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

Over a 21-month period 200 fetal echocardiographic studies were performed on pregnancies at high risk for congenital heart disease referred to the NSW Fetal Echocardiography Service. Four cases of hypoplastic left heart syndrome were diagnosed in-utero during this period. These cases demonstrated the heterogeneity of this lesion, its known association with other defects and chromosomal anomalies, and evidence of in-utero evolution. Hypoplastic left heart syndrome is reliably diagnosed by fetal echocardiography. This lesion is heterogenous and may evolve beyond the first trimester. Careful attention to ventricular function, endocardial echoes and Doppler flow may assist with diagnosis.
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PMID:Prenatal diagnosis of hypoplastic left heart syndrome. 817 75

Hypoplastic left heart syndrome (HLHS) has been widely viewed as a uniformly fatal form of congenital heart disease. Between January 1984 and December 1990, 387 patients with the diagnosis of HLHS were treated at this institution. Mesenteric ischemia was clinically diagnosed in 31 patients (8% incidence) and confirmed by pathology or surgery in 25 of those patients. The mean age at the time of onset was 17.5 +/- 5.4 weeks and only 13% were premature newborns. In 80% of the patients a low perfusion state and significant hypotension were documented within 48 hours prior to the diagnosis of bowel ischemia. Nine patients (29%) required operative intervention (bowel resection 4, diffuse ischemia 3, and simple drainage 2). Overall, at operation or at autopsy, nine patients (29%) had diffuse gastrointestinal ischemia. Of 31 patients with mesenteric ischemia, 26 children (84%) died shortly after onset of the gastrointestinal symptoms regardless of means of management. Five patients (10%) initially improved with aggressive medical and/or surgical management; however, 4 subsequently died secondary to complications of their primary cardiac disease. Therefore, the overall mortality of patients with mesenteric ischemia was 97%. Previous reports have estimated that up to 7% of full-term newborns with symptomatic congenital heart disease may develop necrotizing enterocolitis (NEC). Our unique group of patients with HLHS is comprised mostly of full-term infants who developed onset of mesenteric ischemia at a mean age of 4 months associated with an underlying low perfusion state. This mesenteric ischemia has been erroneously identified as NEC.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Mesenteric ischemia in hypoplastic left heart syndrome. 848 77

Hypoplastic left heart syndrome (HLHS) is the most severe form of congenital heart disease (CHD), which until recently was considered to be fatal. However, surgical intervention is now possible using neonatal heart transplantation or three-stage palliative surgery (Norwood's staged operations). In Malta, during the period 1977-1994, there were four cases of classic HLHS, with an additional three cases of HLHS-like cases. The birth prevalence of classical HLHS was 0.04 per 1000 live births, whereas that of combined HLHS-like cases was 0.06 per 1000 live births for the same period. One neonate with HLHS is expected every 3 or 4 years. Despite a high rate of ascertainment of CHD, the birth prevalence of HLHS in Malta was significantly lower than that quoted in historical studies (p < 0.006) and in the European Congenital Anomalies and Twins Registry (p < 0.002). Malta has the expected birth prevalence of CHD, but the spectrum of CHD exhibits a predominance of lesions causing right ventricular outflow obstruction and a deficit of lesions causing left ventricular outflow tract obstruction. CHD is caused by a genetic-environmental interaction. The low rate of HLHS in Malta appears to be a true divergence from the usual observed spectrum of CHD and may be caused by genetic and/or environmental factors.
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PMID:Decreased prevalence of hypoplastic left heart syndrome in Malta. 1044 91

Hypoplastic left heart syndrome (HLHS) is the most common cause of death from heart disease in the first week of life. There are reports about familial concordance by presumed morphogenetic mechanisms of abnormal embryonic blood flow with phenotypes of varying severity. The risk of having a child with a left heart lesion after a previously affected child may be as high as 5% to 12%. We present case reports from four families in which sustained fetal arrhythmia (three ectopic atrial tachycardias and one severe bradycardia due to excessive ectopic atrial beats) was demonstrated. Within these four families a close relative of the mother (a previous child, a brother, or a nephew) had severe left heart abnormality (three with HLHS and one with severe aortic valve stenosis). The association of sustained fetal arrhythmia of ectopic atrial origin and severe left heart abnormalities could be expected to occur by chance in a very low percentage of cases. We conclude that sustained fetal atrial ectopic arrhythmia is a congenital abnormality and should be considered as a risk factor for inherited congenital heart abnormalities.
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PMID:Familial association of congenital left heart abnormalities and sustained fetal arrhythmia. 1044 94

Hypoplastic left heart syndrome (HLHS) is found in a wide spectrum of changes of the mitral valve, left ventricle and aorta. It represents a critical congenital heart disease with a small left ventricle and stenosis or atresia of the aortic and the mitral valve. The knowledge of a detailed anatomy of this lesion is necessary from the viewpoint of assessment of all possibilities of the surgical treatment, interventional cardiological procedures or foetal cardiac surgery. Sixty one hearts with HLHS from the collection of heart specimens with congenital heart malformations were reviewed. The existence of a wide spectrum of malformations of all heart structures was proved. In all the cases enlarged right atrium and right ventricle was found. In 23.0% of specimens foramen ovale was restrictive or closed. The left ventricle was small in all the hearts, extreme hypoplasia was observed in 26.2% in presence of a combination of the mitral and the aortic atresia. Most commonly, the combination of mitral stenosis with aortic atresia (36.1%) and combination of mitral and aortic stenosis (32.8%) were present. Mitral atresia with aortic stenosis was identified in 4.9% cases. The mitral valve was usually dysplastic with short and thick chordae and smaller annulus. In 39.3% of all hearts fibroelastosis of the left ventricular endocardium was present. In these cases moderate degree hypoplasia of the left ventricle in combination with mitral and aortic stenosis was often found (65.0%). All the aorta was hypoplastic starting from the aortic valve, across the ascendent part and aortic arch until the isthmus. Its diameter usually varied between 2 and 4 mm. Coarctation of the aorta was found in 16.4% hearts. Persistent left superior caval vein represented the most common associated heart lesion. From the surgical point of view, nearly in all reviewed hearts Norwood surgery, consisting in aortic reconstruction from aorta, main pulmonary artery and a patch from the pulmonary homograft could be performed. Reconstruction would be very difficult in presence of an extreme hypoplasia of the ascending aorta. Until present, possibilities to prevent the development of the left ventricular hypoplasia by a foetal surgical intervention or possibility of the surgical augmentation of the left ventricle by excision of a thick endocardium do not cross the horizons of theory.
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PMID:[Morphology of the hypoplastic left heart syndrome from the surgical perspective]. 1145 12

Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.
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PMID:The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome. 1549 71

Hypoplastic left heart syndrome (HLHS) represents a lethal congenital heart disease, which used to be considered inoperable. The improvement of results of cardiac surgery in neonates improved also the prognosis of patients with this heart lesion. HLHS, however, continues to be an unresolved problem from ethical, psychological, social and economical points of view. Opinions regarding the care for these patients depend on economical, political, cultural and religious factors. Based on recent experience it is evident, that HLHS is an operable disease in a child, who without surgery would otherwise die. Approximately 40% of patients with HLHS can live 15 years after surgery. The long-term prognosis remains, however, unclear. Because of the need of demanding care for a patient with HLHS, it is necessary to see also the quality of life and hear the opinion of parents. Psychomotor development and the quality of life may be altered by complications. An individualized approach in fetuses and neonates with HLHS on the basis of prenatal diagnosis, analysis of risk factors, provision of detailed informations to parents and indications for surgery in neonates having chance for an acceptable result is considered to be the most acceptable solution. In the future it will be necessary to concentrate the research on prenatal diagnostics, treatment and postoperative follow-up including psychomotor development and quality of life. In the preparation of legislative, which would reflect more sensitively the contemporary possibilities of medicine and at the same time regard the problems of health care organization, as well as social and economic aspects, narrow interdisciplinary cooperation will be required.
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PMID:[Ethical, psycho-social, legislative and economic aspects of surgical treatment of the hypoplastic left heart syndrome]. 1207 83

Hypoplastic left heart syndrome (HLHS), a complex congenital heart disease, is the most common lethal cardiac defect in neonates. Its treatment includes cardiac transplantation and/or surgical palliation. Associated extracardiac congenital abnormalities are exceptional. We report the case of a neonate with HLHS and anorectal atresia who required urgent surgical management to relieve intestinal obstruction. The surgery was successfully performed under spinal anaesthesia.
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PMID:Spinal anaesthesia in a child with hypoplastic left heart syndrome. 1264 89

Fetal echocardiogram is a reliable tool for prenatal diagnosis of congenital heart disease. It is an important adjunction to obstetrical ultrasonography in caring for women during pregnancy. With improvement in resolution of 2-D echo images as well as the addition of parameter software, fetal echocardiography has found ever-expanding applications in both the structural and functional assessment of the fetal cardiovascular system. From the author's 12 years experience in fetal echocardiogram, 1,000 fetal hearts were studied and 12 cases had congenital heart defects (CHD): 4 ventricular septal defects, 1 Ebstein's anomaly, 1 Tetralogy of cantrell, 1 single atrium and single ventricle, 1 Hypoplastic left heart syndrome, 2 abnormal tricuspid valve with tricuspid regurgitation, 1 calcification RV, LV and interventricular septum with hydrops fetalis. Three hydrops fetalis were of unknown cause. Fetal heart size was evaluated by comparing the heart area/size with the chest area/size. The ventricular dimension was assessed by M-mode and 2-D echo. The ventricular volume could be measured by 2-D echo with a special formula. Evaluation of ventricular function was important and could be evaluated by M-mode, 2-D echo and Tissue Doppler Image (TDI). Pulsed Doppler and Doppler Colour Flow Image gave more information about the hemodynamics. The assessment of the foramen ovale, ductus venous, ductus arteriosus arteries including the umbilical artery and vein were also important and would be abnormal if the fetal heart had ventricular dysfunction and congestive heart failure. The fetal echocardiographic findings will help the obstetrician for further investigation and/or treatment including termination of pregnancy. The author would like to recommend that fetal echocardiogram be performed in all pregnant women at 18-22 weeks of gestation. The incidence of CHD in low risk pregnancy is as significant a finding as in high-risk pregnancy.
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PMID:Some aspects of fetal echocardiogram: 12 years of experience. 1286 67

Hypoplastic left heart syndrome (HLHS) is a challenge for the pediatric cardiologist and the surgeon. It is generally assumed that the postoperative outcome after surgery for congenital heart disease is influenced by the institutional size. We present the results of 43 patients with true HLHS (situs solitus and atrioventricular and ventriculoarterial concordance) referred for operation between 1992 and 2002 in our center. Two children had atrioseptostomy: one died soon after the operation, and the other one was transplanted successfully but died at the age of 6 months following acute rejection. The remaining 41 underwent Norwood I palliation, 21 stage II palliation, and 10 stage III palliation. Early mortality was 29% after stage I operation, 4.7% after stage II palliation, and 0% after stage III operation. Overall mortality was 39% after stage I, 9.5% after stage II, and 10% after stage III operation. Low birth weight was associated with a higher mortality (p < 0.05). Mortality declined with increasing experience, comparable to the results of very large cardiosurgical centers with many more patients. The quality of surgery and perioperative management in smaller pediatric cardiosurgical centers can reach the level of very large centers.
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PMID:A decade of staged Norwood palliation in hypoplastic left heart syndrome in a midsized cardiosurgical center. 1613 81


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