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

Between February 1983 and December 1985 authors have studied 26 newborn infants with severe congenital heart disease who received prostaglandin E1 infusion as therapy. Onset of therapy was before 5 days of age in 15 patients, between 5 and 7 days of age in 7 cases and after 14 days of age in the remaining ones. Initial dose was 0.05 mcg/kg/minutes and mean duration of infusion was 131.9 +/- 19.0 hours. Patients were divided into 3 groups according to pulmonary and systemic blood flows: Group I, with cyanosis and diminished pulmonary blood flow (12 cases); Group II, with diminished systemic blood flow (10 cases); and group III, with cyanosis and normal or increased pulmonary blood flow (4 cases). Most patients of groups I (10/12) and II (9/10) improved levels of blood PO2: mean increments of 17.8 +/- 7.4 and 17.5 +/- 13.3 torr respectively. Peripheral pulses also improved in four extremities in patients from group II; systolic and diastolic pressures increased 16 +/- 9.6 and 5 +/- 2.2 mmHg, respectively. No improvement was observed in patients from group III, with exception of patients with transposition of great vessels. Most frequent complication of prostaglandin infusion was hyperthermia, which was observed in 16 cases but disappeared in all with moderate reduction of doses. Appropriate surgery was performed in 19 patients, with an overall survival of 52.6%. In conclusion, prostaglandin infusion appears to be a very valued tool to improve the hemodynamic condition in newborn infants with severe congenital heart disease and thus allow the practice of corrective surgery during first month of life.
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PMID:[Prostaglandins in newborn infants with severe cardiopathy]. 360 73

Prior to echocardiography, the recognition of serious heart disease in the cyanotic newborn or young infant could be extremely difficult. The profound hemodynamic changes taking place in the heart and lungs after birth influence the clinical manifestations of many cardiac disorders, and sometimes suggest the existence of a cardiac disorder when none is present. Real time echocardiography has revolutionalized the diagnosis of the cyanotic infant. If the reason for the infant's cyanosis or respiratory distress is not apparent from the history, physical examination, laboratory values, and chest radiograph; real time echocardiography should be performed to exclude or diagnose cyanotic congenital heart disease and persistent fetal circulation. This will prevent misdiagnosis in cyanotic infants and assure rapid and appropriate treatment.
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PMID:Radiologic and real time echocardiographic evaluation of the cyanotic newborn. 368 8

To define settings in which use of prostaglandin E1 before transfer from a community hospital to a tertiary care center benefits neonates with possible heart disease, information theory was used to predict the probability of a favorable response to prostaglandin therapy from the limited information of clinical variables. Records of 250 patients, newborn to 7 days old, with suspected heart disease were reviewed to assess six clinical variables (cyanosis, respiratory distress, heart murmur, pulse contour, hepatomegaly and prematurity). According to the anatomic and hemodynamic cardiovascular condition, each case was categorized as to whether a favorable response to prostaglandin E1 could be anticipated. Information content of each clinical variable with respect to prostaglandin responsiveness was determined, and patients were classified according to the most informative clinical variable. Stepwise extraction of information proceeded until remaining clinical variables added no significant information. Bayes' rule gave estimates of probability of prostaglandin-responsive defect in final subgroups for use in decision analysis. Cyanosis, murmur, small volume pulses and prematurity gave information about prostaglandin-responsive defects. Decision analysis indicated that frequency of poor outcome is minimized by early prostaglandin treatment of cyanotic term infants with a murmur or poor pulses, regardless of how ill they appear, and by treating any critically ill term newborn who has either cyanosis or poor pulses. Acyanotic patients with normal pulses are best untreated with prostaglandin until after definitive diagnosis is made. Advantage to either course was not seen in some small subgroups. Information theory with decision analysis is a rigorous approach to identify relevant clinical variables and define their roles in critical decisions in pediatric cardiology.
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PMID:Application of information theory to decision analysis in potentially prostaglandin-responsive neonates. 376 Mar 86

We report a case of previously healthy male with hypotension and cyanosis with no evidence of underlying congenital heart disease, pulmonary hypertension, pulmonary artery aneurysm, or central venous or pulmonary artery catheter insertion. Autopsy revealed a spontaneous rupture of the pulmonary artery with hemopericardium.
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PMID:Spontaneous rupture of pulmonary artery. 381 63

Peripheral neuroblastic tumors (PNT), including neuroblastomas, pheochromocytomas, ganglioneuromas, and paragangliomas, have been reported in association with congenital heart disease (CHD). If a significant correlation between PNT and CHD could be demonstrated, it would suggest that peripheral neuroblastic tumors might represent reactive, secondary phenomena rather than independent or incidental events. We examined this question by reviewing the autopsy files of The Johns Hopkins Hospital and found that the frequency of congenital cardiovascular problems among patients with PNT was significantly greater than the frequency of CHD in the general autopsy population (p less than 0.001). There was no correlation between CHD and central neuroblastic tumors (CNT), i.e., medulloblastomas, retinoblastomas, or pinealoblastomas, or between CHD and metastatic malignant melanoma. We examined the hypothesis that erythropoietic activity, as might be stimulated by hypoxia, was correlated with the presence of peripheral neuroblastic tumors. We found that most (87%) cardiac malformations associated with PNT produced cyanosis, and histologic assessment of hematopoiesis revealed greater activity in patients with PNT (with or without CHD) compared to those with CNT (p less than 0.05) or malignant melanoma (p less than 0.05). In addition, patients with PNT and CHD had higher frequencies of extramedullary hematopoiesis compared to age-matched controls with similar congenital heart defects (p less than 0.05), and compared to patients with CNT or metastatic malignant melanoma (p less than 0.01). These results suggest that peripheral neuroblastic tumors may arise in a setting of chronic hypoxia produced by congenital heart disease.
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PMID:Peripheral neuroblastic tumors and congenital heart disease. Possible role of hypoxic states in tumor induction. 384 56

Seventeen patients 1 day to 28 months old with congenital cyanotic heart disease underwent a modification of the Blalock-Taussig shunt. Eight were newborn infants weighing 2.6 to 4.8 kg. All infants had complex congenital heart defects that were not considered amenable to early correction. There were no early deaths and no shunt failures. Postoperative complications were restricted to prolonged intubation in 2 patients and a subcutaneous wound infection in a 14-day-old infant. Follow-up from 1 to 31 months revealed minimal cyanosis, stable hemoglobin levels, and good shunt murmurs, and there have been no upper extremity complications. There were 2 late deaths; 1 (the oldest patient) was related to medication, and the second resulted from small bowel necrosis. The concept of the azygos vein patch modified Blalock-Taussig shunt involves two factors: (1) mobilizing as much length as possible of the subclavian artery in spite of its distal small size to allow for a tension-free shunt to prevent tension on the anastomosis as growth occurs, and (2) enlarging the subclavian artery distal to the vertebral artery origin with an autologous azygos patch. During performance of a standard Blalock-Taussig shunt, a longitudinal incision is made through the pulmonary artery across the anastomosis into the upper subclavian artery. The appropriate length of harvested azygos vein is used as a patch angioplasty across the shunt. A tension-free shunt with a patulous distal portion is achieved. Pulmonary overcirculation is avoided by the limiting size of the proximal subclavian artery. Temporary occlusion of the shunt at operation resulted in an increased mean blood pressure from 6 to 18% in all infants.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Technique and experience with azygos patch modified Blalock-Taussig anastomosis for congenital cyanotic heart disease. 400 96

The authors have studied 787 women with cardiac disease out of 78,594 pregnant women between 1971 and 1980. They found 133 women who had congenital heart disease who were delivered in their service, and they compared these with 260 pregnant women who were normal. They used the classification of the Cardiological Society of New York to grade the functional state of the patients. Five pregnancies had to be terminated before the 16th week because of a worsening of the cardiovascular state of the patient. The majority (79%) of the patients delivered vaginally and 21% had caesarean operations. 20.5% babies were born prematurely (p less than 0.005). 13.4% of the infants weighed less than 2,000 g at birth. Perinatal mortality was 62.9 per 1,000. Maternal mortality was 23.6 per 1,000. The authors conclude that these pregnancies are high-risk pregnancies where the prognosis is only favourable if the heart disease is moderate. This becomes worse when there is cardiac decompensation or cyanosis due to heart disease.
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PMID:[Non-surgically corrected congenital cardiac malformations in pregnant women]. 403 29

A neonate with total anomalous pulmonary venous connection draining via the right cardinal vein into the superior vena cava had a chest-X-ray unsuspicious for congenital heart disease, and initially was treated for neonatal sepsis. But as the clinical state impaired and cyanosis increased, sectorechocardiography revealed the right diagnosis. The child died soon after corrective surgery.
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PMID:[Unremarkable heart and mediastinal configuration in a newborn infant with totally anomalous pulmonary venous anastomosis to the superior vena cava]. 405 33

The liver in children contains a greater amount of connective tissue in relation to parenchymal tissue than in adults and there is often more in the left than in the right lobe. Livers from 150 children who died between birth and 12 years of age, under different circumstances, were studied.The findings suggested that hypoxia was an important factor leading to increase in fibrous tissue in children's livers. Children who had cyanosis at birth and prolonged hypoxia associated with pathological conditions showed the highest ratio of connective tissue to liver parenchyma. The predominant non-hepatic lesions found were cyanotic congenital heart disease, coarctation of the aorta, acute respiratory distress, congenital malformation of the central nervous system, and shock after abdominal surgery.
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PMID:Hypoxia and asymmetrical fibrosis of the liver in children. 473 3

The differential diagnosis of congenital heart disease which presents cyanosis or respiratory distress, or both, in the first two weeks of life, is difficult. Close correlation of clinical features, electrocardiogram and chest roentgenogram is most helpful. The diagnosis of congenital heart disease should lead to immediate cardiac catheterization, angiocardiography and appropriate therapy.
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PMID:Diagnosis of congenital heart disease in the first two weeks of life. 577 78


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