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

In this article, the determinants of hypoxemia and cyanosis are analyzed and discussed. The pathophysiology, clinical presentation, diagnostic evaluation, and treatment of major cyanotic forms of congenital heart disease also are reviewed.
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PMID:Evaluation of the cyanotic newborn. 240 97

A standard Glenn anastomosis between the superior vena cava and the right pulmonary artery has been the accepted mode of treatment for patients with complex cyanotic congenital heart disease. We report our experience in 18 patients with such disease who underwent a bidirectional cavopulmonary shunt because of increasing cyanosis and growth cessation. All patients were considered less than "ideal" candidates for a Fontan procedure. We divided the patients into two groups: group 1 had azygos continuation and group 2 did not. Fourteen patients required hypothermic cardiopulmonary bypass. Bidirectional pulmonary blood flow was achieved in all patients. Only 1 death occurred (group 2). The improvement in oxygen saturation and overall clinical condition of these patients, together with the low mortality and morbidity, is encouraging. However, long-term follow-up is mandatory for a comprehensive evaluation of this surgical approach as definitive palliation or as a first stage for a Fontan operation.
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PMID:Bidirectional cavopulmonary shunts: clinical applications as staged or definitive palliation. 246 31

A characteristic sinusoidal tcPO2 pattern was observed in 17(27%) of 62 patients with congenital heart disease manifested by cyanosis or heart failure during the neonatal period. All of these 17 patients were definitively diagnosed by cardiac catheterization, as having 8 PPA; 3 ToF; 2 TGA + VSD + PA; 2 asplenia with PA; 1 TGA + VSD + PS; and 1 TA (no TGA type). The transcutaneous oxygen pressure pattern of these patients showed a sinusoidal change with a cycle length of 6-20 min with the maximum pressure not exceeding 50 torr and the minimum pressure occasionally close to 0 torr. This sinusoidal tcPO2 pattern was associated with various types of ductus-dependent congenital heart diseases and hence may be of diagnostic value. Transcutaneous oxygen pressure monitoring is also useful in evaluating the ductal response to PG. Other possible mechanisms underlying this phenomenon including biological oscillation should be considered.
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PMID:The value of the sinusoidal tcPO2 pattern in the evaluation and management of cyanotic congenital heart disease with ductus dependent lesions. 250 23

The patent ductus arteriosus occlusion device (Rashkind-USCI) was employed to occlude a residual ventricular septal defect after Fontan procedure in a patient with hypoplastic right ventricle, ventricular septal defect, and pulmonary stenosis. There was significant right-to-left shunting across the ventricular septal defect, with cyanosis exacerbated by exercise. After placement of the 17 mm occlusion device, the right-to-left shunt was markedly diminished, and the cyanosis resolved. Occlusion of ventricular septal defects in patients with complex congenital heart disease may be performed with the patent ductus arteriosus occluder in selected instances.
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PMID:Transcatheter occlusion of ventricular septal defect. 252 5

Two patients with complex cyanotic congenital heart disease and without previous Glenn anastomosis underwent modified Fontan operations. Postoperatively, pulmonary arteriovenous malformations developed that resulted in cyanosis from significant intrapulmonary right-to-left shunting. These malformations were detected by pulmonary angiograms and contrast echocardiograms. Pulmonary arteriovenous malformations may be a major cause of late clinical deterioration in patients treated with modified Fontan operations.
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PMID:Development of pulmonary arteriovenous malformations after modified Fontan operations. 258 19

The manifestations of carcinoid heart disease are usually right heart failure due to pulmonary and tricuspid valve disease and sometimes high output failure due to peripheral vasodilation. Recently there has been one case report of progressive central cyanosis due to right to left shunting through a patent foramen ovale. We present a similar case where such a shunt developed and was also complicated by significant valvular disease affecting the left side of the heart.
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PMID:Left heart involvement with cardiac shunt complicating carcinoid heart disease. 263 66

The Epstein's anomaly--a congenital heart disease--is an exceptional rarity in medicine and cardiology. A case of this anomaly is described. The patient was admitted to hospital with cardiac and neurologic complaints. The accompanying Wolf-Parkinson-White (WPW) syndrome type B created diagnostic difficulties concerning the basic diagnosis. The lipothymic states in childhood, the presence of cyanosis, bulging neck veins, divided I cardiac tone, systolic murmur at the cardiac apex and the conduction anomaly directed the diagnostic thinking towards a congenital heart disease of the right cavities.
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PMID:[Ebstein's anomaly observed in an adult patient]. 263 84

As this translation of Fallot's conclusions makes clear, he emphasized the clinical diagnostic usefulness of his findings because of the high frequency of this malformation in cyanotic congenital heart disease. Terming this anomaly "tetralogy," he clearly and simply delineated its four cardinal features. He stated that cyanosis was not due to a patent foramen ovale. He attributed the morphogenesis of the tetralogy to an intrauterine pathologic process at the level of the pulmonary valve leaflets and the subpulmonary infundibulum. The tetralogy and Fallot's contribution are reassessed in the light of present understanding.
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PMID:Etienne-Louis Arthur Fallot and his tetralogy: a new translation of Fallot's summary and a modern reassessment of this anomaly. 233 64

Intracardiac masses in infancy are not common. Tumors, thrombi and vegetations of bacterial endocarditis are exceedingly rare in this age group. These masses are seldom diagnosed before the infant's death. Two-dimensional echocardiography is a noninvasive technique that can detect and differentiate intracardiac masses that occur in infants. It can serially detect changes in the size or location of the masses, thus guiding subsequent medical or surgical intervention. An 11-day-old male newborn had suffered from lip and finger nail cyanosis, and dyspnea since 2 days old. Physical examination revealed subcostal retractions and no heart murmur during admission but a grade II/VI systolic murmur with a sometime grade II/VI continuous murmur which could be heard at the left upper sternal border 3 days after admission. The liver was palpable 2 cm below the right costal margin. The electrocardiogram was normal. The chest X-ray showed a normal sized heart (CTR = 0.58) with decreased pulmonary vascularity. Arterial blood gases revealed hypoxemia (PO2 24.1 mmHg) and metabolic acidosis, even though the patient was receiving O2 at 50%. The two-dimensional echocardiogram from the parasternal long axis view showed an echo-dense tumor mass in the right ventricular cavity. At autopsy, a complex congenital heart disease was found. The pathologic findings were: patent ductus arteriosus (1.0 x 0.5 cm), fenestrated foramen ovale, unopening pulmonary valve with thrombus (0.3 x 0.2 cm), right ventricular hypoplasia and hypertrophy of the right ventricular wall (1 cm in thickness). A ball like, organized and calcified thrombus, measuring 2.0 x 1.3 x 1.0 cm was found in the right ventricular cavity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Right ventricular thrombus mimicking a tumor mass in a newborn with pulmonary atresia--an autopsy report]. 273 76

Since 1984 five patients who had previously had cavopulmonary shunts for cyanotic congenital heart disease have returned to the Birmingham Childrens' Hospital with increasing breathlessness and cyanosis. Two had had a classic Glenn (unidirectional) cavopulmonary shunt and three, a bidirectional shunt; all shunts had been performed 5 to 18 years earlier. Each patient was reinvestigated and underwent creation of a brachial artery-basilic vein fistula. This procedure is known to have good patency rates when used in other situations (for example, for hemodialysis), but it has not been previously described for use in this context. Of the five patients, four had a considerable and sustained symptomatic improvement on follow-up of 12 to 49 months (mean 31 months), but the condition of one continued to deteriorate; that child has subsequently undergone a Fontan procedure, also with no improvement. Only one patient complained of coldness of the arm, and there were no other complications. We believe a brachial artery-basilic vein fistula has considerable advantages in terms of ease of operation and postoperative complications when compared with an axillary arteriovenous fistula, used as a supplement to an inadequate cavopulmonary shunt.
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PMID:Creation of brachial artery-basilic vein fistula. A supplement to the cavopulmonary shunt. 275 54


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