incidence of coloboma is greatly increased in Kabuki syndrome. Thus, ophthalmologic abnormalities are frequently associated with Kabuki syndrome, and an ophthalmologic evaluation should be performed for each patient. Phenotypic overlap, including congenital heart, ear, and renal defects, can lead to the diagnosis of CHARGE association, especially since the typical facial features of Kabuki syndrome may not be apparent in early infancy. Thus, Kabuki syndrome should be considered in patients with coloboma if other features consistent with this condition are present, and follow-up evaluations are indicated for establishing the proper diagnosis.
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10/19. noninvasive ventilation for the management of pulmonary hypertension associated with congenital heart disease in children.

    The use of noninvasive ventilation to support children with secondary pulmonary hypertension has not previously been reported. We present four children with secondary pulmonary hypertension in association with complex congenital and acquired cardiorespiratory anomalies who have been successfully managed in hospital and then discharged into the community on noninvasive ventilation, thus placing them in environments more suited for growth and development.
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