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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

New developments in pediatric heart transplantation have influenced the nursing management of these patients. The patient population has changed over the past few years with an increase in patients with congenital heart disease and larger numbers of infants and young children. The management of patients prior to transplantation has become more complex, with new pharmacologic agents and the use of mechanical assist devices as a bridge to heart transplantation. The importance of accurate assessment and management of pulmonary hypertension both before and after transplantation have been recognized. FK506, a new immunosuppressant in clinical trial, appears promising as both a maintenance therapy and a rescue drug with fewer side effects than cyclosporine. The trend toward outpatient and home care for many transplant-related therapies reflects the wider trend in health care. The practice of the critical care nurse involved in pediatric heart transplantation has been influenced by these recent changes.
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PMID:New directions in pediatric heart transplantation. 159 42

Thirteen out of 268 children (less than 18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD.
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PMID:Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome). 162 41

Five children underwent lung transplantation for end-stage pulmonary hypertension and respiratory insufficiency associated with congenital heart disease. One (17 mo) had pulmonary hypertension with a patent ductus arteriosus and required two periods of preoperative extracorporeal membrane oxygenation before successful bilateral sequential lung transplantation. One (21 mo) required bilateral lung transplantation for pulmonary hypertension and bronchopulmonary dysplasia associated with iatrogenic injury to the left pulmonary artery. This child also had patent ductus arteriosus ligation and preoperative catheter closure of an atrial septal defect. Extracorporeal membrane oxygenation was required for early postoperative pulmonary support. One child underwent right single-lung transplantation and closure of an atrial septal defect for pulmonary hypertension. Two patients had single-lung transplantation for Eisenmenger's syndrome: 1 with muscular inlet ventricular septal defect closure, atrial septal defect closure, and right single-lung transplantation; 1 with ventricular septal defect closure, patent ductus arteriosus ligation, right ventricular outflow tract patch repair, and single-lung transplantation. All patients survived operation, with one late death (lymphoproliferative disease). The 4 survivors are all ambulatory without oxygen and have evidence of normal pulmonary artery pressure 9 to 12 months after transplantation.
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PMID:Pediatric lung transplantation for pulmonary hypertension and congenital heart disease. 163 8

The relation between pulmonary vascular pathology and hemodynamics in 81 patients with congenital heart disease complicated by pulmonary hypertension was studied. Slight to marked increase of mean pulmonary pressure (MPAP) was seen in 5 of them, but no vascular lesion was found. 59 of the 81 cases had marked increase of MPAP and Grade 1-2 pulmonary vascular lesions, and cyanosis was seen clinically. Postoperative effects were satisfactory, indicating that not all patients with cyanosis contraindicated surgical treatment. The rest (17) of the 81 patients had most marked increase of MPAP and Grade 3-4 pulmonary vascular lesions with good postoperative results, but long-term results await follow-up observations. It is thought that Grade 4 pulmonary vascular lesion should be considered as a contraindication for corrective surgery.
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PMID:[Comparison between pulmonary vascular pathology and hemodynamics in congenital heart disease with pulmonary hypertension]. 164 27

A case of congenital mitral stenosis with pulmonary hypertension is presented. The severity and rapid development of the pulmonary vascular changes are unusual, especially for a young child. The case underscores the importance of early detection of correctable congenital heart disease and emphasizes the unpredictability of the time course and severity of irreversible pulmonary hypertension.
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PMID:Pulmonary hypertension with necrotizing arteritis secondary to congenital mitral stenosis. 167 84

The patient presented is a 27-month-old male with complex congenital heart disease consisting of severe left ventricular outflow tract obstruction and ventricular septal defect who had undergone a pulmonary trunk-to-aorta graft and a pulmonary artery banding procedure as a neonate. Sometime after this repair, but at least 15 months prior to presentation to this institution for placement of an aortic homograft, the pulmonary trunk band apparently slipped and migrated over the right pulmonary artery, severely limiting blood flow to the right lung and increasing flow to the left. Severe pulmonary hypertension developed, with a main pulmonary artery pressure of 94/53 mm Hg. We present clinical and radiographic evidence that the resulting chronic high blood flow and pressure in the left lung ultimately resulted in hypoperfusion of that lung, presumably secondary to chronic vascular changes with greatly increased vascular resistance. Upon surgical repair and removal of the constrictive band from the previously banded right PA, blood flow was increased to the low resistance right lung causing right-sided unilateral pulmonary edema, ventilation/perfusion mismatching, and severe hypoxemia. Perfusion studies documented that less than 10% of blood was directed to the left lung, with greater than 90% to the right. Perfusion studies 9 months postoperatively continued to demonstrate minimal blood flow to the left lung. Discussion focuses on the effects of mechanical forces and the interaction with hypoxia in causing pulmonary vascular remodeling.
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PMID:Unilateral pulmonary hypertension as a result of chronic high flow to one lung. 169 72

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

The state of the greater and lesser hemodynamics in patients with rheumatic mitral heart disease complicated by pulmonary hypertension was analyzed after treatment with peripheral vasodilators and hemosorption. It is suggested that inclusion of hemosorption in the treatment complex favoured reduction of the degree of pulmonary hypertension, improvement of the central hemodynamics which may be directly related to improvement of the rheological properties of the blood.
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PMID:[Hemodynamic changes in the greater and lesser circulations of patients with a mitral heart defect and primary pulmonary hypertension under the influence of differentiated treatment]. 180 46

Children with Down syndrome have many predisposing factors for the obstructive sleep apnea syndrome (OSAS), yet the type and severity of OSAS in this population has not been characterized. Fifty-three subjects with Down syndrome (mean age 7.4 +/- 1.2 [SE] years; range 2 weeks to 51 years) were studied. Chest wall movement, heart rate, electroculogram, end-tidal PO2 and PCO2, transcutaneous PO2 and PCO2, and arterial oxygen saturation were measured during a daytime nap polysomnogram. Sixteen of these children also underwent overnight polysomnography. Nap polysomnograms were abnormal in 77% of children; 45% had obstructive sleep apnea (OSA), 4% had central apnea, and 6% had mixed apneas; 66% had hypoventilation (end-tidal PCO2 greater than 45 mm Hg) and 32% desaturation (arterial oxygen saturation less than 90%). Overnight studies were abnormal in 100% of children, with OSA in 63%, hypoventilation in 81%, and desaturation in 56%. Nap studies significantly underestimated the presence of abnormalities when compared to overnight polysomnograms. Seventeen (32%) of the children were referred for testing because OSAS was clinically suspected, but there was no clinical suspicion of OSAS in 36 (68%) children. Neither age, obesity, nor the presence of congenital heart disease affected the incidence of OSA, desaturation, or hypoventilation. Polysomnograms improved in all 8 children who underwent tonsillectomy and adenoidectomy, but they normalized in only 3. It is concluded that children with Down syndrome frequently in have OSAS, with OSA, hypoxemia, and hypoventilation. Obstructive sleep apnea syndrome is seen frequently in those children in whom it is not clinically suspected. It is speculated that OSAS may contribute to the unexplained pulmonary hypertension seen in children with Down syndrome.
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PMID:Obstructive sleep apnea in children with Down syndrome. 182 51

Laparoscopic tubal sterilization under local anesthesia with intravenous sedation has been shown to be a safe procedure. However, the use of laparoscopy in patients with cyanotic cardiovascular disease is controversial and is generally contraindicated. Five women were referred with uncorrectable cyanotic heart disease and pulmonary hypertension. The mean preoperative arterial oxygen pressure was 56.2 +/- 5 mmHg (N = 5). After cardiology and cardiovascular anesthesia consultation and clearance, the patients underwent laparoscopic sterilization with Silastic rings under local anesthesia using direct trocar entry. Continuous hemodynamic monitoring and pulse oximetry were employed. The patients were kept in the intensive care unit or the hospital for 24 hours for monitoring, and all did well. This hospital for 24 hours for monitoring, and all did well. This small retrospective series demonstrates that laparoscopic sterilization under local anesthesia is a sterilization technique that may be suitable and safe for such patients when appropriate monitoring is performed. Tubal sterilization may be the contraceptive method of choice in women with heart disease when pregnancy is contraindicated.
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PMID:Laparoscopic tubal sterilization under local anesthesia in women with cyanotic heart disease. 183 53


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