Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 13 day old baby was admitted to hospital with multiple abnormalities, increasing cyanosis, and cardiac failure. Cardiac catheterisation was performed on the day of admission and he died shortly after the procedure. A rare variant of common arterial trunk, in which the pulmonary arteries arose directly from the underside of the aortic arch, was found at necropsy.
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PMID:A rare variant of common arterial trunk. 381 57

Four male patients with pulmonary arterio-venous fistulae are reported. Familiar occurrence was noticed in 2 brothers. Physiological effects, clinical pictures and treatment of these lesions depend on the size and number of the fistulae. A 4-day-old baby presented with severe cyanosis and cardiomegaly. Cardiac catheterization and angiography demonstrated a large arterio-venous fistula affecting the whole of the right upper lobe and a small ventricular septal defect resulting in a high output cardiac failure. The patient underwent emergency lobectomy with good early and long-term results. The other 3 patients (mean age of 7.6 years) developed progressive cyanosis and polycythemia. Cardiac catheterization showed normal intracardiac pressures and peripheral arterial desaturation. Pulmonary angiogram demonstrated multiple fistulae in both lungs. Surgery was not performed because of the diffuse nature of the lesions. The patients reported in this paper illustrate the wide spectrum of clinico-pathological conditions of this anomaly.
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PMID:The spectrum of pulmonary arterio-venous fistulae. Clinico-pathological correlations. 616 19

Four infants with Down syndrome developed cor pulmonale and heart failure in association with chronic upper airway obstruction. Features of the sleep apnea syndrome were conspicuous; namely, noisy breathing with retraction, cyanosis and frequent apnea during sleep, and daytime lethargy and somnolence. The clinical picture masqueraded as cyanotic congenital heart disease. Arterial blood gas analyses revealed alveolar hypoventilation, especially during sleep. The nature of the obstructive element was variable. Adenoidectomy provided partial relief in one patient, and tonsillectomy and adenoidectomy resulted in temporary improvement in two others. Three patients were markedly benefitted by tracheostomy. Functional inspiratory pharyngeal closure was demonstrated fluorographically in one patient. Infants with Down syndrome may be predisposed to upper airway obstruction by virtue of hypoplasia of facial and oropharyngeal structures and generalized hypotonia. Additional obstructive elements may be contributed by hypertrophied lymphoid tissue, excessive secretions, and glossoptosis. Removal of the obstructive element is helpful, but functional obstruction may only be relieved by tracheostomy.
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PMID:Alveolar hypoventilation and cor pulmonale associated with chronic airway obstruction in infants with Down syndrome. 645 3

Pulmonary arteriovenous malformation is an uncommon cause of cyanosis and heart failure in newborns. A case is presented with review of the literature in order to emphasize its clinical and radiographic features.
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PMID:Pulmonary arteriovenous malformation: an uncommon cause for cyanosis in the newborn. 664 88

Tetralogy of Fallot and absent pulmonary valve (T-APV) is associated with massively dilated pulmonary arteries that cause tracheobronchial compression in the newborn and heart failure and cyanosis in older patients. Corrective operations have been attended by high mortality rates due to pulmonary insufficiency causing right heart failure (RHF) and pulmonary complications. Pulmonic valve insertion (PVI) with complete repair has resulted in improved survival. During the last 5 years, 152 patients with tetralogy were corrected. Of these, 10 patients (ages 51 days to 34 years) had absent pulmonary valve. One patient (age 51 days) presented with severe RHF and pulmonary insufficiency and 9 patients presented with mild RHF and cyanosis. Chest roentgenograms showed increased cardiothoracic ratio and pulmonary prominence in all. Arteriography revealed massively enlarged pulmonary arteries with a mean ratio of 2:1 for size of pulmonary artery to aorta. Associated pulmonic stenosis and insufficiency was present in all. Seven patients underwent closure of ventricular septal defect (VSD) and PVI. Of these, 3 had PVI (2 tissue and 1 prosthetic) with outflow patch and 4 had right ventricle to pulmonary artery (RV-PA) tissue valved conduits. Two patients had repair without PVI, and 1 had repair with a monocusp pericardial valve patch. Nine patients have done well with no episodes of thromboembolism or infection. Death occurred in a 51-day-old infant who had VSD closure and relief of pulmonic stenosis. Pulmonary valve insertion seems to be indicated in these patients because it lowers peak pulmonary artery pressure and, thus, reduces compression effects on the trachea and bronchi. When PVI was used, RHF did not occur post-operatively.
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PMID:Surgical management of tetralogy of Fallot with absent pulmonary valve. 665 71

A patient with pronounced dyspnoea and cyanosis was found to have severe hypoxaemia with normal spirographic values. His past history included arterial hypertension, myocardial infarction and phlebitis of the lower limb. Airways resistance was normal, but maximal expiratory flow rates at low lung volume (Flow-volume curves) were reduced, suggesting "peripheral" airways obstruction. This was confirmed by the presence of pulmonary hyperinflation and mechanical non-homogeneity accompanied by unevenly distributed ventilation, as shown by alveolar nitrogen gradient. There was marked hyperventilation with hypocapnia. Since transfer values (measured by the CO single-breath method) and lung distensibility values were normal, emphysema could be ruled out as a cause of obstruction. Analysis of pressure-flow relationship confirmed that the obstruction of peripheral airways was "intrinsic" in character. It could be due to an increase in lung extravascular fluid (interstitial oedema due to left cardiac failure), or to repeated micro-emboli in the lungs, or to hypocapnia, these three mechanisms possibly being associated.
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PMID:[Peripheral airway obstruction involving cardiovascular factors. A case report (author's transl)]. 677 51

A case of single ventricle with pulmonary stenosis operated at 20 years of age by direct atrio-pulmonary anastamosis is presented. This procedure in contrast to classical techniques does not use an intraventricular patch or a valved ventriculo-pulmonary conduit. It is an adaptation of the Fontan technique initially proposed for tricuspid atresia. The operation consisted of closing the right atrioventricular orifice with a piece of Dacron, suturing the pulmonary valves (which were stenosed) and connecting the right atrium and pulmonary artery by a direct anastamosis using the auricle. The clinical result remains satisfactory one year after surgery: the cyanosis has regressed, the functional tolerance is perfect, there is no hepatomegaly and the patient is in sinus rhythm. The technique is very simple and involves less risk to the His bundle than intraventricular septalisation. It should be reserved to cases of single ventricle with low pulmonary pressures and resistance without cardiac failure. The long-term prognosis depends on the tolerance of the right atrium and is, at present, unknown.
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PMID:[Treatment of single ventricle by direct atriopulmonary anastomosis without tube or valve. Apropos of a case operated on]. 681 Jul 87

Pulmonary vascular structure was analyzed in the lungs of 10 patients with a secundum atrial septal defect (ASD) in whom pulmonary hypertension had developed. Four patients were aged 6 months or less, 5 were aged 2 to 9 years, and 1 was 21 years old. Pulmonary vascular structure was analyzed using lung biopsy tissue in 5 and autopsy material in the other 5. All the infants presented with heart failure and all had a marked increase in pulmonary arterial smooth muscle; only 1 infant survived surgery. Of the 5 older children, 1 presented with cyanosis, but in the rest the ASD was incidental to the presentation. Three patients had severe pulmonary vascular disease, similar to that seen in adults with a hypertensive ASD. Only 2 older children underwent successful surgery. In 1 child and in the 1 adult, the severity of the pulmonary vascular disease precluded surgery. The ASD was closed in 8 patients, but only 3 survived. Pulmonary hypertension develops rarely in secundum ASD in childhood.
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PMID:Pulmonary vascular disease in secundum atrial septal defect in childhood. 682 35

Two new cases of type B congenital interruption of the aortic arch are described. Both had other congenital cardiovascular malformations as well as absence of the thymus and parathyroids. They were newborn male and female, their parents were young and healthy, admitted in our institution because of feeding difficulty, respiratory distress and cyanosis with signs of heart failure. Both were diagnosed at necropsy. According to the site of the interruption of the aortic arch, three types (A, B and C) of absence of the aortic arch are recognized. In our patients as in the majority of reported cases, the interruption of the aortic arch was associated with patent ductus arteriosus, ventricular septal defect and atrial septal defect, and other cardiovascular anomalies. The association of Di George syndrome with cardiovascular anomalies is commented.
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PMID:[Steidele complex (author's transl)]. 708 60

Authors described three infants under three months of age with cardiac rhabdomyoma observed during a period of eight years. There was no clinical or pathological evidence of tuberous sclerosis. During this period of time they found two cases of tuberous sclerosis, without associated rhabdomyoma, among 2,457 necropsies. On the other hand none of 11 children with tuberous sclerosis under observation in this centre had any clinical evidence of rhabdomyoma. Presenting symptoms of the three cases were cyanosis, heart failure, heart murmurs and alterations of heart rhythm. Diagnosis was suspected on the bases of eco and angiocardiographic studies and was finally confirmed by necropsy. Surgical exeresis of the tumor was attempted in one of the cases, but was not possible because it was too large.
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PMID:[Rhabdomyoma of the heart: three case report (author's transl)]. 708 61


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