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)

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

The case of a 59-year-old man with corrected transposition of the great arteries (CTGA) is reported. This anomaly is rare in adult patients, uncommon in patients over 50. Most of the cases with CTGA are diagnosed in childhood because of associated cardiac abnormalities. When isolated or complicated by other mild anomalies, CTGA can cause no symptoms until adult life or can even go unrecognized. However, most of these patients present with congestive heart failure when they are 40 or 50 years old. Our patient was admitted to the hospital because of heart failure. Cardiac catheterization and selective angiography demonstrated a CTGA associated with severe pulmonary valve stenosis. The case here reported shows that CTGA even when associated with other cardiac abnormalities, can be a relatively benign lesion, and can be revealed in adult life by signs of cardiac failure.
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PMID:[Corrected transposition of the great vessels associated with severe pulmonary stenosis in a 59-year-old man]. 668 49

Forty-one patients with subaortic stenosis (SAS) and ventricular septal defect (VSD) were identified from the cardiac records of the Hospital for Sick Children, Toronto, Ontario. The diagnosis of an associated SAS was made clinically in only 1 patient, who had findings of left ventricular (LV) hypertrophy with strain on the electrocardiogram. There was a delay of 3.1 years between initial presentation and detection of SAS. The SAS was not diagnosed at initial catheterization in 17 patients and was confirmed at subsequent catheter studies in 8 patients, surgery in 5 and autopsy in 4. Associated defects included coarctation of the aorta in 12 patients, mitral valve abnormalities in 4, and right-sided obstructions, including anomalous right ventricular muscle bundles in 6 patients, tetralogy in 4 and pulmonic stenosis in 1 patient. The mean gradient across the LV outflow tract was 25 mm Hg. Nineteen patients had serial catheters without intervening surgery, and the outflow gradient increased from a mean of 9 to 36 mm Hg. The mechanism of SAS consisted of fibrous diaphragm and fibromuscular obstruction in 31 cases, muscular narrowing in 4, protruding tricuspid valve leaflet in 2, hypertrophic cardiomyopathy in 2, anterolateral twist in 1 patient and redundant tissue tag in 1. Thirty-eight patients had a perimembranous VSD, 19 of whom had an associated so-called aneurysm of the membranous septum; 2 had an infundibular VSD and 1 patient had a central muscular defect. Although the SAS was located below the VSD in 30 cases, the associated heart failure and reduced cardiac output can mask the presence or severity of associated SAS.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Ventricular septal defect and subaortic stenosis: an analysis of 41 patients. 668 70

Pulmonary vascular disease and heart failure are the two major problems in complete transposition of the great arteries (TGA) with increased pulmonary flow. Hypertensive pulmonary vascular disease was observed in patients with complete TGA and increased flow (3.6%) as well as in those without increased pulmonary flow (3.3%). An intact ventricular septum or pulmonic stenosis did not appear to prevent the occurrence of progressive pulmonary vascular disease in all patients. The pulmonary lesion observed in these subjects cannot be explained by chronic severe volume overload alone. Clinically unrecognized pulmonary microthrombi are an additional cause for the development of pulmonary vascular disease in patients with complete TGA. Therefore cyanosis and its complications can be a major factor beside increased pulmonary flow in causing pulmonary vascular changes. Early corrective surgery performed after the age of 3 months is the therapy of choice to avoid progressive pulmonary vascular damage and other thrombo-embolic accidents.
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PMID:[Hypertensive pulmonary vascular disease in complete transposition of the great arteries (author's transl)]. 726 36

Infundibular pulmonic stenosis with intact ventricular septum of primary origin is an uncommon condition. We report 15 such patients (nine males and six females, aged 7-36 years) who had undergone surgical correction for the anomaly during the period between 1975 and 1992. The occurrence of this clinical setting represents 0.19% (15/7826) of all cardiac operations and 0.46% (15/3222) of congenital heart diseases undergoing surgical correction during that period of time. The lesion was of discrete fibromuscular hypertrophy of the infundibulum in all 15 patients. The presenting symptoms of most patients were exertional dyspnea and syncope; however, five patients with severe obstruction were asymptomatic. The peak systolic pressure gradient across the infundibulum ranged from 71 to 230 mmHg. There was only one operative death; the remainder had remained well following the surgery over a mean follow-up period of 35 months. Surgical correction for infundibular pulmonic stenosis is rewarding in the absence of heart failure.
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PMID:Infundibular pulmonic stenosis with intact ventricular septum: a report of 15 surgically corrected patients. 828 34

Four patients with suprarenal coarctation of the abdominal aorta were managed from 1978 to 1993 (mean follow-up 8.75 years). Ages at the time of diagnosis were 2 months, 8 months, 4.5 years, and 15 years, respectively. Three children presented with severe hypertension, two of whom were in congestive heart failure, and the fourth child presented with a cold, ischemic leg. The 8-month-old patient had Williams syndrome (supravalvular aortic and pulmonic stenosis, bilateral renal artery stenosis and celiac artery occlusion, "elfin" facies, and mental retardation) and was treated nonoperatively. After 12 years of follow-up, he was given five medications to control hypertension, cardiac arrhythmias, and heart failure. Three patients with abdominal aortic coarctation were treated operatively and none died. Two patients underwent bypass grafting from the supraceliac aorta to the infrarenal aorta, with bilateral renal artery reconstruction in one. Postoperative arteriograms obtained 1 year or more after operation were normal in both cases. The 2-month-old patient underwent patch aortoplasty, with subsequent reoperation 1.5 years later for recurrent hypertension and heart failure with a bypass graft to the left kidney and removal of an infarcted right kidney. In all three patients, operative repair of the suprarenal aortic coarctation has resulted in long-term control of blood pressure and cardiac and renal function.
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PMID:Coarctation of the abdominal aorta. 852 35

Retinoid-dependent pathways play a central role in regulating cardiac morphogenesis. Recently, we characterized gene-targeted RXR alpha -/- embryos, which display an atrial-like ventricular phenotype with the development of heart failure and lethality at embryonic day 14.5. To quantitate the frequency and complexity of cardiac morphogenic defects, we now use microdissection and scanning electron microscopy to examine 107 wild-type, heterozygous, and homozygous embryos at embryonic day 13.5, 14.5, and 15.5. RXR alpha -/- embryos display complex defects, including ventricular septal, atrioventricular cushion, and conotruncal ridge defects, with double outlet right ventricle, aorticopulmonary window, and persistent truncus arteriosus. In addition, heterozygous RXR alpha embryos display a predisposition for trabecular and papillary muscle defects, ventricular septal defects, conotruncal ridge defects, atrioventricular cushion defects, and pulmonic stenosis. Lastly, we show that the intermediate anatomic phenotype displayed by heterozygous embryos is mirrored in the molecular marker MLC-2a. The intermediate phenotype of RXR alpha heterozygous embryos documents a gene dosage effect for RXR alpha in maintaining normal cardiac morphogenesis. In addition, some defects in RXR alpha mutant mice are phenocopies of human congenital heart defects, thereby suggesting that a relative deficiency in RXR alpha or molecules downstream in its signaling pathway may represent congenital heart disease-susceptibility genes.
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PMID:RXR alpha deficiency confers genetic susceptibility for aortic sac, conotruncal, atrioventricular cushion, and ventricular muscle defects in mice. 882 98

Percutaneous balloon valvuloplasty has been established as an effective treatment for pulmonary valve stenosis (PS) in children and adults. However, there are few reports of the use of this technique in patients with other cardiac anomalies. We report the performance of balloon pulmonary valvuloplasty in a 72-year-old woman with a large atrial septal defect (ASD). This patient had a PS with a right ventricular pressure of 108/18 mmHg and a pulmonary arterial pressure of 42/21 mmHg, corresponding to a 66 mmHg pressure gradient. The ASD measured 32 x 27 mm. Balloon pulmonary valvuloplasty reduced the pressure gradient to 5 mmHg immediately after the procedure and to 2 mmHg 40 days after the procedure. The left-to-right shunt ratio was reduced from 17% before to 12% immediately after and to 36% 40 days after the procedure. In contrast, the right-to-left shunt ratio showed a transient increase from 16% before to 28% immediately after the procedure before decreasing to 11% 40 days after the procedure. The arterial blood oxygen tension was 53 Torr before, 46 Torr immediately after and 55 Torr 40 days after the procedure. The grade of heart failure decreased from New York Heart Association class IV before to class II in the chronic stage. These findings indicated that balloon pulmonary valvuloplasty in a patient with ASD transiently increased the right-to-left shunting associated with a decrease in oxygen tension, but that the change in the chronic stage was slight. In our patient with ASD valvuloplasty was useful in relieving the symptoms associated with elevated right ventricular pressure but could not reduce the hypoxemia caused by right-to-left shunting. In conclusion, valvuloplasty in patients with ASD should be considered as a preoperative treatment with the aim of reducing the risk of surgery and to treat symptoms in patients who refuse to undergo surgery.
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PMID:Effects of balloon pulmonary valvuloplasty on atrial shunting--a patient with pulmonary valve stenosis and a large secundum atrial septal defect. 907 Sep 76

The case records of 151 dogs diagnosed with congenital heart disease were reviewed retrospectively. The most common defect was aortic stenosis, accounting for 35 per cent of all cases, followed by pulmonic stenosis (20 per cent), ventricular septal defect (12 per cent), patent ductus arteriosus (11 per cent), mitral valve dysplasia (8 per cent), tricuspid valve dysplasia (7 per cent), endocardial fibroelastosis (1.9 per cent) and tetralogy of Fallot (0.6 per cent). Fifty-one breeds were represented, with golden retrievers, German shepherd dogs and boxers predominating. No overall sex predilection was obvious. Seventy-five per cent of the dogs were asymptomatic at presentation. The defects most often associated with presenting symptoms, such as dyspnoea, syncope, ascites, failure to grow and depression, were mitral valve dysplasia, atrial septal defect, tricuspid valve dysplasia and endocardial fibroelastosis. The latter presented with the most severe signs of heart failure. In some cases of aortic stenosis and pulmonic stenosis, where the defect could not be accurately visualised with two-dimensional echocardiography, Doppler echocardiographic examination was needed for definitive diagnosis.
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PMID:Retrospective study of congenital heart defects in 151 dogs. 909 39

Congenital cutis laxa is an uncommon disorder of generalized elastolysis. The clinical picture is characterized by inelastic, loose, hanging skin that gives the appearance of premature aging. The disease is inherited most commonly in a severe autosomal recessive form, or as a relatively benign, autosomal dominant form. There is often systemic organ involvement in patients with the autosomal recessive form. Cardiopulmonary abnormalities are common and mainly determine the prognosis and life expectancy. Pulmonary emphysema, cor pulmonale, and right-sided heart failure generally caused by pulmonary disease are often seen in infancy. Various cardiovascular abnormalities including aortic aneurysm, pulmonary artery multiple branch stenosis have been reported in patients with this form of congenital cutis laxa. We report a 10-month-old boy with the autosomal recessive form of congenital cutis laxa who had pulmonary valve stenosis. To the best of our knowledge, this is the first case of this association to be reported in the English language literature. We also emphasize the systemic complications that may be associated with congenital cutis laxa.
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PMID:Autosomal recessive form of congenital cutis laxa: more than the clinical appearance. 1238 97


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