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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with clinical and radiological features similar to those of Singleton and Merten's patients are described. These patients exhibit features of a unique clinical syndrome of unknown etiology: generalized muscular weakness with secondary hip and foot deformities, progressive calcification of the thoracic aorta beginning in childhood, calcific aortic stenosis leading to heart failure, dysplasia of the teeth, poor physical development, osteoporosis, expanded medullary cavities of the metacarpal and metatarsal bones, and chronic psoriaform skin lesions.
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PMID:A syndrome of widened medullary cavities of bone, aortic calcification, abnormal dentition, and muscular weakness (the Singleton-Merten syndrome). 17 95

Four cases of absent pulmonary valve in combination with ventricular septal defect are reported. In this syndrome hypo- and dysplasia of the pulmonary valve is constantly associated with a big ventricular septal defect, formation of a huge pulmonary artery aneurysm and absence of the ductus arteriosus. Presence or absence of a right ventricular outflow tract obstruction is the criteria for classification into two forms. Absence of the pulmonary valve, right ventricular outflow tract obstruction and a malalignment-type ventricular septal defect produced by a conotruncal malseptation process represent the primary complex of malformations. Consecutive intrauterine cardiac failure is most probably prevented by prenatal closure of the ductus arteriosus. Pulmonary artery aneurysm and also dilatation of the right ventricular outflow tract as well as a whole lot of other coexisting deformities can be explained by a cascade of hemodynamical sequelae started by this ductus closure in utero. An embryological scheme explaining the genesis of this syndrome is derived from a morphological analysis of the constituting incoherent-appearing anatomical features.
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PMID:The syndrome of absent pulmonary valve and ventricular septal defect--anatomical features and embryological implications. 43 24

A case of a child affected by Shwachman's syndrome with a chronic diarrhea, cyclic neutropenia and bone defects is described. The child died at the age of eight months from an acute cardiac failure which showed analytical alterations compatible to an acute myocardial infarction. Pathologic study showed a pancreatic lipomatous hypoplasia, myocardial fibrosis and chondrometaphyseal dysplasia. Main hypothesis that relate pancreatic pathology and development of myocardial fibrosis are discussed, although there is a lack of definitive evidence for confirming such relation.
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PMID:[Myocardial fibrosis in Shwachman's syndrome (author's transl)]. 48 46

Three male sibs from consanguienous parents were found to have a strikingly similar pattern of multiple congenital anomalies. The main features were polyhydramnios; low birthweight; dwarfism; epicanthal folds; abnormal ears; microretrognathia; microstomia; microglossia; glossoptosis; webbed neck; severe cardiac defects; radial and ulnar hypoplasia; radial deviation of the hands; brachymetacarpalia; thumb hypoplasia; clinodactyly and hypoplasia of the 5th finger; simian creases; fibular and tibial hypoplasia; talipes varus with hypoplastic heels; wide gap between 1st and 2nd toes; and delayed bone age. Neonatal death occurred in the 3 babies by severe cardiac failure. Differential diagnosis permits one to conclude that this is a new type of faciocardiomelic dysplasia with a probable autosomal recessive inheritance.
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PMID:Lethal faciocardiomelic dysplasia- a new autosomal recessive disorder. 121 41

We report the cases of eight children with Marfan syndrome. Seven (87.5%) were diagnosed with cardiopathy, everyone with auscultatory findings. Electrocardiographic patterns were nonspecific. Enlargement of the aortic root was present in two of the cases as seen by thorax x-rays. Echocardiography detected the presence of mitral valve prolapse in 87.5% of the patients (7 cases) and aortic enlargement in 75% of the patients (6 cases). Echocardiography also detected the presence of aortic dysplasia, tricuspid valve prolapse and right and left ventricular hypertrophy. During the follow-up period, no case had cardiac failure. There was no mortality. The aortic enlargement was progressive and was not modified by propranolol treatment. Surgical treatment was not needed. Family history related to this condition was present in a very small percentage (37.5%). We comment on one infantile form of Marfan syndrome with its own phenotype different from that of classical Marfan syndrome.
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PMID:[Marfan syndrome in childhood: cardiovascular manifestations. Echocardiographic changes]. 141 16

Eight patients with the middle aortic syndrome are described. They were aged 2 months to 14 years at diagnosis; follow up was one to 11 years. Clinical presentations included asymptomatic hypertension (n = 5), severe headache, nose bleed, and chest pain (n = 1), and cardiac failure (n = 1). All had severe hypertension requiring multiple drug treatment. Diminished peripheral pulses were not helpful in the diagnosis, which is made on aortography. Associated clinical findings were Williams' syndrome (n = 3) and appreciable eosinophilia (n = 3). The differential diagnosis includes Takayasu's arteritis, fibromuscular dysplasia, and neurofibromatosis. Blood pressure was adequately controlled by medical treatment in six patients. Surgical angioplasty was performed in two. One patient remained normotensive without drug treatment 21 months after operation; the other died of sepsis and uncontrollable haemorrhage in the postoperative period. Medical treatment is satisfactory in most cases: surgery should be reserved for those in whom blood pressure cannot be controlled without unacceptable side effects of drug treatment. Although rare, the middle aortic syndrome should be considered in the differential diagnosis of hypertension when commoner causes have been excluded. Aortography is necessary for diagnosis.
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PMID:Middle aortic syndrome: clinical and radiological findings. 158 Jun 80

Thirteen out of 268 children (less than 18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD.
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PMID:Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome). 162 41

The thirty nine patients (30 men and 9 women, mean age 49 +/- 14 years) who were implanted with an automatic cardioverter defibrillator (AICD) between October 1982 and April 1990 were reviewed retrospectively. This group included 22 patients with ischaemic cardiomyopathy (55%), 9 primary cardiomyopathies (23%) 5 ventricular tachycardias in patients with normal hearts (13%), 1 case of arrhythmogenic right ventricular dysplasia and two of mitral valve prolapse. The mean ejection fraction for the whole group was 38 +/- 14%. The indication for implantation of the AICD was life-threatening ventricular arrhythmias for which other forms of treatment were either ineffective (29 patients), impossible to assess (9 patients) or poorly tolerated (1 patient). Two patients died during the perioperative period and the follow-up is too short in two other cases. Therefore, 35 patients were studied over an average of 23.7 +/- 16 months. During follow-up, 5 patients died, 3 of progressive cardiac failure and 2 suddenly, one of whom was waiting for a replacement of an exhausted generator. The AICD never functioned in 40% of patients. In addition, it was impossible to determine for the majority of shocks delivered if they had been triggered by a sustained ventricular arrhythmia. Long term tolerance was generally good but two patients developed cardiac constriction induced by the epicardial patch electrodes. The results of this review confirm the efficacy of AICD in preventing sudden death at the expense of a low operative mortality and with good long term tolerance. Quite a large proportion of AICDs never functioned and the interpretation of the delivered shocks remains questionable.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Implantable automatic defibrillator. Evaluation after 8 years of use]. 176 17

Between September 1955 and January 1990, 94 pediatric patients were managed for renovascular hypertension caused by renal artery occlusive disease. Patients (50 boys and 44 girls) were aged 4 days to 17 years (median age: 7 years). At initial evaluation, 34 patients had symptoms of hypertensive encephalopathy or acute heart failure, 36 had moderate symptoms, and 24 were symptom-free. Sixty-five cases were classified on the basis of clinical, radiological, and histological features, as follows: neurofibromatosis (17), fibromuscular dysplasia (11), diffuse arterial calcified elastopathy (11), renal artery thrombosis (10), Williams syndrome (4), Takayashu disease (3), and miscellaneous diseases (9). In the 29 remaining patients, classification was based only on radiological features: causes included unilateral renal artery stenosis (15), bilateral renal artery stenosis with or without aortic stenosis (11), and miscellaneous disorders (3). Surgical treatment consisted in 47 renal revascularization, procedures (14 aortorenal bypasses, 8 aortorenal reimplantations, 9 anastomoses in the upper mesenteric arterial system, 7 autotransplantations, 4 resection-reanastomosis procedures, and 5 miscellaneous procedures). Renal revascularization failed in 15 cases (32%) (because of thrombosis in 14 cases and dehiscence in one). Residual or recurrent stenosis was seen in 7 arteries, whereas the anatomic result was satisfactory in 25 arteries (53%). Blood pressures returned to normal in 16 of 40 (40%) patients successfully treated by revascularization. An additional 25 patients recovered normal blood pressure values after primary nephrectomy (21), partial nephrectomy (4) or nephrectomy after failed renal revascularization.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Renal artery pathology and its therapeutic indications in the child]. 192 4

A 30-year-old male died suddenly after two years of clinically progressive episodes of recurrent ventricular tachycardia. In spite of recurrent episodes of palpitation, the patient had never shown signs or symptoms of heart failure before death. Post mortem examination revealed massive left ventricular involvement in addition to complete absence of myocardial tissue in the right ventricle, replaced by adipose tissue. This case illustrates that patients with arrhythmogenic right ventricular dysplasia may die of arrhythmia before failure of the left ventricle.
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PMID:Arrhythmogenic right ventricular dysplasia with massive involvement of the left ventricle. 193 37


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